Congenital hemicerebral arterial ectasia complicating unilateral megalencephaly

Abstract

Cerebral arterial ectasia, also called megadolichoartery (Boeri & Passerini, 1964), aneurysmal malformation (Scott & Stauffer, 1964), or fusiform arterial dilatation (Eldevik & Gabrielsen, 1975), is a relatively rare condition most commonly occurring in the carotid and vertebro-basilar systems (Greitz & Lofstedt, 1954; Hulten-Gyllensten et al, 1959; Boeri & Passerini, 1964; Sacks & Lindenburg, 1969; Goldstein & Tibbs, 1981; Yu et al, 1982; Tsukamoto et al, 1985; Yamada et al, 1985). The abnormality is characterised by pathological tortuosity, elongation, and dilatation of the arteries at the base of the brain (Hulten-Gyllensten et al, 1959; Bladin & Donnan, 1963; Boeri & Passerini, 1964; Sacks & Lindenburg, 1969; Banna & Romeo, 1979; Goldstein & Tibbs, 1981; Tsukamoto et al, 1985). We recently encountered an interesting case with arterial ectasia showing generalised dilatation of the entire arterial tree but confined to the right cerebral hemisphere accompanying the ipsilateral hemimegalencephaly. This association is the first, to our knowledge, appearing in the neuroscience literature. The main purpose of this report is to present an unusual finding concerning this disease.