Imperforate Anus *
Results in 63 Cases and Some Anatomic Considerations
JOHN E. S. Scorr,** M.A. (Cantab.), M.B., B.Chir., F.R.C.S. (Eng.),
ORVAR SWENSON, M.D., F.A.C.S.
From Tufts University Medical School, Boston, Massachusetts
THE ACCEPTED way of assessing the treat-
ment of any surgical condition is by analyz-
ing the long-term results. In the voluminous
literature on the subject of imperforate
anus such analyses are notable by their
absence. Most of the published work is
concerned with methods of treatment and
the criteria used by an author in judging
a result good or bad are frequently not
stated. Sixty-three cases treated at The
Boston Floating Hospital are discussed in
this paper with particular reference to the
long-term results. Anatomic studies were
carried out in one type of deformity in
which the results were extremely poor in
order to determine whether any improve-
ment could be obtained.
Several methods of classifying the dif-
ferent types of imperforate anus have been
recommended since Bodenhammer 5 pub-
lished his monograph in 1860.6 8, 11, 14 In
1934, Ladd and Gross 13 suggested group-
ing the deformities into four types, and this
is the classification which is most frequently
used in the United States at the present
time. Denis Browne 9, 10 introduced the
concept of two major groups of deformity,
those with rectal agenesis and those
without but with an anus that was mal-
formed and misplaced; the essential differ-
*Senior Surgical Registrar, The Hospital for
Sick Children, Great Ormond Street, London,
England. At present Surgical Research Fellow,
Tufts University Nledical School, Boston, Massa-
chusetts, during the period of tenure of a Harkness
Fellowship of the Commonwealth Fund.
477
ence being that in the former the bowel
ended above the levator ani whereas in
the latter it passed through this important
portion of the sphincter mechanism. This
idea is strongly supported by the embryo-
logic and morphologic investigations of
Wood Jones 20-22 and Keith,12 and has been
emphasized with particular reference to
the relationship between the bowel and
the pelvic floor by Stephens.'6-'8 In 1936,
Stone 19 suggested that the low recto-
vaginal fistula was in fact an ectopic vulval
anus, and the possibility of local malforma-
tions or misplacements of the anus has been
recognized more recently by Bill and
Johnson.1-4
The most important feature in the im-
perforate anus is the development level of
the rectum and its relation to the levator
ani. Although the external anal sphincter
develops independently of the rectum, it is
poorly formed if the proctodeum is absent
and in most cases it fails to function even
though efforts are made to preserve it.
The levator ani is a more important muscle
layer because its central portion, known as
the pubo-rectalis, can act as a sphincter if
the bowel passes through it. In normal
anatomy, this muscle, taking origin from
the under surface of the pubic bone, curves
round the posterior aspect of the ano-
rectal junction to form a sling. By contrac-
tion it increases the angle of the junction
and closes the rectum. In some cases of
imperforate anus the bowel ends above
whereas in others it passes through the
 478 SCOTT AND SWENSON Annals of Surgery
September 1959

BLADDER

CMS I 2 3 4 5
L--- L--L----- L . ...i

...

URETER

I ~~~~~~~ ~~~~~~~~~~~~~~~~~...
..

E
CR T U M P R O S T A T E P E L V I P L SSRHYPAPASTRIC N ER
E X U

RECTUM PROSATE PELVIC PLEXUS S CAL PLEXUS

FIG. 1. Normal right hemipelvis. Medial aspect showing the pelvic plexus.
The rectum has been retracted medially.

muscle sling to open in the vulva or peri- When the dissection was complete histo-
neum. There are other important features logic sections were made to confirm the
which distinguish between these two of
presence nerve fibers. The blood vessels
groups of cases. When the bowel ends at a of three of the normal cases were injected
high level the major part of the rectum with colored liquid latex solution prior to
which is responsible for producing rectal dissection in order to determine the rela-
sensation is absent, and there is good phys- tionship between the nerves and the
iologic evidence that this is one of the vessels.
important features of the defecation reflex. In the normal pelvis (Fig. 1, 2) the find-
The pelves from four cases of high im- ings were similar to Michell's 15 description
perforate anus were dissected and com- except that no contribution to the pelvic
pared with nine normals. The half pelvis plexus from the second sacral nerve was
including the whole rectum and bladder found in any of the specimens. This feature
was removed at autopsy, fixed in 10 per was also described by Caria Mendes.7 The
cent formal saline and dissected in stages. hypogastric nerves carrying sympathetic
Volume 150 IMPERFORATE ANUS
Number 3 479
~~~~~~~~~~~~~~~~~~~~~~.
e_t'- PELVIGC PARASYMPATHETIC
NERVES
PELVIC PLEXUS
... ./

. .si. ._-~ l~ ~ ~ ~ ~ ~ .
ANI

OBTURATOR

_ _ _ ....~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

paay ptei
n:.:e:
JF~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~S1TIC_
i
. ~ ~ ~ ~ HI SACRAL
PLEXUS
FI.2 .oiilrgthmpevs etclve hwn
*~~ ~ ~~~onn pev:peis

.s.ah..

fibers and the pelvic nerves carrying para-
sympathetic fibers mainly from the third
sacral nerve with additional fibers from the
fourth, entered the pelvic plexus which
formed a meshwork of nerve fibers and
ganglion cells extending from the postero-
lateral surface of the rectum to the infero-
lateral angle of the bladder and sent fibers
into both these structures. The areolar tis-
sue surrounding the seminal vesicles, the
bladder base and the posterior surface of
the prostate was noted to be particularly
well endowed with nerve fibers and gan-
glion cells (Fig. 3).
Of the abnormal pelves, one had a recto-
urethral fistula, one a recto-vesical fistula,
one no fistula and one had complete colonic
agenesis with a caeco-vesical fistula. In
 480 SCOTT AND
FIG. 3. Photomicrograph showing nerve fibers and ganglion cells in the
neighborhood of seminal vesicle.
these cases the pelvic plexus was closely
applied to the under surface of the bladder
and was deficient posteriorly, though many
fibers were seen travelling upwards to sup-
ply the region of the fistula and the termi-
nal bowel (Fig. 4-6). The pelvic nerves of
the two sides were closer together than
normal, there being no rectum to separate
them, and in on case with severe sacral
agenesis, the only contribution wvas from
the third sacral nerve (Fig. 7). The hypo-
gastric nerves described the same course
as in the normal and joined the upper mar-
gin of the pelvic plexus. Numerous ganglion
cells were again seen in the pelvic plexus
and around the seminal vesicles, the blad-
der base and the posterior surface of the
prostate.
According to Wood Jones 21 the extra-
peritoneal part of the definitive rectum is
formed from the hind gut, which is repre-
sented in the high imperforate anus by the
SWVENSON Annals of Surgery
September 1959
71t.. -w^ ;.. -A
terminal centimeter or two of bowel. The-
oretically this bowel is capable of produc-
ing some rectal sensation and so it is im-
portant not to remove it or damage its
nerve supply. The anatomic studies show
that wide surgical mobilization would de-
stroy these nerves, and therefore, during
the operative correction of the deformity,
mobilization of the rectum should be re-
duced to the minimum compatible with
obtaining sufficient length to bring down to
the perineum. This is easier when the
bowel is stretched and distended as it is
immediately after birth. The nerve supply
to the bladder must be avoided during the
pelvic dissection by creating the tunnel for
the bowel as far posteriorly as possible on
the sacrum and during the perineal part of
the operation care should be taken to sepa-
rate the levator ani from the posterior as-
pect of the prostate and urethra so that
the rectum may be drawn through anterior
 Volume 150 INIPERFORATE ANUS
Number 3 481
SYMPATHETIC
CHAIN
ECTUM I

/ \ ~~~~~PELVIC PARASYMPATHETIC
LEVATOR ANI RECTO URETHRAL
FISTULA
FIG. 4. Mledial view of hemipelvis of a case of imperforate anus with recto-urethral fistula.

to the pubo-rectalis muscle. This reproduces or rarely in males, in the penile urethra
the normal ano-rectal angle and the sphinc- associated with hypospadias. This nomen-
teric action of the levator ani.
The cases in this series are therefore di-
vided into two groups, termed high level TABLE 1. A utizor's Classification of Types of Imperforate
A nis Compared wit/i That of Ladd and Gross
when the bowel ends on or above the
pelvic floor and low level when it passes Author Ladd and Gross
through the pelvic floor (Table 1). The Low Level
low level cases are classified as stenosed Stenosed anus Type I
anus, covered anus and ectopic anus. The Covered anus Perineal fistula Type III
covered anus is a diagnosis based on the Ectopic anus
Perineal Perineal fistula Type III
studies of Wood Jones.22 Excessive fusion Vulval Vaginal fistula Type III
of the inner genital folds covers a mal- Male urethral Type III
formed anus with a layer of skin and forms Congenital cloaca Type III
Rectal atresia Type IV
a midline track which runs forwards to a
small opening on the under surface of the High Level
scrotum or penis. The ectopic anus is one Recto-urethral fistula
which is malformed and situated anterior Recto-vesical fistula
Imperforate anus with g No fistula SType III
to the normal position, usually in the four- High recto-vaginal
chette or just outside the vulva in females, ( fistula J
 482 SCOTT AND SWENSON Annals of Surgery
September 1959

LEVATOR ANI
I

URETER

RECTUMI

0%-*.,Nk OBTURATOR
| NERVE
PLI_YIc.
PLIEXUS

......I.."...............

PELVIC PARASYMPATHETIC SYMPATHETIC SACRAL

NERVES OKHAINY PT
PLEXUS
FIc. 5. Pelvis viewed from
iabove showing parasympathetic nerves joining pelvic plexus.

clature is not strictly accurate as there is
no anal canal with an epithelial lining even
in the low level cases but it denotes the
embryologic background of the deformities
more accurately than the term imperforate
anus with fistula.
FIG. 6. Photomicrograph showing
nerve
in the outer coat of the terminal bowel in
of imperforate anus.
The two groups may be differentiated
easily in most instances as the majority of
low level cases possess an external bowel
opening whereas the high level do not.
The discovery of squamous cells in the
urine indicates a urinary fistula and there-
fore a high level deformity. Help may also
be obtained from a study of the routine
preoperative x-rays. In an accurate lateral
film a line joining the lower surface of the
pubis and the sacrococcygeal junction indi-
cates the position of the pelvic floor. If the
gas shadow in the bowel terminates on or
above this line the deformity is high level,
if it passes beyond the line it is low level
(Fig. 8).
Thirty cases in the series were regarded
as high level and 33 as low level. Half the
high level cases were treated by an abdom-
ino-perineal anoplasty and all but three of
fibers the remainder by a perineal operation. The
a case
incidence of postoperative complications
 'olume 150 INIPERFORATE ANUS
Ntmber 3 483
.. ......
............

BLADDER

1 . .

0 -

go-
I,

DI VIDE

m_ i RECTAL
URETHRA
MESENTERY
- a....

DIVIDED
KIERSE
k
KIDNEY

PARASYMPATHETIC RECTO VESICAL FISTULA

NERVE' = AGENESIS
...E ..

FIG. 7. MIedial view of hemipelvis of a case of imperforate anus with recto-vesical fistula.
Note the presence of sacral agenesis.

requiring additional surgery was 56 per
cent higher in the latter group indicating
that the abdomino-perineal operation is the
procedure of choice in the high level cases.
The low level cases were treated by en-
largement of the posterior anal margin or
complete posterior anal transplant.
In assessing the results the efficiency of
rectal control was graded in the following
manner:
(a) Normal spontaneous bowel move-
ments without soiling.
(b) Normal spontaneous bowel move-
ments with occasional soiling.
(c) Obstinate constipation relieved by
regular bowel irrigations without soiling.
(d) As in (c) but with soiling despite
the irrigations.
(e) Complete incontinence.
Groups (a) and (b) were regarded as
good, (c) as moderate and (d) and (e)
as bad results.
Only those
cases over the age of three
the time of follow up were in-
years at
cluded in the analysis. In the high level
group there were seven deaths, one case
untraced and three under the age of three
years. Of the remaining 19 cases, four were
regarded as good, two as moderate and 13
as bad results. This represents 68 per cent
bad results. In the low level group there
was one death, four cases untraced and
nine below the age of three years. Of the
remaining 19 cases, 17 were regarded as
good, one as moderate and one as bad re-
sults. This represents 89 per cent good
results. Although the numbers are small the
difference between the two groups is sig-
 484 SCOTT AND SWENSON Annals of Surgery
September 1959

FIG. 8. X-ray demonstrating the relationship of the gas shadow in the terminal bowel in a
case of high level and a case of low level imperforate anus.

nificant and reveals the true nature of the

problem. The results of treatment of the
high level group leave much to be desired.
Another feature which affects the prog-
nosis is the presence of sacral agenesis
(Fig. 9), the incidence of which is higher
in the high level group. With severe agene-
sis, bladder innervation is abnormal result-
ing in derangements of bladder function,2
sacra agenesis and the pelvic floor is poorly formed,
increasing the difficulty of creating a
sphincter.
The most frequent problem in high level
cases is constipation which if untreated
proceeds to massive fecal retention with
overflow incontinence. The causes of the
constipation are difficult to enumerate. In
the absence of rectal sensation the child
does not appreciate the need to evacuate
the bowel and therefore, other activities
being more interesting, it does not do so
at all. This cannot be the only cause of
w the problem as a colostomy on the abdom-
inal wall will usually evacuate spontane-
FIG.
acrayshwigaseveres csofof
9.FIG.9. -rashoinga sverecas
ously and without assistance.
 Volume 150 IMPERFORATE ANUS
Number 3
Colonic and rectal peristalsis studies
were carried out to determine whether
there was any difference between the post-
operative high level imperforate anus and
the normal. Twelve normal and nine ab-
normal children were investigated, changes
in bowel pressure being recorded at differ-
ent levels by means of a four lumen poly-
vinyl chloride tube 0.6 cm. in diameter.
Water was run through the lumina in order
to fill them, and pressure changes were
transmitted through a small side hole in
each lumen to Statham strain gauges ampli-
fied by a Grass recorder. Qualitatively there
were no differences between the two groups
of cases, though it was noted that those
children who had become severely consti-
pated postoperatively produced no peri-
staltic activity.
The postoperative management of the
imperforate anus is as important as the
operation itself. If the child is seen regu-
larly through the years many of the mis-
eries usually associated with these cases
may be avoided. Parents must be instructed
in the prevention of fecal impaction and be
encouraged to institute a program of bowel
training. Daily rectal irrigations may be
necessary to prevent fecal impaction if
spontaneous evacuations do not empty the
bowel efficiently. Improvement in the con-
dition of many children in this series was
obtained by emptying the colon with irri-
gations and establishing a regular bowel
evacuation program. Soiling was frequently
abolished and the development of regular
unassisted evacuations encouraged. Above
all the morale of both parent and child was
improved.
Greater attention to anatomic detail dur-
ing the operative treatment of the high
level imperforate anus, in particular the
correct placement of the rectum in relation
to the levator ani and the preservation of
its nerve supply, may improve postopera-
tive rectal control.
485
Summary
Reasons are given for classifying cases
of imperforate anus as high and low level.
In a series of 63 cases there were 68 per
cent bad results in the high level group and
11 per cent bad results in the low level
group.
Anatomic studies suggest that results in
the high level group may be improved by
preserving the nerve supply to the terminal
bowel and its correct placement in relation
to the levator ani muscle during abdomino-
perineal anoplasty.
A plea is made for a careful postopera-
tive follow up to prevent the distressing
developments that occur if this is neglected.
Acknowledgment
John E. S. Scott expresses his gratitude to the
Harkness Fellowships of the Commonwealth Fund
for a generous grant for travel and study in the
United States.
References
1. Bill, A. H. and R. J. Johnson: Congenital
Median Band of the Anus. Surg., Gynec. &
Obst., 97:307, 1953.
2. Bill, A. H. and R. J. Johnson: Failure of
Migration of the Rectal Opening as the
Cause for Most Cases of Imperforate Anus.
Surg., Gynec. & Obst., 106:643, 1958.
3. Bill, A. H., R. J. Johnson and R. A. Foster:
Anteriorly Placed Rectal Opening in t-he
Perineum. "Ectopic Anus." Ann. Surg., 147:
173, 1958.
4. Bill, A. H., Jr.: Pathology and Treatment of
"Imperforate Anus." J. A. M. A., 166:1429,
1958.
5. Bodenhammer, W.: Congenital Malformations
of the Rectum and Anus. New York, Samuel
S. and William Wood, 1860.
6. Brenner, E. C.: Congenital Defects of the
Anus and Rectum. Surg., Gynec. & Obst.,
20:579, 1915.
7. Caria Mendes, J.: Quoted by Walls, E. W.
Ano-Rectal Anatomy. Proc. Roy. Soc. Med.,
51:425, 1958.
8. Cripps, H.: Diseases of the Rectum and Anus.
New York, MacMillan Co., 1914, p. 26.
9. Denis Browne: Some Congenital Deformities
of the Rectum, Anus, Vagina and Urethra.
Ann. Roy. Coll. Surgeons England, 8:173,
1951.
 486 SCOTT AND SWENSON
10. Denis Browne: Congenital Deforml-ities of the
Anus and Rectum. Arch. Dis. Childhood,
30:42, 1955.
11. Fitchet, S. M.: Imperforate Anus. Boston Mied.
& Surg. J., 195:25, 1926.
12. Keith, A.: MIalformations of the Hind End of
the Body. Brit. Med. J., 2:1736, 1908.
13. Ladd, W. E. and R. E. Gross: Congenital
Mlalformations of Anus and Rectum. Am. J.
Surg., 23:167, 1934.
14. MIatas, R.: The Surgical Treatment of Con-
genital Ano-Rectal Imperforation Considered
in the Light of MIodern Operative Proce-
dures. Tr. Am. Surg. A., 15:453, 1897.
15. Mlitchell, G. A. G.: Surgical Progress. London,
Butterworth, 1951, p. 39.
16. Stephens, F. D.: Congenital Imperforate Rec-
tum, Recto-Urethral and Recto-Vaginal Fis-
tulae. Australian and New Zealand J. Surg.,
22:161, 1953.
DISCUSSION
DR. ROBERT E. GROSS: To some physicians,
abnormalities of the anus and rectum might ap-
pear to be rare and of little interest, yet there is
little doubt that these malformations are fairly
common. The surgeon treating such a condition
must aim not only to save the life of the child by
relieving an obstruction, but in addition should
adopt operative technics which will best insure
that continence and proper rectal function will
follow during the many decades in which the pa-
tient will have to use this part of his anatomy.
In a series of over 700 operations for treat-
ment of imperforate anus, the great majority of
repairs we have made have been by the perineal
approach alone. In 125 it has been managed by
the combined abdominal-perineal procedure. With
the low-lying abnormalities, requiring only a peri-
neal operation, our results of bringing about satis-
factory anal function are almost identical with
those reported by Drs. Scott and Swenson. About
90% of subjects can be given excellent repair and
complete anal continence.
For babies who have a high rectum, requiring
abdominal-perineal correction, the long-term out-
look concerning anal-rectal function is not nearly
so bright. We have found that about 60% of these
develop good anal control; about 20%G have only
fair control, and about 20% have permanent anal
laxity.
Despite the disappointments which we know
will occur in some of these cases, it is advisable
never to resort to an end colostomy on the abdom-
inal wall. It is always best to perform the ab-
dominal-perineal repair. Bringing down the rectum
from a high position will in some instances be
followed by laxity for several years, but then
Annals of Surgery
Septeniber 1959
17. Steplhens, F. 1).: Malformations of the Anus.
Australian and New Zealand J. Suirg., 23:
9, 1953.
18. Stephens, F. D.: Imperforate Rectum. A New
Surgical Technique. M. J. Australia, 1:202,
1953.
19. Stone, H. B.: Imperforate AnLus with Recto-
Vaginal Cloaca. Ann. Surg., 104:651, 1936.
20. Williams, D. I. and M. H. Nixon: Agenesis
of the Sacrum. Surg., Gynec. & Obst., 105:
84, 1957.
21. Wood Jones, F.: The Nature of the Malfor-
mation of the Rectum and Urogenital Pas-
sages. Brit. MIed. J., 2:1630, 1904.
22. Wood Jones, F.: The Delimitation of the
Rectuim and Its Subdivisions. Proc. Roy.
Soc. Med. (Surg. Sect.), 4:85, 1911.
23. Wood Jones, F.: The Explanation of a Recto-
Urethral Anomaly and Some Points in Nor-
mal Anatomy. Lancet, 2:860, 1915.
subsequently there can be considerable improve-
ment in muscular tone. Even in the desperate
cases, where little or no muscle power ever ap-
pears, the child can be managed reasonably well
after the age of 4 or 5 years by giving a consti-
pating diet, accompanying this by daily cleansing
and lavage of the bowel. In this way the indi-
vidual can be kept reasonably clean.
It is important to point out that all poor re-
sults which follow the abdominal-perineal opera-
tion should not invariably be attributed to poor
surgery. There might be a pre-existing deficiency
in some of these children which no surgery can
overcome. Given a newly born baby with a high
rectum, who requiires a combined approach
through the abdomen and perineum, it is essential
preoperatively to examine carefully the perineum,
for much can be learned thereby. If there is no
upholding of tissues between the lower parts of
the buttocks, if the perineum is lax and bulging
downward, and if the perineal structures do not
retract when the skin is pinched or pricked with
a needle, one can be quite sure that there is a
marked weakness of the neuro-muscular mech-
anism of the levator ani sling. Operation on such
a baby is likely to be followed by a poor func-
tional result. Conversely, if the child before oper-
ation has a good midline crease, and the perineum
retracts upward when it is stimulated, it is evi-
dent that there is to start with a good levator ani
system, and that good anal continence should fol-
low if the surgical correction is properly per-
formed. In the technical steps of such a repair
the operator can be helped greatly by the studies
such as those that Dr. Swenson and Dr. Scott
have made, and by the suggestions which they
have formulated.