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Scholars Journal of Medical Case Reports | Volume-11 | Issue-12
Rare Case of a Dyggve-Melchior-Clausen Syndrome Revealed by a Spinal Cord Compression
Dr Yassine Amane, Dr Nadia El Mahi, Dr Lamya Diouri, Dr Zakaria Haddadi, Dr Oussama El Abbassi, Pr. Siham Nasri, Pr. Imane kamaoui, Pr. Imane Skiker
Published: Dec. 18, 2023 |
156
92
DOI: 10.36347/sjmcr.2023.v11i12.013
Pages: 2219-2221
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Abstract
Dyggve-Melchior-Clausen syndrome (DMSC) is an rare autosomal recessive skeletal disorder that belongs to the category of spondyloepiphyseal dysplasia. The present work reports a case of DMCS in a nine- year-old kid who was admitted for spinal cord compression accompanied by mental retardation and dysmorphic syndrome. The patient had shown a C1-C2 dislocation with compression of the bulbo-medullary junction, platyspondyly with double ondulation of all the vertebral endplates, widely open acetabulums and epi- metaphyseal dysplasia of the femoral heads. Discussion: The syndrome is clinically characterized by a staturo- ponderal delay of varying severity. Radiological characteristics consist of a scalloped appearance of the iliac wings, spondyloepimetaphyseal dysplasia and platyspondyly. The diagnosis of Dyggve-Melchior-Clausen syndrome can be suspected after a clinical evaluation, a detailed patient history, and identification of characteristic clinical and radiological signs.