CASE REPORT Pulmonary Mucoraceous Fungal Ball LK. Lahiri, (Mrs) D. Agarwal, G.E.C.V. Sagar Reddy and Archana Bajoria Department of Cardiovascular and Thoracic Surgery, Institute of Medical Sciences, Banaras Hindu University, ‘Varanasi (Uttar Pradesh) ABSTRACT A case of opportunistic pulmonary infection in the form of fungal ball produced by the family of mucoraceae in the class of phycomycetes having nonseptate hyphae (cellophane tubules) with haphazard branching in a post-tubercular immunocompetent patient is described. Clinical course was chronic with right upper lobe cavity invaded by fungi of mucor species, pathology was granuloma with blood vessel thrombosis, and a fungus ball. The host had no associated predisposing diseases. Segmental resectional surgery of the right upper lobe along with removal of fungus ball under the coverage of modified dose of amphotericin B was per- formed. Literature scanning revealed rarity of mucormycosis in immunocompetent host. Key words : Mucorales infection, Lung, Surgery, Amphotericin B. INTRODUCTION Phagocyte opportunists infection caused by the fungi of mucorales order is an occasional entity, and has six distinct clinical presentations. In order of frequency, these are thinocerebral, pulmonary, abdominopelvic, cutaneous, disseminated and vascular with endocardial form!, Pulmonary manifestations include from rapidly progressive fulminant pneumonia, slowly progressive pulmonary infection, endobronchial lesion with confinement to a discrete area, intracavitary fungus ball, and chronic necrotizing, pulmonary mucormycosis in which fungus invades, abnormal lung and adjacent pleura®. Pulmonary mucoraceous fungus ball (PMEB) is a matted collection of fungal hyphae, cellular debris, fibrin and mucous, The rarity of such reports prompted us to describe this case of chronic colonizing pulmonary mucoraceous infection along with intracavitary fungal ball in Undian J Chest Dis Allied Sci 2001; 43 : 107-110] anon-immunocompromised patient. - CASE REPORT ‘A48-year-old male presented with cough and expectoration for four years along with increasing dyspnoea for six months. The expectorant was variable, occasionally viscid and foul smelling and often blood tinged. There was no history of diabetes, smoking or jaundice. The patient had received antitubercular drugs for nine months, 12 years prior to this admission. General examination revealed pallor, grade II clubbing and generalized lymphadenopathy including right lower cervical, bilateral axillary and bilateral inguinal varying from 0.5 cm to 1.5 cm in size, firm but matted at places. Respiratory system examination revealed asymmetric, flat right upper chest along with prominent accessory muscles of respiration and diminished breath Correspondence : Dr TK. Lahiri, Professor and Head, Department of Cardiovascular and Thoracic Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi-221 005 (Uttar Pradesh); Tele. : 91-0542-316151 (R), 91- 0542307530 (0); Telefax : 91-0542-316068; E-mail : .108 Pulmonary Mucormycosis sounds in the right infraclavicular and suprascapular regions. His biochemical and hematological examinations were within normal limits. Sputum was negative for acid-fast bacilli, and ELISA for HIV was negative. Chest roentgenogram revealed an opacity in a cavity in the right upper zone along with retraction of the right upper intercostal spaces (Figure 1). Bronchoscopy disclosed distorted right main bronchus along with crowding and mucopurulent secretions. The bronchial aspirate ‘was negative for malignant cells and also for acid fast bacilli. Bronchoalveolar lavage (BAL) and brush cytology revealed lymphocyte and plasma cells only. Computed tomography (CT) scan revealed the presence of an irregular, soft tissue attentiating mass at the level of right upper lobe encasing and compressing the right main bronchi. The CT also disclosed secondary pulmonic consolidation, and bronchiectatic changes as evidenced by air bronchogram (Figure 2). Chronic bronchitis was present in both lung fields. There was no evidence of calcification. Contrast enhanced computed Figure 1. Chest roentgenogram showing fungus ball in the right upper lobe. TK Lahiri et al tomography (CECT) showed heterogeneous enhancement of the mass. Right posterolateral thoracotomy revealed marked adhesions, markedly thickened and vascular parietal pleura along with complete destruction of the posterior segment of the right upper lobe due to a post tubercular cavity. This cavity contained a fungus ball about 7 cm x 6 cm x 6 cm along with pus. Removal of the fungus ball along with segmental resection of right posterior segment and wedge resection of the apical segment of the right upper lobe performed. Gross pathological examination revealed multiple friable, soft masses from 0.2 5m diameter (Figure 3), and also fibrofatty tissue of 3.5 cm and 2 cm with a greyish white appearance. Microscopic examination demonstrated sheets of unseptate hyphae with haphazard branching and parallel wall. No other viable cellular elements had been seen. The section from wedge resected specimen revealed pseudostratified columner lining with chronic inflammatory cells comprising plasma cells and lymphocytes. There was associated Figure 2. CT scan showing fungus ball on the posterior segment of the right upper lobe. Figure 3. Resected large tungus ball.2001; Vol. 43 endothelial cell proliferation. Histologic study of the fungal mass using hematoxylin-eosin stain revealed features consistent with pulmonary mucorales family. Granuloma formed of colonies of broad nonseptate hyphae with oval zygophores and sporangiophores (asexual propagules-Figure 4). Figure 4. Photomicrograph showing granuloma formed of colonies of broad nonseptate hyphae with parallel branching and with oval zygophores and sporangiophores of mucorales family [H & E x 400] DISCUSSION Mucormycosis also known as zygomycosis or phycomycosis includes infection produced by fungi of the class of Zygomycetes of the family Mucoraceae. The species of several allied genera like Rhizopus, Rhizomucor, Mucor, Absidia, Conidiobolus, Saksenaca and Cunninghanulla can produce human infection. The spores of these organisms are a part of normal respiratory flora. Predisposing factors of mucorales infection are associated diabetes, immunosuppressed host, lymphoma, leukaemia, neutropenia, solid tumours, elastoplast bandages, burns, renal disease, long term treatment with steroids and antimetabolites. Most human infections are acquired by inhalation of sporangiospores usually in immunocompromised states. They are capable of growth under aerobic, anaerobic and microaerophilic conditions*. ‘The clinical onset may be acute or chronic and may be life threatening when invasive or indolent in the immunocompetent hosts. The fungi may disseminate or result in a pneumonia, fungus ‘The Indian Journal of Chest Diseases & Allied Sciences 109 ball, or may present with and allergic manife- station. The main clinical association is with diabetes or hematological malignancy in mucorales infection. In minimally immunocompromised hosts the disease may be asymptomatic, or produce a slowly progressive upper lobe cavity colonization with or without a fungal ball. Fungal balls may be associated with 11 to 17% cases of tuberculosis, 10% cases of sarcoidosis, bronchiectasis, chronic lung abscess, radiation fibrosis, cavitary carcinoma, intralobar pulmonary sequestration and bronchial ankylosing spondylitis’. In more severe immunocompromised host the illness begin as an acute pneumonia with fever and cough along with blood vessel invasion, and followed by pulmonary infarction with haemoptysis and pleuritic pain with rapidly progressive downhill course”, The chest roentgenogram may reveal nodular, lobar, or wedge shaped infiltrate, mediastinal widening, hilar or mediastinal adenopathy, bronchopneumonia, solitary nodule, miliary pattern, cavitation, intracavitary masses and pleural effusion. A solid rounded mass of intense density within an ovoid or spherical cavity, separated from the wall by an air space of variable size and shape along with or without movement in different position is classic for pulmonary fungus ball, roentgenographically’. ‘Computed tomographic scan has been used to image pulmonary mucormycosis. Involvement of multiple segments, walls with only thickening of membranous linings, no air fluid level, minimal bone destruction, demonstration of associated infarction, haemorrhage or abscess, bronchial occlusion, extrapulmonary invasion and pulmonary artery pseudoaneurysm may be found’, Macroscopically pulmonary mucorales with fungus ball consists of a round to oval shaped mass of greenish yellow or brown granular material situated within a cavity composed of fibrous wall of variable thickness. The diagnosis of mucorales is based on the presence of broad, nonseptate (cellophane tubules), upto right angled branching hyphae stained with methamine siliver’. Mycologic isolation of the organism plays a smaller role in diagnosis*. The110 Pulmonary Mucormycosis organisms are also seen in aqueous potassium hydroxide preparation. Reliable serologic tests are not available. Management lies in timely diagnosis with a high index of suspicion. The proper therapy of pulmonary mucoraceous fungus ball is to treat promptly with amphotericin B and resect the involved pulmonary tissue*™", Sporadic reports suggest that cavitary pulmonary lesions are often. treated beneficially by intracavitary irrigation with antifungal agents, In clinically stable patients with acceptable pulmonary reserve, localized pulmonary disease, and improving clinical and immune status, strong consideration should be given for surgical resection of a pulmonary mass or nodule caused by mucorales infection". The use of hyperbaric oxygen therapy as an adjunctive measure has been used. Of twenty cases of mucormycosis with diabetes mellitus, nine cases survived segmental resection or lobectomies", The motality has been improved from six percent to more than 73 percent. In patients with widespread bilateral disease in whom surgical resection is contraindicated intravascular coil embolism therapy may be found to terminate hemorrhage, and is life saving’. Amphotericin B is a naturally occurring, polyene antibiotic and at a dose of 1.0 to 1.5 mgm/kg/day is effective in pulmonary mucormycosis". AMBISONE is a smail unilamellar liposomal preparation in which amphotericin B is associated noncovalently with a pure phospholipid bilayer. This can be administered at 3-5 mgm/kg /day and is superior to amphotericin B both in terms of efficacy and safety?" 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