Surgical pitfalls in patients with Ehlers-Danlos type IV: a case of spontaneous sigmoid perforation in a 17-year-old male.
Ehlers-Danlos syndrome (EDS) is a group of well described connective tissue disorders in which collagen production is impaired. The surgical management of affected individuals remains challenging, with no general consensus. We report a case of spontaneous sigmoid perforation in a 17-year-old Eurasian male, in whom we subsequently established the diagnosis of EDS type IV (EDS-IV). We review the literature to discuss the clinical features and diagnosis, and the recommended therapeutic management.
Division of Colorectal Surgery, University Surgical Cluster, National University Health System, Singapore. ngkailyn@gmail.com
Kai Lyn
KL
Ng
Wai Kit
WK
Cheong
D000293:Adolescent; D003082:Colectomy; D003125:Colostomy; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007416:Intestinal Perforation / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D008297:Male; D012810:Sigmoid Diseases / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery