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Iridocorneal Endothelial (ICE) Syndrome

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Title: Iridocorneal Endothelial (ICE) Syndrome

Author: Krishna Mallem, Drexel University College of Medicine, MD Class of 2024; . Austin Nakatsuka, MD

Date: July 2023

Keywords/Main Subjects: Iridocorneal endothelial syndrome; ICE; Gonioscopy assisted transluminal trab

eculotomy; GATT

Introduction:

Iridocorneal endothelial (ICE) syndrome is an unusual disorder of the eye that involves an abnormality of the corneal endothelium. ICE syndrome typically affects middle aged women, and occurs sporadically and unilaterally 1,2. It consists of three clinical variants, which are Chandler syndrome, essential iris atrophy, and iris nevus or Cogan-Reese Syndrome. The distinction between these variants is based on clinical exam findings. Abnormalities of the corneal endothelium in ICE syndrome can lead to varying degrees of corneal edema, iris atrophy, and secondary angle closure glaucoma. This condition can be potentially blinding due to glaucoma recalcitrant to conventional therapies and recurrent corneal edema1-4. We present a case of a patient with ICE syndrome who undergoes gonioscopy assisted transluminal trabeculotomy (GATT) for uncontrolled IOP on medical therapy.

Photos taken from Moran Eye Center archives.

Pathophysiology:

Similar to corneal guttata found in Fuchs endothelial dystrophy, the corneal endothelium in patients with ICE syndrome is described to have a “beaten bronze” appearance. Scanning and tunneling electron microscopy of endothelial cells in patients with ICE syndrome reveal a population of well differentiated epithelial-like cells in place of normal endothelium. This cell population displays migratory characteristics, and is believed to migrate posteriorly onto the trabecular meshwork and the iris. As this tissue contracts within the angle and the iris, peripheral anterior synechiae (PAS) may form, and the iris may undergo morphological changes. Contraction of tissue can also predispose patients to secondary angle closure glaucoma. Corneal edema can develop as a consequence of elevated intraocular pressure as well as due to failure of the normal pump function of the dysfunctional endothelium3-8.

Case presentation:

Our patient is a 51 year old male who presented in February 2017 for glaucoma evaluation in the left eye (OS). IOP on examination was 34. Corneal pachymetry showed a corneal thickness of 523 microns OS. Gonioscopy revealed a broad area of peripheral anterior synechiae (PAS) temporally, with smaller areas of PAS inferiorly. Slit lamp exam was significant for diffuse endothelial guttae-like changes in the cornea with an oval shaped pupil. IOP and slit lamp exam of the right eye were unremarkable. Baseline visual fields were obtained and showed scattered defects in both eyes. Baseline optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL) revealed normal thickness in both eyes. Given examination findings at the baseline visit, the patient was diagnosed with ICE syndrome and was started on latanoprost once daily OS for intraocular pressure control.

The patient was monitored regularly and over the course of several years his medical therapy was escalated due to persistently high IOPs as well as changes on field and OCT testing. In March 2022, the patient was on maximal medical therapy with netarsudil-latanoprost, dorzolamide-timolol, and brimonidine in the left eye. Despite this, his IOP was 37 on the left. Slit lamp examination at this time showed a distorted pupil with high PAS temporally. Visual field testing showed a subtle superior arcuate scotoma OS. OCT RNFL showed significant thinning inferiorly and superiorly OS. The decision to proceed with surgery was made given the patient’s young age and long term prognosis due to medically uncontrolled IOP.

Patient underwent gonioscopy assisted transluminal trabeculotomy (GATT) with anterior goniosynechiolysis without cataract extraction in May of 2022. After successful surgery without complications, the patient’s iop quickly normalized and was maintained over a year later on two drops. OCT findings 6 months after surgery revealed stability, however, there were signs of progression 1 year following the procedure, despite well controlled IOPs. Additionally, the patient has recently developed corneal guttae and edema which is currently being treated medically with hypertonic . Although the patient’s IOP continues to be well controlled, the current focus is now on controlling corneal edema, with consideration for corneal transplantation in the future if the cornea does not recover with medical therapy alone.

Discussion:

This is a unique case of a patient with ICE syndrome who was managed with GATT and goniosynechiolysis in the left eye for uncontrolled IOP and progressive glaucomatous changes. To our knowledge, there is not another case of GATT or other microinvasive glaucoma surgery (MIGS) being performed in a patient with ICE syndrome in the literature. It is well documented that filtering procedures in ICE syndrome are only modestly successful at 5 years (53% with drainage device and 29% with trabeculectomy)⁸ and often need to be repeated to obtain IOP control. Although GATT has successfully controlled IOP one year out from surgery, there is still evidence of OCT progression and further surgical treatment may be necessary. Furthermore, the patient has developed more clinically significant corneal edema and guttae, suggesting corneal decompensation, which may require surgical intervention with a corneal transplant. This case highlights the difficulty in managing patients with ICE syndrome and documents the novel use of GATT for IOP control in ICE syndrome.

References:

  1. Laganowski, H. C., Muir, M. G. K., & Hitchings, R. A. (1992). Glaucoma and the iridocorneal endothelial syndrome. Archives of Ophthalmology, 110(3), 346-350.
  2. Dubey, S., Jain, K., Singh, S., & Mukhejee, S. (2021). Iridocorneal Endothelial Syndrome with Coexisting Macular Edema and Neurosensory Detachment: An Unusual Case Report. Journal of Current Glaucoma Practice, 15(3), 149.
  3. Campbell, D. G., Shields, M. B., & Smith, T. R. (1978). The corneal endothelium and the spectrum of essential iris atrophy. American journal of ophthalmology, 86(3), 317-324.
  4. Basic and Clinical Science Course (BCSC). American Academy of Ophthalmology, (2010-2011), 142-144.
  5. Eagle, R. C., Font, R. L., Yanoff, M., & Fine, B. S. (1979). Proliferative endotheliopathy with iris abnormalities: the iridocorneal endothelial syndrome. Archives of Ophthalmology, 97(11), 2104-2111.
  6. Doan A, Alward W: Iridocorneal Endothelial Syndrome (ICE) – essential iris atrophy : 63-year-old female with PAS, “iris mass”, corectopia, and increased IOP OS. February 21, 2005; Available from: http://www.EyeRounds.org/cases/case14.htm.
  7. Levy, S. G., Kirkness, C. M., Moss, J., Ficker, L., & McCartney, A. C. (1996). The histopathology of the iridocorneal-endothelial syndrome. Cornea, 15(1), 46-54.
  8. Doe, E. A., Budenz, D. L., Gedde, S. J., & Imami, N. R. (2001). Long-term surgical outcomes of patients with glaucoma secondary to the iridocorneal endothelial syndrome. Ophthalmology, 108(10), 1789-1795.