2013 - 1 Issue

Case report

Retinitis Pigmentosa Mimicking Uveitis. A Case Report

Full Text links

Summary

Purpose: To describe a case report of a 23-year-old patient with retinitis pigmentosa (RP) misdiagnosed as uveitis.

Methods: A comprehensive eye examination including automated visual field assessment, contrast sensitivity, colour vision discrimination, ultrasound examination (US), spectral domain optical coherence tomography (SD-OCT) and full-field electroretinography (ERG) was performed in a patient diagnosed elsewhere as having intermediate uveitis because of the observation of a cellular reaction in the anterior chamber, bilateral cystoid macular oedema and suspected left optic disc swelling.

Results: The patient reported nyctalopia. The best corrected visual acuity in both eyes was 6/12. Concentric visual field constriction was detected bilaterally (less than 25 degrees in the right eye and 15 degrees in the left eye). Fundus examination revealed a few pigment clumps and cystoid macular edema in both eyes confirmed by SD-OCT. Contrast sensitivity was decreased to 1,20 in the right and 0,9 in the left. No colour vision disturbance was present. The B scan ultrasound showed left optic disc drusen. Rod ERG responses were bilaterally not detectable and cone ERGs were abnormally reduced. Based on the examination results, a diagnosis of nonsyndromic RP was made.

Conclusion: Clinicians should be aware of various manifestations of RP, including mild inflammation, to avoid possible confusin with uveitis.

References

  1. Appolloni, R., Modesti, M., Pecorella, I. et al.: Uncommon cause of juvenile cataract: adenoma of the nonpigmented ciliary epithelium. J Cataract Refract Surg, 34; 2008, 11:1997–2001.
  2. Biswas, J., Neelakantan, A., Rao, B.S.: Adenoma of nonpigmented epithelium of the ciliary body presenting as anterior uveitis and glaucoma: a case report. Indian J Ophthalmol, 43; 1995, 3: 137–140.
  3. Boudet, C., Maisongrosse, G., Navarre, L. et al.: Adenome du corps ciliare chez l’enfant (A propos d’1 cas). [Adenoma of the ciliary body in the child (apropos of 1 case).] Bull Soc Ophtalmol Fr, 79; 1979: 969–972.
  4. Cursiefen, C., Schlotzer-Schrehardt, U., Holbach, L.M. et al.: Adenoma of the nonpigmented ciliary epithelium mimicking a malignant melanoma of the iris. Arch Ophthalmol, 117; 1999, 1: 113–116.
  5. Doro, S., Werblin, T.P., Haas, B. et al.: Fetal adenoma of the pigmented ciliary epithelium associated with persistent hyperplastic primary vitreous. Ophthalmology, 93; 1986: 1343–1350.
  6. Elizalde, J., Ubia, S., Barraquer, R.I.: Adenoma of the nonpigmented ciliary epithelium. Eur J Ophthalmol, 16; 2006, 4: 630–633.
  7. Grossniklaus, H.E., Lim, J.I.: Adenoma of the nonpigmented ciliary epithelium. Retina, 14; 1994, 5: 452–456.
  8. Chen, Z.Q., Fang, X.Y.: Case Report: Adenoma of nonpigmented epithelium in ciliary body: literature review and case report. J Zhejiang Univ Sci B, 8; 2007, 9: 612–615.
  9. Mansoor, S., Qureshi, A.: Ciliary body adenoma of nonpigmented epithelium. J Clin Pathol, 57; 2004, 9:997–998.
  10. Nakazawa, T., Abe, T., Sato, Y. et al.: Magnetic resonance imaging of a nonpigmented adenoma of the ciliary epithelium. Acta Ophthalmol Scand, 78; 2000, 4: 470–473.
  11. Papastefanou, V.P., Cohen, V.M.L.: Ciliary body adenoma of the non-pigmented epithelium with rubeosis iridis treated with plaque brachytherapy and bevacizumab. Eye, 26; 2012, 10: 1388–390.
  12. Patrinely, J.R., Font, R.L., Campbell, R.J. et al.: Hamartomatous adenoma of the nonpigmented ciliary epithelium arising in iris-ciliary body coloboma. Light and electron microscopic observations. Ophthalmology, 90; 1983, 12: 1540–7.
  13. Pecorella, I., Ciocci, L., Modesti, M. et al.: Adenoma of the non-pigmented ciliary epithelium: a rare intraocular tumor with unusual immunohistochemical findings. 2009, 12: 870–5.
  14. Rennie, I.G., Parsons, M.A., Palmer, C.A.: Congenital adenoma of the iris and ciliary body: light and electron microscopic observations. Br J Ophthalmol, 76; 1992, 9:563–566.
  15. Romanowska-Dixon, B., OrΠowska- Heitzman, J.: Adenoma of the Iris and Ciliary Body. Case Report. Pol J Pathol, 54; 2003, 3: 187–190.
  16. Shields, J.A., Augsburger, J.J., Wallar, P.H. et al.: Adenoma of the nonpigmented epithelium of the ciliary body. Ophthalmology, 90; 1983, 12: 1528–1530.
  17. Shields, J.A., Eagle, R.C.Jr., Shields, C.L. et al.: Acquired neoplasms of the nonpigmented ciliary epithelium (adenoma and adenocarcinoma). Ophthalmology, 103; 1996, 12: 2007–2016.
  18. Shields, J.A., Eagle, R.C.Jr., Shields, C.L.: Adenoma of nonpigmented ciliary epithelium with smooth muscle differentiation. Arch Ophthalmol, 117; 1999, 1:117–9.
  19. Suzuki, J., Goto, H., Usui, M.: Adenoma arising from nonpigmented ciliary epithelium concomitant with neovascularization of the optic disk and cystoid macular edema. Am J Ophthalmol, 139; 2005, 1: 188–190.
  20. Xian, J., Xu, Q., Wang, Z. et al.: MR imaging of adenomas of the nonpigmented ciliary epithelium of the eye. Am J Neuroradiol, 31; 2010, 5: 886–90.