RAPUNZEL SYNDROME - AFRICAN VARIATIONS ON A EUROPEAN FAIRYTALE.

BACKGROUND

Trichobezoars are intraluminal accretions of ingested hair. Rapunzel syndrome is a rare and extreme presentation with extension down through into the small intestine. Most frequently reported in children and psychiatric patients, we report on an African series of five patients.

METHODS

Five patients presenting with trichobezoars were retrospectively reviewed and analysed with regard to patient background, demographics, clinical presentation, diagnosis, surgical management and complications.

RESULTS

Five female patients with a median age of 19 years (range 12-27 years) presented with a clinical spectrum including early satiety, intermittent vomiting with gastric outlet obstruction, abdominal pain and weight loss. Three patients were of Indian ethnicity and two of African ethnicity. Four highly functional individuals (three high school graduates/ students, one university student) and a 12 year old cerebral palsy child known with trichotillomania were diagnosed with trichobezoars following either endoscopy, abdominal CT scan, barium meal or plain abdominal radiography. Two patients presented with sealed/contained gastric perforations, and one with a small bowel perforation. All five bezoars extended into the jejunum, the longest of which measured 1.4 m, and two of which consisted entirely of artificial hair extensions. Various options for removal are described including chemical dissolution, endoscopy, laparoscopy and laparotomy. All five bezoars were removed by laparotomy. All recovered well and four were referred for psychiatric evaluation.

CONCLUSIONS

While trichobezoars are a rare entity, they may present with significant complications in the form of obstructions and perforations. In view of the risk for infection, and the considerable size of many of these bezoars, an open removal is probably safer than any minimally invasive attempt.