Search Encyclopedia:    
List of Topics Print This Page
 

Diaphragmatic hernia


Definition:

A diaphragmatic hernia is a birth defect in which there is an abnormal opening in the diaphragm, the muscle that helps you breathe. The opening allows part of the organs from the belly (stomach, spleen, liver, and intestines) to go up into the chest cavity near the lungs.

Alternative Names:

Hernia - diaphragmatic; Congenital hernia of the diaphragm

Causes, incidence, and risk factors:

A diaphragmatic hernia is caused by the improper joining of structures during fetal development. As a result, the abdominal organs such as the stomach, small intestine, spleen, part of the liver, and the kidney appear in the chest cavity. The lung tissue on the affected side is thus not allowed to completely develop.

Congenital diaphragmatic hernia is seen in 1 out of every 2,200 to 5,000 live births. Most affect the left side. Having a parent or sibling with the condition slightly increases your risk.

Symptoms:

Severe breathing difficulty almost always develops shortly after the baby is born, because of ineffective movement of the diaphragm and crowding of the lung tissue, which causes collapse.

Other symptoms include:

Signs and tests:

The pregnant mother may have excessive amounts of amniotic fluid. Fetal ultrasound may show abdominal contents in the chest cavity.

Examination of the infant shows:

  • Irregular chest movements
  • Absent breath sounds on affected side
  • Bowel sounds heard in the chest
  • Abdomen feels less full on examination by touch (palpation)

A chest x-ray may show abdominal organs in chest cavity.

Treatment:

A diaphragmatic hernia is an emergency that requires surgery. Surgery is done to place the abdominal organs into the proper position and repair the opening in the diaphragm.

See: Diaphragmatic hernia repair - congenital

The infant will need breathing support until he or she recovers from surgery. Some infants are placed on a heart/lung bypass machine, which gives the lungs a chance to recover and expand after surgery.

If a diaphragmatic hernia is diagnosed during pregnancy (around 24 to 28 weeks), fetal surgery may be considered.

Expectations (prognosis):

The outcome of surgery depends on how well the baby's lungs have developed and also on whether there are any other congenital problems. Usually the outlook is very good for infants who have enough lung tissue and have no other problems.

With advances in neonatal and surgical care, survival is now greater than 80%.

Complications:
  • Lung infections
  • Other congenital problems
Calling your health care provider:

Go to the emergency room or call the local emergency number (such as 911). A diaphragmatic hernia is a surgical emergency.

Prevention:

There is no known prevention.

References:

Ehrlich PF, Coran AG. Diaphragmatic hernia. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 101.

Keijzer R, Puri P. Congenital diaphragmatic hernia. Semin Pediatr Surg 2010 Aug; 19(3): 180-5.

Puri P, Nakazawa N. Congenital diaphragmatic hernia. In: Puri P, Hollworth M, eds. Pediatric surgery: diagnosis and management. Springer, 2009: chapter 31.


Review Date: 6/18/2011
Reviewed By: Kimberly G Lee, MD, MSc, IBCLC, Associate Professor of Pediatrics, Division of Neonatology, Medical University of South Carolina, Charleston, SC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

   View History
  Diaphragmatic hernia

   
   

 

About UPHS   Contact Us   Site Map   Privacy Statement   Legal Disclaimer   Terms of Use

The University of Pennsylvania Health System, Philadelphia, PA 1-800-789-PENN © 2014, The Trustees of the University of Pennsylvania