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

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.

Pigg, MarittaUppsala universitet,Medicinsk genetik (författare)
Kragsterman, BjörnUppsala universitet,Kärlkirurgi(Swepub:uu)bjkra123 (författare)
Bergqvist, DavidUppsala universitet,Kärlkirurgi(Swepub:uu)daviberg (författare)
Uppsala universitetKärlkirurgi (creator_code:org_t)

Ingår i:Gynecologic and Obstetric Investigation: S. Karger AG63:3, s. 173-1750378-73461423-002X

Av författaren/redakt...: Björck, Martin; Pigg, Maritta; Kragsterman, Bjö ...; Bergqvist, David

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