The trachea and esophagus develop from a common foregut tube that separates early in gestation. Incomplete separation produces:

• Esophageal atresia (EA): the esophagus ends in a blind upper pouch, so saliva and feeds cannot reach the stomach.
• Tracheoesophageal fistula (TEF): an abnormal connection between the trachea and esophagus, allowing saliva or feeds into the lungs (upper fistula) or air into the stomach and gastric acid into the lungs (lower fistula).

Types (Gross classification)

Associated anomalies (in about half of infants): the VACTERL association — Vertebral, Anal atresia, Cardiac, Tracheo-Esophageal, Renal, and Limb (radial) anomalies. Also CHARGE syndrome, trisomy 18 and 21, and prematurity. Hearing loss is not part of VACTERL.

Prenatal clue: polyhydramnios (the fetus cannot swallow amniotic fluid) and a small or absent stomach bubble on ultrasound.

At birth or with the first feed

• Excessive frothy saliva and drooling, frequent need for suctioning — often the first sign, before any feeding
• The "3 Cs" with feeding: coughing, choking, cyanosis; milk returns through the nose and mouth
• Respiratory distress, crackles, aspiration pneumonia
• Abdominal distension (air entering the stomach through a distal fistula, especially with crying or positive-pressure ventilation) — or a scaphoid, gasless abdomen in pure EA
• In an intubated infant, gastric contents may appear in the endotracheal tube, or ventilation may be difficult because air escapes into the stomach
• H-type TEF: coughing and choking with feeds, recurrent pneumonia, and intermittent distension in an older infant

Screen for VACTERL: spine and limbs (especially thumbs and radius), anus (patent and normally placed?), heart murmur, urine output, kidney findings.

Never give the first feed to an infant with excessive drooling or respiratory distress until the esophagus is shown to be patent.

Preoperative stabilization (the priority is preventing aspiration)

• NPO immediately; IV fluids with dextrose
• Replogle (double-lumen sump) tube in the upper pouch on continuous low suction to remove saliva
• Head of bed elevated (commonly about 30–45 degrees) to reduce pooling of secretions and reflux of gastric acid through a distal fistula into the lungs
• Avoid bag-mask ventilation when possible — air forced through the fistula can distend and even perforate the stomach and worsen breathing; if ventilation is needed, experienced staff intubate and use careful pressures
• Antibiotics if aspiration pneumonia is present
• Evaluation of associated anomalies before surgery

Surgery

• Division (ligation) of the fistula and end-to-end esophageal anastomosis, through a thoracotomy (often extrapleural) or thoracoscopically, usually in the first days of life.
• Long-gap EA (usually type A or B): initial gastrostomy for feeding, continued upper-pouch suction, and delayed repair after growth or staged lengthening; esophageal replacement (stomach, colon, jejunum) if primary repair is impossible. Some infants have a cervical esophagostomy and receive sham feeds (small oral feeds that drain out the neck stoma) to keep oral skills.

Postoperative management

• Mechanical ventilation, chest tube, pain control, parenteral nutrition
• Feeding via a transanastomotic tube or gastrostomy until healing is confirmed
• A contrast esophagram (often around the end of the first week) checks for leak before oral feeding; feeds are then advanced gradually according to the surgeon's protocol

Medications

• Opioid analgesia (e.g., morphine, fentanyl) — respiratory depression, hypotension, urinary retention, tolerance and withdrawal after prolonged use; monitor sedation scores and SpO₂. Acetaminophen — dose by weight; hepatotoxicity with overdose.
• Acid suppression after repair (reflux is common and worsens strictures):
  • Proton pump inhibitors (e.g., omeprazole, lansoprazole) — associated with respiratory and GI infections (including C. difficile) and possible fracture risk with long use; use the lowest effective duration.
  • H2 blockers (e.g., famotidine) — headache, irritability; dose adjustment in renal impairment.
• Antibiotics perioperatively and for aspiration pneumonia — allergy, diarrhea.

Listed in priority order.

1. Airway and aspiration prevention (preoperative)
   • Keep NPO; maintain continuous suction of the upper pouch — check patency, flush as ordered, and note the amount and character of secretions
   • Elevate head of bed; suction the mouth and pharynx as needed; keep suction and resuscitation equipment at the bedside
   • Monitor respiratory rate, work of breathing, SpO₂, and lung sounds; report increasing distress or abdominal distension
2. Postoperative airway
   • Suction only to the premeasured depth marked by the surgical team (post a sign at the bedside) so the catheter does not reach and damage the anastomosis
   • Avoid neck hyperextension, which puts tension on the anastomosis; keep the head in neutral or slight flexion as ordered
   • Do not reinsert a dislodged transanastomotic or nasogastric tube — notify the surgeon
   • If reintubation is needed, alert the team to the anastomosis
3. Chest tube care — secure, patent system; report saliva-like or milky drainage (anastomotic leak), air leak, or sudden respiratory change
4. Fluid and nutrition — parenteral nutrition, strict intake and output, daily weight; gastrostomy care (site clean and dry, open to drainage or elevated as ordered, then feeds)
5. Pain control and comfort — use a neonatal pain scale; minimize crying
6. Transition to oral feeding — start only after the surgeon clears it; feed slowly, upright, with small volumes; watch for coughing, choking, color change
7. Family support and teaching — involve parents in care; teach gastrostomy care, suction, signs of complications, infant CPR

• Hold the baby upright during feeds and for about 30 minutes afterward (reflux is common)
• Give acid-suppressing medicine as prescribed
• Signs of stricture: difficulty swallowing, drooling, coughing or choking with feeds, food "sticking," vomiting of undigested food, poor weight gain — call the surgical team; dilations may be needed
• Tracheomalacia: a harsh, barking ("seal-like") cough is common and often improves with growth; seek emergency care for breathing pauses, blue color, or severe difficulty breathing
• When solids start: soft textures first; later cut food into small pieces, chew well, drink fluids with meals, avoid hard chunks (meat, raw vegetables, nuts)
• Report recurrent pneumonia or coughing with drinks (possible recurrent fistula)
• Gastrostomy: site care, tube securement, what to do if the tube comes out
• Keep follow-up for growth, feeding, and associated anomalies

• Early sign: excessive frothy saliva and drooling; with feeds: coughing, choking, cyanosis
• Prenatal polyhydramnios is a clue
• Most common type: proximal EA with distal TEF (air in the stomach)
• Diagnosis: orogastric tube cannot pass; X-ray shows it coiled in the upper pouch
• Preoperative priorities: NPO, continuous upper-pouch suction, head elevated, avoid bag-mask ventilation if possible
• Screen for VACTERL (vertebral, anal, cardiac, TE, renal, limb) — hearing loss is not part of it
• Postoperative: suction only to premeasured depth, no neck hyperextension, never reinsert a dislodged tube
• Oral feeds only after the surgeon confirms healing (contrast study)
• Long-term: stricture, reflux, tracheomalacia (barking cough)
• H-type TEF presents later with recurrent pneumonia