Orthopedic Manifestations of the TAR Syndrome

CHARLES D. BUKREY, MD*


The TAR (thrombocytopenia absent radius) syndrome is a rare genetic disorder that follows an autosomal recessive pattern of inheritance. It is to be distinguished from the Fanconi variants. Several types of limb deficits are associated with this syndrome. The Area Child Amputee Center has been involved in the evaluation and care of six children with this syndrome. Patients tend to have a rather typical bilateral intercalary radial hemimelia. The marked foreshortening in the upper extremities make them the clinical homologues of an upper total phocomelia. They also tend to have lower-limb anomalies, which consist primarily of a marked genu varum. The deformity is accompanied by a variety of roentgenographic manifestations. Upper- and lower-limb problems are addressed as regards clinical presentation and prosthetic/orthotic management.

*Area Child Amputee Center, 235 Wealthy SE, Grand Rapids, MI 49503