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Rabih Hage,1 Claire Alapetite,2 Hervé Brisse,3,4 Kevin Zuber,5 Augustin Lecler,6 Guillaume Lot,7 Caroline Le Guerinel,7 Catherine Vignal-Clermont,1 Herve Boissonnet7 1Neuro-ophthalmology Department, Hôpital Fondation A. de Rothschild, Paris, France; 2Radiation Oncology Department, and Proton Center Institut Curie, Paris-Orsay, France; 3Imaging Department, Institut Curie, Paris, France; 4Université des Sciences et Lettres, Paris, France; 5Department of Statistics and Clinical Research, Hôpital Fondation A. de Rothschild, Paris, France; 6Radiology Department, Hôpital Fondation A. de Rothschild, Paris, France; 7Neurosurgery Department, Hôpital Fondation A. de Rothschild, Paris, FranceCorrespondence: Rabih Hage Tel/Fax +33148036322Email rhage@for.parisPurpose: Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France.Methods: We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at each follow-up MRI examination.Results: Sixty patients (50 women, 10 men; mean age, 45.2± 11.1y) were included, of whom 29 underwent surgery. At presentation, 52 (87%) of them had decreased vision (average visual acuity: 0.6 logMAR). Fundus examination showed optic disc swelling (n=27; 46.5%), optic disc pallor (n=22; 37.9%), optic disc cupping (n=2; 3.4%), opto-ciliary shunt (n=8; 13.8%), or choroidal folds (n=5; 8.6%). Otherwise, it was unremarkable (n=7; 12.1%). After treatment, visual function was stable overall. Fundus examination showed pallor (n=47; 83.9%), swelling (n=3; 5.4%), or cupping (n=2; 3.4%) of the optic disc, or was unremarkable (n=5; 8.9%). The visual field of 8 patients worsened, while 3 developed asymptomatic retinal hemorrhages. Tumor shrunk significantly in 8 patients at 1 year after PBT and remained stable in size in all others. Patients with opto-ciliary shunts had significantly worse visual outcome than other patients. Retinal abnormalities were observed in 11 patients during follow-up.Conclusion: PBT alone or in association with surgery appears to be a safe and efficient treatment for ONSM, reducing the tumor size and stabilizing visual function. The risk of developing radiation retinopathy seems to be higher when patients had upfront surgery.Keywords: optic nerve sheath meningioma, ONSM, proton beam therapy, PBT, compressive optic neuropathy, brain tumor, visual loss, opto-ciliary shunt, radiation retinopathy, RON

René Hage,1,2 Fiorenza Gautschi,1,2 Carolin Steinack,1,2 Macé M Schuurmans1,2 1University Hospital Zurich, Division of Pulmonology, Zurich, Switzerland; 2University of Zurich, Faculty of Medicine, Zurich, SwitzerlandCorrespondence: René HageUniversity Hospital Zurich, Division of Pulmonology, Raemistrasse 100, Zurich 8091, SwitzerlandTel +41 44 2559111Email rene.hage@usz.chBackground: Combined pulmonary fibrosis and emphysema (CPFE) is an underrecognized syndrome characterized by chronic, progressive disease with a dismal prognosis. Frequent co-morbidities with a higher incidence than in idiopathic pulmonary fibrosis or emphysema alone are pulmonary hypertension (WHO group 3) in 47– 90% of the patients and lung cancer in 46.8% of the patients.Objective: Review current evidence and knowledge concerning diagnosis, risk factors, disease evolution and treatment options of CPFE.Methods: We searched studies reporting CPFE in original papers, observational studies, case reports, and meta-analyses published between 1990 and August 2020, in the PubMed, Embase, Cochrane Library, Wiley Online Library databases and Google Scholar using the search terms [CPFE], [pulmonary fibrosis] OR [IPF] AND [emphysema]. Bibliographies of retrieved articles were searched as well. Further inclusion criteria were publications in English, French, German and Italian, with reference to humans. In vitro data and animal data were not considered unless they were mentioned in studies reporting predominantly human data.Results: Between May 1, 1990, and September 1, 2020, we found 16 studies on CPFE from the online sources and bibliographies. A total of 890 patients are described in the literature. Although male/female ratio was not reported in all studies, the large majority of patients were male (at least 78%), most of them were current or former heavy smokers.Conclusion: CPFE is a syndrome presenting with dyspnea on exertion followed by disruptive cough and recurrent exacerbations. The disease may progress rapidly, be aggravated by pulmonary hypertension WHO group 3 and is associated with an increased risk of lung cancer. Smoking and male sex are important risk factors. There is a need for more research on CPFE especially relating to etiology, influence of genetics, treatment and prevention options. Antifibrotic therapy might be an interesting treatment option for these patients.Keywords: COPD, pulmonary hypertension, autoimmune hypothesis

Harold Merle,1 Stéphane Olindo,2 Séverine Jeannin,2 Rabih Hage,1 Angélique Donnio,1 Raymond Richer,1 Philippe Cabre2 1Service d'Ophtalmologie, 2Service de Neurologie, Centre Hospitalier Universitaire de Fort de France, Fort-de-France, Martinique, France Purpose: Optic neuritis (ON) observed during neuromyelitis optica (NMO) is in most cases very severe and with poor prognosis. This study's objective was to analyze visual field (VF) abnormalities observed in the absence of ON and post-ON episode. Methods: Twenty-seven cases of both NMO and multiple sclerosis (MS) were selected. Thorough ophthalmologic exam was performed at least 6 months post-ON attack. The VF was collected using the Humphrey 750 perimeter. We used the central threshold tests 24-2 with FASTPAC strategy. The abnormalities were categorized based on the Optic Neuritis Treatment Trial classification. Results: After one ON, 40% of the NMO group's eyes showed total VF loss (P = 0.01), 21% showed abnormalities of neurologic aspect, and 27% showed fascicular abnormalities of which 12% were altitudinal. Given the total VF loss, the positive predictive value in favor of an NMO was 92.8% and the negative predictive value was 47.3%. Conclusion: Alterations of the VF during the NMO differ from those observed in the course of the MS. One ON, blinding from the first attack, must call to mind an NMO. The altitudinal deficits point to a vascular mechanism. Keywords: optic neuritis, visual field, neuromyelitis optica, multiple sclerosis

Rabih Hage, Albert Jean-Charles, Jérôme Guyomarch, Olivier Rahimian, Angélique Donnio, Harold MerleDepartment of Ophthalmology, University Hospital of Fort-de-France, Martinique, French West IndiesAbstract: Posterior scleritis is a rare underdiagnosed condition that can potentially cause blindness. Its varied presentations lead to delayed or incorrect treatment. We present here the cases of two patients with nodular posterior scleritis mimicking a choroidal metastasis. Two female patients presented with a sudden unilateral visual loss associated with ocular pain. Fundus examination revealed temporomacular choroidal masses with exudative detachments that, due to angiographic presentation, were suggestive of choroidal metastasis. Systemic examinations were unremarkable. In the two cases, a local or general anti-inflammatory treatment led to the complete recovery of the lesions, which were, thus, considered nodular posterior scleritis. The diagnosis of nodular posterior scleritis has to be evoked in all patients presenting with a choroidal mass in fundus examination. It represents the principal curable differential diagnosis of malignant choroidal tumor.Keywords: choroidal tumor, choroidal mass, visual loss, ocular pain, blindness, posterior scleritis