MEDICAL & SURGICAL MANAGEMENT

MEDICAL MANAGEMENT

GOAL OF TREATMENT
Because of associated persistent pulmonary hypertension of the newborn (PPHN) and pulmonary hypoplasia, medical therapy in patients with congenital diaphragmatic hernia (CDH) is directed toward optimizing oxygenation while avoiding barotrauma.

Delivery Room Management

If known or suspected to have congenital diaphragmatic hernia:
place a vented orogastric tube 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.

Mechanical ventilation strategies

targeted at avoiding high peak inspiratory pressures and synchronizing ventilation with the infant's respiratory effort. (Deprest et al. Clin
Perinatol. Jun 2009)

In some instances, high-frequency ventilation (HFV) may be helpful in avoiding the use of high peak inspiratory pressures. Early use of HFV showed 90% survival rate. (Bohn Am J Resp
Crit Care Med, 2002)

Mechanical ventilation strategies

PaO2concentrations 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. Barotrauma contributes to up to 25% of CDH deaths.

Mechanical ventilation strategies

Similarly, infants with congenital diaphragmatic hernia often have hypercarbia because of pulmonary hypoplasia. Whether to maintain a low PaCO2 for pulmonary vasodilation, to allow permissive hypercapnia, or to maintain normocarbia remains controversial. Retrospective studies suggest that gentle ventilation and permissive hypercarbia with stable hypoxemia (>80%), may be associated with improved survival (76%). (Boloker et al. J Ped Surg,
2002)

Alkalinization
Alkalinization was frequently used in the past because of its ability to produce a rapid pulmonary vasodilation. Forced alkalosis can be accomplished either by using hyperventilation to induce hypocarbia or by alkali infusions.

Alkalinization
However, benefits of alkalosis have never been demonstrated in any prospective clinical trial, and these therapies are considered controversial. In addition, alkalosis may result in undesirable side effects. For instance, hypocarbia constricts the cerebral vasculature and reduces cerebral blood flow.

Alkalinization
Extreme alkalosis and hypocarbia are strongly associated with later neurodevelopmental deficits, including a high rate of sensorineural hearing loss. Previous studies indicate that the use of alkali infusions may be associated with increased use of ECMO and an increased use of oxygen at age 28 days (Walsh-Sukys et al. Pediatrics. Jan 2000).

Fluids and electrolytes

Maintain glucose and ionized calcium concentrations within reference range. 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.

Cardiac Support
Inotropic support with dopamine, dobutamine, or milrinone may be helpful in maintaining adequate systemic blood pressure; dobutamine and milrinone may be particularly helpful if myocardial dysfunction is present. Epinephrine infusions may be necessary in severe cases; low-dose epinephrine (< 0.2 mcg/kg/min) may help to promote pulmonary blood flow and improve cardiac output.

Nitric Oxide
Nitric oxide does not reduce mortality or the need for ECMO in infants with congenital diaphragmatic hernia, although it may immediately stabilize infants with critical hypoxemia and reduce the chances of cardiopulmonary arrest. (Fliman et al. J Pediatr. May 2006)

Nitric Oxide
Inhaled nitric oxide should be used with caution if ECMO is not immediately available. New studies indicate a potential role for longterm low-dose inhaled nitric oxide therapy in the treatment of late or recurrent pulmonary hypertension.

Sedation
Sedation is an important adjunctive therapy, but the use of paralytic agents remains highly controversial. Although diminished swallowing may be beneficial, paralysis may promote:
both atelectasis of dependent lung regions and ventilation-perfusion mismatch generalized edema decreased chest wall compliance

Surfactant administration
Surfactant administration has also been shown to produce a transient improvement in oxygenation in some infants with CDH. Preliminary reports from the CDH Study Group showed surfactant administration may worsen outcome. (Lally et al. J Pediatr Surg 2004)

SURGICAL MANAGEMENT

 Theoretically, fetal surgery for congenital diaphragmatic hernia provides an elegant solution to the difficult problem of congenital diaphragmatic hernia. However, a randomized trial showed that in utero repair did not improve survival compared with standard therapy. (Harrison et al. N
Engl J Med. Nov 2003)

 Subsequent trials of fetal intervention focused on occluding the fetal trachea. The fetal lung secretes fluid by active ion transport through gestation, and this lung fluid provides a template for lung growth.

 Occlusion of the fetal trachea traps this fluid and stimulates lung growth, either by retention of growth factors within the lung or stimulation of local growth factors by the gentle distension provided by the fluid.

 Unfortunately, a randomized trial in humans found that fetal tracheal occlusion did not improve outcome compared with standard treatment. (Jelin Clin Perinatol. Jun 2009) Currently, fetal intervention is not indicated in congenital diaphragmatic hernia, although some groups continue to offer it on an experimental basis.

 Until recently, specialists believed that reduction of the herniated viscera and closure of the diaphragmatic defect should be emergently performed following birth. However, a delayed surgical approach that enables preoperative stabilization decreases morbidity and mortality.

 This change in protocol is due to the recent understanding that the medical problems of pulmonary hypoplasia and PPHN are largely responsible for the outcome of congenital diaphragmatic hernia and that the severity of these pathophysiologies is largely predetermined in utero. Herniated viscera in the chest does not appear to exacerbate the pathophysiology as long as bowel decompression with a nasogastric tube is adequate.

 Several reports indicate that circulatory stability, respiratory mechanics, and gas exchange deteriorate after surgical repair. The ideal time to repair a congenital diaphragmatic hernia is unknown. Some suggest that repair 24 hours after stabilization is ideal, but delays of up to 7-10 days are typically well tolerated, and many surgeons now adopt this approach. Some surgeons prefer to operate on these neonates when normal pulmonary artery pressure is maintained for at least 24-48 hours based on echocardiography.

 Chest tube drainage is necessary when a tension pneumothorax is present; however, whether routine chest drainage following surgical repair has a role is controversial. Some clinicians report improved survival when chest drainage is not used. Others think that balanced intrathoracic drainage, in which a closed gated pressure system is used to maintain intrathoracic pressure within the normal physiologic range, may minimize risk of pulmonary injury and improve respiratory mechanics.

Approaches for surgical repair

Abdominal subcostal
Preferred because the accompanying malrotation may be addressed if necessary, and the abdominal wall may be left open with skin only closed or a Silastic pouch applied if abdominal pressure is considered excessive

Thoracotomy

Approaches for surgical repair

Laparoscopic vs Thoracoscopic
MIS ideal for Morgagni hernias but can be challenging because the peumoperitoneum widens the defect. Laparoscopy for Bochdaleks has a high failure rate and is associated with pCO2 and acidemia. Thoracoscopy is better approach for Bochdalek hernias with recurrence of 14%. Open approach 322%.(Marjorie et al, J Ped Surg, Nov 2003.)

Approaches for surgical repair

Small defect can be repaired primarily. Large defect will require abdominal or thoracic muscle flaps, or prosthetic patch (tension free). Synthetic patch (polytetrafluoroethylene) is now preferred over autologous muscle transfer or tight primary closure for large defects.

PROBLEM LIST

Problem # 1 RESPIRATORY
S> O> A> PPHN sec to Bochdaleks Hernia P>
Dx: Tx:

Problem # 2 INFECTIOUS
S> O> A> R/O Sepsis P>
Dx: Tx:

Problem # 3 FLUIDS & ELECTROLYTES

S> O> A> R/O Sepsis P>
Dx: Tx: