Dandy-Walker syndrome

Clinical analysis of 23 cases

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✓ A clinical analysis of 23 patients with Dandy-Walker malformation indicates that more than 85% of them were diagnosed at or before 1 year of age, and that the incidence of associated anomalies is approximately 50%, with a 17% incidence for agenesis of the corpus callosum. The significance of the presence of these anomalies is substantiated by the fact that of the seven deaths recorded, six were related to this disease and five were affected by associated anomalies. The high mortality rate (26%) is comparable to that of other series. The differential diagnosis with posterior fossa extra-axial cysts is discussed.

Ten patients were primarily treated with excision of the cyst membrane; all of them required subsequent shunting to control the intracranial pressure, demonstrating the futility of this approach. None of the patients treated with lateral ventricle shunting suffered an upward herniation of the posterior fossa contents, suggesting that combined shunting of the lateral and fourth ventricles is rarely necessary. The technical advantages of posterior fossa shunting alone are outlined. Of the 16 survivors, 14 were assessed for their mental development by means of standard psychometric testing. The results of the intelligence quotient (IQ) scoring have indicated that 71% of the patients have subnormal mental development (IQ < 83). There was no significant relationship between retardation and associated anomalies, although agenesis of the corpus callosum was related to poor intellectual development in the two patients so affected (IQ's of 50 and 73).

Article Information

Address reprint requests to: Raymond Sawaya, M.D., Division of Neurosurgery, University of Cincinnati Medical Center, 31 Bethesda Avenue, Cincinnati, Ohio 45267.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 22. Left vertebral angiography showing a large avascular space in the posterior fossa with elevation of the posterior cerebral arteries and downward displacement of the posterior inferior cerebellar artery (arrow).

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    Case 22. Computerized tomography scan showing the typical features of Dandy-Walker malformation. Note the symmetrical involvement of both cerebellar hemispheres, as well as the communication between the fourth ventricle (arrow) and the cystic mass.

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    Computerized tomography scan in a patient with a large cystic mass in the posterior fossa. The mass is compressing the left cerebellar hemisphere more markedly than the right, displacing the fourth ventricle anteriorly (arrow) and causing hydrocephalus. Multiple air-cerebrospinal fluid levels are noted following ventriculography. A posterior fossa extra-axial cyst was found at the time of posterior fossa exploration.

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    A prominent cisterna magna was found incidentally on this computerized tomography scan. Note the normal size and position of the fourth ventricle.

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    Case 23. Computerized tomography scans at age 3 months (left) and 3 years (right) after posterior fossa shunting. Note the persistence of a cystic cavity within the posterior fossa with the improved cerebellar development.

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