Familial unilateral Brown syndrome
(Syndrome de Brown unilatéral familial)

Kenawy N, Pilz DT, Watts P.
St Paul's Eye Unit, Royal Liverpool University Hospital, Liverpool, L7 8XP, United Kingdom.

We present a two-generation family with Brown syndrome. The proband was a six and a half-year-old female who presented with a history of failure of dextro-elevation of her left eye. A full ophthalmic evaluation was consistent with a left Brown syndrome. Family history revealed that her mother was operated on as a child for left Brown syndrome and examination of her four and a half-year-old sibling showed similar affection in the left eye. Autosomal dominant inheritance has been postulated in this condition. To our knowledge this is the first report of three members of a two-generation family with left-sided Brown syndrome. Genetic counseling of Brown syndrome cases is advised; nevertheless, identification of the responsible gene should shed more light on its genetics.

 Retour à la bibliographie de Septembre 2008

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