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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)