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CDH Etiology

Failure of closure of the pleuro-peritoneal canal
(8-10 weeks)
Development of the diaphragmatic primordium (3-4 weeks).
At the end of this phase the primitive diaphragm is composed of two parts:
1. Ventral transverse septum
2. Dorsal diaphragmatic mesentery

Development of the pleural cavity and closure of the pleuro-peritoneal canals
(4 to 8 weeks)
The diaphragm is formed from the septum transversum and the dorsal mesentery, which separate the thoracic system from the abdominal organs. The poster-olateral areas are the last to close and the left side closure occurs later than the right.

Different concept of development (Iritani, 1984)
Primitive diaphragm consisted of 3 parts:
1. Transverse septum ventrally
2. Pleuroperitoneal canals caudally
3. Posthepatic mesenchymal plate (interposed), leads to the closure of the canals.

Pathophysiology (dynamic lesion)
– Migration of intraperitoneal structures in the hemithorax leads to compression and secondary pulmonary hypoplasia.
– V/Q mismatch
– Pulmonary hypertension
– Relative left ventricular hypoplasia
– Right to left shunt through foramen ovale or ductus arteriosus
– Surfactant deficiency

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