International Journal of Ophthalmology & Visual Science

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Endogenous Endophthalmitis by Candida Albicans Associated with a Primary Immunodeficiency Due to CARD9 Deficiency

Received: 01 September 2019    Accepted: 04 October 2019    Published: 15 October 2019
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Abstract

This report encopasses a case of endogenous endophthalmitis by Candida albicans in an otherwise healthy young adult patient, which led to the diagnosis of a primary immunodeficiency, CARD9 deficiency, associated with familial candidiasis. A 31-year-old female patient with a one-month history of low visual acuity (VA) in the right eye (RE) was referred after no response to treatment of toxoplasmosis and acute retinal necrosis. She presented no comorbidities, but a history of recurrent paronychia, oral candidiasis and tinea capitis since childhood. Her parents were first-degree cousins. Visual acuity was 20/2000 in the RE and 20/20 in the left eye (LE). There was an elevated exudative lesion occupying the retinal posterior pole, associated with fluffy white cotton balls in the adjacent posterior vitreous. Optical coherence tomography (OCT) showed macular lesions with the "rain cloud" sign. Fungal endogenous endophthalmitis was hypothesized. Imaging and laboratory examinations discarded other infections and eliminated involvement of other organs. Diagnostic vitreous puncture was performed in addition to Amphotericin B injection and intravenous Fluconazole. As culture results were negative, a vitreous biopsy was performed, in which Candida albicans was grown, followed by a second Amphotericin B injection. As there was no improvement, pars plana posterior vitrectomy, with placement of silicone oil, was performed. In spite of infection control, VA remained 20/2000. Due to a severe ocular condition with no other invasive sites of infection, associated with superficial recurrent fungal infections, a primary fungal specific immunodeficiency was suspected, resulting in referral to Immunology. An immunogenetic panel was performed, demonstrating heterozygosity for two CARD9 gene mutations, a molecule related to familial candidiasis. Follow-up in Ophthalmology and Immunology was maintained and oral Fluconazole was proposed to be taken for at least 6 months. Three months later, she presented with an oral abscess secondary to Candida, managed by the Otorhinolaryngology team. Fungal endogenous endophthalmitis is an important cause of morbidity and low visual acuity, especially in the population of immunocompromised patients. Early ophthalmological diagnosis should preferably be performed in order to prevent lesions from increasing, affecting noble areas such as the macula, and reaching the vitreous. When the fundus exam presents signs of fungal endophthalmitis in immunocompetent, young and healthy patients, diagnosis of primary immunodeficiencies should be considered.

DOI 10.11648/j.ijovs.20190404.13
Published in International Journal of Ophthalmology & Visual Science (Volume 4, Issue 4, December 2019)
Page(s) 71-76
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Endogenous Endophthalmitis, Candida albicans Endophthalmitis, CARD9 Deficiency, Familial Candidiasis

References
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[2] Coneell PP, O’Neill EC, Fabinyi D, Islam FMA, Buttery R, McCombe M, Essex RW, Roufail E, Clark B, Chiu D, Campbell W, Allen P. Endogenous endophthalmitis: 10-year experience at a tertiary referral centre. Eye. Vol. 25, 2011, pp. 66-72.
[3] Chhablani J. Fungal endophthalmitis. Expert Rev. Anti Infect. Ther. Vol. 9, No. 12, 2011, pp. 1191-1201.
[4] Sallam A, Lynn W, McCluskey P, Lightman S. Endogenous Candida endophthalmitis. Expert Rev. Anti Infect. Ther. Vol. 4, No. 4, 2006, pp. 675-685.
[5] Ueda T, Takesue Y, Tokimatsu I, Miyazaki T, Nakada-Motokawa N, Nagao M, Nakajima K, Mikamo H, Yamagishi Y, Kasahara K, Yoshihara S, Ukimura A, Yoshida K, Yoshinaga N, Izumi M, Kakeya H, Yamada K, Kawamura H, Endou K, Yamanaka K, Yoshioka M, Amino K, Ikeuchi H, Uchino M, Miyazaki Y. The incidence of endophthalmitis or macular involvement and the necessity of a routine ophthalmic examination in patients with candidemia. Plos One. Vol. 14, No. 5, 2019, e0216956.
[6] Drewniak A, Gazendam RP, Tool AT, van Houdt M, Jansen MH, van Hamme JL, van Leeuwen EM, Roos D, Scalais E, de Beaufort C, Janssen H, van den Berg TK, Kuijpers TW. Invasive fungal infection and impaired neutrophil killing in human CARD9 deficiency. Blood. Vol. 121, No. 13, 2013, pp. 2385-2392.
[7] Gavino C, Cotter A, Lichtenstein D, Lejtenyi D, Fortin C, Legault C, Alirezaie N, Majewski J, Sheppard DC, Behr MA, Foulkes WD, Vinh DC. CARD9 Deficiency and Spontaneous Central Nervous System Candidiasis: Complete Clinical Remission With GM-CSF Therapy. Clinical Infectious Diseases. Vol. 59, No. 1, 2014, pp. 81–84.
[8] Jones N, Garcez T, Newman W, Denning D. Endogenous Candida endophthalmitis and osteomyelitis associated with CARD9 deficiency. BMJ Case Rep. Published online: 2016. Doi: 10.1136/bcr-2015-214117.
[9] Gavino C, Mellinghoff S, Cornely OA, Landekic M, Le C, Langelier M, Golizeh M, Proske S, Vinh DC. Novel bi-allelic splice mutations in CARD9 causing adult-onset Candida endophthalmitis. Mycoses Vol. 61, No. 1, 2018, pp. 61-65.
[10] Feman SS, Nichols JC, Chung SM, Theobald TA. Endophthalmitis in patients with disseminated fungal disease. Trans. Am. Ophthalmol. Soc. Vol. 100, 2002, pp. 67-71.
[11] Chavan R, Mustafa MZ, Narendran N, Tarin S, Yang Y. A case of Candida albicans endophthalmitis with no predisposing risk factors and a distant source of infection. Case Rep. Ophthalmol. Vol. 3, 2012, pp. 277-282.
[12] Yeşiltaş YS, Özcan G, Demirel S, Yalçindağ N. Culture-Proven Candida Albicans Endogenous Endophthalmitis in a Patient with Onychomycosis. Ocul Immunol Inflamm. Vol. 22, 2019, pp 1-4.
[13] Lavine JA, Mittelu M. Multimodal imaging of refractory Candida chorioretinitis progressing to endogenous endophthalmitis. Journal of Ophthalmic Inflammation and Infection. Vol. 5, No 24, 2015, pp. 1-5.
[14] Invernizzi A, Symes R, Miserocchi E, Cozzi M, Cereda M, Fogliato G, Staurenghi G, Cimino L, McCluskey P. Spectral Domain Optical Coherence Tomography findings in endogenous Candida Endophthalmitis and their clinical relevance. Retina. Vol. 38, No. 5, 2018, pp. 1011-1018.
[15] Lingappan A, Wykoff CC, Albini TA, Miller D, Pathengay A, Davis JL, Flynn Jr HW. Endogenous fungal endophthalmitis: causative organisms, management strategies, and visual acuity outcomes. American journal of ophthalmology. Vol. 153, No. 1, 2012, pp. 162-6.
[16] Binder MI, Chua J, Kaiser PK, Procop GW, Isada CM. Endogenous endophthalmitis: an 18-year review of culture-positive cases at a tertiary care center. Medicine (Baltimore). Vol. 82, No. 2, 2003, pp. 97-105.
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Author Information
  • Faculty of Medicine, University of Itaúna, Itaúna, Brazil

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    Juliana Albano de Guimarães, Maria Campos Pires, Camillo Carneiro Gusmão, Andrea Mara Simões Torigoe. (2019). Endogenous Endophthalmitis by Candida Albicans Associated with a Primary Immunodeficiency Due to CARD9 Deficiency. International Journal of Ophthalmology & Visual Science, 4(4), 71-76. https://doi.org/10.11648/j.ijovs.20190404.13

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    Juliana Albano de Guimarães; Maria Campos Pires; Camillo Carneiro Gusmão; Andrea Mara Simões Torigoe. Endogenous Endophthalmitis by Candida Albicans Associated with a Primary Immunodeficiency Due to CARD9 Deficiency. Int. J. Ophthalmol. Vis. Sci. 2019, 4(4), 71-76. doi: 10.11648/j.ijovs.20190404.13

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    AMA Style

    Juliana Albano de Guimarães, Maria Campos Pires, Camillo Carneiro Gusmão, Andrea Mara Simões Torigoe. Endogenous Endophthalmitis by Candida Albicans Associated with a Primary Immunodeficiency Due to CARD9 Deficiency. Int J Ophthalmol Vis Sci. 2019;4(4):71-76. doi: 10.11648/j.ijovs.20190404.13

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  • @article{10.11648/j.ijovs.20190404.13,
      author = {Juliana Albano de Guimarães and Maria Campos Pires and Camillo Carneiro Gusmão and Andrea Mara Simões Torigoe},
      title = {Endogenous Endophthalmitis by Candida Albicans Associated with a Primary Immunodeficiency Due to CARD9 Deficiency},
      journal = {International Journal of Ophthalmology & Visual Science},
      volume = {4},
      number = {4},
      pages = {71-76},
      doi = {10.11648/j.ijovs.20190404.13},
      url = {https://doi.org/10.11648/j.ijovs.20190404.13},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.ijovs.20190404.13},
      abstract = {This report encopasses a case of endogenous endophthalmitis by Candida albicans in an otherwise healthy young adult patient, which led to the diagnosis of a primary immunodeficiency, CARD9 deficiency, associated with familial candidiasis. A 31-year-old female patient with a one-month history of low visual acuity (VA) in the right eye (RE) was referred after no response to treatment of toxoplasmosis and acute retinal necrosis. She presented no comorbidities, but a history of recurrent paronychia, oral candidiasis and tinea capitis since childhood. Her parents were first-degree cousins. Visual acuity was 20/2000 in the RE and 20/20 in the left eye (LE). There was an elevated exudative lesion occupying the retinal posterior pole, associated with fluffy white cotton balls in the adjacent posterior vitreous. Optical coherence tomography (OCT) showed macular lesions with the "rain cloud" sign. Fungal endogenous endophthalmitis was hypothesized. Imaging and laboratory examinations discarded other infections and eliminated involvement of other organs. Diagnostic vitreous puncture was performed in addition to Amphotericin B injection and intravenous Fluconazole. As culture results were negative, a vitreous biopsy was performed, in which Candida albicans was grown, followed by a second Amphotericin B injection. As there was no improvement, pars plana posterior vitrectomy, with placement of silicone oil, was performed. In spite of infection control, VA remained 20/2000. Due to a severe ocular condition with no other invasive sites of infection, associated with superficial recurrent fungal infections, a primary fungal specific immunodeficiency was suspected, resulting in referral to Immunology. An immunogenetic panel was performed, demonstrating heterozygosity for two CARD9 gene mutations, a molecule related to familial candidiasis. Follow-up in Ophthalmology and Immunology was maintained and oral Fluconazole was proposed to be taken for at least 6 months. Three months later, she presented with an oral abscess secondary to Candida, managed by the Otorhinolaryngology team. Fungal endogenous endophthalmitis is an important cause of morbidity and low visual acuity, especially in the population of immunocompromised patients. Early ophthalmological diagnosis should preferably be performed in order to prevent lesions from increasing, affecting noble areas such as the macula, and reaching the vitreous. When the fundus exam presents signs of fungal endophthalmitis in immunocompetent, young and healthy patients, diagnosis of primary immunodeficiencies should be considered.},
     year = {2019}
    }
    

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    AU  - Maria Campos Pires
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    AB  - This report encopasses a case of endogenous endophthalmitis by Candida albicans in an otherwise healthy young adult patient, which led to the diagnosis of a primary immunodeficiency, CARD9 deficiency, associated with familial candidiasis. A 31-year-old female patient with a one-month history of low visual acuity (VA) in the right eye (RE) was referred after no response to treatment of toxoplasmosis and acute retinal necrosis. She presented no comorbidities, but a history of recurrent paronychia, oral candidiasis and tinea capitis since childhood. Her parents were first-degree cousins. Visual acuity was 20/2000 in the RE and 20/20 in the left eye (LE). There was an elevated exudative lesion occupying the retinal posterior pole, associated with fluffy white cotton balls in the adjacent posterior vitreous. Optical coherence tomography (OCT) showed macular lesions with the "rain cloud" sign. Fungal endogenous endophthalmitis was hypothesized. Imaging and laboratory examinations discarded other infections and eliminated involvement of other organs. Diagnostic vitreous puncture was performed in addition to Amphotericin B injection and intravenous Fluconazole. As culture results were negative, a vitreous biopsy was performed, in which Candida albicans was grown, followed by a second Amphotericin B injection. As there was no improvement, pars plana posterior vitrectomy, with placement of silicone oil, was performed. In spite of infection control, VA remained 20/2000. Due to a severe ocular condition with no other invasive sites of infection, associated with superficial recurrent fungal infections, a primary fungal specific immunodeficiency was suspected, resulting in referral to Immunology. An immunogenetic panel was performed, demonstrating heterozygosity for two CARD9 gene mutations, a molecule related to familial candidiasis. Follow-up in Ophthalmology and Immunology was maintained and oral Fluconazole was proposed to be taken for at least 6 months. Three months later, she presented with an oral abscess secondary to Candida, managed by the Otorhinolaryngology team. Fungal endogenous endophthalmitis is an important cause of morbidity and low visual acuity, especially in the population of immunocompromised patients. Early ophthalmological diagnosis should preferably be performed in order to prevent lesions from increasing, affecting noble areas such as the macula, and reaching the vitreous. When the fundus exam presents signs of fungal endophthalmitis in immunocompetent, young and healthy patients, diagnosis of primary immunodeficiencies should be considered.
    VL  - 4
    IS  - 4
    ER  - 

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