Iridocorneal Endothelial Syndrome

Iridocorneal endothelial (ICE) syndrome is a spectrum of acquired unilateral abnormalities of the corneal endothelium, anterior chamber angle, and iris. There are 3 recognized clinical variants of ICE (when combined, the first letter of each variant also forms the mnemonic ICE):

• iris nevus (Cogan-Reese) syndrome

• Chandler syndrome

• essential progressive iris atrophy

Figure 7-5 Axenfeld-Rieger syndrome. A, Clinical photograph of the anterior segment in a patient with Axenfeld-Rieger syndrome. Iris atrophy, pseudopolycoria, and iris strands in the periphery are present. Posterior embryotoxon can be seen laterally (arrows).B, Gross photograph shows a prominent Schwalbe line and the anterior insertion of iris strands (Axenfeld anomaly). C, Light micrograph shows the anterior attachment of iris strands (arrow) to the Schwalbe line (arrowhead).

(Part A courtesy of Wallace L.M. Alward, MD. Copyright University of Iowa. Part B courtesy of Robert Y. Foos, MD. Part C modified with permission from Yanoff M, Fine BS. Ocular Pathology: A Color Atlas. New York: Gower; 1988.)

All forms of ICE syndrome, which typically affects young to middle-aged adults, have the following 2 features in common: epithelial-like metaplasia of the corneal endothelium and abnormal proliferation of the corneal endothelium. Abnormal endothelial cells migrate over the anterior chamber angle while laying down new basement membrane (Descemet membrane), leading to the formation of peripheral anterior synechiae (PAS) and subsequent secondary angle-closure glaucoma in approximately half of patients with this condition (Fig 7-7). See BCSC Section 8, External Disease and Cornea, and Section 10, Glaucoma, for further discussion.

• Silva L, Najafi A, Suwan Y, Teekhasaenee C, Ritch R. The iridocorneal endothelial syndrome. Surv Ophthalmol. 2018;63(5):665–676.

Figure 7-6 Collection of neutrophils and necrotic debris in the anterior chamber (hypopyon). The cornea (bracket) has a dense inflammatory infiltrate in the stroma, resulting in the hypopyon. Inflammation may result in the formation of peripheral anterior synechiae in the angle, leading to secondary angle-closure glaucoma.

(Courtesy of Steffen Heegaard, MD.)

Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.