Ehlers-Danlos syndrome during pregnancy: a case report and review of the literature.

Since Ehlers-Danlos syndrome encompasses a wide spectrum of disease and only has an estimated incidence of approximately 1:150,000 (Beighton, 1968a), there is a paucity of data from large studies on which to base firm conclusions about the risks of pregnancy in association with the syndrome. Most reports refer to women with the gravis (type I) and ecchymotic (type IV) forms, unlike pregnancy in association with the mitis (type II), which is rarely reported despite the fact that it constitutes 35 per cent of all cases of Ehlers-Danlos syndrome (Beighton, 1970). We suspect this is because complications with the mitis form are rare. Women with the two severe forms of the syndrome, mentioned above, risk hemorrhage, severe trauma at delivery, and preterm labor, but whether this is the case in the mitis form is unproved. Our patient's course was uneventful, delivery by cesarean section being undertaken because of her hip fixity. Tyson (1972) suggested that delivery by cesarean section was indicated in all cases of Ehlers-Danlos syndrome, but this pertains to the severe forms of the syndrome. The question of vaginal delivery in the mitis form remains unanswered. We would hope that in future reports of pregnancy in association with Ehlers-Danlos syndrome that patients are classified according to type so that prognosis, particularly in the mitis form, can be more accurately assessed.