Presumed Ocular Histoplasmosis Syndrome in a Five-Year-Old Girl in a Non-Endemic Area

Background

Presumed ocular histoplasmosis syndrome (POHS) is a noninflammatory chorioretinal disorder with a distinct fundus appearance that consists of

1. circumferential peripapillary atrophy (PPA), usually pigmented,

2. bilateral mid-peripheral disseminated “punched-out” chorioretinal scars, called “histo spots”, and

3. the possible development of secondary choroidal neovascularization (CNV) or corresponding sequelae such as disciform scars [1].

There is no consensus on its pathogenesis. POHS is commonly found in regions endemic to the inhalable dimorphic fungus Histoplasma capsulatum (HC), i.e., the Midwest and Southeast of the USA (Mississippi and Ohio River valley regions). Although histoplasmosis is the most endemic mycosis in the world, POHS has only been reported in a few countries outside the USA, such as Mexico, India, the United Kingdom, and the Netherlands. Its incidence is largely unknown, and males and females are equally affected [2].

The primary infection presumably occurs many years before the development of symptoms and the diagnosis is usually made during midlife when the disease becomes symptomatic due to macular CNV. POHS is therefore considered one of the principal causes of central vision loss among young adults in endemic areas, secondary to subfoveal CNVs, resulting in significant visual impairment (≤ 0.1 of visual acuity) [2], [3].

Publication History

Received: 16 October 2022

Accepted: 28 December 2022

Article published online:
25 April 2023

• References

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