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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