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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 3570 gram male newborn was born to a 29-year-old mother at the gestational age of 40 weeks with gross abnormalities of flexion contracture of third and fifth fingers and fourth toes bilaterally. After birth, cyanotic spell while feeding was found, and a series of examinations were done. The abnormalities were focused on brain echogram, computed tomogram and magnetic resonance imaging examination, which showed a large posterior fossa cyst, cerebellar hypoplasia complicated with multiple severe supratentorial anomalies, including marked ventricular dilatation, cerebral agyria, agenesis of corpus callosum, absence of thalamus and basal ganglion, and optic nerve atrophy. Clinically, the patient had neonatal seizure and high fever. The diagnosis of Dandy-Walker complex type A was made according to the new classification presented by Barkovick et al. in 1989.
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PMID:[Dandy-Walker complex with multiple anomalies: report of one case]. 206 87

Partial trisomy 22 usually occurs through a 3:1 meiotic segregation of the parental 11q;22q translocation carrier, which is the most common balanced translocation in man. Common neurologic findings are delayed psychomotor development and muscular hypotonia. There have been a few neuroradiologic and neuropathologic studies, which include ventricular dilatation, arrhinencephaly, and aplasia or hypoplasia of the cerebellar vermis, corpus callosum and pons. We here add one patient with partial trisomy 22 who had, in addition to the usual features, Dandy-Walker malformation, which, to our knowledge, is a previously undescribed feature.
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PMID:Partial trisomy 22 with Dandy-Walker malformation. 228 36

Walker-Warburg syndrome (WWS) is an autosomal recessive disorder manifest by characteristic brain and eye malformations. We reviewed data on 21 of our patients and an additional 42 patients from the literature. From this review, we expand the phenotype to include congenital muscular dystrophy (CMD) and cleft lip and/or palate (CLP), and revise the diagnostic criteria. Four abnormalities were present in all patients checked for these anomalies: type II lissencephaly (21/21), cerebellar malformation (20/20), retinal malformation (18/18), and CMD (14/14). We propose that these comprise necessary and sufficient diagnostic criteria for WWS. Two other frequently observed abnormalities, ventricular dilatation with or without hydrocephalus (20/21) and anterior chamber malformation (16/21), are helpful but not necessary diagnostic criteria because they were not constant. All other abnormalities occurred less frequently. Congenital macrocephaly with hydrocephalus (11/19) was more common than congenital microcephaly (3/19). Dandy-Walker malformation (10/19) was sometimes associated with posterior cephalocele (5/21). Additional abnormalities included slit-like ventricles (1/21), microphthalmia (8/21), ocular colobomas (3/15), congenital cataracts (7/20), genital anomalies in males (5/8), and CLP (4/21). Median survival in our series was 9 months. A related autosomal recessive disorder, Fukuyama congenital muscular dystrophy, consists of similar but less severe brain changes and CMD. It differs from WWS because of consistently less frequent and severe cerebellar and retinal abnormalities. We think that WWS is identical to "cerebro-oculo-muscular syndrome" and "muscle, eye, and brain disease."
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PMID:Diagnostic criteria for Walker-Warburg syndrome. 236 44

Dandy-Walker cyst associated with occipital meningocele is very rare. Only 12 patients have been reported. We report a female infant with Dandy-Walker cyst and occipital meningocele whose diagnosis was suspected antenatally by in utero ultrasonography. At birth, head circumference was normal for 37 weeks gestation. She underwent surgical repair of the occipital meningocele immediately after birth. The post-operative course was uneventful until the sixth day of life when progressive enlargement of the head with progressive ventricular dilatation was recognized. Communication between the posterior fossa cyst and the occipital meningocele was confirmed neuroradiologically; the occipital meningocele may have compensated for the increased intracranial pressure in fetal life.
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PMID:Dandy-Walker cyst associated with occipital meningocele. 266 80

Widely diverging opinions on the optimal therapy for intracranial cerebrospinal fluid cysts (CSF), mainly arachnoid cysts and the Dandy-Walker cysts, exist. Excision of the cyst walls in the treatment of the Dandy-Walker cyst has been replaced by shunting procedures, but the recommended method for primary treatment of arachnoid cysts in childhood is still cyst wall excision. Membrane excision is, however, often complicated by recurrence, subsequently requiring shunting-procedures. In a series of 19 cases primary shunting of intracranial CSF cysts proved to be a reliable method. In those cases where hydrocephalus (ventricular dilatation) is present at the time of the primary operation the ventricles should be shunted as well as the cyst. The catheter from the ventricle and that from the cyst should be connected to the same valve, otherwise an increased risk of intracranial herniation exists. The prognosis for infants and children suffering from intracranial CSF cysts is in general good; in 17 out of 19 cases mental development was normal and in 15 out of 19 motor development was normal. The risk of permanent motor damage seems to be particularly high when an arachnoid cyst is located on the quadrigeminal plate.
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PMID:Shunting procedures in the management of intracranial cerebrospinal fluid cysts in infancy and childhood. 402 14

The ependyma of the spinal central canal in cases of hydrocephalus shows abnormalities which vary with the aetiology of ventricular dilatation. To determine whether these ependymal changes are developmental or reactive in nature, immunohistochemical findings were compared between nine normal controls and 12 cases of hydrocephalus (three each of congenital aqueductal stenosis. Dandy-Walker malformation, Chiari type II malformation, and post-haemorrhagic hydrocephalus) using antisera to nestin, vimentin and glial fibrillary acidic protein. The main pathological findings were disruption of ependymal layer, apparent pseudostratification of ependyma, expansion, cleft or syrinx formation in relation to the central canal, and ependymal rosette formation. In normal developing fetal spinal cord, nestin and vimentin were expressed mainly in pseudostratified ependymal cells and radial fibres in the median septum. In cases with congenital hydrocephalus (congenital aqueductal stenosis. Dandy-Walker malformation, and Chiari type II malformation), nestin was overexpressed in immature ependymal cells, and strong vimentin immunoreactivity was detected in the long tract of radial fibres in the median septum. Nestin and vimentin were also expressed in small cells and their fibres which covered areas denuded of ependymal cells in cases of Chiari type II malformation and post-haemorrhagic hydrocephalus. Two conclusions are suggested by this report. First, the ependyma of the spinal central canal in congenital hydrocephalus shows a delay in maturation of radial glial cells into mature astrocytes and ependymal cells. Second, areas of ependymal denudation may be repaired by the immature glial cells derived from subependymal cells.
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PMID:Overexpression of nestin and vimentin in the ependyma of spinal cords from hydrocephalic infants. 906 85

We sought to assess the sonographic findings and postnatal outcome in fetuses with the prenatal diagnosis of asymmetric hydrocephalus. The sonograms from cases of asymmetric hydrocephalus diagnosed prenatally at our institution were reviewed. Postnatal outcome was obtained from maternal, neonatal, and pediatric records. Fourteen fetuses at 17.3 to 38.9 weeks' gestational age on prenatal sonography had a maximum ventricular measurement of 10.2 to 48.8 mm, with the degree of asymmetry ranging from 2.2 to 27.3 mm. Thirteen of 14 had a normal-sized contralateral ventricle. Other fetal anomalies identified at sonography included Dandy-Walker malformation, intraventricular hemorrhage, porencephalic cyst, hydronephrosis, pleural effusion, and mild dilatation of a renal pelvis. Eleven fetuses had follow-up prenatal sonography. Among these, ventricular dilatation resolved in 5, remained the same in 3, increased in 2, and decreased in 1. Postnatal outcome was normal in 6 cases (43%) and abnormal in 8 (57%), including 2 cases of in utero intracranial hemorrhage, 2 with congenital syndromes, 1 with an imperforate foramen of Monro, 1 with tuberous sclerosis, 1 with developmental delays, and 1 with cerebral palsy. Asymmetric unilateral hydrocephalus appears to represent an entity different from bilateral hydrocephalus in that there is less risk of perinatal death, there are fewer associated anomalies, and the overall prognosis is better. Outcome may be normal, but fetuses with increasing unilateral ventriculomegaly and cases associated with other brain abnormalities tend to have a poor neurologic outcome.
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PMID:Postnatal outcome of fetuses with the prenatal diagnosis of asymmetric hydrocephalus. 1127 May 20

Dilatation of the fetal cerebral ventricles (ventriculomegaly) is a generic sonographic sign common to several pathological entities carrying different prognoses. The main causes of fetal ventriculomegaly are aqueductal stenosis, Chiari II malformation, Dandy-Walker complex and agenesis of the corpus callosum. Ventriculomegaly is easily recognized by ultrasound by measuring the atrial width. This simple measure allows the recognition of mild forms of ventricular dilatation and is used as a screening method for ventriculomegaly. However, although the diagnosis of ventriculomegaly is easy, the prenatal identification of the cause of ventricular dilatation is a more difficult task. To this end, the evaluation of the posterior fossa in association with the visualization of the corpus callosum is a useful landmark. Research into the cause of ventriculomegaly is clinically useful, since the prognosis mainly depends on the etiology and on the presence of associated anomalies. In this article the role of prenatal sonography in recognizing the cause of ventriculomegaly and the prognostic value of the prenatal sonographic findings are discussed.
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PMID:The role of ultrasonography in recognizing the cause of fetal cerebral ventriculomegaly. 1500 80

Dilatation of the fetal cerebral ventricles (ventriculomegaly) is a generic sonographic sign that is common to several pathological entities carrying different prognoses. The main causes of fetal ventriculomegaly are aqueductal stenosis, Chiari II malformation, Dandy-Walker complex, and agenesis of the corpus callosum. Ventriculomegaly is easily recognized by ultrasound by measuring the atrial width. This simple measure allows the recognition of mild forms of ventricular dilatation and is used in screening for ventriculomegaly. However, although the diagnosis of ventriculomegaly is easy, the prenatal identification of the cause of ventricular dilatation is a more difficult task. For this purpose the evaluation of the posterior fossa in association with the visualization of the corpus callosum is useful. Research into the causes of ventriculomegaly is clinically useful, since the prognosis mainly depends on the etiology and on the presence of associated abnormalities. In this article the role of prenatal sonography in determining the cause of the ventriculomegaly is reviewed, as well as the prognostic value of the prenatal sonographic findings.
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PMID:Sonographic diagnosis of fetal cerebral ventriculomegaly: an update. 1743 93

Dandy-Walker syndrome (DWS) is a congenital brain malformation involving the cerebellum and fourth ventricle. We report a 6-month-old girl with DWS presenting an initially normal ventricular system and mild cyst-like lesion over the posterior fossa as assessed by postnatal brain sonography. However, symptoms and signs of increased intracranial cerebral pressure in terms of frequent vomiting and tense anterior fontanel developed, and these were associated with mild hypotonia and poor neck support, and upward-gaze palsy at the age of 6 months. Magnetic resonance imaging revealed a huge cystic lesion of the fourth ventricle, which filled the posterior fossa and ventricular dilatation. The tentorium was progressively displaced upward by the cyst. A nearly complete agenesis of the cerebellar vermis was also confirmed. After a successful endoscopic third ventriculostomy, a series of brain magnetic resonance imaging scans, taken during a follow-up survey, showed normal lateral and third ventricles. Consequently, symptoms of intracranial cerebral pressure resolved, and a developmental milestone was achieved. In conclusion, DWS can be confirmed postpartum, and endoscopic third ventriculostomy was found to be a preferential operative procedure for DWS with hydrocephalus. It may be effective for patients younger than 1 year.
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PMID:Successful treatment of Dandy-Walker syndrome by endoscopic third ventriculostomy in a 6-month-old girl with progressive hydrocephalus: a case report and literature review. 2138 57


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