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[Orbital exenteration in therapy-resistant painful scleritis-associated inflammation of orbital soft tissues].

PATIENT: Presentation of a 67-year-old female with persisting left hemifacial pain - very intense in the periorbital area, and an ipsilateral red eye. The patient had received analgesics and corticosteroids systemically, but no significant improvement was achieved. She had a history of anterior nodular scleritis in her left eye three years ago. Immunologic screening, especially for Wegener's granulomatosis, was negative. The ophthalmic evaluation revealed a left blind eye with diffuse anterior scleritis, an intercalar staphyloma nasally and superiorly, absolute angle closure glaucoma with rubeotic iris and mature cataract. With a 3 mm proptosis with a totally immurable globe, the MRI demonstrated a diffuse orbital tumour with involvement of intraconal space and extraconal muscles. A lid-sparing exenteration of the left orbit was performed. Postoperatively, the patient recovered rapidly and had no more pain. Macroscopically, the orbital fat was replaced by dense scar tissue. Microscopically, a focal necrotising granulomatous vasculitis of small vessels and scleritis with zonal granulomatous reaction on scleral collagen was observed. CONCLUSIONS: Unilateral proptosis with anterior and posterior scleritis combined with granulomatous inflammation of orbital soft tissues may be the first symptom of Wegener's granulomatosis. In addition to systemic immunosuppression, the lidsparing orbital exenteration may be one option of therapy.

Aged↗

Deep intramuscular methylprednisolone treatment of recurrent scleritis.

PURPOSE: To evaluate the efficacy of deep intramuscular methylprednisolone (IMMP) in the treatment of recurrent scleritis. METHODS: A total of 15 patients with scleritis (12 anterior, 3 pan) underwent IMMP injection deep into the thigh. Thirteen patients were already being treated with oral non-steroidal anti-inflammatory drugs, oral prednisolone, an oral immunosuppressive agent, or a combination of these drugs. A second IMMP injection was given to nine patients, making a total of 24 injections. Follow-up ranged from 4 to 18 months. RESULTS: There was a documented improvement in scleritis after 21/24 (87.3%) injections with a mean duration of improvement of 5.7 months. No patient required the introduction of oral corticosteroid or immunosuppressive agent, and only one patient required an increase in methotrexate to control the disease. No systemic, injection site, or ocular side effects were noted. CONCLUSIONS: Deep IMMP is a safe and effective treatment for scleritis. It ensures compliance, avoids the systemic side effects of oral corticosteroids, and is easily repeatable in the outpatient setting.

Adult↗

Recurrent nodular scleritis preceding an adult TINU syndrome.

PURPOSE: To describe the occurrence of nodular scleritis in a patient developing tubulointerstitial nephritis and uveitis (TINU) syndrome. DESIGN: Observational case report. METHOD: The case notes of a 57-year-old female presenting with recurrent nodular scleritis, who later developed interstitial nephritis with bilateral uveitis, were reviewed. RESULTS: Common and established causes of scleritis were not present in an adult who developed the TINU syndrome a decade later. CONCLUSIONS: Although scleritis has not been reported as a tubulointerstitial nephritis-associated ocular inflammation, it may be part of the possible spectrum of ocular inflammation occurring as part of the TINU syndrome.

Acute Disease↗

Management of infectious scleritis after pterygium excision.

PURPOSE: We sought to describe the clinical features, responsible pathogens, management, and prognosis of infectious scleritis after pterygium excision. METHODS: A retrospective study through review of medical records of patients diagnosed with infectious scleritis after pterygium excision over a 10-year period at our institution. RESULTS: A total of 16 cases of infectious scleritis after pterygium excision was identified. Among them, eight were associated with sclerokeratitis, and six had multifocal scleral nodules with subconjunctival abscesses. Culture results were positive in 15 (93.8%) cases. Pseudomonas was isolated in 13 (81.3%) patients, fungus in three (18.8%), and two had a mixed growth (12.5%). Based on the in vitro susceptibility test, four (31%) Pseudomonas isolates were resistant to gentamicin, whereas all isolates were sensitive to amikacin. During the course of treatment, eight cases were complicated by vitreous opacity, four developed glaucoma, four had serous retinal or choroidal detachment, and two had secondary cataract. Scleral infection recurred in two patients after cessation of therapy. Among the nine patients treated with medical therapy, two eyes were enucleated, whereas only two attained a visual acuity of > or =2/200 at the end of the follow-up period. On the other hand, seven patients had combined antibiotic therapy and surgical debridement. The number of surgical debridement ranged from one to three, with an average of 1.4. In this combined-treatment group, only one patient required enucleation, and five cases attained a visual acuity of > or =2/200. The duration of hospitalization for patients with combined treatment was 21.2+/-4.8 days compared with the 28.4+/-5.0 days for those with medical treatment alone (p = 0.035). CONCLUSION: Surgical debridement in combination with appropriate antimicrobial therapy shortens the course of treatment and improves the visual outcome of severe infectious scleritis after pterygium excision.

Aged↗

Recognition of posterior scleritis and its treatment with indomethacin.

Posterior scleritis is a rare disease, the clinical signs of which may vary. There is no consensus on the appropriate method of treatment for this disease. Some have advocated treatment with nonsteroidal antiinflammatory drugs, whereas others have reported variable success with potentially more toxic therapy. Patients with posterior scleritis constitute approximately 1% of the uveitis clinic population at the Casey Eye Institute in Portland, Oregon. The cases of 6 patients with posterior scleritis, the diagnosis of which was elusive, are reported. Posterior scleritis was generally confirmed by ultrasound examination. Each of the patients responded to treatment with indomethacin, which was usually the sole method of nontopical therapy. Thus, recognition of this relatively rare disease had marked implications for treatment.

Adult↗

Posterior scleritis secondary to Pseudomonas aeruginosa keratitis.

PURPOSE: To report a case of Pseudomonas aeruginosa keratitis that progressed to posterior scleritis. METHODS: Observational case report. RESULTS: A 64-year-old woman was treated for P. aeruginosa keratitis. Twelve days later, she was diagnosed with posterior scleritis and treated with oral ciprofloxacin. Her condition progressed, ultimately resulting in enucleation. Histopathology confirmed posterior scleritis, a previously unreported complication of P. aeruginosa keratitis. CONCLUSION: P. aeruginosa keratitis may progress to posterior scleritis, a potentially devastating complication.

Anti-Infective Agents↗

Rheumatoid arthritis-associated necrotizing scleritis and peripheral ulcerative keratitis treated successfully with infliximab.

In patients with rheumatoid arthritis (RA), necrotizing scleritis, an ocular manifestation of a systemic vasculitic process, is associated with significant ocular morbidity and high mortality. We present a 60-year-old man with RA who developed necrotizing scleritis and peripheral ulcerative keratitis. The scleritis was refractory to local measures, systemic corticosteroids, and cyclophosphamide but responded rapidly to infliximab. Our case illustrates that biologic agents may be considered in refractory cases of sight- and life-threatening scleritis.

Antibodies, Monoclonal↗

Diffuse granulomatous necrotizing scleritis.

CASE REPORT: A 34-year-old man presented with anterior scleral thinning in the right eye (RE) and a painful nodular scleritis in the left eye (LE). Fundus examination showed a healed vasculitis and an inferior epiretinal neovascular membrane in the LE. Topical and systemic oral steroids and antiviral medication were prescribed. One year later, optic disc hyperaemia and swelling and macular oedema became apparent in the LE. Pulsed intravenous steroids were administered for 1 year, when a nasal septum perforation and vitreous haemorrhage in the LE were diagnosed. The eye was enucleated 3.5 years after the initial complaint. Necrotizing granulomatous tissue replacing the sclera and subconjunctival granulomatous tissue were observed. Six months later, oedema and neovascularization of the right optic disc were observed and cyclophosphamide was started, with regression of the clinical signs. No systemic abnormalities have so far become apparent. DISCUSSION: Posterior scleritis is most often observed in patients with no signs of associated systemic autoimmune disease. The diagnosis in this case is most probably one of Wegener's granulomatosis (WG). In WG, the diagnosis is based on necrotizing granulomas of the respiratory tract, generalized focal necrotizing vasculitis and focal necrotizing glomerulonephritis. Eye involvement with WG has been reported in up to 58% of cases. Evaluation of the scleritis patient should include a detailed medical history, chest X-rays, blood tests, autoantibody serology and analysis of urinary sediment. Cyclophosphamide is the treatment of choice for patients with ocular manifestations of WG, polyarteritis nodosa or rheumatoid arthritis, either alone or in combination with systemic steroids. Visual loss is expected in 85% of individuals with severe necrotizing posterior scleritis.

Adult↗

Haemophilus influenzae associated scleritis.

AIMS: To describe the clinical course and treatment of Haemophilus influenzae associated scleritis. METHODS: Retrospective case series. RESULTS: Three patients developed scleritis associated with ocular H influenzae infection. Past medical history, review of systems, and laboratory testing for underlying collagen vascular disorders were negative in two patients. One patient had arthritis associated with an antinuclear antibody titre of 1:160 and a Westergren erythrocyte sedimentation rate of 83 mm in the first hour. Each patient had ocular surgery more than 6 months before developing scleritis. Two had cataract extraction and one had strabismus surgery. Nodular abscesses associated with areas of scleral necrosis were present in each case. Culture of these abscesses revealed H influenzae in all patients. Treatments included topical, subconjunctival, and systemic antibiotics. Scleral inflammation resolved and visual acuity improved in each case. CONCLUSION: H influenzae infection may be associated with scleritis. Accurate diagnosis and treatment may preserve ocular integrity and good visual acuity.

Aged↗

Bilateral posterior scleritis.

PURPOSE: To describe an unusual presentation of posterior scleritis in a healthy young patient. METHOD: We reviewed the medical record of 21-year-old man for clinical presentation, course, and ancillary studies. RESULTS: The healthy patient had a painless, bilateral posterior scleritis that presented with a combination of circumscribed fundus masses and exudative macular detachment. On fluorescein angiography, different hyper-fluorescent patterns characterized the two manifestations of posterior scleritis. An ultrasound confirmed the scleral thickening. Fundoscopic abnormalities resolved with oral prednisone. CONCLUSION: Pain is not always present in this disease. A combination of exudative macular detachment and circumscribed fundus masses can be a form of presentation in posterior scleritis.

Adult↗

Necrotizing scleritis following diode laser trans-scleral cyclophotocoagulation.

We report a case of necrotizing scleritis following diode cyclo-photocoagulation (CPC). A-64-year-old-Asian Indian male presented with pseudophakic corneal decompensation with secondary angle closure glaucoma that was uncontrolled with maximum medical therapy. Trans-scleral diode CPC was performed in the inferior 180-degree section using a standard diode laser with a G-probe. One month later, the patient developed features suggestive of necrotizing scleritis that resolved with systemic steroid therapy. Necrotizing scleritis following diode CPC has been rarely reported. Ophthalmologists should be aware of the possible occurrence of surgically induced nectotizing scleritis following diode CPC.

Follow-Up Studies↗

Posterior scleritis: clinical profile and imaging characteristics.

Posterior scleritis is relatively uncommon and is often misdiagnosed due to its protean manifestations. We report eight cases of posterior scleritis to analyse the clinical profile, ultrasonographic and computed tomography (CT) scan features of this rare disorder. Fundus findings included serous retinal detachment, choroidal folds, retinal folds, subretinal mass, choroidal detachment, disc edema, and macular edema. There was associated anterior scleritis and anterior uveitis in the majority of the cases. In all cases ultrasound with or without CT scan confirmed the clinical diagnosis. All patients responded to systemic steroids except one who required immunosuppressive therapy. This paper describes the clinical profile of a series of posterior scleritis cases highlighting varied clinical presentation, and the role of ultrasound and CT scan findings in the diagnosis.

Adolescent↗

[Transeptal steroids in necrotizing scleritis].

PURPOSE: To analyze the efficacy of transeptal corticosteroid injections in necrotizing scleritis as additional treatment to systemic immunosuppressors. METHOD: Eleven patients with necrotizing scleritis treated with systemic immunosuppressors received transeptal corticosteroid injections during periods of active inflammation. RESULTS: Transeptal corticosteroid injections in addition to systemic immunosuppressors halted active imflammation in 10 of 11 patients with necrotizing scleritis, showing few side effects. Because of that, there was no need to increase dosage nor to switch systemic immunosuppressors. There was no evidence of scleral perforation, scleral thinning and no cases of staphyloma were observed. CONCLUSIONS: Systemic immunosuppressors remain as the first choice treatment for necrotizing scleritis; however, transeptal corticosteroid injections may be helpful as additional treatment during periods of active inflammation.

Adult↗

Intravital microscopy of leukocyte-endothelial dynamics using the Heidelberg confocal laser microscope in scleritis and allergic conjunctivitis.

PURPOSE: To examine leukocyte-endothelial cell rolling and arrest in human ocular vessels overlying sites of inflammation in various ocular inflammatory diseases in comparison to normal controls using the Heidelberg confocal laser microscope, which provides images with greater clarity and resolution than the tandem scanning microscope that uses white light. METHODS: Healthy controls (n=8) and patients with active anterior scleritis (n=7) or allergic eye disease (n=4) were scanned using the Heidelberg confocal laser microscope (HRT II) with the Rostock cornea module attachment for a minimum of 5 min at a depth of 45-120 microm from the conjunctival epithelial surface. RESULTS: There was a marked increase in the number of rolling leukocytes in scleritis patients (534+/-119 cells per mm2/min) versus controls (6+/-6 cells per mm2/min; p=0.0002) or allergic patients (59+/-44 cells per mm2/min; p=0.009). No statistically significant increase was seen in allergic patients compared to controls (p=0.059). A similar pattern was seen in the number of arrested leukocytes in patients with scleritis (56+/-23 cells per mm2) in comparison to either those with allergic eye disease or controls (each=0 cells per mm2; p=0.02). CONCLUSIONS: Patients with scleritis have a significantly increased number of rolling and arrested leukocytes in superficial ocular vessels in comparison to patients with mild allergic conjunctivitis and controls. The image quality with this microscope is superior to prior studies with a scanning microscope.

Adult↗

Episcleritis and scleritis.

Episcleritis and scleritis are two common inflammatory disorders encountered by primary care practitioners. Although episcleritis is a self-limited disease that usually resolves within 2 to 3 weeks, severe symptoms may require the use of topical steroids or, in recalcitrant cases, nonsteroidal anti-inflammatory agents (NSAIAs). Scleritis is a painful, generalized inflammation of the eye that is often associated with systemic disease. The necrotizing form of scleritis is the most serious, requiring aggressive treatment with NSAIAs and systemic steroids. Significant sequelae of scleritis include uveitis, glaucoma, cataract, and retinal detachment.

Administration, Topical↗

Scleritis associated with the fibromyalgia syndrome.

We present a case of a patient with scleritis and the fibromyalgia syndrome. Scleritis and the fibromyalgia syndrome are both thought to be caused by an immunological process. This is the first report of a possible association of scleritis and the fibromyalgia syndrome. Additional cases might suggest that the fibromyalgia syndrome be added to the list of etiologies of scleritis.

Female↗

Posterior scleritis. A cause of diagnostic confusion.

The referring diagnoses in seven women with posterior scleritis included intraocular neoplasm, retrobulbar tumor, choroiditis, and idiopathic central serous choroidopathy. In all cases, a localized area of intense posterior scleritis was responsible for the misdiagnosed ocular findings. Features that helped to correctly identify posterior scleritis were as follows: female sex; a history of anterior scleritis; a fundus mass the same color as normal adjacent pigment epithelium; choroidal folds; serous retinal detachment with cloudy fluid; early pinpoint leaking spots from fluorescein angiography; and thickening of the posterior coats of the eye, retrobulbar edema, and high internal reflectivity on ultrasonography. Corticosteroids given for retrobulbar or systemic effect provided effective treatment.

Adult↗

Posterior scleritis.

Posterior scleritis must be considered in the differential diagnosis of many ocular conditions, including angle closure glaucoma, choroidal folds, optic disk edema, circumscribed fundus mass, choroidal detachment, and exudative retinal detachment. Because it is rare, a high index of suspicion is necessary. Anterior scleritis, pain, or a history of collagen-vascular disease, when present, help to alert the clinician to the correct diagnosis. Posterior scleritis affects women more often than men, but annular ciliochoroidal effusion and choroidal folds are more common in men. Exudative macular detachment and a circumscribed fundus mass are more common in women. This paper reviews the world literature on posterior scleritis and describes findings in a series of 43 patients seen at Wills Eye Hospital. It stresses the clinical features and ancillary diagnostic tests that help to establish the diagnosis.

Diagnosis, Differential↗