Pediatr Neonatol 2010;51(3):186−189

C ASE R EPORT

Congenital Colonic Atresia: Report of One Case

Chieh-Teng Hsu1, Shie-Shan Wang2, Jia-Fu Houng1, Pei-Jung Chiang1,
Chung-Bin Huang3,4*
1
Department of Pediatrics, Chang Gung Memorial Hospital at Chia-Yi, Chia-Yi, Taiwan
2
Department of Pediatric Surgery, Chang Gung Memorial Hospital at Chia-Yi, Chia-Yi, Taiwan
3
Department of Pediatrics, Kuang Tien General Hospital, Taichung, Taiwan
4
Department of Nursing, Hung Kuang University, Taichung, Taiwan

Received: Feb 26, 2009 Colonic atresia is a very rare cause of intestinal obstruction, and surgical manage-
Revised: Jul 13, 2009 ment is the mainstay of therapy. A case of congenital colonic atresia is reported in
Accepted: Aug 3, 2009 a full-term neonate who presented with delayed passage of meconium, abdominal
distention and bilious vomiting. The present case and the pertinent literature are
KEY WORDS: discussed, with an emphasis on surgical management.
atresia;
colon;
surgery

1. Introduction admitted with abdominal distention, bilious vomit-

Colonic atresia (CA) is a rare cause of congenital
bowel obstruction, accounting for 5−15% of all cases
of bowel atresia in newborns.1 Various incidences
had been reported, ranging from 1 in 1500 to 1 in
66,000 live births.2,3 CA was first recorded by Binniger
in 1673;4 the first survivor was reported by Gaub5 in
1922 and was treated using a diverting colostomy.
Potts6 reported the first survivor following primary
repair in 1974. Because of its rarity, little informa-
tion has been available about the management and
predictors of outcome. We present a case and review
the literature, both of which raise interesting points
regarding the management of this rare disease.
2. Case Report
A 3-day-old full-term female baby with a birth weight
of 2770 g was born to a 16-year-old mother. She was
ing and failure to pass meconium. The family history,
maternal history and prenatal examinations were
all unremarkable. No drugs or infectious exposure
was documented during pregnancy. Physical exam-
ination revealed dehydration, with a body weight
of 2465 g. Her respiratory condition revealed dis-
tress with subcostal retraction. A distended abdo-
men accompanied by hypoactive bowel sounds was
also observed. X-ray examination of the chest and
abdomen revealed increased intestinal gas, mainly
in the colon. A contrast enema showed ascending
colon atresia and distal microcolon (Figure 1).
Type IIIa atresia of the colon was found at lapa-
rotomy. A perioperative photograph of the patient
with transverse colon atresia showed dilated as-
cending colon (Figure 2). The discrepancy in diam-
eters between the proximal and distal segments
was 9:1. Biopsies of the distal colon and appendix
were performed and revealed the presence of gan-
glion cells. A temporary double-barrel colostomy

*Corresponding author. Department of Pediatrics, Kuang Tien General Hospital, 117 Shatien Road, Taichung 433, Taiwan.
E-mail: cb2147@ktgh.com.tw

©2010 Taiwan Pediatric Association

Congenital colonic atresia
Figure 1 Lower gastrointestinal barium enema showed
small caliber of transverse colon, descending colon, sig-
moid colon and rectum, compatible with microcolon.
Figure 2 Interruption of gastrointestinal tract at the
transverse colon level with double blind ends. There was
marked discrepancy in the size of the proximal and distal
colon on either side of the atretic segment.
was performed. The postoperative course was un-
eventful. Delayed anastomosis was performed at
4 months of age, despite gross disparity between
the proximal and distal colon sizes (diameter dis-
crepancy of 5:1, proximal:distal). A subsequent con-
trast enema confirmed smooth barium passage with
smooth retrograde filling up to the terminal ileum
(Figure 3).
A follow-up at 7 months of age showed the baby
was feeding well, with a body weight of 6100 g (third
percentile), body length of 67 cm (fiftieth percen-
tile) and head circumference of 42.5 cm (fiftieth
percentile).
3. Discussion
The etiological basis of CA is believed to be a vas-
cular insult to the mesenteric vessels during fetal
187
Figure 3 Contrast enema confirmed smooth retrograde
filling up to the terminal ileum, after closure of colostomy.
development. This theory was first proposed by Louw
and Barnard7 in 1955. Segmental ischemia may be
caused in vivo by hematological events (embolus
from the placenta or spontaneous thrombosis), or
mechanical events (volvulus, intussusception or stra-
ngulated herniation).8 A report by Benawra et al9
of three cases occurring amongst first-degree rela-
tives of a family supports a genetic basis. CA has
been associated with numerous anomalies, including
omphalocele, gastroschisis, exomphalos, vesico-
intestinal fistula, Hirschsprung’s disease and im-
perforate anus, as well as choledochal cyst.10−14
CA presents from birth with typical features of ob-
struction: abdominal distention, bilious vomiting
and failure to pass meconium. Plain X-rays typically
show dilated loops of proximal bowel. The diagnosis
is made following contrast enema studies showing
a narrowed distal colon coming to an abrupt halt at
the level of the atresia. Our case presented with the
above clinical features and radiological findings.
The classification of intestinal atresias has also
been applied to CA, initially proposed by Louw15 and
later refined by Martin and Zerella16 and Grosfeld
et al,17 to include the apple-peel deformity and
multiple atresias. According to this classification,
a type I defect represents a mucosal defect with
an intact mesentery; a type II defect consists of
fibrous cord connecting the atretic bowel ends; a
type IIIa lesion consists of an atretic segment with
a V-shaped mesenteric gap defect, while a type IIIb
lesion consists of the apple-peel deformity, where
there is proximal colonic atresia and the distal bowel
is supplied by a single retrograde blood vessel; a type
IV lesion describes instances of multiple atresias
(“string of sausage” effect). Most cases present with
type IIIa defects, as in the current case.
Operative management of CA has been modified
over the years. Earlier studies recommended resec-
tion with primary anastomosis for atresia proximal
188
to the splenic flexure, and colostomy with delayed
anastomosis for atresia distal to this point.17−20 In
recent years, however, several authors have advo-
cated the use of resection and primary anastomosis,
regardless of the location of the atresia.21,22 How-
ever, primary anastomosis may be technically diffi-
cult because of the very large discrepancy between
the sizes of the distal and proximal bowels,23 and
the loss of length associated with resection of the
dilated proximal colon. Cox et al24 suggested that
primary anastomosis could be safely performed with
a diameter variance of 3:1 (proximal:distal) and with-
out distal mechanical or functional obstruction. In
our case, delayed anastomosis without bowel resec-
tion was performed 4 months later, with a diameter
variance of 5:1.
Microcolon generally results from intrauterine
underutilization of the colon, including conditions in
which the intestinal contents are not passed into
the colon during gestation. We administered warmed
normal saline solution to the distal microcolon three
times a day before the colostomy was closed, with
the aim of stimulating the bowel to grow. The re-
sult was encouraging, and the diameter discrepancy
decreased from nine-fold to five-fold over a period
of 4 months. Dalla Vecchia et al25 tested a protocol
using glutamine and complex carbohydrate diets in
four patients with bowel atresia after primary anas-
tomosis, and the total parenteral nutritional require-
ments were significantly reduced in all cases. One
patient became independent of total parenteral
nutrition, while three others were able to receive
more of their intake enterally than parenterally.25
Further studies are needed to determine if this pro-
tocol stimulates growth of the gut that is already
there, or if it accelerates adaptation.
The prognosis of CA in the absence of other se-
rious pathologies is good if prompt action is taken,
with an overall mortality of 10% or less;26 however,
a delay in diagnosis beyond 4 days may result in a
mortality as high as 100%.27
In conclusion, we emphasize that although very
rare, CA must be considered as a cause of distal
neonatal bowel obstruction. A single, disproportion-
ately enlarged air-filled bowel loop on abdominal
radiograph is suggestive of CA. Diagnosis can be con-
firmed with a contrast enema. Operative correction
of CA has evolved over the years. Although primary
anastomosis can safely be performed after resection
of the dilated proximal colon in right-sided lesions,
this necessitates sacrifice of the whole proximal
colon and ileocecal valve. If there is a significant
atretic segment with loss of colon length and if the
proximal colon is to be preserved, then primary
anastomosis may not be safe, and we recommend
a staged approach. The importance of performing
rectal histology to exclude Hirschsprung’s disease
C.T. Hsu et al
before colostomy closure cannot be over emphasized.
A subsequent contrast enema is optional.
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