Protocolo de tratamiento del edema macular quístico asociado a retinosis pigmentaria y otras distrofias hereditarias de la retina

  1. Jaume Català-Mora 1
  2. Juan Francisco Santamaría-Álvarez 2
  3. D. Kyriakou 3
  4. Socorro Alforja 4
  5. Marina Barraso-Rodrigo 1
  6. Paula Belén Blasco-Palacio 5
  7. Ricardo P. Casaroli-Marano 4
  8. Estefanía Cobos-Martin 2
  9. Rosa M. Coco-Martin 6
  10. C. Esmerado 7
  11. Amanda Garcia-Tirado 3
  12. Purificación Piñas García 3
  13. Alba Gómez-Benlloch 8
  14. Carmen Antía Rodríguez-Fernández 2
  15. F. Vilaplana Mira 9
  1. 1 Hospital Sant Joan de Deu
    info

    Hospital Sant Joan de Deu

    Barcelona, España

    ROR https://ror.org/001jx2139

  2. 2 Hospital Universitari de Bellvitge
    info

    Hospital Universitari de Bellvitge

    l'Hospitalet de Llobregat, España

    ROR https://ror.org/00epner96

  3. 3 Servicio de Oftalmología, Consorci Sanitari del Maresme, Mataró, Barcelona, España
  4. 4 Hospital Clinic Barcelona
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    Hospital Clinic Barcelona

    Barcelona, España

    ROR https://ror.org/02a2kzf50

  5. 5 Hospital Universitario de Basurto
    info

    Hospital Universitario de Basurto

    Bilbao, España

    ROR https://ror.org/00j4pze04

  6. 6 Universidad de Valladolid
    info

    Universidad de Valladolid

    Valladolid, España

    ROR https://ror.org/01fvbaw18

  7. 7 Hospital de Viladecans
    info

    Hospital de Viladecans

    Barcelona, España

    ROR https://ror.org/00t4w1v80

  8. 8 Hospital General de Granollers
    info

    Hospital General de Granollers

    Barcelona, España

    ROR https://ror.org/0190kj665

  9. 9 Hospital Universitari Germans Trias i Pujol
    info

    Hospital Universitari Germans Trias i Pujol

    Barcelona, España

    ROR https://ror.org/04wxdxa47

Revista:
Archivos de la Sociedad Española de Oftalmologia

ISSN: 0365-6691

Año de publicación: 2024

Volumen: 99

Número: 2

Páginas: 67-81

Tipo: Artículo

DOI: 10.1016/J.OFTAL.2023.09.005 DIALNET GOOGLE SCHOLAR

Otras publicaciones en: Archivos de la Sociedad Española de Oftalmologia

Resumen

Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the working population. Cystic macular edema (CME) is one of the treatable causes of visual loss, affecting up to 50% of the patients. A bibliographic review has been carried out combining “inherited retinal dystrophy”, “retinitis pigmentosa”, “macular edema” and a diagnostic-therapeutic protocol according to the levels of evidence and recommendations of the “US Agency for Healthcare Research and Quality”. This protocol has been discussed in the monthly meetings of the XAREA DHR group with the participation of more than 25 experts, creating a consensus document. The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pigment epithelium, and Müller cells, inflammation, and vitreous traction. OCT is the test of choice for the diagnosis and follow-up of CME associated with IRD. The drugs with the highest degree of scientific evidence are carbonic anhydrase inhibitors (IAC). Intravitreal corticosteroids, anti-VEGF, and vitrectomy with peeling of the internal limiting membrane do not have sufficient evidence. A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patients and an another for IRD and cataract surgery. Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment with corticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinical trials are required to establish the therapeutic scale in these patients.