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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 3  |  Page : 792-796

Rhinocerebral mucormycosis: a case report and literature review


1 Department of Pathology, Faculty of Medicine, Al-Azhar University, Cairo; Department of Pathology, Histopathology Consultant, Khamis MUshyte General Hospital, Egypt
2 Department of Pathology, Faculty of Medicine, Al-Azhar University, Cairo, Egypt
3 Department of Ophthalmology, Oculoplastic Consultant, Khamis MUshyte General Hospital
4 Department of Otolaryngology, & Head and Neck Surgery Consultant, Khamis MUshyte General Hospital

Date of Submission30-Oct-2019
Date of Decision30-Oct-2019
Date of Acceptance27-Nov-2019
Date of Web Publication10-Feb-2020

Correspondence Address:
MD Reda A Elhawary
Department of Pathology, Faculty of Medicine, Al-Azhar University, Cairo, 62433
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sjamf.sjamf_90_19

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  Abstract 


Mucormycosis is a rare rapidly progressing and lethal invasive fungal infection that involves the nose, paranasal sinuses, and orbit of the head and neck regions. Mucormycosis commonly affects patients with uncontrolled diabetes, especially those with ketoacidosis. The authors report a case of rhinocerebral mucormycosis in a 45-year-old diabetic patient who presented at Khamis Mushayt General Hospital, Aseer region, south of Saudi Arabia, with ketoacidosis, right nasal sinusitis, right orbital cellulitis, and loss of vision. Rhinocerebral mucormycosis is suspected clinically, so surgeon immediately did tissue biopsy. Mucormycosis fungal organisms were detected pathologically as well as in fungal culture. The patient responded well after aggressive surgical debridement with antifungal medication. The authors conclude that the diagnosis of rhinocerebral mucormycosis as early as possible is essential for a good therapy effect as well as better survival and limited morbidity of patients.

Keywords: fungal, ketoacidosis, mucormycosis, orbital, rhinocerebral


How to cite this article:
Elhawary RA, Abdelwahed MS, Odadi AS, Etwadi AA. Rhinocerebral mucormycosis: a case report and literature review. Sci J Al-Azhar Med Fac Girls 2019;3:792-6

How to cite this URL:
Elhawary RA, Abdelwahed MS, Odadi AS, Etwadi AA. Rhinocerebral mucormycosis: a case report and literature review. Sci J Al-Azhar Med Fac Girls [serial online] 2019 [cited 2020 Feb 29];3:792-6. Available from: http://www.sjamf.eg.net/text.asp?2019/3/3/792/278054




  Introduction Top


Mucormycosis (zygomycosis) is an emerging infection associated with high mortality and caused by infection with fungi in the order of Mucorales. Rhizopus spp. are the most common causative organisms. Most mucormycosis infections are life-threatening, and risk factors such as diabetic ketoacidosis are present in most cases, which can lead to complications [1]. Mucormycosis patients present with periorbital facial pain, facial cellulitis, proptosis, nasal ulcer and stuffiness, headache, and acute loss of vision [2]. A high index of clinical suspicion for mucormycosis as well as rapid surgical and medical intervention play a crucial role in the treatment of patients with rhinocerebral mucormycosis and are important for recovery of patients from such a devastating infection [3].


  Case report Top


A 45-year-old male patient presented at Khamis Mushayt General Hospital (KMGH), Aseer region, south of Saudi Arabia, with type 1 diabetes mellitus complicated with ketoacidosis without coma and also presented with purulent foul smelling, right nasal discharge and right nasal cavity crustation. We have approval ethical committee from Khamis Mushayt General Hospital as well as patient consent for publication. The right eye showed swelling, proptosis, and loss of vision.

The clinical differential diagnosis includes fungal mucormycosis, midline granuloma, and midline malignancy.

MRI examination without contrast of the brain showed marked right orbital cellulitis with right proptosis, bilateral mastoiditis and nasal, ethmoidal, frontal, maxillary, and sphenoidal mucosal thickening with acute sinusitis of right maxillary and sphenoidal sinuses. No obvious intracranial extension was detected.

Multislice postcontrast computed tomography study of the orbits showed right orbital edema and swelling of the ocular muscles with extraconal, intraconal fat stranding, and small collection on the medial orbital wall. Evidence of exophthalmos with abnormal shape of the globe (slightly coned posteriorly) and evidence of chronic pansinusitis were reported ([Figure 1]).
Figure 1 (a) MRI showing right orbital cellulites and pansinusitis; (b) computed tomography showing right orbital cellulites and pansinusitis.

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A nasal biopsy was taken and sent for histopathological and microbiological evaluations.

Pathological gross examination indicated multiple soft black grayish tissue pieces.

Pathological microscopic examination showed nasal mucosa with irregular tissue fragments composed of acute neuroinflammatory neutrophilic reaction, admixed with bloody proteineous crustation and heavy fungal infiltrate of broad, right-angle branching, nonseptate ribbon-like hyphae that invade the blood vessels ([Figure 2] and [Figure 3]). There was also associated focal giant cell reaction and mature bony trabeculae with no evidence of malignancy. Periodic Acid Schiff (PAS) stain showed heavy fungal infiltrate of broad, right-angle branching, nonseptate ribbon-like hyphae with angioinvasion ([Figure 2]).
Figure 2 (a) Histopathological examination showing heavy fungal infiltrate of broad, right-angle branching, nonseptate ribbon-like hyphae (hematoxylin and eosin stain, ×200); (b) histopathological examination showing heavy fungal infiltrate of broad, right-angle branching, nonseptate ribbon-like hyphae (PAS stain, ×400).

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Figure 3 (a) Heavy fungal infiltrate of mucormycosis with angioinvasion as fungal hyphae present within the wall and inside the lumen of blood vessel (hematoxylin and eosin stain, ×100); (b) heavy fungal infiltrate of mucormycosis with angioinvasion as fungal hyphae present within the wall and inside the lumen of blood vessel (hematoxylin and eosin stain, ×200).

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Microbiological examination colonies grew rapidly, filling the culture plate within a few days. White growth was first observed and then colonies became grayish to brownish in a cottony or woolly pattern. Hyphae were broad (6–15 µm), aseptate, hyaline, and ribbon like. Sporangiophores were long, unbranched, and branched with terminal, round, spore-filled sporangia ([Figure 4]).
Figure 4 (a) Culture showed long, unbranched, and branched sporangiophores with terminal, round, spore-filled sporangia (Giemsa stain, ×100); (b) culture showed long, unbranched, and branched sporangiophores with terminal, round, spore-filled sporangia (Giemsa stain B, ×400).

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Aggressive surgical debridement was performed by otolaryngology & head and neck surgeon and ophthalmic surgeon together. Surgeons remove necrotic tissue, with local and intravenous injection of antifungal amphotricin-B. After operation, oral antifungal amphotricin-B and insulin medications were prescribed. The patient responded well to therapy and was completely cured from fungal infection after 5 months of antifungal treatment; currently, the patient is on regular follow-up at the endocrine outpatient clinic for controlling diabetes mellitus by medications.


  Discussion Top


Mucormycosis is a dangerous, infrequent invasive fungal infection and one of the most aggressive and lethal invasive mycoses [4]. The risk factors for mucormycosis development include uncontrolled diabetes (especially in patients with ketoacidosis) and hematological malignancies. Patients with renal failure, organ transplant, on immunosuppressive therapy, liver cirrhosis, and AIDS have also been reported to have mucormycosis [2],[5].

Mucorales species are vasotropic organisms leading to necrosis of the affected tissues. Clinically, the spectrum of presentation of mucormycosis ranges from rhinocerebral, pulmonary, cutaneous, gastrointestinal, to disseminated and frequently fatal infections, especially in immunocompromised hosts [6].

In diabetic patients, rhinocerebral mucormycosis is the most common form of mucormycosis. The infection develops by inhalation of fungal sporangiospores into the paranasal sinuses. The invading fungus germinates and may spread inferiorly to invade the palate, posteriorly to invade the sphenoid sinus, laterally into the cavernous sinus to invade the orbits, or cranially to invade the brain, which may be fatal [7].

In patients with diabetes mellitus, especially with elevated blood sugar levels, the spores germinate, hyphae develop, and involve blood vessels that become thrombosed, resulting in tissue necrosis; as fungi continue to grow, devitalized tissue occurs, with further damage to surrounding tissues [7].

Our patient had uncontrolled diabetes complicated with ketoacidosis, which is a well-known predisposing factor for mucormycosis, along with spread of lesion from nasal sinuses into adjacent tissues.

Signs and symptoms that suggest rhinocerebral mucormycosis in susceptible patients include unilateral periorbital facial pain, facial cellulitis, orbital inflammation, edema of the eyelid, proptosis, acute changes of ocular motility, nasal discharge, nasal stuffiness, headache, and acute loss of vision [2].

Blindness develops if blood supply to the eye is affected when invasion of retinal artery occurs. A black necrotic eschar is considered the hallmark of mucormycosis [2].

Histological study with hematoxylin and eosin stain showed the presence of broad nonseptate hyphae with branching at a right angle extended to be found in the wall and lumen of existing blood vessels along with areas of necrosis [8].

In terms of the differential diagnosis, rhinocerebral mucormycosis may be initially similar to bacterial sinusitis and may mimic malignant neoplasms. Rhinocerebral mucormycosis may be indistinct from allergic fungal sinusitis caused by phaeohyphomycoses occurring in individuals with histories of allergic rhinitis, immunoglobulin E level elevation, nasal polyps, and recurrent chronic sinusitis. Allergic fungal sinusitis slowly advances over months to years; although it may lead to proptosis and a large rhinocerebral mass, it does not invade tissue or meninges [9].

Aspergillosis can cause a similar disease, with central nervous system invasion, and carries a poor prognosis. An important difference is that itraconazole may play a role in the treatment of aspergillosis fungal infection. Aspergillosis is characterized by narrow, septate hyphae with narrow-angle branching [9],[10].

In this case, the fungus was identified by hematoxylin and eosin stain, PAS stain, and microbiological examination study.

The treatments of mucormycosis should be fast and aggressive because by the time even the presumptive diagnosis is made, the patient may suffer significant tissue loss that cannot be reversed. Both surgical and medical treatments are required in most patients. The treatment for such cases requires vigorous surgical debridement of the affected areas; however, medications play an important role. Antifungal medications are used to slow or prevent fungal spread; at the same time, other medications are used to treat any debilitating underlying diseases [11],[12].

Initial intravenous Amphotericin B is the usual drug of choice for antifungal treatment. Proper control of diabetes is mandatory in diabetic patients. Patients may need additional surgeries and usually need antifungal therapy for an extended time period (weeks to months) depending on the severity of the disease. The prognosis of mucormycosis is usually fair to poor; it depends on the health status of the patient, time taken for diagnosis and treatment, the patient’s response to treatments, and the complete debridement of the affected zone. In this patient, treatment included aggressive surgical debridement of the infected area in parallel with administration of amphotericin B as it is the drug of choice in the treatment of mucormycotic infection [11],[12].

The patient was under observation for his response to treatment for 5 months until he was completely cured from fungal infection; regular follow-up continues for controlling diabetes mellitus.


  Conclusion Top


The current report emphasizes the importance of having a high index of suspicion when dealing with patients with diabetes mellitus presenting with orbital cellulites and prompt initiation of medical therapy along with surgical debridement for control of rhinocerebral mucormycosis. A delay in diagnosis can be fatal. Institution of surgical and medical therapy is critical in maximizing the likelihood of good outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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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–S34.  Back to cited text no. 1
    
2.
Reddy SS, Rakesh N, Chauhan P, Sharma S. Rhinocerebral mucormycosis among diabetic patients: an emerging trend. Mycopathologia 2015; 180:389–396.  Back to cited text no. 2
    
3.
Roden MM, Zaoutis TE, Buchanan WL, Knudsen TA, Sarkisova TA, Schaufele RL et al. Epidemiologyand outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis 2005; 41:634.  Back to cited text no. 3
    
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Kauffman CA, Malani AN. Zygomycosis: an emerging fungal infection with new options for management. Curr Infect Dis Rep 2007; 9:435.  Back to cited text no. 4
    
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Berdai MA, Labib S, Harandou M. Rhinocerebral mucormycosis complicating ketoacidosis diabetes. Presse Med 2016; 45:145–146.  Back to cited text no. 5
    
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Gonzalez CE, Rinaldi MG, Sugar AM. Zygomycosis. Infect Dis Clin North Am 2002; 16:895–914.  Back to cited text no. 6
    
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Hosseini SM, Borghei P. Rhinocerebral mucormycosis: pathways of spread. Eur Arch Otorhinolaryngol 2005; 262:932–938.  Back to cited text no. 7
    
8.
Sravani T, Uppin SG, Uppin MS, Sundaram C. Rhinocerebral mucormycosis: Pathology revisited with emphasis on perineural spread. Neurol India 2014; 62:383–386.  Back to cited text no. 8
    
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Nallapu V, Vuppalapati HB, Sambhana S, Balasankulu B. Rhinocerebralmucormycosis: a report of two cases. J Indian Acad Oral Med Radiol 2015; 27:147–151.  Back to cited text no. 9
    
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Jeffrey D. Jenks and Martin Hoenigl: treatment of aspergillosis. J Fungi (Basel) 2018; 4:98.  Back to cited text no. 10
    
11.
Sipsas NV, Gamaletsou MN, Anastasopoulou A, Kontoyiannis DP. Therapy of Mucormycosis. J Fungi 2018; 4:90.  Back to cited text no. 11
    
12.
Sun HY, Singh N. Mucormycosis: its contemporary face and management. Lancet Infect Dis 2011; 11:301–311.  Back to cited text no. 12
    


    Figures

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



 

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