Neurodevelopment after prenatal diagnosis of isolated agenesis of the corpus callosum: an integrative review

Objective: To systematically review published data on the neurodevelopment of children that were diagnosed prenatally with isolated agenesis of the corpus callosum.

Study design: Medline and Scopus searches (1960-July 2011); cross-referencing of retrieved articles.

Results: Sixteen reports (132 cases of apparently isolated agenesis of the corpus callosum) were included in the analysis. The rates of normal outcome, borderline or moderate disability and severe disability were 94/132 (71.2%; 95% confidence interval [CI], 63.0-78.3), 18/132 (13.6%; 95% CI, 8.8-20.5), and 22/132 (15.2%; 95% CI, 10.0-22.2), respectively. Magnetic resonance imaging detected additional cerebral abnormalities in 22.5% (95% CI, 12.3-37.5) of apparently isolated agenesis of the corpus callosum cases. In truly isolated agenesis of the corpus callosum (confirmed by magnetic resonance imaging), the rates of normal neurodevelopment and severe disability were 52/69 (75.4%; 95% CI, 64.0-84.0) and 8/69 (11.6%; 95% CI, 6.0-21.2), respectively.

Conclusion: Prenatally diagnosed, isolated agenesis of the corpus callosum is usually associated with a favorable outcome. Larger, prospective series are required, as current data are limited, inconsistent, and prevent subgroup analyses (eg, complete vs partial agenesis of the corpus callosum).