Abstract
Interruption in the normal development of the esophagus and trachea may result in a wide variety of esophageal malformations. This chapter will review the anatomic and clinical spectrum of this disease including the diagnosis and evaluation.
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References
Mathieu A, Goldsmith HE. Congenital atresia of the esophagus with tracheo-esophageal fistula. Am J Surg. 1933;22.
Durston W. A narrative of a monstrous birth in Plymouth. Phil Trans R Soc. 1670.
Gibson T. The anatomy of humane bodies epitomized. 5th ed. London: Awnsham and Churchill; 1697.
Hill TP. Bost Med Surg J. 1840;21. Brewer LA 3rd. History of surgery of the esophagus. Am J Surg. 1980;139(6):730–43.
Hirschsprung H. Hospitalstidende. 1895. Muensterer OJ, Berdon WE. From Vogt to Haight and Holt to now: the history of esophageal atresia over the last century. Pediatr Radiol. 2015;45(8):1230–5.
Mackenzie M. Malformation of the esophagus. Arch Laryngol. 1880;1.
Plass ED. Congenital atresia of the esophagus with tracheoesophageal fistual: associated with fused kidney. A case report and a survey of the literature on congenital anomalies of the esophagus. Johns Hopkins Hosp Rep. 1919;18.
Vogt EC. Congenital esophageal atresia. Am J Roentgenol. 1929;22:463–5.
Ladd W. The surgical treatment of esophageal atresia and tracheoesophageal fistulas. N Engl J Med. 1944;230:625–37.
Gross RE, Piotti E. The surgery of infancy and childhood: its principles and techniques. Philadelphia: Saunders; 1953.
Holder TM, Cloud DT, Lewis Jr 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;34:542–9.
Mustard WT, Simpson Jr JS, Stephens CA. Congenital atresia of the esophagus with tracheo-esophageal fistula. Surg Clin North Am. 1956;36:1465–78.
Swenson O, Lipman R, Fisher JH, Deluca FG. Repair and complications of esophageal atresia and tracheoesophageal fistula. N Engl J Med. 1962;267:960–3.
Kluth D. Atlas of esophageal atresia. J Pediatr Surg. 1976;11(6):901–19.
Myers NA. Unusual varieties of esophageal atresia. Pediatr Surg Int. 1987;2:195–7.
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.
McKinnon LJ, Kosloske AM. Prediction and prevention of anastomotic complications of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1990;25(7):778–81. doi:S0022346890002494 [pii].
Boyle Jr EM, Irwin ED, Foker JE. Primary repair of ultra-long-gap esophageal atresia: results without a lengthening procedure. Ann Thorac Surg. 1994;57(3):576–9. doi:0003-4975(94)90548-7 [pii].
Waterston DJ, Carter RE, Aberdeen E. Oesophageal atresia: tracheo-oesophageal fistula. A study of survival in 218 infants. Lancet. 1962;1(7234):819–22.
Poenaru D, Laberge JM, Neilson IR, Guttman FM. A new prognostic classification for esophageal atresia. Surgery. 1993;113(4):426–32.
Rokitansky AM, Kolankaya VA, Seidl S, Mayr J, Bichler B, Schreiner W, Engels M, Horcher E, Lischka A, Menardi G, et al. Recent evaluation of prognostic risk factors in esophageal atresia – a multicenter review of 223 cases. Eur J Pediatr Surg. 1993;3(4):196–201.
Randolph JG, Tunell WP, Lilly JR. Gastric division in the critically ill infant with esophageal atresia and tracheoesophageal fistula. Surgery. 1968;63(3):496–502.
Randolph JG, Tunell WP, Lilly JR, Altman RP. Gastric division: a surgical adjunct in selected problems with esophageal anomalies. J Pediatr Surg. 1971;6(5):657–63. doi:0022-3468(71)90393-9 [pii].
Koop CE, Hamilton JP. Atresia of the esophagus: increased survival with staged procedures in the poor-risk infant. Ann Surg. 1965;162(3):389–401.
Richter HM. Congenital atresia of the oesophagus: an operation designed for its cure. Surg Gynecol Obstet. 1913;27.
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(5):526–30; discussion 530–521.
Spitz L. Esophageal atresia and tracheoesophageal fistula in children. Curr Opin Pediatr. 1993;5(3):347–52.
Spitz L. Esophageal atresia: past, present, and future. J Pediatr Surg. 1996;31(1):19–25. doi:S0022-3468(96)90313-9 [pii].
Brown AK, Tam PK. Measurement of gap length in esophageal atresia: a simple predictor of outcome. J Am Coll Surg. 1996;182(1):41–5.
Haight C. Some observations on esophageal atresias and tracheoesophageal fistulas of congenital origin. J Thorac Surg. 1957;34(2):141–72.
Myers NA. Oesophageal atresia: the epitome of modern surgery. Ann R Coll Surg Engl. 1974;54(6):277–87.
Sulamaa M, Gripenberg L, Ahvenainen EK. Prognosis and treatment of congenita; tresia of the esophagus. Acta Chir Scand. 1951;102(2):141–57.
Shapiro RN, Eddy W, Fitzgibbon J, O’Brien G. The incidence of congenital anomalies discovered in the neonatal period. Am J Surg. 1958;96(3):396–400. doi:0002-9610(58)90932-2 [pii].
Ingalls TH, Prindle RA. Esophageal atresia with tracheoesophageal fistula; epidemiologic and teratologic implications. N Engl J Med. 1949;240(25):987–95.
Harris J, Kallen B, Robert E. Descriptive epidemiology of alimentary tract atresia. Teratology. 1995;52(1):15–29. doi:10.1002/tera.1420520104.
Whitman-Elia GF, Plouffe Jr L, Craft K, Khan I. Esophageal atresia in an in vitro fertilization pregnancy. South Med J. 1997;90(1):86–8.
Lanman TH. Congenital atresia of the esophagus: a study of thirty-two cases. Arch Surg. 1940;41:1060–83.
Blank RH, Prillaman Jr PE, Minor GR. Congenital esophageal atresia with tracheoesophageal fistula occurring in identical twins. J Thorac Cardiovasc Surg. 1967;53(2):192–6.
Hansmann PF, Close AS, Williams LP. Occurrence of tracheoesophageal fistula in three consecutive siblings. Surg Gynecol Obstet. 1957;41.
Engel PM, Vos LJ, de Vries JA, Kuijjer PJ. Esophageal atresia with tracheoesophageal fistula in mother and child. J Pediatr Surg. 1970;5(5):564–5.
Kiesewetter WB, Bower RJ. Tracheoesophageal fistula in parent and offspring: a rare occurrence. Am J Dis Child. 1980;134(9):896.
Dennis NR, Nicholas JC, Kuvar I. Esophageal atresia: three cases in two generations. Dis Arch Child. 1983;48.
German JC, Mahour GH, Woolley MM. The twin with esophageal atresia. J Pediatr Surg. 1979;14(4):432–5. doi:S0022346879000713 [pii].
David TJ, O’Callaghan SE. Twinning and oesophageal atresia. Arch Dis Child. 1974;49(8):660–2.
Pletcher BA, Friedes JS, Breg WR, Touloukian RJ. Familial occurrence of esophageal atresia with and without tracheoesophageal fistula: report of two unusual kindreds. Am J Med Genet. 1991;39(4):380–4. doi:10.1002/ajmg.1320390403.
Forrester RM, Cohen SJ. Esophageal atresia associated with an anorectal anomaly and probably laryngeal fissure in three siblings. J Pediatr Surg. 1970;5(6):674–5.
Lipson AH, Berry AB. Oesophageal atresia in father and daughter. Aust Paediatr J. 1984;20(4):329.
Warren J, Evans K, Carter CO. Offspring of patients with tracheo-oesophageal fistula. J Med Genet. 1979;16(5):338–40.
Chen H, Goei GS, Hertzler JH. Family studies on congenital esophageal atresia with or without tracheoesophageal fistula. Birth Defects Orig Artic Ser. 1979;15(5C):117–44.
Nora AH, Nora JJ. A syndrome of multiple congenital anomalies associated with teratogenic exposure. Arch Environ Health. 1975;30(1):17–21.
Szendrey T, Danyi G, Czeizel A. Etiological study on isolated esophageal atresia. Hum Genet. 1985;70(1):51–8.
Ozimek CD, Grimson RC, Aylsworth AS. An epidemiologic study of tracheoesophageal fistula and esophageal atresia in North Carolina. Teratology. 1982;25(1):53–9. doi:10.1002/tera.1420250108.
Imperatori CJ. Congenital tracheo-esophageal fistula without atresia of the esophagus. Report of a case with plastic closure and cure. Arch Otolaryngol Head Neck Surg. 1939;30:352–9.
Haight C. Congenital tracheoesophageal fistula without esophageal atresia. J Thorac Surg. 1948;17(5):600–12.
Le Roux BT, Williams MA. Congenital oesophagobronchial fistula with presentation in adult life. Br J Surg. 1969;55:306–8.
German JC, Mahour GH, Woolley MM. Esophageal atresia and associated anomalies. J Pediatr Surg. 1976;11(3):299–306. doi:S0022346876000298 [pii].
Iuchtman M, Brereton R, Spitz L, Kiely EM, Drake D. Morbidity and mortality in 46 patients with the VACTERL association. Isr J Med Sci. 1992;28(5):281–4.
Quan L, Smith DW. The VATER association. Vertebral defects, anal atresia, T-E fistula with esophageal atresia. Radial and renal dysplasia: a spectrum of associated defects. J Pediatr. 1973;82(1):104–7.
Weigel W, Kaufmann HJ. The frequency and types of other congenital anomalies in association with tracheoesophageal malformations. Radiologic studies of 83 such infants. Clin Pediatr (Phila). 1976;15(9):819–20, 827–819, 832–814.
Weaver DD, Mapstone CL, Yu PL. The VATER association. Analysis of 46 patients. Am J Dis Child. 1986;140(3):225–9.
Touloukian RJ, Keller MS. High proximal pouch esophageal atresia with vertebral, rib, and sternal anomalies: an additional component to the VATER association. J Pediatr Surg. 1988;23(1 Pt 2):76–9. doi:S002234688800017X [pii].
Temtamy SA, Miller JD. Extending the scope of the VATER association: definition of the VATER syndrome. J Pediatr. 1974;85(3):345–9.
Weber TR, Smith W, Grosfeld JL. Surgical experience in infants with the VATER association. J Pediatr Surg. 1980;15(6):849–54. doi:S0022346880001534 [pii].
Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Oesophageal atresia and associated anomalies. Arch Dis Child. 1989;64(3):364–8.
Landing BH. Syndromes of congenital heart disease with tracheobronchial anomalies. Edward B. D. Neuhauser Lecture, 1974. Am J Roentgenol Radium Ther Nucl Med. 1975;123(4):679–86.
Valente A, Brereton RJ. Oesophageal atresia and the CHARGE association. Pediatr Surg Int. 1987;2:93–4.
Kutiyanawala M, Wyse RK, Brereton RJ, Spitz L, Kiely EM, Drake D, Blake K. CHARGE and esophageal atresia. J Pediatr Surg. 1992;27(5):558–60. doi:0022-3468(92)90445-D [pii].
Spitz L, Kiely EM, Morecroft JA, Drake DP. Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg. 1994;29(6):723–5. doi:0022-3468(94)90354-9 [pii].
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(10):1055–9. doi:S0022346889000485 [pii].
Hennekam RC, Huber J, Variend D. Bartsocas-Papas syndrome with internal anomalies: evidence for a more generalized epithelial defect or new syndrome? Am J Med Genet. 1994;53(2):102–7. doi:10.1002/ajmg.1320530203.
Parent P, Bensaid M, Le Guern H, Colin A, Broussine L, Chabarot A, Cozic A, Jehannin B, de Parscau L. Clinical heterogeneity of Townes-Brocks syndrome. Arch Pediatr. 1995;2(6):551–4. doi:0929693X96812000 [pii].
Pul N, Pul M, Gedik Y. McKusick-Kaufman syndrome associated with esophageal atresia and distal tracheoesophageal fistula: a case report and review of the literature. Am J Med Genet. 1994;49(3):341–3. doi:10.1002/ajmg.1320490320.
Hoffman MA, Superina R, Wesson DE. Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: report of two cases. J Pediatr Surg. 1989;24(10):1084–5. doi:S0022346889000527 [pii].
Steadland KM, Langham Jr MR, Greene MA, Bagwell CE, Kays DW, Talbert JL. Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula. Ann Thorac Surg. 1995;59(2):511–3. doi:000349759400383I [pii].
Havard AC, MacDonald LM. Oesophageal atresia and other disorders with a similar antenatal presentation. Br J Radiol. 1991;64(762):557–8.
Depaepe A, Dolk H, Lechat MF. The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT Working Group. Arch Dis Child. 1993;68(6):743–8.
Carvalho O. Hidramnio e atresia esophagiea congenita. Ann Bras Ginecol. 1954;38(3):137–42.
Farrant P. The antenatal diagnosis of oesophageal atresia by ultrasound. Br J Radiol. 1980;53(636):1202–3.
Vijayaraghavan SB. Antenatal diagnosis of esophageal atresia with tracheoesophageal fistula. J Ultrasound Med. 1996;15(5):417–9.
Stringer MD, McKenna KM, Goldstein RB, Filly RA, Adzick NS, Harrison MR. Prenatal diagnosis of esophageal atresia. J Pediatr Surg. 1995;30(9):1258–63. doi:0022-3468(95)90480-8 [pii].
Borsellino A, Zaccara A, Nahom A, Trucchi A, Aite L, Giorlandino C, Bagolan P. False-positive rate in prenatal diagnosis of surgical anomalies. J Pediatr Surg. 2006;41(4):826–9. doi:10.1016/j.jpedsurg.2005.12.024. S0022-3468(05)00969-3 [pii].
Manchester DK, Pretorius DH, Avery C, Manco-Johnson ML, Wiggins J, Meier PR, Clewell WH. Accuracy of ultrasound diagnoses in pregnancies complicated by suspected fetal anomalies. Prenat Diagn. 1988;8(2):109–17.
Nicolaides KH, Snijders RJ, Cheng HH, Gosden C. Fetal gastro-intestinal and abdominal wall defects: associated malformations and chromosomal abnormalities. Fetal Diagn Ther. 1992;7(2):102–15.
Shulman A, Mazkereth R, Zalel Y, Kuint J, Lipitz S, Avigad I, Achiron R. Prenatal identification of esophageal atresia: the role of ultrasonography for evaluation of functional anatomy. Prenat Diagn. 2002;22(8):669–74. doi:10.1002/pd.375.
Choudhry M, Boyd PA, Chamberlain PF, Lakhoo K. Prenatal diagnosis of tracheo-oesophageal fistula and oesophageal atresia. Prenat Diagn. 2007;27(7):608–10. doi:10.1002/pd.1745.
Eyheremendy E, Pfister M. Antenatal real-time diagnosis of esophageal atresias. J Clin Ultrasound. 1983;11(7):395–7.
Centini G, Rosignoli L, Kenanidis A, Petraglia F. Prenatal diagnosis of esophageal atresia with the pouch sign. Ultrasound Obstet Gynecol. 2003;21(5):494–7. doi:10.1002/uog.58.
Yagel S, Sonigo P, Rousseau V, Sarnacki S, Cohen S, Benachi A. Esophageal atresia diagnosed with three-dimensional ultrasonography. Ultrasound Obstet Gynecol. 2005;26(3):307–8. doi:10.1002/uog.1982.
Satoh S, Takashima T, Takeuchi H, Koyanagi T, Nakano H. Antenatal sonographic detection of the proximal esophageal segment: specific evidence for congenital esophageal atresia. J Clin Ultrasound. 1995;23(7):419–23.
Kalache KD, Chaoui R, Mau H, Bollmann R. The upper neck pouch sign: a prenatal sonographic marker for esophageal atresia. Ultrasound Obstet Gynecol. 1998;11(2):138–40. doi:10.1046/j.1469-0705.1998.11020138.x.
Has R, Gunay S, Topuz S. Pouch sign in prenatal diagnosis of esophageal atresia. Ultrasound Obstet Gynecol. 2004;23(5):523–4. doi:10.1002/uog.1041.
Levine D, Barnewolt CE, Mehta TS, Trop I, Estroff J, Wong G. Fetal thoracic abnormalities: MR imaging. Radiology. 2003;228(2):379–88. doi:10.1148/radiol.2282020604. 2282020604 [pii].
Matsuoka S, Takeuchi K, Yamanaka Y, Kaji Y, Sugimura K, Maruo T. Comparison of magnetic resonance imaging and ultrasonography in the prenatal diagnosis of congenital thoracic abnormalities. Fetal Diagn Ther. 2003;18(6):447–53. doi:10.1159/000073141. FDT2003018006447 [pii].
Langer JC, Hussain H, Khan A, Minkes RK, Gray D, Siegel M, Ryan G. Prenatal diagnosis of esophageal atresia using sonography and magnetic resonance imaging. J Pediatr Surg. 2001;36(5):804–7. doi:10.1053/jpsu.2001.22965. S0022-3468(01)07270-0 [pii].
Goldberg S, Ringertz H, Barth RA. Prenatal diagnosis of horseshoe lung and esophageal atresia. Pediatr Radiol. 2006;36(9):983–6. doi:10.1007/s00247-006-0242-y.
Kuti K, Patel R, Chapman S, Jawaheer G. A rare pitfall in the diagnosis of oesophageal atresia. Pediatr Radiol. doi:10.1007/s00247-010-1685-8.
Behnia M, Tarver RD. Unilateral bronchiectasis and esophageal dysmotility in congenital adult tracheoesophageal fistula. Intern Med. 2001;40(7):620–3.
Holinger PH, Johnston KC, Potts WJ. Congenital anomalies of the esophagus. Ann Otol Rhinol Laryngol. 1951;60(3):707–30.
Skandalakis JE, Gray SW. Embryology for surgeons: the embryological basis for the treatment of congenital anomalies. 2nd ed. Baltimore: Williams & Wilkins; 1994.
Girdany BR, Sieber WK, Osman MZ. Traumatic pseudodiverticulums of the pharynx in newborn infants. N Engl J Med. 1969;280(5):237–40.
Talbert JL, Rodgers BM, Felman AH, Moazam F. Traumatic perforation of the hypopharynx in infants. J Thorac Cardiovasc Surg. 1977;74(1):152–6.
Krasna IH, Rosenfeld D, Benjamin BG, Klein G, Hiatt M, Hegyi T. Esophageal perforation in the neonate: an emerging problem in the newborn nursery. J Pediatr Surg. 1987;22(8):784–90. doi:S0022346887002677 [pii].
Blair GK, Filler RM, Theodorescu D. Neonatal pharyngoesophageal perforation mimicking esophageal atresia: clues to diagnosis. J Pediatr Surg. 1987;22(8):770–4. doi:S0022346887002653 [pii].
Pal K, Alwabari A, Abdulatif M. Traumatic pharyngeal pseudodiverticulum mimicking esophageal atresia. Indian J Pediatr. 2007;74(2):195–6.
Emil SG. Neonatal esophageal perforation. J Pediatr Surg. 2004;39(8):1296–8. doi:S0022346804002842 [pii].
Goh DW, Brereton RJ, Spitz L. Esophageal atresia with obstructed tracheoesophageal fistula and gasless abdomen. J Pediatr Surg. 1991;26(2):160–2. doi:0022-3468(91)90898-4 [pii].
Dudgeon DL, Morrison CW, Woolley MM. Congenital proximal tracheoesophageal fistula. J Pediatr Surg. 1972;7(5):614–9. doi:0022-3468(72)90221-7 [pii].
Kosloske AM, Jewell PF, Cartwright KC. Crucial bronchoscopic findings in esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1988;23(5):466–70. doi:S0022346888001903 [pii].
Mellins RB, Blumenthal S. Cardiovascular anomalies and esophageal atresia. Am J Dis Child. 1964;107:160–4.
David TJ, O’Callaghan SE. Cardiovascular malformations and oesophageal atresia. Br Heart J. 1974;36(6):559–65.
Berdon WE, Baker DH, Schullinger JN, Santulli TV. Plain film detection of right aortic arch in infants with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1979;14(4):436–7. doi:S0022346879000725 [pii].
Stringel G, Coln D, Guertin L. Esophageal atresia and right aortic arch. Right or left thoracotomy? Pediatr Surg Int. 1990;5:103–5.
Allen SR, Ignacio R, Falcone RA, Alonso MH, Brown RL, Garcia VF, Inge TH, Ryckman FC, Warner BW, Azizkhan RG, Tiao GM. The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 2006;41(3):479–83. doi:10.1016/j.jpedsurg.2005.10.051. S0022-3468(05)00802-X [pii].
Bowkett B, Beasley SW, Myers NA. The frequency, significance, and management of a right aortic arch in association with esophageal atresia. Pediatr Surg Int. 1999;15(1):28–31.
Harrison MR, Hanson BA, Mahour GH, Takahashi M, Weitzman JJ. The significance of right aortic arch in repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1977;12(6):861–9. doi:0022-3468(77)90595-4 [pii].
Harrison MR, Weitzman JJ, deLorimier AA. Localization of the aortic arch prior to repair of esophageal atresia. J Pediatr Surg. 1980;15(3):312.
Stewart JR, Kincaid OW, Titus JL. Right aortic arch: plain film diagnosis and significance. Am J Roentgenol Radium Ther Nucl Med. 1966;97(2):377–89.
Knight L, Edwards JE. Right aortic arch. Types and associated cardiac anomalies. Circulation. 1974;50(5):1047–51.
Katz M, Konen E, Rozenman J, Szeinberg A, Itzchak Y. Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies. J Comput Assist Tomogr. 1995;19(4):564–8.
Burrows PE, MacDonald CE. Magnetic resonance imaging of the pediatric thoracic aorta. Semin Ultrasound CT MR. 1993;14(2):129–44.
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Klein, J.D., Jennings, R.W. (2017). Esophageal Atresia and Tracheoesophageal Fistula: The Clinical Spectrum, Diagnosis, and Evaluation. In: Till, H., Thomson, M., Foker, J., Holcomb III, G., Khan, K. (eds) Esophageal and Gastric Disorders in Infancy and Childhood. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-11202-7_7
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