Recently, the prognosis of congenital hydrocephalus has improved by shunting operation. There are some cases, however, showing poor prognosis even when this procedure has been performed. Thus, postoperative courses of hydrocephalus are variable, and do not always produce the same results. From the clinical viewpoint, we have studied preoperative signs and symptoms, and tried to find some factors which influence the prognosis. This report consists of 41 cases of congenital hydrocephalus and 7 cases of hydroencephalodysplasia over the past six years. For the purpose of observations before and after operation, we adopted some neurological symptoms, head circumference, thickness of cerebral mantle in pneumoventriculogram, intraventricular pressure and others. The average period of follow up of all the cases was two years and six months. Of the 41 patients of congenital hydrocephalus, 24 survived and 14 died. Of the 7 patients of hydroencephalodysplasia, 5 survived and 2 died. As to the age of the patients on admission, 57% of all the cases were infants from 2 months to 4 months old. The patients who had such neurological signs already on admission as motor disability, opisthotonic posture and severe optic nerve atrophy, had a tendency to show poor prognosis in general and to result in a higher mortality. The convulsion and so-called setting sun sign had no significant correlation to poor prognosis. From the morphological evaluation, we have examined increase of circumference and enlargement rate of the head. In the death group, abnormal increase of head circumference was recognized within the first three months of life. When the enlargement was higher than Nellhaus's standard rate within four months after birth, over half of the cases died. In the survival cases, the cerebral mantle thickness was found to be variable while a remarkable decrease was often noted in the death cases. If the mantle is less than 14 mm within three months after birth, prognosis of these cases was generally poor. In the evaluation of intraventricular pressure under endotracheal anesthesia, we found little difference between the survival cases and death cases. However, the death cases exhibited slightly higher average intraventricular pressure. In hydroencephalodysplasia, the cases with abnormal enlarged head marked dysplasia of cerebral hemisphere and thin cerebral cortex showed a tendency toward death, however, the cases with localized partial brain or a lobe dysplasia survived. In some cases by using the ventriculoscope, we could see directly the defect of the brain and obtain beneficial information on diagnosis. Due to the influence of various factors, it is impossible to predict or decide exactly the prognosis of congenital hydrocephalus before operation, but it is of critical necessity to anticipate the postoperative prognosis from preoperative synthetic information.
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