SKELETAL ABNORMALITIES OF HANDS AND FEET IN LAURENCE-MOON-BARDET-BIEDL (LMBB) SYNDROME - A RADIOGRAPHIC STUDY

Objective. To identify radiological changes of the hands and feet in a large group of patients with Laurence-Moon-Bardet-Biedl syndrome. Des ign. Postero-anterior views of hands and feet were obtained and analys ed. Patients. The material consists of 43 Scandinavian patients with t he syndrome (24 males and 19 females; age 3 weeks to 57 years, median 23 years at the time of radiological examination). Results and Conclus ions. Polydactyly of the hands and feet is one of the main criteria. T his was noted clinically in 33 of 43 patients, but all but 3 had been operated on before this radiological study. Remnants of the extirpated finger or toe noted as exostoses, additional joint surfaces of duplic ation were found in half the hands and feet, while the remainder showe d no radiological changes. Other features found were short, broad bone s and flat joint surfaces of the metacarpophalangeal or metatarsophala ngeal joints. We also found a high frequency of short or long ulna in relation to the radius and Madelung deformity of the wrist in several patients. Thus, the radiographs showed several non-specific normal var iations besides remnants or postoperative changes after polydactyly.