Figure 12-15 Iridocorneal endothelial syndrome with corectopia. (Courtesy of Stephen Orlin, MD.)

ICE syndrome becomes apparent most commonly in middle-aged females and is almost always unilateral. Asymmetric posterior polymorphous dystrophy, as well as other causes of unilateral corneal edema, must be included in the differential diagnosis of ICE syndrome.

Penetrating keratoplasty and endothelial keratoplasty are treatment options for the corneal component of this syndrome. Glaucoma is an important feature of ICE syndrome. Long-term graft clarity depends on the successful control of intraocular pressure, which can be difficult (see BCSC Section 10, Glaucoma).

Carpel EF. Iridocorneal endothelial syndrome. In: Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. 3rd ed. Vol 1. Philadelphia: Elsevier/Mosby; 2011:889–899.

Price MO, Price FW Jr. Descemet stripping with endothelial keratoplasty for treatment of iridocorneal endothelial syndrome. Cornea. 2007;26(4):493–497.

Peripheral cornea guttae

Peripheral cornea guttae (Hassall-Henle bodies) are small, wartlike excrescences that appear in the peripheral portion of Descemet membrane. A normal aging change, they result from the thickening of Descemet membrane that happens throughout life; they occur on the posterior part of the membrane and protrude toward the anterior chamber. With the slit lamp, Hassall-Henle bodies have the appearance of small, dark dimples within the endothelial mosaic; these are best seen by specular reflection. Rarely seen before age 20, they then increase steadily in number with age. When they appear in the central cornea, they are pathologic and are called cornea guttae. Central cornea guttae associated with progressive stromal and eventually epithelial edema represent Fuchs endothelial dystrophy (see Chapter 10).

Melanin pigmentation

Deposits of melanin on the corneal endothelium can be observed in patients with glaucoma associated with pigment dispersion syndrome. Typically, the cluster of vertically oriented pigments is known as

Krukenberg spindle (see Table 12-3).

Scleral Degenerations

Scleral rigidity increases in older people, and there is a relative decrease in scleral hydration and the