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Multiple Exostosis Syndrome Joseph Junewick, MD FACR 08/22/2010 History 13 year old with cough and fever. Diagnosis Multiple Exostosis Syndrome Discussion Multiple exostosis syndrome (MES) is a polynomic entity and may be referred to as multiple hereditary osteochondromatosis, osteochondromatosis, diaphyseal aclasis, external chondromatosis among others. MES is an autosomal dominant disorder. Osteochondromas may appear in any portion of the skeleton but tend to be more numerous and larger near the fastest growing joints (wrists and knees); osteochondromas of the carpal bones, sesamoid bones, vertebrae and skull are rare. Fifty percent of patients with multiple exostosis syndrome have lesions in the ribs. Complications include fractures, pneumothorax, vascular or neural compression, and malignant transformation to chondrosarcoma. Findings CR-Multiple osteochondromas involving the humeri, scapulae and ribs. Reference Levine BD, Motamedi K, Chow K, Gold RH, et al. CT of Rib Lesions. AJR (2009); 193:5-13

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Page 1: Multiple Exostosis Syndrome

Multiple Exostosis SyndromeJoseph Junewick, MD FACR

08/22/2010

History13 year old with cough and fever.

DiagnosisMultiple Exostosis Syndrome

DiscussionMultiple exostosis syndrome (MES) is a polynomic entity and may be referred to as multiplehereditary osteochondromatosis, osteochondromatosis, diaphyseal aclasis, external chondromatosisamong others. MES is an autosomal dominant disorder. Osteochondromas may appear in any portionof the skeleton but tend to be more numerous and larger near the fastest growing joints (wrists andknees); osteochondromas of the carpal bones, sesamoid bones, vertebrae and skull are rare. Fiftypercent of patients with multiple exostosis syndrome have lesions in the ribs. Complications includefractures, pneumothorax, vascular or neural compression, and malignant transformation tochondrosarcoma.

FindingsCR-Multiple osteochondromas involving the humeri, scapulae and ribs.

ReferenceLevine BD, Motamedi K, Chow K, Gold RH, et al. CT of Rib Lesions. AJR (2009); 193:5-13

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