Eye

Cornea

Acanthamoeba keratitis



Last author update: 20 November 2023
Last staff update: 20 November 2023

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PubMed Search: Acanthamoeba keratitis

Purvi Patel, M.D., Ph.D.
Gabrielle Yeaney, M.D.
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Cite this page: Patel P, Yeaney G. Acanthamoeba keratitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyecorneaacanthamoeba.html. Accessed April 19th, 2024.
Definition / general
  • Rare, serious parasitic infection of the cornea occurring mostly in contact lens wearers
  • Acanthamoeba are free living, ubiquitous protozoa found in soil and freshwater (tap water, hot tubs, swimming pools)
Essential features
  • Clinically has a waxing and waning course and may be difficult to diagnose and treat
  • Direct correlation with contact lens wearing or corneal trauma with exposure to soil or contaminated water (Parasite 2015:22:10)
  • Microscopic features include ulceration, necrosis, heavy neutrophilic and macrophage infiltrates
  • Encysted forms may be highlighted with PAS or GMS stains (Hum Pathol 2013;44:918)
Terminology
  • Amebic keratitis
ICD coding
  • ICD-10
    • B60.13 - keratoconjunctivitis due to Acanthamoeba
    • H16.8 - other keratitis
Epidemiology
Sites
  • Cornea, typically unilateral but can be bilateral (Am J Ophthalmol 2008;145:193)
  • Sclera or conjunctiva
  • In prolonged cases, chorioretinitis
Pathophysiology
Etiology
Diagrams / tables

Images hosted on other servers:
Life cycle of Acanthamoeba

Life cycle of Acanthamoeba

Clinical features
Diagnosis
Laboratory
Prognostic factors
Case reports
  • 20 year old man with orthokeratology contact lenses, right eye pain and redness for 2 days and dendrite-like anterior stromal keratitis coinfected with Acanthamoeba and Pseudomonas (Taiwan J Ophthalmol 2019;9:131)
  • 26 year old woman with history of immunocompetence and contact lens use presented with severe pain, photophobia, tearing and decreased visual acuity of her left eye for 2 months (Parasitol Res 2021;120:1121)
  • 53 year old woman with contact lens use and a 2 month history of a persistent left corneal ulcer (Hum Pathol 2013;44:918)
  • 65 year old man presented with a 10 year history of bilateral uveitis, scleritis and eventual complete loss of vision and severe pain in both eyes (bilateral enucleation) (Am J Ophthalmol Case Rep 2020:20:100970)
  • 74 year old woman with history of extended soft contact lens use and intense painful ring corneal ulcer refractory to conventional antibiotics (Pathogens 2021;10:323)
  • 76 year old man with rigid gas permeable lens use in the setting of keratoconus presented after a 4 month history of corneal ulcer of the left eye (BMJ Case Rep 2021;14:e241864)
Treatment
  • Acanthamoeba trophozoite form is susceptible but the cystic form is highly drug resistant and may persist for months
  • Principal initial treatment is topical biguanide, such as polyhexamethylene biguanide (PHMB) 0.02 - 0.08% or chlorhexidine 0.02 - 0.06% (J Clin Med 2021;10:942)
  • In severe cases, therapeutic keratoplasty
Clinical images

Contributed by Gabrielle Yeaney, M.D.
AK slit lamp exam AK slit lamp exam AK slit lamp exam

Acanthamoeba keratitis on slit lamp examination

Gross description
  • Transparent to opacified / cloudy disc shaped tissue
Microscopic (histologic) description
  • Ulceration, necrosis, heavy neutrophil and macrophage infiltrates
  • May have granulomatous inflammation
  • Encysted forms
  • Typically, absence of lymphocytes (Hum Pathol 2013;44:918)
Microscopic (histologic) images

Contributed by Gabrielle Yeaney, M.D.
Stromal microabscess and necrosis

Stromal microabscess and necrosis

Multiple amoebic forms

Multiple amoebic forms

2 Acanthamoeba in stroma

2 Acanthamoeba in stroma

Ulceration and necrosis

Ulceration and necrosis


Granuloma with eosinophils

Granuloma with eosinophils

Contrast to reactive stromal keratocyte

Contrast to reactive stromal keratocyte

PAS with encysted amoebae

PAS with encysted amoebae

GMS with encysted amoebae

GMS with encysted amoebae

Cytology description
  • Impression cytology with double walled Acanthamoeba cysts and occasional trophozoites scattered among corneal epithelia (Pathogens 2021;10:323)
  • Acanthamoeba cysts identified as unstained, hollow structures on Diff-Quik stain (Diagn Cytopathol 2023;51:98)
Positive stains
  • GMS highlights walls of amoebic cysts (silver / black)
  • PAS highlights walls of amoebic cysts
  • Immunohistochemical stains for Acanthamoeba spp. exist but are not readily available in most clinical settings (Mod Pathol 2007;20:1230)
Negative stains
  • CD68 or CD163 highlight macrophages in background; stain is negative in amoebic organisms
Electron microscopy description
  • Typically for education / research rather than diagnosis: transmission electron micrograph showing an encysted Acanthamoeba organism with outer ectocyst layer, mitochondria, lipid vacuoles and lysosomes; adjacent neutrophils (Am J Ophthalmol 1987;103:626)
Videos

Clinical presentation of Acanthamoeba keratitis

Sample pathology report
  • Eye, (side) cornea, biopsy or keratoplasty:
    • Acanthamoeba keratitis (see comment)
    • Comment: The cornea is ulcerated with necrosis and neutrophils. PAS and GMS stains highlight amoebic forms. PAS also highlights the Descemet membrane.
Differential diagnosis
  • Herpetic keratitis:
    • Ulceration, may or may not have Cowdry A intranuclear inclusions in corneal epithelium at edge of ulcer (may be IHC HSV1 / HSV2 positive)
    • Lymphocytes, plasma cells, vascularization
    • Multinucleated giant cells around Descemet membrane
  • Mycotic (fungal) keratitis:
    • Granulomatous, chronic nongranulomatous or rarely purulent inflammation
    • May have associated hypopyon
    • PAS or GMS positive for yeast or hyphal forms
Board review style question #1

A 25 year old man with orthokeratology (specially fitted lenses) presents with severe pain in his left eye. The slit lamp examination shows a pattern of radial perineural and ring infiltrate in cornea. Corneal biopsy following negative direct smear examination on corneal scrapes shows focal ulceration of the surface with loss of epithelium; infiltrate of inflammatory cells, mainly polymorphonuclear cells and macrophages, are shown in the image above. What is the most likely diagnosis?

  1. Acanthamoeba keratitis
  2. Bacterial keratitis
  3. Herpetic keratitis
  4. Mycotic (fungal) keratitis
Board review style answer #1
A. Acanthamoeba keratitis. H&E sections at high power show several Acanthamoeba infiltrating corneal stroma in varying states of degeneration. Microscopic features on corneal biopsy also include ulceration, necrosis, neutrophilic and macrophage infiltrates; may show nonnecrotizing granulomatous inflammation. Encysted forms may be highlighted with PAS or GMS stains. Answer C is incorrect because a corneal biopsy for herpetic keratitis shows inflammatory infiltrates of mainly lymphocytes, plasma cells with stromal vascularization on H&E sections. Multinucleated giant cells around Descemet membrane are also identified. Answer D is incorrect because a corneal biopsy for mycotic (fungal) keratitis shows granulomatous, chronic nongranulomatous or rarely purulent inflammation on H&E sections; PAS or GMS positive for yeast or hyphal forms. Answer B is incorrect because microscopic findings of infiltration of the stroma by polymorphonuclear leukocytes occur in acute bacterial keratitis. The endothelium is often damaged and may have associated hypopyon and corneal perforation. Gram stain often shows bacteria in the stroma bordering the inflammatory infiltrate.

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Reference: Acanthamoeba keratitis
Board review style question #2

A 52 year old man who is a soft contact lens wearer presents with left eye pain and vision changes. Ophthalmic exam shows a ring shaped corneal stromal defect. Corneal biopsy reveals necrosis, neutrophilic infiltrates as shown in the image above. Which of the following is the best choice of stain to confirm the diagnosis?

  1. AFB Fite
  2. AFB Ziehl-Neelsen
  3. GMS
  4. Gram
  5. Mucicarmine
Board review style answer #2
C. GMS. GMS staining will highlight the encysted forms of amoeba in this description of Acanthamoeba keratitis. Answer A is incorrect because AFB Fite is used for Nocardia and would not stain amoebic forms. Answer B is incorrect because AFB Ziehl-Neelsen identifies acid fast organisms such as tuberculosis but would not stain amoebic forms. Answer D is incorrect because Gram stain is typically for bacterial organisms and would not be helpful in amoebic keratitis. Answer E is incorrect because mucicarmine is a stain for mucin and highlights cryptococcal forms, for example but is not helpful in this case of Acanthamoeba keratitis.

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Reference: Acanthamoeba keratitis
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