title: Management of Cystoid Macular Edema in Retinitis Pigmentosa.
abstract: Retinitis pigmentosa is a genetically heterogeneous disorder with an estimated prevalence of one in 4,000 that is classically characterized by the progressive constriction of peripheral vision and a later deterioration of visual acuity. Central vision can be compromised earlier in disease, however, in the approximately 25% of patients that have cystoid macular edema. This poorly understood problem can thus significantly impair patient quality of life, particularly as available treatments have limited efficacy. We will review clinical features of retinitis pigmentosa-associated cystoid macular edema, potential causative mechanisms, and finally, evidence supporting currently employed therapies with emphasis upon which management strategies require further evidence-based evaluation.
authors.affiliation: a Department of Ophthalmology , Massachusetts Eye and Ear , Boston , MA , USA.
authors.forename: Rachel M
authors.initials: RM
authors.lastname: Huckfeldt
authors.affiliation: a Department of Ophthalmology , Massachusetts Eye and Ear , Boston , MA , USA.
authors.forename: Jason
authors.initials: J
authors.lastname: Comander
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D002257:Carbonic Anhydrase Inhibitors / Q000627:therapeutic use; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008269:Macular Edema / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D013508:Ophthalmologic Surgical Procedures; D012174:Retinitis Pigmentosa / Q000150:complications*; D041623:Tomography, Optical Coherence
chemical_list: D020533:Angiogenesis Inhibitors; D002257:Carbonic Anhydrase Inhibitors; D005938:Glucocorticoids
keywords: Carbonic anhydrase inhibitor; cystoid macular lesion; inherited retinal degeneration