Outcome of congenital diaphragmatic hernia

Abstract

The outcome of congenital diaphragmatic hernia (CDH) differs for different stages of the fetus or infant's life (i.e., antenatal, immediate postnatal, and postoperative). Assessing combined data from nonrandomized studies is technically difficult. Following recognized methods of reviewing such trials, we aimed to review the available literature on the outcome of CDH to provide a guide to clinicians when counselling parents who have a fetus/infant with this condition. Thirty-five studies reporting data for CDH from 1985 to March 1998 were identified using a high sensitive search strategy, hand-searching journals, and reviewing references of relevant studies. These were systematically reviewed.The median overall mortality was 58% (interquartile range (IQR), 43-65%) for babies diagnosed in utero, 48% (IQR, 35-55%) if born alive, and 33% (IQR, 18-54%) postoperatively. Diagnosis before 25 weeks of gestation is not a uniformly bad prognostic indicator (median mortality, 60%). Outcome was worse for those fetuses with other anomalies (median mortality, 93%). The median percentage mortality for all infants born alive and treated in extracorporeal membrane oxygenation (ECMO) centers was 34% (IQR, 26-47%). Median percentage mortality for all ECMO-treated infants was 44% (IQR, 35-50%). Different treatment strategies may have a variable impact on outcome. These figures, together with local data, may help in parental counselling on prognosis for fetuses/infants with CDH.

Factors influencing survival in newborns with congenital diaphragmatic hernia: the relative role of timing of surgery

“…This likely reflects the fact that the combined degree of pulmonary hypoplasia and pulmonary hypertension determine survival rate. 1,3 Although there were slight changes in our protocol for managing patients with CDH over the last 10 years, particularly with respect to the use of gentle ventilation, these did not affect outcome to any great degree, which remained relatively stable over the 10-year study period.…”

“…1 The timing in which surgery is performed has generated significant controversy, as many surgeons have gradually shifted away from performing an emergent repair, and instead emphasize the importance of waiting 48 hours or more for the "honeymoon period" to elapse. 2 However, survival of infants with CDH may be influenced to a greater degree by physiologic parameters that reflect the degree of pulmonary hypoplasia, thereby minimizing the overall importance of the timing of repair on survival.…”

Survival of severe congenital diaphragmatic hernia has morbid consequences

“…This anomaly carries a significant mortality of 40% to 60% [7][8][9][10][11][12]. The most severely affected fetuses with left CDH can be identified by a diagnosis at less than 25 weeks' gestational age, the presence of liver herniated into the hemithorax, and a low lung-tohead ratio (LHR) of less than 1.4 [13,14].…”

Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

“…Despite recent advances in medical and surgical management, the prognosis of such neonates continues to be uncertain because of persisting lung hypoplasia and pulmonary hypertension. 1,2 However, long-term cardiopulmonary sequelae of CDH survivors are still unclear, and understanding of developmental processes of hypoplasic lung is limited. 3 In the last decades, several studies reported the presence of persisting pulmonary abnormalities in a minority of CDH survivors, 4-8 but recent improvement in treatment led to survival of more severely affected infants, and little is known about these patients.…”