GLAUCOMANUR AINA BINTI AB KADIR

CONTENTS Primary Angle Closed Glaucoma(PACG) Congenital/developmental glaucoma Secondary glaucoma

Apposition of peripheral iris against the trabecular meshwork(TM) obstruction of aqueous outflow by closure of an already narrow angle of the anterior chamber

PRIMARY ANGLE-CLOSURE DISEASE

Primary angle closure glaucoma(PACG)

Epidemiology Etiopathogenesis Classification Clinical profile Management

Primary angle closure glaucoma(PACG)

EPIDEMIOLOGY(International Society of Geographical and Epidemiological Ophthalmology (ISGEO) Every 10 occludable angles(PAC suspects)

seen there is only one case of PACG Chronic PACG(asymptomatic): acute

PACG(symptomatic) = 3:1 Great ethnic variability in the prevalence of

PACG Major cause of world glaucoma blindness is

PACG

Ethnic group POAG PACG

Europeans, Africans, Hispanics 5 1

Urban Chinese 1 2

Mongolian 1 3

Indian 1 1

Etiopathogenesis

Predisposing risk factorsPathogenesis of rise in IOP

Predisposing risk factors Demographic risk factor

Age: PACG + pupillary block6th & 7th decades

Gender: male to female ratio (1:3) Race: South-East Asians, Chinese, Eskimos

(more common)

Predisposing risk factors Anatomical and ocular risk factors

Hypermetropic eyes Eyes in which iris-lens diaphragm is placed

anteriorly Eyes with narrow angle of anterior chamber Plateau iris configuration Heredity

Pathogenesis of rise in IOP

Pupillary block mechanism

Plateau iris configuration& syndrome

Phacomorphic mechanism

Pupillary block mechanism PRECIPATING FACTORS

Physiological mydriasis Pharmacological mydriasis:

bronchodilators,antidepressant Pharmacological miosis: echothiophate,

pilocarpine Valsalva manoevure

Mechanism of rise in IOP after mydriasis

Due to effect of precipitating factorsmid dilatation of the pupil

Relative pupil block

Iris bombe formation

Appositional angle closure High IOP(transient)synechial angle closure

Plateau iris configuration and syndrome

Anterior chamber angle is closed by pushing mechanism because of the anterior positioned ciliary processes displacing the peripheral iris anteriorly

Iridotomy Syndrome: acute ACG occurs either

spontaneously/ after pharmacological dilatation, in spite of patent iridotomy

Treat: miotics, laser peripheral iridoplasty

Phacomorphic mechanism Acute secondary angle closure glaucoma

caused by the intumescent/other lens morphological abnormalities

Ultrasonic biomicroscopy (UBM) and Anterior,Segment OCT (AS-OCT) acute primary ACG in predisposed patient

Base of lens extraction for acute PACG

Classification ISGEO,based on natural history

Primary angle closure suspect (PACS)

Primary angle closure(PAC)

Primary angle closure glaucoma (PACG)

Classification –traditional clinicalLatent PACG

Subacute(intermittent) PACG

Acute PACG

Chronic PACG

Primary Angle-Closure Suspect (PACS)

No symptoms PRESENTING SITUATIONS

Suspicious clinical sign, glaucoma screening programme

Eclipse sign Slit-lamp biomicroscopic signs

Decreased axial anterior chmaber length Convex shaped iris lens diaphragm Close proximity of the iris to cornea in the

periphery Van Herick slit-lamp grading of the angle

Primary Angle-Closure Suspect (PACS)

Van Herick slit-lamp grading of the angle: Grade 4 pacd = ¾ to 1 CT (wide open

angle) Grade 3 pacd = ¼ to ½ CT (mild narrow) Grade 2 pacd = ¼ CT (moderate narrow) Grade 1 pacd < ¼ CT (extremely narrow) Grade 0 pacd = NIL (closed)

Primary Angle-Closure Suspect (PACS) DIAGNOSTIC TEST

IOP measurements

Gonioscopy Ultrasonic

biomicroscopy Anterior segment

OCT Optic disc

evaluation Visual filed

analysis

DIAGNOSTIC CRITERIA Gonioscopy: irido-

trabecular contact >270’, no peripheral anterior synechia

IOP: normal Optic disc: no

glaucomatous change

Visual fields: normal

Management Prone dark room (provocative test)

Dark room, prone position without sleeping 1hour

IOP >8mmHg ( diagnostic) Mydriatic test (O.5% tropicamide)

produce mid-dilated pupil pressure rise > 8mmHg (positive)

Inferences Positive: angle is capable of spontaneous closure Negative: presence of occludable angles on

gonioscopy does not rule out the possibility

Treatment Prophylactic laser iridotomy : >270’ of

oppositional iridotrabecular contact(gonioscopy) in the fellow eye of all patients (acute PAC or PACG in one eye)

Periodic follow up Education

Primary Angle Closure (PAC)

Subacute Acute

Chronic

Criteria Irido-trabecular contact: >270’ IOP elevated and/or peripheral anterior

synechiae(PAS) present Optic disc: normal Visual field: normal

Subacute PAC Transient rise of IOP (40-50mmhg) may

last minutes- 1-2 hours

Precipitating factor Physiological mydriasis (dark room or

sympathetic response) Physiological shallowing of anterior

chamber (lying in prone position) Pharmacological mydriasis- fundus

examination

Symptoms Unilateral transient blurring of vision Colored halos around light

accumulation of fluid in corneal epithelium and corneal lamellae

Mild headache & brow ache due to raised IOP

In between recurrent attacks FREE from symptoms

Signs: eye is white, not congested DD: acute purulent conjunctivitis, early

cataractous changes Treatment: peripheral laser iridotomy

Acute PAC Attack of acute rise in IOP in patients

with PAC may occur due to pupillary block sudden closure of angle

SYMPTOMS: Pain Nausea,vomiting, prostrations associated

with pain Rapidly progressive impairment of vision,

redness, photophobia, lacrimation Past history

Signs Lids: oedematous Conjunctiva: chemosed,congested Cornea: oedematous, insensitive Anterior chamber: shallow

Angle: completely closed Iris: discolored Pupil: semidilated,vertically oval & fixed IOP: markedly elevated Optic disc: oedematous, hyperamic Fellow eye: shallow anterior chamber,

occludable angle

Differential Diagnosis Other causes of acute red eye

Acute conjunctivitis Acute iridocyclitis

Acute secondary glaucomas Phacomorphic glaucoma Acute neovascular glaucoma Glaucomatocyclitic crisis

Management IMMEDIATE MEDICAL THERAPY TO LOWER IOP Systemic hyperosmotic agent (IOP 40mmHg)

Intravenous mannitol (1gm/kg) Oral hyperosmotics (glycerol)

Systemic carbonic anyhydrase inhibitor Acetazolamide 500 mg IV

Topical antiglaucoma drugs Beta blocker(timolol), PG analogues

Analgesics and antiemetic Topical steroid

Definitive therapy Laser peripheral iriditomy (LPI) Filtration surgery Clear lens extraction

PROPHYLACTIC: prophylactic laser iridotomy should be performed on the fellow asymptomatic PACS

Sequale of acute PAC Postsurgical acute PAC

Successful: normalized IOP Failed: high IOP trabeculectomy

Spontaneous angle reopening: rare Ciliary body shutdown: AH production,

ischaemic, similar CF, treatment Vogt’s triad

Glaukomflecken (anterior subcapsular lenticular opacities)

Patches of iris atrophy Slightly dilated nonreacting pupil

Primary Angle-Closure Glaucoma (PACG)

Gradual synechiael closure of the angle of anterior chamber

Untreated patient of PAC

Clinical features Subacute and acute PACG

Similar Glaucomatous optic disc changes Visual field defect

Chronic PACG IOP remains constantly raised Eyeball remains white and no congestion Optic disc show galucomatous cupping Visual field defect Gonioscopy >270 angle closure + PAS

Diagnosis Irido-trabecular

contact >270 on gonioscopy

PAS are formed IOP elevated Optic disc show

glaucomatous sign

Visual field defect

Treatment Laser iriditomy Trabeculectomy Prophylactic laser

iriditomy

Absolute Primary Angle-closure Glaucoma

CLINICAL FEATURES Painful blind eye with no perception of light Perilimbal reddish blue zone Caput medusae Cornea (bullous keratopathy/filamentary keratitis) Anterior chamber is very shallow Iris atrophic Pupil fixed, dilated, greenish hue Optic disc (optic atrophy) High IOP Stony hard eyeball

Management

Retrobulbar alcohol injection

• Relieve pain• 1 ml of 2% xylocaine• After 5-10 min:1ml of 80% alcohol-destroy ciliary ganglion

Destruction of secretory ciliary epithelium

• Lower IOP• Cyclocryotherapy / cyclophotocoagulation/cyclodiathermy

Enucleation of eyeball

• Painful blind eye+malignant growth

Complications Corneal ulceration •Prolonged epithelial edema & insensitivity

•Perforate

Staphyloma formation •High IOPvery thin sclerabulges out

Atrophic bulbi •Ciliary body degenerates, IOP falls•Eyeball shrinks

CONGENITAL/DEVELOPMENTAL GLAUCOMAS

Types Pathogenesis Clinical features Examination(evaluation) Differential diagnosis Treatment

TYPES

Primary developmental/ congenital glaucoma

DG with associated congenital ocular anomalies

DG with associated systemic anomalies

Primary developmental/ congenital glaucoma

Abnormal high IOP d/t developmental anomaly of the angle of the anterior chamber

NOT associated with ocular/systemic anomaly

Newborn 40% •True CG,IOP raised during intrauterine life•Born with ocular enlargement

Infantile 55% •Manifest prior to the child’s 3rd birthday

Juvenile 5% •After 3 year but before adulthood•Aka juvenile POAG(10-35y/o)•35%: myopes

Prevalence & genetic pattern Sporadic occurrence (90%) Autosomal recessive inheritance with

incomplete penetrance (10%) Loci linked with PCG : 2p21(GLC3A),

1p36(GLC3B), and 14q24(GLC3C) Sex linkage ,>65% are boys Bilateral occurrence (70%) , asymmetric 1:10 000 births

Pathogenesis Maldevelopment, from neural crest derived cells, of

trabeculum including the iridotrabecular junction (trabeculodysgenesis)

Absence of angle recess with iris insertion Flat iris insertion (commoner) : iris insert flatly &

abruptly into the thickened trabeculum either at or anterior to scleral spur (more often) or posterior, often possible to visualize a portion of ciliary & scleral spur.

Concave iris insertion: superficial iris tissue sweeps over the iridotrabecular junction & the trabeculum obsecure the scleral spur, ciliary body

Clinical features Lacrimation, photophobia, blepharospasm Corneal signs Corneal oedema Corneal enlargements Tears and breaks in Descemet’s

membrane(Haab’s striae) Sclera: thin, appears blue Anterior chamber: deep Iris: iridodonesis, atrophic patches in late

stages

Clinical features Lens: flat, stretching of zonules,

subluxate backward IOP: increased Axial myopia

Examination(evaluation) Measurement of IOP with

Schiotz/preferably hand held Perkin’s applanation tonometer

Measurement of corneal diameter by callipers

Slit-lamp examination: portable slit-lamp Ophthalmoscopy: optic disc Gonioscopic examination of angle of

anterior chamber

Differential diagnosis Cloudy cornea Large cornea Lacrimation: congenital nasolacrimal

duct blockage Photophobia Raised IOP in infants Optic disc changes

Treatment MEDICAL TREATMENT

Hyperosmotic agents, acetazolamide, beta-blocker

Miotics- not used Alpha-2 agonist(brimonidine): CNS

depression CI

Treatment SURGICAL TREATMENT

INCISIONAL ANGLE SURGERY Goniotomy Trabeculotomy

FILTERATION SURGERY Trabeculectomy Combined trabeculectomy & trabeculectomy

with antimetabolites

GLAUCOMA DRAINAGE DEVICES (GDD)

Goniotomy Barkan’s goniotomy knife Through the limbus of temporal side Anterior chamber to the nasal part Incision: midway between root of the iris

and Schwalbe’s ring(75’)

Trabeculotomy Corneal clouding prevents visualisation of the

angle/failed goniotomy Canal of Schlemm is exposed at about 12

o’clock position by a vertical scleral incision conjunctival flap & partial thickness scleral flap

Lower prong of Harm’s trabeculotome is passed along the Schlemm’s canal on one side and the upper prong is used as guide

Rotate break the inner wall over one quarter of the canal

DG with associated congenital ocular anomalies

Glaucoma associated with iridodysgenosis

Glaucoma associated with iridocorneal dysgenesis

Glaucoma associated with iridodysgenosis

Glaucoma associated with: Aniridia-50% Familial iris hypoplasia Congenital ectropion uvea Congenital microcornea Congenital nanophthalmos

Glaucoma associated with iridocorneal dysgenesis

Posterior embryotoxon: prominent Schwalbe’s ring

Axenfeld-Rieger syndrome Axenfeld anomaly: post. Embryotoxon with

attachment of strands of peripheral iris tissue.

Rieger anomaly:post. Embryotoxon, iris stomal hypoplasia, ectropion uveal corectopia, full thickness iris defect

Rieger syndrome: Rieger anomaly+dental anomalies (hypodentia/microdental), facial anomalies(maxillary hypoplasia),other anomalies(hypospadias)

Glaucoma associated with iridocorneal dysgenesis

Peter’s anomaly: central cornea opacity with/ without irido-corneal/lenticulocorneal adhesions

Combined Rieger’s syndrome & Peter’s anomaly

DG with associated systemic anomalies

Glaucoma associated with: Chromosomal disorders: trisomy 13-15,18,21,

turner’s syndrome Ectopia lentis syndrome: Marfan’s syndrome,

Weil-Marchesani syndrome Phakomatosis: Sturge-Weber syndrome(50%),

Von-Recklinghausan’s neurofibromatosis(25%) Metabolic syndrome:

Lowe’s syndrome(oculo-cerebrorenal sydndrome) Hurler’s syndrome(mucopolysaccharidosis) Zellweger syndrome(hepato-cerebral renal

syndrome)

Group of disorders High IOP + primary ocular/systemic

disease

SECONDARY GLAUCOMAS

Classifications Mechanism of

rise in IOP Secondary OAG

Pretrabecular membrane

Trabecular clogging oedema&scarring Post-trabecular

elevated episcleral venous pressure

Secondary ACG +/- pupil block

Causative primary disease Lens induced

(phacogenic) glaucomas

Inflammatory glaucoma

Pigmentary glaucoma

Steroid induced Trumatic glaucoma

Lens-induced (phacogenic) glaucomas

IOP raised 2’ to some disorder of lens Classifications :

Lens-induced 2’ angle closure glaucoma Phacomorphic glaucoma (swollen lens) Phacotopic glaucoma (ant. lens

displacement) Lens-induced 2’ open angle glaucoma

Phacolytic glaucoma Lens particle glaucoma Phacoanaphylactic glaucoma

Phacomorphic Glaucoma Causes :

Intumescent lens – swollen cataractous lens (rapid maturation of cataract or traumatic rupture of capsule

Ant. subluxation/dislocation of the lens & spherophakia congenital smaller, more spherical optic lens cause for phacotopic glaucoma

Pathogenesis : Swollen len pushes iris forward & obliterates the angle Further increase iridolenticular contact (pupillary block

& iris bombe)

Clinical presentation

As in acute congestive glaucoma with features of primary angle closure glaucoma

Lens is always cataractous & swollen

Treatment : Medical treatment –

IV mannitol, systemic acetazolamide & topical BB

Surgical – laser iridotomy (breaking closure-angle attack)

Cataract extraction with implantation of PCIOL

Phacolytic glaucoma (Lens protein glaucoma)

Pathogenesis Trabecular meshwork is clogged by lens protein,

macrophages which have phagocytosed lens protein & inflammatory debris

Leakage of lens proteins occurs through intact capsule in hypermature cataractous lens

CLINICAL FEATURES Features of acute congestive glaucoma Pseudohypopyon Open ant. Chamber (gonioscopy) MANAGEMENT: Extraction of hypermature

cataractous lens

Lens particle glaucoma Pathogenesis Trabecular meshwork

is blocked by lens particles floating in aqueous humour

After accidental/planned ECCE (Extracapsular Cataract Extraction) or following traumatic rupture of lens

Clinical features: Symptoms of acute

rise in IOP assoc. lens particles in anterior chamber

Treatment: Irrigation-aspiration

of lens particles from ant. chamber

Phacoantigenic glaucoma Fulminating acute inflammatory reaction due to Ag-Ab

reaction Same mechanism & management with acute inflammatory

glaucoma Typical finding – granulomatous inflammation in involved

eye after it goes surgical trauma

Pathogenesis : There is preceding distruption of lens capsule by ECCE,

penetrating injury or leaks of protein from capsule Trabecular meshwork is clogged by both inflammatory cells &

lens particles

Management : Treatment of iridocylitis (streroid & cycloplegics) Irrigation-aspiration of lens matter from ant. Chamber

Glaucoma due to uveitis IOP raised because of iricocyclitis or even

due to keratitis and scleritis Types :

Non specific inflammatory glaucoma Open angle inflammatory glaucoma (acute/chronic) Angle closure inflammatory glaucoma

Specific hypertensive uveitis syndromes Fuchs’ uveitis syndrome Glaucomatocyclitic crisis

Acute OAIG

C/F :• Features of acute iridocylitis + increased IOP + open

angle of ant. Chamber• Returns to normal after acute episode of

inflammationManagement :• Treat iridocylitis• Medical therapy to lower IOP (hyperosmotic agents,

acetazolamide, BB)

Mechanism • Trabecular clogging, trabecular edema and

prostaglandin-induced rise in IOP

Chronic OIAGMechanism • Chronic trabeculitis and trabecular scarring

C/F • Raised IOP, open angle, no active inflammation• Signs of prev episode of uveitis present• Glaucomatous disc changes & field defectsTreatments • Medical therapy – topical BB , CAI & alpha agonist (avoid

pilocarpine & PGs)• Trabeculectomy • Cyclodestructive procedure (cycloiodide)

Angle closure inflammatory glaucoma

PAS are formed causing synechiae angle closureMechanism :• 2’ angle closure with pupil block (due to annular synechiae or

acclusio pupillae – iris bombe)• 2’ angle closure without pupil block (organization of

inflammatory debris in the angle causing pulling of iris over the trabeculum during contraction – gradual & progressive synechiae angle closure + increased IOP)C/F :

• Raised IOP, seclusion papillae, iris bombe, shallow ant. Chamber

Management• Prophylaxis (treat acute iridocylitis – local steroids & atropine)• Curative – medical therapy to reduced IOP, surgical or laser iridotomy

in pupil block without angle closure and filtration surgery in presence of angle closure

References Comprehensive Opthalmology, 6th

Edition, AK Khurana, New Age International publisher, page 219-256

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