[Biliary atresia and polysplenia syndrome]

Area of Science:

• Pediatric Surgery
• Medical Genetics
• Developmental Biology

Background:

• Polysplenia syndrome is a rare congenital disorder characterized by multiple spleens and associated visceral anomalies.
• It frequently involves cardiac, vascular, intestinal, and biliary system malformations, posing complex clinical challenges.

Observation:

• A neonate presented with upper intestinal obstruction and situs inversus, diagnosed with polysplenia syndrome.
• Surgical findings included multiple spleens, a preduodenal vein, duodenal atresia, and biliary atresia.
• The patient underwent duodenoduodenostomy and Kasai hepatoportoenterostomy, with successful short- and long-term outcomes over 23 years.

Findings:

• This case demonstrates a 23-year survival in a patient with polysplenia syndrome and biliary atresia.
• Early diagnosis and surgical treatment of biliary atresia are crucial for improving outcomes and native liver survival.
• Long-term survivors, even without jaundice, often develop biliary cirrhosis requiring lifelong monitoring.

Implications:

• The study underscores the importance of early intervention for biliary atresia in patients with polysplenia syndrome.
• Understanding the interplay between polysplenia and biliary atresia prognosis is vital for patient management.
• Lifelong follow-up is essential for survivors due to the high incidence of biliary cirrhosis.