Recurrent ventral herniation in Ehlers-Danlos syndrome

Ehlers-Danlos syndrome is an inherited collagen disorder characterized by s kin hyperextensibility,joint laxity, and tissue friability. In this study, it was hypothesized that Ehlers-Danlos syndrome is frequently undiagnosed i n patients who present for repair of ventral abdominal wall hernias. A retr ospective chart review was conducted, and patients who had presented for el ective repair of recurrent abdominal wall herniation were identified. In al l patients, one or more prior attempts at repair with either mesh or autolo gous tissues had failed. Patients in whom abdominal wall components were lo st secondary to extirpation or trauma, patients who had required acute clos ure, and patients with less than 2 months of follow-up were excluded. Twenty patients met these criteria. Twenty cases of recurrent ventral herni a repairs were reviewed, with special attention to identification of the pr eoperative diagnosis of Ehlers-Danlos syndrome. Patients ranged in age from 29 to 15 years, with a mean age of 54 years. Five patients were male (25 p ercent), and 15 were female (75 percent). The majority (95 percent) were Ca ucasian. The most common initial procedures were gynecologic in origin (35 percent). A precise closure technique that minimizes recurrence after ventr al 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 fo llow-up duration, 25.7 months). Two patients with Ehlers-Danlos syndrome were identified, and their cases a re presented in this article. The "components separation" technique with pr imary component approximation and mesh overlay was used for defect closure in the two cases presented. The identification of these two patients sugges ts the possibility of underdiagnosis of Ehlers-Danlos syndrome among patien ts who undergo repeated ventral hernia repair and who have had previous adv erse postoperative outcomes. There are no previous reports in the literature that address recurrent vent ral abdominal herniation in patients with Ehlers-Danlos syndrome.