Nail - patella syndrome

an autosomal dominant disorder, also referred to as osteo-onychodysostosis, hereditary osteo-onychodysplasia (HOOD), or Fong's syndrome. In this disorder patients have dysplastic fingernails, hypoplastic or absent patellae, other bone deformities, iliac horns ( Fig.1), widespread soft tissue changes and renal dysplasia, sometimes leading to renal osteodystrophy and death. In the knee, asymmetric development of the femoral condyles and a sloping tibial plateau can be observed. The elbow may also reveal asymmetric development of the humeral condyles, hypoplasia of the capitulum, and subluxation or dislocation of the radial head (Fig.2). The bilateral outgrowths from the posterior ilium (iliac horns) are virtually pathognomonic of nail-patella syndrome. Other bone changes can be evident in the shoulder, wrist, ankle and subtalar joints.

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Fig.2

AP (a) and lateral (b) radiographs of the elbow demonstrate hypoplasia of the capitulum and posterior subluxation of the radial head.


Nail - patella syndrome, Fig.1 (a)	Nail - patella syndrome, Fig.1 (b)	Nail - patella syndrome, Fig.2 (a)

Nail - patella syndrome, Fig.2 (b)