Radial Club Hand

Deviation of the axis of the wrist in a radial direction is referred to as radial club hand. It is often associated with radial hypoplasia, which can range from mild hypoplasia of the thumb to complete absence of the radial ray (case above). Other anomalies (listed below) are often seen in patients with radial club hand.

• Aneuploidies: Trisomy 18, 21; chromosome 13 deletion
• TAR syndrome: Thrombocytopenia absent radius: Radial agenesis with thumb and metacarpals present; humerus and ulna may be also absent; congenital heart disease in about 1/3 of cases.
• Fanconi anemia: Genetically and phenotypically heterogeneous recessive disorder of progressive pancytopenia, various congenital malformations, and predisposition to hematologic and solid malignancies.
• Holt-Oram syndrome: Congenital heart disease; radial aplasia or hypoplasia; limb defects usually asymmetric.
• VACTERL association: Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal, and Limb (radial) malformations.
• Aase-Smith syndrome: Hypoplastic anemia; hypoplastic distal radius; triphalangeal thumb (thumb has three phalanges instead of the usual two); congenital heart disease

References

Rypens F, Dubois J, Garel L, Fournet JC, Michaud JL, Grignon A. Obstetric US: watch the fetal hands. Radiographics. 2006 May-Jun;26(3):811-29.