Alagille syndrome associated with myelinated retinal nerve fibers

Ophthalmologica. 2009;223(5):348-50. doi: 10.1159/000226259. Epub 2009 Jul 1.

Abstract

Purpose: Alagille syndrome is frequently associated with optic disc anomalies. This is the first report of a patient with Alagille syndrome and myelinated retinal nerve fibers.

Methods: A 5-year-old female patient was referred to the Centre for Ophthalmology before a liver transplantation. Ocular examinations including slit lamp examination and funduscopy as well as anterior segment and fundus images were performed.

Results: We observed myelinated retinal nerve fibers in both eyes of a 5-year-old female patient with Alagille syndrome.

Conclusions: A broad spectrum of ocular anomalies is associated with Alagille syndrome. To our knowledge, this is the first reported case of myelinated retinal nerve fibers in a patient with Alagille syndrome.

Publication types

  • Case Reports

MeSH terms

  • Alagille Syndrome / complications*
  • Child, Preschool
  • Eye Abnormalities / etiology*
  • Female
  • Humans
  • Nerve Fibers, Myelinated / pathology*
  • Optic Disk / abnormalities*
  • Retinal Ganglion Cells / pathology*