Congenital Diaphragmatic Hernia: Experience with Preoperative Stabilization and Delayed Surgery without ECMO and Inhaled Nitric Oxide

Abstract

Despite many advances in the management of congenital diaphragmatic hernia (CDH), the condition carries a mortality rate of 40-50% usually consequent to pulmonary hypoplasia and/or persistent pulmonary hypertension. Several centers have reported improved survival with preoperative stabilization and delayed surgery, which is now an accepted method of management. This is a retrospective analysis of all neonates with respiratory distress at birth due to CDH who were treated at our institution with neither extracorporeal membrane oxygenation nor nitric oxide being used. The medical records of all neonates with CDH and respiratory distress at birth who were treated at this institution from August 1, 1992 through March 1, 1997 were reviewed. There were 21 patients, 11 male and 10 female. There were 17 full-term and 4 premature infants; two premature infants at 30 and 34 weeks' gestation were not resuscitated because of severe associated congenital anomalies. Surgery was performed from 5 to 144 hr (mean 45 hr) in 18 infants. One infant died during preoperative stabilization from severe pulmonary hypoplasia and pulmonary hypertension and one infant died postoperatively from the same conditions. Seventeen of 19 infants (89.5%) survived and were discharged home. Three infants (17.6%) who failed to thrive due to severe gastroesophageal reflux (GER) required fundoplication. Eleven infants (64.7%) who had sepsis proven by blood culture responded satisfactorily to appropriate antibiotics. Preoperative stabilization and delayed surgery has been a satisfactory form of management in our series. The significant complication was sepsis, which must be addressed.

The immediate and long-term outcomes of newborns with congenital diaphragmatic hernia

“…The aim of ventilatory support was to maintain the blood pH between 7.45 and 7.55, the PaO 2 above 100 mmHg, the SaO 2 above 96% and PaCO 2 between 25 and 35 mmHg. The preoperative management has already been reported [1]. Since 2003, permissive hypercapnia has been used instead of hyperventilation; however, none of the patients were included in this review.…”

“…Although improved survival of up to 90% has been reported in some centers using delayed surgery [1][2][3], this has not been the experience of other groups [5,15]. Delayed surgery allows for stabilization of CDH newborns with pulmonary hypoplasia and associated pulmonary hypertension and also avoids surgery in newborns that are most likely to die.…”

“…Staphylococci are a common cause of hospital-acquired infections in many NICUs [25]. Al-Hathal et al [1] reported coagulase-negative staphylococci as the causative organism in approximately 82% of CDH patients with proven sepsis. In this series, the same organism was the cause of sepsis in 24% of newborns with CDH.…”

“…With the management of CDH including delayed surgery, extracorporeal membrane oxygenation (ECMO), high-frequency oscillatory ventilation (HFOV), inhaled nitric oxide (NO), exogenous surfactant, partial liquid ventilation and synchronized ventilation, the survival rate ranges from 53 to 90% [1][2][3][4][5][6][7][8]. After Sakai et al [9] reported that early surgical repair may reduce pulmonary compliance, impede mechanical ventilation and increase circulatory instability, the treatment of CDH is no longer considered a surgical emergency.…”

“…After Sakai et al [9] reported that early surgical repair may reduce pulmonary compliance, impede mechanical ventilation and increase circulatory instability, the treatment of CDH is no longer considered a surgical emergency. Since the early 1990s delayed surgery has been the accepted management of newborns with CDH at King Fahad National Guard Hospital, Riyadh, Saudi Arabia [1].…”