Fatal bleeding following delivery : a manifestation of the vascular type of Ehlers-Danlos' syndrome

• Introduction: The vascular form of Ehlers-Danlos' syndrome (type IV) is a potentially lethal genetic condition because of rupture of major arteries, often in the peri-partum period. Case Report: We report a 31-year-old primipara who died from a rupture of the right subclavian artery. The patient had several symptoms and signs typical of the disease. The rupture occurred during the expulsion-phase of delivery but was recognized only on day 9. Conclusion: Early recognition is crucial to avoid maternal mortality due to this genetic disorder. Once the condition is suspected, the clinical diagnosis is straightforward.

Nyckelord

Ehlers-Danlos' syndrome

Subclavian artery

Delivery

Bleeding

Rupture

MEDICINE

MEDICIN

Publikations- och innehållstyp

ref (ämneskategori)

art (ämneskategori)