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Table of Contents   
CASE REPORT  
Year : 2020  |  Volume : 31  |  Issue : 4  |  Page : 652-655
Rhinocerebral maxillary mucormycosis: A palatal ulcer


1 Department of Oral Medicine and Radiology, AME'S, Dental College and Hospital, Raichur, Karnataka, India
2 Department of Orthodontia, AME'S, Dental College and Hospital, Raichur, Karnataka, India

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Date of Submission15-Mar-2018
Date of Decision26-Mar-2018
Date of Acceptance15-May-2018
Date of Web Publication16-Oct-2020
 

   Abstract 


Mucormycosis, caused by saprophytic fungi of the order Mucorales of the class zygomycetes, is a rare opportunistic fungal infection, which has a rapidly progressive and fulminant course with fatal outcome. Mucormycosis can result in an acute, rapidly advancing and occasionally fatal disease caused by different fungi typically found in the soil in association with decaying organic matter such as leaves, compost piles, or bread molds. Mucormycosis is not contagious and does not spread from person to person. We report a case of palatal perforation by rhino-maxillary mucormycosis in an immunocompromised patient. The patient was treated and had a good prognosis for such a fatal condition.

Keywords: Amphotericin B, palatal ulcer, rhinocerebral mucormycosis

How to cite this article:
V Ramesh D N, Anjum G, Rukmangada T, Patil N. Rhinocerebral maxillary mucormycosis: A palatal ulcer. Indian J Dent Res 2020;31:652-5

How to cite this URL:
V Ramesh D N, Anjum G, Rukmangada T, Patil N. Rhinocerebral maxillary mucormycosis: A palatal ulcer. Indian J Dent Res [serial online] 2020 [cited 2020 Oct 31];31:652-5. Available from: https://www.ijdr.in/text.asp?2020/31/4/652/298401



   Introduction Top


Mucormycosis is an emerging angioinvasive infection caused by the ubiquitous filamentous fungi of the Mucorales order of the class of zygomycetes.[1] It is an aggressive, frequently fatal invasive fungal infection that can develop in immunocompromised patients.[2] It is usually documented in patients with uncontrolled diabetes. Mucormycosis is often recognized as a triad of symptoms, such as uncontrolled diabetes mellitus, periorbital infection, and meningoenchephalitis.[3] The prognosis is poor, with severe sequelae and high mortality even in patients with a prompt diagnosis and correct treatment.[4]


   Case Report Top


A 23-year-old male presented with a chief complaint of pain in the posterior upper jaw region with foul odor for two months. History revealed that the pain was sudden in onset, severe, throbbing, and continuous in nature. It was associated with an extraoral swelling on left middle third of the face, followed by an intraoral ulcerative growth on the left side of the palate. Medical history revealed that the patient had dengue fever 15 days prior for which patient was administered high doses of antibiotics. There was associated nasal congestion and purulent discharge from the left side of the nose, loss of sensation in the infraorbital region and upper lip on the same side. The pain was moderate, aggravated on bending the head and chewing food. Detailed medical history revealed that the patient was not satisfied with the treatment regimen for dengue, henceforth patient had changed three physicians for the same, with three different treatment strategies. He had the habit of chewing tobacco (gutkha) for 1 year.

After general examination, the patient was found to be moderately built, moderately nourished but febrile (temperature 38.9°C). The patient was advised routine blood investigations in which RBS was 240 mg/dl. Later, the patient was subjected to blood investigations which revealed raised ESR (34 mm/1st h) and was advised fasting and postprandial blood sugar level by which patient was declared to be type 2 diabetic. The patient was unaware of his diabetic status. Extraoral findings revealed ptosis of the left eye and swelling over the left maxillary sinus region with paresthesia in the left half of the upper lip and bilateral inflammatory submandibular lymphadenitis.

On intraoral examination, an infiltrating ulcer approximately 2 cm × 3 cm in diameter with irregular borders was appreciated over the hard palate. The ulcer was covered over by necrotic slough and areas of bone denudation on the periphery in the anterior aspect of the ulcer [Figure 1]. Ulcer was nontender with few areas of erythema surrounding the ulcer. Considering the patient's medical history and a rapidly proliferating ulcer denuding the underlying bone, and clinical presentation a provisional diagnosis of deep mycotic infection of palate was made. The differential diagnosis of mucormycosis, Wegener's granulomatosis, squamous cell carcinoma, and necrotizing sialometaplasia was made [Table 1].
Figure 1: Intraoral photograph of the patient shows ulcerative lesion covered with yellowish slough with everted borders

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Table 1: Differential diagnosis for rhinocerebral mucormycosis

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Radiological investigations

Imaging studies revealed diffuse haziness over the left maxillary antrum extending into the left nasal cavity. Computed tomography (CT) scan in axial, coronal and sagittal sections revealed left maxillary sinus opacification [Figure 2], with moderate mucosal thickening of left maxillary sinus and blockage of left ethmoid infundibula; there was a sclerotic reaction of the bone [Figure 3]. However, the “Black Turbinate sign” is a characteristic early finding of mucormycosis seen on magnetic resonance imaging and an incisional biopsy was advised. Hard tissue specimen along with the adjacent soft tissue was excised under local anesthesia and sent for histopathological examination. The histopathological report suggests the presence of numerous aseptate fungal hyphae branching at 90° with neutrophilic infiltrate invading the smaller blood vessels (periodic acid–Schiff [PAS] staining). Grocott's modified silver methenamine special staining technique further identified these nonseptate branching hyphae of mucormycosis. Based on history, clinical findings, laboratory investigations, radiological investigations, and histopathological reports a final diagnosis of maxillary mucormycosis was established.
Figure 2: Left maxillary sinus in coronal and axial section

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Figure 3: Postoperative image after surgical debridement

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The patient was hospitalized, and physicians controlled blood sugar levels with insulin. An informed written consent was obtained. The necrotic bone along with 1 cm of adjacent bone was excised under general anesthesia. The patient was administered amphotericin-B 0.8 mg/kg/day intravenously for 2 weeks. Surgical debridement was done in higher center (on the 10th day of hospitalization). However, orbital plate of maxilla was preserved, nasal endoscopy was done after a week. The patient was under observation, and two weeks following surgery, the whole necrotic material of the hard palate got sloughed off, exposing the underlying bone, which was brownish in color [Figure 4]. Surgical debridement was repeated, and further amphotericin-B was continued for two more weeks. Electrolyte corrections were done as advised by nephrologist. Ophthalmologist opinion was taken and a diagnosis of healed neurotrophic ulcer was made. The patient was very concerned regarding esthetics and function following extraction of teeth in the involved region. An obturator was therefore fabricated in order to enable him to feed on solid and semisolid food. He was able to carry out routine activities of daily living and was called for monthly checkups for a year following surgery [Figure 5]. Morbidity from the treatment was reduced since diagnosis was prompt followed by definitive treatment.
Figure 4: (a) Preoperative image. (b) Postoperative image

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Figure 5: Computed tomography image showing mucoperiosteal thickening

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   Discussion Top


Mucormycosis is the term used to refer to a rare and lethal fungal infection caused by the family of Mucoraceae, which belongs to the class of phycomycetes or zygomycetes.[5],[6]

These saprophytic fungi exist widely in nature, and their spores may be found in soil, air, spoilt food, and other decaying organic material. Due to their low virulence potential, it may be present in the nasal mucosa of normal people as a commensal.[7] These fungi are ubiquitous, usually harmless and become pathogenic in man under certain conditions such as immunosuppression, diabetic acidosis, antibiotic, steroids and cytotoxic therapy, with other predisposing factors such as malignancy, burns, malnutrition, renal failure, and blood dyscrasias.[8]

Clinical variants[9] Eisenberg etal. described six clinical variants:

  • Rhinocerebral (rhinomaxillary)
  • Pulmonary
  • Cutaneous
  • Gastrointestinal
  • Central nervous system
  • Disseminated type.


Pathogenesis

Everyone is exposed to and inhales the spores; the nasociliary system transports these spores down in the pharynx to be cleared by the gastrointestinal tract. The spores inhaled into the lungs are cleared by the phagocytes. In the susceptible individuals, the infection begins in the middle and inferior nasal turbinates. From the nasal cavity, the infection spreads to the paranasal sinus region and then to the retro-orbital region through direct extension or either through ethmoidal, lachrymal, and angular vessels. Infection can also spread to the brain through the cribriform plate and the ophthalmic vessels. Following spread, the function of the cranial nerves II, III, IV, and VI may be impaired and can lead to ptosis, proptosis, pupillary dilatation, visual loss, periorbital cellulitis, and numbness.[10]

This condition was first described by Paltauf in 1885 in human beings,[8] but only in the past two decades, the condition is being reported with increasing frequency. In dentistry, this condition gains increasing interest because of its first manifestation in the facial and oral tissue. Oral manifestations are usually in the palate where ischemic necrosis of the mucoperiosteum with bony denudation may occur,[11] similar signs and symptoms were reported in our case.

Mucormycosis that involves the nose and the paranasal sinus generally begins with signs and symptoms of an acute bacterial rhinosinusitis. When it hits the orbit, it causes cellulitis, proptosis, ophthalmoplegia, and eventually blindness. If the infection spreads beyond the first site of infection, then the central nervous system can be reached. Usually, in these cases, there are signs of bone destruction in CT scans [Figure 6].[12] In the present case, the patient had ptosis of the left eyelid with paraesthesia in the left half of the upper lip, discharge from the nose, and bilateral inflammatory submandibular lymphadenitis and necrotic eschar, these findings were concomitant with previous case reports.[13],[14],[15],[16] Radiological features include nodular thickening of sinus mucosa, cloudy sinusitis without fluid levels, and spotty destruction of bony walls of paranasal sinuses were noted.
Figure 6: Coronal computed tomography image showing the depth of involvement as shown in the present case

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CT findings show opacification of paranasal sinuses, thickening of sinus mucosa, and erosions of the bony walls[17] which were present in the radiographs taken except for the destruction of walls in CT. Later, it was confirmed by histopathological examination, PAS which showed irregularly shaped, broad, hyphae that branch in the right angle.

Recent studies emphasized the importance of an aggressive surgical treatment that may involve exenteratio orbitae, partial or total maxillectomy, partial rhinectomy, and other techniques. Exclusively medically treated patients usually present a less favorable prognosis in relation to severity and aggressiveness of the disease. The timing of surgery does not seem to play a fundamental role in prognosis; most patients are initially treated with drugs and surgically treated with in a week from diagnosis if it is possible. Surgical timing is strictly related to the medical treatment response and the general conditions of the patient.[12] As the disease progresses with alarming rapidity, prompt, and aggressive therapy is essential. Successful treatment of mucormycosis consists of aggressive and repeated surgical debridement of necrotic tissue, systemic antifungal therapy and immediate control of underlying systemic diseases along with continuous monitoring of amphotericin-induced nephrotoxicity.[8]


   Conclusion Top


A prompt diagnosis is crucial to improve the survival and reduce the sequela of patients with Rhinocerebral maxillary mucormycosis.[4] Prognosis can be improved with early diagnosis, modern imaging techniques, and therapy progress, both surgical and pharmacologic.[12] The gold standard of the medical treatment is amphotericin B, although the new antifungal generation is obtaining promising results with fewer adverse effects.

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

1.
Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh TJ, Kontoyiannis DP, et al. Epidemiology and clinical manifestations of mucormycosis. Clin Infect Dis 2012;54 Suppl 1:S23-34.  Back to cited text no. 1
    
2.
Breiman A, Sadowsky D, Friedman J. Mucormycosis. Discussion and report of a case involving the maxillary sinus. Oral Surg Oral Med Oral Pathol 1981;52:375-8.  Back to cited text no. 2
    
3.
Vijayabala GS, Annigeri RG, Sudarshan R. Mucormycosis in a diabetic ketoacidosis patient. Asian Pac J Trop Biomed 2013;3:830-3.  Back to cited text no. 3
    
4.
Martín-Moro JG, Calleja JM, García MB, Carretero JL, Rodríguez JG. Rhinoorbitocerebral mucormycosis: A case report and literature review. Med Oral Patol Oral Cir Bucal 2008;13:E792-5.  Back to cited text no. 4
    
5.
Ferguson BJ. Mucormycosis of the nose and paranasal sinuses. Otolaryngol Clin North Am 2000;33:349-65.  Back to cited text no. 5
    
6.
Uçkay I, Chalandon Y, Sartoretti P, Rohner P, Berney T, Hadaya K, et al. Invasive zygomycosis in transplant recipients. Clin Transplant 2007;21:577-82.  Back to cited text no. 6
    
7.
Elinav H, Zimhony O, Cohen MJ, Marcovich AL, Benenson S. Rhinocerebral mucormycosis in patients without predisposing medical conditions: A review of the literature. Clin Microbiol Infect 2009;15:693-7.  Back to cited text no. 7
    
8.
Doni BR, Peerapur BV, Thotappa LH, Hippargi SB. Sequence of oral manifestations in rhino-maxillary mucormycosis. Indian J Dent Res 2011;22:331-5.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Garg R, Gupta VV, Ashok L. Rhinomaxillary mucormycosis: A palatal ulcer. Contemp Clin Dent 2011;2:119-23.  Back to cited text no. 9
[PUBMED]  [Full text]  
10.
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. 10
    
11.
Ramon Y, Oberman M, Horowitz I, Freedman A. Extensive maxillary s equestration resulting from rhinocerebralmucormycosis. J Oral Surg 1977;35:989-91.  Back to cited text no. 11
    
12.
Viterbo S, Fasolis M, Garzino-Demo P, Griffa A, Boffano P, Iaquinta C, et al. Management and outcomes of three cases of rhinocerebral mucormycosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:e69-74.  Back to cited text no. 12
    
13.
Tabachnick TT, Levine B. Mucormycosis of the craniofacial structures. J Oral Surg 1975;33:464-9.  Back to cited text no. 13
    
14.
Tugsel Z, Sezer B, Akalin T. Facial swelling and palatal ulceration in a diabetic patient. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:630-6.  Back to cited text no. 14
    
15.
Buhl MR, Joseph TP, Snelling BE, Buhl L. Temporofacial zygomycosis in a pregnant woman. Infection 1992;20:230-2.  Back to cited text no. 15
    
16.
Iatta R, Napoli C, Borghi E, Montagna MT. Rare mycoses of the oral cavity: A literature epidemiologic review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:647-55.  Back to cited text no. 16
    
17.
O'Neill BM, Alessi AS, George EB, Piro J. Disseminated rhinocerebral mucormycosis: A case report and review of the literature. J Oral Maxillofac Surg 2006;64:326-33.  Back to cited text no. 17
    

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Correspondence Address:
Dr. Gulnaaz Anjum
Department of Oral Medicine and Radiology, AME'fS Dental College and Hospital, Raichur, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdr.IJDR_234_18

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