Unilateral cerebral ventriculomegaly: is one better than two?

Benacerraf, Beryl R.

doi:10.7863/jum.2001.20.3.179

Cited by 3 publications

(5 citation statements)

References 15 publications

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“…In our study, the foetopathological examination established the diagnosis of alobar holoprosencephaly and mesencephalosynapsis. Chromosomal abnormalities are classically reported in minor ventriculomegaly (up to 27%) [12]. In our study, karyotypes were normal.…”

Section: Discussionsupporting

confidence: 54%

Fetal Ventriculomegaly and Outcomes: About 3 Cases

Diallo¹,

Poaty

Azonbankin

et al. 2019

OJPathology

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Introduction: Ventriculomegaly is one of the most common abnormalities detected by prenatal ultrasound. This entity can be associated with brain abnormalities and other malformations. In order to perform a good prenatal counseling, the diagnosis is essential. Objective: Our purpose was to report the management of 3 cases of ventriculomegaly in our practice and underline the benefits of the neuropathologic exam. Methods: We analyzed retrospectively 3 medical files concerning fetus that were diagnosed as having one or two ventricles of >10 mm and a follow up for prenatal diagnosis. Results: All cases were diagnosed between 22 and 27 weeks of gestation by an ultrasound. In a case, the ventriculomegaly evolved rapidly to a hydrocephalus. In two cases, the pregnancy was terminated with medical interruption by the parent's request. Amniocentesis confirmed the diagnosis of congenital toxoplasmosis in case 2 there were no chromosomal abnormalities after karyotyping. Causes included holoprosencephaly, congenital toxoplasmosis and mesencephalosynapsis. Conclusion: Ultrasonography may detect ventriculomegaly nerverthless in the cases presented, pathology exam was essential to assess the diagnostic. Causes are numerous and prognosis variable. We recommand MRI that is more and more accessible in our regions in the follow up of this entity and neuropathology exam in case of termination of the pregnancy. The establishment of main causes of ventriculomegaly in our regions (infectious causes for example) could help to prevent this outcome in our practice.

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Section: Discussionsupporting

confidence: 54%

Fetal Ventriculomegaly and Outcomes: About 3 Cases

Diallo¹,

Poaty

Azonbankin

et al. 2019

OJPathology

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“…There are few studies which assess the developmental outcome of unilateral ventriculomegaly 2,6,7,10,11 and only one study on the outcome of asymmetric ventricles 1 . Benacerraf, 12 based on a review of the literature, states that the prognosis of a fetus with unilateral ventriculomegaly clearly depends on the degree of the ventriculomegaly and the presence or absence of other associated abnormalities. When the unilateral ventriculomegaly is isolated, mild and stable in utero , a favourable neurologic outcome is expected.…”

Section: Introductionmentioning

confidence: 99%

Neuropsychological outcome of children with asymmetric ventricles or unilateral mild ventriculomegaly identified in utero

Sadan

Malinger

Schweiger

et al. 2007

BJOG

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Design To assess the neuropsychological outcome of children with asymmetric ventricles and unilateral ventriculomegaly identified in utero.Setting Fetal neurology clinic.Population We assessed 21 children with asymmetric ventricles (group 1) and 20 children with unilateral ventriculomegaly (group 2) identified in utero and compared them with a group of 20 children with symmetric ventricles using a formal neuropsychological tool: the Bayley Scale of Infant Development II (BSID-II).Main outcome measures The group of children with unilateral ventriculomegaly scored significantly lower than the control group on the mental developmental index (MDI) and on the behaviour rating scale (BRS) but not on the psychomotor index. The group of children with asymmetric ventricles did not differ significantly from the control group on either the MDI or psychomotor developmental index but differed from the latter on the BRS. Fifteen percent of the children in the asymmetric ventriculomegaly group performed two SDs below average compared with 4% of children in the asymmetrical ventricles group and none of the control.Conclusion Our results indicate that prenatally observed unilateral ventriculomegaly is a significant risk factor for developmental delay. The mental and motor outcome of children with asymmetric ventricles is similar to that of the control group, but these children are at a significant risk for behavioural abnormalities.

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“…Prognosis of unilateral ventriculomegaly is uncertain. However, it depends on the severity of dilatation, therapeutic options as well as absence of associated anomalies [Benacerraf, 2001; Sadan et al, 2007]. Patients with mild unilateral ventriculomegaly have favorable outcome with adequate cognitive function and normal to mild physical impairment [Patten et al, 1991].…”

Section: Discussionmentioning

confidence: 99%

“…Patients with mild unilateral ventriculomegaly have favorable outcome with adequate cognitive function and normal to mild physical impairment [Patten et al, 1991]. Mild to moderate developmental delay, squint strabismus, hemianopia, hemiparesis, mild speech difficulties and learning problems have been ascertained in the few reports that assessed developmental outcome of unilateral ventriculomegaly [Patten et al, 1991; Tsao et al, 1996; Durfee et al, 2001; Benacerraf, 2001; Sadan et al, 2007]. Both patients had mild developmental delay, hemiparesis contralateral to the involved hemisphere, and mild intellectual impairment.…”

Section: Discussionmentioning

confidence: 99%

“…Gaglioti et al 2005 mentioned that the rate of chromosomal anomalies in isolated ventriculomegaly is fairly low, and these were normal in this family. Isolated ventriculomegaly has been associated with chromosomal aneuploidy in few reports [Terry et al, 2000; Benacerraf, 2001; Ouahba et al, 2006]. Nevertheless, the presence of a closely similar picture in the sibship is highly suggestive of a genetic basis.…”

Section: Discussionmentioning

confidence: 99%

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Familial congenital unilateral cerebral ventriculomegaly: Delineation of a distinct genetic disorder

Zaki¹,

Afifi²,

Barkovich³

et al. 2009

American J of Med Genetics Pt A

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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.

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Unilateral cerebral ventriculomegaly: is one better than two?

Cited by 3 publications

References 15 publications

Fetal Ventriculomegaly and Outcomes: About 3 Cases

Fetal Ventriculomegaly and Outcomes: About 3 Cases

Neuropsychological outcome of children with asymmetric ventricles or unilateral mild ventriculomegaly identified in utero

Familial congenital unilateral cerebral ventriculomegaly: Delineation of a distinct genetic disorder

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