Pediatric glaucoma Flashcards

1
Q

Which gene is most commonly associated with Primary Congenital Glaucoma?

A

The CYP1B1 gene is the most commonly associated gene with PCG.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What causes Primary Congenital Glaucoma (PCG)?

A

PCG is caused by genetically-determined abnormalities in the trabecular meshwork and anterior chamber angle, leading to elevated intraocular pressure (IOP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the three variants of PCG based on age of presentation?

A

The variants are newborn onset (0-1 month), infantile onset (>1-24 months), and late onset or late-recognized (>24 months).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the classic symptom triad associated with elevated IOP in PCG?

A

The classic symptom triad includes photophobia, epiphora, and blepharospasm.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is Haab striae, and how is it related to PCG?

A

Haab striae are horizontal or oblique breaks in Descemet’s membrane, associated with corneal enlargement and PCG.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the potential outcomes for children with PCG?

A

Outcomes vary; some achieve good vision, while others may become blind due to corneal scarring, optic nerve damage, or amblyopia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the primary treatment modality for PCG?

A

Surgical management is the primary treatment for controlling intraocular pressure in PCG.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is “spontaneously arrested” PCG?

A

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 well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How does the 2013 International Classification System for Childhood Glaucoma define childhood glaucoma?

A

t is defined as irreversible or reversible damage to the whole eye, not just the optic nerve, as in adults.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What can result from untreated or uncontrolled elevated IOP in PCG?

A

Irreversible vision loss and optic nerve damage can occur, leading to permanent corneal scarring and opacification.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

How can corneal scarring affect vision in PCG?

A

It can obscure the visual axis, cause astigmatism, and lead to refractive amblyopia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What intraocular pressure (IOP) is considered elevated in Primary Congenital Glaucoma?

A

Elevated IOP in PCG is greater than 21 mmHg.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are Haab striae in PCG?

A

Haab striae are horizontal or oblique breaks in Descemet’s membrane associated with corneal edema.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

At what corneal diameter should glaucoma suspicion be high in children under 1 year of age?

A

A corneal diameter greater than 12 mm suggests an abnormality and raises glaucoma suspicion.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What causes corneal clouding in PCG?

A

Elevated IOP leads to diffuse and/or focal corneal edema, causing clouding.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is buphthalmos in the context of PCG?

A

Buphthalmos is the enlargement of the eye due to stretching from high IOP.

17
Q

What are signs of eye distension in PCG

A

Signs include myopia, anisometropia, astigmatism, and optic nerve cupping

18
Q

How does optic nerve cupping occur in young children with PCG?

A

It occurs due to optic canal stretching and posterior bowing of the lamina cribrosa without neuroretinal rim loss.

19
Q

What is the main surgical treatment for Primary Congenital Glaucoma?

A

The main surgical treatments are goniotomy or trabeculotomy to improve aqueous outflow and lower intraocular pressure (IOP)

20
Q

What surgical options are available if angle surgery is unsuccessful in PCG?

A

Trabeculectomy enhanced with mitomycin C or glaucoma implant surgery with devices like Molteno, Baerveldt, or Ahmed implants.

21
Q

What procedures can be used in refractory cases of PCG?

A

Cycloablation with an Nd
laser, diode laser, or cryotherapy, with the diode laser being the most commonly used.

22
Q

What role does medical therapy play in the treatment of PCG?

A

Medical therapy is used temporarily before surgery to reduce corneal clouding and to supplement IOP control after surgery.

23
Q

What is the first-line treatment for secondary congenital glaucoma?

A

Medical therapy is the first-line treatment for secondary congenital glaucoma.

24
Q

What is the first-choice medication for pediatric glaucoma?

A

Timolol is the first-choice medication for pediatric glaucoma.

25
Q

What combination of medications is used if timolol alone is insufficient in reducing IOP in children?

A

Timolol once a day combined with dorzolamide twice a day.

26
Q

Why are alpha-2 agonists contraindicated in children under 2 years of age?

A

They can cause serious side effects like severe sleepiness, respiratory depression, and coma.

27
Q

What are the potential side effects of beta-blockers like timolol in children?

A

Systemic side effects include respiratory distress and bradycardia.

28
Q

Which oral carbonic anhydrase inhibitor is used in PCG treatment and what are its side effects?

A

Acetazolamide is used, with side effects like lethargy, decreased appetite, and metabolic acidosis

29
Q

What is the role of prostaglandin analogs like latanoprost in PCG

A

Latanoprost reduces IOP by 15-20%, but is less effective in children than adults.

30
Q

How do miotic agents function in PCG treatment?

A

Miotics are used perioperatively for angle surgery but are less effective due to immature angle anatomy in children.

31
Q

When is goniotomy preferred over trabeculotomy in PCG?

A

Goniotomy is preferred when the cornea is clear enough to visualize anterior segment structures.

32
Q

What is trabeculotomy ab externo, and when is it performed?

A

Trabeculotomy ab externo is performed when the cornea is not clear enough for angle visualization or due to technical factors or surgeon preference.

33
Q

What is the purpose of using Mitomycin C (MMC) in trabeculectomy?

A

MMC is used to enhance surgical success by reducing scarring and facilitating aqueous flow