Treatment

Whilst the definitive treatment of diaphragmatic hernia is surgical closure, the timing of this is not of paramount importance and should be undertaken once the infant is stable from a cardiovascular and respiratory point of view. The respiratory management of these infants can be problematic owing to the severe lung hypoplasia and associated pulmonary hypertension. Most require conventional ventilatory support as a minimum and many require high-frequency oscillatory ventilation to ensure adequate oxygenation. Other measures to reduce pulmonary hypertension, including inhaled nitric oxide, adenosine or sil-denafil, may be effective. Resistant cases may be candidates for extracorporeal membrane oxygenation, during which the infant is placed on a life-support system in the hope that the lungs and in particular the pulmonary vasculature will mature. Various criteria for extracorporeal membrane oxygenation exist47 with the aim of

Figure 2.7 Chest radiograph of an infant with congenital diaphragmatic hernia. Loops of intestine are clearly seen within the left hemithorax and there is mediastinal shift to the right.

reserving it for those who have the most severe respiratory failure and those who are most likely to benefit. Unfortunately, there remain a number of infants with congenital diaphragmatic hernia whose lung disease presents too great a challenge and these do not survive.

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