International Ophthalmology

, Volume 29, Issue 2, pp 103–107 | Cite as

Intravitreal pegaptanib sodium (Macugen) for refractory cystoid macular edema in pericentral retinitis pigmentosa

  • Giuseppe Querques
  • Francesco Prascina
  • Cristiana Iaculli
  • Nicola Delle Noci
Case report


The purpose of this paper was to describe a patient with pericentral retinitis pigmentosa (RP) and cystoid macular edema (CME) refractory to oral acetazolamide alone who was successfully treated with adjunctive pegaptanib sodium. A 33-year-old man presented with decreased vision and a best-corrected visual acuity (BCVA) in his left eye of 20/200 due to CME secondary to RP. Although he had been treated daily for 1 month with 500 mg of oral acetazolamide, the edema was unresponsive. When informed of the available treatment options, the patient requested adjunctive intravitreal pegaptanib sodium. One month after receiving an injection of pegaptanib sodium 0.3 mg and continued daily acetazolamide, the patient’s BCVA had improved to 20/40. At the 4-month follow-up visit, no recurrence of CME was found on fundus biomicroscopy, fundus-related perimetry, and optical coherence tomography. We conclude that intravitreal pegaptanib sodium combined with daily doses of acetazolamide appears to provide benefits in CME refractory to oral acetazolamide alone with regards to the improvement of visual acuity.


Cystoid macular edema Fundus-related perimetry Macugen Optical coherence tomography Pegaptanib sodium Pericentral retinitis pigmentosa 


  1. 1.
    Sandberg MA, Gaudio AR, Berson EL (2005) Disease course of patients with pericentral retinitis pigmentosa. Am J Ophthalmol 140:100–106PubMedGoogle Scholar
  2. 2.
    Noble KG, Carr RE (1978) Peripapillary pigmentary retinal degeneration. Am J Ophthalmol 86:65–75PubMedGoogle Scholar
  3. 3.
    Hayasaka S, Fukuda K, Tsuchiya M, Mizuno K (1985) Pericentral pigmentary retinal degeneration. Jpn J Ophthalmol 29:161–169PubMedGoogle Scholar
  4. 4.
    Yamaguchi K, Kin-Para Y, Tamai M (1991) Idiopathic central serous choroidopathy in a patient with pericentral pigmentary retinal degeneration. Ann Ophthalmol 23:251–253PubMedGoogle Scholar
  5. 5.
    Fenkenhour CL, Choromokos E, Weinstein J, Shoch D (1977) Cystoid macular oedema in retinitis pigmentosa. Trans Am Acad Ophthalmol Otolaryngol 83:515–521Google Scholar
  6. 6.
    Fishman GA, Maggiano JM, Fishman M (1977) Foveal lesions seen in retinitis pigmentosa. Arch Ophthalmol 95:1993–1996PubMedGoogle Scholar
  7. 7.
    Cox SN, Hay E, Bird AC (1988) Treatment of chronic macular edema with acetazolamide. Arch Ophthalmol 106:1190–1195PubMedGoogle Scholar
  8. 8.
    Saraiva VS, Sallum JM, Farah ME (2003) Treatment of cystoid macular edema related to retinitis pigmentosa with intravitreal triamcinolone acetonide. Ophthalmic Surg Lasers Imaging 34:398–400PubMedGoogle Scholar
  9. 9.
    García-Arumi J, Martinez V, Sararols L, Corcostegui B (2003) Vitreoretinal surgery for cystoid macular edema associated with retinitis pigmentosa. Ophthalmology 110:1164–1169PubMedCrossRefGoogle Scholar
  10. 10.
    Gragoudas ES, Adamis AP, Cunningham ET Jr, Feinsod M, Guyer DR; VEGF Inhibition Study in Ocular Neovascularization Clinical Trial Group (2004) Pegaptanib for neovascular age-related macular degeneration. N Engl J Med 351:2805–2816PubMedCrossRefGoogle Scholar
  11. 11.
    Vinores SA, Youssri AI, Luna JD, Chen YS, Bhargave S, Vinores MA, Schoenfeld CL, Peng B, Chan CC, LaRochelle W, Green WR, Campochiaro PA (1997) Upregulation of vascular endothelial growth factor in ischemic and non-ischemic human and experimental retinal disease. Histol Histopathol 12:99–109PubMedGoogle Scholar
  12. 12.
    Melo GB, Farah ME, Aggio FB (2007) Intravitreal injection of bevacizumab for cystoid macular edema in retinitis pigmentosa. Acta Ophthalmol Scand 85:461–463PubMedCrossRefGoogle Scholar
  13. 13.
    Nao-i N, Fukiyama J, Sawada A (1996) Retinitis pigmentosa with recurrent vitreous hemorrhage. Acta Ophthalmol Scand 74:509–512PubMedCrossRefGoogle Scholar
  14. 14.
    Kadayifçilar S, Eldem B, Kiratli H (2000) Retinitis pigmentosa associated with peripheral sea fan neovascularization. Acta Ophthalmol Scand 78:593–595PubMedCrossRefGoogle Scholar
  15. 15.
    Arden GB (2001) The absence of diabetic retinopathy in patients with retinitis pigmentosa: implications for pathophysiology and possible treatment. Br J Ophthalmol 85:366–370PubMedCrossRefGoogle Scholar
  16. 16.
    Lahdenranta J, Pasqualini R, Schlingemann RO, Hagedorn M, Stallcup WB, Bucana CD, Sidman RL, Arap W (2001) An anti-angiogenic state in mice and humans with retinal photoreceptor cell degeneration. Proc Natl Acad Sci USA 98:10368–10373PubMedCrossRefGoogle Scholar
  17. 17.
    Heckenlively JR, Jordan BL, Aptsiauri N (1999) Association of antiretinal antibodies and cystoid macular edema in patients with retinitis pigmentosa. Am J Ophthalmol 127:565–573PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2007

Authors and Affiliations

  • Giuseppe Querques
    • 1
  • Francesco Prascina
    • 1
  • Cristiana Iaculli
    • 1
  • Nicola Delle Noci
    • 1
  1. 1.Department of Ophthalmology, Policlinico Riuniti di FoggiaUniversity of FoggiaFoggiaItaly

Personalised recommendations