Cherukuri Venkat Kumar Raju, Radhakrishnan Shilpa, Pillai R Bhanu Vikraman
Pediatrics, Amrita Institute of Medical Sciences and Research Center, Kochi, IND.
Radiodiagnosis, Amrita Institute of Medical Sciences and Research Center, Kochi, IND.
Cureus. 2025 Jun 21;17(6):e86499. doi: 10.7759/cureus.86499. eCollection 2025 Jun.
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.
目的 胆总管囊肿是一种罕见的先天性胆道异常，关于其临床谱及小儿群体（尤其是印度南部）预后的数据有限。本研究旨在分析一家三级医疗中心20年间儿童胆总管囊肿的临床表现、类型、合并症、治疗及长期预后。方法 本回顾性医院记录研究在印度喀拉拉邦科钦市的阿姆里塔医学科学与研究中心开展。分析了147例患胆总管囊肿儿童患者的数据，包括临床表现、影像学检查结果、实验室检查、治疗及预后。结果 147例患者中，103例为女性（男:女比例 = 1:2.4）。患者就诊时的平均年龄为3.4个月。腹痛（58%，n = 85）是最常见症状，而疼痛、黄疸及可触及肿块的经典三联征罕见（4%，n = 6）。I型囊肿（63%，n = 93）最常见，其次是IV型（31%，n = 46）和卡罗利病（5.4%，n = 8）。14%（n = 21）的患者存在胰胆管异常汇合（APBJ），其中43%患有胰腺炎。总体而言，20%的患者（n = 29）发生胰腺炎，12%（n = 18）发现肾脏异常。75%（n = 110）的患者接受了手术干预，手术时的平均年龄为4.1岁。组织病理学检查显示3%的病例（n = 3）有化生改变。长期并发症包括慢性肝病（7%，n = 11）和门静脉高压（4%，n = 6）。结论 本研究突出了印度南部小儿胆总管囊肿的临床特征，强调早期诊断和手术干预，这可能减少并发症并改善预后。需要开展多中心研究以增进对疾病进展和预后的了解。
