Biliary Atresia with Congenital Anomalies
 
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Ira Shah
Medical Sciences Department, Pediatric Oncall, Mumbai, India

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056


Clinical Problem :
A 1½ month old girl born of non consanguineous marriage presented with jaundice and clay colored stools since birth. On examination, she had cleft lip and palate with jaundice and hepatomegaly. Investigations are depicted in Table 1. Child underwent portoenterostomy after 1 week. On table, it was noted that child had atretic gall bladder with malrotation with Meckel’s diverticulum and megaureter. A karyotype was sent but subsequently on discharge, patient was lost to follow up.


Question :
What are the anatomical malformations associated with biliary atresia_?

Expert Opinion :
Biliary atresia is characterized by obliteration or discontinuity of the extra hepatic biliary system, resulting in obstruction to bile flow. There are 3 types of biliary atresia:
Type 1: Atresia restricted to common bile duct
Type 2: Atresia of the common hepatic duct
Type 3: Atresia of the right and left hepatic duct

Patients with biliary atresia generally pass acholic stools with onset at about 2 weeks of life. They are average birth weight. They have firm hepatomegaly. {1} There is a female predominance. They may have associated polysplenia syndrome, heterotaxy, and reverse rotation of intestine, in isolation or in various combinations and intra-abdominal vascular anomalies. {2} In addition, malrotation, Meckel’s diverticulum and jejunal atresia have also been reported with biliary atresia. {3} Biliary atreisa in association with other congenital structural anomalies may have a poor prognosis. These patients have poor bile secretion after hepatic portoenterostomy. {4}


References
1. Shah I, Parikh S. Clinical and Biochemical Factors Associated With Biliary Atresia. Tropl Gastroenterol. 2012` 33: 214-217
2. Rasool F, Mirza B. Polysplenia syndrome associated with situs inversus abdominus and type I jejunal atresia. APSP J Case Rep. 2011` 2: 18
3. Kataria R, Kataria A, Gupta DK. Spectrum of congenital anomalies associated with biliary atresia. Indian J Pediatr. 1996`63:651-654
4. Tanano H, Hasegawa T, Kawahara H, Sasaki T, Okada A. Biliary atresia associated with congenital structural anomalies. J Pediatr Surg. 1999` 34: 1687-90


Correct Answers :  yes 19%
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