eMedicine Specialties > Ophthalmology > Retina

Presumed Ocular Histoplasmosis Syndrome: Differential Diagnoses & Workup

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

Differential Diagnoses

ARMD, Exudative
Toxoplasmosis

Other Problems to Be Considered

Birdshot chorioretinopathy
Punctate inner choroidopathy
Coccidioidomycosis
Multifocal choroiditis
Idiopathic choroidal neovascular membranes

Workup

Laboratory Studies

  • Although the diagnosis is clinical, certain ancillary tests help in confirming it. Other than fluorescein angiography (FA), most patients do not require ancillary testing.
  • HLA typing B7 and DRw2 may be indicated.
  • Complement fixing antibodies are only positive in 16-68% of patients with POHS.
  • Lymphocyte stimulation assay by Histoplasma antigens may be indicated.
  • Focal calcifications in the liver or the spleen may be present.

Imaging Studies

  • FA is essential in diagnosing and managing the maculopathy associated with POHS.

    • The typical angiographic pattern is early hyperfluorescence with late leakage.
    • According to its location relative to the center of the fovea, CNV has been classified as extrafoveal (200-1500 µm), juxtafoveal (1-199 µm), and subfoveal (under the center of the fovea).
  • Chest x-ray film findings usually reveal calcifications of the hilar areas.

Other Tests

  • Skin testing should not be performed on patients with a maculopathy; some report that it may exacerbate this condition.

Histologic Findings

Few cases report H capsulatum isolated from the human eye. In most cases, isolation of the organism in either atrophic scars or CNV is not possible. Histologically, the peripapillary and peripheral spots are seen as areas where there is partial loss of the RPE and the photoreceptor cell layer. The Bruch membrane often presents with focal breaks in it. Sometimes, the overlying inner retina shows cystic degeneration. These areas often are surrounded by a lymphocytic choroidal infiltrate.

In the macular area, most CNV develops adjacent to an atrophic histo spot, although de novo neovascularization can occur. The new capillaries and fibroblasts originate from the choroid and grow through a defect in the Bruch membrane into the subretinal space, not the sub-RPE space (type 2 CNV). Reactive hyperplastic RPE is present at the advancing edge of CNV.

Specimens obtained during surgical excision of CNV reveal that the most common cellular components are vascular endothelium and RPE; they were present in more than 85% of samples. Fibrocytes and macrophages have been identified in more than 50% of specimens. Extracellular components include collagen and fibrin.

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

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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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