Panuveitis of undetermined origin after diagnostic pars plana vitrectomy: clinical characterization and long-term outcome from a tertiary referral center.

PURPOSE: To identify the characteristics and outcome of panuveitis of undetermined origin (PUO) after diagnostic vitrectomy. METHODS: Retrospective analysis of all patients who underwent vitrectomy for diagnostic/therapeutic purposes from 2013 to 2020, whose vitreous biopsies turned out negative and final diagnoses were not clinically supported. RESULTS: Of 122 operated eyes, 36 eyes (29.5%) were defined as PUO (67.8 ± 14.9 years). The presenting clinical picture revealed a predominantly bilateral condition (70% of eyes) with significant posterior segment involvement: 3.1 ± 0.6 vitritis, 61.1% of eyes with retinal vasculitis, 44.4% with macular edema, and 30.6% with exudative retinal detachment. Presenting visual acuity was 1.2 ± 0.7 logMAR, and up to 90% remained stable or improved vision over a ~ 3.5 year observation period. None of the presenting clinical features turned out to be predictive of final visual outcome or survival. CONCLUSIONS: PUO is present in up to 30% of cases after diagnostic/therapeutic vitrectomy. This mainly bilateral condition shows chronic and overall stable long-term outcome, generally with retained steady visual function.

Vision-threatening bilateral panuveitis and TRAPS in a child: an uncommon association.

PURPOSE: To report a childhood case of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) carrying the R92Q variant with a vision-threatening bilateral panuveitis. METHODS: Case report and review of the literature. RESULTS: A 7-year-old boy presented with an active bilateral panuveitis and a macular rash associated with fever. Fundus examination showed two choroidal lesions on the posterior pole of the right eye, and fluorescein angiography revealed early hypofluorescence and late hyperfluorescence of the lesions, which were hyper-autofluorescent. Extensive clinical laboratory analyses ruled out autoimmune diseases and systemic infection. The only remarkable finding was a positive IgG for herpes simplex 1. He underwent two successive diagnostic pars plana vitrectomies as well as cataract and glaucoma surgeries. Genetic analysis revealed a mutation in the TNFRSF1A gene, and the patient was diagnosed with TRAPS-associated bilateral panuveitis. He was treated with adalimumab and has been free of active inflammation since then. CONCLUSIONS: We present here the first case reported of panuveitis in a patient with TRAPS. This finding stresses the increasing importance of genetic analysis in search of autoinflammatory diseases to establish an adequate diagnosis and treatment in cases of uveitis of unknown etiology.

A hairy affair: tarantula setae-induced panuveitis requiring pars plana vitrectomy.

Cases of ocular inflammation following penetration by urticating hairs from caterpillars and tarantulas have been previously reported and although rare, the condition is increasingly being recognised as a cause of chronic panuveitis. The long-term outcomes and prognosis of this condition are not well known. This article describes a case of chronic panuveitis as a result of ocular penetration by tarantula setae, and its challenging management that ultimately required pars plana vitrectomy. Interventional case report: A 29-year-old male presented with chronic panuveitis secondary to tarantula-hair penetration. Initial management with mechanical removal of hairs from the cornea and intensive topical steroid therapy for 18 months did not adequately control his symptoms. Pars plana vitrectomy was carried out and at 6-month follow-up the patient was symptom-free without any pharmacological therapy. We propose early pars plana vitrectomy as a management option for treatment-resistant panuveitis in cases of ophthalmia nodosa secondary to setae-related injury.

Clinicopathological and immunohistochemistry correlation in a case of Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada (VKH) disease is a systemic disorder causing bilateral panuveitis. Histopathological documentation along with molecular diagnostic evidence in VKH eye is a rarity. We present a 46-year-old woman with VKH with several ocular complications and subsequently enucleation of the right eye was done because of painful blind eye. Patient had clinical complications of VKH and some of the complications were observed in histopathology. Pathology of the case showed nongranulomatous uveitis, indicating the disease in chronic recurrent stage. Immunohistochemistry showed predominant T-cell involvement in this case. The case showed clinicopathological and immunohistochemistry correlation in a case of VKH disease.

Vitrectomy for persistent panuveitis in Behçet's disease.

PURPOSE: To determine whether vitrectomy is safe and effective for the management of vitreoretinal complications in Behçet's patients with persistent panuveitis. METHODS: Patients who had undergone therapeutic vitrectomy for Behçet's panuveitis were reviewed retrospectively. Those patients who had shown persistent uveitis despite maximum medical therapy were included. Surgical outcomes were measured by an improvement in vision of 0.3 logarithm of minimum angle of resolution, acute relapse rates, and postoperative complications. RESULTS: Twenty-one patients with a mean postoperative follow-up of 30 months were identified. For almost all patients visual acuity and inflammatory control were significantly improved after vitrectomy. However, patients with optic disc neovascularization (NVD) showed no improvement in their vision or inflammatory control. The postoperative rise of intraocular pressure was frequently associated with intravitreal triamcinolone acetonide injections. CONCLUSIONS: Our study suggests that vitrectomy may be both safe and effective for the management of vitreoretinal complications in Behçet's patients with persistent uveitis. Patients with NVD, however, may have poor outcomes.

Episodic acute hypotonia after Nd:YAG laser capsulotomy--retinal function and choroidal swelling.

In a patient with uveitis who had been treated with Nd:YAG laser capsulotomy after cataract surgery, several episodes of acute hypotonia occurred which were associated with changes in clinical tests of the eye and of visual function. Immunosuppressive and immunomodulating treatment appeared to reverse the changes in intraocular pressure and normalise the test results. The significance of these observations is discussed.

[Cataract extraction and intraocular lens implantation in patients with uveitis].

Thirty two patients with uveitis had a posterior chamber intraocular lens implantation after extracapsular cataract extraction. After a mean follow-up period of 5.4 months (range 3 to 29 months), the corrected visual acuity in 93.7% of the patients reached to 0.5 or better. Postoperative complications included posterior capsule opacification and iris posterior synechiae. The results suggest that careful selection of candidates for IOL implantation be crucial for successful cataract surgery in uveitis patients.

[Panuveitis associated with papillary carcinoma of the thyroid]. / Panuvéite bilatérale associée à un carcinome papillaire de la thyroïde.

Ocular involvement secondary to thyroid carcinomas is uncommon. Uveal metastasis may occur. More rarely, they can be responsible for paraneoplastic syndromes. We report the case of a 64-year-old woman who presented with a severe bilateral panuveitis with venous vasculitis associated with hyperthyroidism from a multinodular goiter, complicated by papillary carcinoma. Systemic steroid therapy was initiated; ocular symptoms resolved completely after total thyroidectomy. Other causes of panuveitis with venous vasculitis were ruled out. This is the first reported case of panuveitis associated with papillary thyroid carcinoma. The occurrence of the ocular symptoms with hyperthyroidism and their remission after surgery supports the possibility that this association may not be coincidental. A paraneoplastic phenomenon is suspected.

Pars plana vitrectomy in the treatment of severe complicated toxoplasmic retinochoroiditis.

PURPOSE: To present the anatomic and functional results of pars plana vitrectomy performed in severe complicated toxoplasmic retinochoroiditis. METHODS: Three patients, 2 women and 1 man aged 57, 22, and 57 years, are presented. The first patient was under immunosuppressive therapy for dermatomyositis and underwent diagnostic/therapeutic vitrectomy for severe toxoplasmic panuveitis with dense vitritis. The other 2 patients underwent vitrectomy for macula-off rhegmatogenous retinal detachment that developed after severe toxoplasmic panuveitis. RESULT: Preoperative visual acuity was hand movement for the first 2 patients and 20/400 for the third. All patients received pars plana vitrectomy with epiretinal membrane peeling, laser photocoagulation, and SF6 gas tamponade. The second and third patients needed 5 and 3 additional operations, respectively, including extensive retinotomies and silicone-oil tamponade, for recurrent retinal detachment due to proliferative vitreoretinopathy. At the end of the follow-up period (11, 5, and 1 year, respectively), the retina was attached and visual acuity was 20/30 for the first patient but counting fingers for the other 2 patients. CONCLUSIONS: Severe panuveitis and/or recurrent retinal detachment may develop in some cases of ocular toxoplasmosis, compromising the visual prognosis. Retinal detachment due to toxoplasmosis is generally complex, and long-acting tamponade with silicone oil should be contemplated for anatomic retinal reattachment.

Visual outcome of vitrectomy in seasonal hyperacute pan uveitis.

INTRODUCTION: Seasonal Hyper Acute Panuveitis (SHAPU) is a sight threatening condition often affecting children. Its management as yet is a challenge in ophthalmic practice. Most of the eyes even after treatment end up in pthisis bulbi. MATERIALS AND METHODS: A retrospective hospital based analysis of 18 patients with SHAPU managed with vitrectomy at Himalaya Eye Hospital (HEH) Pokhara over a period of two years was carried out. RESULTS: Out of 18 patients 10 were males. Majority of them were below the age of 15 years. Best corrected visual acuity (BCVA) at presentation was less then 3/60 in 72% of them. BCVA was better then 6/60 in 7 (50%) out of 14 patients, who underwent vitrectomy. CONCLUSION: Vitrectomy is a useful procedure in management of SHAPU.

Vitrectomy in multifocal chorioretinitis.

In nine patients suffering from multifocal chorioretinitis with panuveitis, we obtained no impressive therapeutic benefit from vitrectomy. Seven women and two men ranging in age from 63 to 86 years underwent pars plana vitrectomy at 3-28 months after the first signs of uveitis had appeared. Previous medical treatment had always been unsuccessful. Vitrectomy was performed in one eye of eight patients and in both eyes of one patient. In addition, in two patients a cataract was removed by phacoemulsification and an intraocular lens was implanted. A visual improvement of one or two lines was achieved postoperatively in most cases, but the visual acuity decreased to preoperative values or less within 6 months. The surgical treatment showed no obvious effect on the intensity or frequency of uveitis relapse. In all cases, vitrectomy specimens were serologically negative for herpes-group viruses. Histologically, no malignancy was detectable. Immunohistochemistry studies revealed that a large number of the vitreous cells bore T-lymphocyte markers (mostly CD2-, CD3-, and CD4-positive); about one-third of the vitreous cells were macrophages, and B-lymphocytes were only rarely detected. In conclusion, pars plana vitrectomy in multifocal chorioretinitis achieved no positive long-term result. There was no diagnostic hint as to whether the cause of the uveitis might have been of a viral, malignant, or autoimmune origin. Therefore, multifocal chorioretinitis remains poorly understood in terms of its etiology and adequate treatment.

[Pars plana vitrectomy in uveitis]. / Vitrectomie par la pars plana au cours des uveites.

A vitrectomy was performed in 30 eyes suffering from different forms of uveitis. An improvement of visual acuity was obtained in 29 cases and was explained by removal of vitreous haze and/or of the cataract, but not by the improvement of the inflammatory process.

[Uveitis in ancylostomiasis]. / Uveitis bei Ankylostomiasis.

A 42-year-old patient from Sri Lanka presented with unilateral panuveitis. The only possible cause detected was ancylostoma duodenale, a parasitosis occurring frequently on the Indian subcontinent. The link between this and uveitis, and the therapeutic consequences, are discussed.

[Zinsser-Engman-Cole syndrome (dyskeratosis congenita) with severe sicca syndrome, panuveitis and corneal perforation--a case report]. / Zinsser-Engman-Cole-Syndrom (Dyskeratosis congenita) mit schwerem Sicca-Syndrom, Panuveitis und Hornhautperforation--eine Kasuistik.

BACKGROUND: Zinsser-Engman-Cole syndrome (Z.E.C.) is a very rare type of ectodermal dysplasia, inherited in X-linked recessive manner and characterised by poikiloderma, nail dystrophy, lingual leucoplakia, bone marrow hypoplasia, hyperkeratosis and hyperhidrosis of planta and palms, dental anomalies and caries, premature grey hair. PATIENT AND METHODS: We report on a 46-year-old man who presented with occlusion of lacrimal puncta, trichiasis, severe dry eye, recurrent corneal ulceration and perforation, uveitis. HLA typing, flow cytometry of peripheral lymphocytes, bone marrow biopsy, conjunctival biopsy and extensive laboratory evaluation towards autoimmune and infectious diseases were performed. RESULTS: CD4+ T cells fraction was decreased, CD8+ and CD3+ HLA DR+ elevated. The patient was HLA-B27 positive. Laboratory studies revealed increased erythrocyte sedimentation rate and C-reactive protein level, hypochromic and hypoplastic anaemia, negative serum titers of antibodies to Epstein-Barr virus, HIV, HTLV-I, toxoplasma gondii and treponema pallidum, repeated titers to cytomegalovirus, herpes simplex and herpes zoster viruses--IgM negative, IgG positive. Corneal perforation was treated with amniotic membrane transplantation and corneal transplantation. CONCLUSION: The defect in cell-mediated immune mechanisms in Z.E.C. syndrome explains the corneal perforation, sicca syndrome and uveitis, first reported in this syndrome.