eMedicine Specialties > Pediatrics: Surgery > General Surgery

Congenital Anomalies of the Esophagus: Follow-up

Author: Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy
Coauthor(s): Alison Snyder-Warwick, MD, Research Fellow, Department of Developmental Biology and Department of Surgery, Division of Plastic and Reconstructive Surgery, Washington University School of Medicine; Mark V Mazziotti, MD, Assistant Professor of Pediatric Surgery, Department of Surgery, Baylor College of Medicine, Texas Children's Hospital; Jacob C Langer, MD, Professor, Department of Surgery, University of Toronto Faculty of Medicine
Contributor Information and Disclosures

Updated: Oct 31, 2008

Outcome and Prognosis

The survival rate of patients with esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) has immensely improved since Haight's first successful repair in 1941. Early diagnosis and advancements in neonatal anesthesia, surgical technique, treatment of associated anomalies, and intensive care management have improved the prognosis. Most children treated for EA have a normal lifespan. Despite an increased number of patients with severe congenital anomalies, survival rates have been reported as high as 95%. In uncomplicated cases, survival rates are virtually 100%.

Traditionally, prognosis for children with EA-TEF was based on birth weight and the presence of pneumonia and associated congenital anomalies. Because of advancements in neonatal care, birth weight does not affect survival rate unless it is severely low, and pneumonia may be treated successfully. Currently, cardiac and chromosomal abnormalities are the most significant causes of death. Infants with a birth weight less than 1500 g, major congenital cardiac abnormalities, severe associated anomalies, preoperative ventilator dependence, and/or long gap are at increased risk.

Dysphagia, frequent night coughs, dyspepsia, and recurrent respiratory infections are frequent results of the less distensible esophagus and gastroesophageal reflux. Gastroesophageal reflux occurs in as many as one half of these patients and many require antireflux operations. Feeding difficulties also are common, particularly during the first several years after repair. Choking, vomiting, and food impaction occur. These symptoms, like many following EA repair, diminish over time, and 70-80% of adolescents report no or only occasional swallowing impairment. Most patients who have undergone EA repair have abnormal peristalsis with decreased contractile activity and inefficient clearance capacities.

In one series, after an average of 8.8 years of follow-up care, all patients were reported to eat excellently or satisfactorily, with more than 90% eating no differently than their siblings. Normal respiratory function is observed in half of patients 3 months postoperatively. Tracheomalacia, vascular rings, and decreased lung volumes account for the abnormal respiratory function in the other children. Tracheomalacia occurs in 10% of patients with TEF. Most outgrow this problem; however, some children require more aggressive therapy.

Growth retardation has been observed in some children who have had EA repair, but this observation varies. Patients treated for EA-TEF are at higher risk for developing esophagitis and Barrett epithelium. Reports of esophageal carcinoma decades after EA-TEF repair are becoming more frequent as the first generation of survivors progresses through adulthood. Surveillance esophagoscopy has been proposed to provide early detection for esophageal abnormalities.

Despite the complications, the results of EA-TEF repair have dramatically improved. Many symptoms are alleviated over time, and most children and adults enjoy normal lifestyles and have no complaints concerning their quality of life or eating habits. Even by school age, children who had many complications in infancy reported few restrictions at school or in participation in sports with little or no effect on school attendance and social activities. The outcome for these children and children treated for other congenital lesions is generally good.

Future and Controversies

The prognosis and treatment course for infants with esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and other congenital lesions has improved over the past 60 years. Advances in perinatal and neonatal care have been paramount in reducing the morbidity and mortality rates associated with these conditions. Currently, associated congenital anomalies and pulmonary complications contribute most significantly to adverse outcomes.

Infants with very low birth weight or serious cardiac abnormalities are at increased risk for poor outcome. Improvements in the prevention and management of these high-risk infants would improve outcome and survival rates. In addition, enhanced prenatal detection of EA and/or TEF and other congenital anomalies allows for better prenatal counseling and preparation for the delivery at a tertiary medical center.

Esophageal defects are currently repaired with thoracoscopy; robotic-assisted surgery may be used in the future.6

Tissue engineering for esophageal replacement, in utero intervention, and minimally invasive techniques such as thoracoscopy and robotic assistance may be used in years to come to further improve treatment of these infants.

 


More on Congenital Anomalies of the Esophagus

Overview: Congenital Anomalies of the Esophagus
Workup: Congenital Anomalies of the Esophagus
Treatment: Congenital Anomalies of the Esophagus
Follow-up: Congenital Anomalies of the Esophagus
Multimedia: Congenital Anomalies of the Esophagus
References
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Further Reading

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Keywords

congenital anomalies of the esophagus, esophageal atresia, EA, tracheoesophageal fistula, TEF, esophageal stenosis, esophageal cyst, tracheobronchial remnant, esophageal atresia and tracheoesophageal fistula, EA-TEF, esophageal web, esophageal muscular hypertrophy, esophageal duplications, esophageal rests, columnar epithelium–lined lower esophagus, Barrett esophagus, Barrett's esophagus, laryngotracheoesophageal cleft, LTEC, gastroesophageal reflux, GERD, oculodigitoesophageoduodenal, ODED, Feingold syndrome, trisomy 18, Down syndrome, pneumonia, atelectasis, polyhydramnios, pneumonitis, tetralogy of Fallot, imperforate anus

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Contributor Information and Disclosures

Author

Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy
Robert K Minkes, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Alison Snyder-Warwick, MD, Research Fellow, Department of Developmental Biology and Department of Surgery, Division of Plastic and Reconstructive Surgery, Washington University School of Medicine
Alison Snyder-Warwick, MD is a member of the following medical societies: Alpha Omega Alpha, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Mark V Mazziotti, MD, Assistant Professor of Pediatric Surgery, Department of Surgery, Baylor College of Medicine, Texas Children's Hospital
Mark V Mazziotti, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Jacob C Langer, MD, Professor, Department of Surgery, University of Toronto Faculty of Medicine
Jacob C Langer, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Society for Surgery of the Alimentary Tract, and Society of University Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Robert Kelly, MD, Chairman, Department of Surgery, Departments of Surgery and Pediatrics, Children's Hospital of the King's Daughters; Associate Professor, Eastern Virginia Medical School
Robert Kelly, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society of Abdominal Surgeons, Medical Society of Virginia, Norfolk Academy of Medicine, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
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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