Fungi are ubiquitous organisms in our environment. Invasive fungal rhinosinusitis is characterized by mycotic infiltration of the mucosa of the nasal cavity and the paranasal sinuses. Invasive fungal rhinosinusitis is rapidly destructive. IFS requires urgent diagnosis and treatment; otherwise, the mortality rate can be as high as 50–80% [1, 2]. The disease occurs primarily in immunocompromised patients. Most patients with AIFR are already in a state of poor health and are expected to have a poor prognosis because of the underlying disease.

Invasive fungal sinusitis has a worldwide distribution. Most cases have been reported in adults, but immunocompromised children are also at risk; men and women are equally affected. (3,4,5) . Medical advances, such as new chemotherapeutic agents and long-term use of immunosuppressive agents following bone marrow or solid organ transplantation, have resulted in an increase in the population at risk of developing AIFR. Poorly controlled type 1 diabetes mellitus, malnutrition, and excessive storage of iron in hematological diseases can also be predisposing factors for the development of AIFR [6].

There are very few landmark studies on invasive fungal rhinosinusitis in our country, there is insufficient data regarding the causative agents from southern part of the country. Varied presentations and progress of the disease need to be studied in detail.

Our institute is a tertiary center, we get to see good number of invasive fungal rhinosinusitis cases and would be a good opportunity to study the diagnostic  and therapeutic management and outcome of these patients.

Aim: To study multimodal diagnostic and therapeutic approaches for the management of invasive fungal rhinosinusitis.

Objectives: 1. To study the underlying risk factors. 2. To study the isolated pathogens 3. To study the radiological features of invasive fungal sinusitis. 4. To study various treatment modalities.

This is a prospective and observational study conducted in the Department of ENT, Subbaiah Institute of Medical Sciences, Shivamogga over a period of 2 years.

Inclusion criteria:

• All cases of invasive fungal rhinosinusitis attending Hospital.

Exclusion criteria:

• Patients not willing to participate in
• Patients lost in follow

Investigations: Haematological investigations, Biochemical investigations, Microbiological investigations.Pathological investigations &  Radiological investigations

Treatment modalities:

Surgical: Endoscopic sinus surgery under general anesthesia.

Medical: Systemic Antifungal Therapy. Management of comorbid conditions.

FOLLOW UP:

DURATION: All the patients are followed up for at least 3 months.

FREQUENCY: Once in a week for first one-month post discharge, once in a month        thereafter. Endoscopic assessment is done in all these cases in the follow up.

Table 1: AGE

Out of 60 cases, 5 cases (8.3%) were under 20 years age group, 11 cases (18.3%) were in the 3^rd decade, 14 cases (23.3%) were in the 4^th decade, 15 cases (25%) were in the 5^th decade, 8 cases (13.3%) were above 60 years of age group. No age preponderance was noted.

TABLE 2: SEX INCIDENCE

Incidence of the disease was more in the male population with 40 cases (66.6%) whereas the female incidence was 20 cases (33.3%)

TABLE 3: CLINICAL EXAMINATION:

In our clinical findings 28 cases had necrotic tissue in the nasal cavity, purulent discharge in the nose in 11 cases, polyposis in 3 cases, septal erosions in 2 cases, hard palate erosion in 14 cases, maxilla erosion in 4 cases, so the commonest clinical finding was necrotic tissue in the nasal cavity.

Table 4: CT FINDINGS

Study of CT scans of 60 patients revealed unilateral pan sinusitis in (74%) of cases with incidentally more on the left side with 47% of cases, bilateral pan sinusitis was seen in 27% of cases, with bony erosions of lamina papyracea in 57% , hard palate erosion in 47%,maxilla erosion was seen in 13% of cases and intracranial extension was seen in 23% of cases.

Table 5: Pathogen isolated

On fungal culture of the nasal tissue 53.3% of cases were found to isolate mucor species and 10% of cases isolated to have aspergillous species where as in 36.6% of cases no fungus was grown.

Table 6: Treatment

All cases that were diagnosed as invasive fungal sinusitis either clinically or based on histopathology were treated with IV Amphotericin in which 60% of cases were treated with amphotericin deoxycholate and 40% of cases were treated with lipid emulsion amphotericin purely based on the financial status of the patient. 87 % of cases       underwent endoscopic sinus surgery and debridement of fungal debris was done other were excluded as they were not fit for the surgery.

Table 7 Complications

During the course of treatment or disease 14 cases (47%) developed oroantral fistula, (57 %)17 cases developed orbital cellulitis, 05 cases (17%) of cases developed  permanent loss of vision, 20% of cases developed meningitis and 3% of cases developed CSF leak, 6% of cases developed cerebral mucormycosis, 20% of cases were deceased.

Table 8 Result

Out of 60 cases clinical improvement was seen in 48 cases (80%) and 12    cases (20%) were deceased

In our study the age of the patients were between 5 to 65 years with a mean age group of 47.8 years. According to Patorn Piromchai et al(⁷) mean age was around 51.7 years and according to navya BN et al (⁸) mean age was around 30 years. According to Sakeena J. Payne et al (⁹) the mean age at time of evaluation                                                      was 49.4 years, with 60.3% being males and 39.7% female.

In our study the disease was more prevalent in male population with 66.6% of cases, Male: female ratio of 2.25:1, whereas according to Matin ghazizade et al (¹⁰) Male: female was 1.56:1 according to Patorn Piromchai et al (¹¹) Male: female was 1:1.5.

According to Ahmet Emre Süslü · (¹²) Nasal obstruction was the predominant symptom (94.7%), followed by fever (73.7%), facial pain (68.4%), headache (42.1%), and facial swelling (26.3%). Loss of visual acuity and ophthalmoplegia were detected in two patients. Our study showed nasal obstruction (47%) and nasal discharge (43%) was commonest presenting complaint followed by eye pain and eye swelling in (40%) of cases and fever was presenting complaint in only 27% of cases. Orbital symptoms in a case of invasive fungal sinusitis is a late presentation, nasal obstruction and nasal discharge are part of acute rhinitis hence usually neglected and leading to presentation in late stage.

In our clinical findings 28 cases had necrotic tissue in the nasal cavity, purulent discharge in the nose in 11 cases, polyposis in 3 cases, septal erosions in 2 cases, hard palate erosion in 14 cases, maxilla erosion in 4 cases. So the commonest clinical finding was necrotic tissue in the nasal cavity. According to Rupa Mehta et al (¹³)lamina erosion was the commonest bony erosion that was observed, followed by roof of maxillary sinus.

According to Chien-Yuan Chen et al(¹⁴) Sinus imaging by computed tomography (CT) or magnetic resonance (MRI) were performed in 44 (96%) of                                  patients with IFS and 37 (59%) of 63 patients with chronic nonspecific sinusitis. Evidence of extra-sinus tissue involvement and/or bony destruction were detected in 15 (33%) of patients with IFS. Where as in our study Computed tomography of paranasal sinuses revealed unilateral pan sinusitis in (74%) of cases with incidentally more on the left side with 47% of cases, bilateral pan sinusitis was seen in 26% of cases, with bony erosions of lamina papyracea in 57%, hard palate erosion in 47%,maxilla erosion was seen in 13% of cases and intracranial extension was seen in 23% of cases.

According to E.H.Middlebrooks et al(¹⁵) Aspergillus species was isolated in 42.9%, 23.8% of cases mucor species and 19% of cases pathogen was unknown. According to Rupa Mehta et al(⁴²) positive fungal growth was seen in 57.7% (Aspergillus sps) and negative for fungal growth in 42% of cases. According to Matin ghazizade et al (¹⁶) study showed mucor species was isolated in 80.5% of cases. According to Chien-Yuan Chen et al (¹⁷) The fungal isolates from 25 patients with proven invasive fungal sinusitis included Aspergillus flavus (12), Aspergillus fumigatus (2), Aspergillus vesicolor (1), Aspergillus sybowii (1), unidentified Aspergillus species (1), Mucor (4), Phaeohyphomyces (1), Fusarium (1), Penicillium (1), and unidentified fungal species (1).

According to Patorn Piromchai et al (¹⁸) orbital cellulitis was the commonest complication seen in 25% of cases and cavernous sinus thrombosis in 23 % of cases and intracranial complication in 5% of cases. Whereas our study revealed (47%) developed oroantral fistula (57%)17cases developed orbital cellulitis, 5cases (17%) of cases developed permanent loss of vision, 20% of cases developed meningitis   and 3% of cases developed CSF leak,6% of cases developed cerebral mucormycosis,20% of cases were deceased.

According to Ahmet Emre Süslü · Ofuz Öfretmenoflu et al (¹⁹) early aggressive sinonasal debridement should be performed for all patients with biopsy-proven AIFR. In the early stages, when the disease is limited to the nasal cavity and paranasal sinuses, an endoscopic approach may be sufficient for the debridement procedure. Surgical debridement accomplishes the following: (1) slows the progression of the disease, allowing time for bone marrow recovery, (2) reduces fungal load, which in turn, reduces the burden on recovering neutrophils, (3) prevents easy growth of fungus in devitalized tissues, (4) enhances the ability of antifungal drug.

In Ahmet Emre Süslü · Ofuz Öfretmenoflu et al (²⁰) study Amphotericin B was            added to the treatment regimen of each patient after invasive fungal infection was considered. Although aggressive surgical debridement was planned for all patients, only 13 underwent surgery. In remaining patients surgical debridement could not be performed because of severe thrombocytopenia or poor health status related to the systemic effects of the underlying disease. Similar modality of treatment was given in all studies where 100% of cases were treated with Injection Amphotercin and endoscopic debridement of necrotic and fungal debris in most of the cases who are fit for surgery. Few patients were treated with injection amphotericin deoxycholate 69% and few patients 31% of them with lipid emulsion amphotericn B purely based on the financial status of the patients. However clinically significant complications were not noted in the patients who are given amphotericin b deoxycholate.

According to Rupa Mehta et al (²¹) itraconazole has the similar efficacy in treating the cases of invasive fungal sinusitis with fewer side effects compared to amphotericin B. According to Rupa Mehta et al (²²) study mortality rate was 11% in cases of invasive fungal sinusitis. According to Matin ghazizade et al (²³) study clinical improvement was seen in 80% of cases.

Invasive fungal rhinosinusitis is a relatively rare disease with high morbidity and mortality. The most common risk factor is uncontrolled diabetes mellitus. Maintaining a high index of suspicion in at- risk patient populations, followed by prompt evaluation and management, is crucial in suspected AIFRS. Early diagnosis of IFR requires a high level of suspicion because of the non- specific initial symptoms and radiological signs. Diagnostic nasal endoscopy and CT PNS helps in early detection of bony erosions and necrotic changes and are complementary to microbiology and pathological investigations. IFRS can be successfully treated with a combination of endonasal surgical debridement and antifungal medication.

Invasive fungal rhinosinusitis is a relatively rare disease with high morbidity and mortality. The most common risk factor is uncontrolled diabetes mellitus. Maintaining a high index of suspicion in at- risk patient populations, followed by prompt evaluation and management, is crucial in suspected AIFRS. Early diagnosis of IFR requires a high level of suspicion because of the non- specific initial symptoms and radiological signs. Diagnostic nasal endoscopy and CT PNS helps in early detection of bony erosions and necrotic changes and are complementary to microbiology and pathological investigations. IFRS can be successfully treated with a combination of endonasal surgical debridement and antifungal medication.

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