Oral mucosa for reconstructive surgery in a case of severe inflammatory necrotizing sclero-uveitis

The purpose of this case is to show the efficacy of buccal mucosa as an alternative to treat a case of severe necrotizing sclero-uveitis (NSU) associated with ocular perforation. We show a severe inflammatory NSU case that did not improve with topical treatment and scleral patch. We performed a buccal mucosa graft taken from the lower lip with excellent functional and anatomical result, with no signs of relapse of the NSU after 2 years of follow-up. Buccal mucosa can be a safe, useful, and effective alternative for the reconstruction of the scleral wall.

A case of idiopathic necrotizing scleritis with secondary glaucoma treated successfully with golimumab and Ahmed valve implantation

A 52-year-old woman presented with idiopathic active scleritis not responding to oral cyclophosphamide, azathioprine, and oral steroid. Her intraocular pressure in the left eye was 45 mm of Hg in spite of using combination of brinzolamide 1% and brimonidine 0.2% (thrice a day), timolol maleate 0.5% (twice a day) eye drops in both eyes and oral acetazolamide. She was administered subcutaneous golimumab 50 mg injection every 4 weeks along with oral methotrexate 15 mg/week. The scleral inflammation responded and she underwent Ahmed glaucoma valve implantation after two months of initiation of golimumab therapy. After one week of surgery her IOP in left eye was the reduced to 8 mm of Hg. The index case showed that Golimumab can be a useful drug in the management of necrotizing scleritis refractory to the conventional therapy.

Alteraciones anatomopatológicas en la escleritis anterior nodular necrotizante / Pathological alterations in necrotizing nodular anterior scleritis

RESUMEN La presencia de la escleritis necrotizante es rara; necesita de un diagnóstico y de un tratamiento oportuno, ya que su curso es rápido, severo y destructivo de la integridad del globo ocular. Se presenta una paciente de 43 años de edad, quien requirió atención oftalmológica al presentar el ojo derecho rojo con intenso dolor. Se le encontraron 3 nódulos en la región antero-inferior escleral. Se le realizó punción-aspiración con aguja fina en los nódulos esclerales y se obtuvo un fragmento de uno de ellos para el corte histológico. En ambas muestras se observó infiltrado inflamatorio de linfocitos y neutrófilos con algunas células plasmáticas. Se le impuso tratamiento con prednisona oral (0,5 mg/kg/día), prednisolona colirio y azatioprina a dosis de 1,5 mg/kg/día. La evolución al mes fue satisfactoria con mejoría gradual de las lesiones. El tratamiento se mantuvo por 3 meses con chequeos hematológicos reiterados. Se disminuyeron posteriormente las dosis medicamentosas hasta su eliminación a los 6 meses. La curación de las lesiones fue total. No se encontró enfermedad sistémica en el chequeo clínico efectuado(AU)

ABSTRACT The presence of necrotizing scleritis is rare; it needs a diagnosis and an opportune treatment, since its course is fast, severe and destructive of the integrity of the eyeball. We present a 43-year-old patient who required ophthalmological attention when presenting the red right eye with intense pain. Three nodules were found in the antero-inferior scleral region. Fine needle aspiration was performed on the scleral nodules and a fragment of one of them was obtained for the histological section. In both samples, inflammatory infiltrate of lymphocytes and neutrophils with some plasma cells was observed. He was prescribed treatment with oral prednisone (0.5 mg /kg/day), prednisolone eye drops and azathioprine at a dose of 1.5 mg/kg/day. The evolution to the month was satisfactory with gradual improvement of the lesions. The treatment was maintained for 3 months with repeated blood tests. Drug doses were subsequently reduced until their elimination at 6 months. The healing of the injuries was total. No systemic disease was found during the clinical check-up(AU)

Antineutrophil cytoplasmic antibody-positive scleritis: Clinical profile of patients from a tuberculosis-endemic region

Purpose: To report the clinical profile of a series of antineutrophil cytoplasmic antibody (ANCA)-associated scleritis in Indian population. Methods: We conducted a retrospective review of medical records of 33 eyes of 26 consecutive patients with scleritis, who tested positive for either antibody to proteinase 3 [anti-PR3/cytoplasmic antineutrophil cytoplasmic antibody (cANCA)] or myeloperoxidase [anti-MPO/perinuclear anti-neutrophil cytoplasmic antibody (pANCA)] between 2006 and 2015. Results: The mean age at presentation was 54.1 (11.1) years and 61.5% of the patients were female. Underlying systemic disorder was found in 46.2% of patients and includes granulomatosis with polyangitis (30.8%) and tuberculosis (15.4%). Necrotizing scleritis (48.5%) was the most common scleritis observed, followed by diffuse anterior scleritis (42.4%). Positive cANCA was found in 65.4% of patients and 34.6% was found positive for pANCA. Four of the six patients with positive Mantoux test were started on anti-tuberculosis treatment (ATT) by pulmonologist. Cyclophosphamide was the most common immunosuppressive and 11.5% of the patients required combination of two immunosuppressives. Seventeen eyes developed cataract and four eyes required patch graft. Female gender was more frequently associated with pANCA-associated scleritis than cANCA (P = 0.037). Incidence of necrotizing scleritis was higher in patients with positive cANCA, but this difference was not statistically significant (P = 0.806). cANCA-positive patients had statistically significant higher association with systemic rheumatic diseases (P = 0.021). Conclusion: Necrotizing scleritis is the most common subtype of scleritis in ANCA-positive individuals and even in the absence of systemic involvement. All patients with ANCA positivity should be thoroughly screened to rule out any evidence of tuberculosis, especially in tuberculosis-endemic region before planning aggressive immunomodulatory therapy.

Idiopathic Necrotizing Scleritis, Anterior Uveitis, and Localized Retinal Detachment.

Aim: To present clinical picture and treatment of anterior idiopathic necrotizing scleritis. Methods: Clinical and laboratory examination; B-scan ultrasound; tissue biopsy and histological analysis, were performed. Results: Herewith we depict a case of 74 year old man, with unilateral granulomatous, anterior, necrotizing scleritis. Etiology has, through extensive testing, both laboratorial and clinical, not been proven and thus the patient has been classified as having idiopathic scleritis. Complications on both anterior (anterior uveitis) and posterior (subretinal granulomatous infiltrates with localized retinal detachment) segments of the eye are a consequence of granulomatous necrotizing scleritis. Both tissue biopsy of granulomatous scleral infiltrates and histological analysis showed that this is the case of granulomatous, partially necrotizing scleritis with some elements of vasculitis. Progression of granulomatous scleral infiltrates into the eye has also been shown through clinical examination and B-scan ultrasound, and depicted subretinal lesion of medium reflectivity that is in contact with the epibulbar lesion. Retinal detachment in this area had progressed and required excessive laser photocoagulation barrage and resulted in absorption of subretinal fluid. Positive therapeutic outcome was achieved through the use Methotrexate and corticosteroids. Conclusion: Granulomatous infiltrates that spread towards the subretinal space and result in localized retinal detachment are a rare complication that may occur during the evolution of necrotizing scleritis and require regular monitoring and followup. Treatment, both pharmaceutical and laser photocoagulation, should be adjusted in order to affect progression and prevent possible complications of the disease.

Efficacy of Autologous Tragal Perichondrium Graft after Proper Antifungal Treatment in Fungal Necrotizing Scleritis

PURPOSE: To compare the efficacy of an autologous tragal perichondrium graft after proper antifungal treatment between 2 cases of fungal necrotizing scleritis. CASE SUMMARY: A 58-year-old female was referred to our clinic with fungal necrotizing scleritis of the left eye which had occurred after pterygium removal. Scleral melting around calcification was observed. After proper treatment with antifungal agents, the authors performed autologous tragal perichondrium graft; however, 3 months after surgery, a necrosis of sclera recurred and the, patient underwent additional treatment with antifungal agents. No complication has been observed up to 3 months postoperatively. A 36-year-old male visited our clinic with ocular pain and decreased visual acuity associated with necrotizing scleritis which occurred after local conjunctival resection. After 4 weeks of antifungal treatments, scleral lesions were stabilized and the authors confirmed negative findings with repetitive fungus smear test. Therapeutic autologous tragal perichondrium graft was performed, and no complication was observed 3 months postoperatively. CONCLUSIONS: When treating a patient with fungal necrotizing scleritis, preoperative antifungal therapy and confirmation of negative findings in repetitive fungus smear test are important. Autologus tragal perichondrium graft accompanied with proper antifungal therapy is an effective treatment of fungal necrotizing scleritis.

A Case of Necrotizing Keratoscleritis in Primary Sjogren's Syndrome

We report on a case of necrotizing keratoscleritis in primary Sjogren's syndrome. A 66-year-old female patient who was complaining of ocular pain, tearing and decreased vision in her right eye for the previous two days was admitted to our hospital. Visual acuity in the right eye was hand movement, and initial examination showed a 3.0 x 1.8 mm uveal mass bulging through a corneoscleral melting site in the nasal region of the right eye. Positive anti-nuclear antibody was identified at a titer of 1:320 with a speckled pattern, and both Sjogren's syndrome A and Sjogren's syndrome B antibody tests were positive, with titers >200 U/mL. A technetium 99m pertechnetate salivary scan revealed chronic sialoadenitis in the submandibular glands. We diagnosed the lesion as necrotizing keratoscleritis due to primary Sjogren's syndrome. A corneoscleral patch graft was performed, followed by immunosuppression including oral cyclosporin and topical prednisolone. During a follow-up period of 12 months, the corneoscleral graft was well maintained with no recurrence.

Ocular Reconstruction Using Autologous Tragal Perichondrium for a Refractory Necrotizing Scleral Perforation: A Case Report

PURPOSE: To report the effectiveness of an autologous tragal perichondrium graft for a necrotizing scleritis case which was refractory to conventional surgery. CASE SUMMARY: A 75-year-old woman was referred to our clinic with recurrent necrotizing scleritis of the left eye which occurred after pterygium removal five years earlier. The patient underwent scleral graft, pericardium graft, and amniotic membrane graft in other clinics; however, necrosis of the sclera progressed. The best corrected visual acuity was 0.06, and choroidal tissue was nearly exposed below the melted pericardium graft in the nasal area. The authors harvested tragal perichondrium from the right ear, and the scleral defect was successfully reconstructed with an autologous tragal perichondium graft. The graft showed rapid epithelization and neovascularization within a week and conjunctivalization after three months. No complications have been observed up to one year after surgery. CONCLUSIONS: Autologous tragal perichondrium graft is an effective treatment to alter necrotized sclera via neovascularization and rapid epithelization in refractory necrotizing scleritis cases.

Diagnóstico de granulomatosis de Wegener en pacientes con enfermedades oculares inflamatorias / Diagnosis of Wegener's granulomatosis in patients with ocular inflamatory disease

Objetivo. Informar las manifestaciones oftalmológicas en pacientes con granulomatosis de Wegener (GW). Método. Se revisó la base de datos del Instituto de Oftalmología Conde de Valenciana. Se recolectó la exploración oftalmológica completa, los exámenes de laboratorio y de gabinete, el tratamiento y la evolución. Resultados. Se incluyeron 11 pacientes con GW (18 ojos). Siete pacientes masculinos y 4 femeninos con edad promedio de 43.7 años (28-55). Tres habían tenido diagnóstico previo de GW y los 8 restantes se diagnosticaron en nuestro departamento. Siete presentaron cuadros bilaterales y 4 unilaterales. Las formas de presentación clínica fueron escleritis necrosante con queratitis ulcerativa periférica (QUP) (7/18), escleritis difusa (3/18), escleritis nodular (1/18), uveítis anterior no granulomatosa (1/18), neuropatía óptica isquémica (1/18), neuropatía óptica retrobulbar (1/18), desprendimiento de retina seroso (2/18) y dacriocistitis (2/18). De los 18 ojos, la capacidad visual final fue mejor o igual a 20/40 en 13, 20/400 en 3, cuenta dedos a 30cm o no-percepción de luz en 1. Actualmente 7 pacientes se encuentran en fase inactiva. Conclusiones. Las manifestaciones oftalmológicas más frecuentes en pacientes con GW fueron: escleritis necrosante y QUP. En la mayoría, la GW se diagnosticó después de las manifestaciones oftalmológicas, sin embargo, todos presentaron síntomas sistémicos u oftalmológicos previos.

OBJECTIVE: Report the ophthalmologic manifestations among patients with Wegener 's Granulomatosis (WG). METHOD: We reviewed the database of the Instituto de Oftalmologia Fundación Conde de Valenciana in order to collect information regarding complete ophthalmic examination, laboratory and cabinet tests, treatment, and disease progression. RESULTS: We included 11 patients with WG (18 eyes). Seven men and four women, mean age 43.7 years (range = 28-55). Three patients had a prior diagnosis of WG and the remaining eight patients were diagnosed by our study team. Seven subjects developed a bilateral affection and four had unilateral involvement. The clinical presentation was necrotizing scleritis with peripheral ulcerative keratitis (PUK) (7/18), diffuse scleritis (3/18), nodular scleritis (1/18), non-granulomatous uveitis (1/18), optic ischemic neuropathy (1/18), retrobulbar neuritis (1/18), serous retinal detachment (2/18), and dacryocystitis (2/18). Final visual acuity was better or equal to 20/40 (13/18), 20/400 (3/18), finger-counting or no-perception of light in 1/18. Currently, seven patients are symptom free. CONCLUSIONS: The most frequent ophthalmic manifestations among our patients with WG were: necrotizing scleritis and PUK. In most cases, WG was diagnosed after ophthalmic manifestations; however, all patients displayed prior systemic or ocular symptoms.

A Case of Nonspecific Orbital Inflammation in Anophthalmia

PURPOSE: We report a case of orbital pseudotumor developed in an anophthalmic socket, presenting no typical symptoms or signs. METHODS: A 67-year-old woman was referred for treatment of necrotizing scleritis of her left eye and painful orbital pseudotumor of her right eye. The right eye had been removed 10 years previously. There was an irregular mass in superomedial portion of anterior orbit. Orbital MRI showed poorly defined T1 iso and low T2 signal intensity of a lesion in the medial anterior portion of the right orbit. Because systemic steroid administration was limited, 2 mg of betamethasone was injected locally, after which the size of the lesion was decreased and the orbital pain improved. CONCLUSIONS: The diagnosis of an orbital pseudotumor developed in an anophthalmic socket may be difficult because of the lack of typical eyeball-related signs or symptoms such as proptosis, red eye, or decreased vision. But, the easy access to the lesion can provide early and effective treatment through direct injection of glucocorticoid.

The Effect of Amniotic Membrane Transplantation on the Treatment of Necrotizing Scleritis after Pterygium Excision

PURPOSE: To investigate the efficacy of amniotic membrane transplantation (AMT) in patients who were diagnosed with necrotizing scleritis after pterygium excision and who were refractory to systemic corticosteroid and immunosuppressive therapy. METHODS: Six patients who had persistent scleral melting with systemic corticosteroid or immunosuppressive agents underwent double AMT as a permanent patch graft and temporary overlying barrier. Postoperatively, the symptom improvements, reepithelization times and visual acuity changes were evaluated. RESULTS: Choroidal detachment was detected in two patients preoperatively. In all patients, ocular pain improved at 4.7 +/- 3.1 days, and reepithelization of the scleral lesions was completed at 13.2 +/- 8.1 days postoperatively. Choroidal detachment in two patients disappeared at postoperative 2 and 8 days. Complete remission was found in 5 of the 6 eyes (83.3%). One eye which achieved full epithelization in the necrotic area experienced recurrent nodular scleritis in another quadrant, resulting in remission after changing the regimen of immunosuppressive agents. Mean values of initial and final visual acuities were 0.898 logMAR and 0.428 logMAR, respectively, and the visual acuities improved in all patients. CONCLUSIONS: AMT is effective in promoting reepithelization of the necrotic area, resulting in complete remission of necrotizing scleritis in patients who were refractory to systemic corticosteroid or immunosuppressive therapy.

Increased Tear TNF-alpha and MMP-9 in Patients with Necrotizing Scleritis after Pterygium Excision

PURPOSE: We collected tear or scleral tissues of necrotizing scleritis after pterygium excision, and evaluated them for tumor necrosis factor (TNF)-alpha and matrix metalloproteinase (MMP)-9 to elucidate the molecular basis and seek for treatment of this disease. METHODS: Three patients with necrotizing scleritis after pterygium excision were evaluated for MMP-9 and TNF-alpha in tear and scleral tissue by Western blot analysis. RESULTS: Before treatment with corticosteroid, the patients' tear samples showed increased expression of TNF-alpha and MMP-9 compared to those of the contralateral eye. After treatment, the expression of TNF-alpha and MMP-9 was decreased compared to those of the pre-treated tear samples. The patients' sclera showed increased expression of MMP-9 compared to that of the donors' sclera and the patients' conjunctiva. CONCLUSIONS: Our results suggest that cytokine-related inflammation plays a role in the pathophysiology of necrotizing scleritis and strongly supports, under the guarantee of negative microbiological culture, the prompt use of corticosteroid and immunosuppressive agents to help suppress the progression of this disease.

Use of Immunosuppressant in the Treatment of Surgically Induced Necrotizing Scleritis (SINS) after Pterygium Excision

PURPOSE: To assess the proper differential diagnosis and treatment of scleritis with scleral melt following pterygium excision. METHODS: A retrospective study through a review of medical records of 5 patients diagnosed with surgically induced necrotizing scleritis (SINS) after pterygium excision and treated with systemic immunosuppressant and steroid. RESULTS: All the patients responded to the treatment and showed significant improvement. CONCLUSIONS: Immunosuppressive treatment without surgical intervention is effective in resolution of SINS after pterygium excision.

A Case of Enucleation due to Extensive necrotizing Scleritis after Pterygium Excision in a Rheumatoid Arthritis Patient

The scleritis is developed after taruma, infection or associated with systemic disease in 505 such as connective tissue disease, granulomatous disease & metabolic disease, in which the most common disease is rheumatoid arthritis[RA]. Scleromalacia perforans characterized by progressive thinning of the sclera in the absence of symtoms and with minimal inflammatory signs is a typical type of scleritis in rhematoid arthritis patient. And necrotizing scleritis is associated with long-standing and severe RA in 4-10%. However, there has been no effective method for preventing those diseases. In order to prevent recurrence of pterygium, anti-metabolic agent or radioactive material has been applied to excision site. However, in case of RA, excessive application of above method or aggressive excision of pterygeal mass is rather avoided to prevent postoperative necrotizing scleritis. In this study, we reported a case of enucleation with histology due to severe necrotizing scleritis with extensive exposure of choroid in a 67-year-old man having a past history of RA for 20 years and pterygium excision[OS] 15 years ago.

Two Cases of Necrotizing Scleritis After Pars Plana Vitrectomy

Necrotizing scleritis is an uncommon but most severe form in scleritis both in terms of pain and scleral destruction. It occasionally associated with systemic connective tissue diseases or idiopathic in orgin. Adequate diagnosis and therapy are essential to every ophthalmologist because it takes disastrous clinical course frequently. Histopathologically, localized granulomatous vasculitis was found and in pathogenesis, immunologic mechanism is suggested. We treated two cases of necrotizing scleritis which developed after pars plana vitrectomy, with topical or systemic corticosteroids and in one case, whose sclera was extensively melted, grafting with fascia lata was made with sucessful result. Two eyes became quiet after months of treatment without sequelae by scleritis itself We thought that this conditions occured by electrical low current diathermy which was given for hemostasis around sclerotomy incisions and by diabetic microcirculatory disturbances although not proven histologically.