Rapunzel syndrome associated with trichotillomania and pica causing bowel obstruction in a 4 year-old-female: A rare case report from Nepal.

INTRODUCTION

Bezoars are an accumulation of human or vegetable fibers in the gastrointestinal tract which if extend to the small intestine are referred to as Rapunzel syndrome. They are primarily a psychiatric issue rather than a surgical condition and are associated with trichotillomania, with subsequent trichophagia, pica, and other psychiatric conditions usually seen in adolescent females. This case report describes an extremely rare diagnosis of Rapunzel syndrome in a 4-year-old female.

CASE PRESENTATION

A 4-year-old female presented with pain and a lump over the epigastric region for 1 week associated with an episode of vomiting, early satiety, and a history of ingestion of hair. There was a 4X4cm non-tender, firm, mobile, intraabdominal lump on examination. CT showed a moderately distended stomach with ill-defined heterogenous content extending up to the duodenum and suggesting bezoars. An open gastrotomy was performed to remove bezoars.

DISCUSSION

If bezoars extend beyond the pylorus to the small intestine, it is referred to as Rapunzel syndrome. Clinical features of RS include epigastric pain, epigastric lump, nausea and vomiting, early satiety, loss of appetite, halitosis, weight loss, and constipation. CT scan is the key to diagnosis and surgical intervention via open or laparoscopic approach is required. Psychiatric evaluation is essential for long-term management and prevents its recurrence.

CONCLUSION

Bezoars are a rare cause of intraluminal intestinal obstruction and diagnosis should be considered if associated trichotillomania and pica are present in a paediatric patient who presents with abdominal pain, vomiting, early satiety, and weight loss.