A disorder that results in anatomical anomalies of the
hands and feet.
Triphalangeal Thumbs and Brachyectrodactyly Syndrome.
The few cases reported relate to
four kindreds mainly from Mexico. It occurs either sporadically or as an
autosomal dominant inherited syndrome with highly variable expression.
The syndrome is diagnosed based on the clinical
findings of congenital anomalies of the hands and feet. These findings are
principally restricted to the extremities and include triphalangeal thumbs,
brachy(syn)dactyly of the index fingers and third toes and occasionally
ectrodactyly of the hands and feet. Short stature, ectrodactyly (more often
of the feet than of the hands), and onychodystrophy occasionally occur.
Anesthesia for patients with this
condition has not been reported. The mainly peripheral nature of the
features and the absence of associated organ dysfunction suggest no specific
precautions should be necessary.
Carnevale A, Hernandez M, del Castillo V: A new syndrome of triphalangeal
thumbs and brachyectrodactyly. Clin Genet
Silengo MC, Biagioli M, Bell GL, et al: Triphalangeal thumb and
brachy-ectrodactyly syndrome: Confirmation of autosomal dominant
inheritance. Clin Genet
Zenteno JC, Aguinaga M, Chavez V, et al: Triphalangeal thumb and
brachyectrodactyly syndrome: An uncommon entity with evidence of geographic
distribution. Clin Genet