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Introduction: Choledochal cysts in adults are commonly associated with hepatobHiary pathology and complications of previous cyst related procedures. Portal hypertension is a rare complication of choledochal cyst. The treatment of choledochal cyst complicated by portal hypertension has evolved from internal drainage of cysts to single stage excision of cyst with bHio-enteric anastomosis. Case Report: A is-year-old female presented with typical triad of abdominal pain, abdominal lump and jaundice. Magnetic resonance cholangiopancreatography (MRCP) was suggestive of type-I choledochal cyst with portal hypertension. An upper gastrointestinal endoscopy revealed grade 1 esophageal varices with proximal gastropathy. Intraoperatively, the posterior wall of the choledochal cyst was densely adherent to the portal vein and hence a partial excision of cyst with stripping of the mucosa of the posterior wall of the cyst along with Roux-en-Y hepaticojejunostomy was done. Conclusion: Single stage excision of choledochal cyst with bilio-enteric anastomosis is the treatment of choice of choledochal cyst with portal hypertension. Portal decompression is reserved for cases with extensive collaterals in the hepatoduodenal ligament.

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