WOFAPS 2025 8th World Congress of Pediatric Surgery

Nedra Zouabi, Sabrine Ben Youssef, Syrine Laribi, Sami Sfar, Myriam Ben Fredj, Samia Belhssan, Amine Ksia, Mongi Mekki, Mohsen Belghith, Lassaad Sahnoun

Pediatric surgery department, Fattouma Bourguiba Hospital, Monastir, Tunisia

Purpose: Biliary atresia (BA) is a congenital malformation characterized by an inflammatory, destructive, and sclerosing process affecting the intrahepatic and extrahepatic bile ducts. This leads to progressive fibrosis and obliteration of the biliary tract, resulting in cholestasis and ultimately hepatic cirrhosis. In the absence of surgical intervention, the outcome is invariably fatal, with death typically occurring within the first 19 months of life due to liver failure and complications of portal hypertension.This study aimed to evaluate the clinical, biological, ultrasonographic, and therapeutic characteristics of infants diagnosed with biliary atresia.

Methods: We conducted a retrospective study from 2019 to 2025, including cases of biliary atresia managed at our department.

Results: A total of 24 infants were included: 15 girls and 9 boys. The mean age at initial presentation was 20 days. The primary reason for consultation was jaundice, which was present in all cases. Hepatic cytolysis was observed in 23 patients, while all had biochemical evidence of cholestasis. A hallmark biological feature was markedly elevated serum gamma-glutamyl transpeptidase (GGT) levels, with a mean value of 650 IU/L (range: 110–1890 IU/L). Abdominal ultrasound was performed in all patients: it was normal in 2 cases, while 22 showed findings suggestive of BA. All patients underwent Kasai portoenterostomy as the initial surgical management. The mean age at surgery was 2 months, with a range from 19 days to 3 months and 22 days. Postoperatively, all infants received ursodeoxycholic acid and supplementation with fat-soluble vitamins.

Conclusion: Serum GGT levels play a pivotal role in the diagnosis of BA when combined with conventional ultrasound findings. However, a normal abdominal ultrasound does not rule out the diagnosis. Early recognition and timely surgical intervention remain key to improving outcomes.