Case Report
Year: 2018 | Month: January | Volume: 3 | Issue: 1 | Pages: 5-10
Pregnancy-Related Death in a Woman with Vascular Ehlers-Danlos Syndrome; Dissection and Rupture of Internal Carotid Artery Aneurysm Accompanied by Preeclampsia
Maasoumeh Saleh1, Maasoumeh Mirzamoradi1, Soraya Saleh Gargari1, Maasoumeh Panahi2
1Department of Gynecology and Obstetrics, Mahdiyeh Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
2Midwife - Department of Gynecology and Obstetrics, Vali-Asr Hospital,Shiraz University of Medical Sciences
Corresponding Author: Maasoumeh Saleh
ABSTRACT
Ehlers-Danlos syndrome is a rare inherited disorder of connective tissue, characterized by a collagen synthesis defect. Vascular type is the most serious type. Dissections of the vertebral and carotid arteries in their extra- and intra-cranial segments are typical. Pain is the initial symptom of a spontaneous internal carotid artery dissection presenting to a physician. Headache (including neck and facial pain) is usually described as constant and severe and is commonly ipsilateral to the dissected artery. Failure to consider the diagnosis in young patients presenting with neurologic symptoms is a potential medico-legal pitfall. We describe a 37 year old pregnant woman with undiagnosed Ehlers-Danlos syndrome type IV, presenting with dissection and rupture of extra-cranial segment of internal carotid aneurysm associated with preeclampsia in 33 weeks of gestation.
Key words: Ehlers-Danlos syndrome, Carotid artery, Dissection, Pregnancy