Mortality In Congenital Diaphragmatic Hernia Research Articles

Lethal nonpulmonary anomalies associated with congenital diaphragmatic hernia: Implications for early intrauterine surgery

Recently, interest has been expressed in the correction of diaphragmatic hernia in utero as a solution to the problem of high mortality from this condition. Before such a procedure can be adopted in the human, studies aimed at establishing the contribution such advanced techniques would make in reducing mortality are required. Between 1973 and 1982, there were 36 cases of congenital diaphragmatic hernia among 75,512 births--an incidence of 1 in 2097 births. there were 11 (31%) stillbirths and 25 (69%) livebirths. the liveborn patients were divided into two groups: group A included 15 patients who died prior to transfer to the referral center; group B included ten neonates who arrived at the referral center and were operated upon. Twenty (56%) cases of diaphragmatic hernia (11 stillbirths and 9 liveborns who did not live long enough to be transferred to the referral center) were found to have lethal nonpulmonary associated anomalies at autopsy. In view of the high association of lethal anomalies it appears that fetal surgery would have a limited role in reducing mortality in congenital diaphragmatic hernia.

Iatrogenic pneumothorax and mortality in congenital diaphragmatic hernia

In a review of 22 infants who underwent surgical repair of congenital diaphragmatic hernias, it was noted that one of the most common factors correlating with death was perioperative tension pneumothorax. Each of the seven infants who died developed an iatrogenic pneumothorax with air leak due to face-mask or endotracheal ventilation with pressures in excess of 34 cm H2O, or to thoracentesis. Only two infants with tension pneumothorax survived. Infants who developed tension pneumothorax experienced severe respiratory distress at an earlier age and required more vigorous resuscitative measures than those infants without an air leak.

Pulmonary and ductal hemodynamics in studies of simulated diaphragmatic hernia of fetal and newborn lambs

Congenital Posterolateral Diaphragmatic Hernia is associated with ipsilateral pulmonary hypoplasia and blood flow to the affected lung remains diminished into late childhood. 1 Although this unilateral pulmonary hypoplasia is physiologically significant, it is probably not primarily responsible for the high mortality of congenital diaphragmatic hernia. The major immediate threat to a baby's life is the tension effect of the herniated abdominal viscera, but even after successful reduction of these organs, a greater than 50% mortality occurs. The mortality and morbidity is often due to continuing and progressive hypoxemia and metabolic acidosis. Several authors 2–4 have reported pulmonary hypertension, patent ductus arteriosus and variable right-to-left shunting in babies with diaphragmatic hernias. Right-to-left shunting via a patent ductus may contribute significantly to increasing hypoxemia and acidosis in addition to the efect of pulmonary hypoplasia. To test this hypothesis, we have developed a simplified fetal model to simulate diaphragmatic hernia and permit studies of pulmonary and ductal hemodynamics. The fetal lamb was chosen for studies of the pathophysiology of diaphragmatic hernia because pulmonary development in fetal lambs occurs late in gestation and because many studies have been made of normal pulmonary function in newborn lambs. 5–8 This cumulative data is available for comparison with laboratory models of pulmornary abnormalities. These studies were therefore designed to test our belief that right-to-left shunting via the patent ductus arteriosus (PDA) is due to persistent fetal circulation and associated with altered blood flow to the hypoplastic lung.