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Voi..

112,

No.

HIRSCHSPRUNGS
IROBLEMS
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By

DISEASE*
INTERPRETATION
HARVEY WHITE,
M.D4

IN

THE

ROENTGEN
M.D.,f
CHICAGO,

WILLIAM

L. SCHEY,

and
ILLINOIS

classic roentgenographic features of Hirschsprungs disease have been well documented.5 They include narrowing and spasm of the aganglionic distal segment of bowel in continuity with more dilated proximal, normally innervated bowel. Classic findings are not always present, especially in the neonate.83 Some roentgeno-

HE

plug passed wilicil did support ilis contention. Consultation with other radiologists alerted him to a possible localized area of anorectal spasm and hence the possibility of Hirschsprungs disease. A repeat study io days later demonstrated relatively persisten t anorectai spasm (Fig. I, C and D). After the second
examination a correct diagnosis of Hirsch-

graphic features exist, however,


lying

in the difficult cases may which either reflect underHirschsprungs disease, or mask or are to reported, possibly
details findings

sprungs biopsy.

disease

was

made

and

confirmed

by

Initial
Final

impression.
diagnosis.

Meconium

plug
ngs disease.

syndrome.

mimic it. Seven cases were considered


sprungs tories, disease.

irschspru

all of which have Hirschof the and hiscon-

The

roentgenographic

firmed diagnoses are discussed. It is the purpose of this paper to present case material which demonstrates some problems leading to difficulty in making or excluding the diagnosis of Hirschsprungs (disease.
REPORT CASE I. R.C.
27,

CASE II. B.L. This white male infant was born at Michael Reese Hospital on November 14, 1968. He passed no meconium in the first 24 hours of life and was becoming distended. A barium enema examination denuonstrated no
spasm, no differential bowel caliber, and

OF

CASES

This delivery.

white After

November
pregnancy

1968, following

male was born an uncomplicated the first

on

and

only scanty passed and


The abdominal

quantities of meconium his abdomen became


plain roentgenograms

36 hours had been distended. suggested

a low bowel
genograms

obstruction.
demonstrated

Barium
no

enema

roentrectal

the way to the anorectal junction (Fig. 2, A and B). After instillation of barium and roentgenography, evacuation was allowed to proceed. There was no explosive-type evacuation and one or two large meconium boluses were passed. The distention subsided and the infant was returned to the nursery with the diagnosis of meconium plug syndrome. He experienced no reported difficulty under expert care while in the nursery. He was discharged 5 days later. The child was readmitted on December II, 1968. He had done well at home for 2 weeks having 4 to 5 pasty yellow stools per day, spontaneously. At
2

meconium

in

the

rectum

all

significant

weeks diluted

of

age prune
at
i

he

did juice.

have

40

hour

period

spasm but much meconium packed into the rectum (Fig. i, A and B). The radiologist had had little experience with this problem and obtained spot roentgenograms only in the anteroposterior position. His diagnosis was meconium plug syndrome. Some meconium
with
*

without
oral
admission

bowel

evacuation.
month bowel

This

was relieved

by

hours

without

Three days prior to his of age he had gone 72 evacuation. A barium

enema
findings and D).
and Medical

examination
of Hirschsprungs

now

showed
disease

the
(Fig.

classic
2,

a yellow-green
From the Departments

phrygian
of Radiology,

cap
Michael

or leading
Reese Hospital

Center,

and

Childrens

Memorial

Hospital,

Chicago,

Illinois.

t Director,

Pediatric

Radiology,

Michael

Reese Hospital
Memorial

and Medical
Hospital,

Center,
Chicago,

Chicago,
Illinois;

Illinois;
Professor,

Instructor,
Department

Department

of Radiology, North-

University of Chicago. Director, Department western University Medical

of Radiology, School.

Childrens

of Radiology,

105

i o6

\\ill

ia m

Schev

a nil

Ha

rvcv

Wii ite

MAY,

1971

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11G.
later,

1.

(A

and

B)
the of

Initial
diagnosis

examination of meconium narrowing

of

suggesting because a repeat tonus, sprungs

suspicious
was

study
was

made

which

Case ,. There is meconium in the rectum and much of the Colon plug syndrome (anteroposterior view only). (C and D) Ten days of the rectum on the first examination and persistence of the synupshowed spasm and narrowing of the rectunu. A diagnosis of Hirsch-

disease

entertained.

Initial Final CsE was

iiii/)ressiofl.

I econ
Hirsciusprungs L.T. to this

iu

Ill plug

syndroiuu

e.

tion
O
ing.

diagnosis.
iii.

disease. old
wilite

tile

and bloody stools. Blood second day of life.Tiuere


Roentgenograms

was

first

noted
vomit-

was

no

on

admission

demon-

3 day

fenuale Hos-

strated without

generalized definite or

distended
obstruction.

transferred
in

Ciuildrens

Menuorial

pital

Cilicago

because

of abdoiuuinal

disteiu-

mucosally

deeper

was

loops of bowel Air dissecting subalso iuoted in tile

VOL.

112,

No.

Hirschsprungs
field
denuonstrated

Disease
operated found 3
the colon
on. cm.

107

colon

projection

(Fig.

3A). marked

Barium
spasticity

enenua
of
of the

exanuination

froiuu

tiue entire

colon,
and again

an irregular
subnuucosal

appearance

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mucosa

(Fig. ease
associated

3B).

was

Tiue raised,

was revealed question of Hirschsprungs distotal or partial iiu degree, with


air
colitis to explain tiue

viability ill for


necrotic

was was anytiuing


ileum

portion of ileum was ileocecal valve. Iiu addition small in caliber and friable. Its questioned. Tlue child was too more than a resection of the
necrotic

tile

and

a double-barreled
colostomy of was

ileotomy.
required be-

necrotizing

Subsequently
cause of obstruction

extraluminal
was colitis ease. primary without On
tile

air.
sepsis
second

The

second
a secondary

consideration
necrotizing

with

underlying
hospital

Hirschsprungs day tiue ciuiid

dis-

was

tlue sigmoid. At 2 later dates aiu ileoproctostomy and colon resection were performed. Tlue patients over-all course was stormy but slue survived and is now doing

FIG.

demonstrating meconium throughout the entire rectum, rectosigmoid and No spasm or aganglionic segment was defined. The diagnosis was meconium plug syndrome. (C and D) One month later, with only 72 hours of problem of obstipation, a repeat examination demonstrated classic findings of anorectal narrowing.
2.

(A and

B) Roentgenograms

much

of the descending

colon.

io8

\Villiam

L.

Sciley

and

Harvey

Wiuite

MAY,

1971

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11G.
tile

(il)

Roentgenogram
of

demonstrating

distended roentgenogram

ioops

of bowel

throughout
one area

the
of rectal

abdomen
spasm

and
noted

air

within
Huoros-

wall

the

copy,

although

large bowel. (B) the entire colon 3,

The
was

demonstrates

at

frequently bowel

in severe

spasm

during

the
suspected

examination.
aiu inconuplete progression

well,
tuiuction.

at

age

years,

witiu

excellent

i tug

su rgeoiu

bowel
of tiue

obstruction impression. secondary 1)ssiI)le


secondary

Witil

Initial colitis

partial
consideration,

or

total ileo-

iiufaiuts coiuditioiu to be ruled out.


revealed
as

unfavorable ; Hirschsprungs
A entered
bariuiuu

disease
exaiuuination

had

eneiua

aganglionosis;

iiuumediate bariullu

spasm tiue
rectosigiuuoid

of

tiue

rectosigiuuoid sigiuuoid. a to
Tiue areas

to sepsis.
Necrotizing

tiue

descending
tinue

Final
CAsE

diagnosis.
to IV.

colitis

and

ileitis

The
few
recognize spot

spasnu
seconds
it

of

the

secondary

sepsis.

but
O

enougiu
tile

lasted only was allowed


iuuonitor. the

was
tober

l)orn
i6,

B.R. Tiuis 8 lb., NIichael Reese 1969. Tiue patient


at

8 oz.

Negro

feiuuale

fluoroscope demonstrated

Hospital did well

on Octthe first
noriuual by the On the

roentgenograms

in question

already reiuuainder

re-expanded of
tile colon

(Fig. filled

4, B and unevent-

c days. quantities
usual tftil with

Bowel nuoveiuuents consisted of of meconium passage followed stools after


enuesis was of

C). Tiue
fully. position

The

the

first

few

days.

cecum and this

was

noted

in

an
was

abnoriuual
reported

iuualrotation

day
feedings

yellowish material iuuixed noted. Bowel evacuation


and Similar noted mild no abdoiuuinal disepisodes of inon tile following abdoiuuinal dis-

(Fig.
was and
witil

Tile aganglionosis, malrotation.

4D).

diagiuosis considered at least of the At surgery a

most
rectosigmoid, malrotation

likely

still

retuuained was

nornual observed. eiuuesis were

telution ternuittent few


tention stools

volvulus
was was
colon

was
in observed

found.
tiue

Soiuue
peritoneal 10 to 15

inflaiuumatory
cavity. minutes abnormality

fluid bowel

noted

The
after

days

associated

with
soiuuewhat septic

for

and
became
of a

diminished

stool
condition

evacuation.
Consideraor gastritis

The

watery. during
of
tile

untwisting of tile
was
reoperate

the
was

iuualrotation.
demonstrated.

No
intention

The
second

abdonuen

lion
secondary

possible to

closed.
tile

The
following
of

surgeons

was

to

1)100(1

ingested
tile cause

delivery

was ticulty. strated

postulated

as
the

the
infant

childs
for Soiuue

diftiue bile

investigation

On

eigilth

day

deiuuon-

expired
was Initial

prior

to

day for a bowel viability. tilat procedure.

look Tiue infant No autopsy

nuoderate

abdominal

distention

performed.
impression.

first
of

time.
the was in al

Supine
noted roen

aiud
were

upright
unrenuarkabie.
following

roentgenogranus
Hirschsprungs 1\Ialrotat ion
with

abdomen

disease
volvulus

with

emesis

abdom

on tile tgenograph

day. revealed

Repeat some of a

nu alrotation. Final diagnosis. vascular vitalization

and
de-

bowel distention without mecilanical obstruction

definite evidence (Fig. 4z1). The

attend-

compronuise; secondary and peritonitis.

bowel

\OL.

112,

No.

Hirschsprungs

Disease rectum disclosed tiue anal orifice.


prior to the

109

CASE

v.

E.H.
of

Fhis

month

old

male with

entered
a diagnosis

Childrens
possible

Memorial
nuegacolon

white feHospital
made at

a rectal stricture This correct diagnosis


pull-through

4 cm. was

from made

definitive

procedure

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the referriiug hospital. tion demonstrated


Hirschsprungs
surgery, while

Barium
findings

enema
consistent

examinawitil

of Swenson. After tigation suggested


culty in the newborn

the procedure that the child


period

disease
under

(Fig.
anesthesia,

c, A examination

and
the

B).
patient

At
tile

significant
demonstrated

nature.
some

Since

the

further inveshad some diffibut not of any resected bowel


changes it is

was

re-examined.

Digital

of

inflammatory

FIG.

. (A) Plain roentgenogram of the abdomen demonstrating the abdomen. (B and C) Barium study demonstrating already

moderate
re-expanded

dilatation

rectosigmoid

severe cecum.

spasm

for 4 to

seconds.

(D)

Evacuation

roentgenogram

demonstrating

bowel throughout which had been in abnormal position of the

of the

110

William

L.

Schey

and

Harvey

White

MAY,

197!

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11G. of

the

found

(A) Overhead lower rectum in abundant that some

and (B) strongly numbers trauma,

spot

roentgenograms

suggestive
all the way

of the rectosigmoids of a zone of aganglionosis. to tile anorectal junction

demonstrating persistent This was a stricture and n the resected specimen.


biopsied (Fig.

narrowing
ganglia were

assumed

such
lilis

its
is

a adequ

tiler-

inadequately

6A).

bariulul seg-

mometer,
speculation

neurogenic
Initial Final

created tile stricture. . Fiue resected bowel components.


Hirschspru lugs

oiuly
ate

eneiuua

exaiuuination

was

repeated.
a

A siuort

iu ad

fluent of rectunu tiuaiu the iuore


colon biopsy

did

iliVe

bowel

caliber
and

siuualler
tower,

proxinual

rectuilu

descending normal. doing


soft for

impression. diagnosis. possibly


VI.

disease.

Inflatuuiuuatory
trauiuuatic. This 9 lulolltil and
duratiolu.

stricture
old Negro

of the male with


dis-

(Fig. 6, B and C). A second, was obtained and it too was iiue child was disciuarged and is presently well. but Most bowel movenuents mild requires are iue is oiu iilteriuuitteiut

rectuiuu,
CASE

Spontaneous,

A.W.
of

cathartics, an enema

was
a

brought

to the
constipation

Michael

Reese

Clinics

history

abdoiuuiiual

foods and occasionally adequate bowel evacuation. Initial Final


etiology. CASE VII.

tention was

of 2 nuonths

7 lb., ii oz. He had the first 6 nuonths of life. lue had diarrhea for i
green stools per day.

His birth weight no bowel probleiuus for At the age of 6 moiuths weeks, passing 3 to 4
He was given soiuue

impression. diagnosis.

Sluort disease. Coiustipatioiu,

aganglionic

segment,

Hirsciuspruiugs

of

unknown

loose

oral nledication and placed oiu a tea and skiiuuiuued nuilk reginue. He did well but tilereafter became constipated. He passed one hard, yellow, small stool daily for the subsequent 2 to 3 iuuonths. At tiue age of 9 iuuontius he was brought
to
tilat

B.C.

This

6 year

old

Negro had his been

iuuale ad-

entered of

Michael
2 years

Reese
and
earlier

Hospital
soiling.
for

for
He

evaluation
and problenu.
wilen

constipation

mitted exclude
File his

investigatioiu

to

an
history

organic dated
began to giving

etiology 4 to
hinu oral

of

tiue

clinic

for

evaluatioiu.

His
frolul bariuiuu

mother abdoiuuinal
tiue tulle

stated

5 months
of tile

of age
catluartics rectuiuu

the

child
and episode.

had

iluterluuittelut A

disof tile

mother

and
to

tention diarriueal
tiOIu

constipatioiu a colon

occasioiual

digital colonic frequency


prior to her

evacuation

eneiuua

exanuina-

encourage and diminishing


months
enuptying.

eiuuptying. of
attempts

Siue
to years

had for

noted
2 to

demoiustrated

filled

with

feces

evacuation aid
an

no transitioiual vealed a full

segiuueiut. complement

rectal biopsy of iueurogenic


but aganglionic

reeletiue

the

bowel
iuuegaBarium with a

At
ill a

the
diagnosis biopsy

age

of
of was was not

evaluation

meluts.
probleiuu

The It
was

patietut
He suggested

was
was iuad

discilarged readmitted that an

resulted colon.
eiueiuua diagnosis

psychogeiuic performed. consistent disease.

persisted.

2 iuuonths

No
examination

later. portion

of tiuc rectum

been

overlooked

and

of

Hirschsprungs

Psychiatric

VOL.

112,

No.

Hirschsprungs

Disease

III

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consultation suggested some exposure to significant traumatic episodes and supported the diagnosis of psychogenic constipation. On tlue final admission, 2 years later, a repeat barium enema examination (Fig. 7, A-C) and biopsies were obtained. A rectal myomectomy and anal sphincterotomy were perfornued. The biopsies

features
enema

can
study.

be defined
These

with
findings

routine
include

barium
a nar-

row
onic

segment
portion)

of distal
and normally the

colon
wider, innervated

(the
usually

aganglifecally proximal the from


may

impacted,

showed
anorectal

no ganglion
junction,

cells
a few

within
ganglia ganglia

cnu.

of 2

tile cm.

bowel. Some difficulty true site of normally

in demarcating innervated bowel

at at

bowel
occur. matter

without This may the be

myentenic

plexuses
the into infant impacted the more

proximally, the anorectal


Initial Final

and scattered junction.

3 cnu.

froiuu

is because forced

fecal distal in a few

abnormal
impression.
diagnosis. Psychogenic

bowel,
neonate children may be

widening
and

the

caliber.
and

iiu egacolon.
agatuglionosis.

In older nosis

Silort

segnlent

with constipation the diageasy to make; it may also be

DISCUSSION

The in the

diagnosis older child

of

Hirschsprungs generally presents

disease little

extremely Careful
details After of an

confusing. attention
the barium infant-size

must
enema plastic

be

given

to

tile
is

difficulty.

All

the

classic

roentgenographic

examination. rectal tip

112

\\itlianu

L.

Schev

and

Harvey

White

MAY,

1971

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inserted

the Premature a

patient

is

adequately

immobilmay A No. be used. re22

is taped

into

position

and

the

buttocks

are

ized.
quire

or

snlall

infants

then
months plastic

taped
easily eienut

together.
accommodate tip. A

Children
an balloon

over
adult-size catheter

6
or

French

snualler soft rubber

rectal device. catheter can

It

VOL.

112,

No.

Hirschsprungs

Disease

113

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Bardex catheter is never used as the roentgenographic findings may be obscured. The barium is then instilled while the patient is in the frontal position. Only a small amount of barium is delivered initially. The patient is then rolled onto the left side or into the left posterior oblique position and observed again.2 More barium may be needed but flooding rapidly is to be avoided. Spasm and/or narrowing of the aganglionic segment yields the classic picture in the neonate, especially when the more proximal portion of bowel is seen with its nonspastic, normal caliber juxtaposed. The spastic portion may show irregularity of the mucosa suggesting early submucosal inflammation. The authors believe that this pic_ ture ofmucosal irregularity may be present in the absence of associated inflammation. It is wise to initiate the patients care rapidly, however, as submucosal inflammation in an infant with Hirschsprungs disease may precede and cause sepsis and death.3 The aganglionic segment may be of any length but it may be assumed that wherever the aganglionosis begins, all the bowel from that point distal is devoid of normal neurogenic elements. Evidence to the contrary has been reported (skip areas) but these cases are extremely rare.4 Difficulties in diagnosis occur when the classic findings are not present. In Case iv the classic fluoroscopic image presented but only for an abbreviated period of time. Spasm of the rectosigmoid was definitely present. It was not demonstrated in the descending colon. Although the findings were only temporarily observed (about -6 seconds) it was believed that the appearance was that of Hirschsprungs disease. The remainder of the colon demonstrated a malrotation but intrinsically the colon was otherwise normal. An upper gastrointestinal examination at this time would have demonstrated a volvulus, and possibly devitalization of bowel might have been considered. The patient was operated upon immediately, however, and much inflammatory fluid was found in the penitoneal
cavity. An early peritonitis was also found

as well believed

as the findings that irritating

noted above. inflammatory

It is fluid

and the peritonitis caused spasticity rectosigmoid noted at fluoroscopy, creating the findings interpreted
rectly as Hirschsprungs disease.

of the thus incor-

Masking of the findings occurred in Case I. Initially the examiner obtained only frontal spot roentgenograms and interpreted the findings as a meconium plug syndrome. The patient did evacuate the
barium and much meconium, as first stated in

the case presentation. done within 10 days


clearly demonstrated

A repeat
of the
the

examination
examination

Hirschsprungs oblique and observing the the pitfall to Absence of be interpreted have a classic plug syndrome Clearing of
barium enema

classic findings of disease. Not obtaining lateral roentgenograms and patient in these positions is be guarded against. the findings in Case ii cannot as a pitfall. The patient did history for either meconium or Hirschsprungs disease. the symptoms following the
examination, absence of a

spastic
symptoms

segment
for

and
i month

elimination
supported

charge
drome.

diagnosis
The infant

of

meconium
also did not

of the the displug synhave ex-

plosive-type bowel evacuations of watery stool as is frequently seen in Hirschsprungs disease and/or colitis and did not become
distended. after
i

The month and

symptoms the correct

recurred diagnosis

only of

Hirschsprungs was made over the telephone by the history (albeit unusual), by physical examination (absence of stool in the nectum), and visually by barium enema study. This case suggests that Hirschsprungs disease need not cause daily difficulties in the
neonatal period and may occur in an infant

who plug
urn

initially syndrome.
plug

presented That
may

a classic meconium infants with meconilater have Hirsch-

syndrome

sprungs disease has been cited b\ other authors.7 Colitis with or without sepsis can cause spasm of the bowel. The neonate is seldom, if ever, the object of classic idiopathic ulcerative colitis. Generally the neonate with

114

William

L.

Schev

and error

Harvey
in

White

MAY,

197!

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colitis has an underlying sepsis and/or Hirschsprungs disease. These children are usually very toxic and appear acutely ill. Tetanic spasms of the colon can cause explosive bursts of watery stool. Colitis,
sepsis, and is Hirschsprungs disease should be

the

diagnosis

of

Hirschsprungs or questionable zone is puz_ megacolon) is a


and fre-

disease.

The tapered anorectum short segment aganglionic zling. Case vi (psychogenic


perfect example. These

infants

considered.
tion

The
confusing

barium

colon
great rectum

did

go into

enema interpretain Case in. The entire spasm. The spasm was so
flow proximal be achieved by to placing the a

quently
of bowel rower in

older
that caliber

children
tapers than they Many as

present
or the

with

a zone
narrectum Hirschand mega-

is definitely proximal

that retrograde could only

soft catheter into the distal sigmoid and delivening barium in this manner. Prior to this examination the supine and upright views of the abdomen demonstrated pneumatosis
cystoides intestinalis. A necrotizing colitis

and colon. Are sprungs disease? many are labeled

cases with are constipated psychogenic

colon. fancy, a real

In some the history dates to inwhich should favor the diagnosis of aganglionosis. That a history sugaganglionosis can with histologically be obtained normal the narnor-

was suspected. Was this Hirschsprungs disease with colitis, a total colonic aganglionosis, or sepsis with colitis and no aganglionosis ? As noted in the Case iii presentation, surgery was performed. When the colon was removed because of the necrosis, ganglion cells were present. The primary vasculature to the bowel was also intact excluding a possible arterial thrombosis. The final diagnosis was sepsis with secondary endarterial obliteration, secondary necrotizing colitis. Sepsis the sprungs
to

gesting true in patients

bowel

has
also

been
been

noted
with

in the

literature.80
that

It
mal

has

suggested

rowing trophy.

in patients

histologically

bowel

may
Biopsy
of

be due
is

to muscular
for

hypercomplete

necessary

evaluation therapeutic
myomectomy. If one

these patients when performed


an extremely

and may be with rectal


short seg-

assumes

focal with

ileitis and colitis should

necrotizing alert one Hirschwithout


iii.

ment of aganglionosis, the findings of Case vii (short segment aganglionosis) are clearly
understood. Here the rectum is full, no

possibility disease. and

of an underlying That it may exist


yet mimic

aganglionosis disease

Hirschsprungs

is demonstrated

in Case

tapered or spastic postevacuation strate excellent roentgenographic


glionosis. tive, The however.

segment is noted and the roentgenograms demonemptying. There is no evidence of typical aganwas strongly suggesabsence of revealed

congenital

narrowing
created disease by may

of
mechanical

the

rectum6
and/or Hirsch-

history A biopsy

or narrowing inflammatory

mimic

sprungs perience
ture dence rectum based of

disease. Indeed this was with Case v (inflammatory


the that in on rectum). stricture this case the The supportive or narrowing was inflammatory evidence.

our

exstricevi-

of

the is

from the distal rectum to within 2 cm. of the anal mucosa. This child then does have an aganglionic zone and will benefit from surgery. Rectal myomectomy may be the procedure of choice.9 It has been suggested that the same procedure
is helpful, and tient exemplified at times curative, in the by Case vi (psychogenic pa-

ganglion

cells

pathologic

historic
was well

data
and

were
robust

vague
when

but

that

the

initially

The child seen was

megacolon),

as noted
CONCLUSIONS

above.

obvious. This did not deter us from making the diagnosis of Hirschsprungs disease on the barium enema examination, as many of these patients are robust in appearance. These findings created another source for

Adequate administration for appropriate

roentgenography techniques investigation are

and

barium

mandatory of Hirsch-

VOL.

112,

No.

Hirschsprungs

Disease

I 15 investigation. led cases disease.


L. Schey, M.1). Reese Hospital

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sprungs disease. Anorectal narrowi ng a nd/or should strongly suggest, but is not nomonic of, aganglionosis. It may
in patients tis, tis, infectious and in with patients sepsis penitoneal with and fluid secondar\ and structure

roen

tgenographic in of

The

misand

spasm pathogbe seen


colipenitonifonma-

interpretation both

to true

incorrect aganglionosis

diagnoses

those
sprungs
William Michael

later

proved

not

to

have

Hirsch-

tion. The meconium plug syndrome may be the earliest manifestation of a patient with Hirschsprungs disease and may mask it entirely. Findings of Hirschsprungs disease may be difficult to recognize or be nonexistent in the first month of life, however, when the meconium plug syndrome is encountered. The tapered anorectum, as noted on the barium enema examination, may mimic Hirschsprungs disease, whereas the patient
with true short segment aganglionosis

and Medical Center Chicago, Illinois 6o6i6


REFERENCES I. ALDRIDGE,

R. T.,
cell

and

CAMPBELL,

P.
normal

E. Gan-

glion

distribution

in

2.

by anorectal 968, 4, 475-490. BERMAN, C. Z. Roentgenographic manifestations of congenital megacolon (Hirschsprungs ease) in early infancy. Pediatrics, 1956, z8, Pediat. Surg.,
i
237. BILL,

anal canal: basis sprungs disease

for

diagnosis

rectum of Hirschbiopsy. 7.

dis227-

3.

A. H., and
70-74.

JR.,

and treatment.

CHAPMAN,

N. D. Enteroits natural Surg., 1962,

may demonstrate barium enema psychogenic


Adequate

no study problem.
biopsy,

aganglionic and be
including

zone labeled
rectal

on a
4.

colitis

of Hirschsprungs

history
103,
CAFFEY,

disease: Am. 7.

myo-

mectomy, in patients with suspicious aganglionosis is necessary for complete evaluation. It may be therapeutic in both cases of short segment aganglionosis and psychogenic megacolon.
SUMMARY

J. Aganglionic X-Ray Diagnosis. Medical Publishers, 626-633.


B. D. Congenital
1962, 104,

megacolon. In: Pediatric Fourth edition. Year Book Inc., Chicago, 1961, pp.

COHN,

atresia

of colon.

Am.

7.

Surg., 6.
GILLI5,

86i-86.
GRANTMYRE,

plug
Canad. 7.
KOTTMEIR,

D. A., and syndrome


M.

and

E. B. Meconium Hirschsprungs disease.

Pitfalls

in the

roentgenologic

diagnosis

of

A. 7., 1965, 92, 225-227. P. K., and CLATWORTHY, H. W., JR. Aganglionic and functional megacolon in children: diagnostic dilemma. Pediatrics, 1965,
572-582.

Hirschsprungs disease occur, the neonate and infant. Seven cases are reported,

especially each demon-

in
8.

36,
LYNN,

H. B. Rectal megacolon. Mayo


289-295.

myomectomy Clin. Proc.

for agangiionic
Staff Meet.,
1966,

strating where errors in the diagnosis been made. In cases Hirschsprungs ease was suggested as the initial

have dis-

41,

9.

RAFFENSPERGER,

enterology.

Volume

diagnosis,
o.

ter
RAY,

64.

W.
1966,

E. C. Megacolon. In: GastroII. Second edition. ChapB. Saunders Company, Philapp.

but was not. In 2 cases the lesion was initially called meconium plug syndrome, but was aganglionosis. The seventh case was
roentgenographically considered to have

delphia,

II.

psychogenic megacolon, but was found to have a short segment type Hirschsprungs disease by biopsy. The unique details of each case are presented. An analysis of the findings shows some of the causes for misinterpretation of the

12.

13.

for idiopathic megacolon 7. Surg., 1968, 55, io6-io. RIKER, W. I)iagnosis and treatment of aganglionosis of myenteric plexus. A.M.A. Arch Surg., 1957, 75, 362-376. SHANDLING, B., and DESJARDINS, J. G. Anal myomectomy for constipation. 7. Pediat. Surg., 1969, 4, ii5i8. SHIM, W. K. T., and SWENSON, 0. Treatment of congenital megacolon in 50 infants. Pediatrics, 1966,38, 185-193.

A. I). Resection

647-662. of hindgut in adolescence. Brit.