Management of thrombocytopenia-absent radius (TAR) syndrome.

Abstract

Thrombocytopenia-absent radius (TAR) syndrome is defined by bilateral absence of the radius and hypomegakaryocytic thrombocytopenia (<150,000/mm3). Lower extremity and nonorthopaedic anomalies also are frequently present. Charts and radiographs of 23 patients with TAR syndrome were reviewed, with extremity and other anomalies documented. Upper and lower extremity management, which included surgery and multiple attempts at orthotic and prosthetic fitting, was evaluated. Upper extremity prostheses were generally rejected, as most patients were able to perform tasks by approximating themselves closely enough to an object to use their own hands. Adaptive devices for feeding, dressing, and toileting were well tolerated. In the lower extremity, most affected patients either rejected any lower extremity intervention or had involvement that eventually precluded functional ambulation, necessitating power wheelchair or motorized cart use. The greatest degree of independence for these patients comes not from surgical, prosthetic, or orthotic intervention, but from the use of simple adaptive devices and powered mobility aids if required.