Late-onset unilateral primary developmental glaucoma associated with iridotrabecular dysgenesis, congenital ectropion uveae and thickened corneal nerves: a new neural crest syndrome?

Ophthalmic Surg Lasers. 1999 Jul-Aug;30(7):567-70.


The association of unilateral primary developmental glaucoma with iridotrabecular dysgenesis and congenital ectropion uveae has been well documented in the literature. The glaucoma in this entity may present at birth, infancy or may develop at a later stage in life. I report the case of a child with late-onset unilateral primary developmental glaucoma due to iridotrabecular dysgenesis, congenital ectropion uveae, and who had a previously undescribed association with ipsilateral thickened corneal nerves in the stroma.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple*
  • Child
  • Corneal Stroma / innervation*
  • Diagnosis, Differential
  • Ectropion / congenital*
  • Ectropion / diagnosis
  • Female
  • Glaucoma / congenital*
  • Glaucoma / diagnosis
  • Glaucoma / surgery
  • Humans
  • Iris / abnormalities*
  • Neural Crest / pathology*
  • Ophthalmic Nerve / pathology*
  • Syndrome
  • Trabecular Meshwork / abnormalities*
  • Trabeculectomy