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  • • 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

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Symptoms and Signs

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  • • Dyspnea

    • Chest retractions

    • Decreased breath sounds on affected side

    • Polyhydramnios prenatally (80%)

    • Cyanosis

    • Scaphoid abdomen

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Laboratory Findings

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  • • Respiratory acidosis

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Imaging Findings

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  • 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

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  • • Cardiac and neural tube defects

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Rule Out

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  • • Associated neural tube or cardiac defects

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  • • History and physical exam

    • Chest x-ray

    • ABG measurements

    • Echocardiogram

    • US for neural tube defects

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Surgery

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Indications

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

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Medications

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  • • Mechanical ventilation if necessary

    • NG decompression

    • High frequency oscillatory ventilation if necessary

    • Extracorporeal membrane oxygenation (ECMO) if necessary

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Complications

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  • • Chronic lung disease

    • Skeletal muscle abnormalities

    • Acute respiratory failure postoperatively

    • Developmental delay

    • Growth delay

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Prognosis

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  • • 39-95% survival

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Prevention

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

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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]

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