Volume 1, Issue 2

 

TWIN PREGNANCY COMPLICATED BY MOYAMOYA DISEASE

August 5, 2010

 SYNOPSIS

We report a 22 year old twin gestation female successfully delivered preterm with postpartum complications which turned out to be secondary to Moyamoya disease.

Keywords: Moya moya disease, Cerebro vascular disease

 ABSTRACT

Background: There are only 53 reported cases of Moya Moya disease (MMD) associated with pregnancy in the literature1

Case: Our report highlights the difficulty in diagnosing and treating newly diagnosed pregnant patients with MMD. We report a case of twin pregnancy in a 23 year old patient who sustained a cerebral hemorrhage less than 24 hours after a Cesarean Delivery and was found to have MMD.

Conclusion:  The differential diagnosis of stroke in pregnancy is extensive and the cause must be extensively investigated. Although MMD is a rare condition, it must be considered in the differential diagnosis.

CASE REPORT   

A 22 year old Bangladeshi primigravida with diamniotic/dichorionic twin pregnancy was admitted to the labor and delivery floor of our facility at 35 weeks gestation because of frontal headaches.  There was no history of visual disturbances or epigastric pain.  Her blood pressure was 150/100 mm Hg and she had +2 proteinuria.  The fundal height measured 40 cm.  Limited sonogram confirmed diamniotic/dichorionic twin pregnancy with breech/cephalic presentations. Fetal cardiac motion was present in each twin.  Pelvic examination revealed a long, closed cervix.  Her complete blood count and liver function tests were normal. She had no significant past medical or surgical history and family history was significant only for hypertension in both parents. Her antepartum course was uneventful prior to admission.   

The patient had an uncomplicated emergency Cesarean delivery of live male infants with Apgar scores of 9/9 and 9/9 at one and five minutes respectively. Following transfer to the postpartum floor, her blood pressure was noted to be elevated and she was treated with Labelatol. She also had Magnesium Sulphate for seizure prophylaxis. On post operative day one, the patient was noted to be increasingly lethargic by family members.  On evaluation by the on-call resident, she was unresponsive to verbal and painful stimuli. Blood pressure was 160/112mm hg and she had sinus tachycardia of 110 beats per minute.     

Neurosurgery and neurology consults were obtained. The patient was transferred to the intensive care unit and intravenous phenytoin and nicardipine were started.  A computed tomography (CT) scan of the head with contrast showed subependymal right basal ganglia and intraventricular hemorrhage measuring 1.2x1 cm (figure 1).  On postoperative day two, the patient was transferred to a tertiary care center where she underwent an emergency cerebral angiogram.  The result demonstrated irregular, severe stenosis and occlusion of the supraclinoid internal carotid arteries (ICA) with multiple collaterals throughout the basal cisterns consistent with MMD (figures 2 and 3).  A follow-up magnetic resonance imaging (MRI) showed persistent hemorrhage in the right hypothalamus/thalamus region and so patient underwent a ventriculo-peritoneal (VP) shunt placement (figure 4). 

Her postoperative course was complicated by clostridium difficile colitis which was treated with oral Metronidazole and postpartum hypertension controlled with oral Captopril, Metoprolol and Clonidine. The patient was discharged home after two weeks in the neurosurgical unit.  She had residual left upper extremity weakness for which physical and occupational therapy were required on an outpatient basis. Currently two and a half years after the cerebrovascular accident, she still complains of weakness in the left upper extremity.  She was prescribed Escitalopram and Amitryptiline for mood disorders.

 A repeat MRI of the brain done recently showed stable microvascular ischemic disease in the right pre and post central gyrus (around the position of the shunt) with a stable old right caudate lobe infarct.  Surgical intervention to correct the MMD is currently under consideration pending determination of suitability by the neurovascular team at the patient’s referral hospital.

DISCUSSION      

MoyaMoya is a Japanese word which means ‘something hazy like a puff of cigarette smoke drifting in the air’2 and in MMD denotes a hazy network of stenosed basilar vessels resembling a puff of smoke3. The cause of the disease is obscure but a high familial occurrence rate (approximately 15%4,5) has been noted. Although MMD occurs primarily in Asians, it has been reported among Caucasians, African Americans, Haitians, and Hispanics6. The female to male ratio is 1.8:17 and it has an incidence (in the western hemisphere) of 0.086 cases per 100,000 persons7.

Symptoms and signs are variable from mild transient ischemic attacks to severe intracranial hemorrhage, seizures, coma, and death8. Nearly half of affected individuals experience a gradual deterioration of cognitive function presumably from recurrent strokes8. In pregnancy these signs and symptoms are difficult to differentiate from other cerebrovascular diseases of pregnancy (like eclampsia, stroke and pre-eclampsia) and, as in our patient, may present with elevated blood pressure and headaches – two of the most common complaints in patients with MMD9. Seol et al found that 25% of patients with MMD initially complained of headaches of which 67% were refractory to medical treatment9.

      Patients with undiagnosed MMD prior to pregnancy have worse outcomes and higher complication rates compared with patients diagnosed prior to pregnancy1. Poor outcomes in pregnancy due to MMD are usually related to hemorrhagic events rather than ischemia and are more likely to occur in women whose first presentation occurs during pregnancy1. In a study by Komiyama et al, there was a greater than 50% complication rate including death, hemiplegia and intellectual impairment in patients diagnosed with MMD during pregnancy. In comparison, there was only one patient with any complication in those diagnosed with MMD prior to pregnancy1 therefore showing the improved prognosis in MMD cases diagnosed early or preterm. Surgical treatment of MMD before pregnancy has reduced complications such as intracranial hemorrhage in pregnancy (which has a mortality of 14-22%10) and shown a reduced incidence of repeat hemorrhages in surgical re-vascularization patients compared to MDD patients managed conservatively (12% versus 14-33%)10.

This is only the second case reported in the literature of cerebral hemorrhage secondary to MMD occurring within 24 hours after delivery. There is limited information about the management of pregnancy and delivery in patients with MMD. No in-depth study has been done to compare the benefit of adequate blood pressure control in the management of these patients but in anesthesiology current recommendations for the delivery of an MMD patient include avoiding hypocapnia and fluctuations in blood pressure as much as possible11. Cesarean Delivery meets these requirements better than vaginal delivery and is the delivery mode of choice in an elective setting11. However, as in our patient, the disease might present as an intracranial hemorrhage even after a Cesarean delivery. There are case reports of patients with MMD who have had vaginal deliveries without neurological sequelae12 in the literature showing that vaginal delivery is not contraindicated in MMD as long as delivery is not prolonged1 and vital signs are stable.   

LEGEND

Figure 1: CT Head without contrast showing subependymal right basal ganglia and intraventricular hemorrhage measuring 1.2x1 cm

Figure 2: Cerebral angiogram showing left Internal carotid artery stenosis and extensive collateral circulation in basilar circulation

Figure 3: Cerebral angiogram showing left Internal carotid artery stenosis and extensive collateral circulation in basilar circulation

 

Figure 4: CT Head without contrast showing reduced intraventricular hemorrhage status post ventriculo-peritoneal shunt placement

REFERENCES

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  2. Suzuki J, Takaku A. Cerebral vascular “Moyamoya” disease. A disease showing abnormal net-like vessels in base of brain. Arch Neuro 20:288-299, 1969
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