Moya-moya disease
DOI:
https://doi.org/10.36958/sep.v7i1.214Keywords:
Moyamoya, vasculopathy, cerebrovascular event, intracerebral hemorrhageAbstract
Moyamoya disease is a vasculopathy distinguished by progressive stenosis of cerebral arteries with the consequent formation of multiple fragile collateral vessels. Genetic studies identify RNF213 as the gene that provides susceptibility to develop the disease, but other genetic triggers are needed to express it. Moyamoya vessels are fragile and provide insufficient cerebral perfusion leading to a wide variety of clinical presentations such as: ischemic or hemorrhagic cerebrovascular events, epileptic seizures, cognitive impairment, disability or death.
The case of a male patient admitted for a hemorrhagic cerebrovascular event is presented. An arteriogram with digital subtraction showed severe stenosis at the level of the internal carotid arteries and collateral circulation from multiple basal and dural arteries, highlighting in the arterial phase that the collateral circulation emits an image similar to chimney smoke puffs.
In adults it occurs as intracranial hemorrhage due to hyperflow generated in anomalous vessels, which causes their rupture. Indirect revascularization by pial synangiosis or direct revascularization by bypass of the superficial temporal artery with the middle cerebral artery are the surgical treatments and and future ischemic or hemorrhagic cerebrovascular events should be prevented. The case is exposed because it is a pathology whose incidence is very low in Guatemala and an update on the pathophysiology and therapeutic approach will be provided.
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Copyright (c) 2024 Ligia Ibeth Portillo Rivera, Sergio Raúl Chang Menéndez, María Ximena López Cervantes, Francisco Quijivix Cojulun, David Navarro Loarca, Juan Pablo Pineda
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