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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 22  |  Issue : 1  |  Page : 28-31

Mucormycosis: Atypical presentation and the associated red flags


1 Department of ENT, BDF Hospital, Kingdom of Bahrain, Dharan, KSA
2 King Fahad Military Medical Complex, Dharan, KSA

Date of Submission06-Sep-2019
Date of Decision06-Oct-2019
Date of Acceptance19-Nov-2019
Date of Web Publication01-Jun-2020

Correspondence Address:
Dr. Muneera Al-Khalifa
BDF - ENT Senior Resident, Kingdom of Bahrain
KSA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/SJOH.SJOH_14_19

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  Abstract 


Mucormycosis previously known as zygomycosis is a type of scarce infection caused by fungi. Mucorales is the order that mucormycosis is related to, with Rhizopus being the most common genus. Mucormycosis occurs usually in immunocompromised individuals such as diabetics or posttransplant patients. Diabetes is the most common predisposing factor for rhino-orbital-cerebral mucormycosis. The first documented case of an upper airway mucormycosis was described by Paltauf, in 1885. Thereafter, Gregory reported three cases of rhinocerebral mucormycosis in 1943. In rhino-orbital-cerebral mucormycosis, the spores adhere to the nasal mucosa and rapidly proliferate in the ideal metabolic hypoxic state found in diabetics. Being an angiotropic fungus, mucor swiftly invades the elastic lamina of the blood vessels, leading to necrosis and ischemic infarctions. Patients with rhino-orbital-cerebral mucormycosis may present with sinusitis, rhinitis, ocular complaints, or facial pain. Here, we present a subtle presentation of an isolated case of rhinosinusitis mucormycosis in a 50-year-old male presenting to the emergency department with 1-week history of headache aggravated at night and the red flags that lead to the diagnosis. On presentation, the patient was afebrile and generally well. Nasal examination revealed a patch of necrotic mucosa on the nasal septum. The patient was admitted with the differentials of either invasive rhinosinusitis or granulomatous disease. Nasal swabs were collected revealing a colonization of methicillin-resistant Staphylococcus aureus; nevertheless, the red flags that lead to suspecting a more aggressive infection were the complain of paresthesia of the cheek and upper lip, a necrotic patch on the hard palate, and the aggravated nocturnal headache. The definitive diagnosis was achieved by histopathology demonstrating the importance of high index of suspicion in such cases.

Keywords: Chronic sinusitis, fungal sinusitis, mucormycosis


How to cite this article:
Al-Khalifa M, Alsaif S, Al Bahrani S. Mucormycosis: Atypical presentation and the associated red flags. Saudi J Otorhinolaryngol Head Neck Surg 2020;22:28-31

How to cite this URL:
Al-Khalifa M, Alsaif S, Al Bahrani S. Mucormycosis: Atypical presentation and the associated red flags. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2020 [cited 2020 Jul 16];22:28-31. Available from: http://www.sjohns.org/text.asp?2020/22/1/28/285550




  Introduction Top


Mucormycosis is a fungal infection isolated usually in immunocompromised individuals.[1],[2],[3],[4] Mucormycosis is a type of rare fungi. Mucorales is the order that mucormycosis is related to; with Rhizopus being the most common genus.[1] Mucormycosis occurs usually in immunocompromised individuals. Diabetes is The most common predisposing factor for rhino-orbital-cerebral mucormycosis.[2],[3],[4] Paltauf described the first documented case of mucormycosis in 1885.[5] Followed by three cases of rhinocerebral mucormycosis reported by Gregory in 1943.[5] In rhino-orbital- cerebral mucormycosis the spores adhere to the nasal mucosa and rapidly proliferate due to the metabolic hypoxic state in diabetics. Being an angiotropic fungus mucor invades the elastic lamina of the blood vessels leading to ischemic infarctions.[2],[4],[6] Sinusitis, rhinitis, ocular complains or facial pain are all potential presenting symptoms in rhino-orbital- cerebral mucomycosis.[2],[4],[6] Advancement of the disease in infected patients is mostly rapid and violent especially in poorly controlled immune defenses. Due to its rapid course, early recognition of its subtle signs is lifesaving. Epidemiology of mucormycosis varies from one region to another with the disease being more common in developed countries among the individuals with diabetes mellitus, hematological malignancies, and organ transplants, while in developing countries such as India, mucormycosis incidences are more common in uncontrolled diabetic patients or posttrauma.[7]


  Case Report Top


History and physical examination

A 50-year-old retired Saudi male presented to the King Fahad Military Medical Complex with a history of constant frontal headache of 1-week duration that increased with bending forward, no history of visual disturbance, no recent dental procedure, or recent upper respiratory tract infection. According to the patient, he had a previous history of nasal polyposis long standing that was not followed neither did he complain of it. One day before presentation, the patient started to notice dark discharge from his nasal cavity with hyposmia; however, there was no fever or nasal blockage.

In the emergency, the patient was investigated to rule out acute cerebrovascular assault owing to his headache. Computed tomography (CT) scan brain was ordered and found to be negative for acute assault. Incidentally, the patient was found to have a high blood sugar of 29.1 with positive urinary ketones and he was newly diagnosed with diabetes mellitus. The patient was discharged from emergency as a case of epistaxis with conservative management.

At the same night, the patient presented back to the emergency room with severe headache where he was referred to our otolaryngology department to rule out sinusitis.

Initial blood investigations showed C-reactive protein of 69.8, erythrocyte sedimentation rate 63, white blood cell 8.4, Hgb 18.7 g/dl, iron 11.8 μmol/L, platelets 181 × 10[3]/μl, and glycosylated Hb 13.9%.

On examination, the patient was vitally stable with no facial swelling or change of skin color. Anterior rhinoscopy with zero-degree rigid scope showed bilateral pale Grade1 polyps, erythematic engorged nasal mucosa, and what was thought to be an ulcer on the nasal septum with purulent discharge. Oral examination showed ulcer in the hard palate [Figure 1]a.
Figure 1: (a) Hard palate ulceration, (b) after 6 weeks of treatment

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Hospital course

Nasal swab was collected that grows methicillin-resistant Staphylococcus aureus (MRSA). Initial differential of granulomatous disease was made and the patient was started on antibiotics (cefuroxime, vancomycin, and metronidazole) as a superimposed MRSA infection. The endocrine team followed the patient and tight blood sugar control was insured. CT scan sinuses were done and showed partial opacification of the maxillary, frontal, anterior ethmoids, posterior ethmoids, and sphenoid sinuses bilaterally with the disease being more evident on the left side [Figure 2]. During hospital stay day 2, the patient started to complain of upper lip paresthesia and the left cheek paresthesia. Magnetic resonance imaging (MRI) sinuses were then ordered and showed enhancing sinus mucosal diseases [Figure 3]a.
Figure 2: Computed tomography scan sinus showing the opacification filling the maxillary and anterior ethmoids

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Figure 3: (a) Magnetic resonance imaging sinus before the debridement surgery and (b) magnetic resonance imaging sinus after the surgery and initiation of treatment

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Multiple biopsies were taken from the nasal cavity and nasal septum under general anesthesia that also revealed to have necrotic tissue and septal perforation under the engorged mucosa [Figure 4].
Figure 4: (a) Nasal septum initial presentation covered with dark crusts. (b) Nasal septum perforation and engorged erythematous nasal mucosa

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The histopathology report came back confirming the diagnosis of mucormycosis [Figure 5]. The patient was then taken back to the operating theater and underwent functional endoscopic sinus surgery and extensive sinonasal debridement. Intraoperative findings revealed an intact lamina papyracea and base of skull. Infectious disease team was consulted and the patient was started on liposomal amphotericin-B (5 mg/kg/day) with regular nasal debridement till clinical and radiological improvement that took about 6 weeks in duration. The patient improved immensely after few days of starting the liposomal amphotericin-B the palate ulcer started [Figure 1]b, headache improved and he regained the sense of smell. The patient was discharged with step-down therapy of oral posaconazole suspension 400 mg BID to be continued till complete clinical and radiological resolution of the infection and close follow-up in outpatient clinic with repeated MRI that showed improvement [Figure 3]b.
Figure 5: Histopathology nasal mucosa showing mucormycosis as broad nonseptated hyphae with irregular branching

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  Discussion Top


Invasive fungal disease such as mucormycosis usually has a vast impact on the infected individuals. Mucormycosis is an opportunistic being cunning its way in hosts weakened by diseases such as diabetes or posttransplant patients although emerging cases of mucormycosis in immunocompetent patients have been reported in literature.[8],[9]

In a systemic review conducted by Jeong et al. between January 2000 and January 2017, 851 individual cases were reviewed showing that the majority of reported cases came from Europe with 34% followed by Asia and the Americas with 31% and 28%, respectively.[7] Alas, documentation of mucormycosis incidence in the Middle East region is scarce; nonetheless, a recent study conducted in Jordan reported that annual rates in Iraq and Algeria were estimated, as in Jordan, at about 0.2/100,000, while 0.034/100,000 for Saudi Arabia.[10]

The mass of mucormycosis cases is rhino-orbital-cerebral while cutaneous, pulmonary, hepatic, isolated cerebral, and gastric cases have been described.[11],[12] Inhalation of the sporangiospores is the most common route for infection in rhino-orbital-cerebral form where the spores can adhere and germinate the mucosa of the respiratory system.[10]

In rhino-orbital-cerebral mucormycosis, diabetes mellitus was found to be the most common underlying compromising disease in most patients. In literature review piloted by Roden et al., 929 documented cases were revised, out of those 337 cases had diabetes mellitus.[12] Another study conducted in France disclosed an increase in mucormycosis incidence from 0.7 to 1.2/million in 1997–2006 with an increase in diabetes-associated mucormycosis by 9%.[11],[13],[14]

Similar to our case, our patient was found to have high blood sugar and urinary ketones as an underlying cause for his compromised defenses.

Presentation of rhino-orbital-cerebral mucormycosis patients is mostly rapidly progressive sinusitis with ocular and cerebral extension.[2],[4]

In contrast, our patient presented with slow-progressing subtle signs and symptoms that diverted the primary caregivers from suspecting mucormycosis as a possible cause. In a retrograde view of the case, the red flags of having severe headache associated with paresthesia of the face should have raised the suspicion of having a serious underlying cause earlier. Although these signs are nonspecific to mucormycosis, they will still alert the physician to promptly and aggressively investigate the patient. A recent multicenter study in Mexico reported several cases of indolent type of mucormycosis that mainly presents with facial pain or headache analogous to our case that lacked the swiftness and fierceness of the fulminant type of mucormycosis.[9]

Mucormycosis has neither specific presenting signs, symptoms nor pathognomonic radiological signs. The accurate diagnosis is made only by identification of the fungus in the affected host tissue still the cultural isolation yield is recognized to be between 50% and 71%.[12],[15]

Once mucormycosis is diagnosed with an aggressive treatment, protocol should be initiated as the reported mortality overall is exceeding 50%.[15] The treatment strategies must include the correction of the underlying risk factors, surgical debridement of the affected tissues, and antifungal medications.[8],[9

In our case, an aggressive debridement of the rhino-sinus cavities was done with concomitant antifungal therapy. The correction of the acidotic state and high blood glucose levels to restore a balanced electrolytic equilibrium was also essential in the treatment plan.

Liposomal amphotericin-B showed effective outcome in regressing the advancement of mucormycosis with a combination of surgical therapy.[16]

The use of liposomal amphotericin-B in our case showed dramatic improvement in the symptoms of the patient after <72 h of commencement, therefore concluding the necessity of early recognition and treatment for a better outcome.


  Conclusion Top


Indolent mucormycosis cases might be difficult to recognize in its early presentation; the necessity of better awareness of the subtle signs of headache with facial paresthesia in patients presenting in primary care or emergency is vital to insure better outcomes in such patients.

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

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Spellberg B, Edwards J Jr., Ibrahim A. Novel perspectives on mucormycosis: Pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556-69.  Back to cited text no. 1
    
2.
Spellberg B, Walsh TJ, Kontoyiannis DP, Edwards J Jr., Ibrahim AS. Recent advances in the management of mucormycosis: From bench to bedside. Clin Infect Dis 2009;48:1743-51.  Back to cited text no. 2
    
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Ibrahim AS, Spellberg B, Walsh TJ, Kontoyiannis DP. Pathogenesis of mucormycosis. Clin Infect Dis 2012;54 Suppl 1:S16-22.  Back to cited text no. 3
    
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Waness A, Dawsari GA, Al Jahdali H. The rise of an opportunistic infection called “Invasive Zygomycosis”. J Glob Infect Dis 2009;1:131-8.  Back to cited text no. 4
    
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Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54 Suppl 1:S23-34.  Back to cited text no. 5
    
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Nithyanandam S, Jacob MS, Battu RR, Thomas RK, Correa MA, D'Souza O. Rhino-orbito-cerebral mucormycosis. A retrospective analysis of clinical features and treatment outcomes. Indian J Ophthalmol 2003;51:231-6.  Back to cited text no. 6
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Prakash H, Chakrabarti A. Global epidemiology of mucormycosis. J Fungi (Basel) 2019;5. pii: E26.  Back to cited text no. 7
    
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Mignogna MD, Fortuna G, Leuci S, Adamo D, Ruoppo E, Siano M, et al. Mucormycosis in immunocompetent patients: A case-series of patients with maxillary sinus involvement and a critical review of the literature. Int J Infect Dis 2011;15:e533-40.  Back to cited text no. 8
    
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Celis-Aguilar E, Burgos-Páez A, Villanueva-Ramos N, Solórzano-Barrón J, De La Mora-Fernández A, Manjarrez-Velázquez J, et al. An emergent entity: Indolent mucormycosis of the paranasal sinuses. a multicenter study. Int Arch Otorhinolaryngol 2019;23:92-100.  Back to cited text no. 9
    
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Wadi J, Denning DW. Burden of Serious Fungal Infections in Jordan. J Fungi (Basel) 2018;4. pii: E15.  Back to cited text no. 10
    
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Roden MM, Zaoutis TE, Buchanan WL, Knudsen TA, Sarkisova TA, Schaufele RL, et al. Epidemiology and outcome of zygomycosis: A review of 929 reported cases. Clin Infect Dis 2005;41:634-53.  Back to cited text no. 11
    
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Skiada A, Pagano L, Groll A, Zimmerli S, Dupont B, Lagrou K, et al. Zygomycosis in Europe: Analysis of 230 cases accrued by the registry of the European Confederation of Medical Mycology (ECMM) Working Group on Zygomycosis between 2005 and 2007. Clin Microbiol Infect 2011;17:1859-67.  Back to cited text no. 12
    
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Lanternier F, Dannaoui E, Morizot G, Elie C, Garcia-Hermoso D, Huerre M, et al. A global analysis of mucormycosis in France: The RetroZygo study (2005-2007). Clin Infect Dis 2012;54 Suppl 1:S35-43.  Back to cited text no. 13
    
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Bitar D, Van Cauteren D, Lanternier F, Dannaoui E, Che D, Dromer F, et al. Increasing incidence of zygomycosis (mucormycosis), France, 1997-2006. Emerg Infect Dis 2009;15:1395-401.  Back to cited text no. 14
    
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Katragkou A, Walsh TJ, Roilides E. Why is mucormycosis more difficult to cure than more common mycoses? Clin Microbiol Infect 2014;20 Suppl 6:74-81.  Back to cited text no. 15
    
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Cagatay AA, Oncü SS, Calangu SS, Yildirmak TT, Ozsüt HH, Eraksoy HH. Rhinocerebral mucormycosis treated with 32 gram liposomal amphotericin B and incomplete surgery: A case report. BMC Infect Dis 2001;1:22.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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