2. Tracheoesophageal fistula
1. Introduction: Tracheoesophageal fistula (TEF) is a congenital anomaly resulting from faulty embryonic development; although there are numerous “types” of TEF, the major problem is an anatomical defect that results in an abnormal connection between the trachea (respiratory tract) and the esophagus (GI system). No exact cause has been identified; however, infants born with TEF are often premature, with a maternal history of polyhydramnios. Diagnosis should be made immediately, within hours after birth, and preferably before feeding (to avoid aspiration pneumonia). Associated anomalies include: CHD, anorectal malformations, and genitourinary anomalies.
2. Assessment:
   1. Perinatal history: maternal polyhydramnios, premature birth.
   2. Most important system affected is respiratory:
      1. Shortly after birth, infant has excessive amounts of mucus.
      2. Mucus bubbles or froths out of nose and mouth as infant literally “exhales” mucus.
      3. “Three C’s”: coughing, choking, cyanosis - because mucus accumulates in respiratory tract.
      4. “Pinks up” with suctioning, only to experience repeated respiratory distress within a short time as mucus builds up again.
      5. Aspiration pneumonia occurs early.
      6. Respiratory arrest may occur.
   3. Second system affected is GI:
      1. Abdominal distention because excessive air enters stomach with each breath infant takes.
      2. Inability to aspirate stomach contents when attempting to pass NG tube.
      3. If all these signs are not correctly interpreted and feeding is attempted, infant takes two to three mouthfuls, coughs and gags, and forcefully “exhales” formula through nostrils.
3. Analysis/nursing diagnosis:
   1. Ineffective breathing pattern/ineffective airway clearance related to excess mucus.
   2. Altered nutrition, less than body requirements, related to inability to take fluids by mouth.
   3. Anxiety related to surgery, condition, preterm delivery, and uncertain prognosis.
   4. Knowledge deficit regarding discharge care of infant related to gastrostomy tube, feeding.
4. Nursing care plan/implementation:
   1. Goal: prepare neonate for surgery.
      1. Stress to parents that surgery is only possible treatment.
      2. Allow parents to see neonate before surgery to promote bonding and attachment.
      3. Maintain NPO - provide IV fluids, monitor I&O, gastrostomy tube.
      4. Position: elevate HOB 20-30 degrees to prevent aspiration.
      5. Administer warmed, humidified oxygen, as ordered, to relieve hypoxia and to prevent cold stress.
   2. Goal: postoperative - maintain patent airway.
      1. Position: elevate HOB 20-30 degrees.
      2. Care of chest tubes (open-chest procedure).
      3. Care of endotracheal tube/ventilator (neonate frequently requires ventilatory assistance for 24 - 48 hour postoperatively).
      4. Monitor for symptoms and signs of pneumonia (most common postoperative complication):
         1. Aspiration.
         2. Hypostatic, secondary to anesthesia.
      5. Monitor for symptoms and signs of respiratory distress syndrome (preterm infant).
      6. Use special precautions when suctioning: “suction with marked catheter” to avoid exerting undue pressure on newly sutured trachea.
      7. Administer prophylactic/therapeutic antibiotics, as ordered.
      8. Administer warmed, humidified oxygen, as ordered; monitor arterial blood gases (ABGs).
   3. Goal: maintain adequate nutrition.
      1. Maintain NPO for 10-14 days, until esophagus is fully healed (offer pacifier).
      2. 48-72 hour postoperatively: IV fluids only.
      3. When condition is stable: begin gastrotomy tube (G-tube) feedings, as ordered.
         1. Start with small amounts of clear liquids.
         2. Gradually increase to full-strength formula.
         3. Postoperative: leave G-tube open and elevated slightly above level of stomach to prevent aspiration if infant vomits.
         4. Offer pacifier ad lib.
      4. Monitor weight, I&O.
      5. Between 10th and 14th postoperative day: begin oral feedings.
         1. Start with clear liquids again.
         2. Note ability to suck and swallow.
         3. Offer small amounts at frequent intervals.
         4. May need to supplement postop feeding with G-tube feeding prn.
   4. Goal: prepare parents to successfully care for the infant after discharge.
      1. Teach parents that infant will probably be discharged with G-tube in place; teach care of G-tube at home.
      2. Teach parents symptoms and signs of most common long-term problem, i.e., stricture formation.
         1. Refusal to eat solids or swallow liquids.
         2. Dysphagia.
         3. Increased coughing or choking.
      3. Stress need for long-term follow-up care.
      4. Offer realistic encouragement, because prognosis is generally good.
5. Evaluation/outcome criteria:
   1. Neonate survives immediate surgical repair without untoward difficulties.
   2. Patent airway is maintained; adequate oxygenation is provided.
   3. Adequate nutrition is maintained; infant begins to gain weight and grow.
   4. Parents verbalize confidence in ability to care for infant on discharge.