Congenital stenosis involving a long segment of the trachea: Further experience in reconstructive surgery

Congenital Stenosis Involving a Long Segment of the Trachea: Further Experience in Reconstructive Surgery By Chikara Tsugawa, Ken Kimura, Toshihiro Muraji, Eiji Nishijima, Yoichi Matsumoto, and Hiroshi Murata Kobe, Japan and New Hyde Park, New York 9 Since w e introduced the surgical technique for the repair of congenital stenosis involving the entire trachea with a successfully treated patient in 1982, four more patients have undergone a tracheoplasty employing the same technique for congenital stenosis involving a long segment of trachea. This article describes the refinement of surgical technique, the management of patients during and after operation, and the long-term outcome. 9 1988 by Grune & Stratton, Inc.

INDEX WORDS: Congenital tracheal stenosis; tracheoplasty; costal cartilage graft.

O N G E N I T A L STENOSIS involving a long seg-

C ment of the trachea has been regarded as a fatal disease. Attempts of surgical repair had been totally unsuccessful 1'2 until we introduced a new reconstructive procedure using a costal cartilage graft? Since we reported the first patient who was successfully treated in 1982, 3 four more patients have undergone the same technique for congenital tracheal stenosis involving more than 50% of the entire length of trachea. In four of five patients, the endotracheal tube was removed and the patients have been free of respiratory problems. MATERIALS AND METHODS During the period from 1982 to 1987, five patients with congenital stenosis involving a long segment of the trachea have undergone surgical treatment at the Kobe Children's Hospital (Table 1). There were two males and three females. The age of patients ranged between 5 and 20 months at operation. The patients developed a variety of respiratory symptoms such as apneic spell, stridor, and/or pneumonia since early age. Four patients developed severe respiratory distress requiring mechanical ventilation. Three of them developed cerebral palsy due to hypoxemia prior to operation. Preoperative workup included bronchoscopy, CAT scan and xerography of trachea. Tracheography was mandatory to investigate the anatomic feature of the distal tracheobronchial tree. This was performed just before the operation because of the possible airway obstruction induced by instilled contrast materials, which may require an urgent thoracotomy. Bronchoscopy demonstrated a narrowed tracheal lumen of 2 to 3 mm, starting at the level of cricoid ring or the inlet of thorax. The tracheal cartilages in these patients usually formed a complete ring with an absence of membraneous portion. The tracheogram showed a variety in the extent of stenosis; entire length of the trachea in one patient and more than 50% of the entire length of the trachea in the other four patients (Fig 1). In two patients, mechanical ventilation did not alleviate expiratory and inspiratory obstruction, resulting in hypercarbia. High pressure manual ventilation was required with external compression of the chest wall to assist expiration, and emergency thoracotomy

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was carried out. In the other two patients, respiratory condition deteriorated after tracheography, requiring an emergency thoracotomy.

Operative Technique The trachea was reached through a median sternotomy in three patients. At median sternotomy, the head and neck were fully extended by placing a roll under the shoulders, and the neck and anterior chest were draped. A skin incision was extended from the suprasternal notch to the xiphoid process with adjunctive small transverse neck incision. Immediately after entering the thorax, the distal end of stenosis, which had been determined by tracheogram, was reached and the distal normal segment of trachea or bronchus was incised longitudinally and cannulated with a 3.0 or 3.5 Portex tube to secure ventilation. Thereafter, the strap muscles were retracted laterally and the lobes of thymus were separated to visualize the proximal trachea. Dissection was carried out along the anterior wall of the trachea extending from the cricoid ring down to the carina with an effort to minimize the dissection along the midline of the trachea. The innominate vein and the aortic arch were separately taped and retracted downward. The longitudinal tracheal incision was advanced upward to the proximal end of the stenosis. The narrowed stenotic segment was opened, which permitted an advance of a nasotracheal stent tube along the inner surface of the longitudinally divided trachea. At this point, ventilatory route was switched from the tube in the operative field to the nasotracheal stent tube. The right seventh costal cartilage was excised with the perichondrium intact. The intercostal and rectus abdominis muscles were freed from the cartilage by a fine needle cautery, avoiding any damage to the perichondrium. The cartilage was split into two pieces, and then each piece was shaped into an ellipse according to the size of the tracheal incision. The cartilages were used to fill the longitudinal defect of the trachea with the perichondrium facing toward the lumen, and were sutured to the cut edges of the trachea with interrupted 5-0 or 4-0 Vicryl sutures. A hemoc!ip was placed to mark the lower end of the suture line for radiological visualization. A retrosternal drain tube was placed and the chest was closed (Fig 2). A standard right thoracotomy was employed in two patients. At entering the mediastinum through the right thoracotomy, a longitudinal incision was made along the right lateral wall of the stenotic portion of the trachea. The technique employed for reconstruction of the trachea was essentially the same as the previously described in median sternotomy approach.

From the Departments of Surgery and Anesthesiology, Kobe Children's Hospital, Kobe, Japan and Schneider Children's Hospital, Long Island Jewish Medical Center, New Hyde Park, NY. Presented at the 20th Annual Meeting of the Pacific Association of Pediatric Surgeons, Seattle and Rosario, WA, April 26 to May 1, 1987. Address reprint requests to Chikara Tsugawa, MD, Kobe Children's Hospital, 1-1-I Takakuradai, Sumaku, Kobe 654, Japan. 9 1988 by Grune & Stratton, Inc. 0022-3468/88/2305-0020$03.00/0

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Table 1. Summary of Tracheopiasty Using Costal Cartilage Graft Case

Age

1

12 mo

2 3

5 mo 8 mo

4

1 yr

5

8 mo 3 mo

Associated Abnormalities

Complication

Imperforate anus,

Eventration of 1 diaphragm,

agenesis of the right lung

compression of the trachea by the aorta aortopexy

Pulmonary artery

Remaining stenosis aortopexy

sling

Biliary atresia

Necrosis of the graft, recur-

Results

Duration of Follow-up

Doing well

5 yr, 9 mo

Doing well Died 11 mo after

4 yr

operation Doing well

10 mo

Doing well

4 mo

rent stenosis

Postoperative Management The patients were managed in the ICU on the ventilator for 2 to 3 weeks. Continuous muscle relaxation was employed in all patients to immobilize the trachea to avoid tube trouble. A chest x-ray was frequently taken to assure the location of the distal end of the tube as well as for possible development of pulmonary complications such as pneumonia, atelectasis and/or other respiratory problems. Intravenous nutrition was employed through a central venous catheter during the period of muscle relaxation. At 4 to 5 weeks postoperatively, bronchoscopy was performed and the endotracheal stent tube was removed. One patient was complicated by left diaphragmatic eventration requiring a plication. Two patients required aortopexy for tracheomalacia.

trachea was incised longitudinally, and a piece of the costal cartilage was implanted. He was managed on the ventilator with muscle relaxant for 3 weeks. He was extubated on postoperative day 25 without any difficulty. The patient is free of respiratory symptoms at the age of 4 years. Case 3 An 8-month-old male was hospitalized with two episodes of respiratory distress with cardiac arrest.

CASE R E P O R T S

Case 1 A 1-year-old female was referred with congenital stenosis of the entire trachea associated with an agenesis of the right lung (Fig 3). She was successfully treated by tracheoplasty employing costal cartilage graft at 12 months of age. Bronchoscopies performed every year demonstrate a growth in tracheal lumen. She is 6 years 9 months old at present, and has been completely free of airway diseases. This patient is the first survivor, who has been previously reported. 3 Case 2 A 5-month-old male was transferred with a tracheal stenosis involving the mid portion of trachea. Through a right standard thoracotomy, the lateral wall of 2

l

4

3

5

0 mr

5 mm

6 mm

Fig 1.

Type of stenosis and age of patients.

Fig 2. The stenotic trachea is longitudinally incised and the tracheal defect is reconstructed with the costal cartilage. During the operation jet ventilation is employed through a fine catheter introduced into the nasotracheal stent tube for ventilation.

CONGENITAL LONG-SEGMENT TRACHEAL STENOSIS

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Bronchoscopy and contrast studies demonstrated the stenosis extending from the inlet of chest cavity to the carina. At operation, the left pulmonary artery was found to rise from the right artery, running around stenotic segment forming a pulmonary artery sling. The reconstruction of pulmonary artery sling and tracheoplasty were simultaneously carried out. Postoperatively, attempts at tracheal decannulation were unsuccessful. Tracheoscopy demonstrated a tracheal collapse above the carina. An external silastic stent wrapping the collapsed segment of trachea was implanted but failed to restore the tracheal lumen. A sternal aortopexy was performed with an improvement of tracheomalacia. The endotracheal tube was finally removed on day 144 after tracheoplasty. The patient suddenly developed severe respiratory distress at home 11 months after tracheoplasty and died. An autopsy was not performed. Case 4

A 1 year and 8 month old female had been hospitalized seven times for recurrent pneumonia prior to referral. Serious cerebral retardation was evident probably because of previous hypoxic episodes. Bronchoscopy and bronchography demonstrated tracheal stenosis beginning from 2 cm below the cricoid ring (40 mm in length). A tracheoplasty was performed through a median sternotomy. The endotracheal tube was removed 5 weeks after operation. She is 2 years and 6 months of age, 10 months after operation, and occasionally requires oxygen for persisting bronchopulmonary dysplasia. Case 5

A 3-month-old female was referred with biliary atresia. At proposed Kasai operation for biliary atresia, the endotracheal tube could not be advanced further than 1.5 cm below the cricoid ring. A bronchoscopy was carried out, which demonstrated tracheal stenosis. Emergency tracheoplasty was carried out through a median sternotomy. Kasai operation for biliary atresia was undertaken three days after this operation. On postoperative day 7, she developed massive air leakage through the chest tube. Emergency exploration of the mediastinum revealed necrosis of the grafted cartilage, and regrafting of the costal cartilage was performed employing the same procedure. Attempts at decannulation were unsuccessful over the following 8 months. Bronchoscopy and bronchography revealed recurrence of stenosis throughout the grafted lumen (Fig 4). Eight months after the first operation, she underwent the third operation. The cartilage graft, which was implanted at previous operations, seemed to have shrunk in width

Fig 3. Case 1. Trachaobronchogram before operation. The entire trachea is stanotic. The right bronchus is obstructed while the left bronchus is of relatively normal caliber.

and length. The longitudinal incision was made in the previous cartilage and was filled with new costal cartilage. At this procedure, the abdomen was opened to mobilize the omentum to bring up through the retrosternal space into the mediastinum. The grafted portion of the trachea was then wrapped by the omentum. The endotracheal tube was finally removed 53 days after the last operation. She has been free of respiratory symptoms and anicteric. DISCUSSION

Various surgical techniques have been attempted for the repair of congenital stenosis involving the entire or long segment of trachea employing dilation of stenosis using a bougie, an implantation of synthetic materials and autografts of pericardium, esophagus, or periosteum for surgical repair. 48 These attempts had been

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Fig 4. Case 5. Bronchogram before the third operation. The stenosis extends from just below the ericoid ring down to 2.0 cm above the carina.

unsuccessful until the first patient in this series was successfully treated employing costal cartilage graft. 3 Thereafter, the technique that we described has been successfully employed at several centers. 9'1~ Most of the patients have been successfully treated, but failure in treatment was seen in some patients complicated by anastomatic leakage, necrosis, or malatic change of the cartilage graft in the early postoperative period. Recurrent stenosis or formation of granulation tissue at the suture line were the problems in the late postoperative course. For the purpose of overcoming these problems, we have provided several technical refinements in this series of patients. Tracheal dissection should be limited in the narrow area along the line of tracheotomy for preservation of vascularities of the trachea. Since the trachea receives its blood supply from the arterial capillary network running along the lateral wall, H extensive dissection may produce local devascularization, compromising

the blood supply to the grafted cartilage, particularly in the patient with extensive stenosis of the trachea. The inadequate blood flow at the tracheotomy may invite infection of the graft and/or subsequent granulation formation. In one patient (patient 5) in whom two previous operations were unsuccessful due to graft necrosis, the revised costal cartilage was wrapped with omentum. The viability of implanted costal cartilage is preserved with oxygen supplied through the perichondrium by diffusion from the surrounding tissue. The use of the omentum attached to trachea was effective for prevention of ischemic change of the poorly perfused tracheal tissueJ 214 In the recent two patients, jet ventilation was employed during operation employing a fine catheter that was introduced into the distal airway through the nasotracheal tube placed proximally to the stenosis. The patient was adequately ventilated without disturbing the operative procedure. Since it is difficult for a surgeon to determine the extent of stenosis by observation of the outer surface of trachea, a mark using a 26 G needle placed at the upper margin of the stenosis in the operative field is helpful to determine the accurate point of the corner of stenotic segment employing a simultaneous bronchoscopy during the tracheoplasty procedure. As the commercially available endotracheal tube is not adequately long to be used as a stent for the reconstructed trachea, we designed the tubes with adequate length for this purpose. A radiopaque marking at the distal end of the grft by a hemoclip was helpful to determine the proper location of the endotracheal tube in postoperative respiratory care. The report of laryngotracheoplasty employing a similar procedure indicates that the inner surface of grafted costal cartilage is epithelialized in several weeks. In the long-term follow-up, the patients are relieved with gradual improvement from the respiratory symptoms.15 Our early two patients have been free of respiratory symptoms during 4 to 6 years follow-up. In these patients, repeat bronchoscopies demonstrated satisfactory tracheal enlargements. These results encourage us to continue this procedure as a life-saving and definitive reconstructive operation for the congenital stenosis involving the long segment of trachea.

REFERENCES

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12. Williams R, White H: The greater omentum: Its applicability to cancer surgery and cancer therapy, in Ravitch MM (ed): Current Problems in Surgery. Chicago, Year Book Medical, 1986 13. Nelson RJ, Goldberg L, White RA, et al: Neovascularity of a tracheal prosthesis/tissue complex. J Thorac Cardiovasc Surg 86:800-808, 1983 14. Morgan E, Lima O, Goldberg M, et al: Successful revascularization of totally ischemic bronchial autografts with omental pedicle flaps in dogs. J Thorac Cardiovasc Surg 84:204-210, 1982 15. Cotton RT: Pediatric laryngotracheal stenosis. J Pediatr Surg 19:699-704, 1984