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
- Contrast Agents:
- 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