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

https://doi.org/10.1016/j.ajog.2011.12.024Get rights and content

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).

Section snippets

Objective

The aim of this study was to systematically review published evidence about the prognosis of prenatally diagnosed, apparently isolated ACC, pool the evidence, and explore for potential favorable or adverse prognostic factors.

Sources

We searched the literature (last update July 2011 for studies with prenatal diagnosis of ACC and postnatal follow-up of these children. MEDLINE and SCOPUS searches (1960-July 2011) used combinations of the terms “agenesis corpus callosum” AND “prenatal*.” We deliberately used wide search criteria, as the relevant evidence is sparse. These searches were complemented by perusal of the references of the retrieved articles and additional automated search using the “search for related articles”

Study Selection

Only cases with prenatal diagnosis of ACC were considered. Inclusion criteria included: cohort or series with 3 or more cases of prenatally diagnosed ACC, for which postnatal follow-up of neurodevelopment was available; provision of sufficient details to identify only cases with isolated (ie, without coexisting cerebral or extracerebral congenital anomalies) ACC. There were no language restrictions, and we included cases with complete, partial agenesis, and corpus callosum hypoplasia.

Exclusion

Results

Our initial search yielded 246 results; after perusing the abstracts, 20 studies were further assessed. The analysis included in total 16 studies reporting on the prenatal diagnosis of 132 live born infants with ACC and postnatal follow-up of neurodevelopment.9, 14, 17, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33 Pre- and/or postnatal ascertainment of cerebral lesions with the use of MRI was undertaken in 12 studies (totally 99 cases of ACC: 59 CACC, 21 PACC, 4 cases with hypoplasia of

Comment

ACC is one of the most common CNS abnormalities diagnosed in the fetus. However, prenatal counseling is currently based on scattered data, coming mainly from small case series. Therefore, we systematically reviewed published reports on the neurodevelopment outcome of children prenatally diagnosed with ACC, and we pooled the data when appropriate. We found that the pooled rates of normal outcome and severe disability across all studies were 71.2% (95% CI, 63.0–78.3) and 15.2 (95% CI, 10.0–22.2),

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    The authors report no conflict of interest.

    Reprints will not be available from the authors.

    Cite this article as: Sotiriadis A, Makrydimas G. Neurodevelopment after prenatal diagnosis of isolated agenesis of the corpus callosum: an integrative review. Am J Obstet Gynecol 2012;206:337.e1-5.

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