HEREDITARY OSTEO-ONYCHO-DYSPLASIA

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Abstract
Hereditary osteo-onycho-dysplasia, a disorder inherited in an autosomal dominant manner, is manifested by dysplasia of the knees, elbows, and nails. The presence of iliac horns, bony projections arising from the posterior aspect of the iliac bones, seems to be pathogno-monic of this condition. Although a completely penetrant syndrome, it exhibits extremely variable expression not only of the components of the tetrad but also of a number of other anomalies of the skeleton. Occasionally, the kidneys and eyes are also affected. The incidence of major involvement in patients with hereditary osteo-onycho-dysplasia in previously reported cases is as follows nails 98%; elbows 90%; knees 92% (agenesis of the patellae 11%, hypoplastic patellae 79%, patellar dislocation 29% and iliac horns 81% (70% are clinically palpable). A review of the literature, and the clinical and roentgenographic observations and pedigree of an affected family renal dysplasia exists, are presented.

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