Dandy Walker variant mimicking as cerebral palsy with severe neurological impairment

Vaishali Ramchandra Ghane, Kailash Chandra Patra, Narjohan Meshram, Arpan Chauhan, Anjali Kalbhande, Samir Wankhede

Abstract


Dandy Walker Variant (DWV) is a less severe form of the spectrum of Dandy Walker Malformation (DWM). The DWM is a rare congenital intracranial posterior fossa malformation comprising cystic dilatation of the fourth ventricle, complete or partial agenesis of the cerebellar vermis and an enlarged posterior fossa. Clinically it presents with variable degrees of neurological impairment. Definitive diagnosis of DWM or DWV depends on neuroimaging as most of the clinical signs are not conclusive. This child was clinically suspected as spastic quadriplegic cerebral palsy had incidental finding of DWV on neuroimaging. Hence a definitive diagnosis of DWV was made by MRI in this case. Here we discuss the clinical and radiological aspects of this case with DWV without other associated anomalies yet presenting with clinically significant neurological impairment. 


Keywords


Dandy Walker malformation, Dandy Walker variant, MRI, Neurological impairment

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References


Osenbach RK, Menezes AH. Diagnosis and management of the Dandy-Walker malformation: 30 years of experience. Paediatr Neurosurg. 1992;18:179-89.

Brown JR. The Dandy-Walker syndrome. In: Vinken PJ, Bruyn GW, Klauns HL, eds. Handbook of Clinical Neurology. 2nd ed. Amsterdam: Elsevier publications; 1977: 623-646.

Lavanya T, Cohen M, Gandhi SV, Farrell T, Whitby EH. A case of Dandy-Walker variant: the importance of a multi-disciplinary team approach using complementary techniques to obtain accurate diagnostic information. Br J Radiol. 2008;81:242-5.

Hosam A. Hamid. Dandy-Walker malformation. Egypt J Hum Genet. 2007;8(2):115-20.

Murray JC, Johnson JA, Bird TD. Dandy-Walker malformation: etiologic heterogeneity and empiric recurrence risk. Clin Genet. 1985;28:272-6.

Lavanya T, Cohen M, Gandhi SV, Farrell T, Whitby EH. A case of Dandy-Walker variant: the importance of a multi-disciplinary team approach using complementary techniques to obtain accurate diagnostic information. Br J Radiol. 2008;81:242-5.

Ecker JL, Shipp TD, Bromley B, Benacerraf B. The sonographic diagnosis of Dandy Walker and Dandy Walker variant: associated findings and outcomes. Prenat Diag. 2000;20:328-32.

Altman NR, Naidich TP, Braffman BH. Posterior fossa malformations. AJNR Am J Neuroradiol. 1992;13(2):691-724.

Alam A, Chander BN, Bhatia M. Dandy Walker variant: prenatal diagnosis by ultrasonography. Med J Armed Forces India. 2004;60:287-9.

Kalidasan V, Carroll T, Allcutt D, Fitzgerald RJ. The Dandy-Walker syndrome: a 10-year experience of its management and outcome. Eur J Paediatr Surg. 1995;5(Suppl 1):16-8.

Hosam A. Hamid Dandy-Walker malformation: review article. 2007;8(2):115-20.

Barkovich AJ. Toxic and metabolic brain disorders. In: Barkovich AJ, eds. Paediatric Neuroimaging. 3rd ed. New York: Raven Press; 2000: 71-76.

Jyothi Tadakamadla, Santhosh Kumar, G. P. Mamatha. Dandy Walker malformation: an incidental finding. Indian J Hum Genet. 2010 Jan-Apr;16(1):33-5.