Congenital Diaphragmatic Hernia

Congenital Diaphragmatic Hernia is present in approximately 1 in every 3000 births. A Diaphragmatic Hernia is defined as a “hole” located in the diaphragm (the large muscle that separates the abdominal cavity form the chest cavity) that allows the abdominal organs including the bowel, stomach, spleen, and sometimes the liver to move upward into the chest cavity. Congenital refers to the fact that the defect is present at birth. Because this defect occurs during the fetal period, having the abdominal contents in the chest cavity can severely restrict the growth of the lung on the affected side. Infants born with Congenital Diaphragmatic Hernia usually present with profound respiratory distress after birth. This defect may or may not be associated with other congenital abnormalities.

Because of the seriousness of CDH at birth some infants require emergent intubation (placement of a breathing tube), administration of a pulmonary vasodilation drug known as Nitric Oxide, placement on an ECMO circuit (heart/ lung bypass machine), and/or surgery within the first few weeks of life. The availability of treatments such as ECMO and Nitric Oxide has given doctors the ability to stabilize babies for a few hours or days prior to surgically repairing the defect. Because these babies are prone to having a problem known as pulmonary hypertension (high pressures in the lungs that make ventilating the babies difficult) utilizing these treatments prior to surgical repair has increase the survival rate for these infants who once saw a mortality of 40-50%.

Once the infant is stabilized and lung pressures are improved the Doctor will recommend surgical closure of the defect. Surgical treatment for CDH includes a Thoracotomy (and incision on the effected side of the rib cage). The hole in the diaphragm is located and the abdominal organs are gently placed by the surgeon back in place in the abdominal cavity. A patch made from Gortex is sewn over the hole separating the abdominal cavity from the chest cavity. Alternatively, an incision is made in the abdomen (Laparotomy), and the repair is made through the abdomen instead of the chest. Once the surgery is complete the affected lung may or may not expand/ grow to fill the chest cavity, depending on the number on abdominal organs located in the chest cavity during development of the lungs.

Long term survival for infants with Congenital Diaphragmatic Hernia is related to the size of the lungs at the time of the repair. Some survivors have long term evidence of restrictive lung disease. Survival rates for severe CDH are increasing with the utilization of pre- operative ECMO and/ or Nitric oxide. Our experience with Congenital Diaphragmatic Hernia’s, consist of 65 patients with 10% of these patients requiring ECMO support before the surgical repair. Survival is close to 100% for babies not requiring ECMO, with the number decreasing to 70- 80% when ECMO is needed.

Contact Information:

John Mark Morales MD, FACS, FAAP
Chief of Cardiothoracic, Director of Perfusion Services
Certified by the American Board of Surgery, American Board of Thoracic Surgery

Mark Bielefeld, MD
Driscoll Children's Hospital Chief of Staff
Certified by the American Board of Surgery, American Board of Thoracic Surgery

Thoracic surgeons are available for questions and consultations: (361) 854-0201. For appointments, assistance, and physician references in Corpus Christi call: (361) 854-0201 or 800-DCH-LOVE
Fax : 361-855-7572
E-MAIL : jmarkmorales@aol.com

For further information on any surgical procedures you can contact Carol Kaplan, RN, Surgical Nurse Liaison at (361) 694-5150. Consultation and surgery for inpatients is provided in concert with neonatology and pediatric cardiology departments. Complete evaluation and management for infants, children, adolescents and adults with congenital or acquired cardiac, vascular or thoracic anomalies.

Cardiothoracic Associates
3533 S. Alameda, Suite 202
Corpus Christi, Texas 78411
Phone: (361) 694-5150
Fax: (361) 855-7572
Hours: 9am to 6pm
Fri 9am to 5pm

Congenital Diaphragmatic Hernia

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