Anti-IL-2 receptor therapy is an effective therapeutic approach for disorders with a predominant Th1 profile. Infliximab (3– 10 mg/kg given as intravenous infusion followed with additional similar doses at 2 and 6 weeks after the first infusion, and every 8 weeks thereafter) and etanercept (50 mg per week given as two 25 mg subcutaneous injections at separate sites) are tumor necrosis factor alpha (TNF-α) blockers.

Medical

In patients with infectious scleritis surgical debridement of the infected tissues, systemic, topical and subconjunctival antibiotic treatments are required. Treatment is needed for six or more weeks. In active stages of the disease corticosteroid and immunosuppressive treatments are contraindicated.

Surgical

In cases of extreme scleral thinning or perforation reinforcement is required. Donor sclera, fascia lata, periosteum, splitthickness dermis, aortic tissue, or artificial materials such as Gore-Tex can be used for this purpose. To maintain the integrity of the globe this tectonic surgery should always be accompanied with systemic immunosuppressive therapy.

COMPLICATIONS

Ocular complications are not uncommon and are more frequently seen with necrotizing scleritis. Corneal complications are the most frequent, and include peripheral corneal thinning, peripheral ulcerative keratitis, stromal keratitis, and sclerosing keratitis. Peripheral ulcerative keratitis is the most severe form of corneal involvement. It is usually associated with necrotizing scleritis and if not treated may lead to perforation. Stromal keratitis is the extension of the inflammation into the cornea that can lead to sclerocornea. Uveitis is seen in about one-third of patients. It is more frequently anterior and occurs later during the course of the inflammation. Posterior uveitis is relatively rare and usually appears in patients with posterior scleritis. Increased intraocular pressure can develop due to the uveitis and scleral edema. Open angle glaucoma, angle closure glaucoma and neovascular glaucoma may occur. Cataracts can also develop due to prolonged inflammation secondary to longterm steroid treatment.

348 SCLEROMALACIA PERFORANS

379.04

Gregory J. McCormick, MD

Rochester, New York

James V. Aquavella, MD

Rochester, New York

ETIOLOGY

The term scleromalacia perforans was coined by van der Hoeve in 1934 to describe a rare form of scleritis that occurs mainly in postmenopausal women with long-standing polyarticular rheumatoid arthritis. The condition also occurs as a sequela of ocular carcinoma in acquired immune deficiency syndrome (AIDS) and in predisposed patients following a latent period after ocular surgery with or without intraoperative antimetabolite application.

COURSE/PROGNOSIS

The principal feature is the formation of multiple areas of deep scleral ulceration that coalesce to reveal the underlying uveal tissue. The condition is of insidious onset and is typically painless and characterized by a lack of clinical signs of inflammation. Yellowish necrotic lesions appear in the anterior sclera between the limbus and the equator ultimately resulting in sequestration of necrotic tissue, scleral thinning, and a characteristic bluish hue. Tissue loss apparently follows occlusion of smaller episcleral capillaries with pathology characterized as immune complex-mediated vasculitis. Although the overlying conjunctiva may be thinned, spontaneous perforation and anterior staphyloma formation are rare and are usually associated with glaucoma. However, these eyes are at high risk from the occurrence of even minor trauma (Figure 348.1).

DIAGNOSIS

The diagnosis is made by identifying a necrotizing anterior scleritis in the absence of significant pain or objective signs of

inflammation. Although the number of lesions varies, they can coalesce into large areas of scleral necrosis. Associated findings may include uveitis, secondary glaucoma, cataract, and perforation. All of the lesions share the same pathologic features. A history of rheumatoid arthritis and the presence of rheumatoid nodules in other parts of the body are common in this patient population.

Clinical signs and symptoms

Ocular or periocular

●Cornea: aseptic necrosis, pannus, ulceration.

●Extraocular muscles: limitation of motion, tendonitis.

●Sclera: episcleritis, scleritis, anterior staphyloma.

●Other: cataract, retinal vasculitis, secondary glaucoma, uveitis.

TREATMENT

Ocular

Although the clinical appearance is non-inflammatory, the underlying cause is believed to be inflammatory in nature and therefore the mainstay of treatment is immunomodulatory and is reserved for patients with symptoms, progressive disease or complications. Topical prednisolone and prednisone, typically 100 to 250 mg daily, in combination with other antiinflammatory agents such as phenylbutazone or cytotoxic agents (cyclosphamide), may be helpful. Low-dose cyclosporin A (5 mg/kg per day) has been reported to be effective in conjunction with a minimum controlling systemic steroid dosage. Intensive anti-inflammatory therapy seems to produce rapid resolution and may prevent progression of lesions. Patients with scleromalacia perforans often suffer from rheumatoid arthritis, so a comprehensive medical approach is indicated. Nonsteroidal anti-inflammatory drugs have not been proven to be of value. Once the inflammatory process has been stabilized, steroids should be slowly tapered over a period of several weeks.

Surgical

Surgical techniques are directed towards reinforcing areas of thinned sclera, anterior staphyloma or perforation. Free or rotational scleral autografts have been used with success in treating some lesions. Larger areas of thinning have been repaired with preserved sclera, fascia lata from the lateral thigh, cadaveric aorta, periosteum from the anterior tibial crest, tarsus, dura mater, preserved pericardium, and split thickness dermis. The surgeon should be careful not to rupture the choroid during the placement of the graft. Intraoperative cautery of prolapsing areas has been repored to safely shrink tissue without evidence of fundus abnormalities 6 months after grafting surgery. It is important for the grafts to be covered totally by sliding or free conjunctival grafts when possible. Larger grafts have been successfully epithelialized after being covered with buccal mucous membrane and more recently with amniotic membrane transplantation (Figure 348.2).

FIGURE 348.2. Postoperative appearance after placement of periosteal graft and hydrophilic bandage contact lens.

effects of these drugs. Periocular injections of corticosteroids are absolutely contraindicated. When surgical therapy is undertaken, the surgeon should be aware of the potential for recurrence. Finally, it is important to differentiate true scleromalacia perforans from other forms of scleral necrosis secondary to inflammatory conditions in which patients are more symptomatic.

COMMENTS

Scleromalacia perforans, although uncommon, poses a major management problem. If the patient is asymptomatic in the absence of complications, it may be that no therapy is necessary. Once a decision to intervene has been made, comprehensive medical therapy with both topical and systemic corticosteroids, often accompanied by immunosuppressive drugs, must be instituted. If these measures fail, surgical modalities using overlying grafts with various materials may have to be considered.

REFERENCES

Bick MW: Surgical treatment of scleromalacia perforans. Arch Ophthalmol 61:907–917, 1959.

Breslin CW, Katz JI, Kaufman HE: Surgical management of necrotizing scleritis. Arch Ophthalmol 95:2038–2040, 1977.

Hakin KN, Ham J, Lightman SL: Use of cyclosporin in the management of steroid-dependent non-necrotizing scleritis. Br J Ophthalmol 75:340– 341, 1991.

Watson PG, Hazleman BL: The sclera and systemic disorders. Philadelphia, WB Saunders, 1976.

Watson PG: The nature and the treatment of scleral inflammation. Trans Ophthalmol Soc UK 102:257–281, 1982.