©1991 S. Karger AG, Basel 0025-793W9I/D584-Ü207 S 2.75/0
Respiration 1991;58:207-210
Tracheobronchomegaly Associated with Interstitial Pulmonary Fibrosis C. Vidal, F. Peña, M. Rodriguez Mosquera. A. Gonzalez Quiniela Clínica Puerta de Hierro, Madrid, Spain
Key Words. Tracheobronchomegaly • Interstitial pulmonary fibrosis
Introduction
Tracheobronchomegaly (TBM) is a rare condition first described by Mounier-Kuhn [1] in 1932. It is char acterized by a marked dilatation of the trachea and major bronchi. Katz et al. [2] clearly defined this enti ty and suggested this name on the basis of both ana tomic and radiographic findings. TBM has frequently been associated with recurrent and chronic respira tory tract infections, although some patients remain completely asymptomatic [3— 5]. In this report we de scribe a 42-year-old male with TBM and diffuse in terstitial pulmonary fibrosis (IPF). Case Report A 42-ycar-old male was admitted to our hospital with a clin ical picture of progressive dyspnea on exertion, non-productive cough, pleuritic chest pain and easy fatigue for the last 8 years. He had smoked 30 cigarettes per day for 10 years until he was 32year-old. He had worked as a car painter but he was retired by the time of admission. For the last 2 months, the patient's dys pnea began to appear at low levels of exertion and he had lost 12 kg of weight. Physical examination revealed a malnourished patient. His voice was low in pitch. There were scattered dry. late inspiratory
rales on chest auscultation. ORL exploration demonstrated two laryngeal cysts. No abnormality was detected in any other system. Routine complementary studies showed an erythrocyte sedimen tation rate of 42 mm in the first hour; pulmonary function tests showed a vital capacity of 1.423 liters (33% of the predicted vol ume); 1-second forced expiratory volume of 0.950 liter; Tiffeneau was 66.7%; total lung capacity was 4.259 liters (41% of the pre dicted value) and the residual volume was 2.759 liters (the pre dicted value was 1.960). Arterial blood gases (FIO,: 21%) were as follows; pH 7.39; pO:, 50 mm Hg; pCO,, 49 mm Hg; CO,H. 30 mEq/1: Sat O,. 90% and carboxvhemoglobin. 1.1%. The electrocar diogram revealed right axis deviation and evidence of right ven tricular enlargement; chest roentgenograms (fig. 1) showed dilata tion of the trachea and an important bilateral interstitial disease with a destructive pattern compatible with long-standing lung fi brosis; no mediastinal masses were noted (previous chest x-ray films, 8 years before, were normal). Upper gastrointestinal series (fig. 2) showed spontaneous reflux of barium contrast to the tra chea and bronchial tree; the hypopharynx and the cervical esopha gus were narrowed because of an extrinsic mass which in a CT scan (fig. 3) was demonstrated to be the enlarged trachea (diame ters of the trachea, right main bronchus and left main bronchus measured by this method were 36, 19 and 20 mm, respectively). A diffuse increased uptake in both lungs was observed in a 67 Ga scintigraphy; bronchoscopy disclosed no alterations other than a diffuse distortion of the bronchial tree with hypotrophic mucosae; cellularity of bronchoalveolar lavage was composed almost exclu sively of neutrophils (98%); transbronchial biopsy yielded scarce alveolar tissue, but it showed a fibrotic reaction with little inflam matory response. The rest of the complementary studies perform-
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
Abstract. Tracheobronchomegaly (TBM) is the syndrome of enlarged trachea and main bronchi associated with recurrent and chronic respiratory tract infections. A 42-year-oid man with TBM and diffuse interstitial pulmonary fibrosis is described. The possible relationship between the two entities is discussed and the etiol ogy, pathogenesis, clinical manifestations, prognosis and treatment of TBM are reviewed.
Vidal/Pcna/Mosqucar/Quintela
208
Fig. 1. Dilaiation of the trachea and bilateral interstitial disease with a destructive pattern. Posteroanterior (a) and lateral (b) chest X-ray films.
ed in serum (SMA-12, antinuclear antibodies, a,-antitrypsin. VDRL. fractions 3 and 4 of complement, quantification of immu noglobulins and protein electrophoresis) were negative or within normal limits. Prednisone treatment (1 mg/kg of body weight) was begun with prompt improvement of the general status, the exercise perform ance and the objective parameters of pulmonary function (vital capacity of 1.703 liters. 39.4% of the predicted value). Two months later, corticosteroids were tapered and finally discontinued after (> months. The patient maintained a satisfactory condition with moderate dyspnea on exertion; (pO. of 66 mm Hg) for 1 month. Afterwards, the patient complained of renewed dyspnea at low levels of exertion and malaise. Arterial basal pO, was 52 mm Hg. Neither symptoms nor radiologic findings of bronchopulmonary infection were present. Corticosteroid therapy was reinstituted at a dose of 10 mg prednisone daily with prompt improvement. An alternate-day schedule with the same dose was not tolerated be cause of increasing dyspnea. Presently, the patient needs a dose of 10 mg prednisone daily to maintain his standard exercice tolerance and an arterial basal pO, of 64 mm Hg.
Fig. 2. Barium study of the esophagus: contrast retention in the cervical esophagus with spontaneous reflux into the tracheobron chial tree. Diameter of the esophagus is diminished by extrinsic compression.
Katz et al. [2], after studying 50 normal adults, es tablished that the normal maximum transverse diame ters of the trachea and the main bronchi should be 20 and 14.5 mm, respectively [2], Himalstein and Gal lagher [6] dealt with 69 reported cases of TBM in a
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
Discussion
Tracheobronchomegaly
Fig. 3. Thorax CT-sean. a Destructive lung pattern, more evi dent in upper lobes, is present, b Increased tracheal lumen with no mediastinal masses, c Bronchial dilatation, air cysts with rough walls can be seen, which do not spare the subpleura! space.
Acknowledgment The authors thank Mrs. Martha Messman for the English translation of the manuscript.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
1973 review of the literature. They indicate that the diagnosis should be suspected when the transverse di ameter of the trachea exceeds 25 mm; the right main bronchus, 23 mm; and the left main bronchus, 20 mm. Fraser and Pare [7] consider a tracheal measurement greater than 30 mm as diagnostic of TBM. TBM usu ally becomes manifest between the ages of 30 and 50 years, and occurs predominantly in males [7, 8], The etiology of this rare condition remains un known. It has been suggested that a congenital con nective tissue defect could be responsible [9]. This is corroborated by the occasional association of TBM with Ehler-Danlos syndrome [10] and cutis laxa [11]. Other authors consider TBM an acquired disease [12, 13], Parris and Johnson [13] presented a case of an adult TBM secondary to intensive radiotherapy. TBM has also been associated with bronchiectasis and em physema [7], The clinical picture of TBM can be high ly variable, ranging from no symptoms to the full spectrum of infection and respiratory failure. For in stance, Guest and Anderson [3] reported a case of TBM leading to death from uncontrolled infection and respiratory insufficiency [3, 9]. The usual clinical features include productive cough of copious and pu rulent sputum, low-grade fever, dyspnea and hoarse ness [8]. The medical treatment of TBM is limited to appropriate systemic and topical antibiotics combined with physical measures. However, even with optimal care, respirator)' tract infections limit pulmonary function [4]. The pathogenesis of IPF in the case reported here remains unclear. On the one hand, it is well known that TBM predisposes to bronchial infections [3-5]; in fact, in our case, spontaneous reflux to the respiratory tree was observed during swallowing of barium con trast. In this sense, lung fibrosis could be due either to chronically infected bronchiectasis or to recurrent as piration pneumonitis or to both, respectively. On the other hand, lung uptake in 67 Ga scintigraphy, neu trophil predominance in bronchoalveolar lavage and response to corticosteroids point to an inflammatory lung disease such as idiopathic interstitial pneumo nitis. It is unlikely that TBM is secondary to a respira tory disease associated with chronic cough because of the different pattern of enlargement of the trachea often seen in cases of pulmonary fibrosis and chronic bronchitis, in which only the membranous part of the tracheal wall is affected [5] rather than both membra nous and cartilaginous parts of this organ, as in the case presented here.
209
210
Vidal/Peña/Mosquear/Quintcla
1 Mounier-Kuhn P: Dilatation de la trachée; constatations ra diographiques et bronchoscopiques. Lyon Méd 1932;150:106— 109. 2 Katz I, Levine M, Herman P: Tracheobronchiomegaly: The Mounicr-Kuhn syndrome. Am J Roentgenol 1962;88:10841093. 3 Guest JL, Anderson JN: Tracheobronchomegaly (MounierKuhn syndrome). JAMA 1977;238:1754-1755. 4 Reisz G. Rosa U, Pingleton SK: Tracheobronchomegaly caus ing recurrent pneumonia. JAMA 1981;246:1705-1706. 5 Al-Mallah Z. Quantock OP: Tracheobronchomegaly. Thorax 1968;23:320-324. 6 Himalstein MR, Gallagher JC: Tracheobronchomegaly. Ann Otol Rhinol Laryngol 1973;82:223-227. 7 Fraser RG, Paré FAP: Diagnosis of diseases of the chest. Phi ladelphia, Saunders, 1979, pp 1322-1328. 8 Martinez Orozco F, Ancochea L, Villalta. Ingclmo M, BalcellsGorina A: Traqueobroncomegalia asociada a diverticulosis di gestiva multiple. Med Clin (Bare) 1981;77:435-438. 9 Johnston RF, Green RA: Tracheobronchiomegaly: Report of five cases and demonstration of familial occurrence. Am Rev Respir Dis 1965;91:35-50.
10 Aaby GV. Blake HA. Tracheobronchiomegaly. Ann Thorac Surg 1966;2:64-70. 11 Wanderer AA, Ellis EF. Goltz RW, Cotton EK: Tracheobron chiomegaly and acquired cutis laxa in a child. Pediatrics 1969:44:709-715. 12 Feist JH. Johnson TH, Wilson RJ: Acquired tracheomalacia: Etiology and differential diagnosis. Chest 1975:68:340-345. 13 Parris WC, Johnson AC: Tracheomegaly. Anesthesiology 1982;56:141-143.
Received: April 23, 1990 Accepted after revision: February 18, 1991 Carmen Vidal, MD Servicio de Alergia Clínica Puerta de Hierro c/ San Martin de Porres, 4 E-28035 Madrid (Spain)
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
References
Respiration 1991;58:207-210
Tracheobronchomegaly Associated with Interstitial Pulmonary Fibrosis C. Vidal, F. Peña, M. Rodriguez Mosquera. A. Gonzalez Quiniela Clínica Puerta de Hierro, Madrid, Spain
Key Words. Tracheobronchomegaly • Interstitial pulmonary fibrosis
Introduction
Tracheobronchomegaly (TBM) is a rare condition first described by Mounier-Kuhn [1] in 1932. It is char acterized by a marked dilatation of the trachea and major bronchi. Katz et al. [2] clearly defined this enti ty and suggested this name on the basis of both ana tomic and radiographic findings. TBM has frequently been associated with recurrent and chronic respira tory tract infections, although some patients remain completely asymptomatic [3— 5]. In this report we de scribe a 42-year-old male with TBM and diffuse in terstitial pulmonary fibrosis (IPF). Case Report A 42-ycar-old male was admitted to our hospital with a clin ical picture of progressive dyspnea on exertion, non-productive cough, pleuritic chest pain and easy fatigue for the last 8 years. He had smoked 30 cigarettes per day for 10 years until he was 32year-old. He had worked as a car painter but he was retired by the time of admission. For the last 2 months, the patient's dys pnea began to appear at low levels of exertion and he had lost 12 kg of weight. Physical examination revealed a malnourished patient. His voice was low in pitch. There were scattered dry. late inspiratory
rales on chest auscultation. ORL exploration demonstrated two laryngeal cysts. No abnormality was detected in any other system. Routine complementary studies showed an erythrocyte sedimen tation rate of 42 mm in the first hour; pulmonary function tests showed a vital capacity of 1.423 liters (33% of the predicted vol ume); 1-second forced expiratory volume of 0.950 liter; Tiffeneau was 66.7%; total lung capacity was 4.259 liters (41% of the pre dicted value) and the residual volume was 2.759 liters (the pre dicted value was 1.960). Arterial blood gases (FIO,: 21%) were as follows; pH 7.39; pO:, 50 mm Hg; pCO,, 49 mm Hg; CO,H. 30 mEq/1: Sat O,. 90% and carboxvhemoglobin. 1.1%. The electrocar diogram revealed right axis deviation and evidence of right ven tricular enlargement; chest roentgenograms (fig. 1) showed dilata tion of the trachea and an important bilateral interstitial disease with a destructive pattern compatible with long-standing lung fi brosis; no mediastinal masses were noted (previous chest x-ray films, 8 years before, were normal). Upper gastrointestinal series (fig. 2) showed spontaneous reflux of barium contrast to the tra chea and bronchial tree; the hypopharynx and the cervical esopha gus were narrowed because of an extrinsic mass which in a CT scan (fig. 3) was demonstrated to be the enlarged trachea (diame ters of the trachea, right main bronchus and left main bronchus measured by this method were 36, 19 and 20 mm, respectively). A diffuse increased uptake in both lungs was observed in a 67 Ga scintigraphy; bronchoscopy disclosed no alterations other than a diffuse distortion of the bronchial tree with hypotrophic mucosae; cellularity of bronchoalveolar lavage was composed almost exclu sively of neutrophils (98%); transbronchial biopsy yielded scarce alveolar tissue, but it showed a fibrotic reaction with little inflam matory response. The rest of the complementary studies perform-
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
Abstract. Tracheobronchomegaly (TBM) is the syndrome of enlarged trachea and main bronchi associated with recurrent and chronic respiratory tract infections. A 42-year-oid man with TBM and diffuse interstitial pulmonary fibrosis is described. The possible relationship between the two entities is discussed and the etiol ogy, pathogenesis, clinical manifestations, prognosis and treatment of TBM are reviewed.
Vidal/Pcna/Mosqucar/Quintela
208
Fig. 1. Dilaiation of the trachea and bilateral interstitial disease with a destructive pattern. Posteroanterior (a) and lateral (b) chest X-ray films.
ed in serum (SMA-12, antinuclear antibodies, a,-antitrypsin. VDRL. fractions 3 and 4 of complement, quantification of immu noglobulins and protein electrophoresis) were negative or within normal limits. Prednisone treatment (1 mg/kg of body weight) was begun with prompt improvement of the general status, the exercise perform ance and the objective parameters of pulmonary function (vital capacity of 1.703 liters. 39.4% of the predicted value). Two months later, corticosteroids were tapered and finally discontinued after (> months. The patient maintained a satisfactory condition with moderate dyspnea on exertion; (pO. of 66 mm Hg) for 1 month. Afterwards, the patient complained of renewed dyspnea at low levels of exertion and malaise. Arterial basal pO, was 52 mm Hg. Neither symptoms nor radiologic findings of bronchopulmonary infection were present. Corticosteroid therapy was reinstituted at a dose of 10 mg prednisone daily with prompt improvement. An alternate-day schedule with the same dose was not tolerated be cause of increasing dyspnea. Presently, the patient needs a dose of 10 mg prednisone daily to maintain his standard exercice tolerance and an arterial basal pO, of 64 mm Hg.
Fig. 2. Barium study of the esophagus: contrast retention in the cervical esophagus with spontaneous reflux into the tracheobron chial tree. Diameter of the esophagus is diminished by extrinsic compression.
Katz et al. [2], after studying 50 normal adults, es tablished that the normal maximum transverse diame ters of the trachea and the main bronchi should be 20 and 14.5 mm, respectively [2], Himalstein and Gal lagher [6] dealt with 69 reported cases of TBM in a
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
Discussion
Tracheobronchomegaly
Fig. 3. Thorax CT-sean. a Destructive lung pattern, more evi dent in upper lobes, is present, b Increased tracheal lumen with no mediastinal masses, c Bronchial dilatation, air cysts with rough walls can be seen, which do not spare the subpleura! space.
Acknowledgment The authors thank Mrs. Martha Messman for the English translation of the manuscript.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
1973 review of the literature. They indicate that the diagnosis should be suspected when the transverse di ameter of the trachea exceeds 25 mm; the right main bronchus, 23 mm; and the left main bronchus, 20 mm. Fraser and Pare [7] consider a tracheal measurement greater than 30 mm as diagnostic of TBM. TBM usu ally becomes manifest between the ages of 30 and 50 years, and occurs predominantly in males [7, 8], The etiology of this rare condition remains un known. It has been suggested that a congenital con nective tissue defect could be responsible [9]. This is corroborated by the occasional association of TBM with Ehler-Danlos syndrome [10] and cutis laxa [11]. Other authors consider TBM an acquired disease [12, 13], Parris and Johnson [13] presented a case of an adult TBM secondary to intensive radiotherapy. TBM has also been associated with bronchiectasis and em physema [7], The clinical picture of TBM can be high ly variable, ranging from no symptoms to the full spectrum of infection and respiratory failure. For in stance, Guest and Anderson [3] reported a case of TBM leading to death from uncontrolled infection and respiratory insufficiency [3, 9]. The usual clinical features include productive cough of copious and pu rulent sputum, low-grade fever, dyspnea and hoarse ness [8]. The medical treatment of TBM is limited to appropriate systemic and topical antibiotics combined with physical measures. However, even with optimal care, respirator)' tract infections limit pulmonary function [4]. The pathogenesis of IPF in the case reported here remains unclear. On the one hand, it is well known that TBM predisposes to bronchial infections [3-5]; in fact, in our case, spontaneous reflux to the respiratory tree was observed during swallowing of barium con trast. In this sense, lung fibrosis could be due either to chronically infected bronchiectasis or to recurrent as piration pneumonitis or to both, respectively. On the other hand, lung uptake in 67 Ga scintigraphy, neu trophil predominance in bronchoalveolar lavage and response to corticosteroids point to an inflammatory lung disease such as idiopathic interstitial pneumo nitis. It is unlikely that TBM is secondary to a respira tory disease associated with chronic cough because of the different pattern of enlargement of the trachea often seen in cases of pulmonary fibrosis and chronic bronchitis, in which only the membranous part of the tracheal wall is affected [5] rather than both membra nous and cartilaginous parts of this organ, as in the case presented here.
209
210
Vidal/Peña/Mosquear/Quintcla
1 Mounier-Kuhn P: Dilatation de la trachée; constatations ra diographiques et bronchoscopiques. Lyon Méd 1932;150:106— 109. 2 Katz I, Levine M, Herman P: Tracheobronchiomegaly: The Mounicr-Kuhn syndrome. Am J Roentgenol 1962;88:10841093. 3 Guest JL, Anderson JN: Tracheobronchomegaly (MounierKuhn syndrome). JAMA 1977;238:1754-1755. 4 Reisz G. Rosa U, Pingleton SK: Tracheobronchomegaly caus ing recurrent pneumonia. JAMA 1981;246:1705-1706. 5 Al-Mallah Z. Quantock OP: Tracheobronchomegaly. Thorax 1968;23:320-324. 6 Himalstein MR, Gallagher JC: Tracheobronchomegaly. Ann Otol Rhinol Laryngol 1973;82:223-227. 7 Fraser RG, Paré FAP: Diagnosis of diseases of the chest. Phi ladelphia, Saunders, 1979, pp 1322-1328. 8 Martinez Orozco F, Ancochea L, Villalta. Ingclmo M, BalcellsGorina A: Traqueobroncomegalia asociada a diverticulosis di gestiva multiple. Med Clin (Bare) 1981;77:435-438. 9 Johnston RF, Green RA: Tracheobronchiomegaly: Report of five cases and demonstration of familial occurrence. Am Rev Respir Dis 1965;91:35-50.
10 Aaby GV. Blake HA. Tracheobronchiomegaly. Ann Thorac Surg 1966;2:64-70. 11 Wanderer AA, Ellis EF. Goltz RW, Cotton EK: Tracheobron chiomegaly and acquired cutis laxa in a child. Pediatrics 1969:44:709-715. 12 Feist JH. Johnson TH, Wilson RJ: Acquired tracheomalacia: Etiology and differential diagnosis. Chest 1975:68:340-345. 13 Parris WC, Johnson AC: Tracheomegaly. Anesthesiology 1982;56:141-143.
Received: April 23, 1990 Accepted after revision: February 18, 1991 Carmen Vidal, MD Servicio de Alergia Clínica Puerta de Hierro c/ San Martin de Porres, 4 E-28035 Madrid (Spain)
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 6/23/2018 1:49:35 AM
References
