Rapunzel syndrome leading to multiple bowel perforations: A case report

Abstract

Introduction

Although patients with Rapunzel syndrome are often asymptomatic, rare complications such as isolated gastric or duodenal perforation, pancreaticobiliary or intestinal obstruction, and intussusception have been described.

Case presentation

A 5-year-old female with no known medical history presented after nearly two weeks of worsening constipation, abdominal distension, and abdominal pain. A CT scan demonstrated a large gastric bezoar, pneumoperitoneum, and large volume complex free fluid. The patient was taken to the operating room for an abdominal exploration. We found a large gastric trichobezoar with hair extending into the small bowel, consistent with Rapunzel syndrome. The gastric trichobezoar was connected to a mid-ileal trichobezoar through interdigitating hair fibers. At multiple locations along the small bowel, garroted linear perforations had occurred due to tension created by the intestinal peristalsis along the interconnecting hair fibers, creating mucosal ulcerations and transmural pressure necrosis that required an extensive small bowel resection. Ultimately, we did one end-end small bowel anastomosis and placed a gastrostomy tube. Gastrostomy tube feeds were started on postoperative day 6 after confirming with a contrast study that there were no intestinal leaks. She developed a superficial wound infection that was managed with antibiotics and negative pressure wound therapy. She was discharged tolerating regular diet on post-operative day 23, and is undergoing psychiatric therapy.

Conclusion

Although rare, Rapunzel syndrome can lead to severe complications. When two trichobezoars are present, interconnecting hair fibers can cause small bowel perforations.