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Franceschetti-Leber phenomenon

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The affected child constantly or occasionally sticks the fingers of one or both hands into the eye socket, manipulates the eyelid, and thus exerts a heavy pressure on the bulbus. If a rest of eyesight is present, the child also makes fanning movements with the fingers spread out, to provoke an impression of light.

Theodor Leber mentioned the phenomenon in 1877 in von Graefe-Saemisch Handbuch der gesammten Augenheilkunde (7 volumes, Leipzig 1874-1880) under the section «Angeborene Amaurose durch Retinalatrophie», but gave it no special designation. It was Franceschetti who, in 1939, termed it «oculo-digital phenomenon». The sign does not mark any particular form of amaurosis, but is a general symptom in any form of impairment of vision in child age.

Bibliography

  • Th. Leber:
    Die Krankheiten der Netzhaut und des Sehnerven.
    In: von Graefe-Saemisch, Handbuch der gesamten Augenheilkunde, 5 volumes, 1st edition, section 5, page 649. Leipzig, 1877,
  • A. Franceschetti in:
    XI Congre Franç, O. N. O. Bordeaux. 3.-5.-6. 1939. Rev O. N. O., 1939, 17: 244. Rubéole pendant la grossesse et cataracte congénitale chez l‘enfant accompagnée du phénomene digito-oculaire.
    Ophthalmologica, Basel, 1947, 114: 332-339.
  • A. Franceschetti, S. Forni:
    Dégénérescence tapeto-rétinienne infantile (type Leber) avec aspect marbré du fund de oeil périphérique.
    Ophthalmologica, Basel, 1958, 135: 610-616.

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