Presumed ocular histoplasmosis syndrome (POHS) is a syndrome affecting the eye, which is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the

optic disc The optic disc or optic nerve head is the point of exit for ganglion cell axons leaving the eye. Because there are no rods or cones overlying the optic disc, it corresponds to a small blind spot in each eye. The ganglion cell axons form t ...

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

Presentation

The diagnosis of POHS is based on the clinical triad of multiple white, atrophic choroidal scars, peripapillary pigment changes (dark spots around optic disc of the eye), and a maculopathy caused by choroidal neovascularization. Completely distinct from POHS, acute ocular

histoplasmosis Histoplasmosis is a fungal infection caused by ''Histoplasma capsulatum''. Symptoms of this infection vary greatly, but the disease affects primarily the lungs. Occasionally, other organs are affected; called disseminated histoplasmosis, it can ...

may rarely occur in

immunodeficiency Immunodeficiency, also known as immunocompromise, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that aff ...

Causes

Despite its name, the "presumed" relationship of POHS to ''

Histoplasma capsulatum ''Histoplasma capsulatum'' is a species of dimorphic fungus. Its sexual form is called ''Ajellomyces capsulatus''. It can cause pulmonary and disseminated histoplasmosis. ''Histoplasma capsulatum'' is "distributed worldwide, except in Antarc ...

'' is controversial and has been questioned by a number of medical professionals. The fungus has rarely been isolated from cases with POHS,
Presumed Ocular Histoplasmosis Syndrome the condition has also been found in locations where

is rare, and there appears to be a relationship with

tobacco smoking Tobacco smoking is the practice of burning tobacco and ingesting the resulting smoke. The smoke may be inhaled, as is done with cigarettes, or released from the mouth, as is generally done with pipes and cigars. The practice is believed to hav ...

Diagnosis

Fluorescein angiography Fluorescein angiography (FA), fluorescent angiography (FAG), or fundus fluorescein angiography (FFA) is a technique for examining the circulation of the retina and choroid (parts of the fundus) using a fluorescent dye and a specialized camera. ...

is usually performed for diagnosis and follow-up of patients with POHS.

Treatment

Treatment requires careful consideration of angiographic findings when a choroidal neovascular membrane is suspected which is a condition that responds to treatment. A vitreo-retinal specialist (an ophthalmologist specialized in treatment of retinal diseases) should be consulted for proper management of the case. Presumed ocular histoplasmosis syndrome and

age-related macular degeneration Macular degeneration, also known as age-related macular degeneration (AMD or ARMD), is a medical condition which may result in blurred or no vision in the center of the visual field. Early on there are often no symptoms. Some people experien ...

(AMD) have been successfully treated with laser, anti-vascular endothelial growth factors and

photodynamic therapy Photodynamic therapy (PDT) is a form of phototherapy involving light and a photosensitizing chemical substance used in conjunction with molecular oxygen to elicit cell death ( phototoxicity). PDT is used in treating acne, wet age-related macula ...

. Ophthalmologists are using anti-vascular endothelial growth factors to treat AMD and similar conditions since research indicates that

vascular endothelial growth factor Vascular endothelial growth factor (VEGF, ), originally known as vascular permeability factor (VPF), is a signal protein produced by many cells that stimulates the formation of blood vessels. To be specific, VEGF is a sub-family of growth factors ...

(VEGF) is one of the causes for the growth of the abnormal vessels that cause these conditions.

References

External links