Ehlers-Danlos type IV in pregnancy. A case report and a literature review

Abstract

Background: Ehlers-Danlos syndrome is a heterogeneous group of connective tissue disorders, characterized by a defect in the synthesis of collagen. The syndrome is subdivided into different clinical subtypes, the most hazardous of which is type IV, the vascular type. It can manifest itself in various complications such as rupture of arteries and hollow organs. The obstetrical manifestations are the risk of uterine rupture during labor, damage to the vagina and perineum, bleeding and rupture of blood vessels and colon during the puerperium.

Case report: We describe a primigravida suffering from Ehlers-Danlos type IV who was followed and successfully delivered by a cesarean section at 36 weeks of gestation and made an uneventful recovery.

Conclusion: Following a thorough literature review, it seems wise to perform early delivery by cesarean section for pregnant women suffering from Ehlers-Danlos type IV.