Esophageal
Tracheoplasty
for Congenital
Tracheal
Stenosis
By Shingi Sasaki, Fuji0 Hara, Toshinobu Ohwa, Takeshi Eguchi, and Akira Masaoka Nagoya, Japan l Two infants with congenital long tracheal stenosis underwent operation by means of an esophageal tracheoplasty. The first patient had previously undergone tracheal reconstruction using the pericardium. Although ventilation improved somewhat following this procedure, the pericardial patch suddenly ruptured 12 days after the operation, requiring an immediate esophageal tracheoplasty. The esophageal portion of the reconstructed trachea epithelialized 1 month later, with the lumen maintaining its proper size. However, the patient died 3 months after the second tracheoplasty. The cause of death was thought to be due to complications arising from prolonged high-pressure use of mechanical ventilation. He had been on a respirator for 6 months before the first tracheoplasty. The second patient has been doing well with no recurring respiratory problems for 25 months now. Her reconstructed trachea has adapted with her growth. This technique should be considered along with other forms of treatment for tracheal reconstruction because it is relatively simple and pliable. Copyright o 7992 by W. B. Saunders Company
INDEX plasty.
WORDS:
Tracheal
stenosis,
congenital;
tracheo-
C tion
ONGENITAL tracheal stenosis, whereby a porof the membrane is absent (complete cartilage rings), is a rare and lethal anomaly. Management of this severe condition remains controversial, especially when the entire or nearly entire trachea is involved (generalized type).’ Various surgical techniques using autologous tissues to repair the trachea have been reported. 2-6Kimura et al3 reported using costal cartilage as a graft in tracheopIasty in 1982, and this technique is now widely used. However, with such a method, the isolated cartilage sometimes develops into necrosis, leading to restenosis of the tracheal lumen.7,8 We chose the esophagus as the grafting source for tracheal reconstruction in two infants with congenital long tracheal stenosis. Because the esophagus is an autologous tissue that can be used without requiring isolation, this technique is considered simpler and less risky than other methods. Herein, we present the operative technique, rigidity, and potential for growth of the constructed trachea. MATERIALS AND METHODS Case Reports Case 1. This case was reported previously in Japanese.9 A boy was born after a normal pregnancy and remained well for the first month of life except for a tendency of turning the face to the right. Stridor and cyanosis developed shortly thereafter. At a peripheral hospital, he initially received therapy for bronchitis, but intubation and management with a ventilator were soon required Journal of Pediafric Surgery, Vol27, No 5 (May). 1992: pp 645-649
as his respiratory distress rapidly worsened. A bronchogram taken 2 weeks after artificial ventilation was begun showed tracheal stenosis extending from the second thoracic vertebra to the carina (Fig 1). With these conditions, he was transferred to this hospital. Bronchofiberscopy showed remarkable narrowing of the tracheal lumen just below the top of the endotracheal tube (Fig 2A). A fiberscope (2.7 mm in diameter) could not be inserted into more distal trachea because of acute stenosis in the region. A right posterolateral thoracotomy was performed after several failed attempts to intubate the stenotic portion with a small Portex tube (inner diameter of 2.0 to 2.5 mm). During the operation, we found a pulmonary artery sling (PA sling) arising from the right pulmonary artery and passing between the trachea and esophagus to the left lung. A pulmonary arteriopelry was performed whereby the PA sling was pulled toward the right posterior region by means of a Goretex sheet that was sutured to the pleural wall. This served to alleviate extrinsic compression of the right main bronchus and/or lower end of the trachea. Although his respiratory condition improved slightly immediately following this procedure, it was not sufficient enough to warrant a reduction in the high pressure required to control ventilation. Therefore, a total tracheoplasty of the stenotic segment was planned, but his parents refused such treatment. He continued to require assistance with high-pressure mechanical ventilation. For the next 5 months, he was also given muscle relaxants and morphine, during which time he sometimes suffered from severe difficulties in ventilation, as well as cyanosis, hypotension, and bradycardia. When he was 7 months old, his parents agreed to a second surgery in an attempt to alleviate his symptoms. We performed a tracheoplasty with a pericardial patch. The trachea was reached through the right posterolateral thoracotomy and was incised posteriorly. The tracheal wall defect was sutured with the pericardial patch which had been resected earlier. Ventilation during the operation was maintained by the same method as was used for the esophageal tracheoplasty (see details under Operative Technique below). The arteriopexy was removed because no compression of the lower trachea or right main bronchus by the PA sling was evident. His respiratory condition improved somewhat immediately following the tracheoplasty, although it was necessary to continue monitoring under highpressure ventilation in order to maintain adequate respiration. On the 12th postoperative day, high-pressure ventilation had to be increased as his respiratory condition deteriorated even further, and, as a consequence, the pericardial patch ruptured. A second tracheoplasty was immediately performed using the esophagus according to Ein’s technique4 without extracorporeal circulation. A bronchoscopy taken 1.5 months later showed the lumen of the trachea had become adequately dilated with evidence of some granulation. The grafted esophageal wall also appeared to have epithelialized completely, resembling that of normal trachea (Fig
From the Second Department of Surgery, Nagoya City University Medical School, Nagoya, Japan. Date accepted: April 16, 1991. Address reprint requests to Shingi Sasaki, MD, The Second Department of Surgery, Nagoya City UniversityMedical School, Kawasumi-I, M&ho-cho, M&ho-ku, Nagoya 467, Japan. Copyright o I992 by WB. Saunders Company 0022-3468/92/2705-0028$03.00f0
645
SASAKI ET AL
646
now 2 years 9 months old, and she has experienced no recurring respiratory difficulties except for slight stridor at times.
Operative Technique The trachea and esophagus were reached through right posterolateral thoracotomy. After entering the thorax, the azygosvein was ligated and dissected. The pleura of the mediastinal side was then opened. The trachea and esophagus were mobilized and taped separately. The posterior wall of the distal end of the stenotic region, immediately above the carina, was incised longitudinally and intubated into the left main bronchus with a 3.0-mm or 3.5-mm Portex tube to secure ventilation. However, in case 1 adequate ventilation could not be achieved using only a Portex tube with PO2 at 67.0 mm Hg and PC01 at 71.6 mm Hg. Therefore, a smaller tube (8F) connected to a separate anesthetic system was inserted into
Fig 1. Preoperative tracheobronchogram in case 1 showing marked stenosis of the trachea from the second thoracic vertebra to the carina (approximately 3.5 cm in length). Main bronchi are of almost normal caliber.
However, despite these favorable signs his respiratory difficulty never improved enough to allow for extubation. The patient died 3 months after the second tracheoplasty. An autopsy was not performed in compliance with the parents’ wishes. Case 2. An 8-month-old girl with severe respiratory distress was transferred to the intensive care unit of our hospital from a peripheral hospital. Weighing 3,100 g, she was born uneventfully after 39 weeks of a normal pregnancy. She remained well until 7 months of age when her respiratory condition deteriorated rapidly following an upper airway infection. She was intubated and put on a respirator at the first hospital, where a diagnosis of tracheal stenosis was made by bronchogram (Fig 3). Bronchofiberscopy performed at this hospital showed severe narrowing of the tracheal lumen with the carina no longer visible because of stenosis (Fig 4A). Esophageal tracheoplasty was performed using the same technique as in case 1. A PA sling was identified during the operation; however, no surgical treatment was considered necessary as a countermeasure. Extubation was attempted unsuccessfully at 7 days postoperatively, and was finally achieved at 14 days following surgery. Her respiratory condition remained unstable for the first 3 months after extubation as she experienced periods of stridor, retraction, and cyanosis that were severe at times. She was readmitted to the intensive care unit twice. The first time, she was reintubated for 3 days, and her condition was monitored under continuous positive airway pressure with satisfactory extubation. The second time, she recovered in 2 days with administrations of a mild sedative and nasal oxygen. Bronchoscopy (Fig 4B) and computed tomography were repeated to examine the condition of the tracheal lumen. Epithelialization in the esophageal section was complete at 1 month after surgery, and no collapse of the esophageal wall occurred. The size of the reconstructed trachea continues to increasing gradually with her growth (Fig 5). She is
2B).
Fig 2. Bronchofiberscopic findings in case 1. (A) Preoperative: fiberscope (2.7 mm in diameter) could not be inserted completely due to acute stenosis of the trachea. (B) Postoperative (at 1.5 months): tracheal lumen has become dilated with mucosa and some granulation present.
ESOPHAGEAL TRACHEOPLASTY
FOR TRACHEAL STENOSIS
647
reasons, we chose the pericardium as the grafting source for the first tracheoplasty in case 1 because it is pliant and the slit in the trachea could be covered easily. In addition, the pericardium can be used to reconstruct even major defects covering as much as three quarters of the circumference of the trachea. However, the grafted pericardial patch suddenly ruptured on the 12th postoperative day, requiring high-pressure manua1 ventilation in an attempt to improve his poor respiratory condition. In such cases requiring high-pressure ventilation, thicker material should be considered for grafting so that such high pressure can be tolerated better. Although an au-
Fig 3.
Preoperative tracheobronchogram
in case 2 showing stenoBoth
sis of the entire intrathoracic trachea (approximately 4.0 cm). main bronchi are also relatively stenotic.
the right main bronchus to achieve adequate ventilation. The PA sling was adjusted slightly downward so that an incision could be made on the posterior side of the trachea. Care was also taken so that the PA sling would not interfere with either the trachea or the esophagus. The longitudinal incision (approximately 4 cm in case 1, and 4.5 cm in case 2) made in the posterior side of the trachea just above the carina advanced upward to the proximal end of the stenotic area, determined at the point where the tip of the orally intubated tracheal tube (3.5-mm Portex tube) had reached. The left side of incised trachea was first sutured to the anterior esophageal wall with continuous 4-0 vicryl. The Portex tube was then passed down to expand the lumen in the affected trachea to an appropriate size. In both cases, the trachea had to be expanded to about twice the original diameter in order for the tube to pass through. The right edge of the incised trachea was then sutured to the esophageal wall. The main bronchus was extubated when suturing from the proximal end had nearly reached the distal end. At this point, the route of ventilation was switched from the cannulated tube in the left main bronchus to the stent tube which had been intubated orally, and suturing of the defect was completed. An intrathoracic drainage tube was then inserted, and the chest wall was closed in the usual manner. DISCUSSION
types of surgery for the treatment of Different congenital tracheal stenosis have been described using various autologous tissues, such as the dura mater,‘O esophagus,‘s4 costal cartilage,3 pericardium,5 and periosteum. l1 Rigid prosthetic materials have also been used in combination with some of these analogous tissues. 11,12Some factors should be considered when choosing grafting material to repair the trachea in children: (1) biocompatibility, (2) rigidity, and (3) the potential for growth. With the pericardium, Idriss et al5 have shown that early extubation can be achieved despite its pliability. For these
Fig 4. Bronchofiberscopic findings in case 2. (A) Preoperative: narrowing of the tracheal lumen is severe. Carina cannot be seen. (B) Postoperative (at 1 month): tracheal lumen is adequately dilated, and the esophageal section (lower region) is well epithelialized.
648
SASAKI ET AL
b-no)
Age
7
12
17
23
Post Op
(mo)
Pre Op
Weight
(kg)
9.1
12.1
13.2
14.2
Height
(cm)
69.0
73.4
79.2
87.6
lntrathoracic Trachea (from
CT)
L--l
1cm
topsy was not conducted in case 1 in compliance with the parents’ wishes, it is believed that high-pressure mechanical ventilation used over an extended period of about 6 months to maintain adequate ventilation may have induced further complications such as interstitial emphysema. Malacia of the main or more distal bronchi may also have been a possible contributing factor in this case. It is known that highpressure mechanical ventilation increases the incidence of overinflation unless an endotracheal tube can be placed beyond the stenotic lesion, which is usually impossible. l3 In the second tracheoplasty, which was urgently needed, we chose to use the esophageal wall for reconstruction because: (1) the technique is simpler than with other tissues, (2) the esophagus is available without requiring isolation and can therefore be well perfused, and (3) the esophagus is thick enough to tolerate high pressure. Since Kimura et al3 reported new tracheoplasty in 1982, tracheoplasty using the costal cartilage as a graft has been widely used.7,8J4J5 However, cartilage as well as isolated free materials tend to become necrotic, with restentosis being a common problem.7,8J6 Tsugawa et al7 attempted wrapping the grafted cartilage with the greater omentum in order to maintain adequate blood supply in the region. The esophagus is the only autologous tissue that does not require isolation for use in tracheoplasty and, therefore, poses no subsequent problems in blood supply. It is thought that the esophagus is too weak for maintaining the lumen. Ein et al4 and Loeff et al” used the esophagus as the donor site in three patients, two of whom died while the remaining one survived and recovered well. Of the two that did not
Fig 5. Growth of the constructed intrathoracic trachea in case 2.
survive, the cause of death in one case was presumed to be from the collapse of the esophageal wall into the tracheal lumen. The other patient survived for 7 months after the operation, but eventually died during a bronchoscopic examination for prolonged hypoxia. Donahoe and Gee17 reported using a similar reconstruction technique for a complete laryngotracheoesophageal cleft. They used the mucosal side of the esophagus to the lumen of the trachea with the patient doing well and with no malacia occurring. In the two cases in our experience as well, no malacia was noted. In case 2, respiratory conditions remained unstable for the first 3 months following extubation when periods of stridor, retraction, and cyanosis were observed, severely at times. Such instability in respiration may be attributed to a temporary collapse of the esophageal wall into the tracheal lumen. However, her condition has improved greatly and continues to remain stable. Although experience is still limited, the results of these two cases may serve to show that the esophageal wall can be used successfully for tracheoplasty in congenital tracheal stenosis. With the esophageal wall, we found that epithelialization could be achieved in 1 month with rigidity seen after 3 months of surgery. In the surviving case (case 2) the reconstructed trachea has also adapted well with the child’s growth. We believe this technique should be considered along with other methods in the surgical treatment for congenital tracheal stenosis because the esophagus is simple to use and pliable in adapting to a child’s growth. A PA sling has often been associated with cases of congenital tracheal stenosis.“J8 Such a phenomenon
ESOPHAGEAL
TRACHEOPLASTY
649
FOR TRACHEAL STENOSIS
has been termed the “ring-sling syndrome” by Berdon et ali9 The two cases in our experience were also of the ring-sling syndrome; however, surgical correction of the PA sling was considered unnecessary in both cases. The primary cause of respiratory distress in both cases seems to have been from long segment tracheal stenosis, with the PA sling having little or nothing to do with the compression to the trachea and right main bronchus. Chest x-rays showed no difference in lucency between the left and right lungs.
Postoperative bronchoscopies likewise showed no compression to the lower trachea nor to either main bronchi. From these findings, we conclude that primary operation should focus on the trachea in cases with the ring-sling syndrome. It should also be noted that the rate of arterial patency is low following the repair of a PA sling. l* In fact, patients surviving operations that combine treatment for ring-sling syndrome are very rare.2o,21
REFERENCES 1. Cantrell JR, Guild HG: Congenital stenosis of the trachea. Am J Surg 108:297-305,1964 2. Fonkalsrud EW, Sumida S: Tracheal replacement with autologous esophagus for tracheal stricture. Arch Surg 102:139-142, 1971 3. Kimura K, Mukohara N, Tsugawa C, et al: Tracheoplasty for congenital stenosis of the entire trachea. J Pediatr Surg 17:869-871, 1982 4. Ein SH, Friedberg J, Williams WG, et al: Tracheoplasty: A new operation for complete congenital tracheal stenosis. J Pediatr Surg 17:872-878,1982 5. Idriss FS, Delson SY, Ilbawi MN, et al: Tracheoplasty with pericardial patch for extensive tracheal stenosis in infants and children. J Thorac Cardiovasc Surg 88:527-536,1984 6. Choen RC, Filler RM, Konuma K, et al: The successful reconstruction of thoracic tracheal defects with free periosteal grafts. J Pediatr Surg 20:852-858, 1985 7. Tsugawa C, Kimura K, Muraji T, et al: Congenital stenosis involving a long segment of the trachea: Further experience in reconstructive surgery. J Pediatr Surg 23:471-475, 1988 8. Ueoka K, Okawa H, Ikebukuro K, et al: A case report of congenital extensive tracheal stenosis. J Jpn Sot Pediatr Surg 25:1025-1030,1989 9. Sasaki S, Naganawa N, Hara F, et al: Pulmonary artery sling with long segment tracheal stenosis: A case report and review of cases reported in Japan. J Jpn Sot Pediatr Surg 23:1277-1283,1987 10. Sabas AA, Uez JB, Rojas 0, et al: Replacement of the trachea with dura matter. J Thorac Cardiovasc Surg 74:761-765, 1977
11. Loeff DS, Filler RM, Vinograd I, et al: Congenital tracheal stenosis: A review of 22 patients from 1965 to 1987. J Pediatr Surg 23~744-748,1988 12. Moghissi K: Tracheal reconstruction with a prosthesis of marlex mesh and pericardium. J Thorac Cardiovasc Surg 69:499506,1975 13. Benjamin B, Pitken J, Choen D: Congenital tracheal stenosis. Ann Otol Rhino1 Laryngol90:364-371, 1981 14. Saad SA, Falla A: Management of intractable and extensive tracheal stenosis by implantation of cartilage graft. J Pediatr Surg 18:472-474, 1983 15. Campbell DN, Lilly JR: Surgery for total congenital tracheal stenosis. J Pediatr Surg 21:934-935, 1986 16. Dykes EH, Bahoric A, Smith C, et al: Reduced tracheal growth after reconstruction with pericardium. J Pediatr Surg 2525-29, 1990 17. Donahoe PK, Gee PE: Complete laryngotracheoesophageal cleft: Management and repair. J Pediatr Surg 19:143-148.1984 18. Campbell CD, Wernly JA, Koltip PC, et al: Aberrant left pulmonary artery (pulmonary artery sling): Successful repair and 24 year follow-up report. Am J Cardiol45:316-320, 1980 19. Berdon WE, Baker DH, Wung J-T, et al: Complete cartilagering tracheal stenosis associated with anomalous left pulmonary artery: The ring-sling complex. Radiology 152:57-64,1984 20. Bertolini A, Pelizza A, Panizzon A, et al: Vascular rings and slings: Diagnosis and surgical treatment of 49 patients. J Cardiovast Surg 26:7, 1985 (abstr) 21. Yamaguchi M, Ohshima Y, Ienaga T, et al: Successful repair of pulmonary sling associated with congenital tracheal stenosis in 8-month-old infant. J Jpn Assoc Thorac Surg 34:85-89, 1986
Tracheoplasty
for Congenital
Tracheal
Stenosis
By Shingi Sasaki, Fuji0 Hara, Toshinobu Ohwa, Takeshi Eguchi, and Akira Masaoka Nagoya, Japan l Two infants with congenital long tracheal stenosis underwent operation by means of an esophageal tracheoplasty. The first patient had previously undergone tracheal reconstruction using the pericardium. Although ventilation improved somewhat following this procedure, the pericardial patch suddenly ruptured 12 days after the operation, requiring an immediate esophageal tracheoplasty. The esophageal portion of the reconstructed trachea epithelialized 1 month later, with the lumen maintaining its proper size. However, the patient died 3 months after the second tracheoplasty. The cause of death was thought to be due to complications arising from prolonged high-pressure use of mechanical ventilation. He had been on a respirator for 6 months before the first tracheoplasty. The second patient has been doing well with no recurring respiratory problems for 25 months now. Her reconstructed trachea has adapted with her growth. This technique should be considered along with other forms of treatment for tracheal reconstruction because it is relatively simple and pliable. Copyright o 7992 by W. B. Saunders Company
INDEX plasty.
WORDS:
Tracheal
stenosis,
congenital;
tracheo-
C tion
ONGENITAL tracheal stenosis, whereby a porof the membrane is absent (complete cartilage rings), is a rare and lethal anomaly. Management of this severe condition remains controversial, especially when the entire or nearly entire trachea is involved (generalized type).’ Various surgical techniques using autologous tissues to repair the trachea have been reported. 2-6Kimura et al3 reported using costal cartilage as a graft in tracheopIasty in 1982, and this technique is now widely used. However, with such a method, the isolated cartilage sometimes develops into necrosis, leading to restenosis of the tracheal lumen.7,8 We chose the esophagus as the grafting source for tracheal reconstruction in two infants with congenital long tracheal stenosis. Because the esophagus is an autologous tissue that can be used without requiring isolation, this technique is considered simpler and less risky than other methods. Herein, we present the operative technique, rigidity, and potential for growth of the constructed trachea. MATERIALS AND METHODS Case Reports Case 1. This case was reported previously in Japanese.9 A boy was born after a normal pregnancy and remained well for the first month of life except for a tendency of turning the face to the right. Stridor and cyanosis developed shortly thereafter. At a peripheral hospital, he initially received therapy for bronchitis, but intubation and management with a ventilator were soon required Journal of Pediafric Surgery, Vol27, No 5 (May). 1992: pp 645-649
as his respiratory distress rapidly worsened. A bronchogram taken 2 weeks after artificial ventilation was begun showed tracheal stenosis extending from the second thoracic vertebra to the carina (Fig 1). With these conditions, he was transferred to this hospital. Bronchofiberscopy showed remarkable narrowing of the tracheal lumen just below the top of the endotracheal tube (Fig 2A). A fiberscope (2.7 mm in diameter) could not be inserted into more distal trachea because of acute stenosis in the region. A right posterolateral thoracotomy was performed after several failed attempts to intubate the stenotic portion with a small Portex tube (inner diameter of 2.0 to 2.5 mm). During the operation, we found a pulmonary artery sling (PA sling) arising from the right pulmonary artery and passing between the trachea and esophagus to the left lung. A pulmonary arteriopelry was performed whereby the PA sling was pulled toward the right posterior region by means of a Goretex sheet that was sutured to the pleural wall. This served to alleviate extrinsic compression of the right main bronchus and/or lower end of the trachea. Although his respiratory condition improved slightly immediately following this procedure, it was not sufficient enough to warrant a reduction in the high pressure required to control ventilation. Therefore, a total tracheoplasty of the stenotic segment was planned, but his parents refused such treatment. He continued to require assistance with high-pressure mechanical ventilation. For the next 5 months, he was also given muscle relaxants and morphine, during which time he sometimes suffered from severe difficulties in ventilation, as well as cyanosis, hypotension, and bradycardia. When he was 7 months old, his parents agreed to a second surgery in an attempt to alleviate his symptoms. We performed a tracheoplasty with a pericardial patch. The trachea was reached through the right posterolateral thoracotomy and was incised posteriorly. The tracheal wall defect was sutured with the pericardial patch which had been resected earlier. Ventilation during the operation was maintained by the same method as was used for the esophageal tracheoplasty (see details under Operative Technique below). The arteriopexy was removed because no compression of the lower trachea or right main bronchus by the PA sling was evident. His respiratory condition improved somewhat immediately following the tracheoplasty, although it was necessary to continue monitoring under highpressure ventilation in order to maintain adequate respiration. On the 12th postoperative day, high-pressure ventilation had to be increased as his respiratory condition deteriorated even further, and, as a consequence, the pericardial patch ruptured. A second tracheoplasty was immediately performed using the esophagus according to Ein’s technique4 without extracorporeal circulation. A bronchoscopy taken 1.5 months later showed the lumen of the trachea had become adequately dilated with evidence of some granulation. The grafted esophageal wall also appeared to have epithelialized completely, resembling that of normal trachea (Fig
From the Second Department of Surgery, Nagoya City University Medical School, Nagoya, Japan. Date accepted: April 16, 1991. Address reprint requests to Shingi Sasaki, MD, The Second Department of Surgery, Nagoya City UniversityMedical School, Kawasumi-I, M&ho-cho, M&ho-ku, Nagoya 467, Japan. Copyright o I992 by WB. Saunders Company 0022-3468/92/2705-0028$03.00f0
645
SASAKI ET AL
646
now 2 years 9 months old, and she has experienced no recurring respiratory difficulties except for slight stridor at times.
Operative Technique The trachea and esophagus were reached through right posterolateral thoracotomy. After entering the thorax, the azygosvein was ligated and dissected. The pleura of the mediastinal side was then opened. The trachea and esophagus were mobilized and taped separately. The posterior wall of the distal end of the stenotic region, immediately above the carina, was incised longitudinally and intubated into the left main bronchus with a 3.0-mm or 3.5-mm Portex tube to secure ventilation. However, in case 1 adequate ventilation could not be achieved using only a Portex tube with PO2 at 67.0 mm Hg and PC01 at 71.6 mm Hg. Therefore, a smaller tube (8F) connected to a separate anesthetic system was inserted into
Fig 1. Preoperative tracheobronchogram in case 1 showing marked stenosis of the trachea from the second thoracic vertebra to the carina (approximately 3.5 cm in length). Main bronchi are of almost normal caliber.
However, despite these favorable signs his respiratory difficulty never improved enough to allow for extubation. The patient died 3 months after the second tracheoplasty. An autopsy was not performed in compliance with the parents’ wishes. Case 2. An 8-month-old girl with severe respiratory distress was transferred to the intensive care unit of our hospital from a peripheral hospital. Weighing 3,100 g, she was born uneventfully after 39 weeks of a normal pregnancy. She remained well until 7 months of age when her respiratory condition deteriorated rapidly following an upper airway infection. She was intubated and put on a respirator at the first hospital, where a diagnosis of tracheal stenosis was made by bronchogram (Fig 3). Bronchofiberscopy performed at this hospital showed severe narrowing of the tracheal lumen with the carina no longer visible because of stenosis (Fig 4A). Esophageal tracheoplasty was performed using the same technique as in case 1. A PA sling was identified during the operation; however, no surgical treatment was considered necessary as a countermeasure. Extubation was attempted unsuccessfully at 7 days postoperatively, and was finally achieved at 14 days following surgery. Her respiratory condition remained unstable for the first 3 months after extubation as she experienced periods of stridor, retraction, and cyanosis that were severe at times. She was readmitted to the intensive care unit twice. The first time, she was reintubated for 3 days, and her condition was monitored under continuous positive airway pressure with satisfactory extubation. The second time, she recovered in 2 days with administrations of a mild sedative and nasal oxygen. Bronchoscopy (Fig 4B) and computed tomography were repeated to examine the condition of the tracheal lumen. Epithelialization in the esophageal section was complete at 1 month after surgery, and no collapse of the esophageal wall occurred. The size of the reconstructed trachea continues to increasing gradually with her growth (Fig 5). She is
2B).
Fig 2. Bronchofiberscopic findings in case 1. (A) Preoperative: fiberscope (2.7 mm in diameter) could not be inserted completely due to acute stenosis of the trachea. (B) Postoperative (at 1.5 months): tracheal lumen has become dilated with mucosa and some granulation present.
ESOPHAGEAL TRACHEOPLASTY
FOR TRACHEAL STENOSIS
647
reasons, we chose the pericardium as the grafting source for the first tracheoplasty in case 1 because it is pliant and the slit in the trachea could be covered easily. In addition, the pericardium can be used to reconstruct even major defects covering as much as three quarters of the circumference of the trachea. However, the grafted pericardial patch suddenly ruptured on the 12th postoperative day, requiring high-pressure manua1 ventilation in an attempt to improve his poor respiratory condition. In such cases requiring high-pressure ventilation, thicker material should be considered for grafting so that such high pressure can be tolerated better. Although an au-
Fig 3.
Preoperative tracheobronchogram
in case 2 showing stenoBoth
sis of the entire intrathoracic trachea (approximately 4.0 cm). main bronchi are also relatively stenotic.
the right main bronchus to achieve adequate ventilation. The PA sling was adjusted slightly downward so that an incision could be made on the posterior side of the trachea. Care was also taken so that the PA sling would not interfere with either the trachea or the esophagus. The longitudinal incision (approximately 4 cm in case 1, and 4.5 cm in case 2) made in the posterior side of the trachea just above the carina advanced upward to the proximal end of the stenotic area, determined at the point where the tip of the orally intubated tracheal tube (3.5-mm Portex tube) had reached. The left side of incised trachea was first sutured to the anterior esophageal wall with continuous 4-0 vicryl. The Portex tube was then passed down to expand the lumen in the affected trachea to an appropriate size. In both cases, the trachea had to be expanded to about twice the original diameter in order for the tube to pass through. The right edge of the incised trachea was then sutured to the esophageal wall. The main bronchus was extubated when suturing from the proximal end had nearly reached the distal end. At this point, the route of ventilation was switched from the cannulated tube in the left main bronchus to the stent tube which had been intubated orally, and suturing of the defect was completed. An intrathoracic drainage tube was then inserted, and the chest wall was closed in the usual manner. DISCUSSION
types of surgery for the treatment of Different congenital tracheal stenosis have been described using various autologous tissues, such as the dura mater,‘O esophagus,‘s4 costal cartilage,3 pericardium,5 and periosteum. l1 Rigid prosthetic materials have also been used in combination with some of these analogous tissues. 11,12Some factors should be considered when choosing grafting material to repair the trachea in children: (1) biocompatibility, (2) rigidity, and (3) the potential for growth. With the pericardium, Idriss et al5 have shown that early extubation can be achieved despite its pliability. For these
Fig 4. Bronchofiberscopic findings in case 2. (A) Preoperative: narrowing of the tracheal lumen is severe. Carina cannot be seen. (B) Postoperative (at 1 month): tracheal lumen is adequately dilated, and the esophageal section (lower region) is well epithelialized.
648
SASAKI ET AL
b-no)
Age
7
12
17
23
Post Op
(mo)
Pre Op
Weight
(kg)
9.1
12.1
13.2
14.2
Height
(cm)
69.0
73.4
79.2
87.6
lntrathoracic Trachea (from
CT)
L--l
1cm
topsy was not conducted in case 1 in compliance with the parents’ wishes, it is believed that high-pressure mechanical ventilation used over an extended period of about 6 months to maintain adequate ventilation may have induced further complications such as interstitial emphysema. Malacia of the main or more distal bronchi may also have been a possible contributing factor in this case. It is known that highpressure mechanical ventilation increases the incidence of overinflation unless an endotracheal tube can be placed beyond the stenotic lesion, which is usually impossible. l3 In the second tracheoplasty, which was urgently needed, we chose to use the esophageal wall for reconstruction because: (1) the technique is simpler than with other tissues, (2) the esophagus is available without requiring isolation and can therefore be well perfused, and (3) the esophagus is thick enough to tolerate high pressure. Since Kimura et al3 reported new tracheoplasty in 1982, tracheoplasty using the costal cartilage as a graft has been widely used.7,8J4J5 However, cartilage as well as isolated free materials tend to become necrotic, with restentosis being a common problem.7,8J6 Tsugawa et al7 attempted wrapping the grafted cartilage with the greater omentum in order to maintain adequate blood supply in the region. The esophagus is the only autologous tissue that does not require isolation for use in tracheoplasty and, therefore, poses no subsequent problems in blood supply. It is thought that the esophagus is too weak for maintaining the lumen. Ein et al4 and Loeff et al” used the esophagus as the donor site in three patients, two of whom died while the remaining one survived and recovered well. Of the two that did not
Fig 5. Growth of the constructed intrathoracic trachea in case 2.
survive, the cause of death in one case was presumed to be from the collapse of the esophageal wall into the tracheal lumen. The other patient survived for 7 months after the operation, but eventually died during a bronchoscopic examination for prolonged hypoxia. Donahoe and Gee17 reported using a similar reconstruction technique for a complete laryngotracheoesophageal cleft. They used the mucosal side of the esophagus to the lumen of the trachea with the patient doing well and with no malacia occurring. In the two cases in our experience as well, no malacia was noted. In case 2, respiratory conditions remained unstable for the first 3 months following extubation when periods of stridor, retraction, and cyanosis were observed, severely at times. Such instability in respiration may be attributed to a temporary collapse of the esophageal wall into the tracheal lumen. However, her condition has improved greatly and continues to remain stable. Although experience is still limited, the results of these two cases may serve to show that the esophageal wall can be used successfully for tracheoplasty in congenital tracheal stenosis. With the esophageal wall, we found that epithelialization could be achieved in 1 month with rigidity seen after 3 months of surgery. In the surviving case (case 2) the reconstructed trachea has also adapted well with the child’s growth. We believe this technique should be considered along with other methods in the surgical treatment for congenital tracheal stenosis because the esophagus is simple to use and pliable in adapting to a child’s growth. A PA sling has often been associated with cases of congenital tracheal stenosis.“J8 Such a phenomenon
ESOPHAGEAL
TRACHEOPLASTY
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FOR TRACHEAL STENOSIS
has been termed the “ring-sling syndrome” by Berdon et ali9 The two cases in our experience were also of the ring-sling syndrome; however, surgical correction of the PA sling was considered unnecessary in both cases. The primary cause of respiratory distress in both cases seems to have been from long segment tracheal stenosis, with the PA sling having little or nothing to do with the compression to the trachea and right main bronchus. Chest x-rays showed no difference in lucency between the left and right lungs.
Postoperative bronchoscopies likewise showed no compression to the lower trachea nor to either main bronchi. From these findings, we conclude that primary operation should focus on the trachea in cases with the ring-sling syndrome. It should also be noted that the rate of arterial patency is low following the repair of a PA sling. l* In fact, patients surviving operations that combine treatment for ring-sling syndrome are very rare.2o,21
REFERENCES 1. Cantrell JR, Guild HG: Congenital stenosis of the trachea. Am J Surg 108:297-305,1964 2. Fonkalsrud EW, Sumida S: Tracheal replacement with autologous esophagus for tracheal stricture. Arch Surg 102:139-142, 1971 3. Kimura K, Mukohara N, Tsugawa C, et al: Tracheoplasty for congenital stenosis of the entire trachea. J Pediatr Surg 17:869-871, 1982 4. Ein SH, Friedberg J, Williams WG, et al: Tracheoplasty: A new operation for complete congenital tracheal stenosis. J Pediatr Surg 17:872-878,1982 5. Idriss FS, Delson SY, Ilbawi MN, et al: Tracheoplasty with pericardial patch for extensive tracheal stenosis in infants and children. J Thorac Cardiovasc Surg 88:527-536,1984 6. Choen RC, Filler RM, Konuma K, et al: The successful reconstruction of thoracic tracheal defects with free periosteal grafts. J Pediatr Surg 20:852-858, 1985 7. Tsugawa C, Kimura K, Muraji T, et al: Congenital stenosis involving a long segment of the trachea: Further experience in reconstructive surgery. J Pediatr Surg 23:471-475, 1988 8. Ueoka K, Okawa H, Ikebukuro K, et al: A case report of congenital extensive tracheal stenosis. J Jpn Sot Pediatr Surg 25:1025-1030,1989 9. Sasaki S, Naganawa N, Hara F, et al: Pulmonary artery sling with long segment tracheal stenosis: A case report and review of cases reported in Japan. J Jpn Sot Pediatr Surg 23:1277-1283,1987 10. Sabas AA, Uez JB, Rojas 0, et al: Replacement of the trachea with dura matter. J Thorac Cardiovasc Surg 74:761-765, 1977
11. Loeff DS, Filler RM, Vinograd I, et al: Congenital tracheal stenosis: A review of 22 patients from 1965 to 1987. J Pediatr Surg 23~744-748,1988 12. Moghissi K: Tracheal reconstruction with a prosthesis of marlex mesh and pericardium. J Thorac Cardiovasc Surg 69:499506,1975 13. Benjamin B, Pitken J, Choen D: Congenital tracheal stenosis. Ann Otol Rhino1 Laryngol90:364-371, 1981 14. Saad SA, Falla A: Management of intractable and extensive tracheal stenosis by implantation of cartilage graft. J Pediatr Surg 18:472-474, 1983 15. Campbell DN, Lilly JR: Surgery for total congenital tracheal stenosis. J Pediatr Surg 21:934-935, 1986 16. Dykes EH, Bahoric A, Smith C, et al: Reduced tracheal growth after reconstruction with pericardium. J Pediatr Surg 2525-29, 1990 17. Donahoe PK, Gee PE: Complete laryngotracheoesophageal cleft: Management and repair. J Pediatr Surg 19:143-148.1984 18. Campbell CD, Wernly JA, Koltip PC, et al: Aberrant left pulmonary artery (pulmonary artery sling): Successful repair and 24 year follow-up report. Am J Cardiol45:316-320, 1980 19. Berdon WE, Baker DH, Wung J-T, et al: Complete cartilagering tracheal stenosis associated with anomalous left pulmonary artery: The ring-sling complex. Radiology 152:57-64,1984 20. Bertolini A, Pelizza A, Panizzon A, et al: Vascular rings and slings: Diagnosis and surgical treatment of 49 patients. J Cardiovast Surg 26:7, 1985 (abstr) 21. Yamaguchi M, Ohshima Y, Ienaga T, et al: Successful repair of pulmonary sling associated with congenital tracheal stenosis in 8-month-old infant. J Jpn Assoc Thorac Surg 34:85-89, 1986
