Nail-patella syndrome: long term evolution

Abstract

Nail-patella syndrome is a rare dysplasia characterized by a typical tetrad: hypoplasic nails, hypoplasic or absent patella, radial head dislocation and iliac horns. We review eight patients, four men and four women, aged from 20 to 70 years. The main complaint at consultation was with their knees due to patellar instability and pain. Five of them required surgical treatment due to patellar dislocation and three patients were not treated. The Krogius-Lecène procedure was performed in four patients with a good result following the Insall classification of pain and instability and with an average follow-up of 24 years (range, 18-28 years). Patellectomy was performed in the eldest patient due to femoropatellar arthritis present at first consultation. No patients presented with elbow or nail disorders or with iliac horns.