Pregnancy With Moyamoya Disease

Author Information

Dalvi P*, Parulekar S V**
(* Third Year Resident, ** Professor and Head, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)


Moyamoya disease  is a progressive cerebrovascular disease characterized  by bilateral occlusion of the arteries supplying the circle of Willis and resulting in a prominent arterial collateral circulation, the vessels of which are fragile. The disease is characterized by thickening of the intima in the walls of the internal carotid arteries bilaterally. Intraventricular, subarachnoid, and intracerebral hemorrhage are known to occur in these patients. We present a case of a woman with Moyamoya disease  who underwent an emergency lower segment cesarean section. 


The meaning of Japanese word “Moyamoya” is hazy or like “a puff of smoke in the air.” The term was originally used to describe the angiographic appearance of the affected vascular collateral network.[1] 
The exact etiology of the disease is not known, but the incidence is higher in the Japanese and other Asian people. A familial inheritance occurs in around 10 percent of cases.[2] It is twice as often seen in women as in men. A considerable number of women with Moyamoya disease may be in the child bearing age group. Clinical features include cerebrovascular events such as transient ischemic attacks (50-75%), hemorrhagic stroke (10-40%), and infarction. Diagnosis is made by demonstration of stenotic or occlusive lesions in the distal internal carotid arteries and in the arteries proximal to the circle of Willis with vascular imaging with magnetic resonance (MR), computerized tomographic (CT), or conventional cerebral angiography.

Case Report

A 35 year old woman,  married for 1.5 years, second gravida with one prior stillbirth was referred to us at 39 weeks of gestation for breech presentation and two loops of cord around the neck on ultrasonography. She was a known of Moyamoya disease diagnosed at the time of her previous pregnancy on CT angioangiography . She had history of  severe preeclampsia in her first pregnancy which had to be terminated at 6 months’ gestation for maternal indications.  Her previous pregnancy had been further complicated by intracerebral frontoparietal hemorrhage which had been managed conservatively by the neurosurgery department.   There was no history of any other major medical or surgical illness. She was on tablet labetalol 100 mg twice a day for three months. She had also been taking tablet aspirin 75 mg until 3 months ago. There was no history of any bowel or urinary bladder related symptoms. Her clinical examination revealed a pulse of 90 beats per minute, blood pressure was 100/80 mm of Hg, no pallor no edema. The uterus was full term, the fetus in a breech presentation, fetal heart rate 140 per minute, regular. General and systemic examination revealed no abnormality. The uterus was relaxed. There was no bleeding or leaking per vaginum. The cervix was 2.5 cm dilated, 40% effaced, station was -1, membranes intact and the pelvis was adequate. Her hematological and biochemical investigations for fitness for anesthesia were normal. Neurosurgeons found her to fit for undergoing surgery. A decision for an emergency lower segment caesarean section (LSCS) was taken in view of breech presentation of the fetus.  LSCS was done under general anesthesia and a female child of 2.6 kg with Apgar 9/10 at 1 and 5 minutes was delivered. Intraoperative and postoperative course was uneventful. The patient made an uneventful recovery.


Moyamoya disease has no definitive treatment other than palliation. Individuals experiencing TIAs and strokes are prescribed aspirin, vasodilators, or anticoagulants to reduce the risks of future recurrent neurological sequelae.[3]. A large number of cases in literature have initially presented as an intracranial hemorrhage with neurological dysfunction.[4,5,6] The control of blood pressure is of utmost importance. Risk of thrombosis is also increased in pregnancy; thus, aspirin may even be more essential during this period. Maintenance of hemodynamic stability during pregnancy is important in avoiding neurological events. It depends on adequacy of analgesia, maintaining fluid balance, avoiding fluctuations in blood pressure, and reducing Valsalva's maneuver. Many neurosurgeons believe that vaginal delivery has more risk than cesarean section without data supporting the same. To avoid problems at delivery, scheduled cesarean section is preferred in by some workers for fear of litigation. 
The preferred mode of delivery remains unclear. LSCS was chosen in the majority of previously reported cases. This has been suggested to better control changes in blood pressure and potential variations in cerebral blood flow that may occur during the active and second stages of labor. Women with Moyamoya disease having had vaginal deliveries without neurological sequelae have been reported.[4,7]
Lee et al reported the use of  single­photon emission computed tomography to assess cerebral hemodynamic status to help determine women at greater risk of development of neurological events during pregnancy and puerperium. They postulated that revascularization surgery prior to conception may help avoid neurological events during pregnancy and puerperium.[8]
General anesthesia may provide stable systemic condition, but hypertension, vomiting at the time of endotracheal intubation may occur. Spinal and epidural anesthesia may cause hypotension and the patient may present hyperventilation or hypertension due to anxiety. Epidural anesthesia can provide good pain control in the post-delivery period.


There is no consensus regarding the safest method of delivery, or anesthesia.  So the best approach would be the one with which obstetricians, anesthesiologists and neonatologists are familiar. Adequacy of anesthesia and maintenance of hemodynamic status seem to be more important than the mode of delivery. LSCS under epidural anesthesia is a good option. Alternatively vacuum or obstetric forceps assisted vaginal delivery can also be achieved with due care.

  1. Suzuki J, Kodama N. Moyamoya disease: a review. Stroke. 1983;14(1):104–109. 
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  3. Cunningham FG, Leveno KJ, Bloom SL. Williams Obstetrics. New york, NY, USA: 2010. Neurological and psychiatric disorders; pp. 1167–1169.
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  8. Lee SU, Chung YS, Oh CW, Kwon OK, Bang JS, Hwang G, et al. Cerebrovascular Events During Pregnancy and Puerperium Resulting from Preexisting Moyamoya Disease: Determining the Risk of Ischemic Events Based on Hemodynamic Status Assessment Using Brain Perfusion Single-Photon Emission Computed Tomography. World Neurosurg. 2016 Jun;90:66-75.

Dalvi P, Parulekar SV. Pregnancy With Moyamoya Disease. JPGO 2017. Volume 4 No. 3. Available from: