the University of Michigan Medical School; Surgeon-in-Chief, C. S. Mott. Children's Hospital ... independently by Leven of St. Paul and Ladd of Boston in patients ...
with Esophageal Atresia
Beginning with Cameron Haight's First Operation in 1935
PETER B. MANNING, M.D.* ROSS A. MORGAN, M.D.* ARNOLD G. CORAN, M.D.t
JOHN R. WESLEY, M.D.4 THEODORE Z. POLLEY, JR., M.D.§
Four hundred twenty-six patients with esophageal atresia with or without tracheoesophageal fistula have been primarily cared for at the University of Michigan Medical Center since Cameron Haight's initial experience with this entity. Over the period of observation, the incidence of new cases as well as the number of associated anomalies has remained constant. The long-term survival of these patients has steadily improved over the past half-century from 36% in the pre-1950 era to 84% during the most recent 20 years. Conversely, operative mortality has shown a progressive decline from 56% early in the authors' series to 6.9% more recently, despite a steady increase in the proportion of high-risk neonates seen at the University of Michigan Medical Center during this time span. In the last 9 years, there have been no postoperative deaths in group A or B risk infants (36 patients), while the rate has been 18.2% in group C risk babies (27 patients); almost all of these deaths were due to severe associated anomalies. During the last 10 years, the authors have changed their technique of anastoinosis from a two- to a one-layer method while still advocating a primary repair via an extrapleural approach. Although this change has resulted in a modest increase in the rate of anastomotic leak (17% vs. 6.2%, p < 0.03), the leaks have been small and asymptomatic because of the extrapleural approach and, as a result, have been managed conservatively without any untoward sequelae. Conversely, there has Presented at the 106th Annual Meeting of the American Surgical Association, Hot Springs, Virginia, April 24-26, 1986. * Surgical Resident, the University of Michigan Medical School. t Professor of Surgery and Head of the Section of Pediatric Surgery, the University of Michigan Medical School; Surgeon-in-Chief, C. S. Mott Children's Hospital. t Associate Professor of Pediatric Surgery, the University of Michigan Medical School. § Assistant Professor of Pediatric Surgery, the University of Michigan Medical School. 1 Professor of Thoracic Surgery, the University of Michigan Medical School. ¶ Professor of Thoracic Surgery, Former Head of the Section of Thoracic Surgery, the University of Michigan Medical School; Chiefof Clinical Affairs, the University of Michigan Hospitals. Reprint requests: Arnold G. Coran, M.D., C. S. Mott Children's Hospital, Room F7516, Box 66, Ann Arbor, MI 48109. Submitted for publication: April 28, 1986.
DOUGLAS M. BEHRENDT, M.D.II MARVIN M. KIRSH, M.D.II HERBERT E. SLOAN, M.D.¶
From the Department of Surgery, the University of Michigan Medical School, and C. S. Mott Children's Hospital, Ann Arbor, Michigan
been a significant decrease in the rate of stricture formation with the one-layer anastomosis (4.3% vs. 23.3%, p < 0.002). While this may in part be explained by the change in anastomotic technique, it is felt that the more aggressive diagnosis and surgical management of gastroesophageal reflux (seen in 37.9% of our recent group) have contributed greatly to this decrease. The steady improvement in survival over this 50-year period, in spite of the increasing number of high-risk infants, is attributable to major improvements in neonatal care before, during, and after operation.
F aIFTY YEARS AGO, the first attempt at surgical management of esophageal atresia (EA) with tracheoesophageal fistula (TEF) was undertaken at our institution. Within a decade, Cameron Haight had reported the successful outcome of primary esophageal anastomosis and fistula division.' With few modifications in the surgical approach, great strides have been made in more recent decades primarily because of improved anesthetic management, appropriate fluid and antibiotic management, and developments in neonatal intensive care. Drastic reduction in operative mortality and corresponding improvement in long-term survival have accompanied these advances. The earliest description of congenital EA is found in a report by Durston from 1670 in one member of conjoined (Siamese) twins.2 A TEF was not present. The first report of the more common form of this anomaly (atresia with
TEF) is credited to Thomas Gibson in his fifth edition of The Anatomy of Humane Bodies Epitomized, published in 1697.3 Management by nonsurgical means resulted in 100% mortality over the next 2.5 centuries. A surgical approach to this entity awaited the devel-
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ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA
opment of thoracic surgery as a specific discipline in the 1920s. In 1936, Lanman first attempted a primary repair of EA.4 The first survivors with the anomaly were reported independently by Leven of St. Paul and Ladd of Boston in patients admitted on successive days in late 1939.5,6 Both cases were managed using a staged approach with initial gastrostomy, secondary fistula ligation, or division with cervical esophagostomy, followed by the creation of an antethoracic skin tube conduit from the esophagostomy to the gastrostomy. The first reported patient with EA at the University of Michigan was seen in 1935 and was managed unsuccessfully with gastrostomy alone. In 1939, Haight first attempted a primary repair. Following four failed attempts at achieving survival with primary repair, little enthusiasm was present when the next patient with this disorder was transferred to the University of Michigan Hospital in early 1941. The infant was an "unusually robust" 12-day-old child weighing 8 lb 4 oz at admission. In this patient, the first successful primary repair of EA with TEF was accomplished using a left extrapleural approach and a singlelayer anastomosis. After operation, the patient developed an anastomic leak, which was managed without surgery. She later developed a stricture at the anastomosis, which responded to a single dilatation. No other complications followed, and the child ate and continued to grow normally. In 1943, Haight revised his procedure to a right extrapleural approach, as he felt that better exposure of the distal segment was obtained from this side. He also moved to a modified two-layer, "telescoping" anastomosis in the hope of decreasing the risk of leak. Many ofHaight's initial teachings continue to guide our current management of the infant with congenital atresia of the esophagus. Our current approach continues to follow the principles of primary repair whenever feasible and the use of an extrapleural exposure of the esophagus. In the last 10 years, we have departed from the use of the classic Haight, two-layer, telescoping anastomosis in favor of a singlelayer method. The various advantages of these points of management remain a topic of discussion. In this report, we update the experience with EA and TEF at the University of Michigan and present trends observed over the past half century and a detailed analysis of our more recent results in an attempt to address current controversies in management.
1943,' 1957,7 and 1966,8 and by Strodel et al. in 1979.9 To update the series, we reviewed all charts of patients admitted from January 1977 through September 1985 with a diagnosis of EA and/or TEF. The information obtained was compared with data from the previous reviews. All cases were classified into anatomic type ofanomaly based on both the criteria described by Haight (groups A to G, plus H-type TEF)8 and the more condensed classification of Gross (groups A to E).'0 Infants were also assigned to risk groups A, B, or C, as described by Waterston et al.' 1,12: group A-birth weight greater than 2500 g and otherwise well; group B-birth weight 2000-2500 g and well or higher weight with moderate associated anomalies; group C-birth weight less than 2000 g or higher with severe associated anomalies. A severe anomaly was defined as one that required operative intervention or intensive care management to sustain life. Pneumonia was omitted from consideration in the Waterston classification as suggested by Louhimo and Lindahl'3 and is discussed more thoroughly below. Patient survival for determination of operative mortality was defined as a feeding child who was discharged from the hospital regardless of duration of stay following operation. Overall survival data include analysis of all cases in which the anomaly was diagnosed regardless of whether the patients were operated on.
Materials and Methods From March 1935 to September 1985, a total of 428 patients with EA and/or TEF were admitted to the University of Michigan Medical Center. Patients initially operated on at other institutions have been excluded. Earlier reviews of our experience were presented by Haight in
Associated Anomalies The proportion of patients with associated congenital anomalies has not changed significantly over the period of observation (Table 2). Cardiovascular and gastrointestinal malformations are the most frequently seen major anomalies, while skeletal and limb defects are the most
Results Sixty-three new cases of EA with or without TEF were seen at the University of Michigan, C. S. Mott Children's Hospital, since our series was last compiled in 1976. The year-to-year incidence of cases has varied somewhat, but over the course of the last 50 years a fairly constant rate of eight to nine new cases per year has been observed. An incidence rate of 1 in 4500 births has been estimated based on early data from our series. Table 1 shows the distribution of patients by Waterston risk class over the last 2 decades. Overall survival data are also given. Figure 1 displays the anatomic type of anomaly based on both the Haight and Gross classifications. Currently, we find that the more simplified scheme of Gross minimizes confusion. A proximal blind pouch with distal TEF remains by far the most common form. No patient with a distal pouch and proximal fistula has been seen at this
TABLE 2. Severe Associated Anomalies
TABLE 1. Risk Groups 1977-1985
A B C
35 20 22 77
97 95 59 86
30 6 27 63
100 83.3 63 82.5
common minor types seen. The constellation of defects referred to as the "VATER" or "VACTERL" syndrome'4"15 was seen in 17.5% (11/63) of patients in the most recent group reviewed. The presence of three or more anomalies was used as the criterion for this designation. The most frequent cardiovascular anomalies encountered included patent ductus arteriosus and ventricular or atrial septal defects. One case each of tetralogy of Fallot, common atrio-ventricular canal, total anomalous pulmonary venous return, and single ventricle were seen. The most frequently encountered gastrointestinal mal-
Proxaa', TEF nd dista pouch
At,zia wnlhout TEF
F I0.5% )
7.3 %) I-
C I 85.4 % )
TEF without ttngia
Avesia with proximal disul TEF
Ann. Surg. * October 1986
MANNING AND OTHERS
2.1 % )
H-tyPe (2.8 X ) E42.5%)
FIG. 1. Classification and incidence of anatomic variants of EA and TEF. Both the Haight and Gross schemes are demonstrated.
Cardiovascular Gastrointestinal Neurological Genitourinary Orthopedic Other Total Per cent
44 31 9 4 0 3 91 31.6%
12 8 1 1 0 1 23 27.3%
10 10 2 1 0 1 22 34.0%
formations were imperforate anus, duodenal atresia (with or without annular pancreas), and pyloric stenosis. Operative Management Primary repair of the esophagus was performed in 90.3% (371/411) of patients for the entire series. In the overall series, a staged approach was used in 9.7% (40/ 41 1). In the most current group, three patients underwent a staged approach. In one infant, this was chosen because of the high-risk status ofthe baby. In the other two, it was not possible to perform a primary anastomosis because of the great distance between proximal and distal segments. These two patients later underwent esophageal substitution with colon or stomach. In 14 patients overall (5 in the last 9 years), no repair was attempted because of the severity of additional defects and their associated grave prognoses. These patients were palliated with gastrostomy and sump suction of the proximal pouch to decrease the risk of pulmonary complications. A classic Haight two-layer, telescoping anastomosis was performed in 87.5% (358/41 1) of the entire series. Since the time of the most recent review, however, a change was made to a single-layer technique that appears to have gained preference worldwide. Therefore, while a two-layer repair was performed on 98% of patients prior to 1977, this has been used in only 17% (9/53) of cases since that time (all of these were from the earlier years ofthis update). Cases of H-type fistulas (without EA) were considered as a separate management group. Simple division of the fistulawasaccomplished without complication in all cases either via a thoracotomy or cervical approach (the cervical approach was used exclusively in the recent group). In the group from 1977 to the present, the median length of hospitalization for survivors was 24 days, with a range of 9-174 days.
Survival In our most recent group of 63 patients, the overall survival was 82.5% (52/63). Five of these patients were not operated on because of severe associated anomalies.
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Survival rates by Waterston risk class are given in Table 1. Four deaths occurred in the immediate postoperative period for an operative mortality rate of 6.9%. As seen in Figure 2, this continues the trend of decline in operative mortality we have seen in the past 50 years. Of the operative deaths, two were related to the esophageal anastomosis and two died of complications related to severe underlying congenital anomalies. Late deaths occurred in two patients, both resulting from pulmonary complications that developed at times remote from the operative period.
Complications Accurate data concerning complication rates have been compiled only during the past 30 years. For that reason, our analysis of complications deals only with patients treated from 1956 to the present. During the most recent 30-year period (1956-1985), the incidence of anastomotic leak was 8.5% (19/224). As seen in Table 3, the rate was 17% for those undergoing a single-layer repair and 6.2% for those treated with the classic two-layer telescoping anastomosis. In all cases, the leak remained in the extrapleural space and was almost always asymptomatic. Ninety-five per cent were treated conservatively with spontaneous resolution. One patient had complete disruption of the anastomosis (which was performed under tension despite upper pouch myotomy) and underwent cervical esophagostomy, followed later by colon interposition. Two patients who developed anastomotic leaks died in the postoperative period, both secondary to recurrent aspiration and resistant pneumonia. During the period 1956-1985, the incidence of recurrent fistula was 6.3% (14/224). There was no significant difference between the one- and two-layer anastomosis groups (see Table 3). As mentioned in a previous review of this series,9 the
incidence of anastomotic stricture was difficult to establish precisely because most patients in the earlier years underwent routine postoperative esophageal "sounding." For purposes of comparison, anastomotic strictures have been defined as those requiring more than two dilatations. During the past 30 years, the incidence of stricture has been 19% (43/224). There was a significant difference in stricture rate between those undergoing one- versus twolayer repair (4.3 vs. 23.2%, see Table 3). All strictures responded to esophageal dilatation. As noted by several investigators,16-18 gastroesophageal reflux (GER) is now recognized as a significant postoperative problem in patients undergoing repair of EA. The rate of GER was not documented in prior reports of this series. In our present update, the incidence of symptomatic GER is 37.9% (22/58). Seventy-seven per cent of these 22 patients have undergone fundoplication at a median age of 4 months.
OPERATIVE MORTALITY LATE SURVIVAL
FIG. 2. Trends in operative mortality and late survival over the 50-year period.
In the recent group, less frequently occurring complications include tracheomalacia in 10.3% (6/58), three of whom required aortopexy, and esophageal foreign body impaction in 12.1% (7/58).
Discussion A review of the University of Michigan's experience with repair of esophageal atresia and tracheoesophageal fistula over the past 50 years has led to several interesting observations. Changing referral patterns in our region have led to an increased proportion of neonates with severe associated congenital anomalies or significant prematurity undergoing treatment at this institution. In the most recent decade, we have seen more Waterston group C patients than were seen in the previous decade (41% vs. 26%). In spite of this, operative mortality has continued to decline in recent years. Continued advances in the perioperative intensive care management of high-risk neonates account for much of this decrease in operative mortality. In agreement with Louhimo and Lindhal,'3 we have TABLE 3. Complications Versus Type of Anastomosis: 1956-1985
Type of Repair
One-layer (N = 47) Two-layer (N = 177)
41(23.2%) (p < 0.002)
11(6.2%) (p < 0.03)
MANNING AND OTHERS
Ann. Surg. * October 1986
fashion. We have found this to be necessary only rarely and in very selected infants. Factors that should influence one's decision in favor of a staged approach have been emphasized by others1920 and include: (1) prematurity; (2) established pneumonia; (3) severe associated anomalies; (4) widely separated proximal and distal segments, precluding anastomosis; and (5) deterioration of the patient in the operating room. Most recent studies have found that with current intensive care management, primary repair in the high-risk infant carries no more morbidity or mortality, and probably less, than a staged approach. A recent review by Hicks and Mansfield2' in which all high-risk infants were routinely staged showed an operative mortality of 27% and overall survival of 45%. Comparable figures in our most current group are 18.2% and 63% for Waterston group C risk patients undergoing
MANAGEMENT OF ESOPHAGEAL ATRESIA
AND TRACHEOESOPHAGEAL FISTULA
\>/ I The choice between a retropleural and transpleural op~~~~~~erative has become amatter ofdebate. Thel1964
! 1 _ > /
FIG. 3. The management of EA and TEF. Upper left. Lateral roentgenogram. Small arrows indicate air pouchogram Afrowhead indicates air in distal esophagus. Open arrow indicates air in stomach confirming
fistula. Upper right. Contrast pouchogram (recommended in all cases). Lower left. Sump suction of upper pouch, tube gastrostomy, and upright posture as initial therapeutic interventions. Lower right. Division of fistula and esophageal anastomosis via extrapleural approach.
eliminated pneumonia as a parameter for determining the Waterston risk category. They emphasized that pneumonia was more a complication of management rather than of the underlying anomaly. In fact, in no case did preoperative pneumonia or respiratory distress syndrome affect the classification or outcome in our present group. Our basic scheme for perioperative management (from diagnosis to repair) has not changed significantly since this series was last reviewed and is summarized in Figure 3. We continue to stress the importance of urgent gastrostomy tube placement for decompression (performed in 95% of patients in our recent group). This allows fistula division and repair of the EA to be performed on an elective basis after further preoperative preparation and evaluation for associated anomalies has been accomplished. It also allows for early enteral feeding after the repair. While we have always attempted to perform a primary repair in the patient with congenital EA, some surgeons prefer routinely to approach certain neonates in a staged
survey of the Surgical Section of the American Academy of Pediatrics reported by Holder et al.22 showed that in patients who developed anastomotic leaks, survival was 2 for those who had undergone retropleural dissection better (60% vs. 32%). Even excluding those who developed leaks, they found improved survival with a retropleural approach. Other authors have echoed this preference, emphasizing the greater margin of safety afforded by the retropleural technique, particularly in cases in which a leak develops ( 10-20% of most series).9'20'23 Two recent series, however, note that the choice of exposure did not affect overall mortality.13'21 With a retropleural approach, the leak becomes an easily managed esophagocutaneous fistula, which in most instances closes spontaneously (95% in our series), whereas, if a transpleural approach has been used, a leak becomes a potentially life-threatening empyema, which often requires operative management. The type of anastomosis used for the esophageal repair has undergone a transition over the past 25 years. While Haight used a single-layer technique in his earliest cases, he soon adopted his classic two-layer, telescoping method. In the 1964 survey of Holder et al.,22 nearly half of those polled used a single-layer technique. Their report noted a significantly higher leak rate but a lower incidence of stricture with a single-layer repair. More recently, Hicks and Mansfield2' found a 17 and 18% leak rate with oneand two-layer anastomoses, respectively, while they reported strictures three times more often with a two-layer repair. Louhimo and Lindahl'3 noted a similar incidence of leak with the two types of anastomoses, but no difference in the stricture rate when all patients underwent routine postoperative dilatation. They favor the single-layer technique, however, because it requires less distal mobilization (thus preserving the vascular supply to this segment), a point they feel to be important in decreasing the
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ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA
occurrence of all anastomotic complications. We have also adopted a single-layer technique since our series was last reviewed. The comparison of results with a one- versus two-layer anastomosis in our series over the most recent 30 years is not without bias, as it examines two historically different populations. We found that the rate of anastomotic leaks is significantly higher with the more recently adopted single-layer repair (17% vs. 6.2%). Our previous rate with the two-layer repair, however, was markedly lower than that reported in most other series. Our experience over the past 30 years shows that significantly fewer strictures occurred with the single-layer technique. Part of this may be explained by inherent differences in the anastomoses; however, other factors may be involved. A strong association between gastroesophageal reflux and persistent stricture was pointed out by Pieretti et al.,'8 and the importance of early recognition and management of reflux in these patients was further emphasized by Ashcraft et al. 16 In fact, three of the four patients with significant strictures seen at our institution in the last 9 years had reflux. That gastroesophageal reflux is common in infants following repair of EA is emphasized by the incidence seen in our most current group (37.9%). A long-term follow-up study of patients at this institution showed moderate to severe reflux in over half of those studied and significant dysmotility in nearly all an average of 15 years following operation.'7 Our tendency toward more aggressive management of postoperative gastroesophageal reflux over the past decade is evidenced by the 77% fundoplication rate for symptomatic reflux. This has undoubtedly contributed to our decreased incidence of stricture formation. Important to the changing economic environment in which we now practice is the fact that the median length of hospitalization has continued to decrease. This is, in part, due to more intensive parenteral nutritional support during the perioperative period, in addition to all the other improvements in neonatal care. Interestingly, Haight's first successful patient remained hospitalized for 20 months following operation, apparently much ofthis time to receive enteral nutritional support. In summary, in the last half century, we have seen congenital esophageal atresia transformed from an anomaly with 100% mortality to one with an expected survival exceeding 80%. This has largely come about through advances in both the operative and, more important, the perioperative management of the high-risk neonate. A plateau has probably been reached so that further improvement in the outcome of these infants will primarily depend on improvement in management of the other severe anomalies that may occur simultaneously.
References 1. Haight C, Towsley H. Congenital atresia of the esophagus with tracheoesophageal fistula: extrapleural ligation of fistula and endto-end anastomosis of esophageal segments. Surg Gynecol Obstet 1943; 76:672-688. 2. Durston W. A narrative of a monstrous birth in Plymouth, October 22, 1670; together with the anatomical observations, taken thereupon by William Durston Doctor in Physik, and communicated to Dr. Tim Clerk, Philosophical Tr., London 1670; 5:2096. 3. Gibson T. The Anatomy of Humane Bodies Epitomized, 5th ed. London: printed by TW for Awnsham and John Churchill, 1697. 4. Lanman TH. Congenital atresia of the esophagus: a study of thirtytwo cases. Arch Surg 1940; 41:1060. 5. Ladd WE. The surgical treatment of esophageal atresia and tracheoesophageal fistulas. N Engl J Med 1944; 230:625. 6. Leven NL. Congenital atresia of the esophagus with tracheoesophageal fistula: report of successful extrapleural ligation of fistulous communication and cervical esophagotomy. J Thor Surg 1941; 10:648. 7. Haight C. Some observations on esophageal atresias and tracheoesophageal fistulas of congenital origin. J Thorac Surg 1957; 34: 141. 8. Haight C. Congenital Esophageal Atresia and Tracheoesophageal Fistula. In Mustard WT, Ravitch MM, Snyder WH, Welch KJ, Benson CD, eds. Pediatric Surgery, 2d ed. Chicago: Year Book Medical Publishers, 1969; 357-382. 9. Strodel WE, Coran AG, Kirsh MM, et al. Esophageal atresia: A 41 year experience. Arch Surg 1979; 114:523. 10. Gross RE. The Surgery of Infancy and Childhood. Philadelphia: WB Saunders Co, 1953. 11. Waterston DJ, Bonham-Carter RE, Aberdeen E. Oesophageal atresia: tracheo-oesophageal fistula: a study of survival in 218 infants. Lancet 1962; 1:819. 12. Waterston DJ, Bonham-Carter RE, Aberdeen E. Congenital tracheooesophageal fistula in association with oesophageal atresia. Lancet 1963; 2:55. 13. Louhimo I, Lindhal H. Esophageal atresia: primary results of 500 consecutively treated patients. J Pediatr Surg 1983; 18:217. 14. Barry JE, Auldist AW. The VATER association: one end of a spectrum of anomalies. Am J Dis Child 1974; 128:769. 15. Heyman MB, Berquist WE, Fonkalsrud EW, et al. Esophageal muscular ring and the VACTERL association: a case report. Pediatrics 1981; 67:683. 16. Ashcraft KW, Goodwin C, Amoury RA, Holder TM. Early recognition and aggressive treatment of gastroesophageal reflux following repair of esophageal atresia. J Pediatr Surg 1977; 12:317. 17. Orringer MB, Kirsh MM, Sloan H. Long-term esophageal function following repair of esophageal atresia. Ann Surg 1977; 186:436. 18. Pieretti R, Shandling B, Stephens CA. Resistant esophageal stenosis associated with reflux after repair of esophageal atresia: a therapeutic approach. J Pediatr Surg 1974; 9:355. 19. Abrahamson J, Shandling B. Esophageal atresia in the underweight baby: a challenge. J Pediatr Surg 1972; 7:608. 20. Myers NA, Aberdeen E. Congenital Esophageal Atresia and Tracheoesophageal Fistula. In Ravitch MM, Welch KJ, Benson CD, Aberdeen E, Randolph JG, eds. Pediatric Surgery, 3d ed. Chicago: Year Book Medical Publishers, 1979. 21. Hicks LM, Mansfield PB. Esophageal atresia and tracheoesophageal fistula: review of thirteen years' experience. J Thorac Cardiovasc Surg 1981; 81:358. 22. Holder TM, Cloud DT, Lewis JE, Pilling GP. Esophageal atresia and tracheoesophageal fistula: a survey of its members by the surgical section of the American Academy of Pediatrics. Pediatrics 1964; 34:542. 23. Holder TM, Ashcraft KW. Developments in the care of patients with esophageal atresia and tracheoesophageal fistula. Surg Clin North Am 1981; 61:1051.