Delhi Journal of Ophthalmology

Cogan- Reese Syndrome: A Variant of Iridocorneal Endothelial Syndrome

Guddeti Praveena, Malleswari Medikonda, Nandish Mashru 
Sankara Eye Hospital, Pedakakani, Guntur, India

Corresponding Author:

Guddeti Praveena 
DNB
Sankara Eye Hospital, 
Pedakakani, Guntur, India
Email id: Venus.Reddy88@Gmail.Com

Published Online: 30-APR-2018

DOI:http://dx.doi.org/10.7869/djo.366

Abstract
Iridocorneal Endothelial Syndrome (ICE) is a unique ophthalmic disorder characterized by the presence of an abnormal corneal endothelium which leads to varying degrees of corneal edema, progressive iris atrophy and secondary angle closure glaucoma without pupillary block. Cogan Reese syndrome is one of the three clinical variants of Iridocorneal Endothelial Syndrome (ICE) characterized by nodular, pigmented lesions of the iris which are hallmark, and may be seen with the entire spectrum of corneal and other iris defects. Secondary glaucoma is the main sight threatening complication. Management depends on severity of ocular findings and is targeted to control secondary glaucoma medically and surgically. We hereby report two middle aged male patients who were diagnosed to have the Cogan-Reese variant of ICE Syndrome based on clinical findings.

Keywords :Cogan Syndrome, Iridocorneal Endothelial Syndrome, Iris Nevus Syndrome

Introduction

The term Iridocorneal Endothelial Syndrome (ICE) was suggested by Eagle and Yanoff which denotes a spectrum of disease characterized by primary corneal endothelial abnormality.1 Three clinical variants viz. Chandler Syndrome, Essential (Progressive) Iris Atrophy, Cogan-Reese Syndrome (Iris Nevus Syndrome) represent a spectrum of ICE syndrome.

The clinical features in general includes presentation in early to middle adulthood, predilection for women, reduced visual acuity, pain and unilateral occurrence with varying degrees of corneal edema, iris atrophy, angle abnormalities and secondary glaucoma.2,3 

The true etiology of ICE syndrome is unclear. Alvarado et al.4 have proposed a viral cause for the disease, based on the presence of inflammatory cells on histological analysis. The abnormal endothelial cells may migrate posteriorly, forming a membrane that covers the adjacent structures, iris and trabecular meshwork.3 The contraction of this membrane leads to characteristic iris changes, iridotrabecular synechiae, corectopia with the pupil being drawn towards the area where the synechiae are most prevalent and to secondary angle-closure glaucoma.

Case Report- 1

A 35 year old male patient presented to us with blurring of vision the left eye. On examination of the left eye, BCVA was 6/9. The cornea was clear, iris showed mammillary bodies, pupil was irregular with ectropion uvea. IOP  with applanation tonometry (AT) was 56 mm of Hg. Gonioscopy showed synechial angle closure of more than 270°. Fundus showed cup-disc ratio of 0.9:1. The right eye was within normal limits. The patient was investigated with CCT (546µ) and HFA (Figure 1) and diagnosed to be a case of OS Cogan Reese Syndrome. The patient was treated with antiglaucoma medication. Nd:Yag laser peripheral iridotomy was performed in view of shallow anterior chamber. Trabeculectomy with peripheral iridectomy with anti-metabolites (MMC) was performed because of uncontrolled IOP in spite of maximal medical treatment. The surgery was uneventful and IOP was under control for 8 months after which the patient was restarted with AGM. Repeat trabeculectomy with peripheral iridectomy with anti-metabolites (MMC) was performed later (Figure 2 & Figure 3) and the IOP  was under control for a year. Later, he presented with circum-corneal congestion and corneal edema with IOP of 46 mm of Hg and he was advised glaucoma drainage device surgery.





Case Report - 2

A 39 year old male patient who was a known case of glaucoma with no relevant family history, presented to us with blurring of vision in the left eye. On examination, BCVA in the left eye was 6/36 with head turned to the right. The cornea was clear, iris showed mammillary bodies (Figure 4), pupil was irregular with ectropion uvea. IOP by applanation tonometry was 30 mm of Hg. Gonioscopy showed synechial angle closure of more than 270°. Fundus showed cup-disc ratio of 0.9:1. The right eye was within normal limits. The patient was investigated with CCT (457µ) and ONH OCT (Figure 5). The patient was unable to perform HFA as there was no target view in the primary position. He was diagnosed to be a case of OS Cogan Reese Syndrome. The patient was treated with antiglaucoma medication.


Discussion

Long term follow up is necessary in cases of ICE syndrome as the disease itself is progressive in nature. Serial IOP monitoring and HFA are to be performed to assess the progression and severity of the disease. 

Management of glaucoma in ICE is complex. Glaucoma is initially treated with full range of medical therapy but as the entire angle is progressively covered by a membrane or sealed by synechia, medical therapy or angle surgery eventually fail because of relentless angle closure. Functioning filtering blebs often fail after two to five years probably due to proliferation of a membrane over the internal opening of the sclerostomy, despite the use of adjunctive anti-metabolite therapy.5 Repeat trabeculectomy with antimetabolite therapy6 or glaucoma drainage device surgeries can be attempted in such cases.

The reported success rate for trabeculectomy with anti-fibrotic agents ranged from 60% to 73% at one year and 21% to 29% at 5 years.7,8 Success rates with drainage device surgery have been reported to be about 70% at one year and 53% at 5 years.9 

In our case-1, despite maximum medical therapy and repeated trabeculectomies with adjunctive antimetabolite therapy, IOP was not under control and hence glaucoma drainage device surgery was opted. In view of failed trabeculectomies with adjunctive antimetabolite in a span of one year glaucoma drainage device surgery was opted as the first, choice of surgery in case-2. The outcome of glaucoma drainage device surgery is yet to be studied.

In conclusion, though ICE syndrome is uncommon in routine practice, it attracts much attention in its diagnosis and treatment. This case reports stands out due to its controversies and complexities because ICE syndrome typically affects middle aged women whereas in our study we report two middle aged male patients affected with it.

References
  1. Eagle RC Jr, Font RL, Yanoff M, et al. Proliferative endotheliopathy with iris abnormalities. The iridocorneal endothelial syndrome. Arch Ophthalmol 1979; 97:2104-11.
  2. 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.
  3. Hirst LW, Quigley HA, Stark WJ, et al. Specular microscopy of iridocorneal endothelia syndrome. Am J Ophthalmol 1980; 89:11-21.
  4. Alvarodo JA, Murphy CG, Jusrer RP, Herheringron J. Pathogenesis of Chandler’s syndrome, essential iris atrophy and the Cogan-Reese syndrome. II. Estimated age at disease onset. Invest Ophthalmol Vis Sci 1986; 27:873-82.
  5. Wright MM, Grajewski AL, Cristol SM, Parrish RK. 5-Fluorouracil after trabeculectomy and the iridocorneal endothelial syndrome. Ophthalmology 1991; 98:314-6. 
  6. Kidd M, Hetherington J, Magee S. Surgical results in iridocorneal endothelial syndrome. Arch Ophthalmol 1988; 106:199-201.
  7. Laganowski HC, Kerr Muir MG, Hitchings RA. Glaucoma and the iridocorneal endothelial syndrome. Arch Ophthalmol 1992; 110:346-50. 
  8. Doe EA, Budenz DL, Gedde SJ, et al. Long-term surgical outcomes of patients with glaucoma secondary to the iridocorneal endothelial syndrome. Ophthalmology 2001; 108:1789-95.
  9. Kim DK, Aslanides IM, Schmidt CM Jr, Spaeth GL, Wilson RP, Augsburger JJ. Long-term outcome of aqueous shunt surgery in ten patients with iridocorneal endothelial syndrome. Ophthalmology 1999; 106:1030-4.

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Guddeti Praveena, Malleswari Medikonda, Nandish MashruCogan- Reese Syndrome: A Variant of Iridocorneal Endothelial Syndrome.DJO 2018;28:72-74

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Guddeti Praveena, Malleswari Medikonda, Nandish MashruCogan- Reese Syndrome: A Variant of Iridocorneal Endothelial Syndrome.DJO [serial online] 2018[cited 2021 Dec 6];28:72-74. Available from: https://www.djo.org.in/articles/28/4/Cogan-Reese-Syndrome.html