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.