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European Glaucoma Society Terminology and Guidelines for Glaucoma, 4th Edition - Chapter 2: Classification and terminology
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2 Classification and Terminology

2.1 - Primary Congenital Forms/Childhood Glaucomas

Primary congenital glaucoma is a rare disease but has a major impact on the child’s development and quality of life over his/her whole life span. Early diagnosis and appropriate therapy can make a huge difference in the visual outcome and can prevent lifelong disability. Surgical treatment is always necessary1, 2 [I,C].

2.1.1 Primary congenital glaucoma (PCG): from birth to >2 years of life

  1. Neonatal or newborn onset (0-1 month)

  2. Infantile onset (>1 until 24 months)

  3. Late onset or late recognized (>2 years)

  4. Spontaneously non-progressing cases with normal IOP but typical signs of PCG may be classified as PCG

Etiology:

Angle dysgenesis is caused by incomplete development of the trabecular meshwork before and/or after birth. Strong monogenetic influence. Heredity shows recessive inheritance with variable penetrance in most cases or is sporadic. Specific chromosomal abnormalities have been identified at chromosomes 1p36 and 2q212.

Pathogenic mechanism:

Decreased aqueous outflow causes significant elevation of IOP. Isolated trabeculodysgenesis is the most common form of primary congenital glaucoma.

Epidemiology:

Congenital glaucoma occurs in about 1 in 12-18,000 births among Caucasians. Incidence can be 5 to 10 times higher if consanguinity of parents is present. Severe visual disability is common. PCG is more common in males (65%), and is bilateral in 70% of patients.

Symptoms:

Crying unhappy child during first weeks or year of life. Not always symptomatic.

Signs:

Photophobia, tearing, blepharospasm, and eye rubbing are typical early signs.

Eyes are larger compared to age, with corneal diameter usually >10.5 mm at birth and >12 mm in the first year of life. Axial length is increased, >20 mm at birth or >22 mm after 1 year. Corneal oedema is frequent; epithelial and stromal oedema can de associated with ruptures of Descemet’s Membrane, or Haab’s striae, not to be confused with forceps delivery trauma.

IOP can sometimes be measured in the awaked child with hand-held tonometers. …

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