Median nerve compression in Weill-Marchesani syndrome

Weill-Marchesani syndrome is a rare, generalized disorder of connective tissue manifested by short stature, brachymorphia, and spherophakia. Inheritance is autosomal recessive. In the less than 50 reported cases, joint stiffness in the hands and thenar atrophy have been noted in adults. A kindred is reported here in which release of multiple trigger fingers and bilateral carpal tunnel syndrome in childhood has improved hand function in a brother and sister.