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Information

  1. CLEFT LIP AND CLEFT PALATE
    1. Introduction: Cleft lip and cleft palate are congenital facial malformations resulting from faulty embryonic development; there appear to be multiple factors involved in the exact etiology: mutant genes, chromosomal abnormalities, teratogenic agents, etc. The infant may be born with cleft lip alone, cleft palate alone, or with both cleft lip and cleft palate. Cleft lip and palate may occur unilaterally or bilaterally. Table 5-15. Comparison of Cleft Lip and Cleft Palate compares these conditions.
    2. Assessment:
      1. Cleft lip—obvious facial defect, readily detectable at time of birth.
      2. Cleft palate—must feel inside infant's mouth to check for presence of palatal defect and to note extent of defect: soft palate only or soft palate and hard palate.
      3. Both—major problems with feeding: difficult to feed, noisy sucking, swallows excessive amounts of air, prone to aspiration.
      4. Parent-infant attachment (bonding) may be adversely affected due to "loss of perfect infant," multiple hospitalizations; note amount and quality of parent-infant interaction.
    3. Analysis/nursing diagnosis:
      1. Altered nutrition, less than body requirements, related to physical defect.
      2. Impaired physical mobility (postoperative) related to postoperative care requirements.
      3. Altered parenting related to birth of child with obvious facial defect.
      4. Knowledge deficit, actual or risk for, potential, related to treatment and follow-up.
    4. Nursing care plan/implementation:
      1. foodImageGoal: maintain adequate nutrition.
        1. Preoperative: first encourage parents to watch nurse feed infant, then teach parents proper feeding techniques:
          1. Use Breck feeder or Asepto syringe.
          2. Deposit formula on back of tongue to facilitate swallowing and to prevent aspiration.
          3. Rinse mouth with sterile water after feedings, to prevent infection.
          4. Feed slowly, with child in sitting position, to prevent aspiration.
          5. Burp frequently, because infant will swallow air along with formula due to the defect.
          6. Monitor weight.
        2. Postoperative
          1. Begin with clear liquids when child has fully recovered from anesthesia (see Table 5-15. Comparison of Cleft Lip and Cleft Palate ).
          2. Monitor weight gain carefully, to ensure adequate rate of growth.
          3. No sucking for either cleft lip or palate repair until incision is healed.
          4. Avoid stretching or pulling at incision site; metal "Logan bow" may be used as external brace for cleft lip repair.
      2. Goal: promote parent-infant attachment.
        1. Show no discomfort handling infant; convey acceptance.
        2. Stay with parents the first time they see/hold infant.
        3. Offer positive comments about infant.
        4. Give positive reinforcement to parents' initial attempts at parenting.
        5. Encourage parents to assume increasing independence in care of their infant.
        6. Allow rooming-in on subsequent hospitalizations.
      3. Goal: teach parents particulars of feeding and need for long-term follow-up care.
        1. Teach parents regarding long-term concerns (see Table 5-15. Comparison of Cleft Lip and Cleft Palate ).
        2. Make necessary referrals before discharge:
          1. Specialists: speech, dentition, hearing.
          2. Home health nurse.
          3. Social service.
          4. Disabled children's services for financial assistance.
          5. Local craniofacial malformations support group.
        3. Refer parents to genetic counseling services because of mixed genetic/environmental etiology.
        4. Encourage parents to promote self-esteem in infant/child as child grows and develops.
    5. Evaluation/outcome criteria:
      1. Adequate nutrition is provided, and infant grows at "normal" rate for age.
      2. Parent-infant attachment is formed.
      3. Parents verbalize confidence in their ability to care for infant.
  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) (Figure 5-9. Esophageal Malformations). 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. bulbImageThe "3 Cs": 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. pillImage Maintain NPO—provide IV fluids, monitor I&O, gastrostomy tube.
        4. infoImage  Position: elevate HOB 20 to 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. infoImage  Position: elevate HOB 20 to 30 degrees.
        2. Care of chest tubes (open-chest procedure).
        3. Care of endotracheal tube/ventilator (neonate frequently requires ventilatory assistance for 24 to 48 hours 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. pillImage Administer prophylactic/therapeutic antibiotics, as ordered.
        8. Administer warmed, humidified oxygen, as ordered; monitor arterial blood gases (ABGs).
      3. foodImageGoal: maintain adequate nutrition.
        1. pillImage48 to 72 hours postoperatively: IV fluids only.
        2. Maintain NPO for 10 to 14 days, until esophagus is fully healed (offer pacifier).
        3. When condition is stable: begin gastrostomy 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. 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.
        5. Monitor weight, I&O.
      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 problems (i.e., esophageal reflux, 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.