NEOVASCULAR GLAUCOMA /

AQUEOUS MISDIRECTION /
UVEITIC GLAUCOMA

CHAIRPERSON - DR.A.K.GUPTA
MODERATOR - DR.REENA CHOUDHRY
PRESENTOR - DR. GAURAV SHUKLA
NEOVASCULAR GLAUCOMA

 Definition
 History
 Etiology
 Pathophysiology
 Clinical course
 Clinical features
 D/D’S
 Investigations
 Treatment
 DEFINITION-

 Severe form of secondary glaucoma characterised by fibro

vascular proliferation in the anterior chamber angle

 ALSO KNOWN AS-

 1. Thrombotic glaucoma
 2. Rubeotic glaucoma
 3. Congestive glaucoma
 4.Hemorrhagic glaucoma
 HISTORY-

 1906 - Coats , NVI in CRVO termed as RUBEOSIS IRIDIS

 1937 - Kurtz , NVA leading to PAS formation

 1963 - Weiss et al, coined the term NEOVASCULAR GLAUCOMA

 ETIOLOGY-

 Proliferative diabetic retinopathy (M.C.C)- approx. 33%

 CRVO - 28% (CRVO>CRAO)

 Ocular ischemic disease d/t carotid artery obstruction-13%

 Others-
 PATHOPHYSIOLOGY-

 CHRONIC RETINAL ISCHAEMIA

 ANGIOGENIC FACTORS RELEASED &

DIFFUSED

 NEOVASCULARISATION OF IRIS AND

ANGLE,

 NEOVASCULAR GLAUCOMA
 Lens and vitreous act as mechanical barriers and also releases
vaso inhibitory factors

 So any complicated cat sx with PCR, aphakia more predisposition

 VEGF conc 50-100 times more in aqueous humour in NVG

 STAGES-

 PRE RUBEOSIS STAGE

 PRE GLAUCOMA (RUBEOSIS IRIDIS )
 OPEN ANGLE GLAUCOMA
 ANGLE CLOSURE GLAUCOMA
 Pre rubeosis stage-

 In patients with predisposing risk factors such as PDR, CRVO, etc

 It is important to understand the risk of developing rubeosis irides

and the chances for progression to NVG

 2. Look carefully for NVI and NVA under high magnification

 Pre glaucoma stage : rubeosis iridis

 a. NVI +/- NVA

 b. IOP normal

 d. dilated tufts of preexisting capillaries

and fine, randomly oriented vessels on
the surface of the iris
 Open angle glaucoma

 1. Elevated IOP
 2. NVA and NVI increased
 3. AC inflammatory reaction
 4. Hyphema may be present
 5. No PAS
 6. Angles open
 Fibro-vascular fibrovascular membrane
covering the angle and anterior surface of the
iris and may even extend onto the posterior
iris
(HALLMARK)
 Angle closure glaucoma-
 Most patients are detected in this
stage
 Iris is dilated and pulled
anteriorly from the lens
 PAS formation

 Fibro vascular membrane

contracts leads to flat iris

 Ectropion uvea present

 IOP very high

CLINICAL FEATURES

 SYMPTOMS  SIGNS
 Severe pain  Reduced vision
 Headache ,vomiting  Ciliary injection
 Redness  Corneal edema
 Watering  AC with flare
 Defective vision  Hyphema
 Photophobia  Fixed dilated pupil
 NVI, NVA
 Raised IOP
 DIFFERENTIAL DIAGONOSIS
 1. PACG - no NVI and NVA

 2. UVEITIC GLAUCOMA - KP’S + ,Complicated cataract, band shaped

keratopathy

 3. Fuch Heterochromic Iritis - stellate KP’S, NVA+ ,NVI and NVG are rare

 4. ICE syndrome - corneal decompensation,iris atrophy

 5. Old trauma - angle recession,iris pigment clumps, no NVI

 6. Lens induced glaucoma

 INVESTIGATIONS
 OCULAR :-
 Fundus examination-
 Fundus Fluorescein Angiogram- to assess retinal ischemia
 Iris angiography- in cases of doubtful NVI, to confirm the diagnosis
 B scan ultrasound- if retina is not visible
 Electroretinogram – to assess for retinal ischemia

 SYSTEMIC :-

 - BP, FBS PPBS, Carotid Doppler, lipid profile, renal profile

 TREATMENT-

 A. Identifying the underlying etiology and start adequate

treatment to prevent the development and progression of NVG

 B. Once NVG develops and IOP is high, the major point of

management is control of high IOP to prevent optic nerve
damage
 Management-
 Intravitreal bevacizumab- injecting 1.25-mg bevacizumab in the
vitreous cavity or 1.0- to 1.25-mg bevacizumab in the anterior chamber
before or concomitant with pan retinal photocoagulation PRP

 Pan retinal photocoagulation (PRP)- Ist line treatment

 DM - In established cases of PDR, PRP +/- IVB done to prevent NVG
 CRVO -PRP indicated only after 2 clock hours of NVA/NVI (CVOS)
 Ocular Ischemic Syndrome -PRP indicated for cases with retinal
ischemia on FFA & refer for neurological and cardiology assessment
 Pan retinal photocoagulation-
 Make ischemic retina anoxic by reducing O2 demand
 Decreased angiogenic factor
 Decreased new vessels
 Reduces neo vascularization
 Transscleral Panretinal cryotherapy+ cyclocryotherapy

 Goniophotocoagulation-

 a. Adjunct to PRP

 b. LASER therapy aimed at directly treating the NVA before

development of NVG

 c. No role once glaucoma is established

 d. Low-energy argon laser shots (0.2 seconds, 50-100 um, 100 - 200
mW) are applied to the neovascular tufts
 Medical management-

 Aqueous suppressants- beta blockers, carbonic anhydrase

inhibitors, alpha2 agonists

 Topical prostaglandin analogues can be tried though they may

increase ocular inflammation

 Miotics are c/I- as they can increase inflammation and discomfort

 Surgical management-

 Trabeculectomy

 Tube shunts

 Cycloablation
 Surgical management :-

 1. Medical management of glaucoma with intravitreal anti-VEGF along

with retinal ablation

 It may be sufficient to control the IOP in the open angle stage of NVG

 2. Advanced stage with synechial angle closure surgical intervention

for IOP lowering is often required.
 Trabeculectomy :-

 a. Intraoperative use of anti-fibrotic agents -to reduce the risk of

bleb failure due to subconjunctival scarring

 b. The success rate of trabeculectomy with MMC in NVG at 1 year

has been reported to be around 62.6% and reduced to 51.7% at 5
years *

 c. With the use of preoperative Bevacizumab, success rate may

improve up to 95%**

 * Takihara Y, Inatani M, Fukushima M, Iwao K, Iwao M, Tanihara H. Trabeculectomy with mitomycin C for neovascular glaucoma: prognostic factors
for surgical failure.Am J Ophthalmol 2009; 147:912–8.
 ** Saito Y, Higashide T, Takeda H, Ohkubo S, Sugiyama K.Beneficial effects of preoperative intravitreal bevacizumab on trabeculectomy outcomes
in neovascular glaucoma. Acta Ophthalmol 2010; 88:96–102.
 Tube shunts-
 I. Glaucoma drainage devices - considered as a primary
surgical procedure especially NVG, high risk for failure of
conventional filtering surgery

 II. Scarred conjunctiva, active inflammation, vigorous new

vessel growth and prior failure of trabeculectomy -
indications to consider tube shunt surgery in NVG
 Cycloablation :-
 For refractive NVG, no PL eye to relieve pain-

 Cyclocryotherapy

 TSCPC, other contact and non contact trans scleral cyclo destructive
procedures

 Cyclo photocoagulation- 12-24 burn spots ,posterior to limbus over

360 degrees , 1500-2000 MW, 1.5-2 secs
AQUEOUS – MISDIRECTION /
MALIGNANT GLAUCOMA
 Malignant Glaucoma / Aqueous Misdirection Syndrome
 Definition-

 Von Graefe 1869

 A shallow or flat anterior chamber with an inappropriately high

intraocular pressure despite a patent iridectomy

 European Glaucoma Society; II edition

 Secondary angle closure glaucoma with ‘’posterior’’ pushing
mechanism, without pupillary block, caused by the ciliary body and
iris rotating forward
 Malignant Glaucoma – Etiology
 Surgery for angle-closure glaucoma
 Cessation of topical cycloplegic therapy
 Initiation of topical miotic therapy
 Laser iridotomy
 Laser capsulotomy
 Laser cyclophotocoagulation
 Cataract extraction
 Central retinal vein occlusion
 Argon laser suture lysis
 Hyperopia
 Short axial lengths, or nanophthalmos
 Spontaneously
 Pathogenesis-
 Posterior misdirection of aqueous flow

 Through hyaloid membrane into or behind the

vitreous body
 Increase in vitreous volume

 Anterior rotation of the ciliary body, forward

movement of the lens-iris diaphragm

 Shallower anterior chamber

 Increase in intraocular pressure

Malignant glaucoma: cilio-lenticular
block
Malignant glaucoma: cilio-vitreal
block
 Clinical Presentation-
 Shallow or flat anterior chamber both centrally and peripherally

 No iris bombe

 IOP is elevated
 Great posterior resistance may be noted, during reformation of
anterior chamber postoperatively through the paracentesis site with
viscoelastic substance

 Anterior chamber may not deepen

 IOP may rise substantially
 Patent pi may be there
 Lens iris diagphram ant placed
 Trigger factors-

 Small, crowded anterior segment

 Angle closure

 Swelling and inflammation of the ciliary processes

 Anterior rotation of the ciliary body

 Forward movement of the lens-iris diaphragm

Differential diagnosis-
Pupillary block v/s Malignant Glaucoma
 Investigations-

 Ultrasound biomicroscoscopy (UBM)

 Ultrasound B scan: exclude other pathologies

 Ultrasound biomicroscopy-

 Confirm the diagnosis by the

visualization of the anterior segment
structures:-
 Irido-corneal touch
 Appositional angle closure
 Anterior rotation of the ciliary body
 Apposition to the iris
 MANAGEMENT-

 Medical therapy
 Laser therapy
 Pars plana vitrectomy
 Medical treatment-
 First step (good results in 50% of cases)
 Cycloplegia with atropin 1%x 4-6/d
 Mydriasis with phenilephrin 2,5%x 4-6/d
 Anti glaucoma medications
 Steroids

 Mechanism of action-
 posterior push of the iris lens diaphragm
 Cilliary muscles relaxation
 Long time treatment with atropin required to prevent recurrence
 β blockers, α agonists
 Hyperosmotics agents: Glycerol (po), Manitol (2g/kg iv)
 Laser Therapy
 The second line of treatment
 Neodymium : yttrium-aluminum-garnet (Nd:YAG) laser
 Anterior hyaloid rupture to release the trapped
aqueous from the vitreous
 Several openings are made peripherally
 Placement of the iridectomies should be peripheral
to enable anterior migration of the aqueous
 Pars plana vitrectomy

 Mechanism-
 To debulk the vitreous
 To disrupt the anterior hyaloid face
 Ac reformation
 +/_ lensectomy / phaco
 Needed if-
 Medical or laser therapy fails
 Phakic eyes for which laser treatment is not
possible
 Large pi through in phakic eye a good
option,
 Pars plana vitrectomy-

 Pseudophakic
 vitrectomy + anterior hyaloidotomy

 Phakic-
 Pars plana vitrectomy ± lensectomy
 Fellow eye-
 Narrow angle is present

The laser peripheral iridotomy is performed before

 Risk of aqueous misdirection may be reduced after iridotomy if the

angle remains open and the IOP is normal

 Failure to provide prompt therapy to the fellow - bilateral blindness

UVEITIC GLAUCOMA
 Uveitis is one of the most common of the inflammatory processes

 Acute cases (< 3 months duration) involves the anterior uvea (iritis
or iridocyclitis)

 Chronic forms are seen having frequencies:-

 Anterior (45%), intermediate (15%), posterior (14%) and panuveitis
(24%)

 • Iridocyclitis most common form causing increased IOP

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

Uveitic glaucoma
 Multifactorial
 Hypertensive uveitis ( trabeculitis)
 Open angle sec glaucoma - steroid induced , trabeculitis,
chronic inflammatory glaucoma – trab scarring, neovascular
glaucoma

 Closed angle sec glaucoma- acute pupil block , chronic

angle closure , nvg

 Hypotony (acute with active acute inflammation or chronic

due to ciliary shut down )
 Pathophysiology-
 Secondary OAG
 Obstruction of TM by inflammatory debris, RBCs, WBCs, fibrin,
viscous inflammatory aqueous

 Direct inflammation and swelling of the TM endothelial cells

(trabeculitis)

 Steroid responsiveness

 Formation of vascular or cuticular membranes overlying the TM as

a result of chronic recurrent Inflammation
 Pathophysiology-
 Secondary ACG

 Pupillary block secondary to

posterior synechiae

 Extensive peripheral anterior

synechiae

 Choroidal/ciliary effusions with

anterior rotation of the ciliary body
and resultant angle closure

 Neovascular glaucoma
 General Principles
 It is uncommon for acute anterior uveitis of short duration (< 3 months)
to cause persistent IOP elevation

 Main aim of treatment - suppressing inflammation

 Full physical, CXR, SI joint films, ACE levels, HLA-B27, RPR, FTA-ABS,
ANA, RF, HIV

 HLA B27 positive accounts for 50% of cases in Caucasians,

 Signs and symptoms
 Pain, photophobia,
tearing, decreased VA

 Ciliary flush, miosis, cells

& flare, KPs, vitreous
cells,

 Ant/post synechiae, iris

atrophy, corneal edema
and IOP changes.
 Treatment for Uveitic Glaucoma-

 Depending upon the degree of inflammation present

 Aimed to reduce the acute inflammation and controlling the IOP

 A long term treatment goal is to prevent any permanent structural

damage such as cataract, corneal decompensation and glaucoma

 Specific Tx required in certain cases – eg: ABx for STD, TB,

Toxoplasmosis
 Treatment Principles for Uveitic Glaucoma
 Identification of the mechanism of IOP elevation and treatment of the
underlying cause
 Cycloplegics and corticosteroids - mainstays of treatment to control
inflammation and prevent synechiae

 Steroid potency:-
 Difluprednate (Durezol)>Dexamethasone>Prednisolone>
 Loteprednol (Lotemax)>FML Steroids must be tapered off
 Topical NSAIDS as adjunct in known steroid responders
 Systemic immunosuppresive Tx successful in 70% of patients
unresponsive to other Tx
 Treatment Principles for Uveitic Glaucoma
 Select target IOP based on duration of IOP elevation
 Elevated IOP initially treated with B blockers and CAIs, both topical
and systemic
 Alpha agonists effective, although granulomatous anterior uveitis in
patients taking brominidine 0.2%
 Miotics are C/I
 Laser trabeculoplasty generally ineffective in uveitic glaucoma and
may result in IOP spikes.
 Laser PI is the treatment of choice although higher rate of secondary
closure - only in pupillary block
 Goniosynechiolysis, both laser and surgical- effective in reversing PAS
( combined with cat sx )

 Tube shunt surgery

 Slightly higher success rate than Trabeculectomy but often need

medications

 Cyclodestructive surgery an option in eyes with poor visual potential

 Common Uveitic Entities Associated with
Glaucoma-
 Fuchs Heterochromic Iridocyclitis (FHI)-
 Chronic low grade inflammatory condition
 Heterochromia, KPs, vitreous opacities, cataract
and glaucoma
 M=F
 U/L in 95% of cases.
 Pts usually present with a white, quiet eye
complaining of decreased VA due to cataract
 Minimal flare and cell, fine stellate KPs
 Stromal iris atrophy d/t iris Transillumination
defects.
 PAS, posterior synechiae are unusual

 Light NV of the iris and AC angle

 Fine iris nodules occur in 20% of

patients

 FA shows delayed filling, sector

ischemia and NV of the iris

 Iris transillumination may be present.

 Management of FHI
 Cataract surgery in these patients is generally associated with
good visual outcomes
 Higher incidence of post op CME, IOP spikes, hyphema and PO
uveitis
 Glaucoma Incidence is greater with longer term follow up
 Does not respond to aggressive steroid therapy and may develop
steroid induced glaucoma if used inappropriately.
 Standard glaucoma medications are useful but often ineffective in
the long term
 Avoid pilocarpine
 Trabeculectomy with MMC as well as tube shunt sx =
good success rates if medical treatment fail
 Glaucomatocyclitic Crisis (Posner-Schlossman Syndrome)

 Recurrent unilateral attacks of mild anterior uveitis with marked

elevations of IOP
 Young to middle aged adults, age 20-50 yrs.
 Symptoms, slight in relation to the level of IOP, such as slight
ocular discomfort, blurred vision and
 Recurrences = monthly to yearly.
 Mild ciliary flush, epithelial edema, faint flare and scant KPs
 Angle is open, no synechiae
 A/W subsequent POAG.
 Etiology unknown
 IOP range = 40-60 mmHg

 IOP return to normal between attacks

 Self limiting condition

 Corticosteroids = controlling the inflammatory process (Unlike FHI)

 CAIs, B-blockers and Alpha agonists are all effective in controlling IOP
during acute attacks

 Prophylactic antiglaucoma meds and corticosteroids are not necessary

between attacks
 NSAIDS ? Study showed benefits of Indomethacin

 Glaucoma surgical procedures may be indicated if severe

or prolonged attacks lead to progressive ON damage.
 Common Uveitis Entities Associated
with Glaucoma
 Sarcoidosis-
 A multi system inflammatory disorder

 Young adults and African Americans

 Non caseating granulomas involving the lungs,

liver, spleen, skin, eyes and CNS

 Glaucoma occurred in 10% of pts c Sarcoid

related iridocyclititis
 Ocular manifestations occur in 38-50% of
sarcoidosis patients

 MC involves -anterior uveitis,

chorioretinitis,retinal periphlebitis, optic
neuritis and lacrimal gland involvement

 Diagnosis is made by Chest Xray=(hilar

lymphadenopathy),

 ACE levels and lymph node or skin

biopsy
 Both an acute form and chronic relapsing form
 Initially u/l with an insidious onset

 Frequently develops into a chronic phase often

becoming b/l

 Characterized by mutton fat KPs, iris nodules

(Busacca and Koeppe), nodules in the AC angle
and synechiae.
 Glaucoma is mc associated with chronic relapsing form of sarcoid
anterior uveitis

 Corticosteroids are usefull in the treatment of systemic and ocular

sarcoidosis

 May require prolonged course and taper of corticosteroids

 Cycloplegics

 CAIs, B-Blockers and Alpha agonists effective

 As always avoid miotics
 Trabeculectomy c MMC, tube surgery.
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