Primary nasal tuberculosis is rare. We report a case that was all the more extraordinary because of the age and sex of the patient (an 11-year-old boy), the unusual associated symptoms (epistaxis and grand mal seizures), and the presence of intracranial extension. Clinical and radiologic findings on our initial evaluation suggested that the patient had a large sinonasal malignancy. The patient manifested no evidence of pulmonary tuberculosis. The diagnosis of primary nasal tuberculosis was established only after we obtained the results of histopathology of the excised mass and a subsequent tuberculin skin test; the diagnosis was confirmed by the patient's rapid response to antituberculosis drug therapy. We also review the relevant literature on this rare condition.
Primary nasal tuberculosis is exceedingly rare; fewer than 100 cases have been reported in the literature. According to Sim and Crowther, the first such case was reported by Clarke in 1876. (2) Most reported cases have occurred in middle-aged women and in immunocompromised patients. In this article, we report a new case of primary nasal tuberculosis that is all the more remarkable for its unusual associated features, which included intracranial extension into the frontal lobe, epistaxis, grand mal seizures, and the age and sex of the patient. This confusing clinical picture presented us with a diagnostic dilemma.
An 11-year-old boy came to our outpatient department with complaints of an intermittent low-grade fever of 4 months' duration, two episodes of grand mal seizure during the preceding 3 months, and a bilateral nasal obstruction that had gradually worsened over the preceding 2 months. He also reported a recent history of epistaxis with crusting and anorexia with weight loss.
On physical examination, the boy was found to have mild right-sided proptosis and a visible expansion of the dorsum of the nose. Anterior rhinoscopy revealed the presence of a smooth bilateral septal bulge that completely obstructed the lumen of the nose. The patient's eye movements and vision were normal. No palpable neck nodes were detected, and systemic examination did not yield any significant findings.
Contrast-enhanced computed tomography (CT) of the head and paranasal sinuses detected a large expansile soft-tissue mass that filled most of both nasal cavities, more so on the right (figure 1). The mass also involved the right ethmoid sinuses and the orbit, and it extended intracranially into the frontal lobe.
The patient was administered general anesthesia, and endoscopic biopsy specimens were taken from different points on the proliferative mass--from the septum and from both nasal cavities. Histopathology revealed that the mass contained granulomas with caseous necrosis as well as Langhans' giant cells and numerous epithelioid cells. Subsequent culture for acid-fast bacilli (AFB) was positive, and an assay for purified protein derivative (PPD) was strongly positive (1.8 x 1.8 cm). The patient's erythrocyte sedimentation rate was 65 mm during the first hour. The results of his kidney function tests were normal, and his total leukocyte count was normal. His alkaline phosphatase level was elevated (178 U/L). Analysis of sputum for AFB was negative, and findings on chest x-ray were normal (figure 2).
On the basis of histopathology and other laboratory values, we established a diagnosis of primary caseous nasal tuberculosis with intracranial extension. The patient was started on standard four-drug antituberculosis therapy (rifampin, isoniazid, ethambutol, and pyrazinamide). He responded quickly and was discharged from the hospital. After 6 months on the antituberculosis drug regimen, the patient was asymptomatic. Follow-up CT revealed that the lesion had resolved completely.
Nasal tuberculosis is a rare, chronic, granulomatous infection caused by Mycobacterium tuberculosis. It can occur either as a primary infection or secondary to an infection at another site. The infection can be introduced into the nose by inhalation of infected droplets or dust and by inoculation via the finger. (3) The rarity of this disease can be explained by the protective functions provided by the ciliary action of the nasal mucosa, the bactericidal properties of the nasal secretions, and the protective mechanisms of the nasal vibrissae.
The usual initial symptoms of nasal tuberculosis are unilateral or bilateral nasal obstruction, which is frequently associated with a mucoid or mucopurulent rhinorrhea and postnasal drip. Epistaxis is sometimes frequent but brief. Pain is usually absent. The infection typically involves the lateral nasal wall and appears as a bright red or nodular thickening in the mucosa, with or without ulceration. (4) The nodular thickening is usually soft and bleeds easily when touched. Onodi (quoted by Chamberlain (5)) described three basic types of nasal tuberculosis: ulcerative, infiltrative, and proliferative. Goguen and Karmody found that exophytic, granular lesions were more common than the ulcerative and infiltrative types. (6) In descending order of frequency, involved sites include the anterior septum, the turbinates, and the nasal floor. Bilateral involvement occurs with septal lesions.
Nasal tuberculosis is most common in immunocompromised patients and in those who live in crowded and unhygienic conditions. There is a distinct female preponderance (female-to-male ratio: 3:1), and the mean age at diagnosis has been reported to be 40 years. (7)
The diagnosis is based on the results of Ziehl-Neelsen staining for AFB, culture of organisms obtained from tissue or nasal washings, characteristic findings on histopathology, and the response to therapy. The typical histopathologic feature is the presence of granulomas with central caseous necrosis; the areas of necrosis are surrounded by epithelioid cells and by Langhans' giant cells that feature multiple nuclei arranged in a horseshoe-shaped pattern. Compared with other types of granuloma, the tubercular variety tends to exhibit a greater number of epithelioid and giant cells. A positive reaction to PPD testing provides additional confirmation of the diagnosis.
Extrapulmonary tuberculosis occurs in 70% of patients who have acquired immunodeficiency syndrome and pulmonary tuberculosis. (8) Therefore, it is essential to test all patients who have extrapulmonary tuberculosis for the presence of human immunodeficiency virus.
The differential diagnoses of nasal tuberculosis include other granulomatous diseases of the nose (e.g., Wegener' s granulomatosis and lethal midline granulomatosis), specific granulomas (e.g., syphilis, rhinoscleroma, and rhinosporidiosis), and fungal granulomas (e.g., blastomycosis, histoplasmosis, and coccidioidomycosis). Leishmaniasis and sarcoidosis also have similar characteristics, which can produce a confusing clinical picture. (9)
In our patient, clinical findings (i.e., epistaxis and fever) and CT findings (i.e., an extensive bilateral nasal and ethmoidal mass with intracranial extension into the frontal lobe and involvement of the right orbit) suggested a malignancy. Our patient did not exhibit any other symptoms that would have suggested a diagnosis of tuberculosis. Our final diagnosis was not established until we obtained the results of histopathology and other tests. The diagnosis was confirmed by the patient's rapid response to antituberculosis drug therapy. We found no evidence of immunosuppression.
This case was unusual in that it occurred in a boy; most cases occur in middle-aged women. Also, our review of the literature turned up no other case that featured intracranial involvement or epilepsy, which makes our case all the more unusual.
In any suspected case of nasal tuberculosis, tissue should be sent for culture and Ziehl-Neelsen staining, although M tuberculosis can be grown only rarely. The more recently developed polymerase chain reaction (PCR) assay holds great promise as a diagnostic tool; as few as five organisms are all that is needed to allow for identification of DNA, even in a preserved specimen. (10) However, the exact specificity of PCR has yet to be determined.
Among the treatments for nasal tuberculosis that have been used are surgical excision, diathermy, cautery, and radium. However, Goguen and Karmody reported that the recurrence rate following excision was greater than 50%. (6) Current recommendations call for a 2-month course of the standard four-drug regimen followed by maintenance therapy with rifampin and isoniazid for 4 months or longer, depending on individual patient requirements, culture results, and sensitivity rates when available.
(1.) Sim DW, Crowther JA. Primary nasal tuberculosis masquerading as a malignant tumour. J Laryngol Otol 1988;102:1150-2.
(2.) Clarke WF. Tuberculous lupus of the tongue, palate, and gums. Trans Pathol Soc (Lond) 1876;27:248-9.
(3.) Messervy M. Primary tuberculoma of the nose with presenting symptoms and lesions resembling a malignant granuloma. J Laryngol Otol 1971;85:177-84.
(4.) Weir N. Acute and chronic inflammation of the nasal cavities. In: Ballantyne J, Groves J, eds. Scott-Brown's Diseases of the Ear, Nose, and Throat. 4th ed. London: Butterworths, 1979:185-7.
(5.) Chamberlain WB. Report of two cases of tuberculosis of the nose. Laryngoscope 1911;21:873-5.
(6.) Goguen LA, Karmody CS, Nasal tuberculosis. Otolaryngol Head Neck Surg 1995;113:131-5.
(7.) Friedmann I. The changing pattern of granulomas of the upper respiratory tract. J Laryngol Otol 1971;85:631-82.
(8.) Dhamgaye TM. Nasal tuberculosis. Otolaryngol Head Neck Surg 1996;114:841-2.
(9.) Waldman SR, Levine HL, Sebek BA, et al. Nasal tuberculosis: A forgotten entity. Laryngoscope 1981;91:11-6.
(10.) Clarridge JE III, Shawar RM, Shinnick TM, Plikaytis BB. Largescale use of polymerase chain reaction for detection of Mycobacterium tuberculosis in a routine mycobacteriology laboratory. J Clin Microbiol 1993;31:2049-56.
From the Department of Otorhinolaryngology, Safdarjung Hospital, New Delhi.
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