Updated: 5/6/2020

Congenital Diaphragmatic Hernia

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  • A newborn infant girl is evaluated 2 hours after birth for signs of trouble breathing. The patient was born via an uncomplicated vaginal delivery at 39 weeks of gestation to a 32-year-old mother. The girl's APGAR scores were 9 at 1 minute of life and 10 at 5 minutes after delivery, with a score of 2 for respiration with a vigorous cry at both time points. Her temperature is 96.9°F (36.1°C), pulse is 168/min, and respirations are 58/min. On physical examination, the patient's point of maximal impulse is markedly displaced to the right and lung sounds are absent on the left side. A chest radiograph is obtained and shown in the image.
  • Overview
    • a congenital diaphragmatic hernia occurs due to congenital defects in the diaphragm, causing bowel to protrude through the diaphragm into the thorax
  • Epidemiology
    • incidence
      • 1 in 3000 newborns
    • demographics
      • 90% of patients present in the neonatal period or within 1 year of age
    • location
      • commonly presents on the left posterolateral side
  • Pathophysiology
    • muscular entities and pleuroperitoneal membrane of the diaphragm fail to develop normally
      • diaphragm develops between 4-12 weeks of gestation
        • Bochdalek hernia
          • failure of pleuroperitoneal membrane closure in utero causes a defect in the posterior attachment of the diaphragm
        • Morgagni hernia
          • failure of formation of the anteromedial portion of the diaphragm, where the septum transversum and the anterior-inferior thoracic wall intersect
      • results in displacement of bowel into the thorax
  • Symptoms
    • dyspnea and/or respiratory distress
    • cyanosis
      • occurs within the first 24 hours of life if neonatal onset
    • chest tightness or fullness
  • Physical exam
    • ↓ breath sounds on the affected side
    • bowel sounds in the thorax
    • displaced point of maximal impulse
    • cyanosis
    • scaphoid abdomen
      • due to herniation of abdominal contents into thorax
  • Chest radiograph
    • findings
      • bowel and stomach are present in the chest cavity
      • shifting of the mediastinum to one side
  • Medical
    • resuscitation with ventilatory support
      • indications
        • respiratory distress
      • modalities
        • conventional mechanical ventilation
        • high-frequency oscillatory ventilation (HFOV)
    • ECMO (extracorporeal membrane oxygenation)
      • indications
        • reserved for patients who fail to improve with both conventional mechanical ventilation and HFOV
    • nasogastric tube for decompression
  • Surgical
    • surgical repair of the hernia
      • definitive treatment
  • Small bowel obstruction
    • risk factors
      • late-presenting congenital diaphragmatic hernia in neonates
      • left-sided hernia associated with ↑ bowel complications (liver protects from herniation on the right side)
      • adhesions from surgical repair of hernia
    • treatment
      • surgery for small bowel obstruction repair

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