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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 256-258

Candida albicans keratitis complicated with endophthalmitis after penetrating keratoplasty in Cogan's syndrome


Institut Clínic d'Oftalmologia (ICOF), Hospital Clínic de Barcelona, Barcelona, Spain

Date of Submission27-Jun-2020
Date of Acceptance20-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Carlos Rocha-de-Lossada
Institut Clínic d'Oftalmologia (ICOF), Hospital Clínic de Barcelona, Barcelona, C/Sabino Arana, 1, Barcelona, Spain
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2068_20

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  Abstract 


A 30-year-old woman diagnosed of Cogan's syndrome (CS) with a chronic interstitial keratitis suffered from fungal keratitis and endophthalmitis after penetrating keratoplasty (PK). Microbiological cultures revealed a Candida albicans infection of the corneal graft. The corneoscleral rim was found out to be contaminated by this fungal species. Antifungal treatment, lensectomy and PK were performed. Fungal-related postoperative complications might be considered in a torpid postoperative course after PK specifically in immunosuppressed patients such CS. Routine corneoscleral rim culture may help to identify those patients who could benefit of prophylactic treatment.

Keywords: Candida albicans, Cogan's syndrome, endophthalmitis, keratoplasty, keratitis


How to cite this article:
Romero-Nunez B, Rocha-de-Lossada C, Sabater-Cruz N, Peraza-Nieves J, Torras Sanvicens J. Candida albicans keratitis complicated with endophthalmitis after penetrating keratoplasty in Cogan's syndrome. Indian J Ophthalmol Case Rep 2021;1:256-8

How to cite this URL:
Romero-Nunez B, Rocha-de-Lossada C, Sabater-Cruz N, Peraza-Nieves J, Torras Sanvicens J. Candida albicans keratitis complicated with endophthalmitis after penetrating keratoplasty in Cogan's syndrome. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jun 14];1:256-8. Available from: https://www.ijoreports.in/text.asp?2021/1/2/256/312355



Post-keratoplasty keratitis and endophthalmitis are rare but devastating complications.[1] A recent retrospective review of 17.035 corneas revealed a low overall rate of infection in grafts from an eye bank center (2,3 in 10.000).[2] The prevalence of post-penetrating keratoplasty (PK) endophthalmitis is 0.1 to 0.5%[3] and C. albicans is responsible for most cases.[3]

Cogan's syndrome (CS) is a rare multisystemic disorder characterized by a non-syphilitic interstitial keratitis (IK) and audio-vestibular symptoms. This association was first described in 1934, but named in 1945 by David G Cogan.[4] Its prevalence is unknown though, approximately 300 cases have been reported.[5] The most frequent ophthalmological manifestation of CS is bilateral IK, which is present in 80% of cases[6] requiring sometimes a corneal transplant.[6]

As far as we know, we describe the first report of fungal endophthalmitis following Candida albicans keratitis after a PK in CS.


  Case Report Top


A 30-year-old woman diagnosed of CS presented to our clinic with photophobia and blurred vision in both eyes. The patient had a medical history of recurrent ocular inflammations since childhood and progressive severe bilateral hypoacusis. She was treated with 10 mg/ml rimexolone eye drops, systemic methotrexate, and folinic acid.

The ophthalmological examination revealed a best-corrected visual acuity (BCVA) of 20/25 in the right eye (RE), 20/20 in the left eye (LE) and an intraocular pressure (IOP) within normal limits in both eyes. Slit-lamp examination showed corneal ghost vessels and lipidic stromal degeneration poorly preserving the corneal central area. All those features were compatible with inactive IK [Figure 1].
Figure 1: Slit lamp examination of the right (a) and left (b) eyes showing lipidic stromal corneal degeneration and ghost vessels. Enlarged slit lamp image showing ghost vessels (c)

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A follow up every 6 months was performed, and intensification of topical and systemic treatment was required as the patient presented ocular and systemic inflammation. Specifically, as systemic immunosuppressants azathioprine, cyclosporine, mycophenolate mofetil, tacrolimus, and adalimumab were tried out with poor response. As for topical treatment, corticosteroids and mydriatic eye drops were required.

During the follow-up, a progressive visual acuity deterioration was noticed, BCVA sloped to 20/40 in the RE and 20/50 in the LE. Also, a bilateral progression of the IK and development of cortical cataract in the left eye were found. A PK of the left cornea was performed. Oral and topical corticosteroids were prescribed and tapered gradually. The corneal graft maintained its transparency throughout the initial postoperative follow-up, although later, corneal graft melting, and persistent cellularity in the anterior chamber led us the suspicion of a possible IK recurrence. So that, 40 mg subtenonian triamcinolone 4 weeks and after checking the microbiological results which at that time were negative was prescribed.

At the end of the first postoperative month, slit lamp examination revealed perikeratic hyperemia, keratic precipitates, and round infiltrates suggesting infective keratitis [Figure 2]a and [Figure 2]b. Corneal scraping microbial culture was performed and revealed the presence of Candida albicans in the corneal graft. Topical and systemic antifungal treatments were initiated. Since infective keratitis diagnosis, a frequent follow up was performed. A week after fungal keratitis the patient visited the emergency room because of ocular pain. AS-OCT showed an endothelial graft hyperreflective irregularities and a large amount of hyperreflective material in the anterior capsule of the crystalline lens were found out [Figure 2]c and [Figure 2]d.
Figure 2: Perikeratic hyperemia and round infiltrates in the corneal graft after penetrating keratoplasty of the left cornea (a and b). Anterior Segment Optical Coherence Tomography (AS-OCT) showing hyperreflective endothelial abnormalities (c) and hyperreflective material in the anterior capsule of the crystalline (d). Slit lamp examination after reintervention: penetrating keratoplasty and lensectomy (e)

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The corneoscleral rim (CSR) was found out to be contaminated by Candida albicans as well. Thioglycollate Medium was used for the isolation and cultivation of aerobes, anaerobes and fungi on the CSR remnants. The CSR were incubated for up to 45 days at 35 ± 2°C. Growth was evidenced at day 35 by the presence of turbidity in the tubes and identification of microorganism was done by Matrix-assisted laser desorption/ionization time-of-flight (MALDI-TOF) Mass Spectrometry.

A new surgery was performed including keratoplasty, anterior chamber material extraction, lensectomy, 23-gauge pars plana vitrectomy and B amphotericin and voriconazole intravitreal injections. Currently, her LE is in aphakia and an intraocular lens implantation is about to be conducted [Figure 2]e.


  Discussion Top


Fungal keratitis and endophthalmitis are uncommon but devastating complications after PK.[1] CS is a rare chronic inflammatory disorder of un unknown origin characterized by ocular and audio-vestibular symptoms.[7] In the past 10 years, an increase in the incidence of exogenous fungal endophthalmitis has been noticed.[8] The frequent steroid and broad-spectrum antibiotic use and also contaminated corneal graft or preservation medium may play an important role.[8] It is a fact that fungal infections tend to progress in an immunosuppressive context, so the systemic and local immunosuppressive treatments that the patient was receiving may have had an important role in the progression to endophthalmitis. As in the case reported here, most of post-PK fungal endophthalmitis reported in the literature are related to contaminated CSR.[1] Although the vast majority of literature regarding OCT in endophthalmitis report its usefulness in assessing the posterior segment status,[9] here we show its relevance characterizing anterior segment affectation.

In the context of fungal keratitis or endophthalmitis after PK further surgical intervention is usually required to achieve cure.[10] Although lens fungal deposits are rare, its presence may require lensectomy. Positive fungal donor CSR cultures are uncommon but can carry devastating complications. Systematic cultures of CSR may have prognostic implications as early treatment can be established.[10] Although it has not been determined yet whether prophylactic antifungal treatment would be of any benefit in the presence of a positive CSR culture, some authors support it.[1]

In the case, the CSR contamination was known after the infectious keratitis was established. We believe that prior knowledge of graft contamination could have had prognostic implications in our case since prophylactic treatment could have been initiated. Although infectious keratitis is a rare complication after PK and endophthalmitis is even more infrequent, patient's immunosuppression and graft contamination may play an important role. Systematic culture of CSR may provide diagnostic orientation and may have prognostic implications. We consider that frequent follow-up should be conducted specially in immunosuppressed patients as the case reported.


  Conclusion Top


We describe a rare case of fungal endophthalmitis following Candida albicans keratitis after a PK in CS, and to the best of our knowledge it is the first reported in the literature.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Keyhani K, Seedor J, Shah M, Terraciano A, Ritterbrand D. The incidence of fungal keratitis and endophthalmitis following penetrating keratoplasty. Am J Ophthalmol 2005;140:569-70.  Back to cited text no. 1
    
2.
Mathes K, Tran K, Mayko Z, Stoeger C, Straiko M, Terry M. Reports of post-keratoplasty infections for eye bank-prepared and non–eye bank-prepared corneas. Cornea 2019;38:263-7.  Back to cited text no. 2
    
3.
Merchant A, Zacks CM, Wilhelmus K, Durand M, Dohlman CH. Candidal endophthalmitis after keratoplasty. Cornea 2001;20:226-9.  Back to cited text no. 3
    
4.
Cogan D. Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 1945;33:144-9.  Back to cited text no. 4
    
5.
Cimaz R, Giani T. Cogan syndrome. Orphanet encyclopedia, 2019. Available from: https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=1467.  Back to cited text no. 5
    
6.
Gluth M, Baratz K, Matteson E, Driscoll C. Cogan syndrome: A retrospective review of 60 patients throughout a half century. Mayo Clin Proc 2006;81:483-8.  Back to cited text no. 6
    
7.
D'Aguanno V, Ralli M, de Vincentiis M, Greco A. Optimal management of Cogan's syndrome: A multidisciplinary approach. J Multidiscip Healthc 2017;11:1-11.  Back to cited text no. 7
    
8.
Wykoff CC, Flynn HW, Miller D, Scott IU, Alfonso EC. Exogenous fungal endophthalmitis: Microbiology and clinical outcomes. Ophthalmol 2008;115:1501-7.  Back to cited text no. 8
    
9.
Zhou T, Aptel F, Bron A, Cornut PL, Palombi K, Thuret G, et al. Longitudinal study of retinal status using optical coherence tomography after acute onset endophthalmitis following cataract surgery. Br J Ophthalmol 2017;101:1211-6.  Back to cited text no. 9
    
10.
islisel JM, Goins KM, Wagoner MD, Schmidt GA, Aldrich BT, Skeie JM, et al. Incidence and outcomes of positive donor corneoscleral rim fungal cultures after keratoplasty. Ophthalmol 2017;124:36-42.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2]



 

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