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Ann Maxillofac Surg. 2018 Jan-Jun;8(1):131-136. doi: 10.4103/ams.ams_34_16.

Dandy-Walker Syndrome with Giant Cell Lesions and Cherubism.

Author information

1
Department of Oral and Maxillofacial Surgery, Terna Dental College, Nerul, India.
2
Department of Oral and Maxillofacial Surgery, Nair Hospital Dental College, Mumbai, Maharashtra, India.

Abstract

It has been very aptly quoted, "Variety is the spice of life"; and so variations exist in all forms and kinds good or bad, and for the worst or the best! Mother nature in all her glory and beauty has been very generous, but what when she fails to provide? It is this very character of nature that gives us variations which sometimes manifest in a cruel way on the human body and gives us the eponym of syndromes. Dandy-Walker malformation is an abnormality of the central nervous system, which leads to hydrocephalus and is associated with other abnormalities. Neurologic symptoms are the norm in afflicted patients due to the inherent nature of the disease in that it affects the very center of human function-the brain. This article brings to you a very unique, challenging and rare case of a young patient with this debilitating disorder who was also affected with giant cell lesions of the maxilla and mandible along with cherubism. It highlights the unpredictable course and progression of the disease in a child and our unique protocol employed for the management of the same. It adds providence and a new perspective to the still ambiguous nature of this disorder and the unprecedented maxillofacial anomalies, i.e., giant cell lesions and Cherubism associated with the same.

KEYWORDS:

Cherubism; Dandy–Walker syndrome; dexamethasone injections; giant cell lesions; surgical curettage

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