Moyamoya disease: review and demographic description of a series of cases in Bogotá, Colombia

Johann D. Serrato-Perdomo, Neurosurgery Service, Hospital Militar Central, Universidad Militar Nueva Granada, Bogotá D.C., Colombia
Laura D. Ramírez-Melo, Department of Neurosurgery, Federación Latinoamericana de Sociedades de Neurocirugía, Bogotá D.C., Colombia
Juan C. Luque-Suarez, Neurosurgery Service, Hospital Militar Central, Universidad Militar Nueva Granada, Bogotá D.C., Colombia

Introduction: Moyamoya disease (EM) is an unusual pathology that presents with unilateral or bilateral occlusion of the supraclinoid portion of the internal carotid arteries accompanied by vascular neoformation as a compensatory method of arterial blood flow to the brain parenchyma. Objective: The objective of the study is to describe the experience in the diagnosis, management, and treatment of EM in a fourth level hospital in Bogotá, Colombia, in relation to the literature available in the databases. Methods: A retrospective study was carried out through the search in medical records, and collecting patients diagnosed with EM, demographic variables, risk factors, diseases associated with the diagnosis, treatment, and follow-up of the patients included in this study were obtained. Results: Six cases of different ages were collected, on average 24 years of age, a case with neurofibromatosis type 1 was found, and a case with a first-degree family history, the most frequent clinical presentations were headache, convulsion, and hemorrhagic or ischemic accidents. Arteriography was the gold-standard diagnosis; 83% of the patients received surgical treatment; no relapses or associated mortality was documented at follow-up; however, in 50% of the cases, some type of neurological deficit was identified. Conclusions: The clinical manifestations presented in our cases are compatible with those described in the literature, revascularization surgery was effective taking into account the sequelae and neurological status of the patients, we consider that a long-term follow-up of all cases is necessary to establish sequelae and recurrences.

Keywords: Ischemic stroke. Hemorrhagic stroke. Moyamoya disease. Moyamoya syndrome. Revascularization.