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Bilateral ice syndrome with cystoid macular oedema

Poster Details


First Author: P.Gupta UK

Co Author(s): H. Gupta                    

Abstract Details

Purpose:

To report a rare case of early bilateral Irido-Corneal Endothelial (ICE) syndrome with bilateral cystoid macular oedema (CMO), which elaborates the importance of subtle signs exhibited by serious conditions in their early phase as well as associated complications which may not be correlated to them because of confounding factors (in this case- diabetes). A 51 year old female presented to our emergency clinic with complaints of blurry vision in left eye (OS); (as if a translucent film had been stretched over it) and moving black spots in front of both eyes (OU). History of Liver transplant and Diabetes-II.

Setting:

Tertiary Eye Care Hospital, Manchester, UK.

Methods:

Ocular examination revealed UCVa of 0.1 (OD) and 0.2 (OS). Anterior segment evaluation showed symmetrical corectopia- pear shaped pupils peaking (at 4’o clock OD; 8’o clock OS) with bilateral ectropion uveae, heterochromia and iris naevus OS. Gonioscopy (with UBM) revealed pigment stippling of temporal angles OU but no synaechie or adhesions. IOP was OD/OS-12/14 mmHg. Fundus examination exhibited bilateral macular elevation with thickening and distortion of foveal reflex. OCT recorded cystic oedematous spaces with spongy thickening at the macula and central macular thickness of 285μm OD and 337μm OS. OCT-RNFL was within normal limits.

Results:

The patient was scheduled for OS intra-vitreal Ranibizumab injection and in the interim was initiated on topical Nepafenac 0.1% OU. Her haematological investigations were found normal. OCT performed prior to her injection revealed a reduction in macular oedema with central macular thickness of 265μm and 287μm recorded in OD and OS respectively. As of present the intra-vitreal anti-VEGF injection has been withheld and the patient is continuing with topical Nepafenac. Serial follow-ups have been scheduled with IOP assessment. The patient has been educated about her condition and the possibility of a surgery in the future.

Conclusions:

ICE syndrome generally presents unilaterally with a direct effect on Intra-ocular pressure. Till date there have been precious few cases reported in literature which exhibit bilateral early ICE or its association with CMO respectively. It has been hypothesised that proliferation of abnormal endothelial cells at the angle in ICE leads to circumferential traction which may ultimately lead to collapse of the inner blood-retinal barrier. This would ultimately cause seepage and development of CMO. Our patient is in the characteristic age bracket associated with this disease which interestingly has presented itself by exhibiting a complication rarely associated with it.

Financial Disclosure:

None

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