Recurrent ventral herniation in Ehlers-Danlos syndrome.
Ehlers-Danlos syndrome is an inherited collagen disorder characterized by skin hyperextensibility, joint laxity, and tissue friability. In this study, it was hypothesized that Ehlers-Danlos syndrome is frequently undiagnosed in patients who present for repair of ventral abdominal wall hernias. A retrospective chart review was conducted, and patients who had presented for elective repair of recurrent abdominal wall herniation were identified. In all patients, one or more prior attempts at repair with either mesh or autologous tissues had failed. Patients in whom abdominal wall components were lost secondary to extirpation or trauma, patients who had required acute closure, and patients with less than 2 months of follow-up were excluded. Twenty patients met these criteria. Twenty cases of recurrent ventral hernia repairs were reviewed, with special attention to identification of the preoperative diagnosis of Ehlers-Danlos syndrome. Patients ranged in age from 29 to 75 years, with a mean age of 54 years. Five patients were male (25 percent), and 15 were female (75 percent). The majority (95 percent) were Caucasian. The most common initial procedures were gynecologic in origin (35 percent). A precise closure technique that minimizes recurrence after ventral hernia repairs was used. With use of this technique, there was only one recurrence over a follow-up period that ranged from 2 to 60 months (mean follow-up duration, 25.7 months). Two patients with Ehlers-Danlos syndrome were identified, and their cases are presented in this article. The "components separation" technique with primary component approximation and mesh overlay was used for defect closure in the two cases presented. The identification of these two patients suggests the possibility of underdiagnosis of Ehlers-Danlos syndrome among patients who undergo repeated ventral hernia repair and who have had previous adverse postoperative outcomes. There are no previous reports in the literature that address recurrent ventral abdominal herniation in patients with Ehlers-Danlos syndrome.
Girotto
J A
JA
Division of Plastic and Reconstructive Surgery, The Johns Hopkins School of Medicine, Baltimore, MD, USA. jgirotto@jhmi.edu
Malaisrie
S C
SC
Bulkely
G
G
Manson
P N
PN
D000328:Adult; D000368:Aged; D003937:Diagnosis, Differential; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D017558:Elective Surgical Procedures; D005260:Female; D005500:Follow-Up Studies; D018662:Fundoplication / Q000009:adverse effects; D006555:Hernia, Ventral / Q000209:etiology* / Q000601:surgery; D006801:Humans; D007044:Hysterectomy / Q000009:adverse effects; D010535:Laparoscopy / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012086:Reoperation; D012189:Retrospective Studies; D013526:Surgical Mesh; D013536:Suture Techniques; D014182:Transplantation, Autologous; D016896:Treatment Outcome