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To determine the accuracy of commonly utilized ultrasound formulas for estimating birth weight (BW) in fetuses with gastroschisis. A retrospective review was conducted of all inborn pregnancies with gastroschisis within the five institutions of the University of California Fetal Consortium (UCfC) between 2007 and 2012. Infants delivered at ⩾28 weeks who had an ultrasound within 21 days before delivery were included. Prediction of BW was evaluated for each of the five ultrasound formulas: Hadlock 1 (abdominal circumference (AC), biparietal diameter (BPD), femur length (FL) and head circumference (HC)) and Hadlock 2 (AC, BPD and FL), Shepard (AC and BPD), Honarvar (FL) and Siemer (BPD, occipitofrontal diameter (OFD), and FL) using Pearson’s correlation, mean difference and percent error and Bland–Altman analysis. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for the ultrasound diagnosis of intrauterine growth restriction (IUGR) were assessed. We identified 191 neonates born with gastroschisis within the UCfC, with 111 neonates meeting the inclusion criteria. The mean gestational age at delivery was 36.3±1.7 weeks and the mean BW was 2448±460 g. Hadlock (1) formula was found to have the best correlation (r=0.81), the lowest mean difference (8±306 g) and the lowest mean percent error (1.4±13%). The Honarvar and Siemer formulas performed significantly worse when compared with Hadlock 1, with a 13.7% (P

Introduction: We sought to determine the relationship between the degree of stomach herniation by antenatal sonography and neonatal outcomes in fetuses with isolated left-sided congenital diaphragmatic hernia (CDH). Materials and Methods: We retrospectively reviewed neonatal medical records and antenatal sonography of fetuses with isolated left CDH cared for at a single institution (2000-2012). Fetal stomach position was classified on sonography as follows: intra-abdominal, anterior left chest, mid-to-posterior left chest, or retrocardiac (right chest). Results: Ninety fetuses were included with 70% surviving to neonatal discharge. Stomach position was intra-abdominal in 14% (n = 13), anterior left chest in 19% (n = 17), mid-to-posterior left chest in 41% (n = 37), and retrocardiac in 26% (n = 23). Increasingly abnormal stomach position was linearly associated with an increased odds of death (OR 4.8, 95% CI 2.1-10.9), extracorporeal membrane oxygenation (ECMO; OR 5.6, 95% CI 1.9-16.7), nonprimary diaphragmatic repair (OR 2.7, 95% CI 1.4-5.5), prolonged mechanical ventilation (OR 5.9, 95% CI 2.3-15.6), and prolonged respiratory support (OR 4.0, 95% CI 1.6-9.9). All fetuses with intra-abdominal stomach position survived without substantial respiratory morbidity or need for ECMO. Discussion: Fetal stomach position is strongly associated with neonatal outcomes in isolated left CDH. This objective tool may allow for accurate prognostication in a variety of clinical settings.

Background/Purpose Gastroschisis is a resource-intensive birth defect without consensus regarding optimal surgical and medical management. We sought to determine best-practice guidelines by examining differences in multi-institutional practices and outcomes. Methods Site-specific practice patterns were queried, and infant–maternal chart review was retrospectively performed for gastroschisis infants treated at 5 UCfC institutions (2007–2012). The primary outcome was length of stay. Univariate analysis was done to assess variation practices and outcomes by site. Multivariate models were constructed with site as an instrumental variable and with sites grouped by silo practice pattern adjusting for confounding factors. Results Of 191 gastroschisis infants, 164 infants were uncomplicated. Among uncomplicated patients, there were no deaths and only one case of necrotizing enterocolitis. Bivariate analysis revealed significant differences in practices and outcomes by site. Despite wide variations in practice patterns, there were no major differences in outcome among sites or by silo practice, after adjusting for confounding factors. Conclusions Wide variability exists in institutional practice patterns for infants with gastroschisis, but poor outcomes were not associated with expeditious silo or primary closure, avoidance of routine paralysis, or limited central line and antibiotic durations. Development of clinical pathways incorporating these practices may help standardize care and reduce health care costs.

We describe 2 infants with congenital diaphragmatic hernia with severe pulmonary hypertension at 6 weeks. Treprostinil was used with rapid clinical improvement. Repeat cardiac catheterization showed dramatic improvement. Both infants were weaned off the drug, representing the first reports of successful short-term treprostinil use in neonates with congenital diaphragmatic hernia.

To assess the utility of clinical predictors of persistent respiratory morbidity in extremely low gestational age newborns (ELGANs).We enrolled ELGANs (29 weeks' gestation) at ≤7 postnatal days and collected antenatal and neonatal clinical data through 36 weeks' postmenstrual age. We surveyed caregivers at 3, 6, 9, and 12 months' corrected age to identify postdischarge respiratory morbidity, defined as hospitalization, home support (oxygen, tracheostomy, ventilation), medications, or symptoms (cough/wheeze). Infants were classified as having postprematurity respiratory disease (PRD, the primary study outcome) if respiratory morbidity persisted over ≥2 questionnaires. Infants were classified with severe respiratory morbidity if there were multiple hospitalizations, exposure to systemic steroids or pulmonary vasodilators, home oxygen after 3 months or mechanical ventilation, or symptoms despite inhaled corticosteroids. Mixed-effects models generated with data available at 1 day (perinatal) and 36 weeks' postmenstrual age were assessed for predictive accuracy.Of 724 infants (918 ± 234 g, 26.7 ± 1.4 weeks' gestational age) classified for the primary outcome, 68.6% had PRD; 245 of 704 (34.8%) were classified as severe. Male sex, intrauterine growth restriction, maternal smoking, race/ethnicity, intubation at birth, and public insurance were retained in perinatal and 36-week models for both PRD and respiratory morbidity severity. The perinatal model accurately predicted PRD (c-statistic 0.858). Neither the 36-week model nor the addition of bronchopulmonary dysplasia to the perinatal model improved accuracy (0.856, 0.860); c-statistic for BPD alone was 0.907.Both bronchopulmonary dysplasia and perinatal clinical data accurately identify ELGANs at risk for persistent and severe respiratory morbidity at 1 year.ClinicalTrials.gov: NCT01435187.

Tracheotomy is sometimes performed in extremely low gestational age newborns requiring prolonged ventilation. Studies suggest better neurodevelopmental outcomes in preterm newborns undergoing earlier tracheotomy (120 days); however, guidelines for who should undergo tracheotomy and when to perform tracheotomy are unclear regarding infants receiving long-term positive-pressure support.To determine the characteristics associated with tracheotomy in high-risk, extremely low gestational age newborns.This secondary analysis of infants enrolled in the double-blind, randomized clinical trial known as the Trial of Late Surfactant (TOLSURF) was conducted from January 10, 2010, to September 3, 2013, in neonatal intensive care units. Participants included 511 premature infants (≤28 weeks' gestational age) who were intubated and mechanically ventilated anytime between 7 and 14 days of life. Infants were randomized to receive late surfactant plus inhaled nitric oxide or inhaled nitric oxide alone. All data were collected prospectively. A mixed-effects model, with patient-level random effects included to account for individual homogeneity, was used to compare mean airway pressure (MAP) during the first 120 days in infants who did not undergo tracheotomy vs those who underwent tracheotomy. The present analysis was conducted from July 1, 2015, to March 29, 2016.Mean airway pressure, comorbidities of prematurity, airway stenosis, and airway malacia.Tracheotomy.Of the 511 infants enrolled in TOLSURF, the mean (SD) gestational age was 25 (1.2) weeks, with a birth weight of 701 (165) g. Fifteen infants (2.9%) underwent tracheotomy. Among those undergoing tracheotomy, 7 infants (46.7%) had airway stenosis or malacia, none of whom died. Of the 8 infants who underwent tracheotomy without airway stenosis or malacia, 4 (50%) died. Mean age at tracheotomy was 126 days (95% CI, 108-144 days). In general, MAP increased over time in the group undergoing tracheotomy (+0.09 cm H2O/wk; 95% CI, 0.06-0.11 cm H2O/wk) but decreased in those who did not undergo tracheotomy (-0.20 cm H2O/wk; 95% CI, 0.19-0.21 cm H2O/wk; P .001 for interaction).In this cohort of high-risk, extremely low gestational age newborns, trends in MAP can be a clinical indicator for infants requiring long-term positive-pressure ventilation who are at highest risk for receiving tracheotomy. Knowledge of this information may identify infants who would benefit from earlier consideration for tracheotomy.clinicaltrials.gov Identifier: NCT01022580.

In fetuses with left-sided congenital diaphragmatic hernia, intrathoracic herniation of the spleen is a common occurrence. The herniated spleen can reside posterior to the left atrium of the heart in the right hemithorax and is increasingly differentiated from the lung with the use of newer sonographic equipment. Estimation of the neonatal prognosis relies on accurate measurement of fetal lung size, particularly with commonly used measurements such as the lung-to-head ratio. Here we describe how herniation of the spleen behind the heart can complicate measurement of the lung-to-head ratio on sonography and lead to overestimation, with implications for perinatal prognostication and management.

ObjectiveCongenital diaphragmatic hernia (CDH) results in morbidity and death from lung hypoplasia and persistent pulmonary hypertension (PH). We sought to define the relationship between fetal ultrasound markers of severity in CDH and the time to resolution of neonatal PH.Study designWe conducted a retrospective study of fetuses with an antenatal ultrasound scan and left-sided CDH cared for at the University of California San Francisco (2002-2012). Fetal liver position was classified on ultrasound scan as abdominal (entire liver within the abdomen) or thoracic (any portion of the liver within the thorax). Fetal stomach position was classified from least to most aberrant: abdominal, anterior left chest, mid-posterior left chest, or retrocardiac (right chest). Lung-to-head ratio (LHR) was determined from available scans at 20-29 weeks of gestational age (GA). Routine neonatal echocardiograms were performed weekly for up to 6 weeks or until PH resolved or until discharge. PH was assessed by echocardiogram with the use of a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity. Days to PH-free survival was defined as the age at which pulmonary artery pressure was estimated to be

ObjectivesTo describe the natural history of pulmonary hypertension (PH) and the risk of death and pulmonary morbidity associated with the persistence of PH through the neonatal hospitalization for these infants.Study designWe performed a retrospective cohort study of infants with congenital diaphragmatic hernia (CDH) cared for at University of California San Francisco (2002-2012). Infants with other major anomalies or syndromes were excluded (n = 43). Clinical echocardiograms were performed weekly for up to 6 weeks or until PH resolved off respiratory support or until hospital discharge. Echocardiograms were re-read by a blinded reviewer and categorized by severity of elevation in estimated pulmonary arterial pressure. PH was defined as ≥2/3 systemic blood pressure. Severity was determined by a hierarchy of ductus arteriosus level shunt, interventricular septal position, and tricuspid regurgitant jet velocity.ResultsOf 140 infants with ≥1 echo, 98 resolved their PH prior to death/discharge. Mean time to resolution was 18 days (median 14 days, IQR 8, 21 days). Those with persistence of PH had a higher rate of extracorporeal membrane oxygenation (P < .001) and death (P < .001), and fewer ventilator-free days (P < .001). Persistence of PH at 14 days predicted mortality (area under the receiver operating characteristic curve 0.87) and adverse respiratory outcomes (area under the receiver operating characteristic curve 0.80-0.83).ConclusionsThe majority of infants with CDH resolve PH between 1 and 3 weeks of life. At 2 weeks of age, severity of PH by echocardiogram strongly predicts short-term pulmonary morbidity and death. Further evaluation of physiological alterations during that time may lead to novel therapies for severe CDH.

Background: Right congenital diaphragmatic hernia (CDH) occurs less frequently than left CDH. Therefore, prognostic indicators for right CDH are not as well studied as for left CDH. Methods: A retrospective review from a single, tertiary referral center (from 1994 until July 2013) of patients with unilateral right CDH was conducted. Prenatal characteristics were evaluated and correlated with survival to discharge and need for extracorporeal membranous oxygen (ECMO). Results: In total, 34 patients were identified. There were 12 postnatal deaths and 2 fetal demises (6%), representing an overall mortality of 41%. Six patients required ECMO. Nine patients underwent fetal intervention and were analyzed separately. For patients not undergoing fetal intervention, the survival rate was 52% and a higher mean (±SD) lung-to-head ratio (LHR) was associated with survival (1.1 ± 0.4 vs. 0.8 ± 0.2, p = 0.03). There were no deaths or need for ECMO in any patient with an LHR ≥1.0. Of the 9 patients who underwent fetal intervention, survival was 78% and only 1 patient required ECMO. Fetal intervention was primarily tracheal occlusion (n = 8). Conclusions: An LHR

Background B-type natriuretic peptide (BNP) has not been evaluated in newborns with congenital diaphragmatic hernia (CDH). We hypothesized that BNP and severity of pulmonary hypertension (PH) would predict clinical outcome in these infants. Methods We measured BNP levels and assessed severity of PH by echocardiography at one day and one week of life. Outcome was classified by status at 56 days (or prior discharge): Good (n=13) if alive on room air and Poor (n=14) if expired or receiving respiratory support. We estimated area under the curve (AUC) and 95% confidence interval (CI). Results BNP levels were higher at one day in newborns with Poor outcome (median 220 pg/mL versus 55 pg/mL, P

Infants with congenital diaphragmatic hernia (CDH) have prolonged respiratory failure, loss of lean body mass, and failure to thrive. Chylothorax is an uncommon complication after CDH repair. The purpose of this study was to determine whether the presence of chylothorax predisposes infants with CDH to worse nutritional outcomes. We performed a retrospective cohort study of 164 newborns with …