- Email: [email protected]
Tuberculosis of the trachea
Tubercle (1987) 68. 225-228 0 Longman Group UK Ltd. 1987
C. G. Wathen,” Departments
of *Medicine,
K. M. Kerr,t D. L. Cowan*
tfathology
and SOtolaryngology,
and A. C. Douglas*
Royal Infirmary,
Edinburgh
EH3 9YW, Scotland
Summary A patient is described who presented with stenosis of the upper trachea causing respiratory distress. There was no evidence of tuberculosis elsewhere in the body but biopsy showed a granulomatous lesion and there was a good response to anti-tuberculosis chemotherapy. A temporary tracheostomy was needed.
Les auteurs rapportent le cas d’un patient qui pr&ente une stbnose de la trachee supbrieure entrainant des difficult&s respiratoires. II n’y avait nulle part d’evidence de tuberculose dans I’organisme, mais la biopsie a montrCI une I&ion granulomateuse qui a bien repondu B la chimiotherapie antituberculeuse. Une tracheotomie temporaire a 6te n&essaire.
Resumen Se describe el case de un un paciente que presenta una estenosis de la tr6quea superior que provoca dificultades respiratorias. No habia evidencias de tuberculosis en parte alguna del organismo, pero la biopsia mostr6 una lesi6n granulomatosa que respondi bien a la quimioterapia antituberculosa. Se hizo necesaria una traqueotomia temporal. Introduction Tuberculosis of the larynx associated with pulmonary tuberculosis is well recognised [II and presents as hoarseness, haemoptysis or dysphagia. Tracheobronchial tuberculosis in association with pulmonary disease is also well documented 121. Upper tracheal swelling with stridor as a presentation of tuberculosis does not appear to have been described previously although development of distal tracheal stenosis during treatment of sputum positive tuberculosis has been recorded (31.
Case report A 46-year-old woman presented with extreme dyspnoea and stridor. She had progressive breathlessness over about 5 weeks associated with lethargy, tiredness and occasional sweats, particularly at night. Two years previously she was diagnosed as having episcleritis and had been prescribed corticosteroid eye drops. Her other past medical history was of varicose veins, tubal sterilisation and mild hypertension. There were no features of thyroid disease. At the time of presentation her only therapy was hydrochlorothiazide. On examination she was apyrexial and was noticed to have episcleritis of the right eye. ‘Address
for correspondence:
Dr C G Wathen.*
Department
of Medicine,
Royal Infirmary,
Edinburgh Eli3 9YW
226
Wathen
and others
She had stertorous respiration, 20 breaths per min and her heart rate was 120/min. General examination of the chest, cardiovascular system, abdomen and central nervous system failed to show any other abnormality. Indirect laryngoscopy was carried out at the time of admission and this showed a smooth non-ulcerating concentric swelling of the upper trachea immediately below the vocal cords. Tracheostomy was performed and biopsies of the swelling, trachea and thyroid isthmus were taken. Biopsy specimens from the region of subglottic stenosis had a non-dysplastic but metaplastic squamous epithelium. The lamina propria showed focal fibrosis, mild chronic inflammation and occasional poorly formed epitheloid and giant cell granulomas without caseation. The tracheal mucosal biopsy showed a more marked chronic inflammation but otherwise similar changes. Cartilage present in the biopsies appeared normal. Tissue taken from the thyroid isthmus also showed scattered non-caseating granulomata but there were no features of a primary thyroiditis. Special stains for fungi, tubercle bacilli and amyloid were negative and foreign material was not identified. The histological findings were non-specific. She was commenced on prednisolone 30 mg daily, reducing to complete withdrawal 10 weeks later. This produced some improvement in the patient’s symptoms but on stopping treatment she again complained of extreme dyspnoea which worsened dramatically whenever the tracheostomy was occluded. A repeat indirect laryngoscopy showed that the sub-glottic swelling was still present and unchanged in size. Further biopsy showed a metaplastic epithelium with underlying
Figure 1.
Laryngeal
tomograms
in phonation,
before
(a) and after (b) antituberculosis
chemotherapy.
Tuberculosis
of the
trachea
227
scarring and a focal, heavy lymphocyte and plasma cell infiltrate. No granulomata or other more specific features were present. Other investigations had shown a normal haemoglobin and white cell count, a high ESR between 74 and 110 mm per hour, falling with the corticosteroid therapy and rising again when this was stopped, and a slightly raised platelet count at 485x10g/l. Urea and electrolytes, bilirubin, alkaline phosphatase and serum angiotensin converting enzyme were all within the normal range. Antinuclear factor testing and a standard autoantibody screen were negative as were viral serology and tests for syphilis. The chest X-ray was normal. Laryngeal tomograms showed a concentric swelling, 3 cm long, extending down from the true cords in the upper trachea (Fig. la). Repeat tomography showed improvement during the period of prednisolone treatment but there was subsequent deterioration. The Mantoux test (1 T.U.) was carried out after withdrawal of prednisolone and resulted in 30 mm of induration. At the time of initial presentation, the Mantoux test had been only weakly positive. Early morning urine specimens and laryngeal swabs were negative on culture for tuberculosis. Two Kveim tests were negative and renal biopsy showed no evidence of renal disease: in particular, there was no evidence to support a diagnosis of either sarcoidosis or Wegener’s granuloma. On the basis of the pathological reports and the very strongly positive Mantoux test, a trial of antituberculosis chemotherapy was begun with rifampicin, ethambutol and isoniazid for 8 weeks, followed by rifampicin and isoniazid for 7 months. Over the 9 months there was progressive improvement in the patient’s breathing and the tracheostomy was blocked off after about 8 weeks. The forced expiratory volume (FEV,) increased from 1.8 to 2.3 I. On completion of the course, the tracheostomy was removed and repeat laryngoscopy showed no evidence of the previous disease in the upper trachea. After treatment was completed the Mantoux test (1 T.U.) gave a 15 mm induration. Tracheal tomograms were entirely normal (Fig. lb), the ESR had fallen to 6 mm and the patient was asymptomatic. She has remained well for 18 months and repeat laryngoscopy was normal. Discussion In view of the very strongly positive tuberculin test and the response to the anti-tuberculosis chemotherapy, tuberculous aetiology seems fairly certain. The results of the biopsy also give some support to the diagnosis. The appearances of the biopsies, however, are non-specific being those of granulomatous chronic inflammation with oedema and fibrosis. The presence of a few granulomas in the adjacent thyroid is perhaps surprising. However, there is evidence that laryngeal tuberculosis may arise as a result of haematogenous rather than aerogenous spread from a pulmonary focus [41.In this patient, haematogenous spread from the trachea may explain the thyroid lesions. Alternative diagnoses such as Wegener’s granuloma, sarcoidosis and polychondritis are untenable on the basis of the clinicopathological findings. Tuberculous tracheitis is extremely rare and usually occurs in the presence of overt pulmonary disease [2,31. In our patient there was no clinical evidence of tuberculosis which might have led to earlier diagnosis and her stridor was treated initially with a tracheotomy which was required for a period whilst the subglottic lesion was producing symptoms.
Acknowledgements The authors would like to thank Dr A. J. Wightman Johnstone for typing the manuscript.
for reviewing
the radiology
and Mrs J.
228
Wathen
and others References
1 Salmon, L. F. W. (1979). Chronic laryngitis, Chapter Throat, Volume 4. Butterworths, London.
14 (p. 402) In: Scott-Brown’s
Diseases
of the Ear, Nose and
2 Auerbach, 0. (1949). Tuberculosis of the trachea and major bronchi. American Review of Tuberculosis 60, 604. 3 Mellem, H., Boye, N. P., Arnkvaern, R., Fgeld, N. Bj. (1986). Stenotic tuberculous tracheitis treated with resection and anastomosis. European Journal Respiratory Disease, 66, 224. 4 Hunter, A. M., Millar, J. W., Wightman, A. J., Horne, N. W. (1981). The changing pattern of laryngeal tuberculosis. Journal of Laryngology and Otology 95, 393.
C. G. Wathen,” Departments
of *Medicine,
K. M. Kerr,t D. L. Cowan*
tfathology
and SOtolaryngology,
and A. C. Douglas*
Royal Infirmary,
Edinburgh
EH3 9YW, Scotland
Summary A patient is described who presented with stenosis of the upper trachea causing respiratory distress. There was no evidence of tuberculosis elsewhere in the body but biopsy showed a granulomatous lesion and there was a good response to anti-tuberculosis chemotherapy. A temporary tracheostomy was needed.
Les auteurs rapportent le cas d’un patient qui pr&ente une stbnose de la trachee supbrieure entrainant des difficult&s respiratoires. II n’y avait nulle part d’evidence de tuberculose dans I’organisme, mais la biopsie a montrCI une I&ion granulomateuse qui a bien repondu B la chimiotherapie antituberculeuse. Une tracheotomie temporaire a 6te n&essaire.
Resumen Se describe el case de un un paciente que presenta una estenosis de la tr6quea superior que provoca dificultades respiratorias. No habia evidencias de tuberculosis en parte alguna del organismo, pero la biopsia mostr6 una lesi6n granulomatosa que respondi bien a la quimioterapia antituberculosa. Se hizo necesaria una traqueotomia temporal. Introduction Tuberculosis of the larynx associated with pulmonary tuberculosis is well recognised [II and presents as hoarseness, haemoptysis or dysphagia. Tracheobronchial tuberculosis in association with pulmonary disease is also well documented 121. Upper tracheal swelling with stridor as a presentation of tuberculosis does not appear to have been described previously although development of distal tracheal stenosis during treatment of sputum positive tuberculosis has been recorded (31.
Case report A 46-year-old woman presented with extreme dyspnoea and stridor. She had progressive breathlessness over about 5 weeks associated with lethargy, tiredness and occasional sweats, particularly at night. Two years previously she was diagnosed as having episcleritis and had been prescribed corticosteroid eye drops. Her other past medical history was of varicose veins, tubal sterilisation and mild hypertension. There were no features of thyroid disease. At the time of presentation her only therapy was hydrochlorothiazide. On examination she was apyrexial and was noticed to have episcleritis of the right eye. ‘Address
for correspondence:
Dr C G Wathen.*
Department
of Medicine,
Royal Infirmary,
Edinburgh Eli3 9YW
226
Wathen
and others
She had stertorous respiration, 20 breaths per min and her heart rate was 120/min. General examination of the chest, cardiovascular system, abdomen and central nervous system failed to show any other abnormality. Indirect laryngoscopy was carried out at the time of admission and this showed a smooth non-ulcerating concentric swelling of the upper trachea immediately below the vocal cords. Tracheostomy was performed and biopsies of the swelling, trachea and thyroid isthmus were taken. Biopsy specimens from the region of subglottic stenosis had a non-dysplastic but metaplastic squamous epithelium. The lamina propria showed focal fibrosis, mild chronic inflammation and occasional poorly formed epitheloid and giant cell granulomas without caseation. The tracheal mucosal biopsy showed a more marked chronic inflammation but otherwise similar changes. Cartilage present in the biopsies appeared normal. Tissue taken from the thyroid isthmus also showed scattered non-caseating granulomata but there were no features of a primary thyroiditis. Special stains for fungi, tubercle bacilli and amyloid were negative and foreign material was not identified. The histological findings were non-specific. She was commenced on prednisolone 30 mg daily, reducing to complete withdrawal 10 weeks later. This produced some improvement in the patient’s symptoms but on stopping treatment she again complained of extreme dyspnoea which worsened dramatically whenever the tracheostomy was occluded. A repeat indirect laryngoscopy showed that the sub-glottic swelling was still present and unchanged in size. Further biopsy showed a metaplastic epithelium with underlying
Figure 1.
Laryngeal
tomograms
in phonation,
before
(a) and after (b) antituberculosis
chemotherapy.
Tuberculosis
of the
trachea
227
scarring and a focal, heavy lymphocyte and plasma cell infiltrate. No granulomata or other more specific features were present. Other investigations had shown a normal haemoglobin and white cell count, a high ESR between 74 and 110 mm per hour, falling with the corticosteroid therapy and rising again when this was stopped, and a slightly raised platelet count at 485x10g/l. Urea and electrolytes, bilirubin, alkaline phosphatase and serum angiotensin converting enzyme were all within the normal range. Antinuclear factor testing and a standard autoantibody screen were negative as were viral serology and tests for syphilis. The chest X-ray was normal. Laryngeal tomograms showed a concentric swelling, 3 cm long, extending down from the true cords in the upper trachea (Fig. la). Repeat tomography showed improvement during the period of prednisolone treatment but there was subsequent deterioration. The Mantoux test (1 T.U.) was carried out after withdrawal of prednisolone and resulted in 30 mm of induration. At the time of initial presentation, the Mantoux test had been only weakly positive. Early morning urine specimens and laryngeal swabs were negative on culture for tuberculosis. Two Kveim tests were negative and renal biopsy showed no evidence of renal disease: in particular, there was no evidence to support a diagnosis of either sarcoidosis or Wegener’s granuloma. On the basis of the pathological reports and the very strongly positive Mantoux test, a trial of antituberculosis chemotherapy was begun with rifampicin, ethambutol and isoniazid for 8 weeks, followed by rifampicin and isoniazid for 7 months. Over the 9 months there was progressive improvement in the patient’s breathing and the tracheostomy was blocked off after about 8 weeks. The forced expiratory volume (FEV,) increased from 1.8 to 2.3 I. On completion of the course, the tracheostomy was removed and repeat laryngoscopy showed no evidence of the previous disease in the upper trachea. After treatment was completed the Mantoux test (1 T.U.) gave a 15 mm induration. Tracheal tomograms were entirely normal (Fig. lb), the ESR had fallen to 6 mm and the patient was asymptomatic. She has remained well for 18 months and repeat laryngoscopy was normal. Discussion In view of the very strongly positive tuberculin test and the response to the anti-tuberculosis chemotherapy, tuberculous aetiology seems fairly certain. The results of the biopsy also give some support to the diagnosis. The appearances of the biopsies, however, are non-specific being those of granulomatous chronic inflammation with oedema and fibrosis. The presence of a few granulomas in the adjacent thyroid is perhaps surprising. However, there is evidence that laryngeal tuberculosis may arise as a result of haematogenous rather than aerogenous spread from a pulmonary focus [41.In this patient, haematogenous spread from the trachea may explain the thyroid lesions. Alternative diagnoses such as Wegener’s granuloma, sarcoidosis and polychondritis are untenable on the basis of the clinicopathological findings. Tuberculous tracheitis is extremely rare and usually occurs in the presence of overt pulmonary disease [2,31. In our patient there was no clinical evidence of tuberculosis which might have led to earlier diagnosis and her stridor was treated initially with a tracheotomy which was required for a period whilst the subglottic lesion was producing symptoms.
Acknowledgements The authors would like to thank Dr A. J. Wightman Johnstone for typing the manuscript.
for reviewing
the radiology
and Mrs J.
228
Wathen
and others References
1 Salmon, L. F. W. (1979). Chronic laryngitis, Chapter Throat, Volume 4. Butterworths, London.
14 (p. 402) In: Scott-Brown’s
Diseases
of the Ear, Nose and
2 Auerbach, 0. (1949). Tuberculosis of the trachea and major bronchi. American Review of Tuberculosis 60, 604. 3 Mellem, H., Boye, N. P., Arnkvaern, R., Fgeld, N. Bj. (1986). Stenotic tuberculous tracheitis treated with resection and anastomosis. European Journal Respiratory Disease, 66, 224. 4 Hunter, A. M., Millar, J. W., Wightman, A. J., Horne, N. W. (1981). The changing pattern of laryngeal tuberculosis. Journal of Laryngology and Otology 95, 393.