Presumed ocular histoplasmosis syndrome

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Presumed ocular histoplasmosis syndrome
Classification and external resources
File:Presumed ocular histoplasmosis syndrome-11192007.png
DiseasesDB 31279
eMedicine oph/406

Presumed ocular histoplasmosis syndrome (POHS) is a disease syndrome affecting the eye that is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the optic disc and maculopathy.

The loss of vision in POHS is caused by choroidal neovascularization.

Causes

It is thought to be caused by Histoplasma capsulatum, but the fungus has rarely been isolated from cases with POHS.

The word "presumed" is also emphasized because the condition has also been found in locations where histoplasmosis is rare.[1]

Diagnosis

Fluorescein angiography is required for diagnosis and follow-up of patients with POHS.

Treatment

Treatment requires careful consideration of FA findings and few cases may respond to corticosteroids and laser photocoagulation. A vitreo-retinal specialist should be consulted for proper management of the case.

Presumed ocular histoplasmosis syndrome and age-related macular degeneration (AMD) have been successfully treated by the drug Bevacizumab (trade name Avastin, Genentech/Roche).[2]Ophthalmologists are using Avastin "off-label" to treat AMD and similar conditions since research indicates that vascular endothelial growth factor (VEGF) is one of the causes for the growth of the abnormal vessels that cause these conditions. Some patients treated with Avastin had less fluid and more normal-appearing maculas, and their vision improved. Avastin injections into the affected eye have been used by retina specialists since early 2005. Thus there is no long term 10 to 15 year follow up data for possible late complications.[3] Early treatment is critical to maintaining vision.

Other treatments include Ranibizumab (trade name Lucentis) which is approved by the FDA for intraocular use. Lucentis uses a smaller molecule compared to Avastin and according to GenenTech, the smaller molecule helps lower the systemic toxicity of the drug, thereby lowering the overall risks compared to Avastin.[4] However, Lucentis costs approximately $1,600 per injection compared to less than $100 per injection for Avastin. Research has shown[5] that Avastin and Lucentis to be equally effective in the treatment of POHS and AMD.

External links

References

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  1. Stefan Dithmar; Frank Gerhard Holz (28 April 2008). Fluorescence Angiography in Ophthalmology. Springer. pp. 168–. ISBN 9783540783596. Retrieved 29 June 2010. 
  2. Ehrlich R, Ciulla TA, Maturi R; et al. (2009). "Intravitreal bevacizumab for choroidal neovascularization secondary to presumed ocular histoplasmosis syndrome". Retina (Philadelphia, Pa.). 29 (10): 1418–23. doi:10.1097/IAE.0b013e3181babdf1. PMID 19898179. 
  3. The Retina Group of Washington: "Informed Consent for Avastin (Bevacizumab) Intravitreal Injection, Feb. 2006.
  4. "Off-Label Avastin Treatment for Macular Degeneration", Macular Degeneration Support Canada. Published April 18, 2008; http://www.amdsupport.ca/2008/04/18/off-label-avastin-treatment-for-macular-degeneration/
  5. "Lucentis vs Avastin: Which Drug is Better?", Macular Degeneration Support Canada, Published August 10, 2009, http://www.amdsupport.ca/2009/08/10/lucentis-vs-avastin-which-drug-is-better/