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-
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.
irschspru
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
on
and
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
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-
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
of
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.
iiii/)ressiofl.
I econ
Hirsciusprungs L.T. to this
iu
Ill plug
syndroiuu
e.
tion
O
ing.
diagnosis.
iii.
disease. old
wilite
tile
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
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,
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
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
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
Witil
Initial colitis
partial
consideration,
or
total ileo-
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
was
tober
l)orn
i6,
8 oz.
Negro
feiuuale
fluoroscope demonstrated
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
C). Tiue
fully. position
The
the
first
few
days.
was
noted
in
an
was
abnoriuual
reported
iuualrotation
day
feedings
(Fig.
was and
witil
4D).
most
rectosigmoid, malrotation
likely
still
retuuained was
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
postulated
as
the
the
infant
childs
for Soiuue
diftiue bile
investigation
On
eigilth
day
deiuuon-
expired
was Initial
prior
to
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
and
de-
attend-
bowel
\OL.
112,
No.
Hirschsprungs
109
CASE
v.
E.H.
of
Fhis
month
old
male with
entered
a diagnosis
Childrens
possible
Memorial
nuegacolon
white feHospital
made at
4 cm. was
from made
definitive
procedure
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Barium
findings
enema
consistent
examinawitil
disease
under
(Fig.
anesthesia,
c, A examination
and
the
B).
patient
At
tile
significant
demonstrated
nature.
some
Since
the
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
of the
110
William
L.
Schey
and
Harvey
White
MAY,
197!
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11G. of
the
found
spot
roentgenograms
suggestive
all the way
narrowing
ganglia were
assumed
such
lilis
its
is
a adequ
tiler-
inadequately
6A).
bariulul seg-
mometer,
speculation
neurogenic
Initial Final
oiuly
ate
eneiuua
exaiuuination
was
repeated.
a
A siuort
iu ad
did
iliVe
bowel
caliber
and
siuualler
tower,
proxinual
rectuilu
disease.
Inflatuuiuuatory
trauiuuatic. This 9 lulolltil and
duratiolu.
stricture
old Negro
(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
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.
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
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
evacuation
eneiuua
exanuina-
eiuuptying. of
attempts
Siue
to years
had for
noted
2 to
demoiustrated
filled
with
feces
evacuation aid
an
segiuueiut. complement
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
resulted colon.
eiueiuua diagnosis
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
impacted,
showed
anorectal
no ganglion
junction,
cells
a few
within
ganglia ganglia
cnu.
of 2
tile cm.
at at
bowel
occur. matter
myentenic
plexuses
the into infant impacted the more
3 cnu.
froiuu
is because forced
abnormal
impression.
diagnosis. Psychogenic
bowel,
neonate children may be
widening
and
the
caliber.
and
iiu egacolon.
agatuglionosis.
In older nosis
Silort
segnlent
DISCUSSION
The in the
of
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
112
\\itlianu
L.
Schev
and
Harvey
White
MAY,
1971
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inserted
the Premature a
patient
is
adequately
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
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
It is fluid
and the peritonitis caused spasticity rectosigmoid noted at fluoroscopy, creating the findings interpreted
rectly as Hirschsprungs disease.
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
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
plosive-type bowel evacuations of watery stool as is frequently seen in Hirschsprungs disease and/or colitis and did not become
distended. after
i
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
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
disease.
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
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
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
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
assumes
focal with
ment of aganglionosis, the findings of Case vii (short segment aganglionosis) are clearly
understood. Here the rectum is full, no
aganglionosis disease
Hirschsprungs
is demonstrated
in Case
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
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
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
and
barium
mandatory of Hirsch-
VOL.
112,
No.
Hirschsprungs
Disease
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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
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
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,
for
diagnosis
rectum of Hirschbiopsy. 7.
dis227-
3.
A. H., and
70-74.
JR.,
and treatment.
CHAPMAN,
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
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,
and
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,
in
8.
36,
LYNN,
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,
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