9 - secondary glaucomas

What is pseudo exfoliation syndrome (PXE)?

systemic condition associated with abnormal basement membranes due to mutation in LOXL 1 gene

Who gets pseudoexfoliation syndrome (PXE)?

caucasians

60-80 years

What are the systemic associations with pseudoexfoliation syndrome (PXE)?

1. vascular insuffienceny

2. TIAs

3. heart attack

4. stroke

5. alzheimers

What is the pathogenesis of pseudoexfoliation syndrome (PXE)?

weakened BM causes loss of epi cells from iris + CB, these cells slough off + accumulate in the anterior chamber/neighboring tissue

What are the signs of pseudoexfoliation syndrome (PXE)?

1. bulls-eye on lens

2. accumulation of material + pupil margin

3. peripupillary TIDs

4. frayed/broken zonules --> lens subluxation

5. pigment in angle

6. difficult cataract sx

Who gets pseudoexfoliation glaucoma (PXG)? What percentage of the disease and what sex?

1. 60% of those with PXE

2. more women

What is the pathogenesis of pseudoexfoliation glaucoma (PXG)?

sticky deposition clogging the TM + UVS

How does pseudoexfoliation glaucoma (PXG) compare to POAG?

1. PXG has higher IOP with more fluctuations

2. PXG has worse ONH + VF at time of diagnosis

3. PXG progresses more rapidly

4. PXG has greater risk of treatment failure

What is the first line treatment for pseudoexfoliation glaucoma (PXG)?

PGAs or ALT/SLT

sx can delay need for medical managment by 8 years

What treatment options are used in pseudoexfoliation glaucoma (PXG)?

1. PGAs

2. ALT/SLT

3. pilocarpine (if PGA fails)

don't use PGAs and pilocarpine together --> CB muscle fibers contract, limiting ECM for PGAs to carryout MOA

What concentration of pilocarpine should be used to treat pseudoexfoliation glaucoma (PXG)?

2%

unless darker iris

What treatments are avoided in pseudoexfoliation glaucoma (PXG)?

1. cataract surgery

2. aqueous suppressors

Why is cat sx difficult with pseudoexfoliation glaucoma?

1. compromised zonules and capsules

2. poor pupil dilation

How can IOP be affected with uveitis?

can increase IOP

BUT

initially can decrease IOP due to cyclists (sick CB) causing decreased AH production

What are the ways that uveitis can increase IOP?

1. increase aqueous viscosity

2. trabeculitis

3. PAS

4. posterior synechiae/iris bombe/angle closure

5. accumulation of cells + inflam material in TM

What is the treatment for uveitis causing glaucoma?

treat inflammation: steroids, cycloplegics

treat IOP (if >30) add aqueous suppressors

What meds are contraindicated in uveitic glaucoma?

1. PGAs

2. pilocarpine

Why is pilocarpine contraindicated for uveitic glaucoma?

compromises the blood-brain barrier

What is the age of onset of Fuch's Heterochromic iridocyclitis?

20-40 years old

What is the cause of Fuch's Heterochromic iridocyclitis?

unknown, potentially rubella

trabeculitis causing increased IOP

What type of glaucoma is Fuch's Heterochromic iridocyclitis?

uveitic glaucoma

What is the triad for Fuch's Heterochromic iridocyclitis?

1. iris heterochromia

2. cyclitis (CB inflammation)

3. cataract

What are the signs of Fuch's Heterochromic iridocyclitis?

triad: heterochromia, cycltitis, cataract

1. unilateral mild uveitis

2. white eye

3. fine stellate diffuse KPs on corneal endothelium

4. angle neovascularization

5. posterior subcapsular cataract

What are the symptoms of Fuch's Heterochromic iridocyclitis?

asymptomatic unless cataract affecting vision

What kind of cataract is seen in Fuch's Heterochromic iridocyclitis?

PSC initially

What is the treatment of Fuch's heterochromic iridocyclitis?

1. aqueous suppressors

2. ALT/SLT

3. steroids for short-term mod/sev episodes

uveitic glaucoma -> contraindications: PGAs + pilocarpine

What is the only uveitic glaucoma treated with ALT/SLT?

Fuch's heterochromic iridocyclitis

What is glaucomatocyclitic crisis (aka posner-schlossman syndrome)?

special type of unilateral uveitic glaucoma causing severe IOP increase (40-60) with mild uveitis

Who gets glaucomatocyclitic crisis (posner-schlossman syndrome)?

1. young to middle aged

2. males

3. those who have had it before (prone to recurrence)

What is the pathogenesis of glaucomatocyclitic crisis (posner-schlossman syndrome)?

trabeculitis

What are the signs of glaucomatocyclitic crisis (posner-schlossman syndrome)?

1. IOP ≥40

2. mild anterior uveitis with white or trace injection eye

3. mild corneal edema

4. open angle with PAS

What are the symptoms of glaucomatocyclitic crisis (posner-schlossman syndrome)?

1. mild discomfort

2. mild blurred or distorted vision

What is the treatment for glaucomatocyclitic crisis (posner-schlossman syndrome)?

in office: treat IOP spike with B-blocker +/or alpha-2 until IOP below 30 mmHg

at home:

1. B-blocker QAM x 1 week

2. Pred Forte QID or Durezol BID x 1 week

What causes UGH (uveitis-glaucoma-hyphema) syndrome?

complication of ACIOL or iris supported IOL causing iris chafing - sub-class of uveitic glaucoma

When does UGH (uveitis-glaucoma-hyphema) syndrome onset?

weeks to months after cataract surgery

What are the signs of UGH (uveitis-glaucoma-hyphema) syndrome?

triad: uveitis, glaucoma, hyphema

What is the treatment for UGH (uveitis-glaucoma-hyphema) syndrome?

steroids and topical glaucoma meds for mild forms, but usually IOL removal

What is traumatic glaucoma?

acute or chronic rises in IOP associated with blunt trauma to the eye

Who gets traumatic glaucoma?

1. young males

2. lower socioeconomic groups

What are the causes of traumatic glaucoma?

1. play

2. sports

3. assaults

4. occupational accidents

What are the signs of early stage traumatic glaucoma?

traumatic uveitis +/or hyphema

When does late stage traumatic glaucoma onset?

develops months to years after initial injury

What is the treatment for early stage traumatic glaucoma?

uveitis: topical steroid, cycloplegic, beta-blockers

hyphema: cycloplegia with 1% atropine, AH suppressors

What is the leading cause of unilateral glaucoma?

traumatic glaucoma

What are the two types of late stage traumatic glaucoma?

1. angle recession

2. ghost cell

How much of the angle must be affected in angle recession glaucoma to cause clinically significant IOP?

180º of angle

What causes increased IOP in angle recession glaucoma?

disruption of TM

What is the treatment for angle recession glaucoma?

aqueous suppressors only

why? - outflow mechanism is completely negated

What is the appearance of angle recession glaucoma?

widening of CB on gonio

What is the cause of ghost cell glaucoma?

RBCs from vitreous heme blocking the TM

What are the signs of ghost cell glaucoma?

1. RBCs in inferior angle in gonio

2. "candy striped" sign

What is the treatment of ghost cell glaucoma?

B-blockers

What are the causes of neovascular glaucoma?

1. ischemic CRVO (90 day glaucoma) - most common

2. PDR

3. ocular ischemic syndrome

What is the pathogenesis of neovascular glaucoma?

retinal ischemia causes iris neo that spreads to angle, fibrovascular membrane forms & contracts causing angle closure

What are the signs of neovascular glaucoma?

1. NVI on pupil margin

2. fibrovascular membrane in the angle - crispy vessels

What is the treatment for neovascular glaucoma?

1. PRP

2. anti-VEGF (Avastin)

3. aqueous suppressors

PAIN

- cycloplegics (atropine)

- steroids

often requires advanced surgical intervention (trabeculectomy, tubes, and shunts)

What is the pathogenesis of phacomorphic glaucoma?

mature cataract mechanically pushing iris forward causing shallow anterior chamber

increases IOP by obscuring angle or pupillary block

What are the signs of phacomorphic glaucoma?

1. mature cataract

2. shallow anterior chamber

What is the treatment for phacomorphic glaucoma?

cataract surgery

What is the cause of phacolytic glaucoma?

leakage of lens proteins due to mature/hypermature cataract (foreign substance)

What is the pathogenesis of phacolytic glaucoma?

proteins impede outflow through TM, inducing an immune response

What are the signs of phacolytic glaucoma?

1. mature cataract

2. increased IOP

3. white, puffy debris in AC

4. cells and flare

What is the treatment for phacolytic glaucoma?

1. cat sx

2. aqueous suppressors

3. topical steroid

What is the cause of lens particle glaucoma?

release of lens material into AH due to cataract surgery or penetrating trauma

What is the pathogenesis of lens particle glaucoma?

lens particles collect in angle + obstruct TM outflow

What are the signs of lens particle glaucoma?

1. white puffy debris in anterior chamber

2. cells + flare

What is the treatment for lens particle glaucoma?

1. aqueous suppressors

2. topical steroids

possible surgical removal of retained cortical material

what is pigmentary dispersion syndrome?

release of pigment granules from posterior epithelium of iris

What is the onset of pigmentary dispersion syndrome? When are they diagnosed?

onset ~ mid teens

dx at 20-40 years

Which sex converts more to pigmentary glaucoma?

males > females (3:1 ratio)

What is the refractive error of the majority of pigmentary glaucoma patients?

myopic with deep anterior chamber

What is the pathogenesis of pigmentary dispersion syndrome?

posterior bowing iris (concave) causes mechanical friction of iris against zones causing iris pigment release

1. literal blockage

2. may compromise the TM endothelial by forcing them to phagocytize the pigment granules instead of facilitating active transport

What are the signs of pigmentary dispersion syndrome?

1. radial mid periphery TID

2. krukenberg spindle

3. sampaolesi's line

4. zentamyer's line - pathognomonic

5. heavily pigmented angle

What is krukenberg's spindle?

liberated pigment accumulates on posterior surface of the corneal endothelium

not pathognomonic

What is zentamyer's line?

pigment granules stuck to the zonules or peripheral anterior lens surface

pathognomonic for PDS

Why is gonioscopy required when you observe krukenberg spindle or iris transillumination defects?

strongly suggests the presence of pigment dispersion syndrome (PDS), or its more advanced form, pigmentary glaucoma (PG)

not pathognomonic

Why is the risk of conversion in pigmentary dispersion syndrome low around 45?

1. burnout: iris has no more pigment to release

2. lens growth forces the iris away from zones

risk of IOP spike nad conversion to PG decreases with age

What IOP checks should be done for a newly diagnosed pigmentary dispersion syndrome patient?

if newly dx and less than 45 years old

1. IOP checks every 3 months for first year, then every 6 months after that

2. IOP check immediate after workout

3. IOP check post-dilation

What is the treatment for pigmentary glaucoma?

treat like POAG

1. PGAs

2. ALT/SLT

3. LPI for <45 yo - decreases peripheral iris concavity

young patients

pilocarpine: increase outflow and decreases the concave configuration of the iris

What are the side effects of pilocarpine?

1. pre-presbyopes: accommodative spasms, miosis, blurred vision

2. retinal detachment

Who gets steroid induced glaucoma?

1. older adults

2. those with glaucoma

3. those with FHx of glaucoma

What is the onset of steroid induced glaucoma?

typically 10 days after steroid use, but can also be seen anytime from 1-4 weeks

What genes is steroid-induced glaucoma associated with?

1. MYOC

2. GLC1A

3. TIGR

What is the pathogenesis of steroid induced glaucoma?

decrease outflow due to changes in the microstructure of TM

What is the treatment for steroid induced glaucoma?

1. stop/taper steroid

OR

2. switch to less penetrative steroid

OR

3. treat with B-blocker or other AH suppressor until off steroid

What are steroid responders more at risk for developing?

steroid responders are at an increased risk for developing POAG

What percentage of steroid responders end up developing POAG later in life?

30%

What glaucoma med class do you want for any secondary glaucoma that is not PG or PXG?

aqueous suppressors not outflow enhancers

use PGAs for PG and PXG