Moyamoya disease is a cerebrovascular condition predisposing affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Patients with characteristic moyamoya vasculopathy plus associated conditions are categorized as having moyamoya syndrome. This review describes the demographic characteristics, pathogenesis, evaluation, and treatment of moyamoya disease and syndrome.

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Moyamoya Disease and Moyamoya Syndrome

Background: Intracranial aneurysm with and without subarachnoid haemorrhage (SAH) is a relevant health problem: The overall incidence is about 9 per 100,000 with

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European Stroke Organization Guidelines for the Management of Intracranial Aneurysms and Subarachnoid Haemorrhage

Moyamoya disease is an uncommon cerebrovascular disease that is characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. The disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed moyamoya vessels. The incidence of moyamoya disease is high in east Asia, and familial forms account for about 15% of patients with this disease. Moyamoya disease has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years. Most paediatric patients have ischaemic attacks, whereas adult patients can have ischaemic attacks, intracranial bleeding, or both. Extracranial-intracranial arterial bypass, including anastomosis of the superficial temporal artery to the middle cerebral artery and indirect bypass, can help prevent further ischaemic attacks, although the beneficial effect on haemorrhagic stroke is still not clear. In this Review, we summarise the epidemiology, aetiology, clinical features, diagnosis, surgical treatment, and outcomes of moyamoya disease. Recent updates and future perspectives for moyamoya disease will also be discussed.

https://www.thelancet.com/pdfs/journals/laneur/PIIS1474-4422(08)70240-0.pdf

Moyamoya disease: current concepts and future perspectives

Purpose— The purpose of this statement is to review the literature on childhood stroke and to provide recommendations for optimal diagnosis and treatment. This statement is intended for physicians ...

/pdf/management-of-stroke-in-infants-and-children-a-scientific-21glewj7ma.pdf

Management of Stroke in Infants and Children A Scientific Statement From a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young

Background and Purpose— We sought to better define the morbidity of endovascular Guglielmi detachable coil (GDC) treatment of unruptured cerebral aneurysms and to discuss its role in the prevention of subarachnoid hemorrhage. Methods— We conducted an observational study from August 1992 to June 1999 of 125 unruptured aneurysms treated with GDC in 116 patients: 91 women (78.4%) and 25 men (21.6%), aged 30 to 78 years (mean age, 50.6 years). Immediate and late clinical results were recorded for any neurological event or hemorrhage related to the treated unruptured aneurysm. Angiographic results are reported as immediate, early (2 to 12 months), intermediate (12 to 30 months), and late follow-up (>30 months). Results— Immediate angiographic results showed complete obliteration (class 1) in 59 (47.2%) or residual neck (class 2) in 53 aneurysms (42.4%), leaving 6 residual aneurysms (4.8%) and 7 failures (5.6%). Early follow-up angiograms, available in 100 treated aneurysms (84%), revealed class 1 in 52% and cl...

Endovascular Treatment of Unruptured Aneurysms

An analysis of the natural history of cavernous malformations

Natural history of Moyamoya disease: comparison of activity of daily living in surgery and non surgery groups

Spontaneous subarachnoid haemorrhage with negative initial angiography: a review of 143 cases.

Ruptured blister-like aneurysms arising at non-branching sites of the internal carotid artery (BLICAA’s) sometimes cause disaster during aneurysm repair because of their characteristic configurations. Our study was designed to establish the best surgical strategies for such aneurysms. Eighteen BLICAA’s (0.9% of all treated aneurysms) treated at our institute from January 1994 to July 2006 were retrospectively analysed using the database and imaging studies. We assessed the management outcome with the modified Rankin Scale (mRS). The average follow-up period was 17 months. Their ages ranged from 30 to 68 years with a mean age of 50. There were 16 females, and two males). The angiographic diameter of the aneurysmal sac sranged from 1.4 to 5 mm with a mean diameter of 2.5 mm. The common origins were dorso-medial (n = 7) or dorsal (n = 6) wall of the ICA. Fifteen patients underwent wrapping with cellulose fabric and clipping. Of the remainder, each underwent direct clipping, suturing, or trapping. The overall outcome was mRS 1 in 11 patients (78.0%), two in three patients, three in one patient, one in one patient, and five in two patients. Intra-operative premature rupture occurred in six patients. There was no rebleeding during the follow-up period. Cerebral infarctions following carotid trapping after premature rupture and stenosis after suturing of perforated carotid wall were causes of mortality. The causes of morbidity included initial brain insult and vasospasm. The surgeon should be ware of the high risk of premature rupture during dissection of BLICAA’s. Wrapping with cellulose fabric (Bemsheet®) and holding clipping technique could be chosen as the optimal surgical modality for prevention of rebleeding from these lesions.

Kyu Chang Lee

Papers

An analysis of the natural history of cavernous malformations

Natural history of Moyamoya disease: comparison of activity of daily living in surgery and non surgery groups

Spontaneous subarachnoid haemorrhage with negative initial angiography: a review of 143 cases.

Surgical strategies for ruptured blister-like aneurysms arising from the internal carotid artery: a clinical analysis of 18 consecutive patients

Recanalization of completely thrombosed giant aneurysm: case report