Recurrent Rapunzel syndrome - A rare tale of a hairy tail.

BACKGROUND

Rapunzel syndrome is a rare condition predominantly found in trichophagic females that occurs when gastric trichobezoar extends beyond the pylorus and into duodenum, jejunum, ileum or even to colon. There are only five cases reported in literature. This case report has been reported in line with the SCARE criteria.

METHODS

A 19 year old female, with a history of trichotillomania with trichophagia, who presented with epigastric pain and vomiting for 12 months. She underwent laparotomy ten years back to extract a trichobezoar, which was diagnosed to be Rapunzel syndrome and lack of follow-up resulted in recurrence. Further examination and imaging showed a large trichobezoar extending into small intestine. She underwent gastrotomy and entrerotomy and extraction of hairball.

CONCLUSIONS

In a trichophagic female with abdominal pain, nausea and vomiting, and abdominal mass, diagnosis is done by ultrasound, CT scan and upper gastrointestinal endoscopy. Small trichobezoars can be removed endoscopically. Large (>20 cms)acutely symptomatic trichobezoars, especially when accompanied by Rapunzel syndrome, must be removed surgically by gastrotomy and enterotomy is required if impacted extension is present. Long-term surgical and psychiatric follow-up with psychotherapy and cognitive behavioural therapy along with selective serotonin reuptake inhibitors are critical to prevent recurrences.

CONCLUSIONS

Although Rapunzel syndrome is an extremely rare disease, it should be considered when diagnosing young women, especially those with a history of trichophagia or trichotillomania. After the surgical removal of trichobezoars by laparotomy, long-term follow-up combined with psychiatric consultation is recommended to prevent recurrences.