Rapunzel Syndrome Complicated by Cholecystoduodenal Fistula Secondary to Biliary Compression: A Case Report of a Patient With Cerebral Palsy

TL;DR

A rare case of Rapunzel syndrome with a cholecystoduodenal fistula in a patient with cerebral palsy is reported, highlighting complex surgical challenges and outcomes.

Contribution

This case report presents a unique combination of Rapunzel syndrome and a cholecystoduodenal fistula in a patient with cerebral palsy.

Findings

A gastric and duodenal trichobezoar was identified along with a vesicoduodenal fistula.

The patient's condition mimicked type V Mirizzi syndrome due to gallbladder compression.

A Roux-en-Y hepaticojejunostomy was performed, but the patient died from nosocomial pneumonia.

Abstract

Rapunzel syndrome is a rare form of gastric trichobezoar that extends into the small intestine, leading to intestinal obstruction. Biliary-enteric fistulas are abnormal communications between the biliary system and the gastrointestinal tract, generally occurring spontaneously. We report the case of a 26-year-old female patient with a history of cerebral palsy who presented to the emergency department with an acute abdomen. Computed tomography revealed a mass occupying the stomach and intestine. A laparotomy was performed, identifying a gastric and duodenal trichobezoar complicated by an acute perforated gastric ulcer, in addition to a vesicoduodenal fistula secondary to extrinsic compression of the gallbladder. This fistula clinically mimicked a type V Mirizzi syndrome. A Roux-en-Y hepaticojejunostomy was carried out. The patient died from nosocomial pneumonia 30 days later. This case highlights the importance of a multidisciplinary approach in patients with underlying neurological conditions and underscores the surgical feasibility of hepaticojejunostomy in complex scenarios of secondary biliary involvement.

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