Non-cirrhotic portal hypertension secondary to cholangiointestinal anastomotic stricture after choledochal cyst excision: a case report

Abstract

BackgroundCystectomy accompanied by biliary system reconstruction is an important treatment option for choledochal cysts, but the risk of post-operative complications is high. The most famous long-term complication is anastomotic stricture, whereas non-cirrhotic portal hypertension secondary to cholangiointestinal anastomotic stricture is rare.Case summaryHere we report the case of a 33-year-old female patient with a type I choledochal cyst who underwent choledochal cyst excision with Roux-en-Y hepaticojejunostomy. Thirteen years later, the patient presented with severe esophageal and gastric variceal bleeding, splenomegaly, and hypersplenism. Furthermore, cholangiointestinal anastomotic stricture with cholangiectasis was identified on imaging. A pathological examination of the liver suggested intrahepatic cholestasis, but the fibrosis was mild and inconsistent with severe portal hypertension. Therefore, the final diagnosis was portal hypertension secondary to a cholangiointestinal anastomotic stricture after choledochal cyst surgery. Fortunately, the patient recovered well after endoscopic treatment and dilated cholangiointestinal anastomotic stricture.ConclusionCholedochal cyst excision with Roux-en-Y hepaticojejunostomy is the recommended standard of care for type I choledochal cysts; however, the long-term risk of cholangiointestinal anastomotic stricture requires consideration. Moreover, cholangiointestinal anastomotic stricture can lead to portal hypertension, and the degree of elevated portal pressure may be inconsistent with the degree of intrahepatic fibrosis.

Association of TP53 Germline Variant and Choledochal Cyst among Clinically Diagnosed Filipino Pediatric Patients

“…55 Portal hypertension is the most common complication among the cases in this study but is rare in other populations. 3,56 It occurs secondary to biliary cirrhosis, as seen in 8 out of 14 patients (57.14%) with portal hypertension who also had liver cirrhosis. [56][57][58][59] Other possible etiologies for portal hypertension include compression or obstruction of the portal vein and extrahepatic portal vein thrombosis.…”

“…3,56 It occurs secondary to biliary cirrhosis, as seen in 8 out of 14 patients (57.14%) with portal hypertension who also had liver cirrhosis. [56][57][58][59] Other possible etiologies for portal hypertension include compression or obstruction of the portal vein and extrahepatic portal vein thrombosis. [56][57][58] Cholangitis is another common complication found in the study population that is also seen in 30% of cases in other populations.…”

“…[56][57][58][59] Other possible etiologies for portal hypertension include compression or obstruction of the portal vein and extrahepatic portal vein thrombosis. [56][57][58] Cholangitis is another common complication found in the study population that is also seen in 30% of cases in other populations. 55,57,59 Several studies observed cholangitis to be predominant in Type I and Type IVA cysts, which are the most common types in the study population.…”