Skip to Main Content

+

  • • 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]

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessSurgery Full Site: One-Year Subscription

Connect to the full suite of AccessSurgery content and resources including more than 160 instructional videos, 16,000+ high-quality images, interactive board review, 20+ textbooks, and more.

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessSurgery

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

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