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Hereditary multiple exostoses causing cord compression.

Author(s)
Md. Shafiqul Bari Department of Medicine, Sylhet M.A.G. Osmani Medical College, Sylhet, Bangladesh
M. M. Jahangir Alam Department of Medicine, Sylhet M.A.G. Osmani Medical College, Sylhet, Bangladesh
Fazle Rabbi Chowdhury Department of Medicine, Sylhet M.A.G. Osmani Medical College Sylhet, Bangladesh
Pulin Bihari Dhar Department of Medicine, Sylhet M.A.G. Osmani Medical College Sylhet, Bangladesh
Afsana Begum Department of Medicine, United Hospital Dhaka, Bangladesh
Abstract
Hereditary multiple exostoses (HME) is an autosomal dominant skeletal disorder characterized by the presence of multiple osseous prominences. It can occur sporadically or within families (22 – 56%). Two genes, EXT1 and EXT2 located respectively at 8q24 and 11p11-p12, have been isolated to cause HME. It can cause gross deformity of limbs and growth disturbance which is quite a common complication. Malignant transformation to chondrosarcoma can also occur. Neurological presentations are rare and usually happened due to direct compression of a peripheral nerve or nerve root or less often the spinal cord. This case is possibly the first case of HME described from Bangladesh, presented with dorsal cord compression. Decompression was done and the complaints of myelopathy were improved.
Publication Details
Page(s) 797-799
DOI DOI not available
Published Journal: Journal of College of Physicians and Surgeons--Pakistan : JCPSP, Volume: 22, Issue: 12, Year: 2012

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