Both paralysis and eventration of the diaphragm are caused by a weakness of the diaphragmatic musculature that interferes with normal diaphragmatic function, resulting in respiratory compromise. The former is usually caused by dysfunction or iatrogenic interruption of the ipsilateral phrenic nerve, whereas the latter is more commonly because of congenital absence of functional diaphragmatic musculature. Adult patients with paralysis may have minimal or no signs of respiratory distress, whereas infants and children may present with total respiratory collapse. Although the etiology of these two processes may differ, the pathophysiology is similar, and both problems can be corrected with similar surgical techniques. Wood proposed plication of the diaphragm as a treatment for the compromised patient in 1916,1 but it was not until 1923 that Morrison reported the first successful surgical plication of the diaphragm.2 The goal of surgical plication is to stabilize the diaphragm by preventing paradoxical movement during inspiration. The technique is indicated in patients with eventration, diaphragmatic paralysis with ventilatory dependence, or symptomatic dyspnea.
Surgical intervention for congenital eventration should be offered only after a thorough evaluation has confirmed the diagnosis of diaphragmatic eventration and identified any associated anomalies. Seventy percent of affected patients have an associated congenital anomaly, such as an undescended testicle, abdominal visceral transposition, cleft palate, hypoplastic arch disorder, patent ductus arteriosus, ventricular septal defect, coarctation of the aorta, gastric volvulus, cleft lip and palate, arthrogryposis multiplex congenita, Werdnig-Hoffmann disease, hemivertebrae, horseshoe kidney, or trisomy 18 syndrome.3 Lung hypoplasia in association with any of these congenital defects is a particular concern.
Most cases of diaphragm dysfunction in children are managed without the necessity of surgical intervention. The first step is supportive care, which includes oxygen (at or above 92% saturation), elevation of the head, and nasoenteric feeding if aspiration is a concern. Continuous positive airway pressure can be added to reduce paradoxical movement of the diaphragm.6 The nasoenteric feeding tube is weaned gradually as the infant grows and the rib cage and other musculature begin to mature. Parenteral nutrition may be needed if there is gastroesophageal reflux. The use of prophylactic antibiotics is controversial, however, and should be considered only on a case-by-case basis.
If the eventration is sufficiently large that adequate ventilation becomes a critical concern, surgical treatment is warranted. Surgical repair is an emergent procedure in newborns with respiratory compromise. The infant should be intubated and surgical plication performed early.4,5 If phrenic nerve injury is suspected without identifiable cause, spontaneous recovery may occur, but surgery is recommended if diaphragmatic function on fluoroscopy fails to return within 2 weeks.3 Early intervention in neonates may prevent the complications of long-term ventilatory insufficiency because the rate of atelectasis or pneumonia will continue to increase over time, amplifying the potential risks of surgery.6,7 Recently, this approach has gained greater acceptance,8 especially in patients in whom the paralysis is caused by a complication of surgery ...