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Journal of Korean Neurosurgical Society 1980;9(2): 601-608.
Two Cases of Infantile Hydranencephaly.
Se Ki Kang, Sang Kun Chang, Joon Ki Kang, Jin Un Song
Department of Neurological Surgery, Catholic Medical School, Seoul, Korea.
ABSTRACT
The term hydranencephaly designates the condition in which large protions of the cerebral hemispheres have been reduced to membranous sacs within a cranium of relatively normal size. Only basal ganglia and remnants of the mesencephalon are found at the base of the skull. We have experienced the 2 cases of infantile hydranencephaly. In surveying the pathogenesis of the hydranencephaly, it becomes apparent that the distribution of most of the lesions falls into certain distinct patterns, which is characterized by almost completely absent of both cerebral hemispheres and by relative gross preservation of the basal ganglia, a portion of lower temporal and occipital and contents of the posterior fossa in our experienced 2 cases. In our cases, the condition may present a defect in vascular ontogenesis or the outcome of vascular occlusion of both internal carotid arteries and their main branches on carotid angiogram and CT scan. This paper presents the essential features for the clinical diagnosis, suggesting pathogenesis in 2 cases of infantile hydranencephaly.
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