We studied six cases of unilateral hydrocephalus detected prenatally to analyze the sonographic features of the abnormality and to determine the cause and clinical outcome. In all cases, third-trimester sonograms showed marked unilateral lateral ventriculomegaly (mean atrial width, 4.4 cm) and normal contralateral lateral, third, and fourth ventricles. Five of the six cases had marked thinning of the cortical mantle on the affected side and shift of midline structures to the contralateral side. The causes of unilateral hydrocephalus were agenesis or stenosis of the foramen of Monro in three cases, transient obstruction of the foramen in one fetus with an intraventricular hematoma, underlying brain dysplasia in one fetus with a variant of holoprosencephaly, and undetermined in one case. All six neonates had placement of a ventriculoperitoneal shunt catheter; four of these have had normal cognitive development at follow-up. The remaining two infants have moderate to severe developmental impairment. Unilateral hydrocephalus is a rare anomaly that can be recognized by prenatal sonography. Even though unilateral ventriculomegaly may be marked, early diagnosis and treatment may result in a favorable clinical outcome.
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