Periocular Corticosteroid Research Articles

Oculoleptomeningeal Amyloidosis in a Patient with a TTR Val30Gly Mutation in the Transthyretin Gene

To describe a patient with oculoleptomeningeal amyloidosis associated with infiltration of the vitreous, elevated intraocular pressures (IOPs), and seizures caused by a TTR Val30Gly mutation in the transthyretin gene. Interventional clinicopathologic report. A 44-year-old man with refractory bilateral intermediate uveitis, elevated IOPs, and seizures. The patient underwent diagnostic pars plana vitrectomy in both eyes. Intraocular and intracerebral pressures were controlled by trabeculectomy and cerebroventricular shunt placement, respectively. Visual acuity, histopathologic analysis of vitreous and leptomeningeal tissue, genetic testing, and magnetic resonance imaging of the brain. After failure of the patient to respond to topical and periocular corticosteroid therapy, a pars plana vitrectomy was performed and revealed amorphous material that showed green birefringence and dichroism on Congo red staining, establishing the diagnosis of vitreous amyloidosis. A full medical workup, including genetic testing, revealed a TTR Val30Gly mutation in the transthyretin gene. The patient subsequently developed elevated IOPs requiring bilateral trabeculectomy surgery and episodic seizures associated with leptomeningeal enhancement on magnetic resonance imaging. Histopathological analysis of a leptomeningeal biopsy taken at the time of surgery revealed amyloid infiltration, confirming the diagnosis of oculoleptomeningeal involvement by his amyloidosis. A ventriculoperitoneal shunt was placed. Amyloidosis should be considered in patients who present with vitritis that is unresponsive to corticosteroid therapy. Vitreous biopsy with histopathological analysis is recommended in these cases. Additionally, sequencing of the transthyretin gene should be considered in patients with vitreous amyloidosis to help establish known genetic syndromes and predict both ocular and systemic comorbidities. Although not described previously, elevated IOP may develop in patients with vitreous amyloidosis due to a TTR Val30Gly mutation in the transthyretin gene.

Periocular Triamcinolone and Photodynamic Therapy for Subfoveal Choroidal Neovascularization in Age-Related Macular Degeneration

To evaluate fluorescein angiographic and visual acuity (VA) outcomes from patients enrolled in a trial of a single periocular corticosteroid injection immediately before photodynamic therapy (PDT) versus PDT alone for subfoveal choroidal neovascularization secondary to age-related macular degeneration (AMD). Randomized 2-center clinical trial. Sixty-seven subjects with AMD, subfoveal choroidal neovascularization, and best-corrected VA of 20/20 to 20/320 in the study eye who had received no more than 1 prior PDT treatment. Subjects were randomized to receive PDT alone (no corticosteroid) or a single periocular corticosteroid injection given via the posterior superior sub-Tenon's capsule route before PDT (corticosteroid) and assessed 1, 3, and 6 months after enrollment. Best-corrected VA and intraocular pressure (IOP) measurements were taken during each examination. Color photographs and fluorescein angiograms were taken at baseline and 3 and 6 months. Presence or absence of fluorescein leakage from choroidal neovascularization 3 months after randomization. Between the 34 participants randomized to periocular corticosteroid and 33 to no corticosteroid, baseline features appeared balanced. Thirty-three corticosteroid participants and 30 no corticosteroid participants returned for the 3-month follow-up, at which time 56 had fluorescein leakage. Proportions of participants with leakage at 3 months for the 2 treatment groups did not statistically significantly differ; 94% of the corticosteroid group and 90% of the no corticosteroid group had fluorescein leakage at 3 months (P = 0.66). Mean VAs at 3 months after enrollment were 20/100 and 20/125 in the corticosteroid and no corticosteroid groups, respectively, decreasing on average 1.5 and 0.9 lines from baseline (P = 0.50). Adverse events included IOP > 21 mmHg in 7 corticosteroid participants (21%) and 1 (3%) no corticosteroid participant (P<0.05) and ptosis of the study eyelid in 1 (3%) corticosteroid participant. In contrast to previously reported uncontrolled studies and 1 controlled study, this trial did not find a reduction in the amount of fluorescein leakage 3 months after a single periocular injection of corticosteroid and PDT compared with PDT alone.

Risk Factors for the Development of Cataract Requiring Surgery in Uveitis Associated with Juvenile Idiopathic Arthritis

To identify the possible risk factors for the development of cataract requiring surgery in children with juvenile idiopathic arthritis (JIA)-associated uveitis. Retrospective cohort study. Data of 53 children with JIA-associated uveitis, of whom 27 had undergone cataract extraction (CE), were obtained. The main outcome measure, the interval between the onset of uveitis and the first CE (U-CE interval), was examined in relation to clinical and ophthalmologic characteristics and treatment strategies before CE. A shorter U-CE interval was found for children with posterior synechia vs those without posterior synechia (hazard ratio [HR], 3.57; 95% confidence interval [CI], 1.33 to 10.00). No significant difference was found for children in whom the uveitis was the first manifestation of JIA vs those in whom arthritis was the first manifestation of JIA (HR, 1.59; 95% CI, 0.63 to 4.00) and children treated with periocular corticosteroid injections vs those not treated with periocular corticosteroid injections (HR, 3.23; 95% CI, 0.95 to 11.11). Children treated with methotrexate (MTX) had a longer U-CE interval than children not treated with MTX (HR, 0.29; 95% CI, 0.10 to 0.87). The risk factor for development of early cataract requiring surgery in children with JIA-associated uveitis is the presence of posterior synechia at the time of diagnosis of uveitis. However, early treatment with MTX is associated with a mean delay in the development of cataract requiring surgery of 3.5 years.

Pharmacologic strategies in the prevention and treatment of corneal transplant rejection

Corneal transplantation remains one of the most successful organ transplantation procedures in humans. The unique structure of the cornea, with its absence of blood vessels and corneal lymphatic, allows the survival of corneal allograft. Recent advances in sutures, storage media, microsurgical instrumentation, and new pharmacological strategies have greatly improved the success of corneal transplantation and the prevention of corneal allograft rejection. Our strategies in the management and prevention of corneal graft rejection can modify and improve the survival of corneal allografts. Preoperative evaluation, understanding the risk factors, and management of ocular surface disorders may greatly improve the survival of the corneal transplant. Early recognition of corneal allograft rejection and aggressive treatment may improve the survival of the corneal graft. Furthermore, patients who undergo corneal transplantation should be maintained under close ophthalmic surveillance and patients should be informed to report immediately whenever symptoms of corneal graft rejection occur. The mainstay of therapy is topical corticosteroids. In severe cases, periocular, intravenous, and oral corticosteroids therapy can be rendered. New therapeutic modalities such as cyclosporine, tacrolimus, daclizumab, mycophenolate mofetil, leflunomide, rapamycin, and others may prove to be of help in the prevention and treatment of corneal graft rejection. Early recognition of corneal graft rejection and prompt treatment are mandatory for the successful survival of the corneal allograft.

Pars Planitis in Children: Epidemiologic, Clinical, and Therapeutic Characteristics

To report the demographics and clinical characteristics, therapy logarithm, and prognosis of children with pars planitis. The medical records were reviewed of all patients diagnosed with pars planitis between June 1995 and December 2005 in the Department of Pediatric Ophthalmology at Hospital Universitario La Paz, Madrid, Spain. A retrospective, descriptive, and longitudinal study of 30 eyes in 16 children was performed. Pars planitis was bilateral in 87.5% and more frequent in males (68.8%). Average age at onset was 9.2 years. The main ophthalmologic findings recorded were snowballs (96.7%) and vitritis (93.3%). Cataract formation was the most prevalent complication (36.7%). Mean initial and final best-corrected visual acuities were 0.640 and 0.840, respectively. Periocular corticosteroids were used in 33.3% of cases and cryotherapy or laser photocoagulation in 16.7%. Complications requiring surgical management occurred in 4 eyes (13.3%). Pars planitis treated with adequate medical and surgical procedures has a good prognosis in most cases.

Intravitreal steroids in the management of macular oedema

The use of intravitreal corticosteroids in the management of macular oedema has recently gained widespread acceptance. New long-acting steroid preparations and methods of delivery have facilitated the use of these new modalities. This review describes the various types of macular oedema for which this therapeutic option is used and the results.

Cogan Syndrome: A Retrospective Review of 60 Patients Throughout a Half Century

To evaluate the disease manifestations and clinical course of patients affected by Cogan syndrome (a syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms) at a single institution during roughly a half century. Medical records of all patients diagnosed as having Cogan syndrome at the Mayo Clinic in Rochester, Minn, and who were followed up from 1940 to 2002 were comprehensively reviewed. Otolaryngologic, ophthalmologic, and systemic manifestations of disease were analyzed. Analysis included patient demographics, presenting manifestations, delayed manifestations, laboratory testing, physical examination features, therapeutic interventions, disease course, and hearing and vision outcomes. Sixty patients were identified as having Cogan syndrome, with follow-up from 1940 to 2002. Most patients presented initially with vestibuloauditory symptoms, most commonly sudden hearing loss (50%). The most common inflammatory ophthalmologic condition noted was bilateral interstitial keratitis. Headache (40%), fever (27%), and arthralgia (35%) were the most frequently encountered systemic manifestations. Evidence of aortitis was found in 12% of patients. Complete hearing loss was eventually noted in 52% of affected patients, whereas permanent loss of any degree of vision was uncommon. Cochlear implantation outcomes were uniformly good. Death directly or indirectly attributed to the effects of Cogan syndrome was noted in 4 patients. The major disease-related morbidities were due to vestibuloauditory disease and only infrequently due to systemic manifestations such as vasculitis, with or without aortitis. Cochlear implantation has been of major benefit in modern hearing rehabilitation for this patient population. We advise caution before institution of protracted courses of high-dose corticosteroids and/or chemotherapy for patients without pronounced systemic disease or severe eye disease unmanageable by topical or periocular corticosteroids alone.

Outcome of Intravitreal Triamcinolone in Uveitis

To report the short-term outcome of intravitreal triamcinolone acetate (TA) in the treatment of uveitic cystoid macular edema (CME). Retrospective noncomparative (nonrandomized, uncontrolled) interventional case series. Sixty-five eyes of 54 patients with uveitis-related CME inadequately responsive to treatment combinations of oral corticosteroid, periocular orbital floor corticosteroid injections, and second-line immunosuppressive agents. Intravitreal injection of 4 mg/0.1 ml of TA. Visual acuity (VA), intraocular pressure (IOP), levels of inflammation, and immunosuppressive therapy were assessed. Other potential complications, including cataract progression, vitreous hemorrhage, endophthalmitis, and retinal detachment (RD), were looked for. The mean follow-up was 8.0 months (range, 3-51), and the mean improvement of VA after intravitreal TA was 0.26 (from 0.65 to 0.39 logarithm of the minimum angle of resolution; Snellen, 6/24-6/12, approximately). This occurred at a mean of 4 weeks (range, 1-30). The improvement in VA was more significant if the duration of CME before intravitreal TA was < or =12 months (P = 0.006) and if patients were < or =60 years old (P = 0.005). Patients with the worst vision before treatment also improved the least. The most important side effect was raised IOP (mean rise, 10.3 mmHg), with 28 eyes (43.1%) experiencing an IOP rise of >10 mmHg. Patients younger than 40 years were more likely to experience this IOP rise than those older than 40. Thirty-three eyes (51%) were treated with antiglaucoma medications, with a mean duration of treatment of 17.4+/-13.3 weeks, and no patient required trabeculectomy or lost vision. The dosage of oral corticosteroids and/or second-line immunosuppressive medication was reduced or stopped altogether in 18 of 33 eyes (54.5%) during the study period. There were no cases of injection-related vitreous hemorrhage, endophthalmitis, or RD. In patients with uveitic CME, intravitreal TA can effectively reduce CME and improve VA and, in some eyes, allows the cessation and/or reduction of immunosuppressive therapy. The period of effectivity varies in different patients and, in some eyes, is limited. Treatment was associated in 43.1% with a rise in IOP, which was transient and treatable medically.

Response to ‘Efficacy of periocular corticosteroid injections in the management of posterior uveitis’

Response to ‘Efficacy of periocular corticosteroid injections in the management of posterior uveitis’

Bilateral uveitis in a patient with autoimmune lymphoproliferative syndrome

We report a case of autoimmune lymphoproliferative syndrome (ALPS) presenting with bilateral uveitis. Observational case report. Review of case record, serum and aqueous IL-10 and IL-6 cytokine results, and immunosuppressive treatment of a patient with a mutation in the gene encoding Fas. Control of the intermediate uveitis required sustained doses of topical and periocular corticosteroids as well as systemic cyclosporine. The serum IL-10 level was elevated, as commonly seen in ALPS, but the aqueous IL-10 was not. Despite a Th2 immune predominance in ALPS, uveitis, a Th1-mediated disease, may still manifest in these patients. The pathogenesis of uveitis in ALPS may differ from that of the systemic disease overall. Long-term follow-up is required for patients with uveitis associated with ALPS.

Indocyanine Green Angiography Findings in Sympathetic Ophthalmia

A 78-year-old woman with a recurrent retinal detachment in the right eye presented 2 years after her last surgery with clinical and fluorescein angiographic evidence of sympathetic ophthalmia in the left eye. Fluorescein angiography showed discrete multifocal areas of central hypofluorescence with a hyperfluorescent ring. After immunosuppressive therapy, fluorescein angiography displayed multiple hypofluorescent spots without the hyperfluorescent collar. Prior to systemic and periocular corticosteroid therapy, indocyanine green angiography (ICGA) revealed multifocal hypo-fluorescent spots that became more prominent as the study progressed. The early stages of the posttreatment ICGA appeared normal, but the hypofluorescent spots reappeared in the late stage of ICGA. ICGA is a useful diagnostic adjunct to fluorescein angiography and clinical examination in helping to secure the diagnosis and monitor the treatment progress of sympathetic ophthalmia.

HLA-B27-Associated Uveitis With a Chorioretinitis Manifestation

A 29-year-old man who had been treated for acute anterior uveitis in a local medical office and observed for 1 month presented complaining of distorted vision in his left eye for 1 week. On ophthalmic examination, the anterior segment was relatively quiet with few cells. A posterior segment examination revealed cystoid macular edema and multiple splinter retinal hemorrhages. Results of all laboratory and imaging studies were negative, except for a positive HLA-B27 haplotype. Fluorescein angiography revealed massive leakage in the mid and late phase, consistent with chorioretinitis. Periocular corticosteroid injections and oral prednisolone were administered. The patient responded to the treatment well with subsequent resolution of chorioretinitis 2 months later. Although rare, chorioretinitis can occur in the setting of uveitis associated with HLA-B27 and seems to respond well to corticosteroid treatment.

Intermediate Uveitis

Intermediate uveitis is an intraocular inflammation involving the anterior vitreous, peripheral retina and pars plana. It usually affects patients from 5 to 30 years old, without gender or racial preferences. The etiology is unknown but there are several associated diseases: multiple sclerosis, idiopathic optic neuritis, autoimmune corneal endotheliopathy, sarcoidosis, thyroid diseases and inflammatory bowel diseases. Symptoms are blurry vision, floaters and distortion of central vision. The syndrome is bilateral in 80% of the patients and chronic with periods of exacerbation and remission. Clinical presentation includes: mild to moderate anterior chamber inflammation, thin keratic precipitates in the inferior portion of the cornea, autoimmune endotheliopathy, vitreitis, vasculitis in the peripheral retina, intravitreal “snowballs,” retinal “snowbanking,” optic neuritis and cystoid macular edema. Cataract and glaucoma are frequent complications. Treatment of intermediate uveitis is based on periocular and oral corticosteroids. Cryotherapy or laser photocoagulation of the peripheral retina are options in patients with snowbanking when there is an insufficient response to periocular or systemic corticosteroids. Imunosuppression may also be used when other therapies fail, and Cyclosporin A is the first drug of choice. Pars plana vitrectomy is indicated in patients with chronic significant inflammation, non-responsive cystoid macular edema, non-clearing vitreous hemorrhage, tractional retinal detachment and epiretinal membranes. The long-term prognosis of intermediate uveitis is usually good, particularly with strict control of inflammation and with proper management of complications. Patients can often maintain a vision of 20/50 or better.

Long-Term Follow-Up of Patients with Birdshot Retinochoroidopathy Treated with Systemic Immunosuppression

Purpose: Birdshot retinochoroidopathy (BRC) is a rare uveitis syndrome of presumed autoimmune etiology. Therapy with systemic and periocular corticosteroids is of inconsistent efficacy, attendant with numerous potential long-term side effects. Corticosteroid-sparing strategies with agents such as cyclosporine A or azathioprine have been suggested for this disease. Methods: We retrospectively reviewed the medical charts of patients with BRC who were evaluated consecutively at a tertiary-care, referral-based North American uveitis clinic over a 15-year period. Results: Eleven Caucasian patients (22 eyes) were diagnosed with BRC, representing approximately 1% of all cases seen at the uveitis clinic. HLA-A29 was positive in all 11 patients. We elected to treat five patients with azathioprine, methotrexate, cyclosporine A, mycophenolate mofetil, and/or IvIg, as well as systemic or periocular corticosteroid injections. The median period of follow-up for the five treated patients was six years (range: 8 months–13 years). Inflammation was reduced or stabilized in five of five patients. Conclusion: Although the definitive strategy for the management of BRC is unknown, control of intraocular inflammation and preservation of vision is possible with corticosteroid-sparing immunosuppressive agents.

CASE REPORT Sympathetic Ophthalmia after a Hyphema due to Nonpenetrating Trauma

Purpose: To report a case of sympathetic ophthalmia occurring after nonpenetrating ocular trauma. Methods: Interventional case report. Results: An 18-year-old white male presented with sympathetic ophthalmia four weeks after a hyphema from a nonpenetrating bungee cord injury. Vision was 20/20; there were keratic precipitates, anterior chamber cells, vitritis, and discrete yellow deep choroidal lesions consistent with Dalen-Fuch's nodules OU. The uveitis was treated with topical, oral, and periocular corticosteroids and the oral immunosuppressives cyclosporine and mycophenolate mofetil. Vision remained 20/20 OU for the first year of follow-up; vision OD then deteriorated to 20/50 over the next year with the formation of a posterior subcapsular cataract OD. Conclusion: Sympathetic ophthalmia may result from nonpenetrating ocular trauma. Ophthalmologists should be aware of this as prompt intervention is warranted.

Clinical trial to compare efficacy and side‐effects of injection of posterior sub‐Tenon triamcinolone versus orbital floor methylprednisolone in the management of posterior uveitis

To compare the efficacy and side-effects of posterior sub-Tenon injection of triamcinolone acetonide (Kenalog) with orbital floor injection of methylpredisolone acetate (Depomedrone) in the management of posterior uveitis. Non-randomized comparative prospective clinical study. Sixty-four eyes from 60 consecutive patients with non-infectious posterior uveitis requiring treatment were allocated on an alternate 1:1 basis to receive either orbital floor methylprednisolone or sub-Tenon triamcinolone using standard procedures and assessed at 6 and 12 weeks. After five eyes of five patients who had received the same treatment bilaterally were excluded from the statistical analysis, 14 out of 29 eyes treated with orbital floor methylprednisolone and 10 out of the 30 eyes given sub-Tenon triamcinolone improved at 6 weeks. There was no statistically significant difference in the improvement rate between the two groups. However, two patients given triamcinolone had prolonged upper lid ptosis, which required surgery, and another two developed markedly raised intraocular pressure, neither of which occurred in the methylprednisolone-treated group. Although the two drugs and routes compared were of similar efficacy, lid ptosis occurred in the triamcinolone-treated but not the methylprednisolone group. This should be borne in mind when choosing the preferred route of delivery of periocular corticosteroid in the treatment of posterior uveitis.

Macular edema as the only ocular finding of tuberculosis

To describe an infrequent presentation of ocular tuberculosis. Retrospective observational case report. A 61-year-old woman presented with decreased vision in her right eye and cystoid macular edema resistant to periocular corticosteroid treatment. There were no other findings. Diagnosis of probable underlying tuberculosis uveitis with macular edema as the only ocular manifestation was established based on a clinical history of possible tuberculosis during her infancy. Additionally, there were residual findings in the patient's chest x-ray, a positive Mantoux reaction, and Mycobacterium tuberculosis in sputum. Specific treatment was implemented for nine months. Cystoid macular edema resolved completely, visual acuity improved (20/20), and no remission occurred during two years of follow-up. In cases of idiopathic cystoid macular edema, tuberculosis should be considered as a potential cause. When treated, both are curable.

A review of evidence guiding the use of corticosteroids in the treatment of intraocular inflammation

Purpose:To review the published laboratory and clinical studies pertaining to the efficacy of corticosteroids in the treatment of intraocular inflammation. Methods:A Pubmed computer search was conducted of all publications focusing on the efficacy of corticosteroids in treating ocular inflammation, with an additional search of pertinent citations culled from these articles. Results:Experimental evidence has established the efficacy of corticosteroid eyedrops in treating intraocular inflammation. The effectiveness of periocular corticosteroid injections as well as of systemic corticosteroids is supported by extensive clinical experience, although placebo-controlled trials are lacking. Few studies have documented differences in efficacy between the common corticosteroid agents or between the different routes of administration. Subconjunctival injection appears to achieve higher intraocular corticosteroid levels than do other routes of periocular injection or systemic therapy. Clinical experience with intravitreous corticosteroid injection is insufficient to firmly attest to its efficacy or safety. Conclusion:The efficacy of corticosteroids in treating intraocular inflammation is based on limited experimental evidence and considerable clinical experience.

Sclerosing lipogranuloma of the orbit after periocular steroid injection

Purpose To report the clinical and histopathologic findings of a large sclerosing lipogranuloma of the orbit arising after a sub-Tenon’s corticosteroid injection. Design Interventional case report and review of the literature. Methods A complete ocular and systemic evaluation was performed on a 81-year-old patient, who developed a large orbital mass subsequent to a periocular corticosteroid injection producing proptosis, ptosis, and ocular motility impairment. The lesion was biopsied and submitted for histopathologic analysis. Main outcome measures Orbital, computed tomography, and histopathologic findings. Results Histopathologic examination revealed lipogranulomatous inflammation. Specifically, this type of reaction was consistent with a diagnosis of sclerosing lipogranuloma. Conclusions It is extremely rare to find a large granulomatous orbital lesion arising subsequent to a periocular corticosteroid injection. Only one case has been reported in the English-language literature to date. It is important to include this type of lesion in the differential diagnosis of an orbital mass seen after the injection of periocular corticosteroids.

Pars planitis in the Mexican Mestizo population: Ocular findings, treatment, and visual outcome

Purpose: To describe the clinical manifestations of classic pars planitis (CPP) in Mexican patients. We report here the most frequent complications, medical and surgical treatment, and visual prognosis. Material and methods: A retrospective, descriptive case series examined the clinical features, complications, and treatment (medical and surgical) of CPP patients seen at the Inflammatory Eye Disease Clinic from January 1990 to September 1999. Results: One hundred and sixty patients met inclusion criteria for the study. Mean age at presentation was 10 years and males were more frequently affected. Both eyes were affected in 84.4% of the cases. The most frequent complaint was decreased visual acuity. Initial visual acuity (VA) ranged from no light perception to 20/20 (mean 20/50), and mean final VA was 20/30. The most frequent clinical manifestations were vitritis (99.7%), snowballs (99.3%), retinal vasculitis (89.2%), and snowbanks (63.1%). The most common complications were cystoid macular edema (63.4%) and cataract (47.5%). Periocular corticosteroids were used in 97.5% of cases, systemic corticosteroids in 68.1%, and other immunosuppressive drugs in 21.3%. Conclusions: CPP in the Mexican population is more frequent in males and usually presents in patients less than 14 years of age. It is typically bilateral, and the most common symptom is decreased visual acuity. The most important clinical findings are located in the vitreous and retina. Cataract and cystoid macular edema are the most frequent complications. Treatment comprises periocular and systemic corticosteroids or other immunosuppressive drugs.