Trichobezoar presenting with the 'comma sign' in Rapunzel Syndrome: a case report and literature review

Abstract

Introduction: Rapunzel Syndrome is an uncommon presentation of trichobezoar, involving strands of swallowed hair extending as a tail through the small intestine, beyond the stomach. This was first described in 1968 and until 2003 only thirteen cases have been reported.

Case presentation: A 55-year old man of East Indian descent presented to the surgical team via the emergency department in Trinidad, West Indies, with an acute abdomen and small bowel obstruction. He had a recent psychiatric history and patchy alopecia as well as a family history of schizophrenia. A supine abdominal radiograph revealed a small bowel obstruction as well as an unusual air shadow in the left hypochondrium in the region of the duodeno-jejunal flexure associated with the stomach bubble, which resembled a 'comma'. At laparotomy, the stomach and third part of the duodenum were distended and contained a large mass of dark, foul-smelling hair that occupied the stomach, crossed the pylorus and extended into the small bowel. A retroperitoneal perforation of the third part of duodenum was found and repaired. The large trichobezoar was removed via a gastro-enterotomy and the patient made a slow, but complete recovery.

Conclusion: We wish to report another case of Rapunzel Syndrome and describe an unusual radiologic sign associated with a retroperitoneal perforation of the third part of duodenum - the comma sign. To the best of our knowledge this is the first reported case of a spontaneous retroperitoneal perforation of the third part of duodenum associated with Rapunzel Syndrome.

Figure 1

Abdominal radiograph of the 'comma sign' in our patient.

Figure 2

Barium meal showing filling defect due to trichobezoar. .

Figure 3

CT image of trichobezoar. [K M. Vitellas, K Vaswani and W F. Bennett: Small-Bowel Bezoar AJR 2000; 175:876–878].