What is CDH?

What is Congenital Diaphragmatic Hernia (CDH)

Congenital diaphragmatic hernia, or CDH, is one of the most common birth defects, affecting about one in 2,500 newborns today. CDH occurs when the diaphragm develops abnormally during gestation (around week 7-8), creating a defect, or hole, through which organs such as the intestines migrate into the chest. The result is compression and impairment in the development of the lungs, including pulmonary hypertension, a dangerous condition involving high blood pressure in the lungs. Among babies with CDH, pulmonary hypertension can lead to heart failure and is the most significant cause of illness and death. Fortunately, medicine has made tremendous progress against pulmonary hypertension during the past 25 years.

                   

CDH Facts: 

  • "Less than two decades ago, an infant born with CDH had a 50-50 chance of survival."  (1)
  • “Overall, worldwide survival of infants born with diaphragmatic hernia has increased from about 50 percent to 68 percent in the last 20 years.” (2)
  • "In CDH, most (85%) lesions are left sided, 13% are right-sided, and 2% bilateral." (3)
  • "Diaphragmatic hernia is caused by a failure of the diaphragm to close or to form, for unknown reasons, at approximately eight weeks’ gestation. Most commonly seen on the left side of the body, the defect may also occur on the right side or in the central portion of the diaphragm. The opening left in the diaphragm allows abdominal organs to herniate into the chest cavity, leading to pulmonary hyperplasia. Complications arise when the lungs fail to develop and the exchange of gases necessary for survival is restricted." (4)
  • “The lungs of infants with CDH are developmentally retarded, with fewer alveoli, thickened alveolar walls, increased interstitial tissue, markedly diminished alveolar air space and gas-exchange surface area." (5)
  • Combining all hernia types, CDH occurs 80-90% of the time on the left side, 10-20% on the right side, and less than 5% of the time bilaterally 
  • Infants with CDH often present in the neonatal period with severe respiratory distress; pulmonary hypoplasia is common 
  • Newborns with CDH are intubated immediately to avoid bag-mask ventilation and inflation of the bowel that has herniated into the chest 
  • Almost all individuals with CDH have some degree of pulmonary hypoplasia. Many infants require oxygen supplementation and diuretics following surgical correction of CDH 
  • The male:female ratio is reported as 1.25 ,the recurrence risk is estimated at approximately 2%, the median overall mortality rate is 58% for babies diagnosed in utero, 48% if born alive, and 33% postoperatively 
  • "Failure to thrive" with growth parameters less than the third centile of normal is common among infants with more significant pulmonary hypoplasia and/or a more prolonged hospitalization following surgical repair of CDH
  • With the proper medical care, resources, emotional support, and the strength which these babies possess...they will overcome their struggle to stay alive and provide joy and smiles to all...

References:

(1) Lally, Kevin, MD.  “Congenital Diaphragmatic Hernia: Toward Better Evidence-Based Management.” Children’s Memorial Hermann Physicians Advance: Advancing the Frontiers of Medicine  (Jan, 2007) A3

(2) Ibid, A2

(3)Deprest, Jan, MD; Jani, Jacques, MD; Gratacos, Eduardo, MD, Vandecruys, Hilde, MD, Naulaers, Gunnar, MD; Delgado, Julian, MD; Greenough, Anne, MD; Nicolaides, Kypros, MD; and The FETO Task Group. “Fetal Intervention for Congenital Diaphragmatic Hernia: The European Experience.”  Seminars in Perinatology 29:2 (2005) 95

(4) Lally, Kevin, MD.  “Congenital Diaphragmatic Hernia: Toward Better Evidence-Based Management.” Children’s Memorial Hermann Physicians Advance: Advancing the Frontiers of Medicine  (Jan, 2007) A2

(5) Deprest, Jan, MD; Jani, Jacques, MD; Gratacos, Eduardo, MD, Vandecruys, Hilde, MD, Naulaers, Gunnar, MD; Delgado, Julian, MD; Greenough, Anne, MD; Nicolaides, Kypros, MD; and The FETO Task Group. “Fetal Intervention for Congenital Diaphragmatic Hernia: The European Experience.”  Seminars in Perinatology 29:2 (2005) 95