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Successful term delivery of the second twin after rescue cervical cerclage at 21 weeks with the placenta of the first twin in situ

denoting a hazy network resembling a puff of smoke. The term is not descriptive of the specific manifestations of the disease but rather of its initial angiographic appearance (Suzuki and Kodama 1983). The cause of this disease is obscure; it could be a true vascular malformation or an acquired arteritic condition. Pathologically, it is characterised by intimal thickening in the walls of the terminal portions of the internal carotid vessels reducing the calibre of the vessels significantly. The proliferating intima may contain lipid deposits. The occlusive process begins in the region of the carotid siphon and spreads distally, approaching the anterior and middle cerebral arteries. Eventually, cerebral perfusion may become dependent on collaterals arising from the vertebral arteries. Moyamoya disease occurs primarily in Asians, but also reported among Caucasians, African Americans, Haitians and Hispanics. The female:male ratio is 1.8:1, varying from ages 6 months to 67 years. The highest peak is reported in the first decade with incidence ebbing in the subsequent years. The symptoms vary widely from mild transient ischaemic attacks to severe intracranial haemorrhage, seizures, coma and death. Nearly half of the affected individuals experience a gradual deterioration of cognitive function, presumably from recurrent strokes (Miyakawa et al. 1986). There are only a few case reports of this condition in pregnancy. Those that have been reported have largely presented as intracranial haemorrhage (Sucholeiki 2006). There are no extensive reviews of the management of pregnancy and delivery in pregnancy associated with Moyamoya disease. Blood pressure must be strictly controlled during pregnancy, delivery and puerperium. The most important issue is the mode of delivery. Hyperventilation and hypertension at delivery are the main concerns in a vaginal delivery, as these may trigger an intracranial bleed. Caesarean section remains the mode of delivery of choice in order to avoid hypertension and possible cerebral ischaemia with active labour in these women. However, the disease might present as a cerebral infarct even after a caesarean section. There are very few case reports of patients with this condition who have had vaginal deliveries without neurological sequence (Amin-Hanjani et al. 1993). There is no treatment for Moyamoya disease. Individuals experiencing transient ischaemic attacks and strokes are prescribed aspirin, vasodilators or anticoagulants to reduce the risks of future attacks (Williams et al. 2000). Operative indications for intracranial haemorrhage in pregnancy are similar to those for the nonpregnant state. Children respond better to revascularisation surgery than adults.