Abstract

URRUTIA-RUIZ, Marco et al. Moyamoya disease. Bol. Med. Hosp. Infant. Mex. [online]. 2007, vol.64, n.2, pp.99-106. ISSN 1665-1146.

Introduction. Objective: Describe the evolution of moyamoya disease in children attended in the Hospital Infantil de Mexico from 1995 to 2005. Material and methods. Retrospective and descriptive study. We retrospectively reviewed the medical records of 10 children with this diagnosis; 7 with the established criteria; 3 were excluded for not counting with angiographic conventional and angioresonance for their diagnosis. Demographic and personal information, clinical manifestations, diagnostic studies, treatment and evolution were recorded and the cases analyzed using descriptive statistics. Results. The average age was 6.5 years and 6/7 was females. The clinical manifestation in all patients was acute or subacute hemiparesis. Associated pathologies included: Down's syndrome (4), microcephaly (3), and congenital heart disease (2). The diagnosis was confirmed with angiography (5), magnetic resonance angiography (2). A duro-arterio-synangiosis was practiced to 2 and a temporal angiosinostosis to 1, with resolution of the motor deficit without posterior deterioration. The remaining 4 patients only received symptomatic treatment, with persistence of motor deficit and seizures. Conclusion. The diagnosis of moyamoya disease continues to be with angiography and angioresonance. When possible, surgical correction is the treatment of choice.

Keywords : Moyamoya's disease; clinical manifestations; diagnosis; treatment.