Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death.
Ehlers-Danlos syndrome (EDS) is a rare connective tissue disorder known to be associated with complications during pregnancy. Recently, the syndrome has been subdivided into a number of types with different clinical manifestations. We report a maternal death with type IV, and review the literature for obstetric complications of the syndrome by type. A very high risk of maternal death with type IV is identified. Recommendations for managing pregnancies complicated by EDS are given.
D000328:Adult; D000740:Anemia / Q000209:etiology; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D006473:Postpartum Hemorrhage / Q000150:complications / Q000209:etiology*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000209:etiology; D012077:Renal Artery; D012422:Rupture, Spontaneous