Internal carotid artery triple dissection and Ehlers-Danlos-like skin lesions related to pregnancy and childbirth: hormonal-implicated transient connective tissue disorder?

The association between cerebral ischemia and pregnancy has long been recognized. Furthermore, stroke related with pregnancy is characterized by significant morbidity and mortality. We report the case of a 35 years old woman presenting with acute left hemiplegia three weeks after caesarean section. Cerebral MRI demonstrated acute right frontoparietal ischemic stroke, while angiographic investigations provided acute occlusion of the proximal right internal carotid artery due to a dissection and two additional dissections of the proximal and distal contralateral internal carotid artery. The patient was not suffering from common cardiovascular (hypertension, diabetes mellitus, hyperlipidemia, and tobacco use) or pregnancy related risk factors like eclampsia or preeclampsia. Cardiac investigations were unremarkable. Furthermore coagulopathies have been excluded by an extensive laboratory testing. To rule out a connective-tissue disorder a skin biopsy was performed, which yielded lesions reminiscent of those found in some types of Ehlers-Danlos Syndrome. After ablactation oral anticoagulation with phenprocoumon was initiated and continued over 6 month. Two years later with complete recanalisation of the left ICA and persistent right-sided carotid-artery occlusion a second pregnancy was accomplished, a caesarean section was performed without any complications.

In the presented case of a triple dissection a skin biopsy demonstrated lesions that were reminiscent of those found in some types of Ehlers-Danlos Syndrome. Such changes are well known in pregnant women with preterm premature rupture of membranes and might represent a hormonal implicated transient connective tissue disorder which may lead to a general transient vasculopathy. Although the case of dissection relapse may be a rare event, we feel gestational guidance for patients already have been suffering from dissection during pregnancy to be extremely difficult. Our case is demonstrating that dissections even associated with connective tissue disorders in antecedent gravidity should not necessarily lead to a general rejection of further conception.