Erica Wymore

Enteral Feeding in Prostaglandin-Dependent Neonates: Is It a Safe Practice?

In many centers presurgical term neonates with prostaglandin-dependent cardiac lesions experience nutritional deficiency because of postponed enteral feeds. We recently adopted early enteral feeding in these infants. This retrospective study demonstrates feeding tolerance in 33 of 34 neonates fed enterally while receiving prostaglandin, suggesting the safety of this practice.

Enteral feeding in neonates with prostaglandin-dependent congenital cardiac disease: international survey on current trends and variations in practice

BACKGROUND The benefits of early enteral feeding in neonates are well known and yet the optimal pre-operative nutrition of prostaglandin-dependent infants with congenital cardiac disease remains ill-defined. This survey delineates international nutritional practices and trends with this population. MATERIALS AND METHODS Paediatric practitioners responded to an Internet-based survey that explored assessment of feeding practices, criteria for feeding readiness, medication dosing, concurrent feeding with umbilical catheters, and the observed incidence of feeding intolerance. Documented nutritional strategies were not correlated with patient outcomes. RESULTS A total of 200 caregivers responded to the survey. Fewer United States caregivers (56%) reported routine pre-operative enteral feeding in prostaglandin-dependent infants when compared with caregivers outside the United States of America (93%). Of those respondents willing to feed, approximately two-thirds did not base their decision on the ductal flow direction. Numerous and heterogeneous parameters were reported to assess feeding readiness. Many caregivers report scepticism with regard to enteral feeding safety in neonates with an umbilical artery catheter, and to a lesser extent in the presence of an umbilical venous catheter. In summary, there is a prevailing lack of consensus regarding pre-operative enteral nutrition to prostaglandin-dependent neonates. CONCLUSIONS This survey demonstrates noticeable variations in pre-operative nutritional practices between providers from around the world. Arguments that support or refute this practice have little support in the medical literature. Future studies should aim to demonstrate the safety of such practice and compare the outcomes of prostaglandin-dependent neonates who were pre-operatively enterally fed with those who were not.

Cannabis Use During the Perinatal Period in a State With Legalized Recreational and Medical Marijuana: The Association Between Maternal Characteristics, Breastfeeding Patterns, and Neonatal Outcomes

Objectives To evaluate state‐level prevalence estimates of prenatal and early postnatal cannabis use in a state with legalized medical and recreational marijuana and the association with adverse neonatal outcomes. Study design We conducted a cross‐sectional study on 3,207 respondents from the 2014‐2015 Colorado Pregnancy Risk Assessment Monitoring System with state‐developed questions on cannabis use. Differences in perinatal cannabis use were evaluated according to maternal characteristics, breastfeeding patterns, and pregnancy intendedness. Multiple logistic regression models evaluated the relationship between prenatal cannabis use and adverse neonatal outcomes including low birth weight, small for gestational age, preterm birth, and admission to the neonatal intensive care unit. Results The self‐reported prevalence of cannabis use at any time during pregnancy was 5.7 ± 0.5% and the prevalence of early postnatal cannabis use among women who breastfed was 5.0% (95% CI, 4.1%‐6.2%). Prenatal cannabis use was associated with a 50% increased likelihood of low birth weight, independent of maternal age, race/ethnicity, level of education, and tobacco use during pregnancy (OR, 1.5; 95% CI, 1.1‐2.1; P = .02). Small for gestational age, preterm birth, and neonatal intensive care unit admission were not associated with prenatal cannabis use, independent of prenatal tobacco use. Conclusions Our findings underscore the importance of screening for cannabis use during prenatal care and the need for provider counselling about the adverse health consequences of continued use during pregnancy and lactation.

The Impact of Pulmonary Hypertension in Preterm Infants with Severe Bronchopulmonary Dysplasia through 1 Year

Objectives To assess the effect of pulmonary hypertension on neonatal intensive care unit mortality and hospital readmission through 1 year of corrected age in a large multicenter cohort of infants with severe bronchopulmonary dysplasia. Study design This was a multicenter, retrospective cohort study of 1677 infants born <32 weeks of gestation with severe bronchopulmonary dysplasia enrolled in the Childrens Hospital Neonatal Consortium with records linked to the Pediatric Health Information System. Results Pulmonary hypertension occurred in 370 out of 1677 (22%) infants. During the neonatal admission, pulmonary hypertension was associated with mortality (OR 3.15, 95% CI 2.10‐4.73, P < .001), ventilator support at 36 weeks of postmenstrual age (60% vs 40%, P < .001), duration of ventilation (72 IQR 30‐124 vs 41 IQR 17‐74 days, P < .001), and higher respiratory severity score (3.6 IQR 0.4‐7.0 vs 0.8 IQR 0.3‐3.3, P < .001). At discharge, pulmonary hypertension was associated with tracheostomy (27% vs 9%, P < .001), supplemental oxygen use (84% vs 61%, P < .001), and tube feeds (80% vs 46%, P < .001). Through 1 year of corrected age, pulmonary hypertension was associated with increased frequency of readmission (incidence rate ratio [IRR] = 1.38, 95% CI 1.18‐1.63, P < .001). Conclusions Infants with severe bronchopulmonary dysplasia‐associated pulmonary hypertension have increased morbidity and mortality through 1 year of corrected age. This highlights the need for improved diagnostic practices and prospective studies evaluating treatments for this high‐risk population.

Validation of the Colorado Retinopathy of Prematurity Screening Model

Importance The Colorado Retinopathy of Prematurity (CO-ROP) model uses birth weight, gestational age, and weight gain at the first month of life (WG-28) to predict risk of severe retinopathy of prematurity (ROP). In previous validation studies, the model performed very well, predicting virtually all cases of severe ROP and potentially reducing the number of infants who need ROP examinations, warranting validation in a larger, more diverse population. Objective To validate the performance of the CO-ROP model in a large multicenter cohort. Design, Setting, Participants This study is a secondary analysis of data from the Postnatal Growth and Retinopathy of Prematurity (G-ROP) Study, a retrospective multicenter cohort study conducted in 29 hospitals in the United States and Canada between January 2006 and June 2012 of 6351 premature infants who received ROP examinations. Main Outcomes and Measures Sensitivity and specificity for severe (early treatment of ROP [ETROP] type 1 or 2) ROP, and reduction in infants receiving examinations. The CO-ROP model was applied to the infants in the G-ROP data set with all 3 data points (infants would have received examinations if they met all 3 criteria: birth weight, <1501 g; gestational age, <30 weeks; and WG-28, <650 g). Infants missing WG-28 information were included in a secondary analysis in which WG-28 was considered fewer than 650 g. Results Of 7438 infants in the G-ROP study, 3575 (48.1%) were girls, and maternal race/ethnicity was 2310 (31.1%) African American, 3615 (48.6%) white, 233 (3.1%) Asian, 40 (0.52%) American Indian/Alaskan Native, and 93 (1.3%) Pacific Islander. In the study cohort, 747 infants (11.8%) had type 1 or 2 ROP, 2068 (32.6%) had lower-grade ROP, and 3536 (55.6%) had no ROP. The CO-ROP model had a sensitivity of 96.9% (95% CI, 95.4%-97.9%) and a specificity of 40.9% (95% CI, 39.3%-42.5%). It missed 23 (3.1%) infants who developed severe ROP. The CO-ROP model would have reduced the number of infants who received examinations by 26.1% (95% CI, 25.0%-27.2%). Conclusions and Relevance The CO-ROP model demonstrated high but not 100% sensitivity for severe ROP and missed infants who might require treatment in this large validation cohort. The model requires all 3 criteria to be met to signal a need for examinations, but some infants with a birth weight or gestational age above the thresholds developed severe ROP. Most of these infants who were not detected by the CO-ROP model had obvious deviation in expected weight trajectories or nonphysiologic weight gain. These findings suggest that the CO-ROP model needs to be revised before considering implementation into clinical practice.

Validation of the CHOP model for detecting severe retinopathy of prematurity in a cohort of Colorado infants

Editor, A recently published large-scale study (Mimouni et al. 2016) identified hyperopia as the parameter that demonstrated the strongest association with retreatment after excimer laser refractive surgery. We do share this observation as we also note less accurate refractive predictability and, most frequently, final undercorrection after hyperopic corneal refractive surgery. To improve refractive predictability, preceding studies (Zaldivar et al. 2005; Spadea et al. 2006) suggested that preoperative cycloplegic or manifest refraction, or a combination of both (Zadok et al. 2003), could be used in the laser nomogram. Previously, we reported that a manifest-cycloplegic difference (MCD) in spherical equivalent (SE) of 1.00 dioptre (D) or more occurs in about 13% of hyperopic eyes. In these cases, a correction of the manifest SE only did not appear to be adequate (Frings et al. 2016a). In a recent multicentre study, we thus evaluated the efficacy, predictability and safety of LASIK retreatment based on manifest refraction in hyperopic patients with a preoperative difference between cycloplegic and manifest refraction of 1.00 D or less who had LASIK retreatment based on manifest refraction. We analysed the refractive outcome of 113 hyperopic eyes according to standard graphs for reporting the efficacy, predictability and safety of refractive surgery. We found that efficacy (p < 0.001) and safety (p = 0.004) were statistically significant improved by the retreatment without being negatively influenced by preoperative manifest SE, manifest cylinder or keratometry, which we analysed. Still showing a trend towards undercorrection, retreatment resulted in 88 eyes (78.0%) that reached 0.50 D of the attempted correction. The optical zone diameter of the retreatment did not correlate with efficacy, predictability or safety. Treatment predictability, however, was statistically significant worse in eyes with a preoperative SE of more than 2.50 D (p = 0.005, tested with chisquare test). In our opinion, the main objective criterion for a retreatment is a difference between targeted and achieved manifest SE of 0.50 D or more after at least 6 months. We do not retreat eyes earlier after LASIK as keratometric and refractive changes are likely to occur up to 6 months (Frings et al. 2016b). The results of our study indicate that in hyperopic eyes with a preoperative difference between cycloplegic and manifest refraction of 1.00 D or less a LASIK retreatment is efficient, predictable and safe and therefore, finally meets preoperative patients’ expectations. To summarize, the improvement of hyperopic LASIK still is a matter of discussion, hyperopes should not be treated earlier than 6 months and the difference between cycloplegic and manifest refraction should be taken into account during treatment planning. Many hyperopic cases will likely need a retreatment, most of which result in high efficacy, predictability and safety after all. We would highly recommend clarifying this fact to hyperopic LASIK candidates before the treatment.

Lethal neonatal hyperammonemia in severe ornithine transcarbamylase (OTC) deficiency compounded by large hepatic portosystemic shunt

A term male infant presented at 48 h of age with apnea, lethargy, and seizures. Plasma ammonia was 1180 μmol/ L, and biochemical studies (plasma citrulline <2 μM; urine orotate 1142 μg/ng creatinine) were consistent with a proximal urea cycle disorder (UCD). Initially, hyperammonemia improved with continuous renal replacement therapy (cRRT) and ammonia scavengers. Upon discontinuation of cRRT, hyperammonemia worsened and lactic acidosis developed, with coexistent fulminant hepatic and renal failure. Color Doppler ultrasound of the liver (Fig. 1a–d identified a large congenital intrahepatic portosystemic shunt (PSS) with enlarged middle hepatic vein (MHV) with abnormal tortuous portosystemic shunt to the left portal vein (LPV). Congenital PSS occurs in approximately 1:25,000 births and is classified as either intraor extrahepatic (Stringer 2008). Shunts bypassing hepatic circulation can be associated with hyperammonemia and encephalopathy, even in the setting of normal urea cycle function (Witters et al. 2008; Kim et al. 2012; Sokollik et al. 2013; Van Straten et al. 2014). Plasma glutamine was initially massively elevated (>3000 μM), but it normalized with cRRT and ammonia scavengers despite persistence of recalcitrant hyperammonemia. OTC deficiency was confirmed by identification of a 4.3-Mb hemizygous deletion involving Xp21.1–Xp11.4 and incorporating the entire OTC locus, portending absent functional OTC enzymes and severe disease. The hemizygous Xp deletion, also involving other key genes (including CYBB and XK, associated with chronic granulomatous disease and McLeod syndrome, respectively), together with the PSS, caused recalcitrant, fatal hyperammonemia and lactic acidosis off cRRT. Autopsy revealed extensive hepatic centrilobular necrosis and dilated vascular spaces. This case illustrates the devastating effects of hepatic vascular anomalies in patients with coexistent UCD.