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KMID : 1152420220280010017
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Advances in Pediatric Surgery
2022 Volume.28 No. 1 p.17 ~ p.20
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Biliary Atresia Splenic Malformation With Situs Inversus: A Case Report
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Solanki Shailesh
Kanojia Ravi
Reddy Manasa
Ahmad Mohammad Fahim
Lal Sadhna B.
Samujh Ram
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Abstract
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Biliary atresia is a progressive sclerosing cholangiopathy of bile ducts. Most of the time it is an isolated anomaly but can present with syndromic forms. The biliary atresia splenic malformation (BASM) syndrome is associated with splenic anomalies, vascular anomalies, and visceral asymmetry along with biliary atresia. The surgical anatomy of BASM is distinctive and creates a challenge for the surgeons. We are describing a case of BASM with situs inversus and highlighting the approach, intraoperative anatomy, and surgical intricacies of Kasai portoenterostomy in such a situation with the review of pertinent literature.
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KEYWORD
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Biliary atresia, Polysplenia, Situs inversus, Kasai procedure, Jaundice
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