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


METHODS
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.


CONCLUSIONS
Early recognition is crucial to avoid maternal mortality due to this genetic disorder. Once the condition is suspected, the clinical diagnosis is straightforward.
Björck
M
M
Department of Surgery, Section of Vascular Surgery, Uppsala University Hospital, Uppsala, Sweden. martin@bjorck.pp.se
Pigg
M
M
Kragsterman
B
B
Bergqvist
D
D
D000328:Adult; D036861:Delivery, Obstetric / Q000009:adverse effects*; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000235:genetics; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000235:genetics*; D012422:Rupture, Spontaneous; D013348:Subclavian Artery / Q000293:injuries*