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Late-recognized primary congenital glaucoma

David S Walton1, Karina Nagao, Helen H Yeung

  • 1Department of Ophthalmology, Massachusetts Eye and Ear Infi rmary, Harvard Medical School, Boston, Massachusetts, USA. walton.blackeye@gmail.com

Journal of Pediatric Ophthalmology and Strabismus
|April 26, 2013
PubMed

Insights

Late-recognized primary congenital glaucoma (LRPCG) often presents with subtle signs and requires prompt surgical intervention. Early detection and goniosurgery are crucial for managing this condition in children.

Area of Science:

  • Ophthalmology
  • Pediatric Ophthalmology
  • Glaucoma Research

Background:

  • Primary congenital glaucoma (PCG) is a rare condition that can lead to vision loss if not diagnosed and treated early.
  • Late-recognized primary congenital glaucoma (LRPCG) presents unique diagnostic challenges due to delayed presentation beyond infancy.

Purpose of the Study:

  • To characterize a cohort of children diagnosed with LRPCG.
  • To detail the age of presentation, diagnostic indicators, clinical features, and surgical outcomes in LRPCG patients.

Main Methods:

  • Retrospective review of medical records for 31 patients (49 eyes) with PCG diagnosed after one year of age.
  • Confirmation of PCG based on elevated intraocular pressure (IOP), anterior segment abnormalities, and gonioscopy findings.
  • Evaluation of glaucoma surgery outcomes, including IOP control, complications, and need for re-operation.

Main Results:

  • Average age at diagnosis was 4.7 years, with 36% diagnosed over 4 years old.
  • Common initial signs included corneal enlargement (46%) and photophobia (20%); corneal cloudiness was not an initial sign.
  • Goniotomy success rate was 95% overall, with 65% achieving complete success; 31% had final visual acuity of 20/200 or worse.

Conclusions:

  • Increased awareness of subtle signs is vital for differentiating LRPCG from juvenile glaucoma, enabling earlier diagnosis.
  • Glaucoma surgery, particularly goniosurgery, is frequently necessary for LRPCG management.
Abstract

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