Clinical Course of Unilateral Acanthamoeba Keratitis in a Cosmetic Contact Lens Wearer

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Available online at www.annclinlabsci.org 366 Annals of Clinical & Laboratory Science, vol. 45, no. 3, 2015 Clinical Course of Unilateral Acanthamoeba Keratitis in a Cosmetic Contact Lens Wearer

Raavi Gupta1, Matthew Gorski2, Triona Henderson1, Douglas Lazzaro2, and M. A. Haseeb1

1Department of Pathology and 2Department of Ophthalmology, Downstate Medical Center, State University of New York, and Kings County Hospital Center, Brooklyn, New York, USA

Abstract. Purpose. To report the features and clinical course of Acanthamoeba keratitis in a cosmetic contact lens wearer. Case Report. A 29-year-old man sought medical attention for severe ocular pain, blurry vision, photophobia, and a foreign body sensation in the left eye for the past 3-4 days. He had been wearing a single sapphire cosmetic soft contact lens for 1-2 months. The left upper eyelid was edematous and the conjunctiva was hyperemic; the best corrected distance visual acuity was 20/400. A slit lamp examination revealed circular and perineural linear stromal opacities and diffuse keratic precipitates. A clinical diagnosis of herpes simplex keratitis was made and the patient was started on antiviral therapy. After initial improvement, the patient returned with worsening pain and vision; the corneal lesions had exacerbated. Unresponsiveness to antiviral therapy prompted examination of corneal scrapings, which revealed Acanthamoeba developmental forms. Antimicrobial therapy was prescribed resulting in relief of symptoms despite corneal opacities and poor vision. Conclusion. This case illustrates the importance of considering Acanthamoeba keratitis in contact lens wearers at presentation, particularly in those with symptomatic keratitis unresponsive to antiviral and antifungal therapy.

Key words: Keratitis, Acanthamoeba keratitis, protozoa, contact lens, cornea

Introduction

Acanthamoeba keratitis (AK) is a severe sight- The incidence of AK is on the increase in industrial- threatening ocular condition caused by a free-living ized countries; among the contact lens wearers it opportunistically pathogenic amoeba [1]. Both the varies from 1.65-2.01 cases per million in the trophozoite and cyst forms of Acanthamoeba are United States to 17.53-19.50 per million in the ubiquitous in the environment and have been iso- United Kingdom [3,5]. The increased incidence is lated from various fresh surface water sources, in- most likely attributable to increase in the number cluding tap and recreational water, and ventilation of contact lens wearers, greater awareness among systems. However, contact lenses and their cases health care providers, and better diagnostics [4-6]. and cleaning solutions are the most likely sources of The clinical diagnosis of AK is challenging because human infection. AK has been diagnosed in a mi- of the non-specific nature of signs and symptoms; nority (3-15%) of non-contact lens wearers who severe pain, photophobia and blindness may be have had corneal injury with vegetal material, abra- present in viral, bacterial, and fungal keratitis, fre- sion during water or snow related sport activities, quently leading to misdiagnosis and delayed treat- and invasive corneal procedures [2-4]. AK most ment. Early diagnosis and institution of specific often affects one eye,weve ho r, 7.5% of patients in therapy are imperative for a good prognosis as AK England and Wales were found to have bilateral is unresponsive to commonly used antimicrobials. disease [1,5]. Here we document the clinical course and diagnos- Address correspondence to M.A. Haseeb, Ph.D., Department of tic features of unilateral Acanthamoeba keratitis in a Pathology, State University of New York, Downstate Medical Center, 450 Clarkson Avenue, Brooklyn, New York 11203, USA; phone: monocular cosmetic contact lens wearer who was +1 718 270 1610; fax: +1 718 270 2656; e mail: haseeb.siddiqi@ diagnosed with herpetic keratitis. downstate.edu

Figure 1. Left eye before specific therapy on days 3 and 13 of initial presentation (a,b), respectively, and after specific therapy on days 21, 42 and 112 of initial presentation (c,d, and e), respectively. a. shows subtle linear haze near corneal nerve; b. shows epithelial defect with ring infiltrate, perineural corneal infiltrate and hypopyon; c. shows epithelial defect, ring infiltrate and hypopyon; d. shows diffuse circular stromal infiltrate transitioning to scar; e. shows corneal scar with neovascularization. Case Report

The publication of this report has received exemption from full review by the Institutional Review Board.

A 29-year-old man from Brooklyn sought medical attention for severe pain, blurry vision, photophobia, and a foreign body sensation in the left eye for the past 3-4 days. He had been wearing a single sapphire cosmetic Rainbowlens™ soft contact lens in the left eye for 1-2 months that he purchased from a corner drug store. The patient cleaned the lens with a solution that came from the same store and denied wearing the lens during sleep. At presentation, the left upper eyelid was edematous and the conjunctiva hyperemic; the best corrected visual acuity was 20/400. A slit lamp examination revealed severe microcystic corneal edema, circular and perineural linear stromal opacities, and diffuse keratic precipitateve co ring 40% of the corneal endothelium. The right eye wase- unr markable. A clinical diagnosis of herpes simplex keratitis was made and the patient was started on oral acyclovir, ophthalmic Pred Forte (prednisolone ace- tate), ofloxacin, and Cyclogyl (cyclopentolate hydro- chloride). The patient returned 2 days later reporting alleviation in pain and slight improvement in vision, essentially validating our initial clinical diagnosis; the medication regimen was continued. Ten days later, he returned with worsening pain and decreasing vision; an examination showed exacerbated corneal lesions. There was a 9x9 mm paracentral circular epithelial defect, ring stromal infiltrate, and a 4 mm hypopyon in the anterior chamber (Figure 1). His unresponsiveness to therapy prompted debridement of the corneal epithelium. Microscopic examination of the scrapings stained with Giemsa and haematoxylin and eosin revealed Acanthamoeba trophozoites and cysts. The trophozoites measured 20.40 µm (range: 12- 22.5 µm) and cysts measured 18.25 µm (range: 10- 22.5 µm) (Figure 2). Acyclovir and prednisolone were discontinued, and oral itraconazole, ophthalmic desomedine, polyhexamethylene biguanide, chlorhexidine, and cyclogyl were prescribed. When last seen at 112 days, the patient reported compli- ance, but had severe central corneal scarring and poor vision (Table 1). Table 1. Clinical and laboratory findings, and management and treatment of the patient for each of the outpatient clinic visits. 368 Day 1 Day 3 Day 13 Day 21 Day 42 Day 112

Presentation Progressively worsening Improvement in Severe worsening Mild improvement in Minimal pain. No pain. severe pain, redness and pain and vision. of pain, decreased severe pain. Ann

decreased vision in left vision after not al

eye for 2 days; returning for s photophobia. follow-up. of Cl

Clinical BCVA 20/20 OD, BCVA 20/80 OS BCVA: CF OS BCVA: HM OS BCVA: HM OS BCVA: HM OS in

Findings 20/400 OS ic Severe conjunctival Mild conjunctival Severe conjunctival Severe conjunctival Mild conjunctival No conjunctival al injection; mild diffuse injection; mild stromal injection; 9x9 mm injection; 6x6 mm injection; 2x3 mm injection; no & punctuate keratopathy, and endothelial edema; epithelial defect epithelial defect corneal epithelial epithelial defect; La severe stromal and diffuse endothelial with ring infiltrate; and ring infiltrate; defect; mild 7x7 mm corneal bora

endothelial edema with keratic precipitates; peri-neural corneal diffuse stromal stromal edema; scar with to

diffuse inferior two 1 mm stromal infiltrate; 4 mm edema; <0.5 mm diffuse circular neovascu- ry

endothelial keratic opacities; subtle linear hypopyon with hypopyon; mild stromal infiltrate larization; Sc

precipitates; two 1 mm haze near corneal fibrinous anterior anterior iritis. transitioning to scar; no iritis. ie nc

stromal opacities; subtle nerves; stable chamber reaction; mild anterior e,

linear haze near corneal mild anterior iritis. no vitreous debris. iritis; no hypopyon. vo

nerves; mild anterior iritis. l. Presumptive diagnosis: Presumptive diagnosis: Diagnosis: 45 ,

herpes simplex keratitis herpes simplex keratitis Acanthamoeba no

keratitis . Laboratory H&E and Giemsa bacterial, fungal, 20 3, Findings stains of corneal viral cultures: scrapings showed negative 15 Acanthamoeba cysts and trophozoites Management Acyclovir (400 mg) Continue present Aggressive debridement Continue Desomedine, Continue Desomedine, PHMB and Treatment PO 5x/day; Pred management; of epithelium with PHMB, Chlorhexidine PHMB, ophthalmic Forte ophthalmic return in 2 days 15 blade; Desomedine ophthalmic drops Chlorhexidine drops QID drops 6x/day; 0.1% (brolene substitute) every 1-2 hr, ophthalmic drops Ofloxacin ophthalmic 1 drop, c-PHMB Itraconazole 200 mg every 2-3 hr, Plan for drops QID; Cyclogyl 0.02% drops, PO daily Itraconazole penetrating ophthalmic drops TID Chlorhexidine 0.02% 200 mg PO Daily keratoplasty drops alternating every 30 min; Itraconazole 200 mg PO daily; Cyclogyl ophthalmic drops TID

Abbreviations: BCVA-best corrected visual acuity; CF-count fingers vision; H&E-haematoxylin and eosin; HM-hand motion vision; OD-oculus dexter (right eye); OS-oculus sinister (left eye); PHMB-polyhexamethylene biguanide; PO-per oral; QID-four times a day; TID-three times a day. Clinical course of Acanthamoeba keratitis 369

Figure 2. Preparations of the patient’s corneal scrapings collected on day 13 of initial presentation (A-C). Giemsa-stained Acanthamoeba trophozoite with vacuolated cytoplasm and large, distinctive nucleolus in the nucleus (A), and double- walled polyhedral cysts (B). Haematoxylin and eosin-stained polymophonuclear leukocytes attached to a polyhedral Acanthamoeba cyst (C).

Discussion by examination of corneal scrapings or biopsy and demonstrating Acanthamoeba cysts or trophozoites, Our patient presented with intense ocular pain, culture, in vivo tandem-scanning confocal micros- redness, blurry vision and photophobia. These fea- copy or by PCR [1,3,12,13]. tures are not unique to Acanthamoeba keratitis (AK) and may also be found in bacterial, viral, or fungal The prolonged chemotherapy in AK is aimed at keratitis, possibly contributing to delayed or misdi- eradication of viable trophozoites and cysts, the lat- agnosis. Although presence of ring infiltrates, radial ter being more resistant to amebicidal agents. Also, perineuritis, and severe pain are considered pathog- most cysticidal agents (biguanides and diamidines) nomonic for AK, the presenting clinical features in use are toxic to corneal cells and their prolonged encompass a wide variety of non-specific subjective use may result in keratopathy [3,4]. In addition to and objective findings, making a clinical diagnosis antimicrobials, gene silencing using specific small difficult [3,7,8]. AK is also known to present with iRNAs, and corneal transplantation are other thera- minimal pain or as painless keratitis [9-11]. The peutic options [4]. Use of corticosteroids is contro- clinical management is challenging, not only be- versial and should only be used judiciously. cause of the overlapping features, but a proportion Although AK comprises only 5% of the microbial of patients have a history of or concurrent herpes keratitis cases [14-16], it should be considered infection and other medical conditions (rosacea, when there is a failure to respond to first-line ther- trauma, diabetes) contributing to keratopathy [8]. apy for bacterial or viral keratitis even when culture is positive for another organism as 10-23% of AK AK progresses from epithelial to stromal disease. cases may be polymicrobial [3]. Prevention through The initial presentation of pain, redness, photopho- education and adherence to good hygiene practices bia, and sensation of foreign body results from de- could reduce the current incidence of AK among struction of the anterior cornea by the invading contact lens wearers. Acanthamoeba trophozoites. Amoebic trophozoites adhere to the corneal surface by binding to man- AK is on the rise with the use and easy availability nose receptors. Once attached, amoebae penetrate of non-prescription, inexpensive, cosmetic contact the lamellae of the cornea and cause neutrophil in- lenses. As illustrated by this case, high index of sus- filtration and tissue necrosis [1,3]. Perineural infil- picion and clinical awareness are essential in estab- trates, virtually pathognomonic of AK, are believed lishing the diagnosis of AK. Acanthamoeba keratitis to occur because trophozoites cluster around nerves. should be considered in contact lens wearers at pre- As the disease progresses there is ulceration, desce- sentation particularly in those with symptomatic matocele formation, ring infiltrates, and possibly keratitis unresponsive to antiviral and antifungal perforation [1]. Definitive diagnosis of AK is made therapy. 370 Annals of Clinical & Laboratory Science, vol. 45, no. 3, 2015

Acknowledgment MG and DL looked after the patient. TH, RG and MAH did laboratory diagnosis, investigation and analysis, and wrote the report.

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