CaseWise 12

Iridocorneal Endothelial Syndrome

Dr. Shreya Jaiswal, Dr. Vinay Nangia
Suraj Eye Institute, Nagpur, India

Introduction :

Iridocorneal Endothelial Syndrome (ICE) is a rare disorder with abnormal corneal endothelium that leads to varying amount of corneal edema, iris atrophy, and secondary angle closure glaucoma. It typically affects young females, and is usually unilateral.

Case Report :

A male, 15 years of age, presented with chief complaints of blurring of vision and coloured haloes in left eye since 1 year. On examination he had a best corrected visual acuity (BCVA) of 6/6, N6 in right eye and counting fingers at 2m, less than N36 in left eye. He had microcystic corneal edema, atrophic iris patches and polycoria in left eye and right eye was within normal limits (Fig.1). Intraocular pressure (IOP) of right eye was 18 mmHg and left eye was 48 mmHg. Fundus examination of right eye showed healthy disc and left eye showed a vertical cup disc (VCD) ratio of 0.75, pale neuroretinal rim (NRR) with thinning superiorly and inferiorly and areas of retinal nerve fibre layer (RNFL) loss along the superior and inferior arcade (Fig. 2).

Management :

Patient was started on topical alpha agonist and beta blockers, following which there was significant reduction in the corneal edema. And he was advised Ahmed Glaucoma Valve (AGV) implantation surgery, which was done on 14/06/2019 in left eye in infero-nasal quadrant using standard operating technique. Post operative visual acuity was 6/60 in left eye after 4 months.

Post operative IOP control :

Post operative IOP was well control in early post operative period. At 3 weeks postoperatively, IOP recorded was 28 mmHg which was suspected to be due to hypertensive phase post AGV implantation. Patient was advised to restart topical alpha agonist and beta blockers and IOP recorded at 5 weeks was 12 mm Hg which was stable at 16 mmHg till 6 months follow up.

Discussion :

As the etiology of ICE syndrome remains unclear, the treatment is mainly focused on managing the glaucoma. Trabeculectomy may be done in these patients, but it has a higher rate of failure due to subconjunctival scarring.4 Glaucoma drainage device may provide an effective and relatively safe alternative treatment in cases of patients with ICE syndrome.5 In view of the above we decided to perform a primary AGV for this patient and we achieved satisfactory IOP control over a period of 6 months.

References :

  1. Malhotra C, Seth NG, Pandav SS, Jain AK, Kaushik S, Gupta A, Raj S, Dhingra D. Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. Indian journal of ophthalmology. 2019 May;67(5):604. doi: 10.4103/ijo.IJO_1237_18
  2. Olawoye O, Teng CC, Liebmann JM, Wang FM, Ritch R. Iridocorneal endothelial syndrome in a 16-year-old. Journal of glaucoma. 2011 Jun 1;20(5):294-7. doi: 10.1097/IJG.0b013e3181e664b0
  3. Salim S, Shields MB, Walton D. Iridocorneal endothelial syndrome in a child. Journal of pediatric ophthalmology and strabismus. 2006 Sep 1;43(5):308-10.     https://doi.org/10.3928/01913913-20060901-06
  4. Doe EA, Budenz DL, Gedde SJ, Imami NR. Long-term surgical outcomes of patients with glaucoma secondary to the iridocorneal endothelial syndrome. Ophthalmology. 2001 Oct 1;108(10):1789-95. https://doi.org/10.1016/S0161-6420(01)00725-4
  5. Ray VP, Rao DP, Gulati I. Primary implantation of non-valved glaucoma-drainage-device in sulcus in iridocorneal endothelial syndrome. International journal of ophthalmology. 2019;12(11):1809. doi: 10.18240/ijo.2019.11.23
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