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Moyamoya syndrome in pregnancy – management of two consecutive pregnancies
  1. EH Shaw,
  2. RG Hayman
  1. Department of Obstetrics and Gynaecology, Cheltenham General Hospital, Cheltenham, UK


Moyamoya syndrome is a rare (1:2000000), progressive, vaso-occlusive disorder that affects the internal carotid arteries and their branches. It can present as a cerebral vascular accident (CVA) or intracranial haemorrhage. Pregnancy may increase the risk of these occurring particularly if pregnancy becomes complicated by hypertension. There are limited case reports of moyamoya syndrome during pregnancy due to the rarity of the condition.

The authors cared for a woman with moyamoya during 2 successful pregnancies. She had been diagnosed following a CVA in 1996 and required surgical treatment with encephalomyosynangiosis bypass surgery in 1996/7.

She was treated with aspirin throughout each pregnancy. Blood pressure was closely observed and aggressively managed. Her first pregnancy became complicated with pre-eclampsia, resulting in delivery at 34+5. During her second pregnancy mild hypertension developed at 19 weeks, which required titration of labetalol over subsequent weeks. At 34+4 weeks she developed severe headache and visual disturbance, but without proteinuria. The authors considered that she may be developing complications of her moyamoya so each pregnancy was delivered by caesarean to reduce the possible risk of intracranial haemorrhage. Combined spinal-epidural anaesthetic management was used, with very strict control of her blood pressure, and postpartum HDU care. Following each pregnancy she required a short duration of 4-6 weeks antihypertensive treatment.

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