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Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 244-245

A rare case of infectious crystalline keratopathy caused by Burkholderia cepacia

1 Aravind Eye Hospital, Cornea Department, Pondicherry, India
2 Aravind Eye Hospital, Retina Department, Pondicherry, India
3 Wilmer Eye Institute, John Hopkins University, USA

Date of Submission08-May-2020
Date of Acceptance04-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Vaibhav Khanna
Aravind Eye Hospital, Main Cuddalore Road, Thavalakuppam, Pondicherry . 605 007
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1402_20

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We report a unique case of infectious crystalline keratopathy (ICK) caused by Burkholderia cepacia in a 47-year-old patient with history of multiple penetrating keratoplasties (PKPs). Slit-lamp examination revealed a white nonsuppurative needle-like branching anterior stromal infiltrate. Microscopic examination of the Gram-stained smear showed gram-negative bacilli. Cultures revealed Burkholderia cepacia, which was multidrug resistant and sensitive to piperacillin/tazobactam. Based on antibiotic sensitivity report, patient was started fortified piperacillin-tazobactam (0.32%) eye drop on an hourly basis. After 8 weeks of aggressive therapy, the infiltrate resolved with subsequent corneal scarring and vascularization. To our knowledge, this is the first reported case of Burkholderia-related ICK. ICK, first described by Gorovoy et al.[1] in 1983, is an indolent keratitis with characteristic needle-like branching opacities in the absence of corneal or anterior segment inflammation.[2] The presence of an immunocompromised corneal state, such as chronic topical steroid use post PKP, is a known risk factor for the development of ICK. Other risk factors include surgical procedures such as lamellar keratoplasty, corneal cross-linking, laser-assisted in situ keratomileusis, pterygium excision, and trabeculectomy with 5- FU. Topical anaesthetic abuse as a risk factor has also been described.[3] Burkholderia is a group of Gram-negative bacteria, composed of at least 20 different species. We describe a case of Burkholderia keratitis presenting as ICK in a patient who underwent multiple PKPs and a history of long-term topical steroid therapy.

Keywords: Burkholderia cepacia, infectious crystalline keratopathy, therapeutic penetrating keratoplasty

How to cite this article:
Khanna V, Christy J, Jayarajan AP, Chhabra K, Mishra K. A rare case of infectious crystalline keratopathy caused by Burkholderia cepacia. Indian J Ophthalmol Case Rep 2021;1:244-5

How to cite this URL:
Khanna V, Christy J, Jayarajan AP, Chhabra K, Mishra K. A rare case of infectious crystalline keratopathy caused by Burkholderia cepacia. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2022 Dec 5];1:244-5. Available from: https://www.ijoreports.in/text.asp?2021/1/2/244/312328

  Case Report Top

A 47-year-old immunocompetent Asian male with past history of repeat penetrating keratoplasty (PKP), core vitrectomy, and scleral fixated IOL (SFIOL) in the left eye was on regular follow-up for 5 years in our hospital. Best corrected visual acuity (BCVA) was stable post PKP and SFIOL surgery in the left eye for 2 years, while the right eye on examination was unremarkable. Patient visited subspecialty cornea clinic in November 2019 with complaints of pain, redness, and diminution of vision in the left eye, BCVA dropped from 20/60 to 20/250. The patient's medical history and review of systems were unremarkable. He was diagnosed with graft rejection and was treated aggressively with intravenous and topical steroids. Thereafter, on subsequent visits, he maintained a BCVA of 20/250 in the left eye. Slit-lamp examination demonstrated mild graft stromal haze with no signs of active inflammation, and SFIOL in situ.

In January 2020, the patient presented with pain in the left eye and decreased vision to count fingers close to face. Slit-lamp examination revealed a central 5 mm by 4 mm epithelial defect, and a paracentral, anterior stromal infiltrate with well-demarcated crystalline borders measuring 5 mm by 3m [Figure 1]a. Anterior chamber was deep and quiet with SFIOL in situ. Posterior segment examination was unremarkable. Microbiological examination of corneal scraping revealed Gram-negative bacilli [Figure 1]b. Patient was initiated on empirical therapy, which included topical Amikacin eye drops every 2 h, and Gatifloxacin eye drops every hour. Blood agar revealed cream colored growth similar to pseudomonas species [Figure 1]c. Biochemical tests and Vitek 2 microbial identification system confirmed the organism as Burkholderia cepacia. Vitek 2 antibiotic sensitivity system and disk diffusion method revealed sensitivity to piperacillin and tazobactam, but resistant to fluoroquinolones, aminoglycosides, polymyxin B, colistin, and imipenem. The antibiotic regimen was changed to hourly topical fortified piperacillin-tazobactam 0.32% eye drops. Eye drops were reconstituted using piperacillin-tazobactem 4.5 gm powder used for i.v. (intravenous) injection and reconstituted with 20 mL of sterile distilled water. For 0.32% eye drops 4 mL of this solution was reconstituted with 10 mL of sterile distilled water. The patient was advised to store the reconstitute at 4°C. After 1 week, the patient's epithelial defect started to heal, and the antibiotic was reduced to six times a day. The infiltrate continued to resolve, and the treatment was gradually tapered. Six weeks after initial presentation, BCVA in the left eye was count fingers close to face. Slit-lamp examination revealed a small, central epithelial defect measuring 1 mm by 1 mm in the graft with corneal stromal scarring [Figure 1]d, which completely healed by 8 weeks.
Figure 1: (a) Slit-lamp photograph of the left eye at initial visit, demonstrating conjunctival congestion, a central epithelial defect with a nonsuppurative, branching, needle-like crystalline corneal stromal infiltrate.(b) Gram-negative bacilli isolated postcorneal scraping. (c) Blood agar showing growth after 1 week of inoculation. (d) Slit-lamp photograph after 6 weeks of topical antibiotic treatment; the epithelial defect decreased significantly along with healed stromal infiltrate with scarring and peripheral neovascularization

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  Discussion Top

The pathophysiology of ICK is not clearly understood; however, a break in Bowman's membrane and subsequent entry of the organism into stroma via a suture track may play a role in its pathogenesis in certain cases.[4] Christakopoulos CE et al. hypothesized the role of a biofilm secreted by the organism as a result of decreased infiltration of inflammatory mediators secondary to a suppressed immune response.[5]

The most common organism associated with ICK is Streptococcus viridans, and it may be because of the organism's ability to produce mucopolysaccharides as a biofilm.[6] Other causative organisms reported include Streptococcus pneumoniae, coagulase-negative Streptococcus spp., Pepto streptococcus spp., Haemophilus aphrophilus, Mycobacterium spp., Pseudomonas aeruginosa, Enterococcus spp., Candida spp., Alternaria spp., and Serratia marcescens.[7]

The diagnosis of ICK is mainly clinical, which includes a careful history such as topical or systemic steroid use, other topical eye drops, and prior ocular surgery. The management includes corneal scrapings of the infiltrate for microbiological evaluation, including light microscopic examination, culture with antibiotic sensitivities, and prompt initiation of antimicrobial eye drops.

Burkholderia cepacia is one of the opportunistic human pathogens that most often causes pneumonia in immunocompromised individuals with underlying lung disease such as cystic fibrosis or chronic granulomatous disease.[8] Ocular infections, including keratitis and endophthalmitis, are very rare.[9] Risk factors for keratitis caused by Burkholderia include contact lens wear, trauma, and PKP. Sunita et al. have suggested topical corticosteroids could be a risk factor for Burkholderia keratitis in a published case series.[10]

  Conclusion Top

To conclude ICK in PKP patients is a rare complication. Many organisms have been associated with this entity; however, Burkholderia cepacia has never been reported until now. ICK being indolent in nature threatens the vision and the graft survival rate in a keratoplasty patient. Immediate discontinuation of topical steroids with targeted therapy after performing a good microbiological workup is imperative for faster recovery. A repeat keratoplasty after complete resolution of infection is often required to restore good visual outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Gorovoy MS, Stern GA, Hood I. Intrastromal non-inflammatory bacterial colonization of a corneal graft. Arch Ophthalmol 1983;101:1749-52.  Back to cited text no. 1
Stern GA. Infectious crystalline keratopathy. Int Ophthalmol Clin 1993;33:1-7.  Back to cited text no. 2
Kintner JC, Grossniklaus HE, Lass JH. ICK associated with topical anaesthetic abuse. Cornea 1990;9:77-80.  Back to cited text no. 3
James CB, McDonnell PJ, Falcon MG. Infectious crystalline keratopathy. Br J Ophthalmol 1988;72:628-30.  Back to cited text no. 4
Christakopoulos CE, Prause JU, Heegaard S. Infectious crystalline keratopathy histopathological characteristics. Acta Ophthalmol Scand 2003;81:659-61.  Back to cited text no. 5
Hunts JH, Matoba AY, Osato MS. Infectious crystalline keratopathy: The role of bacterial exopolysaccharide. Arch Ophthalmol 1993;111:528-30.  Back to cited text no. 6
Porter AJ, Lee GA, Jun AS. Infectious Crystalline Keratopathy. Surv Ophthalmol 2018;63:480-99.  Back to cited text no. 7
Speert DP. Advances in Burkholderia cepacia complex. Pediatr Respir Rev 2002;3:230-5.  Back to cited text no. 8
Ornek k, Ozdemir M, Ergin A. Burkholderia cepacia keratitis with endophthalmitis. J Med Microbiol 2009;58:1517-8.  Back to cited text no. 9
Chaurasia S, Muralidhar R, Das S. Keratitis caused by Burkholderia cepacia. Br J Ophthalmol 2011;95:746-7.  Back to cited text no. 10


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