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At a glance

A disorder that results in anatomical anomalies of the hands and feet.

Synonym

Triphalangeal Thumbs and Brachyectrodactyly Syndrome.

Incidence

The few cases reported relate to four kindreds mainly from Mexico.

Genetic inheritance

It occurs either sporadically or as an autosomal dominant inherited syndrome with highly variable expression.

Diagnosis

Based on the clinical findings.

Clinical aspects

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.

Precautions before anesthesia

Routine preoperative assessment.

Anesthetic considerations

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. Peripheral vascular access could be challenging if ectrodactyly is severe.

Pharmacological implications

None specific to this disorder.

References

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Carnevale  A, Hernandez  M, del Castillo  V: A new syndrome of triphalangeal thumbs and brachyectrodactyly. Clin Genet 18:244–252, 1980.  [PubMed: 7438506]
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Silengo  MC, Biagioli  M, Bell  GL,  et al: Triphalangeal thumb and brachy-ectrodactyly syndrome: Confirmation of autosomal dominant inheritance. Clin Genet 31:13–18, 1987.  [PubMed: 3568429]
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Zenteno  JC, Aguinaga  M, Chavez  V,  et al: Triphalangeal thumb and brachyectrodactyly syndrome: An uncommon entity with evidence of geographic distribution. Clin Genet 50:152–155, 1996.  [PubMed: 8946115]

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