Prenatal Diagnosis and Perinatal Outcome of 38 Cases With Congenital Diaphragmatic Hernia: 8-Year Experience of a Tertiary Brazilian Center

Abstract

diagnosis and perinatal outcome of 38 cases with congenital diaphragmatic hernia: 8-year experience of a tertiary Brazilian center. Clinics. 2006;61(3):197-202. PURPOSE:To evaluate the perinatal results for neonates with congenital diaphragmatic hernia diagnosed prenatally. METHOD: We reviewed data from 38 cases of congenital diaphragmatic hernia diagnosed prenatally from January 1995 to December 2003 in the Fetal Medicine Unit of the Department of Obstetrics and Gynecology, São Paulo University Medical School. The main data analyzed were gestational age at diagnosis, fetal karyotyping, side of diaphragmatic defect, presence of associated structural malformations, hepatic herniation, and severe mediastinal shift. Perinatal outcomes were obtained by reviewing hospital documents or by directly calling the patients' immediate relatives. RESULTS: Mean gestational age at diagnosis was 29 weeks (range, 16-37 weeks).Thirty (79%) cases had a left diaphragmatic defect and 8 (21%) had a right lesion. Associated structural malformations were observed in 21 (55%) cases, in which 12 fetuses had a normal karyotype and 9 had chromosomal abnormalities. Isolated congenital diaphragmatic hernia was confirmed in 17 (45%) cases. The overall perinatal mortality rate was 92%. Rates of fetal deaths, early neonatal deaths, late neonatal deaths, and survival were 42%, 50%, 0%, and 8%, respectively, in cases with associated structural malformations but normal karyotyping; 56%, 44%, 0%, and 0% for cases with chromosomal abnormalities; and, 0%, 76%, 12%, and 12% in cases with isolated congenital diaphragmatic hernia. The neonatal mortality rate was 89% in cases with isolated congenital diaphragmatic hernia. CONCLUSION: Perinatal mortality was very high in prenatally diagnosed cases of congenital diaphragmatic hernia. Earlier perinatal deaths are associated with the presence of other structural defects or chromosomal abnormalities. In cases of isolated congenital diaphragmatic hernia, mortality is related to the presence of herniated liver, right-sided lesion, and major mediastinal shift.

Ipsilateral Lung Volumes Assessed by Three-Dimensional Ultrasonography in Fetuses with Isolated Congenital Diaphragmatic Hernia

“…1 ). When it was not possible to identify the ipsilateral lung, we considered the volume almost zero, giving a volume of 0.01 cm 3 , as there was no agenesis of the ipsilateral lung in all cases of CDH studied here.…”

The Role of Predictors Parameters in the Management Policy and Outcome of Congenital Diaphragmatic Hernia with the Lack of Resources in Single Pediatric Surgery Unit

“…Other researchers reported, the higher OI predicts a high mortality and has been widely used as criteria for ECMO [27]. Studies using multiple logistic regression analysis identified two adverse prognostic factors: an Apgar score at 1 min (Ap1) of 0-4, and a best oxygenation index (OI) 8.0, low Apgar scores and high oxygenation index (OI) were associated with poor outcome in infants with CDH (higher OI also reflects and triggering the more severe PPHN in comparison to survivors [28].…”

Prediction of Neonatal Respiratory Function and Pulmonary Hypertension in Fetuses with Isolated Congenital Diaphragmatic Hernia in the Fetal Endoscopic Tracleal Occlusion Era: A Single-Center Study

“…Other predictors of outcome following FETO are lung size prior to balloon insertion and the ability to remove the balloon prior to birth [34]. Increased survival rates were recently confirmed in a Brazilian randomized trial, though the natural disease of CDH may be different in that setting [35,36]. Therefore, in Europe and Northern America FETO remains an investigational procedure, which now is evaluated in a randomized trial [37,38,39].…”