Esophageal Atresia and Tracheoesophageal Fistula

Transcription

1 Esophageal Atresia and Tracheoesophageal Fistula James V. Richardson, M.D., Sharon E. Heintz, P.A., Nicholas P. Rossi, M.D., Creighton B. Wright, M.D., Donald B. Doty, M.D., and Johann L. Ehrenhaft, M.D. ABSTRACT Fifty-seven babies were surgically treated for esophageal atresia and tracheoesophageal fistula between 1968 and Forty-eight (84%) had proximal esophageal atresia and a distal tracheoesophageal fistula, 2 (4%) had proximal and distal esophageal atresia and no tracheoesophageal fistula, and 7 (12%) had a tracheoesophageal fistula without esophageal atresia. Primary repair was accomplished in, 43 patients (75%), colon interposition was required in 5 (9%), while the remainder had staged or palliative repairs. Forty-six (81%) survived surgical treatment. All 21 babies in Waterston Category A, 90% of 20 in Category B, and 44% of 16 in Category C survived surgical treatment. Serious complications occurred in 17 (30%), and dilatable strictures and other minor problems developed in 27 (47%). Late follow-up (mean, 48 months) revealed 3 (7%) late deaths, 2 of which were due to congenital heart disease. Three patients required late colon interposition, and several require frequent dilatations of the esophagus. The Category A and B survivors are all functionally well, while the 5 surviving Category C patients are all significantly impaired by associated anomalies. Esophageal atresia and tracheoesophageal fistula were described by Thomas Gibson in 1697 [l]. Ladd and Levin advanced the management of this anomaly some 243 years later when they successfully treated 2 patients by staged repairs [I]. Successful primary surgical repair was accomplished in 1941 by Haight [71. Since then, important advancements in supportive and surgical care for babies with this condition have From the Division of Thoracic and Cardiovascular Surgery, Department of Surgery, the University of Iowa Hospitals and Clinics, Iowa City, IA. Presented at the Twenty-sixth Annual Meeting of the Southern Thoracic Surgical Association, Nov 1-3,1979, San Antonio, TX. Address reprint requests to Dr. Ehrenhaft, Division of Thoracic and Cardiovascular Surgery, The University of Iowa Hospitals and Clinics, Iowa City, IA resulted in a steady improvement in results [9, 18,21, 22, 25, 261. Concomitant complex anomalies, preoperative pneumonia and sepsis, and prematurity continue to be the primary determinants of mortality in most recent surgical series [9, 18, 21, 22, 25, 261. This paper reviews the surgical experience with esophageal atresia and tracheoesophageal fistula of the Division of Thoracic and Cardiovascular Surgery at the University of Iowa Hospitals and Clinics between 1968 and The results are encouraging, but have prompted us to reexamine some of the details of our management. Material and Methods Fifty-seven babies with esophageal atresia and tracheoesophageal fistula were treated surgically in the Division of Thoracic and Cardiovascular Surgery at the University of Iowa Hospitals and Clinics from 1968 to Thirty were boys and 27 were girls. Many were born prematurely, and 25 (44%) weighed less than 2,500 gm. Weight, pneumonia, and associated anomalies were used to group these patients according to the method of Waterston and associates [261. Twenty-one (37%) of the babies had nearly normal to normal birth weight and no major pneumonia or other congenital anomalies (Category A). Another 20 (35%) were small, well babies or normal weight babies with moderate pneumonia or mild congenital anomalies (Category B). The remaining 16 babies (28%) were very ill, small babies with severe pneumonia or severe congenital anomalies (Category C). The anatomical defects followed the usual distribution: 48 patients (84%) had Type I defects, 2 (4%) Type I1 defects, and 7 (12%) Type I11 defects (Figure). Thirty-five patients also had other important congenital anomalies. These associated congenital anomalies included the following: gastrointestinal complications, 11 patients; cardiovascular, 9; skeletal, 6; respiratory, 6; genitourinary, 2; and Down s syndrome, 1 patient by James V. Richardson

2 365 Richardson et al: Esophageal Atresia and TEF TYPE I TYPE H Table 2. Complications of Surgical Treatment Complication Major and minor leaks Strictures Mediastinal abscesslempyema 8 Recurrent TEF 4 Duodenal perforation 2 Total 44 No. of Patients 2% Distribution of anatomical types of esophageal atresia arid tracheoesophageal fistula. TEF = tracheoesophageal fistula. Primary surgical repair (ligation of the tracheoesophageal fistula, esophagoesophagostomy, and usually gastrostomy) was performed in 43 patients (75%). Five complete repairs were staged. Colon interposition was necessary in 5 patients (9%); two were staged. Palliative procedures (esophagostomy, ligation of the tracheoesophageal fistula, and gastrostomy) were performed in 9 patients (l6%), primarily those in Category C. Early and Late Results In the entire group, 46 (81%) survived (Table 1). Serious complications occurred in 17 patients (30%) (Table 2). Forty-three (93%) of these early survivors are alive with a mean follow-up of 48 months. Each Waterston [261 category is discussed in turn. Category A Babies in this category have normal or nearly normal weight without major pneumonia or other anomalies. All of the 21 babies survived surgical repair. Major complications of anas- Table 1. Mortality of Surgical Repair Waterston No. of No. of Category Patients Deaths Percent A B C Total tomotic leaks requiring reoperation or prolonged drainage and recurrent tracheoesophageal fistula occurred in 4 (19'/0). Minor leaks and dilatable strictures occurred in 15 (71%). There have been no late deaths. One child required a colon interposition and the remainder are well. Category B Babies in this category are small and well or they have normal weight and moderate pneumonia or other anomalies. Eighteen (90%) of these 20 patients survived surgical repair. Serious complications occurred in 4 (20%) and minor complications in 12 (60%). There was 1 late death 2'/2 months postoperatively, which was due to a mediastinal abscess and tracheostomy obstruction. The remaining 17 patients are doing well. Category C Babies in this category are small and have severe pneumonia and other severe anomalies. Seven (44%) of the 16 children in this category survived operation. Five (71%) of the 7 who had primary definitive repair died. Four (57%) of the 7 patients who underwent palliative repairs died. Both patients who had staged repairs survived, however. Five (56%) of the 9 deaths were due to severe pneumonia and sepsis existing preoperatively. Serious complications occurred in 9 patients (56%), 7 of whom died. Of those who survived, 2 (29%) died late of congenital heart disease. Both of them had

3 366 The Annals of Thoracic Surgery Vol 29 No 4 April 1980 palliative procedures. Two others have required colon interposition, and an additional 2 have other serious congenital malformations. Commeht Esophageal atresia occurs probably as a result of epithelial overgrowth within the lumen of the esophagus. Abnormal septation of the laryngotracheal groove results in tracheoesophageal fistula. Both of these conditions are thought to develop before the fourth week of gestation. Variations of classic esophageal atresia and tracheoesophageal fistula can be explained by partial maldevelopments in these areas [10,201. Several distinct types of esophageal atresia and tracheoesophageal fistula are described [lo, 11, 171. Most commonly, esophageal atresia occurs with a distal tracheoesophageal fistula (approximately 85% of cases). Esophageal atresia without esophageal fistula (approximately 8%), tracheoesophageal fistula without atresia (approximately 4%), esophageal atresia with proximal fistula (approximately YO), and esophageal atresia with double fistulae (approximately 1%) comprise the remainder of the well known anatomical forms [lo, 11, 171. Our experience closely parallels these known incidences (see Figure). Waterston and associates [26] devised a method of classifying infants with this condition. This classification has a strongly positive correlation with survival. Generally speaking, term babies with minimal pneumonia and associated anomalies can be expected to do well. Small babies with severe pneumonia and other severe congenital anomalies do less well and are destined to difficult, long-term illnesses. Our experience illustrates that this classification is very useful in predicting the early results of surgical treatment (see Table 1). Proper medical therapy of infants with esophageal atresia and esophageal fistula begins with the pediatrician or family physician who first sees the child and makes the diagnosis. Initial management should consist of esophageal sump suction [19], hydration, and antibiotics. Rapid transport to a facility that has a qualified surgeon is the next important step. Primary surgical repair, accomplished by Haight [7] in 1941, should be considered for all good-risk babies. Extrapleural exposure of the esophagus is preferred by us and most other authors [5, 6, Ligation of the fistula, repair of the tracheal defect, and esophagoesophagostomy with drainage of the area completes the operation. One-layer anastomosis, endto-end, end-to-side [15, 241, or Haight s [6, 221 method, seem to be acceptable. Gastrostomy is performed before, as a temporizing decompressing procedure, or as a part of the primary repair by most authors [6, 9-11, 13, 14, 18, 21, 221. In 1976, Tyson [251 advocated omission of this step, a concept with which we disagree. Delayed primary repair is preferred by several groups [5, 10, 11, 181 for moderaterisk babies to allow stabilization and improvement of medical problems. Gastrostomy is added to the primary management, and definitive repair is postponed for several days. Staged repairs for high-risk babies is proposed by us and several other groups [13, 14, 18, 221. Variations on this management program include prdonged delayed management or initial management coupled with gastrostomy, esophagostomy, and ligation of the tracheoesophageal fistula with late primary reconstruction. Late reconstruction, after resolution of other congenital anomalies and severe medical problems, may be accomplished primarily or by colon interposition. Colon interposition is generally reserved for babies in whom approximation of the two ends of the atretic esophagus is impossible. Elongation of the proximal pouch by pneumatic dilatation has been recommended by some authors [12, 231, but we have no experience with this technique. In 1965, Bentley [21 advocated primary colon interposition in this situation, even in high-risk babies. It is not unusual for colonic interposition to be necessary after attempts at primary reconstruction. We believe that this procedure should be used rarely and that every effort should be made to approximate the ends of the atretic esophagus, recognizing that a higher leakage rate will occur. Most of these leaks, however, can be controlled and are well tolerated by babies if the extrapleural approach has been used. Early results are primarily related to the preoperative status of the patients [26]. For good-

4 367 Richardson et al: Esophageal Atresia and TEF risk babies (Waterston Categories A and B), early survival of 70 to 100% can be anticipated [18, 21, 22, 261. Survival in these groups in our series was 95% (39 of 41 patients). For severely ill patients (Category C), results are uniformly suboptimal [18, 21, 22, 261. Early overall survival in this series was 44% (7 of 16 patients) for this group of babies. Five (71%) of our 7 patients in Category C who underwent attempts at primary repair died, while both patients who had staged repairs survived. This experience is in agreement with that of Koop and colleagues [13, 141 who reported a 92% survival (12 of 13 patients) in these severely ill babies. Stothert [21], Strode1 [22], and their co-workers reported similar good results with staged repairs in this group. Late results depend on operative results, in part, and on the initial clinical state. Despite acceptable early results in Category C babies, these patients may die of other congenital anomalies or have a poor quality of life because of mental retardation and other severe problems. Functional results for babies in Categories A and B are generally good. Many patients require dilatation of late esophageal strictures, but can be managed well and have fairly normal growth and development. Burgess [3], Duranceau [4], Lind [161, and their associates demonstrated definite esophageal motor abnormalities in patients who have had repair of esophageal atresia and tracheoesophageal fistula. While stricture formation may be a manageable problem, these motor dysfunctions of the esophagus may be a troublesome and often untreatable condition resulting in dysphagia. The majority of our patients had good long-term (mean, 48 months) functional results. Some do have dysphagia, probably related to stricture formation and also possibly to these motor derangements. We conclude that primary surgical treatment of esophageal atresia and tracheoesophageal fistula can be expected to give excellent early results in good- and moderate-risk patients with expected good long-term results. Staging of high-risk babies is most appropriate and probably will result in improved early survival although long-term functional results are often suboptimal. We believe that the extrapleural approach is preferable and that every effort should be made to approximate the atretic esophagus. Gastrostomy remains an essential part of our management of these children. References 1. Ashcraft KW, Holder TM: The story of esophageal atresia and tracheoesophageal fistula. Surgery 65:332, Bentley JFR: Primary colonic substitution for atresia of the esophagus. Surgery 58:731, Burgess JN, Carlson HC, Ellis FH Jr: Esophageal function after successful repair of esophageal atresia and tracheoesophageal fistula. J Thorac Cardiovasc Surg 56:667, Duranceau A, Fisher SR, Flye MW, et al: Motor function of the esophagus after repair of esophageal atresia and tracheoesophageal fistula. Surgery 82:116, Ferguson CC: Management of infants with esophageal atresia and tracheoesophageal fistula. Ann Surg 172:750, Haight C: Some observations on esophageal atresias and tracheoesophageal fistulas of congenital origin. J Thorac Surg 34:141, Haight C, Townsley H: Congenital atresia of the esophagus with tracheoesophageal fistula: extrapleural ligation of fistula and end-to-end anastomosis of esophageal segments. Surg Gynecol Obstet 76:672, Hertzler JH: Congenital esophageal atresia: problems and management. Am J Surg 109:780, Holden MP, Wooler GH: Tracheo-oesophageal fistula and oesophageal atresia: results of 30 years experience. Thorax 25:406, Holder TM, Ashcraft KW: Esophageal atresia and tracheo-esophageal fistula. Curr Probl Surg: August, Holder TM, Ashcraft KW: Esophageal atresia and tracheoesophageal fistula (collective review). Ann Thorac Surg 9:445, Howard R, Myers NA: Esophageal atresia: a technique for elongating the upper pouch. Surgery 58:725, Koop CE, Hamilton JP: Atresia of the esophagus: increased survival with staged procedures in the poor-risk infant. Ann Surg 162:389, Koop CE, Schnaufer L, Broennle AM: Esophageal atresia and tracheoesophageal fistula: supportive measures that affect survival. Pediatrics 54:558, Leix F, Schwab CE: End to side operative technic for esophageal atresia with tracheoesophageal fistula. Am J Surg 118:225, Lind JF, Blanchard RJ, Guyda H: Esophageal motility in tracheoesophageal fistula and esophageal atresia. Surg Gynecol Obstet 123:557, Randolph JG, Altman RP: Pediatric surgery, in Surgery: A Concise Guide to Clinical Practice.

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