Suma B. Hoffman

Predictors of survival in congenital diaphragmatic hernia patients requiring extracorporeal membrane oxygenation: CNMC 15-year experience.

Objective:To review outcomes of patients with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) at a level IIIC neonatal intensive care unit and to determine if pre-ECMO respiratory status can help predict mortality.Study Design:A single-center retrospective chart review was conducted on all infants with CDH treated with ECMO in the past 15 years. Demographic and clinical information, including pre-ECMO ventilatory and blood gas data, was collected. Differences between survivors and non-survivors were evaluated using independent samples t-/Mann–Whitney U-and Fishers exact/χ 2-tests for continuous and categorical data, respectively. Cox regression analysis was performed to evaluate predictors of survival while controlling for covariates. Significant predictors were further explored with receiver operating characteristic (ROC) curve and Kaplan–Meier survival analysis.Result:Overall survival of the population of 62 patients treated with ECMO was 50%. Survivor and non-survivors were similar in birth weight, gestational age, gender, race and Apgar scores. Approximately 80% of patients in both groups had a left-sided defect. Less than half of patients were prenatally diagnosed in either group. Patients in the non-survivor group had associated anomalies (42 vs 23% for survivors) but this was not statistically significant (P=0.303). Non-survivors were more likely to be put on ECMO earlier, stay on ECMO longer and be operated upon later. On pre-ECMO blood gas analyses, survivors had higher pH and PaO2, and lower oxygenation index and PaCO2 compared with non-survivors. After controlling for covariates, a lower minimum PaCO2 and side of defect were the only independent predictors of survival. ROC curve for minimum pre-ECMO PaCO2 had a significant area under the curve (0.72, P=0.003). Survival was 27% in babies unable to achieve a pre-ECMO PaCO2 <60 mm Hg whereas no patients survived if their lowest pre-ECMO PaCO2 was >70 mm Hg.Conclusion:Minimum achievable pre-ECMO PaCO2 is an independent predictor of survival in patients with CDH requiring ECMO life support. These data provide useful prognostic information for counseling families and may facilitate direction of care in extreme cases where the degree of pulmonary hypoplasia may be incompatible with life.

Survival in congenital diaphragmatic hernia: use of predictive equations in the ECMO population.

Background: Equations have been proposed by the Wilford Hall/Santa Rosa (WHSR) and Congenital Diaphragmatic Hernia Study Group (CDHSG) for predicting survival in patients with CDH. The CDHSG stratifies risk based on a logistic regression equation incorporating birth weight and 5-min Apgar score, while the WHSR group uses the difference between maximum pO2 and maximum pCO2 as an index of risk. These models have not been applied specifically to the CDH ECMO (extracorporeal membrane oxygenation) population, a group at highest mortality risk. Objectives: To evaluate the WHSR and CDHSG predictive equations when applied to a population of patients with CDH requiring ECMO life support. Methods: A single-center retrospective review was conducted on infants with CDH treated with ECMO between 1993 and 2007. Predicted and actual outcomes were compared using receiver operating curve (ROC) analyses in which an area under the curve (AUC) of 1 denotes 100% agreement between predicted and actual outcomes. Kaplan-Meier analyses were also used to compare survival of patients who were risk-categorized according to each prediction model. Minimum pre-ECMO pCO2 was likewise evaluated as a predictor of survival. Results: Overall survival was 50% in 62 CDH patients treated with ECMO during the study period. The CDHSG equation did not discriminate between survivors and nonsurvivors (AUC 0.55, p = 0.499). The modified WHSR formula showed better discrimination of survival (AUC 0.71, p = 0.004). Lowest achievable pre-ECMO pCO2 had the highest AUC (0.723, p = 0.003). Patients with minimum pre-ECMO pCO2 <50 mm Hg had 56% survival, while those with >70 mm Hg had 0% survival. Conclusions: Equations proposed to predict survival in CDH patients may not discriminate survivors from nonsurvivors in the ECMO population. In this highest risk group, factors such as birth weight and Apgar score are less critical in estimating mortality risk than indicators of ventilation and oxygenation that reflect the degree of pulmonary hypoplasia.

Impact of High-Flow Nasal Cannula Use on Neonatal Respiratory Support Patterns and Length of Stay

BACKGROUND: Heated humidified high-flow nasal cannula (HFNC) is thought to be comparable with nasal CPAP. The effect of multimodality mid-level respiratory support use in the neonatal ICU is unknown. The objective of this work was to evaluate the effect of introducing HFNC on length of respiratory support and stay. METHODS: A chart review was conducted on subjects at 24–32 weeks gestation requiring mid-level support (HFNC/nasal CPAP) 1 y before and after HFNC implementation. The 2 groups were compared for clinical and demographic data using t test or chi-square analysis. Further, multivariate linear and logistic regression was done to determine significant risk factors for outcomes controlling for covariates. RESULTS: Eighty subjects were eligible in the pre-HFNC group, and 83 were eligible in the post-HFNC group. Subjects were similar in their baseline characteristics. In clinical outcomes, the post-HFNC group had higher rates of retinopathy of prematurity (P = .02) and a trend toward higher bronchopulmonary dysplasia rates (P = .063). The post-HFNC subjects had longer duration of mid-level support and were older at the time they were weaned to stable low-flow nasal cannula (P < .05). Although the length of respiratory support and stay and corrected gestational age at discharge were similar, those in the pre-HFNC period were more likely to be receiving full oral feeds and be discharged home versus being transferred to an intermediate care facility (P < .05). CONCLUSIONS: HFNC introduction was significantly associated with a longer duration of mid-level respiratory support, decrease in oral feeding at discharge, increased retinopathy of prematurity rates, and higher use of intermediate care facilities, leading us to examine our noninvasive ventilation and weaning strategies.

Hypotension and Adverse Outcomes in Prematurity: Comparing Definitions

Background: In the premature neonate, there is no consensus regarding normal blood pressure (BP). The most common definition used is a mean arterial BP (MAP) less than the gestational age (GA); however, studies indicate that the neuroprotective mechanism of autoregulation is lost below a MAP of 30 mm Hg. Objective: To determine whether hypotension defined as MAP <30 mm Hg or MAP less than the infants GA better predicts adverse outcomes of intraventricular hemorrhage (IVH) and death. Study Design: For this retrospective study, demographic, clinical, and BP data in epochs of 12 h were collected during the first 72 h of life in 188 subjects 24-28 weeks of gestation. For each definition, outcomes of severe IVH (grade 3 or 4), death, or the composite outcome of either were evaluated using bivariate testing. Logistic regression determined independent predictors of composite outcome of death and/or grade 3 or 4 IVH. Results: Hypotension by either definition was significant for death and the composite outcome (p < 0.0001). Only the MAP <30 mm Hg definition was associated with severe IVH (p = 0.02). On logistic regression, significant predictors of the composite outcome were GA (OR 0.59, 95% CI 0.39-0.89) and vasopressor therapy (OR 5.5, 95% CI 2-17). Conclusions: Neither definition of hypotension independently predicts adverse outcome in multivariate logistic regression. Vasopressor therapy, however, is an independent predictor of IVH and death in premature infants.

Management of asymptomatic neonates born in the setting of chorioamnionitis: a safety comparison of the well-baby and intensive care setting

Background:Evaluate the safety of a change in care setting for asymptomatic neonates born to mothers with chorioamnionitis from the neonatal intensive care unit to the well baby nursery.Local problem:The neonatal intensive care unit evaluation and management of babies born to mothers with chorioamionitis often involves separation of the mother-baby dyad and more invasive interventions.Methods:A single-center pre/post-intervention study of neonates born from January 2011 to November 2016, comparing safety outcomes in the neonatal intensive care unit (pre-intervention) and well baby nursery (post-intervention), following initiation of a triage protocol.Interventions:A protocolized, systematic change was done in the practice location.Results:Groups were similar for time to first antibiotic administration, sepsis symptom development and positive blood cultures. Length of stay (median 73.5 vs 64.4 h, P=0.0192) and % of neonates with intravenous fluid exposure (50.4% vs 7.6%, P<0.0001) were lower in the post-intervention group. Exclusive breastfeeding rates improved (pre—7.3% vs post—46.1%, P<0.0001).Conclusions:Asymptomatic neonates born to mothers with chorioamnionitis were safely treated in a well baby nursery under the guidance of a protocol for triage, thereby reducing NICU exposure for these neonates.

Utility of prenatal Doppler ultrasound to predict neonatal impaired cerebral autoregulation

ObjectiveDetermine if abnormal prenatal Doppler ultrasound indices are predictive of postnatal impaired cerebral autoregulation.Study designProspective cohort study of 46 subjects, 240–296 weeks’ gestation. Utilizing near-infrared spectroscopy and receiver-operating characteristic analysis, impaired cerebral autoregulation was defined as >16.5% time spent in a dysregulated state within 96 h of life. Normal and abnormal Doppler indices were compared for perinatal outcomes.ResultsSubjects with abnormal cerebroplacental ratio (n = 12) and abnormal umbilical artery pulsatility index (n = 13) were likely to develop postnatal impaired cerebral autoregulation (p ≤ 0.02). Abnormal cerebroplacental ratio was associated with impaired cerebral autoregulation between 24 and 48 h of life (p = 0.016). These subjects have increased risk for fetal growth restriction, lower birth weight, lower Apgar scores, acidosis, and severe intraventricular hemorrhage and/or death (p < 0.05).ConclusionAbnormal cerebroplacental ratio and umbilical artery pulsatility index are associated with postnatal impairment in cerebral autoregulation and adverse outcome.

Noninvasive Respiratory Support for the Premature Infant: Choosing the Optimum Interface

Preventing lung injury to reduce the burden of bronchopulmonary dysplasia is a crux of neonatal care. We know that decreasing the duration of intubation and duration of mechanical ventilation is a major component of injury prevention. As practices leading to earlier extubation become more common,

IMAGe syndrome in the era of genetic testing: clues to diagnosis

Abstract IMAGe syndrome (Intrauterine growth restriction (IUGR), Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies) is a rare, multisystem disorder caused by mutations in the PCNA-binding domain of CDKN1C. Reported here is a male infant diagnosed with IMAGe syndrome by CDKN1C sequencing at 3 months of age. He presented with IUGR, primary adrenal insufficiency with adrenal crisis in the neonatal period, dysmorphic facies, and bilateral cryptorchidism. Interestingly, he demonstrates several additional clinical findings not previously reported with IMAGe syndrome including congenital hypothyroidism, recurrent bacterial infections, and severe eczema.

Contents Vol. 99, 2011

S. Andersson, Helsinki E. Bancalari, Miami, Fla. J. Bhatia, Augusta, Ga. G. Buonocore, Siena W. Carlo, Birmingham, Ala. I. Choonara, Derby T. Curstedt, Stockholm C. Dani, Florence B. Darlow, Christchurch M. Fujimura, Osaka M. Hallman, Oulu W.W. Hay, Jr., Aurora, Colo. S.E. Juul, Seattle, Wash. M. Kaplan, Jerusalem B. Kramer, Maastricht R.J. Martin, Cleveland, Ohio C.J. Morley, Cambridge J. Neu, Gainesville, Fla. P.C. Ng, Hong Kong M.W. Obladen, Berlin A.G.S. Philip, Sebastopol, Calif. M. Post, Toronto E. Saliba, Tours O.D. Saugstad, Oslo M.S. Schimmel, Jerusalem B. Schmidt, Philadelphia, Pa. M.P. Sherman, Columbia, Mo. E.S. Shinwell, Rehovot K. Simmer, Perth, W.A. J. Smith, Tygerberg B. Sun, Shanghai N. Vain, Buenos Aires F. van Bel, Utrecht J.N. van den Anker, Washington, D.C. M. Vento Torres, Valencia M. Weindling, Liverpool J.A. Widness, Iowa City, Iowa Fetal and Neonatal Research