dos_feb_2015

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 Figure 6: Specular Microscopy (LE)
endothelialisation2.
The patient was followed up to 6 weeks after surgery. the etio-pathogenesis of this disorder, the most popular
IOP was well controlled and BCVA OS returned to 6/12 amongst these is the Campbell’s Membrane theory6 which
by the end of one month. The filtering bleb was healthy, states that due to an unknown triggering factor (? Viral7),
moderately elevated with normal vascularity. endothelial cells of the cornea undergo metaplasia and turn
To summarise, ICE Syndrome which consists of three into epithelial-like cells and acquire the proliferative and
variants- 1) Progressive/ Essential Iris atrophy; 2) Cogan contractile properties of the same. This results in formation
Reese Syndrome and 3) Chandler Syndrome; is a unilateral, of endothelial membrane extending over the angle and
acquired, corneal endothelial abnormality3, usually first subsequently causing secondary angle closure glaucoma.
seen in young adulthood with a predilection for women Hence, ICE Syndrome should be strongly suspected in any
along with anterior chamber angle abnormalities and case of unilateral glaucoma without other obvious causes8,9.
glaucoma4,5. Various theories have been put forth to explain Also, corneal edema at normal or slightly elevated IOP and

66 l DOS Times - Vol. 20, No. 8 February, 2015

Ocular Trauma

Figure 7: HFA 30-2 showing Glaucomatous Field changes Careful evaluation of the constellation of symptoms and
signs needs to be done to differentiate it from Posterior
any unilateral change in the iris surface or irregularity of the Polymorphous Corneal Dystrophy and Axenfeld-Rieger
pupil, in the absence of a history of trauma or inflammation Syndrome, apart from various other similar appearing
should raise suspicion for the ICE Syndrome. Gonioscopy, conditions as early diagnosis and treatment (ranging from
when possible, should always be performed. Even subtle medical management of glaucoma to surgical management
pupillary distortion may reveal underlying angle changes of glaucoma and Keratoplasty) are essential for better visual
in very early cases. Specular microscopy is invaluable recovery.
for early diagnosis of ICE Syndrome. In cases with severe References
corneal edema interfering with gonioscopic view or
specular microscopy, Ultrasound Biomicroscopy is useful. 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;89:11-
21.

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.

www. dosonline.org l 67

DOS Times Quiz Delhi
Ophthalmological
Society

Instructions:

1. Please return your answers to [email protected] or mail them to “The Quizmaster, DOS Times Quiz,
Dr. Rajesh Sinha, Room No. 479, Dr. R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences,

Ansari Nagar, New Delhi - 110029”. Please write your DOS membership number along with your answers.
2. The answers should reach not later than 8th April, 2015.
The quiz can also be viewed and directly answered on our website www.dosonline.org
3. The results will be announced at the DOS Annual General Meeting meeting on 12th April, 2015.. The correct entry

will be given a prize of Rs. 2,500. If there are more than one correct entries, the winner of the prize will be decided by
draw of lots.

Quiz compiled by Dr. Parul Jain

1. Which is the major cause of post traumatic 3. Identify the type of cataract seen after trauma?
endophthalmitis?

2. Which holi color has been reported to be
associated with higher incidence of ocular
toxicity?

4. Identify the type of fracture?

## ##

Membership No. __________ Name : _______________________Mobile No. _____________Email: _________________

Answer to DOS Times Quiz February, 2015 B. ___________________________________________
A. __________________________________________ D. ___________________________________________
C. __________________________________________

TearsheetDisease Aetiopathogenesis Clinical presentation Management
Commotio retinae/ Self limited opacification of retina
Chorioretinopathies from occular traumaBerlins edemasecondary to shock waves from bluntTransient whitening at the level ofNo treatment available or
www. dosonline.org l 73Retinal concussionocular traumadeep sensory retina. Disruption ofrecommended for it.
Choroidal rupture the photoreceptor outer segments Only observation. Visual prognosis
Milder condition of traumatic retinal with associated retinal pigment is good.
Traumatic macular opacification, Clinical changes are epithelium(rpe) damage.
hole reversible.
Traumatic On FFA, the areas of opaque Only observation. Retinal whitening
chorioretinal rupture Is a tear of inner choroid and overlying retina block background choroidal clears spontaneously in a few days
Bruch’s membrane and RPE due to blunt fluorescence. Leakage from retinal with complete vision recovery.
trauma.(5-10%). Direct choroidal rupture-at vessel is not observed.
the direct site of impact. Usually anterior
to equator. Indirect choroidal rupture-d/t Confirmed after FFA or ICGA If CNV secondary to traumatic
compressive injury to the posterior pole of (indocyanin green angiography). choroidal rupture, observation/
eye. More common (about 80%). Majority On ICGA, appear as hypofluroscent laser photocoagulation/submacular
temporal to disc and involve fovea. streaks. surgery/photodynamic therapy/anti-
Traumatic theory, cystic degeneration Long term risk of choroidal VEGF.
theory, vascular theory, and vitreous theory. neovascularization (CNV) in 5-10%. Visual prognosis depends on
Due to combination of factors and in some whether fovea is affected or CNV
cases vitreous traction plays a role. developed as late complication.
Simultaneous break in retina and choroid
resulting from high velocity missile passing Macular holes were elliptical with No surgical management.
and coming in contact with globe and irregular edges in 19 eyes (95%) and
entering orbit without scleral rupture. ranged in size from 0.2 to 0.5 DD In young patient, with small
(disc diameter) traumatic macular hole without fluid
cuff, wait for 6 months.

Fundus examination. Observation

Chorioretinal defect, bare sclera, Sx rarely for RD or Non resolving
pigment proliferation and scar vitreous haemorrhage
formation. Visual prognosis depends on on the
ICG dye helpful to demonstrate extent and location of intraocular
rupture that are difficult to injury.
appreciate clinically.

Traumatic RPE tears Well known complication of ARMD. A hypo pigmented area Spontaneous resolution of traumatic
But if blunt trauma with force sufficiently corresponding to RPE loss and RPE tears with good visual recovery.
large to cause RPE tear but not so large to exposed Bruch’s membrane. If involve fovea, poor visual
cause choroidal rupture. FFA shows window defect. prognosis.
OCT is helpful.
Traumatic retinal tears 84.4% with retinal tear develop RRD. Ocular contusion may result in Treatment according to RD sx
and detachments. horseshoe tear, operculated holes, principles.
macular holes and retinal dialysis

Retinal dialysis (8-15% Is circumferential retinal tear located along Inferotemporal dialysis is Treatment according to RD sx
of RD) its marginal attachment at the ora serrata. characteristic of blunt trauma. principles.
A blow from the fist is m/c mechanism. There is no known effective surgical
and medical treatment for this.
Optic nerve avulsion When an object intrudes between globe and High dose corticosteroids and optic
Suden loss of vision orbital wall and displaces the eye. canal decompression have been
Motor vehicle/bicycle Complete avulsion attempted but not effective.
accidents basketball Incomplete avulsion Without treatment, resolve
injuries. spontaneously with in 1 month

Chorioretinopathies from indirect ocular injuries. 3-6 month of observation after acute
event followed by vitrectomy, if no
Purtscher’s retinopathy In patients with severe head trauma Purtscher flecken, cotton wool visual improvement occurs…t
Loss of acuity in one or both eyes. spots, retinal haemorrhage, optic
disc swelling.
On FFA, choroidal fluorescence
masked by retinal whitening or
blood.

Terson’s syndrome Vitreous haemorrhage secondary to Haemorrhage in vitreous /
subarachnoid haemorrhage or subdural subhyloid space, sub-internal
haemorrhage. limiting membrane, intraretinal and
subretinal space.

Shaken baby syndrome Infant under 3 yr. U/L Or B/L Most intraretinal, subretinal and
Subdural haemorrhage, intraocular bleeding Post pole retinal haemorrhage, preretinal haemorrhage clear
and metaphyseal fracture. retinal fold, choroidal rupture, spontaneously with in 4 week.
retinoschisis. Vitreous haemorrhage may clear or
Disc edema secondary to elevated persists for longer time.
ICP Conservative mx, sx,,Nd;YAG laser
Premacular haemorrhage is membranectomy.
may be sub ILM or subhyloid or
combination of both.
OCT is helpful for differentiating
Valsalva retinopathyTearsheet: Chorioretinopathies from occular traumaPre retinal and hemorrhagic secondary to
sudden increase in intrathoracic pressure
74 l DOS Times - Vol. 20, No. 8 February, 2015
Fat embolism Direct # of long bones such as femur and is Cotton wool spot., flame like Retinal lesion disappear after few
syndrome(5% of all associated with paralysis,tremor,delirium,st haemorrhage. week. Scotoma may persists.
fracture) upor,coma.
Mortality rate is 30%. Retinopathy reported in 50% of patient with Ashwini Behera MBBS
Whiplash retinopathy FES and 4% pt of long bone #.
A slight,grayish retinal haze,a crater like
depression of less then 100 micrometre in
diameter and slight disturbance in RPE in
fovea following a flexion and extension type
of head and neck injury.

Ashwini Behera MBBS
Dr. R.P. Centre, A.I.I.M.S., New Delhi