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

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 d...

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Bibliographic Details
Published in:Surgical case reports 2020-12, Vol.6 (1), p.312-312, Article 312
Main Authors: Kodikara, Hemal, King, Sebastian K., McLeod, Elizabeth
Format: Article
Language:English
Subjects:
Case Report
Case reports
Ehlers–Danlos syndrome type IV
Enteroatmospheric fistula
Medicine
Medicine & Public Health
Negative pressure wound therapy
Ostomy
Paediatric
Sigmoid perforation
Surgery
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Summary: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.
ISSN:2198-7793
2198-7793
DOI:10.1186/s40792-020-01051-0