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To report a case of overlapping choriocapillaritis that initially presented as multifocal choroiditis (MFC) but later showed features compatible with acute zonal occult outer retinopathy (AZOOR) resistant to standard immunosuppression that responded only to adalimumad therapy. A 41-year-old patient presented with multiple small, discrete yellow-whitish spots in both eyes, compatible with MFC. A few weeks later, despite treatment with sub-Tenon and systemic corticosteroids, a choroidal neovascularization occurred in the right eye. The patient was treated with intravitreal anti-vascular endothelial growth factor. After 2 months, reduced visual acuity, photopsia and visual field defect in the left eye occurred. Spectral domain optical coherence tomography revealed photoreceptor outer segment defects common to all choriocapillaritis. The additional finding of an annular scotoma and a 360° ring on indocyanine green angiography led us to make the diagnosis of presumed AZOOR. Despite the combination of several immunosuppressive agents leading to temporary control of the disease, the patient experienced a further worsening. At that point, adalimumab was introduced, which led to an obvious improvement. This case supports the hypothesis that two different entities of the so-called AZOOR complex can be possible in the same eye, even asynchronously. In our case, anti-tumor necrosis factor alpha monoclonal antibody therapy represented a valid treatment option in a patient unresponsive to traditional immunosuppressive treatments.

Objectives: The aim of this paper is to present two cases of severe idiopathic non-infectious paediatric panuveitis, unresponsive to traditional therapy, successfully treated with Adalimumab (HumiraTM, Abbott Pharmaceutical Inc.) in the long term., Methods: The data of the two cases are presented and the literature is reviewed., Results: At base line, case 1 had 0.2 in the RE and 0.5 in the LE, while case 2 had 0.5 and 0.4 in the RE and LE, respectively. The anterior chamber (AC) of case 1 had 3+ cells and 3+ flare in both eyes, as well as diffuse keratic precipitates (Kps). Case 2 presented 2+ cells and 3+ flare in both eyes, as well as tiny Kps in the inferior part of the endothelium. The Binocular Indirect Ophthalmoscopy (BIO) score was +2 in both eyes of case 1 and case 2 at first examination. After Adalimumab initiation, both patients presented a dramatic resolution of the ocular inflammation, as well as a rapid improvement of the BCVA. Case 1 had 0.8 and 1.0 in the RE and the LE, respectively, while case 2 presented 1.0 in both eyes. At the last visit, both patients presented a quiet uveitis and stable BCVA: case 1 had 0.8 and 1.0 in the RE and the LE, respectively, while case 2 presented 1.0 in both eyes. No side effects were recorded during this time., Conclusions: Adalimumab can be a promising drug for the therapy of severe, refractory paediatric uveitis, although further studies are needed on its application in uveitis.

Purpose: To evaluate the morphologic features of the photoreceptor layer (by spectral-domain optical coherence tomography) and functional parameters in patients with a lamellar macular hole., Design: Prospective, multicenter, observational case series., Methods: Fifty-four patients with lamellar macular hole were enrolled in the study. All patients underwent a complete ophthalmologic examination, including best-corrected visual acuity (BCVA) testing, MP1 microperimetry, and spectral-domain optical coherence tomography. For each patient, 2 experienced masked observers evaluated the integrity of photoreceptor inner segment/outer segment (IS/OS) junction and external limiting membrane (ELM) line., Results: Spectral-domain optical coherence tomography analysis showed complete integrity of the IS/OS junction and ELM line in 40 eyes (group A), partial or complete disruption of the IS/OS junction with an intact ELM line in 8 eyes (group B), and an alteration of both IS/OS junction and ELM line in 6 eyes (group C). Mean BCVA, total retinal sensitivity, and fixation stability were significantly better in groups A and B than in group C (both P < .05, Tukey-Kramer test), whereas there was no significant difference between groups A and B. Mean central retinal sensitivity was significantly different among all 3 groups (all P < .05, Tukey-Kramer test). The grade of integrity of the foveal photoreceptor layer was correlated significantly with mean BCVA (r = -0.57; P < .001), mean central retinal sensitivity (r = 0.52; P < .001), and total retinal sensitivity (r = 0.44; P < .001)., Conclusions: In lamellar macular hole, the morphologic features of the foveal photoreceptor layer consistently are correlated with BCVA and central retinal sensitivity. Preservation of the ELM is related to the maintenance of visual acuity., (Copyright © 2012 Elsevier Inc. All rights reserved.)

To study the functional recovery of a patient with cerebrovascular injury using combined functional magnetic resonance imaging (fMRI) and diffusion tensor imaging (DTI). A 24-year-old woman with left hemianopsia underwent fMRI and DTI in a 1.5-tesla machine both in the acute phase and 1 month after an ischaemic stroke involving the right calcarine cortex. Acute-phase fMRI demonstrated that peripheral left visual field stimulation did not activate the right primary visual cortex, whereas stimulation 1 month later activated the visual cortex bilaterally. Analysis of acute-phase DTI data disclosed that a reduction of fractional anisotropy in the right optic radiation had almost resolved after 1 month. Fibre direction was normal at either time point. fMRI and DTI can demonstrate functional damage and recovery in patients with neuro-ophthalmological lesions.

Tumor necrosis factor alpha (TNF-α) is a key soluble mediator involved in the inflammatory cascade of many disorders including uveitis. Among the anti-TNF-α agents, one of the most used in immune-mediated diseases, such as inflammatory arthropathies, is adalimumab (Humira™, Abbott Pharmaceutical Inc.), a fully humanized antibody. The purpose of this review is to analyze the main pharmacological and clinical aspects of adalimumab and its efficacy both in systemic and ocular inflammatory disorders. Adalimumab was effective in treating several autoimmune diseases, such as rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis. In recent years, adalimumab has been used successfully in refractory cases of intraocular inflammation. Moreover, this biological agent showed good safety and efficacy profiles in ocular use including childhood uveitis. Switching from other anti-TNF-α agents to adalimumab may offer several advantages, such as easier administration, better patient compliance, and lower rate of adverse events. Adalimumab is a promising drug for the therapy of uveitis, although further studies are needed on its application in uveitis.

To study the efficacy of systemic steroids (SS) associated with mycophenolate mofetil (MMF) for the control of juxta/sub-foveal uveitic choroidal neovascularization (CNV) unresponsive to the traditional immunosuppressive agents. Patients with juxta/sub-foveal uveitic CNV unresponsive to the traditional immunosuppressive drugs were treated with SS and MMF. The study was designed as a prospective, consecutive, open-label, interventional case series. Visual gain and loss were defined as improving or worsening of two or more lines of best-corrected visual acuity (BCVA), respectively. CNV size outcome was dichotomized as "increased" or "stable/reduced", if increased >200 μm(2), or reduced ≥ 200 μm(2) or not modified by 200 μm(2), respectively. Nine cases (12 eyes) have been considered; ages ranged from 27 to 56 years. The mean follow-up time was 18.2 ± 2.9 months (min: 14 months, max: 23 months). At base-line, the mean BCVA was 0.3 ± 0.17, improving up to 0.57 ± 0.25 and to 0.63 ± 0.22 (P < 0.001, paired t-test) at the 6 and 12-month follow-ups, respectively. At the last follow-up, all the patients had stable/improved BCVA (P < 0.0001, Fisher's exact test) and stable/reduced lesion size (P < 0.0001, Fisher's exact test). None of the patients complained of any severe adverse event during the treatment. The combination of SS and MMF seems to be a promising strategy in order to control uveitic CNVs unresponsive to the traditional immunosuppressive agents. Further studies are needed to validate the data of this case series.

To describe a case of macular pucker treated by non-vitrectomizing vitreoretinal surgery using a 25-gauge high-flow infusion cannula with a 27-gauge illumination source (Photon II; both from Synergetics USA Inc., O'Fallon, MO). A 42-year-old man complaining of visual reduction and metamorphopsia was referred to the Vitreoretinal Surgery Unit, where he underwent full ophthalmic examination. Best corrected visual acuity (BCVA) at baseline was 0.5 in the left eye (LE) and 1.0 in the right eye. He was diagnosed with epiretinal membrane (ERM) in the LE. The surgical procedure envisaged two-port sclerotomy, one for the infusion line of the 27-gauge Photon II light source, the other for the 25-gauge trocar in the superotemporal quadrant. The ERM was removed with a 25-gauge disposable forceps. The infusion line was placed for safety in the event of additional intraocular pressure being needed, but was not required. The 27-gauge light source afforded good visualization of the posterior pole and the ERM. At 12 months, the patient's BCVA had improved to 1.0, and his cataract has not progressed. A 25-gauge infusion line used with the photon illumination may further improve non-vitrectomizing surgery, minimizing risk and optimizing outcome. Further trials are required to validate these preliminary observations.

Purpose: To evaluate the efficacy and safety of photodynamic therapy (PDT) in the long-term control of subfoveal choroidal neovascularization (CNV) associated with toxoplasmic retinochoroiditis., Methods: The records of 13 patients with classic subfoveal CNV associated with toxoplasmic retinochoroiditis treated with PDT were reviewed. All patients were followed up for at least 48 months. Postoperative visual acuity was defined as a gain or loss of two or more lines of best-corrected visual acuity (BCVA), respectively. Post-treatment CNV size was dichotomized into "increased" if the major CNV diameter (CMD) had increased by >or=300 microm, and as "stable/reduced" if it had decreased by >or=300 microm or had not changed by >300 microm., Results: Nine patients [four males (44.4%) and five females (55.6%)] with a mean age of 20.1 +/- 4.3 years (range 14-27 years) were enrolled in the study. All had unilateral involvement. The median follow-up was 55 months (minimum 48, maximum 65 months). At the 48-month follow-up, all patients had stable/improved BCVA and a mean stable/reduced CMD (846 +/- 326.5 microm), with the BCVA having improved significantly (p < 0.0001) from 0.29 +/- 0.19 at baseline to 0.54 +/- 0.16 at 48 months., Conclusion: Photodynamic therapy seems to be a safe and effective approach to the long-term control of subfoveal CNV associated with toxoplasmic retinochoroiditis. Further trials are needed to validate these findings.

Purpose: To evaluate the long-term safety and efficacy of Mycophenolate Mofetil (MMF) for the control of cystoid macular oedema (CMO) secondary to noninfectious uveitis (NU)., Methods: The medical records of 19 consecutive patients with inflammatory CMO treated with MMF were retrospectively reviewed. Patient demographics, best corrected visual acuity (BCVA), fluorescein angiography (FA), and optical coherence tomography (OCT) findings were evaluated., Results: There were eight females and 11 males with a mean age of 32.9 +/- 8.9 years. After a 1-year follow-up, 18/19 patients (31 eyes, 96.9%, P < 0.05) no longer had signs of CMO, as per their FA and OCT findings; the mean central foveal thickness (CFT) was 167.2 +/- 12.8 microm. At the last follow-up, only 3/19 patients, all affected by Behçet panuveitis, had recurrences of CMO. Mean BCVA improved from 0.34 +/- 0.14 SD at baseline to 0.65 +/- 0.2 SD at last follow-up., Conclusions: MMF was safe and effective in controlling CMO and in reducing the uveitis relapse rate in patients not responding to traditional immunosuppressants. Further case-controlled studies are mandatory to validate those preliminary results.

Purpose: To investigate the presence of the fibronectin isoform containing the extradomain B (B-FN), a marker-protein of angiogenesis, in surgically excised human choroidal neovascular membranes (CNVM) to evaluate whether B-FN could be used as a therapeutic target for specific antibody-photosensitizer immunoconjugates., Design: Laboratory investigation., Methods: The setting was an institutional practice. The study population consisted of 15 eyes (15 patients) with CNVM undergoing membrane excision (four eyes with age-related macular degeneration, seven with pathologic myopia and four with multifocal choroiditis). The control group consisted of eight eye bank eyes (four subjects) without choroidal neovascularization. Light microscopic immunohistochemistry on cryostat sections of tissues was obtained. B-FN was detected by a human recombinant antibody, CGS-1, and compared with immunostaining for endothelial cells with factor VIII-related antigen. The main outcome measure was the presence of CGS-1 positively stained cells or areas of the extracellular matrix. Staining of CGS-1 was scored on a scale from 0 to 3., Results: Fourteen of 15 neovascular membranes stained strongly with CGS-1 (score 2 or 3). One membrane from a patient with pathologic myopia was negatively stained (score 0). CGS-1 positive staining was detected around endothelial cells and in the extracellular matrix of CNVMs. The retina of eyes without choroidal neovascularization was negative with CGS-1 in all eight donor eyes, while the choroid contained some weakly CGS-1 positive cells (score 0 and 1, respectively)., Conclusions: The extradomain B is abundantly expressed in CNVMs, but its expression is more restricted in eyes harboring no apparent choroidal neovascularization. In the future, B-FN might serve as a target for the delivery of antibody-photosensitizer immunoconjugates to newly developed vessels to enhance the selectivity of photodynamic therapy.