Matthieu Randon1, Christine Lévy-Gabriel2, Rachid Abbas3,4, Rémi Dendale5, Livia Lumbroso2, Laurence Desjardins2, Nathalie Cassoux2,6. 1. Ocular Oncology, Institut Curie, Paris, France. matthieu.randon@gmail.com. 2. Ocular Oncology, Institut Curie, Paris, France. 3. Biostatistics Unit, Gustave Roussy, Villejuif, France. 4. Team-Oncostat, Paris-Sud University, Villejuif, France. 5. Radiation Oncology, Institut Curie, Paris, France. 6. School of Medicine Paris V Descartes, Université PSL, Paris, France.
Abstract
PURPOSE: The Sturge-Weber Syndrome (SWS) is a phacomatosis which include facial nevus flammeus, glaucoma, diffuse choroidal hemangioma, and leptomeningeal hemangiomatosis. External beam radiotherapy (EBRT) using photons was used to treat retinal detachment. We investigate the anatomical and functional results in a long-term basis. METHODS: Retrospective review of SWS patients treated by EBRT (20 Gy in 10 fractions) for an exudative diffuse choroidal hemangioma. Visual acuity, B-scan tumor thickness, size of retinal detachment, intra-ocular pressure, and hypotonic treatment were collected before EBRT, 1 year after, and at the latest news. RESULTS: Twenty-five patients (26 eyes) were treated between 2001 and 2014. Retinal detachment including the macula was found among twenty-six eyes before treatment. The average follow-up time was 47 months. The mean tumor thickness was initially 4.5 mm, 2.8 mm at first year, and 2.7 mm at the last visit. The retina was reattached at the last visit for all eyes except two. The visual acuity was stable or better for 20 eyes (p = 0.02). Four patients developed mild cataract during the follow-up. CONCLUSION: EBRT using 20 Gy in 10 fractions is efficient, decreases tumor thickness, reattaches the retina, and stabilizes visual acuity. In the long term, retinal reattachment allows ocular conservation by preventing phthisis bulbi.
PURPOSE: The Sturge-Weber Syndrome (SWS) is a phacomatosis which include facial nevus flammeus, glaucoma, diffuse choroidal hemangioma, and leptomeningeal hemangiomatosis. External beam radiotherapy (EBRT) using photons was used to treat retinal detachment. We investigate the anatomical and functional results in a long-term basis. METHODS: Retrospective review of SWSpatients treated by EBRT (20 Gy in 10 fractions) for an exudative diffuse choroidal hemangioma. Visual acuity, B-scan tumor thickness, size of retinal detachment, intra-ocular pressure, and hypotonic treatment were collected before EBRT, 1 year after, and at the latest news. RESULTS: Twenty-five patients (26 eyes) were treated between 2001 and 2014. Retinal detachment including the macula was found among twenty-six eyes before treatment. The average follow-up time was 47 months. The mean tumor thickness was initially 4.5 mm, 2.8 mm at first year, and 2.7 mm at the last visit. The retina was reattached at the last visit for all eyes except two. The visual acuity was stable or better for 20 eyes (p = 0.02). Four patients developed mild cataract during the follow-up. CONCLUSION: EBRT using 20 Gy in 10 fractions is efficient, decreases tumor thickness, reattaches the retina, and stabilizes visual acuity. In the long term, retinal reattachment allows ocular conservation by preventing phthisis bulbi.
Authors: Christine Léauté-Labrèze; Eric Dumas de la Roque; Thomas Hubiche; Franck Boralevi; Jean-Benoît Thambo; Alain Taïeb Journal: N Engl J Med Date: 2008-06-12 Impact factor: 91.245
Authors: R V Paul Chan; Yoshihiro Yonekawa; Anne Marie Lane; Dimitra Skondra; John E Munzenrider; J Michael Collier; Evangelos S Gragoudas; Ivana K Kim Journal: Ophthalmologica Date: 2009-11-24 Impact factor: 3.250