Skip to Main Content
Home Books Quick Answers Surgery

Diaphragmatic Hernia, Congenital

  • • 1/2000 to 1/5000 births

    • 33% with associated congenital defects, including neural tube defects and cardiac defects

    • 80% are left-sided; 20% are right-sided

    • Right side, hepatic veins may drain ectopically into right atrium

    • 10-20% present after first 24 hrs

    • Associated with pulmonary hypoplasia on affected side

Symptoms and Signs

  • • Dyspnea

    • Chest retractions

    • Decreased breath sounds on affected side

    • Polyhydramnios prenatally (80%)

    • Cyanosis

    • Scaphoid abdomen

Laboratory Findings

  • • Respiratory acidosis

Imaging Findings

  • • Prenatal US: Accurate in 40-90% of cases showing herniation of abdominal contents in thorax

    • Chest film: Herniation of abdominal contents into thorax and associated pulmonary hypoplasia with occasional compression of mediastinum away from affected side

  • • Cardiac and neural tube defects

Rule Out

  • • Associated neural tube or cardiac defects

  • • History and physical exam

    • Chest x-ray

    • ABG measurements

    • Echocardiogram

    • US for neural tube defects

Surgery

Indications

  • • Primary repair or using mesh once respiratory status has been stabilized and optimized

Medications

  • • Mechanical ventilation if necessary

    • NG decompression

    • High frequency oscillatory ventilation if necessary

    • Extracorporeal membrane oxygenation (ECMO) if necessary

Complications

  • • Chronic lung disease

    • Skeletal muscle abnormalities

    • Acute respiratory failure postoperatively

    • Developmental delay

    • Growth delay

Prognosis

  • • 39-95% survival

Prevention

  • • In utero intervention under investigation but not yet consistently beneficial

References

+
+
Congenital Diaphragmatic Hernia Study Group: Defect Size determines survival in infants with congenital diaphragmatic hernia. Pediatrics 2007;120:3651.
+
+
Dutta S, Albanese CT: Use of a prosthetic patch for laparoscopic repair of Morgagni diaphragmatic hernia in children. J Laparoendosc Adv Surg Tech A 2007;17:391.  [PubMed: 17570796]
+
+
Harrison MR et al: A randomized trial of fetoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. New Engl J Med 2003;349:1916.  [PubMed: 14614166]

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.

This site uses cookies to provide, maintain and improve your experience. MHE Privacy Center Close