Pediatric glaucoma Flashcards
Which gene is most commonly associated with Primary Congenital Glaucoma?
The CYP1B1 gene is the most commonly associated gene with PCG.
What causes Primary Congenital Glaucoma (PCG)?
PCG is caused by genetically-determined abnormalities in the trabecular meshwork and anterior chamber angle, leading to elevated intraocular pressure (IOP
What are the three variants of PCG based on age of presentation?
The variants are newborn onset (0-1 month), infantile onset (>1-24 months), and late onset or late-recognized (>24 months).
What is the classic symptom triad associated with elevated IOP in PCG?
The classic symptom triad includes photophobia, epiphora, and blepharospasm.
What is Haab striae, and how is it related to PCG?
Haab striae are horizontal or oblique breaks in Descemet’s membrane, associated with corneal enlargement and PCG.
What are the potential outcomes for children with PCG?
Outcomes vary; some achieve good vision, while others may become blind due to corneal scarring, optic nerve damage, or amblyopia.
What is the primary treatment modality for PCG?
Surgical management is the primary treatment for controlling intraocular pressure in PCG.
What is “spontaneously arrested” PCG?
It is a rare form where classic findings like eye stretching and Haab striae are present but with normal IOP; these patients are monitored as glaucoma suspects.
How does the 2013 International Classification System for Childhood Glaucoma define childhood glaucoma?
t is defined as irreversible or reversible damage to the whole eye, not just the optic nerve, as in adults.
What can result from untreated or uncontrolled elevated IOP in PCG?
Irreversible vision loss and optic nerve damage can occur, leading to permanent corneal scarring and opacification.
How can corneal scarring affect vision in PCG?
It can obscure the visual axis, cause astigmatism, and lead to refractive amblyopia.
What intraocular pressure (IOP) is considered elevated in Primary Congenital Glaucoma?
Elevated IOP in PCG is greater than 21 mmHg.
What are Haab striae in PCG?
Haab striae are horizontal or oblique breaks in Descemet’s membrane associated with corneal edema.
At what corneal diameter should glaucoma suspicion be high in children under 1 year of age?
A corneal diameter greater than 12 mm suggests an abnormality and raises glaucoma suspicion.
What causes corneal clouding in PCG?
Elevated IOP leads to diffuse and/or focal corneal edema, causing clouding.