Alicia A. Moise

Long-term neurodevelopmental outcome after intrauterine transfusion for the treatment of fetal hemolytic disease

OBJECTIVE The aim of the study was to assess the developmental outcome of neonatal survivors of hemolytic disease of the neonate treated with modern intrauterine transfusion techniques. STUDY DESIGN In this prospective, observational study, auditory evoked-response tests were performed in the nursery. Neurodevelopmental evaluation with the Gesell Developmental Schedules was performed between 9 and 18 months of corrected age to assess motor skills, language development, comprehension capacity, and social skills. The McCarthy Scales of Childrens Abilities were administered between 36 and 62 months. RESULTS Forty children who survived severe fetal hemolytic disease were followed up until 62 months old. Demographic data included gestational age at first intrauterine transfusion (26.4 +/- 3.7 weeks), median number of intrauterine transfusions (4, range 1-8), lowest fetal hematocrit (20.2% +/- 7.8%), peak fetal bilirubin (7.1 +/- 2.1 mg/dL), incidence of hydrops fetalis (45%), and mean gestational age at delivery (35.6 +/- 2.2 weeks). One case of severe bilateral deafness and 1 case of right spastic hemiplegia were diagnosed. The Gesell Developmental Schedules score was assessed between 9 and 18 months of corrected age in 22 infants. The global developmental quotient was 101.9 +/- 9.5 (mean for normal population is 100). Regression analysis revealed no correlation between the global developmental quotient and gestational age at the first intrauterine transfusion, gestational age at birth, or the severity of the fetal hemolytic disease (fetal hematocrit, fetal bilirubin, presence of hydrops fetalis, total number of intrauterine transfusions, duration of neonatal phototherapy, and number of neonatal exchange transfusions). Eleven of the 40 children were followed up until they were 62 months old, and the McCarthy Scales of Childrens Abilities were administered. The mean cognitive index was 107.6 +/- 9.4 (90-109 is considered average). CONCLUSION Despite severe fetal hemolytic disease, normal developmental outcome can be expected for children treated with intrauterine transfusions.

Percutaneous Central Catheters and Peripheral Intravenous Catheters Have Similar Infection Rates in Very Low Birth Weight Infants

OBJECTIVE:We performed this study to determine if percutaneous central lines (PCLs) were associated with infection more often than peripherally placed intravenous catheters (PIVs).STUDY DESIGN:We conducted a retrospective, cohort study of 53 infants with PCLs inserted from March 1993 to February 1995 for evidence of catheter-related bloodstream infection and 97 cohorts with PIVs who were matched to the infants with PCLs by admission date and birth weight. We considered an infant to have catheter-related bloodstream infection if bacteremia occurred while the PCL or PIV was in place with no other identifiable infection focus. Statistical analyses were performed by using either Student’s t test or the Mann-Whitney U test where appropriate.RESULTS:There were eight infections per 1000 catheter days of PCL use and nine infections per 1000 catheter days of PIV use.CONCLUSION:PCLs do not become infected more often than PIVs.

Repair of congenital diaphragmatic hernias on Extracorporeal Membrane Oxygenation (ECMO): Does early repair improve patient survival?

INTRODUCTION The optimal timing of repair for congenital diaphragmatic hernia (CDH) patients that require ECMO is controversial. Early repair on ECMO theoretically allows for restoration of normal thoracic anatomy but entails significant bleeding risks. The purpose of this study was to examine the institutional outcomes of early CDH repair on ECMO. METHODS The records of infants with CDH placed on ECMO from 2001 to 2011 were reviewed. Since 2009, a protocol was instituted for early repair while on ECMO. For this study, three cohorts were analyzed: early repair (<72 h), late repair (>72 h), and post-decannulation. These groups were compared for outcomes regarding morbidity and survival. RESULTS Forty-six CDH patients received ECMO support with an overall survival of 53%. Twenty-nine patients (11 early/18 late) were repaired on ECMO, while 17 patients had repair post-decannulation. Survival was 73%, 50%, and 64% for those repaired early, late, or post-decannulation, respectively. Despite significantly worse prenatal factors, patients repaired early on ECMO had a similar survival. When comparing patients repaired on ECMO, the early group patients were decannulated 6 days earlier (p-value=0.009) and had significantly lower circuit complications (p=0.03). CONCLUSION In conclusion, early repair on ECMO was associated with decreased ECMO duration, decreased circuit complications, and a trend towards improved survival.

Reversal of Venous Blood Flow with Atrial Tachycardia and Hydrops in Fetal Sheep

ABSTRACT: The purpose of this project was to characterize the reversal of blood flow in the proximal inferior vena cava (IVC) seen in fetal sheep with pacing-induced atrial tachycardia and hydrops. We successfully operated on seven pregnant ewes at 118–130 d gestation to attach ECG and pacing wires, insert vascular catheters, and place Doppler flow probes around the common aortic trunk and the IVC. We also performed two-dimensional and Doppler ultrasonographic exams at baseline, after initiation of pacing, and daily thereafter. All fetuses developed hydrops. Ultrasonographic appearance of ascites and pleural effusion occurred within 4 h in four fetuses and within 48 h in all fetuses. Atrial pacing did not affect arterial pH or arterial O2 tension, but arterial CO2 tension increased by a small amount. Mean IVC pressure increased 75%, whereas mean aortic pressure remained the same. Concentrations of plasma protein and albumin and the hematocrit did not change with atrial pacing. Doppler ultrasound examination and Doppler IVC flow tracings showed that flow reversal began immediately with atrial pacing and disappeared immediately with cessation of pacing. Reversed flow was 21% of forward flow. Inspection of simultaneous recordings of ECG, Doppler aortic and IVC flows, and aortic and IVC pressure tracings revealed that the reversed blood flow occurred in diastole in conjunction with atrial contraction and, therefore, could not be due to tricuspid insufficiency. Our findings of increased venous pressure and reversed venous blood flow suggest that ventricular function is impaired and further suggest that oxygen supply to the ventricles may not be sufficient for the increased demand.

Tracheostomy placement in infants with bronchopulmonary dysplasia: safety and outcomes.

Optimizing the timing and safety for the placement of a tracheostomy in infants with bronchopulmonary dysplasia (BPD) has not been determined. The purpose of the present study was to describe the data from a single institution about the efficacy and safety of tracheostomy placement in infants with BPD needing long‐term respiratory support. We established a service line for the comprehensive care of infants with BPD and we collected retrospective clinical data from this service line. We identified patients that had a trachostomy placed using the local Vermont‐Oxford database, and obtained clinical data from chart reviews. We identified infants who had a tracheostomy placed for the indication of severe BPD only. Safety and respiratory efficacy was assessed by overall survival to discharge and the change in respiratory supportive care from just before placement to 1‐month post‐placement. Twenty‐two patients (750 ± 236 g, 25.4 ± 2.1 weeks gestation) had a tracheostomy placed on day of life 177 ± 74 which coincided with a post‐conceptual age of 51 ± 10 weeks. At placement these infants were on high settings to support their lung disease. The mean airway pressure (MAP) was 14.3 ± 3.3 cmH2O, the peak inspiratory pressure was 43.7 ± 8.0 cmH2O, and the FiO2 was 0.51 ± 0.13. The mean respiratory severity score (MAP × FiO2) 1 month after tracheostomy was significantly (P = 0.03) lower than prior to tracheostomy. Survival to hospital discharge was 77%. All patients with tracheostomies that survived were discharged home on mist collar supplemental oxygen. In conclusion, the high survival rate in these patients with severe BPD and the decreased respiratory support after placement of a tracheostomy suggests that high ventilatory pressures should not be a deterrent for placement of a tracheostomy. Future research should be aimed at determining optimal patient selection and timing for tracheostomy placement in infants with severe BPD. Pediatr Pulmonol. 2013; 48:245–249.

Reduction in Plasma Protein Does Not Affect Body Water Content in Fetal Sheep

ABSTRACT: We performed this study to determine if isolated hypoproteinemia and low colloid osmotic pressure cause formation of fetal edema. We successfully operated on six sets of twin fetal sheep at 114 d gestation to insert catheters into arteries and veins of both fetuses, allowing us to chronically perform partial exchange transfusions. One twin underwent protein reduction by repeated partial exchange transfusion over 3 d, and the other twin underwent simultaneous sham procedures. We removed an average of 18 g of protein, causing a 41% decrease in plasma protein concentration and a 44% decrease in colloid osmotic pressure. Vascular pressures, heart rate, hematocrit, plasma osmolarity, arterial pH, and arterial Po2 were not affected by protein reduction or by sham procedure, whereas Pco2 increased by a small amount in both groups. At autopsy, none of the fetuses in either group were edematous. Measurements of total body water by the wet to dry method, chloride space, and amniotic and allantoic fluid volumes were similar in both groups. We conclude that hypoproteinemia of a short duration does not affect the body water content of fetal sheep.

Prenatal ultrasonographic description and postnatal pathological findings in atelosteogenesis type 1

Atelosteogenesis type 1 (AO1) is a rare lethal chondrodysplasia characterized by incomplete ossification of cartilage anlagen. Histologically, the cartilage contains irregular clusters that occasionally include giant chondrocytes. Pulmonary hypoplasia is a characteristic finding that has been presumed to be the cause of neonatal lethality. We report on a male fetus with AO1 and document the early ultrasonographic/ radiologic progression of this disorder from 15 weeks gestation until delivery at 41 weeks. While the radiological findings we describe are typical of AO1 by the lack of proximal and middle phalangeal ossification, the complete radiological picture showed considerable overlap with boomerang dysplasia. Although pulmonary hypoplasia was present, it was moderate and considered unlikely to be the sole cause of death. Detailed neonatal and postmortem examination showed severe subglottic hypoplasia and tracheomalacia. The tracheal walls were supported by thin and pliable cartilaginous plates that allowed luminal collapse with minimal pressure. The marked luminal narrowing, tracheomalacia, and temporal proximity of extubation to demise support tracheal collapse as a major contributor to the death in AO1. The detailed description of this patient should contribute to earlier diagnosis of this condition; anticipation of the poor prognosis in AO1 is essential for appropriate genetic counseling of the parents and for determining postnatal treatment options.

A Single Dose of Dexamethasone (Dex.) Given at Delivery to Infants < 28 Weeks Gestation Results in Improved Lung Function, Early Extubation, Higher BP and Less PDA |[dagger]| 1046

Study: We tested the hypothesis that a 0.2 mg/kg dex. dose at delivery improves lung function, increases BP and prevents IVH in .2. Results: Within hours of the dex. dose the Ventilation Index improved and the effect persisted as shown by the following p values: Table Mean BP was initially similar between groups, but in the dex. infants it became significantly higher on day 1 and remained higher (within the physiologic range) through day 6 (p<.025 on each day). The dex. infants had fewer PDAs (10/32 vs 18/31, p<.03), and fewer received indocin (10/32 vs 17/31, p <.06). At the study hospital where early extubation was practiced more dex. infants were extubated during the first week (10/22 vs 2/16, p <.03). There was no difference in IVH. No adverse effects occurred. Conclusion: A single 0.2 mg/kg dex. dose given at delivery to infants < 28 weeks gestation resulted in rapid improvements which lasted through the first week and included reduced ventilator settings and earlier extubations, higher BP and fewer PDAs.

Is Activated Clotting Time an Accurate Measure of Heparinization for Neonates Receiving ECMO? † 999

Is Activated Clotting Time an Accurate Measure of Heparinization for Neonates Receiving ECMO? † 999

PERCUTANEOUS CENTRAL LINES AND PERIPHERAL INTRAVENOUS CATHETERS HAVE SIMILAR INFECTION RATES IN VERY LOW BIRTH WEIGHT INFANTS. 1401

PERCUTANEOUS CENTRAL LINES AND PERIPHERAL INTRAVENOUS CATHETERS HAVE SIMILAR INFECTION RATES IN VERY LOW BIRTH WEIGHT INFANTS. 1401