Esophageal Atresia With or Without Tracheoesophageal Fistula Workup

  • Author: Amulya K Saxena, MD, PhD; Chief Editor: Marleta Reynolds, MD   more...
 
Updated: Apr 25, 2012
 

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
Next

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 the images below) is mandatory and should be performed as soon as possible if esophageal atresia is suspected. This chest radiograph reveals esophageal atresia aThis chest radiograph reveals esophageal atresia and distal tracheoesophageal fistula. Note the Replogle tube in the upper pouch and the GI air below the diaphragm. This chest radiograph reveals esophageal atresia wThis chest radiograph reveals esophageal atresia without tracheoesophageal fistula. Note the absence of gas below the diaphragm.
    • 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.[19] 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 the image below) is indicated if the limbs appear abnormal. The possibility of associated radial-ray deformities should be investigated. This radiograph reveals a radius without a radial This radiograph reveals a radius without a radial ray deformity.
  • 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 the image below) is useful in assessing that distance. Contrast material has been administered, and a proContrast material has been administered, and a probe has been placed through the gastrostomy in this child with pure esophageal atresia. The air-filled upper pouch can be observed superiorly, with a Replogle tube within it. This gap-o-gram reveals a very wide gap (>5 vertebral bodies), which requires esophageal replacement. This study is a dynamic investigation, one in which the surgeon and radiologist should be present to view the real-time fluoroscopic images.
    • 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.
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Contributor Information and Disclosures
Author

Amulya K Saxena, MD, PhD  Associate Professor, Department of Pediatric and Adolescent Surgery, Medical University of Graz, Austria

Amulya K Saxena, MD, PhD is a member of the following medical societies: Austrian Society for Pediatric and Adolescent Surgery, European Pediatric Surgeons Association, German Society of Pediatric Surgery, German Society of Surgery, International Pediatric Endosurgery Group, and Tissue Engineering and Regenerative Medicine International Society (TERMIS)

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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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.

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, Northwestern University, The Feinberg School of Medicine; Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric 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.

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Esophageal atresia classification according to Gross.
This chest radiograph reveals esophageal atresia and distal tracheoesophageal fistula. Note the Replogle tube in the upper pouch and the GI air below the diaphragm.
This chest radiograph reveals esophageal atresia without tracheoesophageal fistula. Note the absence of gas below the diaphragm.
This radiograph reveals a radius without a radial ray deformity.
Contrast material has been administered, and a probe has been placed through the gastrostomy in this child with pure esophageal atresia. The air-filled upper pouch can be observed superiorly, with a Replogle tube within it. This gap-o-gram reveals a very wide gap (>5 vertebral bodies), which requires esophageal replacement. This study is a dynamic investigation, one in which the surgeon and radiologist should be present to view the real-time fluoroscopic images.
This postoperative contrast-enhanced radiograph reveals esophageal gastric tube replacement. The anastomosis to the upper pouch is in the chest. The linear staple line of the tube can be observed.
This esophagogram was obtained using water-soluble contrast material 6 days after a standard repair was performed. The chest tube is still in place. No leak is present. The waist observed here at the site of the recently performed anastomosis is usual and does not, at this stage, necessarily indicate a stricture. The child went home and was eating well 3 days later.
 
 
 
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