Congenital
Diaphragmatic Hernia
A congenital hernia of the diaphragm (CDH) develops as a result of a
primary developmental diaphragmatic defect that allows proximal herniation of
abdominal organs into the thoracic cavity. Approximately 75% of the time, the
diaphragmatic defect is posterolateral (Bochdalek hernia) and typically
on the left side, whereas 25% are anteromedial (Morgagni hernia).
CDH
may present as respiratory disease due to extrinsic compression of abdominal
organs on the developing lungs with pulmonary hypoplasia. Association of CDH
with multiple welldefined genetic syndromes, chromosomal abnormalities, or
other congenital anomalies strongly suggests a genetic predisposition. Although
the stomach is the most common abdominal organ to herniate into the chest, the
small and large bowel, liver, spleen, and pancreas may also herniate. Diagnosis
is typically made with prenatal ultrasound or is suspected at birth due to pulmonary
compromise. In some patients, the presentation may be much later in life,
including adulthood. Symptoms in later life include postprandial upper
abdominal and chest pain or acute symptoms of incarceration. Diagnosis is best
made through computed tomography of the chest and/or barium studies. The only
effective treatment is surgery; visceral organs are reduced into the abdomen,
and diaphragmatic defects are either oversewn or patched, depending on the size of
the defect.
