CONGENITAL DIAPHRAGMATIC HERNIA
Category: Child Health
Abstract : congenital diaphragmatic hernia treatment - Medical Care: Because of associated PPHN and pulmonary hypoplasia, medical therapy is directed toward optimizing oxygenation while avoiding barotrauma. • If the infant has CDH, or if the diagnosis is suspected in the delivery room, immediately place a vented orogastric tube and connect it to continuous suction to prevent bowel distension and fu
congenital diaphragmatic hernia treatment - Medical Care: Because of associated PPHN and pulmonary hypoplasia, medical therapy is directed toward optimizing oxygenation while avoiding barotrauma.
• If the infant has CDH, or if the diagnosis is suspected in the delivery room, immediately place a vented orogastric tube and connect it to continuous suction to prevent bowel distension and further lung compression.
For the same reason, avoid mask ventilation and immediately intubate the trachea. Avoid high peak inspiratory pressures and alert the resuscitation team to the possibility of early pneumothorax if the infant does not stabilize. Many infants benefit from exogenous surfactant administration during the first few hours of life.
o Infants with CDH have immature lung development and may be surfactant deficient. Administration of exogenous surfactant in the delivery room or shortly thereafter may be useful.
o Inhaled nitric oxide may be used in infants with hypoxia not responding to assisted ventilation and surfactant therapy to lower their pulmonary vascular resistance. However, the use of nitric oxide has not been shown to reduce mortality or the need for extracorporeal membrane oxygenation (ECMO) in infants with CDH. Therefore, it should be used with extreme caution if ECMO is not immediately available.
o Use ECMO when optimal ventilator and medical therapy do not maintain acceptable oxygenation and perfusion. Fewer than 100 centers in the United States provide ECMO, which is an adaptation of cardiopulmonary bypass performed via catheters inserted into the neck vessels. Recent developments allow support with a double lumen catheter in the internal jugular vein, thus avoiding ligation of the right common carotid artery.
• Provide meticulous attention to detail for subsequent medical care, including continuous monitoring of oxygenation, blood pressure, and perfusion. Provide care using a minimal stimulation approach, which reduces handling and invasive procedures such as suctioning. Maintain reference range glucose and ionized calcium concentrations. If necessary, support blood pressure using volume expansion and inotropic agents. An adequate circulating volume is necessary to maintain right ventricular filling and cardiac output; however, once circulating volume is normalized, repeated boluses of crystalloid solutions, colloid solutions, or both do not provide additional benefit. Inotropic support with dopamine, dobutamine, or both may be helpful in maintaining adequate systemic blood pressure while avoiding excessive volume administration.
• Mechanical ventilation is almost always required. Target ventilator strategies to avoid high peak inspiratory pressures and synchronize ventilation with the infant's respiratory effort. High-frequency ventilation may be helpful in some instances to avoid the use of high peak inspiratory pressures, although this modality is best used at a center with experience in assessing and maintaining optimal lung distension.
• The appropriate targets for PaO2 and PaCO2 are controversial. PaO2 concentrations greater than 50 mm Hg typically provide for adequate oxygen delivery at the tissue level. Aiming for higher PaO2 concentrations may lead to increased ventilator support and barotrauma. Similarly, because of pulmonary hypoplasia, infants with CDH often have hypercarbia. Whether to maintain a low PaCO2 for pulmonary vasodilation (see below), to allow permissive hypercapnia, or to maintain normocarbia is controversial. No reliable controlled studies exist, and debate continues in the medical literature.
• Alkalinization is a popular therapy because of its ability to produce a rapid pulmonary vasodilation. Forced alkalosis can be accomplished using hyperventilation and hypocarbia by alkali infusions or their combination, although benefits have never been demonstrated in any prospective clinical trial. In many centers, these therapies are considered controversial. For instance, hypocarbia constricts the cerebral vasculature and reduces cerebral blood flow. Extreme alkalosis and hypocarbia are strongly associated with later neurodevelopmental deficits, including a high rate of sensorineural hearing loss. A recent study by Walsh-Sukys and colleagues indicates that the use of alkali infusions may be associated with increased use of ECMO and an increased use of oxygen at 28 days of age.
• The use of paralytic agents is also highly controversial. Paralysis may promote both atelectasis of dependent lung regions and ventilation-perfusion mismatch.
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