Rapunzel syndrome: how to orient the diagnosis
Rapunzel syndrome is a rare form of tricobezoar with a tail extending from the stomach into the small bowel; surgical removal is generally required. About 60 cases have been reported and described in the literature since 1968. We present the case of an 8-year-old girl who, during the course of a gen...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2018-06-01
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| Series: | Pediatric Reports |
| Subjects: | |
| Online Access: | https://www.pagepress.org/journals/index.php/pr/article/view/7689 |
| Summary: | Rapunzel syndrome is a rare form of tricobezoar with a tail extending from the stomach into the small bowel; surgical removal is generally required. About 60 cases have been reported and described in the literature since 1968. We present the case of an 8-year-old girl who, during the course of a genetic consultation, was initially assigned with a clinical suspicion of ectodermal dysplasia. Surgical intervention, which resulted in the extraction of a tricobezoar of enormous size and extension, led us to the diagnosis of Rapunzel syndrome. The possibility of a tricobezoar should be considered in all cases of adolescent patients who present signs of intestinal occlusion or sub-occlusion, suffer from psychiatric disorders, and have a history of trichotillomania. Endoscopic methodology, medical imaging and clinical diagnostics are fundamental for a differential diagnosis. Psychiatric follow-up is advised to prevent recurrence. |
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| ISSN: | 2036-749X 2036-7503 |