General information
The hole in the diaphragm must be repaired surgically within the first few days of the baby’s life. The displacement of abdominal organs into the chest, however, presents a serious problem. In severe cases, the displaced organs take up the room the lungs need to develop normally. The lungs are an essential organ for the newborn immediately after birth and removal of the umbilical cord. The baby’s prognosis will depend on the size of the hole in the diaphragm and how much the abdominal contents herniated into the chest cavity. This means that it is not only the size of the hole that is important, but also the clinical implications of the diaphragmatic defect. Unborn babies and newborns with CDH have abnormalities of the pulmonary blood vessels: there are fewer blood vessels and their muscular layer is thicker. In addition, the bronchial tree has fewer airway branches and the blood-gas barrier is thicker than normal. In many cases of severe CDH, these anatomical abnormalities prevent normal and effective gas exchange immediately after birth and removal of the umbilical cord.
The chances of survival of a newborn baby depend on how severe is the lung underdevelopment (hypoplasia), which depends on the size of the hole in the diaphragm, and how much the abdominal organs herniated into the chest. Once born, your baby will require intensive neonatal care, and most likely intubation and mechanical ventilation. Sadly, babies with a severe form of CDH, associated with greater degrees of lung hypoplasia, have a poor prognosis of survival. In extremely severe forms of CDH, the prognosis is very poor and the survival rates can be as low as 10% without appropriate treatment. These survival rates can be improved up to 50% with in-utero procedures (FETO) and appropriate neonatal care. It is therefore essential to choose the right healthcare center with an experienced team of specialists in maternal fetal medicine, neonatologists, cardiologists and surgeons.