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ENGLISH ABSTRACT
JOURNAL ARTICLE
[Fetal cerebral ventriculomegaly. Etiology and outcome, report of 141 cases].
OBJECTIVE: To present the different etiologies of fetal cerebral ventriculomegaly (VA) and try to evaluate prognosis.
MATERIALS AND METHODS: A retrospective analysis was made in 141 cases of fetal cerebral ventriculomegaly diagnosed in our obstetrics department between January 1988 and December 1996.
RESULTS: Mean gestational age at diagnosis was 24.8 weeks. Myelomeningoceles were the most frequent etiology (50 cases, 36%), usually diagnosed late in pregnancy (25 weeks gestation). Termination of pregnancy was the most frequent outcome. Agenesis of the corpus callosum (ACC) was observed in 16 cases (11%), 8 of them being diagnosed before birth. This etiology has been an important diagnostic problem until recent years. The advent of improved imaging techniques has facilitated diagnostic procedures. Thus in 1996, the 4 ACC were all diagnosed by prenatal ultrasound. The 8 live infants presented developmental retardation. Termination of pregnancy has been performed more and more frequently in accordance with parent wishes. In 6 cases (4%), the etiology was congenital infection. When a chromosome anomaly was found (7%), ventriculomegaly was always associated with other ultrasonographic anomalies. Ventriculomegaly was associated with other malformations in 23% of the cases but only 17% were discovered prenatally; all the live infants but one had severe neurological impairment. Ventriculomegaly was considered to be the unique anomaly in 37% of the prenatal cases and was truly unique in 24%, an etiology being found in 6%. No explanation was found in 18%. The outcome of live infants is known in 139 cases (2 children lost to follow-up) after a 6 month to 6 year follow-up. Overall prognosis was severe as among the 42 infants (30%), 14 (10%) are well including 12 who had ventriculomegaly alone.
CONCLUSION: Prenatal diagnosis of ventriculomegaly implies a severe prognosis. Only children presenting limited ventriculomegaly alone have normal neurological development. In case of ventriculomegaly, clinicians must search carefully for other anomalies, always associated with poor prognosis.
MATERIALS AND METHODS: A retrospective analysis was made in 141 cases of fetal cerebral ventriculomegaly diagnosed in our obstetrics department between January 1988 and December 1996.
RESULTS: Mean gestational age at diagnosis was 24.8 weeks. Myelomeningoceles were the most frequent etiology (50 cases, 36%), usually diagnosed late in pregnancy (25 weeks gestation). Termination of pregnancy was the most frequent outcome. Agenesis of the corpus callosum (ACC) was observed in 16 cases (11%), 8 of them being diagnosed before birth. This etiology has been an important diagnostic problem until recent years. The advent of improved imaging techniques has facilitated diagnostic procedures. Thus in 1996, the 4 ACC were all diagnosed by prenatal ultrasound. The 8 live infants presented developmental retardation. Termination of pregnancy has been performed more and more frequently in accordance with parent wishes. In 6 cases (4%), the etiology was congenital infection. When a chromosome anomaly was found (7%), ventriculomegaly was always associated with other ultrasonographic anomalies. Ventriculomegaly was associated with other malformations in 23% of the cases but only 17% were discovered prenatally; all the live infants but one had severe neurological impairment. Ventriculomegaly was considered to be the unique anomaly in 37% of the prenatal cases and was truly unique in 24%, an etiology being found in 6%. No explanation was found in 18%. The outcome of live infants is known in 139 cases (2 children lost to follow-up) after a 6 month to 6 year follow-up. Overall prognosis was severe as among the 42 infants (30%), 14 (10%) are well including 12 who had ventriculomegaly alone.
CONCLUSION: Prenatal diagnosis of ventriculomegaly implies a severe prognosis. Only children presenting limited ventriculomegaly alone have normal neurological development. In case of ventriculomegaly, clinicians must search carefully for other anomalies, always associated with poor prognosis.
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