Rupture of the external iliac artery during pregnancy: a case of type IV Ehlers-Danlos syndrome.
Ehlers-Danlos Syndrome is a group of connective tissue disorders involving defects in collagen synthesis. Ten different types have been described. The most lethal of these is type IV, with up to 25% maternal mortality described. The defect in type III collagen can lead to rupture of arteries, bowel, or uterus. We present a maternal mortality that occurred in a patient not previously known to have Ehlers-Danlos syndrome. She presented at 29 weeks gestational age with a ruptured external iliac artery. Further complications, including jejunal rupture and aortic disruption ultimately led to her demise. Postmortem examination ultimately confirmed the diagnosis of type IV Ehlers-Danlos syndrome. Although type IV Ehlers-Danlos syndrome is rare, it carries a high risk for maternal morbidity and mortality. Therefore, it is important to be aware of the diagnosis prior to pregnancy and to have a high index of suspicion for arterial or viscus perforation in the face of acute abdominal findings.
Brees
C K
CK
Department of Obstetrics and Gynecology, University of Louisville, KY, USA.
Gall
S A
SA
D000328:Adult; D004535:Ehlers-Danlos Syndrome / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007083:Iliac Artery / Q000601:surgery; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000175:diagnosis* / Q000601:surgery; D012422:Rupture, Spontaneous; D014652:Vascular Diseases / Q000209:etiology