9.2.1.6Adamantiades–Behçet’s Disease

Diffuse scleritis associated with Adamantiades– Behçet’s disease usually responds to an oral NSAID plus colchicine, 0.6 mg twice daily. The nodular scleritis associated with Adamantiades– Behçet’s disease, however, almost always requires the addition of a short course of systemic corticosteroids as described above for rheumatoid arthritis. Necrotizing scleritis associated with Adamantiades–Behçet’s disease is extraordinarily rare, but like the retinal vasculitis associated with this disease, it too requires the addition of a major immunosuppressive agent, such as chlorambucil (0.01 mg/kg body weight per day), cyclophosphamide (oral or intravenous pulse), or cyclosporine A (5 mg/kg body weight per day). Infliximab is especially effective in treating the ocular inflammation seen in such patients.

9.2.1.7Posterior Scleritis

Posterior scleritis is usually idiopathic and almost never requires the use of a chemotherapeutic drug. Our approach to this disorder has been combination oral NSAID and corticosteroid therapy. Such a combination approach, described in Sect. 9.2.1.1, requires special attention to the gastrointestinal tract (see Sect. 9.2.2). In those instances of posterior scleritis that have not responded to this combination NSAID and systemic corticosteroid therapy, we have used, judicially, retrobulbar orbital floor steroid injections. We have used triamcinolone acetonide (40 mg) in an effort to judge the effect of such regional steroid therapy, and have continued such injections to a maximum of eight.

9.2.1.8 Infectious Scleritis

Our experience in treating infectious scleritis with antimicrobial agents specific for the microbe causing the scleritis is described in Chap. 7.

Ancillary anti-inflammatory therapy may be required. For example, the patient with scleritis associated with syphilis may require not only the intravenous penicillin therapy appropriate for the syphilis, but also systemic NSAID therapy and/or corticosteroids for control of the debilitating inflammation. A major caveat in this regard is that any patient with fungal scleritis must not

under any circumstances, be treated with corticosteroids, and a patient with bacterial scleritis may be treated judicially with steroids only after the physician is certain that the infectious agent has been properly identified, specific antibiotic therapy to which the agent is susceptible has been instituted, and the infectious process has begun to respond.

9.2.2Ancillary Therapy

It is not uncommon for patients who require prolonged therapy with oral NSAIDs to require ancillary treatment for the gastrointestinal side effects caused by this class of drugs. Patients who take systemic steroid and NSAIDs are at high risk for gastrointestinal mucosal ulceration, and fatal gastrointestinal bleeding or peritonitis from ulcer perforation can occur. We use a “step-ladder” approach to ancillary therapy so as to prevent these side effects, beginning with the use of oral antacids and gastric mucosal coating materials, such as Carafate (sucralfate). We add an H2 receptor blocker, such as Zantac (ranitidine hydrochloride), when treating a patient with gastrointestinal mucosal irritation symptoms or a patient with a past history of such symptoms, and we add Cytotec (misoprostol) to the regimen of any patient with a past history of a documented peptic ulcer or any patient who is taking NSAIDs while also taking systemic steroids.

9.2.3Drug Management Responsibility

We have emphasized repeatedly that the responsibility for the details of management of the medications of patients requiring the use of multiple medications or in patients who are taking immunosuppressive drugs must lie with an individual who is, by virtue of training and experience, truly expert in the use of those multiple drugs, and in the anticipation of, recognition of, and treatment of side effects produced by the drugs. Few ophthalmologists are trained to do this and, happily, few are inclined to take on the responsibility.

A “hand-in-glove” collaboration between the ophthalmologist and a chemotherapist works well in the management of patients requiring these medications. Timely communication is essential, with the ophthalmologist apprising the chemotherapist of the ocular condition, for example, whether or not the ocular inflammatory lesions are not completely controlled, whether it is safe and prudent to increase the dose of medication. Rheumatologists and dermatologists, as well as oncology and hematology chemotherapists, must collaborate with the ophthalmologists vis-à-vis the ocular needs of the patient for more or less therapy.

9.2.4Surgical Treatment

Fig. 9.1 Keratectomy and sclerectomy in a patient with necrotizing scleritis and associated peripheral ulcerative keratitis

Surgical therapy is rarely necessary in the care of patients with scleritis. It is virtually never necessary except in instances of necrotizing scleritis that have advanced to the point of perforation of the globe or to such a point of threatened perforation that prudent physicians would deem to require scleral reinforcement. The central, critical element in the successful surgical plan in these cases of necrotizing scleritis requiring scleral grafting is, in fact, not surgical: The ophthalmologist must understand that the immunological processes that resulted in destruction of the patient’s sclera will invariably result in destruction of any graft material, scleral or otherwise (periosteum, fascia lata, etc.), used to reestablish the integrity of the globe unless such immunological processes are suppressed. Therefore the most essential element of the successful surgical plan for treating a patient with necrotizing scleritis is the institution of the appropriate systemic medical therapy to interrupt the destructive immune phenomenon.

We have successfully treated patients with peripheral ulcerative keratitis and/or necrotizing scleritis by using allograft ocular material, without additional nonocular materials [11]. For patients with necrotizing scleritis associated with peripheral ulcerative keratitis we prefer a preserved (frozen) whole globe. Evidence suggests that immunoreactivity of such tissue without

Fig. 9.2 A lamella of corneoscleral tissue being elevated by forceps, disclosing occult areas of tissue digestion not observable prior to surgery

living cells is extremely low, and we know of no instance in which rejection of such material has complicated the course of the patient with peripheral ulcerative keratitis and necrotizing scleritis who required grafting and surgical reinforcement of the globe. The recipient surgical bed is prepared, first by a generous conjunctival resection surrounding the area of peripheral ulcerative keratitis, then by removal of the ulcerating corneal lamellae, then by removal of the thin border of necrotic conjunctiva surrounding the focus of scleral necrosis, and finally by removal of necrotic sclera itself (Figs. 9.1–9.3). This latter step can be extremely tedious, and carries with it the potential risk of perforation of the choroid with subsequent ocular hemorrhage. We have not personally encountered this, but in instances in

Fig. 9.3 Same eye as in Figs. 9.1 and 9.2: The necrotic tissue has been resected and the surgical bed prepared for receiving graft tissue

Fig. 9.5 Same eye as in Figs. 9.1–9.3, after securing of the corneoscleral graft

Fig. 9.6 Same patient as in Fig. 9.5, after mobilizing a conjunctival flap over the corneoscleral graft

Fig. 9.4 Diagrammatic illustration of the fitting of a corneoscleral graft to the surgical bed previously prepared (see Fig. 9.3)

which the choroid is exposed or in which the only tissue overlying the choroid is the necrotic sclera to be removed (lest it remain a stimulant for nonspecific inflammation) the scleral dissection requires great care, patience, and often a great deal of time.

Once the limits of the surgical bed to be reinforced with donor material have been defined, we create a template, using a piece of plastic surgical drape, of the surgical bed; this template is then used to outline the size of the graft to be excised from the donor eye. The outlined graft material is excised, trimmed of nonscleral material, trimmed to fit the surgical recipient bed (Fig. 9.4), and secured with interrupted 10–0 and/or 9–0 nylon

sutures (Fig. 9.5). The knots are rotated into the tissue (buried) and, if possible, conjunctiva surrounding the surgical site is undermined and advanced over the graft and secured with 7–0 Vicryl sutures (Fig. 9.6). In instances in which uveal ectasia is pronounced, securing the graft into the graft bed can be facilitated by first performing an anterior chamber paracentesis to partially decompress the globe.

In instances in which peripheral ulcerative keratitis is not part of the destructive lesion either frozen sclera, a frozen globe or glycerinpreserved sclera can serve as a source for the graft material. Glycerine-preserved sclera must be thoroughly washed and allowed to rehydrate in balanced salt solution for approximately 10 min prior to grafting.

Vascularization of the graft and repopulation of it by recipient fibroblasts may take many