Home About us Editorial board Ahead of print Current issue Archives Submit article Instructions Subscribe Search Contacts Login 
  • Users Online: 771
  • Home
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 27  |  Issue : 1  |  Page : 147-151

Rhinocerebral mucormycosis: A report of two cases


Department of Oral Medicine and Radiology, Government Dental College and Hospital, Hyderabad, Telangana, India

Date of Submission10-Jan-2015
Date of Acceptance14-Sep-2015
Date of Web Publication12-Oct-2015

Correspondence Address:
Hima Bindu Vuppalapati
Room: 109, Department of Oral Medicine and Radiology, Government Dental College and Hospital, Hyderabad - 500 012, Telangana
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.167142

Rights and Permissions
   Abstract 

Mucormycosis is an acute opportunistic infection caused by saprophytic fungus of class Phycomycetes order Mucorales and family Mucoracae, found in soil, bread molds, and decaying fruits and vegetables. Even though this fungus is ubiquitous in the nature, the disease is usually prevented by immune system and is therefore rare. Rhizopus, Rhizomucor, Absidia and Cunninghamella are the most common species associated with Mucormycosis. Diabetes, chronic kidney disease, desferroxamine use in dialysis, leukemia, lymphoma, immunocompromised state, burns and open wounds are the risk factors of mucormycosis. Out of six clinical entities of mucormycosis, rhinocerebral mucormycosis is the most common variety which includes three subtypes rhinomaxillary, rhino-orbital and rhino-orbitocerebral mucormycosis. Here are two case reports of Rhinocerebral mucormycosis of different subtypes emphasizing the role of early detection of the disease to limit the spread of infection and reducing the morbidity and mortality.

Keywords: Chronic kidney disease (CKD), diabetes, mucormycosis, rhinocerebral mucormycosis, rhinomaxillary mucormycosis, rhinoorbital mucormycosis, rhinoorbitocerebral mucormycosis, risk factors


How to cite this article:
Nallapu V, Vuppalapati HB, Sambhana S, Balasankulu B. Rhinocerebral mucormycosis: A report of two cases. J Indian Acad Oral Med Radiol 2015;27:147-51

How to cite this URL:
Nallapu V, Vuppalapati HB, Sambhana S, Balasankulu B. Rhinocerebral mucormycosis: A report of two cases. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2019 Apr 19];27:147-51. Available from: http://www.jiaomr.in/text.asp?2015/27/1/147/167142


   Introduction Top


Mucormycosis is an angioinvasive mycosis with high morbidity and mortality rates that predominantly occurs in immunocompromised patients. It was first described by Paultauf in 1885. Mucormycosis is an acute opportunistic infection caused by saprophytic fungus of class Phycomycetes order Mucorales and family Mucoracae, found in soil, bread molds, and decaying fruits and vegetables. Most common species associated are Rhizopus, Rhizomucor, Absidia and Cunninghamella. The term rhinocerebral mucormycosis (RCM) is used if the facial, palatal, orbital, paranasal sinus or cerebral regions are involved and the patients generally present with signs and symptoms primarily located in these regions. [1] Rhizopus is the predominant pathogen, accounting for 90% of the cases of RCM. [2] The three subtypes of RCM are rhinomaxillary, rhino-orbital and rhino-orbitocerebral mucormycosis.


   Case Reports Top


Case 1

A 48-year-old male patient had a chief complaint of pain in the upper right back teeth region since 1 month and swelling of right side of face since 20 days. He was a known diabetic for 10 years. On extra-oral examination a mild diffuse swelling was seen on right side of the face. On intraoral examination chronic generalized periodontitis with multiple periodontal abscesses was present [Figure 1] with generalized mobility of teeth in the upper arch and recession with bone exposure and oro-antral fistula in the region of 15 and 16. Provisional diagnosis of osteomyelitis of maxilla in the region of 15 and 16 was considered.
Figure 1: Multiple periodontal abscesses with bone exposure in the region of 14, 15, 16

Click here to view


Investigations showed increased blood sugar levels (FBS 158 mg/dl, RBS 226 mg/dl), which gave a clue for considering mucormycosis in the differential diagnosis. Orthopantomograph (OPG) showed haziness of right maxillary sinus (maxillary sinusitis was considered in differential diagnosis) [Figure 2]. 3D computed tomography (CT) showed destruction of part of antero-inferior wall of maxillary sinus in the region of 15 and 16 [Figure 3]. CT showed pansinusitis on the right side with mucosal thickening [Figure 4] and [Figure 5], discontinuity of part of antero-inferior wall of right maxillary sinus and destruction of part of right alveolus with antral communication [Figure 6] (osteomyelitis and malignancy were considered in the differential diagnosis).
Figure 2: OPG showing haziness of right maxillary sinus

Click here to view
Figure 3: Focal defect of antero-inferior cortex of right maxillary sinus

Click here to view
Figure 4: Frontal, maxillary and ethmoidal sinusitis on the right side with focal defect on antero inferior wall of maxillary sinus

Click here to view
Figure 5: Bilateral mucosal thickening of maxillary sinus, ethmoidal sinus on right side and deviation of nasal septum to left side

Click here to view
Figure 6: Destruction of bone in the region of right alveolus of maxilla with antral communication

Click here to view


Histopathological findings (HP) included broad non-septate fungal hyphae along with necrosis of the tissue [Figure 7]. Final diagnosis of rhinomaxillary mucormycosis (RCM) was considered as maxilla, associated sinuses and nasal fossa of right side were involved. The patient was kept under human mixtard insulin (SC), liposomal amphotericin B (IV 50 mg with 5% dextrose) and antibiotics for 1 week and later planned for surgery under GA. Debridement of maxillary sinus, correction of deviated nasal septum and complete extraction of teeth in maxillary arch except 26 and 27, irrigation and suturing were done [Figure 8].
Figure 7: Fungal hyphae which are aseptate ribbon-like and broad in necrotic background suggestive of mucormycosis

Click here to view
Figure 8: Postoperative picture after debridement of maxillary sinus, correction of deviated nasal septum and extraction of teeth in maxillary arch

Click here to view


Case 2

A 50-year-old female patient complained of pain and ulcer in the mouth since 3 months and passage of fluids from mouth into nose since 1 month. She was a known diabetic for 15 years with chronic kidney disease and a paralytic attack 3 months back. On extra oral examination, asymmetry with mask like face on the right side was seen. On intraoral examination bone exposure extending up to midline of the palate with palatal perforation and pathological fracture from 11 extending posteriorly up to 18 with missing 12 and chronic generalized periodontitis were seen [Figure 9]. Provisional diagnosis of fungal osteomyelitis was considered.
Figure 9: Ulcer on the right side of palate with oronasal communication

Click here to view


On investigation patient was anemic (Hb- 9g%) with poorly controlled diabetes mellitus (Hb A1c- 8.2%) and increased serum creatinine (3.9) levels. The OPG showed irregular destruction of bone in the region of 13, 14, 15, 16 with haziness of right maxillary sinus [Figure 10] (malignancy and osteomyelitis were considered in radiographic differential diagnosis). The CT findings included mucosal thickening of all maxillary, ethmoidal and sphenoidal sinuses on the right side [Figure 11] (Pansinusitis was considered under the differential diagnosis). Magnetic resonance imaging (MRI) showed infarct with encephalomalacia in the left parietal lobe abutting the sylvian fissure, and right maxillary, ethmoidal and sphenoidal sinus opacification extending into the right orbit with compression of optic nerve [Figure 12]. Nasal swab sent for KOH mount direct microscopy was positive for fungal elements.
Figure 10: OPG showing irregular destruction of bone in the region 13, 14, 15, 16 with haziness of right maxillary sinus

Click here to view
Figure 11: CT scan showing maxillary, ethmoidal, sphenoidal sinusitis on right side with orbital involvement

Click here to view
Figure 12: MRI showing infarct in the left parietal region abutting the sylvian fissure, and ethmoid and sphenoid sinus opacification extending into the right orbit with compression of the right optic nerve at the apex

Click here to view


HP findings showed necrotic tissue with numerous bacterial colonies, necrotic vascular tissue and fungal elements including broad, pauciseptate, and ribbon-like non-parallel wall hyphae suggestive of mucormycosis [Figure 13]. Final diagnosis of rhino-orbitocerebral mucormycosis was established as sinuses, nasal cavity, orbit and cerebrum were involved. The patient was administered antifungal drugs (itraconazole 100 mg BD), antibiotics and human mixtard insulin therapy (SC) for 4 weeks after which the nasal swab culture was negative for fungal elements and microscopy on KOH mount too was negative. Patient had poor prognosis for surgical resection because of anemia, age criteria, uncontrolled diabetes, chronic renal disease and cerebral involvement. Surgery was not performed as the patient was not willing for the surgery. She is under follow up.
Figure 13: Broad pausi septate ribbon-like hyphae occluding the blood vessel suggestive of mucormycosis with angioinvasion

Click here to view



   Discussion Top


Studies have shown that diabetes mellitus alters the immunologic capability to resist mucormycosis through reduction of the phagocytic ability of granulocytes. [3] In addition, Rhizopus species thrive best in an acidic and glucose-rich environment. [4] Mucormycosis often invades blood vessels; thrombosis, infarction and necrosis are the major characteristics. [5] Differential diagnosis includes aspergillosis, histoplasmosis, paracoccidioidomycosis, cryptococcosis (lungs contain foci of infection in addition to oral lesion), malignancy (features of malignancy such as induration, bleeding, paresthesia, metastasis, association with habits were not present in the present case), sinusitis (bone involvement in the present case made us to consider osteomyelitis especially RCM and other fungal infections which has association with osteomyelitis) and Gumma. Cavernous sinus thrombosis is a serious complication of maxillary infections. [4] A definitive diagnosis of mucormycosis can only be made by a biopsy and culturing the fungus in the laboratory. [6] Histologically, mucormycosis is characterized by extensive tissue necrosis, large fungal hyphae, which are nonseptate and have a ribbon-like appearance, with budding and dichotomous branching. [7] In the present cases nonseptate hyphae with tissue necrosis were present which helped in confirmation of the diagnosis.

The initial medical approach to mucormycosis is to treat aggressively any underlying predisposing disorder. [6] Surgical management also should be initiated early in the course of treatment. This should involve debridement of all infected tissues, which was done in the first case. The same treatment was proposed by some others according to which early antifungal and surgical treatment has good prognosis in early cases but patients with cerebral involvement had poor survival rate even after antifungal and surgical debridement. [8] There was poor prognosis in the second case because of cerebral involvement. In some cases, radical resection may be required. [3] The use of amphotericin B in patients with mucormycosis has been a widely published and accepted treatment, with a survival rate of up to 72%. Although combined treatment of surgery and amphotericin B has a survival rate of 80%, 70% of those who do survive will encounter some type of functional deficit. [6] In one of the present cases liposomal amphotericin B was used for the treatment which is the antifungal drug of choice for the treatment [9] of mucormycosis in combination with surgical debridement. Itraconazole and posaconazole are antifungal drugs which acts by inhibiting ergosterol synthesis. [9] In the present case one case was treated by administering itraconazole. Hyperbaric oxygen (HBO) helps in treatment by reducing tissue hypoxia and acidosis caused by vascular invasion of the fungus. [2],[10] Rifampin [11] and anticoagulants [12] has also been used to treat RCM in various studies. The present cases reinforce the concept that awareness and knowledge of potentially fatal complications may help in rapid diagnosis and prevention of disease dissemination. Early aggressive therapy to prevent cerebral involvement by this severe infection provides the best chance for a good outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Parfrey NA. Improved diagnosis and prognosis of mucormycosis. A clinicopathologic study of 33 cases. Medicine (Baltimore) 1986;65:113-23.  Back to cited text no. 1
[PUBMED]    
2.
Ferguson BJ, Mitchell TG, Moon R, Camporesi EM, Farmer J. Adjunctive hyperbaric oxygen for treatment of rhinocerebral mucormycosis. Rev Infect Dis 1998;10:551-9.  Back to cited text no. 2
    
3.
Lador N, Polacheck I, Gural A, Sanatski E, Garfunkel A. A trifungal infection of the mandible: Case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:451-6.  Back to cited text no. 3
    
4.
McNulty JS. Rhinocerebral mucormycosis: Predisposing factors. Laryngoscope 1982;92:1140-3.  Back to cited text no. 4
[PUBMED]    
5.
Tierney MR, Baker AS. Infections of the head and neck in diabetes mellitus. Clin Infect Dis 1995;9:195-216.  Back to cited text no. 5
    
6.
Sugar AM. Mucormycosis. Clin Infect Dis 1992;14:126-9.  Back to cited text no. 6
    
7.
Sapp JP, Eversole LR, Wysocki GP. Contemporary Oral and Maxillofacial Pathology. St Louis: Mosby; 1997. p. 237-48.  Back to cited text no. 7
    
8.
Nussbaum ES, Hall WA. Rhinocerebral mucormycosis: Changing patterns of the disease. Surg Neurol 1994;41:152-6.  Back to cited text no. 8
    
9.
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;48:1743-57.  Back to cited text no. 9
    
10.
Kajs-Wyllie M. Hyperbaric oxygen therapy for rhinocerebral fungal infection. J Neurosci Nurs 1995;27:174-81.  Back to cited text no. 10
    
11.
Christenson JC, Shalit I, Welch DF, Guruswamy A, Marks MI. Synergistic action of amphotericin B and rifampin against Rhizopus species. Antimicrob Agents Chemother 1987;31:1775-8.  Back to cited text no. 11
    
12.
Price DC, Hameroff SB, Richards RD. Cavernous sinus thrombosis and orbital cellulitis. South Med J 1971;64:1243-7.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

   Abstract Introduction Case Reports Discussion Article Figures
  In this article
 References

 Article Access Statistics
    Viewed811    
    Printed13    
    Emailed0    
    PDF Downloaded152    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]