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The 1998 Wendell Hughes Lecture. Evisceration: is sympathetic ophthalmia a concern in the new millennium?

PURPOSE: To investigate a possible relationship between evisceration and sympathetic ophthalmia. METHODS: Data from Mt. Sinai Medical Center and University Hospitals of Cleveland were collected and histopathologic specimens were reviewed for 51 of 90 patients who underwent evisceration between 1980 and 1996 and who returned for follow-up examinations. Additionally, a survey was sent to members of the American Society of Ophthalmic Plastic and Reconstructive Surgery, the Uveitis Society, and the Eastern Ophthalmic Pathology Society to determine the number of enucleations and eviscerations performed and the documented incidence of sympathetic ophthalmia after evisceration. RESULTS: No clinical or histopathologic evidence of sympathetic ophthalmia after evisceration was found among patients treated at the two medical centers. The collective surveys showed a strong preference for enucleation over evisceration, but did not document evidence of sympathetic ophthalmia after evisceration. CONCLUSIONS: Evisceration is an effective and safe procedure with a low risk for sympathetic ophthalmia.

Adolescent↗

Sympathetic ophthalmia associated with pars plana vitrectomy without antecedent penetrating trauma.

PURPOSE: To evaluate, describe, and categorize the clinical presentation, clinical course, histopathology, and response to therapy in patients without a history of penetrating ocular trauma who developed sympathetic ophthalmia following pars plana vitrectomy. METHODS: The records of patients without a history of trauma who underwent pars plana vitrectomy and developed sympathetic ophthalmia were retrospectively reviewed. Cases were analyzed with respect to clinical presentation, fluorescein angiographic findings, anatomic and visual outcomes, histopathology, and response to therapy. RESULTS: Eight eyes were identified. The median age at presentation was 55 years, with a range of 14 to 62 years. The time from vitrectomy to diagnosis of sympathetic ophthalmia ranged from 2 months to greater than 2 years, with a median of 7 months. Six of eight patients (75%) presented with anterior chamber reaction. All eight patients presented with a vitreous inflammatory response. The optic nerve was inflamed clinically or angiographically in four of eight cases (50%). Small yellow-white sub-retinal pigment epithelial deposits were present in four of eight cases (50%). Two eyes had lesions characterized as multifocal choroiditis. One eye had larger yellow placoid-like lesions. One eye presented with vitritis but no retinal lesions. Subretinal choroidal neovascularization was noted in the inciting eye of one patient. Vision improved in the sympathizing eye with immunosuppressive therapy in five of eight cases (62.5%). CONCLUSIONS: Sympathetic ophthalmia can be seen following pars plana vitrectomy in patients without penetrating injuries or a history of trauma. Indeed, it may be seen after successful vitrectomy for retinal detachment. Diverse clinical presentations are possible, and persistent or atypical uveitis following vitrectomy should alert the surgeon to the development of sympathetic ophthalmia.

Adolescent↗

Ophthalmia neonatorum in northern Norway. I: Epidemiology and risk factors.

During one year, 1928 of all 1958 neonates born in the county of Troms in Northern Norway were followed until 6 weeks of age, regarding the development of ophthalmia neonatorum. Conjunctivitis was found in 364 cases (189 per 1000 neonates), including mild and self-limiting cases. In 14.5 cases per 1000 neonates the ophthalmia neonatorum was recorded as severe. Boys were more often affected than girls (P = 0.001). The age of the mother and obstetric events did not significantly influence the occurrence of conjunctivitis. 31.4% of the neonates received silver nitrate instillation. The frequency of conjunctivitis in the group with and without prophylaxis was 15.9 and 20.3%, respectively (P = 0.023). The incidence of chlamydial ophthalmia was 8 per 1000 neonates. At present, chlamydial ophthalmia is a common disease in neonates. Efforts should be made to screen fertile women for chlamydial genital infection and to encourage prompt microbiological examination in cases of ophthalmia neonatorum.

Adolescent↗

A comparison of silver nitrate with erythromycin for prophylaxis against ophthalmia neonatorum.

Although prophylactic eyedrops to treat ophthalmia neonatorum is mandated nationwide, states and hospitals are free to choose specific drugs. To compare two of these agents, we studied the incidence and characteristics of ophthalmia neonatorum in two UCLA teaching hospitals over a five-year period. One, which used 1% silver nitrate solution exclusively, had 50 cases in 34,772 births, a frequency of 0.14%. The other used 0.5% erythromycin ointment exclusively and had 43 cases in 12,652 births, a frequency of 0.34%. Ophthalmia neonatorum was more frequent in the hospital using erythromycin (p less than 0.001), as was chlamydial conjunctivitis (p less than 0.02). Although not statistically significant, gonococcal conjunctivitis was found in four infants, all in the hospital using silver nitrate. Because silver nitrate was found more effective in decreasing the total frequency of all cases of ophthalmia neonatorum, and cases caused by Chlamydia and gram-negative bacteria specifically, this drug still should be considered as a primary prophylactic agent against ophthalmia neonatorum.

Bacteria↗

[Sympathetic ophthalmia].

The authors give an account of a case of sympathetic ophthalmia which was successfully managed by reoperation of the injured eye, corticoids and immunosuppressive drugs. Sympathetic ophthalmia developed after perforation of the cornea along with traumatic cataract one month following the accident. It was manifested by irritation of the anterior segment of both eyes and marked exudation into the area of the posterior pole of the sympathizing eye. On the injured eye surgical reconstruction of the anterior segment was performed and concurrently broad spectrum antibiotics and corticoids were administered. On this therapy the anterior segment of both eyes began to improve, while the exudation into the area of the posterior pole of the sympathizing eye proceeded; therefore immunosuppressive therapy was started. The manifestations of sympathetic ophthalmia receded gradually. After an alternative way of corticoid reduction was started, a relapse of sympathetic ophthalmia occurred. The relapse was suppressed by a temporary increase of the dosage. Three months after the development of sympathetic ophthalmia, using the treatment described, the uveal inflammation of both eyes receded. At present, two years after the accident, both eyes are in a satisfactory condition with normal visual acuity--on the injured eye with an aphakic correction.

Adolescent↗

Relationship between sympathetic ophthalmia, phacoanaphylatic endophthalmitis, and Vogt-Koyanagi-Harada disease.

The more than coincidental occurrence of phacoanaphylatic endophthalmitis (PE) in sympathetic ophthalmia, and the similarity of the dissimilarity between Vogt-Koyanagi-Harada (VKH) disease and sympathetic ophthalmia have been well described both clinically and histopathologically. The etiology and pathogenesis of these three diseases are still not fully understood. Identifying and distinctive characteristics among them include the history of ocular trauma in sympathetic ophthalmia, rupture of the lens capsule in phacoanaphylatic endophthalmitis, and involvement of the skin, ear, and central nervous system in VKH disease. A T-cell-mediated immune reaction to ocular antigens seems to play a major role in sympathetic ophthalmia and VKH disease. A B-cell-related Arthus reaction to lens antigen seems to be the principle mechanism of PE. Thus, these three diseases may represent a spectrum of uveitis. At one end is the delayed-type hypersensitivity disease of sympathetic ophthalmia, whereas at the opposite end is the immune complex disease of PE, with VKH disease in the middle of this uveitic spectrum.

Adrenal Cortex Hormones↗

Ophthalmia nodosa caused by casual handling of a tarantula.

PURPOSE: To present the diagnostic and therapeutic challenges of ophthalmia nodosa secondary to tarantula hairs. METHODS: We present a case of a 28-year-old male with a one-week history of skin rash followed by ocular irritation, and blurring of vision after handling a Chilean rose tarantula. A Medline-guided literature search was performed to review the current and historical knowledge of ophthalmia nodosa. The clinical presentation and therapeutic options in ophthalmia nodosa were reviewed. RESULTS: Ophthalmia nodosa is a granulomatous, nodular reaction to vegetable or insect hairs including tarantula hairs. Tarantula hairs can cause inflammation in all levels of the eye, from conjunctiva to retina. Therapy includes surgical removal of offending hairs and medical management of inflammation. CONCLUSIONS: Although tarantulas are considered harmless pets, ophthalmia nodosa is a potential danger when handling a tarantula. Furthermore the management of exposure to tarantula hairs can be difficult.

Adult↗

32 cases of sympathetic ophthalmia. A retrospective study at the National Eye Institute, Bethesda, Md., from 1982 to 1992.

OBJECTIVE: To examine the relationship between visual outcome and the clinical management of patients with sympathetic ophthalmia. METHODS: Thirty-two patients with sympathetic ophthalmia who were seen at the National Eye Institute, Bethesda, Md, between 1982 and 1992, were retrospectively reviewed. RESULTS: There were equal numbers of males and females. Sympathetic ophthalmia occurred after trauma in 23 patients and surgery in nine patients. Sixteen of the 32 patients had a final visual acuity of 20/40 or better; 10 patients had a visual acuity worse than 20/200. Good visual outcome was associated with early and aggressive treatment with corticosteroids, sometimes in combination with other immunosuppressive agents. Poor visual acuity was associated with glaucoma, chorioretinal scars in the macula, and persistent uncontrolled inflammation. CONCLUSION: Prompt and aggressive use of antiinflammatory therapy can improve the visual outcome of patients with sympathetic ophthalmia.

Adolescent↗

Chlamydia ophthalmia neonatorum in Cameroon.

In the Ndoungué Hospital in Cameroon 449 new-born babies were examined for a month to check on the occurrence of ophthalmia neonatorum. Silver nitrate eyedrops 1% were applied at birth. The incidence of ophthalmia neonatorum was 19.4%; Chlamydia was found in 8 cases (incidence 1.8%) and gonococci in 4 cases (0.9%). Chemical conjunctivitis was suspected in 13 cases (2.9%). Slight conjunctivitis was seen in 68 cases and severe conjunctivitis in 19 cases (4.2%). A sexually transmitted agent was demonstrated in 6% of the slight cases and in 42% of the severe cases. Ophthalmia neonatorum due to Chlamydia was significantly more frequently severe and without specific features than those due to other agents. Although silver nitrate is still considered to be one of the most effective prophylactic measures for gonococcal ophthalmia neonatorum, in 4 cases this disease still developed. Silver nitrate is ineffective against Chlamydia and is also often the cause of a chemical conjunctivitis. The search is advocated for more efficient medicines in Africa which are effective against both Chlamydia and resistant gonoccoci.

Cameroon↗

[Further investigations for the demonstration of a specific microorganism in sympathetik ophthalmia].

19 Following the inoculation of tissue culture cells with material obtained from 3 human eyes suffering from sympathetic ophthalmia (2 sympathizing = sy-l, 1 sympathized = sy-2), no growth of bacteria was observed. On the other hand, changes occurred such as are typical for a massive infection with mycoplasmas. The morphology of these microbes corresponds, down to the details, with micro-organisms which the author has frequently demonstrated and illustrated since 1948 in human, chicken and monkey eyes suffering from sympathetic ophthalmia. 2. With the cultures of mycoplasmas obtained from the eyes of human patients suffering from sympathetic ophthalmia, it was possible to produce the same symptoms in chickens as were described by the author in 1950 in sympathizing and sympathized human eyes, namely: torpid uveitis and papillitis, which dragged on for months, and affected not only the inoculated right eye, but also, after 3 weeks and more, the untouched left eye. 3. Identical phenomena involving both eyes have been observed in chickens, the right eyes of which were inoculated with a strain of mycoplasma hominis orale 1 cultivated from other material in the Institut für medizinische Mikrobiologie der Universität Mainz (Prof. Bredt) and given to the author. A chronic intraocular inflammation, which persisted for several months--up to 6 months and more--developed not only in the inoculated right eye of the animals, but also, after an interval of several weeks, in the untouched left eye. 4. Viewed critically and with due care, these findings suggest that mycoplasmas might well play a role in triggering off sympathetic ophthalmia. 5. The results reported on here indicate that intraocular inoculation with mycoplasmas is of importance both in microbiology and in general medicine.

Animals↗

Intravitreal triamcinolone acetonide in sympathetic ophthalmia.

PURPOSE: To report the result of intravitreal triamcinolone acetonide in the treatment of sympathetic ophthalmia. METHODS: A 29-year-old woman who suffered from sympathetic ophthalmia and who was being treated with systemic corticosteroid therapy received an intravitreal injection of 4 mg of triamcinolone acetonide. RESULTS: By the 15th day after injection visual acuity had improved from 20/200 to 20/40 and serous retinal detachment had almost completely resorbed. Systemic corticosteroid therapy was reduced sequentially. By the third month after injection, the patient was in clinical remission. Her visual acuity was 20/20 and no serous detachment was observed. CONCLUSIONS: In this study, short-term improvement in the clinical picture of a patient with sympathetic ophthalmia after intravitreal triamcinolone acetonide injection was described. The results suggest that intravitreal triamcinolone acetonide injection may be an additional tool in the treatment of sympathetic ophthalmia.

Acute Disease↗

Sympathetic ophthalmia after surgical resection of iridociliary melanoma. A case report.

BACKGROUND: We report a case of sympathetic ophthalmia with systemic findings following resection of a malignant melanoma of the iris and ciliary body, and describe the treatment and clinical outcome. METHODS: A 49-year-old man underwent sector iridocyclectomy of a malignant iridociliary melanoma of the right eye. Five weeks later, he was diagnosed with sympathetic ophthalmia. Snellen's best-corrected visual acuity, fluorescein angiography, electroretinography, cerebrospinal fluid analysis and audiometry were performed. High-dose systemic steroid and immunosuppressive (cyclosporine and azathioprine) therapy was prescribed. Two months later chorioretinitis and macular edema persisted, and intravitreous triamcinolone was injected into the right eye. RESULTS: Five weeks after resection of an iridociliary melanoma, our patient had reported acute bilateral vision loss. Visual acuity was hand motion in both eyes. Examination showed bilateral granulomatous uveitis, diffuse choroiditis with Dalen-Fuchs nodules, papillitis and vitritis. On fluorescein angiography multiple hyperfluorescent dots, which coalesced in areas of exudative retinal detachment, were evident. The patient presented meningismus with pleocytosis on cerebrospinal fluid analysis, and sensorineural deafness. Sympathetic ophthalmia was diagnosed. High-dose intravenous steroids followed by oral prednisone at a tapering dose and immunosuppressive agents (cyclosporine and azathioprine), topical steroids in both eyes and intravitreal steroids in the right eye were administered. Phacoemulsification and intraocular lens implantation were performed to treat a dense cataract of the right eye. After 24 months of follow-up, best-corrected visual acuity was 20/200 in the right eye and 20/25 in the left; no signs of intraocular inflammation were observed and neurological signs had resolved. Low maintenance doses of systemic steroids and immunosuppressive agents were administered up to month 18 of follow-up to avoid recurrence. CONCLUSIONS: Sympathetic ophthalmia is a rare, but severe disease that can occur after resection of iridociliary melanoma. High-dose steroid therapy and supplementation with immunosuppressive agents early in the course of the disease was effective in resolving the condition.

Ciliary Body↗

Chlorambucil therapy in sympathetic ophthalmia.

PURPOSE: We used chlorambucil therapy in a 28-year-old man with sympathetic ophthalmia, which was incompletely controlled with systemic corticosteroids, and the patient developed serious side effects. METHODS: The patient sustained a penetrating injury with an intraocular metal foreign body. Attempts to remove it with a magnet failed. Vitrectomy with lensectomy successfully removed the intraocular foreign body. Five weeks after the injury and one week after the vitrectomy, sympathetic ophthalmia developed, with severe visual impairment and inflammation and serous retinal detachment in the fellow eye. Sympathetic ophthalmia was poorly responsive to topical cycloplegics, topical corticosteroids, and systemic corticosteroid therapy. The patient developed side effects to the corticosteroids, which were reduced to prednisolone 60 mg daily. Chlorambucil therapy was begun at 2 mg daily, increased by 2 mg per day each week to the maximum of 8 to 12 mg per day. The total dose of chlorambucil was 793 mg; the duration of therapy was 23 weeks. RESULTS: Successful treatment was achieved with chlorambucil therapy. There was clinical remission of inflammation and absorption of exudative retinal detachment. The neurosensory retina sealed down; the retinal pigment epithelium demonstrated severe destruction, with the characteristic sunset-glow and moth-eaten appearance. No malignancy and no serious side effect developed during one year of follow-up. After termination of therapy, the patient had sustained remission of ocular disease. CONCLUSION: Chlorambucil immunosuppressive therapy is an alternative to corticosteroids for the treatment of corticosteroid-resistant sympathetic ophthalmia; however, because chlorambucil has potentially serious late side effects, prolonged follow-up is necessary.

Adult↗

[Sympathetic ophthalmia 50 years after penetrating injury. A case report].

BACKGROUND: Sympathetic ophthalmia is a rare form of autoimmune uveitis and manifests in 90% of cases within the first year after penetrating injuries or surgical interventions. PATIENTS AND METHODS: In the present case the sympathetic ophthalmia started 50 years after a penetrating injury by a shell splinter. The injured eye was amaurotic and phthitic and the sympathizing eye showed an anterior uveitis. After an initial treatment with local and systemic corticosteroids the uveitis improved. The clinical diagnosis of sympathetic ophthalmia was made after a second inflammation course with substantial visual loss and subtotal chorioidal detachment. After enucleation of the exciting eye the diagnosis was confirmed by histological examination. An immunosuppressive therapy including azathioprine and cyclosporine became necessary to control the uveitis. RESULTS: After enucleation the corticosteroid treatment was not sufficient. Additional therapy with azathioprine resulted in a recovery of the symptoms but had to be stopped because of adverse reactions. The alternative therapy by means of cyclosporine was tolerated well, but dose reduction was difficult because of recurrences. After a 30 month lasting cyclosporine therapy the patient shows stabile results since 6 months with visual acuity of 20/30. CONCLUSIONS: The present case report demonstrates that a delayed onset of sympathetic ophthalmia 50 years after initial trauma may occur but can be controlled by an immediate, high dose immunotherapy.

Aged↗

[Tyndallometry in monitoring therapy of sympathetic ophthalmia].

BACKGROUND: The Laser Flare-Cell Meter allows noninvasive quantitative measurement of aqueous flare in human eyes. In this study we analysed changes of aqueous flare in eyes with sympathetic ophthalmia under immunosuppressive treatment. PATIENTS AND METHOD: Two men (54- and 59-year-old) with sympathetic ophthalmia (histological examination of the enucleated eyes confirmed the clinical diagnosis) were examined regularly with the Laser Flare-Cell Meter (Kowa). According to the flare values the dose of immunosuppressive treatment with cyclosporin A and prednisolone was gradually titrated. RESULTS: Under immunosuppressive treatment flare values of patient 1 normalized within 21 months. The dose of immunosuppressive treatment was carefully reduced according to flare values. Patient 2 showed very high flare values before treatment, which decreased to flare values of 20 flare counts/ms within 5 months of treatment with cyclosporin A and prednisolone. Both patients stopped immunosuppressive treatment because of systemic side-effects. Thereafter flare values increased acutely and a recurrence of sympathetic ophthalmia was observed in both patients. Under renewed immunosuppressive treatment aqueous flare values decreased again. CONCLUSION: Measurement of aqueous flare with the LFCM appears to be an effective tool for follow-up examinations, and especially for titrating of immunosuppressive treatment in eyes with manifest sympathetic ophthalmia.

Cyclosporine↗

Peripapillary choroidal atrophy in sympathetic ophthalmia and management with triple-agent immunosuppression.

PURPOSE: To describe the occurrence of peripapillary choroidal atrophy and experience with triple-agent immunosuppression in three cases of sympathetic ophthalmia. MATERIALS AND METHODS: Retrospective chart review of three cases of sympathetic ophthalmia with peripapillary choroidal atrophy. RESULTS: Three patients with sympathetic ophthalmia were managed with steroid pulse therapy and triple-agent immunosuppression. All three patients subsequently developed peripapillary choroidal atrophy. CONCLUSIONS: Peripapillary choroidal atrophy can occur in sympathetic ophthalmia and may indicate a severe form of inflammation which would benefit from triple-agent immunosuppression.

Adult↗

Sympathetic ophthalmia.

Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis that occurs after either surgical or accidental trauma to one eye. The ocular inflammation in the fellow eye becomes apparent usually within 3 months after injury. Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic precipitates. The posterior segment manifests moderate to severe vitritis, usually accompanied by multiple yellowish-white choroidal lesions. Evidence suggests that sympathetic ophthalmia represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells. Diagnosis is based on clinical findings and a history of previous ocular trauma or surgery. Other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis should be considered. Treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents with high dose oral corticosteroid as the drug of choice. However, if the inflammation cannot be controlled, cyclosporine is then used. Other immunosuppressive agents, such as chlorambucil, cyclophosphamide or azathioprine, may be necessary for the control of inflammation. The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt wound repair and appropriate immunomodulatory therapy.

Diagnosis, Differential↗

Ophthalmia neonatorum in a trachoma endemic area.

BACKGROUND AND OBJECTIVES: Chlamydia trachomatis can be directly transmitted by sexual or perinatal contact and indirectly transmitted by flies or fomites. Whether distinct epidemiologic forces among human populations or biologic characteristics of the organism are responsible for the different routes of transmission is uncertain. STUDY DESIGN: To determine if ophthalmia neonatorum and trachoma are linked epidemiologically, 38 infants with ophthalmia and 277 children with trachoma were studied for evidence of C. trachomatis infection using culture, antigen and DNA detection tests. The study was performed in a trachoma endemic area of central Kenya. RESULTS: Of infants with ophthalmia neonatorum, 8% to 9% had microbiologic evidence of ocular C. trachomatis infection. Of the children with trachoma, 31% had evidence of chlamydial infection. Ninety-two percent of the 59 identified strains causing trachoma belonged to the classic trachoma serovars (A, B, Ba and C). Neither of the two chlamydial strains recovered from infants with ophthalmia was a trachoma serovar. Mothers rarely (3%) had cervical C. trachomatis infection. CONCLUSION: This study does not support a major role for perinatally transmitted C. trachomatis infection in trachoma epidemiology.

Adolescent↗