Irido-Corneal-Endothelial (ICE) Syndrome

OcularCorner
Trauma
Monthly Meeting
Irido-Corneal-Endothelial
(ICE) Syndrome
Geetika Dogra
MBBS
Geetika Dogra MBBS, M.C. Agarwal MS
Deen Dayal Upadhyay Hospital, Hari Nagar, New Delhi
A
32 years old female presented with gradual, painful,
progressive eiminution of vision in left eye, which was
worse in the morning and improving during the day for the
past 3 years. It was associated with photophobia and glare
for the past 6 months. She had no past history of trauma or
inflammation or any family history of similar complaints.
She had no known history of any systemic illness.
Examination revealed a normal general physical condition,
including ear, nose throat and dental evaluation. Her Best
Corrected Visual Acuity (BCVA) was 6/6 OD and 6/12
OS and Intra Ocular Pressure was 18mm Hg OD and
28mm Hg OS. There was endothelial haze and irregular
excrescences over the endothelium of cornea (Hammered
silver appearance) (Figure 1) along with an irregular depth
of anterior chamber. The iris showed patches of atrophy;
small, fine, raised pigmented nodules; multiple iris holes
(polycoria), both partial and full thickness; irido-Corneal
adhesion in the inferior periphery and heterochromia
(Figure 2,3). Pupil was peaked inferiorly (Corectopia). On
evaluation of the fundus of the left eye, the vertical cup-disc
ratio was 0.9:1 and thinning of the inferior and superior
neuro retinal rim was present (Figure 4).
Gonioscopy was done which revealed a synechial angle
closure in >180° and broad tent like irido-corneal
adhesions with a flat, stretched out, effaced iris contour and
a shiny translucent membrane covering it in the Left eye.
Anterior Segment-Optical Coherence Tomography (ASOCT) (Figure 5) also corroborated these findings in addition
to showing a comparatively thickened endothelium in the
left eye. Specular microscopy (Figure 6) showed a decreased
endothelial count in the left eye along with loss of normal
endothelial mosaic with obscuration of cell borders, loss of
hexagonality, dark areas in the centre and brighter reflection
from the borders. Diurnal variation of intra ocular pressure
was significant (>8mm) and water drinking test showed
decreased facility of outflow. OCT for optic disc confirmed
a glaucomatous disc and Humphrey Field Analyser 30-2
also suggested glaucomatous field changes in the Left eye
(Figure 7).
With these clinical findings and investigations we arrived
at the diagnosis of Irido-Corneal-Endothelial Syndrome
(Progressive/Essential Iris Atrophy and Cogan Reese
Syndrome) with Secondary angle closure glaucoma.
So we put the patient on Medical therapy with aqueous
suppressants in the left eye (initially Timolol 0.5% eye
drops 2 times daily, followed by addition of Brimonidine
0.15% eye drops 2 times daily in fixed dose combination).
However, no favourable reduction in Intra Ocular Pressure
(IOP) was achieved and in view of Glaucomatous field
defects, advanced cupping and failure of medical therapy,
we proceeded with trabeculectomy with Mitomycin
C which offers reasonable intermediate-term relief in
patients with ICE Syndrome1 as these patients are usually
young and are hence more prone to bleb failure due to
(1)
(2)
Figure 1: Hammered Silver Appearance of Corneal Endothelium
Figure 2: Anterior segment picture (LE) showing corectopia,
polycoria, iris atrophy, heterochromia
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Monthly Meeting Corner: Irido-Corneal-Endothelial (ICE) Syndrome
(3)
(4)
Figure 3: Magnified picture showing fine, raised, pigmented iris nodules
Figure 4: Fundus picture (LE) showing Glaucomatous cupping of the Disc
Figure 5: AS-OCT (LE) picture showing fine, raised,
iris nodules on a stretched out, effaced iris contour
with narrowing of angle
usual reasons as well as late failure of filtering bleb due to
endothelialisation2.
The patient was followed up to 6 weeks after surgery.
IOP was well controlled and BCVA OS returned to 6/12
by the end of one month. The filtering bleb was healthy,
moderately elevated with normal vascularity.
To summarise, ICE Syndrome which consists of three
variants- 1) Progressive/ Essential Iris atrophy; 2) Cogan
Reese Syndrome and 3) Chandler Syndrome; is a unilateral,
acquired, corneal endothelial abnormality3, usually first
seen in young adulthood with a predilection for women
along with anterior chamber angle abnormalities and
glaucoma4,5. Various theories have been put forth to explain
66 l DOS Times - Vol. 20, No. 8 February, 2015
Figure 6: Specular Microscopy (LE)
the etio-pathogenesis of this disorder, the most popular
amongst these is the Campbell’s Membrane theory6 which
states that due to an unknown triggering factor (? Viral7),
endothelial cells of the cornea undergo metaplasia and turn
into epithelial-like cells and acquire the proliferative and
contractile properties of the same. This results in formation
of endothelial membrane extending over the angle and
subsequently causing secondary angle closure glaucoma.
Hence, ICE Syndrome should be strongly suspected in any
case of unilateral glaucoma without other obvious causes8,9.
Also, corneal edema at normal or slightly elevated IOP and
Ocular Trauma
Careful evaluation of the constellation of symptoms and
signs needs to be done to differentiate it from Posterior
Polymorphous Corneal Dystrophy and Axenfeld-Rieger
Syndrome, apart from various other similar appearing
conditions as early diagnosis and treatment (ranging from
medical management of glaucoma to surgical management
of glaucoma and Keratoplasty) are essential for better visual
recovery.
References
1. Lanzl IM, Wilson RP, Dudley D, et al. Outcome of trabeculectomy
with mitomycin-C in the iridocorneal endothelial syndrome.
Ophthalmology. 2000;107:295-97.
2. Kidd M, Hetherington J, Magee S. Surgical results in iridocorneal
endothelial syndrome. Arch Ophthalmol. 1988;106:199-201.
3. Eagle RC Jr, Font RL, Yanoff M, et al. Proliferative endotheliopathy
with iris abnormalities. The iridocorneal endothelial syndrome. Arch
Ophthalmol. 1979;97:2104-11.
4. Shields MB. Progressive essential iris atrophy, Chandler’s syndrome,
and the iris nevus (Cogan-Reese) syndrome: a spectrum of disease.
Surv Ophthalmol. 1979;24:3-20.
5. Hirst LW, Quigley HA, Stark WJ, et al. Specular microscopy of
iridocorneal endothelia syndrome. Am J Ophthalmol. 1980;89:1121.
Figure 7: HFA 30-2 showing Glaucomatous Field changes
any unilateral change in the iris surface or irregularity of the
pupil, in the absence of a history of trauma or inflammation
should raise suspicion for the ICE Syndrome. Gonioscopy,
when possible, should always be performed. Even subtle
pupillary distortion may reveal underlying angle changes
in very early cases. Specular microscopy is invaluable
for early diagnosis of ICE Syndrome. In cases with severe
corneal edema interfering with gonioscopic view or
specular microscopy, Ultrasound Biomicroscopy is useful.
6. Campbell DG, Shields MB, Smith TR. The corneal endothelium
and the spectrum of essential iris atrophy. Am J Ophthalmol.
1978;86:317-24.
7. Alvarado JA, Murphy CG, Maglio M, et al. Pathogenesis of Chandler’s
syndrome, essential iris atrophy and the Cogan-Reese syndrome. I.
Alterations of the corneal endothelium. Invest Ophthalmol Vis Sci.
1986;27:853-72.
8. Lichter PR: The spectrum of Chandler’s syndrome: an often
overlooked cause of unilateral glaucoma.
Ophthalmology
1978;85:245-51.
9. Laganowski HC, Kerr-Muir MG, Hitchings RA: Glaucoma and
the iridocorneal endothelial syndrome.
Arch Ophthalmol
1992;110:346-50.
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