Surgical failures: is it the surgeon or the patient? The all too often missed diagnosis of Ehlers-Danlos syndrome
Abstract
Ehlers-Danlos syndrome (EDS) is a closely related group of disorders caused by a heritable defect in collagen synthesis, which leads to marked healing difficulties. It has been estimated to occur in between one in 2500 and one in 5000 individuals but likely occurs more frequently than reported. EDS has probably been seen by all general surgeons several times over the course of a career. The purpose of this report is to describe the findings that should raise the index of suspicion, to aid in the diagnosis, and to characterize the general surgical procedures seen in patients with EDS by reviewing a single surgeon's experience in managing such patients with a review of the literature. Recommendations for treatment are given. A retrospective review of the experience of a single surgeon of 25 procedures in 15 patients with EDS is being reported. This is believed to be the largest series by one surgeon as yet reported. There was a wide variety of procedures performed, including ventral hernia repair (n = 6), inguinal hernia repair (n = 4), colectomy (n = 3), anal fistula (n = 3), and one each of an exploratory laparotomy, an appendectomy, a closure of a dehiscence, a Hickman catheter placement, an open lysis of adhesions for small bowel obstruction (SBO), a laparoscopic lysis of adhesions for SBO, an open cholecystectomy, a laparoscopic cholecystectomy, and an excision of a round ligament endometrioma. There was only one death, which was in a patient with Type IV EDS who was the…
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