Successful endovascular treatment of superior mesenteric artery-duodenal fistula secondary to Rapunzel syndrome

Abstract

Rapunzel syndrome is a rare clinical entity in pediatric patients with a history of trichotillomania and trichophagia that has only been mentioned a few times in the literature. It is characterized by abnormal gastric bezoar formation that sometimes extends to the duodenum, jejunum, or colon. Here, we present a case of a 16-year-old previously healthy female patient who had prolonged hospitalization due to complications related to a significant gastric bezoar that led to massive bleeding due to a superior mesenteric artery (SMA)-duodenal fistula successfully treated with stent graft placement. Undiagnosed trichobezoar can lead to rare and unexpected complications, such as SMA-duodenal fistula, with life-threatening hemorrhagic shock. Prompt activation of massive transfusion protocol and endovascular control of the hemorrhage was vital to successfully treating our patient.

Keywords

• Rapunzel Syndrome
• Sma-duodenal fistula
• Pediatric patients
• Trichobezoar
• Hemorrhagic shock
• Endovascular treatment