C

figure 14-23 Bronchoscopic views of tracheopathia osteoplastica. A, Patient is described in Figure 14-22A, having characteristic overall deformation of the trachea, with multiple submucosal nodules of varied size. There was marked luminal narrowing. B, Trachea of the patient described in Figure 14-22B. Note the extreme compromise of the airway and the viscid secretions. C, Postoperative view of the patient viewed in Figure 14-23A, 9 years later, showing a completely adequate lumen. See also Figures 42 and 43 (Color Plate 16.)

of the tracheal and central bronchial walls are markedly elongated and deformed, particularly in the mid and lower trachea.67,68 Malacia is present, sometimes to an extreme degree. Dynamic change is observed, with collapse occurring at expiration. The trachea, right main bronchus, and left main bronchus greatly exceed normal maximum values for transverse diameters of 25, 23, and 20 mm, respectively (in men).69 Peripheral airways are of normal caliber. The membranous wall is markedly widened. Tracheal sacculation can occur between the dysplastic rings and in the membranous wall and is bronchoscopically and radio-logically visible. The curve of the cartilages becomes so distorted that there may be actual reversal of the anterior wall in some, so that it pushes in against the membranous wall. With expiration and with cough, the mucosa of the membranous wall approximates to a greater or lesser degree to the deformed anterior cartilaginous wall. High degrees of obstruction follow, particularly with respiratory or tussive effort.

In one patient, an attempt was made in several operations to reshape the anterolateral tracheal wall and the main bronchi, using polypropylene ring splints to support the cartilaginous wall, and

figure 14-24 Computed tomography scans showing tracheobronchomegaly in a 44-year-old male, with dyspnea for 10 years, much worse in the last year. There is massive dilation of the trachea (A) with elongated membranous wall and of main bronchi at the carina (B). More marked bronchiectatic changes were present at both lung bases.

posterior splints to narrow the widened membranous wall. When these procedures failed, the patient was successfully managed over the long term with a large diameter silicone tracheal T tube. A T tube was used as primary treatment in subsequent patients, with successful results. A tracheal Y stent provides symptomatic relief if the bronchi also require support.70 At the present time, placement of a silicone stent of large diameter appears to promise certain relief, and it is to be favored over extensive (and, therefore, more risky) surgical tracheobronchoplasty. The stent may have to be specifically made for the individual patient's tracheobronchial anatomy.

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