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Int J Colorect Dis (1990) 5:1-5

Coloree|al
Disease

9 Springer-Verlag 1990

Original articles

Compartment syndrome after prolonged surgery with leg supports


D. Bergqvist 1, M. Bohe 1, G. Ekelund t, S. Hellsten 2, H. Jiborn 1, N . H . Persson 1 and R. Takolander 1
Departments of 1Surgery and 2Urology, University of Lund, Malm6 General Hospital, Malta6, Sweden
Accepted: 20 April 1989

Abstract. Compartment syndrome has been reported in a


few cases after prolonged surgery with patients in leg
supports. A recent case in our hospital (57-year-old man
undergoing cystourethrectomy because of cancer) made
us interested in the problem. This case together with six
from the literature are analysed. Moreover, the first 11
cases operated on with a pelvic pouch and ileoanal anastomosis at our department were reviewed. They had been
in the leg support position for a median duration of 6.4
(5.8-8) h. In four of them leg pain and swelling developed within 12 h. Three showed regression within a few
days, one after a week. In one patient with swelling compartment pressure was measured with a transducer
tipped catheter. Intermittently the pressure was up to
50 m m H g . There was an obvious decrease in pressure on
knee bending. Also, in a patient without swelling large
pressure variations were seen but not to critical levels.

Introduction
There are three main causes of compartment syndrome:
too small compartment (e.g. burn injuries), too large volume of compartmental contents (e.g. reperfusion after
ischaemia) and externally applied pressure (e.g. too tight
plaster). When the intracompartmental pressure increases above the perfusion pressure muscular ischaemia
will develop. Irreversible muscular and nerve changes occur within 4 6 h. The muscle necrosis and nerve ischaemia are the prerequisites for late sequelae in the form
of Volkmann's contracture. In cases of extensive muscle
trauma, as originally described after crush injury [1],
myoglobinuria may develop and change the picture into
the severe generalised syndrome rhabdomyolysis [2] with
acute renal failure, metabolic acidosis, hyperkalaemia
and shock. Thus there is a real threat not only to the
extremities but to the patient's life. In 1953 G o r d o n and
N e w m a n [3] described a 35-year-old male patient developing a fatal "lower nephron syndrome" after prolonged lumbar disk surgery in the knee-chest position.

Recently pressure in the anterior tibial muscle during


surgery in that position has been measured as high as
105-240 m m H g [4], and this is far above the critical level
for a compartment syndrome to develop. After the case
described by G o r d o n and Newman there have been a few
reports on compartment syndromes after surgery in the
lithotomy position (Table 1).
The purpose of this report is threefold: to describe a
patient who developed compartment syndrome necessitating fasciotomy after surgery in the lithotomy position,
to report 11 patients with surgery of long duration in the
low lithotomy position and to review the problem based
on data from the literature.

Case report
57-year-old male with a cancer of the urinary bladder was treated
initially with partial cystectomy and subsequently with several
transurethral resections and intravesical chemotherapy. In 1985 the
bladder cancer had progressed and the patient underwent cystourethrectomy after preoperative radiation (20 Gy). Urinary diversion was achieved using an ileal conduit (Bricker). The removal of
the urinary bladder was impaired by a pronounced pelvic fibrosis
and the operation performed with the patient in the lithotomy
position lasted for 8 h. Postoperatively he developed fever (39 ~
and the day after operation he complained of severe pain in both
calves, which were swollen, hard and tender. The clinical picture of
a fully developed compartment syndrome was present and bilateral
fasciotomy was made 21 h postoperatively. The muscles were swollen and oedematous, especially in the anterior compartments. Because of the leg pain he received prolonged epidural analgesia. For
almost a year postoperatively he had bilateral paresthaesia, hyperaesthesia and weakness and sometimes pain, especially in the left
leg. The part of his calf muscles that had been under direct pressure
during surgery became fibrotic.

Prolonged surgery in leg supports


The first 11 patients (8 males, 3 females; median age 28.5 (range
17-45) years) operated on with a pelvic pouch and ileoanal anastomosis at the Department of Surgery have been analysed. One with
a diagnosis of familial adenomatosis coli had a proctocolectomy

2
Table 1. Compartment syndrome after operation with legs in lithotomy position
Author

Sex

Age

Procedure

Duration of
operation

Fasciotomy

Renal
failure

Dialysis

Outcome

Goldberg et al. [8]

52

Urethral stricture
repair

6h

Discharged with
normal creatinine

Khalil [9]

23

Total colectomy

7h

+ bilat
c:a 12 h

3 months hyperaesthesia

Left and Shapiro


[10]

38

Urethroplasty (post
trauma)

6.5 h

+ bilat
c:a 24 h

Skin necrosis

Lydon and Spielman [11]

44

Resection of pelvic
tumour

9h

+ bilat
few hours

Reddy and Kaye


[12]

Ma

65

Radical prostatectomy

+ bilat
several hours

Drop foot

Sehlin et al. [13]

20

Operation a.m. Soave


(Mb Hirschsprung)

7h

+ left side c:a


24 h

Skin transplantation

Present case

57

Cystourethrectomy
(cancer)

8h

+ bilat
21 h

Muscle fibrosis left


leg

(cancer)

10 days

Muscle weakness

a The patient had a right-sided femoropopliteal bypass 6 years previously


b Extreme tenderness, swelling, pain in both legs but no fasciotomy was made

Fig. 1. Standard stirrups

Fig. 2. Allen stirrups. Note that there is no pressure on the calf

and pouch in one stage. All the others had had a previous colectomy
for ulcerative colitis. All operations were carried out by the same
surgical team. The median duration of anaesthesia was 9.2 (7-10.3)
h, the duration of surgery 7.1 (6-8.9) h and the duration of leg support 6.4 (5.8-8) h. For most of the patients our ordinary (Maquet)
stirrups (Fig. 1) and later Allen stirrups (Fig. 2) were used, but the
legs wee probably not correctly positioned as the calf rested on the
stirrup. In seven cases (five males, two females) the postoperative
course was uneventful. In four patients (three males, one female)
either leg developed swelling and pain within about 12 h. In one case
the regression was spontaneous, in two mannitol was given with
total regression within a few days, whereas one patient had a reversible peroneal palsy lasting for about one week. There was no
difference in the duration of leg support or of surgery between those
with and those without symptoms.
In one of the patients with postoperative swelling, compartmental pressure was measured postoperatively (Figs. 3, 4). For pressure
measurement a transducer tipped catheter (Compartmental pressure monitoring kit, Model lt0-4D, Camino Laboratories) was in-

serted under local analgesia in the anterior and superficial posterior


compartments of the right lower leg (the leg with the most pronounced tenseness). There was no bleeding complication in relation
to the catheterisation. The catheters were connected to a monitor
for recording. They were left in place for 2 days and measurements
were made at various intervals. Intermittently the pressure was high
(up to 50 m m Hg), but on clinical grounds fasciotomy was not considered necessary. Moreover, there was an obvious decrease in pressure during knee bending when muscles were relaxed. Aspartate
amino transferase (ASAT) in muscle tissue fluid was 20.5 gkat/l,
alanine amino transferase (ALAT) 4.3 gkat/1 and creatinine kinase
(CK) 1129 Ixkat/l. The serum values of ASAT and ALAT were at
the same time 4.7 and 1.7 Ixkat/1, respectively. Normal serum values
at the laboratory are _<0.7 gkat/1.
In another patient pressure was monitored before, during as
well as after surgery with results shown in Figs. 5 - 7 . Especially
postoperatively large pressure variations were seen. The pressure in
this patient never reached a critical level (see Discussion), and the
patient did not develop any symptoms from the legs.

mrnHi

mmH,

50

50

~0

40

30

20
~o

ooo]!
10 o~
p

Ib'~

O0

"o.

'

'

'

o
I
-o-o" ~o /

%o--o--o.

10

o..................

12

36

24

i/c" - ~

~o__o..d

43

~8

hours

~,

hours

Fig. 3. Pressure in the anterior tibial compartment of a patient with


immediate postoperative swelling. The continuous line represents
compartment preSsure with an extended knee and the dotted line the
pressure at the same point but with the knee in flexion. The effect
of knee bending (muscle relaxation) is clearly seen

Fig. 5. Preoperative compartment pressure in a patient undergoing


pelvic pouch surgery. The continous line represents the anterior
compartment and the dotted line the posterior compartment. Arrow indicates the release from leg support

mmHg
mmHg
5O

50

,o

40

30

30

20
20
10

0 /

'
i

/',o

a'~ ~176
i

12

\,

"

"~

10

"-.20.
,

hours

36

48
o

-10

12

2/+

36

48

Ioi

72

hours

Fig. 4. Postoperative pressure in the superficial posterior compartment of the same patient as in Fig. 3

Fig. 6. Pressure in the anterior tibial compartment of the same


patient as in Fig. 5 from the start of surgery through to 72 h postoperatively

Discussion

those with a compartment syndrome (Table 1) there have


been local sequelae in all, and it is important to note the
high frequency of transient leg symptoms in all 1] patients.
Whilst in the lithotomy position there is a decrease in
perfusion pressue due to limb elevation. If there is in
addition pressure on the calf muscles from the leg brace,
then the risk of a compartment syndrome is higher. Initially there may be a low perfusion pressure in a localised
area resulting in local injury and oedema leading to a

In this paper a serious complication after prolonged


surgery in the modified lithotomy position is described.
Only a few cases have been reported previously (Table 1).
These and the present patients developing pain and
swelling after pelvic pouch construction were relatively
young compared to those operated on for carcinoma.
This may indicate that a critical muscle mass is a prerequisite for the development of the syndrome. Amongst

mmHg

50

t~0

30

10

I2

2#

36

z,8

60

72

hours

Fig. 7. Pressure in the posterior superficial compartment of the


same patient as in Fig. 5 and 6 from the start of surgery through to
72 h postoperatively

generalised compartment syndrome. The popliteal artery


may also be compressed further diminishing distal perfusion. During prolonged surgery there are other factors
which may further increase the risk of a compartment
syndrome including hypovolaemia with a low flow state
because of fluid undersubstitution, and the administration of drugs with a vasoconstructing effect. As we were
not aware of the risk we have not previously paid enough
attention to patient positioning. We now use Allen stirrups where the pressure is mainly on the heel, and it is
usually possible to avoid pressure on the calf muscles.
The pressures in the anterior tibial and superficial
posterior compartments are increased in these patients
with symptoms. However, there were large variations in
pressure and we could not define a set level necessitating
fasciotomy. Pressures of 5 0 - 5 5 m m H g for 4 - 8 h will
cause irreversible muscle damage [4]. It is also clear that
at least when the pressure is within the range seen in this
study, it is sensitive to alterations in position. As long as
there is reserve capacity reflected in a decrease in pressure
during knee flexion the danger of developing a compartment syndrome is probably low. Pressure decreases with
altered leg position may also be of diagnostic value in
identifying those patients with a low risk for the development of a muscular injury.
The diagnosis of a compartment syndrome is made
mainly from patient history and by clinical examination.
The syndrome is easily overlooked and it is important to
remember that normal peripheral pulses do not exclude
the diagnosis.
When available, intracompartmental pressure measurements may be helpful in deciding when to perform a
fasciotomy, but they should not delay treatment. The
advantage of a transducer tipped catheter compared to
a conventional fluid-filled system is that the position of
the measuring point can be moved without influencing the zero setting. A continuous pressure exceeding

3 0 - 4 0 m m H g with no decrease following knee flexion


indicates the need for fasciotomy. Even the suspicion of
compartment syndrome is enough to warrant exploratory subcutaneous fasciotomy. There is no indication
for angiography in the diagnosis of compartment syndromes.
Although the precise time limit for legs placed in the
lithotomy position is not known, any prolongation of the
operation should be avoided. Care must be taken not to
compress the popliteal artery during surgery. Leg braces
constructed to minimise pressure should be used, and the
patient must be properly positioned. Postoperatively the
patient must be closely observed and examined at frequent intervals with careful documentation, especially
when epidural analgesia is used as it can mask pain. In
patients with some swelling and pain but without neurological symptoms we have seen rapid relief of symptoms
after the infusion of mannitol ( 3 0 - 6 0 g). Mannitol has
two possible beneficial effects: it induces an osmotic diuresis and acts as a superoxide radical scavenger [5-7].
When there is a suspicion of compartment syndrome
compression bandages are contraindicated. In cases of an
established compartment syndrome elevation of the legs
should be avoided as it decreases the arterial perfusion
pressure even more.
In cases of an established compartment syndrome the
treatment is prompt fasciotomy. All four compartments
must be opened and when the muscles are under tension
the skin must be opened as well. Obvious necrotic tissue
is removed, but judging muscle viability is initially difficult and therefore it is reasonable to be conservative and
to make subsequent revisions as necessary. Moist dressings are used since after a fulminant compartment syndrome there might be a significant plasma leakage. The
skin incisions are preferably closed after a few days, with
or without skin grafts.
If a fasciotomy is performed for the clinical suspicion
of a compartment syndrome, and the muscles are found
not to be under pressure, a subcutaneous fasciotomy of
the suspected compartments is enough.
Where myoglobinuria is suspected a high urinary output and alkalinising the urine may decrease any nephrotoxic effect. When there is rhabdomyolysis with myoglobinuria and renal failure, temporary dialysis may be
necessary.
Finally, we would emphasise that prevention is most
important. The choice of leg stirrups is important, and a
careful check of the position should be made before
surgery starts.

Acknowledgement. The study was supported by a grant from the


Swedish Medical Research Council (No. 00759).

References
1. Bywaters EGL, Beall D (1941) Crush injuries with impairment
of renal function. Br Med J 1: 427
2. Gabow APA, Kaehny WD, Kelleher SP (1982) The spectrum of
rhabdomyolysis. Medicine 61: 141 - 152
3. G o r d o n BS, Newman W (1953) Lower nephron syndrome
following prolonged knee-chest position. J Bone Joint Surg
35-A: 764-768

4. Mubarak SJ, Hargens AR (1981) Acute compartment syndromes. Surg Clin North Am 63:539 565
5. Buchbinder D, Karmody AM, Leather RP, Shah DM (1981)
Hypertonic mannitol. Its use in the prevention of revascularization syndrome after acute arterial ischemia. Arch Surg 114:
414 421
6. Hutton M, Rhodes RS, Chapman G (1982) The lowering of
postischemic compartment pressures with mannitol. J Surg Res
32:239-242
7. Shah DM, Powers SR, Stratton HH, Newell JC (1981) Effects
of hypertonic mannitol on oxygen utilisation in canine hind
limbs following shock. J Surg Res 30:593-601
8. Goldberg M, Stecker JF, Scarff JE, Wombolt DG (1980) Rhabdomyolysis associated with urethral stricture repair: report of a
case. J Urol 124:730-731
9. Khalil IM (1987) Bilateral compartment syndrome after prolonged surgery in the lithotomy position. J Vasc Surg 5:879-881
10. Left RG, Shapiro SR (1979) Lower extremity complications of
the lithotomy position: prevention and management. J Urol
122:138-139

1t. Lydon JC, Spielman FJ (1984) Bilateral compartment syndrome following prolonged surgery in the lithotomy position.
Anesthesiology 60:236-238
12. Reddy PK, Kaye KW (1984) Deep posterior compartmental
syndrome: a serious complication of the lithotomy position. J
Urol 132:144-145
13. Sehlin J, Dolk A, Holmstrom B, Netz P (1985) Patienten tick
compartment-syndrom efter Mb Hirschsprung-operation (in
Swedish). L/ikartidn 82:4323-4324
D. Bergqvist, M.D., Ass. Prof.
Department of Surgery
General Hospital
S-214 01 Malm6
Sweden

Int J Colorect Dis (1990) 5:6-11

Col6recial
Disease

9 Springer-Verlag 1990

Perineal colostomy and electrostimulated gracilis "neosphincter"


after abdomino-perineal resection of the colon and anorectum:
a surgical experience and follow-up study in 47 cases
E. Cavina, M. Seccia, G. Evangelista, M. Chiarugi, P. Buccianti, A. Tortora and A. Chirico
Emergency Surgery, Department of Surgery, University of Pisa, Pisa, Italy
Accepted: 3 July 1989

Abstract. A series o f 47 patients u n d e r g o i n g a b d o m i n o perineal resection o f the distal colon and a n o r e c t u m and
c o n s t r u c t i o n o f a continent perineal c o l o s t o m y using
electrostimulated gracilis muscle is described. External
and implanted pulse generators have b o t h been used. A n
analysis o f complications and oncological data are reported. There was no operative mortality. The incidence
o f complications, divided into three classes, mild (62%),
m o d e r a t e (27%) and severe (11%), has not significantly
altered the functional results, with the exception o f early
ischaemia o f the colonic stump in two cases. D u r i n g the
first 22 cases, no preoperative oncological staging was
performed. In the last 23 patients endorectal ultrasonogr a p h y and C T scanning were carried out. F u n c t i o n a l results were evaluated by e l e c t r o m a n o m e t r y , electromyostimulation and d y n a m i c defaecography. Clinical d a t a
assessed postoperatively showed g o o d function in 65 % o f
cases, fair in 22.5% and p o o r in 12.5%. The quality o f life
in 15 patients with a perineal c o l o s t o m y and electrostimulated gracilis was significantly better than in 15 patients
having an a b d o m i n o - p e r i n e a l resection w i t h o u t gracilis
plastic reconstruction.

Introduction
The possibility o f restoring faecal continence after abdomino-perineal resection by a perineal c o l o s t o m y with a
reconstructed " a n a l " sphincter has been investigated
sporadically in the past [1-4]. M o r e recently it has been
d e m o n s t r a t e d that electromyostimulation (EMS) is able
to m a i n t a i n the t r o p h i s m o f transposed muscle and also
to establish a different physiological functional behaviour
[5]. T h r o u g h pilot studies [ 6 - 8 ] we have standardized a
technique for graciloplasty with E M S and have f o u n d
this to be useful in establishing continence o f a perineal
c o l o s t o m y after abdominal-perineal resection. The technique has been applied to 47 cases operated in our clinic
since 1985. In a previous publication [9] we described the
surgical technique a n d E M S details as well as the post-

operative course and results o f 32 cases having this operation.


In this study we report the increased experience
gained in a total n u m b e r o f 47 patients. We have in the
m o r e recent cases implanted fixed pulse generators m o d ulated externally aimed to apply trophic E M S and provide bio-feedback. The features o f this new procedure
will be described.

Patients and methods


Between January 1985 and March 1989, 47 patients were treated.
Postoperative investigations included electromyography of the transposed muscles, electromanometry and defaecography. Manometry
was performed using an open-sided catheter connected to a Statham
Gould P231D pressure transducer.
The operation is carried out by two surgical teams with a total
operating time averaging about 3 h. The first carries out the abdominal procedure including a radical rectal resection and the preparation of the distal colon to be brought down as the perineal colostomy. Of particular importance is the need to ensure adequate colonic
vascularization. Simultaneously the second team mobilizes both
gracilis muscles as described by Pickrell [10] preserving the vascular
and nerve supply (Fig. 1 c, d). The electromyostimulation electrodes
are inserted into these pedicles. After resection of the distal colon,
rectum and anus, the neo-sphincter is constructed (Fig. 1 d). One of
the gracilis muscles is applied "sling-like" in a retrocolic position,
the other is looped around the end of the sub-terminal colon. The
first muscle is placed to simulate as closely as possible the physiological function of the puborectalis, to maintain an adequate angle of
the colon at the level of the excised pelvic floor. The second gracilis
muscle constricts the terminal colon to simulate the external anal
sphincter. It is important to create an ample tunnel from the thigh
to the perineum for the passage of each gracilis and to anchor them
to the remaining edges of the stumps of the levators by sutures
(Fig. 2 a, b).
The final stage is to perform the perineal colostomy (Fig. 3).
The EMS electrodes are fixed to the nerves to the gracili and
brought out onto the skin of the thigh or abdominal wall for connection to an external stimulator. Subsequently when the electrodes
have been removed, an endoluminal probe can be used for EMS.
Recently an implantable pulse generator has been utilized. A temporary right abdominal colostomy is routinely performed.

'if

Fig. 1. a, b Mobilizing and tunnelling the gracilis sling around the colon, c, d Mobilizing and tunnelling the gracilis to encircle the colon

ze,~., k_ ,,~

Fig. 2. a, b Identification and resection of the levator ani muscles during the perineal resection. Suture of the gracilis to the levator muscles.
e, d Implantation of the pulse generator

8
Table 1. Preoperative patient details
Patients

Mean age

Dukes:

LL

A
Rectal cancer
Other

45
2

33
l

12
1

62.6
40.5

I (2%)
.

47

34

13

61.7

31 (69%)
.

12 (27%)
.

1 (2%)

LL = lymphocytic lymphoma

Table 2. Follow-up (47 patients)


Patients
(n = 47)
Withdrawn (ischaemia
of colonic stump)
Available for follow-up

Right colostomy present,


follow-up incomplete
In early training with EMS,
follow-up incomplete

--

Complete follow-up

r\

Rectal cancer Other


(n = 45)
(n = 2)

45

43

40

39

100.0%

80.0%

60.0%
%

Survival

..................

n ........................................ u

40.0%

20.0%

---~-

Dukes'

*
.--m
0.0%

9
0

b
Fig. 3. a Gracilis encircling the colon, b The completed perineal
colostomy

Electromyostimulation ( E M S )
EMS was carried out postoperatively in hospital and after discharge
by providing patients with a portable pulse generator for a period
of about 3 months. Dislocation of the electrode was the major
obstacle to longer myostimulation times. For this reason we used a
subcutaneous unipolar pulse generator (PG) implanted in a subcutaneous pocket of the left abdominal wall in the last seven patients.
This stimulator was connected, via a tunnelled wire, to a platinum
electrode positioned and anchored beside the nerve. The effectiveness of the EMS apparatus was tested in all cases intraoperatively.
Electrostimulation was begun on the third postoperative day.
All pacemaker functions were programmed telemetrically by use of
a portable remote control console to produce a pulse amplitude of
0.5-10.5 volt, at a rate of 50 Hz, pulse width of 270 s, cycle on-time

........
,
6

A+B

Overall

Dukes'
,
12

C
,
18

,
24

,
30

36

Months

Fig. 4. Cumulative survival rates for Dukes' A + B and Dukes' C


carcinomas (43 patients with " M + M d P " operation)

of 64 s and a cycle off-time of 10 min. Utilizing the above parameters, a battery life of at least 48 months could be expected. All patients tolerated EMS well with no discomfort. No complications
developed at the implantation site nor was there any PG displacement.
Function was assessed postoperatively at regular intervals by the
same investigator. Continence was recorded based on parameters
previously standardized by Corman [11] as excellent, good, fair and
poor. This was simplified to three categories of good, fair and poor
by grouping "excellent" and "good" of Corman together.
A study was conducted on the psychological state of 15 patients
who underwent neosphincter constructive surgery as compared with
J 5 patients having had standard abdomino-perineal resection. The
investigation was conducted by psychiatrists using tests specifically
designed to measure as far as possible the quality of life. The SAD

Table 3. Cancer specific outcome in 43 patients with rectal cancer


Patients

At discharge
Deaths from cancer
Deaths from other causes
Actually "at risk"

43
8
2
33

Operative
death

Distant
recurrence

0
0

8
8

Local
recurrence

Dukes' stage

LL

29

12

26

LL = lymphocytic lymphoma

Table

4. Complications of the perineal phase


Severity
+++

2
1

Stricture
Stricture and fistula
Local sepsis and stricture
Perineal fistula
Mucosal prolapse
Sepsis and perineal stump dislocation
Perineocoele and mucosal prolapse
Perineocoele

2
1
6
2
1

16

Clinical
outcome

Functional
failure

Converted to abdominal colostomy


Stump resection, perineal
reconstruction
Dilatation, plastic reconstruction
Dilatation, plastic reconstruction
Drainage, dilatation
Drainage
Mucosectomy
Drainage, perineal plastomy
Perineal reconstruction mucosectomy
Perineal reconstruction

Good
Good

2 stump
Not eval. a

Ischaemia of colon
Prolapse of colonic stump

1
1
1

Treatment

Good
Good
Good
Healed
Healed
Healed
Healed
Good

4.5% b

" Right colostomy still present; incomplete functional evaluation


b Three of the four patients, waiting for a right colostomy closure, are still to be evaluated. In all of these, the gracilis contraction is good

scale (Scala per Autovalutazione della Depressione) [12] with 31


items rating the degree of depression and anxiety, and the SAS
(Social Adjustment Scale) [13] with 51 items measuring the level and
quality of social adaptation were used.

Results

Basic p a t i e n t details are s h o w n in Tables 1 a n d 2. Deaths


due to d i s t a n t a n d local recurrence are given in Table 3.
The c u m u l a t i v e percentage of survivors at 36 m o n t h s was
70.5% while c o m p a r i s o n between the patients with a
D u k e s ' A + B a n d D u k e s ' C stage shows a highly signific a n t difference (Fig. 4).
C o m p l i c a t i o n s o f b o t h the perineal a n d a b d o m i n a l
phase are given in Tables 4 a n d 5. C o n t i n e n c e was good
in 26 (65%) o f patients. Five (13%) were i n c o n t i n e n t of
b o t h liquid a n d solid faeces (Table 6). There was n o difference in the o u t c o m e w h e t h e r the p a t i e n t h a d h a d local
c o m p l i c a t i o n s or not.
N o n e u r o m u s c u l a r d a m a g e was f o u n d postoperatively by E M G in a n y o f the patients. The e l e c t r o m a n o m e t r y
showed the area of highest resting pressure 2 - 3 c m from
the colo-perineal m u c o - c u t a n e o u s j u n c t i o n was less t h a n
10 m m H g in 20 cases, b e t w e e n 10 a n d 15 m m H g in 10
cases a n d b e t w e e n 15 a n d 20 m m Hg in 8 cases. Maxi-

Table 5. Complications of the abdominal phase


Complication

Early

Late

Treatment

Intestinal obstruction
Colostomy hernia
Iliac phlebothrombosis
Systemic candidosis
Enteritis
Tibial nerve compressive damage

1
1
1
1
1

2
2
-

Surg.
Surg.
Med.
Med.
Med.
Med.

m u m v o l u n t a r y c o n t r a c t i o n s (MVC) p r o d u c e d pressures
between 40 a n d 80 m m H g in one third of cases a n d
a b o v e 80 m m H g in the rest. I n 12 patients the v o l u n t a r y
c o n t r a c t i o n s exceeded 150 m m Hg.
D e f a e c o g r a p h y clearly showed the phases of evacuation a n d d e m o n s t r a t e d the n e o s p h i n c t e r a p p a r a t u s anchored to the levator muscles (Fig. 5).
The overall psychological score of patients with a n
a b d o m i n a l c o l o s t o m y (15 cases) as c o m p a r e d with patients h a v i n g a perineal c o l o s t o m y with a n e o - s p h i n c t e r
(15 cases) indicates t h a t the latter have a better quality of
life (Table 7).

10

Fig. 5. Defaecography. Left: complete


arrest of barium due to neo-sphincter
contraction; right: voluntary evacuation

Table 6. Continence in 40 patients

Good

Fair

Poor

Free of local complication


Local complication

8
18

4
5

2
3

Total n (%)

26 (65%)

9 (22%)

5 (13%)

Table 7. SAD scale (15 Miles vs 15 M+MdP)

SAD factors

Depressive mood
Psychic and
somatic anxiety
Somatic symptoms
Diurnal variations
Suicidal tendencies
Total

M + mP

Miles

1.3
1.3

0.3
0.3

1.7
1.6

0.6
0.5

-2.4
-1.9

0.02
0.06

1.2
1.1
1.0

0.2
0.3
0.0

1.3
1.5
1.1

0.3
0.7
0.2

-1.6
-1.9
-1.9

0.12
0.07
0.03

38.2

6.1

46.2 12.9

-2.2

0.03

M = median of results; s = standard deviation; T = T test value

Discussion

The technique described appears to be satisfactory in the


positioning of the muscles transposed to the perineum.
The most crucial surgical factor is the vascular supply of
the colon brought down to the perineum. Local complications of suppuration and necrosis were a consequence
of ischaemia in this segment. The technique of EMS appeared to maintain and augment the trophism of muscles.
The electrodes brought externally through the skin of the
thigh are necessarily temporary. In the case of their removal it is preferable for EMS to be performed with
endoluminal electrodes. The installation procedure of the
subcutaneous pacemaker does not present any particular
difficulties and the programmable regulation of the electric stimulus appears to be functionally promising. The
use of this device is easy for the patient. Experience with
this method, only very recently acquired, is limited however and full evaluation must await further experience. A

bipolar pacemaker for use in two muscles is now available and is ready for implantation.
A m o n g late complications, stenosis is easily correctable, as is mucosal prolapse. The latter, when it occurs
may cause nocturnal soiling. More difficult to correct is
a true perineocoele which occurred in three patients, especially in those with a poor muscle tone or with a tendency to obesity. Special attention may be required for
the pelvic-sacral fixation o f the lowered colic stump in
subjects who are at risk. We are expecting improvement
in EMS techniques using permanent pacemakers.
Function was satisfactory in the majority of patients.
Only 13 % were incontinent to both liquid and solid stool.
This was despite low resting pressures in all cases. Presumably voluntary contraction which was satisfactory
was sufficient to maintain continence in most cases. The
requirement of this technique was that a radical resection
based on accepted oncological criteria should be the priority. In the event cancer specific survival was most satisfactory with only one case of local recurrence.
There are few reports of the use of an implanted muscle stimulator as an essential component in muscle transposition for incontinence [5]. Our results now extending
over 4 years suggest that this method may be applied
more widely in the management of perineal colostomy by
sphincteric reconstruction following anorectal excision.
The technique offers a satisfactory quality of life in
the majority of patients, being somewhat better in cases
undergoing chronic muscle stimulation. Acceptable continence is likely without any sacrifice of cancer specific
survival. Despite a moderate complication rate, function
is ultimately satisfactory in most patients.

References

1. Chittenden AS (1930) Reconstruction of anal sphincter by muscle slips from the glutei. Ann Surg 92:152
2. Toupet A (1978) L'op6ration de Pickrell modifi6e. Chirurgie
104:367-368
3. Beche' M (1952) Continence apr6s amputation abdominoperineale du rectum. Marseille Chir 4:333
4. Shafik A (1986) A new concept of the anatomy of the anal
sphincter mechanism and the physiology of defecation. Reversion to normal defecation after combined excision operation
and end colostomy for rectal cancer. Am J Surg 151:278-284

11
5. Baeten C, Spaans F, Fluks A (1988) An implanted neuromuscular stimulator for fecal continence following previously implanted gracilis muscle: report of a case. Dis Col Rectum 31:
134 137
6. Cavina E, Seccia M, Evangelista G (1982) Neosphincter and
neostomy. New surgical views for myoelectric stimulated continence. Preliminary report. Am J Proctol Gastroenterol Colon
Rectum Surg 33:16
7. Cavina E, Seccia M, Chiarugi M, Evangelista G, Buccianti P,
Chirico A, Rossi B, Sartucci F (1985) Continenza di colostomia
perineale dopo operazione di Miles: neosfintere elettrostimolato. Boll Soc It Chir 6:3
8. Cavina E, Seccia M, Evangelista G (1981) Neosfintere e neostoma. Nuove tecniche chirurgiche in funzione delle prospettive
di elettrostimolazione per la continenza. Min Chir 36:389
9. Cavina E, Seccia M, Evangelista G, Chiarugi M, Buccianti P,
Chirico A, Lenzi M, Bortolotti P, Bellomini G, Arganini M,
Pieri L (1987) Construction of a continent perineal colostomy
by using electrostimulated gracilis muscles after abdominoperineal resection: personal technique and experience with 32 cases.
It J Surg Sci 17:305-314

10. Pickrell K, Masters F, Georgiade N, Horton C (1954) Rectal


sphincter reconstruction using gracilis muscle transplant. Plast
Reconstr Surg 13:46
11. Corman ML (1985) Gracilis muscle transposition for anal incontinence: late results. Br J Surg 72 [Suppl]: 21-22
12. Cassano GB, Castrogiovanni P, Ghiozzi M, Principe S (1977)
Rilevazione standard dei dati socio-ambientali mediante un
questionario di autovalutazione. Atti XXXIII Congresso
Nazionale della Societfi Italiana di Psichiatria. Napoli 1:289
13. Schooler NR, Hogarty GE, Weissman MM (1979) Social Adjustment Scale. In: Hargreaves WA et al (eds) Resource material
for community mental health program evaluation. A.D.M. 79,
328, 291 (Printing Authors, Washington D.C.)
Prof. Enrico Cavina
Emergency Surgery
Department of Surgery
University of Pisa
1-56010 Pontasserchio, Pisa
Italy

Int J Colorect Dis (1990) 5:12-14

Col6reeial
Disease

9 Springer-Verlag 1990

Conservative treatment of low rectovaginal fistula in Crohn's disease


Y. Francois, L. Descos and J. Vignal
Department of Surgery, HSpital Claude Bernard, Centre Hospitalier Lyon-Sud, France
Accepted: 15 September 1989

Abstract. A c o n s e r v a t i v e o p e r a t i v e t r e a t m e n t o f a n o v a g i n a l fistula in C r o h n ' s disease is described. This consists o f


s i m p l y l a y i n g o p e n the fistula w i t h section o f the rectov a g i n a l s e p t u m a n d the p o r t i o n o f e x t e r n a l s p h i n c t e r superficial to the fistula. T h e o p e r a t i o n m a y be p e r f o r m e d
in the presence o f rectal i n v o l v e m e n t even d u r i n g a n a c u t e
e x a c e r b a t i o n o f the disease; a t e m p o r a r y d e f u n c t i o n i n g
s t o m a is n o t r e q u i r e d . T h e fistula was o f the h i g h
t r a n s s p h i n c t e r i c t y p e in t h r e e p a t i e n t s a n d low
t r a n s s p h i n c t e r i c in six. A l l w o u n d s h e a l e d in less t h a n 3
m o n t h s w i t h o u t a n y f u r t h e r surgery. A t a m e a n f o l l o w - u p
o f 29 m o n t h s , 6 h a d perfect c o n t i n e n c e a n d 3 c o u l d c o n t r o l solid b u t n o t liquid stools n o r flatus.

Introduction
A n o v a g i n a l fistula is the c o m m o n e s t f o r m o f f i s t u l a t i o n
f r o m the g u t t u b e to the female genital t r a c t in C r o h n ' s
disease. T h e i r low l o c a t i o n allows a c o n s e r v a t i v e o p e r a tive t r e a t m e n t w i t h g o o d result. We r e p o r t o u r experience
w i t h nine such cases.

"vulvar fourchette" in eight patients and 1 cm higher in one patient.


The anal opening was at the level of the dentate line anteriorly. All
the fistulae were transsphincteric. In six the primary track traversed
the external sphincter in its lower half whereas in the three other
patients the track was high transsphincteric. Three patients were
found to have a secondary track. Colonoscopy revealed the presence of Crohn's disease in three patients not previously known to be
affected. Eight cases had rectal involvement. In two instances, the
operation was done during an acute exacerbation of the intestinal
disease.

Surgical procedure
All patients were taking steroids and metronidazole. The two patients operated upon during an acute exacerbation also received
parenteral hyperalimentation for 15 days post-operatively.
Under general anaesthesia or spinal anaesthesia the patient was
placed into the lithotomy position and the track probed from the
internal opening to the vagina. The track was then laid open as a
medial perineotomy with section of the rectovaginal septum and
external sphincter superficial to the fistula. Great care was taken to
excise all secondary tracks. A temporary defunctioning stoma was
not felt to be necessary in any of the patients.

Results (Table 1)

Patients
Nine women with a mean age of 33 years (range 20 to 54 years)
presented in our unit with an anovaginal fistula between 1983 and
1988. Six had known Crohn's disease but in three the fistula presented as the first manifestation. The condition had been previously
misdiagnosed as "pseudo Bartholin abscess" in eight cases and
haemorrhoids in one. All patients were incontinent to flatus. One
had mild faecal anal incontinence during an exacerbation of
Crohn's disease associated with microrectum, and the other eight
patients complained of soiling per vagina on defaecation when the
stool was loose. Three patients had had previous surgery for
anoperineal lesions, two had had a total qolectomy with ileorectal
anastomosis and one a right hemicolectomy. One patient had had
a rectovaginal fistula repaired 4 years previously.
In eight cases the fistula was diagnosed on clinical examination
and one was recognized during operation. Their location was always very low with the vaginal opening lying at the level of the

H e a l i n g o f the w o u n d o c c u r r e d in less t h a n 3 m o n t h s
w i t h o u t a n y surgical revision. We never o b s e r v e d a rectov a g i n a l k e y h o l e d e f o r m i t y c o n s e q u e n t to the l a y i n g open,
b e c a u s e the s c a r r i n g achieved a t r a n s v e r s e b a n d o f fib r o u s tissue (Figs. 1 - 3 ) f o r m i n g a p r o j e c t i o n o f the
anovaginal septum.
T h e p a t i e n t s were f o l l o w e d for a m e a n o f 29 m o n t h s
(range 3 to 64 m o n t h s ) . Six p a t i e n t s h a d perfect continence a n d t h r e e h a d c o n t r o l o f solid stool b u t were i n c o n t i n e n t to liquid stool a n d flatus. Two o f the three p a t i e n t s
with d i s t u r b a n c e o f c o n t i n e n c e h a d h a d a c o l e c t o m y w i t h
ileorectal a n a s t o m o s i s , one o f w h o m h a d a m i c r o r e c t u m ,
a n d the t h i r d p a t i e n t h a d h a d a right h e m i c o l e c t o m y .

13
Table 1. Patient population

Age Duration of
disease
(months)

Location of the
trans-sphincteric
fistulae

Secondary Distribution of disease


track
Small Colon
Rectum
bowel

Follow up
(months)

Patient ]
Patient 2

50
32

0
60

High
Low

+
0

+
+

Patient 3

27

72

High

Patient 4
Patient 5
Patient 6

54
22
25

0
84
96

Low
Low
Low

0
0
+

0
+
+

Patient 7
Patient 8
Patient 9

27
20
40

48
3
0

High
Low
Low

0
0
0

0
0
0

Fig. 1. Patient 1: Complex anovulvar and anal fistula


Fig. 2. Patient 1: Laying open of the anovulvar fistula, of the sec- ;
ondary track, and of the associated anal fistulae
Fig. 3. Patient 1:(9 months later) Transverse indolent scar between
vagina and rectum. Perfect continence

Discussion

Rectovaginal fistula is an u n c o m m o n complication o f


C r o h n ' s disease occurring in 5 to 15% o f affected w o m e n
[1-3]. M o s t c o m m o n l y they are low transsphincteric
anovaginal [3-6], resulting f r o m infection originating in
the anal crypt glands [1, 3]. Less c o m m o n l y they m a y be
rectovaginal extrasphincteric secondary to transmural inf l a m m a t i o n o f the rectum, resulting in a cavitating ulcer
leading to fistula f o r m a t i o n [3, 7].
Medical treatment is aimed at minimising s y m p t o m s
and avoiding surgery [3]. M e t r o n i d a z o l e m a y have a ben-

+
0
Total
+
colectomy
Total
+
colectomy
+
+
+
+
Right hemi- +
colectomy
+
+
+
+
+
+

Continence
Solid
stool

Liquid Flatus
stool

54
26

+
+

+
0

+
0

24

64
60
9

+
+
+

+
+
_+

+
+
0

10
3
9

+
+
+

+
+
+

+
+
+

eficial effect on healing o f the anoperineal lesions and


parenteral hyperalimentation can be useful when patients
are operated on during an acute exacerbation as in two o f
our cases.
True rectovaginal fistula. Local repair o f the fistula,
regardless o f the surgical technique and whether or n o t a
defunctioning s t o m a is employed, is associated with a
healing rate o f less than 50% [4 6] and m a y be undertaken only if the fistula is extrasphincteric. In the latter
instance, e n d o a n a l a d v a n c e m e n t is the m e t h o d preferred
by Radcliffe et al. [3]. M o r e o v e r , local repair is possible
only when the rectum is relatively healthy and the intestinal disease in remission [3, 4, 6]. Faecal diversion is often
necessary [6 8].
Neither intestinal resection n o r faecal diversion [3, 6,
8, 9] results in complete healing o f rectovaginal fistula.
P r o c t e c t o m y is the m o s t c o m m o n l y used procedure [8, 10,
11]. However, the resulting disability, particularly in
y o u n g w o m e n , should be avoided for as long a time as
possible. In the series reported f r o m St. M a r k ' s Hospital

14
[3], p r o c t e c t o m y was c a r r i e d o u t in 48 o f 90 w o m e n w i t h
r e c t o v a g i n a l fistula d u e to C r o h n ' s disease, b u t the fistula
itself w a s the m a j o r f a c t o r in the d e c i s i o n to o p e r a t e in
o n l y 10 patients.
A n o v a g i n a l fistula. L a y i n g o p e n o f the fistula (or first
stage o f M u s s e t ' s o p e r a t i o n [12]), as u s e d in this g r o u p o f
p a t i e n t s , is a v e r y simple a n d efficacious solution. It allows the excision o f all f i b r o t i c r a m i f i c a t i o n s o f the fistulous t r a c k s a n d g o o d d r a i n a g e . T h e o p e r a t i o n d o e s n o t
result in i n c o n t i n e n c e o f solid stool, a l t h o u g h s o m e p a tients h a v e difficulty with liquid stool [3, 12]. Thus, a
t e m p o r a r y d e f u n c t i o n i n g s t o m a [3, 12] o r a s t a g e d r e c o n s t r u c t i o n , either d u r i n g the s a m e o p e r a t i o n [4] or at a
later d a t e [12], are u n n e c e s s a r y [3]. T h e o p e r a t i o n can be
p e r f o r m e d d u r i n g an a c u t e f l a r e - u p o f C r o h n ' s disease
a n d even in the presence o f rectal i n v o l v e m e n t . D e s p i t e
the s h o r t f o l l o w u p , the results in this series s h o w the
effectiveness o f this m e t h o d . O t h e r s h a v e h a d a similar
experience. Thus, R a d c l i f f e et al. [3] r e p o r t e d a successful
o u t c o m e in 12 o u t o f 16 p a t i e n t s t r e a t e d while M u s s e t [12]
m e t w i t h success in 4 o u t o f 4 p a t i e n t s .

3, Radcliffe AG, Ritchie JK, Hawley PR, Lennard-Jones JE,


Northover JMA (1988) Anovaginal and rectovaginal fistulas in
Crohn's disease. Dis Colon Rectum 31:94 99
4. Bandy LC, Addison A, Parker RT (1983) Surgical management
of rectovaginal fistulas in Crohn's disease. Am J Obstet Gynecol 147:359 363
5. Faulconer HT, Muldoon JP (1975) Rectovaginal fistula in patients with colitis. Dis Colon Rectum 18:413-415
6. Tuxen PA, Castro AF (1979) Rectovaginal fistula in Crohn's
disease. Dis Colon Rectum 22:58-62
7. Hughes LE (1978) Surgical pathology and management of
anorectal Crohn's disease. J R Soc Med 71: 644-65i
8. Givel JC, Hawker P, Allan RN, Alexander-Williams J (1982)
Enterovaginal fistulas associated with Crohn's disease. Surg
Gynecol Obstet 155:494-496
9. Macilrath DC (1971) Diverting ileostomy or colostomy in the
management of Crohn's disease of the colon. Arch Surg
103:308-310
10. Wolff BG (1984) Surgical treatment of Crohn's disease. Probl
General Surg i: 51 - 59
11. Marks CG, Ritchie JK, Lockhart-Mummery HE (1981) Anal
fistulas in Crohn's disease. Br J Surg 68:525-527
12. Musset R (1979) Mort exp6rience du traitement des fistules
rectovaginales des deux tiers inf4rieurs du vagin ni radiques, ni
nhoptasiques. Ann Gastroenterol Hepatol 15:427 436

References
I. Crohn BB, Yarnis H (1958) Regional enteritis, 2nd edn. Grune
and Stratton, New York
2. Hudson GN (1970) Aquired fistulae between the intestine and
the vagina. Ann R Coll Surg Engl 46:20

Dr. Y Francois
H6pital Claude Bernard
22 Grande Rue d'Oullins
F-69600 Oullins
France

Int J Colorect Dis (1990) 5:15-20

Col6~
Disease
9 Springer-Verlag 1990

Endorectal ultrasonography with a 7.5 M H z linear array scanner


for the assessment of invasion of rectal carcinoma
E Konishi 1, H. Ugajin 1, K. Ito 2 and K. Kanazawa 1
Departments of 1Surgery and 2 Clinical Pathology, Jichi Medical School, Tochigiken, Japan
Accepted: 4 July 1989

Abstract. A n e n d o r e c t a l 7.5 M H z linear a r r a y s c a n n e r


was used for the a s s e s s m e n t o f i n v a s i o n in rectal t u m o u r s .
A s a p r e l i m i n a r y s t u d y n o r m a l rectal wall o f the resected
s p e c i m e n was s c a n n e d w i t h the 7.5 M H z linear a r r a y
scanner. T h e rectal wall was d e p i c t e d in seven layers. T h e
a n a t o m i c a l i d e n t i f i c a t i o n o f these seven layers was m a d e .
F o r t y nine p a t i e n t s w i t h rectal t u m o u r s were e x a m i n e d
b y e n d o r e c t a l u l t r a s o n o g r a p h y with the 7.5 M H z linear
a r r a y scanner. T h e sensitivities for T1, T2 a n d T3 tum o u r s using the U I C C T N M classification were 8 1 % ,
100% a n d 9 0 % respectively, a n d the specificities were
100%, 85% a n d 100% respectively. D e f i n i t e d i s c r i m i n a tion o f lesions c o n f i n e d to the m u c o s a f r o m those i n v a d ing s u b m u c o s a was difficult, even using this k i n d o f high
f r e q u e n c y u l t r a s o n o g r a p h y . W h e n the s o n o g r a p h i c assessment was m u c o s a l , o r m u c o s a l o r s u b m u c o s a l inv o l v e m e n t , local excision was p e r f o r m e d . W h e n the assessment was i n v a s i o n in the m u s c u l a r i s p r o p r i a o r inv a s i o n p e n e t r a t i n g the m u s c u l a r i s p r o p r i a , b o w e l resection was p e r f o r m e d . E x c e p t in o n e case, the h i s t o l o g y o f
the resected specimens o f the 49 cases c o n f i r m e d the adeq u a c y o f the o p e r a t i o n m e t h o d s selected. A l t h o u g h there
was s o m e difficulty in m a k i n g the d i s t i n c t i o n b e t w e e n
m u c o s a l a n d s u b m u c o s a l lesions, e n d o r e c t a l u l t r a s o n o g r a p h y with the 7.5 M H z linear a r r a y s c a n n e r was considered to p r o v i d e useful i n f o r m a t i o n in d e c i d i n g on the
t y p e o f o p e r a t i o n offered to the p a t i e n t .

Introduction
The preoperative assessment of invasion of rectal carcinoma is important when we decide the method of operation. The extent of invasion has been assessed mainly by
digital examination [1]. With this method assessment of
invasion is relatively accurate only by a very experienced
examiner. With CT scanning only a gross assessment of
invasion is possible [2]. During recent years endorectal
ultrasonography has been used for the assessment of invasion of rectal carcinoma [3-7]. The advantage of en-

d o r e c t a l u l t r a s o n o g r a p h y is t h a t it is p o s s i b l e to visualize
the l a y e r e d s t r u c t u r e o f the rectal wall. U p until n o w
v a r i o u s types o f t r a n s r e c t a l u l t r a s o n i c s c a n n e r h a v e been
used. I n m o s t o f the r e p o r t s r a d i a l scanners w i t h a
s o n o f r e q u e n c y o f 5.0 to 7.5 M H z were used. U n t i l 3 y e a r s
a g o we h a d been using a 5.0 M H z linear a r r a y s c a n n e r [4].
I n this s t u d y we used a n e w l y d e v e l o p e d 7.5 M H z linear
a r r a y s c a n n e r w h i c h has a b e t t e r r e s o l u t i o n t h a n the
5.0 M H z linear a r r a y scanner. O u r experience with this
scanner a n d its influence o n the o p e r a t i v e m e t h o d subseq u e n t l y selected has been assessed.

Materials and methods


In a preliminary study, normal bowel wall from fresh and fixed
specimens of the resected colon and rectum were scanned in a water
bath using the 7.5 MHz linear array scanner (UST-660-7.5, SSD331, Aloka Japan). The probe used in this study was T-shaped and
the transducer of this probe was identical to the one for endorectal
use with a sonofrequency of 7.5 MHz. In this experimental study,
the layered structure of the bowel walt visualized by ultrasonography was analysed by injecting saline into the submucosa, stripping
off the mucosa and the submucosa and scanning the bowel wall
adjacent to a taenia coli.
Forty nine patients with rectal cancer were examined with the
7.5 MHz linear scanner (UST-660-7.5, SSD-331, Aloka Japan). Tumours with marked stenosis were excluded from this study because
insertion of the probe was not always possible. Since the probe was
rigid, the distal margin of tumours suitable for scanning was less
than 10 cms from the anal verge. A thin balloon was attached to the
probe, and after insertion into the rectum it was filled with degassed
water. In selected cases, usually those with only small lesions, the
probe was inserted without a balloon and water was infused into the
lumen of the rectum. The entire tumour was scanned by turning the
probe around its long axis. The depth of invasion was assessed
according to the interruption of the layers of the rectal wall by the
tumour. Extramural invasion was divided into two categories according to the following findings for both ultrasonography and
histology. When the tumour invasion reached only a few millimetres
below the level of the interrupted muscle coat, the invasion was
assessed as slight. When the extramural invasion was greater than
that it was assessed as moderate to extensive. The assessment by
ultrasonography was compared with histological findings of the
resected specimens. The formulae for sensitivity and specificity for

16
Table 1. Formule for the sensitivity and specificity in the sonographic assessment of the TNM staging
Sensitivity = TP/TP + FN
Specificity= TN/TN + FP
TP: True positive. Number of cases correctly assessed as a particular
grade
TN: True negative. Number of cases correctly assessed as other
grades
FP: False positive. Number of cases incorrectly assessed as a particular grade
FN: False negative. Number of cases incorrectly assessed as other
grades

each grade of UICC TNM classification [8] are shown in Table 1. In


35 cases in which one experienced surgeon did the digital examination, the assessment of invasion by digital examination was compared to that using endorectal ultrasonography. The operative
method selected by the findings on ultrasonography was audited
and the detection of lymph node metastasis by uttrasonography in
49 cases was assessed.

Results
T h e n o r m a l rectal wall o f the resected s p e c i m e n was visualized in seven layers w h e n s c a n n e d with a 7.5 M H z linear
a r r a y s c a n n e r in a w a t e r b a t h . W h e n saline was injected
into the s u b m u c o s a o f a fresh specimen, the injected saline was v i s u a l i z e d in the t h i r d e c h o g e n i c layer (Fig. 1). I n
the n e x t e x p e r i m e n t m u c o s a was s t r i p p e d o f f in one p a r t
o f a fixed s p e c i m e n a n d the m u c o s a a n d the s u b m u c o s a
were s t r i p p e d o f f in the a d j a c e n t p a r t o f the s p e c i m e n a n d
s c a n n e d in the w a t e r b a t h . I n the p a r t w h e r e the m u c o s a
was s t r i p p e d off, the t h i r d l a y e r w h i c h was e c h o g e n i c was
e x p o s e d o n the surface. I n the p a r t w h e r e the m u c o s a a n d
the s u b m u c o s a were s t r i p p e d o f f o n l y a thin e c h o g e n i c
l a y e r was seen o n the surface o f the e c h o p o o r l a y e r
(Fig. 2). W i t h these results the t h i r d layer, w h i c h was
echogenic, was c o n s i d e r e d to be the s u b m u c o s a , a n d the
e c h o p o o r layers u n d e r n e a t h this layer were c o n s i d e r e d to
b e the m u s c u l a r i s p r o p r i a . In the t h i r d e x p e r i m e n t , b o w e l
wall with a t h i c k t a e n i a coli was scanned. T h e a r r o w in
Fig. 3 shows the site o f the t a e n i a coli. T h e e c h o p o o r
l a y e r c o n t i n u o u s w i t h the t a e n i a coli was c o n s i d e r e d to be
the l o n g i t u d i n a l m u s c l e layer. T h e e c h o p o o r layer a b o v e
the thin h i g h echoic l a y e r was c o n s i d e r e d to be the circular m u s c l e layer. We h a v e s u m m a r i z e d the l a y e r e d structure o f the rectal wall v i s u a l i z e d b y u l t r a s o n o g r a p h y in
Fig. 4.
W i t h the 7.5 M H z p r o b e n o r m a l rectal wall w a s visualized in five to seven layers. T h e thin e c h o g e n i c layer
(layer 5 in Fig. 4) w h i c h is c o n s i d e r e d to be the b o u n d a r y
b e t w e e n the two m u s c u l a r layers was n o t a c o n s t a n t finding.
I n Table 2 a c c u r a c y o f a s s e s s m e n t o f i n v a s i o n in the
49 cases e x a m i n e d is shown. I n this t a b l e the d e p t h o f
i n v a s i o n was g r a d e d into five c a t e g o r i e s a n d the a c c u r a c y
o f a s s e s s m e n t for each o f these is shown. Cases in w h i c h
it w a s difficult to assess i n v o l v e m e n t as m u c o s a l o r sub-

Fig. 1. Saline was injected into the submucosa of a normal rectal


wall and scanned in a water bath with the 7.5 MHz linear array
scanner, sin, submucosa; pm, proper muscle
Fig. 2. The mucosa was stripped off in the part indicated with a
thick line, and the mucosa and the submucosa were stripped off in
the part indicated with the thin line. sin, submucosa, pro, proper
muscle
Fig. 3. Ultrasonic scanning of a colonic wall with a thick taenia coli
in a water bath. The taenia coli was visualized in the part indicated
with the arrow
Fig. 4. Identification of the layers visualized with 7.5 MHz ultrasonography

17

Fig. 6. A rectal tumour measuring 2.5 cms invading the superficial


part of the muscularis propria (endorectal scan), sm, submucosa;
pm, proper muscle; T, tumour
Fig. 5. A rectal tumour confined to the mucosa, measuring 3 cms
(endorectal scan), sin, submucosa; pm, proper muscle; T, tumour

Table 2. Accuracy of assessment with endorectal ultrasonography


Depth of invasion
(histology)

Correct assessment
on ultrasonography

Mucosa

8/12 (67%)

Submucosa

0/4

(0%)

Muscularis propria

10/10 (100%)

Slight extramural invasion

14/16 (88%)

Moderate to extensive
extramural invasion

5/5 (100%)

Table 3. Results of preoperative staging with endorectal ultrasonography (UICC TNM classification)
Histological
depth of invation

Sensitivity

Specificity

Positive
predictive
value

Negative
predictive
value

T1 mucosa,
submucosa

81%

100%

100%

92%

T2 muscularis
propria
T3 extramural
invasion

100%

85%

100%

100%

90%

100%

100%

93%

mucosal were classified as "inaccurate". S o n o g r a p h i c assessment was correct in 8 o f the 12 cases with mucosal
involvement only, in none o f the 4 cases with invasion in
the submucosa, in all o f the 10 cases with invasion confined to the muscularis propria, in 14 o f 16 cases with
slight extramural invasion, and in all o f the 5 cases with
m o d e r a t e to extensive extramural invasion. The histological depth o f invasion was reported according to the
U I C C T N M classification [8], and the sensitivity,
specificity, positive predictive value and negative predictive value in each grade are shown in Table 3. In this table
two cases with invasion to an adjacent structure (T4) are
excluded because o f the small n u m b e r s o f cases in this
category.
Figure 5 shows a rectal t u m o u r with mucosal involvement. The t u m o u r is visualized in a m e d i u m echo level as
a definite elevation above the mucosal surface. The lesion
is visualized a b o v e the echogenic layer which is considered to be the submucosa. Figure 6 shows a case with
invasion into the superficial part o f the muscularis propria. The s u b m u c o s a which is visualized as an echogenic
layer is interrupted at b o t h margins o f the turnout, and
the t u m o u r reaches the superficial part o f the thickened
muscularis propria. Figure 7 shows a case with slight ext r a m u r a l invasion. There is an interruption o f the low
echoic layer which corresponds to the muscularis propria. Figure 8 summarises the s o n o g r a p h i c assessment
and the selected operative methods. I n 10 cases local excision was performed because o f the s o n o g r a p h i c assessm e n t o f " m u c o s a l " or " m u c o s a l or s u b m u c o s a l " involvement. In 8 o f these 10 cases t u m o u r was confined to the
mucosa, and in 2 other cases t u m o u r was invading the
superficial p a r t o f the submucosa. L y m p h node involve-

18
Table 4. Accuracy - a comparison between digital examination and

endorectal ultrasonography
Histological depth
of invasion

Digital
examination

Endorectal
ultrasonography

Mucosa or
submucosa

(Freely mobile)
12/14

12/14

Muscularis propria

(Mobile)
4/6

Extramural
invasion

(Tethered mobility)
14/15

6/6
14/15

Table 5. Detection of lymph node metastasis by endorectal ultra-

sonography
Lymph node enlargement
on ultrasonography
(+)
(-)

Fig. 7. A rectal tumour measuring 4 cms with a slight extramural


invasion (endorectal scan), sm, submucosa; pro, proper muscle;
2", tumour

Ultrasonic Assessment

of Invasion

Histology

Operation
~

Mucosa, Mucosa or - - L o c a l
Submucosa (10 cases)
Excision

mucosa

(8)

submucosa
(2)
(slight to moderate)
(0)
submucosa
(massive invasion)
[ - - mucosa

(1)

I - - submucosa .

(0)

(slight to moderate)

Muscularis Propria
(1 5 cases)

Bowel

Resection

---~--_
- _ submucosa

I~

(2)

(massive invasion)

mucularis

l Propria

(1 O)

L.__ slight extramural


invasion
(2)

Fig. 8. Operative methods selected according to the ultrasonic assessment and the histological results. In 24 other cases correct assessment of extramural invasion was made which led to bowel resection

ment was not present in the specimens of these 10 cases.


Bowel resection was performed because where the sonographic assessment demonstrated invasion reaching the
muscularis propria in 15 cases, a bowel resection was
indicated. In 10 of these 15 cases sonographic assessment
was correct. In two other cases histology showed invasion
into the deeper part of the submucosa, and as a result the
selected operative method was considered to be appropriate. In two other cases histology showed slight extramural invasion. The tumour was limited to the mucosa in
only one case and here sonographic assessment led to

Lymph node
metastasis ( + )

Lymph node
metastasis ( - )

34

inappropriate treatment. In all 24 cases in which the


sonographic assessment showed extramural invasion
bowel resection was performed. Histology of these resected specimens showed extramural invasion and confirmed the adequacy of the operative method selected. In
35 out of 49 patients digital examination was performed
by one experienced examiner. The assessment of depth o f
invasion was made according to York-Mason's criteria.
Here, freely mobile corresponds to mucosal or submucosal involvement, mobile corresponds to invasion in the
muscularis propria and tethered mobility corresponds to
extramural invasion. The accuracy of digital examination
compared to endorectal ultrasonography in 35 cases is
shown in Table 4.
Enlarged lymph nodes suggestive of metastatic involvement were seen as a round mass with a medium echo
level. Our success in detecting lymph node metastasis
with endorectal ultrasonography was not satisfactory.
The sensitivity for the detection of enlarged lymph nodes
was 36% (5/13), the specificity 94% (34/36) and the positive predictive value 71% (5/7) (Table 5).

Discussion

In the treatment of rectal carcinoma preoperative assessment of depth of invasion is important, particularly when
there may be an indication for local excision. If the lesion
is confined to the mucosa or in certain cases the invasion
reaches the submucosa, local excision is considered to be
appropriate.
Although Morson stated that the rate of lymph node
metastasis in cases with invasion into the muscularis pro-

19
pria is only 10% [9], in other reports lymph node metastasis in 20% or 30% have been observed [10-12]. For this
reason, when invasion reaches the muscularis propria,
bowel resection with lymph node dissection is considered
to be necessary.
The preoperative assessment of invasion of rectal carcinoma has usually been made by digital examination.
York-Mason proposed his own criteria of assessment
with digital examination [1]. Although previous reports
and our own experience of digital examination showed a
fairly good accuracy, the reliability of this examination is
high only when performed by an experienced examiner.
CT scans are disappointing since the layered structure of
the rectal wall cannot be demonstrated.
Since the advent of endorectal ultrasonography, it has
become possible to visualize the layered structure of the
rectal wall. In some earlier reports the rectal wall was
depicted in three layers on endorectal ultrasonography
[3, 4]. Endorectal transducers with a higher frequency
have made it possible to visualize the rectal wall in five to
seven layers and our results agree with other reports [13,
14]. The first layer, which is echogenic, is considered to be
generated by the b o u n d a r y effect at the surface of the
mucosa. We consider that the second echopoor layer does
not correspond to the muscularis mucosa itself. The reason for this is that the muscularis mucosa usually measures 25 to 30 microns, and is much thinner than the
second echopoor layer. The second echopoor layer is
considered to be mucosa, and possibly to include muscularis mucosa. According to the results of our study the
other layers can be identified as shown in Fig. 3.
In our present study, a 7.5 M H z linear array scanner
was used, and the disadvantage of this higher frequency
is that the ultrasound does not penetrate deeply in the
tissue. Considering that the thickness of a rectal carcinoma is 4 - 5 cms at most, the penetration of 7.5 M H z
ultrasound would be sufficient. In a previous report the
linear array scanner had better resolution than a radial
scanner with the same ultrasonic frequency [15]. The reason for this may be that in the linear array scan the
scanning is electrical, resulting in a truly real time scan,
whilst in a radial scan the scanning is mechanical.
The result of the assessment of invasion in our 49
cases was satisfactory when analysed for invasion reaching the muscularis propria or invasion which had gone
through the muscularis propria, and compares well with
recent reports using 7.0 or 7.5 M H z transducers. An accurate assessment of lesions confined to the mucosa or in
which the invasion had reached the submucosa was more
difficult. Except in one case, tumours confined to the
mucosa were treated by local excision. The incorrect assessment which led to a bowel resection may be due to the
attenuation of ultrasound caused by the thick protruberant turnout confined to the mucosa. This does not suggest that the penetration of ultrasound with the 7.5 M H z
transducer was insufficient since images obtained with a
5.0 M H z transducer in the same case showed a poorer
visualization of the layers. In all cases with submucosal
invasion the sonographic assessment was incorrect. However, in two of these cases in which histology showed
invasion in the superficial part of the submucosa local

excision was performed. Two other cases in which histology showed invasion nearly reaching the muscularis propria had a bowel resection and lymph node dissection
(Fig. 8). We think that in the latter two cases bowel resection was an appropriate procedure because invasion
nearly reached the muscularis propria. K u d o has reported lymph node metastasis in 20% of tumours invading the deep part of the submucosa [16]. It may therefore
be appropriate to treat tumours with invasion into the
deep part of the submucosa by bowel resection rather
than by local excision unless the patient is aged or has a
poor general condition.
Although there is still some difficulty in making an
accurate sonographic assessment of lesions confined to
the mucosa or those with invasion into the submucosa,
ultrasonography with a 7.5 M H z linear array scan is a
useful method for deciding the indications for local excision. In a recent report by Wang et al. [17], in which
resected specimens were scanned with an 8.5 M H z linear
array scanner, assessment for tumours invading the submucosa was better than ours. The development of an
endorectal linear array scanner with a frequency higher
than that in our present study will make it possible to
make a more accurate assessment of invasion in such
tumours.

References
1. Mason AY (1976) Rectal cancer: the spectrum of selective surgery. Proc R Soc Med 69:237-244
2. Williams MP, Husband JE (1987) CT scanning in carcinoma of
the rectum: a review. J R Soc Med 80:701-703
3. Hildebrandt U, Feifel G (1985) Preoperative staging of rectal
cancer by intrarectal ultrasound. Dis Colon Rectum 28:42-46
4. Konishi F, Muto T, Takahashi H, Itoh K, Kanazawa K,
Morioka Y (1985) Transrectal ultrasonography for the assessment of invasion of rectal carcinoma. Dis Colon Rectum 28:
889-894
5. Saitoh N, Okui K, Sarashina H, Suzuki M, Arai T, Nunomura
M (1986) Evaluation of echographic diagnosis of rectal cancer
using intrarectal ultrasonic examination. Dis Colon Rectum
29:234-242
6. Beynon J, Foy DMA, Roe AM, Temple LN, Mortensen
NJMcC (1986) Endoluminal ultrasound in the assessment of
local invasion in rectal cancer. Br J Surg 73:474-477
7. Yamashita Y, Machi J, Shirouzu K, Mototomi T, Isomoto H,
Kakegawa T (1988) Evaluation of endorectal ultrasound for the
assessment of wall invasion of rectal cancer. Dis Colon Rectum
31:617-623
8. Spiessel B, Hermanek P, Schiebe O, Wanger G (eds) (1985)
UICC TNM atlas. Springer, Berlin Heidelberg New York
9. Morson BC (1966) Factors influencing the prognosis of early
cancer of the rectum. Proc R Soc Med 59:607
10. Astler VB, Coller FA (1953) The prognostic significance of
direct extension of carcinoma of the colon and rectum. Ann
Surg 130:846-852
11. Copeland EM, Miller LD, Jones RS (1968) Prognostic factors
in carcinoma of the colon and rectum. Am J Surg 116:875-881
12. Hojo K, Koyama Y, Moriya Y (1982) Lymphatic spread and its
prognostic value in patients with rectal cancer. Am J Surg
144:350-354
13. Beynon J, Foy DMA, Temple LN, Virjee J, Channer JL,
Mortensen NJMcC (1986) The endosonic appearances of normal colon and rectum. Dis Colon Rectum 29:810-813

20
14. Aibe T (1984) A study on the structure of layers of the gastrointestinal wall visualized by means of the ultrasonic endoscope
II. The structure of layers of the esophageal wall and the colonic
wall. Gastrointest Endosc 26:1465-1473

17. Wang KY, Kimmey MB, Nyberg DA, Mack LA, Haggit RC,
Shuman WP, Franklin DW, Silverstein FE (1987) Colorectal
neoplasms: accuracy of US in demonstrating the depth of invasion. Radiology 165:827-829

15. Konishi F, Takahashi H, Itoh K, Sunouchi T, Muto T, Kanazawa K, Morioka Y (1985) Transrectal ultrasonography of
rectal carcinoma - Our present result and a study on the quality
of a newly developed 7.5 MHz linear scanner. Jpn J Med Ultrasonics 12:715-716

Dr. Fumio Konishi


Department of Surgery
Jichi Medical School
3311-1 Yakushiji
Minamikawachimachi
Kawachigun
Tochigiken 392-04
Japan

16. Kudo S, Soga J, Yamamoto M, Koyama S, Muto T (1984)


Treatment of colorectal sm-carcinomas. Stomach and Intestine
12:1349-1355

Announcements
2 3 - 2 4 April 1990 - Marseille]France

Journ~es d'H~pato-Gastroent~rologie M~dico-Chirurgicale

2 5 - 2 6 April 1990 - Marseille]France

Journ~es d'Endoscopie, de Radiologie et d'Echographie Digestive (ERED)


Organizers: Prof. H. Sarles, Prof. J. C. Sarles, Prof. J. Sahcl, Prof. A.Gerolami, Prof. R. Lagier,
Prof. B. Sastre, Dr. A. Arnaud.
For further information contact: Sud Congr~s Services, 277, chemin du Vallon de l'Oriol,
F-13007 Marseille, France. Tel: (33) 91 59 43 33, Fax: (33) 91 52 63 68

20
14. Aibe T (1984) A study on the structure of layers of the gastrointestinal wall visualized by means of the ultrasonic endoscope
II. The structure of layers of the esophageal wall and the colonic
wall. Gastrointest Endosc 26:1465-1473

17. Wang KY, Kimmey MB, Nyberg DA, Mack LA, Haggit RC,
Shuman WP, Franklin DW, Silverstein FE (1987) Colorectal
neoplasms: accuracy of US in demonstrating the depth of invasion. Radiology 165:827-829

15. Konishi F, Takahashi H, Itoh K, Sunouchi T, Muto T, Kanazawa K, Morioka Y (1985) Transrectal ultrasonography of
rectal carcinoma - Our present result and a study on the quality
of a newly developed 7.5 MHz linear scanner. Jpn J Med Ultrasonics 12:715-716

Dr. Fumio Konishi


Department of Surgery
Jichi Medical School
3311-1 Yakushiji
Minamikawachimachi
Kawachigun
Tochigiken 392-04
Japan

16. Kudo S, Soga J, Yamamoto M, Koyama S, Muto T (1984)


Treatment of colorectal sm-carcinomas. Stomach and Intestine
12:1349-1355

Announcements
2 3 - 2 4 April 1990 - Marseille]France

Journ~es d'H~pato-Gastroent~rologie M~dico-Chirurgicale

2 5 - 2 6 April 1990 - Marseille]France

Journ~es d'Endoscopie, de Radiologie et d'Echographie Digestive (ERED)


Organizers: Prof. H. Sarles, Prof. J. C. Sarles, Prof. J. Sahcl, Prof. A.Gerolami, Prof. R. Lagier,
Prof. B. Sastre, Dr. A. Arnaud.
For further information contact: Sud Congr~s Services, 277, chemin du Vallon de l'Oriol,
F-13007 Marseille, France. Tel: (33) 91 59 43 33, Fax: (33) 91 52 63 68

Int J Colorect Dis (1990) 5:21-24

Col6ree|al
Disease

9 Springer-Verlag 1990

The investigation of anorectal dysfunction in the solitary


rectal ulcer syndrome
E.J. Mackle, J.O. Manton Mills and T.G. Parks
1 Department of Surgery, The Queen's University of Belfast, Belfast City Hospital, Belfast, UK
2 Department of Radiology, Royal Victoria Hospital, Belfast, UK
Accepted: 26 October 1989

Abstract. To investigate a n o r e c t a l f u n c t i o n in s o l i t a r y
rectal ulcer s y n d r o m e 22 p a t i e n t s were s t u d i e d b y m e a n s
o f b a l l o o n expulsion, intestinal t r a n s i t time, b a r i u m ene m a a n d e v a c u a t i o n p r o c t o g r a p h y . H a l f o f the p a t i e n t s
tested h a d difficulty in expelling a w a t e r filled b a l l o o n .
D e l a y in intestinal t r a n s i t was n o t e d in o n l y three p a tients. B a r i u m e n e m a was o f little benefit in d i a g n o s i n g
the c o n d i t i o n . E v a c u a t i o n p r o c t o g r a p h y was the investig a t i o n o f choice in t h a t it s h o w e d at least one a b n o r m a l ity o f pelvic f l o o r f u n c t i o n in all o f the p a t i e n t s a n d can
help select p a t i e n t s for surgery.

Introduction
S o l i t a r y rectal ulcer s y n d r o m e is a benign c o n d i t i o n affecting the r e c t u m m a i n l y in y o u n g to m i d d l e a g e d adults.
T h e c o n d i t i o n was first d e s c r i b e d in detail b y M a d i g a n
a n d M o r s o n in 1969 [1]. T h e title is far f r o m ideal in t h a t
a s o l i t a r y ulcer is only f o u n d in a p p r o x i m a t e l y 4 0 % o f
p a t i e n t s with the s y n d r o m e , while 2 0 % h a v e m u l t i p l e
ulcers a n d the r e m a i n d e r h a v e either b r o a d b a s e d p o l y p o i d a l lesions o r a p a t c h y g r a n u l a r h y p e r a e m i c m u c o s a
[2]. T y p i c a l l y the p a t i e n t s describe difficulty in defaecation a n d the p a s s a g e o f m u c u s a n d b l o o d p e r rectum.
B i o p s y f r o m the edge o f a n ulcer o r f r o m the g r a n u l a r
m u c o s a in the n o n - u l c e r a t i v e p h a s e will g e n e r a l l y s h o w
the c h a r a c t e r i s t i c h i s t o l o g i c a l changes. T h e d i a g n o s i s is
m a d e on b o t h clinical a n d h i s t o l o g i c a l g r o u n d s as similar
m o r p h o l o g i c a l c h a n g e s have been r e p o r t e d in a r e a s o f
p r o l a p s i n g m u c o s a elsewhere in the g a s t r o i n t e s t i n a l t r a c t

Materials and methods


We have studied 22 patients with a clinical and histological diagnosis of solitary rectal ulcer syndrome. There were 7 men and 15
women whose ages ranged from 19 to 63 years with a mean of 38
years. A detailed history was taken from each patient, paying particular attention to the duration of symptoms prior to assessment and
whether the patients needed to practice self digitation to assist
evacuation. To help quantify the magnitude of the problem associated with defaecation the patients were requested to keep a stool
chart for a week, recording how often they attempted defaecation
and how long they spent straining at stool.
Difficulty experienced during evacuation was quantified using
a balloon expulsion test [7, 8]. A small balloon mounted on a fine
catheter was inserted into the rectum with the patient lying in the
left lateral position. The balloon was then inflated with 50 ml of
water at body temperature and the attached catheter passed over a
pulley system (Fig. 1). An attempt was made to expel the balloon
and if this proved impossible traction was applied to the end of the
catheter by adding weights in 50 g increments until eventually expulsion occurred. We have confirmed that normal control subjects
have no difficulty in expelling the bailoon without the addition of
weights.
Twelve of the 22 patients had a double contrast barium enema
examination prior to entry to this study and the results of these were
reviewed. In order to assess the intestinal transit time we used the
radio-opaque marker technique of Hinton et al. [9]. The patients
swallowed 20 markers on day 1 and then on day 6 a plain radiograph of the abdomen was taken and the number of residual mark-

[3].
T h e precise a e t i o l o g y o f the c o n d i t i o n is u n k n o w n ;
however, its a s s o c i a t i o n with rectal p r o l a p s e a n d disordered pelvic f l o o r f u n c t i o n is well r e c o g n i s e d [ 2 - 6 ] . We
r e p o r t the results o f a s t u d y u n d e r t a k e n to assess the
difficulty in d e f a e c a t i o n d e s c r i b e d b y p a t i e n t s with the
condition.

Fig. 1. Technique of balloon expulsion

22
ers counted, the presence of more than 4 markers (20%) being
indicative of delay in intestinal transit. No laxatives or antidiarrhoeal drugs were consumed during the period of the test.
We carried out evacuation proctography using a modification
of the technique described by Mahieu et al. [10]. A suspension of
barium sulphate was thickened with potato starch and 140 ml were
then injected rectally. The patients then sat upon a perspex commode bolted to a standard screening table and a video record of the
screening during evacuation was taken as well as spot radiographs
at rest, whilst straining, during evacuation and post-evacuation.
With the screening time being usually less than 3 min the dose of
radiation was significantly less than that used for barium enema.

Results
All of the patients gave a history of problems with evacuation with the duration of s y m p t o m s prior to assessment ranging from 3 months to 20 years (mean 7.5 years).
Self digitation to assist evacuation was practised by 20 of
the 22 patients. One patient felt that she was pushing a
blockage out of the way to enable evacuation to progress
normally, while the other 19 felt that they were digitally
evacuating their bowels. F r o m the stool charts it was
noted that each patient attempted evacuation on average
4 times per day (1 - 11) and spent an average of 33 min per
day (range 3 - 2 0 0 ) straining at stool.
O f the 15 patients tested 8 had no difficulty in expelling a water filled balloon. However, 7 required assistance with a range of 100 to 350 g (mean 200 g) of traction needing to be applied to the tubing to achieve
expulsion.
The barium enema examination was a b n o r m a l in
only three cases. One case showed a granular appearance
of the rectum, one a thickening of the wall in the upper
rectum and diverticula of the sigmoid colon, whilst the
third had sigmoid diverticula.
A delay in intestinal transit of ingested radio-opaque
markers was noted in only 3 of the 22 patients. One had
13 residual pellets present in the rectum after 5 days, and
the other 2 patients had 7 and 8 pellets respectively distributed along the large intestine distal to the splenic
flexure.
Evacuation p r o c t o g r a p h y demonstrated an abnormality in all of the 22 patients and in some more than one
abnormality was present. The anorectal angle failed to
increase beyond 90 degrees in 5 patients indicating a failure of the puborectalis muscle to relax properly (Fig. 2).
Excessive descent of the pelvic floor was noted in 17
patients and in one patient there was a m a r k e d descent of
the posterior pelvic floor. Six of the patients with excessive pelvic floor descent also had a rectocele while three
patients had prolapse of the anterior rectal wall mucosa.
Rectal emptying was almost non-existent after 30 s
straining in 2 cases and was visually considered t o - b e
p o o r in a further 4.
The presence of an intussusception of the rectum was
demonstrated in nine patients (Fig. 3). Intussusception of
the rectum is usually described as commencing in the
midrectal region [11]; however in one of the nine cases it
commenced at the anorectal junction.

Fig. 2. Proctogram showing failure of relaxation of puborectalis


(arrowed) and the development of a rectocele during evacuation

Fig. 3. Proctogram showing recto-anal intussusception

Discussion
The exact aetiology of solitary rectal ulcer syndrome is
unknown and several theories have been proposed to
account for the clinical and histological changes seen in
the syndrome. It is generally accepted that there is disordered pelvic floor function associated with the condition.
In particular the association of rectal intussusception,
excessive perineal descent and the failure of the puborectalis muscle to relax during evacuation are well recognised [12-14].
The majority of patients with the syndrome experience difficulty in evacuating their bowels and this is m a n ifest in this series by the average time of 33 min spent
straining at stool per day. It has previously been thought
that the cause of the ulceration was self digitation [15];
however, the histological changes are not those expected
f r o m simple trauma. Furthermore, avoidance of the prac-

23
tice does not produce healing of the ulcers. A review of
260 male homosexuals with bowel complaints produced
only 6 cases of rectal ulceration and only one of these
fitted the histological criteria for diagnosis as solitary
rectal ulcer syndrome [15].
Failure to expel an intrarectal balloon without assistance was demonstrated in almost 50% of those tested.
While the balloon may simulate the presence of stool in
the rectum, it is less likely to induce peristaltic activity in
the sigmoid which might be expected to be associated
with the onward passage of stool in a caudad direction in
the distal large intestine. We found the test to be simple
to perform and normal controls to have no difficulty in
expelling the balloon; however we must admit that the
left lateral position and balloon expulsion per se is not
very physiological. Indeed, of the eight patients shown to
have difficulty in expelling the balloon, only one was
considered on proctography to have poor evacuation and
a further one showed failure of the anorectal angle to
increase beyond 90 degrees during defaecation.
A delay in the passage of radio-opaque markers was
seen in only 3 (14%) patients while 17 showed transit
times within the normal range. Furthermore, the distal
location of the pellets in the three patients does not reasonably indicate colonic inertia since outlet obstruction
can lead to the distribution of the markers throughout
the colon at the fifth day [16].
Barium enema examination may show ulceration,
polypoid lesions, stricturing or granularity. However, it is
rare for the radiologist to be able to make a correct diagnosis on radiological changes alone [17]. We found that a
barium enema was of little benefit in the diagnosis of
solitary rectal ulcer syndrome and really was only of use
in excluding other colorectal pathology.
Evacuation proctography proved to be the single
most useful test as it demonstrated at least one disorder
of pelvic floor function in each of the 22 patients. Using
evacuation proctography we were able to confirm the
association of rectal intussusception and solitary rectal
ulcer syndrome in 41% of patients, while in nearly a
quarter failure of the puborectalis to relax was suggested
by a lack of increase o f the anorectal angle beyond 90
degrees during evacuation.
The amount of pelvic floor descent and measurements of the anorectal angle while straining and during
evacuation were made from the radiographs taken during
each of the phases. While abnormalities in the rectal configuration were present on the radiographs, changes
could best be judged from the video recording of the
screening. We feel that this technique for evacuation
proctography [18] combines the best of balloon proctography [19] and defaecography using cineradiography [10]
or video recording [20] in that anorectal angles and pelvic
floor movement are more accurately measured from a
radiograph than a video screen. Furthermore, it is easier
to make a more accurate allowance for magnification
using radiographs.
Caution, however, must be exercised in interpreting
evacuation proctograms as a study on 48 healthy young
adult subjects showed that a degree of perineal descent,
rectocele and even intussusception can occur in asymp-

tomatic subjects [21]. We feel therefore that while evacuation proctography can show abnormalities, reliance
should not be placed on these results alone and confirmation should be sought by other techniques including
E M G as well as correlation with clinical findings.
It has been postulated that excessive perineal descent
causes outlet obstruction by allowing the anterior rectal
mucosa to prolapse into the anal canal during defaecation [22]. More recently it has been suggested that it is not
just the rectal mucosa which prolapses but rather the full
thickness anterior rectal wall [23]. We found that prolapse
of the anterior rectal wall mucosa occurred in only 18%
of those patients with excessive perineal descent. This
prolapse does not seem to play a significant part in obstructing defaecation as the three patients involved practised self digitation to remove faeces and not to push an
obstruction out of the way.
It has been postulated that a high intrarectal voiding
pressure combined with rectal prolapse is the cause of
solitary ulcer syndrome [12]. However, this theory does
not account for the 10 patients in this study who had no
evidence of either :full thickness or partial prolapse, and
the question as to w h y some patients with the typical
histological changes have ulcers and others do not remains to be answered satisfactorily.
The condition has proven difficult to treat and this is
to some extent a reflection that the exact aetiology of the
condition is unknown. If either occult or overt prolapse
is present then a rectopexy relieves many of the symptoms
associated with the condition [1, 2, 24]. Some workers
have claimed success with internal sphincterotomy [25] or
partial division ofpuborectalis muscle [26], but the results
of these procedures have largely proved unpredictable
and unsatisfactory. We feel that for those patients without prolapse or intussusception, education about bowel
habit with avoidance of straining at stool and the institution of a high fibre diet should be the main line of approach.

Acknowledgements. We wish to express our appreciation for the help


and support of Sister E. Crawford, Departmant of Surgery, The
Queen's University and Miss L. Irwin, Superintendent Radiographer, Royal Victoria Hospital. E. J. Mackle was supported by the
Royal Victoria Hospital Research Fund.

References

1. Madigan MR, Morson BC (1969) Solitary ulcer of the rectum.


Gut 10:871-881
2. Martin CJ, Parks TG, Biggart JD (1981) Solitary rectal ulcer
syndrome in Northern Ireland 1971 1980. Br J Surg 68:744747
3. Rutter KRP (1985) Solitary ulcer syndrome of the rectum: its
relation to mucosal prolapse. In: Henry MM, Swash M (eds)
Coloproctology and the pelvic floor. Pathophysiology and
management. Butterworths, London, pp 282-298
4. Rutter KRP, Riddel RH (1975) The solitary ulcer syndrome of
the rectum. Clin Gastroenterol 4:505-530
5. Lane RH (1974) Clinical application of anorectal physiology.
Proc R Soc Med 68:28-30
6. Mackle EJ, Parks TG (1986) The pathogenesis and pathophysiology of rectal prolapse and solitary rectal ulcer syndrome.
Clin Gastroenterol 15:985 1002

24
7. Barnes PRH, Lennard-Jones JE (1985) Balloon expulsion from
the rectum in constipation of different types. Gut 26:10491052
8. Preston DM, Lennard-Jones JE (1985) Anismus in chronic constipation. Dig Dis Sci 30:404-418
9. Hinton JM, Lennard-Jones JE, Young AC (1969) A new technique for studying gut transit times using radio-opaque markers.
Gut 10:842-847
10. Mahieu P, Pringot J, Bodart P (1984) Defecography, 1. Description of a new procedure and results in normal patients.
Gastrointest Radiol 9:247-251
11. Broden B, Snellman B (1968) Procidentia of the rectum studied
with cineradiography: a contribution to the discussion of the
causative mechanism. Dis Colon Rectum 11:330 347
12. Womack NR; Williams NS, Holmfield JHM, Morrison JF
(1987) Anorectal function in the solitary rectal ulcer syndrome.
Dis Colon Rectum 30:319 323
13. Kuijpers HC, Schreve RH, ten Cate Hoedemakers HC (1986)
Diagnosis of functional disorders of defaecation causing the
solitary rectal ulcer syndrome. Dis Colon Rectum 29: 126-129
14. Snooks SJ, Nicholls RJ, Henry MM, Swash M (1985) Electrophysiological and manometric assessment of the pelvic floor in
the solitary rectal ulcer syndrome. Br J Surg 72:131-133
15. ThomsonH, Hill D (1980) Solitary rectal ulcer: always a self-induced condition? Br J Surg 67:784 785
16. Read NW, Timms JM (1986) Defaecation and the pathophysiology of constipation. Clin Gastroenterol 15:937-965
17. Millward SF, Bayjoo P, Dixon MF, Williams NS, Simpkins KC
(1985) The barium enema appearances in solitary rectal ulcer
syndrome. Clin Radiot 36:185-189

18. Shorvon PJ, Stevensen GW (1989) Defaecography: setting up a


service. Br J Hosp Med 41:460 466
19. Preston DM, Lennard-Jones JE, Thomas BM (1984) The balloon proctogram. Br J Surg 155:45-48
20. Ekberg O, Nylander G, Fort FT (1985) Defaecography. Radiology 155:45-48
21. Shorvon PJ, McHugh F, Somers S, Stevensen GW (1987) Defaecographic findings in young healthy volunteers. Gut
28:A1361-1362
22. Parks AG, Porter NH, Hardcastle J (1966) The syndrome of the
descending perineum. Proc R Soc Med 59:477-482
23. Bartolo DCC, Roe AM, Virgee J, Mortensen NJMcC, LockeEdmunds JC (1988) An analysis of rectal morphology in obstructed defaecation. Int J Colorect Dis 3:17-22
24. Nicholls RJ, Simson JNL (1986) Anteroposterior rectopexy in
the treatment of solitary rectal ulcer syndrome without overt
rectal prolapse. Br J Surg 73:222-224
25. Dough JH, Wright FF (1981) Acute and chronic benign ulcers
of the rectum. Surg Gynecol Obstet 153:398-400
26. Wasserman IF (1964) Puborectalis syndrome. Dis Colon Rectum 7:87-97
Mr. E. J. Mackle
Department of Surgery
The Queen's University of Belfast
University Floor
Belfast City Hospital
Lisburn Road
Belfast BT9 7AB
UK

Int J Colorect Dis (1990) 5:25-30

Col6reeial
Disease

9 Springer-Verlag 1990

Surgery for carcinoma of the colon in people aged 75 years and older
J.P. Ozoux, L. de Calan, M. Perrier, C. Berton, J.P. Favre and J. Brizon
Service de Chirurgie Digestive, H6pital Bretonneau, Tours, France
Accepted: 6 December 1989

Abstract. F r o m J a n u a r y 1976 to June 1986, 154 p a t i e n t s


(75 m e n a n d 79 w o m e n ) w h o were 75 years o f age or o l d e r
were a d m i t t e d for the surgical t r e a t m e n t o f a c o l o n i c
cancer. T h e m e a n age was 80.7 years. P a t i e n t s were div i d e d into two g r o u p s : 66 p a t i e n t s , b e t w e e n 75 a n d 80
years o f age, were in G r o u p I; 88 p a t i e n t s , 80 y e a r s o f age
a n d older, were in G r o u p II. O n e h u n d r e d a n d f o r t y - t h r e e
p a t i e n t s ( 9 3 % ) were o p e r a t e d o n a n d a resection was
c a r r i e d o u t in 125 p a t i e n t s (87%). T h e overall p o s t - o p e r ative m o r t a l i t y r a t e was 12% ( G r o u p I: 5%; G r o u p II:
17%; p < 0.02). T h e a c t u a r i a l survival r a t e o f p a t i e n t s
o p e r a t e d o n was 32_+8% at 3 y e a r s a n d 26+_8% at 5
y e a r s ( G r o u p I: 3 7 % ; G r o u p II: 17%). In b o t h g r o u p s the
survival rate was closely r e l a t e d to the extent o f the disease a c c o r d i n g to D u k e s classification. These results suggest t h a t in the 75 80 y e a r g r o u p age a l o n e s h o u l d no
l o n g e r be c o n s i d e r e d a m a j o r risk f a c t o r for i m m e d i a t e
surgical o u t c o m e . I n p a t i e n t s a g e d 80 a n d older, the results are w o r s e b u t it is n o t a d v a n c e d age p e r se w h i c h
influences m o r t a l i t y , r a t h e r the p h y s i o l o g i c status o f the
patient.

Introduction

Patients and methods


P a tien ts

In accordance with the World Health Organisation Classification of


Elderly People and geriatric classifications (Silverberg and Cassel
cited by Bader [5]), the minimum age was set at 75 years old. One
hundred and fifty-four patients older than 75 years were hospitalised between January 1976 and June 1986 for colonic cancer,
excluding rectal cancers (tumours located less than 15 cm from the
anal margin). There were 75 men and 79 women; median age was
80.7 (82 for women and 79.5 for men; range from 75 to 97 years old)
(Fig. 1). The patients were separated into 2 groups: group I, patients
between 75 and 79 years old (n = 66) and group II, patients older
than 80 years old (n = 88). Tumour distribution according to location is shown in Fig. 2. There were no differences in localisation
with respect to age (30% right-sided cancers and 50% left-sided
cancers in group I compared to 37% right-sided cancers and 47%
left-sided cancers in group II). More women had a right-sided cancer (77% vs 23%) while more men had a left sided cancer (65% vs
35%) (p < 0.01). Sixty-two percent of left-sided cancers and 37% of
right-sided cancers were discovered following a change in bowel
habit. Twenty-seven percent of the patients had a palpable abdominal mass. Most of these were located in the right colon and were

No of p a t i e n t s
70

T h e n u m b e r o f elderly p e o p l e is i n c r e a s i n g in m a n y c o u n tries [1]. In 1985, the N a t i o n a l I n s t i t u t e o f Statistics a n d


E c o n o m i c Studies census f o u n d 700 000 F r e n c h p e o p l e
o l d e r t h a n 85 years o f age. T h e r e will be 1 m i l l i o n in the
y e a r 2000 a n d 2.5 m i l l i o n in 2040. C a n c e r is the s e c o n d
l e a d i n g cause o f d e a t h in these elderly p e o p l e , with colorectal c a n c e r being the s e c o n d m o s t f r e q u e n t cancer,
b e h i n d b r e a s t c a n c e r in w o m e n a n d b r o n c h o g e n i c c a n c e r
in m e n [2]. In F r a n c e , as in o t h e r countries, c a n c e r registries i n d i c a t e t h a t one t h i r d o f the c o l o r e c t a l cancers
o c c u r in elderly p e o p l e [3, 4]. We h a v e a t t e m p t e d to a n a lyse the s h o r t - t e r m a n d l o n g - t e r m t r e a t m e n t results in this
p o p u l a t i o n in o r d e r to d e t e r m i n e w h e t h e r c o l o n i c c a n c e r
has a w o r s e p r o g n o s i s in elderly p e o p l e a n d w h e t h e r age
s h o u l d be a f a c t o r in t h e r a p e u t i c d e c i s i o n - m a k i n g .

60

50

40

30

20

10

Years

0
75 - 8 0

80 - 85

85 - 90

90 a n d +

Fig. 1. Carcinoma of the colon in patients aged 75 years and over.


Age and sex distribution

26
clinically detectable in 53 %. Thirty-ninecancers (25%) were discovered following a complication. Left sided obstruction was more
frequent than right sided obstruction (22% vs 7%) (p < 0.05); group
II had more frequent complications (32%) than group I (17%)
(p<0.05). The symptom duration was less than 6 months in 82%
of the patients.

Methods
Eleven patients did not undergo surgery: 3 had peritoneal carcinomatosis, 3 had another associated cancer, 2 had associated
disease which contraindicated surgery while the remaining 3 patients, who were older than 85 years old, were asymptomatic. One
hundred and forty-three patients out of 154 (93%) underwent
surgery: 63 in group I and 80 in group II. One hundred and twentyfive (87%) of these had tumour resection (Table 1). The resectability
rate was not significantlyrelated to sex (males: 91%, females: 83%)
or to localisation in the left or right colon (right: 92%, left 80%).
The resectability rate was lower in group II, 66 of 80 (83%) than in
group I, 59 of 63 (94%) (p<0.05). Similarly, the resectability rate
in cancers with complications was lower (62%) than in cancers
without complications (96%) (p<0.01). Among the 47 patients
having a resection for right colon cancers, 46 underwent a right
hemicolectomy and one a segmental resection. Resection on the left
colon comprised 48 segmental resections, 4 left hemicolectomies
and 6 Hartman procedures. The inferior mesenteric artery was
ligated at its origin in only 12% of:cases. Of 11 patients who had
synchronous cancers, 8 were operated on (5 subtotal colectomies, 2
.right hemicolectomies, I left colectomy). Six patients had a temporary colostomy followed by re-anastomosiS later on and 3 anastomoses were protected by a temporary colostomy. In 14 patients,
cotectomy had to be extended to include neighbouring organs. Two
patients had metachronous cancers and had to be re-operated on 2
and 4 years after the first operation.

RC

53 (37%)

//

kC

75 (53%)

66

Fig. 2. Site of colonic cancer in 143 patients aged 75 years and over.
RC = right colon; LC = left colon; TC = transverse colon

Table 1. Treatment of carcinoma of the colon in 154 patients aged 75 years and over

The tumours were well-differentiated adenocarcinomas in 24%


of cases, fairly well-differentiated in 37%, poorly differentiated in
11%, mucinous in 6% and polymorphic with mixed mucinous and
adenocarcinoma cells in 22% of cases. In group II there were fewer
well-differentiated cancers (16%) than in group I (30%) (p< 0.05)
and more polymorphic cancers (28 vs 13 %) (p < 0.05). Twenty-eight
per cent of the operative specimens contained one or more colonic
adenomas. Peroperatively, 6% of the patients had peritoneal carcinomatosis and 11% had liver metastasis. Analysis of the classification of the patients according to Dukes staging (Table 2) failed to
show any significant differences with respect to age or tumour
location. However, there were more stage D tumours among the
cancers with complications than among those without (p<0.05).
Survival studies were terminated on November 1, 1986. Followup was obtained from the attending physicians or from mortality
records. None of the patients was lost to follow-up. Survival curves
were calculated according to the actuarial method and the logrank
sum test was used for the difference.

Results

Seventeen of the p a t i e n t s w h o were o p e r a t e d o n died


before the 30th p o s t - o p e r a t i v e day (12%). O f these, six
deaths occurred in patients who only h a d palliative
surgery for cancers with c o m p l i c a t i o n s , five were related
to medical c o m p l i c a t i o n s (cerebrovascular accident, pulm o n a r y e m b o l i s m , m y o c a r d i a l infarction, p n e u m o t h o rax, haematemesis), five were related to surgical c o m p l i c a t i o n s (Table 3), a n d o n e d e a t h r e m a i n e d u n e x p l a i n e d .
M o r t a l i t y was greater in g r o u p II: 14 o f 80 patients ( 1 7 % )
t h a n in g r o u p I: 3 o f 63 patients (5%) ( p < 0 . 0 2 ) . It was
also greater following surgery for c o m p l i c a t i o n s o f cancer
(8 o f 37 patients: 2 2 % ) t h a n for u n c o m p l i c a t e d cancers
(9 o f 106 patients: 8 % ) (p < 0.05). A f t e r curative surgery,
m o r t a l i t y was greater i n g r o u p II: 10 o f 59 patients (17%)
t h a n in g r o u p I: 2 o f 48 patients (4%) (p < 0.02). Twentyfive o f the 143 p a t i e n t s (17%) w h o were o p e r a t e d o n died
d u r i n g the first 3 p o s t o p e r a t i v e m o n t h s : 20 p a t i e n t s o f the
g r o u p II (25%) a n d 5 patients o f g r o u p I; all the p a t i e n t s
w h o died b e t w e e n i a n d 3 m o n t h s following surgery died
f r o m their cancer.
Sixty-two patients experienced m e d i c a l c o m p l i c a tions, especially p u l m o n a r y (50%). F o r t y - f o u r p a t i e n t s
o p e r a t e d o n h a d one or m o r e a b d o m i n a l c o m p l i c a t i o n s
(Table 3) r e q u i r i n g 8 reoperations. Six p a t i e n t s h a d a fistula with p u r u l e n t discharge t h r o u g h their drains; the
discharge was t r a n s i e n t in all o f the cases. A m o n g the
r e m a i n i n g eight patients, three died w i t h o u t a n y o p p o r t u -

Group I
75- 79

No surgery
Resection
- Curative resection
- Palliative resection
Colostomy
Laparotomy alone

Group II
%

> 80

11

125
107
18

81

59
48
11

89

66
59
7

75

14

11

13

27
Table 2. Carcinoma of the colon in patients aged 75 years and over. Dukes' classification in 129 patients
Stage

No. of
patients

13

No complication

Complication

No. of
patients

No. of
patients

10

12

11

75-79 years
No. of
patients

> 80 years
No. of
patients

4
NS

58

45

48

46

10

40

23

35

39

30

32

31

28

18

21

19

15

12
12
t--p<O.O5--J

28

10

60

69

129

104

25

NS = not significant
Table 3. Postoperative complications (44 patients)

Complication

No. of
patients"

No. of deaths
without
reoperation

No. of
reoperations

Wound haematoma
Wound infection
Evisceration

2
27
2

No. of deaths
after
reoperation

Abnormal discharge
Through drain
Anastomotic leakage
Peritonitis

7
1

2
1

1
-

Complication of col 9149


Intestinal obstruction
Intraabdominal bleeding

1
2
4

1
2
2

a One or more complication per patient


Percent
survival

Percent
survivar

100 9
90

S~

70

~ o

.o- TOTAL (N=1431

.o-

60, . . . .

.-.

8o, ....

ool "<:>..\o

100D
llll

,N:8o,

,o,

60
50
40

55%

STAGE A (n=13)

45 %

STAGE B (n=58)

30

32 %

STAGE C (n=39)

11%

STAGED(n=19)

(631
(143)

20'
(881
10'
o

8O

40'
30 '

9- -

20
10

12

18

24

30

38

42

48

54

60

0
months

12

18

24

30

36

Months

Fig. 3. Carcinoma of the colon in patients aged 75 years and over.


Actuarial survival curves

Fig. 4. Carcinoma of the colon in patients aged 75 years and over.


Actuarial survival curves according to stage (Dukes' classification)

nity for re-operation, four closed spontaneously, and one


f o l l o w i n g r e - o p e r a t i o n ( T a b l e 3).
The mean hospital stay of operated patients who did
n o t die b e f o r e t h e 3 0 t h p o s t - o p e r a t i v e d a y w a s 23 d a y s
( r a n g e f r o m 14 to 74 days); it w a s n o t m o d i f i e d b y t h e
p a t i e n t s age.

The actuarial survival rate of operated patients was


6 5 _ + 8 % at ~ y e a r , 3 2 _ + 8 % at 3 y e a r s a n d 26___8% at 5
years. G r o u p I h a d a 5 y e a r s s u r v i v a l r a t e o f 3 7 % w h i l e
g r o u p I I ' s w a s 1 7 % (Fig. 3). T h e g r e a t e s t n u m b e r o f c a n c e r - r e l a t e d d e a t h s o c c u r r e d d u r i n g t h e first 2 years. H o w ever, 2 4 % o f t h e p a t i e n t s d i e d f r o m n o n - c a n c e r r e l a t e d

28
causes (21% in group I and 28% in group II). I f one
excludes patients who died during the first 30 post-operative days and those who died of incidental causes, the
corrected actuarial survival rate was 34% at 5 years (41%
in group I and 26% in group II. Survival was studied as
a function of sex, t u m o u r location, complication, treatment, histologic type and Dukes staging. N o significant
difference was observed at 5 years concerning sex (females:
34%; males: 20%) and right (31%) or left (30%) t u m o u r
location. Survival of patients with cancers with complications was much worse than for those with non-complicated ones (40% vs 4% at three years) (p<0.001). When
curative resection was undertaken, the 5 years survival rate
was 33% (47% in group I and 23% in group II), whereas
it was only 11% at 3 years in the case of palliative surgery.
After 3 years, survival of patients with mucinous and
p o l y m o r p h o u s cancers was less than for differentiated
cancers (28% vs 44%), but the difference was not significant. Figure 4 shows survival differences at 3 years as a
function of Dukes staging. When there were no distant
metastases, the presence or absence of lymph node involvement significantly affected 3 years survival (47%
Dukes A and B vs 32% in Dukes C) ( p < 0.02). Ten of 11
patients who had associated liver metastases died within
2 years, while one patient survived for 26 months. Eight
of 9 patients with peritoneal carcinomatosis died within
12 months while one patient lived for 19 months.
Twenty-three out of 107 patients (21%) who underwent curative surgery experienced a loco-regional,
metastatic or peritoneal recurrence; 21 of these recurrences occurred before the second year. Nine of these
patients were operated on a second time: only one patient
underwent a resection, while all the others had either a
simple l a p a r o t o m y or palliative colostomy. Every patient
who had a recurrence died within 1 year following diag-

nosis and all re-operated recurrences died within the first


6 post-operative months.

Discussion
Our results indicate that colon cancer is not necessarily
more advanced in elderly people and that surgical results
are comparable in the 75 to 80 years old population and
in the younger population. However, after eighty years of
age, mortality is greater and survival worse.
In our series, there were as m a n y males as females as
in colon cancer series from all ages [6-9]. Nevertheless,
some authors have reported a greater proportion of
males to females in elderly patients [4, 10]. Localisation to
the left colon remains predominant, but there is a 30 to
40% right colon cancer rate in elderly people [4-6, 1 0 14], a rate greater than that seen in younger patients (22
to 30%) [8, 15-17]. An increase in the number of polyps
in the right colon with age could explain this difference
[14]. However, the predominance of female right colon
cancers in our series has not been reported elsewhere.
While the clinical symptoms are not different in elderly people, we have noted, along with Jensen [10], that
one quarter of the tumours were detectable on physical
examination. The percentage of cancers with complications, especially obstruction (Table 4), is globally comparable in elderly and in younger patients [2, 8]. Nonetheless, if patients are separated into two age groups, cancer
with complications is seen twice as frequently in patients
older than eighty years old.
Operability diminishes with age [5, 10, 11] but this
seems related more to associated diseases than to age
alone. The resectability rate seems to have increased over
time and is greater than 85% in more recent series

Table 4. Carcinoma of the colon in patients aged 70 years and over. Comparison between series
Authors

Jensen" [10]
Denmark 1969
Adam b [11]
USA 1972
Calabrese ~ [6]
USA 1973
Cohen b [12]
Australia 1978
Bader b [5]
USA 1986
Payne b [4]
Australia 1986
Waldron b [14]
UK 1986
Ozoux" [present series]
France 1989

Year

No. of
patients

Age
years

No.
surgery
%

Resectability
%

Emergency
cases
%

Postoperative
mortality
%

5-year
survival
%

1950-1964

225

_>70

12

67

24

27

25

12 years

226

>_80

30

68

21

32.5

22.5

1955-1966

226

>80

31

72

21.7

32.6

22.4

1971 1976

192

_>70

84.9

23

10

1982-1985

96

_>75

11

86

24

1971-1983

310

_>75

1972-1982

522

>70

16

85

52

29

1976-1986

154

_>75

87

25

11.9

" Carcinoma of the colon; bcarcinoma of the colon and rectum

7.4

7.1
9

3O

26

29
(Table 4). It has become comparable to the rate in younger patients (83 to 91% [8, 15, 17]) and is not influenced
by t u m o u r location or sex. However, resectability falls
with age when greater than 80 years old, as A d a m and
Cohen have underlined [11, 12]. Along with Jensen [10],
we found that resectability was lower in cases of complicated cancer while being of little influence in our series of
younger patients [8].
Cancers in the elderly patient are often advanced as
seen in most series including patients of all ages [11, 18];
45 % of our patients had lymphatic or distant metastases.
After 80 years of age, cancers were not more advanced
and there was no significant difference a m o n g the Dukes
staging as a function of age [4, 5, 12, 14]. However, our
series showed that tumours were less well differentiated in
patients over 80 years of age. Payne [4] noted that the
degree of infiltration into the colon wall was greater in
older patients.
In agreement with the majority of published series, we
defined post-operative mortality as deaths occurring during the first 30 days after surgery. Wilder [19] considers
that it would be better to include all deaths before 3
months in the case of elderly patients; however, while it
m a y be true that 25% of our operated patients older than
80 died during the first 3 months, one must consider that
all of the deaths between 1 and 3 months were due to the
cancer and not to post-operative complications. Our
post-operative mortality rate is comparable to other recent series [20, 21], and is lower than older series reported
(Table 4). While the death rate of 5% found in the 7 5 - 8 0
age group is the same as that in series obtained from all
age groups published [8, 9, 15, 16, 18], the post-operative
mortality rate over 80 years of age was significantly
greater. M a n y authors have estimated mortality to be
two to five times greater in the elderly, but have used
quite variable age limits: 70 years [1, 10, 14, 18], 75 years
[4], 80 years [6, 11, 12]. In fact, a past history of lung or
cardio-vascular disease counts more than age by itself [2,
5, 6, 11]. Surgical complications were the cause of one
half of the deaths in our study [1, 10, 19] and of two
deaths out of three in Payne's series [4]; in particular,
anastomotic fistulae were fatal one out of three times,
with elderly patients experiencing greater difficulty dealing with the slightest surgical complication. The existence
of a complication with cancer also had a negative effect
on mortality and morbidity, a finding seen in other older
patient groups [2, 5, 11, 14, 21] as well as in younger
patients [8, 18]. Using the criteria of a serum albumin
level lower than 30 g/l, Bader [5] has isolated a group of
elderly patients having a high post-operative risk, but this
parameter was not considered in our series. We have observed, along with other authors [4, 19] a large number of
respiratory and urinary complications, with a rate exceeding that of younger patients [5].
In our series, the global five-year survival rate is comparable to that seen in other series of elderly people
(Table 4). Survival comparison with younger patients is
difficult because a large n u m b e r of deaths in elderly people are due to intercurrent causes, being 25% in our series. However, the group I survival rate was 37% at 5
years with a corrected survival rate o f 4 1 % and 47% with

respect to curative operation. These results are comparable to those obtained in young patients from our own
(35%) [8] and other series ( 2 9 - 3 6 % ) [9, 15]. They are
even better if one considers corrected survival or curative
treatment [8, 22]. On the other hand, survival is worse
over 80 years old whether one considers global survival,
corrected survival or survival after curative surgery.
M a n y authors consider age to be a negative factor, but
when one compares survival with that of a similarly aged
population, the prognosis seems to be equal to or even
better than in younger patients [1, 4, 10, 11, 20].
Kragelund has calculated that colorectal cancer lowers
life-expectancy by 40% in both under and over 70 year
old age groups [1].
Considering a number of prognostic factors, tumour
location and sex do not seem to be as important as they
are in younger patients [8, 15, 17], and the degree of
tumour differentiation does not seem to be as significant
as reported elsewhere [8, 17]. However, our results are in
agreement with the majority of other colon cancer series:
curative treatment [8, 20], whether the cancer has a complication or not [1, 7, 10], and the Dukes staging [8-10,
17, 20] all have a significant prognostic value.
In conclusion, mortality and survival in the 75 to 80
year old range are at least equal to that found in younger
patients, suggesting that this group of patients should be
treated in the same fashion. Patients who are older than
80 more often have cancers with associated complications, have a higher operative mortality rate and a worse
prognosis [7, 12]. Morbidity and operative mortality
should be improved by providing better medical preparation in elderly patients, because physiologic status is more
important than age by itself. Finally, improving anaesthesia by the use of combined techniques, such as
epidural and general anaesthesia, can also help achieve
better results [2, 11].

References
1. Kragelund E, Balslev I, Bardram L, Jensen HE, Nielsen J
(1974) Resectability, operative mortality and survival of patients in old age with carcinoma of the colon and rectum. Dis.
Colon Rectum 17:617-621
2. Boyd JB, Bradford B, Watne AL (1980) Operative risk factors
of colon resection in the elderly. Ann Surg 192:743-746
3. Faivre J, Gouget N, Martin F, Michiels R, Cabanne F, Klepping
C (1979) Incidence des cancers colorectaux dans une population
bien d6finie de 450 000 habitants. Gastroenterol Clin Biol
3:820-825
4. Payne JE, Chapuis PH, Pheils MT (1986) Surgery for large
bowel cancer in people aged 75 years and older. Dis Colon
Rectum 29:733-737
5. Bader TF (1986) Colorectal cancer in patients older than 75
years of age. Dis Colon Rectum 29:728 732
6. Calabrese CT, Adam YG, Volk H (1973) Geriatric colon cancer.
Am J Surg 125:181-184
7. Chapuis PH, Dent OF, Fisher R, Newland RC, Pheils MT,
Smyth E, Colquhoun K (1985) A multivariate analysis of clinical and pathological variables in prognosis after resection of
large bowel cancer. Br J Surg 72:698-702
8. De Calan L, Ozoux JP, Brizon J (1983) Les cancers du colon.
Rbsultats du traitement chirurgical. Gastroenterol Clin Biol
7:802-807

30
9. Peloquin A (1972) Cancer du colon et du rectum. Etude de 1228
cas. Ann Chir (Paris) 26:1189-1197
10. Jensen HE, Nielsen J, Balslev I (1970) Carcinoma of the colon
in old age. Ann Surg 171:107-115
11. Adam YG, Calabrese C, Volk H (1972) Colorectal cancer in
patients over 80 years of age. Surg Clin North Am 52:883-889
12. Cohen JR, Theile DE, Holt J, Davis NC (1978) Carcinoma of
the large bowel in patients aged 70 years and over. Aust. NZ J
Surg 48:405-408
13. Slater G, Papatestas A, Tartter PI, Mulvihill M, Aufses AH
(1982) Age distribution of right and left sided colorectal cancers. A m J Gastroenterol 77:63-66
14. Waldron RP, Donovan IA, Drumm J, Mottram SN, Tedman S
(1986) Emergency presentation and mortality from colorectal
cancer in the elderly. Br J Surg 73:214-216
15. Evans JT, Vana J, Aronoff BL, Baker HW, Murphy GP (1978)
Management and survival of carcinoma of the colon: results of
a National Survey by the American College of Surgeons. Ann
Surg 188:716-720
16. Gardner B, Dotan J, Shaikh L, Feldman J, Herbsman H, Alfonso A, Iyer SK (1981) The influence of age upon the survival
of adult patients with carcinoma of the colon. Surg Gynecol
Obstet 153:366-368

17. Moreaux J, Catala M (1985) Les cancers coliques. R6sultats du


traitement chirurgical et pronostic. Cinq cent soixante dix neuf
observations. Nouv Presse Med 14:463-466
18. Irvin GL, Robinson DS, Hubbard S (1985) Operative risks in
patients with colorectal cancer. Am Surg 51:418-422
19. Wilder RJ, Fishbein R (1961) Operative experience with patients over 80 years of age. Surg Gynecol Obstet 113:205-212
20. Hobler KE (1986) Colon surgery for cancer in the very elderly.
Ann Surg 203:129-131
21. Reiss R, Deutsch AA, Eliashiv A (1983) Decision-making process in abdominal surgery in the geriatric patient. World J Surg
7:522-526
22. Wied U, Nilsson T, Knudsen JB, Sprechler M, Johansen A
(1985) Post operative survival of patients with potentially curable cancer of the colon. Dis Colon Rectum 28:333-335
Dr. J. P. Ozoux
Service de Chirurgie Digestive
H6pital Bretonneau
2 Bd Tonnell6
F-37044 Tours Cedex
France

Int J Colorect Dis (1990) 5:31-36

Colb~
Disease

9 Springer-Verlag 1990

The role of transient internal sphincter relaxation in faecal incontinence?


Wei Ming Sun, N.W. Read, P.B. Miner, D.D. Kerrigan and T.C. Donnelly
Sub Department of Gastrointestinal Physiology and Nutrition, The University, Sheffield, UK
Accepted: 4 October 1989

Abstract. Twenty-five (18%) of 140 incontinent patients


and 6 (17%) of 35 normal controls showed episodes of
spontaneous internal sphincter relaxation during 30 rain
multiport manometric and electromyographic recording
under resting conditions. The episodes lasted at least 15 s
and reduced the pressure in the outermost anal channels
by at least 20 cm of water. Patients exhibited more
episodes of relaxation than controls (4.3 0.6 vs 2.3 + 0.2
per subject; mean _+SEM; p < 0.05) and the pressures fell
to lower values (19 + ] vs 42 5 cm water, p < 0.01), but
the duration of relaxation was not significantly different
(53 + 4 vs 40 + 7 s). Episodes o f spontaneous relaxation
were associated with simultaneous rectal contractions in
33% of the normal subjects and 45% of incontinent patients. Unlike normal subjects, most of the episodes of
transient relaxation recorded in the incontinent group
were not associated with compensatory increases in the
electrical activity of the external anal sphincter (77% vs
1 7 % ; p < 0.05). Over 50% of the incontinent patients who
showed spontaneous relaxation also showed post squeeze
or post-strain IAS relaxations whereas these were seen in
less than 6% of the normal subjects with spontaneous
relaxation. The rectal volumes, required to elicit anal relaxation (10_+0 vs 28_+7ml; p < 0 . 0 5 ) , to incude sustained relaxation (60 _+8 vs 82 _+5 ml; p < 0.05), to elicit a
sensation of 'wind' (19-+3 vs 27-+8 ml; p < 0 . 0 5 ) and to
cause a desire to defaecate (36_+4 vs 63_+9 ml; p < 0 . 0 5 )
were all lower in the incontinent patients who showed
spontaneous relaxations than in the incontinent control
group. In conclusion, spontaneous relaxation of the internal sphincter m a y be an important factor leading to
faecal incontinence in patients with a sensitive rectum,
especially as they tend to occur in these subjects in the
absence of a compensatory increase in external sphincter
activity.

Introduction
The rapid entry o f faeces into the rectum is thought to
induce reflex relaxation of the internal anal sphincter,

and a compensatory contraction of the external anal


sphincter, which maintains the anal pressure barrier and
p r e v e n t s incontinence. During routine multiport
m a n o m e t r y and electromyography carried out under
resting conditions on 140 patients with faecal incontinence, we found that 18% exhibited spontaneous
episodes of sphincter relaxation. Less than half of these
episodes were associated with increases in rectal pressure.
Does the presence of spontaneous sphincter relaxations
make the patients more susceptible to faecal incontinence? This paper attempts to answer that question by
comparing; (i) the features of spontaneous relaxations in
incontinent patients and in normal subjects, and (ii) the
responses to provocative tests of sphincter function in
incontinent patients with and without spontaneous
sphincter relaxations during the period of basal recording.

Material and methods

Subjects
A total of 140 patients (20 male, 120 female; aged between 19 and
80 years, median = 59 years), referred for sphincter function tests
because of faecal incontinence, and 35 healthy controls (15 male, 20
female; aged between 20 and 60 years, median =48 years) were
studied. Of these, 25 patients (17 female, 8 male, aged between 34
and 76 years, median = 60 years) and 6 controls (4 female, 2 male,
aged between 20 and 52 years, median=45 years) demonstrated
prolonged (> 15 s) and profound (>20 cm water) spontaneous
sphincter relaxations. These were termed the incontinent and norreal study groups respectively.
All of the female patients and normal subjects who exhibited
transient relaxations had had children though the average number
of children was higher in the incontinent study group than in the
normal study group (3.4 vs 2.0). These were a group of patients with
idiopathic faecal incontinence, presumably neurogenic in origin;
patients with obvious aetiological factors such as sphincter trauma, systemic neuropathy or CNS disease were excluded. All patients were incontinent of stool at least once a month. Four of the
patients in the incontinent study group (16%) had had a hysterectomy, 2 (8%) had a solitary rectal ulcer, 2 (8%) had prolapse and
2 (8%) had diabetes mellitus. By comparison, 8 patients in the

32
incontinent control group (7%) had undergone hysterectomy, 6
(5%) had solitary rectal ulcer, 26 had prolapse (23%) and 4 (3%)
had diabetes mellitus.
None of the normal subjects had any disturbance in bowel
activity or any condition that could affect anal function and were
recuited from university staff and students.
Each of the subjects was informed of the nature and aims of the
tests and gave their consent for the study to be carried out. The
protocol was approved by the Ethical Sub-committee of the
Sheffield Area Health Authority (Southern District) in September
1985.

Methods
With the subject lying in the left lateral position with the hips flexed
to 90 degrees, a manometric probe, consisting of a polyvinyl 7-1umen tube with an external diameter of 4 mm and bearing a terminal
inflatable balloon (Durex Dry, LRC Products Ltd. London, U.K.)
was inserted into the rectum. When correctly positioned, manometric side holes were situated in the anal canal at approximately 0.5,
1.0, 1.5, 2.0 and 2.5 cm from the anal verge and in the rectum at
4.5 cm from the anal verge and the outermost pole of the balloon
was 8 cm from the anal margin. The side holes were perfused with
water at a rate of 0.4 ml per minute by a low compliance pressurised
perfusion system (Mui, PIP 2, Mississauga, Toronto, Canada), and
pressures were measured by means of pressure transducers
(Statham P23ID, Oxnard, California, USA), situated in each perfusion line and connected via amplifiers to a multichannel chart
recorder (Hewlett Packard, 7758A, Waltham, Mass. USA).
The electrical activity of the sphincter was recorded using a
bipolar electrode [1, 2], consisting of two trimel coated wires (diameter = 0.025 ram) with their ends bared, hooked and offset to avoid
electrical contact. The wires were inserted into the superficial external anal sphincter (EAS) or into the groove between the EAS and
the internal anal sphincter (IAS) inside a fine gauge hypodermic
needle, which was subsequently withdrawn, leaving the hooked
ends of the wires in situ. The free ends of the wires were bared and
attached to an amplifier (Differential type 21C01 URO-DISA,
Copenhagen, Denmark; frequency range I H z - 1 0 KHz (-3 dB)),
which was connected via an integrator (AC to RMS convertor;
Analog Devices AD536, U.K.) to the chart recorder. Both raw and
integrated records (170 ms time constant) were displayed on the
chart recorder. The activity of the IAS was represented on the raw
E M G record as regular oscillations, which occurred at a frequency
of between 10 and 20 per minute and increased in amplitude as the
activity of the muscle increased [3]. The activity of the EAS appeared on the E M G record as successive spikes that increased in
both amplitude and frequency as the activity of that muscle increased, and appeared on the integrated record as an elevation
above the baseline.

Protocol
Anorectal motility was recorded under resting conditions for
30 min. Episodes of spontaneous relaxation were identified during
this time as decreases in anal pressure of at least 20 cm water,
occurring in at least three anal channels and lasting at least 15 s
before returning to the original baseline. Then the subject was instructed to contract the anal sphincter as hard as he or she could for
a period of 1 min. This was repeated two more times with gaps of
at least a minute between the contractions. The rise in pressure
during each conscious contraction was measured and any decrease
in pressure below resting levels, that occurred immediately after the
conscious contraction was noted. Then the rectal balloon was serially inflated with 10, 20, 40, 60 and 100 ml of air. Each inflation was
maintained for 1 rain and a gap of the least 1 min was allowed
before the next inflation. Rectal distension usually causes a burst of

activity in the EAS, that is often associated with a transient spike in


anal pressure. This is followed by a decrease in anal pressure, best
seen in the innermost anal channels and caused by relaxation of the
internal anal sphincter. The anal relaxation is usually accompanied
by a phasic increase in rectal pressure. Deflation of the balloon,
particularly after inflation with higher volumes, elicits a brief contraction of the EAS, followed by a more prolonged increase in
pressure, associated with increased activity in the IAS. The existence
of these phenomena at each distending volume was noted as well as
the pre-inflation pressure, the residual pressure and the post-inflation or rebound pressure in each channel during each inflation. In
addition, subjects were asked to report their subjective sensations
during each inflation, and the lowest distending volumes at which
subjects perceived the balloon and reported a feeling of a gas in the
rectum (wind), a desire to defaecate, and a sensation of pain were
recorded.
Next, after a rest period of at least 10 minutes, the subject was
instructed to blow up a batloon (Sainsbury's Partytime Round Balloons, 747/350, J. Sainsbury plc., London, U.K.) and to strain as if
to defaecate. Each manoeuvre was repeated on three occasions,
separated by gaps of at least a minute. The increases in intra-abdominal pressure, induced by these manoeuvres, were observed as
increases in rectal pressure and were associated with compensatory
increases in EAS electrical activity, which usually maintained the
anal pressure above the rectal pressure so that continence was preserved. The presence of an external sphincter response and the
existence of a relaxation in pressure below basal values, occurring
immediately after the increase in intra-abdominal pressure, were
noted.

Statistical analysis
An analysis of variance was used to evaluate differences in sphincter
pressures and in the responses to rectal distension or increases in
intra-abdominal pressure between the groups. Mann-Whitney " U "
test was used to analyse the sensory data. Chi-square test was used
to compare the percentages of subjects in each group that demonstrated specific phenomena.

Results

Basal recordings
More episodes of spontaneous relaxation were recorded
in p a t i e n t s t h a n in n o r m a l s u b j e c t s (108 in 25 p a t i e n t s a n d
14 in 6 n o r m a l s u b j e c t s ; a v e r a g e : 4.3 v e r s u s 2.3; p < 0.05).
A l t h o u g h t h e fall in p r e s s u r e in t h e p a t i e n t s w a s n o t sign i f i c a n t l y d i f f e r e n t f r o m t h a t in t h e c o n t r o l s (50 +_ 1 vs
37 -t- 4 c m w a t e r , m e a n _ + S E M ; p > 0.05), b o t h t h e m a x i m u m a n d t h e m i n i m u m b a s a l p r e s s u r e s w e r e l o w e r in t h e
p a t i e n t s t h a n in n o r m a l s u b j e c t s ( T a b l e 1) a n d c o n s e quently the residual pressure during spontaneous relaxa t i o n was m u c h l o w e r in t h e i n c o n t i n e n t p a t i e n t s t h a n in
t h e n o r m a l s u b j e c t s (19 + 1 vs 4 2 _ 5 c m w a t e r , p < 0.01).
I n 4 p a t i e n t s , b u t n o n o r m a l subjects, t h e a n a l p r e s s u r e
d u r i n g e p i s o d e s o f s p o n t a n e o u s r e l a x a t i o n fell to levels
t h a t w e r e e q u a l to t h o s e r e c o r d e d in t h e r e c t u m , a n d
l e a k a g e o f f l u i d o c c u r r e d at t h e s e t i m e s in 2 o f t h e s e
patients. The duration of spontaneous relaxation was not
s i g n i f i c a n t l y l o n g e r in p a t i e n t s t h a n in c o n t r o l s (47 +_ 5 vs
40_+5 s).
Episodes of spontaneous relaxation usually commenced within 3 to 5 min of insertion of the probe, when
less t h a n 10 m l f l u i d h a d b e e n i n f u s e d v i a t h e m a n o m e t r i c

33
Channel crnH20

cmH20

120] ~
1

3
4
S

_--

Channel cmH20

80]

120]

40"

80]

40 ~

1 __

40 ~
1201
40 J "
120

~
~

0
80]
0
80]

40 ] ~

80]

,o]

_ .

EMG 140juV] ~

] '~,~t~

. ~

Integrated
EMG

EMG 140)JV]

Integrated
EMG
I

1 min

1 rain

Fig. 1. Multiport recordings of anorectal pressures and the electrical activities of the EAS and IAS during and after an episode of
spontaneous anal relaxation in a normal subject (left) and an incontinent patient (right). Channels 1 to 6 represent ports situated 0.5,
1.0, 1.5, 2.0, 2.5 and 4.5 cm from the anal verge. The relaxation
lasted longer in the patient and was not associated with a compensatory increase in external sphincter activity

Fig. 2. Recordings of anorectal pressures and the electrical activity


of the sphincter during maximum conscious contraction of the external sphincter in a patient, who exhibited spontaneous relaxation.
Channels 1 to 6 represent ports, situated 0.5, 1.0, 1.5, 2.0, 2.5 and
4.5 cm from the anal verge. The post squeeze relaxation is associated with suppression of the IAS slow wave, but no decrease in the
electrical activity of the EAS below baseline values

sideholes. T h e r e were no significant differences in the


number of episodes of spontaneous relaxation between
the first 15 m i n a n d the s e c o n d 15 m i n o f b a s a l r e c o r d i n g ,
suggesting t h a t the p h e n o m e n o n was n o t i n d u c e d b y
the presence o f a n i n c r e a s i n g v o l u m e o f w a t e r in the
rectum.
I n t e r n a l s p h i n c t e r slow waves were o b t a i n e d o n the
electrical r e c o r d s f r o m 9 o f the i n c o n t i n e n t p a t i e n t s w h o
e x h i b i t e d s p o n t a n e o u s r e l a x a t i o n , a n d f r o m 6 o f the n o r m a l c o n t r o l s w h o also e x h i b i t e d s p o n t a n e o u s r e l a x a t i o n ,
a n d were a t t e n u a t e d in all o f these subjects d u r i n g s p o n t a n e o u s r e l a x a t i o n (Fig. 1). I n c o n t r a s t , the electrical activity o f the E A S was n o t decreased, b u t s h o w e d c o m p e n s a t o r y increases in activity d u r i n g 83 % o f the e p i s o d e s o f
s p o n t a n e o u s s p h i n c t e r r e l a x a t i o n in n o r m a l subjects, b u t
o n l y 2 3 % o f the e p i s o d e s in i n c o n t i n e n t p a t i e n t s
(p < 0.05) (Fig. i). T h i r t y p e r cent o f the e p i s o d e s o f s p o n t a n e o u s r e l a x a t i o n in n o r m a l subjects a n d 4 5 % episodes
in i n c o n t i n e n t p a t i e n t s o c c u r r e d in a s s o c i a t i o n with increases in rectal pressure.

Table 1. Anorectal pressures (cm water) recorded under resting


conditions, and during a maximum contraction and during rectal
distension with 100 ml air
Normal subjects

Incontinent patients

Control
n=29

Study
n=6

Control
n=115

Study
n=25

76_+ 4
34_+ 3

80+ 6
36_+ 4

49_+ 4 a
20_+ 2"

50_+4"
24_+2a

88

90_+8~

Anal pressure during


rectal distension
Pre-inflation
74_+ 6 74-+14
Residual
28 4- 5 24_+ 5
Rebound
100-+ 9 104-+27

52+ 8 a
23 -+ 3
54-+ 8"

60_+8
23 -+4
67 a

Rectal pressure during


rectatdistension
17_+ 1

29_+ 3"

48

Anal pressure at
Max. basal
Min. basal
Squeeze pressure

188

179-+17

20-+ 3

ab

Results are expressed as mean_+ SEM


a Results significantly different from the normals
b Results significantly different from the patient controls

Squeeze pressures
T h e m a x i m u m sequeeze pressures at all a n a l p o r t s were
l o w e r in the i n c o n t i n e n t p a t i e n t s t h a n in the n o r m a l subjects ( p < 0 . 0 5 ) (Table 1), b u t there were n o significant
differences b e t w e e n either the p a t i e n t s o r the n o r m a l subjects w h o e x h i b i t e d s p o n t a n e o u s r e l a x a t i o n s a n d t h o s e
w h o d i d n o t (Table 1). F i f t y - t w o p e r cent o f the incontin e n t patients, w h o s h o w e d t r a n s i e n t s p h i n c t e r relax-

ations, also s h o w e d p o s t squeeze r e d u c t i o n s in s p h i n c t e r


p r e s s u r e to values t h a t were at least 5 c m w a t e r l o w e r
t h a n b a s a l p r e s s u r e a n d lasted m o r e t h a n 6 s (Fig. 2).
These p o s t squeeze r e l a x a t i o n s were a s s o c i a t e d with a
r e d u c t i o n in I A S activity, b u t no decrease in the electrical
activity o f the E A S b e l o w b a s a l values. P o s t squeeze rel a x a t i o n s were also seen in 15 % o f the i n c o n t i n e n t c o n t r o l

34
Table 2. Responses to rectal distension
Normal subjects

Incontinent patients

Control

Control ~ Study

Study

Threshold for anal relaxation (ml)


Initial
19_+4
14+ 2
Sustained
81-+6
77_+11
Relaxation at 10 ml distension
Duration (s)
10_+2
Threshold for sensation
Perception
Wind
Desire to defaecate

(ml)
12 _ 1
29_+3
66_+6

28-+7
82-+5

60-{-8 ab

104-0 "b

9_+ 3

9-+2

22-t-5 =b

13 _ 2
25-+ 8
53-+11

15 + 5
27-+8
63_+9

134-1
194-3 =b
3 6 - t - 4 ab

Data are expressed as mean_+SEM


= Results significantly different from the normal study subjects
b Results significantly different from the normal subjects and incontinent patients who do not exhibit spontaneous relaxations
~ Patients who showed no anal relaxation to rectal distension were
excluded

group (p<0.01, c o m p a r e d with the incontinent study


group) and in 2 normal subjects (p < 0.01); one of w h o m
showed spontaneous relaxations.

Responses to rectal distension


Inflation of the rectal balloon in all of the normal subjects
and incontinent patients, who exhibited spontaneous relaxations, caused reductions in pressure at all anal ports,
increasing in amplitude and duration as the inflation volume increased. Rectal distension did not elicit anal relaxation in 30% of the patients in the incontinent control
group; this was because the internal sphincter tone was
absent in these patients [4]. The distension volumes required to cause IAS relaxation, and to induce relaxation
that was sustained for the duration of the distension
(1 min), were significantly lower in the incontinent study
group than in the patients in the incontinent control
group, who exhibited internal sphincter tone at rest
(Table 2). A similar trend was seen in the normal subjects
who exhibited spontaneous relaxations. The duration of
sphincter relaxation in response to distending volumes of
between 10 and 60 ml was significantly longer in the incontinent study group than in the incontinent control
group (Table 2), though the corresponding values in the
two groups of normal subjects were very similar. There
were no differences in pre-inflation, post-inflation and
residual pressures between the study groups and control
groups, although pre-inflation and post-inflation pressures were significantly lower (p < 0.05) in the incontinent
patients c o m p a r e d with the normal controls (Table 1).
The rectal pressures during rectal distension were significantly higher in the incontinent study group compared with the incontinent control group (Table 1). In
m a n y instances, the rectal pressures in the incontinent
patients exceeded the anal pressures, favouring incontinence. Seventy-seven per cent of the patients who exhibited spontaneous relaxations and 52% of incontinent

controls (p<0.05) leaked the perfusion fluid f r o m the


rectum during rectal distension. N o n e of the normal subjects leaked.
All of the normal subjects showed increases in the
electrical activity of the external anal sphincter in response to rectal distension. These changes were often
brief, but became more prolonged as the distension volume and the duration of sphincter relaxation increased.
Corresponding increases in external sphincter electrical
activity were also seen in all except 2 of the incontinent
patients who exhibited spontaneous relaxations.
Incontinent patients who exhibited spontaneous relaxatations, perceived sensations of wind and a desire to
defaecate at volumes that were much lower than the
thresholds in the incontinent control group, and also
lower than the corresponding values in the normal subjects (Table 2). There were no significant differences in
sensation between the two normal groups. Seventy-seven
percent of patients in the incontinent study group, but
only 48% in the incontinent control group, perceived
pain during rectal distension with 100 ml air (p<0.05).
Similarly, 50% of the normal study group c o m p a r e d with
24% of the normal control group (p>0.05) perceived
pain at 100 ml rectal distension.

Anorectal changes during increases


in intra-abdominal pressure
All normal subjects, including those that exhibited spontaneous anal relaxations, increased the electrical activity

Channel cmH20

8o]
8o]
8o]
0

-----A/~

--

-__]~.

--

o
EMG

_L_
140,uV]

Integrated
EMG

__j~
i

1 rain

Fig. 3. Recording of anorectal pressures at ports situated 0.5, 1.0,


1.5, 2.0, 2.5 and 4.5 cm from the anal margin (channels I to 6) and
the electrical activity of the sphincter complex in a patient in the
incontinent study group during straining. The post strain relaxation
is associated with the reduction in the IAS slow wave, but no
decrease in electrical activity of EAS below baseline values

35
of the external anal sphincter when they attempted to
inflate a balloon, and all except 6 females increased the
EAS electrical activity when they strained. These increases in EAS activity were accompanied by increases in
anal pressure above rectal pressure so that continence
was maintained. Thirty-six per cent of the incontinent
patients failed to show an EAS response to increases in
intra-abdominal pressure, caused by inflating a balloon,
and 82% failed to show an EAS response to straining, but
there were no significant differences between those patients who showed spontaneous relaxation and those who
did not.
Fifty-four per cent of the patients who showed spontaneous sphincter relaxation also had a post strain reduction in anal pressure of at least 5 cm water, lasting at least
6 s (Fig. 3). This p h e n o m e n o n was only observed in 10%
of the incontinent control group (p < 0.01) and 1 normal
subject (p < 0.001). In each case, the reduction in pressure
was not associated with any reduction in EAS electrical
activity below basal values, but was associated with attenuation of IAS activity. Seventy-five percent o f the patients who had post-strain relaxation also exhibited postsqueeze relaxations.

Discussion
Spontaneous relaxations of the anal sphincter occur in
a b o u t 18% of normal subjects and incontinent patients,
although they are more frequent in the incontinent patients. Simultaneous myoelectrical recordings of the
sphincter complex indicate that this p h e n o m e n o n is
caused by a relaxation of the internal anal sphincter. IAS
relaxations can be normally evoked by rectal distension,
such as might be caused by the entry of faeces into the
rectum, or by rectal contraction [3, 5-7]. However, less
than 50% of the episodes of spontaneous sphincter relaxation, recorded in this study, were associated with a corresponding increase in rectal pressure. Thus, the majority
of the episodes appeared to be due either to a u t o n o m o u s
losses of internal sphincter tone or to changes in rectal
contractile activity or content that are too small to induce
a recordable change in rectal pressure [8, 9]. Our observation that the rectum is abnormally sensitive to distension
in these patients is compatible with this theory. N a u d y
and his colleagues [10] have shown that relaxation of the
sphincter can be induced by distension of more proximal
regions of the colon, so perhaps the 'spontaneous relaxations' are related to contractile activity and movements
of the contents in the more proximal colon. It is unlikely
that spontaneous relaxations are an artefact, caused by
the volume of fluid infused via the manometric channels,
for the following reasons: (i) the relaxations commenced
within the first 5 rain of recording When less than 10 ml
fluid had been infused and did not increase in frequency
as more fluid was infused, (ii) similar p h e n o m e n a have
been reported from studies that have used ambulatory
anorectal manometry. In one study 15 normal subjects
showed an average of 7 spontaneous relaxation per hour
and those were associated with a feeling of rectal flatus
[11], (iii) patients with spontaneous relaxations also show

a much higher incidence of post-strain and post-squeeze


relaxations, indicating a more sensitive IAS.
Spontaneous IAS relaxation is probably o f little or no
significance in normal subjects, because simultaneous
contraction of the EAS maintains the anal pressure barrier in most of them and guards against incontinence, It
is more important in the incontinence patients, who have
lower resting and squeeze pressures and a lower incidence
of compensatory increases in EAS activity.
The results of sphincter function tests showed that
incontinent patients, who exhibited spontaneous relaxation, were more sensitive to rectal distension than the
remainder of the incontinent patients. Rectal distension
also induced more prolonged sphincter relaxation in response to lower volumes of rectal distension, and gave
rise to higher rectal pressures in the incontinent study
group compared with the incontinent control group. Similar trends were observed in the results from the normal
subjects, though it must be emphasised that normal subjects did not exhibit spontaneous relaxations to the same
extent as the patients. A more striking feature of the
incontinent study group was the markedly increased incidence of post squeeze and post strain sphincter relaxations.
Thus, our results suggest that patients who exhibit
transient anal relaxations are more susceptible to incontinence in that they have an unstable internal anal sphincter, and enhanced rectal contraction and IAS relaxation
in response to rectal distension. This predisposition is
exacerbated by the absence of a compensatory EAS contraction. Even patients who show an increase in EAS
activity in association with IAS relaxation could be at
risk. Since the external sphincter is unable to compensate
for a relaxation of the IAS during sleep [12], transient
relaxation could cause episodes of nocturnal incontinence.

References
1. Basmajian JV, Stecko G (1962) A new bipolar electrode for
electromyography. J Appl Physiol 17:849
2. Haynes WG, Read NW (1982) Anorectal activity in man during
rectal infusion of saline. A dynamic assessment of the anal
continence mechamism. J Physiol 330:45-56
3. Monges H, Salducci J, Naudy B, Raniere F, Gonella J, Bouvier
M (1980) The electrical activity of the internal anal sphincter:
a comparative study in man and cat. In: Christensen J (ed)
Gastrointestinal motility. Raven Press, New York, pp 495-501
4. Sun WM, Donnelly TC, Read NW (1989) Impaired internal
anal sphincter in a subgroup of patients with idiopathic fecal
incontinence. Gastroenterology 97:130-135
5. Callaghan RP, Nixon HH (1984) Megarectum: physiological
observation. Arch Dis Child 39:153-157
6. Denny-Brown D, Robertson EG (1935) An investigation of the
nervous control of defaecation. Brain 58:256-310
7. Ustach T, Tobon F, Hambrecht T, Schuster MM (1970) Electrophysiological aspects of sphincter function. J Clin Invest
49:41-48
8. Schuster MM, Hendrix TR, Mendeloff AI (1963) The internal
anal sphincter response: Manometric studies on its normal
physiology, neutral pathways, and alteration in bowel disorders. J Ctin Invest 42:196-207

36
9. Meunier P, Mollard P (1977) Control of the internal anal
sphincter (manometric study with human subjects). Pflfigers
Arch 370:233-239
10. Naudy B, Planche D, Monges B, Salducci J (1983) Relaxations
of the internal anal sphincter elicited by rectal distension and
extrarectal distension in man. In: Roman C (ed) Gastrointestinal motility. MTP Press Limited, Lancaster, pp 451-458
11. Miller R, Lewis GT, Bartolo DCC, Cervero F, Mortensen
NJMcC (1988) Sensory discrimination and dynamic activity in
the anorectum: evidence using a new ambulatory technique. Br
J Surg 75:1003-1007

12. Whitehead WE, Orr WC, Engel BT, Schuster MM (1982) External anal sphincter response to rectal distension: learned response of reflex, Psychophysiology 19:57-72
Prof. N. W. Read
Sub Department of Gastrointestinal
Physiology and Nutrition
Floor K
Royal Hallamshire Hospital
Sheffield S10 2JF
UK

lnt J Colorect Dis (1990) 5:37-40

Col6ree|al
Disease

9 Springer-Veflag 1990

Rectal compliance: a critical reappraisal


R . D . M a d o f f ~, W . J . Orrom 2, D . A . Rothenberger 2 and S . M . Goldberg 2
1 University of Massachusetts Medical School and Worcester MemorialHospital, Worcester, Massachusetts, USA
2 Division of Colon and Rectal Surgery University of Minnesota Hospital and Clinic, Minneapolis, Minnesota, USA
Accepted: 15 September 1989

Abstract. Compliance is a widely measured parameter of


rectal function. Its value is determined clinically by
recording pressure changes associated with volume infusion into a rectal balloon. This paper examines the inherent assumptions of the rectal balloon technique and discusses several of its shortcomings. A stricter definition of
rectal compliance is needed, and in vivo compliance
should be correlated with the directly measured mechanical properties of the rectal wall.

The balloon technique for measuring rectal distensibility rests on these assumptions:
1. The rectum can be modelled as a closed cylinder.
2. Rectal size does not influence measured rectal compliance.
3. Extrarectal tissues do not contribute to measured
rectal compliance.
4. The rectum is mechanically passive.
Each assumption is discussed in greater detail below.

Introduction
To maintain continence, the rectum must serve as a faecal
reservoir. This storage function requires the rectum to be
distensible to accommodate the incoming faecal load. In
the surgical literature, distensibility is most commonly
measured as rectal compliance, which is defined as the
change in rectal volume per unit change in rectal pressure.
In most laboratories, rectal compliance is determined
with a rectal balloon technique in which serial volumes of
fluid are infused to generate a pressure-volume curve.
The proctometrogram, a technical refinement of this
method, is based on the same principles [1]. Using these
techniques, decreased rectal compliance has been demonstrated in such pathological states as active ulcerative
colitis [2] and radiation proctitis [3].
Despite the apparent simplicity of determining rectal
compliance, different investigators report a wide range of
normal values (Table 1) [2, 4-7]. We believe these discrepancies reflect fundamental theoretical defects inherent in the rectal balloon technique that call its accuracy
and relevance into question.
The hypothesis underlying the techniques of determining rectal compliance suggests that: the mechanical
properties of the rectal wall are altered by pathologic
processes, and that, these alterations adversely affect faecal continence, and can be measured with reasonable accuracy in vivo.

Is it legitimate to model the rectum as a closed cylinder?


It is obvious that no anatomical barrier exists between the
lumina of the rectum and sigmoid colon, nor indeed has
any functional barrier been described in this location. To
measure rectal "compliance," a balloon is necessary to
prevent reflux of the infused fluid into the colon. It is
unknown whether such reflux plays a physiological role
in faecal storage between evacuations. With the balloon
technique, an arbitrary proximal limit to the rectum is
assigned where none, in fact, exists. Furthermore, because the balloon technique is "blind," the proximal limit
of the balloon's axial expansion during volume infusion
is not legitimate. This point becomes the newly assigned
" t o p " of the rectum.
Consider two rectums with mechanically identical
walls. Should axial expansion of the rectal balloon be
Table 1. Normal rectal compliance
Author, year

Compliance

(ml/cm H2O)
Suzuki, 1982 [4]
Varma, 1985 [3]
Roe, 1986 [5]
Womack, 1986 [6]
Rao, 1987 [2]
Allan, 1987 [7]
" Calculated from published graph

15.7
9.0
5.1
6.6"
11.5
5.9

38
Does rectal size influence measured compliance?

Fig. 1 A, B. Effect of rectal balloon axial distension on measured


rectal compliance. Even if rectums A and B are mechanically identical, measured compliance in A will be greater

Fig. 2A, B. Effect of rectal size on measured rectal compliance.


Regardless of its wall stiffness, rectum A will have a higher measured compliance than B

limited in midrectum (e.g. by a kink or stricture), radial


rectal distension will begin at low volumes and measured
compliance will be low. Conversely, if an obtuse rectosigmoid junction permits expansion of the balloon into the
distal sigmoid, radial distension of the rectum will occur
only after a large volume has been infused and the measured compliance will appear to be high (Fig. 1). Indeed,
the balloon infusion technique rests squarely on the assumption that the balloon's axial expansion will stop precisely at the rectosigmoid junction. This supposition has
never been tested. It is worth considering that, given an
adequately compliant balloon, even a lead pipe would
appear to be compliant by this technique; the infused
volume could be accommodated without a corresponding rise in pressure by simple axial expansion of the balloon.

Patients with acquired megacolon have been found to


have high rectal compliance [1]. This stands in contrast to
the operative observation that acquired megarectum frequently appears to be thick wailed and, if anything, stiffer
than normal rectum. The discrepancy is explained by the
different resting volumes of normal rectum and megarecturn and by the confusion that exists between the concepts of capacity and compliance.
Given a very large rectum, a very large volume of
fluid must be infused to initiate rectal distension. The
volume of fluid adequate to distend a normal sized rectum would not even begin to stretch the walls of a
megarectum (Fig. 2).
Conversely, the volume of fluid needed to distend a
megarectum would, at a minimum, stretch a normal rectum beyond its usual physiological range, even to the
point of rupture. Once again, given a large enough size,
a totally rigid rectum would appear to be highly compliant with the balloon technique.
A related problem of size discrepancy has been addressed by pulmonary physiologists. The in vivo compliance of the pediatric lung is less than that of the adult
lung, even though the distensibility of the lung tissue itself
is identical [8]. This disparity is due to the differences in
lung size, and is reconciled by normalizing the measured
compliance to lung volume:
Specific compliance =

measured compliance
resting volume

(1)

In the lung, the resting volume is easily determined and is


known as the "functional residual capacity". The specific
compliance of normal lungs is similar irrespective of lung
size [8]. Unfortunately, because the rectum has no functional proximal limit, there is no such thing as a resting
rectal volume, and specific rectal compliance therefore
can not be calculated.

Do extrarectal tissues influence measured rectal


compliance?
The rectum is surrounded by extrarectal fat, vascular and
nervous tissues, genitourinary structures, and the bony
pelvis. Determining rectal compliance with the balloon
technique actually measures several compliances in series: the compliance of the balloon, the rectum, and the
extrarectal tissues. The relationship of these serial compliances is described by this equation:
1
C .......

d - - Cballoon "~- Crectu~ --t- Cext . . . . . tal ti . . . .

(2)

where C = compliance
It is widely accepted that balloon compliance must not
significantly alter the measurement of rectal compliance,
and to prevent such alteration all investigators use highly
compliant rectal balloons. As can be seen from equation
(2), a very large Cba.oon term causes its reciprocal to be-

39
Table 2. Compliance and tissue elastic properties: a comparison of active inflammatory
bowel
Compliance (mls/mm Hg)
Initial modulus (kg/cm 2)
Stiff modulus (kg/cm 2)
come negligibly small. One is then left with this equation:
1

Cmeasured

Crecturn

Cextrareetal

(3)
tissue

The relative values of Crectum and Cext..... t,lt~.... are unknown. Nonetheless, in all probability, it is not legitimate
simply to ignore the t e r m J/Cex t..... talti . . . . . Indeed, it is
entirely possible that, given the a n a t o m y of the bony
pelvis surrounding the rectum, the l/Crectum t e r m is negligibly small in equation (3) and that the measured compliance actually reflects the size, anatomy, and composition
of the extrarectal pelvic structures.
Is the rectum

mechanically

passive?

It has long been known that the rectum passively accommodates a volume load [9]. Thus, if a balloon is inserted
into the rectum and inflated, the initially recorded rise in
rectal pressure gradually returns to baseline over the
course of one to two minutes (Fig. 3). Current measurement techniques for rectal compliance do not take this
p h e n o m e n o n into account.
A measured decrease in rectal compliance m a y be due
to structural changes in the bowel wall, or simply to increased smooth muscle tone in the rectum. R a o demonstrated that the diminished rectal compliance seen in patients with active ulcerative colitis returns to near normal
levels when the disease is quiescent [2]. R a o also found
that rectal reactivity, defined as the peak minus steady
state rectal pressure in response to rectal balloon inflation, was significantly greater in active ulcerative colitis
than in normal controls or in patients with quiescent
disease. These findings strongly suggest a role of increased muscle tone or muscle spasm in the genesis of
diminished rectal compliance in ulcerative colitis.

0tj'"

Rectal Accommodation

o
I
0

I
1

I
2

Active inflammatory
bowel disease (n = 5)
mean (range)

Controls
(n = 5)
mean (range)

3.11 (0.84 - 6.24)


0.016 (0.006- 0.440)
3.600 (1.20 -25.10)

7.00 (6.03 - 9.38)


0.014 (0.005- 0.034)
6.600 (1.920-15.700)

0.043
N.S.
N.S.

The implication of R a o ' s findings is clear: a "stiff"


rectum in a patient with active ulcerative colitis is insufficient grounds for the rectum's removal, as the stiffness
m a y well be reversible. R a o ' s findings also underline the
confusion inherent in current usage of the term "compliance" with reference to the rectum. To some, compliance
is a mechanical property of the rectal wall. To others, it
simply implies the results of a functional measurement
made on the rectum, usually with the balloon technique.
We are unaware of any studies that correlate rectal compliance determined in vivo with the mechanical properties of the rectal wall determined in vitro.
In some preliminary work, we have compared rectal
compliance measured in vivo (using a standard balloon
p r o c t o g r a m technique as described by Preston and Lennard-Jones [10]), with tissue elasticity measured with an
Instron tensiometer. This ex vivo method measures the
"modulus of elasticity," i.e., the relationship between
stress and strain for a particular material. Stress is the
a m o u n t of force applied to a material and strain is a
measure of the change in length of a material following
an applied stress. The larger the number, the stiffer the
material. The initial modulus refers to this relationship
when measured during the initiation of stress (stretch),
and the stiff modulus refers to measurements made just
prior to breakage of the material. While in vivo measurement of compliance demonstrated low values in active
inflammatory bowel disease when compared with controls, no such differences were seen comparing these two
groups using ex vivo measurements of elasticity (Table 2).

Conclusion

We do not believe that the balloon infusion technique


accurately measures physical alterations in the rectal
wall. At the same time, we do not believe that such
changes are necessarily immeasurable, nor that they lack
functional importance.
In order to improve on the current techniques of determining rectal compliance, in vivo measurement must
be based on a method that ensures and accurately determines radial rectal distension alone. Measured rectal distensibility must be normalized to the rectum's resting
dimensions. Distensibility measured in vivo must be correlated with distensibility measured in vitro. Once these
studies have been performed, the true meaning of rectal
compliance can be ascertained.

Time (rain.)

Fig. 3. Pressure response to inflation of a rectal balloon. Intrarectal


pressure decreases with time

Acknowledgement. The authors would like to thank O. Douglas


Waugensteen, Ph.D., for his helpful comments and suggestions.

40

References
1. Varma JS, Smith AN (1986) Reproducibility of the proctometrogram. Gut 27:288-292
2. Rao SSC, Read NW, Davison PA, Bannister JJ, Holdsworth
CD (1987) Anorectal sensitivity and responses to rectal distention in patients with ulcerative colitis. Gastroenterology
93:1270 1275
3. Varma JS, Smith AN, Busuttil A (1985) Correlation of clinical
and manometric abnormalities of rectal function following
chronic radiation injury. Br J Surg 72:875-878
4. Suzuki H, Matsumoto K, Amano S, Fujioka M, Hozumi M
(1980) Anorectal pressure and rectal compliance after low anterior resection. Br J Surg 67:655-657
5. Roe AM, Bartolo DCC, Mortensen NJM (1986) Diagnosis and
surgical management of intractable constipation. Br J Surg
73:854-861
6. Womack NR, Morrison JFB, Wiliams NS (1986) The role of
pelvic floor denervation in the aetiology of idiopathic faecal
incontinence. Br J Surg 73:404-407

7. Allan A, Ambrose NS, Silverman S, Keighley MRB (1987)


Physiology study of pruritus ani. Br J Surg 74:576-579
8. Comroe JH, JR (1974) Physiology of Respiration: An Introductory Text. Year Book Medical Publishers, Chicago
9. Duthie HL (1975) Dynamics of the rectum and anus. Clin
Gastroenterol 4:467-477
10. Preston DM, Barnes PRH, Lennard-Jones JE (1983). Proctometrogram: does it have a role in the evaluation of adults with
constipation? Gut 24:86

David A. Rothenberger, M.D.


Division of Colon and Rectal Surgery
Box 450
University of Minnesota Hospital and Clinic
420 Delaware Street SE
Minneapolis, MN 55455
USA

Int J Colorect Dis (1990) 5:41-43

C ol0ree/al
Disease

9 Springer-Verlag 1990

Restorative proctocolectomy:
a procedure for the district general hospital?
B . R . Davidson * and J. Thornton-Holmes

Surgical Unit, Peterborough District Hospital, Thorpe Road, Peterborough, UK


Accepted: 15 September 1989

Abstract. Restorative proctocolectomy is widely regarded as the surgical procedure of choice for patients
with ulcerative colitis or familial adenomatous polyposis,
the majority being carried out within specialised regional
centres. The use of this procedure outside such centres
has been investigated by reviewing the results from a
District General Hospital ( D G H ) over the 8 year period
1981-1989. Seventeen patients (11 male and 6 female
with a median age of 36 years) underwent total colectomy
and ileoanal anastomosis with formation of a pelvic reservoir ( T C - I A ) . Fourteen had ulcerative colitis (UC),
2 familial a d e n o m a t o u s polyposis (FAP) and one a
colonic and rectal cancer. Three pouch designs were used
("S" in 7, " J " in 8 and " W " in 2) with no operative or
perioperative deaths. Further laparotomy was required in
two patients for adhesions and pelvic sepsis. Functional
results were assessed in 16 patients at a mean of 5 years
after surgery. The median daily stool frequency was 5
(range 2 6). Twelve of the 16 patients defaecate spontaneously, 2 regularly self-catheterise and 2 do so occasionally. N o n e of the patients is incontinent of formed or
liquid stool but one has occasional soiling. These results
suggest that TC IA m a y be statisfactorily performed
outside a specialised unit.

Introduction

The cure of ulcerative colitis (UC) and familial adenomatous polyposis (FAP) is dependent on the complete removal of all colonic and rectal mucosa. Panproctocolectomy, although curing these conditions, leaves the patient
with a permanent ileostomy with its associated physical
and psychological sequelae [1, 2]. D a m a g e to pelvic autonomic nerves is an occasional complication resulting in

* Present address: Academic Department of Surgery, Royal Free


School of Medicine, Rowland Hill Street, Hampstead, London
NW3 2PF, UK

urinary and sexual difficulties [3, 4]. The development of


a continent ileostomy by K o c k [5], went some way to
improving ileostomy m a n a g e m e n t but is far from an ideal
solution. One alternative is colectomy and ileorectal
anastomosis. Although satisfactory results have been
achieved in some centres [6], this procedure leaves the
diseased rectum and therefore exposes the patient to the
risk of developing a rectal carcinoma subsequently [7].
Total colectomy, mucosal proctectomy and ileoanal
anastomosis ( T C - I A ) with formation of a pelvic reservoir as originally described by Sir Alan Parks over a
decade ago [8] appears to offer a better solution, and
modifications in technique and pouch design have gradually improved the results obtained [9-11]. Despite this
m a n y patients requiring surgery for U C or FAP are left
with a permanent ileostomy due to a reluctance to perform this procedure except in a specialised centre. In the
Trent Region with a population of 4.5 million, for example, 25 patients with U C required a colectomy in 1985.
Fifteen (60%) were left with a permanent ileostomy and
only one patient had ileoanal anastomosis, despite 14
(56%) of the patients being less than 50 years of age and
therefore likely to have been suitable for reconstructive
surgery [12].
Whether TC IA could be made more widely available to patients by carrying out this procedure in a district rather than a regional or national centre has been
investigated by reviewing the experience and results from
a District General Hospital (DGH).

Patients and methods

Over an eight year period, 1981-1989, 17 patients underwent total


colectomy, mucosal proctectomy and iteoanal anastomosis with
formation of a pelvic reservoir. There were 11 males and 6 females,
aged 15 to 58 years (median 36 years). Fourteen patients had ulcerative colitis, one of whom was known also to have a colonic carcinoma (Dukes stage C). Two patients had FAP. The final patient
had a low rectal carcinoma (Dukes B), a family history of colonic
cancer and had had a previous left hemicolectomy for carcinoma
(Dukes B) when 32 years of age.

42

Indications for surgery

Table 1. Post-operative complications

Patients with UC or FAP who required surgery were considered for


T C - I A except those over 60 years of age or with previous rectal
disease resulting in poor sphincter function. Crohn's disease was
excluded by multiple colonic biopsies although preoperative histological classification was occasionally indeterminate.

Complication

Incidence

No. in series

Surgery

Perineal sinus
Anal stricture
Pelvic abscess
Adhesional obstruction
Abdominal fistula

1
1
1
1
1

6
9
10
10
12

Yes
No
Yes a
Yes"
Yes

Operative procedure

a Patient required surgery for two separate complications

In 8 of the 17 patients total colectomy and the formation of a


mucous fistula was carried out as an initial procedure, with mucosal
proctectomy and ileoanal pouch formation being carried out when
the patients' general condition improved. Two patients with pyoderma gangrenosum had a total colectomy and mucosal proctectomy as an initial procedure, and the remaining 7 patients had a one
stage procedure. All patients received pre-operative parenteral antibiotics (metronidazole+ampicillin or cefuroxime). Total colectomy was carried out along standard lines. The rectum was mobilised to 2 - 3 cm above the dentate line, dissection being carried
out close to the rectal wall in the first eight patients and in the post
mesorectal plane in the subsequent nine. The rectum was transected at this level and the lower rectal mucosa removed in its
entirety by endoanal dissection without eversion of the rectum.
Three different pouch designs were used. Seven patients had
'~ pouches formed as originally described by Parks [8], two with
a short efferent limb (2-3 cm). Eight patients had the " J " pouch as
described by Utsonimiya [9] and two a four limbed " W " pouch [I 1].
Ileoanal anastomosis was carried out using interrupted Vicryl sutures and an intersphincteric Penrose drain was brought out in the
perineum. A right iliac fossa defunctioning loop ileostomy was
formed in all cases.

Operative problems and their management


There were no significant operative difficulties in 13 (76%) of the 17
procedures. In four patients insufficient length of small bowel
mesentery was available for pouch formation without tension and
a vascular arcade of the superior mesenteric artery had to be divided. This resulted in pouch ischaemia in one of these four patients
and the pouch had to be reconstructed.
Five patients had minor operative difficulties due either to adhesions from the previous colectomy or the presence of active rectal
disease.

Operative mortality and post-operative complications


There was no operative or perioperative mortality. Post-operative
complications are shown in Table 1. Twelve (70%) of the 17 patients
had no significant post-operative complications. Three (18%) of the
/7 patients have required further surgery. Of these two have required further laparotomy, one on two separate occasions. The first
patient had division of small bowel adhesions and at a later date
laparotomy for pelvic sepsis which may have followed pouch perforation at sigmoidoscopy. The second patient developed a mucocutaneous fistula from the apex of the pouch to the lower anterior
abdominal wall necessitating exploration and closure, which may
have been related to previous long term steroid therapy. The third
patient developed an intersphincteric sinus related to the perineal
drain site which healed following exploration and curettage.

Follow up and functionaI results


The median period of follow up is 53 months (range 3 - 9 2 months).
Sixteen of the 17 patients were available for review, the remaining

Table 2. Functional results (16 patients)


Number
Continence
Normal
Occasional mucous leakage
Occasional soiling
Defaecation
Spontaneous
Catheterisation

Always
Occasional

Anti-diaorrhoeal medication
Never
Regular

(% of series)

9
6
1

56
37
6

11
2
3

69
12
19

8
8

50
50

patient having diecl from liver metastases one year following pouch
formation. At the time of surgery he had a Dukes' C carcinoma of
the transverse colon arising in an area of chronic ulcerative colitis
but no evidence of metastatic disease. He had previously refused
total colectomy and ileostomy. Functional results for the 16 patients are shown in Table 2. The median daily stool frequency was
five (range 2-6). Three of the five patients who catheterise have "S"
pouches, two with 5 - 6 cm and one with a 2 - 3 cm efferent limb.
Two patients with "J" pouches occasionally catheterise, one of
whom has only recently had ileostomy closure. None of the patients
has had problems related to urgency of defaecation nor any urinary
or sexual difficulties. All patients but one are satisfied with the
functional result and none has expressed a desire to return to an
ileostomy. The dissatisfied patient self-catheterises up to 10 times
daily. Further investigation of this problem has been hampered by
poor patient co-operation and clinic attendance. This patient had a
long history of psychiatric problems and drug abuse although he
had reformed prior to surgery being considered. All patients wishing to do so have been able to return to full time employment.
Two patients had late complications on follow up, one having
recurrent bouts of"pouchitis" which respond to metronidazole and
the other a stricture above the ileo-anal anastomosis which was
successfully treated with a dilator.

Discussion
Many factors are of importance when considering patients for total colectomy and ileo-anal anastomosis
(TC-IA). Elderly patients and those having undergone
previous anorectal surgery may have inadequate anal
sphincter function following TC-IA. As further deterior a t i o n in f u n c t i o n is to b e e x p e c t e d w i t h a d v a n c i n g a g e
and follow up remains limited, the upper age limit for
p a t i e n t s e l e c t i o n r e m a i n s to be e s t a b l i s h e d . T h e o n l y p a t i e n t in t h e p r e s e n t series w i t h a n y i n c o n t i n e n c e o f s o l i d
o r l i q u i d f a e c e s w a s 58 y e a r s o f a g e a t t h e t i m e o f s u r g e r y .

43
Lower rectal cancer is commonly considered to be
an absolute contraindication to T C - I A [13]. In the present series, however, one such patient underwent T C - I A .
This patient had a strong family history of colonic carcinoma and presented with a rectal carcinoma (Dukes
stage B) at 34 years of age, 2 years following a left hemicolectomy for carcinoma of the descending colon (Dukes
stage B). The remainder of the colon was removed leaving
a short rectal stump and an ileostomy. Two years later
mucosal proctectomy with ileo-anal anastomosis was
performed and 5 years later he shows no evidence of
recurrent disease. Where total colectomy is required and
a rectal lesion would normally be managed by anterior
resection there seems no logical reason to withhold the
sphincter preservation of T C - I A .
Difficulty in excluding Crohn's disease on pre-operative histology is not uncommon, which would support
T C - I A being carried out in stages, with the initial total
colectomy allowing histological examination of the entire
specimen. M a n y reported series of T C - I A include patients with Crohn's disease who were initially misdiagnosed [10, 14].
Although minor operative difficulties with the technique of T C - I A are common, major problems are rare.
Insufficient length of small bowel mesentery to allow
pouch formation and ileo-anal anastomosis without tension was encountered in four patients (23%), and was
considered to be the major operative difficulty. Our experience in the present study suggests that if the intended
site of ileo-anal anastomosis can reach 2 cm over the
pubic crest then ileo-anal anastomosis will be carried out
without tension. This surface landmark is simpler than
drawing the intended site of anastomosis down to the
dentate line as has been suggested previously [11].
Five patients in the present series, three of whom have
"S" pouches, require to self-catheterise. This problem,
most commonly encountered in patients with "S"
pouches, appears to be related to the length of the distal
ileal segment and to be rare if a short segment is used [15].
Both " J " and " W " pouch constructions usually avoid the
need to catheterise [16].
In the present series the incidence of post-operative
complications was comparable to that reported from specialised centres [8, 9, 11, 14, 16]. O f interest is that the
complications occurred in the 6th, 9th, 10th and 12th
patients in the series which does not suggest that a learning curve was an important factor [17]. This may, however, reflect the senior author's (J.T.H.) previous experience of coloanal anastomosis.
In conclusion, although this series is small, the results
achieved from a District General Hospital are comparable to those from major centres in terms of operative
mortality, post-operative complications and functional
results. This would support the use of T C - I A in a district

hospital setting if care is taken with patient selection, the


operative procedure and post-operative management.
It's wider availability would allow many patients the opportunity to avoid life with a permanent ileostomy.

References
1. Koudahl G, Kristensen M (1976) Post-operative mortality and
complications after colectomy for ulcerative colitis. Scand J
Gastroenterol [Suppl] 11: 117- 122
2. Druss RG, O'Connor JF, Prudden JF, Stern LO (1968) Psychological response to colectomy. Arch Gen Psych 18:53-59
3. Neal DE, Parker AJ, Williams NS, Johnston D (1982) The long
term effects of proctectomy on bladder function in patients with
inflammatory bowel disease. Br J Surg 69:349-352
4. Burnham WR, Lennard-Jones JE, Brooke BN (1977) Sexual
problems amongst married ileostomists. Gut 18:673-677
5. Kock NG (1973) Continent ileostomy. Progr Surg 12:180-201
6. Aylett SO (1966) Three hundred cases of diffuse ulcerative colitis treated by total colectomy and ileo-rectal anastomosis. Br
Med J 1:1001-i005
7. Baker WNW, Glass RE, Ritchie Jean K, Aylett SO (1978)
Cancer of the rectum following colectomy and ileorectal anastomosis for ulcerative colitis. Br J Surg 65:862-868
8. Parks AG, Nicholls RJ (1978) Proctocolectomy without
ileostomy for ulcerative colitis. Br Med J 2:85-88
9. Utsunomiya J, Iwama T, hnajo M, Matuso S, Sawai S, Yaegashi K, Hirayania R (1980) Total colectomy, mucosal proctectomy and ileoanal anastomosis. Dis Col Rect 23:459-466
10. Fonkalsrud EW (1984) Endorectal ileoanal anastomosis with
isoperistaltic ileal reservoir after colectomy and mucosal proctectomy. Ann Surg 199:151-157
11. Nicholls RJ, Lubowski DZ (1987) Restorative proctocolectomy: the four toop (W) reservoir. Br J Surg 74:564-566
12. Statistics of the Trent Regional Health Authority 1985.
13. Williams NS, Johnston D (1985) The current status of mucosal
proctectomy and ileo-anal anastomosis in the surgical treatment of ulcerative colitis and adenomatous polyposis. Br J Surg
72:159-168
14. Dozois RR (1985) Ileal "J" pouch-anal anastomosis. Br J Surg
72 [Suppl]:80-82
15. Nicholls RJ, Pezim ME (1985) Restorative proctocolectomy
with ileal reservoir for ulcerative colitis and familial adenomatous polyposis; a comparison of three reservoir designs. Br J
Surg 72:470-474
16. Nasmyth DG, Williams NS, Johnston D (1986) Comparison of
the function of triplicated and duplicated pelvic ileal reservoirs
after mucosal proctectomy and ileo-anal anastomosis for ulcerative colitis and adenomatous polyposis. Br J Surg 73:361 -366
17. Keighley MRB, Winslet MC, Flinn R, Kmiot W (1989) Multivariate analysis of factors influencing the results of restorative
proctocolectomy. Br J Surg 76:740-744
Brian R. Davidson, M.D., FRCS
Academic Department of Surgery
Royal Free School of Medicine
Rowland Hill Street
Hampstead
London NW3 2PF
UK

Int J Colorect Dis (1990) 5:44-48

Col6ree/al
Disease

9 Springer-Verlag 1990

A retrospective study of colostomies, leaks and strictures


after colorectal anastomosis
J. R. D. Tuson and W . G . Everett
Addenbrooke's Hospital, Cambridge, UK
Accepted: 30 October 1989

Abstract. A review was u n d e r t a k e n o f 360 p a t i e n t s u n d e r g o i n g elective left-sided c o l o n i c or rectal resections with


p r i m a r y a n a s t o m o s i s , u n d e r the c a r e o f one surgeon, over
a n i n e t e e n y e a r p e r i o d . T h e incidence, a e t i o l o g y a n d
m a n a g e m e n t o f a n a s t o m o t i c leaks a n d strictures was
s t u d i e d a n d the role o f p r o x i m a l d i v e r t i n g c o l o s t o m y c o n sidered. P e r i o p e r a t i v e m o r t a l i t y was 2 . 7 % . T h e incidence
o f a n a s t o m o t i c leaks was 2 4 . 4 % . L e a k s were m o r e c o m m o n w h e n a n a s t o m o s e s were low, were s u t u r e d o r were
c o n s t r u c t e d b y trainees. Strictures d e v e l o p e d in 5.8%.
L o c a l r e c u r r e n c e o f t u m o u r was the cause o f 25 % o f these
strictures. A n a s t o m o t i c l e a k a g e was the p r i n c i p a l cause
o f b e n i g n strictures; t h o s e d e v e l o p i n g in a s s o c i a t i o n w i t h
leaks were m o r e likely to r e q u i r e surgical i n t e r v e n t i o n .
T h e r e was n o evidence t h a t d e l a y in c o l o s t o m y closure
c o n t r i b u t e d to the d e v e l o p m e n t o f b e n i g n a n a s t o m o t i c
strictures. I t was n o t p o s s i b l e to d e t e r m i n e w h e t h e r the
presence o f a c o l o s t o m y affected the incidence o f leaks
b u t the local effects o f such l e a k s were m i t i g a t e d in p a tients w i t h c o l o s t o m i e s . W h e r e a m i n o r l e a k h a d o c c u r r e d
it was n o t necessary to w a i t for c o m p l e t e a n a s t o m o t i c
h e a l i n g b e f o r e closing the c o l o s t o m y . A f t e r m a j o r leaks,
c o l o s t o m y c l o s u r e b e f o r e c o m p l e t e h e a l i n g was associa t e d w i t h f u r t h e r a n a s t o m o t i c p r o b l e m s in 16.0% o f
cases.

Introduction
A review was u n d e r t a k e n o f 360 p a t i e n t s u n d e r g o i n g elective left sided c o l o n i c o r rectal r e s e c t i o n s w i t h p r i m a r y
a n a s t o m o s i s u n d e r the care o f one s u r g e o n ( W G E ) d u r i n g
the p e r i o d 1 9 6 9 - 1 9 8 7 . T h e p u r p o s e was to d e t e r m i n e the
incidence a n d a e t i o l o g y o f a n a s t o m o t i c leaks a n d strictures, to review the m a n a g e m e n t o f these c o m p l i c a t i o n s
a n d to a d d r e s s two p a r t i c u l a r issues: (1) W h e n is it safe to
close a c o l o s t o m y in a p a t i e n t w h o has h a d a n a n a s t o m o t i c leak; is it n e c e s s a r y to w a i t until there is c o m p l e t e
h e a l i n g o f the a n a s t o m o s i s ? (2) D o e s d e l a y in c l o s u r e o f

c o l o s t o m i e s result in a n i n c r e a s e d incidence o f a n a s t o m o t i c strictures?

Patients and methods


Information was obtained on 360 patients whose ages ranged from
23 to 93 years (mean 64.6 yr). 182 were male and 178 female. Most
operations were performed for neoplasms of the colon or rectum
(290- 80.5 %) or diverticular disease (65-- 18.1%). Pre-operative
preparation and operative technique are described elsewhere [1].
Anastomoses were sutured, using a single layer of interrupted inverting Supramid sutures (A. W. Showell, Surgicraft Ltd), or stapled using the non-disposable EEA instrument (Autosuture). Of the
360 anastomoses 271 were sutured and 89 were stapled. From 1979
cases were entered into a prospective trial comparing hand suture
and stapling techniques [1]. This became the principal factor determining the technique chosen for individual cases.
Anastomoses were marked with Cushing's clips for identification on subsequent radiographs. Anastomoses were classified as
"low" when the rectum was divided below the pelvic peritoneal
reflection. Of 360 anastomoses, 119 were low. A proximal diverting
right transverse loop colostomy was fashioned in all 18 cases with
coloanal anastomoses. Other indications included inadequate bowel
preparation, gross faecal contamination, cases where the anastomosis could not be adequately inspected or where there was particular
difficulty in constructing the anastomosis, and stapled anastomoses
where doughnuts were incomplete.
Patients were monitored tbr clinical evidence of anastomotic
leakage, which was considered to be present if any of the following
was observed: post-operative pyrexia or septicaemia with abdominal tenderness; evidence of an intraperitoneal abscess on pelvic
examination or ultrasound; development of faecal fistula from
wound, drain tract or vagina; discharge of pus per rectum or via
drain tract; necessity of a further laparotomy for peritonitis; anastomotic defect palpable or visible at sigmoidoscopy,
On the 9th or 10th post-operative day a limited contrast enema
was performed to determine integrity of the anastomosis. Where
present, colostomies were dosed at the earliest opportunity after a
satisfactory contrast enema examination. The radiographs were reviewed, and leaks classified as "major" where contrast escaped
freely into the peritoneal cavity and "minor" where the escape was
localised. If there was evidence of a major leak closure was usually
delayed until subsequent contrast studies showed healing or limitation of the extent of the leak.
Anastomotic strictures were considered significant if at any
stage during follow-up a 15 mm sigmoidoscope could not be passed
through the anastomosis. All were within sigmoidoscopic range.

45
The chi-squared test with Yates' correction was used to determine
the statistical significance of the observations. Probabilities of
<0.05 were accepted as significant.

Table 1. Factors affecting the incidence of anastomotic leakage


Leak incidence
Number %

Results
There were 9 early deaths, a hospital mortality of 2.5%.
Three were related to septicaemia associated with anastomotic leaks.
Proximal diverting colostomies were fashioned for all
18 coloanal anastomoses. The incidence of colostomy
formation in the remainder was 29.8%, and was greater
for low (68.3%) than for high (13.7%) anastomoses.
Clinically apparent leaks from the primary anastomosis affected 55 cases (15.3 %). Post-operative co ntrast enemas disclosed 33 additional leaks - a combined incidence of 24.4%. Age, sex and pathological diagnosis
exerted no discernable effect on the incidence of leaks.
Factors which significantly influenced the incidence of
anastomotic leaks are considered in Table 1. Coloanal
anastomoses were excluded from those figures in Table 1
pertaining to the effect of seniority of surgeon and anastomotic technique on the incidence of leaks as all were
sutured by the consultant. Both stapled and sutured
groups were well matched for age, sex, pathological diagnosis and level of anastomosis.
M a n a g e m e n t of anastomotic leaks: 50 of the 88 cases
that leaked had proximal diverting colostomies. All 50
were managed conservatively; closure of the colostomy
was delayed if there was clinical evidence of sepsis, or a
major leak was detected radiologically. Closure was effected when a subsequent contrast enema showed either
complete healing of the anastomosis or localisation of the
abscess cavity. Two of the 50 cases died as a result of
sepsis related to leakage. Non-malignant strictures developed in 4 of the 50 cases, 2 of which required late surgical
intervention.
Thirty-eight of the 88 cases with leaks did not have a
colostomy formed at the time of anastomosis. In this
group 8 cases (21.0%) required early re-operation for
faecal contamination - drainage and formation of a diverting colostomy in 6, and H a r t m a n n ' s procedure in 2.
One of the 8 died as a result of sepsis. Non-malignant
strictures developed in 6 of the 38 cases (15.8%). Late
surgical intervention was required in 6 cases - in 5 of
these for strictures.
Anastomotic strictures: We detected 20 strictures, an
incidence of 5.6%. These were caused by either cicatrization or local recurrence of malignancy.
There were 15 non-malignant strictures (4.2%). O f
the possible aetiological factors examined, age, sex,
pathological diagnosis, level of anastomosis, anastomotic technique, seniority of surgeon, presence of a diverting colostomy and the extent of a leak where one had
occurred were all found to exert no statistically significant effect. The development of an anastomotic leak was
the single factor which was found to significantly affect
the incidence of such strictures. Strictures developed in 10
of 88 cases with leaks (11.4%) and 5 of 272 cases with no
leak (1,8%-p<0.001). In 5 cases the stricture was an

Level of
anastomosis

High
Low excluding
coloanal
Coloanal

42/241
35/101

17.4 p<0.001
34.7

11/18

61.1

Anastomotic
technique
Surgical
experience

Sutured
Stapled
Consultant: Stapled
Sutured
Registrar:
Stapled
Sutured

66/253
11/89
7/67
48/216
4/22
18/37

26.1 p<0.01
12.4
10.4 p>0.05
22.1
18.2 p< 0.05
48.6

asymptomatic sigmoidoscopic finding and no intervention was required. In 10 cases the stricture either caused
obstructive symptoms or required treatment before closure of a proximal colostomy. Dilatation was performed
in five cases. Five fight fibrous strictures required resection or stricturoplasty.
Eight of the 10 non-malignant strictures occurring in
association with leaks required surgical intervention (resection in 3, per-anal stricturoplasty in 1 and dilatation in
4). O f the five strictures developing in the absence of a
leak, one sutured anastomosis was resected; of three stapled anastomoses in this group, one was easily dilated by
the passage of a colonoscope and the other two were
observed to dilate spontaneously.
D a t a pertaining to the effect of timing of colostomy
closure on the incidence of non-malignant anastomotic
strictures are presented in Table 2. The term "delay" in
colostomy closure was applied to closure after the 28th
day. Where there was no anastomotic leak, delay in
colostomy closure was not associated with an increased
incidence of strictures. Our results do not provide support for the hypothesis that the presence of a proximal
diverting colostomy predisposes to the development of an
anastomotic stricture.
Timing of colostomy closure after anastomotic leaks:
In 18 cases the colostomy was closed early (mean 18.7
days) despite a minor leak (Table 2). These leaks were
subclinical. In 8 further cases closure was delayed (mean
78.6 days) until there was radiographic evidence of complete anastomotic healing. N o further local complications occurred in any of these 26 cases. In 25 cases closure
of the colostomy was delayed (mean 127.5 days) until
there was radiographic evidence of partial but incomplete
healing, with limitation o f extent o f the leak. Six non-malignant strictures developed in this group. Three developed early and were managed by dilatation before
colostomy closure. Three cases developed symptomatic
strictures late, after colostomy closure. Two were managed by dilatation, while the third required resection.
Malignant strictures: local malignant recurrence occurred in 15 cases (5.3%) and caused a stricture in 5 of
these. The m a n a g e m e n t of this complication is beyond
the scope of this article.

46
Table 2. The effect of leaks, colostomies and the timing of colostomy closure on the incidence of non-malignant strictures

Colostomy
126 cases

Anastomotic leak
(56 cases)

No anastomotic leak
(70 cases)

Stricture
incidence

Management

Results

12.5%

18 cases closed early despite minor leak

No strictures

8 cases closure delayed until radiographic


evidence of complete anastomotic healing
25 cases closed when there was incomplete healing

No strictures

2.9%

5 cases not closed


53 cases closed early

6 strictures
(3 early and 3 late)
1 stricture.
2 strictures

16 cases closure delayed

No strictures

I peri-operative death
No colostomy
234 cases

Anastomotic leak
(32 cases)
No anastomotic leak
(202 cases)

9.4%

3 strictures

1.5%

3 strictures

Discussion
A particular feature of this series is the assessment of all
anastomoses by contrast radiography. In other series
where a post-operative contrast enema was performed
[2-11] the incidence o f leaks ranges from 5.7% to 51.0%.
Whilst the combined incidence of leaks in our series appears high in comparison with some reports [11 15],
these results are matched by those of the senior author [1,
16]. We report the results of all elective left-sided colonic
anastomoses during a 19 year period which perhaps reflect more accurately the experience of a single surgical
firm.
Three factors significantly affected the incidence of
leaks: the level of the anastomosis, the method of its
construction and the degree o f experience of the surgeon.
A n increased incidence of leaks from low anastomoses is well recognised [ 1 - 6 , 11, 15-19] and was reflected in our results. One of the main difficulties encountered with anastomoses in this situation is disparity
between colon and rectum; the diameter of the rectum
m a y be up to three times that of the proximal colon.
When a sutured anastomosis is performed in these circumstances it m a y be impossible to avoid gaps between
sutures. W h e n performing a stapled anastomosis difficulty m a y be experienced gathering the rectum into the
purse-string suture so that the knife blade cuts through
the rectum eccentrically giving an incomplete doughnut.
Where there is gross disparity there is much to be said for
closing the rectum with a linear stapler before completing
the anastomosis with the E E A instrument or, if hand
suturing, forming a side to end anastomosis [20].
The method of construction of the anastomosis did
not significantly affect the incidence of leaks when the
procedure was performed by the consultant. This is in
accordance with other reports [1, 5, 21]. Where anastomoses were stapled, seniority and experience of the sur-

geon did not affect the incidence of leaks. However, the


overall incidence of anastomotic leaks was significantly
greater where anastomoses were fashioned by registrars,
and for registrars the incidence of leaks was significantly
greater for sutured than for stapled anastomoses. Where
leakage occurs it is likely that one of the basic principles
of anastomosis (an adequate blood supply, avoidance of
tension and contamination, and construction of an airtight anastomosis) has been violated. The increased incidence of leaks f r o m anastomoses constructed by registrars supports this contention and highlights the
importance of supervision. After introduction of the stapler cases were entered into a prospective trial, trainees
were more closely supervised, and the incidence of leaks
no longer varied significantly with seniority or technique
[1].
Conservative m a n a g e m e n t of leaks in cases with a
covering colostomy was successful in most instances
(92.0%), the presence of a colostomy making it easier to
resist a further operation for contamination. However,
the 2 deaths amongst 50 cases m a n a g e d this way underline the need for careful case selection, with timely surgical intervention when indicated. In the absence of a
colostomy, conservative m a n a g e m e n t of leaks was successful in a smaller proportion of cases (65.0%). Early
surgery for contamination was necessary in 2 t . 1 % , and
late intervention for anastomotic problems - principally
non-malignant strictures - w a s required in 15.8%. We
therefore conclude that diverting colostomies mitigate
the local and systemic effects of leaks, as suggested by
others [3, 12], although any advantage must be weighed
against the morbidity and mortality of colostomy closure. We do not agree with the contention [12] that a
proximal diverting colostomy should be formed in all
cases where a clinically apparent leak has occurred.
We found that it was not necessary to await complete
healing of a minor leak before closing a colostomy. In

47
contrast, after major leaks further anastomotic problems
may be anticipated if the colostomy is closed before healing is complete.
The sensitivity of our criteria for determining which
anastomoses should be considered at high risk of leakage
and protected by a colostomy was 41.7%, and the
specificity 84.2%. Assuming that the presence of a
colostomy does not make an anastomosis less likely to
leak, these criteria seem poor indicators of outcome of an
anastomosis, which have resulted in unnecessary
colostomies in 19.4% of our 360 cases. Furthermore, our
experience indicates that the majority of anastomotic
leaks may be managed without formation of a colostomy.
Certain groups at particular risk can be identified where
a colostomy should be considered: coloanal anastomoses; low sutured anastomoses performed by registrars;
and cases where there is gross contamination or inadequate bowel preparation.
Anastomotic leakage was the only statistically significant aetiological factor in the development of non-malignant anastomotic strictures and was related to fibrosis
associated with healing and contracture of the associated
abscess cavity [9]. Non-malignant strictures developing in
the absence of leakage may be attributable to compromise of the vascular supply of the proximal colon. With
stapled anastomoses ischaemia may be the consequence
of excessive clearance of the bowel wall before application of the purse-string suture [22, 23]. Crushing of the
bowel between anvil and cartridge may also be a factor,
as may cicatrisation during healing by second intention
of the linear ulcer formed in the gap between mucosal
surfaces which is produced by the action of the stapler
[24]. Furthermore, a diaphragm-like stenosis may result
from the inversion produced by stapling devices [25],
especially where smaller sized cartridges are used [26].
We found no evidence that proximal diverting
colostomies cause non-malignant strictures. Whilst strictures were more c o m m o n amongst cases with
colostomies, the incidence of leaks was also higher in this
selected group.
Many strategies have been employed in the management of non-malignant anastomotic strictures. Such
strictures are often asymptomatic, and probably dilate
spontaneously with the passage of faeces [2, 4]. Others
require dilatation, particularly where a proximal diverting colostomy is to be closed.
We found that strictures developing in the presence of
anastomotic leakage were more likely to require surgical
intervention than those occurring without leaks. Where a
leak culminated in stricture formation there was often
extensive fibrosis and contracture, This was in contrast to
strictures affecting intact anastomoses which were commonly short diaphragm-like lesions, particularly where
the stapler was used to construct the anastomosis. In all
cases where a stricture was dilated we found that a single
dilatation was sufficient.
Antonsen [27] suggested that symptoms of stenosis in
patients without evidence of anastomotic leakage were
usually explained by local recurrence. However, in our
experience such stenoses were more commonly caused by
non-malignant cicatrisation. It is clear that the prognosis

for patients with malignant strictures is poor, irrespective


of the treatment selected. Death was in all our cases
caused by disseminated malignancy rather than local
complications.
In conclusion, we report the combined results
achieved by a surgical firm and highlight the continuing
problem of anastomotic leakage and its sequelae, in spite
of modern suturing and stapling techniques. We question
the place of the diverting proximal colostomy for leftsided cotorectal anastomoses and consider the need for
refinement of the indications for diversion.

Acknowledgements. We wish to acknowledge the contribution of


our colleagues in the Department of Radiology. Mrs. Jackie Foreman provided secretarial assistance.

References
I. Everett WG, Friend PJ, Forty J (1986) Comparison of stapling
and hand-suture for left-sided large bowel anastomosis. Br J
Surg 73:345-348
2. Blamey SL, Lee PWR (1982) A comparison of circular stapling
devices in colorectal anastomoses. Br J Surg 69:19-22
3. Goligher JC, Graham NG, De Dombal FT (1970) Anastomotic
dehiscence after anterior resection of rectum and sigmoid. Br J
Surg 57:109-118
4. Goligher JC, Lee PWR, Lintott DJ (1979) Experience with the
Russian Model 249 suture gun for anastomosis of the rectum.
Surg Gynaecol Obstet 148:517-524
5. Brennan SS, Pickford IR, Evans M, Pollock AV (1982) Staples
or sutures for colonic anastomoses - a controlled clinical trial.
Br J Surg 69:722-724
6. Goligher JC, Lee PWR, Simpkins KC, Lintott DJ 0977) A
controlled comparison of one- and two-layer techniques of suture for high and low colorectal anastomoses. Br J Surg
64:609-614
7. McGinn FP, Gartell PC, Clifford PC, Brunton FJ (1985) Staples or sutures for low colorectal anastomoses: a prospective
randomised trial. Br J Surg 72:603-605
8. Goligher JC, Morris C, McAdam WAF, de Dombal FT, Johnston D (1970) A controlled trial of inverting versus everting
intestinal suture in clinical large-bowel surgery. Br J Surg
57:817-822
9. Kirkegaard P, Christiansen J, Hjortrup A (1980) Anterior resection for mid-rectal cancer with the EEA stapling instrument.
Am J Surg 140:312-314
10. Irvin TT, Goligher JC, Johnston D (1973) A randomised prospective ctinical trial of single-layer and two-layer inverting
intestinal anastomoses. Br J Surg 60:457 460
11. Matheson NA, Irving AD (1975) Single layer anastomosis after
rectosigmoid resection. Br J Surg 62:239-242
t2. Heald RJ, Leicester RJ 0981) The low stapled anastomosis. Dis
Colon Rectum 24:437-444
13. Parks AG, Percy JP (1982) Resection and sutured colo-anal
anastomosis for rectal carcinoma. Br J Surg 69:301-304
14. Steinhagen RM, Weakley FL (1985) Anastomosis to the rectum: operative experience. Dis Colon Rectum 28:105-109
15. Fielding LP, Stewart-Brown S, Hittinger R, BlesovskyL (1984)
Covering stoma for elective anterior resection of the rectum: an
outmoded operation? Am J Surg 147:524 530
16. Everett WG (1975) A comparison of one layer and two layer
techniques for colorectal anastomosis. Br J Surg 62:135-140
17. Fielding LP, Stewart-Brown S, BlesovskyL, Kearney G (1980)
Anastomotic integrity after operations for large bowel cancer:
a multicentre study. Br Med J 281:411-414
18. Irvin TT, Goligher JC (1973) Aetiology of disruption of intestinal anastomoses. Br J Surg 60:461 464

48
19. Morgenstern L, Yamakawa T, Ben-Shoshan M, Lippman H
(1972) Anastomotic leakage after low colorectal anastomosis.
Am J Surg 123:104-109
20. Everett WG, Greatorex RA (1987) Colonic anastomosis - suture or staple? Trop Gastroenterol 8:195-203
21. Beart RW, Kelly KA (1981) Randomised prospective evaluation of the EEA stapler for colorectal anastomoses. Am J Surg
141:143-147
22. Trollope ML, Cohen RG, Lee RH, Cannon WB, Marzoni FA,
Cressman RD (1986) A 7 year experience with low anterior
sigmoid resections using the EEA stapler. Am J Surg 152:1115
23. Gordon PH, Vasilevsky CA (1984) Experience with stapling in
rectal surgery. Surg Clin North Am 64:555-566
24. Polglase AL, Hughes ESR, McDermott FT, Burke FR (1981)
A comparison of end-to-end staple and suture colorectal anastomosis in the dog. Surg Gynaecol Obstet 152:792-796

25. Accordi F, Sogno O, Carnioto S, Fabris G, Moschino P, Coan


B, Carlon C (1987) Endoscopic treatment of stenosis following
stapler anastomosis. Dis Colon Rectum 30:647-649
26. Kissin MW, Cox AG, Wilkins RA, Kark AE (1985) The fate of
the EEA stapled anastomosis: A clinico-radiological study of 38
patients. Ann R Coll Surg Engl 67:20 22
27. Antonsen HK, Kronborg O (1987) Early complications after
low anterior resection for rectal cancer using the EEA stapling
device: a prospective trial. Dis Colon Rectum 30:579-583
J. R. D. Tuson
St. Helier Hospital
Wrythe Lane
Carshalton
Surrey SM5 IAA
UK

Int J Colorect Dis (1990) 5:49-52

Colo~
Disease

9 Springer-Verlag 1990

Gynaecological problems related to anatomical changes after


conventional proctocolectomy and ileostomy
M. Wikland 1, I. Jansson 1, M. Aszt61y 2, Inger Palselius 3, G. Svaninger 3, O. Magnusson 3 and L. Hult~n 3
Departments of 1Obstetrics and Gynecology, 2 Radiology and 3 Surgery II, Sahlgrens' Hospital, University of G6teborg,
G6teborg, Sweden
Accepted: 5 June 1989

Abstract. Seventy-one w o m e n w h o had a proctocolect o m y for ulcerative colitis ( n = 4 1 ) or C r o h n ' s disease


( n = 30) were interviewed in the follow-up clinic a b o u t
gynaecological problems and fertility. All w o m e n were
examined by an independent gynaecologist and abnormalities o f the internal genital tract were registered.
Forty-nine per cent (35/71) o f the w o m e n had a distressing vaginal discharge after p r o c t o c o l e c t o m y , c o m p a r e d
with 9% (6/71) before surgery. A t the gynaecological
examination 4 5 % (32/71) had a heavy vaginal secretion
with- out any signs o f an acute vaginal infection. In
68% (30/44) fluid retention in the vagina was associated
with a caudally firmly fixed and dilatated posterior vaginal fornix. Twelve per cent (8/66) o f the w o m e n reported
dyspareunia before surgery. After surgery, 27% (18/66)
complained o f this s y m p t o m . Fertility was significantly
reduced after surgery since only 37% (10/27) o f the
w o m e n w h o attempted to become p r e g n a n t succeeded
within 5 years follow-up. The c o r r e s p o n d i n g figure before surgery was 72% (39/54). Those w h o conceived went
t h r o u g h p r e g n a n c y and parturition w i t h o u t any incident,
6 o f 21 delivered by caesarean incision. In conclusion,
conventional p r o c t o c o l e c t o m y in w o m e n will result in
distressing vaginal discharge, and dyspareunia in a considerable p r o p o r t i o n o f the patients. The operation also
seems to decrease their chances o f b e c o m i n g pregnant.

Introduction
Disturbance o f sexual function in the male after conventional p r o c t o c o l e c t o m y is a well recognized complication
and its relationship to d a m a g e o f the pelvic a u t o n o m i c
nerves fairly well u n d e r s t o o d [ 1 - 5]. By c o m p a r i s o n with
studies on men, there have been fewer investigations on
gynaecological dysfunction after such an operation, however. The m a i n difficulty encountered by w o m e n in previous studies appears to be dyspareunia and orgasmic fail-

ure [4-6], but i n f o r m a t i o n on other possible disturbances


such as menstrual disorders and vaginal discharge is
superficial and scant. Surprisingly little effort has been
m a d e to study h o w surgery and subsequent postoperative
adhesion f o r m a t i o n m a y change pelvic a n a t o m y and
affect the function o f vagina, uterus, the fallopian tubes
and ovaries.
The aim o f this study was to identify any gynaecological problems and their possible relationship to postoperative anatomical changes o f the genital tract in a series o f
w o m e n subjected to conventional p r o c t o c o l e c t o m y . A n
a t t e m p t was also m a d e to assess the relative chances o f
these w o m e n b e c o m i n g pregnant.

Material and methods


Seventy-one women who had a proctocolectomy for ulcerative colitis (n=41) or Crohn's disease (n=31) were interviewed at the follow-up clinic about their gynaecological history before and after
surgery according to a standard form. The age distribution is shown
in Fig. 1. Eleven of the 71 women were postmenopausal at the time
of the investigation. They were asked about menstrual disorders,
problems of vaginal discharge, sexual disorders such as dyspareunia
and fertility problems. This latter detail was assessed on the basis of
the number of pregnancies achieved after attempts to become pregnant for at least one year. All women were then examined by an
independent gynaecologist. Vaginal secretion was arbitrarily graded
as no secretion, moderate secretion (posterior fornix filled with fluid
but as cervix identified without prior wash out) or heavy secretion
(upper third of vagina filled with fluid, cervix identified after washout). Anatomical changes of the vagina were classified as a = none,
b = caudal fixation of the posterior vaginal fornix but no dilatation,
c = dilatation of posterior vaginal fornix but no caudal fixation and
d=caudal firm fixation and dilatation of the posterior vaginal
fornix (see Table 1).
Bacterial culture from the cervical canal was performed for
Chtamydia trachomatis in 33 women. Cytological evaluation of the
cervical epithelium was performed in 37 women.
Statistics." For comparison between groups, Chi square analysis was

used.

50
Table l. Vaginal discharge related to change in vaginal anatomy

No of
women

201

Amount of vaginal secretion

Anatomical
changes

17

No

Moderate

Heavy

Total

None

13

Caudal fixation of the


post vag. fornix but no
dilatation

Dilatation of the post


vag. fornix but no
caudal fixation

Caudal firm fixation


and dilatation of post
vag. fornix

13

30

44

15

24

32 (45%)

71

15

10

0
20-25

26-30

31-35

36-40 41-45

46-50

51-55 56-60

Age cohorts (5 years)

Total

Fig. 1. Number of women in each 5 years age cohorts


Table 2. Menstrual pattern before and after surgery

Results

Interviews
T h e r e were n o significant differences in the m e n s t r u a l
cycle b e f o r e a n d after s u r g e r y (Table 2). H o w e v e r ,
9 0 % (54/60) o f the p a t i e n t s s t a t e d t h a t m e n s t r u a l bleeding lasted o n a v e r a g e o n e d a y l o n g e r t h a n b e f o r e the
operation.
T h e m a i n g y n a e c o l o g i c a l p r o b l e m was v a g i n a l discharge. Before surgery, 9 % (6/71) o f w o m e n h a d this
p r o b l e m r e g u l a r l y while 4 9 % (35/71) o f the w o m e n
c l a i m e d t h a t they h a d c o n s t a n t p r o b l e m s w i t h v a g i n a l
d i s c h a r g e after s u r g e r y (Table 3). T h e degree o f v a g i n a l
d i s c h a r g e was r e l a t e d to the p o s i t i o n o f the b o d y in t h a t
the s y m p t o m was m o r e p r o n o u n c e d w h e n the w o m e n
rose f r o m sitting or lying.
D y s p a r e u n i a was r e p o r t e d b y 12% (8/66) o f the
w o m e n b e f o r e s u r g e r y a n d b y 2 7 % (18/66) after s u r g e r y
(Table 3). Sexual a c t i v i t y as j u d g e d f r o m f r e q u e n c y o f
i n t e r c o u r s e ( > 1 c o i t u s / w e e k ) d i d n o t differ b e f o r e (44/
66) a n d after s u r g e r y (44/66), however.
Fifty-four women did not take any contraceptive
m e a s u r e s b e f o r e s u r g e r y a n d 7 2 % (39/54) b e c a m e p r e g n a n t . T w e n t y - s e v e n w o m e n a t t e m p t e d to b e c o m e p r e g n a n t after s u r g e r y b u t o n l y 3 7 % (10/27) succeeded. N o n e
o f the w o m e n in this g r o u p h a d a n y h i s t o r y o f g y n a e c o logical infection o r a b d o m i n a l s u r g e r y except the p r o c t o c o l e c t o m y . T h e d e c r e a s e in p r e g n a n c y r a t e was statistically significant (p < 0.001). T h e r e were no differences in
p r e g n a n c y r a t e b e f o r e o r after s u r g e r y w i t h r e s p e c t to
t y p e o f i n t e s t i n a l disease (Table 4). F u r t h e r m o r e , a m o n g
10 w o m e n w h o h a d b e e n p r e g n a n t b e f o r e b u t a t t e m p t e d
to b e c o m e p r e g n a n t after surgery, o n l y 3 succeeded.
T h e r e were no m a j o r p r o b l e m s d u r i n g p r e g n a n c y or
delivery. TwentY-0ne were d e l i v e r e d v a g i n a l l y a n d 6 b y
c a e s a r e a n section.
C o n t r a c e p t i o n was u s e d b y 23 o f the 60 m e n s t r u a t i n g
w o m e n . T h e m e t h o d s used are listed in Table 5.

Characterization
of menstruation

Before surgery
no. of women

After surgery
no. of women

Regular
Irregular
Dysmenorrhoea

52
8
18

48
12
22

Table 3. Gynaecological symptoms before and after surgery

Symptom

Before surgery
no. of women

After surgery
no. of women

Vaginal discharge
Dyspareunia

6/71 (9%)
8 a/66 (12%)

35/71 (49%)*
18/66 (27%)**

a Four of these women did not have any dyspareunia after surgery;
*p<0.001, **p<0.05
Table 4. Fertility before and after surgery

Fertility

Pregnant
Not pregnant
Total

Before surgery
no. of women

After surgery
no. of women

UC

CD

UC

CD

19
8

20 (72%) *
7 (28%)

6
8

4 (27%) *
9 (73%)

54

27

9p < 0.001; UC = ulcerative colitis; CD = Crohn's disease


Table 5. Contraceptive methods used

Method

No. of women

Sterilized
Barrier method
Husband sterilized
Oral contraceptives
Intrauterine device

1
1
1
10
10

Total

23

51

GynaecoIogical examination
The most frequent change in vaginal anatomy observed
in 61% (44/71) of the patients was caudal fixation of the
posterior vaginal fornix which was dilated into a pouch
(Table 1). Heavy secretion was demonstrated in 68%
(30/44) of the women with combined caudal fixation and
pouch formation of the posterior vaginal fornix, while
this symptom was significantly less common (p < 0.05) in
the other subgroups (Table 1).
The uterus could be palpated in 78% (54/69) (two
women were hysterectomized). It was firmly fixed in a
retroverted position in 57% (31/54) of the women.
The ovaries could be identified in 48% (34/71). However, in only 8% (6/71) of the women was at least one
of the ovaries mobile. None of the 33 cultures for
Chlamydia trachomatis was positive. All 37 cytological
evaluations of cervical epithelium were normal.

Discussion

A profuse vaginal discharge was the most common complaint in the present series of women, constituting a daily
problem in almost 50% and being most marked on positional changes. A heavy retention of vaginal secretion
was demonstrated more frequently in those who had the
most exaggerated changes in vaginal anatomy with caudal fixation and dilatation of the posterior vaginal fornix.
The observations are in accordance with those reported
by Emblem and Stray-Pedersen [6]. The postoperative
distortion of vaginal anatomy as demonstrated at the
gynaecological examination probably interferes with
vaginal drainage and appears therefore to be the principal cause of this distressing symptom. These abnormalities have been documented in a recent radiological investigation of these women where the topographic changes
of the vagina and displacement of the adnexa were confirmed [7].
As regards the effect of operation on the act of intercourse information in literature is incomplete and the
results varied. Apart from psychological factors which
may have a considerable impact on sexual life in ileostomy patients, mechanical difficulties during intercourse
with discomfort and pain due to perineal scarification or
suppuration or vaginal stenosis are also reported to occur
in these patients [1, 2, 6]. In the present study about one
third of the women complained of dyspareunia postoperatively which is in accordance with previous reports.
Nevertheless, despite the increased incidence of this complaint after surgery there was no significant change in
their postoperative sexual activities. This is an interesting
observation that has been noted by others. In fact even an
enhancement of sexual relationships is often reported to
occur postoperatively being attributed to improvement in
general health [2, 4].
To what extent proctocolectomy influences fertility is
not known. The disease itself and its impact on fertility
increases the complexity of the problem. About 3/4 of the
women in the present study who attempted to become
pregnant before surgery succeeded, a figure which is close

to that of healthy fertile women [8]. In contrast only one


third of the women who attempted to become pregnant
after surgery succeeded. The pre- and postoperative pregnancy rate was similar in patients with Crohn's and ulcerative colitis. The diminished chances of women becoming
pregnant after this operation is not surprising considering the gross postoperative distortion of pelvic anatomy
caused by adhesion formation in the vicinity of the fallopian tubes and the ovaries. Hysterosalpingography disclosed occlusion of at least one tube in the majority of
these women [7].
Infertility that seems to be present in a high proportion of the women after conventional proctocolectomy
raises two important questions. Which patients are infertile and using contraception unnecessarily, and what can
be done for those who prove to be infertile but wish to be
pregnant? Hysterosalpingography is justified in these
women. Women with occlusion of the fallopian tubes
could then be spared the inconvenience and/or risks of
taking contraceptive pills. As for those with tubal occlusion who have a strong wish to become pregnant, microsurgery is probably an unsuitable approach considering
the likely extent of pelvic adhesions. In vitro fertilization
appears therefore to be a more realistic alternative [9].
It was gratifying that all women who conceived went
through pregnancy and parturition without any incident,
a few delivered by caesarean section. Other authors have
recorded equally reassuring experiences [10, 11].
The results of the present study show that distressing
vaginal discharge, dyspareunia and infertility are common sequelae after conventional proctocolectomy, contributing greatly to long term morbidity. It appears very
likely that these problems are partly or entirely related to
postoperative changes in vaginal anatomy, distortion
and fibrosis in the pelvis and adhesion formation. Since
removal of the sphincters and the pelvic musculature is
not necessary for benign disease, preservation of these
structures by an intersphincteric technique has been advocated by some authors [12] while mucosal proctectomy
preserving the entire sphincter apparatus and perineal
area is recommended by others [13]. The advantages of
such techniques with respect to gynaecological symptoms
are not known. Restorative proctocolectomy with replacement of the rectum by an ileal pouch and an ileoanal
anastomosis has become the first alternative for treatment of ulcerative colitis in many specialist centres. Apart
from preservation of the sphincters and anus the "new"
rectum prevents posterior displacement of the vagina and
caudal sliding of the adnexae. Gratifying results with
fewer gynaecological complaints have been reported [14],
while conflicting views are presented by others [15]. To
what extent the new technique might lessen the risk of
infertility also remains to be shown.

Acknowledgements. This study was supported by the Swedish Medical Research Council (No 2873 and No 3117), the University of
G6teborg, G6teborgs Lfikaresfillskap,Assar Gabrielssons fond and
AB Skandias 100-firsfond.

52

References
1. Watts J, deDombal FT, Goligher JC (1966) The early results of
surgery for ulcerative colitis. Br J Surg 53:1005-1010
2. Watts J, deDombal FT, Goligher JC (1966) Long term complication and prognosis following major surgery for ulcerative
colitis. Br J Surg 53:1014-1022
3. Grfiner OPN, Fretheim B (1977) Marital status and sexual
adjustment after colectomy. Scand J Gastroentero112:193 197
4. Fasth S, Filipsson S, Hellberg R, Hult~n L (1978) Sexual dysfunction following proctocolectomy. Ann Chir Gynecol 67:
8-11
5. Neal DE, Parker AJ, Williams NS, Johnston D (1982) The long
term effects of proctectomy on bladder function in patients with
inflammatory bowel disease. Br J Surg 69:349-352
6. Emblem R, Stray-Pedersen S (1988) Kvinners problemer etter
proktektomi. Tidsskr Nor Laegeforen no. 6, 108:466-467
7. Aszt61y M, Hult6n L, Wikland M (in press) X-ray changes in
the pelvis of proctocolectomized women. Int J Colorect Dis
8. Short RV (1984) When conception fails to become a pregnancy.
Maternal recognition of pregnancy. CIBA foundation symposium. Excerpta Medica, Amsterdam 64:377-394
9. Wikland M, Enk L, Hammarberg K, Nilsson L (1987) Use of
a vaginal transducer for oocyte pick-up in an IVT-ET program.
J Clin Ultrasound 15:245-249

10. Roy PH, Sauer WG, Beahrs OH, Farrow GM (1969) Experience with ileostomies. Evaluation of long term rehabilitation in
497 patients. Am J Surg 119:77-81
11. Hudson CN (1972) Ileostomy in pregnancy. Proc R Soc Med
65:281-283
12. Lyttle JA, Parks AG (1977) Intersphincteric excision of the
rectum. Br J Surg 64:413-416
13. Fasth S, Oresland T, Ahr6n C, Hult6n L (1985) Mucosal proctectomy and ileostomy as an alternative to conventional proctectomy. Dis Colon Rectum 28:31-34
14. Metcalf AM, Dozois RR, Kelly KA (1986) Sexual function in
women after proctocolectomy. Ann Surg 204:624-627
15. Oresland T, Fasth S, Nordgren S, Hult6n L (1989) The clinical
and functional outcome after restorative proctocolectomy. A
prospective study in 100 patients. Int J Colorect Dis 4:50-56

Dr. M. Wikland
Department of Obstetrics
and Gynecology
Sahlgrens' Hospital
S-413 45 G6teborg
Sweden

Int J Colorect Dis (1990) 5:53-60

Croibre | l|
Disease

9 Springer-Verlag 1990

Abstracts from the Third Meeting


of the Leeds Castle Polyposis Group
15-17

June

1989

T h e third m e e t i n g of the Leeds Castle Polyposis G r o u p


t o o k place at D o r m y H o u s e Hotel, Broadway, Worcestershire between 1 5 - 1 7 J u n e 1989 u n d e r the c h a i r m a n ship o f Mr. James P. S. T h o m s o n , H o n o r a r y D i r e c t o r of
The Polyposis Registry, St. M a r k ' s Hospital, L o n d o n .
The abstracts of the 27 free papers presented are p r i n t e d
and, in addition, sessions were devoted to genetics a n d
data collection.
It was decided t h a t the Leeds Castle Polyposis G r o u p
should c o n t i n u e as a n i n t e r n a t i o n a l , m u l t i d i s c i p l i n a r y
scientific o r g a n i s a t i o n to study the p r o b l e m s o f p o l y p o s i s
a n d to foster friendship a m o n g s t those involved. To that
end a C o u n c i l has been elected u n d e r the c h a i r m a n s h i p o f
Dr. D a v i d J a g e l m a n , Cleveland Clinic F l o r i d a , 3000
West Cypress Creek Road, F o r t L a u d e r d a l e , F l o r i d a
33309, U S A (Telephone: 305/978 5236; F a x : 305/
978 5022) with Dr. Steffen B u l o w o f D e n m a r k as d e p u t y
c h a i r m a n . The a d m i n i s t r a t i v e h e a d q u a r t e r s will be T h e
Polyposis Registry at St. M a r k ' s Hospital, City Road,
London ECIV2PS
(Telephone: 0~-601 7958; F a x :
01-250 3741). All enquiries should be m a d e to the a d m i n istrative secretary, Miss K a y Neale, at that address.
10 cases of desmoid tumours associated with familial adenomatous
polyposis (FAP)

R.A. Audisio, C. Rossetti,


L. Gennari

L. Bertario,

P. Sala,

C. Clemente,

Registro Nazionale Delle Poliposi Coliche Familiari, Milan, Italy


Ten patients (6 male) affected by FAP developed desmoid tumours.
The presence of oestrogenic and progestinic receptors was tested on
the desmoid tumour patients with negative results. Two patients had
the diagnosis of FAP (at 47 and 31 years) before developing a
desmoid tumour of the mesentery 6 and 16 months respectively after
abdominal surgery, whereas 8 were first seen for desmoid tumours
(median age 28 years; range 13-45) without any previous surgical
procedure: the first affected site was the abdominal wall in 2 patients, thoracic walt in 3 patients and mesentery in 3 patients.
Among them, 5 patients developed a mesenteric desmoid tumour a
median of 20 months (range 8-38) after abdominal surgery. Overall, 27 operations were performed to remove desmoid tumours occurring in 16 different sites, but only 5 have not recurred (3 patients). Three patients show no evidence of desmoid tumours at 24,
103 and 120 months from diagnosis; 6 patients are alive with

desmoid tumours at a median of 42 months (range 4-96). One


patient died of intestinal occlusion 54 months from diagnosis. This
series suggests that desmoid tumour may occur before surgical procedure although the surgical trauma can play an important role in
subsequent desmoid tumour growth. Surgical excision alone is an
unsatisfactory treatment.
Desmoid tumours and viruses

A.D. Spigelmanl, S. Argent 1, C. Wells1, C. Crofton-Sleigh2,


S. Venitt 2 R. K. S. Phillips 1
1 St. Mark's Hospital, London and Professorial Surgical Unit,
St. Bartholomew's Hospital, London, UK
2 Institute of Cancer Research, Royal Marsden Hospital, Sutton,
UK
Of 197 patients with familial adenomatous polyposis (FAP) who
underwent colectomy and ileo-reetal anastomosis, 17 (9%) had
desmoid tumours (abdominal wall only 6, intra-abdominal only 6,
both 5; deaths 4). A further 17 patients (primary surgery elsewhere
13, different primary surgery 4) give a total experience of desmnid
tumours of 34 cases with 7 deaths (all young). Rogalski [1] postulated that a transmissible tumour-inducing virus is present in cell free
desmoid extracts. Accordingly, we have looked for evidence of a
mutagenic virus in desmoid tissue.
Snap frozen desmoid tissue removed from four patients was
diluted with saline, ground, homogenised, centrifuged and the supernatant aspirated as the extract. Inoculation in viral culture media (human embryo kidney, lung; Rhesus monkey kidney) and
examination using transmission electron microscopy failed to
demonstrate the presence of a virus. Reverse bacterial mutation
assays using 3 bacterial strains (S. typhimurium TA98 and TA100;
E. coli WP2uvrApKMI01) were negative.
Desmoid tumours are a formidable problem in FAP patients.
We have been unable to provide evidence to support a mutagenic
viral-desmoid link.

Reference
1. Rogalski VJ (1972) On carcinogenic effect of extract from colon
polyps and desmoids. Vop Onkol 18:58-63
The use of triphenylethylene drugs with desmoid tumours

M. D. Brooks, A.A. Colletta, M. Baum


Department of Surgery, Kings College Hospital, London, UK
Triphenylethylenes are a group of drugs traditionally classed as
antioestrogens which include tamoxifen and its newer chlorinated

54
analogue toremifene. Until now their use has largely been confined
to malignant breast disease both in the adjuvant and advanced
setting.
Desmoid disease appears to have a predilection for young pregnant women, a fact which leads to the suggestion that oestrogens are
mitogenic to such fibroblast tumours, which in turn implied that
oestrogen blockade may be useful in treatment. A number of reports followed describing desmoid regression with antioestrogen
therapy [1, 2].
Despite the clinical success of the triphenylethylene group in
breast and some desmoid tumours, their mechanism of action has
not yet been clearly defined. Evidence that simple oestrogen blockade may not be the whole story came from trials of adjuvant tamoxifen in breast cancer where a significant response was seen regardless of oestrogen receptor status [3]. Similarly, desmoid tumours
have been shown to be receptor-poor. Recent work has suggested
that triphenylethylenes act in part through the fibroblast stroma of
breast turnouts with paracrine effects on the epithelial elements.
This view has been supported by observations of clinical response
in a series of 10 patients with desmoid disease treated with these
drugs.
The study group consisted of 6 females and 4 males with an
average age of 32 years. Six patients had additional features of
Gardner's syndrome (4 female, 2 male), and 50% of these had a
positive family history. Follow-up was between 2 and 32 months. A
response was seen in 70% of patients, ranging from tumour necrosis
(2 patients), marked tumour softening and shrinkage (4 patients), to
mild softening (1 patient). One patient had static disease, one had
rapidly progressive disease held static for 3 months before disease
progression, and one had no response to progressive disease and
died after 2 months.

References
1. Kinzbrunner B, Ritter S, Domingo J, Rosenthal CJ (1983)
Remission of rapidly growing desmoid tumours after tamoxifen
therapy. Cancer 52:2201-2204
2. Waddell WR, Gerner RE, Reich M P (1983) Nonsteroid antiinflammatory drugs and tamoxifen for desmoid tumours and
carcinoma of the stomach. J Surg Oncol 22:197-211
3. (1988) Early breast cancer triallists. Effects of adjuvant tamoxifen and ofcytotoxic therapy on mortality in early breast cancer:
an overview of 61 randomised trials among 28,896 women. N
Engl J Med 319:1681-1690

Gastroduodenal polyps are more prevalent in familial adenomatous


polyposis (FAP) than formerly recognized
O. Fausa, A. Bergan, K. Elgjo
Section of Gastroenterology, Medical and Surgical Department,
and Institute of Pathology, Rikshospitalet, Oslo, Norway
During a 4 year period, 1985-1988, upper gastrointestinal endoscopy with biopsies was performed routinely in FAP patients.
Either at follow-up after colectomy or preoperatively 41 patients (14
women) were examined, mean age 29 years (range 6-62). Duodenal
polyps were observed in 38 patients (93%) and gastric polyps in 27
(66%) (Table 1).
Gastric polyps were small ( < 6 mm) and multiple, mostly located in the fundus, but also found in the corpus. Histology regularly
revealed gland hyperplasia and dilatation. In addition, a solitary
antral adenoma was observed in one patient. The duodenal lesions
were multiple; keep in more than 20 polyps were detected in the
descending part, showing large variability in size and shape. Most
polyps were small. Seven patients (mean age 38 years) had one or
more large ( > 2 cm) polyps. In 21 patients (mean age 25 years) all
polyps observed were < 6 mm. Definite polyps could not always be
identified - localized mucosal protrusions of an irregular shape were
sometimes seen instead. Irregular adenomatous hyperplasia or nilcroadenomas could be seen on histology, which proved all polyps
to be true tubular or tubulovillous adenomas. Histological exami-

nation of large polyps invariably showed dysplasia and in one patient invasive carcinoma.

Table 1. Findings at gastro-duodenoscopy in FAP patients (n = 40)

Postoperative group

Age (years) mean (range)

Patients with
polyps

At
colectomy

Duo- Gastric
denal

At
endoscopy

25 (10-43) 33 (14-62)

23

16

24 (6-50)

15

11

(n=25)
Preoperative group
(n = 16)

Conclusion: The high frequency of adenomatous lesions observed


may indicate a prevalence of duodenal adenomas close to 100% in
FAP. It is a premalignant condition and these patients should be
submitted to endoscopy at regular intervals. Endoscopic removal
seems appropriate when possible, but prophylactic surgery has to be
considered in selected patients.
Gastro-duodenal adenomas in polyposis - is bile the missing link?
A. D. Spigelman 1, C. B. Williams 1, C. Crofton-Sleigh 2, S. Venitt z,
M. Granowska 1, R. K. S. Phillips 1
1 St. Mark's Hospital, London, and Professorial Surgical Unit,
St. Bartholomew's Hospital, London, U K
2 Institute of Cancer Research, Royal Marsden, Hospital, Sutton,
UK
Familial adenomatous polyposis (FAP) patients have a 100-fold
increased risk of duodenal cancer without an increased risk of gastric cancer. Eighty FAP patients (48 male) underwent gastro-duodenoscopy (side viewing video-endoscope 73, forward viewer 7).
Duodenal adenomas were proven histologically (including 5 microadenomas) in 73 (91%) and gastric adenomas in 5 (6%). Polyps
were seen in the duodenal bulb in 8 patients (10%) and in the second
part in 70 (88%). Gastric and duodenal bulb adenomas were always
accompanied by denser distal duodenal involvement. The ampullary area (within 5 m m of the papilla) was adenomatous in 52/72
(72%).
Four of the gastric adenoma patients were tested for duodenogastric biliary reflux. 80 mBq 99rnWgD I D A was given intravenously,
followed by 5 microgrammes of ceruletide, serial gamma camera
images obtained, and a 30 mBq 99mWClabelled milk drink given to
outline the stomach. Bile reflux was seen in three patients (Grades
1 a, 3 b, 3 b) but not in the fourth, where scanning was interrupted
when the patient complained of nausea (reflux not seen on resumption).
These observations parallel mucosal exposure to bile and may
be explained by abnormalities in FAP bile, mucosa or both.

Restorative proctocolectomy with ileal pouch anastomosis


in familial adenomatous polyposis
E. Tiret, A. Kartheser, J. P. Sales, P. Frileux, R. Parc
Centre de Chirurgie Digestive, Hopital Saint-Antoine, Paris, France
Restorative proctocolectomy with ileal-pouch anastomosis has
modified surgical attitudes towards FAP. The procedure removes
all diseased mucosa and obviates the need for a permanent ileostomy.
O f 221 patients who have undergone this procedure between
October 1983 and February 1989, 96 (43.5%) had FAP (49 men),
with a mean age of 30 years (11-59). Eighteen had undergone a
previous ileorectostomy and 3 a left colectomy. All the patients had
a two loop (J) reservoir and a diverting ileostomy closed 2 months
later.

55
Thirty-one (30%) patients had an adenocarcinoma discovered
at operation. Six (6%) patients had a cancer preoperatively diagnosed.
One patient died of cardiac arrhythmia. Eighteen patients (18 %)
had a complication after the first operation, requiring 4 reinterventions. Six (6%) patients had a complication of ileostomy closure.
No reservoir had to be removed. Mean hospital stay was 24 days
(14-80), including ileostomy closure.
Fifteen patients (15 %) had late complications requiring reoperation in 8 cases. Ten out of 15 developed intra-abdominal desmoid
tumour.
Functional results have been assessed at 1, 3, 6 and 12 months
and yearly thereafter. Mean follow-up was 26 months ( i - 6 0 ) . All
patients could evacuate spontaneously. Mean frequency of defaecation was 4.9 at 1 month, 4.8 at 3 months, 4.2 at 6 months and I year.
Fifty four per cent of patients had night evacuation at 1 month and
30% at 1 year. Daytime continence was normal in 94% of patients
at 1 m o n t h and 95% at 1 year and night-time continence in 81% at
I m o n t h and 88% at 1 year. Except for two patients who had a
radical rectal excision for cancer, leaks were minor. Fifty-four percent of patients had stopped antidiarrhoeal medication at 3 months.
No pouchitis has been observed.
Ileal pouch anastomosis achieves a good functional result in
FAP and avoids the risk of further rectal cancer.

Studies on histogenesis of microadenoma in familial


adenomatous polyposis

bowel were tested on autopsied, resected or biopsied specimens


obtained from the right colon, left colon and rectum of the various
patient groups including 36 non-neoplastic and non-inflammatory
control subjects, 34 with adenoma, 44 with cancer and 10 with
familial adenomatous polyposis (FAP) in order to find their applicability for the cancer high risk surveillance.
U E A - / , PNA and GSA-II were positive in nearly 100% of
cancer tissue regardless of their location, while DBA was negative
in the majority. In adenomatous tissue, all lectins showed positive
binding at a rate of about 80%. In the background normal mucosa,
UEA-1 always stained the right colon but rarely stained the rectum
in the control group. UEA-1 binding ability of the rectal mucosa
was increased to 26.5% in adenoma patients, to 43.2% in cancer
patients, and to 70% in FAP patients. The other lectins such as
PNA, GSA II and DBA were much less apparent in the background
mucosa. Adenoma patients with a U E A - I positive rectum were
found to have a family history of large bowel cancer in 3 3 . 3 % ,
significantly higher (/)<0.05) compared with 4.0% in the patients
with negative rectal mucosa. In cancer patients with a UEA-1 positive rectum, the family history was positive in 63.2%, significantly
(p<0.01) increased above 4.0% seen in cancer patients with a negative rectum.
The result suggests that the apparently normal rectal mucosa of
the person who is at genetically high risk for colon and rectal
cancer has specific lectin binding ability similar to adenomatous
tissue, and that the simple method using UEA-1 staining on a rectal
biopsy specimen can be practically useful for an identification of the
familial cancer high risk group-

S. Baba 1, S. Morioka 1, I. Ogawa 1, S. N a k a m u r a 2


Departments of 1 Surgery and 2 Pathology, Hamamatsu University,
School of Medicine, Hamamatsu, Japan

Brain tumour associated with adenomatons polyposis (Turcot


syndrome): an analysis of seven cases from one registry

In order to study the histogenesis of microadenoma, the following


studies were performed in 25 cases of FAP:
1. Colonic mucosa of the distal colon was observed by magnifying
sigmoidoscopy.
2. Resected colons were irrigated with oxygenated artifcial blood
supplemented with 3H thymidine or BrdU to label the S phase
cells.
3. Microadenomas in the mucosa of the resected specimen were
observed by stereoscopy.

S. R. Hamilton, A.J. Krush, F. Taqi, E M. Giardiello, S.V. Booker

Results
1. Single gland or oligoglandular adenomas were observed by magnifying sigmoidoseopy. The density of microadenomas was examined in the resected specimen revealing the difference in densely
populated type and scattered type.
2. The proliferative zone of background mucosa was confined to the
lower two thirds of the mucosa. Neither expansion nor shift of the
proliferative zone to upper part of the mucosa was observed.
3. Single gland adenomas were detected in the upper part of the
colonic mucosa. It seems that the cell which has a potentiality to
become an adenoma flows upward to the mucosal surface by natural cell flow and when it reaches the surface, develops into a single
gland adenoma.
4. Labelling indices of background normal mucosa, adenoma and
invasive cancer were calculated as 4.7_+2.2, 14.6_+4.3, and 25.2
_+5.4, respectively.

Department of Pathology, Oncology Center and Division of Medical Genetics and Gastroenterology, Department of Medicine, The
Johns Hopkins University School of Medicine and Hospital, Baltimore, Maryland, USA
The definition and inheritance of Turcot syndrome are controversial. We therefore analyzed the seven cases in the Johns Hopkins
Polyposis Registry. All seven patients had a parent with adenomatous polyposis involving the large bowel and five had at least one
sibling with polyposis, but only one patient had another family
member with a brain tumour (sister of unaffected parent). Four
patients had a cerebellar medulloblastoma (ages 5, 6, 14 and 25
years), one had a frontal lobe ependymoma (age 16), and one had
a parietooccipital glioblastoma multiforme (age 54). Polyposis developed before the brain tumour in one patient (interval 6 years)..
Polyposis was diagnosed after the brain tumour in three patients
(intervals 4, 5 and 7 years), and another had adenomatous epithelium in grossly normal colonic mucosa at autopsy (age 5). The other
two patients were not examined for polyposis or were negative
(deceased at ages 13 and 6). Three patients had additional extracolonic lesions (cutaneous cysts, cranial osteomas, papillary carcinoma of the thyroid or pigmented ocular fundus lesions); the
other four had family members with both extracolonic lesions and
polyposis. We conclude the "Turcot syndrome" is a manifestation
of pleiotropy in familial adenomatous polyposis syndrome rather
than a distinct inherited condition.

Lectin staining of neoplastic and normal background mucosa of the


colon and rectum in non-polyposis and polyposis patients

The value of screening and central registration of families


with familial adenomatous polyposis

J. Utsunomiya 1, T. Kuroki 1, y. Miki 1, T. Yamamura 1,


A. K u b o t a 2

H. E A. Vasen, G. Griffioen, G.J.A. Offerhaus, E C. A. den Hartog


Jager, I. S. J. van Leeuwen-Cornelisse, C. B. H. W. Lamers,
E.A. van Slooten

1 Second Department of Surgery and


2 First Department of Pathology, Hyogo College of Medicine,
Hyogo, Japan
The binding activities of the lectins UEA-1, PNA, GSA-II and D BA
to the neoplastic as well as to the background mucosa of the large

Foundation for the Detection of Hereditary Tumours, Utrecht, The


Netherlands
In 1984 a national registry of families with familial adenomatous
polyposis (FAP) was set up in The Netherlands. Eighty-two families

56
had been registered by the end of 1988. The diagnosis FAP was
histologically confirmed in 230 patients. These patients were subdivided into two groups. Group A comprised patients with FAP
referred because they were symptomatic, and group B relatives of
these patients who were found by screening. We compared these
groups with respect to the occurrence of colorectal carcinoma.
Forty-nine of the 104 patients in group A (47%) and five of the 126
patients in group B (4%) were found to have a colorectal carcinoma
at the time of diagnosis of FAP. The average age at diagnosis of the
104 patients in group A was 35 years (range: 13-66) and that of the
126 patients in group B was 24 years (range: 8-59). By the age of
40, 90% and by the age of 50, 97% of the patients in group B had
been diagnosed. Late onset of FAP was found in four families.
These results show conclusively that screening leads to the early
detection of FAP. The value of a national registry is proved by the
finding of many at risk family members who had not previously
been screened. Screening should start between the ages of 10 and 12
and should continue up to the age of 50. In the rare cases of fatuities
with an apparently late onset of FAP, screening should be continued
up to age 60.
Reasons for surveillance failure in familial adenomatous polyposis
(FAP), and the impact of a central register
E A. Macrae, D. J. B. St. John, E. Muir, A. M. Cuthbertson,
B. Teltscher
Department of Gastroenterology and Colorectal Unit, The Royal
Melbourne Hospital, and Esso Familial Polyposis Register, Melbourne, Australia
Until recently, there had been no registration of FAP families or
central organization of surveillance in Australia. In the present
study, the experience of (20) families associated with The Royal
Melbourne Hospital was documented. The reasons for any failure
of management were studied and the impact of a hospital-based
register on disease management was assessed. In each family, results
were categorised according to whether or not "at risk" relatives had
been diagnosed at surveillance examinations, and whether the Hospital register was involved. Reasons for failure of surveillance were
ascertained by interview with family members and their medical
practitioners.
Prior to register involvement, 24 family members presented with
symptomatic polyposis after the first affected case had been diagnosed. Eighteen of these had colorectal cancer (CRC) at diagnosis,
and 16 are now dead. Identifiable reasons for failure of surveillance
were family communication failure (2), family denial (2), failure of
hospital clinic (2), failure to cover extended branches of families
living locally (9), interstate (4) or overseas (3). In contrast, only 2 of
33 (6%) affected cases identified at a planned surveillance endoscopy (24 pre-register, 9 post-register) had CRC at diagnosis.
Without a dedicated system for centralised management of endoscopic surveillance of FAP families, lethal delays in diagnosis are
likely to occur. Most reasons for failure are potentially correctable
by a dedicated register responsible for notifying clinicians and patients about the timing of surveillance procedures.

The value of a local (regional) registry for familial adenomatous


polyposis (FAP) - Experience in the Northern Regional Health
Authority of England
A. G u n n ~, M. Rhodes 1, p.C. Chapman 2, j. Burn 2, C. M. Wood 3,
J. Delhanty 4, D. Roberts 2
Department of Surgery, Ashington Hospital, Ashington, U K
Departments of 2 H u m a n Genetics and 3Ophthalmology, Royal
Victoria Infirmary, Newcastle upon Tyne, U K
r Galton Laboratory, University College, London, U K
In January 1987, the registry for EAP was established in the Northern Regional Health Authority of England (population 3. I million).
All patients known to surgeons in the region were traced and in the
2 years up to January 1989, the number of at risk individuals

screened was increased from 21 to 102 and a further 106 were


contacted with a view to screening. The number of known patients
with FAP was increased from 54 to 59. Of the 59 FAP patients on
the Registry at January 1989, 34 were detected by symptoms at an
average age of 38 years and the 25 detected by screening had an
average age of 21 years. Fourteen of the patients who presented with
symptoms have developed a colorectal cancer and 17 have required
excision of the rectum. None of the screened detected patients has
had a cotorectal cancer and only five have required rectal excision.
A regional or local registry is able to:
1. identify and trace patients with FAP and at risk individuals not
previously detected by hospital and national screening
2. provide counselling and tracing skills to surgeons previously not
readily available
3. apply advanced screening techniques rapidly to patients at risk
from FAP
4. expect to reduce the proportion of patients detected by screening
and therefore reduce the morbidity and mortality of this disease.
At present, the registry is evaluating the different modes of screening to determine the optimal screening programme. It is likely that
local registries are best equipped to deal with patients and relatives
in their region but that co-ordination with a national registry will be
required.
Anthropometric measurements in Mexican kindreds
with familial adenomatous polyposis
L. Herrera
Roswell Park Memorial Institute, New York, USA
There is a great need for a clinical marker that can segregate to
relatives at risk of patients with FAP, who will eventually manifest
the disease.
Osteomas of the jaw and teeth abnormalities have been shown
to be specific and sensitive clinical markers in selected kindreds. In
this study, we decided to gather growth and bone measurements in
kindreds with FAP and to compare them to the average values of
a " n o r m a l " comparable Mexican population.
We studied 57 subjects from 3 kindreds selected at random from
the Mexican Polyposis Registry. Cranial and extracranial standard
anthropometric measurements were obtained. Eighteen members
were affected (10 females). Osteomas of the jaw were detected in
only five of the affected members (four females).
Only affected females demonstrated an upper facial height and
cormic index that exceeded one standard deviation difference over
the non-affected general population. No differences were distinguishable between affected and non-affected members within FAP
kindreds.
Anthropometric measurements were non-invasive, easy to perform, reproducible, and entailed minimal cost and effort. Patients
accept them well. These measurements have the potential to disclose
parameters of significance. Although our conclusions were based on
a small number of subjects, they encourage further studies. We have
added measurements of incisor width - another of the autosomal
Mendelian inherited phenotypic manifestations - for subsequent
clinical correlation and patient stratification.
Linkage studies on familial adenomatous polyposis (FAP)
in the Netherlands
P. Meera Khan ~, C. M. J. Tops 1. C. Breukel ~, J. Th. Wijnen 1, H. M.
yd. Klift t, I.S.J. van Leeuwen-Cornelisse 1,2,3, H. E A. Vasen a,4,
G. Griffioen 4, F. C. A. den Hartog Jager 5, C. B. H. W Lamers 4
1 H u m a n Genetics Institute, University of Leiden, Leiden
2 Clinical Genetics Centre, 3 National Familial Polyposis Registration Centre and 4Department of Gastroenterology, University
Medical Centre, Leiden, The Netherlands
s Netherlands Cancer Institute, Amsterdam, The Netherlands
A systematic registration of families with FAP was initiated in 1984.
In a nation-wide co-operative venture, so far the registration centre

57
has documented clinical, pathological and pedigree information
from more than 90 Dutch families. We employed two polymorphic
D N A probes (or markers), Pi227 and C l l p ] I, in our study. Three
polymorphic sites with Pi227 (BclI, BstXI and PstI RFLP's) and
two polymorphic sites with CI l p l 1 (TaqI R F L P and a 4-base deletion polymorphism) were screened; they showed a rather high degree of polymorphism (the polymorphism information contents
(PIC's) being 0.75 with Pi227 and 0.40 with C l l p l 1). After screening about a quarter of the above families with these markers, a two
locus linkage analysis was performed using Lathrop's computer
program, L I N K A G E . Eighty five persons in 7 families were informative for linkage between FAP and C l l p l l , as were 171 in 15
families between FAP and Pi227. The study revealed that Pi227 is
about 4 centimorgans (cM's) from FAP (peak lod score 12.29) while
C l l p 1 1 is about 6 cM's (maximum lod score 4.39). The distance
between Pi227 and C1 l p l 1 was estimated to be 3 cM's (160 persons
in 13 families; peak lod score 13.59). These data suggest that Pi227
and C1 l p l i are on the same side of the FAP locus on chromosome
5. There is no indication of genetic heterogeneity among these families.
Gene mapping studies in familial adenomatous polyposis
J. D. A. Delhanty 1, K. Tsioupra 1, M. B. Cachon Gonzalez
M. B. Davis 2, j. Burn 3

1 Galton Laboratory, University College London, London, U K


2 National Hospital for Nervous Diseases, London, U K
3 Department of H u m a n Genetics, University of Newcastle-UponTyne, Newcastle-upon-Tyne, U K
Hyperproliferation of epithelial and mesenchymaI tissues is characteristic of the autosomal dominant condition, familial adenomatous
potyposis (FAP). Congenital hypertrophy of the retinal pigment
epithelium, (CHRPE), has been found to occur in over 85% of
patients with FAP and is suggested to be a useful early marker for
the presence of the gene [1]. The gene for FAP has been mapped to
chromosome 5, region q21 22, by linkage to a random D N A segment, C l l p l l [2]. A nurnber of other linked markers have since
been isolated including Pi227 (D5S37) [3], the closest being YN5.48
(D5S81) [4] which has shown no recombinants with FAP. However,
the precise localisation of FAP in relation to these other markers
cannot be determined with any certainty due to lack of informative
meioses.
We have families which segregate for a number of markers
simultaneously, including the first recombinant between FAP and
the locus D5S71 detected by CI l p l 1 [5]. Linkage analysis has been
carried out in 11 three generation families using the probes C1 l p l 1
and Pi227. The maximum lod score obtained for FAP and D5S7]
was 2.68 at a recombination fraction (0) of 0.12, and for FAP and
D5S37 was 2.68 at a 0 of 0.14. No recombinants were observed
between D5S71 and D5S37. These data place the marker loci close
together on the same side of the FAP gene but the precise order of
these three loci cannot yet be obtained.
These families also manifest the C H R P E phenotype in 85% of
known gene carriers [1]. We are monitoring young, clinically unaffected individuals for C H R P E and carrying out D N A typing. So
far, the prediction for 32 patients has been altered by D N A analysis
combined with C H R P E phenotype.

References
1. C h a p m a n PD, Church W, Burn J, G u n n A (1989) Congenital
hypertrophy of retinal pigment epithelium: a sign of familial
adenomatous polyposis. Brit Med J 298:353-354
2. Bodmer WF, Bailey CJ, Bodmer J (1987) Localization of the
gene for familial adenomatous polyposis on chromosome 5.
Nature 328:614-616
3. Meera K h a n P, Tops CMJ, v d Broek M, Breukel C, Wijnen JT,
Oldenburg M, v d Bos J, Van Leeuwen-Cornellisse IS J, Vasen
HFA, Griffioen G, Verspaget HM, den Hartog Jager F C A and
Lamers C B H W (1988) Close linkage of a highly polymorphic
marker (D5S37) to familial adenomatous polyposis (FAP) and

confirmation of FAP localisation of chromosome 5@1-11. Hum


Genet 79:183-185
4. Nakamura Y, Lathrop M, Leppert M, Dobbs M, Nasmuth J,
Wolff E, Carlson M, Fujimoto E, Krapcho K, Sears T (1988)
Localization of the genetic defect in FAP within a small region of
chromosome 5. Am J Hum Genet 43:638
5. Aldred MA, Rees M, Tsioupra K, Leigh SEA, Neale KF, Delhanty JDA (1988) Familial polyposis coll. Lancet II: 565
Molecular and genetic analysis in familial adenomatous polyposis
(FAP)
T. Sasazuki 1, M. Sasaki 1, K. Sugio 1, K. Urabe 1, 5( Yanagawa 1,
M. Okamoto 2, M. Miyak 2, y. Kaneda 3, T. Uchida 3
1 Department of Genetics, Kyushu University, Fukuoka, Japan
2 Department of Biochemistry, Tokyo Metropolitan Institute of
Medical Science, Tokyo, Japan
3 Institute for Molecular and Cellular Biology, Osaka University,
Osaka, Japan
Frequent loss of heterozygosity in colorectal carcinoma for FAP
patients was observed on chromosomes 5 (24%), 14 (20%), 17
(31%), 18 (40%) and 22 (35%), and was also observed in non-polyposis colorectal carcinoma (NPCC) on these chromosomes. This
suggests that tumour suppression genes for colorectal carcinoma
may be located on these chromosomes, and that they may play a
critical role in colorectal carcinogenesis not only in FAP patients
but also in NPCC patients.
Based on these data, we introduced a single h u m a n chromosome
5 into a carcinoma cell line using a microcell technique. H u m a n
chromosome 5, introduced into murine hepatoma cell line 7R.1,
suppressed the tumorogenicity of 7R.1 in nude mice whereas the
truncated chromosome 5 did not do so. However, the truncated
chromosome 5 suppressed the growth of 7R.1 in vitro, as did complete chromosome 5. The human chromosome 5 suppressed the
growth of human colon carcinoma cell line in vitro which showed
partial deletion of chromosome 5q. These data suggest that one of
the tumour suppression genes is located on the long arm of chromosome 5.
Chromosome 5 allele loss in familial and sporadic
colorectal adenomas
J. D. A. Delhanty 1, M. Rees x, S. E. A. Leigh 1, j. R. Jass 2
i Galton Laboratory, Department of Genetics and Biometry, University College London, London, U K
2 Department of Pathology, University of Auckland School of
Medicine, Auckland, New Zealand
D N A extracted from familial and sporadic colorectal neoplasms
was compared with constitutional D N A using a range of hypervariable locus-specific probes to assess the extent of allele loss during
conversion to malignancy. Chromosome 5 allele loss was observed
in 23% of carcinoma samples, as previously found by others [1,2].
However, we have been able to show loss of the D5S43 locus on
chromosome 5 in fore" adenomas from three patients, two of whom
had the precancerous condition Familial Adenomatous Polyposis
(FAP) [3]. These results suggest significant genetic changes involving chromosome 5 are occurring in benign familial adenomas. Use
of the probes YNZ22 and 144D6, on the short arm of chromosome
17, detected no changes in the familial adenomas whereas 30% of
both sporadic and familial carcinomas showed loss of sequences in
this chromosomal region. This is evidence that genetic alterations
on chromosome 5 precede those on 17.
Probes for chromosome ] (loci D1S7 and D1S8) and for chromosome 7 (loci D7S21 and D7S22) revealed no notable alterations
in the adenoma samples. Complete loss of alleles for loci on chromosome 7 was not observed in carcinomas but reduced intensity of
one parental allele was found in 17%. One of these specimens was
known to have multiple copies of this chromosome, suggesting
duplication of one homologous chromosome only. Using probes for
chromosome 1 on samples from 26 carcinoma patients detected no
case with deletion of the D1S7 or D1S8 loci. Deletion of material

58
from chromosome l is not a common event in colorectal carcinogenesis, despite the frequently observed cytogenetic rearrangement
of this chromosome.
Loss of chromosome 5 alleles in adenomas from familial polyposis patients provides evidence that FAP conforms to Knudson's
hypothesis.
References
1. Solomon E, Voss R, Hall V, Bodmer WF, Jass JR, Jeffreys AJ,
Lucibello FC, Patel I and Rider SH (1987) Nature 328:616-619
2. Okamoto M, Sasaki M, Sugio K, Sato C, Iwama T, Ikeuchi T,
Tonomura A, Sasazuki T, Miyaki M (1988) Loss of constitutional heterozygosity in colon carcinoma from patients with familial
polyposis coli. Nature 331:273-277
3. Rees M, Leigh SEA, Delhanty JDA, Jass JR (1989) Chromosome 5 allele loss in familial and sporadic colorectal adenomas.
Br J Cancer 59:361-365

We suggest that the predominance of diploid/near-diploid colorectal carcinoma is one of the characteristics of cancer family
syndrome. This might signify the existence of two or more pathogenetically different subgroups of colorectal carcinoma, and is in
good accordance with the view that cancer family syndrome patients with colorectal carcinoma have an improved prognosis. Previously, it has been shown that the D N A content of colorectal carcinoma measured by flow cytometry correlates well with the
chromosome analysis. Therefore, if D N A content of colorectal carcinomas is consistently diploid in several cancer family syndrome
families, as observed in the present study, this may indicate a uniform karyotype of colonic tumours in cancer family syndrome, and
would further encourage the search for a cancer family syndrome
gene. The present study will be completed by performing flow cytometric D N A analysis for colorectal carcinoma in FAP, and in ulcerative colitis and by comparing those results with the present series.

Association of congenital hypertrophy of the retinal pigment


epithelium (CHRPE) with familial adenomatous polyposis (FAP)

Genetic alterations in benign and malignant turnouts from patients


with familial adenomatous polyposis and Gardner's syndrome

T. Iwama 1 M. Yoshio 1, O. Neiich 2 I. Jirou 1

M. Miyaki 1, M. Okamoto 1, C. Sato 1, K. Tanaka 1, T. Iwama 2,


J. Utsunomiya 3, T. Mori 4

1 Department of Surgery, The Research Center for Polyposis and


Intestinal Disease, Tokyo Medical and Dental University
2 Inoue Eye Hospital, Tokyo, Japan

1 Department of Biochemistry, Tokyo Metropolitan Institute of


Medical Science
2 Polyposis Centre, Tokyo Medical and Dental University
3 Department of Surgery, Hyogo College of Medicine
4 Department of Surgery, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan
To investigate the mechanism of carcinogenesis in familial adenomatous polyposis (FAP) and Gardner's syndrome (GS), we have
analysed the loss of heterozygosity and the mutation of K-ras-2 in
22 Colon adenocarcinomas, 97 adenomatous polyps (3 - 3 0 mm) and
7 desmoid tumours from 37 patients with FAP and GS, using 32
R F L P markers and 8 oligonucleotide probes. The allele losses were
observed in colon carcinomas most frequently on chromosomes 5q
(25%), 17p (40%), 18 (33%) and 22 (33%), and also on chromosomes 6 (25%) and 12 (18%). Numerous carcinomas had multiple
simultaneous losses on different chromosomes and mutation of
K-ras-2. Similar alterations were observed in sporadic colon adenocarcinomas from 36 patients. Frequency of allele loss in adenomatous polyps was low, but several large polyps exhibited the loss on
chromosomes 5 or 17, or mutation of K-ras-2. Benign desmoid
tumours, even large ones, did not show any allele losses, although
one tumour had an abnormally sized fragment of K-ras-1 on chromosome 6.
These results suggest that the inactivation of suppressor genes
on chromosomes 17, 18 and 22, along with the FAP gene on chromosome 5, contributes to the progression of tumours from benign
to malignant stage in FAP and GS.

Ophthalmologic examinations were made on 44 persons (38 patients, 6 normal first degree relatives) from 20 FAP families. The
ophthalmologic examinations were performed by one ophthalmologist. No features of C H R P E were present in six first degree relatives
in whom the bowel appeared normal. Fifteen (51.7%) of 29 patients
who had no Gardner's manifestations had fundus lesions. Eight
(88.9%) of nine cases of Gardner's syndrome had pigmented lesions. Twenty-one of 38 patients had gastric or duodenal polyps. In
this group, the prevalence of C H R P E was 61.9% and in the patients
who had no gastric or duodenal polyps the prevalence was 70%.
There were 10 families in which 2 or more patients underwent
ophthalmologic examinations. In 5 of these families, all the 12 patients were negative for CHRPE. In three of the remaining five
families, all nine patients had CHRPE. In the remaining two families, patients with Gardner's manifestations had C H R P E more frequently than the patients without Gardner's manifestations, irrespective of gastro-duodenal polypoid lesions. The presence of
C H R P E differed between families.

A study of congenital hypertrophy of the retinal pigment epithelium


(CHRPE) in individuals at risk for familial adenomatous polyposis
(FAP and FAPG)
A. J. Krush 1, E.A. Traboulsi 2, I. H. Maumenee 1,
E. J. Gardner 3, J.P. Hughes 4, R. W Burt s, j. E. Garber 6,
E P. Li 6, F. M. Giardiello 1, S. R. Hamilton 1

t Second Department of Surgery


2 Department of Radiotherapy and Oncology and
3 Pathology Laboratory of the Department of Radiotherapy and
Oncology, University Central Hospital, Helsinki, Finland

1 Division of Medical Genetics, Division of Gastroenterology,


Department of Medicine, Department of Pathology, Department of
Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
2 The Center for Sight, Georgetown University Medical Center,
Washington, D.C.
3 U t a h State University, Logan, U t a h
4 Salt Lake City, U t a h
5 University of Utah, Salt Lake City, U t a h
6 N C I - N I H Clinical Epidemiology Branch, Bethesda, Maryland,
USA

Fifty-nine colorectal carcinomas of patients with verified cancer


family syndrome were analysed for D N A ploidy using flow cytometry. Forty tumours (68%) were diploid. Nineteen tumours (32%)
classified as DNA-aneuploid had a median D N A index of 1.24
(range 1.12-1.97). Ninety per cent of all tumours had a D N A index
less than 1.27. In studies of general colorectal carcinomas only one
third of the tumours show a D N A index less than 1.27. This predominance of diploid/near-diploid tumours in the present study was
seen b o t h in primary and in metachronous carcinomas.

From 1984 to 1988, retinas were examined by indirect ophthalmoscopy in members of 26 families with familial adenomatous
polyposis and extra-colonic lesions (FAPG - defined as FAP with
two or more family members having one or more extra-intestinal
lesions such as cutaneous cysts, osteomas, desmoids, tumours of
brain, thyroid, adrenal, duodenum, or hepatoblastoma). Eighteen
individuals at 50% risk of FAPG, due to an affected parent, were
found to have C H R P E (age range 1-30); 5 had previously had a
negative endoscopy and 13 had not yet been examined. Follow-up

Diploid predominance in hereditary nonpolyposis colorectal


carcinomas evaluated by flow cytometry
J. P. Mecklin 1, M. Kouri 2, A. Laasonen 3, K. Franssila 3,
S. Pyrhonen 2, H. Jarvinen 1

59

Table 1. C H R P E and FAP examinations


CHRPE
CHRPE +
CHRPETotal

18
26
44

FAPG +

FAPG-

Not examined

9
0
9

3
14
17

6
12
18

Summary of patients who have undergone endoscopy:


Sensitivity of those positive for C H R P E
Specificity or per cent of those without FAP
who are negative for C H R P E
Predictive value of positive C H R P E
Predictive value of negative C H R P E

9/9 or 100%

= 14/17 or 82%
= 9/12 or 75%
= 14/14 or 100%

Of 24 G r o u p I patients (median age 18 years) 16 had CHRPE,


compared with 14 out of 24 patients (median age 29 years) in
Group II and only 3 out of 30 patients in G r o u p III. The overall
sensitivity of C H R P E for FAP was 62% (_+27%) without any
difference related to ECM; the predictive value was 90.5%. The
specificity calculated from Group III (median age 26 years) was
90%, but the results should not be considered as definitive because
a longer follow-up to determine the appearance of adenomas is
required.
The data suggest that examination for C H R P E is an inexpensive, non-invasive test for FAP, but the absence of retinal lesions
does not eliminate the necessity for adequate follow-up of individuals at risk.

Eye examination in the assessment of carrier risk for familial


adenomatous polyposis (FAP)
endoscopies revealed 9 with FAPG (age range 9-30), 3 with negative examinations until 1989 (age range 16-29), and 6 who have not
yet been examined (age range 1 - 2 2 ) (Table 1).
Twenty six individuals also at 50% risk for FAPG (age range
4 - 3 6 ) had no ocular fundic lesions. Follow-up endoscopies in 14
were negative (age range 12-37). None were positive for FAPG.
Twelve individuals have not yet had an endoscopy (age range 4-18).
Reasons for individuals with positive C H R P E not being examined were: (1) four were too young for endoscopy; (2) two feared the
examination or its outcome; and for those with negative C H R P E
were: (1) four were too young; (2) two had severe financial problems; (3) six were "independent adolescents". Parental fear of the
outcome of the examination may have entered into the procrastination problem as well.
Our findings suggest that the presence of C H R P E is a clinical
marker for FAPG and the continued absence of C H R P E provides
good evidence that the person is not affected.
We pooled our data with studies of at-risk individuals from the
University of Utah, the Clinical Epidemiology Branch of the NCI,
and from recent published reports from the University of Toronto
and the University of Colorado School of Medicine. Total figures
for all five groups are:
At risk for FAP
C H R P E positive
C H R P E negative
FAP positive
FAP negative
Not examined

104
48
56
20
32
52

Of 104 persons at risk for FAP, 44 were from our group, 36 from
the U t a h group, and 24 from the other 3 groups. Eighteen of 44
individuals (41%) in our group have not yet had colon examinations compared with 23/36 (64%) in the U t a h group, 3/i 1 (27%) in
the Toronto group, 1/5 (20%) in the Boston group, and 7/8 (88%)
in the Denver group. Follow-up examinations for all those not yet
examined are needed, as well as the pooling of results from many
other registries in order to determine whether C H R P E is a good
marker for FAP and FAPG, or whether as in our study so far, it is
a marker only for FAPG.

J. Burn 1, p. D. Chapman 1, C. M. Wood 2, E Lalloo 1, j. Delhanty 3,


A. G u n n ,
Departments of 1 H u m a n Genetics and z Ophthalmology, University of Newcastle-upon-Tyne, Newcastle-upon-Tyne, U K
3 Galton Laboratory, University College London, London, U K
4 Department of Surgery, Ashington Hospital, Ashington, U K
An individual whose parent was affected by FAP begins life with a
i in 2 chance of being a gene carrier. If no polyps develop in early
adulthood the probability that the normal gene has been inherited
rises progressively. The small possibility of late onset of the disease,
however, means screening must be continued into middle life. The
use of linked D N A markers offers the prospect of improved discrimination between carriers and noncarriers, which should lead to
greater compliance with screening in those who carry the gene and
considerable psychological and practical benefits for those who do
not.
For many families, however, D N A markers are not informative
and when they are helpful there remains the problem of crossovers
between the marker and the disease which could lead to incorrect
assignment of carrier status. There remains the possibility, in addition, of genetic heterogeneity which limits the approach in several
other diseases.
We have investigated the value of indirect fundoscopy to detect
congenital hypertrophy of the retinal pigment epithelium (CHRPE)
in a total population [l]. The physical sign has proved to be highly
specific and sensitive. Among 40 obligate carriers, 37 had 3 or more
lesions whereas no control had more than 2. In addition, C H R P E ' s
in gene carriers displayed distinct morphological characteristics in
many cases. This physical sign is applicable to carrier assessment
from childhood and eye examination is well tolerated.

Reference
1. Chapman PD, Church W, Burn J, G u n n A (1989) Congenital
hypertrophy of retinal pigment epithelium: a sign of familial
adenomatous polyposis. Br Med J 298:353-354

Familial adenomatous polyposis and congenital hypertrophy


of the retinal pigment epithelium
Congenital hypertrophy of retinal pigment epithelium (CHRPE)
as a marker for familial adenomatous polyposis (FAP)
L. Bertario, C. Rossetti, E Bandello, R. A. Audisio, P. Sala,
E. Fortini, M. Pietriusti
Registro Nazionale Delle Polyposi Coliche Familiari, Milan, Italy
Seventy-eight patients from 29 kindreds were examined to evaluate
the potentiality of C H R P E as a phenotypic marker of FAP.
The examined subjects were divided into three different groups:
I
II
[II

patients with FAP without extracolonic manifestations (ECM)


patients with FAP and E C M (desmoids/osteomas/ugit adenomas)
individuals at risk without FAP.

P. J. Polkinghorne 1, S. Ritchie 2, K. Neale 2, j. p. S. Thomson


B. S. Jay 1

2,

1 Institute of Ophthalmology, University of London


2 The Polyposis Registry, St. Mark's Hospital for Diseases of the
Rectum and Colon, London, U K
In 1980 an association was described between Gardner's syndrome
and congenital hypertrophy of the retinal pigment epithelium
(CHRPE) [1]. The term Gardner's syndrome has recently been
replaced by familial adenomatous polyposis (FAP), and the association of retinal lesions has been extended to include affected patients without extra-colonic manifestations.
The ocular fundi of 74 patients with documented FAP, and 31
"at risk" individuals were studied. A comparison group of 26,

60

Table 1. Absolute number of pigmented retinal lesions in patients


with familial adenomatous polyposis and in the 'at risk' and comparison groups
No. of
lesions

Affected
(n = 72)

'At risk'
(n = 31)

Comparison
(n = 26)

0
1-5
>5

2
25
45

14
15
2

18
8
0

matched for age and sex with the affected group, were also examined. Two patients were excluded from the affected group: one had
x-linked retinitis pigmentosa and the other had a large pigmented
lesion at the macula consistent with a diagnosis of congenital toxoplasmosis.
We found that although a few lesions of C H R P E occurred in
some of the comparison group, in most cases we were able to
distinguish patients with polyposis on the basis of bilaterality, and
type and number of retinal lesions. Using the classification of Berk
et al. [2], most of the affected individuals had more than two types
of lesion and, in particular, exhibited pisciform pigmented lesions.
This latter lesion was not found in any of the comparison group.
Bilateral pigmented fundus lesions were found in 65 out of 72
patients, an additional 5 patients having unilateral lesions. The
absolute number of lesions did not change with age. In the "at risk"
group there were fewer retinal lesions than seen in the affected
group and the variety of lesions was less (Table 1).
This study confirms the observation that multiple pigmented
retinal lesions are reliable markers for FAP and that the examination of the ocular fundus may be useful in screening individuals at
risk from familial adenomatous polyposis.

References
1. Blair NP, Trempe CL (1980) Hypertrophy of the retinal pigment
epithelium associatd with Gardner's syndrome. A m J Ophthalmol 90:661 667
2. Berk T, Cohen Z, McLeod RS, Parker JA (1988) Congenital
hypertrophy of the retinal pigment epithelium as a marker for
familial adenomatous polyposis. Dis Colon Rectum 31:253-257

Mucins in familial adenomatous polyposis


J. R. Jass
University of Auckland School of Medicine, Auckland,
New Zealand
Epithelial mucin histochemistry of normal-appearing colorectal
mucosa from patients with familial adenomatous polyposis (FAP)
was studied. Differences from normal individuals, if any, might be
usefully exploited for biomarker purposes. Material was derived
from 112 patients with FAP and 137 controls with non-neoplastic
conditions. Distribution of neutral mucin was studied indirectly
using periodic acid Schiff (PAS) with and without phenylhydrazine
blockade. Increased expression of neutral mucin was demonstrated
in the right as compared to left colon in both study group and
controls, with no important differences between the two groups.
Sulphation was assessed using high iron diamine/alcian blue. No

differences were detected. The mild PAS technique was used to


assess loss of acetyl groups from sialic acid. Contrary to previous
reports, no differences between FAP patients and controls could be
demonstrated. Binding patterns of three lectins were studied in a
subgroup of 22 age- and sex-matched patients. Ulex europeus I
showed consistent binding in the right colon in study and control
groups. There was variable binding in the left colon for both groups,
with no significant differences. Peanut lectin showed little or no
binding in both groups. The majority of samples were bound by
Helix pomatia, again with no differences. In conclusion, no alterations of mucin expression within normal-appearing mucosa could
be detected despite matching a large series of FAP patients for site,
age and sex.

Mucin abnormality in colonic mucosa in patients


with familial adenomatous polyposis
T. Muto
Department of Surgery 1, University of Tokyo, Tokyo, Japan
Colonic mucosa of the left colon in 41 patients with familial adenomatous polyposis (FAP) was stained by a periodic acid-thionin
Schiff/potassium hydroxide/periodic acid-Schiff method in which
the normal colonic mucosa usually stained red and carcinoma
stained blue or purple. In FAP 82.9% stained blue or purple whereas 35.5% stained blue or purple in normal controls. The data suggest that sialomucin properties of the flat mucosa surrounding
polyps in FAP are different from those of the normal colon and that
this simple technique may be useful for the early detection of highrisk family members in the FAP family.

An automatic family tree generation for familial adenomatous


polyposis (FAP) using our coding system on a commercially available
database and calculation program for personal computers
T. Iwama 1, y Mishima 1, j. Utsunomiya 2
1 Research Center for Polyposis and Intestinal Disease, Department of Surgery, Tokyo Medical and Dental University, Tokyo,
Japan
2 Department of Surgery, Hyogo College of Medicine, Hyogo,
Japan
We have three kinds of files for our polyposis registry. They are a
registry file, a clinical file, and an identity card file. This last one is
a file of the necessary kindred of the registered patients of FAP.
Among the components of the file are family code, personal code,
relationship with the proband, date of birth and death, cause of
death, revealed disease and other medical data. The personal code
was proposed by Utsunomiya in 1980. To generate a family tree
automatically, we prepare three kinds of files or buffers. They are
a buffer for display, a main buffer for calculation of the family code
and a referring file for the marks necessary to show personal features. The most important procedure of this program is the two
dimensional arrangement of family members. The procedure is performed easily without the aid of specialists using the macro functions of a commercially available popular spread sheet program. It
shows that our coding system is easily able to identify a person in
a family. We are of course flexible enough to accept any other good
coding or computer system to monitor the members of familial
adenomatous polyposis families.

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