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Case Report

Biliary Atresia Associated with Jejunal Atresia and

a Review of the Literature in Japan

Koushi Asabe,1,2 Ko Yukitake,3,4 Toshiko Mori,3,4 Akihisa Mitsudome3 and Takayuki Shirakusa,1
1Second Department of Surgery and 3Department of Pediatrics, Fukuoka University School of Medicine, and Divisions of
2Pediatric Surgery and 4Neonatology, Maternity and Perinatal Care Center, Fukuoka University Hospital, Fukuoka, Japan.

An unusual case of biliary atresia with jejunal atresia is herein described. Only 12 cases demonstrating biliary
atresia associated with a jejunal atresia have been previously reported in Japan. The pathogenesis of biliary atresia
is thought to be secondary to the influence of jejunal atresia. [Asian J Surg 2005;28(2):154–7]

Key Words: biliary atresia, jejunal atresia

Introduction demonstrated microcolon, which indicated the presence of

The incidence of jejunoileal atresia (JIA) varies from 1:330 and
1:400 live births in some reports to between 1:1,500 and
1:3,000 live births in others,1 and jejunal atresia (JA) is said to
account for about 40–50% of these.2,3 Biliary atresia (BA) is
probably the result of an insult to the biliary tract following
initial normal development.4,5 The incidence of BA is approxi-
mately 1:10,000 live births in Japan.5 Therefore, the associa-
tion of JA with BA is extremely rare. We present one such case
and review 12 other cases previously reported in Japan.6–14
Case report
A female of 38 weeks’ gestation weighing 2,398 g was born by
spontaneous normal vaginal delivery. The mother had had an
uneventful pregnancy. The Apgar scores were 10 (1 minute)
and 10 (5 minutes). Shortly after birth, the infant developed
marked abdominal distension and continuous vomiting. As
a result, she was transferred to the neonatal intensive care
unit of Fukuoka University Hospital for evaluation and treat-
ment of intestinal obstruction. On admission, she looked
well and had no obvious external anomalies. Plain radio-
graphs showed a dilated small bowel (Figure 1) and a cologram
intestinal atresia. At laparotomy, a type I atresia (Grosfeld’s
classification) was observed at 55 cm from the ileocaecal valve
on the oral side, along with the microintestine and a markedly
distended jejunum proximal to the atresia (Figure 2). There
was no evidence of meconium peritonitis caused by perfora-
tion of small-bowel atresia. A short segment of the JA was
resected and end-to-back anastomosis was performed. The
gallbladder was in the normal position and of normal size.
Postoperatively, the unconjugated bilirubin level remained
elevated and she was treated using phototherapy for 5 days,
which resulted in a decrease in the bilirubin level. However,
the bilirubin level did not return to normal during the first
month of life. Fever and eruption were noted in the infant on
the 12th day. The leucocyte count was 8,700/+L and the
platelet count was 119,000/+L. The C-reactive protein level
was 7.5 mg/dL. Arbekacin was used for suspected bacterial
or viral infection. Stools, at first normally coloured, became
acholic around the 28th day.
Another laparotomy was performed on the 51st day under
suspicion of BA. Although the gallbladder was in the normal
position, the lumen was obliterated. Only a small fibrous cord
of tissue was found in the remainder of the extrahepatic biliary
tree. As a result, operative cholangiography was not possible.

Address correspondence and reprint requests to Dr. Koushi Asabe, Second Department of Surgery, Fukuoka
University School of Medicine, 45-1, 7-chome Nanakuma Jonan-ku, Fukuoka 814-0180, Japan.
E-mail: asabe@fukuoka-u.ac.jp • Date of acceptance: 29 January 2004

© 2005 Elsevier. All rights reserved.

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 ■ JEJUNAL & BILIARY ATRESIA ■
Figure 1. Plain radiograph showing a dilated small bowel.
Figure 2. Type I atresia showing the bulbous jejunal atresia with the
distal collapsed polypoid-like lesion.
The liver became firm and fibrous such as in cirrhosis. Dissec-
tion of the porta hepatis revealed no patent duct or sinus. A
Kasai’s portoenterostomy was thus performed. After this
operation, bile was again slightly excreted. However, the pa-
tient eventually died of sepsis due to ascending cholangitis at
4 months of age. An autopsy could not be performed.
ASIAN JOURNAL OF SURGERY VOL 28 • NO 2 • APRIL 2005
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Discussion
The presence of an atretic segment of the jejunum or ileum
constitutes the most frequently encountered case of small
bowel obstruction in the neonatal period.15 A Japanese Society
of Pediatric Surgeons report showed 304 cases of intestinal
atresia in Japan in 1998, indicating an overall incidence of 2.53
per 10,000 total births.16 About half of intestinal atresias
below the stomach involve the duodenum, while the jejunum
and ileum comprise the other half. Colonic atresia is an ex-
tremely rare condition.2 JIA is not usually associated with
anomalies in organ systems other than those directly con-
cerned with the bowel. Isolated examples of intestinal atresia
associated with cardiovascular anomalies have been reported,
but unless it is associated with duodenal atresia or hindgut
abnormalities, JIA usually occurs as an independent single
abnormality.15 Kishida et al reported that three of 79 JIA
patients had BA.11 Three of Akiyama et al’s 57 patients with JA
had BA, although none of their 51 patients with ileal atresia
had BA.17 The incidence of BA in cases of JIA ranges from 0%
to 0.5%.11
On the other hand, there is a definite association between
BA and polysplenia syndrome, and this is the most common
malformation complex among the reported associated
anomalies. Polysplenia syndrome is seen in 25% of all BA
cases.4 Among the 1,483 Japanese patients evaluated in that
study, 359 (24.3%) had one or more associated anomalies.
Polysplenia was found in 33 patients, a preduodenal portal
vein in 16, situs inversus in 16, accessory spleen in five, asplenia
in two and other organs in 287.18 The association of BA with
intestinal atresia is rare. The most frequent form of intestinal
atresia associated with BA is duodenal atresia.7,9,13 De Lorimier
et al reported only two cases of BA in 619 patients with small
bowel atresia and stenosis.19 Ohi et al revealed that one patient
in 214 with BA had meconium peritonitis due to a perforation
of the JA.10 Kishida et al reported that three of 783 BA patients
had meconium peritonitis due to JIA.11 The incidence of JIA
in cases of BA is reported to range from 0% to 3.2%.11 There-
fore, the association of JIA with BA is extremely rare. We
observed one such case and also found 12 other cases previ-
ously reported in Japan (Table).6–14
BA is characterized as an obliterative cholangiopathy that
may affect not only the extrahepatic but also the intrahepatic
bile duct system. Although a number of factors have been
implicated, such as development malformation, perinatal
viraemia, toxicity of bile constituents and anatomical abnor-
malities in the hepatobiliary duct system, the actual cause
155
 ■ ASABE et al ■

Table. Cases of biliary atresia (BA) with jejunoileal atresia reported in Japan

Gestation Body weight Classification Age at IA Classification Age at BA

No. Study Sex Outcome Follow-up
(wk) (g) of IA surgery of BA surgery

1 Shimura et al6 M 35 2,420 Jejunum II 12 d III 107 d Died 7 mo

2 Arima et al7 F – 3,150 Jejunum III 12 d – 156 d Died 2 yr
3 Akiyama et al8 M 40 3,310 Jejunum III 14 d III 177 d Alive 8 yr 8 mo
4 Yura et al9 F – 2,550 Jejunum III 12 d – 112 d Died 86 d
5 Ohi et al10 F – – Jejunum – – I cyst – Alive –
6 Kishida et al11 M 32 2,528 Jejunum II 15 d – 118 d Alive –
7 Kishida et al11 F 39 1,840 Jejunum – 13 d I cyst 165 d Died 69 d
8 Kishida et al11 M 38 3,300 Jejunum, ileum II 15 d III 169 d Died 4 mo
9 Iijima et al12 F 37 2,984 Multiple – 14 d I cyst 157 d Alive 9 mo
10 Iijima et al12 M 38 3,205 Multiple – 16 d I cyst 144 d Alive 4 mo
11 Yanagihara et al F – 2,775 Multiple – 14 d III 158 d Died 9 mo
and Inoue et al13,14
12 Inoue et al14 F 33 2,528 Ileum – 11 d III 171 d Alive 1 yr 2 mo
13 Present case F 38 2,398 Jejunum I 11 d II 151 d Died 4 mo

IA = intestinal atresia.

remains unknown.20 The association of BA and duodenal
malformation might be presupposed from their common
points of embryonic development.13 However, eight of the 13
BA patients with JIA (Cases 1, 4–9, 12) demonstrated meco-
nium peritonitis.6,9–13 Kishida et al and Yanagihara et al specu-
lated that meconium peritonitis might be one of the causes of
BA.11,13 Although in some cases, including the present case,
the gallbladder is in the normal position and of normal size at
first laparotomy, and bile content is clearly found by nasogas-
tric suction, acholic stool is also found continuously and un-
conjugated bilirubin remains elevated postoperatively. Post-
natal bacterial or viral infection with fever and eruption was
suspected in the present case. We speculate that the relation-
ship between BA and JIA may thus have a peri- or postnatal
origin and may be related to either a viral or bacterial origin.
Seven deaths were reported among the 13 patients demon-
strating BA with JIA in Japan, giving a survival rate of 46.2%.
The reported 6-month survival rate is 82% in BA patients with
a previous portoenterostomy, and the 3-year actuarial survi-
val rate for all patients is 75%.21 The overall survival rate for
BA among 1,483 patients was 88.1% in Japan from 1989 to
2000.18 Three of the six surviving cases had cystic type I atre-
sia. It is generally believed that cystic type I BA has a good
prognosis. The age at BA surgery is the most influential factor
in the prognosis. The survival rate for BA in the USA from 1976
to 1989 was 62.5% for infants treated before 30 days, 43.6%
for those treated between 31 and 60 days, and only 28.6% for
those treated at more than 90 days of age.21,22 On the other
hand, among BA patients with JIA in Japan, 0/1 treated before
30 days survived, 2/5 treated between 31 and 60 days survived
and 3/6 treated after 61 days survived.6–14 As a result, the age
at BA surgery was not a prognostic factor in Japan. Repeated
cholangitis also has a detrimental effect on the surgical re-
sults.21 Han et al revealed risk factors of ascending cholangitis
in BA patients with short bowel syndrome.23 Bacterial over-
growth in the small bowel is a well-recognized problem in
children with short bowel syndrome. Another area of concern
involves the management of short bowel syndrome in BA,
which requires extended total parenteral nutrition, and has
also been shown to increase the risk of cholestasis leading to
liver cirrhosis, sepsis and increased mortality risk. The preven-
tion of ascending cholangitis is very difficult in BA patients
with JIA due to a short small intestine. Therefore, the survival
rate for BA with JIA is very low compared with that for all BA
patients.
In conclusion, we were unable to clearly describe the
pathogenesis of the coexistence of BA and JIA. However, we
postulate that such a relationship may have a peri- or postna-
tal origin due to either a bacterial or viral infection.
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