Hip Dysplasia in CMT - Research from Japan

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Abstract from Journal of Orthopedic Science. 2003;8(4):610-2.

Hip dysplasia in Charcot-Marie-Tooth disease: report of a family.

Ushiyama T, Tanaka C, Kawasaski T, Matsusue Y.

Department of Orthopaedic Surgery, Shiga University of Medical Science,

520-2192, Seta, Otsu, Japan.

Charcot-Marie-Tooth disease is classified into hereditary motor and

sensory neuropathy (HMSN) types I and II, and affected patients present

with progressive peripheral neuropathy. Some previous orthopedic studies

have revealed the association of hip dysplasia with HMSN, in addition to

pes cavovarus, scoliosis, and recurrent dislocation of the patella. We

describe three patients from the same family who were each diagnosed as

having HMSN type I with associated bilateral severe hip dysplasia,

borderline abnormalities of both acetabula, and dysplastic

osteoarthritis. Based on our experience with these patients and a review

of previous reports, we concluded that routine screening of hip joints,

especially for those with a family history of HMSN, is necessary for

early diagnosis.