|Year : 2019 | Volume
| Issue : 2 | Page : 58-62
Mucormycosis in head-and-neck region – Our experiences at a tertiary care teaching hospital of Eastern India
Santosh Kumar Swain1, Ishwar Chandra Behera2, Jatindra Nath Mohanty3
1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
2 Department of Community Medicine, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
3 Medical Research Laboratory, IMS and SUM Hospital, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India
|Date of Submission||24-May-2019|
|Date of Acceptance||20-Oct-2019|
|Date of Web Publication||25-Nov-2019|
Prof. Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O“ Anusandhan University, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
Source of Support: None, Conflict of Interest: None
Introduction: Mucormycosis is a rare clinical entity and often affects immunocompromised patients. It is caused by the fungus of the order Mucorales. Rhizopus species are the most common causative organism associated with mucormycosis. It is an emergency situation and has poor prognosis.Materials and Methods: A retrospective study included eight patients with head-and-neck mucormycosis diagnosed over a 3-year period from December 2016 to March 2019. Results: There are five male and three female patients, aged from 29 to 65 years. Out of eight patients, four were diabetic and the rest were with hematological disorders. There were five sinonasal mucormycosis and three nonsinonasal involvement. Diagnoses were confirmed by histopathological examination. Two cases were fatal due to cerebral involvement.Conclusion: Prompt diagnosis with tissue biopsy, local control of the disease by aggressive surgical debridement, and appropriate systemic antifungal treatment improve the prognosis and survival of the patients. Treatment of mucormycosis needs antifungal agents such as amphotericin B and wide surgical debridement. Early diagnosis and treatment is often needed for survival of the patients.
Keywords: Amphotericin B, head and neck, mucormycosis, surgical debridement
|How to cite this article:|
Swain SK, Behera IC, Mohanty JN. Mucormycosis in head-and-neck region – Our experiences at a tertiary care teaching hospital of Eastern India. Ann Indian Acad Otorhinolaryngol Head Neck Surg 2019;3:58-62
|How to cite this URL:|
Swain SK, Behera IC, Mohanty JN. Mucormycosis in head-and-neck region – Our experiences at a tertiary care teaching hospital of Eastern India. Ann Indian Acad Otorhinolaryngol Head Neck Surg [serial online] 2019 [cited 2020 Aug 15];3:58-62. Available from: http://www.aiaohns.in/text.asp?2019/3/2/58/271605
| Introduction|| |
Mucormycosis is a rare and dreaded disease caused by a fungus of the order Mucorales. It has high morbidity and mortality. It is an opportunistic fungal infection. Mucormycosis was first described by Paltauf in 1885. It is also known as zygomycosis, harmless to a healthy person but fatal in immunocompromised patients. Most commonly, Rhizopus species are causative organisms for the mucormycosis. Diabetic ketoacidosis and neutropenia are common predisposing conditions. This is a life-threatening infection which has remarkable affinity for arteries. They have tendency for spreading into vessels and lymphatics causing formation of Mucor thrombi and lead to ischemia and infarction of the affected tissue. This fungus often dissects internal elastic lamina from the media of the blood vessels, resulting in extensive damage to the endothelium, and leads to thrombosis. Mucormycosis is classified into different forms as per anatomic sites such as rhinomaxillary, central nervous system (CNS), cutaneous, pulmonary, disseminated, and miscellaneous. The rhino-orbito-cerebral is the most common variety of mucormycosis. The most common types of mucormycosis infections are paranasal sinuses (39%), lungs (24%), skin (19%), brain (9%), and gastrointestinal (7%) forms, and other miscellaneous types are extremely rare. The ideal treatment needs correction of underlying risk factors, antifungal treatment with amphotericin B, and aggressive surgery. Here, we present our experiences of managing mucormycosis in head-and-neck region during the last 3 years at our tertiary care teaching hospital.
| Materials and Methods|| |
Eight patients were treated in the department of otorhinolaryngology between December 2016 and March 2019. Four patients were referred from department of hemato-oncology and the rest four were referred from the department of endocrinology/diabetology. Clinical presentations, imaging, histopathological findings, and treatment were documented and discussed. This study was approved by the institutional ethics committee. All the four diabetic mellitus patients were under treatment with oral hypoglycemic agents/insulins regularly, but their blood sugar was poorly controlled. Diagnostic nasal endoscopy was done in all cases and showed details of nasal cavity and nasopharynx. Ophthalmological consultation was done in all cases to find loss of vision or not. Computed tomography (CT) scan of the nose and paranasal sinuses was done to check any spread to the orbit and adjacent facial area. Biopsy sample [Figure 1] was taken from all the cases, which showed the picture of mucormycosis with some foci of nonseptate fungal hyphae with right-angled hyphae branches [Figure 2]. All had undergone radical excision of the mucormycosis along with orbital exenteration in two cases, followed by parenteral amphotericin B (0.5 mg/kg/day). Patient follow-up was done after 6 months' interval after surgery.
|Figure 1: Black necrotic mass from nasal cavity sent for histopathological examination|
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|Figure 2: Histopathology microphotograph showing broad nonseptate hyphae with 90° branching (Eosin stain, ×400)|
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| Results|| |
Out of the eight patients, there are five male and three female patients, aged from 29 to 65 years. The mean age of the patients in this study was 51.5 years. Out of eight patients, four were diabetic. All the four patients were uncontrolled diabetes mellitus which was diagnosed for the first time. All the patients were from poor socioeconomic class. Four patients were farmer, three were roadside vendors, and one was a retired teacher. There were five sinonasal mucormycosis and three nonsinonasal involvement. Out of three nonsinonasal mucormycosis, one was palatal involvement, one was orbitofacial, and last one was otogenic. All patients of sinonasal mucormycosis presented with foul-smelling nasal discharge and nasal block. Diplopia was seen in one patient, headache was presented in three patients, septal perforation in one patient, palatal perforation [Figure 3] in one patient, and cellulitis of the facial area in two patients. Details of patient profiles are given in [Table 1]. One patient had orbitofacial swelling [Figure 4]. One patient had lower cranial nerve palsy [Figure 5]. All patients had more than one clinical symptom. All sinonasal mucormycosis patients presented with black necrotic debris inside the nose. Diagnoses were confirmed by histopathological examination. Culture of the nasal discharge showed Rhizopus oryzae in six patients and the rest showed no growth. Two cases were fatal due to cerebral involvement. All diabetic patients were treated for controlling blood sugars and all attained normal level of blood sugar. All patients had undergone debridement under general anesthesia [Table 1]. All patients were administered intravenous infusion of amphotericin B. The initial dose was 0.5 mg/kg/day of amphotericin B as an intravenous infusion in 5% dextrose, and the dose was adjusted later on basis of patient response and tolerance. Two patients died in the hospital due to intracranial involvement [Table 1]. One patient had permanent vision loss in one eye and one had residual vision loss. One patient lost follow-up, and the remaining were disease free.
|Figure 4: a) Mass at right facio-orbital area of the patient; b) CT scan of the nose and para-nasal sinuses showing a radio-opaque haziness/mass inferior to the right orbit|
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| Discussion|| |
Mucormycosis is caused by saprophytic fungi of many genera related to phycomycetes (zygomycetes) and order Mucorales. The fungus has a great affinity toward arteries and adheres to the arterial wall. It grows along the internal elastic lamina of the blood vessels causing thrombosis, ischemia, and necrosis of the surrounding tissues. Mucorales are abundantly seen in soil, decaying vegetables, animal excreta, and foodstuffs. They grow rapidly in humid environment, and the sporangiospores are released into the environment and spread as airborne propagules. The incidence of mucormycosis in head-and-neck region is not related to sex or age. There are three different modes of transmission such as inhalation, ingestion, and percutaneous introduction spores of mucormycosis. There is no person-to-person spread of the mucormycosis. In India, the airborne spores are more during transition from summer to rainy season as it may be ideal for fungal growth. Mucormycosis is a rare clinical condition and often affect immunocompromised patients. The organisms causing mucormycosis are a fungus, primarily of the genus Rhizopus or Mucor. Mucormycosis may be of rhino-orbital-cerebral and pulmonary infections, and these types of the diseases may lead to emergency situation to the patients. Rhinocerebral mucormycosis is rapidly spreading disease with high mortality rate, which needs immediate surgical debridement, followed by medical intervention. In our case, five cases were sinonasal variety, whereas rests three were nonsinonasal types such as isolated orbitofacial, palatal, and otogenic. In our orbitofacial variety, the disease is affecting only orbit and nearby facial area without involving nose and sinuses. Mucormycosis often spreads to orbit and adjacent areas or brain either directly or vial vessels. The mucormycosis may cause cavernous sinus thrombosis causing either unilateral or bilateral vision loss even within few hours. Mucormycotina are seen as common saprobes over decaying organic material and forest or agricultural soils. These are rapidly growing organisms, characterized by ribbon-like hyphae with no or only few septa. The genera affecting human are Cunninghamella, Absidia (Lichtheimia), Mucor, Rhizomucor, and Rhizopus and on the basis of geographical distribution, Saksenaea and Apophysomyces. These aggressive and highly destructive fungal infections are more often seen in immunocompromised hosts such as patients with hematological malignancies or those taking hematopoietic stem cell transplantation. Diabetic mellitus patients with ketoacidosis and transfusion/dyserythropoietic iron overloaded patients are also important risk groups. The most common predisposing factors associated with mucormycosis are uncontrolled diabetes mellitus, especially with a history of ketoacidosis. Poor farmers with uncontrolled and undiagnosed diabetes mellitus may be at risk for acquiring this fatal disease. Four (50%) of our patients were farmers with uncontrolled and undiagnosed diabetes mellitus. Farmers those use organic manure are more prone to this disease as organic manure often contains animal excreta and decayed substance which has high concentration of Mucorales. During applying manure in the farming field, there is a chance of inhalation of fungal spores. Three (37.5%) patients were street fast food vendors who were working near to dustbin containing decaying food materials which act as good source of Mucorales, particularly in humid environment. Neutropenia, immunosuppressive therapy, acquired immune deficiency syndrome, malnutrition, dialysis, hematological malignancy, and organ transplantation are often predisposing factors. Mucormycosis universally affects immunocompromised patients. The potent T-cell-depleting agents used for immunosuppression as in organ transplantation also often lead to high risk for causing mucormycosis. Although mucormycosis is ubiquitous and rapidly spread, it seldom affects an immunologically competent patients. Therefore, mucormycosis occurs among patients with serious underlying conditions such as diabetic mellitus, leukemia, organ transplantations, acquired immunodeficiency syndrome, severe burn, and immunosuppressive medications. Around 70%–80% of mucormycosis patients have diabetes mellitus. Radiological imaging will not establish, but suggests the diagnosis of mucormycosis. CT or magnetic resonance imaging (MRI) is helpful for facial and cerebral involvement of mucormycosis and determine locoregional extension toward the orbit and brain and also identify the cerebral thrombosis. Cavernous sinus involvement is best assessed by MRI. Difficulties in diagnosis and followed by antifungal treatment with resistance to many commonly prescribed antifungal agents lead to high mortality in certain group of patients. There is no such definitive diagnosis for the mucormycosis except histopathological confirmation by seeing the nonseptate hyphae of the affected tissue. Necrosis of the surrounding tissue prevents antifungal drugs such as amphotericin B to enter the site of infection. Early debridement of the affected tissue gives better prognosis for successful treatment in mucormycosis. Liposomal amphotericin B treatment has better tissue penetration and considered as first-line treatment. However, monotherapy such as surgery alone or amphotericin B alone has limited efficacy, whereas the combination therapy with liposomal amphotericin B or posaconazole and surgical debridement is recommended treatment. Amphotericin B has potential toxicity on renal function and so dose should be individually adjusted between 0.5 mg/kg/day and 1.0 mg/kg/day on the basis of body weight and renal functions of the patients. The total cumulative dosage of amphotericin B is 2–4 g often advocated to the adult patient. Hyperbaric oxygen therapy and certain cytokines such as granulocyte-macrophage colony-stimulating factor and interferon gamma act as adjuvant treatment, and these are under assessment. Hyperbaric oxygen therapy has fungi static effect and helps revascularization of the necrotic or ischemic tissue. The survival rate of mucormycosis patients has been improved by new antifungal agents and combination therapy. Despite advancement in treatment modality and care, the overall mortality rate of mucormycosis patients remains high due to delay in diagnosis. A delayed diagnosis of mucormycosis of more than 5 days is associated with increased chance of death. For successful outcome, correction of predisposing factors, timely diagnosis, surgical debridement, and systemic antifungal are needed in combination. The systemic amphotericin B has its own side effects which need vigilant monitoring. Amphotericin B in classic desoxycholate form in the dose of 1–1.5 mg/kg/day or more preferably in liposomal form of amphotericin B is highly useful for mucormycosis. The liposomal form has less nephrotoxicity and also allows higher doses in 5–15 mg/kg/day for prolonged period. Liposomal form better concentrates in macrophages including deep infected tissue with higher CNS penetration. Posaconazole, a new type of triazole, is useful in failure cases with better efficacy in vitro and in vivo with good tolerance and very fewer side effects. Isavuconazole is a new type of extended spectrum of triazole which act against molds, yeast, and dimorphic fungi and also approved drug for the treatment of invasive aspergillosis and mucormycosis. Total duration of antifungal drug treatment in mucormycosis varies with evolution, but at least advised for 12 weeks. For prevention of the mucormycosis, the risk factors should be properly addressed. The mucormycosis is often seen in patients of uncontrolled diabetes mellitus, which needs a very good control for preventing this dreaded disease.
| Conclusion|| |
Mucormycosis is a rare and dreaded fungal infection in the head-and-neck region. It is a rapidly progressive disease with fatal outcome unless diagnosed early and treated promptly. Delayed diagnosis and treatment may lead to fatal complications and death. Treatment includes both medical and surgical. It needs early and adequate treatment with amphotericin B, and surgical resection and control of risk factors can improve the prognosis. Prognosis depends on multiple factors and needs early initiation of treatment. A multidisciplinary approach often needed and consists of otorhinolaryngologists, ophthalmologists, neurologists, and dentists for successful management of patients with mucormycosis. Identification of risk factors and doing all preventing measures can reduce the incidence of life-threatening mucormycosis in the head-and-neck area.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]