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At a glance

Inherited anomalies of bones and joints of the hands.

Incidence

In 1965, Z. Banki, a Hungarian physician, described the syndrome in three generations of the same Hungarian family.

Genetic inheritance

Assumed to be a unique, autosomal dominant inherited mutation.

Diagnosis

Based on the clinical findings and family history.

Clinical aspects

Affected family members demonstrate fusion of the cuneiform and lunate bones of the wrist, clinometacarpia (bowing of metacarpal bones), brachymetacarpia (shortening of metacarpal bones), leptometacarpia (thinning of metacarpal bones), and clinodactyly. No other systemic manifestations are associated with this condition.

Precautions before anesthesia

None specific to this syndrome.

Anesthetic considerations

Care with positioning of the wrists during anesthesia due to decreased mobility and increased susceptibility of the wrist joints to trauma. If possible, venous access should probably not be attempted in the hands.

Pharmacological implications

None specific to this syndrome.

Reference

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Banki  Z: Kombination erblicher Gelenk-und Knochenanomalien an der Hand. Zwei neue Röntgenzeichen. Fortschr Röntgenst 103:598, 1965.

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