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Introduction: Ibrutinib is a small-molecule drug approved for the treatment of haematological disorders and is known to be associated with visual disturbances, but uveitis has not yet been reported as an adverse effect of this medication. We present two cases of ibrutinib-associated severe uveitis in patients with chronic lymphocytic leukaemia., Case Description: Our first case is a 65-year-old woman who presented with acute onset of bilateral fibrinous anterior uveitis 1 day after starting ibrutinib. Her vision was hand movements in the right eye and 20/120 in the left with hyperaemic discs and subretinal fluid. Ibrutinib was stopped and she experienced a significant improvement under local and oral steroid treatment. The second case is a 64-year-old male with subacute onset of bilateral hypertensive anterior uveitis with pupillary seclusion and right eye hyphaema. He was on ibrutinib for the past 9 months. His vision at presentation was 20/80 and 20/60 for the right and left eye, respectively. He responded poorly to local steroid treatment until ibrutinib was stopped due to cardiac side-effects, after which his uveitis resolved and treatment was stopped., Conclusion: The temporal association between changes in ibrutinib treatment and our patients' ocular inflammation suggests a causative link. Ibrutinib increases Th1-based immune responses which is proposed as a mechanism for drug-induced uveitis. Its antiplatelet effect may explain the fibrinous nature of the inflammation and hyphaema.

Background: Alström syndrome is a rare genetic ciliopathy caused by a mutation in the ALMS1 gene. The syndrome is characterized by cone-rod dystrophy, dilated myocardiopathy, childhood obesity and sensorineural hearing loss. To date, cystoid macular edema has not been reported., Methods: A female affected by Alström syndrome developed bilateral cystoid macular edema evidenced by optical coherence tomography. A topical carbonic anhydrase inhibitor was prescribed., Results: Complete resolution of the cystoid macular edema was achieved, though visual acuity did not improve., Conclusions: Topical carbonic anhydrase inhibitors may have a role in the treatment of macular edema in syndromic retinal dystrophies such as Alström syndrome.

Purpose: To evaluate the treatment outcomes of intravitreal bevacizumab injections as monotherapy in type 1 retinopathy of prematurity (ROP)., Methods: A retrospective chart review was performed for patients with type 1 ROP who had intravitreal bevacizumab injections between November 2013 and January 2015 at La Paz University Hospital in Madrid, Spain. Gestational age at birth, birth weight, sex, ROP zone, ROP stage, mean age at treatment, and follow-up period were recorded. The final clinical status of the retina was noted for each patient. The primary outcome measures included ROP recurrences requiring re-treatment, complete or incomplete peripheral vascularization, mean age at complete vascularization, and refractive errors., Results: From 14 patients enrolled with type 1 ROP, 28 eyes were included. The mean gestational age at birth was 25.9 ± 2.34 weeks (range: 23.6 to 32.4 weeks) and the median birth weight was 694 g (range: 487 to 1,740 g). All eyes showed zone II ROP: 18 eyes (64.3%) had anterior zone II ROP and 10 eyes (35.7%) had posterior zone II ROP. One week after intravitreal bevacizumab injection, 14 eyes (50%) had achieved complete regression of ROP, and a partial regression of ROP was observed in 10 eyes (35.7%). Twenty-two eyes (78.6%) obtained complete vascularization during the follow-up. The median time to complete vascularization was 134 ± 21.45 days. The mean spherical equivalent at last visit was 1.99 diopters., Conclusions: Intravitreal bevacizumab injection used as a monotherapy is an effective treatment approach in patients with zone II ROP. [J Pediatr Ophthalmol Strabismus. 2016;53(6):375-382.]., (Copyright 2016, SLACK Incorporated.)

Purpose: To describe a series of 5 patients with herpes simplex virus keratitis (HSK) and rheumatoid arthritis (RA) under immunosuppressive treatment., Methods: Retrospective study. Detailed data were obtained regarding symptoms and signs at the initial evaluation, treatment, microbiological diagnostic tests, evolution, and outcomes., Results: Five patients with HSK and RA were identified. Bilateral involvement occurred in 2 patients (40%). Epithelial keratitis was diagnosed in 5 eyes. Three eyes showed severe melting with eye perforation. Gram-positive bacterial co-infections were common in the group with stromal keratitis. We did not find differences in the evolution of the disease based on anti-rheumatoid treatment., Conclusions: The characteristics of HSK in patients with RA differed from HSK in immunocompetent patients. The stromal keratitis cases were very aggressive and difficult to manage, with perforation and gram-positive bacterial co-infection as frequently associated conditions. Prophylactic therapy at standard doses was unsuccessful to avoid recurrences.

Purpose: To review the systemic and ocular outcomes and long-term status of ocular rhabdomyosarcoma in pediatric patients in a tertiary center in Spain., Methods: All patients younger than 18 years who were diagnosed as having ocular rhabdomyosarcoma and treated between 1982 and 2011 at La Paz University Hospital, Madrid, Spain, were included. Clinical presentation, management, complications, and ocular and systemic outcomes were reviewed., Results: The mean age at presentation was 8 years (range: 3 months to 12.5 years). In all cases, the rhabdomyosarcoma was located primarily in the orbit. Treatment included surgical debulking and various regimens of chemotherapy and radiotherapy. All of the patients underwent surgical biopsy for diagnosis confirmation. Orbital exenteration was performed in 4 cases (28%). Twelve patients received radiotherapy. The long-term visual outcomes of the 10 patients who maintained their globe was as follows: best corrected visual acuity 20/20 to 20/40 in 6 patients (60%), 20/50 to 20/100 in 2 patients (20%), and 20/200 to no light perception in 2 patients (20%). Intraocular complications (primarily cataracts: 50%) were present in 7 patients (70%), ocular surface lesions occurred in 6 patients (60%), and orbital sequelae were found in 8 patients (80%). Local tumor recurrence was detected in 5 patients (35%) and distant metastasis occurred in 2 patients (14%). Tumor-related death occurred in 1 patient (7%)., Conclusions: Orbital rhabdomyosarcoma has an excellent prognosis; nevertheless, local complications are common, including surgery-related complications. To minimize them, initial surgical planning based on individual patient characteristics and an accurate diagnosis of relapses is mandatory. The clinical presentation, management, and long-term ocular and systemic outcomes are comparable with other series published to date., (Copyright 2016, SLACK Incorporated.)

Ten patients with craniopharyngioma treated for the first time when younger than 18 were included. This study reviews the visual outcomes and provides information on visual field (VF) and optical coherence tomography (OCT) examination of craniopharyngioma. The best kappa concordance coefficients between VF and OCT parameters of atrophy were obtained for the ganglion cell (GC) thickness and the mean retinal nerve fibre layer (RNFL) thickness. The agreement between GC colour maps and VF defects was good. Optic nerve compression may be detected by RNFL measurement and GC analysis, and this may be valuable to predict visual recovery and in uncooperative patients to evaluate visual damage.

Purpose: To report the results of adalimumab therapy in a cohort of children with refractory noninfectious uveitis., Methods: The medical records of patients diagnosed with uveitis and treated with biweekly adalimumab injections for a period of at least 3 months at the University Hospital of La Paz from 2007-2012 were retrospectively reviewed. Improvement in inflammatory activity was graded according to grading schema of the Standardization of Uveitis Nomenclature Working Group., Results: A total of 15 patients participated in the study (12 girls; mean patient age, 12 years). Diagnoses included juvenile idiopathic arthritis in 10 patients, idiopathic uveitis in 4, and familial juvenile systemic granulomatosis or Blau syndrome in 1. Mean follow-up was 32 months (median, 36; range, 15-58 months). Improvement in inflammatory activity was initially observed in 12 (86%) of 14 children, with a mean time to achieve response of 6 weeks (median, 4; range, 1-18). Treatment was effective in 9 patients (60%), mildly effective in 2 (13%), ineffective in 2 (13%), and resulted in worsening in 2 (13%). In the juvenile idiopathic arthritis patients, response was effective in 6 cases (60%), mildly effective in 2 (20%), and ineffective in 2 (20%). Adalimumab therapy was discontinued in 4 patients., Conclusions: Adalimumab was effective in most patients in the initial control of acute inflammatory activity in children with refractory uveitis, although therapy appears to become less effective in the long term., (Copyright © 2013 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)