Presumed Ocular Histoplasmosis Syndrome Treatment & Management

  • Author: Lihteh Wu, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 30, 2012
 

Medical Care

  • Corticosteroids
    • A few anecdotal cases of oral steroids inducing involution of recent-onset subfoveal CNV have been reported.
    • A small series reported on the benefits of an intravitreal injection of 4 mg of triamcinolone acetonide in eyes with subfoveal or juxtafoveal CNV secondary to POHS.[16]
    • Sustained-release steroid implants have been used on a compassionate basis in refractory patients with stabilization or improvement of vision in 6 of 7 cases. However, concomitant submacular surgery was performed in 4 cases.[17]
  • Antifungals are not beneficial.
  • Anti-VEGF
    • Vascular endothelial growth factor (VEGF) has been shown to be a key molecular player in the pathogenesis of CNV. In the current era of anti-VEGF therapy, the extraordinary results obtained in CNV secondary to age-related macular degeneration have been extrapolated to other causes of CNV with apparent good results.[18, 19] Currently available anti-VEGF agents include bevacizumab, ranibizumab, and pegaptanib sodium.
    • In a small retrospective case series of 28 eyes with a relatively short follow-up of 22 weeks, intravitreal bevacizumab was shown to improve the visual acuity in 71% of eyes. In 14%, the visual acuity remained the same, and, in another 14%, the visual acuity decreased despite treatment.[19]
    • A large retrospective comparative case series compared intravitreal bevacizumab (117 eyes) with combination therapy consisting of intravitreal bevacizumab and verteporfin photodynamic therapy (34 eyes). At the end of 2 years of follow up, no difference in visual outcome was noted between the treatment strategies. Approximately 30% of eyes gained at least 3 lines of best-corrected visual acuity over baseline. Subgroup analysis revealed that in juxtafoveal CNV, no difference in the burden of injections was noted between the groups. However, in the subfoveal group, there were statistically significantly fewer injections in the combination group.[20]
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Surgical Care

  • Laser photocoagulation
    • The Macular Photocoagulation Study (MPS), a multicenter prospective randomized clinical trial, demonstrated that laser photocoagulation is indicated in the treatment of extrafoveal and juxtafoveal CNV secondary to POHS.[21]
    • The goal of treatment is to obliterate the entire area of CNV.
    • Prior to laser treatment, a fluorescein angiogram that shows the exact borders of the lesion is essential.
    • Despite its marginal benefits, the MPS recommended laser treatment of peripapillary CNV. Alternatively, pars plana vitrectomy and excision of the peripapillary CNV may be considered. Most surgeons recommend removal of recent subfoveal CNV but not peripapillary lesions.
    • Pilot studies of laser photocoagulation of subfoveal CNV were inconclusive.
  • Photodynamic therapy (PDT): Subfoveal CNV secondary to POHS is a labeled indication for PDT by the US Food and Drug Administration. An open-label, uncontrolled clinical study reported the median improvement of visual acuity of 6 letters after a mean of 3.9 PDT treatments in a 2-year follow-up with no serious ocular or systemic effects reported.[22]
  • Submacular surgery
    • Given that most CNV secondary to POHS grow in the subretinal space, uncontrolled studies have recommended surgical excision of subfoveal CNV via pars plana vitrectomy. The goal is to remove the CNV but to leave the underlying RPE and choriocapillaris intact.
    • The Submacular Surgery Trial (SST), a randomized multicenter prospective trial sponsored by the National Eye Institute (NEI), reported on the modest benefit in eyes with CNV secondary to POHS with a baseline visual acuity of 20/100 or worse.[23]
    • A case series of 45 eyes with extensive peripapillary CNV that were ineligible for laser photocoagulation by the Macular Photocoagulation Study criteria underwent surgical removal. Of these 45 eyes, in 23 eyes the CNV extended subfoveally. In this subgroup, the median visual acuity improved from 20/200 to 20/50. Almost 80% (18/23) of eyes achieved stable or improved visual acuity from baseline. Only 22% (5/23) of eyes experienced a loss of more than 2 lines of visual acuity from baseline. Close to 50% (11/23) of eyes achieved a visual acuity of ≥ 20/40. In the remaining 17 eyes where CNV remained extrafoveal, 88% (15/17) of eyes had an improvement or stability in visual acuity. Only 12% (2/17) of eyes showed a loss of ≥ 2 lines of visual acuity. The median visual acuity improved from 20/60 to 20/20. This suggests that surgical extraction in selected cases of extensive peripapillary CNV secondary to POHS might be beneficial.[24]
  • Macular translocation: Macular translocation surgery is another experimental surgical option to treat subfoveal CNV.
  • Photocoagulating atrophic scars to prevent CNV formation is not recommended.
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Consultations

  • Refer to a vitreoretinal specialist.
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Contributor Information and Disclosures
Author

Lihteh Wu, MD  Consulting Surgeon, Department of Ophthalmology, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica

Lihteh Wu, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Pan-American Association of Ophthalmology, and Retina Society

Disclosure: Nothing to disclose.

Coauthor(s)

Teodoro Evans, MD  Retina Fellow, St Michael's Hospital, University of Toronto, Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Russell P Jayne, MD  Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas

Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Woods AC, Wahlen HE. The probable role of benign histoplasmosis in the etiology of granulomatous uveitis. Am J Ophthalmol. Feb 1960;49:205-20. [Medline].

  2. Rhee P, Dev S, Mieler WF. The development of choroidal neovascularization in pregnancy. Retina. 1999;19(6):520-4. [Medline].

  3. Comstock GW, Vicens CN, Goodman NL, Collins S. Differences in the distribution of sensitivity to histoplasmin and isolations of Histoplasma capsulatum. Am J Epidemiol. Sep 1968;88(2):195-209. [Medline].

  4. Ganley JP. Epidemiologic characteristics of presumed ocular histoplasmosis. Acta Ophthalmol Suppl. 1973;119:1-63. [Medline].

  5. Asbury T. The status of presumed ocular histoplasmosis: including a report of a survey. Trans Am Ophthalmol Soc. 1966;64:371-400. [Medline].

  6. Schlaegel TF Jr, Weber JC, Helveston E, Kenney D. Presumed histoplasmic choroiditis. Am J Ophthalmol. May 1967;63(5):919-25. [Medline].

  7. Hawkins BS, Ganley JP. Risk of visual impairment attributable to ocular histoplasmosis. Washington County Follow-up Eye Study Group. Arch Ophthalmol. May 1994;112(5):655-66. [Medline].

  8. Watzke RC, Klein ML, Wener MH. Histoplasmosis-like choroiditis in a nonendemic area: the northwest United States. Retina. 1998;18(3):204-12. [Medline].

  9. Goswami RP, Pramanik N, Banerjee D, Raza MM, Guha SK, Maiti PK. Histoplasmosis in eastern India: the tip of the iceberg?Trans R Soc Trop Med Hyg. 1999;93:540-542.

  10. Sinha R, Raju S, Garg SP, Venkatesh P, Talwar D. Presumed ocular histoplasmosis syndrome in India. Ocul Immunol Inflamm. Jul-Aug 2007;15(4):315-7. [Medline].

  11. Suttorp-Schulten MS, Bollemeijer JG, Bos PJ, Rothova A. Presumed ocular histoplasmosis in The Netherlands--an area without histoplasmosis. Br J Ophthalmol. Jan 1997;81(1):7-11. [Medline].

  12. Amaro MH, Muccioli C, Abreu MT. Ocular histoplasmosis-like syndrome: a report from a nonendemic area. Arq Bras Oftalmol. Jul-Aug 2007;70(4):577-80. [Medline].

  13. Smith RE, Ganley JP. An epidemiologic study of presumed ocular histoplasmosis. Trans Am Acad Ophthalmol Otolaryngol. Sep-Oct 1971;75(5):994-1005. [Medline].

  14. Chheda LV, Ferketich AK, Carroll CP, et al. Smoking as a risk factor for choroidal neovascularization secondary to presumed ocular histoplasmosis syndrome. Ophthalmology. Feb 2012;119(2):333-8. [Medline].

  15. Hatef E, Turkcuoglu P, Ibrahim M, et al. Importance of proper diagnosis for management: multifocal choroiditis mimicking ocular histoplasmosis syndrome. J Ophthalmic Inflamm Infect. Jun 2011;1(2):55-63. [Medline]. [Full Text].

  16. Rechtman E, Allen VD, Danis RP, Pratt LM, Harris A, Speicher MA. Intravitreal triamcinolone for choroidal neovascularization in ocular histoplasmosis syndrome. Am J Ophthalmol. Oct 2003;136(4):739-41. [Medline].

  17. Holekamp NM, Thomas MA, Pearson A. The safety profile of long-term, high-dose intraocular corticosteroid delivery. Am J Ophthalmol. Mar 2005;139(3):421-8. [Medline].

  18. Adan A, Navarro M, Casaroli-Marano RP, Ortiz S, Molina JJ. Intravitreal bevacizumab as initial treatment for choroidal neovascularization associated with presumed ocular histoplasmosis syndrome. Graefes Arch Clin Exp Ophthalmol. Dec 2007;245(12):1873-5. [Medline].

  19. Schadlu R, Blinder KJ, Shah GK, et al. Intravitreal bevacizumab for choroidal neovascularization in ocular histoplasmosis. Am J Ophthalmol. May 2008;145(5):875-8. [Medline].

  20. Cionni DA, Lewis SA, Petersen MR, et al. Analysis of outcomes for intravitreal bevacizumab in the treatment of choroidal neovascularization secondary to ocular histoplasmosis. Ophthalmology. Feb 2012;119(2):327-32. [Medline].

  21. Macular Photocoagulation Study Group. Five-year follow-up of fellow eyes of individuals with ocular histoplasmosis and unilateral extrafoveal or juxtafoveal choroidal neovascularization. Macular Photocoagulation Study Group. Arch Ophthalmol. Jun 1996;114(6):677-88. [Medline].

  22. Rosenfeld PJ, Saperstein DA, Bressler NM, et al. Photodynamic therapy with verteporfin in ocular histoplasmosis: uncontrolled, open-label 2-year study. Ophthalmology. Sep 2004;111(9):1725-33. [Medline].

  23. Hawkins BS, Bressler NM, Bressler SB, et al. Surgical removal vs observation for subfoveal choroidal neovascularization, either associated with the ocular histoplasmosis syndrome or idiopathic: I. Ophthalmic findings from a randomized clinical trial: Submacular Surgery Trials (SST) Group H Trial: SST Report No. 9. Arch Ophthalmol. Nov 2004;122(11):1597-611. [Medline].

  24. Almony A, Thomas MA, Atebara NH, Holekamp NM, Del Priore LV. Long-term follow-up of surgical removal of extensive peripapillary choroidal neovascularization in presumed ocular histoplasmosis syndrome. Ophthalmology. 2008;115(3):540-545.e5.

  25. Macular Photocoagulation Study Group. Argon laser photocoagulation for neovascular maculopathy. Five-year results from randomized clinical trials. Macular Photocoagulation Study Group. Arch Ophthalmol. Aug 1991;109(8):1109-14. [Medline].

  26. Macular Photocoagulation Study Group. Laser photocoagulation for juxtafoveal choroidal neovascularization. Five-year results from randomized clinical trials. Macular Photocoagulation Study Group. Arch Ophthalmol. Apr 1994;112(4):500-9. [Medline].

  27. Fine SL, Wood WJ, Isernhagen RD, et al. Laser treatment for subfoveal neovascular membranes in ocular histoplasmosis syndrome: results of a pilot randomized clinical trial. Arch Ophthalmol. Jan 1993;111(1):19-20. [Medline].

  28. Macular Photocoagulation Study Group. Laser photocoagulation for neovascular lesions nasal to the fovea. Results from clinical trials for lesions secondary to ocular histoplasmosis or idiopathic causes. Macular Photocoagulation Study Group. Arch Ophthalmol. Jan 1995;113(1):56-61. [Medline].

  29. Alam S, Zawadzki RJ, Choi S, et al. Clinical application of rapid serial fourier-domain optical coherence tomography for macular imaging. Ophthalmology. Aug 2006;113(8):1425-31. [Medline].

  30. Atebara NH, Thomas MA, Holekamp NM, Mandell BA, Del Priore LV. Surgical removal of extensive peripapillary choroidal neovascularization associated with presumed ocular histoplasmosis syndrome. Ophthalmology. Sep 1998;105(9):1598-605. [Medline].

  31. Baskin MA, Jampol LM, Huamonte FU, Rabb MF, Vygantas CM, Wyhinny G. Macular lesions in blacks with the presumed ocular histoplasmosis syndrome. Am J Ophthalmol. Jan 1980;89(1):77-83. [Medline].

  32. Berger AS, Conway M, Del Priore LV, Walker RS, Pollack JS, Kaplan HJ. Submacular surgery for subfoveal choroidal neovascular membranes in patients with presumed ocular histoplasmosis. Arch Ophthalmol. Aug 1997;115(8):991-6. [Medline].

  33. Callanan D, Fish GE, Anand R. Reactivation of inflammatory lesions in ocular histoplasmosis. Arch Ophthalmol. Apr 1998;116(4):470-4. [Medline].

  34. Dawson DW, Volpert OV, Gillis P, et al. Pigment epithelium-derived factor: a potent inhibitor of angiogenesis. Science. Jul 9 1999;285(5425):245-8. [Medline].

  35. Essex RW, Tufail A, Bunce C, Aylward GW. Two-year results of surgical removal of choroidal neovascular membranes related to non-age-related macular degeneration. Br J Ophthalmol. May 2007;91(5):649-54. [Medline].

  36. Gass JD. Stereoscopic Atlas of Macular Diseases. In: Diagnosis and Treatment. 4th ed. St Louis: Mosby Year Book Inc; 1997:130-147.

  37. Godfrey WA, Sabates R, Cross DE. Association of presumed ocular histoplasmosis with HLA-B7. Am J Ophthalmol. Jun 1978;85(6):854-8. [Medline].

  38. Grossniklaus HE, Green WR. Histopathologic and ultrastructural findings of surgically excised choroidal neovascularization. Submacular Surgery Trials Research Group. Arch Ophthalmol. Jun 1998;116(6):745-9. [Medline].

  39. Holekamp NM, Thomas MA, Dickinson JD, Valluri S. Surgical removal of subfoveal choroidal neovascularization in presumed ocular histoplasmosis: stability of early visual results. Ophthalmology. Jan 1997;104(1):22-6. [Medline].

  40. Khalil MK. Histopathology of presumed ocular histoplasmosis. Am J Ophthalmol. Sep 1982;94(3):369-76. [Medline].

  41. Melberg NS, Thomas MA, Dickinson JD, Valluri S. Managing recurrent neovascularization after subfoveal surgery in presumed ocular histoplasmosis syndrome. Ophthalmology. Jul 1996;103(7):1064-7;discussion 1067-8. [Medline].

  42. Oliver A, Ciulla TA, Comer GM. New and classic insights into presumed ocular histoplasmosis syndrome and its treatment. Curr Opin Ophthalmol. Jun 2005;16(3):160-5. [Medline].

  43. Ongkosuwito JV, Kortbeek LM, Van der Lelij A, et al. Aetiological study of the presumed ocular histoplasmosis syndrome in the Netherlands. Br J Ophthalmol. May 1999;83(5):535-9. [Medline].

  44. Prasad AG, Van Gelder RN. Presumed ocular histoplasmosis syndrome. Curr Opin Ophthalmol. Dec 2005;16(6):364-8. [Medline].

  45. Shah GK, Blinder KJ, Hariprasad SM, et al. Photodynamic therapy for juxtafoveal choroidal neovascularization due to ocular histoplasmosis syndrome. Retina. Jan 2005;25(1):26-32. [Medline].

  46. Smith RE, Dunn S, Jester JV. Natural history of experimental histoplasmic choroiditis in the primate. I. Clinical features. Invest Ophthalmol Vis Sci. Jul 1984;25(7):801-9. [Medline].

 
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