title: Cystoid macular lesions are resistant to topical dorzolamide treatment in enhanced S-cone syndrome child.
abstract: OBJECTIVE
  To evaluate whether cystoid macular lesions respond to treatment with dorzolamide 2% drops in the enhanced S-cone syndrome (ESCS) child, as several case reports document favorable efficacy in adults.
  
  
  METHODS
  Seven-year-old boy with ESCS and cystoid macular lesions was treated with dorzolamide 2% in both eyes three times a day for a period of 7 months. The efficacy of treatment was analyzed by visual acuity assessment (ETDRS), multifocal electroretinography and SD-OCT central foveal thickness (CFT) measurement.
  
  
  RESULTS
  Baseline RE CFT was 540 and 453 µm in the LE, with amplitude of P1-wave density 39.8 and 50.4 nV/deg(2), respectively. Best corrected visual acuity (BCVA) was 0.3 logMAR RE and 0.3 logMAR LE at distance. At 7-month follow-up examination, CFT showed no reduction in thickness (RE 599 µm, LE 521 µm). P1-wave density increased (RE 49.1 nV/deg(2), LE 84.9 nV/deg(2)), with BCVA 0.3 logMAR RE and 0.2 logMAR LE.
  
  
  CONCLUSIONS
  To the best of our knowledge, this is the youngest ESCS patient treated with dorzolamide drops and the first report recording that cystoid macular lesions are resistant to topical dorzolamide treatment. Furthermore, these data are in favor of the hypothesis that microcystoid changes in ESCS appear due to defects in cell-to-cell adhesion rather than the disintegration of the retinal barrier. The marked differences in treatment response to carbonic anhydrase inhibitors between the adults and the child here presented suggest that the breakdown of the blood-retinal barrier may play a more important role later in life.
authors.affiliation: University Eye Clinic, Faculty of Medicine Josip Juraj Strossmayer University of Osijek, University Hospital "Sveti Duh", Sveti Duh 64, 10000, Zagreb, Croatia.
authors.forename: Mladen
authors.initials: M
authors.lastname: Bušić
authors.affiliation: University Eye Clinic, Faculty of Medicine Josip Juraj Strossmayer University of Osijek, University Hospital "Sveti Duh", Sveti Duh 64, 10000, Zagreb, Croatia. mbjelos@mef.hr.
authors.forename: Mirjana
authors.initials: M
authors.lastname: Bjeloš
authors.affiliation: University Eye Clinic, Faculty of Medicine Josip Juraj Strossmayer University of Osijek, University Hospital "Sveti Duh", Sveti Duh 64, 10000, Zagreb, Croatia.
authors.forename: Damir
authors.initials: D
authors.lastname: Bosnar
authors.affiliation: University Eye Clinic, Faculty of Medicine Josip Juraj Strossmayer University of Osijek, University Hospital "Sveti Duh", Sveti Duh 64, 10000, Zagreb, Croatia.
authors.forename: Senad
authors.initials: S
authors.lastname: Ramić
authors.affiliation: University of Zagreb School of Medicine, Šalata 3, 10000, Zagreb, Croatia.
authors.forename: Iva
authors.initials: I
authors.lastname: Bušić
mesh_terms: D000287:Administration, Topical; D002257:Carbonic Anhydrase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D002648:Child; D004596:Electroretinography; D015785:Eye Diseases, Hereditary / Q000188:drug therapy* / Q000503:physiopathology; D005451:Fluorescein Angiography; D006801:Humans; D008269:Macular Edema / Q000188:drug therapy* / Q000503:physiopathology; D008297:Male; D009883:Ophthalmic Solutions; D012160:Retina / Q000503:physiopathology; D012162:Retinal Degeneration / Q000188:drug therapy* / Q000503:physiopathology; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use*; D013876:Thiophenes / Q000008:administration & dosage / Q000627:therapeutic use*; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000188:drug therapy* / Q000503:physiopathology; D014792:Visual Acuity / Q000502:physiology
chemical_list: D002257:Carbonic Anhydrase Inhibitors; D009883:Ophthalmic Solutions; D013449:Sulfonamides; D013876:Thiophenes; C062765:dorzolamide
keywords: Amblyopia; Carbonic anhydrase inhibitors; Enhanced S-cone syndrome; Multifocal electroretinography; Optical coherence tomography