A CASE REPORT OF RECURRENT RAPUNZEL SYNDROME

Objectives: Bezoars are concrete masses found within the gastrointestinal tract that can lead to obstructions. One of the most common forms of bezoars is trichobezoar, composed of swallowed hair. Trichobezoars develop in 1% of people who have trichotillomania. Many bezoars are confined to the stomach, however, a small occurrence of Trichobezoars extends past the pylorus and into the duodenum, or small bowel, termed Rapunzel syndrome. Exploratory laparotomy is the treatment of choice for large bezoars. In the literature, there have been few cases of recurrent Rapunzel syndrome. Our case is a 13-year-old female with recurrent Rapunzel syndrome requiring three operative interventions.  

Case report  
A 13-year-old female with a history of sickle cell disease and recurrent Rapunzel syndrome requiring previous operative interventions presented to emergency department with back pain and presumed sickle cell crisis. On hospital day two, after developing epigastric abdominal pain and emesis, an abdominal x-ray was obtained that was concerning for possible obstruction and bezoar. A CT was then obtained for further evaluation and showed a small bowel obstruction with a long segment bezoar extending to the mid-small bowel, portal venous gas, and possible pneumatosis involving the stomach. Pediatric surgery was consulted. The patient was taken to the operating room for exploratory laparotomy with lysis of adhesions, repair of small bowel perforation, reduction of internal hernia, small bowel resection, and primary stapled anastomosis. Postoperatively the patient did well. During this admission, psychiatry was consulted for trichotillomania, and she was started on Luvox.  

Of note, at the age of 9, the patient presented for a clinic visit and was found to have a palpable abdominal mass. Further workup with ultrasound and CT abdomen and pelvis with contrast revealed a large trichobezoar extending into the jejunum. The following day the patient was taken for an upper endoscopy with the gastroenterology team, however they were not able to successfully remove the bezoar. The patient required an exploratory laparotomy with gastrostomy, and partial gastrectomy to remove the bezoar. At the age of 11 she presented to emergency department with a one-day complaint of nausea, vomiting, and abdominal pain. CT scan which showed small bowel loops within the central lower abdomen have abnormal contents concerning for bezoar with no evidence of obstruction. The patient was taken to the operating room for an exploratory laparotomy, and was noted to have obstructions in the mid ileum requiring multiple enterotomies to remove trichobezoar.

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