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Related Experiment Videos

Biliary atresia with situs inversus: an experience shared.

Sina Aziz1, Ghous Buksh Soomro, Nasir Hassan Luck

  • 1Sindh Institute of Urology and Transplantation, Dow University of Health Sciences and Civil Hospital, Karachi.

JPMA. the Journal of the Pakistan Medical Association
|September 17, 2005
PubMed
Summary

Biliary atresia (BA) with situs inversus (SI) is a rare condition. This report documents the first two cases of BA and SI identified in Pakistan, highlighting associated congenital anomalies.

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Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Congenital Anomalies

Background:

  • Biliary Atresia (BA) is a common cause for pediatric liver transplantation.
  • BA can present with various congenital anomalies.
  • Situs Inversus (SI) is a rare congenital condition where major visceral organs are reversed.

Observation:

  • Two cases of Biliary Atresia (BA) with Situs Inversus (SI) are reported from Pakistan.
  • The first case involved an infant with BA, SI, dextrocardia, and hydronephrosis.
  • The second case presented with BA, SI, and mesocardia.

Findings:

  • Liver biopsy confirmed biliary cirrhosis secondary to extrahepatic biliary atresia (EHBA) in the first patient.
  • Ultrasound and hepatobiliary scintigraphy supported BA diagnosis in the second patient with SI.

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  • Echocardiography confirmed SI with mesocardia in the second patient.

    • Implications:

    • This is the first documented association of BA with SI in Pakistan.
    • Highlights the importance of recognizing rare congenital anomalies in pediatric liver disease.
    • Contributes to understanding the spectrum of BA presentations and associated conditions.