eMedicine Specialties > Pediatrics: Surgery > General Surgery

Esophageal Atresia With or Without Tracheoesophageal Fistula: Workup

Author: Amulya K Saxena, MD, Attending Pediatric Surgeon, Department of Pediatric Surgery, Medical University of Graz, Austria
Coauthor(s): Geoffrey Blair, MD, Clinical Professor of Pediatric General Surgery, Department of Pediatric Surgery, University of British Columbia; Head, British Columbia's Children's Hospital; David E Konkin, MD, Staff Physician, Department of Surgery, Royal Columbian Hospital, University of British Columbia
Contributor Information and Disclosures

Updated: Apr 30, 2008

Workup

Laboratory Studies

In babies with esophageal atresia, samples should be obtained to determine baseline values of the following:

  • CBC count
  • Electrolyte levels
  • Venous gas concentrations
  • BUN and serum creatinine levels
  • Blood glucose level
  • Serum calcium level
  • ABG concentrations, as necessary

Imaging Studies

  • Prenatal ultrasonography may reveal the size of the gastric bubble, polyhydramnios, and VACTERL anomalies, all of which may indicate esophageal atresia in the fetus.
    • The sensitivity of prenatal ultrasonography is approximately 40%.
    • A prenatal diagnosis of esophageal atresia may be associated with a worse prognosis.
  • Chest radiography (see Media files 2-3) is mandatory and should be performed as soon as possible if esophageal atresia is suspected.
    • The value of the chest radiography is enhanced if a Replogle tube is in place and if 5-10 mL of air is injected to distend the upper pouch.
    • Great caution should be exercised if liquid contrast material is injected into the proximal pouch. First, only about 1 mL of isotonic water-soluble contrast should be used to prevent spillage into the airway. A catheter with an end-hole should be used. Second, if an upper pouch fistula is present, the contrast material flows directly into the airway. Usually, a contrast-enhanced study is unnecessary.
    • The heart shadow and size should be assessed.
    • Vertebral and rib anomalies should be assessed.
    • The lung fields should be assessed for possible aspiration pneumonitis and for the rarely associated diaphragmatic hernia or congenital lung lesion.
    • The presence or absence of GI air below the diaphragm is an important finding. Complete absence of gas in the GI tract denotes the absence of a distal tracheoesophageal fistula (TEF); however, distal fistulae simply occluded by mucous plugs have been rarely reported. In cases of esophageal atresia without fistula, assume that the distance between the ends of the atretic esophagus is too long for early single-stage primary repair. These infants require a delayed repair (see discussion about gastrostomy in Relevant Anatomy and about gap-o-gram below).
  • Early renal ultrasonography is mandatory and is used to evaluate associated kidney anomalies, ureteral anomalies, or both.
  • Echocardiography is indicated early in the care of infants with esophageal atresia who have clinical signs of cardiovascular disease. However, a 1-day-old neonate with significant congenital heart disease may have normal findings upon physical examination. Therefore, some argue that echocardiography should be performed in all infants with esophageal atresia. This examination also provides the surgeon with information regarding the side of the aortic arch. A right-sided aortic arch is not uncommon in cases of esophageal atresia, and the surgeon should be aware of this finding.
  • Limb radiography (see  Media file 4) is indicated if the limbs appear abnormal. The possibility of associated radial-ray deformities should be investigated.
  • Spinal ultrasonography is a simple test that takes advantage of the neonate's relatively transparent lumber lamina in the assessment of an associated tethered cord. This examination may be performed when the baby is younger than 1 month, although it is not critically important in the early care of the infant.
  • In cases in which the distance between the 2 atretic ends of the esophagus is suspected to be too long for a primary repair, a gap-o-gram (see Media file 5) is useful in assessing that distance.
    • A gastrostomy is created, and the upper pouch is intubated with a 10F Replogle tube with radiopaque markings. A small-diameter Bakes dilator is introduced into the gastrostomy and directed superiorly under fluoroscopic guidance into the distal esophageal segment. With gentle but definite force on both the Bakes dilator and the Replogle tube, the 2 ends are pushed toward each other under fluoroscopic control.
    • At the point of least separation, an image is obtained, and the distance between the 2 ends is determined in terms of vertebral bodies, which provide an inherent reference for measurement.
    • Generally, a separation distance of 2 (some say 3) vertebral bodies or fewer is usually small enough for an anastomosis. If greater distances separate the ends, a delay of weeks to months may be required for the ends to grow closer together, for reassessment with gap-o-grams every 4-6 weeks, or for esophageal replacement or lengthening surgery.

More on Esophageal Atresia With or Without Tracheoesophageal Fistula

Overview: Esophageal Atresia With or Without Tracheoesophageal Fistula
Workup: Esophageal Atresia With or Without Tracheoesophageal Fistula
Treatment: Esophageal Atresia With or Without Tracheoesophageal Fistula
Follow-up: Esophageal Atresia With or Without Tracheoesophageal Fistula
Multimedia: Esophageal Atresia With or Without Tracheoesophageal Fistula
References
[ CLOSE WINDOW ]

References

  1. O'Rahilly R, Muller F. Chevalier Jackson lecture. Respiratory and alimentary relations in staged human embryos. New embryological data and congenital anomalies. Ann Otol Rhinol Laryngol. Sep-Oct 1984;93(5 Pt 1):421-9. [Medline].

  2. Kluth D, Steding G, Seidl W. The embryology of foregut malformations. J Pediatr Surg. May 1987;22(5):389-93. [Medline].

  3. Spilde TL, Bhatia AM, Marosky JK, et al. Fibroblast growth factor signaling in the developing tracheoesophageal fistula. J Pediatr Surg. Mar 2003;38(3):474-7. [Medline].

  4. Crisera CA, Maldonado TS, Longaker MT, Gittes GK. Defective fibroblast growth factor signaling allows for nonbranching growth of the respiratory-derived fistula tract in esophageal atresia with tracheoesophageal fistula. J Pediatr Surg. Oct 2000;35(10):1421-5. [Medline].

  5. Orford J, Manglick P, Cass DT, Tam PP. Mechanisms for the development of esophageal atresia. J Pediatr Surg. Jul 2001;36(7):985-94. [Medline].

  6. Gross RE. The Surgery of Infancy and Childhood. Philadelphia, PA: WB Saunders; 1953:76.

  7. Dunn JC, Fonkalsrud EW, Atkinson JB. Simplifying the Waterston's stratification of infants with tracheoesophageal fistula. Am Surg. Oct 1999;65(10):908-10. [Medline].

  8. Spitz L, Kiely EM, Morecroft JA, Drake DP. Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg. Jun 1994;29(6):723-5. [Medline].

  9. Poenaru D, Laberge JM, Neilson IR, Guttman FM. A new prognostic classification for esophageal atresia. Surgery. Apr 1993;113(4):426-32. [Medline].

  10. 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. May 1989;209(5):526-30; discussion 530-1. [Medline].

  11. Konkin DE, O'hali WA, Webber EM, Blair GK. Outcomes in esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. Dec 2003;38(12):1726-9. [Medline].

  12. Skarsgard ED. Dynamic esophageal lengthening for long gap esophageal atresia: experience with two cases. J Pediatr Surg. Nov 2004;39(11):1712-4. [Medline].

  13. Foker JE, Linden BC, Boyle EM, Marquardt C. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg. Oct 1997;226(4):533-41; discussion 541-3. [Medline].

  14. Foker JE, Kendall TC, Catton K, Khan KM. A flexible approach to achieve a true primary repair for all infants with esophagealatresia. Semin Pediatr Surg. Feb 2005;14(1):8-15. [Medline].

  15. Allal H, Kalfa N, Lopez M, et al. Benefits of the thoracoscopic approach for short- or long-gap esophageal atresia. J Laparoendosc Adv Surg Tech A. Dec 2005;15(6):673-7. [Medline].

  16. Bax KM, van Der Zee DC. Feasibility of thoracoscopic repair of esophageal atresia with distal fistula. J Pediatr Surg. Feb 2002;37(2):192-6. [Medline].

  17. Holcomb GW 3rd, Rothenberg SS, Bax KM, et al. Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis. Ann Surg. Sep 2005;242(3):422-8; discussion 428-30. [Medline].

  18. Rothenberg SS. Thoracoscopic repair of tracheoesophageal fistula in newborns. J Pediatr Surg. Jun 2002;37(6):869-72. [Medline].

  19. Deurloo JA, Ekkelkamp S, Taminiau JA, et al. Esophagitis and Barrett esophagus after correction of esophageal atresia. J Pediatr Surg. Aug 2005;40(8):1227-31. [Medline].

  20. Deurloo JA, Ekkelkamp S, Hartman EE, et al. Quality of life in adult survivors of correction of esophageal atresia. Arch Surg. Oct 2005;140(10):976-80. [Medline].

  21. Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Anastomotic leakage following surgery for esophageal atresia. J Pediatr Surg. Jan 1992;27(1):29-32. [Medline].

  22. Blair GK, Cohen R, Filler RM. Treatment of tracheomalacia: eight years' experience. J Pediatr Surg. Sep 1986;21(9):781-5. [Medline].

  23. Clark DC. Esophageal atresia and tracheoesophageal fistula. Am Fam Physician. Feb 15 1999;59(4):910-6, 919-20. [Medline].

  24. Engum SA, Grosfeld JL, West KW, et al. Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decades. Arch Surg. May 1995;130(5):502-8; discussion 508-9. [Medline].

  25. Stringer MD, McKenna KM, Goldstein RB, et al. Prenatal diagnosis of esophageal atresia. J Pediatr Surg. Sep 1995;30(9):1258-63. [Medline].

  26. Ashcraft KW, Holder TM. Pediatric Esophageal Surgery. Orlando, FL: Grune and Stratton; 1996.

  27. Beasley SW, Hutson JM, Auldist AW. Essential Paediatric Surgery. Oxford, England: Oxford University Press; 1996.

  28. Cotran RS, Kumar V, Robbins T. Robbins Pathological Basis of Disease. 5th ed. Philadelphia, PA: WB Saunders; 1994.

  29. Diaz LK, Akpek EA, Dinavahi R, Andropoulos DB. Tracheoesophageal fistula and associated congenital heart disease: implications for anesthetic management and survival. Paediatr Anaesth. Oct 2005;15(10):862-9. [Medline].

  30. Encinas JL, Luis AL, Avila LF, Martinez L, et al. Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia. Pediatr Surg Int. Feb 2006;22(2):150-3. [Medline].

  31. Greenfield LJ, Mulholland M, Oldham KT. Surgery: Scientific Principles and Practice. 2nd ed. Philadelphia, Pa: Lippincott-Raven; 1997:2011-6.

  32. Jolley SG. A longitudinal study of children treated with the most common form of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. Sep 2007;42(9):1632-3; author reply 1633. [Medline].

  33. Lopes MF, Botelho MF. Midterm follow-up of esophageal anastomosis for esophageal atresia repair: long-gap versus non-long-gap. Dis Esophagus. 2007;20(5):428-35. [Medline].

  34. McCollum MO, Rangel SJ, Blair GK, et al. Primary reversed gastric tube reconstruction in long gap esophageal atresia. J Pediatr Surg. Jun 2003;38(6):957-62. [Medline].

  35. Moore KL, Persaud TV. The Developing Human: Clinically Oriented Embryology. 5th ed. Philadelphia, Pa: WB Saunders; 1993.

  36. O'Neil JA, Rowe MI, Grosfeld JL. Pediatric Surgery. 5th ed. St Louis, Mo: Mosby-Year Book; 1998:941-67.

  37. Pederiva F, Burgos E, Francica I, Zuccarello B, Martinez L, Tovar JA. Intrinsic esophageal innervation in esophageal atresia without fistula. Pediatr Surg Int. Oct 26 2007;[Medline].

  38. Seguier-Lipszyc E, Bonnard A, Aizenfisz S, et al. The management of long gap esophageal atresia. J Pediatr Surg. Oct 2005;40(10):1542-6. [Medline].

  39. Sharma S, Sinha SK, Rawat JD, Wakhlu A, Kureel SN, Tandon R. Azygos vein preservation in primary repair of esophageal atresia with tracheoesophageal fistula. Pediatr Surg Int. Dec 2007;23(12):1215-8. [Medline].

  40. Shaw-Smith CJ. Oesophageal atresia, tracheo-oesophageal fistula and the VACTERL association:review of genetics and epidemiology. J Med Genet. Nov 21 2005;[Medline].

  41. Spitz L. Esophageal atresia: past, present, and future. J Pediatr Surg. Jan 1996;31(1):19-25. [Medline].

  42. Spitz L, Kiely E, Brereton RJ. Esophageal atresia: five year experience with 148 cases. J Pediatr Surg. Feb 1987;22(2):103-8. [Medline].

  43. van der Zee DC, Vieirra-Travassos D, Kramer WL, Tytgat SH. Thoracoscopic elongation of the esophagus in long gap esophageal atresia. J Pediatr Surg. Oct 2007;42(10):1785-8. [Medline].

  44. Way LW, ed. Current Surgical Diagnosis and Treatment. 10th ed. New York, NY: Appleton & Lange; 1994.

  45. Yeming W, Somme S, Chenren S, et al. Balloon catheter dilatation in children with congenital and acquired esophageal anomalies. J Pediatr Surg. Mar 2002;37(3):398-402. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

esophageal atresia, EA, tracheoesophageal fistula, TEF, congenitally interrupted esophagus, malformed esophagus, tracheoesophageal defects, trisomy 21, trisomy 13, trisomy 18, tracheoesophageal separation, esophageal atresia without fistula, pure esophageal atresia, proximal TEF, distal TEF, H-type fistula, congenital esophageal stenosis, polyhydramnios, aspiration pneumonitis, acute gastric perforation, dysphagia, gastroesophageal reflux, tracheomalacia, pneumonia, respiratory distress, VACTERL, vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, limb deformities, hemivertebrae, scoliosis, rib deformities, imperforate anus, cloacal deformities

ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, atrial septal defects, atrioventricular canal defects, aortic coarctation, right-sided aortic arch, single umbilical artery, Potter syndrome, bilateral renal agenesis, horseshoe kidney, polycystic kidneys, urethral atresia, ureteral malformations, radial dysplasia, absent radius, radial-ray deformities, syndactyly, polydactyly, lower-limb tibial deformities, coloboma, heart defects, atresia choanae, developmental retardation, genital hypoplasia, ear deformities, CHARGE, neural tube defects, hydrocephalus, tethered cord, holoprosencephaly, duodenal atresia, ileal atresia, hypertrophic pyloric stenosis, omphalocele, malrotation, Meckel diverticulum, unilateral pulmonary agenesis, diaphragmatic hernia, undescended testicles, ambiguous genitalia, hypospadias, Fanconi syndrome

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Amulya K Saxena, MD, Attending Pediatric Surgeon, Department of Pediatric Surgery, Medical University of Graz, Austria
Amulya K Saxena, MD is a member of the following medical societies: European Pediatric Surgeons Association, German Society of Pediatric Surgery, German Society of Surgery, and International Pediatric Endosurgery Group
Disclosure: Nothing to disclose.

Coauthor(s)

Geoffrey Blair, MD, Clinical Professor of Pediatric General Surgery, Department of Pediatric Surgery, University of British Columbia; Head, British Columbia's Children's Hospital
Geoffrey Blair, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

David E Konkin, MD, Staff Physician, Department of Surgery, Royal Columbian Hospital, University of British Columbia
David E Konkin, MD is a member of the following medical societies: American College of Surgeons, British Columbia Medical Association, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, and Society of American Gastrointestinal and Endoscopic Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Kurt D Newman, MD, Vice Chairman, Department of Pediatric Surgery, Children's National Medical Center; Professor, Departments of Surgery and Pediatrics, George Washington University School of Medicine
Kurt D Newman, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Michael G Caty, MD, Professor of Surgery and Pediatrics, State University of New York at Buffalo; Consulting Staff, Department of Pediatric Surgery, Children's Hospital of Buffalo
Michael G Caty, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Physician Executives, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Association for Academic Surgery, and Association for Surgical Education
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.