Biliary Atresia with Congenital Anomalies

Dr Ira Shah

doi:https://10.7199/ped.oncall.2015.46

Biliary Atresia with Congenital Anomalies

Dr 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

Show affiliations

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.

Table 1. Investigations of patient.

	Patient 1
Bilirubin (mg/dl)	15.6
Direct (mg/dl)	9.4
SGOT (IU/L)	344
SGPT (IU/L)	218
Total proteins (gm/dl)	6.1
Albumin (gm/dl)	3.2
USG Abdomen	Hepatomegaly, No gall bladder seen
Echocardiography	Mild right plumonary artery stenosis
HIDA	Good extraction, no excretion of tracer
TORCH	Toxoplasma, CMV, Rubella IgG positive

What are the anatomical malformations associated with biliary atresia_?

Discussion
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}

Compliance with ethical standards

Funding: None

Conflict of Interest: None

References

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