Congenital diaphragmatic hernias are seen in 1 of every 2000-4000 live births.
84% are left-sided,
13% are right-sided and 2% bilateral (usually fatal).
There are 3 basic types of congenital diaphragmatic hernia: posterolateral Bochdalek hernia,
anterior Morgagni hernia and hiatus hernia.
Hiatal hernias account for only 9% of diaphragmatic hernias in infants younger than 1year.
Congenital Diaphragmatic hernia (CDH) is characterized by a defect in the diaphragm leading to the protrusion of abdominal contents into the thoracic cavity affecting the normal development of the lungs.
The condition may present as an isolated lesion or as part of a syndrome.
It is one of the most common non-cardiac fetal intrathoracic anomalies.
It accounts for 8% of all major congenital anomalies.
Left-sided hernias allow herniation of both the small and large bowel and intraabdominal solid organs into the thoracic cavity.
In right-sided hernias,
only the liver and a portion of the large bowel tend to herniate.
Infants with CDH most commonly present with respiratory distress and cyanosis in the first minutes or hours of life,
although a later presentation is possible.
The respiratory distress can be severe and may be associated with circulatory insufficiency,
requiring aggressive resuscitative measures.
The degree of respiratory distress is dependent on the severity of lung hypoplasia and the development of persistent pulmonary hypertension of the newborn (PPHN).
The most common content of hiatus hernia is the stomach.
It is divided into four types:
Type 1: sliding hiatal hernia (95%)
Type 2: para-esophageal hiatal hernia with the gastro-esophageal junction in a normal position.
Type 3: mixed or compound type,
para-esophageal hiatal hernia with displaced gastro-esophageal junction.
Type 4: mixed or compound type hiatal hernia with additional herniation of viscera.
Other thoracic lesions — congenital pulmonary airway malformation,
bronchopulmonary foregut malformation,
Diaphragmatic eventration — Diaphragmatic eventration refers to elevation of a portion of the diaphragm that is intact but thinned because of incomplete muscularization.