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Early Hum Dev. 2018 Jan;116:9-16. doi: 10.1016/j.earlhumdev.2017.10.004. Epub 2017 Nov 5.

Neurodevelopmental outcome in prenatally diagnosed isolated agenesis of the corpus callosum.

Author information

1
Department of Neonatal Pediatrics and Intensive Care -Neuropediatrics, Rouen University Hospital, Rouen, France. Electronic address: folliot.lise@gmail.com.
2
Department of Neonatal Pediatrics and Intensive Care -Neuropediatrics, Rouen University Hospital, Rouen, France.
3
Department of Radiology, Rouen University Hospital, Rouen, France.
4
Department of Obstetrics, Rouen University Hospital Rouen, France; Normandy University, Neovasc team UNIROUEN, U1245, INSERM, Institute for Research and Innovation in Biomedicine (IRIB), Rouen, France.
5
Department of Genetics, Normandy Centre for Genomic Medicine and Personalized Medicine, Rouen University Hospital, Rouen, France.
6
Department of Neonatal Pediatrics and Intensive Care -Neuropediatrics, Rouen University Hospital, Rouen, France; Normandy University, Neovasc team UNIROUEN, U1245, INSERM, Institute for Research and Innovation in Biomedicine (IRIB), Rouen, France.

Abstract

Neurodevelopmental outcome in children with agenesis of the corpus callosum (ACC) is correlated with the presence or absence of associated brain abnormalities. Indeed, neurodevelopmental outcome shows severe disabilities when the ACC is not isolated whereas in isolated forms, the neurologic development is mainly normal. Contrary to data in several published studies, the prognosis remains uncertain even in isolated forms, which may lead in France to medical termination of pregnancy.

OBJECTIVE:

To evaluate long-term neurodevelopmental outcome in children with prenatally diagnosed isolated ACC.

DESIGN, SETTING AND PARTICIPANTS:

This is a follow-up study conducted in Normandy (France). It included a cohort of 25 children born between January 1991 and June 2016, with a prenatal diagnosis of isolated ACC and who were followed for at least two years.

RESULTS:

The average follow-up was 8±5years. ACC was complete in 17 patients (68%), partial in 5 (20%) and hypoplastic in 3 (12%). Whereas global motor development was normal in each case, normal neurodevelopmental outcome or mild disabilities occurred in 88% children and moderate/severe neuro-disabilities were present in 12% of children. Wechsler Intelligence Scale for Children-IV evaluations and Intellectual Total Quotients were within normal range, but we observed lower scores in verbal comprehension, social judgment, executive functions. A lower score in morphosyntax was observed among 52% of children with oral language disorders.

CONCLUSIONS:

Neurodevelopmental outcome was favorable in most of our patients with isolated ACC, but mild learning disabilities emerged in older children. Long-term follow-up until school age is essential to provide early diagnosis and appropriate care support.

KEYWORDS:

Agenesis of the corpus callosum; Cognitive development; Neurodevelopmental outcome; Prenatal diagnosis

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