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A rare case of preseptal orbital mucormycosis in a kidney transplant recipient

Julia Janeczko1, Agata Góral1, Paweł Poznański2, Aleksandra Zgryźniak3, Dorota Kamińska2, Magdalena Krajewska2
* JJ and AG contributed equally to this work.
1 Student Scientific Club, Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Wrocław, Poland
2 Department of Non‑Procedural Clinical Sciences, Faculty of Medicine, Wrocław University of Science and Technology, Wrocław, Poland
3 Department of Ophtalmology, Jan Mikulicz‑Radecki University Clinical Hospital in Wroclaw, Wrocław, Poland
DOI: 10.20452/pamw.16816
Published online: August 1, 2024.
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In this article

A 47‑year‑old white woman was admitted to a department of nephrology and transplantation medicine 11 years after receiving a kidney transplant from a deceased donor, due to orbital phlegmon and acute kidney allograft injury. At that time, immunosuppressive regimen was based on prednisone 5 mg once daily and mycophenolate mofetil 250 mg twice a day. Prior to the admission, the patient had been treated for 48 hours with topical tobramycin and dexamethasone due to blepharitis of the right eye. At admission, physical examination revealed right‑sided orbital inflammation with phlegmon and focal skin necrosis of the eyelids, accompanied by massive purulent conjunctival discharge. The inflammation, manifested as cellulitis, also involved the skin of the right cheek, right side of the neck, and left eyelids (Figure 1A and 1B). Submandibular lymph nodes were significantly enlarged. Examination of the anterior segment of the right eye was not possible due to narrow palpebral fissure and extreme tenderness of the tissues. Pupillary functions seemed unaltered and relative afferent pupillary defect was not present. Computed tomography (CT) and magnetic resonance imaging (MRI) did not show any orbital, sinal, or central nervous system involvement. Microscopic examination of surgically removed necrotic tissues affirmed morphologic features of Mucor (Figure 1C and 1D). Moreover, the intraoperative swab culture examination showed growth of Streptococcus pyogenes. Considering the high concentration of C‑reactive protein (493 mg/l; reference range [RR] <⁠0.5 mg/l) and procalcitonin (>200 ng/ml; RR <⁠0.05 ng/ml), parenteral treatment with liposomal amphotericin B, meropenem, and clindamycin was initiated. In addition, mycophenolate mofetil was discontinued and intravenous methylprednisolone was administered as the only immunosuppressive agent. Initially, the acute kidney allograft injury occurred with admission serum creatinine levels of 5.1 mg/dl (RR, 0.6–1.3 mg/dl), with subsequent gradual lowering over the course of treatment to 1.46 mg/dl at the time of discharge. During the last outpatient visit prior to the admission, serum creatinine concentration was 1.73 mg/dl. Due to improvement of the patient general condition, cyclosporin A was introduced into the treatment and parenteral glucocorticoids were converted to an oral form. Antifungal therapy was continued for 5 weeks and after the acute phase reactants normalized, the patient was discharged in good general condition and with good allograft function, unaltered visual acuity, and only some scarring on the skin of the eyelids (Figure 1E). Due to immunosuppression, kidney transplant recipients (KTRs) are susceptible to invasive fungal diseases (IFDs). Mucormycosis, which is an opportunistic infection caused by the members of a ubiquitous fungal order of Mucorales, accounts for 0.2%–1.2% of IFDs in KTRs.1 Different manifestations of the disease have been described, such as pulmonary, rhino‑orbito‑cerebral, gastrointestinal, cutaneous, and disseminated form.2 Risk factors for mucormycosis in KTRs include age, deceased donor, tacrolimus, nonwhite race, and Hispanic ethnicity.3 Cutaneous form may manifest as painful erythema with edema, progressing over time to ulceration and necrosis, covering deeper structures with fistulas.4 Diagnosis of mucormycosis is possible through microscopic examination, supported by immunohistochemistry or polymerase chain reaction techniques. Imaging exminations, such as CT or MRI, should be performed to evaluate the extent of changes. Cultures should also be performed, but the results are positive only in 50% of cases.5 Treatment includes administration of lipid formulations of amphotericin B, posaconazole, or both, and, if possible, surgical resection of necrotic tissue. Despite availability of specific treatment, the overall mortality rate ranges from 40% to 80%, depending on the site of infection and on the underlying conditions.5 Early diagnosis, surgical debridement of the infected tissue, and antifungal treatment are crucial in the management of mucormycosis.

Figure 1 A, B – images of the patient’s eyes on admission showing right‑sided orbital inflammation with purulent skin necrosis of the eyelids, accompanied by cellulitis of the right cheek (A) and cellulitis of left eyelids (B); C, D – microscopic examination of necrotic masses and pus containing small fragments of soft tissues undergoing necroinflammatory destruction, showing visible strands and spores of fungal hyphae (morphology consistent with mucormycosis; hematoxylin and eosin staning); E – an image of the patient’s eyes 5 months after treatment showing mild scarring of the skin of the right eyelids and unaltered anterior segment of the right eye
Acknowledgements: Histopathological pictures courtesy of professor Agnieszka Hałoń from the Department of Clinical and Experimental Pathology, Wroclaw Medical University.
Funding: None.
Conflict of interest: None declared.
References
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  2. Steinbrink JM, Miceli MH. Clinical review of mucormycosis. Infect Dis Clin North Am. 2021; 35: 435‑452. | Crossref
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