Tracheal Pulmonary Agenesis

Stenosis with Unilateral


Congenital tracheal stenosis with unilateral pulmonary agenesis is a rare and frequent fatal combination. In an 8-year period, 5 infants (ages 2 to 6 months) with these anomalies were treated. The presenting signs and symptoms consisted of wheezing, stridor, and tachypnea and included frank respiratory failure requiring emergency therapy in several patients. The operative repair consisted of segmental resection and anastomosis in one patient, and rib-cartilage tracheoplasty in the other four. Two infants died, one of cerebral hypoxia, and the other of aortotracheal fistula. Long-term follow-up in the three survivors is satisfactory.

From the Division of Pediatric Surgery, Department of Surgery, St. Louis University School of Medicine and Cardinal Glennon Children's Hospital, St. Louis, Missouri

techniques. Within this group, 20 had normal bilateral lung development, while the remaining five infants had the combination of tracheal stenosis and unilateral agenesis. The latter five patients form the basis for this report. The patients were 2 to 6 months old when the tracheal stenosis was first diagnosed. There were three boys and two girls. In three infants the unilateral pulmonary agenesis was found at the time of the discovery of tracheal stenosis, while two others had known pulmonary aplasia at birth and were being followed closely for the development of respiratory difficulties. In neither of these latter cases was a work-up performed at birth for tracheal stenosis. The characteristics, signs, symptoms, and pulmonary and tracheal anomalies for each patient are shown in Table 1 and Figure 1. Generally the children with shorter stenoses (patients 1 and 2) presented with milder symptoms, although sufficiently significant to warrant work-up. It is interesting to note that these two patients were being treated for 'asthma' but had become refractory to bronchodilation therapy, which prompted further work-up. In contrast the three infants (patients 3, 4, and 5) with extensive tracheal stenosis involving long segments of the trachea presented in severe respiratory failure, with severe symptoms and markedly abnormal blood gases. The work-up in each case consisted of a plain chest xray (Fig. 2) followed by computed tomography (CT) scan in three of the five cases (patients 1, 2, and 5). In patients 1 and 2, the CT scan was obtained before endotracheal intubation, while patient 5 was intubated for respiratory failure and deterioration of blood gases. Although helpful in aiding in the diagnosis of tracheal stenosis, the extent of the stenosis could not be assessed accurately with this


ONGENITAL TRACHEAL STENOSIS with unilateral pulmonary agenesis is a rare but well-known combination of anomalies. This combination generally is fatal, with only a few survivors of attempted repair having been reported. Because tracheal stenosis associated with solitary lung frequently involves a long tracheal segment, or even the entire trachea, simple excision and anastomosis have rarely been possible. In addition the rarity of the combination of malformations has limited the experience that any one center has accumulated. In 1982 Kimura' described a new technique for the repair of congenital tracheal stenosis that involved the use of rib cartilage graft. Since then several successful cases have been reported using this technique. However most of these cases did not involve reconstruction of the trachea to a solitary lung. The present series details the use of rib

cartilage graft tracheoplasty and other techniques in five infants with critical congenital tracheal stenosis and unilateral pulmonary agenesis. Materials and Methods Patients In an 8-year period, 25 infants and children with congenital tracheal stenosis were managed with a variety of Address reprint requests to Thomas R. Weber, M.D., Cardinal Glennon Children's Hospital, 1465 S. Grand Blvd., St. Louis, MO 63104. Accepted for publication March 5, 1990.



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TABLE 1. Patient Characteristics

Initial Blood Gases Patient Number

Age (mos)/ Sex

Symptoms and Signs


Pulmonary Anomaly

Location of Tracheal Anomaly

I 2 3 4 5

6/F 3/F 2/M 2/M 3/M

Wheezing, tachypnea Stridor, tachypnea Respiratory failure Respiratory failure Severe wheezing, stridor, respiratory failure in 6 hours

65/70/7.25 88/50/7.30 55/120/6.95 60/145/6.80 44/90/7.11

Right agenesis Left agenesis Right agenesis Left agenesis Right agenesis

Cervical Cervical to lower trachea Cervical to lung hilum Vocal cords to lung hilum Cervical to lung hilum


technique, thus limiting its usefulness. Tracheograms with dilute barium were performed in two cases, both at the time of bronchoscopy (Fig. 3). Bronchoscopy was performed in each case, which resulted in acute deterioration in every child. The deterioration was manageable in cases 1 and 2 by the placement of a small (2.5 mm) endotracheal tube through the stenosis. This allowed short-term adequate ventilation in these two patients, and each underwent operative repair within 12 hours. The three infants with extensive tracheal stenosis were more difficult to manage before repair. Patient 3 deteriorated rapidly after bronchoscopy and could not be ventilated. He underwent emergency thoracotomy for tracheal repair. Patient 5 was ventilated with a high pressure, high-rate respirator, which gave adequate blood gases for 6 hours, at which time he deteriorated and underwent urgent thoracotomy and tracheal repair. Patient 4 had rapid deterioration after bronchoscopy at another hospital and was transferred in moribund condition. Because it was thought that he would not survive attempted repair, we elected to place him on extracor-

Patient 4

FIG. 1. Extent of stenosis in five infants with unilateral pulmonary agenesis and tracheal stenosis.

poreal membrane oxygenation (ECMO), and repaired his tracheal stenosis 48 hours later. Extracorporeal membrane oxygenation has been used extensively for newborn respiratory failure from other causes,2 but its use in this setting has not, to our knowledge, been reported previously. Briefly, the technique of ECMO involved cannulation of the right jugular vein and carotid artery, after systemic heparinization. This allowed connection to an extracorporeal circulatory apparatus, consisting of a reservoir, roller pump, membrane oxygenator, and heat exchanger.2 With the onset of ECMO, the patient stabilized and the blood gases rapidly returned to normal. The patient then underwent tracheal reconstruction while connected to the extracorporeal circuit and was later weaned from ECMO. Operative Technique The operative repair of patient 1 consisted of segmental resection with end-to-end anastomosis. This was performed without cardiopulmonary bypass, by intubating the distal trachea after transection was completed

(Fig. 4). Patient 2

Patient 1

Patient 3 & 5



Ann. Surg. * January 1991

FIG. 4. Operative technique for patient 1. After resection of the narrow tracheal segment, the distal trachea was intubated while the anastomosis was performed. Ventilation was then resumed via an oral endotracheal tube.

FIG. 2. Chest x-ray of an infant with tracheal stenosis and right pulmonary agenesis. Note hyperinflation of the left lung.

Ventilation of the lung was accomplished through this tube until the sutures (4-0 polydiaxanone) were placed but not tied. At that point the distal endotracheal tube was removed and a standard endotracheal tube was placed

FIG. 3. Tracheogram in an infant with agenesis of the left lung and severe, diffuse tracheal stenosis to the hilum. Reconstruction with rib cartilage graft was successful.

through the vocal cords and advanced through the area of anastomosis, and ventilation was resumed through the oral endotracheal tube. The sutures were then tied. The remaining patients, having more extensive lesions, required more complicated procedures. Rib cartilage tracheoplasty, using the technique of Kimura,l was performed without cardiopulmonary bypass in patients 2, 3 and 5, and with ECMO in patient 4. Briefly, the surgical procedure consists of exposure of the stenotic trachea through a transverse thoracic incision that transects the sternum at the third interspace. The great vessels are mobilized and retracted. The position of the heart in unilateral pulmonary agenesis is variable, but has not presented particular difficulties in tracheal exposure. The trachea itself may not appear extremely abnormal externally. The trachea is gently palpated to ascertain the location of the distal end of the endotracheal tube, which has been positioned just above the most proximal area of narrowing. A rigid or small flexible bronchoscope can be used to aid in positioning the endotracheal tube. The anterior tracheal wall is opened longitudinally, beginning at the endotracheal tube and continuing distally until normal tracheal lumen is encountered. The endotracheal tube is advanced by the anesthesiologist as the trachea is opened. Frequently at this point the ventilation of the patient improves markedly, and the blood gases rapidly become normal (Fig. 5). With the endotracheal tube in place within the lumen, a graft of rib cartilage, harvested from the anterior chest wall, is cut coronally and fashioned to fit into the tracheal defect (Fig. 6), where it is sutured in place with interrupted 4-0 polydiaxanone or 4-0 prolene (Fig.

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the size, length, and configuration of the endotracheal tube. The patients are eventually discharged with this custom-made long tracheostomy tube in place. Periodic reassessment of the graft by outpatient bronchoscopy is performed. When sufficient healing has occurred, as judged by the appearance, lumen size, and rigidity of the trachea and graft, a shorter tracheostomy tube of equivalent diameter is inserted and removed later when appropriate.

FIG. 5. Operative technique for rib cartilage graft tracheoplasty. After opening the narrow trachea anteriorly, the endotracheal tube is advanced and a graft of rib cartilage, prepared as in Figure 6, is sutured with interrupted monofilament sutures.

5). The perichondrium is excised with the cartilage, and the graft is placed with the perichondrium toward the lumen. In addition longitudinal, partial-thickness cuts are made in the nonperichondrium side of the cartilage to allow it to conform to a more natural tracheal shape (Fig. 6). The tube is left in place and converted to a tracheostomy tube in 7 to 10 days. Care is taken to keep the suture material in an extraluminal position because troublesome granulomas can form on suture material within the tracheal lumen. Before closure soft tissue (thymus) is interposed between the graft and any great vessels that it contacts. Postoperative care consists of intubation, either by a nasotracheal tube or tracheostomy, and ventilator support, the latter of which is weaned over several days. On the seventh to tenth postoperative day, the child is returned to the operating room where bronchoscopy is performed using a rigid, ventilating bronchoscope (Stortz, Culver City, CA). In patient 1, in whom resection and end-toend anastomosis were performed, the suture line was found to be well healed, without evidence of stenosis or infection, and thus the endotracheal tube was successfully removed at that time. In each case in which rib cartilage graft was used, the graft was seen to collapse into the airway lumen. Thus stenting of the graft was accomplished, first with an endotracheal tube-tracheostomy tube device that allowed precise placement of the tube through a tracheostomy stoma and through the area of the graft. When, after several days, it is determined that adequate ventilation can be achieved with the tube, a tracheostomy tube (Shiley Corp., Irvine, CA) is manufactured to conform to

Result All five of the patients in this series survived the operative procedure. Three of five patients are long-term survivors, having been discharged from the hospital, and the tracheostomy tube, if used, eventually removed. Patients 4 and 5 died before they could be discharged. Patient 4 died 5 months after extensive cartilage graft reconstruction and had been weaned from the ventilator. Viral pneumonia developed in this patient, with subsequent copious tracheal secretions. These secretions completely occluded his airway on several occasions, resulting in severe hypoxic episodes. Hypoxic encephalopathy and a seizure disorder developed, rapidly progressing to coma. He died 10 days later of encephalopathy. At autopsy his cartilage graft was found to be well healed, with adequate lumen size, but he had evidence of severe bronchopneumonia. Patient 5 died 8 weeks after cartilage graft reconstruction. Refractory Pseudomonas tracheitis developed and significant tracheostomy hemorrhage occurred in this child after a tracheostomy tube change, which stopped spontaneously. Tracheoaortic fistula was suspected, but before further work-up could be accomplished, another hemorrhage intervened that could not be controlled by tamponade. The patient underwent emergency thoracotomy and was placed on extracorporeal circulation but the aortotracheal fistula could not be repaired and the patient died. Autopsy showed that the infected graft had eroded into the aorta.


FIG. 6. Technique for preparing rib cartilage graft. The cartilage is harvested with the perichondrium intact, then is cut as shown. Longitudinal partial-thickness slits allow the graft to assume a dome shape, with perichondrium to the inside of the trachea.



The other three patients are well 3 to 6 years after operation. All are without tracheostomy tubes and have shown normal tracheal growth at bronchoscopy.

Discussion The survival of patients with congenital tracheal stenosis has improved in recent years, due to improvements in anesthesia techniques, operative management, and intensive care. However the outlook of the child with critical tracheal/bronchial stenosis associated with unilateral pulmonary aplasia, a known association, has continued to be extremely poor. Most of the reported patients have undergone desperate attempts at tracheal reconstruction, using a variety of techniques, with few successes reported.3-5 However the present report documents that survival can be achieved in these infants with tracheal anomalies that previously were thought to be lethal. The important points in the management of these infants seem to be individualized therapy, based on as thorough a workup as can be safely performed before operation, careful cooperation between surgeon and anesthesiologist, and flexibility both during and after operation. Most of these repairs can be performed without cardiopulmonary bypass by simply having the anesthesiologist advance the endotracheal tube past the point of obstruction, if possible, after resection or opening the trachea anteriorly. This allows adequate ventilation while the repair is completed. An occasional patient, such as patient 4 in this series, will have generalized narrowing into the hilum. In these instances, the immediate use of extracorporeal membrane oxygenation, followed several days later by work-up and repair of the tracheal stenosis, allows at least an attempt at repair that would not have been possible without extracorporeal circulation. Although the child eventually died of neurologic complication, his graft healed well and he had no tracheal narrowing at the time of his death. In patients with short-segment stenosis, the ideal therapy is excision of the trachea, with end-to-end anasto-

Ann. Surg January 1991 -

mosis. This was performed successfully in one of our patients (patient 1), and was used by Harrison et al.4 Infants with more extensive tracheal narrowing require complex reconstruction. Successful use of rib cartilage grafts reported by Kimura' prompted us to use this technique in four of our five patients, with excellent healing in three patients. Multiple bronchoscopies at postoperative intervals in surviving patients, and autopsy in patient 3 have shown excellent healing and growth of these cartilage grafts. This confirms Kimura's observation.' The long-term follow-up of the three surviving infants has shown normal respiratory function and, equally importantly, normal neurologic outcome and development. This latter observation is particularly important because of the multiple episodes of hypoxia, hypercarbia, and acidosis these infants suffered before and during repair. This re-emphasizes the capacity that infants have for recovery from critical illness in the newborn period and demonstrates that prolonged hospitalization, with multiple clinical 'set-backs,' can still result in an excellent outcome. Nearly successful result with extracorporeal membrane oxygenation used before attempted repair of extensive tracheal stenosis warrants further investigation into the role of this new invasive life-support technique in the management of these critically ill infants. References 1. Kimura K, Mukohara N, Tsugawa C, et al. Tracheoplasty for congenital stenosis of the entire trachea. J Pediatr Surg 1982; 17:

869-871. 2. Weber TR, Pennington DG, Connors R, et al. Extracorporeal membrane oxygenation for newborn respiratory failure. Ann Thorac Surg 1986; 42:529-535. 3. Nelson CS, McMillan KR, Bharucha PK. Tracheal stenosis, pulmonary agenesis, and patent ductus arteriosus. Thorax 1967; 22: 7-12. 4. Harrison MR, Heldt GP, Brasch RC, et al. Resection ofdistal tracheal stenosis in a baby with agenesis of the lung. J Pediatr Surg 1980; 15:938-943. 5. Lobe TE, Hayden CK, Nicolas D, Richardson CJ. Successful management of congenital tracheal stenosis in infancy. J. Pediatr Surg 1987; 22:1137-1142.

Congenital tracheal stenosis with unilateral pulmonary agenesis.

Cogenital tracheal stenosis with unilateral pulmonary agenesis is a rate and frequent fetal combination. In an 8-year period, 5 infants (ages 2 to 6 m...
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