[Osteoarticular deformities in pseudo-achondroplastic dysplasia]

Mistaken in the past for classical achondroplasia, pseudoachondroplasic dysplasia is indeed a clinical and roentgenologic entity. Moreover under electronic microscope and using microchemical technics, specific pathological patterns may be observed in the growth cartilage cells. Aside from severe micromelic dwarfism, osteoarticular deformitites must be considered as an evolutive complication of P.A.D. The osteoarticular complications are well defined and confirme the specifity of the disease. Clinical and roentgenological evaluation are highly desireable and, if necessary, elective surgical orthopedic management should be performed, with prevention being it's principal goal. The authors give the details of the osteoarticular abnormalities observed in 15 cases of P.A.D. and relate their therapeutic experience herein. The atlanto-axial deformity, which is life threatening, must be looked for with special attention, and treated before neurological complications arise. In the lower limbs, deformities are constant and severe. Epiphyso-metaphyseal growth disorders result in axial deviations and aggravate the above-mentioned pre-existing articular-deformitites. Classical surgical orthopedic management is applicable to these types of malformations and should contribute to a better functional prognosis in this constitutional disease.