Biliary atresia splenic malformation syndrome: A case series

Authors

  • Rahul Gupta
  • Atul Gupta

DOI:

https://doi.org/10.46831/jpas.v2i2.173

Keywords:

Biliary atresia, Kasai portoenterostomy, Malrotation, Polysplenia, Preduodenal portal vein, Porto-duodenostomy, Situs inversus

Abstract

Background: Biliary atresia with splenic malformation (BASM) syndrome carries an unfavourable outcome related to complex surgical anatomy that poses technical difficulties during surgery.

Case Presentation: Herein we report two cases of biliary atresia with polysplenia syndrome. Other associated anomalies were complex cardiac anomalies (in both), preduodenal portal vein (in one), midgut malrotation (in one), and right hepatic artery originating from the superior mesenteric artery (SMA) (in one). In one patient Ladd’s procedure along with porto-duodenostomy was done while in the other, Kasai porto-enterostomy was performed. One patient is alive, with normal liver function tests, on a 3 year follow-up.

Conclusion: Patients with biliary atresia and splenic malformation syndrome have complex associated anomalies. Additional procedures or modification of surgical procedures are often required that may affect the already guarded prognosis.

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Author Biographies

Rahul Gupta

SMS Medical College, JLN Marg, Jaipur, Rajasthan 302004, India

Atul Gupta

Senior Consultant, Department of Paediatric Surgery Neoclinic, Jaipur

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Published

2023-12-23

How to Cite

1.
Gupta R, Gupta A. Biliary atresia splenic malformation syndrome: A case series. J Pediatr Adolesc Surg [Internet]. 2023Dec.23 [cited 2024Nov.6];2(2). Available from: http://jpedas.org/ojs/index.php/jpedas/article/view/173
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