Cases reported "Dandy-Walker Syndrome"

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1/8. dandy-walker syndrome associated with occipital meningocele and spinal lipoma--case report.

    A neonate presented with dandy-walker syndrome associated with occipital meningocele and spinal lipoma, manifesting as soft masses on the skull and lumbosacral regions. magnetic resonance imaging demonstrated a large posterior fossa cyst between the fourth ventricle and occipital meningocele, but the aqueduct was patent and there was no sign of hydrocephalus. A cyst-peritoneal shunt was emplaced at the age of 8 days followed by partial removal of the spinal lipoma and untethering of the cord at the 3 months. Follow-up examination of age 3 years found almost normal development, although the cyst still persisted.
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keywords = aqueduct
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2/8. dandy-walker syndrome successfully treated with cystoperitoneal shunting--case report.

    A neonate presented with dandy-walker syndrome manifesting as a large posterior cranial fossa cyst, aplasia of the lower cerebellar vermis, and elevation of the confluence of the sinuses but without hydrocephalus. A cystoperitoneal shunt was placed at one month after birth. The cyst diminished in size, and marked development of the cerebellar hemispheres and descent of the confluence of sinuses were observed, but not vermis development. The primary pathology of dandy-walker syndrome is posterior cranial fossa cyst formation due to passage obstruction in the fourth ventricle exit area and aplasia of the lower cerebellar vermis. The first choice of treatment in patients with dandy-walker syndrome in whom the cerebral aqueduct is open is cystoperitoneal shunt surgery, regardless of the presence or absence of hydrocephalus.
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keywords = aqueduct
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3/8. 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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keywords = aqueduct
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4/8. Endoscopic third ventriculostomy with cystoventricular stent placement in the management of dandy-walker malformation: technical case report of three patients.

    OBJECTIVE AND IMPORTANCE: Dandy-Walker malformation has conventionally been managed with placement of cystoperitoneal or ventriculoperitoneal shunts. However, associated aqueductal obstruction requires simultaneous drainage of both the supratentorial and infratentorial compartments. CLINICAL PRESENTATION: Three children with Dandy-Walker malformation and aqueductal obstruction were managed with endoscopic third ventriculostomy and placement of a stent from the third ventricle to the posterior fossa cyst. INTERVENTION: After an endoscopic third ventriculostomy was performed, the stent was placed from the third ventricle to the posterior fossa cyst through the thinnest part of the posteroinferior wall of the aqueduct. There was no operative morbidity. In one patient, the stent was malpositioned, requiring a repositioning. The endoscopic third ventriculostomy was successful in two patients, whereas it failed in one, requiring a ventriculoperitoneal shunt placement. CONCLUSION: Cystoventricular stent placement with endoscopic third ventriculostomy is a promising alternative in patients with Dandy-Walker malformation with aqueductal obstruction.
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ranking = 4
keywords = aqueduct
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5/8. 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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keywords = aqueduct
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6/8. 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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ranking = 1
keywords = aqueduct
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7/8. Telencephalosynapsis (synencephaly) and rhombencephalosynapsis with posterior fossa ventriculocele ('Dandy-Walker cyst'): an unusual aberrant syngenetic complex.

    Agenesis of the cerebellar vermis (paleocerebellar agenesis) with fusion of the cerebellar hemispheres (rhombencephalosynapsis) is a rare malformation of the central nervous system (CNS). Its combination with synencephaly (telencephalosynapsis), telencephalic ventricular aplasia, aqueductal atresia and cystic fourth ventricle has not yet been described, as far as we know. Here, we report this combination in a 23-weeks' gestation male fetus who was aborted to a 24-year-old diabetic mother. In this fetus with cerebral and cerebellar hemispheric fusion, vermian agenesis was associated with a Dandy-Walker-like posterior fossa cyst, in spite of the fusion of the hypoplastic cerebellar hemispheres. The CNS malformations were further accompanied by dysmorphic facial stigmata such as unilateral atresia of the external ear, ocular hypertelorism and a broad nasal bridge. Preaxial polydactyly and contractures of the upper limbs were the only associated non-cranial abnormalities. Cytogenetic studies revealed a numerically and structurally normal male (46, XY). The malformation complex described in this fetus of a mother with antedating pregnancy diabetes appears to represent a previously undescribed aberrant syngenetic CNS phenotype, some basic teratogenetic aspects of which will be discussed in this paper.
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keywords = aqueduct
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8/8. Observations on the differentiation of hydrocephalus occlusus in infancy and early childhood using computerized axial tomography (CAT).

    hydrocephalus occlusus in children is caused by obstructions of the aqueduct and occasionally by cystic deformations of the posterior cranial cavre presented which were initially discovered by computerized axial tomography (CAT). The value of CAT becomes obvious since it can clearly demonstrate cystic and other lesions, size of ventricles and the course of a hydrocephalus. The method is non-invasive and there is no stress for the patient at all.
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ranking = 1
keywords = aqueduct
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