More than 50% of cases with CDH are detected prenatally by ultrasound examination.
MRI is especially useful for the prenatal diagnosis of
multiple abnormalities and for assessing lung volumes.
CDH occurs in
every 1-2500 live births in the United States.
CDH accounts for 8% of
all major congenital anomalies.
Approximately 1,600 babies are born each
year with CDH.
After having a baby with CDH the chances of their sibling
diagnosed with CDH is 2%.
The chances of reherniation for a baby
born with CDH are as high as 60%. Those with a patch are slightly higher.
Combining all hernia types, CDH occurs approximately 85%of the time on
the left side, 10% on the right side, and less than 5% of the time bilaterally.
Mortality from CDH continues to be high, ranging from 20% to 60%.
determinants of mortality in CDH are:
•Whether the CDH is isolated or
complex. Higher mortality occurs with complex CDH associated with a chromosome
abnormality, a single gene disorder, and/or the coexistence of major
malformations. The presence of a cardiovascular malformation also indicates a
•The size of the diaphragm defect.
•The degree of
•Whether the liver is up in the chest or remains
down below the diaphragm. Individuals with a large amount of "liver up" CDH have
higher mortality than those whose liver remains down below the
•The severity of pulmonary hypertension in the perinatal
period. Pulmonary hypertension, which may progress to a late or chronic phase,
is often not responsive to medical therapy.
•Whether the hernia is
right-sided, left-sided, or bilateral. Some, but not all, studies show that a
right-sided hernia is associated with greater mortality than a left-sided
hernia. Bilateral CDH always confers a high mortality.