Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis.

BACKGROUND: Behçet's disease is a multisystem vasculitis of unknown origin. Standard treatment mainly comprises systemic immunosuppressive agents. Ocular involvement, mostly posterior uveitis with retinal vasculitis, leads to blindness in 20-50% of the involved eyes within 5 years. The efficacy of interferon alfa-2a was studied in patients with sight threatening posterior uveitis or retinal vasculitis. METHODS: 50 patients were included in this open, non-randomised, uncontrolled prospective study. Recombinant human interferon alfa-2a (rhIFNalpha-2a) was applied at a dose of 6 million units subcutaneously daily. Dose reduction was performed according to a decision tree until discontinuation. Disease activity was evaluated every 2 weeks by the Behçet's disease activity scoring system and the uveitis scoring system. RESULTS: Response rate of the ocular manifestations was 92% (three non-responder, one incomplete response). Mean visual acuity rose significantly from 0.56 to 0.84 at week 24 (p<0.0001). Posterior uveitis score of the affected eyes fell by 46% every week (p<0.001). Remission of retinal inflammation was achieved by week 24. Mean Behçet's disease activity score fell from 5.8 to 3.3 at week 24 and further to 2.8 at week 52. After a mean observation period of 36.4 months (range 12-72), 20 patients (40%) are off treatment and disease free for 7-58 months (mean 29.5). In the other patients maintenance IFN dosage is three million units three times weekly. CONCLUSIONS: rhIFNalpha-2a is effective in ocular Behçet's disease, leading to significant improvement of vision and complete remission of ocular vasculitis in the majority of the patients.

[Intracorneal granulomatous inflammation]. / Intrakorneale granulomatöse Entzündung.

INTRODUCTION: We report a patient suffering from lattice corneal dystrophy and with a corneal granuloma 8 months after phototherapeutic keratectomy (PTK). CASE REPORT: PTK was performed in a 33-year-old man with hereditary lattice corneal dystrophy. An intracorneal tumor was found in the right eye 8 months after treatment. There was no indication of previous foreign body injury. Detailed clinical examinations yielded no evidence of the cause or nature of the tumor. For this reason an excision was performed. RESULTS: Histological examination revealed a dense inflammatory infiltration of the deeper corneal stroma with epithelioid cells, histiocytes, lymphocytes, eosinophilic leukocytes, and giant cells of the Touton type. There was no indication of a generalized granulomatous inflammation. CONCLUSION: A monosymptomatic, intracorneal, granulomatous infiltration is very rare and has not previously been described in a case of lattice corneal dystrophy. This may have been a case of xanthogranuloma or sarcoidosis. Strangely, the granuloma occurred only several months after PTK; nevertheless, a pathogenetic connection seems unlikely.

[An attempt to extract an intraretinal nematode located in the papillomacular bundle]. / Extraktionsversuch einer intraretinalen Nematode im papillomakulären Bündel.

BACKGROUND: The nematode infection of the retina is a rare disease with sight threatening prognosis. Untreated eyes usually become blind. There are different types of clinical progression: granuloma causing tractional detachment chronic inflammation called diffuse unilateral subacute neuroretinitis (DUSN) and rapid loss of vision named "ocular wipe-out syndrome". Efficient drugs are not available. The progression of the disease can be stopped by killing the nematode by means of photocoagulation. For parasites in the periphery of the fundus this is the therapy of choice. Photocoagulation of the nematode close to the posterior pole can cause severe damage to the visual functions. There is a need for a less detrimental treatment. PATIENT AND METHODS: We report on a 48-year-old woman with an intraretinal nematode inside the papillomacular bundle. At an early stage of the disease with normal visual acuity the eye was vitrectomized and a posterior vitreous detachment performed. It was intended to extract the worm out of the retina in toto. The retina over the parasite was incised parallel to the nerve fibers, a tiny hook inserted and the worm pulled out. RESULTS: Only one half of the parasite could be removed since the adhesion of the parasite to the retina was stronger than its own cohesion. Nevertheless the inflammation rapidly regressed. Finally a small area of pigment mottling persisted around the former location of the parasite corresponding to a relative scotoma in the visual field. Visual acuity remained unchanged at full vision. CONCLUSION: We demonstrated that nematode larvae can be removed successfully from the central retina using vitrectomy techniques. By that means the damage to the visual functions can be limited. Near the posterior pole surgical extraction of the worm may be favorable compared to photocoagulation.

Peripheral scotoma associated with chronic ethmoidal sinusitis. A case report and review of the literature.

Diseases of the paranasal sinuses may cause visual disturbances, especially diminished visual acuity and visual field defects, by affecting the optic nerve. We report the unusual case of a female patient with unilateral peripheral, quadrantic scotoma and concomitant chronic ethmoidal sinusitis. Visual acuity was not diminished. Despite extensive diagnostic examinations no other cause of the scotoma could be evaluated. As conservative therapy had been unsuccessful, endonasal pansinus operation was performed. Upon surgery, nearly all ethmoidal cells appeared to be filled with polypous mucosa. The sphenoid sinuses, however, contained air. In contrast, pre-operative CT scans had shown only a slight opacity of the ethmoid bone. Already two weeks after surgery a reduction in size of the scotoma could be noted. In addition to this case report, possible causes of visual field defects due to inflammatory diseases of the paranasal sinuses are discussed. In cases of unilateral visual field reduction associated with symptoms of chronic paranasal sinusitis, early operative exploration of the paranasal sinuses should be considered after exclusion of other possible causes, even if radiological findings do not warrant such a procedure.

Visual fields after removal of subretinal hemorrhages and neovascular membranes in age-related macular degeneration.

BACKGROUND: Surgical removal of subretinal hemorrhaging and membranes in eyes with age-related macular degeneration is feasible from a technical point of view, but no advantage over the spontaneous course of the disease has been proven. Visual acuity usually does not improve much. A realistic aim of surgical intervention may be diminution of the central scotoma size, which has not been studied. METHOD: In a prospective study visual fields were measured preoperatively and 6 weeks and 6 months postoperatively in eyes operated on for subretinal hemorrhages and membranes in exudative age-related macular degeneration. The OCTOPUS 1-2-3 program glx, which tests the visual field of 60 degrees diameter at 59 points, was used. Changes in the total size of areas with sensitivity loss of 10-20 dB (= relative scotoma) and > 20 dB (= absolute scotoma) were interpreted as surgical effect. Patients with postoperative retinal detachment or neovascular recurrence were excluded. 30 eyes of 29 patients were enrolled (mean age 75.3 years, 11 male, 18 female) and divided into 3 groups: 14 eyes with massive subretinal hemorrhage of diameters > 30 degrees; 12 eyes with hemorrhages of 10 degrees-30 degrees; 4 eyes with mere neovascular membranes < 10 degrees. RESULTS: (1) The visible area of damage is diminished by surgery (P < 0.01). The reduction for lesions > 30 degrees is 80%; for lesions between 10 degrees and 30 degrees the improvement is 43%. For lesions < 10 degrees there is no significant change. (2) The absolute scotoma size diminishes in all three groups (P = 0.05). The reductions are 63%, 57% and 21%, respectively. (3) Areas of absolute scotoma do not regain full function but are converted to relative scotoma depth, at best. Therefore, in the > 30 degrees group an overall increase (P < 0.01) of the relative scotoma size is observed. (4) The mean sensitivity in the 60 degrees field enhances by 1.6 dB (average over 30 eyes, P = 0.04). (5) Mean preoperative visual acuities were 0.03, 0.12 and 0.17, respectively, with no significant change after surgery. The power of testing is sufficient (0.93) to reject an increase of acuity from 0.12 to 0.2 in group 2. CONCLUSIONS: For hemorrhagic subretinal lesions of diameter > 10 degrees a relevant reduction of central scotoma size is achieved by surgery. The final benefit for the patients depends on the frequency of surgical complications and neovascular recurrence.

[Accidental macular injuries caused by the neodymium:YAG laser]. / Akzidentelle Maculaverletzungen durch Neodym:YAG-Laser.

BACKGROUND: The number of laser-users is steadily increasing. This implies an increasing risk of laser accidents with injuries especially of the posterior eye segment. PATIENTS AND METHODS: Two engineers accidentally suffered severe macular injuries caused by Neodym: YAG-lasers with 1064 and 1064/532/355 nm wavelength, respectively. In both cases very high energies were used. One patient looked directly at the laser beam, while in the other case the laser light was reflected from a detector. RESULTS: Depending on the emitted laser energy serious foveal destruction of retinal and chorioideal tissue, surrounding retinal edema and vitreous hemorrhage occurred, resulting in immediate reduction of visual acuity to 0.125 and 1/24 in 1 m, resp., and central scotoma. The most important damage mechanisms are plasma formation and expansion, emission of acoustic transients and cavitation with jet formation. Subsequently, macular scars evolved which were responsible for the limited increase of visual acuity up to 0.32, resp, 0.16 and remaining absolute central scotoma. CONCLUSION: Since there is no effective therapy, attention must be paid to preventive aspects. Improved information and awareness concerning the serious functional consequences of macular injuries is necessary, especially for non-medical users. Due to distinctive technical conditions (experimental arrangement with unprotected laser beam, comparatively high laser energy, prevailing parallel laser radiation), this circle of laser-users has a high risk of accident. A proper opportunity for an improved education are workshops for laser safety precautions.

[Calculated birth date as critical temporal marker for cryocoagulation of retinopathy of prematurity]. / Errechneter Geburtstermin als kritische Zeitmarke für die Kryokaogulation der Frühgeborenenretinopathie (ROP).

UNLABELLED: The time course of retinopathy of prematurity (ROP) is investigated for all preterm babies who underwent initial ocular therapy (cryocoagulation, encircling band or vitrectomy; 1983-1992; 156 eyes, 82 children; mean gestational age 28.1 weeks; mean birth weight: 864 g). Group I (ROP3+ in 5 adjacent or 8 accumulated clock hours): 63 eyes, 34 babies. The conceptional age at the time of cryo was 37.4 +/- 2.1 weeks. The disease progressed to cryo threshold before the presumed date of birth in 81% of cases. Group 2 (ROP4 and 5): 51 eyes, 27 babies. The gestational age at the time of first observation of ROP4 was 41.7 +/- 3.5 weeks. At least 39% of the detached cases arrived at stage 4 before the presumed date of birth. Group 3 (ROP3+ in less than 5 clock hours): Cryo-application in this grade of ROP is not generally accepted. We treated 42 eyes of 21 babies. Conceptional age at cryo therapy was 39.2 +/- 3.4 weeks. CONCLUSIONS: The time window for efficient ablative cryotherapy of ROP is clearly before the presumed date of birth. Eyes requiring therapy are at high risk for retinal detachment (ROP4) to be already present at 40 weeks conceptional age and later. Exceptions are ROP3+ cases of minor lateral extension.