Objective: Aim of the study was to analyze 10 managed cases of Primary Histoplasmosis of Larynx, over a period of 10 years. Design: Retrospective, non-randomized, interventional case series. Patients and Method: This study was conducted in the Department of Otorhinolaryngology S.M.S Medical College from 2002 to 2011. The study consisted of evaluation of self-reported and referred patients who presented to ENT OPD and emergency. In all the cases a detailed history and clinical examination was done. All the patients underwent fiber optic laryngoscopy and subsequent direct laryngoscopy and biopsy. CT Scans were obtained for few cases. The study was a retrospective analysis of 10 treated cases. Main Outcome and Measures: Success of the treatment was defined as resolution of symptoms, differ-entiation from malignancy, no recurrence and regular follow up. Result: A total of 10 cases were managed between 2002 and 2011 and were reviewed. The main presentation was hoarseness of voice, with lesion simulating malignancy. Most common site of involvement was false cord and aryepiglottic fold (6 cases). Primary laryngeal Histoplasmosis was essentially a disease of adult males in all 10 cases. Treatment was exclusively medical with use of Itraconazole as drug of choice in 7 cases. Conclusion: Isolated laryngeal Histoplasmosis is a rare entity. Because of its simulation with malignancy it needs to be included in the differential diagnosis of neoplasm both benign and malignant. Our study clearly limits this kind of disease to adult males most commonly involving false cord and aryepiglottic fold in an endophytic pattern. Though it looks like cancer; biopsy confirms the diagnosis. The treatment is mainly medical with Itraconazole being the drug of choice.
Histoplasmosis (also known as “Cave disease,” “Darling’s disease,” “Ohio valley disease,” “Reticuloendotheliosis,” “Spelunker’s Lung” and Caver’s disease) [
Histoplasmosis can appear as a mild, flu-like respiretory illness and has a combination of symptoms, including malaise (a general ill feeling), fever, chest pain, dry or nonproductive cough, headache, loss of appetite, shortness of breath, joint and muscle pains, chills, and hoarseness. A chest X-ray can reveal distinct markings on an infected person’s lungs.
Though primary infection of larynx is a rarity and generally associated with generalized disseminated disease, we have seen and collected 10 cases over a period of 10 years, which presented to us primarily affecting the larynx. On initial assessment the patients were diagnosed as carcinoma larynx but on progressive histopathological examination diagnosis of histoplasmosis was confirmed.
Laryngeal involvement occurs in the mucocutaneous form of the chronic disease. Common features are: pain to swallow, hoarseness, gingival ulceration and dysphagia. There can be weight loss, malaise and fatigue. As per the data histoplasmosis of head and neck is more common in adults and exclusively in males.
This study was conducted in the Department of Otorhinolaryngology S.M.S. Medical College from 2002 to 2011. The study consisted of evaluation of self-reported and referred patients who presented to ENT OPD and emergency. In all the cases a detailed history regarding the complaints was taken which included elaboration of all the complaints with regards to onset, duration and progress. A through clinical examination was done which included a comprehensive Ear, Nose and Throat examination. Special care was done to examine the oral cavity, oropharynx, pharynx, larynx and neck as these were the main complaint areas. Indirect laryngoscopy was done in all cases in first sitting and then subsequently all the patients underwent fiber optic laryngoscopy. Then patient was prepared for General anesthesia by getting all the relevant blood examination, which also helped in ruling out any acute infection or other blood related maladies. The OT was prepped and a direct laryngoscopy was done to further confirm the gross feature of the lesion and further taking biopsy to confirm the diagnosis histopathologically. Contrast enhanced CT Scans were obtained for few cases and radiologist was specifically asked to give thinner cuts at the lesion site. The study was a retrospective analysis of 10 treated cases.
Out of 10 cases, 8 cases were in age group of 40 to 60 years. Youngest patient was 32 years old and eldest one was 60 years of age. Disease was exclusively seen in males. The most common presenting symptom was gradually progressing hoarseness of voice (10 cases), and difficulty in swallowing (3 cases). Laryngeal examination revealed endophytic growth in 6 cases, exophytic growth in 2 cases and ulcerative lesion in 2 cases. False cord and aryepiglottic fold was the common site of involvement (6 cases). Epiglottis involvement was seen in 3 cases and only 1 case was with postcricoid lesion.
Histopathological diagnosis was mandatory in all the cases. A sufficient quantity of tissue was taken and sent to the pathologist in all the sterile conditions with 6 hours of the biopsy The pathologist did two types of staining to confirm the diagnosis. Both Hematoxyline & Eosin and Silver methenamine stains (MSN) showed Histiocytes (macrophages) with Histoplasma, granuloma with caseation and pseudoepiteliomatous hyperplasia.
In the past Amphotericin B was the drug of choice, which was used in 3 cases. We have used Itraconazole 200 mg, twice a day, (by mouth) in 7 cases. All cases were successfully treated on 6 months follow-up. Resolution of clinical symptoms and clearance of the positive culture was obtained in these cases. There were no signs of pulmonary or Systemic involvement.
Histoplasmosis is a dimorphic fungal infection first described and named by Darling in 1906. Initally he thought causative agent was a protozoan, later it proved to be a fungus. Though fungal nature was confirmed in early stages it was treated as a deficiency disease [
Clinically it can be classified into 4 types according to Furcolow [
1) Acute Pulmonary Histoplasmosis;
2) Chronic pulmonary histoplasmosis-progressive cavitary;
3) Acute Disseminated Histoplasmosis-benign progressive;
4) Chronic Disseminated Histoplasmosis-Mucocutaneous, Meningitis, Pericarditis, Adrenal insufficiency and Bone marrow involvement.
The source of infection is usually the soil with high nitrogen content which is result of contamination by bat and bird guano. Thus found in areas of bird roosting, caves and chicken houses.
Histoplasma capsulatum infection involving the larynx is a rare manifestation, especially in immunocompetent individuals and a high index of suspicion is needed to establish the diagnosis correctly [
In our series all the 10 cases presented with Hoarseness of voice as main symptom. Difficulty in swallowing was also one of the features in 2 cases. Subramaniam (2005) [
As per the study we could see that primary laryngeal Hiatoplasmosis was a disease of adult males with around 80% cases between age group of 40 - 60. In all the previous reported cases males were exclusively involved. Subramaniam [
The most common site to be involved in our study was false cord and aryepiglottic fold (
Definitive diagnosis of Laryngeal Histoplasmosis is rarely on clinical examination, but a set of differential diagnosis can be considered [
Staloff et al. [
Diagnosis depends on clinical features and high index of suspicion especially in endemic areas. Histopathological examination with Hematoxyline & Eosin (
Treatment is essentially medical with Itraconazole being the drug of choice in our series. Itraconazole was used in a dose of 200 mg twice daily for 3 to 4 months. Amphotericin B was used in initial 3 cases with recurrence in 1 case after 6 months and signs of nephrotoxicity in another, but with use of Itraconazole in rest 7 cases, no recurrence or side effect was noted. Amphotericin B remains the gold standard for treatment and is highly effective, even in immunocompromised individuals; Itraconazole shows promise as an alternative to Amphotericin b for treatment of less severely ill patients [16- 18].
Prognosis of these patients is very good and we had full recovery in all the patients. The treatment being essentially medical both the morbidity and patient compliance is excellent.
Isolated laryngeal Histoplasmosis is a rare entity. Because of its simulation with malignancy it needs to be included in the differential diagnosis of neoplasm both benign and malignant. Our study clearly limits this kind of disease to adult males most commonly involving false cord in an Endophytic pattern. Though it looks like cancer, biopsy confirms the diagnosis. The treatment is mainly medical with Itraconazole being the drug of choice.
• Primary Laryngeal Histoplasmosis is a rare entity;
• It generally presents clinically as Benign or Malignant Laryngeal Pathology;
• Histopathological examination confirms the diagnosis;
• Treatment is essentially medical with Itraconazole showing wondrous results.