WOFAPS 2025 8th World Congress of Pediatric Surgery

Ashley Ramirez ¹, Li-Horng Lee ², Francisca Velcek ¹

¹ Department of General Surgery, Division of Pediatric Surgery, State University of New York Downstate Health Sciences University
² Garden State Pediatric Surgery

Introduction:

Rapunzel syndrome is a rare subset of trichobezoar in which a long tail of hair can extend into the small intestine. Complications associated with this syndrome include cholestasis, obstruction, and even rarer still, pancreatitis. In this case report, we present a 15-year-old girl who presents with pancreatitis secondary to Rapunzel syndrome.

Case presentation:

A 15-year-old girl with past medical history including iron deficiency anemia and alopecia presented with abdominal pain and bilious emesis. Laboratory testing at admission revealed elevated lipase and amylase. Subsequent imaging including magnetic resonance cholangiopancreatography (MRCP) revealed bezoar-like material within the stomach and a mass anterior to the duodenum suggestive of mechanical obstruction with impaired pancreatic and biliary outflow. The patient was taken for exploratory laparotomy which revealed a palpably firm and irregular pancreas consistent with pseudocyst formation. Surgical removal of a large gastric trichobezoar (removed via gastrotomy) and a second trichobezoar (via jejunal enterotomy) was accomplished, in addition to cholecystectomy and feeding jejunostomy tube placement. Postoperatively the patient recovered well with normalization of pancreatic enzymes. The patient was further referred to outpatient psychiatric follow-up for anxiety-related trichotillomania and trichophagia discovered on psychiatric evaluation.

Conclusion:

This case report demonstrates both an extremely rare entity as well as an extremely rare complication. When encountering a patient with history of unexplained iron-deficiency anemia and alopecia who presents with biliary or pancreatic ductal dilatation, it is critical to consider bezoars as a possible source. The timeliness of surgical intervention is key to a successful outcome as was accomplished in this case. As the presenting symptoms can be vague or possibly misleading, this case emphasizes the importance of a high index of suspicion when encountering similar patients.