Congenital / Juvenile Glaucoma

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Definition

  • Primary congenital glaucoma (PCG) <2 years:
    • Neonatal onset (up to 1 month)
    • Infantile onset (1 month to 2 years)
    • Late-onset/ late diagnosis (> 2 years)
  • Juvenile / late-onset glaucoma: Onset from >2 years to puberty
  • Secondary childhood glaucoma:
    • Associated with anomalies e.g., in the context of aniridia, Axenfeld-Rieger anomaly, Peters anomaly, nanophthalmos, PHPV, ROP, tumor, uveitis, etc.
    • In the context of a syndrome: e.g., Lowe, Sturge-Weber, neurofibromatosis, Hallermann-Streiff, Pierre Robin sequence, Stickler, etc.

Primary Congenital Glaucoma

  • Typically sporadic or autosomal recessive with varying penetrance
  • In 70% of cases bilateral, male > female (3:2)
  • Symptoms: Epiphora, photophobia, blepharospasm, eye rubbing
  • Findings:
    • IOP >21mmHg (normal IOP in newborns: 10 – 12mmHg), C/D ratio >0.3
    • Corneal clouding/edema, Haab Striae
    • Buphthalmos
      • Horizontal corneal diameter >10mm in the first month, >12mm in the first year
      • Axial length >20mm in the first month, >22mm in the first year

Treatment

  • almost always initial surgery
    • Goniotomy
    • Trabeculotomy
  • Medication (temporary until surgery or in addition after surgery)
  • If unsuccessful with the above therapies: Trabeculectomy with Mitomycin C, Ahmed/Baerveldt implant, Cyclophotocoagulation, etc.

Sources

  • EyeWiki Congenital or Infantile Glaucoma
  • EyeWiki Juvenile Open Angle Glaucoma
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)