CASE PRESENTATION

Presenter- Dr. Nancy Sehdev

Moderator- Dr. Rita Dhamankar

Name- S WAge- 27 yearsSex- FemaleAddress- Kharghar

Chief complaints First day-14/12/12

Diminution of vision in left eye even with glasses since 2 months.

Pain in left eye since 2 months

History of presenting illness Apparently alright 2 months back when she

noticed diminution of vision in left eye, gradual in onset and progressive in nature.

Associated with pain in her left eye, pricking in nature, present throughout the day.

Sometimes pain used to increase in the evening.

No aggravating and relieving factors and radiating to left brow and forehead area.

Had shown to another ophthalmologist where she was diagnosed to have anterior segment dysgenesis and was prescribed following eye drops- left eye

1. Eyemist forte eye drops( Hydroxypropyl methyl cellulose 3mg, Dextran 70, Glycerin) sos.

2. Travatan eye drops(Travoprost 0.004%) once at night.

3. Dortas T eye drops( Dorzolamide hydrochloride 2%,

Timolol Maleate 0.5%) 2 times per day.

History of using glasses since 7-8 years.Her present glasses were 5 months old.No h\o redness, watering.No h\o photophobia.No h\o coloured halos.No h\o traumaNo h\o similar complaints in the other eye.

• PAST HISTORY

No history of similar complaints in the past.

• FAMILY HISTORY Nothing significant.

GENERAL PHYSICAL EXAMINATION-

Normal

SYSTEMIC EXAMINATION-

Normal

Ocular examinationHead posture- Erect

Facial symmetry- symmetrical

Visual acuitySPH CYL AXIS VA

DIST -2.50 -1.50 160 6/12

NEAR

- - - N18

Extra ocular movements-All uniocular and binocular extraocular movements are full and free.

SPH CYL AXIS VA

DIST -2.00 -2.00 20 6/6

NEAR

- - - N6

Structure Right eye Left eye

lids Normal Normal

Conjunctiva Normal Mild superficial conjunctival congestion

Cornea Clear Few iris pigments on endothelium

Anterior chamber Quiet Irregular in depth, quiet

Anterior segment examination

Anterior segment examinationIris Normal in colour

and patternLoss of pattern,Iris holes

Pupil Round, regular and reacting to light

single, eccentric pupil, round,3mm in size, sluggishly reactive, pseudopolycoria

Lens Clear Clear, iris pigments on anterior lens capsule.

OD Anterior Segment

OS Anterior segment

Intraocular pressureOn applanation tonometer- 14mm Hg in both eyes

OD Fundus

OS Fundus

FundusOD OS

Glow Present Present

Media Clear Clear

Disc Small Small

cup:disc 0.4:1 0.8:1, small inferior notch

Macula Foveal reflex present Foveal reflex present

Background Normal Normal

Gonioscopy(OS) PAS

Provisional diagnosis Right eye refractive error Left eye Iridocorneal endothelial

syndrome

Investigations- Perimetry(OD)

OS- Perimetry

Specular microscopy-

RE WNL

LE shows polymegathism with silver beaten appearance

Definitive diagnosisRight eye refractive error

Left eye Iridocorneal endothelial syndrome

After the above investigations, patient was advised to continue same eye drops-left eye

1. Eyemist forte eye drops( Hydroxypropyl methyl cellulose 3mg, Dextran 70, Glycerin) sos.

2. Travatan eye drops(Travoprost 0.004%) once at night.

3. Dortas T eye drops( Dorzolamide hydrochloride 2%,

Timolol Maleate 0.5%) 2 times per day. Patient was given appointment for diurnal

variation test.

Diurnal variation test-24-01-13Time IOP B.P.

3:oo pm 16 16 120/80

5:00 pm 14 24 110/76

7:00 pm 14 24 108/76

9:00 pm 14 24 108/76

12:00 am 14 24 108/76

3:00 am 14 22 108/78

6:00 am 14 22 100/80

Time IOP B.P.

8:00 am 12 16 110/80

10:00 am 12 16 108/76

12:00 pm 14 16 120/72

3:00 pm 14 16 110/78

Right eye Left eye

Highest IOP 16 24

Lowest IOP 12 16

•Patient was asked to continue same eye drops and review after 6 weeks.•Also, Dortas T eye drops were asked to be added a little later in the day to blunt off the spike.

Subsequently patient was followed up with intervals of 2-3 months for IOP evaluation.

Her IOP was well controlled throughout.

23-08-2013 Patient came for regular follow-up. She was

in first trimester of pregnancy. On examination, BCVA in Right eye was 6/6,

N6 Left eye was

6/12, N18

Anterior and posterior findings were same as before.

Patient was advised to stop Travoprost eye drops for first trimester as it could be an abortifacient and follow up 3 weeks.

On subsequent follow ups till Feb 2014, her pressures were under control.

Then she directly came for follow up on 08-10-14.

She was using both Dortas T eye drops 2 times daily and Travatan eye drops once daily in left eye.

IOP was 10mmHg in right eye and 16 mmHg in left eye.

Perimetry was repeated

Perimetry- OD

Perimetry OS

Not very reliable with too many fixation losses

Patient was asked to continue same treatment and review every 3 months for IOP evaluation.

On 27-07-15, patient came for follow up.IOP was 14 mmHg in both eyes.

Specular microscopy comparison

Repeat perimetry

Perimetry-OS

GPA Printout

There seems to be a trend towards progression on VFI

On her last followup visit- 14-01-16IOP- 12 mmHg in right eye and 14 mmHg in

left eye.

Perimetry-OD

Perimetry- OS

Shows a definitive inf nasal arcuate defect with an early Siedel’s .

OCT

There seems to be a progresion happening. Hence an AGV counselling was done.

Till then, advised to continue same eye drops and review after 1 month.

GLAUCOMA AND CORNEAL DISORDERS

Disorders of the cornea associated with glaucoma

• Peters’ anomaly• Aniridia• Axenfeld-Rieger syndrome

Developmental disorders

• Keratouveitis• Full thickness or

endothelial keratoplasty• Trauma

Acquired conditions

Glaucomas associated with primary disorders of corneal endothelium

1. Iridocorneal endothelial syndrome (Unilateral)

2. Posterior polymorphous dystrophy (Bilateral)

3. Fuch’s endothelial dystrophy (Bilateral) Allingham RR, Damji KF, Freedman SF et al. Editors. SHEILDS. Textbook of Glaucoma.6th ed. Lippincott Williams & Wilkins: 2012

Glaucoma with secondary corneal abnormalitiesA. Pressure

induced corneal changes

1. Epithelial and stromal oedema

2. Endothelial changes

3. Haab’s striae(childhood glaucoma)

B. Exfoliation induced corneal endothelial changes

C. Drug induced changes in the cornea

1. Endothelial decompensation with topical carbonic anhydrase inhibitors. 2. Toxic effects to cornea epithelium(eg: benzalkonium chloride, β- blockers,

miotics.)

Iridocorneal endothelial syndromeCharacterised by primary corneal endothelial

abnormality.

Associated with-1. corneal oedema2. Anterior chamber angle changes3. Alterations in iris4. Glaucoma

Hirst LW, Quigley HA, Stark WJ, et al. Specular microscopy if iridocorneal endothelia syndrome. AM J Ophthalmol. 1990;89(1):11-21.

General features

Clinically unilateralAge- early to middle adulthoodSex- females more commonly affectedFamilial cases rareNo consistent association with any systemic

diseaseMost common clinical manifestations- Abnormalities of iris Reduced vision Pain

Major clinical variations

Progressive iris atrophy•Iris features predominate•Corectopia•Atrophy and hole formation

Allingham RR, Damji KF, Freedman SF et al. Editors. SHEILDS. Textbook of Glaucoma.6th ed. Lippincott Williams & Wilkins: 2012

Chandler syndrome

•Corneal oedema often at normal IOP•Iris changes mild to absent

Cogan –Reese syndrome

•Nodular, pigmented lesions of iris •Corneal and iris defects

Specular microscopyFine hammered

silver appearance of posterior cornea.

Pleomorphism in size and shape.

Dark areas within the cells.

Loss of clear hexagonal margins

Hirst LW, Quigley HA, Stark WJ, et al. Specular microscopy if iridocorneal endothelia syndrome. AM J Ophthalmol. 1990;89(1):11-21.

ICE cells

These endothelial cells appear dark by specular microscopy except for light central spot and a light peripheral zone.

When clustered as continuous cells, known as ICE TISSUE.

Give appearance of negative of a tissue.

Variations of ICE tissue• Disseminated ICE – 1. ICE cells scattered

among large endothelial cells. 2. elevated IOP

• Total ICE- 1. Whole posterior

corneal surface composed of ICE

tissue.Hirst LW, Quigley HA, Stark WJ, et al. Specular microscopy if iridocorneal endothelia syndrome. AM J Ophthalmol. 1990;89(1):11-21.

• Subtotal ICE(+)- 1. clearly defined ICE

tissue 2. remaining cells-distinct

small cells 3. normal IOP

• Subtotal ICE (-)- 1. ICE tissue 2. larger than normal

remaining endothelial cells.

Anterior chamber angle alterationsPeripheral anterior synechia, usually

extending to or beyond Schwalbe’s line is common to all variations of ICE syndrome.

Histology reveals a cellular membrane consisting of a single layer of endothelial cells and Descemet’s like membrane extending down from the peripheral cornea.

Weber PA, Gibb G. Iridocorneal endothelial syndrome glaucoma without peripheral anterior synchias. Glaucoma. 1994;6:128.

Membrane theory of Campbell

Campbell DG, Shields MB, Smith TR. The corneal endothelium and the spectrum of essential iris atrophy. AM J Ophthalmos. 1978;86(3):317-324.

Differential diagnosis

Corneal endothelial disorders

Posterior polymorphous dystrophy

Fuch’s endothelial dystrophy

Dissolution of the iris Axenfeld Rieger syndrome

• Congenital • Bilaterality • Prominant anteriorly

displaced Schwalbe’s line

• Associated ocular and systemic abnormalities.

Iridoschisis

Separation of superficial layers of iris stroma

May be associated with glaucoma

Typically seen in elderly

Nodular lesions of iris

Bilateral diffuse iris nodular nevi-

1. Neurofibromatosis2. Oculodermal melanocytosis3. Axenfeld syndrome4. Peter’s anomaly5. Inflammatory disorders-sarcoidosis

Management Of ICE Syndrome In early stages, drugs that reduce aquoeus

production are used.Aqueous facilitators are of no use, as the TM

is affected . However other pathways may still be targeted.

Prostaglandins may not be advocated, as there is a school of thought that believes the HSV / Epstein Barr virus to be an aetiological factor.

There have been no reported cases of reactivation of the viral infection.

When the IOP can no longer be controlled medically, surgical intervention is indicated.

Laser trabeculoplasty is not effective for this disease, as the angle is affected.

Filtering surgery is reasonably successful, though late failures have occured because of endotheliazation of the filtering bleb & the fact that the surgery is done on relatively young adults, where fibroblastic proliferation is common. .

Glaucoma drainage device surgery, is the surgery of choice & has proven to be more effective.

Kim DK, Aslanides IM, Schmidt CM Jr, et al. Long term outcome of aqueous shunt surgery in ten patients with iridocorneal endothelial syndrome. Ophthalmology. 2000;106(5):1030-1034.

Corneal oedema may be controlled improved by lowering the IOP.

Hypertonic saline solutions can be used.

Penetrating keratoplasty or DSAEK if there is marked dysfunction of corneal endothelium and oedema is not getting cleared.

Alwin PT, Cohen EJ, Rapuano CJ, et al. Penetrating keratoplasty in iridocorneal endothelial syndrome. Cornea. 2001;20(2):134-140.

Glaucoma medications-

pregnancy considerations

Beta-blockers-FDA rating class C Drugs like timolol, levobunolol, betaxolol,

carteolol-should be avoided in the first trimester of pregnancy and discontinued 2-3 days prior to delivery to avoid beta-blockade in the infant.

Beta-blockers being concentrated in breast milk, avoided in mothers who are breastfeeding.

  Timolol - reported to be compatible with lactation according to the American Academy of Pediatrics.Michael J. Trad. Some ophthalmic drugs not safe for

use in lactating or pregnant womenPrimary Care Optometry News, September 2008

Carbonic anhydrase inhibitors- FDA rating Class CContraindicated during pregnancy because of

potential teratogenic effects. Avoided in mothers who are breastfeeding

because of the potential hepatic and renal effects to the infant.

However, acetazolamide has been reported to be compatible with lactation according to the American Academy of Pediatrics.

Miotics-Class CDrugs like pilocarpine, echothiophate,

carbachol appear to be safe during pregnancy.

The toxicity during lactation is unknown. One exception is demecarium, which is toxic

and is contraindicated in pregnancy and mothers who are breastfeeding.

Prostaglandin analogs-Class CProstaglandins are used systemically for

labor induction and termination, and as such, the topical use for glaucoma during pregnancy raises natural concern.

Therefore, caution should be exercised when latanoprost is administered in women who are pregnant or breastfeeding.

Adrenergic agonists- Class CBrimonidine has not demonstrated any fetal

risk. Although no studies were conducted in pregnant patients, it may be used if necessary.

Whether brimonidine is excreted in human milk is not known. Therefore, caution should be exercised since topical brimonidine given to human infants aged younger than 2 months has been reported to cause bradycardia, hypertension, hypothermia, and apnea.

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