Bruno Barthe

Predicting perinatal outcome in isolated congenital diaphragmatic hernia using fetal pulmonary artery diameters

OBJECTIVE The aim of the study was to evaluate the potential of fetal pulmonary artery (PA) diameters to predict perinatal death and pulmonary arterial hypertension (PAH) in congenital diaphragmatic hernia (CDH). STUDY DESIGN In this prospective observational study, observed PA (main, right, and left) diameters were measured at the level of the 3 vessels in 21 fetuses with isolated CDH and in 85 controls at 22 to 36 weeks. The observed/expected (o/e) diameters of the main, contralateral, and ipsilateral PAs were calculated by comparing these measurements with reference values obtained in our previous study and correlated with perinatal death and postnatal PAH. RESULTS The o/e PA diameters were significantly reduced in fetuses with CDH compared to controls (P < .001) and in fetuses with CDH who died (P < .050). However, there was no significant association between PA diameters and PAH (P >or= .050). CONCLUSIONS The PA diameters might be useful to predict perinatal death in isolated CDH but not postnatal PAH, suggesting that PA diameters are probably related to the severity of pulmonary hypoplasia.

Three-dimensional ultrasonographic measurements of the fetal lungs for prediction of perinatal outcome in isolated congenital diaphragmatic hernia

Aim:  To evaluate the potential of different lung measurements using three‐dimensional ultrasonography (3D‐US) to predict perinatal outcome in isolated congenital diaphragmatic hernia (CDH).

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

Objective: To evaluate the precision of three-dimensional ultrasonography (3DUS) in estimating the ipsilateral lung volume and the potential of this measurement to predict neonatal death in congenital diaphragmatic hernia (CDH). Methods: Between January 2002 and December 2004, the ipsilateral lung volumes were assessed by 3DUS using the technique of rotation of the multiplan imaging in 39 fetuses with CDH. The observed/expected ipsilateral lung volume ratios (o/e-IpsiFLVR) were compared to the lung/head ratios (LHR) and to the observed/expected total fetal lung volume ratios (o/e-TotFLVR) as well as to postnatal death. Results: Ipsilateral lung volumes (median 0.12, range 0.01–0.66) were more reduced than the total lung volumes (median 0.52, range 0.11–0.95, p < 0.001) in CDH. The bias and precision of 3DUS in estimating ipsilateral lung volumes were –0.61 and 0.99 cm3, respectively, with absolute limits of agreement from –2.56 to +1.33 cm3. The o/e-IpsiFLVR was lower in neonatal death cases (median 0.09, range 0.01–0.46) than in survivals (median 0.18, range 0.01–0.66), but this difference was not statistically significance (p > 0.05). The sensitivity, specificity, (positive and negative) predictive values and accuracy of o/e-IpsiFLVR in predicting neonatal death was 52.6% (10/19), 83.3% (10/12), 83.3% (10/12), 52.6% (10/19) and 64.5% (20/31), respectively. Conclusion: Although the ipsilateral lung volume can be measured by 3DUS, it cannot be used to predict neonatal death when considering it alone. However, it is important to measure it to calculate the total fetal lung volumes as the o/e-TotFLVR has the best efficacy in predicting neonatal death in isolated CDH.

OP31.08: Predicting perinatal outcome in 55 fetuses with isolated congenital diaphragmatic hernia using different 3DUS lung measurements

Results: All of the ten fetuses who underwent postnatal renal function tests had grade 3 or 4 hydronephrosis in postnatal ultrasonography. Six of them needed surgical correction. Relative kidney volume does not correlate well with relative kidney function (Pearson correlation coefficient r 0.195). Conclusion: This preliminary study demonstrated that relative kidney parenchymal volume measured by antenatal 3D ultrasonography provide no relation with postnatal kidney relative function.

OP19.06: Predicting neonatal prognosis in 40 fetuses with isolated congenital diaphragmatic hernia using the “ultrasonographic fetal lung volume to body weight ratio”

Background: CCAM is an uncommon pathology that involves proliferation of terminal respiratory bronchioles. The diagnosis is often made in the prenatal period using ultrasound. The antenatal diagnosis has become more frequent with increasing sonographer training and higher resolution ultrasound imaging platforms. Methods: This was a retrospective analysis of all fetuses of CCAM diagnosed antenatally that were born in our department during last three years. The ultrasound diagnosis was confirmed by prenatal MRI scan in all cases. Results: In a 3-years period, 14 cases of fetal CCAM were reffered for further management. All the lesions were noted to be unilateral CCAMs. The gestational age at diagnosis was 16–22 weeks. Two cases were diagnosed as CCAM type I (14%), 5 CCAM type II (36%) and 7 CCAM type III (50%). In two cases diagnostic type of CCAM was different from US and MRI investigations. The lesions were usually complicated by varying degrees of mediastinal sfift (78%). Severe hydrops fetalis was present in two cases only (14%). All the fetuses had normal karyotype. Termination of pregnancy was performed in three cases. Spontaneous regression of the sonographic appearances during pregnancy was observed in 46% (5/11) cases. All cases had good perinatal outcome (91% after postnatally surgery). Conclusions: The postnatal outcome of fetal CCAM is better than previously assumed, probably because of the detection ob subtle lesions and the high rate of prenatal spontaneous resolution of such tumors. Supported by grant VZ FNM 00064203/6202.

OP19.05: Ipsilateral lung volumes assessed by three‐dimensional ultrasonography in 39 fetuses with isolated congenital diaphragmatic hernia

Background: CCAM is an uncommon pathology that involves proliferation of terminal respiratory bronchioles. The diagnosis is often made in the prenatal period using ultrasound. The antenatal diagnosis has become more frequent with increasing sonographer training and higher resolution ultrasound imaging platforms. Methods: This was a retrospective analysis of all fetuses of CCAM diagnosed antenatally that were born in our department during last three years. The ultrasound diagnosis was confirmed by prenatal MRI scan in all cases. Results: In a 3-years period, 14 cases of fetal CCAM were reffered for further management. All the lesions were noted to be unilateral CCAMs. The gestational age at diagnosis was 16–22 weeks. Two cases were diagnosed as CCAM type I (14%), 5 CCAM type II (36%) and 7 CCAM type III (50%). In two cases diagnostic type of CCAM was different from US and MRI investigations. The lesions were usually complicated by varying degrees of mediastinal sfift (78%). Severe hydrops fetalis was present in two cases only (14%). All the fetuses had normal karyotype. Termination of pregnancy was performed in three cases. Spontaneous regression of the sonographic appearances during pregnancy was observed in 46% (5/11) cases. All cases had good perinatal outcome (91% after postnatally surgery). Conclusions: The postnatal outcome of fetal CCAM is better than previously assumed, probably because of the detection ob subtle lesions and the high rate of prenatal spontaneous resolution of such tumors. Supported by grant VZ FNM 00064203/6202.

OC36: Predicting postnatal pulmonary arterial hypertension using the vascular indices estimated by three-dimensional power Doppler ultrasonography in isolated congenital diaphragmatic hernia

cage perimeter (RCP), lung area (LA), heart area (HA), right lung diameter (RLD), lung length, abdominal circumference (AC), and femur length (FL) were measured by 2DUS. Pulmonary hypoplasia was confirmed by: 1) lung/body weight ratio ≤ 0.015 < 28 weeks, or ≤ 0.012 ≥ 28 weeks of gestation, and/or 2) autopsy. Sensitivity, specificity, positiveand negative predictive values (PPV/NPV), and diagnostic efficiency of 3DUS and 2DUS parameters to detect lethal pulmonary hypoplasia were compared. 44 fetuses were excluded for lack of a suitable volume dataset to measure (n = 30) or impossibility to confirm the neonatal diagnosis [(n = 14)]. Results: Lethal pulmonary hypoplasia was diagnosed in 34.1% of cases (14/41). The diagnostic efficiency of biometric parameters in predicting lethal pulmonary hypoplasia is shown in the Table.

OC14.03: Prenatal evaluation of pulmonary volume and vascularity in congenital diaphragmatic hernia using three‐dimensional ultrasonography

Objective: In utero diagnosed congenital diaphragmatic hernia (CDH) is associated to high antenatal and neonatal loss rates. Accurate prediction of outcome is crucial in counselling parents about management options. We evaluated lung-to-head ratio (LHR) and liver position in prediction of outcome of isolated left CDH in the previable period. Methods: Retrospective review of consecutive patients diagnosed with isolated LCDH prior to 28 weeks, evaluated at 6 tertiary units from 1995 onwards. Only patients with LHR measurements, obtained by experienced sonographers and with determined liver position by ultrasound or MRI, both ≤ 28 wks, were included. In all participating centers, LHR measurement was performed as previously described (Metkus et al. JPS 1996; 31 : 148–52) by experienced operators. Outcome measure was survival at discharge from NICU. Results: 134 cases were available for review; LHR was obtained at a mean of 24.4 ± 2.8 wks. Eleven patients (8%) opted for termination after being evaluated, all having LHR < 1.4. There were no postnatal diagnoses of chromosomal anomalies. Overall survival rate was 43% (58/134), after substraction of antenatal losses (11 TOP) it was 47% (58/123). LHR correlated to survival irrespective of liver position. In case of liver herniation survival was 35%. Combination of both variables predicted neonatal outcome better: liver up & LHR < 1 predicted a survival of 9%. When LHR < 0.8 & liver up, there were no survivors, but with liver down (37% of cases) survival was 40%. When LHR < 0.6 there were no survivors irrespective of liver position. Conclusions: Combination of liver up & LHR < 1 at ≤ 28 wks predicts a < 10% chance of survival, dropping to 0% if LHR < 0.8 and liver up, or 0% if LHR < 0.6, irrespective of the liver. 8% of patients opted for termination, all with LHR < 1.4, but only in half this coincided with LHR < 1.0 and liver up.