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  • • 85% esophageal atresia with distal TEF, 5-7% pure esophageal atresia, 2-6% TEF alone, other configurations more rare

    • Risk factors:

    • -First pregnancy

      -Advanced maternal age

      -Affected parent

      -Affected siblings

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Epidemiology

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  • • 1/2500 to 1/20,000 births affected

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Symptoms and Signs

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  • • Asymptomatic first few hours of life

    • Excessive drooling

    • Choking, coughing

    • Regurgitation

    • Respiratory distress

    • Cyanosis

    • Scaffoid abdomen if pure esophageal atresia

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Imaging Findings

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  • Prenatal US: Polyhydramnios

    Chest and abdominal x-ray

    • -Blind esophageal pouch when NG inserted

      -Gasless abdomen means no associated TEF

      -Gas filled means associated TEF

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Rule Out

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  • • Other associated abnormalities (present in 50% of patients)

    • -Vertebral

      -Anorectal

      -Cardiac

      -Renal

      -Radial limb

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  • • History and physical exam

    • Chest and abdominal films with presence of NG tube

    • Occasionally esophageal contrast study to verify presence of fistula

    • Occasionally bronchoscopy or esophagoscopy to verify presence of fistula

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Surgery

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  • • Decompressive gastrostomy if TEF causing gastric distention and subsequent difficulty breathing secondary to compression of diaphragm

    • Resection of fistula with primary repair of esophageal atresia

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Indications

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  • • Resolution or stabilization of pulmonary or cardiac abnormalities

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Medications

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  • • Sump suction to esophageal pouch

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Complications

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  • • Anastomotic leak, 10-20%

    • Stricture, 40%

    • Recurrent TEF, 10%

    • Gastroesophageal reflux disease, all

    • Esophageal dysmotility, all

    • Tracheomalacia

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Prognosis

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  • • 85-90% survival

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References

Holcomb GW 3rd et al: Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis. Ann Surg 2005;242:422.  [PubMed: 16135928]

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