Journal
SURGICAL CASE REPORTS
Volume 6, Issue 1, Pages -Publisher
SPRINGER
DOI: 10.1186/s40792-020-01051-0
Keywords
Ehlers– Danlos syndrome type IV; Sigmoid perforation; Negative pressure wound therapy; Enteroatmospheric fistula; Paediatric
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Background Ehlers-Danlos syndrome (EDS) type IV is a rare subtype of EDS, but has important surgical implications. Case presentation Here, we present a case of a spontaneous sigmoid perforation in a 14-year-old boy. He was initially treated with laparotomy, oversew of the sigmoid perforation and a diverting ileostomy. He developed a complete wound dehiscence and enteroatmospheric fistulae. These were managed with a combination of negative pressure wound therapy and Eakin (TG Eakin (TM)) pouch changes. We discuss the clinical features and presentation of EDS type IV, the surgical implications of managing patients with the condition, and the challenges in management of enteroatmospheric fistulae in children. Conclusions Ehlers-Danlos syndrome type IV should be considered as a cause of any spontaneous colonic perforation in children.
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