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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 23  |  Issue : 2  |  Page : 83-86

Facial nerve palsy as infrequent presentation in patients with rhino-orbital–cerebral-mucormycosis: A case report of two cases


1 Department of ENT, King Fahad University Hospital, Khobar, Saudi Arabia
2 Department of ENT, KFSH, Dammam, Saudi Arabia
3 Department of Medical Imaging, KFSH, Dammam, Saudi Arabia

Date of Submission06-Sep-2020
Date of Decision23-Oct-2020
Date of Acceptance21-Nov-2020
Date of Web Publication10-Jun-2021

Correspondence Address:
Dr. Marwah Aljahli
King Fahad University Hospital, Khobar
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sjoh.sjoh_41_20

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  Abstract 


Rhino-orbital-cerebral-mucormycosis (ROCM) is a rare aggressive and fatal infection. Its uncommon clinical encounter and mostly seen in immunocompromised patients, especially uncontrolled diabetic mellites patients. Symptoms of ROCM include nasal stuffiness and discharge, headache, retro-orbital pain, orbital or facial swelling, ophthalmoplegia, and visual loss. Although cranial nerve involvement is not common, palsy of the facial nerve is extremely rare finding. Two uncontrolled diabetic patients presented to our outpatient clinic with rapidly progressive sinusitis and unilateral facial nerve palsy in which further evaluation were proven to be mucormycosis. Facial nerve palsy in diabetic patient could be a red flag and must be investigated for underlying mucormycosis. An early diagnosis and treatment are very important for a favorable outcome in ROCM.

Keywords: Diabetes complication, diabetic ketoacidosis, facial palsy, rhino orbital cerebral mucormycosis


How to cite this article:
Aljahli M, Alhassan F, Almomen A, Molani F. Facial nerve palsy as infrequent presentation in patients with rhino-orbital–cerebral-mucormycosis: A case report of two cases. Saudi J Otorhinolaryngol Head Neck Surg 2021;23:83-6

How to cite this URL:
Aljahli M, Alhassan F, Almomen A, Molani F. Facial nerve palsy as infrequent presentation in patients with rhino-orbital–cerebral-mucormycosis: A case report of two cases. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2021 [cited 2021 Jul 23];23:83-6. Available from: https://www.sjohns.org/text.asp?2021/23/2/83/318132




  Introduction Top


Mucormycosis is a progressive and potentially fetal mycotic infection. It is caused by filamentous Mucorales fungi, most commonly Rhizopus species. Mucorales are considered an opportunistic infection. It is usually found in the soil and respiratory tract of healthy individuals. The route of transmission is by spore's inhalation. It is the most common invasive fungal infection after Aspergillus and Candida. Invasion occurs directly and spread rapidly from the nose to paranasal sinuses. Then, it erodes through the orbit and skull base into the brain causing orbital and cerebral infections, respectively.[1] Mucormycosis can manifest in a different clinical syndrome depending on the site of the infection. Rhino-orbital-cerebral-mucormycosis (ROCM) is the most common form, accounting for about 30%–50% of cases.[1],[2]

Although it is rare, ROCM is a life-threatening fungal infection, frequently seen in immunocompromised patients.[1] Moreover, the most common predisposing factor is diabetic mellites (36%) followed by hematological malignancy (17%) and solid organ or hematopoietic cell transplantation (12%).[3] Typical presentation of ROCM includes nasal stuffiness and discharge, headache, retro-orbital pain, orbital or facial swelling ophthalmoplegia, and visual loss. Cranial nerve involvement is unfrequently seen, yet if present it indicates a severe infection with grave prognosis. Facial nerve palsy is an unusual but significant presentation of mucormycosis. Unfortunately, it could be misdiagnosed as cerebral vascular accident which may delay the treatment.[4]

Early and accurate diagnosis with adequate management are highly required to achieve a better outcome.[2],[4]


  Case Reports Top


Case 1

A 47-year-old female with diabetes presented with chief complain of swelling, pain in the right side of the face, and right eye loss of vision. The patient's symptoms started 3 weeks back when she had an upper teeth infection which was treated oral antibiotics. Later, the patient started to have severe epigastric pain and repeated episodes of vomiting. After further evaluation, she was diagnosed with diabetic ketoacidosis (DKA) with suspected mucormycosis. On examination, the patient was conscious, alert, and oriented with a normal gait. In addition, she had black necrotic debris in the nasal cavity along with multiple ulcers in the hard palate and upper incisors. Ophthalmic examination revealed a right eye complete loss of vision associated with fixed dilated pupil and paralysis of all extraocular muscles. Other significant clinical features observed were decreased sensation on the right side of the face, drooping of the mouth corner, drooling of saliva, and the absence of wrinkles in the right half of the forehead. Accordingly, the House Brackman grade was VI unilateral facial nerve palsy. Cranial nerve examination revealed the involvement of II, III, IV, and VI cranial nerves. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain revealed a right maxillary and bilateral sphenoid inflammatory sinusitis [Figure 1]a and [Figures 1]b. CT Brain venogram showed right-sided cavernous sinus and superior ophthalmic vein thrombosis [Figure 2]. The patient started on insulin infusion, Amphotericin B (liposomal) 10 mg/kg daily, posaconazole 200 mg in addition to ceftriaxone and clindamycin after septic workup. She underwent urgent functional endoscopic sinus surgery. Intraoperative endoscopy showed nasal hyphae and surgical debridement was done. Histopathology report was suggestive of mucormycosis. The patient shifted to the intensive care unit for further management. Unfortunately, the patient's status deteriorated and she died after 1 month.
Figure 1: (a) Computed tomography without contrast showing moderate mucosal thickening of the right maxillary antrum with extension into the right nasal cavity. The antrum shows complete opacification with hyperdense contents, (b) Axial Computed tomography without contrast at the level of the posterior fossa shows interval development of low attenuation of the right side of the pons (arrow). The right sphenoid sinus shows mucosal thickening with hyperdense contents indicating fungal infection (black arrowhead)

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Figure 2: Computed tomography venogram at the level of the cavernous sinus shows the absence of enhancement of the right cavernous sinus (arrow) consistent with thrombosis

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Case 2

A 30-year-old male poorly controlled diabetic was referred to our hospital presenting with headache, fever, right-sided facial pain and numbness, and inability to open right eye. On examination, the patient was unable to open his right eye and there was a mild maxillary tenderness. In addition, a big necrotic ulcer was noted in the hard palate [Figure 3]a. Ophthalmic examination showed a decrease visual acuity in the right eye associated with ptosis, fixed dilated pupil, and restricted extraocular movements. The left eye was apparently normal. Furthermore, a cranial nerve evaluation revealed drooping of the angle of the mouth, drooling of saliva, absence of wrinkles in the right half of the forehead [Figure 3]b, and the absence of corneal sensation with no perception of light. With the aid of nasal endoscopy, an inflamed black mucosa was noted over the middle turbinate, and the inferior turbinate was normal. The patient was started on Amphotericin B (liposomal) 10 mg/kg daily IV cefoperazone, sulbactam, and metronidazole with an insulin infusion.
Figure 3: (a) Showing a necrotic ulcer was in the hard palate, (b) showing right LMN facial palsy

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CT scan, MRI, and MR venogram confirmed the diagnosis of acute ROCM with cavernous sinus thrombosis. Under general anesthesia, the patient underwent uneventful endoscopic endonasal debridement. The black necrotic tissues noted in the nasal cavity, ethmoidal sinuses, and maxilla, was debrided after a nasal biopsy. The biopsy specimens were sent for fungal cultures and histopathology. Microscopic examination revealed a broad, branched hypha, which was consistent with mucormycosis. Despite the grave prognosis, he has gradually improved after 6 weeks of intensive medical and surgical management. The patient was discharged home.


  Discussion Top


Mucormycosis is a rare clinical encounter with high mortality if left untreated. Its uncommon infection and mostly seen in immunocompromised patients, especially uncontrolled diabetic mellites patients. Moreover, high glucose, high iron, and high acidic environment are a favorable circumstance for fungal growth.[1],[5]

Rhino cerebral, pulmonary, gastrointestinal, cutaneous, central nervous system, and disseminated infection are the most common manifestation of mucormycosis.[6] As mention before, ROCM is the most common type and occurs mostly in patient with uncontrolled or poorly controlled diabetes mellitus. Originating from the nasal cavity, the infection spreads rapidly into the sinuses, palate, pharynx, and orbit. Furthermore, if it extends to the brain and meninges, it can result in cerebritis or brain abscess. The hallmark of Mucormycosis is angioinvasion, hypha occlude the vessels causing thrombosis and ischemia. Ultimately, it leads to the characteristic black necrotic areas in the nasal cavity and paranasal sinuses.[1],[2] Clinical manifestation includes nasal obstruction, bloody nasal discharge, black eschar on the palatal or nasal mucosa, facial pain, and swelling. Furthermore, chemosis, ptosis, proptosis, ophthalmoplegia, and blindness can present in advanced stages which indicate orbital invasion.[7] Blindness occurs either due to the central retina artery occlusion or direct involvement of the optic nerve. Multiple cranial nerve palsy (cranial nerves II, III, IV, and VI) also have been reported.[7],[8] All these findings are consistent with our cases and are suggestive of ROCM.

The incidence of facial nerve palsy in poorly controlled diabetic patients with ROCM is 11%.[9] Our patients had right side facial nerve palsy. Although the exact mechanism of facial nerve involvement is unknown and no obvious pathology has been identified. Bakshi S.suggested that if the infection spread through the pterygopalatine fossa and reaches the infratemporal fossa, it may affect the facial nerve as it exits the stylomastoid foramen. On the other hand, some reports indicate that facial nerve may be affected directly by spreading of infection through the  Eustachian tube More Details or by a vascular invasion of the middle ear; this was not seen in our patients as all them had no ear complaints and normal ear examination.[1] Pathology of resistance arteries in diabetic patients may cause edema and localize facial nerve ischemia. This would compromise the blood supply to the nerve leading to palsy. However, the involvement of the facial nerve could be incidental and may be idiopathic as in Bell's palsy.[1],[2]

Radiographically, CT of the sinuses revealed mucosal thickening, fluid filling in paranasal sinuses, destruction of periorbital tissues, and bony walls of paranasal sinuses. However, these findings are nonspecific, it may be useful in identifying the progression of the infection and in guiding surgical debridement. MRI is more sensitive than CT. It is useful for detecting orbital and cerebral involvement. Diagnosis of mucormycosis is difficult. Unlike other fungal infection, definitive diagnosis is made by a combination of a positive Mucorales culture and microscopic evidence. Detecting irregular, broad, nonseptate hyphae is pathognomonic of mucormycosis.[10],[11]

Successful treatment of ROCM includes the reversal of the underlying predisposing factors, early surgical debridement of the infected tissues, and rapid initiation of effective antifungal therapy. It is important to reverse any underlying predisposing factor, such as restoring blood sugar and reaching euglycemia with normal acid-base state in DKA patient. Surgical interventions could be extreme depending on the patient status and the severity of the infection.[10] Its extent from debridement of nasal mucosa to ethmoidectomy, sphenoidectomy, and medial maxillectomy to radical maxillectomy with orbital exenteration by either endoscopic or open approach. First-line antifungal treatment is a high dose of lipid-based amphotericin B 1 mg/kg/day, based on the patient's renal status and other underlying conditions.[1] Surgical intervention along with medical treatment improves the survival rate from 57.5% to 78% when compared to medical management alone.[12] Van der et al. suggested that any diabetic patient with DKA who presents with clinical and radiographic findings of rhinosinusitis should be suspected as having mucormycosis until proven otherwise.[13]

Despite of surgical intervention and systemic therapy with antifungal agents, morbidity is still rising. A high index of clinical suspicion is required along with early detection of the disease and prompt management to improve the outcome.[1],[4]


  Conclusion Top


Rhino-orbital-cerebral-mucormycosis is a rapidly progressive and fatal fungal infection. An early diagnosis and adequate treatment are the keys to improve the outcomes. Facial nerve palsy in diabetic patients is a significant and alerting sign. A high index of suspicion in such patient thorough history, supported by detailed examination confirm by biopsy is required. A combination therapy including immediate control of predisposing factors, surgical debridement, and appropriate antifungal therapy is mandatory to minimize the fatal outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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.
Bakshi S. An unusual cause for facial nerve palsy: Mucormycosis. Int J Diabetes Dev Ctries 2016;36:385-8.  Back to cited text no. 1
    
2.
Shekar V, Sikander J, Rangdhol V, Naidu M. Facial nerve paralysis: A case report of rare complication in uncontrolled diabetic patient with mucormycosis. J Nat Sci Biol Med 2015;6:226-8.  Back to cited text no. 2
    
3.
Yohai RA, Bullock JD, Aziz AA, Markert RJ. Survival factors in rhino-orbital-cerebral mucormycosis. Surv Ophthalmol 1994;39:3-22.  Back to cited text no. 3
    
4.
Mane R, Patil B, Mohite A, Mohanty R. Facial nerve palsy: An unusual presentation in patients with rhino cerebral mucormycosis. Indian J Otolaryngol Head Neck Surg 2019;71:2110-3.  Back to cited text no. 4
    
5.
Aloosi S. Facial nerve palsy as frequent presentation in patient with rhinocerebral mucormycosis. J Oral Dent Res 2020;5:55-69.  Back to cited text no. 5
    
6.
Topazian R, Goldberg M, Hupp J. Oral and Maxillofacial Infections. 4th ed. Philadelphia, PA: Saunders; 2002.  Back to cited text no. 6
    
7.
Sachdeva K. Rhino-oculo cerebral mucormycosis with multiple cranial nerve palsy in diabetic patient: Review of six cases. Indian J Otolaryngol Head Neck Surg 2013;65:375-9.  Back to cited text no. 7
    
8.
Munir N, Jones NS. Rhinocerebral mucormycosis with orbital and intracranial extension: A case report and review of optimum management. J Laryngol Otol 2007;121:192-5.  Back to cited text no. 8
    
9.
Ferguson BJ. Mucormycosis of the nose and paranasal sinuses. Otolaryngol Clin North Am 2000;33:349-65.  Back to cited text no. 9
    
10.
Lam SC, Yuen HK. Management of bilateral rhino-orbital cerebral mucormycosis. Hong Kong Med J 2019;25:408-9.  Back to cited text no. 10
    
11.
Gen R, Horasan E, Vaysoğlu Y, Arpaci R, Ersöz G, Ázcan C. Rhino-orbito-cerebral mucormycosis in patients with diabetic ketoacidosis. J Craniofac Surg 2013;24:e144-7.  Back to cited text no. 11
    
12.
Yadav S, Goel A. Rhino-orbital mucormycosis – A case report. Int J Pediatr Otorhinolaryngol Extra 2010;5:9-12.  Back to cited text no. 12
    
13.
Van der Westhuijzen AJ, Grotepass FW, Wyma G, Padayachee A. A rapidly fatal palatal ulcer: Rhinocerebral mucormycosis. Oral Surg Oral Med Oral Pathol 1989;68:32-6.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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