Diffuse lesions of the trachea

D i f f u s e L e s i o n s o f the T r a c h e a By Robert H. Choplin, William D. Wehunt, and Elias G. Theros

HE T R A C H E A may participate in a systemic process, may be part of a diffuse respiratory disorder, or may give rise to some unique diffuse diseases of its own. We are unaware of statistics indicating overall disease incidence in the trachea, but the diffuse disorders to be discussed in this article probably make up a relatively small proportion. With the exception of scleroma, there are fewer than 100 cases of each disorder described in the English literature. Nonetheless, this may be misleading because some of the diseases or their tracheal components may be asymptomatic and therefore overlooked. Respiratory symptoms, when present, are always nonspecific and include cough, dyspnea, wheezing, and stridor. Recurrent tracheobronchitis, pneumonia, and hemoptysis may also occur. Patients with the more chronic disorders are often thought to have bronchial asthma for some time before the correct diagnosis is made. Recognition of these disorders requires a knowledge of the symptoms and signs, a high index of suspicion, and good quality films. Many of the conditions that diffusely involve the tracheobronchial tree begin in a particular zone and affect the entire trachea only as the disease evolves. Differential diagnosis may be approached by assessing tracheal width and then noting the location and extent of the involvement. Diffuse narrowing is considerably more common and has many more causes than diffuse widening as is observed in Figure 1, which is a

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From the Department of Radiology, Bowman Gray School of Medicine, Wake Forest University, WinstonSalem, N.C., and the Section of Chest and Mediastinal Radiologic Pathology, Armed Forces Institute of Pathology, Washington, D.C. Robert Choplin, Assistant Professor of Radiology, and Elias G. Theros, I. Meschan Distinguished Professor." Department of Radiology, Bowman Gray School of Medicine, Wake Forest University, Winston-Salem, N.C.; William D. Wehunt; L TC, MC, USA Chief, Section of Chest and Mediastinal Radiologic Pathology, Armed Forces Institute of Pathology, and Assistant Chief of Diagnosis, Walter Reed Army Medical Center, Washington D.C. Address reprint requests to Robert H. Choplin, M.D., Department of Radiology, Bowman Gray School of Medicine, Winston-Salem, N.C. 27103. 9 1983 by Grune & Stratton, Inc. 0270-9295/83/1801~9007502.00/0 38

map illustrating the most likely sites of initial involvement for the diseases to be discussed. Those listed at the midlevel of the trachea are usually extensive at presentation while those listed at both ends become diffuse only as the disease progresses. With the exception of chronic mediastinitis, these disorders are intrinsic to the trachea, and mediastinal widening seldom accompanies them. It should be noted that the vast majority of patients with relapsing polychondritis have narrowing of the proximal trachea, though it is listed as causing widening as well. DISORDERS WITH TRACHEAL NARROWING Proximal Trachea

Infectious Disorders Bacterial and viral. Narrowing of the proximal trachea may take place in a variety of infectious disorders and is often associated with laryngeal involvement. In developed countries, narrowing from a pseudomembrane of diphtheria, or a gumma of syphilis is primarily of historic interest.~'2 Most instances of laryngotracheobronchitis in the United States result from viral infection. Subglottic laryngeal narrowing is common but radiographically discernible involvement extending into the subglottic trachea is unusual. Bacterial pseudomembranous tracheitis may be recognized clinically because of unusually high fever and leukocytosis. 3 These patients fail to respond to therapy for croup and usually require intubation with suctioning to clear the airway. Staphylococcus aureus can b e cultured in more than half of the cases while other patients have had pneumococcus, Klebsiella, Hemophilus, or mixed bacterial infections. When viral cultures have been taken, they have been positive for influenza, parainftuenza, or enterovirus in 50% of the patients. It has been suggested that laryngotracheobronchitis represents a bacterial infection superimposed on ordinary viral croup. Neck radiographs in such patients have demonstrated subglottic laryngeal and proximal tracheal narrowing. 4 Mucosal irregularity and irregular soft tissue densities representing the Seminars in Roentgenology, Vol. XVIII, No. 1 (January), 1983

DIFFUSE LESIONS

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DIFFUSE TRACHEAL LESIONS BY LOCATION

';ili!i "!i !i~s riiar~igm~ r;~ie~176

Fig. 1. Locator map for diffuse tracheal lesions. Disorders are listed at the most likely level of the earliest manifestations. The disorders listed in boldface at the center are usually extensive at presentation, but favor the lower trachea. A, narrowing disorders, and B, widening disorders.

Fig. 2. Pseudomembranous laryngotracheobronchitis. Lateral v i e w of the neck shows diffuse subglottic and tracheal narrowing with irregular mucosal surface densities. These latter represent the pseudomembranes formed in these bacterial infections.

pseudomembrane (Fig. 2) have been present in up to 25%. Involvement of the entire trachea and mainstem bronchi has been documented by bronchoscopy in at least three patients. 5 Half of the patients have had a concomitant pneumonia by chest radiograph. Croup and other acute infections of the trachea are discussed by Swischuk (p. 12). Scleroma. This is a chronic granulomatous disorder of the upper respiratory tract associated with the gram negative bacterium Klebsiella rhinoscleromatis. 63 While it is unusual in the United States, it is encountered with some frequency throughout Africa, Central and South America, Asia, and Central and Eastern Europe. The patients are usually poverty stricken young adults from rural areas. Widely known as rhinoscleroma, the disease affects primarily the nose, paranasal sinuses, and pharynx. The disorder is slowly progressive and may evolve over a period as long as 20 yr. Three recognizable stages merge imperceptibly. The catarrhal stage is characterized by watery nasal discharge and difficult nasal breathing. The nasal mucosa is hypertrophied with tiny bluishred nodules and covered with a yellowish crust. As the nodular stage develops, the coryza disappears and granulation tissue progressively infiltrates the nasal mucosa and paranasal regions resulting in a characteristic swelling and defor-

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CHOPLiN, WEHUNT, AND THEROS

Fig. 3. Scleroma. (A) Deformity of the nose (Hebra nose) resulting from infiltration by granulomatous tissue in the nasal cavity and paranasal sinuses. (B) Biopsy of nasal mucosa demonstrates granulomas composed of lymphocytes and plasma cells. The clear spaces are in large foamy histiocytes ( i i k u l i c z cells) that contain gram negative bacilli. (C) The trachea and main bronchi are diffusely narrowed as a result of t h e sclerotic end stage of the infection. (AFIP Neg. No. 82-12069-1,82-12068, and 79-3806-1 .)

mity of the nose (Hebra nose; Fig. 3A). Biopsy during the first two stages reveals granulomatous infiltration with lymphocytes, plasma cells, and a peculiar large foamy histiocyte (Mikulicz cell) that contains gram negative bacilli, K. rhinosclerornatis (Fig. 3B). During the "healing" or cicatricial stage, the granulation tissue is replaced by

the dense fibrous tissue that gives the disorder its name. The cicatrization results in stenosis of the nasal passages, sinus orifices, and nasopharynx and causes considerable morbidity. The 2% to 9% of patients who develop tracheal disease may exhibit diffuse symmetric narrowing or nodular masses. 8-1~While the larynx is always

DIFFUSE LESIONS

involved, the sinuses may be spared in these patients. The proximal trachea is most commonly affected, but the process may proceed distally to involve the entire trachea and mainstem bronchi (Fig. 3C). H Treatment with antibiotics has been successful in arresting the disease, but corrective surgery is often necessary to relieve obstruction if fibrosis is severe. Fungal disease. While the trachea is not a primary site for fungus infection, narrowing or masses have been described in isolated instances of candidiasis, coccidioidomycosis, histoplasmosis, mucormycosis, and rhinosporidiosis. ~2 16 There are no distinguishing radiographic features and diagnosis requires biopsy of the lesion.

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rarely into the distal trachea. ~8'~9 While most patients have well-established sarcoidosis elsewhere, occasionally the larynx may be the presenting site. In either case, biopsy is often necessary for confirmation and for exclusion of neoplasm or an infectious process. Relapsing polychondritis. This disorder has a characteristic clinical syndrome, consisting of recurrent episodes of inflammation of the pinna of the ear, and the nasal, laryngeal, and tracheal cartilages. 2~ These patients may also have a nonerosive polyarthritis, inflammation of ocular structures, vestibulocochlear dysfunction, and

Noninfectious Disorders Sarcoidosis. The larynx may be affected in 1%-3% of patients with sarcoidosis. ~7 The inflammatory process is usually supraglottic, but may extend into the subgiottic region (Fig. 4) or

Fig. 4. Sarcoidosis. There is diffuse thickening of the vocal cords, subglottic larynx, and tracheal walls. The intraluminal soft tissue mass was a polyp composed of sarcoid tissue. AP tomogram.

Fig. 5. Relapsing polychondritis. (A) The trachea is n a r r o w e d from the subglottic region to its bifurcation in this patient w i t h long-standing disease. (Continued on page 42.) (B) Specimen radiographs of the trachea of another patient w i t h relapsing polychondritis (right), and a normal trachea (left). Note the abnormal shape, w i t h loss of structural support and definition of t h e cartilaginous rings in t h e diseased trachea. (C) Low p o w e r photomicrograph of a section of tracheal wall from a third patient demonstrates marked destruction and fibrous replacement of t h e tracheal cartilage. (AFIP Nag. No. 80-11296-1 and 59-4360-1 .)

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CHOPLIN, WEHUNT, AND THEROS

Fig. 5.

arteritis of large to medium-sized vessels. The peak incidence of the disease is in the third and fourth decades but any age group may be affected. Sex incidence is equal and most patients have been Caucasian. Laryngeal and tracheal chondritis, which is present in 50% of the patients, may result in airway obstruction or recurrent pneumonia. In fact, respiratory tract involvement is the most common cause of death in relapsing polychondritis. 2~ The larynx and subglottic trachea are the initial sites of involvement in the early stages. 2~ Symmetric subglottic narrowing is the most frequent manifestation, although a patient with a large obstructing inflammatory mass has been reported. 22'23 The distal trachea and bronchi become~progressively involved, until virtually the entire airway is affected (Fig. 5A). Biopsy of the affected cartilage demonstrates loss of basophilic staining of the matrix, perichondral fibrocytic and capillary endothelial cell proliferation, and perivascular infiltration of lymphocytes and plasma cells. Eventually, fibrous tissue replaces the destroyed cartilage (Fig. 5B and C). 24 Autoantibodies to cartilage have been demonstrated in a high proportion of patients and this is thought to be pathogenetically important. 25 Because of the seriousness of laryngotracheal involvement, hoarseness, cough, dyspnea, choking sensation, laryngeal tenderness, or stridor

(Continued,)

should be sought. If any of these are present, airway films or tomograms of the trachea should be performed to search for areas of narrowing. Wegenergranulomatosis. This disorder is a granulomatous vasculitis chiefly involving the

Fig. 6. Tuberculosis. The trachea is narrowed distally with ulceration of the posterior wall.

DIFFUSE LESIONS

upper and lower respiratory tract and kidneys. 26'27The joints, skin, cardiovascular system, and central nervous system may be affected in a smaller percentage of patients. There are no specific diagnostic tests but the clinical syndrome is typical in over 90% of cases, so that a confident diagnosis may be made. While involvement of either the upper or lower respiratory tract is required for the diagnosis, the larynx and trachea have only rarely been involved. 28 30 The subglottic larynx and proximal trachea show smoothmargined narrowing of variable length which may be symmetric or asymmetric. In patients with a typical clinical presentation, biopsy of the laryngotracheal lesion is not necessary and a nasal or lung biopsy is preferred. 28 Distal Trachea

Infectious Disorders Tuberculosis. While the major airways were often affected by tuberculosis in the past, 3~ tra-

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cheal infection has decreased paralleling the overall decline in tuberculosis. In fact, no case of tracheal involvement was noted in a recent review of upper respiratory tract tuberculosis. 32 The most common manifestation is mucosal ulceration, usually on the distal posterior tracheal wall. 3j Occasionally extensive granulation tissue associated with these ulcerations results in narrowing of the lumen (Fig. 6). Fibrous tracheal stenosis is unusual if the patient responds to treatment, but permanent stricture is common at lobar or segmental bronchial orifices. Since these infections are more common in patients with extensive cavitary disease who expectorate a larger number of organisms, diagnosis is usually not difficult. Mediastinal granuloma and mediastinal fibrosis. These disorders are forms of chronic mediastinitis that may lead to narrowing of the trachea and bronchi. 33 35 In mediastinal granuloma, which is twice as common as mediastinal

Fig. 7. (A) Mediastinal Granuloma. This 4-mo-old child's main bronchi are narrowed from extrinsic compression by enlarged subcarinal lymph nodes secondary to histoplasmosis. Note the smooth inner margin of the bronchial walls (arrows), (B) Mediastinal fibrosis. Tomogram of the trachea and main bronchi shows a long relatively smooth-margined narrowing. Adjacent paratracheal and subcarinal lymph node enlargement is present. Lymph node calcification (arrowheads) suggests the correct diagnosis, histoplasmosis. (AFIP Neg. No. 67-5997-4.)

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fibrosis, the pathologic process is confined to lymph nodes that exhibit capsular fibrosis of variable thickness. In mediastinal fibrosis, on the other hand, the fibrotic reaction infiltrates the entire mediastinum with consequent compression of normal anatomic structures. Infection with histoplasmosis or tuberculosis is thought to be the most common cause of the granulomatous reaction, and mediastinal fibrosis is thought to

CHOPLIN, WEHUNT, AND THEROS

represent the late stages. Although the mechanism by which the florid sclerosis is produced is not well understood, an abnormal immunologic response to a persistent antigenic stimulus appears most likely. 33 The clinical presentation of patients with chronic mediastinitis is governed by the extent of functional impairment. Massive fibrosis may produce large radiographic masses without

Fig. 8. Amyloidosis. (A) Tomogram of the trachea shows irregularity and concentric narrowing at its midportion. (B) Photomicrograph of bronchial amyloid deposition from another patient shows submucosal thickening and narrowed bronchial lumen. (AFIP Neg. No. 71-6932-2 and 67-11656.)

DIFFUSE LESIONS

symptoms if vital structures are spared. However, small to moderate-sized but critically located areas of fibrosis may produce dramatic clinical symptoms related to superior vena caval obstruction, recurrent laryngeal nerve paralysis, pulmonary artery occlusion, or lobar collapse. Respiratory symptoms related to tracheal or

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bronchial compression develop in about 30%50% of patients with mediastinal granuloma or fibrosis. 33,36 Tracheal narrowing from enlarged granulomatous nodes may occur in children, presumably because of their less rigid tracheobronchial cartilages (Fig. 7A). When narrowing takes

Fig. 9. Tracheopathia Osteoplastica, (A) PA v i e w of the upper trachea of an asymptomatic 62-yr-old man, showing marked nodularity of the tracheal air column. (B) Tomogram of the upper trachea demonstrates calcification rimming t h e nodules studding the upper airway. (C) CT shows the classic location of a nodule along the inner anterolateral tracheal surface. The posterior tracheal wall is not involved. (D) L o w p o w e r photomicrograph of the tracheal wall of another patient demonstrates the osteocartilaginous nodules (arrowheads) in the submucosal tissue anterior to the tracheal cartilage and beneath an intact but metaplastic mucosal surface. Note also the metaplastic bone formation in the tracheal cartilage (arrows). (AFIP Neg. No. 81-2831 and 82-208.)

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place in the adult, it is usually a manifestation of fibrous infiltration into the tracheobronchial wall. 33 Both processes almost always involve the distal trachea and one or both main stem bronchi. The narrowing is of variable length and usually has a smooth margin. 34 Calcification within mediastinal lymph nodes (Fig. 7B) is a clue to the correct diagnosis. The associated mediastinal widening often suggests a neoplastic disorder and biopsy is usually necessary for clarification.

Noninfectious Disorders Mediastinal fibrosis. A previous infectious process cannot be demonstrated in many patients with mediastinal fibrosis and the etiology may remain unknown. However, fibrosis may be associated with other disorders and has been described in patients with retroperitoneal fibrosis, sclerosing cholangitis, Riedel thyroiditis, and

CHOPLIN, WEHUNT, AND THEROS

pseudotumor of the orbit, raising the possibility that these are all related in s o m e w a y . 37 While it has been stated that patients taking methylsergide for migraine may develop mediastinal as well as retroperitoneal fibrosis, we have been unable to find any well-documented cases. 38 Amyloidosis. Amyloid is a proteinaceous material that may be deposited in various organ systems and lead to their dysfunction. There is no totally satisfactory classification of the disorders associated with amyloid deposition because they overlap considerably in their clinical and pathologic manifestations. In addition, while the clinical syndromes may be similar, they may be associated with biochemically different amyloid proteins. 39 The lungs and tracheobronchial tree may be infiltrated by amyloid as part of the systemic disorder or may be the only organ involved. 4~ The most dramatic clinical and radiographic

Fig. 10. Saber sheath trachea. (A) Frontal view shows narrowing beginning at the level of the clavicles and extending to just above the bifurcation, The tracheal wall is slightly irregular, (B) The lateral v i e w shows a normal trachea. Coronal width is 12,5; sagittal width is 25.

DIFFUSE LESIONS

manifestations in regard to the trachea have been in patients with localized respiratory tract involvement. The average age is 53 and men are affected twice as frequently as women. The prognosis is not well defined although two-thirds of the deaths from amyloid disease are due to respiratory involvement. The radiographic findings in tracheal amyloidosis are determined by the pattern of gross involvement, the extent of tracheobronchial disease, and the presence of obstructive complications. The radiograph may demonstrate diffuse narrowing or show nodular protrusions into the tracheal lumen that may show calcification (Fig. 8A). Pathologically (Fig. 8B) submucosal amyloid deposition o c c u r s . 41-43 The overlying mucosa remains intact but may demonstrate squamous metaplasia. 44 Obstructive hyperinflation, atelectasis, and recurrent pneumonitis may develop as secondary manifestations of the tracheobronchial involvement. Tracheopathia osteoplastica. This unusual condition is characterized by multiple submucosal osteocartilaginous growths along the inner anterolateral surfaces of the trachea? 5'46Men are more frequently affected than women (3:1), and the average age is over 50 yr (range 1 1-78 yr). The etiology is unknown. Theories have been advanced linking this disorder to chronic inflammation, degenerative processes, amyloidosis, and frank neoplasia. 47-49 Typically the radiograph reveals multiple sessile nodular tumors, with or without calcification, extending over a long segment of the trachea (Fig. 9A). 47 Tomography is extremely helpful in detecting the nodules lining the inner trachea and the rimming calcification (Fig. 9B). Although calcification or ossification is almost invariably present histologically, it may be difficult to demonstrate radiologically. While the nodules in amyloidosis may be circumferential, those of tracheop~thia osteoplastica typically spare the posterior membranous wall (Fig. 9C). Pathologically there are submucosal islands of hyaline cartilage with areas of lamellar bone and occasionally marrow elements (Fig. 9D). The mucosal surface remains intact. The occasional demonstration of a connection of these nodules to perichondrium suggests that the native cartilage may play a role in the pathogenesis of tracheopathia osteoplastica.

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Saber sheath trachea. This tracheal deformity has been associated with chronic obstructive pulmonary disease and is found almost exclusively in men. 5~ The trachea is flattened from side to side so that the coronal diameter is equal to or less than two-thirds of the sagittal diameter when measured 1.0 cm above the top of the aortic arch (Fig. 10). Since the deformity affects only the intrathoracic trachea, there is abrupt widening above the thoracic inlet. The trachea usually has a smooth inner margin, but a nodular appearance has been described. 5~ Calcification of the tracheal cartilages is frequently present. Although over 95% of patients with this

Fig. 11. Idiopathic tracheobronchial narrowing. The trachea exhibits smooth narrowing in the subglottic and distal portions. The left main bronchus is also narrowed.

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CHOPLIN, WEHUNT, AND THEROS

Fig. 12. Tracheobronchomegaly. (A) Contrast examination of the trachea shows diffuse widening, with outpouchings of the tracheal wall between the cartilaginous rings. A bronchographic catheter is present. (B) Gross specimen from another patient. Marked dilatation of the trachea and proximal bronchiectasis are present, (AFIP Neg. No. 66-1066.)

deformity have clinical evidence of chronic obstructive pulmonary disease, only 55% have other radiographic evidence of it. The pathogenesis of the deformity is unknown. Histologic examination in one patient demonstrated diffuse ossification of the cartilaginous rings. Idiopathic narrowing. Fibrosis of uncertain cause, with multiple areas of narrowing in the subglottic region, distal trachea, and main stem bronchus may occur, as in the 39-yr-old black woman illustrated in Fig. 11. A tracheal biopsy showed squamous metaplasia with mild chronic inflammation and fibrosis. Despite extensive evaluation, no cause could be defined. While we have listed a number of disorders resulting in tracheal narrowing, it should be remembered that these are diseases intrinsic to the trachea in which there is fixed narrowing. Primary and secondary neoplasm, thyroid

enlargement, and vascular abnormalities may also result in stenosis. Finally, some patients may have tracheomalacia, in which the abnormal expiratory narrowing may only be detected by fluoroscopic or cineradiographic examination. 52 DISORDERS WITH TRACHEAL WIDENING

Diffuse tracheal widening is much less common than tracheal narrowing and has a more limited differential diagnosis. Since most of the patients have diffuse widening at presentation, there is no advantage to assessing proximal or distal involvement. Tracheobronchomegaly. The MounierKuhn syndrome, which accounts for almost all cases of tracheal widening, primarily affects men in the fourth and fifth decade, s3,s4 In this disorder, the cartilaginous rings dilate and the intercartilaginous portions of the tracheal wall bulge

DIFFUSE LESIONS

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outward, forming broad diverticula-like protrusions. The trachea is involved from the subglottic region to the carina (Fig. 12A). While a diameter greater than 3 cm is required for diagnosis, tracheal widths up to 5.5 cm have been recorded. 55 Bronchiectasis involving the first to fourth order branchings is present in many of the patients (Fig. 12B).54 The etiology and pathogenesis of the disorder is poorly understood. While many observers have considered it to have a congenital basis, others have considered it to be acquired. 53 There is a paucity of information about the histopathology,

but decreased amounts of elastic and connective tissue with thinning .of the muscularis mucosae and absence of the myenteric plexus have been described. Miscellaneous. The remaining cases of tracheal widening consist of isolated reports in patients with the Ehlers-Danlos syndrome and cutis laxa. 56'5vIt is uncertain whether this is part of these :syndromes or a coincidental association with Mounier-Kuhn syndrome. Finally, while narrowing is the usual end result in relapsing polychondritis, diffuse widening has :occasionally devdoped.52

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44. Spencer H: Pathology of the Lung, vol. 2 (ed 3). Oxford, Pergamon Press, 1977, pp 675-680 45. Lundgren R, Stjernberg NL: Tracheobronchopathia osteochondroplastica: A clinical bronchoscopic and spirometric study. Chest 1981 ; 80:706-709 46. Secrest PG, Kendig TA, Beland A J: Tracheobronchopathia osteochondroplastica. Am J Med 1964; 36:815-818 47. Young RH, Sandstrom RE, Mark G J: Tracheopathia osteoplastica: clinical, radiologic, and pathological correlations. J Thorac Cardiovasc Surg 1980; 79:537 541 48. Alroy GG, Lichtig C, Kaftori JK: Tracheobronchopathia osteoplastica: End stage of primary lung amyloidosis? Chest 1972; 61:465-468 49. Way SPB: Tracheopathia osteoplastica. J Clin Pathol 1967; 20:814-820 50. Greene R: "Saber-sheath" trachea: Relation to chronic obstructive pulmonary disease. A JR 1978; 130:441 445 51. Rubenstein J, Weisbrod G, Steinhardt MI: Atypical appearances of "saber-sheath" trachea. Radiology 1978; 127:41-42 52. Feist JH, Johnson TH, Wilson R J: Acquired tracheomalacia: etiology and differential diagnosis. Chest 1975; 68:34(~344 53. Bateson EM, Woo-Ming M: Tracheobronchomegaly. Clin Radiol 1973; 24:354-358 54. Johnston RF, Green RA: Tracheobronchiomegaly: report of five cases and demonstration of familial occurrence. Am Rev Respir Dis 1965; 91:35 50 55. Rouan M: Un cas de tracheom6galie. J Franc Med Chir Thorac 1959; 13:417 56. Cavanaugh M J, Cooper DM: Chronic pulmonary disease in a child with the Ehlers-Danlos syndrome. Acta Paediatr Scand 1976; 65:679 684 57. Wanderer AA, Ellis EF, Goltz RW, et al: Tracheobronchiomegaly and acquired cutis laxa in a child: Physiologic and immunologic studies. Pediatrics 1969; 44:709-715