Moyamoya Syndrome Associated with Polycystic Kidney Disease in a Moroccan Child: A Rare Case

Abstract

Moyamoya disease is a chronic and occlusive cerebro-vascular disease characterized by bilateral steno-occlusive changes in the internal carotid artery and its proximal branches and an abnormal vascular network at the base of the brain. Polycystic Kidney disease (PKD) is known as one of the underlying diseases of moyamoya syndrome which was rarely reported. Only 3 cases of moyamoya syndrome associated with PKD have been reported in the literature. We report here another case of moyamoya syndrome associated with PKD in a Moroccan child. Case Report: 7-year-old girl, was born of a consanguineous marriage, operated at the age of 7 months for bilateral inguinal hernias with a history of polycystic kidney and liver disease, discovered at D15 of life, complicated by arterial hypertension well-balanced under dual therapy, chronic renal insufficiency under conservative treatment and portal hypertension with hypersplenism and esophageal varices grade II. Admitted for a partial afebrile seizure (left hemibody), tonic-clonic associated with intensive headache. Angio-MRI showed features of Moya-Moya syndrome with amputation of the left sylvian artery, and slender aspect of some portions of the right sylvian artery. Treatment was symptomatic with benign evolution without surgery. Conclusion: This is the first report of a Moroccan person with PKD associated with moyamoya syndrome