Abstract
INTRODUCTION: Choledochal cysts are rare congenital anomalies primarily affecting females, with a higher incidence in Asian populations and often diagnosed in childhood. Characterized by an abnormal pancreaticobiliary union, these cysts can lead to serious complications, including malignancy in about 7.5 % of cases. Surgical excision is the preferred treatment to prevent severe outcomes, highlighting the importance of early diagnosis and long-term follow-up. CASE PRESENTATION: A 2-month-old girl, conceived via egg donation and gestational surrogacy, presented with a 2-week history of jaundice and a birth weight of 3 kg. Prenatal findings were unremarkable, but post-admission imaging confirmed a choledochal cyst measuring 93 × 118 mm, alongside a rare finding of total agenesis of the distal common bile duct. Eight days after admission, she underwent exploratory laparotomy, where cholecystectomy and resection of the choledochal cyst were performed, followed by Roux-en-Y hepaticojejunostomy. The patient tolerated the procedure well and was discharged in good condition four days later. CLINICAL DISCUSSION: Choledochal cysts are rare congenital anomalies of the biliary tree, asian populations report a significantly higher incidence of approximately 1 in 1000 live births. The patient showed acceptable outcomes at a two-year follow-up, with a normal liver biopsy report. CONCLUSION: Our report emphasizes the importance of prompt diagnosis and differentiation between giant choledochal cysts and biliary atresia due to their similar presentations, which significantly influence treatment strategies and outcomes. Enhanced awareness and the effective use of imaging techniques are crucial for pediatric surgeons to ensure timely surgical intervention and improve patient management.