Nonprogressive congenital unilateral ventriculomegaly.

Department of Pediatrics, National Taiwan University Hospital, Taipei, Republic of China.
Pediatric Neurology (Impact Factor: 1.5). 02/1996; 14(1):66-8. DOI: 10.1016/0887-8994(95)00256-1
Source: PubMed

ABSTRACT Congenital unilateral ventriculomegaly is a rare condition, usually caused by obstruction of the foramen of Monro. In the past, this condition required surgical intervention. We present a female newborn with nonprogressive unilateral ventriculomegaly which was initially detected by prenatal sonography. No surgical intervention was performed, and during the 9 months of follow-up, she had normal head growth and reached appropriate developmental milestones.

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    ABSTRACT: To report prospectively the prenatal diagnosis, management and outcome of 14 cases of unilateral ventriculomegaly. Fourteen fetuses were diagnosed as having one ventricle of > or = 10 mm, as measured at the level of the atrium. In ten cases, the scan showed mild unilateral ventriculomegaly with an atrium width between 11 and 13 mm and this remained stable up to term. Eight of these fetuses had a magnetic resonance imaging scan in utero between 32 and 34 weeks of gestation which confirmed the diagnosis of mild ventriculomegaly without other brain abnormalities and showed a normal cortical mantle. No obvious cause was found and the outcome was normal in all cases. In four cases, the unilateral ventriculomegaly evolved rapidly with an atrium width up to 20-25 mm. Causes included atresia of the foramen of Monro, toxoplasmosis, brain atrophy and Weaver syndrome. Three underwent termination of pregnancy and the postmortem examination confirmed the diagnosis. The baby with brain atrophy and schizencephaly had a ventriculoperitoneal shunt placed at 1 month of age and has severe developmental delay at 9 months. The prognosis of unilateral ventriculomegaly is uncertain. Examination of both ventricles during the anomaly scan should be performed, as should ultrasound follow-up of these cases up to the end of the third trimester. Fetuses with an isolated, mild, stable unilateral ventriculomegaly seem to have a favourable neurological outcome. However, fetuses with rapidly evolving unilateral ventriculomegaly or cases associated with other brain abnormalities may have a poor neurological outcome.
    Ultrasound in Obstetrics and Gynecology 11/1999; 14(5):327-32. DOI:10.1046/j.1469-0705.1999.14050327.x · 3.14 Impact Factor
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    ABSTRACT: To evaluate the outcome of fetuses with isolated borderline, unilateral ventriculomegaly. A retrospective survey was conducted at four perinatal centers in Israel. Only fetuses with one ventricular width of > or = 11 mm and the other < 10 mm were included in the study. In all cases, the difference of the ventricular width between the two ventricles was > 2.4 mm (two standard deviations). Fetuses with other malformations, chromosomal abnormalities, or those with evidence of in utero infection, were not included in the study. Unilateral ventriculomegaly was found in 27 subjects (after excluding one case with unilateral ventriculomegaly and Down's syndrome). The mean width of the enlarged ventricle was 11.7 +/- 0.9 mm, while the other normal ventricle was 7.2 +/- 0.9 mm. The mean gestational age at diagnosis of the unilateral ventriculomegaly was 23.6 +/- 2.7 weeks. In one case, pregnancy was terminated, and pathological examination of the fetal brain failed to detect any structural abnormality. Twenty-five patients delivered at term and only one at 34 weeks' gestation. The neurological development in all 25 fetuses was normal, and one fetus had petit mal seizures. Fetuses with isolated, borderline unilateral ventriculomegaly, but without other abnormalities, have a good neurological outcome.
    Ultrasound in Obstetrics and Gynecology 07/1998; 12(1):23-6. DOI:10.1046/j.1469-0705.1998.12010023.x · 3.14 Impact Factor
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    ABSTRACT: We identified two female siblings, derived from healthy first cousin parents, with congenital unilateral cerebral ventriculomegaly detected prenatally. Patient 1 underwent ventriculoperitoneal shunt operation at 1 week old, while Patient 2 was followed without surgical intervention. Both patients presented with mild developmental delay and hemiparesis contralateral to the involved hemisphere. Focal seizures were observed in Patient 1, whose neuroimaging revealed posterior insular polymicrogyria in the normal sized ventricle hemisphere and retrocerebellar cyst. Both siblings displayed near absence of white matter with marked thinning of the overlying cortex in the affected hemisphere and very thin corpus callosum. Investigations revealed no other system involvement and karyotyping was normal. Normal TORCH screening in subsequent pregnancies, normal parental coagulation profile and undetectable maternal autoantibodies suggested against the possible role of extrinsic factors as an etiological factor for unilateral ventriculomegaly. Parents had normal brain imaging findings. We suggest delineation of a distinct developmental brain defect, most likely of autosomal recessive inheritance.
    American Journal of Medical Genetics Part A 08/2009; 149A(8):1789-94. DOI:10.1002/ajmg.a.32983 · 2.05 Impact Factor


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May 26, 2014