A Rare Case of Vocal Cord Aspergillosis

Abstract

Primary aspergillosis of larynx is very rare. Till now only 28 cases of isolated laryngeal aspergillosis are documented in the English literature. In the field of otorhinolaryngology, aspergillosis most frequently affects external auditory canal or nasal sinuses and larynx is usually secondarily involved. It usually affects the patients with immunocompromised state and usually presents with hoarseness of voice. Laryngeal lesions usually mimics malignancy, so accurate diagnosis with direct laryngoscopy and biopsy is needed for further management. As these lesions responds very well to antifungal therapy, early diagnosis and starting antifungal therapy is important. Here we are presenting a case report of 67 years old male with aspergillosis of vocal folds without any immunodeficiency.

Introduction

Aspergillosis very rarely involves larynx primarily. It usually involves the larynx secondarily. Lesions may be confined to vocal folds or may involve other sites in the larynx. Laryngeal aspergillosis is known to occur in immunocompromised states particularly diabetes mellitus, TB and HIV infection. It is also associated with use of inhalational corticosteroids, cytotoxic drugs, and in patients after radiotherapy [1]. In immunocompromised patients, aspergillus infection is usually necrotizing, invasive and associated with poor prognosis. In an immunocompetent patient, laryngeal aspergillosis usually presents as colonization rather than invasion requiring no systemic antifungal [2].

Aspergillus fumigates and Aspergillus niger are common species infecting human beings. Despite its opportunistic nature, aspergillus can also cause localized or primary disease in relatively healthy patient in the external auditory canal, paranasal sinuses and orbit. [3, 4]. It gains access to respiratory mucosa via inhalation (spores) with subsequent invasion causing necrosis, ulceration, hemorrhage and thrombosis. In immunocompromised hosts, there is often haematogenous seedling involving multiple organs such as lungs, brain, heart, kidneys, spleen, GI Tract and lymph nodes [5].

When larynx is involved, patient complains of hoarseness, dysphagia and sometimes symptoms of airway obstruction. Depending upon degree of respiratory obstruction, stridor may or may not be present but hoarseness and loss of intensity of voice are almost universal findings. Here we are presenting a case report of 67 years male with aspergillosis of vocal folds without any immunodeficiency.

Case Report

A 67 years male patient presented with progressive hoarseness of voice since 1 month. He lives in rural/urban area and is farmer by occupation. There was no history of dysphagia, dyspnoea or vocal abuse. No history of any generalized immune deficiency, leukemia, malignant disease, diabetes mellitus, or use of any immunosuppressive drugs like corticosteroids. There was no recent loss of weight and apetite, no cough or evening rise of temperature, no history of TB, Asthma or any other chronic disease. He was not addicted to alcohol or tobacco and was otherwise healthy.

General physical examination revealed nothing significant. All routine blood and urine examinations were within normal limits. Chest X-ray and CT PNS were unremarkable. He was seronegative for HIV and HCV. On throat Telescopy using 90 degree rigid endoscope, irregular whitish lesion seen in anterior half of both vocal folds. Microlaryngeal excision done and tissue sent for histopathological examination.

On histopathological examination showed necrotic areas with plenty of fungal organisms with uniform width having dichotomous branching. No evidence of dysplasia or malignancy found. GMS staining was found positive for fungal organism i.e. Aspergillus. He was treated with tablet Itraconazole 100 mg twice a day for 2 months and instructions were given to the patient for vocal hygiene. After 1 month of medication his voice showed significant improvement. Repeated throat telescopy with 90 degree endoscope done after 1 month and 2 months showed apparently normal vocal folds (Fig. 1).

Fig. 1.

Throat telescopy at time of diagnosis and after starting itraconazole therapy

Discussion

Aspergillosis is a fungal infection caused by one of the species of genus Aspergillus and family Aspergillacea. Of various species implicated in human pathology, the Aspergillus fumigates and the Aspergillus niger are the commonest ones, the less common but equally pathogenic being Aspergillus nidulans, Aspergillus flavus and Aspergillus versicolor [6]. Aspergillus species are ubiquitous saprophyte fungi that grow on soil and decaying matter. However they also cause opportunistic infection (sinusitis, bronchitis, allergic bronchopulmonary aspergillosis, aspergilloma, invasive aspergillosis) whose severity depends upon the virulence of the species and hosts immunity [3].

Aspergillosis is classified into two groups: superficial and deep according to the depth of invasion. The superficial variety involves the mucosal lining whereas deep one involves the deeper tissue and may spread to other parts by blood dissemination [7]. Histopathologically Aspergillosis can be divided as necrotizing, suppurative, granulomatous and pseudomembranous variety.

Several factors which may lead to primary aspergillosis of larynx include inhaled corticosteroids for chronic respiratory disease, vocal cord abuse, smoking, severe reflux disease, laryngeal radiation, and settings of prolonged exposure to large amount of fungal spores [8]. These factors cause either decrease in local immunity, direct damage to the protective mucosal barrier, or increase in exposure of aspergillosis. Laryngeal aspergillosis in immunocompetent patient is quite rare and after very thorough review of literature we are presenting 28 cases of laryngeal aspergillosis in immunocompetent patients which are described in literature [1, 9] (Table 1).

Table 1.

Cases of laryngeal aspergillosis in immunocompetent patients described in literature

Cases	References	Age/sex	Presenting features	Associated factors
1.	Rao [12]	48 years/male	Hoarseness of voice	None
2.	Ferlito [6]	76 years/male	Hoarseness of voice	None
3.	Kheir et al. [13]	50 years/male	Hoarseness of voice	COPD
4.	Benson-Mitchell et al. [4]	62 years/male	Hoarseness of voice	None
5–12 (8 cases)	Nong et al. [14]	30–40 years 4 males/4 females	Hoarseness of voice	None
13–14 (2 cases)	Beust et al. [15]	53 years/male 54 years/male	Hoarseness of voice, dyspnoea	Radiotherapy for CA larynx
15.	Fairfax et al. [16]	75 years/male	Hoarseness of voice	Prolonged use of inhalational steroid
16.	Dean et al. [17]	17 years/female	Hoarseness of voice	None
17.	Ogawa et al. [3]	73 years/male	Hoarseness of voice	Radiotherapy for CA larynx
18.	Wittkopf et al. [18]	62 years/female	Hoarseness of voice	Cyst in vocal fold
19.	Ran Y et al. [11]	36 years/male	Hoarseness of voice	Systemic antibiotics and inhalational steroids
20–21 (2 cases)	Liu et al. [19]	30 years/female 32 years/female	Hoarseness of voice	Vocal abuse Vocal abuse and broad spectrum antibiotics
22.	Ran et al. [20]	30 years/male	Hoarseness of voice	Vocal abuse
23.	Ran et al. [21]	23 years/female	Hoarseness of voice	Oral sex
24.	Gangopadhyay et al. [5]	42 years/male	Hoarseness of voice, cough	Smoking, vocal abuse
25.	Al-ogaili et al. [22]	77 years/female	Hoarseness of voice, dysphagia	Smoking, use of inhalational steroids
26.	Doloi et al. [2]	35 years/female	Hoarseness of voice	None
27.	Arpita Saha et al. [23]	28 years/female	Dyspnoea	Vocal abuse, long use of inhalational steroids
28.	Mainak Datta et al. [1]	45 years/female	Hoarseness of voice	None

Lesions of Larynx usually look like malignancy and if not properly diagnosed it can lead to unnecessary surgery to remove the lesion with or without radiotherapy. Accurate and early diagnosis of the lesion is necessary. The hyphae of aspergillus look basophilic with haematoxylin and eosin stain. However periodic Gomori methenamine silver stain, Reticular silver impregnation method, PAS and haematoxylin stain, give a better morphological appearance of the hyphae. Aspergillus is recognized by circular, uniform calibra mycelium on section, septate hyphae and occasional folds with dichotomous branching [10].

Management of laryngeal aspergillosis in immunodeficiency pt is quite problematic. Amphotericin B is still considered as first line agent for this infection, although it is associated with severe systemic side effects. [3–10]. Aerosolized and liposomal Amphotericin B are its newer form with more efficacies and less side effects. In immunocompetent patient’s management of primary laryngeal aspergillosis included systemic administration of Amphotericin B, Itraconazole, and cauterization of lesion with a co2 laser, or reception of infected nidus in severe cases [11]. Most of the previous patients described in literature received Itraconazole, only few undergone surgery with stripping of the lesion. Our patient received 100 mg of Itraconazole twice a day for 2 months with excellent results.

Conclusion

Aspergillosis of larynx is very rare. We are presenting this case, because of its rarity and also very less information is present in literature about the disease. The disease is very easily treatable with oral antifungal drugs provided it is accurately diagnosed. Early and accurate diagnosis of disease can prevent unnecessary sufferings for the patients.