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[Uvetitis and multiple sclerosis].

AIM: To evaluate the type of uveitis, the time of the duration between the first ocular signs and establishing of the diagnosis of multiple sclerosis (MS), effect of the treatment, and the final visual acuity (VA). METHODS: In a retrospective study, medical records of 41 patients (82 eyes) from the total of 1267 patients with uveitis treated at the Center for uveitis diagnosis and treatment at the Department of Ophthalmology of the Faculty Hospital and 1st Medical faculty, Charles University in Prague, Czech republic, during the period 1986-2003 were evaluated. The cohort consisted of 32 females and 9 males, mean age of 27 years (8-46 years). The mean follow-up period was 8 years (1-17 years). RESULTS: The cohort consisted of 41 patients (82 eyes), 32 females and 9 males. The mean age at the beginning of the uveitis was 27 years, at the time of the diagnosis of the MS 29.5 years. In 19 patients the starting of the uveitis preceded the manifestation of the systemic disease. In 23 patients, the uveitis was the first manifestation of MS. The most common type of uveitis in patients with MS was the intermediate uveitis (IMU) and vasculitis of retinal vessels. In 82% of patients, we were able during the follow-up period to stabilize the VA, or even to improve it. The systemic immunosuppressive treatment was necessary in 83 % of patients. SUMMARY: The MS was the second most common systemic disease in uveitis patients. It is necessary to follow-up young patients with unclear etiology of IMU or vasculitis, because the systemic disease may develop even few years after the first sings of uveitis. The collaboration between the neurologist and the ophthalmologist is of a great importance.

Adolescent↗

Ocular tuberculosis. A prospective study in a general hospital.

Ocular tuberculosis has traditionally been considered uncommon or anecdotal. Imprecise and variable diagnostic criteria have contributed to the confusion surrounding this topic. The increase in extrapulmonary manifestations of tuberculosis during the AIDS era established the need for a prospective study of ocular involvement in patients with all types of tuberculosis using well-defined criteria. During a 15-month period, 300 cases had culture-proven tuberculosis at our institution. We randomly selected 100 for systematic ophthalmologic evaluation. Our criteria for ocular tuberculosis were divided as follows: certainty (isolation of Mycobacterium tuberculosis from ocular specimens), probability (patients with isolation of M. tuberculosis from extraocular samples, with ocular lesions not attributable to other causes that respond to anti-tuberculous treatment), and possibility (same as probability but follow-up impossible). Ocular tuberculosis was present in 18 patients (18%) of which 10 patients fulfilled probability and 8 patients fulfilled possibility criteria. Eleven of 18 patients had HIV infection. In 11 patients, ocular involvement was asymptomatic. Almost all patients (17/18) had choroiditis, and other ocular lesions included papillitis, retinitis, vitritis, vasculitis, dacryoadenitis, and scleritis. Multivariate analysis showed as risk factors independently predicting ocular involvement in patients with ocular tuberculosis the presence of miliary disease (odd ratio 43.92, p = 0.002), ocular symptoms (odds ratio 6.35, p = 0.0143), and decreased visual acuity (odds ratio 0.04, p = 0.012). We observed an unexpectedly high (18%) incidence of ocular involvement, frequently asymptomatic, in patients with tuberculosis. Miliary disease is a clear predisposing factor in both HIV-infected and noninfected populations. Ocular examination should be routinely considered in patients with proven or suspected tuberculosis.

AIDS-Related Opportunistic Infections↗

Ocular manifestations of adult T-cell leukemia/lymphoma. A clinicopathologic study.

BACKGROUND: Adult T-cell leukemia/lymphoma (ATLL) is a recently described distinct clinicopathologic entity characterized by an extremely aggressive clinical course, a leukemic or lymphomatous proliferation of hyperlobulated peripheral T cells, and an association with infection by a retrovirus, human T-lymphotropic virus type I (HTLV-I). The authors present a patient with ATLL who initially had clinical features of severe uniocular intraocular inflammation, simulating acute retinal necrosis or herpetic retinitis, but results of histopathologic examination of the globe showed ATLL. PATIENT: A 38-year-old man had severe unilateral intraocular inflammation associated with widespread diffuse retinitis. Two weeks later, a focal retinitis with vasculitis developed nasal to the optic disc in the opposite eye. Shortly thereafter, mental disturbance developed, and the patient died less than 3 months after onset of the ocular symptoms. RESULTS: Results of postmortem examination showed atypical lymphocytic infiltration in the eyes, brain, and other systemic organs. Results of histopathologic and immunohistochemical studies showed that the atypical cells infiltrating the ocular tissues were ATLL cells. CONCLUSIONS: Patients infected with HTLV-I initially can have intraocular T-cell lymphoma that simulates acute retinal necrosis or herpetic retinitis.

Adult↗

Acute posterior multifocal placoid pigment epitheliopathy associated with a systemic necrotizing vasculitis.

PURPOSE: The authors present a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with a systemic necrotizing vasculitis with mixed features of Wegener granulomatosis and polyarteritis nodosa (PAN). METHODS: Case report. RESULTS: A 29-year-old woman developed a severe nosebleed, followed by a low-grade fever, night sweats, and a productive cough. One month later, she began experiencing high fevers, headache, myalgia, neck stiffness, and abdominal pain as well as bilateral blurred vision from APMPPE. Systemic evaluation revealed nasal ulcerations, bilateral pleural effusions, and a bilateral maxillary and ethmoid sinusitis, consistent with Wegener granulomatosis. However, ANCA testing was negative, and a renal and mesenteric angiogram showed aneurysmal dilatations suggestive of PAN. Her ocular and systemic symptoms markedly improved with systemic corticosteroids. CONCLUSIONS: The cause of APMPPE is unknown. This case of APMPPE associated with systemic necrotizing vasculitis provides support for the choroid as being primarily involved by a diffuse vasculitic process that interrupts choroidal perfusion and causes the characteristic fundus findings in APMPPE.

Adult↗

Cotton-wool spots as the initial ocular manifestation in Wegener's granulomatosis.

Various ocular manifestations have been described in Wegener's granulomatosis. Orbital involvement is common and manifestations in the anterior segment of the eye such as keratitis, scleritis, and conjunctivitis may occur. Involvement of the posterior segment presenting with major vascular occlusions or anterior ischemic neuropathy is rare. This case report presents a patient with bilateral cotton-wool spots at the posterior pole associated with histologically proven Wegener's granulomatosis. This finding, as an initial ocular manifestation of the disease, is very uncommon and may be interpreted as focal retinal inflammatory vasculitis. This was supported by the rapid positive response to systemic combined immunosuppressive and steroidal treatment during a 30-month period of review. The presence of cotton-wool spots in association with systemic signs of the disease should be considered in establishing the diagnosis of Wegener's granulomatosis.

Cyclophosphamide↗

[Panuveitis as a possible ophthalmic complication of Kikuchi-Fujimoto disease].

CLINICAL CASE: A 37 year old female with histologically proven Kikuchi-Fujimoto disease is presented. She developed panuveitis, vasculitis and subretinal macular infiltrate, probably as a recurrence of the disease. Immunosuppressive treatment (methotrexate) was initiated in the absence of response to systemic steroid therapy and threat to vision owing to macular involvement. DISCUSSION: Ophthalmic complications of Kikuchi-Fujimoto disease are unusual. We discuss differential diagnosis and emphasize the aggresiveness of our case.

Adult↗

The acute retinal necrosis syndrome. Possible herpes simplex retinitis.

The acute retinal necrosis (ARN) syndrome is a severe necrotic peripheral retinitis with vasculitis and vitritis. It attacks healthy people and carries a poor visual prognosis. Five patients with ARN were seen. Two showed interesting clinical features. One patient developed the ARN syndrome in both eyes but with a 14-year hiatus separating the two episodes, each being preceded by an infectious illness. A second patient had a possible recurrence of the ARN syndrome in the same eye, one year after the original episode. Each episode was preceded by an upper respiratory infection and ipsilateral vesicular skin lesions on the face. Biopsy of one lesion yielded herpes simplex virus (HSV) in culture. These associations, although not definitive, do lend support to the hypothesis that HSV may be the cause of the ARN syndrome.

Acute Disease↗

Eales' disease.

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Hemorrhage↗

Vitrectomy and trabeculectomy combined with interferon alpha treatment in Adamantiades-Behçet's disease: a case report.

BACKGROUND: Adamantiades-Behçet's disease is a multisystem disorder with recurrent oral and/or genital ulcerations, skin lesions, and ocular involvement. Eye involvement is a common manifestation that affects the patients' quality of life more than any other. Left untreated, it leads to blindness and often to loss of the eye through secondary complications such as phthisis or painful glaucoma. METHODS: Case report of a 24-year-old patient of Turkish descent living in Germany since his birth was admitted to our Department of Ophthalmology in 1999 because of visual loss in his right eye. He could only perceive light in that eye and evidenced total retinal detachment. In the left eye he had 20/20 vision but showed signs of retinal vascular inflammation and secondary glaucoma. RESULTS: Treatment with interferon alpha (9x10(6) I.U./3x week s.c.) and prednisolone (100 mg/d p.o.) led to complete regression of the acute inflammation within 2 weeks prior to operation. Vitrectomy was then successfully performed in the right eye under the therapy of interferon alpha (9x10(6) I.U./3x week s.c.) and 10 mg prednisolone p.o. The prednisolone therapy was stopped 1 week following operation. The failure of conservative glaucoma treatment necessitated trabeculectomy in the left eye. The patient has had no further recurrence for 4 years under monotherapy with interferon alpha (3x10(6) I.U./3x week s.c.). CONCLUSION: Interferon alpha is a potent therapy for Adamantiades-Behçet's disease with ocular involvement. It also provides a basis for safe and reliable surgical interventions. There was no intra- or perioperative recurrence of inflammation, which is a common finding in these procedures.

Adult↗

Functional outcome and prognostic factors in 304 eyes managed by retinectomy.

BACKGROUND: This study was undertaken to relate the anatomic and functional results of patients who underwent retinectomy for complex retinal detachment (RD) to preoperative prognostic variables. METHODS: Three hundred and four eyes of 302 patients whose surgery involved retinectomy were included in the analysis. All eyes had established proliferative vitreoretinopathy (PVR grade C). The main outcome measures were (1) postoperative visual acuity of 6/24 or better, (2) status of the retina at the end of follow-up, and (3) incidence of hypotony whilst under review. RESULTS: PVR was secondary to rhegmatogenous RD in 237 eyes (78%), posterior trauma in 51 eyes (16.8%), tractional RD in vasoproliferative vasculitides in 12 eyes (4%), acute retinal necrosis in 2 eyes and endophthalmitis in 2 eyes. Complete reattachment rate after one operation was 51%, with final complete reattachment success rate of 72%. The visual acuity improved in 138 eyes (45%), remained the same in 73 eyes (24%) and became worse in 89 cases (29%). Postoperative visual acuity of 6/24 or better was significantly associated with preoperative vision, the duration of silicone oil tamponade, silicone oil removal and retinectomy size. There was also some evidence of association between visual outcome and the number of clock hours of retinal detachment. Final retinal attachment was significantly associated with silicone oil removal and preoperative vision, and final hypotony was significantly associated with silicone oil removal. The incidence of sympathetic ophthalmia in our study was 0.09% (one case). CONCLUSIONS: Good functional outcome is possible following retinectomy surgery despite advanced pathology and often multiple surgical procedures. Retinal redetachment as a result of reproliferation and hypotony appear to be the main reasons for anatomical and functional failure. The clinical features we have identified as good indicators for improved final visual acuity such as shorter tamponade duration, removal of silicone oil, smaller retinectomy size, fewer previous operations and better preoperative vision are surrogate markers of less advanced PVR and should prompt retinal surgeons to consider retinectomy at an earlier stage in the process of PVR development. Clinicians should be aware of the small risk of sympathetic ophthalmia from complex retinal surgery.

Adolescent↗

Concurrent relapsing central nervous system and ocular involvement in a case of life-threatening Adamantiades-Behçet Disease (ABD).

Adamantiades-Behçet disease (ABD) is characterised by oral and genital ulcerations, skin lesions and ocular manifestations and, rarely, by central nervous system (CNS) involvement. Neuro-Behçet disease (NBD) is categorised to parenchymal or non-parenchymal, while combined CNS disease is rarely reported in the literature. A case of NBD, with severe relapsing ocular and neurological disease of combined pattern is presented. Neurological complications included brainstem manifestations, as well as neurovascular involvement, while ocular involvement consisted of bilateral uveitis and branch retinal vein occlusion. Manifestations responded to corticosteroid plus cyclophosphamide pulse therapy. Maintenance therapy included cyclosporine A, azathioprine and corticosteroids. Case individualities are discussed, focusing on scepticism concerning treatment of NBD relapses in the long term.

Acute Disease↗

Frosted branch angiitis secondary to macular choroidal neovascularization in a chinese woman.

BACKGROUND: This is perhaps the first report of frosted branch angiitis secondary to choroidal neovascularization (CNV) from China. We describe a case of frosted branch angiitis associated with macular CNV. CASE: A 19-year-old Chinese woman complained of metamorphopsia and blurred vision in the right eye on August 27, 2002. OBSERVATIONS: Fluorescein angiography demonstrated CNV in the macula. Five months later, focal and atrophic choroidal scars were observed in the macular area. She complained of blurred vision again on April 14, 2003. Iridocyclitis and frosted branch angiitis were found in both fundi. Following treatment with systemic acycloguanosine and corticosteroids for 4 months, the fundi recovered almost completely. CONCLUSIONS: A case of frosted branch angiitis secondary to macular choroidal neovascularization is reported in a Chinese woman. The relation between frosted branch angiitis and CNV remains to be further explored.

Adult↗