eMedicine Specialties > Ophthalmology > Retina

Presumed Ocular Histoplasmosis Syndrome: Treatment & Medication

Author: Lihteh Wu, MD, Consulting Surgeon, Department of Ophthalmology, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Coauthor(s): Teodoro Evans, MD, Retina Fellow, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Contributor Information and Disclosures

Updated: Jul 24, 2007

Treatment

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.
    • 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.
  • Antifungals are not beneficial.

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.
    • 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.
  • 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), recently reported on the modest benefit in eyes with CNV secondary to POHS with a baseline visual acuity of 20/100 or worse.
  • Macular translocation: Macular translocation surgery is another experimental surgical option to treat subfoveal CNV.
  • Photocoagulating atrophic scars to prevent CNV formation is not recommended.

Consultations

  • Refer to a vitreoretinal specialist.

Medication

Antifungals, such as amphotericin B, are not helpful. Steroids anecdotally have been used in subfoveal CNV by a few observers.

Corticosteroids

Anecdotal, controversial evidence suggests efficacy in treating subfoveal CNV.


Prednisone (Deltasone, Orasone)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Adult

Gass recommends 40-100 mg qd for several wk, then alternate-day basis for several wk; if no response, rapidly taper and stop

Pediatric

Not established

Drugs, such as phenobarbital, phenytoin, and rifampin, induce hepatic enzymes and, therefore, increase clearance of corticosteroids; other drugs, such as ketoconazole, inhibit clearance of corticosteroids; adjust doses accordingly; corticosteroids have an unpredictable effect on anticoagulants, so monitor coagulation indices

Documented hypersensitivity; systemic fungal infections

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Secondary adrenocortical insufficiency may be induced; in periods of stress, an increased dosage is indicated; hold immunizations while the patient takes corticosteroids


Triamcinolone acetonide (Kenalog)

Off-label use of triamcinolone.

Adult

Not established, but most vitreoretinal specialists inject 4-25 mg intravitreally

Pediatric

Not established

Coadministration with barbiturates, phenytoin, and rifampin decreases effects

Documented hypersensitivity; fungal, viral, and bacterial skin infections

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Use sterile technique when injecting triamcinolone into the vitreous cavity to avoid endophthalmitis; infectious and noninfectious endophthalmitis have been reported following intravitreal injection of triamcinolone; elevation of intraocular pressure has been reported to occur in up to 33% of eyes; retinal detachment may occur; long-term use may lead to progression of lens opacities

Photosensitizers for photodynamic therapy

Reduction of leakage from abnormal, neovascular vessels, resulting in reduced visual loss.


Verteporfin (Visudyne)

A benzoporphyrin derivative monoacid (BPD-MA), consists of equally active isomers BPD-MAC and BPD-MAD, which can be activated by low-intensity, nonthermal light of 689-nm wavelength. After activation with light and in presence of oxygen, verteporfin forms cytotoxic oxygen free radicals and singlet oxygen. Singlet oxygen causes damage to biological structures within range of diffusion. This leads to local vascular occlusion, cell damage, and cell death. In plasma, verteporfin is transported primarily by low-density lipoproteins (LDL). Tumor and neovascular endothelial cells have increased specificity and uptake of verteporfin because of their high expression of LDL receptors. Effect can be enhanced by use of liposomal formulation.

Adult

6 mg/m2 (dissolved in 30 mL of solution) IV for 10 min
Second part of treatment consists of activation of drug: Recommended light intensity of 600 mW/cm2, takes 83 s to apply necessary light dose of 50 J/cm2

Pediatric

Not established

None reported; many drugs can influence effect; theoretical examples include concomitant use of other photosensitizer (eg, tetracycline, sulphonamide, phenothiazine, sulphonylurea, hypoglycemic substances, thiazide diuretics, griseofulvin) could increase photosensitivity; compounds that scavenge active oxygen species or radicals (eg, dimethylsulphoxide, beta-carotene, ethanol, formate, mannitol) could reduce activity; calcium channel blockers, polymyxin B, or radiation therapy can increase rate of uptake by vascular endothelium; anticoagulants, vasoconstrictors, or platelet-aggregation inhibitors (eg, thromboxane-A2 inhibitors) can reduce effectiveness

Documented hypersensitivity; porphyria

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Patients remain photosensitive to sunlight and strong artificial light for 48 h after infusion with verteporfin; wearing sunglasses and long-sleeved clothing highly recommended to avoid serious skin and eye burns; indoor lighting is safe in general and recommended over complete darkness because accelerates breakdown of active drug; caution in advanced liver disease; extravasation can cause severe pain, inflammation, swelling, and discoloration at injection site; cold compresses and analgesia help reduce pain and complications of extravasation

More on Presumed Ocular Histoplasmosis Syndrome

Overview: Presumed Ocular Histoplasmosis Syndrome
Differential Diagnoses & Workup: Presumed Ocular Histoplasmosis Syndrome
Treatment & Medication: Presumed Ocular Histoplasmosis Syndrome
Follow-up: Presumed Ocular Histoplasmosis Syndrome
References
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References

  1. Alam S, Zawadzki RJ, Choi S, Gerth C, Park SS, Morse L, et al. Clinical application of rapid serial fourier-domain optical coherence tomography for macular imaging. Ophthalmology. Aug 2006;113(8):1425-31. Epub 2006 Jun 12. [Medline].

  2. 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].

  3. 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].

  4. 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].

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

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

  7. Fine SL, Wood WJ, Isernhagen RD, Singerman LJ, Bressler NM, Folk JC, 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].

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

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

  10. 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].

  11. Hawkins BS, Bressler NM, Bressler SB, Davidorf FH, Hoskins JC, Marsh MJ, 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].

  12. 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].

  13. 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].

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

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

  16. 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].

  17. 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].

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

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

  20. 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].

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

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

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

  24. 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].

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

  26. 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].

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

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

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Further Reading

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Keywords

POHS, ocular histoplasmosis, peripheral atrophic chorioretinal scars, peripapillary scarring, maculopathy, Histoplasma capsulatum, H capsulatum, histoplasmin skin testing, fungal infection, macular choroidal neovascularization, macular CNV, vision loss

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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, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

Coauthor(s)

Teodoro Evans, MD, Retina Fellow, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting

CME Editor

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.

 
 
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