Uveitic GlaucomaUveitic GlaucomaEvaluation and ManagementEvaluation and Management

Howard Kane MD,ODHoward Kane MD,ODMay 22, 2016May 22, 2016

ObjectivesObjectives

• Review the pathophysiology of Uveitic Review the pathophysiology of Uveitic GlaucomaGlaucoma

• Recognize the signs and symptoms of Recognize the signs and symptoms of Uveitic GlaucomaUveitic Glaucoma

• Discuss management options in Uveitic Discuss management options in Uveitic GlaucomaGlaucoma

• Detail several specific Uveitic Glaucoma Detail several specific Uveitic Glaucoma SyndromesSyndromes

General PrinciplesGeneral Principles• Uveitis is the most common of the Uveitis is the most common of the

inflammatory processes which affect the inflammatory processes which affect the eye.eye.

• Most of the acute cases (< 3 months duration) Most of the acute cases (< 3 months duration) involve the anterior uvea (iritis or iridocyclitis)involve the anterior uvea (iritis or iridocyclitis)

• Chronic forms are seen in the following Chronic forms are seen in the following frequencies: Anterior (45%), intermediate frequencies: Anterior (45%), intermediate (15%), posterior (14%) and panuveitis (24%)(15%), posterior (14%) and panuveitis (24%)

• Iridocyclitis most common form causing Iridocyclitis most common form causing increased IOPincreased IOP

• 10% of pts with uveitis will develop 10% of pts with uveitis will develop OHT/OAGOHT/OAG

PathophysiologyPathophysiology Secondary OAGSecondary OAG

•Obstruction of TM by inflammatory debris, Obstruction of TM by inflammatory debris, RBCs, WBCs, fibrin, viscous inflammatory RBCs, WBCs, fibrin, viscous inflammatory aqueousaqueous

•Direct inflammation and swelling of the TM Direct inflammation and swelling of the TM endothelial cells (trabeculitis)endothelial cells (trabeculitis)

•Steroid responsivenessSteroid responsiveness•Formation of vascular or cuticular Formation of vascular or cuticular

membranes overlying the TM as a result of membranes overlying the TM as a result of chronic recurrent Inflammationchronic recurrent Inflammation

PathophysiologyPathophysiology• Secondary ACGSecondary ACG

•Pupillary block secondary to posterior synechiaePupillary block secondary to posterior synechiae•Extensive peripheral anterior synechiaeExtensive peripheral anterior synechiae•Choroidal/ciliary effusions with anterior rotation Choroidal/ciliary effusions with anterior rotation

of the ciliary body and resultant angle closureof the ciliary body and resultant angle closure•Neovascular glaucomaNeovascular glaucoma

•

General PrinciplesGeneral PrinciplesIOP in uveitis depends on a delicate IOP in uveitis depends on a delicate balance between aqueous secretion by balance between aqueous secretion by the ciliary body and aqueous outflow at the ciliary body and aqueous outflow at the TM and the effects of the specific the TM and the effects of the specific inflammatory event on these structures.inflammatory event on these structures.• Basic Principles of glaucoma management Basic Principles of glaucoma management

in uveitis patientsin uveitis patients•Elevated IOP often complicates cases of anterior Elevated IOP often complicates cases of anterior

segment inflammation, rarely in posterior or segment inflammation, rarely in posterior or intermediate Uveitisintermediate Uveitis

General PrinciplesGeneral Principles• It is uncommon for acute anterior uveitis of short It is uncommon for acute anterior uveitis of short

duration (< 3 months) to cause persistent IOP elevationduration (< 3 months) to cause persistent IOP elevation• The etiologic agent for specific cases of uveitis is The etiologic agent for specific cases of uveitis is

frequently unknown, therefore treatment is often frequently unknown, therefore treatment is often nonspecific, aimed at suppressing inflammation.nonspecific, aimed at suppressing inflammation.

• Full medical workup generally done in bilateral, Full medical workup generally done in bilateral, persistent and recurrent cases. Low yield in initial, mild persistent and recurrent cases. Low yield in initial, mild unilateral cases. unilateral cases.

• Focused Hx/ROS should be performed Focused Hx/ROS should be performed • Full physical, CXR, SI joint films, ACE levels, HLA-B27, Full physical, CXR, SI joint films, ACE levels, HLA-B27,

RPR, FTA-ABS, ANA, RF, HIV.RPR, FTA-ABS, ANA, RF, HIV.• HLA B27 positive accounts for 50% of cases in HLA B27 positive accounts for 50% of cases in

Caucasians, predominantly younger men Caucasians, predominantly younger men • Due to the delicate balance between alterations in Due to the delicate balance between alterations in

aqueous production and outflow, IOP in uveitic glaucoma aqueous production and outflow, IOP in uveitic glaucoma may fluctuate greatly.may fluctuate greatly.

• Signs and Signs and symptomssymptoms

• Wide rangeWide range• Pain, photophobia, Pain, photophobia,

tearing, decreased VAtearing, decreased VA• Ciliary flush, miosis, cell Ciliary flush, miosis, cell

& flare, KPs, AC, & flare, KPs, AC, vitreous cells, vitreous cells,

• Ant/post synechiae, iris Ant/post synechiae, iris atrophy, Band atrophy, Band keratopathy, corneal keratopathy, corneal edema and IOP edema and IOP changes.changes.

• Signs and symptomsSigns and symptoms

• Wide rangeWide range• Pain, photophobia, tearing, decreased VAPain, photophobia, tearing, decreased VA• Ciliary flush, miosis, cell & flare, KPs, ant Ciliary flush, miosis, cell & flare, KPs, ant

vitreous cells, hypopion vitreous cells, hypopion • Ant/post synechiae, iris atrophy, Band Ant/post synechiae, iris atrophy, Band

keratopathy, corneal edema and IOP changes.keratopathy, corneal edema and IOP changes.

Treatment Principles for Uveitic Treatment Principles for Uveitic GlaucomaGlaucoma• Treatment is aimed at reducing the acute Treatment is aimed at reducing the acute

inflammation and controlling the IOP.inflammation and controlling the IOP.• A further long term treatment goal is to A further long term treatment goal is to

prevent any permanent structural damage prevent any permanent structural damage that could predispose the eye to that could predispose the eye to secondary conditions such as cataract, secondary conditions such as cataract, corneal decompensation and glaucoma.corneal decompensation and glaucoma.

• Specific Tx required in certain casesSpecific Tx required in certain cases– eg: ABx for STD, TB, Toxoplasmosiseg: ABx for STD, TB, Toxoplasmosis

Treatment Principles for Uveitic Treatment Principles for Uveitic GlaucomaGlaucoma• Prompt identification of the mechanism of IOP elevation and Prompt identification of the mechanism of IOP elevation and

treatment of the underlying cause.treatment of the underlying cause.• Cycloplegics and corticosteroids are the mainstays of Cycloplegics and corticosteroids are the mainstays of

treatment to control inflammation and prevent synechiae.treatment to control inflammation and prevent synechiae.– IOP must be closely monitored while on steroidsIOP must be closely monitored while on steroids– Steroid potency: Steroid potency:

Difluprednate(Durezol)>Dexamethasone>Prednisolone>Difluprednate(Durezol)>Dexamethasone>Prednisolone> Loteprednol (Lotemax)>FML>AlrexLoteprednol (Lotemax)>FML>Alrex– Route of administrationRoute of administration– Steroids must be tapered.Steroids must be tapered.

• Topical NSAIDS may be used adjunctively in patients who Topical NSAIDS may be used adjunctively in patients who are known steroid responders.are known steroid responders.

• Systemic immunosuppresive Tx successful in 70% of Systemic immunosuppresive Tx successful in 70% of patients unresponsive to other Txpatients unresponsive to other Tx

Treatment Principles for Uveitic Treatment Principles for Uveitic GlaucomaGlaucoma• Select target IOP based on level and duration of IOP elevation, Select target IOP based on level and duration of IOP elevation,

presence and degree of optic nerve damage, central corneal presence and degree of optic nerve damage, central corneal thickness.thickness.

• Elevated IOP initially treated with B blockers and CAIs, both topical Elevated IOP initially treated with B blockers and CAIs, both topical and systemic.and systemic.

• Alpha agonists also effective in these patients, although there have Alpha agonists also effective in these patients, although there have been reports of a granulomatous anterior uveitis in patients taking been reports of a granulomatous anterior uveitis in patients taking brominidine 0.2%brominidine 0.2%

• Miotics are contraindicated.Miotics are contraindicated.• PGAs are probably effective. Anecdotal reports of CME, ant uveitis.PGAs are probably effective. Anecdotal reports of CME, ant uveitis.• Laser trabeculoplasty generally ineffective in uveitic glaucoma and Laser trabeculoplasty generally ineffective in uveitic glaucoma and

may result in IOP spikes.may result in IOP spikes.• Laser PI is the treatment of choice for RPB although associated with a Laser PI is the treatment of choice for RPB although associated with a

higher rate of secondary closure.higher rate of secondary closure.

Treatment Principles for Uveitic Treatment Principles for Uveitic GlaucomaGlaucoma• Goniosynechiolysis, both laser and surgical, may be Goniosynechiolysis, both laser and surgical, may be

effective in reversing PAS that have been present effective in reversing PAS that have been present for under a year. for under a year.

• Trabeculectomy with antimetabolite is effective but Trabeculectomy with antimetabolite is effective but should be done in a setting of as little inflammation should be done in a setting of as little inflammation as possible. 60-65% 5 yr success rate.as possible. 60-65% 5 yr success rate.

• Tube shunt surgery may also be effective in these Tube shunt surgery may also be effective in these eyes. Slightly higher success rate than eyes. Slightly higher success rate than Trabeculectomy but often need medications.Trabeculectomy but often need medications.

• Cyclodestructive surgery an option in eyes with Cyclodestructive surgery an option in eyes with poor visual potential.poor visual potential.

Treatment Principles for Treatment Principles for Uveitic GlaucomaUveitic Glaucoma

Ahmed Glaucoma ValveAhmed Glaucoma Valve

Common Uveitic Entities Common Uveitic Entities Associated with GlaucomaAssociated with Glaucoma• Fuchs Heterochromic Iridocyclitis (FHI)Fuchs Heterochromic Iridocyclitis (FHI)• Chronic low grade inflammatory condition Chronic low grade inflammatory condition

characterized by heterochromia, KPs, vitreous characterized by heterochromia, KPs, vitreous opacities, cataract and glaucoma.opacities, cataract and glaucoma.

• There is no gender or ethnic predisposition.There is no gender or ethnic predisposition.• Unilateral in 95% of cases.Unilateral in 95% of cases.• Patients usually present with a white, quiet eye Patients usually present with a white, quiet eye

complaining of decreased VA due to cataract.complaining of decreased VA due to cataract.• Minimal flare and cell present and fine stellate Minimal flare and cell present and fine stellate

KPsKPs• Stromal iris atrophy present with iris TI defects.Stromal iris atrophy present with iris TI defects.

Fuchs Hetereochromic IridocyclitisFuchs Hetereochromic Iridocyclitis• Development of OAG in 20-40% of patients.Development of OAG in 20-40% of patients.• Most commonly misdiagnosed form of uveitis.Most commonly misdiagnosed form of uveitis.• Accounts for 2-5% of all uveitis cases.Accounts for 2-5% of all uveitis cases.• Heterochromia may be missed in AA patientsHeterochromia may be missed in AA patients• Glaucoma develops gradually and often late in Glaucoma develops gradually and often late in

the course of the disease.the course of the disease.

• FuchsFuchs Heterochromic Heterochromic IridocyclitisIridocyclitis

• PAS, posterior synechiae are PAS, posterior synechiae are unusual unusual • Light NV of the iris and Light NV of the iris and

AC angleAC angle• Fine iris nodules occur in Fine iris nodules occur in

20% of patients20% of patients• FA shows delayed filling, FA shows delayed filling,

sector ischemia and NV of sector ischemia and NV of the iris.the iris.

• Iris transillumination may Iris transillumination may be present.be present.

• Management of FHIManagement of FHI• Cataract surgery in these patients is relatively Cataract surgery in these patients is relatively

routine and is generally associated with good routine and is generally associated with good visual outcomes. Higher incidence of post op visual outcomes. Higher incidence of post op CME, IOP spikes, hyphema and PO uveitis.CME, IOP spikes, hyphema and PO uveitis.

• GlaucomaGlaucoma• Incidence is greater with longer term follow upIncidence is greater with longer term follow up• Does not respond to aggressive steroid therapy and Does not respond to aggressive steroid therapy and

may, in fact, develop steroid induced glaucoma if may, in fact, develop steroid induced glaucoma if used inappropriately.used inappropriately.

• Laser trabeculoplasty is ineffective in FHILaser trabeculoplasty is ineffective in FHI• Standard glaucoma medications are useful but often Standard glaucoma medications are useful but often

ineffective but often ineffective in the long term.ineffective but often ineffective in the long term.• Avoid pilocarpine. Avoid pilocarpine.

• Management of FHI (cont)Management of FHI (cont)• GlaucomaGlaucoma

•Trabeculectomy c MMC as well as tube Trabeculectomy c MMC as well as tube shunt procedures have good success rates shunt procedures have good success rates in a majority of these patients if medical in a majority of these patients if medical treatment fail.treatment fail.

Common Uveitis Entities Common Uveitis Entities Associated with GlaucomaAssociated with Glaucoma• Glaucomatocyclitic Crisis (Posner-Schlossman Glaucomatocyclitic Crisis (Posner-Schlossman

Syndrome)Syndrome)• Characterized by recurrent unilateral attacks of Characterized by recurrent unilateral attacks of

mild anterior uveitis with marked elevations of IOPmild anterior uveitis with marked elevations of IOP• Young to middle aged adults, age 20-50 yo.Young to middle aged adults, age 20-50 yo.• Symptoms, slight in relation to the level of IOP, Symptoms, slight in relation to the level of IOP,

include slight ocular discomfort, blurred vision and include slight ocular discomfort, blurred vision and halos lasting several hours to weeks. Recurrences halos lasting several hours to weeks. Recurrences monthly to yearly. monthly to yearly.

• Mild ciliary flush, epithelial edema, faint flare and Mild ciliary flush, epithelial edema, faint flare and scant KPs. Angle is open. Absence of synechiae.scant KPs. Angle is open. Absence of synechiae.

• There is an association with subsequent POAG.There is an association with subsequent POAG.• Etiology unknownEtiology unknown

• Glaucomatocyclitic CrisisGlaucomatocyclitic Crisis• GlaucomaGlaucoma

• IOP typically elevated into the 40-60 mmHg range.IOP typically elevated into the 40-60 mmHg range.• IOP and facility of outflow return to normal between attacks.IOP and facility of outflow return to normal between attacks.• Typically normal appearing ONH and visual fields, early on.Typically normal appearing ONH and visual fields, early on.• GCC is a self limiting condition.GCC is a self limiting condition.• Corticosteroids are beneficial in controlling the inflammatory Corticosteroids are beneficial in controlling the inflammatory

process (Unlike FHI)process (Unlike FHI)• CAIs, B-blockers and Alpha agonists are all effective in CAIs, B-blockers and Alpha agonists are all effective in

controlling IOP during acute attacks. Prophylactic controlling IOP during acute attacks. Prophylactic antiglaucoma meds and corticosteroids are not necessary antiglaucoma meds and corticosteroids are not necessary between attacks.between attacks.

• Glaucomatocyclitic CrisisGlaucomatocyclitic Crisis• NSAIDS ? Study showed benefits of IndomethacinNSAIDS ? Study showed benefits of Indomethacin• Glaucoma surgical procedures may be indicated if Glaucoma surgical procedures may be indicated if

severe or prolonged attacks lead to progressive ON severe or prolonged attacks lead to progressive ON damage.damage.

Common Uveitis Entities Common Uveitis Entities Associated with GlaucomaAssociated with Glaucoma

• SarcoidosisSarcoidosis• A multi system inflammatory disorder with a A multi system inflammatory disorder with a

predilection for young adults and African predilection for young adults and African Americans.Americans.

• Pathology consists of noncaseating granulomas Pathology consists of noncaseating granulomas involving the lungs, liver, spleen, skin, eyes involving the lungs, liver, spleen, skin, eyes and CNS.and CNS.

• Glaucoma occurred in 10% of pts c Sarcoid Glaucoma occurred in 10% of pts c Sarcoid related iridocyclititisrelated iridocyclititis

Common Uveitis Entities Common Uveitis Entities Associated with GlaucomaAssociated with Glaucoma

• SarcoidosisSarcoidosis..• Ocular manifestations occur in Ocular manifestations occur in

38-50% of sarcoidosis patients 38-50% of sarcoidosis patients and include anterior uveitis and include anterior uveitis (most common), (most common), chorioretinitis, retinal chorioretinitis, retinal periphlebitis, optic neuritis and periphlebitis, optic neuritis and lacrimal gland involvement.lacrimal gland involvement.

• Diagnosis is made by Chest X-Diagnosis is made by Chest X-ray (hilar lymphadenopathy), ray (hilar lymphadenopathy), ACE levels and lymph node or ACE levels and lymph node or skin biopsyskin biopsy

• Sarcoid UveitisSarcoid Uveitis

• Both an acute form and Both an acute form and chronic relapsing form.chronic relapsing form.

• Initially unilateral with an Initially unilateral with an insidious onset it frequently insidious onset it frequently develops into a chronic develops into a chronic phase often becoming phase often becoming bilateral.bilateral.

• Characterized by mutton fat Characterized by mutton fat KPs, iris nodules (Busacca KPs, iris nodules (Busacca and Koeppe), nodules in the and Koeppe), nodules in the AC angle and synechiae.AC angle and synechiae.

• Sarcoid GlaucomaSarcoid Glaucoma

• Glaucoma is more commonly associated with the Glaucoma is more commonly associated with the chronic relapsing form of sarcoid anterior uveitis chronic relapsing form of sarcoid anterior uveitis which has a worse visual prognosis.which has a worse visual prognosis.

• Corticosteroids are generally effective in the Corticosteroids are generally effective in the treatment of systemic and ocular sarcoidosis.treatment of systemic and ocular sarcoidosis.

• CycloplegicsCycloplegics• CAIs, B-Blockers and Alpha agonists effective to CAIs, B-Blockers and Alpha agonists effective to

control IOP. As always avoid miotics. PGAs control IOP. As always avoid miotics. PGAs possible.possible.

• Trabeculectomy c MMC, tube surgery. Expect Trabeculectomy c MMC, tube surgery. Expect prolonged course and taper of corticosteroids in prolonged course and taper of corticosteroids in these cases. Treatment can include lower potency these cases. Treatment can include lower potency steroids and NSAIDS.steroids and NSAIDS.