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.
What is buphthalmos in the context of PCG?
Buphthalmos is the enlargement of the eye due to stretching from high IOP.
What are signs of eye distension in PCG
Signs include myopia, anisometropia, astigmatism, and optic nerve cupping
How does optic nerve cupping occur in young children with PCG?
It occurs due to optic canal stretching and posterior bowing of the lamina cribrosa without neuroretinal rim loss.
What is the main surgical treatment for Primary Congenital Glaucoma?
The main surgical treatments are goniotomy or trabeculotomy to improve aqueous outflow and lower intraocular pressure (IOP)
What surgical options are available if angle surgery is unsuccessful in PCG?
Trabeculectomy enhanced with mitomycin C or glaucoma implant surgery with devices like Molteno, Baerveldt, or Ahmed implants.
What procedures can be used in refractory cases of PCG?
Cycloablation with an Nd
laser, diode laser, or cryotherapy, with the diode laser being the most commonly used.
What role does medical therapy play in the treatment of PCG?
Medical therapy is used temporarily before surgery to reduce corneal clouding and to supplement IOP control after surgery.
What is the first-line treatment for secondary congenital glaucoma?
Medical therapy is the first-line treatment for secondary congenital glaucoma.
What is the first-choice medication for pediatric glaucoma?
Timolol is the first-choice medication for pediatric glaucoma.
What combination of medications is used if timolol alone is insufficient in reducing IOP in children?
Timolol once a day combined with dorzolamide twice a day.
Why are alpha-2 agonists contraindicated in children under 2 years of age?
They can cause serious side effects like severe sleepiness, respiratory depression, and coma.
What are the potential side effects of beta-blockers like timolol in children?
Systemic side effects include respiratory distress and bradycardia.
Which oral carbonic anhydrase inhibitor is used in PCG treatment and what are its side effects?
Acetazolamide is used, with side effects like lethargy, decreased appetite, and metabolic acidosis
What is the role of prostaglandin analogs like latanoprost in PCG
Latanoprost reduces IOP by 15-20%, but is less effective in children than adults.
How do miotic agents function in PCG treatment?
Miotics are used perioperatively for angle surgery but are less effective due to immature angle anatomy in children.
When is goniotomy preferred over trabeculotomy in PCG?
Goniotomy is preferred when the cornea is clear enough to visualize anterior segment structures.
What is trabeculotomy ab externo, and when is it performed?
Trabeculotomy ab externo is performed when the cornea is not clear enough for angle visualization or due to technical factors or surgeon preference.
What is the purpose of using Mitomycin C (MMC) in trabeculectomy?
MMC is used to enhance surgical success by reducing scarring and facilitating aqueous flow
