J Radiol Sci 2012; 37: 11-19
Total Colonic Aganglionosis: Reappraisal of
Contrast Enema Study
Ting -Wen Sheng1 Chao -Jan Wang1,4 Wan-Chak Lo1,4 R ey-In Lien2,4 Jin-Yao Lai3,4 Pei-Yeh Chang3,4
Department of Medical Imaging and Intervention1, Division of Neonatology, Department of Pediatrics2, Division of Pediatric Surgery,
Department of Surgery3, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan
College of Medicine4, Chang Gung University, Taoyuan, Taiwan
ABSTRACT
Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease (HD). It is difficult to be diagnosed
on contrast enema because the radiographic findings are variable. The study aims to re-evaluate the contrast enema
findings of TCA. From 2001 to 2009, 14 patients (11 males, 3 females; ages from 1 day to 6 months) with pathologically proven TCA were reviewed for demographic features, clinical presentations, pathology reports, radiographic
and contrast enema findings. In addition, the radiographic and contrast enema findings of 53 patients with non-TCA
HD were reviewed and compared with those of TCA. Among the imaging findings, a short and rigid colon, small
bowel dilatation, microcolon, and radiographic transition zone proximal to the cecum were statistically more significant in patients with TCA than in patients with non-TCA HD (P < 0.001). In conclusion, TCA is a colon disease
presenting as small bowel obstruction clinically and radiographically. Although the radiographic findings of TCA are
variable, TCA could be suspected when the initial radiograph shows small bowel dilatation and contrast enema shows
a short and rigid colon, radiographic transition zone proximal to the cecum, poor rectal distensibility, colonic wall
irregularity and delayed contrast emptying. In addition, biopsy should be performed to make a definite diagnosis.
According to the caliber of the colon on contrast enema, a subgrouping approach may be useful in the differential
diagnosis and be helpful in early diagnosis and exclusion of TCA.
Hirschsprung disease (HD) is a developmental
disorder of the enteric nervous system which is characterized by the absence of ganglion cells in the myenteric and
submucosal plexuses of the distal intestine [1]. The arrest of
craniocaudal migration of the ganglion cells from the neural
crest is thought to be the etiology of HD [2]. Spasm, chronic
contraction and poor coordinated peristalsis of the aganglionic distal bowel lead to the functional intestinal obstruction which is the characteristic presentation of HD. Children with HD typically present in the neonatal period with
abdominal distention, feeding intolerance, bilious vomiting
and delayed passage of meconium. Some patients who do
not present with obvious clinical symptoms in the neonatal
period could present with chronic constipation when they
get older. Other patients could present with enterocolitis
which is characterized by fever, abdominal distention and
diarrhea [1]. In the majority of HD (67% to 79%), the transition zone between the ganglionic and aganglionic bowel is
in the rectosigmoid colon [3, 4]. Total colonic aganglionosis
(TCA) is defined as aganglionosis of the entire colon with or
without extension into the small intestine, which accounts
for 3% to 12% of HD [3-8].
Full-thickness biopsy of the colon in the operation is the gold standard for the diagnosis of HD. For the
Correspondence Author to: Chao-Jan Wang
Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital at Linkou, Taoyuan, Taiwan
No. 5, Fu-Xing Street, Gui-Shan, Taoyuan 333, Taiwan
J Radiol Sci March 2012 Vol.37 No.1
11
Imaging of total colonic aganglionosis
preoperative evaluation, the sensitivity and specificity of
contrast enema are less favorable than other diagnostic
tools such as anorectal manometry and rectal suction
biopsy [9]. However, contrast enema plays an important role
in the diagnosis of HD. First, comprehensive assessment of
the clinical presentations and the radiographic findings on
contrast enema may help in appropriate selection of patients
who need to receive rectal biopsy [10]. Second, besides
TCA, contrast enema may not only identify some diseases
which also cause distal bowel obstruction, but may potentially treat them (e.g., meconium plug syndrome, meconium
ileus) [1]. Third, contrast enema may help the preoperative
evaluation of the level of transition zone. The anticipated
level of transition zone can greatly influence the surgical
approach to HD [11-13]. The preoperative knowledge of the
extent of aganglionic bowel might potentially reduce the
operative time and prevent an incorrect determination of
transition zone in the operation [14].
Previous studies have shown that contrast enema easily
misleads surgeons in evaluating the level of transition zone
in patients with TCA [12, 13]. There is a wide spectrum
of radiographic features of TCA on contrast enema, which
can mimic other causes of distal bowel obstruction in the
neonate and young infants. The diagnosis of TCA is difficult to be established by contrast enema studies [14-18].
Radiographic diagnostic rate was suspected in only 20% to
30% of patients with TCA [19]. In this article, we reviewed
the cases of TCA in our institute and addressed our experiences with contrast enema in establishing the diagnosis of
TCA.
MATERIALS AND METHODS
Research design
This study was approved by our institutional review
board. We retrospectively searched the medical record database for patients who were diagnosed with HD by surgical
pathology at our institution from 2001 to 2009. Eighty
patients with HD were identified (63 males, 17 females;
age from 1 day to 10 years). Among these patients, 15 had
TCA. One patient with TCA was excluded because contrast
enema was not performed. The medical records of the 14
patients (11 males, 3 females; age from 1 day to 6 months)
with TCA were reviewed for demographic features, clinical
presentations, pathology reports, radiographic and contrast
enema findings. In addition, among the 65 patients with
non-TCA HD, 53 had available contrast enema images. The
radiographic and contrast enema findings of the 53 patients
with non-TCA HD were reviewed and compared with those
of TCA.
In our institution, we performed contrast enema with
water-soluble contrast medium in neonates and patients with
enterocolitis. Compared with barium enema, water-soluble
contrast enema is a potential treatment and examination
12
modality of mecoium plug syndrome and meconium ileus.
In addition, it will result in fewer complications if there is
a bowel perforation.
Assessment of imaging findings
The initial abdominal plain radiographs and contrast
enema images of the 14 patients with TCA and the 53
patients with non-TCA HD were reviewed in consensus by
two pediatric radiologists: one with 15 years of experience
(C.-J. W.) and one with 10 years of experience (W.-C. L.).
Seven imaging findings were assessed: small bowel dilatation, microcolon, a short and rigid colon, poor rectal distensibility, colonic wall irregularity, radiographic transition
zone, and delayed contrast emptying.
There were some terms to be defined in the following.
First, the caliber of the colon was further classified into
microcolon (Fig. 1a) and non‑microcolon (Fig. 1b). Microcolon was defined as a colon caliber which was generally
less than the interpedicular space of the L1 vertebra. The
normal caliber colon or dilated colon was classified into
the subgroup of non-microcolon. Second, a short and rigid
colon was defined as the loss of redundancy of the sigmoid
colon with rounding of the splenic and hepatic flexures.
Sometimes, it could appear as a question mark-shaped colon
(Fig. 1c). Third, poor rectal distensibility was defined as a
persistent poor distention of the rectum (Fig. 1d). Fourth,
colonic wall irregularity was defined as a serrated contour
of the colon (Fig. 1e). Fifth, the radiographic transition zone
was the location of obvious caliber change in the bowel
(Fig. 1f). Sixth, delayed contrast emptying was defined as
retained contrast medium proximal to the sigmoid colon on
a radiograph which was obtained from 24 to 48 hours after
the contrast enema.
Statistical analysis
The Fisher’s exact test was used to compare the
imaging findings of the two patient groups, those with TCA
and those with non-TCA HD. A P value of less than 0.05 was
considered to indicate a statistically significant difference.
RESULTS
Demographic features, pathology reports, and imaging
findings of the patients with TCA are shown in Table 1.
Comparison of imaging findings of TCA and non-TCA HD
are shown in Table 2.
Demographic, clinical, pathologic data of TCA
The patients were predominantly males (11 males, 3
females, male-to-female ratio = 4:1) and full-term (12/14).
The ages at the time of contrast enema ranged from 1 day
to 6 months, and were usually during the neonatal period
(10/14). The clinical presentations included vomiting (14/14),
abdominal distention (13/14), feeding intolerance (4/14),
J Radiol Sci March 2012 Vol.37 No.1
Imaging of total colonic aganglionosis
delayed passage of meconium (3/14), enterocolitis (2/14),
and bowel perforation (2/14). Almost all the patients had
aganglionic segments extending to the cecum or the distal
ileum, and just one (case 7) had an aganglionic segment
extending to the proximal ileum (100 cm proximal to the
ileocecal valve). One patient (case 2) had the simultaneous
occurrence of TCA with ileal atresia. There was no related
family history found in patients with TCA.
Imaging findings
Among the imaging findings assessed in our study, a
short and rigid colon, small bowel dilatation, microcolon,
and radiographic transition zone proximal to the cecum
were statistically more significant in patients with TCA
than in patients with non-TCA HD (P < 0.001). A short and
rigid colon was noted in 14 of 14 patients with TCA (100%)
while in one of 53 patients with non-TCA HD (1.9%). Small
bowel dilatation was noted in 14 of 14 patients with TCA
(100%) while in one of 53 patients with non-TCA HD
(1.9%). The contrast enema showed microcolon in three of
14 patients with TCA (21.4%) while in one of 53 patients
with non-TCA HD (1.9%). The radiographic transition
zone of the 14 patients with TCA was noted at the region
from the distal descending colon to the proximal ascending
colon in one (7.1%) and at the region proximal to the cecum
in 13 (92.9%). For the 53 patients with non-TCA HD, the
radiographic transition zone was noted at the region from
the anus to the proximal sigmoid colon in 43 (81.1%), at the
region from the distal descending colon to the proximal
ascending colon in four (7.5%), at the region proximal to
the cecum in one (1.9%) and there was no discernible radiographic transition zone in five (9.4%).
There were no statistically significant differences in
the presence of poor rectal distensibility, colonic wall irregularity, and delayed contrast emptying between patients
with TCA and non-TCA HD. The poor rectal distensibility
Figure 1
1a
1b
1c
1d
1e
1f
Figure 1. a. Microcolon, case 7. b. Non-microcolon, case 8. c. Short and rigid colon with a “question mark” appearance, case 3. d. Poor
rectal distensibility (arrow), case 1. e. Colonic wall irregularity (arrow), case 5. f. Radiographic transition zone at the terminal ileum (arrows),
case 3.
J Radiol Sci March 2012 Vol.37 No.1
13
14
4/F
18/M
188/M
10/M
4/M
1/M
3/M
6/M
58/F
56/F
5/M
7/M
8/M
69/M
No.
1
2
3
4
5
6
7
8
9
10
11
12
13
14
Preterm
Term
Term
Term
Term
Term
Term
Term
Term
Term
Term
Term
Preterm
Term
Gestation
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
Small
bowel
dilatation
✓
✓
✓
Microcolon
N/A: not available; TCA: total colonic aganglionosis
Age at
enema
(days)/
Gender
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
Short and
rigid colon
✓
✓
✓
✓
✓
✓
✓
✓
✓
✓
Poor rectal
distensibility
✓
✓
✓
✓
✓
✓
✓
✓
Colonic
wall
irregularity
Imaging findings
Table 1. Demographic, pathological, and imaging findings of 14 patients with TCA
✓
N/A
✓
N/A
N/A
N/A
✓
✓
✓
✓
Delayed
contrast
emptying
Distal ileum
Distal ileum
Distal ileum
Distal ileum
Distal ileum
Transversal colon
Distal ileum
Proximal ileum
Distal ileum
Distal ileum
Distal ileum
Distal ileum
Distal ileum
Distal ileum
Radiographic
transition
zone
Distal ileum
Distal ileum
Distal ileum
Cecum
Cecum
Distal ileum
Cecum
Proximal ileum
Distal ileum
Distal ileum
Distal ileum
Cecum
Distal ileum
Distal ileum
Pathologic
transition zone
Pseudo-transition zone
Colon perforation during
contrast enema
1) concurrent ileal atresia
and TCA
2) presented with bowel
perforation
Remarks
Imaging of total colonic aganglionosis
J Radiol Sci March 2012 Vol.37 No.1
Imaging of total colonic aganglionosis
Table 2. Comparison of imaging findings of TCA and non-TCA HD
Imaging findings
Small bowel dilatation
Microcolon
TCA (n=14)
Non-TCA HD (n=53)
n (%)
n (%)
14 (100)
1 (1.9)a
<0.001
3 (21.4)
(1.9)a
<0.001
1
P Value
Short and rigid colon
14 (100)
1 (1.9)a
<0.001
Poor rectal distensibility
10 (71.4)
45 (84.9)
0.257
Colonic wall irregularity
8 (57.1)
10 (18.9)
0.082
(60)b
(46.9)c
Delayed contrast emptying
Radiographic
transition zone
6
15
No transition zone
0 (0)
5 (9.4)
Zone 1d
0 (0)
43 (81.1)
1 (7.1)
4 (7.5)
13 (92.9)
1 (1.9)a
Zone
2d
Zone 3d
1
<0.001
aO ne
case in the group of non-TCA HD is skip segment HD with aganglionsis involving the rectosigmoid colon and the terminal ileum. The
patient presented with a short and rigid colon, small bowel dilatation, microcolon, poor rectal distensibility and delayed contrast emptying
on the radiograph and contrast enema imagings.
b10 of 14 patients with TCA had available 24 to 48 hours post-evacuation radiographs. Delayed contrast emptying on 24 to 48 hours postevacuation radiograph was noted in 6 of 10 patients.
c32 of 53 patients with non-TCA HD have available 24 to 48 hours post-evacuation radiographs. Delayed contrast emptying on 24 to 48 hours
post-evacuation radiograph was noted in 15 of 32 patients.
d Zone 1: from the anus to the proximal sigmoid colon; Zone 2: from the distal descending colon to the proximal ascending colon; Zone 3:
proximal to the cecum
HD: Hirschsprung disease; TCA: total colonic aganglionosis
was noted in 10 of 14 patients with TCA (71.4%) while in
45 of 53 patients with non-TCA HD (84.9%). The colonic
wall irregularity was noted in eight of 14 patients with
TCA (57.1%) while in 10 of 53 patients with non-TCA HD
(18.9%). Ten of 14 patients with TCA and 32 of 53 patients
with non-TCA HD had available 24 to 48 hours post-evacuation radiographs for review. Delayed contrast emptying
on 24 to 48 hours post-evacuation radiograph was noted in
six of 10 patients with TCA (60%) while in 15 of 32 patients
with non-TCA HD (46.9%).
DISCUSSION
In the following paragraphs, we will review the demographic and clinical features of TCA. In addition, we will
discuss the imaging findings and the differential diagnosis
of TCA.
Hirschsprung disease is a male predominance with
the male-to-female ratio of 3:1 to 4:1 [4, 8, 20]. Previous
study stated the longer the extent of aganglionosis, the
smaller ratio of male-to-female [3]. The male-to-female
ratio of TCA was reported from 1:1 to 6:1 [8, 18, 21-23],
and the ratio is 4:1 in our study. Most of our patients with
TCA presented in the neonatal period (71%). The results
J Radiol Sci March 2012 Vol.37 No.1
of our study group are consistent with the previous studies
[21, 23]. Some studies reported that the presentation of TCA
might be much later than expected and 14% of TCA present
after the age of six months [24]. In addition, there are few
case reports of TCA presenting as late as adolescence and
early adulthood [25-27]. In our study, the oldest patient with
TCA presented at the age of six months.
The clinical presentations of TCA are similar to those
of classic cases of HD. In our study, most of the patients
presented with symptoms of neonatal bowel obstruction
such as delayed passage of meconium, abdominal distention, feeding intolerance and bilious vomiting. Few patients
presented with chronic constipation and enterocolitis.
Although it is rare, bowel perforation is one of the possible
clinical manifestations and potential complications when
performing contrast enema on these patients. In the literature, bowel perforation was noted in 3% to 4% of patients
with HD [6, 28]. Most of them were long segment HD or
TCA [28]. In our study, two patients (14.3%) developed
bowel perforation— one presented in the initial presentation (case 2) and the other had the attack during the contrast
enema (case 6).
It was reported that the incidence of a positive family
history in TCA is higher than that in other forms of HD [8],
but in our study there was no related family history found
15
Imaging of total colonic aganglionosis
Figure 2
Figure 2. A 2-month-old girl with total colonic
aganglionosis (case 9) had a radiographic pseudotransition zone at the transverse colon (arrows).
in patients with TCA. TCA may be associated with a wide
range of other anomalies such as congenital heart disease,
malrotation, intestinal atresia, Down’s syndrome, mental
retardation, cleft lip and palate [8]. In our study, one patient
with TCA had ileal atresia. The simultaneous occurrence
of TCA with intestinal atresia is rare. There are only a few
case reports in the literature [29, 30].
As compared to the classic HD, TCA is associated with
higher morbidity and mortality rates [4, 5, 8]. Enterocolitis is the most commonly encountered complication with
significant morbidity and mortality [5, 31]. The incidence
of enterocolitis was suggested to increase in patients with
delayed diagnosis [32, 33]. It highlights the importance of
early diagnosis of TCA.
The surgical management of HD has progressed from
a two- or three-stage approach to a one-stage and minimal
access procedure. The treatment of TCA was modified from
the procedures used for classic cases of HD. The choice of
the surgical procedure is partly based on the anticipated
level of transition zone on contrast enema. The preoperative
knowledge of the extent of aganglionic bowel can greatly
influence the surgical planning [11-13]. In addition, it might
reduce the operative time and potentially prevent an incorrect determination of transition zone in the operation [14].
Thus, the findings on contrast enema which might raise the
suspicion of TCA are important.
16
In the literature review, there is a wide spectrum of
radiographic features of TCA on contrast enema and the
diagnosis of TCA is therefore usually difficult to be established [14-18]. There were some retrospective studies of
TCA focusing on the imaging findings [14, 17, 18]. However,
development in the radiographic diagnostic techniques of
TCA has seldom occurred.
In our cases, all patients with TCA showed dilated
small bowel loops on the initial abdominal plain radiographs
which indicate small bowel obstruction. In contrast, small
bowel dilatation was rarely seen in patients with non-TCA
HD except for one case of skip segment HD. This case
presented with small bowel dilatation due to one segment
of agangliosis involving the terminal ileum.
The radiographic transition zone has been reported to
be the most reliable imaging finding of HD [34, 35]. But it
had been documented to be unreliable in TCA [12-14]. In
our study, 13 patients with TCA (92.9%) had radiographic
transition zones at the ileum, which were consistent in the
pathological transition zones. Only one patient (Fig. 2, case
9) had a radiographic pseudo-transition zone at the transverse colon. Based on the results and experiences, the radiographic transition zone is not unreliable in TCA. In fact,
the reason why it is considered to be unreliable is because it
might be difficult to be well evaluated. The location of the
transition zone in TCA is usually at the terminal ileum and
J Radiol Sci March 2012 Vol.37 No.1
Imaging of total colonic aganglionosis
the cecum which are hard to be well demonstrated because
of frequent overlapping by the adjacent bowel loops.
A short and rigid colon was defined as the loss of
redundancy of the sigmoid colon with rounding of the
splenic and hepatic flexures. It is probably related to denervated muscle spasm of the colon. In the literature review,
19% to 23% patients with TCA had this radiographic feature
[17, 18]. However, in our study, all patients with TCA had
short and rigid colon. The discrepancy was probably due
to the various degrees of the presentation and the different
definition of this radiographic feature. The patient with
TCA having obvious short and rigid colon could appear
as a question mark-shaped colon (Fig. 1c) [1, 14, 19]. It is
considered to be a characteristic sign of TCA.
In our study, poor rectal distensibility was used as one
of the radiographic features in TCA instead of the rectosigmoid index. The rectosigmoid index was first described by
Pochaczevsky and Leonidas for aiding the diagnosis of HD
[36]. It is a quantitative method used to assess the caliber
difference between the rectum and sigmoid. However,
previous studies showed that the rectosigmoid index is
valueless in the diagnosis of long segment HD and TCA [13,
14, 37, 38]. Poor rectal distensibility is presumed to result
from poor relaxation of the rectum with aganglionosis.
Although it is not quantitative method as rectosigmoid
index, it can be applied to TCA and long segment HD. That
is the reason why poor rectal distensibility was proposed
instead of rectosigmoid index in our study. It might be
useful to distinguish TCA from other diseases, especially
when the diseases cause microcolon. In other causes of
microcolon such as small bowel atresia and meconium
ileus, the rectal ampulla is often distended, despite the colon
caliber is generally small. This might be the major difference between the TCA and other causes of microcolon.
Colonic wall irregularity was thought to result from
denervation hypersensitivity of smooth muscle or association with colitis [34]. In the literature review, colonic wall
irregularity has been reported to have low sensitivity and
high specificity in HD [10]. In our study, this imaging
finding was noted in 57.1% of patients with TCA and in
18.9% of patients of non-TCA HD. It might be helpful for
diagnosis of TCA.
Delayed contrast emptying was defined as retained
contrast medium proximal to the sigmoid colon on the radiograph which was obtained from 24 to 48 hours after contrast
enema. In the literature review, it has been reported to be
a imaging finding of HD with poor specificity [10, 34, 35].
Delayed contrast emptying might be helpful to differentiate
TCA from meconium plug syndrome [34]. Meconium plug
syndrome could be treated by contrast enema, and it shows
good evacuations of contrast medium on the delayed films.
The imaging features discussed above might be
helpful to distinguish TCA from other diseases with similar
radiographic findings. According to the caliber of colon on
contrast enema, we divide the imaging findings of TCA into
two subgroups— microcolon and non-microcolon to make
differntial diagnosis more systemic. The common differential diagnosis of the two subgroups was discussed in the
following paragraphs and summarized in Fig. 3.
The differential diagnosis of microcolon includes small
bowel atresia, meconium ileus, TCA and other causes of
small bowel obstruction such as adhesion band and postnecrotizing enterocolitis stricture. Small bowel atresia
should be suspected if there is no contrast medium to be
further refluxed into the dilated small intestine. Meconium
ileus should be considered when seeing a dilated distal ileum
with refluxed contrast which containing many round or ovoid
filling defects. Besides microcolon, TCA should have other
Figure 3
Figure 3. Subgrouping approach in the imaging analysis of TCA. HD: Hirschsprung disease; TCA: total colonic aganglionosis; NEC: necrotizing enterocolitis
J Radiol Sci March 2012 Vol.37 No.1
17
Imaging of total colonic aganglionosis
abnormal findings such as a short and rigid colon, radiographic transition zone proximal to the cecum, poor rectal
distensibility, colonic wall irregularity and delayed contrast
emptying, which we have discussed above. Adhesion band
and post-necrotizing enterocolitis stricture may also result
in microcolon, but they are considered in the differential
diagnosis only if the patient has related clinical history.
The differential diagnosis of non-microcolon in
patients with distal intestinal obstruction includes meconium plug syndrome, long segment HD and TCA. Meconium plug syndrome which is an entity in the spectrum of
functional neonatal intestinal obstruction [39] has clinical
symptoms and radiographic features of distal intestinal
obstruction. The symptoms of distal intestinal obstruction
in meconium plug syndrome could be relieved after watersoluble contrast enema [1]. In addition, there is usually no
retained contrast medium in the colon on the 24 to 48 hours
delayed radiograph [34]. Long segment HD is very difficult
to distinguish from TCA by contrast enema. Due to the same
etiology and similar extent of involvement, the radiographic
features of TCA such as a short and rigid colon, poor rectal
distensibility, colonic wall irregularity and delayed contrast
emptying could also be presented in patients with long
segment HD. The major difference between long segment
HD and TCA is the location of transition zone. In TCA,
the transition zone locates at the cecum or small intestine.
However, the pseudo-transition zone could be presented in
patients with TCA like the case in our study (Fig. 2, case
9). It could make us confused TCA with long segment HD.
The imaging findings of patients with non-TCA HD
were reviewed and compared with those of TCA. Among
the imaging findings, a short and rigid colon, small bowel
dilatation, microcolon, and radiographic transition zone
proximal to the cecum were statistically more significant in
patients with TCA than in patients with non-TCA HD (P <
0.001). It is reasonable that most of the cases with non-TCA
HD are short segment HD. The aganglionosis involves short
segment distal colon, which seldom result in a short and
rigid colon, small bowel dilatation, microcolon, and radiographic transition zone proximal to the cecum.
In conclusion, TCA is a colon disease presenting as
small bowel obstruction clinically and radiographically.
Although the radiographic findings of TCA are variable,
they can be divided into two subgroups. In the subgroup
of microcolon, TCA should be differentiated from small
bowel atresia, meconium ileus, adhesion band and postnecrotizing enterocolitis stricture. In the subgroup of nonmicrocolon, TCA should be differentiated from meconium
plug syndrome and long segment HD. We need to take TCA
into consideration when the initial radiograph shows small
bowel dilatation and contrast enema shows a short and rigid
colon, radiographic transition zone proximal to the cecum,
poor rectal distensibility, colonic wall irregularity and
delayed contrast emptying. In addition, biopsy should be
performed to make a definite diagnosis.
18
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