“Childhood uveitis through the eyes of a rheumatologist”
Dr. Thomas J. A. Lehman, Invited Speaker
Chief, Division of Pediatric Rheumatology, Hospital for Special Surgery, and
Professor of Pediatrics, Sanford Weill Cornell Medical Center, Cornell University
Dr. Kesen: Uveitis in Children with Behcet’s Disease
Dr. Garza-Leon: Toxoplasmosis in Pediatric Patients
Dr. Li Cheng: Dengue-Associated Maculopathy in Children
Dr. Orozco-Carroll: Vogt Koyanagi Harada in pediatric patients
Dr. Tsai: Outcome Assessment of VKH Disease treated with oral corticosteroids
Dr. Thorne: Risk Factors & Causes of Visual Acuity Loss in Patients with JIA-uveitis
Dr. Cervantes: Pediatric Cataract Surgery Outcomes in JIA-uveitis
Dr. Anzaar: The Use of Mycophenolate Mofetil in Recalcitrant JIA-uveitis
Dr. Quinones: Biologic Patients in the Treatment of Refractory Pediatric Uveitis
Dr. Lew: Regression of Retinal Neovascularization in a Patient with severe SLE retinal vasculitis using intraocular Avastin
Muge R. Kesen, MD, Debra A. Goldstein, MD, Howard H. Tessler, MD
University of Illinois at Chicago, Department of Ophthalmology and Visual Sciences
Uveitis Service, Chicago, IL
OBJECTIVE
To describe demographics, ocular manifestations, clinical course, complications, treatment and visual outcomes of uveitis in children with Behcet’s disease.
DESIGN
Retrospective case series.
METHODS
From 1975 to 2006, 100 cases of Behcet’s disease were diagnosed at the University of Illinois, Chicago. 10 (10%) of these cases were in children aged 18 or younger. 3 illustrative cases (6 eyes) are reviewed. All patients met the classification criteria of the International Study Group for Behcet’s Disease. Data on the patients’ gender, race, age at onset of uveitis, initial symptoms, ocular manifestations, visual acuity, follow-up period, complications and treatment were collected.
RESULTS
All 3 patients were male. One was Hispanic, one Caucasian and one East Indian. The clinical picture included oral ulcers in all cases. The mean age of uveitis onset was 13.3 years (range 10-15 years of age). Aphthous stomatitis was the initial symptom in 2 cases. All patients had bilateral ocular involvement. Panuveitis with retinal vasculitis was noted upon presentation in 2 patients. All cases received immunosuppressive therapy, including prednisone, chlorambucil, methotrexate, cyclophosphamide, cyclosporine, infliximab. Response to treatment was variable. Follow-up period ranged from 3 to 17 years (mean 8.33 years). Upon presentation 2 out of 6 eyes (33 %) had a visual acuity of 20/200 or worse. Over the follow-up period those remained stabile at 20/200 or worse. 4 out of 6 eyes (67%) had a visual acuity of 20/50 or worse, and only 2 of these eyes (50%) were noted to improve, 1 of which improved better than doubling of visual angle using logMAR units. 1 out of these 4 eyes (25%) remained stable and 1 eye (25%) worsened by 0.1 logMAR unit. 2 out of 6 eyes (33%) had a visual acuity better than 20/50 and remained stable at that.
CONCLUSION
Behcet’s disease has a fairly poor prognosis in children. Retinal vasculitis was seen bilaterally in 2 patients. Despite systemic therapy with various combinations of prednisone, chlorambucil, methotrexate, cyclophosphamide, cyclosporine, infliximab, only 3 out of 6 eyes maintained visual acuity of 20/50 or better.
Garza-León MA, Arellanes-García L.
Purpose: To characterize the clinical features of ocular Toxoplasmosis in pediatric patients.
Design: Retrospective noncomparative case series.
Participants: Sixty nine pediatric patients with diagnosis of toxoplasmosis admitted between January 1990 - March 2006.
Methods: The clinical files of children with ocular toxoplasmosis attending the Inflammatory Eye Disease Clinic of the Asociación para Evitar la Ceguera en México were reviewed. Fundus fluorescein angiography (FFA) and serologic tests for Toxoplasma gondii, were performed in certain cases.
Results: Sixty nine patients (99 eyes) were included. Thirty eight were female. Unilateral involvement was noticed 56.5%. A history of coexistence with puppies was recorded in 63.6% of patients, only 4% had diagnosis of systemic toxoplasmosis. Most common symptom was decreased vision and strabismus (28.2% each one). Most common clinical presentation was an inactive retinal scar in 73.7% of eyes, followed by panuveitis in 15.2% and posterior uveitis in 10.1%, one eye presented with neuroretinitis. 81.2% of eyes with panuveitis (15) showed a non granulomatous anterior uveitis A mild-moderate anterior chamber reaction was observed in 70% of affected eyes. In eyes with posterior uveitis (26), vitritis was moderate to severe in 60%. 68.4% of eyes presented with one retinochoroidal lesion, 19.2% with two, 10.2% with three and only 2 eyes (2%) had four lesions. Most frequent location was the posterior pole (72.7%). Vasculitis, papillitis, choroidal neovascularizarion and retinal detachment were found in 27% of cases. Serologic tests were done in 46 patients, IgG was positive in 84.8%. Patients with active inflammation receive treatment, most with pyrimethamine, trimethoprim/sulfamethoxazole and steroids.
Conclusion: In children, ocular toxoplasmosis is most commonly seen during the inactive stag. However, when the inflammation is present it can be severe and is frequently associated with other complications such vasculitis, papilitis choroidal neovascularization and retinal detachment.
Bob Ching- Li Cheng, Kristine Bacsal, Wee- Kiak Lim, Soon- Phaik Chee
Singapore National Eye Centre
Abstract
Purpose: To describe the clinical spectrum of fundus manifestations and angiographic and optical coherence tomography features of dengue associated maculopathy in a pediatric population.
Methods: Clinical records of patients diagnosed with dengue maculopathy at the Singapore National Eye Centre who were aged 16 and below between 2002 and 2005 were reviewed.
Results: Six patients with serological evidence of dengue fever with ocular signs and symptoms not attributable to other diseases within 1 month following dengue were identified. Four patients had bilateral involvement. Mean best-corrected LogMar visual acuity was 0.6 (range hand motions to 0.097). Clinical manifestations include vitritis (40% of eyes), intraretinal hemorrhages with venous sheathing (50%), yellow subretinal dots (30%). Fluorescein angiography demonstrated venular occlusion in 30%, combined arteriolar and venular occlusion in 10%, knobbly capillary hyperfluorescence in 10% and retinal pigment epithelium transmission window defect in 40% respectively. Half of the eyes had large vessel hyperfluorescence and/or late phase hypofluorescent spots on indocyanine green angiograph. Central or paracentral scotomas were observed in 70%. Three patients received steroid treatment. Mean visual acuity showed significant improvement between weeks 2 and 4, with an increasing proportion of eyes having a BCVA of 20/40 or better across time.
Conclusion: Fluorescein and indocyanine green angiography, optical coherence tomography and visual field testing are useful tools in the diagnosis of dengue maculopathy in the pediatric population.
Orozco - Carroll, M, Garza – León, MA, Arellanes –García, L
Objective: To describe the clinical manifestations and frequency of complications of VKH in Mexican Mestizo pediatric patients.
Material and methods: A two cohort (retrospective and prospective), descriptive and observational study was done. We retrieve the clinical files of all VKH patients and reviewed those in which diagnosis was made at 16 years or younger.
Age, gender, best corrected visual acuity (BCVA), intraocular pressure (IOP), extraocular and ocular manifestations, treatment, complication and its management, surgery and follow up time were recorded.
Results: From a group of 156 VKH patients we reviewed the files of nine (18 eyes). Five were females, mean age at onset was 10.7 years (4 to 15 years).Initial BCVA was: ≤20/400 in 8 eyes. In the last follow up visit it was ≥ 20/40 in 7 eyes, 20/50 -20/200 in 9 and ≤ 20/400 in 2.
Most common extraocular manifestations were: in the prodromic phase, headache and tinnitus; in the convalescent phase vitiligo and alopecia .
Most frequent ocular manifestations in the uveitic phase were: optic disk hyperemia - 83.3% and serous retinal detachment and panuveitis in 55.5%; in the convalescent phase: nummular yellowish lesions in 94.4% and sunset glow fundus in 77% .
Systemic steroids, oral or intravenous were used in all patients, 5 required repeated injections of periocular steroids. In one eye a single intravitreal triamcinolone injection was given. Only 3 patients were treated with other immunosuppressors (azathioprine and cyclophosphamide). At least one recurrence was found in four patients. Seven patients presented complications: cataract and glaucoma.
Conclusions: VKH syndrome is an uncommon disease among children, clinical manifestations and complications are similar to those found in adults. In our series most patients were successfully treated with corticosteroids.
Julie H. Tsai MD1, Somsiri Sukavatcharin MD1, Laurie Dustin MS2, & Narsing A. Rao MD1
1 Doheny Eye Institute and the Department of Ophthalmology, Keck School of Medicine of the University of Southern California, Los Angeles, California.
2 Department of Preventative Medicine, Keck School of Medicine of the University of Southern California, Los Angeles, California.
Abstract
Purpose: To determine the efficacy of oral prednisone as compared to combination therapy with corticosteroid and immunosuppressive agents in the treatment of acute and chronic Vogt-Koyanagi-Harada (VKH) disease.
Methods: A retrospective analysis was conducted on 116 consecutive patients diagnosed with VKH at a tertiary referral center. Sixty-seven patients with a minimum follow up time of six months were included in the study. Demographic characteristics, the stage of disease at presentation, and clinical features were documented.
Results: Overall visual acuity improved in 83.3% of patients with acute VKH over a three month period (p=0.05). Beyond the three months, improvement from baseline was noted in 79.2% (p=0.004). In those patients who did not improve with corticosteroid monotherapy, there was a trend toward improvement with the addition of immunomodulatory agents (p=0.18). In the chronic group, there was no statistically significant improvement in outcomes at the three month time point (p=0.31). The addition of immunosuppressant agents did not significantly alter the visual outcome (p=0.59). Disease recurrence does not appear to differ between disease stages or treatment groups; rather, it appears to be related to disease duration.
Conclusion: High dose oral corticosteroid therapy is an effective treatment for acute VKH in the vast majority of patients. However, this data suggests that if improvement in visual acuity has not been seen after twelve weeks of corticosteroid therapy, the addition of systemic immunomodulatory agents may be warranted. Aggressive therapy to prevent the development of chronic changes should be the goal, as neither corticosteroid monotherapy nor combined steroid and immunosuppressive regimens appear to positively affect the overall visual outcome of chronic VKH.
Jennifer E. Thorne, MD, PhD,1,3 Fasika Woreta, BS,1,3 Sanjay R. Kedhar, MD,1 James P. Dunn, MD1, Douglas A. Jabs, MD, MBA,1,2,3
1Departments of Ophthalmology and 2Medicine, the Johns Hopkins University School of Medicine; the 3Department of Epidemiology, Center for Clinical Trials, the Johns Hopkins University Bloomberg School of Public Health, Baltimore, Maryland.
Purpose: To describe the risk factors for and causes of visual acuity loss at presentation and during follow-up in a cohort of patients with juvenile idiopathic arthritis (JIA)-associated uveitis.
Methods: 75 patients with JIA-associated uveitis were evaluated from July 1984 through August 2005 at a single-center academic practice. Data on patients diagnosed with JIA-associated uveitis were entered retrospectively into a database and analyzed. Outcomes included visual acuity of 20/50 or worse and 20/200 or worse at presentation and during follow-up and the risk factors for and potential causes of such vision loss.
Results: The frequencies of 20/50 or worse and of 20/200 or worse visual acuities at presentation in affected eyes were 36% and 24%, respectively. The presence of > 1+ anterior chamber flare and a history of intraocular surgery prior to presentation were significantly associated with 20/50 or worse and 20/200 or worse vision. Presence of posterior synechiae was associated with 20/200 or worse vision at presentation. Among affected eyes, the incidence rates of loss of visual acuity to 20/50 or worse and to 20/200 or worse were 0.10/eye-year (EY) and 0.08/EY, respectively. Risk factors for incident vision loss in affected eyes included presence of posterior synechiae, > 1+ anterior chamber flare, and an abnormal intraocular pressure (IOP < 5mm or > 21mm Hg) at presentation. The most common causes of vision loss either at presentation or during follow-up for affected eyes were cataract and band keratopathy within the visual axis.
Conclusions: Loss of visual acuity was common in our patients with JIA-related uveitis. Presence of anterior chamber flare, posterior synechiae, and abnormal IOP were statistically significantly associated with poor visual acuity outcomes. Cataract and band keratopathy were the most common causes of vision loss.
Authors: Sarah Acevedo MD, Veena Rao BS, Karina Quinones MD, Rene Cervantes-Casteñada MD, C. Stephen Foster MD, FACS, FRCS
Purpose: To analyze visual outcomes following cataract extraction in patients with juvenile idiopathic arthritis (JIA) related uveitis. While cataract extraction may result in visual improvement, controversy exists regarding IOL implantation and perioperative management. For this reason, our aim was to analyze visual outcomes following cataract surgery in patients with JIA-related uveitis.
Methods: The clinical records of patients <18 years old with JIA related uveitis followed by us from January 1985 to December 2005 were reviewed. We identified 24 patients (30 eyes) who underwent cataract extraction with follow-up of ≥1 year. The main outcome measure was the difference between pre and post-operative visual acuity (Va). Additional parameters followed include intraocular inflammation, perioperative management, and post-operative complications.
Results: The mean age of patients at surgery was 8.95 years (Range: 4.5-16 years). Overall, 93.3% (28/30) of eyes showed improvement or maintenance of Va, while Va 6.67% (2/30) of eyes worsened. 46.67% (14) had final Va of >20/50, 30% (9) between 20/50 and >20/200, and 23.3% (7) with ≤20/200, the majority of which is attributable to pre-existing retinal or optic nerve pathology. Additionally, 76.67% (23) underwent concomitant pars plana vitrectomy, of which 21 (91.3%) showed improved Va. Twenty-three (76.67%) were left aphakic while 7 (23.3%) had lens implantation.
Conclusions: While cataract extraction results in visual improvement in most pediatric patients, this may not hold true for all patients with JIA-related uveitis. Controversy exists regarding perioperative treatment, IOL implantation, and case selection. Chronic inflammation, along with an increased risk of post-operative complications, make the decision of performing surgery in this cohort more challenging. We found that patients with JIA associated uveitis may benefit from cataract extraction given careful patient selection, optimal control of inflammation, and meticulous surgical planning.
Anzaar F1, Bhat P1, Quinones K1, Sobrin L1 , Foster CS1
1 Massachusetts Eye Research and Surgery Institute
Purpose: To report the efficacy and safety of mycophenolate mofetil (MMF) in the treatment of juvenile idiopathic arthritis-associated uveitis failing treatment with other immunomodulatory agents.
Methods: Medical records of all patients with the diagnosis of JIA from 1997-2006 were reviewed. Those who received MMF were identified. We recorded the dose of the drug, response to therapy and corticosteroids used. The side effects associated with use of MMF and other parameters were also recorded.
Results: Thirteen eyes of 9 patients with JIA uveitis were identified. The median age was 12 (range 3-40 years). All patients had been treated with methotrexate (MTX) prior to starting MMF, but the drug had been discontinued for at least 6 months prior to commencing MMF therapy. The mean duration of MMF use was 6 months (range 5-11 months). The median dosage of mycophenolate mofetil used was 1.5 grams (range 400 mg-2.5 g). Treatment was discontinued secondary to side effects in 3 patients: gastro-intestinal symptoms in two and leukopenia in the third. Remission was defined as the absence of cells in the anterior chamber, concurrent with no steroid therapy. Eight of the 9 patients went into remission while one patient showed trace cells on last follow up visit. The mean time to remission was 5 months (range 1-7 months). Visual acuity improved from baseline in 6 eyes (≥ 20/25 in 4 eyes), decreased in 2 eyes, and was unchanged in 5 eyes.
Conclusion: Our study suggests that MMF is an effective corticosteroid sparing agent in the treatment of JIA associated uveitis. Regular monitoring is important in order to prevent potentially serious side effects.
Quinones K1, Gallagher MJ1, Yilmaz T1, Acevedo SM1, Foster CS1.
1Massachusetts Eye Research and Surgery Institute
Purpose: To assess the efficacy and safety of various biologic agents in the treatment of noninfectious, refractory pediatric uveitis.
Methods: All pediatric patients treated by us with a biologic agent for their uveitis were identified. Main outcome measures analyzed included the quantitative measurement of intraocular inflammation, corticosteroid-sparing effects of the agent, and changes in visual acuity.
Results: Twenty five patients with refractory uveitis were identified. The biologics, used adjunctively with immunmodulating agents in 24 patients, included Daclizumab (n=3), Adalimumab (n=7), and Infliximab (n=15). The mean follow-up time for treatment was 7, 9, and 11 months, respectively. Diagnosis of the participants included: juvenile idiopathic arthritis with uveitis (n=21) and one patient each with Adamantiades-Behcet Disease, sarcoid associated uveitis, idiopathic panuveitis, and idiopathic anterior uveitis. There was an objective improvement in the intraocular inflammation in 76% of patients. Sixteen percent remained stable, while there was deterioration in 8% of cases. This correlated with a reduction in the use of topical and/or sytemic corticosteroids. With regards to visual acuities, there was an overall improvement in 64%, while 20% remained stable. No significant complications were reported. Two patients developed infections which did not require dicontinuation of therapy, and one had transient leukopenia.
Conclusion: The biologic agents included this study appear to be effective, well-tolerated, and valuable alternatives in the treatment of refractory pediatric uveitis.
Julie Lew, MD
Steven Yeh, MD
Robert Nussenblatt, MD
Grace Levy-Clarke, MD
National Eye Institute
National Institutes of Health
10 Center Drive
Bethesda MD 20892
Abstract:
Purpose: To describe a pediatric patient with SLE retinal vasculitis whose retinal neovascularization dramatically regressed over a matter of days after intraocular injection of bevacizumab.
Methods: Case report
Results: A sixteen year old female with a history of SLE first presented to our service in March of 2005 with decreased vision more so in the right eye than the left. Fluorescein angiogram (FA) done at that time showed choroidal ischemia OD>OS, active retinal vasculitis and pruned off retinal vasculature OU. The patient was treated at that time with full pan-retinal photocoagulation (PRP) OU and systemic immunosuppression. The patient’s vasculitis and retinal ischemia improved and her vision improved from 20/200 OD, 20/63 OS to 20/20 OD, 20/25 OS. 6 months after her initial presentation the patient developed a frond of retinal neovascularization elsewhere (NVE) in the right eye, and received additional PRP. Despite multiple sessions of PRP, NVE persisted, with development of a vitreous hemorrhage OD. Vision at that time was counting fingers OD. One week following the vitreous hemorrhage, the patient was given one injection of intravitreal bevacizumab (Avastin). Within one week, complete regression of NVE was observed by clinical exam and FA. The vitreous heme cleared centrally, and the patient’s vision recovered to 20/20.
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