eMedicine Specialties > Pediatrics: Surgery > General Surgery

Esophageal Atresia With or Without Tracheoesophageal Fistula: Follow-up

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

Outcome and Prognosis

Statistics regarding mortality rates in esophageal atresia are constantly changing and improving.23 One must consider the classification system used in reporting such statistics.

  • Montreal classification9
    • Class I - Mortality rate of 7.3%
    • Class II - Mortality rate of 69.2%
  • Spitz grouping8
    • Group I - Mortality rate of 3%
    • Group II - Mortality rate of 41%
    • Group III - Mortality rate of 78%
  • Waterston categorization24
    • Category A - Mortality rate of 0%
    • Category B - Mortality rate of 4%
    • Category C - Mortality rate of 11%

Fetuses with prenatal diagnoses of esophageal atresia seem to have a worse prognosis.25 The cohort of babies in whom esophageal atresia is detected prenatally has a 75% mortality rate, whereas the cohort of babies in whom esophageal atresia is not detected prenatally has a 21% mortality rate. Babies who survive have varied morbidities related to any of the associated anomalies and complications. However, most children who undergo a successful repair of esophageal atresia are relatively healthy.

Future and Controversies

The future is bright, with the following considerations: More accurate prenatal diagnosis and prenatal treatment may be possible. Minimally invasive techniques for repair with thoracoscopic surgery are now used in some centers, with good results. A better understanding of the pathoembryologic processes of this condition may reveal its causative agents or genetic factors. This knowledge, in turn, may lead to specific prenatal treatments or preventive techniques. Recently, the incidence of this disorder has decreased, perhaps because of increased usage of prenatal folic acid supplements.

Debates continue about the best operative technique (eg, right-sided or left-sided thoracotomy) for patients with right-sided aortic arches, suture type and technique, esophageal lengthening strategies, and procedures for mobilizing the distal esophagus. Other discussions include when to use cervical esophagostomy and the choice of esophageal replacement. The advent of esophageal atresia repairs that combine both minimally invasive and radiologic interventional techniques may be near.

The management of gastroesophageal reflux in esophageal atresia is particularly challenging; some advocate aggressive fundoplication, and others prefer more conservative medical treatment. In addition, the true incidence and treatment of tracheomalacia continues to be the subject of debate. Lastly, the proper evidence-based guidelines for long-term follow-up are still elusive.

 


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References
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Further Reading

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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

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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.

 
 
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