Rapid Development of High-Grade Dural Arteriovenous Fistula During Pregnancy: A Case Report
Nazanin Sheibani1, Barbara Voetsch2, Emanuele Orru3, David Lerner5, Collin Culbertson4
1Tufts Medical Center, 2Division of Vascular Neurolog, 3Neurointerventional Radiology, 4Division of Vascular Neurology, Lahey Hospital & Medical Center, 5Department of Neurology, Division of Neurocritical Care, Brookdale University Hospital and Medical Center
Objective:

We present a case of minimally symptomatic, radiographically occult dural arteriovenous fistula (dAVF) evolving into intracerebral hemorrhage shortly after pregnancy.

Background:
dAVFs are generally acquired arteriovenous shunts thought to be caused by pathological neo-arterialization within an occluded vein. While a relatively common entity, rapid dAVF development has rarely been described in the setting of pregnancy. 
Design/Methods:
Case report.
Results:

A 30-year-old nulliparous woman was referred to our outpatient clinic following subacute onset of left-predominant pulsatile tinnitus and mild bifrontal headache. Brain magnetic resonance imaging (MRI) revealed diffuse evidence of intracranial hypertension, which was supported by elevated opening pressure on lumbar puncture (LP). MR angiography and venography were within normal limits. She was diagnosed with idiopathic intracranial hypertension and started on acetazolamide with improvement in symptoms. At 6-month follow-up, she was seven weeks pregnant and reported mild worsening of tinnitus

After discontinuation of acetazolamide. She remained clinically stable until three weeks after an uneventful spontaneous vaginal delivery when she presented with thunderclap headache, confusion, and aphasia. Head computed tomography (CT) was significant for a 4 mL acute frontotemporal intracerebral hemorrhage. CT angiography demonstrated a large vascular malformation of the left sphenoid wing. Cerebral angiogram confirmed a dAVF fed by the left inferolateral trunk, middle meningeal arteries, and arteries of the foramina spinosum, ovale, and rotundum, and draining into enlarged cortical veins. The fistula was successfully obliterated transarterially with a liquid embolic system. Three months following embolization, she has made complete neurological recovery with resolution of tinnitus.

Conclusions:
Radiographically occult dAVF can emerge and become symptomatic during pregnancy and the post-partum period. Potential mechanisms include systemic changes in hormone levels, shifting hemodynamics, and local phenomena of thrombosis, hypoxia, and secondary angiogenesis. Neurologists should consider this entity when evaluating women with new headache or focal symptoms in the peripartum setting.
10.1212/WNL.0000000000203313