Wanda Pfeifer

Nine-year results of a volunteer lay network photoscreening program of 147 809 children using a photoscreener in Iowa.

PURPOSE To present the largest cohort of preschool children screened by the MTI PhotoScreener over a 9-year period from a single, statewide vision screening effort. DESIGN Cross-sectional study. PARTICIPANTS We included 147,809 children screened between May 1, 2000, and April 30, 2009 by a photoscreening program. METHODS Retrospective review of results from the Iowa photoscreening program using the MTI PhotoScreener. The photographs were taken by volunteers from local Lions clubs and sent to the University of Iowa for interpretation. Children who failed the photoscreening were referred to local eye care professionals, who preformed a comprehensive eye evaluation and forwarded the results to the Iowa KidSight program. MAIN OUTCOME MEASURES Number of screenings, referral rate, positive predictive value (PPV), follow-up rate, and associated costs per year are described. RESULTS Over the 9 years of the continuously operating program, 147,809 children underwent photoscreens to detect amblyopic risk factors at 9746 sites. Because of abnormal photoscreen results, 6247 children (4.2%) were referred. Of the children, 24.3% were evaluated by local ophthalmologists and 76.7% were seen by local optometrists. Between 2000 and 2009, the follow-up rate ranged from a low of 36.1% to a high of 89.5%, with an overall program follow-up rate after the addition of the follow-up coordinator of 81.3%. The overall PPV of the MTI PhotoScreener was 94.2%. Taking into account overall operating budget including salaries and associated costs, the cost of screening 1 child has been reduced to

Immunosuppressive Treatment for Retinal Degeneration in Juvenile Neuronal Ceroid Lipofuscinosis (Juvenile Batten Disease)

US9 per child. CONCLUSIONS The addition of a part-time follow-up coordinator to the photoscreening program produced 89.5% follow-up rate when screening 147,809 children for amblyopia risk factors over a 9-year period.

The clinical evaluation of infantile nystagmus: What to do first and why

Abstract Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) presents with progressive vision loss at 4–7 years of age. Blindness results within 2 years, followed by inexorable neurologic decline and death. There is no treatment or cure. Neuroinflammation is postulated to play a role in the neurodegeneration. The JNCL mouse model demonstrated decreased neuroinflammation and improved motor skills with immunosuppression. Based on this work, a short-term human clinical trial of mycophenolate mofetil has begun, however longer term effects, and whether immunosuppression modulates vision loss, have not been studied. We report a JNCL patient treated with immunosuppressive therapy in whom visual function was comprehensively characterized over 2 years.

Effect of occlusion amblyopia after prescribed full-time occlusion on long-term visual acuity outcomes.

ABSTRACT Introduction: Infantile nystagmus has many causes, some life threatening. We determined the most common diagnoses in order to develop a testing algorithm. Methods: Retrospective chart review. Exclusion criteria were no nystagmus, acquired after 6 months, or lack of examination. Data collected: pediatric eye examination findings, ancillary testing, order of testing, referral, and final diagnoses. Final diagnosis was defined as meeting published clinical criteria and/or confirmed by diagnostic testing. Patients with a diagnosis not meeting the definition were “unknown.” Patients with incomplete testing were “incomplete.” Patients with multiple plausible etiologies were “multifactorial.” Patients with negative complete workup were “motor.” Results: A total of 284 charts were identified; 202 met inclusion criteria. The three most common causes were Albinism (19%), Leber Congenital Amaurosis (LCA; 14%), and Non-LCA retinal dystrophy (13%). Anatomic retinal disorders comprised 10%, motor another 10%. The most common first test was MRI (74/202) with a diagnostic yield of 16%. For 28 MRI-first patients, nystagmus alone was the indication; for 46 MRI-first patients other neurologic signs were present. 0/28 nystagmus-only patients had a diagnostic MRI while 14/46 (30%) with neurologic signs did. The yield of ERG as first test was 56%, OCT 55%, and molecular genetic testing 47%. Overall, 90% of patients had an etiology identified. Conclusion: The most common causes of infantile nystagmus were retinal disorders (56%), however the most common first test was brain MRI. For patients without other neurologic stigmata complete pediatric eye examination, ERG, OCT, and molecular genetic testing had a higher yield than MRI scan. If MRI is not diagnostic, a complete ophthalmologic workup should be pursued.

Recurrence of amblyopia after occlusion therapy.

PURPOSE To investigate the incidence and characteristics of occlusion amblyopia with prescribed full-time patching and determine its effect on long-term visual acuity outcomes. METHODS The records of patients younger than 10 years diagnosed as having amblyopia between 1970 and 2000 were retrospectively reviewed. Patients were prescribed full-time occlusion and observed until completion of therapy. RESULTS Of 597 patients treated for amblyopia by full-time patching, 115 were diagnosed as having occlusion amblyopia (19.3%). Seventy-five percent (86 of 115) developed occlusion amblyopia during the first episode of full-time patching. Occlusion amblyopia occurred more frequently in children prescribed full-time patching at an earlier age (P = .0002), with an odds ratio of 8.56 (95% confidence interval: 2.73, 26.84) in children younger than 36 months and 2.66 (95% confidence interval: 0.96, 7.37) in children between 36 and 59 months old. Seven of the patients with occlusion amblyopia did not reverse fixation and continued to fixate with the initially amblyopic eye after treatment. Final visual acuity in these eyes with occlusion amblyopia was 20/30 or better. After cessation of treatment, the final interocular difference in visual acuity was less in patients with a history of occlusion amblyopia (P = .003). CONCLUSION Occlusion amblyopia occurred at all ages, but the incidence decreased with increasing age. Patients who developed occlusion amblyopia with prescribed full-time occlusion had less interocular visual acuity difference than patients who did not, suggesting that development of occlusion amblyopia can indicate the potential for the development of better vision in the originally amblyopic eye.