Infection

Madurella mycetoma—a rare case with cranial extensionShradha Maheshwari MBBSa, Antonio Figueiredo MBBSa,

Swati Narurkar MD, DNBb, Atul Goel MChc,d,⁎aDepartments of Neurosurgery

bHistopathology, Lilavati Hospital and Research Center, Mumbai 400 050, IndiacDepartment of Neurosurgery, Seth G.S. Medical College and K.E.M. Hospital, Parel, Mumbai 400 012, India

dLilavati Hospital and Research Center, Mumbai 400 050, India

Received 28 December 2008; accepted 11 June 2009

Abstract Background:Madurella species of fungus causes chronic subcutaneous infection of lower extremities;

Abbreviations: CNS,⁎ Corresponding

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the infection is commonly labeled as Madura foot. We report a case of Madurella infection involvingthe cranial cavity. Such an involvement by Madurella fungal infection is not recorded in the literature.Case description: A 31-year-old nonimmunocompromised male patient presented with complaintsof left hemifacial pain for 1 year and diplopia on looking toward left side for a period of 2 weeks. Onexamination, he had ipsilateral sixth nerve paresis. Investigations revealed a large paranasal sinuslesion that extended in the cavernous sinus. The lesion was partially resected. Histologicexamination revealed that the lesion was a fungus Madurella mycetomi.Conclusion: A rare cranial extension of Madurella fungal infection is reported.© 2010 Elsevier Inc. All rights reserved.

Keywords: Fungal infection; Cavernous sinus; Madurella mycetoma

1. Introduction

Madurella species of fungus more frequently causeschronic subcutaneous infection of the lower extremities, andsuch infections are commonly labeled as Madura foot.Fungal infections related to Madurella species has beenrelatively infrequently identified in several other locations inthe body. We present a case of Madurella infection of theparanasal sinuses that extended into the intracranialcompartment. We could not locate any case of intracranialinvolvement of Madurella fungal infection in the literature.

2. Case report

A 31-year-old male, an Ayurvedic doctor by profession,hailing from a village in the state of Rajasthan in India,

central nervous system; MRI, magnetic resonance imagingauthor. Department of Neurosurgery, Seth G.S.KEM Hospital, Parel, Mumbai 400 012, India. Tel.:001.ulgoel62@hotmail.com (A. Goel).

nt matter © 2010 Elsevier Inc. All rights reserved.009.06.014

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presented with complaints of intermittent extrusion ofblackish “tea leaves-like” granules from his left nostril forabout 2 years. The patient was investigated for the same andfound to have Madurella fungal sinusitis and was started onantifungal drug treatment (ketoconazole, 200 mg BD).Despite the ongoing drug therapy, the nasal dischargecontinued for about 1 year. The patient had left hemifacialpain for about 1 year and diplopia on left lateral gaze forabout 2 weeks. The patient was of generally thin build.Neurologic examination revealed left sixth nerve paresis andno other deficit. The routine blood investigations and chestx-ray were normal. There was no evidence of immunocom-promise of any kind. The MRI scan showed a large lesioninvolving the paranasal air sinus that extended into the leftcavernous sinus. It was a mixed attenuation lesion with areasof hyperintensity and hypointensity on T1-weighted images(Fig. 1) and hypointensity on T2-weighted images (Fig. 2).The lesion enhanced on contrast administration (Fig. 3). Aleft basal temporal craniotomy was carried out. Afterretracting the temporal lobe, the lateral wall of the cavernoussinus was seen to be bulging laterally. The lesion was

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Fig. 1. T1-weighted MRI shows a mixed attenuation tumor in the region ofthe cavernous sinus, encasing the internal carotid artery. Extension of thetumor into the paranasal air sinus can be observed.

Fig. 3. Contrast MRI shows the enhancement in the tumor.

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entirely within the confines of the cavernous sinus. It wasexposed between the laterally stretched fibers of the fifthcranial nerve. The lesion was firm, granulomatous, and onlymoderately vascular and had dense adhesions to theadjoining structures. Black granular mass could be identifiedinterspersed in the lesion. The lesion was radically butincompletely resected. The lesion was found to extend intothe paranasal air sinus during the surgery. The patient hadimmediate postoperative relief in facial pain, and the sixthnerve paresis and related diplopia completely recovered.Histopathologic examination showed black-colored grains

Fig. 2. T2-weighted MRI showing the largely hypointense lesion involvingthe cavernous sinus.

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found amid neutrophilic abscesses (Fig. 4). The abscessshowed brown cement matrix and embedded chlamydos-pores and septate hyphae (Fig. 5). These features confirmedthat the infection was due to fungus Madurella mycetomi.The patient was now placed on liposomal-amphotericin Bantifungal drug treatment. The patient took this drugtreatment for a period of 2 months. The cumulative dosewas 3 g. There were no adverse effects related to the drug.He was followed up 7 months after surgery with recurrenceof diplopia. Examination revealed left sixth nerve palsy,and investigations showed a large recurrence of the lesion.In addition, enhancement of the tentorium could beobserved suggesting the intracranial spread of the lesion.In addition, there were several other abscesses in theparanasal sinuses and in the infratemporal fossa. No further

Fig. 4. Chlamydospores and hyphae in brown cementlike matrix(hematoxylin and eosin, original magnification ×200).

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Fig. 5. Pigmented granule surrounded by neutrophils and granulation tissue(hematoxylin and eosin, original magnification ×100).

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surgical treatment was advocated, and the drug treatmentwas continued. At a follow-up of 18 months, the patient isstill having diplopia and nasal discharge of black granulesbut is free of facial pain.

3. Discussion

Fungi are ubiquitous in nature; they find a nidus in humanbody and adopt to its metabolism either in symbiosis orbecome pathogenic [8]. Fungal infection of brain and of thecranial cavity is a rare and challenging therapeutic problem[2,5,8]. Of late, the frequency of fungal infection appears tobe on the rise, probably due to increase in number of caseswith immunocompromised state such as HIV/AIDS andincreased frequency of organ transplantation [2,8]. Thepathologic examination of CNS fungal infection is largelydictated by the size of the fungus—the small size yeast formsenter the microcirculation to cause microabscess andmeningitis, whereas the larger hyphal forms invade thevasculature and manifest as infarcts [3,7,8].

Aspergilloma and zygomycosis (mucormycosis) are 2most common fungal infections that involve the cranial cavityand cerebral parenchyma [4,5,7,8]. Mycetoma is chronicsubcutaneous infection induced by traumatic inoculation withsaprophytic species of fungi or actinomycetes bacteria foundin soil. Fungal agents of mycetoma include Pseudoal-lescheria boydii, Madurella mycetomi, Madurella grisea,Exophiala jeanselmei, and Acremonium falciforme.

The 2 forms of mycetoma are bacterial mycetoma andfungal mycetoma; bacterial mycetoma is known as actino-mycetoma, whereas the fungal form is called eumycetoma.The true incidence and the geographical distribution ofmycetoma throughout the world is not exactly known due tothe nature of the disease that is usually painless, slowlyprogressive that may lead to the late presentation of mostpatients. Mycetoma has a worldwide distribution, but this is

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extremely uneven. It is endemic in tropical and subtropicalregions. The African continent seems to have the highestprevalence. It prevails in what is known as the mycetomabelt stretching between the latitudes of 15° south and 30°north. The belt includes Sudan, Somalia, Senegal, India,Yemen, Mexico, Venezuela, Colombia, Argentina, andothers. In India, its high prevalence is seen in states ofRajasthan, Tamil Nadu, and Andhra Pradesh. Actinomycoticmycetoma is prevalent in South India, south east Rajasthan,and Chandigarh, whereas eumycetoma, which constitutesone third of total cases, is mainly reported from North Indiaand central Rajasthan [1,6,9].

The generally exposed areas of the body that includehands and feet are more frequently involved with mycetoma.Lesions are usually characterized by suppuration, abscessformation, granulomas, and formation of draining sinuses thatcontains granules. These granules may range up to 2 mm, arehard, and contain intertwined septate hyphae that are typicallydistorted and enlarged at the periphery of the granule. Thesegranules help in the diagnosis of the fungal species [2,8].

Madurella mycetoma usually involves the lower extrem-ities, and intracranial spread of the infection has not beenreported before in the literature. The organisms may reachthe brain via traumatic inoculation or spread from thesurrounding infected structures. In the presented case, therewas evidence of paranasal infection [8]. The typicalhistopathologic picture seen in the lower limb Madurellamycetoma was seen in this patient. The black grains ofMadurella mycetoma found amid neutrophilic abscesses,contain uniform rust brown cement, chlamydospores, andseptate hyphae with expanded terminal ends [2,8].

Surgical debridement followed by treatment with itraco-nazole, ketoconazole, or amphotericin B are found to beeffective in mycetoma of the lower limb [2].

References

[1] Chakrabarti, Singh K. Mycetoma in Chandigarh and surrounding area.Ind J Med Microbiol 1998;16:64-5.

[2] Friedman AH, Bullitt E. Fungal infections. In: Wilkins RH, RengacharySS, editors. Neurosurgery, 2nd ed., vol. 3. New York: McGraw-Hill;1996. p. 3385-94.

[3] Goel A, Nadkarni T, Desai AP. Aspergilloma in paracavernous sinusregion—two case reports. Neurol Med Chir (Tokyo) 1996;36:733-6.

[4] Goel A, Satoskar A, Desai AP. Brain abscess caused by Cladosporiumtrichoides. Br J Neurosurg 1992;6:589-92.

[5] Kothari M, Goel A. Fungi: too evolved to be condemned. Neurol India2007;55:189-90.

[6] Mahogonb ES. Agents of mycetoma. In: Mandell LG, Benett JE,Douglas R, editors. Principles and practice of infectious diseases. 3rd ed.New York: Churchill Livingstone; 1990. p. 1977.

[7] Nadkarni T, Goel A. Aspergilloma of brain: an overview. J PostgradMed 2005;51:37-41.

[8] Shankar SK, Mahadevan A, Sundaram C. Pathobiology of fungalinfections of CNS with special reference to Indian scenario. NeurolIndia 2007;55:198-215.

[9] Sundaram C, Umabala P, Laxmi V, Purohit AK, Prasad VS,Panigrahi M, et al. Pathology of fungal infections of the centralnervous system: 17 years experience from South India. Histopatho-logy 2006;49:396-405.

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