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Gynecologic and Surgical Complications in Type IV Ehlers-Danlos Syndrome

Wheeler, Sarahn M. MD; Russo, Melissa MD; Wilson-Murphy, Molly; Shen, Wen MD

Obstetrics & Gynecology:
doi: 10.1097/AOG.0000000000000001
Case Report
Abstract

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.

In Brief

The decision to proceed with surgery should be weighed against tissue fragility in patients with type IV Ehlers Danlos Syndrome.

Author Information

Departments of Obstetrics and Gynecology and Genetics and the School of Medicine, Johns Hopkins School of Medicine, Baltimore, Maryland.

Corresponding author: Sarahn Wheeler, MD, Department of Obstetrics and Gynecology, Johns Hopkins School of Medicine, 600 North Wolfe Street, Phipps 249, Baltimore, MD 21287; e-mail: wheelersarahn@gmail.com.

Financial Disclosure The authors did not report any potential conflicts of interest.

© 2014 by The American College of Obstetricians and Gynecologists.