Ehlers Danlos Syndrome Type IV and pregnancy

A case report is presented of a 23-year-old patient who was diagnosed with Ehlers Danlos syndrome (EDS) Type IV (vascular type) in the 23rd week of her second pregnancy. EDS Type IV has one of the highest mortality rates for pregnant women of any condition with significant morbidity if the mother survives.

Current literature is presented and supports the necessity for close monitoring of mother and child in a specialist unit with involvement of a multi-disciplinary team. There is no agreed consensus on the mode or timing of delivery but recent literature is supportive of delivery by caesarean section at 32 weeks.

This case report demonstrates a successful outcome for both mother and child with a planned delivery at 34 weeks by caesarean section and emphasises the importance of strategic planning for complicated deliveries and the effectiveness of good communication networks. Patients with EDS Type IV should be counselled about the potential risks for both themselves and the child to enable them to make informed decisions about their obstetric care.