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At a glance

A medical condition characterized by absent or decreased eyelashes and eyebrows. It causes loss of visual. Other clinical features include dysplastic and grooved nails and alopecia.

Synonyms

Choroidal Atrophy; Alopecia Syndrome.

Incidence

There are only two cases described in the literature.

Genetic inheritance

The mode of inheritance is uncertain, but mild macular disease in the father who may be a heterozygote representation of a recessive transmission.

Clinical aspects

Total regional choroidal atrophy and other signs of ectodermal dysplasia, dysplasia of the nails (grooved), and teeth. Fine hair, absent or decreased eyelashes, eyebrows. Visual loss must be suspected. Occasionally syndactyly of fingers.

Anesthetic considerations

Other than special attention to protection of the eye and its sensitivity to the light, no specific measures must be undertaken in the anesthetic care of this patient.

References

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Moloney  JB, Blake  J, Denham  B,  et al: Regional choroidal atrophy and alopecia. A new syndrome. Acta Ophthalmol 66:272, 1988.

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