title: Bevacizumab for choroidal neovascularisation in enhanced S-cone syndrome.
abstract: BACKGROUND
  We present a case of enhanced S-cone syndrome (ESCS)-associated choroidal neovascularisation (CNV) treated successfully with intravitreal bevacizumab therapy.
  A 14-year-old with a known retinal dystrophy presented with acute visual deterioration. Fluorescein angiography demonstrated CNV, and treatment was initiated with an anti-vascular endothelial growth factor (anti-VEGF) agent, with significant improvement in vision. Subsequent electroretinogram examination of the patient and her younger sister showed severely reduced rod responses with accentuated fast cone (S-cone only) response, confirming the diagnosis of ESCS as the underlying dystrophy.
  
  
  CONCLUSIONS
  CNV is a rare complication of ESCS that is responsive to anti-VEGF therapy. Although cystic retinal lesions may develop in patients with retinal dystrophies due to multiple possible aetiologies, CNV is a known cause of macula oedema in these patients that requires treatment with different agents, namely anti-VEGF therapy. Rapid visual loss in patients with inherited retinal disorders should prompt immediate clinical assessment to exclude CNV, and if CNV is detected, anti-VEGF therapy can preserve vision.
authors.affiliation: Save Sight Institute, The University of Sydney, Sydney, NSW, Australia.|Sydney Institute for Vision Science, Sydney, NSW, Australia.|Royal Victorian Eye and Ear Hospital, East Melbourne, VIC, Australia.
authors.forename: G K
authors.initials: GK
authors.lastname: Broadhead
authors.affiliation: Save Sight Institute, The University of Sydney, Sydney, NSW, Australia.
authors.forename: J R
authors.initials: JR
authors.lastname: Grigg
authors.affiliation: Save Sight Institute, The University of Sydney, Sydney, NSW, Australia.
authors.forename: P
authors.initials: P
authors.lastname: McCluskey
authors.affiliation: Save Sight Institute, The University of Sydney, Sydney, NSW, Australia.
authors.forename: M
authors.initials: M
authors.lastname: Korsakova
authors.affiliation: Save Sight Institute, The University of Sydney, Sydney, NSW, Australia. achang@sydneyretina.com.au.|Sydney Institute for Vision Science, Sydney, NSW, Australia. achang@sydneyretina.com.au.
authors.forename: A A
authors.initials: AA
authors.lastname: Chang
mesh_terms: D000293:Adolescent; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D000068258:Bevacizumab / Q000627:therapeutic use*; D020256:Choroidal Neovascularization / Q000188:drug therapy*; D015785:Eye Diseases, Hereditary / Q000150:complications*; D005260:Female; D006801:Humans; D012162:Retinal Degeneration / Q000150:complications*; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors; D014786:Vision Disorders / Q000150:complications*; D014792:Visual Acuity
chemical_list: D020533:Angiogenesis Inhibitors; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab
keywords: Bevacizumab; Choroidal neovascularisation; Electroretinography; Fluorescein angiography; Inherited retinal disease