Cases reported "Dandy-Walker Syndrome"

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1/7. Blake's pouch cyst: an entity within the Dandy-Walker continuum.

    Abnormal cerebrospinal fluid (CSF) collections within the posterior fossa are defined by the Dandy-Walker complex (DWC) and by arachnoid cysts (AC). The DWC includes the Dandy-Walker malformation (DWM), the Dandy-Walker variant (DWV) and the mega-cisterna magna (MCM). In addition, Tortori-Donati et al. added persistent Blake's pouch cyst (BPC) as an independent entity within the DWC. BPC represents a posterior ballooning of the superior medullary velum into the cisterna magna. All of these malformations are overlapping developmental anomalies characterized by varying degrees of malformation of the medullary vela, the cerebellar vermis and hemispheres, the fourth ventricle choroid plexus, the posterior fossa subarachnoid cisterns and the enveloping meningeal structures. We present two cases of persistent BPC detected in two adult women without history of gestational or subsequent growth problems. They underwent neuroradiological investigation because of headache and because of recurrent episodes of loss of consciousness, respectively. The MRI findings included tetraventricular hydrocephalus, wide communication of the fourth ventricle and the cystic posterior fossa (i.e. BPC), inferior posterior fossa mass effect with or without hypoplasia of both the cerebellar vermis and the medial aspects of the cerebellar hemispheres, and absence of communication between fourth ventricle and the basal subarachnoid space in the midline posteriorly. Persistent BPC is defined by a failure of embryonic assimilation of the area membranacea anterior within the tela choroidea associated with imperforation of the foramen of Magendie. Typically this condition becomes symptomatic early in life. In the current cases the normal function of the laterally positioned foramina of Luschka probably helped to maintain some CSF flow between intraventricular and subarachnoid spaces, with the establishment of a precarious equilibrium characterized by a compensatory enlargement of the cerebral ventricular system (i.e. hydrocephalus).
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2/7. Intraoperative direct neuroendoscopic observation of the aqueduct in Dandy-Walker malformation.

    A 3-month-old female infant with Dandy-Walker malformation manifesting as hydrocephalus was treated successfully by only ventriculoperitoneal shunting. A flexible neuroendoscope was used intraoperatively to confirm the patency of the aqueduct, i.e. communication of the ventricular system and the cyst in the posterior fossa. Direct confirmation of the patency of the aqueduct and cyst communication is valuable to select the shunt procedure in the treatment of Dandy-Walker malformation.
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3/7. Dandy-Walker variant malformation, spastic paraplegia, and mental retardation in two sibs.

    Two sibs, a boy and a girl, had both hypoplasia of the cerebellar hemispheres and partial agenesis of the cerebellar vermis with normal communication between the fourth ventricle and arachnoid spaces, i.e., the manifestations of the Dandy-Walker variant malformation associated with agenesis of the corpus callosum. Both sibs were mentally retarded and had spastic paraplegia. The occurrence of a distinct and similar pattern of congenital anomalies in sibs born to healthy parents points toward a "new" syndrome caused by the homozygous state of an autosomal recessive gene. Prenatal ultrasonographic diagnosis is possible at least for the more severe form of the brain anomalies.
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4/7. Upward herniation of the posterior fossa cyst in the shunted child.

    Seventy-two years after Dandy and Blackfan's initial description of the dandy-walker syndrome, controversies still exist regarding the precise methods of treatment for this disorder. One potential complication of simple lateral ventricular shunting is upward herniation of the posterior fossa cyst, which at times can result in precipitous clinical deterioration. We present three examples of posterior fossa cysts with upward herniation and review the pertinent literature. We advocate contrast studies to determine whether aqueductal communication exists, before shunting is performed, as well as close follow-up of the shunted child.
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5/7. dandy-walker syndrome: recognition by sonography.

    The sonographic appearance of the Dandy-Walker malformation is not well known. Experience with sonography in the recognition of the dandy-walker syndrome in four neonates is presented. Three cases were discovered serendipitously: one during obstetric evaluation for uncertain gestational age and two in asymptomatic neonates. The typical sonographic features are a triangular posterior fossa cyst, a dilated aqueduct of Sylvius in communication with the cyst, elevation and hypoplasia of the cerebellum, and variable dilatation of the third and lateral ventricles. Sonography is often the first diagnostic procedure performed on these patients and can be very useful in recognizing the anomaly.
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6/7. Dandy-Walker cyst associated with occipital meningocele.

    Two cases of Dandy-Walker cyst associated with occipital meningocele are presented. Only 9 cases with such association have been described in the literature. In our patients computed tomography clearly demonstrated direct communication of a posterior fossa cyst with an occipital meningocele at one end and with the fourth ventricle at the other. Occipital meningocele might simply be an expression of increased intracystic pressure in Dandy-Walker cyst in embryonic life, or such association might suggest its morphogenetic situation during embryogenesis.
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7/7. prenatal diagnosis of Dandy-Walker malformation: report of a case.

    Dandy-Walker malformation is one of the major causes of congenital hydrocephalus. We report on a case that was diagnosed by sonography in a fetus at 34 weeks' gestation. The diagnosis was confirmed by postnatal computed tomographic (CT) brain scan and magnetic resonance imaging (MRI). The prenatal sonographic findings were a large posterior fossa in communication with the fourth ventricle, a small and splayed cerebellar hemisphere and marked degrees of ventriculomegaly. The postnatal CT scan images were similar to the prenatal sonography. The hypoplasia of the cerebellar vermis and the associated hypogenesis of the corpus callosum can only be clearly delineated by MRI. Dandy-Walker malformation is one form of the Dandy-Walker complex and cystic malformations of the posterior fossa. It should be differentiated from Dandy-Walker variant, or mega-cisterna magna, and arachnoid cyst. Dandy-Walker complex may be associated with chromosomal, intracranial and extracranial abnormalities. Early in utero detection is useful for detailed evaluation of associated anomalies. Obstetric management depends on gestational age, karyotype abnormality and associated intracranial and extracranial anomalies. In the present report, the infant presented no progressive ventriculomegaly and no surgery was required. The infant developed normally.
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