presence of total synechial angle closure, panretinal photocoagulation may improve the success rate of subsequent glaucoma surgery by eliminating the angiogenic stimulus and may decrease the risk of hemorrhage at the time of surgery. More recently, anti-VEGF agents have been successfully employed to promote regression of the neovascular tissue prior to filtering surgery (Fig 5-14).

Figure 5-14 A, Slit-lamp photograph of florid iris neovascularization taken 15 minutes before injection of bevacizumab. B, Regression of iris neovascularization 4 days after treatment with bevacizumab. (Courtesy of Nicholas P. Bell, MD.)

Medical management of neovascular glaucoma yields variable success but often is a temporizing measure until more definitive surgical or laser treatment is undertaken. Topical β-adrenergic antagonists, α2-adrenergic agonists, carbonic anhydrase inhibitors, cycloplegics, and corticosteroids may be useful in reducing IOP and decreasing inflammation either as a long-term remedy or prior to filtering surgery. Filtering surgery has a better chance of success once the neovascularization has regressed after panretinal photocoagulation. The use of the antifibrotics 5-fluorouracil and mitomycin C has been shown to increase the success rate of and decrease the final IOP following trabeculectomy in patients with neovascular glaucoma. A variety of glaucoma drainage devices have also been successfully implanted to control the IOP in neovascular glaucoma and, in many cases, glaucoma drainage device implantation is the surgical procedure of choice. If these therapies fail, either endoscopic or transscleral cyclophotocoagulation, or less often, cyclocryotherapy, may help reduce the IOP.

Iliev ME, Domig D, Wolf-Schnurrbursch U, Wolf S, Sarra GM. Intravitreal bevacizumab (Avastin) in the treatment of neovascular glaucoma. Am J Ophthalmol. 2006;142(6):1054–1056.

Jonas JB, Spandau UH, Schlichtenbrede F. Intravitreal bevacizumab for filtering surgery. Ophthalmic Res. 2007;39(2):121–122. Sivak-Callcott JA, O’Day DM, Gass JD, Tsai JC. Evidence-based recommendations for the diagnosis and treatment of neovascular

glaucoma. Ophthalmology. 2001;108(10):1767–1776.

Iridocorneal Endothelial Syndrome

Iridocorneal endothelial (ICE) syndrome is a group of disorders characterized by abnormal corneal endothelium that causes variable degrees of iris atrophy, secondary ACG, and corneal edema. BCSC Section 8, External Disease and Cornea, discusses the corneal aspects of ICE syndrome. Three clinical variants have been described: Chandler syndrome, essential (progressive) iris atrophy, and Cogan-Reese syndrome (iris nevus).

The condition is clinically unilateral, presents between 20 and 50 years of age, and occurs more often in women. No consistent association has been found with another ocular or systemic disease, and familial cases are very rare. Patients typically present with elevated IOP, decreased vision due to corneal edema, secondary chronic ACG, or an abnormal iris appearance. In each of the 3 clinical

variants, the abnormal corneal endothelium takes on a “beaten bronze” appearance, similar to corneal guttae seen in Fuchs corneal endothelial dystrophy. Microcystic corneal edema may be present without elevated IOP, especially in Chandler syndrome. The unaffected eye may have subclinical irregularities of the corneal endothelium without other manifestations of the disease.

High PAS are characteristic of ICE syndrome (Fig 5-15), and these often extend anterior to the Schwalbe line. Similar to neovascular glaucoma, the degree of angle closure does not always correlate to the elevation in IOP, because some angles may be functionally closed by the endothelial membrane without overt synechial formation.

Figure 5-15 The classic high PAS seen in ICE syndrome. These PAS extend anterior to the Schwalbe line in this patient with essential iris atrophy. With angle closure, the secondary glaucoma occurs. (Courtesy of Steven T. Simmons, MD.)

Figure 5-16 ICE syndrome. Corectopia and hole formation are typical findings in essential iris atrophy. (Courtesy of Steven T.

Simmons, MD.)

Figure 5-17 Ectropion uveae in a patient with Chandler syndrome. (Courtesy of Steven T. Simmons, MD.)

Various degrees of iris atrophy and corneal changes distinguish the specific clinical entities. The essential iris atrophy variant of ICE syndrome is characterized by severe progressive iris atrophy resulting in heterochromia, corectopia, ectropion uveae, iris stromal and pigment epithelial atrophy, and hole formation (Fig 5-16). In Chandler syndrome, minimal iris atrophy and corectopia occur, and the corneal and angle findings predominate (Fig 5-17). Chandler syndrome is the most common of the clinical variants and makes up approximately 50% of the cases of ICE syndrome. The iris atrophy also tends to be less severe in Cogan-Reese syndrome. This condition is distinguished by tan pedunculated nodules or diffuse pigmented lesions on the anterior iris surface.

Glaucoma occurs in approximately 50% of patients with ICE syndrome, and the glaucoma tends to be more severe in essential iris atrophy and Cogan-Reese syndrome. In this condition, the corneal endothelium migrates posterior to the Schwalbe line onto the trabecular meshwork. Electron microscopy has shown this endothelial layer to vary in thickness, with areas of single and multiple endothelial layers and surrounding collagenous and fibrillar tissue. Unlike with normal corneal endothelium, filopodial processes and cytoplasmic actin filaments are present, supporting the migratory nature of these cells. PAS are formed when this migratory endothelium and its surrounding collagenous, fibrillar tissue contract. A viral cause has been postulated for the mechanism of ICE syndrome after lymphocytes were seen on the corneal endothelium of affected patients.

The diagnosis of ICE syndrome must always be considered in young to middle-aged patients who present with unilateral, secondary ACG. It is particularly important to maintain a high index of suspicion as this condition may mimic primary OAG when the iris and corneal features are subtle. Specular microscopy can confirm the diagnosis by demonstrating an asymmetric loss of endothelial