Quantitative lung index, contralateral lung area, or lung-to-head ratio to predict the neonatal outcome in isolated congenital diaphragmatic hernia?

OBJECTIVES: To estimate the accuracy of the quantitative lung index and contralateral lung area for prediction of the neonatal outcome in isolated congenital diaphragmatic hernia in comparison to other available prediction models. METHODS: Between January 2004 and December 2010, 108 fetuses with isolated (82 left-sided and 26 right-sided) congenital diaphragmatic hernia were prospectively evaluated. The quantitative lung index and observed-to-expected contralateral lung area were measured and compared to the neonatal survival rate and severe postnatal pulmonary arterial hypertension, along with the lung-to-head ratio, observed-to-expected lung-to-head ratio, and observed-to-expected total lung volume. RESULTS: Overall neonatal mortality was 64.8% (70 of 108). Severe pulmonary arterial hypertension was diagnosed in 68 (63.0%) of the cases, which was associated with neonatal death (P < .001). Both the quantitative lung index and observed-to-expected contralateral lung area were significantly associated with neonatal survival and pulmonary arterial hypertension (P < .001), with accuracy to predict survival of 70.9% and 70.0%, respectively, and accuracy to predict hypertension of 78.7% and 72.0%; however, they were both less accurate than the observed-to-expected total lung volume (83.3% and 86.1%; P < .01). The lung-to-head ratio (73.1% and 78.7%) and observed-to-expected lung-to-head ratio (75.9% and 72.2%; P > .05) had similar accuracy as the quantitative lung index and observed-to-expected contralateral lung area. CONCLUSIONS: The observed-to-expected total lung volume is the most accurate predictor of the neonatal outcome in cases of isolated congenital diaphragmatic hernia. Both the quantitative lung index and observed-to-expected contralateral lung area, albeit reasonably accurate, do not produce the same level of accuracy and render similar results as the lung-to-head ratio and observed-to-expected lung-to-head ratio.

Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops: a single center cohort and a literature review.

OBJECTIVE: To evaluate the perinatal outcomes in hydropic fetuses with congenital microcystic pulmonary lesions that underwent percutaneous, invasive, laser therapy. METHOD: This retrospective study reviews the literature and our experience between 2004 and 2010. Characteristics of the cystic lung lesions, liquor volume (presence of polyhydramnios or not), localization of ablation (vascular vs interstitial) and gestational age at which the procedure was performed were related to outcome (survival). RESULTS: In total, 16 fetuses with congenital lung lesions underwent 'invasive' percutaneous laser ablation, seven performed in our center and nine published cases. Survival rate was higher in fetuses with a subsequent postnatal diagnosis of bronchopulmonary sequestration (87.5%) compared with congenital adenomatoid malformation (28.6%; p = 0.04). The technique of vascular ablation was more successful (100%) than interstitial ablation (25.0%, p < 0.01). CONCLUSION: Percutaneous vascular laser ablation seems to be effective for bronchopulmonary sequestration in hydropic fetuses. Outcomes were worst following interstitial ablation for microcystic congenital adenomatoid with hydrops.

Fetal pulmonary response after fetoscopic tracheal occlusion for severe isolated congenital diaphragmatic hernia.

OBJECTIVE: To estimate the response in lung growth and vascularity after fetal endoscopic tracheal occlusion for severe congenital diaphragmatic hernia in the prediction of neonatal survival. METHODS: Between January 2006 and December 2010, fetal lung parameters (observed-to-expected lung-to-head ratio; observed-to-expected lung volume; and contralateral lung vascularization index) were evaluated before fetal tracheal occlusion and were evaluated longitudinally every 2 weeks in 72 fetuses with severe isolated congenital diaphragmatic hernia. Thirty-five fetuses underwent fetal endoscopic tracheal occlusion and 37 cases did not. RESULTS: Survival rate was significantly higher in the fetal endoscopic tracheal occlusion group (54.3%) than in the no fetal endoscopic tracheal occlusion group (5.4%, P<.01). Fetal endoscopic tracheal occlusion resulted in a significant improvement in fetal lung size and pulmonary vascularity when compared with fetuses that did not go to the fetal intervention (increase of the observed-to-expected lung-to-head ratio, observed-to-expected total lung volume, and contralateral pulmonary vascularization index 56.2% compared with 0.3%, 37.9% compared with 0.1%, and 98.6% compared with 0.0%, respectively; P<.01). Receiver operating characteristic curves indicated that the observed-to-expected total fetal lung volume was the single best predictor of neonatal survival before fetal endoscopic tracheal occlusion (cutoff 0.23, area under the curve [AUC] 0.88, relative risk 5.3, 95% confidence interval [CI] 1.4-19.7). However, the contralateral lung vascularization index at 4 weeks after fetal endoscopic tracheal occlusion was more accurate in the prediction of neonatal outcome (cutoff 24.0%, AUC 0.98, relative risk 9.9, 95% CI 1.5-66.9) with the combination of observed-to-expected lung volumes and contralateral lung vascularization index at 4 weeks being the best predictor of outcome (AUC 0.98, relative risk 16.6, 95% CI 2.5-112.3). CONCLUSION: Fetal endoscopic tracheal occlusion improves survival rate by increasing the lung size and pulmonary vascularity in fetuses with severe congenital diaphragmatic hernia. The pulmonary response after fetal endoscopic tracheal occlusion can be used to predict neonatal survival.

Comparison between fetal endoscopic tracheal occlusion using a 1.0-mm fetoscope and prenatal expectant management in severe congenital diaphragmatic hernia.

OBJECTIVES: To evaluate if fetal endoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia (CDH) using a 1.0-mm fetoscope improves neonatal outcome. METHOD: Between January 2006 and December 2008, a controlled study was conducted at a single center in which FETO was proposed for fetuses with severe isolated CDH (lung-to-head ratio <1.0) and liver herniation to the thoracic cavity but no other detectable anomalies at diagnosis (<26 weeks). FETO was performed under maternal epidural and fetal intramuscular anesthesia, guided by ultrasonography and 1.0-mm fetoscope between 26 and 30 weeks. All cases submitted to FETO were delivered by ex utero intrapartum therapy procedure. Postnatal therapy was the same for both treated fetuses and controls. The primary outcome was neonatal survival (up to 28 days after birth). RESULTS: A total of 35 women met the inclusion criteria, and in 17 of them, fetal intervention was intended. However, in 1 case, it was not possible to insert the balloon inside the fetal trachea because of placental bleeding. FETO was therefore successfully performed in 16 fetuses with severe CDH. Eighteen cases received no prenatal intervention and served as the control group. Mean gestational age at diagnosis was similar in both groups (p > 0.05). Delivery occurred at 35.6 (range: 28-38) weeks in the FETO group and at 37.5 (range: 31-40) weeks (p = 0.18) among controls. Nine of 17 (52.9%) infants in the FETO group and 1 of 18 (5.6%) in the control group survived (p < 0.01). Severe pulmonary arterial hypertension was present in 8/17 (47.1%) infants from the FETO group and in 16/18 (88.9%) controls (p = 0.01). CONCLUSION: The present study shows that FETO using a 1.0-mm fetoscope is feasible and may improve neonatal outcome in severe CDH.

Congenital intrahepatic arterioportal fistula presenting as severe undernutrition and chronic watery diarrhea in a 2-year-old girl.

Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.

Intimal dissection of the hepatic artery after thrombectomy as a cause of graft loss in pediatric living-related liver transplantation.

HAT is the main cause of graft loss in pediatric living-related LTx. Revascularization of the graft by thrombectomy and re-anastomosis has been reported to be effective for graft salvage in cases of HAT and should be attempted when potential donors are not available for emergency re-transplantation. Immediate complications secondary to revascularization attempts in cases of HAT are not described. Late complications are mainly related to biliary tree ischemia. We report a case of child who experienced intimal hepatic artery dissection, which extended into intra-hepatic branches of the artery after a thrombectomy with a Fogarty balloon catheter in an attempt to restore arterial flow after HAT. This complication led to acute deterioration of the graft and the need for emergency re-transplantation.

The outcome of newborns with abdominal wall defects according to the method of abdominal closure: the experience of a single center.

Recent reports suggest that the technique of abdominal closure in neonates with anterior abdominal wall defects (AWD) correlates with the outcome. The aim of this study is to analyze factors related to mortality and morbidity, according to the technique of abdominal closure of these neonates. Retrospective analysis of charts from 76 consecutive neonates with AWD treated in a single institution. They were divided according to the type of abdominal wall closure: group I: primary closure, group II: silo followed by primary closure and group III: silo followed by polypropylene mesh. Outcome was analyzed separately for neonates with gastroschisis and omphalocele. There were 13 deaths (17.1%). Mortality for neonates with isolated defects was 9.6%. Mortality rate was similar in all groups for either neonates with gastroschisis or omphalocele. Postoperative complications were not significantly different among groups except for a prolonged time of hospitalization in group III. Mortality rate is not correlated with the type of abdominal closure. Neonates with primary closure or with other methods of abdominal wall closure had similar rate of postoperative complications. Neonates with mesh closure of the abdomen have prolonged hospitalization. The use of a polypropylene mesh is a good alternative for neonates whose primary closure or closure after silo placement is not possible.