This document presents a case study of a 27-year-old female patient with iridocorneal endothelial syndrome (ICE) in her left eye. Her symptoms included diminished vision and pain in the left eye for 2 months. Ocular examination revealed findings consistent with ICE including iris defects, irregular anterior chamber depth, and specular microscopy showing polymegathism with a silver beaten appearance in the left eye. The patient was diagnosed with refractive error in the right eye and ICE in the left eye. She was prescribed eye drops and followed up regularly, with her IOP remaining controlled.

1. CASE PRESENTATION
Presenter- Dr. Nancy Sehdev
Moderator- Dr. Rita Dhamankar

2.  Name- S W
 Age- 27 years
 Sex- Female
 Address- Kharghar

3. 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

4. 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.

5.  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.

6.  History of using glasses since 7-8 years.
 Her present glasses were 5 months old.
 No ho redness, watering.
 No ho photophobia.
 No ho coloured halos.
 No ho trauma
 No ho similar complaints in the other eye.

7. • PAST HISTORY
No history of similar complaints in the past.
• FAMILY HISTORY
Nothing significant.

8.  GENERAL PHYSICAL EXAMINATION-
 Normal
 SYSTEMIC EXAMINATION-
 Normal

9. Ocular examination
 Head posture- Erect
 Facial symmetry- symmetrical

10. Visual acuity
SPH 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

11. 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

12. Anterior segment examination
Iris Normal in colour and
pattern
Loss 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.

13. OD Anterior Segment

14. OS Anterior segment

15. Intraocular pressure
 On applanation tonometer-
14mm Hg in both eyes

16. OD Fundus

17. OS Fundus

18. Fundus
OD 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

19. Gonioscopy(OS) PAS

20. Provisional diagnosis
 Right eye refractive error
 Left eye Iridocorneal endothelial syndrome

21.  Investigations-
Perimetry(OD)

22.  OS- Perimetry

23. Specular microscopy-
RE WNL
LE shows
polymegathism
with silver beaten
appearance

24. Definitive diagnosis
 Right eye refractive error
 Left eye Iridocorneal endothelial syndrome

25.  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.

26. Diurnal variation test-24-01-13
Time 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

27. 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

28. 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.

29.  Subsequently patient was followed up with intervals
of 2-3 months for IOP evaluation.
 Her IOP was well controlled throughout.

30. 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.

31.  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.

32. Perimetry was repeated
Perimetry- OD

33. Perimetry OS
Not very reliable with
too many fixation losses

34.  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.

35. Specular microscopy comparison

36. Repeat perimetry
Perimetry-OS

37. GPA Printout
There seems to be a
trend towards
progression on VFI

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

39. Perimetry-OD

40. Perimetry- OS
Shows a definitive inf nasal
arcuate defect with an early
Siedel’s .

41. OCT

42.  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.

43. GLAUCOMA AND CORNEAL
DISORDERS

44. Disorders of the cornea associated
with glaucoma
• Peters’ anomaly
• Aniridia
• Axenfeld-Rieger syndrome
Developmental
disorders
• Keratouveitis
• Full thickness or endothelial
keratoplasty
• Trauma
Acquired
conditions

45.  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

46. Glaucoma with secondary corneal
abnormalities
A. Pressure induced
corneal changes
1. Epithelial and stromal
oedema
2. Endothelial changes
3. Haab’s
striae(childhood
glaucoma)
B. Exfoliation induced
corneal endothelial
changes

47. 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.)

48. Iridocorneal endothelial syndrome
 Characterised by primary corneal endothelial
abnormality.
 Associated with-
1. corneal oedema
2. Anterior chamber angle changes
3. Alterations in iris
4. Glaucoma
Hirst LW, Quigley HA, Stark WJ, et al. Specular microscopy if
iridocorneal endothelia syndrome. AM J Ophthalmol. 1990;89(1):11-21.

49. General features
 Clinically unilateral
 Age- early to middle adulthood
 Sex- females more commonly affected
 Familial cases rare
 No consistent association with any systemic disease
 Most common clinical manifestations-
Abnormalities of iris
Reduced vision
Pain

50. 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

51. Chandler
syndrome
• Corneal
oedema often
at normal IOP
• Iris changes
mild to absent

52. Cogan –Reese
syndrome
• Nodular,
pigmented
lesions of iris
• Corneal and iris
defects

53. Specular microscopy
 Fine 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.

54. 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.

55. 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.

56. • 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.

57. Anterior chamber angle alterations
 Peripheral 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.

58. 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.

59. Differential diagnosis
Corneal endothelial disorders
Posterior
polymorphous
dystrophy
Fuch’s
endothelial
dystrophy

60. 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

61. Nodular lesions of iris
 Bilateral diffuse iris nodular nevi-
1. Neurofibromatosis
2. Oculodermal melanocytosis
3. Axenfeld syndrome
4. Peter’s anomaly
5. Inflammatory disorders-sarcoidosis

62. 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.

63.  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.

64.  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.

65.  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.

66. Glaucoma
medications-
pregnancy
considerations

67. 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 women
Primary Care Optometry News, September 2008

68. Carbonic anhydrase inhibitors- FDA
rating Class C
 Contraindicated 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.

69. Miotics-Class C
 Drugs 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.

70. Prostaglandin analogs-Class C
 Prostaglandins 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.

71. Adrenergic agonists- Class C
 Brimonidine 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.

72. THANK YOU