title: Primary congenital glaucoma outcomes: lessons from 23 years of follow-up.
abstract: OBJECTIVE
  To determine in primary congenital glaucoma whether age of presentation influences surgical success, the degrees of angle surgery needed to achieve glaucoma control, and whether there are critical ages where glaucoma progresses, requiring further surgical management.
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  The medical records of patients with primary congenital glaucoma over a 23-year period were reviewed: 192 procedures were performed on 117 eyes (70 patients). The number and age of angle procedures and final visual acuity was analyzed. Surgical success was defined as stable intraocular pressure and optic disc appearance.
  
  
  RESULTS
  Procedures involving 83 of the 110 eyes (75.5%) undergoing angle surgery were successful, with 2-, 4-, 6-, and 10-year success rates of 92%, 86%, 84%, and 75%, respectively. Subgroup analysis (<3 months; 3-6 months; >6 months) comparing age of diagnosis to visual outcome (<20/200, 20/200-20/40, >20/40) was significant (P = .04). The age at first operation (P = .94), the number of angle operations (P = .43), and their effect on angle surgery success was not significant. Seven of 192 operations were performed after the age of 8 years (3.6%). After the initial angle surgeries within the first year of life, the third procedure occurred at a median age of 2.4 years (interquartile ratio [IQR] 0.6-3.8 years) and the fourth procedure occurred at a median age of 5.3 years (IQR 2.5-6.1 years).
  
  
  CONCLUSIONS
  Children diagnosed at <3 months of age had a visual outcome of <20/200 despite successful glaucoma control. Age of presentation did not affect surgical success. A total of 78.9% of cases undergoing primary trabeculotomy were controlled with 1 operation: 4 clock hours of angle (120 degrees). Analysis of glaucoma progression suggests critical ages where further glaucoma surgery is required at around 2 and 5 years of age.
authors: Discipline of Ophthalmology, University of Sydney, Sydney, NSW, Australia. Electronic address: sophia.zagora@gmail.com.
authors: Sophia L
authors: SL
authors: Zagora
authors: Department of Ophthalmology, The Children's Hospital at Westmead, Sydney, NSW, Australia; Epsom and St Helier University Hospitals, National Hospital Service Trust, London, United Kingdom.
authors: Charlotte L
authors: CL
authors: Funnell
authors: Discipline of Ophthalmology, University of Sydney, Sydney, NSW, Australia; Department of Ophthalmology, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors: Frank J
authors: FJ
authors: Martin
authors: Discipline of Ophthalmology, University of Sydney, Sydney, NSW, Australia; Department of Ophthalmology, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors: James E H
authors: JE
authors: Smith
authors: Department of Ophthalmology, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors: Stephen
authors: S
authors: Hing
authors: Discipline of Ophthalmology, University of Sydney, Sydney, NSW, Australia; Department of Ophthalmology, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors: Francis A
authors: FA
authors: Billson
authors: National Health and Medical Research Council Clinical Trials Centre, University of Sydney, Sydney, NSW, Australia.
authors: Anne-Sophie
authors: AS
authors: Veillard
authors: Discipline of Ophthalmology, University of Sydney, Sydney, NSW, Australia; Discipline of Paediatrics and Child Health, University of Sydney, Sydney, NSW, Australia; Eye and Developmental Genetics Research Group, Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney, NSW, Australia; Children's Medical Research Institute, Westmead, Sydney, NSW, Australia.
authors: Robyn V
authors: RV
authors: Jamieson
authors: Discipline of Ophthalmology, University of Sydney, Sydney, NSW, Australia; Eye and Developmental Genetics Research Group, Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney, NSW, Australia; Department of Ophthalmology, The Children's Hospital at Westmead, Sydney, NSW, Australia.
authors: John R
authors: JR
authors: Grigg
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D002924:Ciliary Body / Q000601:surgery; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D020327:Glaucoma Drainage Implants; D006068:Gonioscopy; D006801:Humans; D006871:Hydrophthalmos / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007429:Intraocular Pressure / Q000502:physiology; D017075:Laser Coagulation; D008297:Male; D012189:Retrospective Studies; D013997:Time Factors; D014065:Tonometry, Ocular; D014130:Trabeculectomy; D016896:Treatment Outcome; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult