Pediatric Surgery: Esophageal Atresia (EA)

Esophageal Atresia (EA)

Definitions

  • Esophageal Atresia (EA) – Congenital Defect of the Esophagus with an Absent Segment
  • Tracheoesophageal Fistula (TEF) – Abnormal Connection Between the Trachea & Esophagus

Gross Classification

  • Type A: No TEF (6%)
  • Type B: Proximal TEF (5%)
  • Type C: Distal TEF (85% – Most Common)
    • “h-Type”
  • Type D: Proximal & Distal TEF (1%)
  • Type E: TEF, No EA (4%)
    • “H-Type”
    • Trachea Opening is Always Proximal to Esophageal Opening
  • Type F: Esophageal Stenosis, No EA or TEF

Esophageal Atresia Classification 1

Associated Anomalies

  • 50-70% Have ≥ 1 Associated Congenital Anomaly
    • Most Common in Type A
    • Least Common in Type E
  • Most Common Associated Anomaly: Cardiovascular
  • VACTERL Syndrome
    • Vertebrae
    • Anorectal
    • Cardiac
    • TA/TEF
    • Renal
    • Limb Anomalies
  • Most Common Cause in Adults: Malignancy

Predictors of Survival (Okamoto Modification of Spitz Classification)

  • Class I (No Major Cardiac Anomaly & Birth Weight ≥ 2000 g): 100% Survival
  • Class II (No Major Cardiac Anomaly & Birth Weight < 2000 g): 81% Survival
  • Class III (Major Cardiac Anomaly & Birth Weight ≥ 2000 g): 72% Survival
  • Class IV (Major Cardiac Anomaly & Birth Weight < 2000 g): 27% Survival

Presentation

  • Most are Symptomatic within Hours of Life
  • Excessive Drooling & Unable to Tolerate Secretions
  • Choking & Coughing After Feeding
  • Cyanosis
  • Respiratory Distress
    • Chemical Pneumonitis from Gastric Acid Reflux Through TEF
    • Diaphragm Elevation from Abdominal Distention

Diagnosis

  • Not Commonly Found Prenatally
  • Diagnosis: Chest X-Ray (CXR) or Esophagram (Barium)
    • Contrast Agents:
      • Gastrografin:Causes Severe Pneumonitis (Should Not Be Used if Concerned for Aspiration)
      • Barium:Cause Severe Inflammatory Mediastinitis (Should Not Be Used if Concerned for Perforation)
    • X-Ray Findings:
      • Orogastric Tube Looped in Proximal Esophagus
      • Gaseous Distention:
        • Type A/B: No Gas in Abdomen
        • Type C: Distended Stomach
    • May Also Require Endoscopy or Bronchoscopy
  • Requires Echocardiogram & Renal US Before Surgery to Evaluate VACTERAL Syndrome

Esophageal Atresia Esophogram 2

Treatment

Initial Management

  • Initial Management: Resuscitation & Stabilization
  • Sump/Replogle Catheter
    • Drains Proximal Stump – Perforations Only at the Tip to Minimize Loss of Oxygenated Air
  • Avoid Routine Endotracheal Intubation – Can Worsen Abdominal Distention & Respiratory Distress

Definitive Treatment

  • Definitive Treatment: Surgical Repair
  • Approach:
    • Type A-D (Non-H-Type): Right Extra-Pleural Thoracotomy & Primary Repair
    • Type E (H-Type): Right Cervical Approach
      • Because Most Are Located at Level of Thoracic Inlet
  • General Measures:
    • Separate the Trachea & Esophagus
    • Ligate Fistula
    • Primary Anastomosis for Atresia

Long Gap (> 2 Vertebral Bodies) Atresia

  • Management: Temporary G-Tube & Delay Repair for 2-3 Months
  • Allows Esophageal Growth to Permit Repair
  • Consider Placement of Internal or External Traction to Reapproximate Until Delayed Repair
  • If Fails: Intestinal Interposition or Gastric Transposition
    • Long Gap EA is the Most Common Indication for Esophageal Replacement in Peds – Caustic Stricture is Second Most Common

Complications

  • GERD (Nearly All – Most Common Complication)
  • Stricture (40%)
  • Anastomotic Leak (10-20%)
    • Most Heal Spontaneously
  • Recurrent Fistula (10%)
  • Tracheomalacia
  • Most Commonly Injured Structure During Dissection of Upper Esophagus: Recurrent Laryngeal Nerve

References

  1. Krishnan U. Eosinophilic Esophagitis in Esophageal Atresia. Front Pediatr. 2019 Nov 29;7:497. (License: CC BY-4.0)
  2. DrM!key. Wikimedia Commons. (License: CC-0)