OBJECTIVE: To establish the perinatal and neurodevelopmental outcomes of fetuses diagnosed with isolated ventriculomegaly (IVM). METHODS: A systematic review of cohort, case/control studies, case series and case reports of IVM (unilateral or bilateral enlargement of the lateral ventricle, >or=10 mm, with no additional diagnosis at the time of the initial ultrasound), identified by searching, without language restrictions, The Cochrane Library, MEDLINE and Embase databases in June 2004. RESULTS: No cohort study was retrieved but 577 cases were identified in one case/control study and 29 case series/case reports. Of 207 cases with follow-up and known gestational age at diagnosis, normal developmental outcomes were found in 82%, mild impairments in 9% and moderate/severe impairments in 10%. Prenatal diagnosis could possibly have reduced the risk of moderate/severe impairments to 7%. In 137 cases followed to at least 20 months of age, 79% had normal neurodevelopment, 10% were mildly delayed and 11% had moderate/severe developmental delays. CONCLUSIONS: Outcome was favorable in 85% of IVM cases, decreasing to 79% when cases were followed to at least 20 months of age. We recommend prospective cohort studies with new diagnostic modalities to better identify the underlying conditions relating to moderate/severe impairments in the remaining cases.
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