BACKGROUND: Although type IV Ehlers-Danlos syndrome is a rare condition, it is critical that physicians be aware of this disease because it presents unique management challenges. CASE: We present a young woman who was admitted with a pelvic abscess after having a miscarriage and an appendiceal perforation. She had an exploratory laparotomy secondary to a persistent pelvic abscess and endured multiple postoperative complications. Based on her family history and physical characteristics, type IV Ehlers-Danlos syndrome was diagnosed. CONCLUSION: The decision to proceed with surgery should be weighed against tissue fragility in patients with type IV Ehlers-Danlos syndrome. A conservative, multidisciplinary team approach addresses whether to proceed with surgery and minimize postoperative complications if surgery is performed.
BACKGROUND: Although type IV Ehlers-Danlos syndrome is a rare condition, it is critical that physicians be aware of this disease because it presents unique management challenges. CASE: We present a young woman who was admitted with a pelvic abscess after having a miscarriage and an appendiceal perforation. She had an exploratory laparotomy secondary to a persistent pelvic abscess and endured multiple postoperative complications. Based on her family history and physical characteristics, type IV Ehlers-Danlos syndrome was diagnosed. CONCLUSION: The decision to proceed with surgery should be weighed against tissue fragility in patients with type IV Ehlers-Danlos syndrome. A conservative, multidisciplinary team approach addresses whether to proceed with surgery and minimize postoperative complications if surgery is performed.
Authors: Gustavo S Oderich; Jean M Panneton; Thomas C Bower; Noralane M Lindor; Kenneth J Cherry; Audra A Noel; Manju Kalra; Timothy Sullivan; Peter Gloviczki Journal: J Vasc Surg Date: 2005-07 Impact factor: 4.268