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views in expiration (left) and inspiration (right).

The patient was fully relieved of respiratory and phonatory complaints and later underwent a further cosmetic procedure. The fat removed from these patients does not seem to regrow. Mounier-Kuhn and Haguenauer treated a similar patient by laryngoscopic excision of a posterior commissure lipoma.31 On the basis of this very limited and scattered experience, the following approach seems prudent in case of upper airway obstruction in symmetric lipomatosis colli:

1) Careful assessment with CT, tomography, laryngoscopy, and bronchoscopy.

2) Cervical exploration for removal of lipomatous tissue.

3) Laryngofissure, if required, for excision of intralaryngeal submucosal lipoma causing obstruction at false cords or glottic structures.

4) Excision of mediastinal fat, if the trachea is compressed.

Intraoperative bronchoscopy should help to assess the extent of anatomic relief obtained at each step. Airway edema must be watched for postoperatively. The long-term prognosis should be satisfactory, given that lipomatous tissue does not seem to recur.

Vascular Compression

Note is made in Chapter 6, "Congenital and Acquired Tracheal Lesions in Children," of the variety of congenital vascular anomalies that may cause airway obstruction. These include aortic vascular rings (double aortic arch) with or without ligamentum arteriosum, pulmonary artery sling with or without congenital tracheal stenosis, and prominent innominate artery. An anomalous subclavian artery, which passes behind the esophagus and trachea, does not usually cause airway compression, except in conjunction with a right-sided and right descending aortic arch with Kommerell's diverticulum (See Chapter 32, "Surgery for Tracheomalacia, Tracheopathia Osteoplastica, Tracheal Compression, and Staged Recontruction of the Trachea.") Aneurysm of the thoracic aorta can obstruct the trachea and carina (Figure 15-13). A rare aneurysm of an anomalous sub-clavian artery, which was otherwise previously asymptomatic, also caused tracheal obstruction.

An enlarged right atrium has been reported to compress the airway, as has massive dilation of pulmonary artery due to congenital heart disease.

Postpneumonectomy Syndrome

In postpneumonectomy syndrome, a severely symptomatic airway compression is caused by extreme medi-astinal shift and rotation, most frequently after a right pneumonectomy in the presence of a normal aortic arch.32 Mirror image airway compression follows a left pneumonectomy in the presence of a right aortic arch. After a right pneumonectomy, the mediastinum moves to the right and posteriorly, as viewed on CT scans. Because of the attachments of the heart to the great vessels, the heart rotates counterclockwise with the great vessels, as viewed on CT scans (Figure 15-14). Herniation of the left lung and overdistension of the lung accompany this shift and rotation. In the most common situation, realignment of thoracic structures results in tracheal displacement to the right, and compression of the left main bronchus and sometimes the distal portion of the trachea, as the airway angles beneath the aorta and is flattened against the vertebral column or the descending aorta. The elongated residual pulmonary artery lies tightly against the anterior wall of the compressed bronchus (Figure 15-15). Such tracheobronchial compression has also been encountered in the analogous situation of displacement and rotation of the heart due to right lung agenesis.33

Similar anatomic distortion after a left pneumonectomy occurs in the presence of a right aortic arch.32 In this case, a clockwise rotation of the mediastinum is seen, with the trachea pulled to the left and the right main bronchus compressed, overlying the vertebral column or aorta (Figures 15-16A,B). Because the right main bronchus is so much shorter than the left, it is not uncommon to find that the right upper lobe bronchus and the bronchus intermedius are also compressed against the vertebral column. Patients have also been observed with this syndrome after a left pneumonectomy with a normal left-sided aortic arch (Figures 15-16C,D).34,35 A further variation was observed in a patient following a right pneumonectomy for infection in a congenitally nonfunctional lung. The left lower lobe bronchus alone was compressed over the aorta, perhaps due to limited left lung shift into a right hemithorax of chronically reduced volume (Figure 15-17).

Such an extreme shift was originally thought to occur principally or entirely in children. However, Grillo and colleagues presented 11 adults with severe symptoms, only 1 of whom had undergone pneumonectomy in childhood.32 Seven were under the age of 30 years.

Radiographic demonstration of the intrathoracic realignment of the lung and mediastinum is an integral part of evaluation of this syndrome. Conventional radiography shows marked lateral and posterior displacement, but CT of the chest makes the rotation clear and shows the relationship of the airway to the great vessels and the spine.36 Three-dimensional reconstruction adds further illumination. Multiplanar images obtained on magnetic resonance scanning may also be of help. With these adjuncts, angiography is no longer necessary for assessment. Bronchoscopy shows anterior-posterior or slightly oblique compression of the lower trachea and/or corresponding main bronchial origin (Figure 15-18). A small number of these patients also develop severe malacia of the cartilages of the compressed segment so that restoration of the mediastinal anatomy centrally fails to correct the airway obstruction. This is much more difficult to identify preoperatively, either by bronchoscopy or by radiography. Intraoperative bronchoscopy after initial surgical correction may reveal malacia.

figure 15-13 Thoracic aortic aneurysm obstructing the trachea and carina. A, Posteroanterior chest roentgenogram. B, Lateral chest roentgenogram. C, Computed tomography scan at the carinal level.

figure 15-13 Thoracic aortic aneurysm obstructing the trachea and carina. A, Posteroanterior chest roentgenogram. B, Lateral chest roentgenogram. C, Computed tomography scan at the carinal level.

Kaunitz and Fisher proposed managing postpneumonectomy patients with indefinitely continued pneumothorax.37 This seems impractical as a long-term solution for the problem. The available experience of attempts at definitive surgical correction of the syndrome formerly consisted mostly of case reports of

figure 15-14 After a right pneumonectomy with a left aortic arch, the heart and aortic arch are displaced to the right of the midline (dashed vertical line) and rotated (arrow). This results in displacement of the trachea and carina to the right and posteriorly. The left main bronchus is compressed between the left pulmonary artery and descending aorta or vertebral column. The mirror image is seen after a left pneumonectomy with a right aortic arch. Reproduced with permission from Grillo HC et al.32

figure 15-14 After a right pneumonectomy with a left aortic arch, the heart and aortic arch are displaced to the right of the midline (dashed vertical line) and rotated (arrow). This results in displacement of the trachea and carina to the right and posteriorly. The left main bronchus is compressed between the left pulmonary artery and descending aorta or vertebral column. The mirror image is seen after a left pneumonectomy with a right aortic arch. Reproduced with permission from Grillo HC et al.32

various techniques of treatment.38-40 Surgical correction is best accomplished by replacement of the mediastinum to normal anatomic relationships, to allow the compromised airway to return to its normal position and patency (Figure 15-19).32,39 Mediastinal repositioning relieves mechanical obstruction of the bronchial tree in those patients in whom malacia has not developed, and it also corrects overdistension of herniated and hyperexpanded lung. Our experience with later recurrences in two earlier patients indicated that simple replacement and suture fixation of the mediastinum alone is undependable, even though it may occasionally work in the long term. Therefore, the empty hemothorax must be filled to prevent recurrence of the disorder. We initially used silicone breast prostheses filled with silicone gel. However, after widespread concern about the possible untoward effects of leaking silicone arose, we used saline-filled prostheses instead.32 This has seemed preferable to ping-pong ball plombage, in light of the very late complications seen with the latter in the treatment of tuberculosis. Expandable prostheses have been used and recommended for children and adolescents.40 The use of breast prostheses has now been applied more generally, with success.41,42 Stenting has also been applied as primary treatment for the syndrome.43 Long-term results of stenting will be needed to clarify both its continuing efficacy and safety.

At corrective operation, the side of the original pneumonectomy is reentered and the scar and adhesions dissected sufficiently to permit repositioning of the heart and mediastinal structures to a normal central location. After scarification of the pericardium anteriorly and of the retrosternal fascia, the pericardium is fixed to the fascia behind the sternum with two rows of 0 Prolene sutures. Care is taken not to produce tamponade by reefing up too much pericardium. Careful cardiac monitoring is essential during this phase of the procedure (See Chapter 32, "Surgery for Tracheomalacia, Tracheopathia Osteoplastica, Tracheal Compression, and Staged Reconstruction of the Trachea").

figure 15-15 Postpneumonectomy syndrome in a 19-year-old woman, within 1 year after right pneumonectomy for bleeding from congenital cystic disease. A, Chest roentgenogram shows the heart and mediastinum displaced to the right with obliteration of right pleural space. The lung is hyperexpanded and herniated. B, Lateral roentgenogram demonstrates posterior displacement of mediastinal contents and anterior herniated lung. C, Computed tomography scan shows the left main bronchus compressed between the pulmonary artery and spine.

figure 15-16 (Continued) Postpneumonectomy syndrome in a 26-year-old woman, 14 years after a left pneumonectomy in the presence of a normal left-sided aorta. Short carinal resection had also been necessary. Dyspnea was of 3 years duration. C, Computed tomography (CT) scan shows the bronchus intermedius (arrow) compressed against the vertebra. A = ascending aorta; D = descending aorta; M = main pulmonary artery; R = right pulmonary artery. D, CT scan after corrective surgery. Lung volume is returned to normal and the main bronchus is on the right of the spine.

figure 15-16 Postpneumonectomy syndrome, following a left pneumonectomy with a right aortic arch, in a 29-year-old woman, 16 months after pneumonectomy for congenital hypoplasia with hemoptysis. She had dyspnea, cough, and increasing stridor. A, Chest roentgenogram is essentially a "mirror image" of Figure 15-15A. Lateral view corresponded. B, Computed tomography scan shows right upper and middle lobe bronchi squeezed between the pulmonary artery and aorta and spine.

figure 15-17 Postpneumonectomy syndrome after a right pneumonectomy with obstruction of the left lower lobe bronchus alone, in a 39-year-old woman who had a right pneumonectomy 1 year before because of abscess, pneumonia, and hemoptysis in a congenitally cystic lung with ectatic bronchi and vascular anomalies. Six months later, she recognized progressive dyspnea, which became incapacitating. A, Computed tomography scan shows near complete obstruction of the left lower lobe bronchus (arrow) between the pulmonary artery and aorta. The upper lobe bronchus was rotated but open. Note the reduced volume of the right hemithorax related to the relatively nonfunctional right lung, which had resided there. Repositioning and insertion of saline-filled prostheses produced complete relief. Pre- (B) and postoperative (C) bronchoscopies. The lower lobe bronchus is a mere slit preoperatively (arrows). Both primary divisions of the upper lobe are seen. Both lobar orifices are widely patent postoperatively.

Repositioning alone is insufficient, since the pericardium will eventually work its way free if the hemithorax is not filled. A prosthetic volume is selected which is sufficient to fill the space without compressing the heart and remaining lung, which have been returned to the heart's original position and to a more normal lung volume. Initially, I dropped four intercostal muscle bundles with their attached costal periosteum against the pericardium, leaving the muscles attached anteriorly and posteriorly in the manner of Kergin's modified thoracoplasty.44 Periosteum was left on the lateral surfaces of the ribs to maintain bony

figure 15-18 Typical bronchoscopic appearance of the junction of the trachea and left main bronchus in postpneumonectomy syndrome, 2 years following a right pneumonectomy for trauma in an 18-year-old male. Also, see Figure 40 (Color Plate 16).

integrity. The intent was to provide a firm partition, which would solidify as the periosteum calcified and so maintain the mediastinal repositioning, even if the prosthetic filler had to be removed later. This proved to be unnecessary, and we now simply fill the hemithorax with saline-containing prostheses of appropriate volume. The patency and stability of the airway is checked intraoperatively by flexible bronchoscopy after repositioning, and again after prosthetic placement. It is then rechecked when the patient is placed supine on the table after completion of the operation. The cardiopulmonary dynamics are also observed closely as the patient is placed on his/her back after the chest has been closed. Sometimes, it is found that as the thoracic incision is closed, the prosthetic volume proves to be too great and produces a tamponade effect. The partially closed incision is reopened and the volume adjusted.

In 10 patients who initially underwent mediastinal repositioning, including 2 who had recurrence prior to the use of prosthetics to maintain repositioning, 5 did well. One died a month later, presumably from pulmonary embolism. The seventh patient showed significant residual airway malacia, and the trachea was later reconstructed. Three other patients who had severe malacic obstruction of the airway after mediastinal repositioning underwent a variety of procedures, most of which involved aortic division to remove the compressive presence of the aorta, using bypass grafts, and in some, tracheobronchial reconstruction. Two patients died postoperatively, and 1 patient remains well many years later. It is clear that the problem of severe malacia in this syndrome has not yet been solved. Today, it is probably best to consider stenting the airway if such a case appears. Mediastinal repositioning generally seems prudent in order not to force a foreign body, such as a stent with semirigid components, into a bronchus that is pinched between the pulmonary artery and sometimes the aorta, where erosion would be disastrous. However, one frail, elderly patient, who had severe obstruction after a right pneumonectomy many years earlier for tuberculosis, was successfully palliated for some years by insertion of a specially constructed, extra-long silicone T tube. As noted, others have reported short-term success, at least, with stents.43 We have not encountered severely malacic airways in 11 additional patients operated upon since the original report.

Pulmonary function studies demonstrated improvement in flow rates and a decrease in hyperinflation of the lung (Tables 15-2, 15-3; Figure 15-20).32 Improved flow is reflected by an increase in the peak

figure 15-19 Postoperative images after mediastinal repositioning with implantation of saline-filled breast prostheses. A, Chest x-ray film of the patient seen in Figure 15-15A. The trachea and mediastinum are returned to the midline and the lung to normal volume. B, Computed tomography scan of this patient shows the outline of the prostheses, and return of the left main and upper lobe bronchi to normal location and patency. C, Chest x-ray film of the patient from Figure 15-16A. D, Computed tomography scan shows right main and upper lobe bronchi widely open and to the right of the spine.

figure 15-19 Postoperative images after mediastinal repositioning with implantation of saline-filled breast prostheses. A, Chest x-ray film of the patient seen in Figure 15-15A. The trachea and mediastinum are returned to the midline and the lung to normal volume. B, Computed tomography scan of this patient shows the outline of the prostheses, and return of the left main and upper lobe bronchi to normal location and patency. C, Chest x-ray film of the patient from Figure 15-16A. D, Computed tomography scan shows right main and upper lobe bronchi widely open and to the right of the spine.

expiratory flow rate and in the ratio of the forced expiratory volume in 1 second to the forced vital capacity (FEVi/FVC). The increase in peak flow was associated with loss of an upper airway obstruction plateau and was primarily due to relief of tracheal compression. The improvement in the FEV1/FVC ratio from a moderately obstructed value of 0.52 to a value in the normal range of 0.75 was due to several factors. First, the decrease in FVC due to relief of hyperinflation was always greater in absolute amount than the decrease in FEV1. Second, upper airway obstruction was so severe in 3 patients that relief of tracheal compression

Table 15-2 Mediastinal Repositioning for Postpneumonectomy Syndrome: Preoperative and Postoperative Spirometry

Preoperative Testing (% Predicted)

Postoperative Testing (% Predicted)

Table 15-2 Mediastinal Repositioning for Postpneumonectomy Syndrome: Preoperative and Postoperative Spirometry

Preoperative Testing (% Predicted)

Postoperative Testing (% Predicted)

FEV1 (L)

FVC (L)

FEV1/FVC

PEFR (L/sec)

FEV1 (L)

FVC (L)

FEV1/FVC

PEFR (L/sec)

1.48 (60%)

2.54 (77%)

0.58 (70%)

2.21 (37%)

1.31 (53%)

1.68 (53%)

0.78 (101%)

3.1 (52%)

1.07 (29%)

1.66 (39%)

0.64 (73%)

1.97 (29%)

1.17 (35%)

1.52 (39%)

0.77 (80%)

2.5 (36%)

0.35 (9%)

1.78 (40%)

0.19 (22%)

0.48 (7%)

1.85 (47%)

2.17 (47%)

0.85 (101%)

3.37 (46%)

1.72 (61%)

2.44 (72%)

0.71 (87%)

2.31 (40%)

1.35 (52%)

1.78 (56%)

0.76 (93%)

2.63 (51%)

0.81 (20%)

1.57 (31%)

0.52 (67%)

1.89 (20%)

1.15 (28%)

1.77 (34%)

0.65 (83%)

3.67 (39%)

1.23 (26%)

3.08 (54%)

0.4 (48%)

2.88 (29%)

1.66 (35%)

2.66 (47%)

0.63 (76%)

4.5 (45%)

1.67 (52%)

2.83 (75%)

0.59 (69%)

3.1 (48%)

1.41 (43%)

1.73 (46%)

0.81 (94%)

3.91 (61%)

Mean ± SE 1.19±0.19

2.27±0.23

0.52±0.07

2.12±0.32

1.41±0.10*

1.90±0.15*

0.75±0.03f

3.38±0.27f

Reproduced with permission from Grillo HC et al.32

FEVi = forced expiratory volume in 1 sec; FVC = forced vital capacity; PEFR = peak expiratory flow rate; SE = standard error. *Not significantly different from the preoperative value. fp < .05 preoperative value.

Reproduced with permission from Grillo HC et al.32

FEVi = forced expiratory volume in 1 sec; FVC = forced vital capacity; PEFR = peak expiratory flow rate; SE = standard error. *Not significantly different from the preoperative value. fp < .05 preoperative value.

Table 15-3 Preoperative and Postoperative Plethysmography

Preoperative Testing (% Predicted)

Postoperative Testing (% Predicted)

Table 15-3 Preoperative and Postoperative Plethysmography

Preoperative Testing (% Predicted)

Postoperative Testing (% Predicted)

TLC (L)

RV (L)

RV/TLC

TLC (L)

RV (L)

RV/TLC

3.5 (72%)

1.06 (64%)

0.3 (90%)

2.43 (51%)

0.63 (40%)

0.26 (76%)

7.55 (105%)

5.98 (241%)

0.79 (229%)

3.62 (49%)

1.85 (73%)

0.51 (148%)

5.69 (76%)

2.61 (141%)

0.46 (186%)

4.2 (56%)

1.54 (83%)

0.37 (150%)

7.36 (144%)

4.53 (290%)

0.62 (203%)

3.36 (66%)

1.63 (104%)

0.49 (161%)

Mean ± SE 6.03±0.94

3.55±1.08

0.54±0.11

3.40±0.37*

1.41±0.27f

0.41±0.06f

Reproduced with permission from Grillo HC et al.32

RV = residual volume; SE = standard error; TLC = total lung capacity.

*p < .05 versus preoperative value.

fNot significantly different from preoperative value.

Reproduced with permission from Grillo HC et al.32

RV = residual volume; SE = standard error; TLC = total lung capacity.

*p < .05 versus preoperative value.

fNot significantly different from preoperative value.

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