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Objectives: To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by the introduction of a new ultrasonographic (US) marker, defined as mediastinal shift angle (MSA) and to evaluate its ability in predicting postnatal survival at discharge.Methods: Twenty-four consecutive fetuses from singleton pregnancies with isolated left-sided CDH were included in the study group and then subdivided into group A (16 survivors) and group B (8 nonsurvivors). The study group was matched with a control group of 95 fetuses from singleton pregnancies free from structural and/or chromosomal anomalies. On the same US stored images commonly used for lung-to-head ratio (LHR) measurement, a landmark line was drawn from a point on the posterior face of the vertebral body, splitting it into two equal parts, to the mid-posterior surface of the sternum. Another landmark line was then traced from the same point of the vertebral body to touch tangentially the lateral wall of the right atrium. The angle between these two lines was used to quantify mediastinal shift and called "mediastinal shift angle" (MSA).Results: Median MSA was significantly different between group A (34.3° range 29.3-45.9°) and group B (42.7° range 34.1-58.9°) (p < .001) and between study group as a whole and the control group (19° range 13.8-25.9°) (p < .001). Statistical analysis confirmed an inverse correlation between MSA values and survival (p = .004). The best cutoff value for MSA was 43.7°, which demonstrated the highest discriminatory power (sensitivity 63%; specificity 93.75%).Conclusions: In fetuses with isolated CDH, the mediastinal shift may be quantified using mediastinal shift angle (MSA) and this US marker, similarly to the widely accepted and used US prenatal prognostic indicators (LHR and O/E LHR), seems to reliably predict survival. [ABSTRACT FROM AUTHOR]

Background: Two randomized controlled trials compared the neonatal and infant outcomes after fetoscopic endoluminal tracheal occlusion with expectant prenatal management in fetuses with severe and moderate isolated congenital diaphragmatic hernia, respectively. Fetoscopic endoluminal tracheal occlusion was carried out at 27+0 to 29+6 weeks' gestation (referred to as "early") for severe and at 30+0 to 31+6 weeks ("late") for moderate hypoplasia. The reported absolute increase in the survival to discharge was 13% (95% confidence interval, -1 to 28; P=.059) and 25% (95% confidence interval, 6-46; P=.0091) for moderate and severe hypoplasia.Objective: Data from the 2 trials were pooled to study the heterogeneity of the treatment effect by observed over expected lung-to-head ratio and explore the effect of gestational age at balloon insertion.Study Design: Individual participant data from the 2 trials were reanalyzed. Women were assessed between 2008 and 2020 at 14 experienced fetoscopic endoluminal tracheal occlusion centers and were randomized in a 1:1 ratio to either expectant management or fetoscopic endoluminal tracheal occlusion. All received standardized postnatal management. The combined data involved 287 patients (196 with moderate hypoplasia and 91 with severe hypoplasia). The primary endpoint was survival to discharge from the neonatal intensive care unit. The secondary endpoints were survival to 6 months of age, survival to 6 months without oxygen supplementation, and gestational age at live birth. Penalized regression was used with the following covariates: intervention (fetoscopic endoluminal tracheal occlusion vs expectant), early balloon insertion (yes vs no), observed over expected lung-to-head ratio, liver herniation (yes vs no), and trial (severe vs moderate). The interaction between intervention and the observed over expected lung-to-head ratio was evaluated to study treatment effect heterogeneity.Results: For survival to discharge, the adjusted odds ratio of fetoscopic endoluminal tracheal occlusion was 1.78 (95% confidence interval, 1.05-3.01; P=.031). The additional effect of early balloon insertion was highly uncertain (adjusted odds ratio, 1.53; 95% confidence interval, 0.60-3.91; P=.370). When combining these 2 effects, the adjusted odds ratio of fetoscopic endoluminal tracheal occlusion with early balloon insertion was 2.73 (95% confidence interval, 1.15-6.49). The results for survival to 6 months and survival to 6 months without oxygen dependence were comparable. The gestational age at delivery was on average 1.7 weeks earlier (95% confidence interval, 1.1-2.3) following fetoscopic endoluminal tracheal occlusion with late insertion and 3.2 weeks earlier (95% confidence interval, 2.3-4.1) following fetoscopic endoluminal tracheal occlusion with early insertion compared with expectant management. There was no evidence that the effect of fetoscopic endoluminal tracheal occlusion depended on the observed over expected lung-to-head ratio for any of the endpoints.Conclusion: This analysis suggests that fetoscopic endoluminal tracheal occlusion increases survival for both moderate and severe lung hypoplasia. The difference between the results for the Tracheal Occlusion To Accelerate Lung growth trials, when considered apart, may be because of the difference in the time point of balloon insertion. However, the effect of the time point of balloon insertion could not be robustly assessed because of a small sample size and the confounding effect of disease severity. Fetoscopic endoluminal tracheal occlusion with early balloon insertion in particular strongly increases the risk for preterm delivery. [ABSTRACT FROM AUTHOR]

Abstract: Background/Purpose: The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. A recent report from CDH Study Group showed that dimension of diaphragmatic defect is the only independent risk factor of mortality. However, the influence of defect size on late morbidity is still controversial. The aim of the study was to evaluate the influence of patch repair (proxy of diaphragmatic defects size) on midterm morbidity. Methods: All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 hours of life) CDH survivors treated at our institution from 2004 to 2008 were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary, and orthopedic evaluations were performed at 6, 12, and 24 months of age. Patient outcomes were compared with respect to +/− patch repair. Results: Of 70 survivors, 61 (87%) were enrolled and prospectively evaluated in follow-up. Poorer auxological outcome, increased rate of gastroesophageal reflux, and altered pulmonary function test were observed during follow-up. Conclusions: Patch repair correlates with higher pulmonary, auxological, and gastroesophageal morbidity without increasing chest wall deformities at long-term follow-up. [ABSTRACT FROM AUTHOR]

Congenital diaphragmatic hernia (CDH) has been rarely described in CHARGE syndrome. We report a patient affected by CHARGE syndrome presenting with a right-sided Bochdalek-type diaphragmatic hernia, and collect the pertinent literature. Furthermore, we review the embryogenesis of the diaphragm and the pathogenesis of CDH to highlight if this malformation could be explained by a developmental anomaly of CHARGE. On the basis of our study, we suggest that patients affected by CDH, facial asymmetry and cardiovascular or urogenital malformations, should be actively screened for CHARGE syndrome findings. [ABSTRACT FROM AUTHOR]

With improving treatment strategies for congenital diaphragmatic hernia (CDH) infants, an increase in survival of more severely affected patients can be expected. Consequently, more attention is now focused on long-term follow up of these patients. Many reports have emphasized associated morbidity, including pulmonary sequelae, neurodevelopmental deficits, gastrointestinal disorders, and other abnormalities. Therefore, survivors of CDH remain a complex patient population to care for throughout infancy and childhood, thus requiring long-term follow up. Much information has been provided from many centers regarding individual institutional improvements in overall survival. Few of these, however, have reported long-term follow up. The aim of this review is to describe the long-term outcome of survivors with CDH and to suggest a possible follow-up protocol for these patients. [Copyright &y& Elsevier]

In congenital diaphragmatic hernia (CDH), chest tube insertion at repair could determine rapid overexpansion of hypoplastic lungs, increasing the risk of pneumothorax. Therefore, in our institution no drainage tube at CDH repair was inserted after 1997. Afterwards several patients needed chest drainage for cardiopulmonary distress due to pleural effusion (PE) during the postoperative course. The aims of this study are to establish the incidence of PE requiring drainage for cardiopulmonary distress during postoperative course in CDH and to assess its eventual increase in patients with patch repair. Furthermore, the aetiology and treatment of PE are highlighted. Records of high-risk CDH operated on from 1998 to 2004 were reviewed. No chest tube was inserted at repair. Drainage was accomplished postoperatively if a cardiopulmonary distress due to PE occurred. Groups with and without patch were compared on gender, gestational age, birth weight, side of hernia, PE, hernial sac, central venous line and venous thrombosis, using the chi(2) and Student's t tests. Overall mortality rate was recorded. Out of 76 patients, 23 (30%) required patch repair. The PE occurred in 22 out of 76 patients, and in 68% of cases a chylothorax resulted. Birth weight was significantly lower, and the PE rate was significantly higher in patients with patch. Pleural drainage yielded improvement of ventilatory and respiratory parameters in all cases. Overall mortality rate was 16 and 23% in patients that required chest drain. The incidence of PE was 30% in CDH and significantly higher in patients with patch. The increased mortality rate in patients with PE causing cardiopulmonary distress warrants chest tube drainage before instability occurs. [ABSTRACT FROM AUTHOR]

Unlabelled: Cystic adenomatoid malformation of the lung (CAML) is a rare pulmonary maldevelopment resulting from an abnormal growth of the terminal bronchial structures. This study proposes a possible management of prenatally diagnosed CAML. A group of nine cases of CAML diagnosed prenatally between January 1990 and December 1995 was studied retrospectively. The evolution of lesions was followed in utero by serial ultrasound monitoring. Chest X-ray was performed at birth in all neonates and CT only in the symptomatic ones. CAML was macrocystic in three cases and microcystic in six. No polyhydramnios, hydrops or associated malformations were seen. In four cases, CAML was confirmed at birth and required surgery. The lesion decreased in size prenatally in five fetuses, of whom only one underwent surgery. In two further cases a prenatal diagnosis of CAML was changed to diaphragmatic hernia.Conclusion: Given the possible clinical disappearance or resolution of cystic adenomatoid malformation of the lung, we believe that surgery is justified at birth only in symptomatic and radiologically positive neonates. It could be safely delayed in those asymptomatic patients with either positive or negative chest X-ray. The former need computed tomography at birth, whereas, in the latter, it should be performed at 6 months of age for a more definitive assessment of the patient. [ABSTRACT FROM AUTHOR]

Background: Newborns with congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) might experience breastfeeding difficulties. The aim of this study was to detect the prevalence of breastfeeding in newborns with CDH and EA at different time points. Methods: We performed an epidemiological study and retrospective survey on the prevalence of breastfeeding in CDH and EA affected newborns. We identified 40 CDH and 25 EA newborns who were fed through breastfeeding procedures according to WHO categorized definitions, and compared the breastfeeding procedures at the beginning of hospitalization and at three months of life. Results: Although all the mothers attempted breastfeeding after birth, only 44 (67.7%) were still breastfeeding at the time of discharge. Exclusive breastfeeding was successful for only 19 (29%) mothers. The rate of exclusive breastfeeding at three months of life did not differ statistically from discharge and between the two groups of study. Conclusion: A large percentage of mothers of children with CDH and EA who breastfed at the beginning of hospitalization did not continue at three months. It would be important to increase the breastfeeding rate in CDH and EA affected newborns by following specific steps for vulnerable infants and sustaining breastfeeding after discharge. [ABSTRACT FROM AUTHOR]