Vascular Ehlers-Danlos Syndrome (previously Ehlers-Danlos IV) is a rare autosomal dominant collagen vascular disorder caused by a 2q31 COL3A1 gene mutation encoding pro-alpha1 chain of type III collagen (in contrast to classic Ehlers-Danlos, caused by a COL5A1 mutation). The vascular type accounts for less than 4% of all Ehlers-Danlos cases and usually has a poor prognosis due to life threatening vascular ruptures and difficult, frequently unsuccessful surgical and vascular interventions. In 70% of cases, vascular rupture or dissection, gastrointestinal perforation, or organ rupture is a presenting sign. We present a case of genetically proven vascular Ehlers-Danlos with fatal recurrent retroperitoneal hemorrhages secondary to a ruptured right common iliac artery dissection in a 30-year-old male. This case highlights the need to suspect collagen vascular disorders when a young adult presents with unexplained retroperitoneal hemorrhage, even without family history of such diseases.
Recent controversies surrounding obstetrics and gynecology devices, including a permanent sterilization device, pelvic meshes, and laparoscopic morcellators, highlight the need for deeper understanding of obstetrics and gynecology medical device regulation. The U.S. Food and Drug Administration premarket approval database was queried for approvals assigned to the obstetrics and gynecology advisory committee from January 2000 to December 2015. Eighteen device approvals occurred in the time period studied. The most common clinical indications included endometrial ablation (33%), contraception (28%), and fetal monitoring (17%). The median approval time was 290 days (range 178-1,399 days). Regarding the pivotal trials leading to approval, there were 11 randomized controlled trials, one randomized crossover study, five nonrandomized prospective studies, and two human factor studies. Fourteen devices (78%) met their primary clinical efficacy endpoint. Only 12 of 18 devices were required to conduct postmarket surveillance. A significant proportion of devices (42%) were approved on the basis of nonrandomized controlled trials. Three devices have been withdrawn after approval, all of which were either not referred or not recommended for approval by the obstetrics and gynecology advisory committee. Of the three devices withdrawn from the market, two failed to demonstrate clinical benefit in their pivotal trials. One device was not required to undergo postmarketing surveillance and was subsequently withdrawn as a result of patient safety concerns. Our results reveal significant weaknesses in the preapproval and postapproval regulation of high-risk obstetrics and gynecology devices. Greater specialty group involvement is necessary to ensure the development of safe and clinically effective devices.
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