7/31/2019 Biliary Atresia Associated with Polysplenia Syndrome,
Situs Inversus Abdominus, and Reverse Rotation of Intestine
1/2
Mirza et al, Biliary atresia polysplenia syndrome
APSP J Case Rep2012; 3: 14
L E T T E R TO TH E E D I T O R OPEN ACCESS
Biliary Atresia Associated with Polysplenia Syndrome, Situs
Inversus Abdominus, and
Reverse Rotation of Intestine
Bilal Mirza,* Shahid Iqbal, Afzal Sheikh
Dear Sir,
In 25% of cases of biliary atresia associated malfor-
mations are present; polysplenia constitute 10-50%
of these associated anomalies. Biliary atresia found
associated with polysplenia syndrome, heterotaxy,
and reverse rotation of intestine, in isolation or in
various combinations; however its association with
polysplenia syndrome, situs inversus abdominus,
and reverse rotation of gut in the same patient is not
reported in English language literature [1,2].
A 2-month-old female infant presented with jaundice,
clay colored stools, and abdominal distension since
the first week of life. She was a product of consan-
guineous marriage and born via normal vaginal de-
livery. General physical examination revealed a vital-
ly stable infant with obvious jaundice and abdominal
distension. Abdominal examination revealed hepa-
tomegaly. Her laboratory investigations showed con-jugated
hyperbilirubinemia (total bilirubin 14 mg/dl,
and direct bilirubin 8 mg/dl). Gallbladder was not vis-
ualized on ultrasound of the abdomen. On HIDA
scan no excretion of the radiopharmaceutical tracer
was noted. At operation the gallbladder was found
atretic (Fig. 1); per-operative cholangiogram con-
firmed extrahepatic biliary atresia. The liver was en-
larged and central in position. Stomach and two big
spleens (non-floating) were present on the right side
of the abdominal cavity (Fig. 1). The duodenum was
entirely intra-peritoneal and transverse colon was
retroperitoneal in position (Fig. 2).
The atretic gallbladder and portal plate were dissect-
ed meticulously. Roux-en Y hepatico-jejunostomy
was performed without going through the transverse
colon window. Both the spleens were left as such.
No effort was made to correct reverse rotation. The
postoperative recovery was uneventful. Patient was
allowed oral feeding on 5th
day of operation and dis-
charged on 7th
day. Liver biopsy showed early cir-
rhotic changes. Postoperatively, echocardiography
and chest x-ray did not show intrathoracic
heterotaxy. She was on follow up of gastroenterolo-
gy department for further management.
Figure 1: Showing atretic gallbladder (AG); stomach (St) and
two
spleens (S) on right side; and intraperitoneal duodenum (D).
Polysplenia syndrome is associated with a number
of anomalies i.e. heterotaxy of abdominal or thoracic
organs, malrotation of gut, biliary atresia, reverse
rotation of gut, intestinal atresia, retroperitoneal
teratoma, vena caval anomalies, cardiac, and lung
anomalies. Few case reports described the associa-
tion of biliary atresia with polysplenia syndrome, si-
tus inversus, and immotile cilia syndrome.
Polysplenia syndrome is more frequently associated
with vascular and cardio-pulmonary anomalies how-
ever the work of Chandra proved that a subclass of
patients of polysplenia syndrome had biliary atresia
7/31/2019 Biliary Atresia Associated with Polysplenia Syndrome,
Situs Inversus Abdominus, and Reverse Rotation of Intestine
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Mirza et al, Biliary atresia polysplenia syndrome
APSP J Case Rep2012; 3: 14
that were associated with gastrointestinal and res-
piratory anomalies [1-3].
Figure 2: Showing intraperitoneal duodenum (D) and
retroperito-
neal transverse colon (TC).
The abdominal heterotaxyin a case of biliary atresia
has few surgical implications. These problems are
related to the orientation of roux-en-y loop. In a usual
case of biliary atresia, the roux-en-y loop is passed
through a rent made in the mesentery of transverse
colon, however in case of malrotation and reverse
rotation of gut the there could be difficulties in the
orientation of the roux-en-y loop. This difficulty is
further incremented by mirrored anatomy of ab-
dominal viscera. In our case the stomach and
spleens were lying on right side of the abdomen. The
duodenum was intraperitoneal and transverse colon
retroperitoneal, therefore mesentery of transverse
colon was not available. Thus we have to take the
roux-en-y loop directly to the portal plate for
hepatico-jejunostomy. To conclude, biliary atresiapolysplenia
syndrome is a rare occurrence. The op-
erating surgeon must be aware of the surgical impli-
cations that may occur in case of associated
malrotation/reverse rotation, and mirrored alimentary
tract anatomy.
REFERENCES
1. Rasool F, Mirza B. Polysplenia syndrome associated with
situs inversus abdominus and type I jejunal atresia. APSP
J Case Rep 2011; 2:18.
2. Chandra RS. Biliary atresia and other structural
anomalies
in the congenital polysplenia syndrome. J Pediatr
1974;85:649-55.
3. Emmanuel J, Danile C, Nicole R, Michelle H. CFC1
Gene Mutation and Biliary Atresia With Polysplenia Syn-
drome. J Pediatr Gastroenterol Nutrit 2002;34:326.
AFFILIATION:Department of Paediatric Surgery
The Childrens Hospital and the Institute of Child Health
Lahore,
Pakistan.
CORRESPONDENCE:* Dr. M. Bilal Mirza, Department of Pediatric
Surgery,
The Childrens Hospital and the Institute of Child Health
Lahore,
Pakistan.
Email:* [email protected]
Received on: 12-01-2012 Accepted on: 18-04-2012
http://www.apspjcaserep.com 2012 Mirza et al ,
This work is licensed under a
CreativeCommonsAttribution3.0UnportedLicense
Competing Interests: None declared Source of Support: Nil
How to cite
Mirza B, Iqbal S, Sheikh A. Biliary atresia associated with
polysplenia syndrome, situs inversus abdominus, and reverse
rotation of intestine.APSP J Case Rep 2012; 3: 14.
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