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Analysis of Morbidity and Mortality in 227 Cases of Esophageal Atresia and/or Tracheoesophageal Fistula Over Two Decades

Analysis of Morbidity and Mortality in 227 Cases of Esophageal Atresia and/or Tracheoesophageal... Abstract Objective: This report analyzes the morbidity and mortality in 227 infants (127 boys and 100 girls) with variants of esophageal atresia and/or tracheoesophageal fistula who were treated from 1971 to 1993. Design: Data were collected retrospectively from hospital and office records. Mean follow-up was 76 months, ranging from 1 month to 22 years. Setting: Patients were treated at a tertiary care children's hospital. Results: The mean birth weight was 2557 g (range, 1100 to 4460 g), and the mean gestational age was 38 weeks (range, 28 to 42 weeks). Classification included 29 cases of type A esophageal atresia (13%); two cases of type B (1%), 178 cases of type C (78%), five cases of type D (2%), and 13 cases of type E (6%). Associated anomalies occurred in 146 infants (64%), including cardiac defects in 86 (38%), skeletal defects in 44 (19%), neurological defects in 34 (15%), renal defects in 35 (15%), anorectal defects in 18 (8%), and other abnormalities in 30 (13%). A single-layer anastomosis was performed in 81%, and a two-layer repair, in 17%. Esophagomyotomy was necessary in 9% of the patients. Anastomotic complications included leakage (16%), symptomatic stricture (35%), and recurrent tracheoesophageal fistula (3%). Gastroesophageal reflux was present in 127 cases (58%), with 56 (44%) requiring an antireflux procedure. Tracheomalacia occurred in 32 cases (15%), and 13 required operative treatment. Postoperative esophageal dysmotility was documented in 56 children (30%). The overall survival rate was 95%. The cause of death in 12 patients included severe cardiac anomalies (n=3), fatal sleep apnea (n=1), renal failure (n=1), trisomy 18 (n=2), accidental decannulation of tracheostomy (n=1), pulmonary failure (n=1), and unknown causes (n=3). Conclusions: Early diagnosis, improved surgical technique, neonatal anesthesia, sophisticated ventilatory support, advanced intensive care management, early treatment of associated anomalies, responsiveness of anastomotic strictures to dilatation, and aggressive treatment of gastroesophageal reflux have influenced survival positively. Improved survival rates were noted irrespective of the traditional Waterston criteria, which now seem outdated. With few exceptions, most infants with esophageal atresia and/or tracheoesophageal fistula should survive in the current era.(Arch Surg. 1995;130:502-508) References 1. Haight C, Towsley HA. Congenital atresia of the esophagus with tracheoesophageal fistula: extrapleural ligation of fistula and end-to-end anastomosis of esophageal segments . Surg Gynecol Obstet . 1943;76:672-688. 2. Abrahamson J, Shandling B. Esophageal atresia in the underweight baby: a challenge . J Pediatr Surg . 1972;7:608-613.Crossref 3. Rickham PP. Infants with esophageal atresia weighing under three pounds . J Pediatr Surg . 1981;16:595-598.Crossref 4. Spitz L, Kiely E, Brereton RJ. Esophageal atresia: five-year experience with 148 cases . J Pediatr Surg . 1987;22:103-108.Crossref 5. Waterston DJ, Bonham-Carter RE, Aberdeen E. Oesophageal atresia: tracheooesophageal fistula: a study of survival in 218 infants . Lancet . 1962;1:819-822.Crossref 6. Randolph JG. Esophageal atresia and congenital stenosis . In: Welch KJ, Randolph JG, Ravitch MM, eds. Pediatric Surgery . 4th ed. St Louis, Mo: Mosby—Year Book; 1986:682-697. 7. Cudmore RE. Oesophageal atresia and tracheo-oesophageal fistula . In: Rickham PP, Lister J, Irving IM, eds. Neonatal Surgery . 2nd ed. London, England: Butterworths; 1978:189-208. 8. Holder TM, Cloud DT, Lewis JE Jr, Pilling GP IV. Esophageal atresia and tracheoesophageal fistula: a survey of its members by the Surgical Section of the American Academy of Pediatrics . Pediatrics . 1964;34:542-549. 9. Hagberg S, Rubenson A, Sillen U, Werkmaster K. Management of long-gap esophagus: experience with end-to-end anastomosis under maximal tension . Prog Pediatr Surg . 1986;19:88-92. 10. O'Neill JA, Holcomb GW, Neblett WW. Recent experience with esophageal atresia . Ann Surg . 1982;195:739-745.Crossref 11. Sillen U, Hagberg S, Rubenson A, Werkmaster K. Management of esophageal atresia: review of 16 years' experience . J Pediatr Surg . 1988;23:805-809.Crossref 12. Howell CG, Davis JB Jr, Parrish RA. Primary repair of esophageal atresia: how long a gap? J Pediatr Surg . 1987;22:42-43.Crossref 13. Louhimo I, Lindahl H. Esophageal atresia: primary results of 500 consecutively treated patients . J Pediatr Surg . 1983;18:217-229.Crossref 14. Holder TM, Ashcraft KW. Esophagus . In: Welch KJ, ed. Complications of Pediatric Surgery: Prevention and Management . Philadelphia, Pa: WB Saunders Co; 1982:199-207. 15. Touloukian RJ. Long-term results following repair of esophageal atresia by end-to-side anastomosis and ligation of the tracheoesophageal fistula . J Pediatr Surg . 1981;16:983-988.Crossref 16. Holder TM, Ashcraft KW. Developments in the care of patients with esophageal atresia and tracheoesophageal fistula . Surg Clin North Am . 1981;61:1051-1061. 17. Lundertse-Verloop K, Tibboel D, Hazebrock FWJ, Molenaar JC. Postoperative morbidity in patients with esophageal atresia . Pediatr Surg Int . 1987;2:2-5. 18. Holder TM, Ashcraft KW, Sharp RJ, Amoury RA. Care of infants with esophageal atresia, tracheoesophageal fistula and associated anomalies . J Thorac Cardiovasc Surg . 1987;94:828-835. 19. Holder TM. Esophageal atresia and tracheoesophageal fistula . In: Ashcraft KW, Holder TM, eds. Pediatric Esophageal Surgery . Philadelphia, Pa: Grune & Stratton; 1987:29-52. 20. Hicks LM, Manfield PB. Esophageal atresia and tracheoesophageal fistula: review of thirteen years' experience . J Thorac Cardiovasc Surg . 1981;81:358-363. 21. Jolly SG, Johnson DG, Roberts CC, et al. Patterns of gastroesophageal reflux in children following repair of esophageal atresia and distal tracheoesophageal fistula . J Pediatr Surg . 1980;15:857-862.Crossref 22. Aprigliano F. Esophageal stenosis in children . Ann Otol . 1980;89:391-395. 23. Manning PB, Morgan RA, Coran AG, et al. Fifty years experience with esophageal atresia and tracheoesophageal fistula . Ann Surg . 1986;204:446-453.Crossref 24. Kafrouni G, Baick CH, Wooley MM. Recurrent tracheoesophageal fistula: a diagnostic problem . Surgery . 1970;68:889-894. 25. Ein SH, Stringer DA, Stephens CA, Shandling B, Simpson J, Filler RM. Recurrent tracheoesophageal fistulas seventeen-year review . J Pediatr Surg . 1983; 18:436-441.Crossref 26. Wheatley MJ, Coran AG. Pericardial flap interposition for the definitive management of recurrent tracheoesophageal fistula . J Pediatr Surg . 1992;27:1122-1126.Crossref 27. Fufezan V, Veleanu C, Duica C, Scoliuc M. L'azygoplastie, une methode chirurgicale complementaire daus le traitement de l'atresie congenitale de l'oesophage . Ann Chir Infant . 1972;13:99-105. 28. Kosloske AM. Azygous flap technique for reinforcement of esophageal closure . J Pediatr Surg . 1990;25:793-794.Crossref 29. Mitchell IM, Goh DW, Roberts KD, Abrams LD. Colon interposition in children . Br J Surg . 1989;76:681-686.Crossref 30. Waterston D. Colonic replacement of esophagus (intrathoracic) . Surg Clin North Am . 1964;41:1441-1447. 31. Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia . J Pediatr Surg . 1994;29:566-568.Crossref 32. Louhimo I, Pasila M, Visakorpi JK. Late gastrointestinal complications in patients with colonic replacement of the esophagus . J Pediatr Surg . 1969;4: 663-673.Crossref 33. Schiller M, Frye TR, Boles ET Jr. Evaluation of colonic replacement of the esophagus in children . J Pediatr Surg . 1971;6:753-760.Crossref 34. Gomez MV. Esophageal replacement in patients under 3 months of age . J Pediatr Surg . 1994;29:487-491.Crossref 35. Poenaru D, Laberge J-M, Neilson IR, Guttman FM. A new prognostic classification for esophageal atresia . Surgery . 1993;113:426-432. 36. Randolph JG, Newman KD, Anderson KD. Current results in repair of esophageal atresia with tracheoesophageal fistula using physiologic status as a guide to therapy . Ann Surg . 1989;209:526-531.Crossref 37. Ein SH, Shandling B, Wesson D, Filler RM. Esophageal atresia with distal tracheoesophageal fistula: associated anomalies and prognosis in the 1980s . J Pediatr Surg . 1989;24:1055-1059.Crossref 38. Beasley SW, Auldist AW, Myers NA. Current surgical management of oesophageal atresia and/or tracheo-oesophageal fistula . Aust N Z J Surg . 1989;59: 707-712.Crossref 39. Shaul DB, Schwartz MZ, Marr CC, et al. Primary repair without routine gastrostomy is the treatment of choice for neonates with esophageal atresia and tracheoesophageal fistula . Arch Surg . 1989;124:1188-1191.Crossref 40. Spitz L, Kiely EM, Morecroft JA, Drake DP. Oesophageal atresia: at-risk groups for the 1990s . J Pediatr Surg . 1994;29:723-725.Crossref 41. Holder TM. Esophageal atresia and tracheoesophageal malformations . In: Ashcraft KW, Holder TM, eds. Pediatric Surgery . 2nd ed. Philadelphia, Pa: WB Saunders Co; 1993:249-269. 42. Parker AF, Christie DL, Cahill JL. Incidence and significance of gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula and the need for anti-reflux following repair of esophageal atresia . J Pediatr Surg . 1977;12:317-321.Crossref 43. Filler RM, Rossello PJ, Lebowitz RL. Life-threatening anoxic spells caused by tracheal compression after repair of esophageal atresia: correction by surgery . J Pediatr Surg . 1976;11:739-748.Crossref 44. Slany E, Holzki J, Holschneider AM, Gharib M, Hugel W, Mennicken U. Flaccid trachea in tracheo-oesophageal malformations . Z Kinderchir . 1990;45:78-85. 45. Rideout DT, Hayashi AH, Gillis DA, Giacomantonio JM, Lau HYC. The absence of clinically significant tracheomalacia in patients having esophageal atresia without tracheoesophageal fistula . J Pediatr Surg . 1991;26:1303-1305.Crossref 46. Blair GK, Cohen R, Filler RM. Treatment of tracheomalacia: eight years' experience . J Pediatr Surg . 1986;21:781-785.Crossref 47. Wailoo MP, Emery JL. The trachea in children with tracheoesophageal fistula . Histopathology . 1979;3:329-338.Crossref 48. Filler RM, Messineo A, Vinograd I. Severe tracheomalacia associated with esophageal atresia: results of surgical treatment . J Pediatr Surg . 1992;27:1136-1141.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

Analysis of Morbidity and Mortality in 227 Cases of Esophageal Atresia and/or Tracheoesophageal Fistula Over Two Decades

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Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1995.01430050052008
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Abstract

Abstract Objective: This report analyzes the morbidity and mortality in 227 infants (127 boys and 100 girls) with variants of esophageal atresia and/or tracheoesophageal fistula who were treated from 1971 to 1993. Design: Data were collected retrospectively from hospital and office records. Mean follow-up was 76 months, ranging from 1 month to 22 years. Setting: Patients were treated at a tertiary care children's hospital. Results: The mean birth weight was 2557 g (range, 1100 to 4460 g), and the mean gestational age was 38 weeks (range, 28 to 42 weeks). Classification included 29 cases of type A esophageal atresia (13%); two cases of type B (1%), 178 cases of type C (78%), five cases of type D (2%), and 13 cases of type E (6%). Associated anomalies occurred in 146 infants (64%), including cardiac defects in 86 (38%), skeletal defects in 44 (19%), neurological defects in 34 (15%), renal defects in 35 (15%), anorectal defects in 18 (8%), and other abnormalities in 30 (13%). A single-layer anastomosis was performed in 81%, and a two-layer repair, in 17%. Esophagomyotomy was necessary in 9% of the patients. Anastomotic complications included leakage (16%), symptomatic stricture (35%), and recurrent tracheoesophageal fistula (3%). Gastroesophageal reflux was present in 127 cases (58%), with 56 (44%) requiring an antireflux procedure. Tracheomalacia occurred in 32 cases (15%), and 13 required operative treatment. Postoperative esophageal dysmotility was documented in 56 children (30%). The overall survival rate was 95%. The cause of death in 12 patients included severe cardiac anomalies (n=3), fatal sleep apnea (n=1), renal failure (n=1), trisomy 18 (n=2), accidental decannulation of tracheostomy (n=1), pulmonary failure (n=1), and unknown causes (n=3). Conclusions: Early diagnosis, improved surgical technique, neonatal anesthesia, sophisticated ventilatory support, advanced intensive care management, early treatment of associated anomalies, responsiveness of anastomotic strictures to dilatation, and aggressive treatment of gastroesophageal reflux have influenced survival positively. Improved survival rates were noted irrespective of the traditional Waterston criteria, which now seem outdated. With few exceptions, most infants with esophageal atresia and/or tracheoesophageal fistula should survive in the current era.(Arch Surg. 1995;130:502-508) References 1. Haight C, Towsley HA. Congenital atresia of the esophagus with tracheoesophageal fistula: extrapleural ligation of fistula and end-to-end anastomosis of esophageal segments . Surg Gynecol Obstet . 1943;76:672-688. 2. Abrahamson J, Shandling B. Esophageal atresia in the underweight baby: a challenge . J Pediatr Surg . 1972;7:608-613.Crossref 3. Rickham PP. Infants with esophageal atresia weighing under three pounds . J Pediatr Surg . 1981;16:595-598.Crossref 4. Spitz L, Kiely E, Brereton RJ. Esophageal atresia: five-year experience with 148 cases . J Pediatr Surg . 1987;22:103-108.Crossref 5. Waterston DJ, Bonham-Carter RE, Aberdeen E. Oesophageal atresia: tracheooesophageal fistula: a study of survival in 218 infants . Lancet . 1962;1:819-822.Crossref 6. Randolph JG. Esophageal atresia and congenital stenosis . In: Welch KJ, Randolph JG, Ravitch MM, eds. Pediatric Surgery . 4th ed. St Louis, Mo: Mosby—Year Book; 1986:682-697. 7. Cudmore RE. Oesophageal atresia and tracheo-oesophageal fistula . In: Rickham PP, Lister J, Irving IM, eds. Neonatal Surgery . 2nd ed. London, England: Butterworths; 1978:189-208. 8. Holder TM, Cloud DT, Lewis JE Jr, Pilling GP IV. Esophageal atresia and tracheoesophageal fistula: a survey of its members by the Surgical Section of the American Academy of Pediatrics . Pediatrics . 1964;34:542-549. 9. Hagberg S, Rubenson A, Sillen U, Werkmaster K. Management of long-gap esophagus: experience with end-to-end anastomosis under maximal tension . Prog Pediatr Surg . 1986;19:88-92. 10. O'Neill JA, Holcomb GW, Neblett WW. Recent experience with esophageal atresia . Ann Surg . 1982;195:739-745.Crossref 11. Sillen U, Hagberg S, Rubenson A, Werkmaster K. Management of esophageal atresia: review of 16 years' experience . J Pediatr Surg . 1988;23:805-809.Crossref 12. Howell CG, Davis JB Jr, Parrish RA. Primary repair of esophageal atresia: how long a gap? J Pediatr Surg . 1987;22:42-43.Crossref 13. Louhimo I, Lindahl H. Esophageal atresia: primary results of 500 consecutively treated patients . J Pediatr Surg . 1983;18:217-229.Crossref 14. Holder TM, Ashcraft KW. Esophagus . In: Welch KJ, ed. Complications of Pediatric Surgery: Prevention and Management . Philadelphia, Pa: WB Saunders Co; 1982:199-207. 15. Touloukian RJ. Long-term results following repair of esophageal atresia by end-to-side anastomosis and ligation of the tracheoesophageal fistula . J Pediatr Surg . 1981;16:983-988.Crossref 16. Holder TM, Ashcraft KW. Developments in the care of patients with esophageal atresia and tracheoesophageal fistula . Surg Clin North Am . 1981;61:1051-1061. 17. Lundertse-Verloop K, Tibboel D, Hazebrock FWJ, Molenaar JC. Postoperative morbidity in patients with esophageal atresia . Pediatr Surg Int . 1987;2:2-5. 18. Holder TM, Ashcraft KW, Sharp RJ, Amoury RA. Care of infants with esophageal atresia, tracheoesophageal fistula and associated anomalies . J Thorac Cardiovasc Surg . 1987;94:828-835. 19. Holder TM. Esophageal atresia and tracheoesophageal fistula . In: Ashcraft KW, Holder TM, eds. Pediatric Esophageal Surgery . Philadelphia, Pa: Grune & Stratton; 1987:29-52. 20. Hicks LM, Manfield PB. Esophageal atresia and tracheoesophageal fistula: review of thirteen years' experience . J Thorac Cardiovasc Surg . 1981;81:358-363. 21. Jolly SG, Johnson DG, Roberts CC, et al. Patterns of gastroesophageal reflux in children following repair of esophageal atresia and distal tracheoesophageal fistula . J Pediatr Surg . 1980;15:857-862.Crossref 22. Aprigliano F. Esophageal stenosis in children . Ann Otol . 1980;89:391-395. 23. Manning PB, Morgan RA, Coran AG, et al. Fifty years experience with esophageal atresia and tracheoesophageal fistula . Ann Surg . 1986;204:446-453.Crossref 24. Kafrouni G, Baick CH, Wooley MM. Recurrent tracheoesophageal fistula: a diagnostic problem . Surgery . 1970;68:889-894. 25. Ein SH, Stringer DA, Stephens CA, Shandling B, Simpson J, Filler RM. Recurrent tracheoesophageal fistulas seventeen-year review . J Pediatr Surg . 1983; 18:436-441.Crossref 26. Wheatley MJ, Coran AG. Pericardial flap interposition for the definitive management of recurrent tracheoesophageal fistula . J Pediatr Surg . 1992;27:1122-1126.Crossref 27. Fufezan V, Veleanu C, Duica C, Scoliuc M. L'azygoplastie, une methode chirurgicale complementaire daus le traitement de l'atresie congenitale de l'oesophage . Ann Chir Infant . 1972;13:99-105. 28. Kosloske AM. Azygous flap technique for reinforcement of esophageal closure . J Pediatr Surg . 1990;25:793-794.Crossref 29. Mitchell IM, Goh DW, Roberts KD, Abrams LD. Colon interposition in children . Br J Surg . 1989;76:681-686.Crossref 30. Waterston D. Colonic replacement of esophagus (intrathoracic) . Surg Clin North Am . 1964;41:1441-1447. 31. Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia . J Pediatr Surg . 1994;29:566-568.Crossref 32. Louhimo I, Pasila M, Visakorpi JK. Late gastrointestinal complications in patients with colonic replacement of the esophagus . J Pediatr Surg . 1969;4: 663-673.Crossref 33. Schiller M, Frye TR, Boles ET Jr. Evaluation of colonic replacement of the esophagus in children . J Pediatr Surg . 1971;6:753-760.Crossref 34. Gomez MV. Esophageal replacement in patients under 3 months of age . J Pediatr Surg . 1994;29:487-491.Crossref 35. Poenaru D, Laberge J-M, Neilson IR, Guttman FM. A new prognostic classification for esophageal atresia . Surgery . 1993;113:426-432. 36. Randolph JG, Newman KD, Anderson KD. Current results in repair of esophageal atresia with tracheoesophageal fistula using physiologic status as a guide to therapy . Ann Surg . 1989;209:526-531.Crossref 37. Ein SH, Shandling B, Wesson D, Filler RM. Esophageal atresia with distal tracheoesophageal fistula: associated anomalies and prognosis in the 1980s . J Pediatr Surg . 1989;24:1055-1059.Crossref 38. Beasley SW, Auldist AW, Myers NA. Current surgical management of oesophageal atresia and/or tracheo-oesophageal fistula . Aust N Z J Surg . 1989;59: 707-712.Crossref 39. Shaul DB, Schwartz MZ, Marr CC, et al. Primary repair without routine gastrostomy is the treatment of choice for neonates with esophageal atresia and tracheoesophageal fistula . Arch Surg . 1989;124:1188-1191.Crossref 40. Spitz L, Kiely EM, Morecroft JA, Drake DP. Oesophageal atresia: at-risk groups for the 1990s . J Pediatr Surg . 1994;29:723-725.Crossref 41. Holder TM. Esophageal atresia and tracheoesophageal malformations . In: Ashcraft KW, Holder TM, eds. Pediatric Surgery . 2nd ed. Philadelphia, Pa: WB Saunders Co; 1993:249-269. 42. Parker AF, Christie DL, Cahill JL. Incidence and significance of gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula and the need for anti-reflux following repair of esophageal atresia . J Pediatr Surg . 1977;12:317-321.Crossref 43. Filler RM, Rossello PJ, Lebowitz RL. Life-threatening anoxic spells caused by tracheal compression after repair of esophageal atresia: correction by surgery . J Pediatr Surg . 1976;11:739-748.Crossref 44. Slany E, Holzki J, Holschneider AM, Gharib M, Hugel W, Mennicken U. Flaccid trachea in tracheo-oesophageal malformations . Z Kinderchir . 1990;45:78-85. 45. Rideout DT, Hayashi AH, Gillis DA, Giacomantonio JM, Lau HYC. The absence of clinically significant tracheomalacia in patients having esophageal atresia without tracheoesophageal fistula . J Pediatr Surg . 1991;26:1303-1305.Crossref 46. Blair GK, Cohen R, Filler RM. Treatment of tracheomalacia: eight years' experience . J Pediatr Surg . 1986;21:781-785.Crossref 47. Wailoo MP, Emery JL. The trachea in children with tracheoesophageal fistula . Histopathology . 1979;3:329-338.Crossref 48. Filler RM, Messineo A, Vinograd I. Severe tracheomalacia associated with esophageal atresia: results of surgical treatment . J Pediatr Surg . 1992;27:1136-1141.Crossref

Journal

Archives of SurgeryAmerican Medical Association

Published: May 1, 1995

References