Moyamoya disease in pregnancy and delivery planning: A case series and literature review

Abstract

Moyamoya disease is a rare condition characterized by stenosis or occlusion of the intracranial internal carotid arteries leading to the development of fragile collateral vessels. Disruption of these vessels can lead to both ischemic and hemorrhagic strokes. As such, these patients are sensitive to changes in intracranial pressure and pose a challenge in pregnancy and delivery planning. Two cases of a parturients with moyamoya disease are presented, and the literature regarding safe method of delivery in similar patients is reviewed. The available evidence suggests that adequate anesthesia, and maintenance of hemodynamic status, takes precedence over the exact method of delivery in these patients. Cesarean delivery under epidural anesthesia can be a safe option for parturients with moyamoya disease, but an uncomplicated vaginal delivery, most often assisted with either vacuum or forceps, has also been frequently reported.

Postpartum Seizure and Subarachnoid Haemorrhage Secondary to Moyamoya Disease

“…Management of Moyamoya disease includes medical treatment for hypertension, vasodilators, anticonvulsants, surgical treatment involving revascularisation surgery, or a combination of these [2,5]. A recent case series has reported that pregnancy-related stroke secondary to Moyamoya disease is related to gestational age, with the highest risk of intracranial haemorrhage occurring antenatally, particular at or greater than 24 weeks [6].…”

“…There is no standard for method of delivery in patients with Moyamoya disease [2]. The aim is to maintain haemodynamic stability by controlling blood pressure, managing pain to minimise hyperventilation causing hypocapnia and cerebral vasoconstriction, and careful fluid management [2,5]. Caesarean section with epidural anaesthetic can be safe, as can an uncomplicated instrumental vaginal delivery [2,5].…”

“…Caesarean section with epidural anaesthetic can be safe, as can an uncomplicated instrumental vaginal delivery [2,5]. Avoidance of valsalva manoeuvres is recommended [5].…”