Choledochal cysts in children and adults with contrasting profiles: 11-year experience at a tertiary care center in Kashmir

Abstract

Background: Choledochal cyst is a surgical problem usually related to infancy and childhood. Despite advancements in management, a large number of cases still present during adulthood. The clinical course and outcome in children varies from that in adults. This study focuses on these variations in terms of presentation, management, histopathology, and outcome.

Methods: An in-depth retrospective analysis was carried out on 79 patients presenting with choledochal cysts between December 2007 and January 1997. The patients were segregated into two groups: group A comprising 32 children and group B with 47 adults. The presentation, clinical evaluation, radiologic, and biochemical findings; operative details; pathologic findings; and early and long-term complications in the two groups were studied on a comparative basis.

Results: The male/female ratios were 1:3 and 1.0:2.3 in groups A and B, respectively. A history of previous biliary surgery, pancreatitis, cholangitis, peroperative difficulties, and early and late postoperative complications were 5.1, 5.4, 6.4, 5.4, 2.0, and 3.3 times more common in group B than in group A. However, the classic triad of jaundice, abdominal pain, and a mass was 6.7 times more common in group A than in group B. The classic triad and cholangitis were the only parameters that were statistically significant. Rare presentations of spontaneous perforation of the cyst or cachexia manifested only in group A patients. The methods of detection and operative treatment were identical for both groups. Histologically, fibrosis of the cyst wall was a feature peculiar to group A, whereas signs of inflammation and hyperplasia were predominantly seen in group B. In the group B series, one patient had cholangiocarcinoma and another gallbladder carcinoma; one more patient developed malignancy during follow-up. Long-term complications were seen in 29.7% of patients in group B versus 9.3% in group A; the most rampant complication was a type IVa cyst, seen in 68.7% of patients.

Conclusions: Choledochal cysts present differently in adults and children; whereas children present with the classic triad, adults present with common biliary or infective complications. Although the methods of detection and surgical treatment are similar for the two groups, the type IVa cyst typically seen in the adult group creates a marked deviation with respect to long-term complications such as ascending cholangitis, anastomotic strictures, stone formation, and development of cholangiocarcinoma. These are areas of grave concern that can be addressed to a large extent by providing an access loop during the initial surgery especially for type IVa cysts. The glaring differences in terms of presentation, histologic picture, and outcome urges us to consider choledochal cysts in children as a separate entity.