Peripartum considerations and challenges in the management of type IV Ehlers-Danlos syndrome

Abstract

Ehlers-Danlos syndrome (EDS) is an uncommon heterogenous autosomal dominant multisystemic connective tissue disorder, which may potentially present with life-threatening emergencies. The prevalence of EDS of all subtypes is from 1 in 10 000 to 1 in 20 000. Pregnancy in women with type IV EDS poses significant challenges, necessitating the care of multidisciplinary team. Potential complications of type IV EDS include arterial dissection, arterial aneurysm, visceral rupture, excessive bleeding, tissue fragility and delayed wound healing. We present an interesting case of a pregnant woman with type IV EDS who had previous cerebrovascular complications and had a successful delivery in our tertiary centre following a collaborative approach to her care.