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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™) 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.

Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child