Choledochal Cysts in Children: A Single-Center Study in South India.

Objectives Choledochal cysts are rare congenital anomalies of the biliary tract with limited data on their clinical spectrum and outcomes in pediatric populations, particularly in South India. This study aimed to analyze the clinical presentation, types, associated comorbidities, management, and long-term outcomes of choledochal cysts in children over a 20-year period at a single tertiary care center. Methods This retrospective audit of hospital records study was conducted at the Amrita Institute of Medical Sciences and Research Center, Kochi, Kerala, India. Data from 147 pediatric patients with choledochal cysts were analyzed, including clinical presentation, radiological findings, laboratory investigations, management, and outcomes. Results Among 147 patients, 103 were female (M:F ratio = 1:2.4). The mean age of the patients at presentation was 3.4 months. Abdominal pain (58%, n=85) was the most common symptom, while the classic triad of pain, jaundice, and palpable mass was rare (4%, n=6). Type I cysts (63%, n = 93) were the most frequent, followed by type IV (31%, n = 46) and Caroli's disease (5.4%, n=8). Anomalous pancreaticobiliary junction (APBJ) was seen in 14% (n=21) of patients, of whom 43% had pancreatitis. Overall, pancreatitis occurred in 20% of patients (n=29), and renal anomalies were noted in 12% (n=18). Surgical intervention was performed in 75% (n=110) of the patients, with a mean age at surgery of 4.1 years. Histopathology revealed metaplastic changes in 3% of the cases (n=3). Long-term complications included chronic liver disease (7%, n=11) and portal hypertension (4%, n=6). Conclusion This study highlights the clinical profile of pediatric choledochal cysts in South India, emphasizing early diagnosis and surgical intervention, which may reduce complications and improve outcomes. Multicenter studies are needed to improve understanding of disease progression and outcomes.