ABSTRACT

The occurrence of pregnancy in females with Ehlers Danlos syndrome is not common. This is a rare inherited connective tissue disorder that has been associated with numerous pregnancy complications ranging from bruising to maternal death. More than 10 varieties of Ehlers Danlos syndrome have been identified based upon either biochemical defects or clinical findings. Various major and minor criteria have been defined for each type. Type I and type II are variants of classical Ehlers Danlos syndrome, with type II a less severe one. Pregnancy in association with type II is rarely reported, despite the fact that it constitutes 35 percent of all cases. This is due to the fact that it is a milder form and complications in pregnancy rarely occur. A case of pregnancy in a 29 year old, second gravida with Ehlers Danlos syndrome(Beighton score 5/9) is reported here. The patient developed non-immune hydrops and intrauterine fetal death at 34 weeks pregnancy. Her previous delivery was by cesarean section.