Isolated corpus callosum agenesis: a ten‐year follow‐up after prenatal diagnosis (How are the children without corpus callosum at 10 years of age?)

Prenatal Diagnosis - Tập 32 Số 3 - Trang 277-283 - 2012
Marie‐Laure Moutard1, Virginie Kieffer2,3, Josué Feingold1, F Lewin4, Jean‐Michel Baron4, C. Adamsbaum5, A. Gélot6, Arnaud Isapof1,7, François Kieffer8, Thierry Billette de Villemeur9,1,7
1Service de Neuropédiatrie, Pathologie du développement Hôpital Trousseau 26 avenue du Dr Arnold Netter 75571 Paris France
2Centre de Suivi et d'Insertion Hôpitaux de Saint‐Maurice 14 rue du val d'osne 94415 Saint‐Maurice France
3Institut Gustave Roussy Département de cancérologie de l'enfant et de l'adolescent 114 rue Edouard Vaillant 94805 Villejuif France
4Service gynécologie‐obstétrique Hôpital Cochin – Port Royal 27 rue du Faubourg saint Jacques 75679 Paris France
5Service de radiologie Hôpital Bicêtre 78 rue du Général Leclerc 94275 Le Kremlin‐Bicêtre France
6Neuropathologie service d'anatomie pathologique Hôpital Trousseau 26 avenue du Dr Arnold Netter 75571 Paris France
7Université Pierre et Marie Curie Paris 6, Paris, France
8Neonatal Intensive Care Unit Institut de Puériculture de Paris 26 boulevard Brune 75014 Paris France
9Pôle Polyhandicap Pédiatrique Hôpital de La Roche Guyon 1 rue de l'Hôpital 95780 LA ROCHE‐GUYON France

Tóm tắt

ABSTRACTBackground

Corpus callosum agenesis (CCA) is generally diagnosed in utero. Outcome appears to be better if the malformation is isolated. The aim of this study, which is the first one with a long (10 years) and standardized follow up, was to report cognitive abilities of children with isolated CCA diagnosed prenatally.

Methods

We prospectively evaluated 17 children. Clinical examinations, neuropsychological tests were performed each year. School achievement and personal and familial data were collected.

Results

Twelve children completed the entire follow up. One child was finally considered to have associated CCA, because signs of fetal alcohol syndrome had become obvious. Of the 11 other children, three (27%) had borderline intelligence whereas the intelligence levels of eight (73%) were in the normal range, although half of these children experienced some difficulties in scholastic achievement. Neither epilepsy nor intellectual deficiency was noted and intellectual quotient scores correlated strongly with the mother's education level.

Conclusion

Although prenatal diagnosis of isolated CCA is reliable, false postnatal diagnoses remain possible (10–20%) even with complete prenatal screening. Outcome is mostly favorable because intelligence is within the normal range for nearly 3/4 of the children. However, they frequently have mild learning difficulties. © 2012 John Wiley & Sons, Ltd.

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