El Paso Children's Hospital

Inadequate treatment continues to be the leading cause of nonunion of slightly displaced fractures of pediatric lateral humeral condyles, despite references to prevention in the orthopaedic literature. In most cases, the elbow was established nonunion can be salvaged by early stabilization and bone grafting, provided that the fragment is in acceptable position and the growth plate of the condylar fragment is open. Procrastination may allow the physis of the condylar fragment to close prematurely, preemptying the golden opportunity to salvage the child's elbow. The author has collected 23 similar cases from correspondence with 17 orthopaedic surgeons.

Influenza is the most common cause of vaccine-preventable morbidity and mortality despite the availability of the conventional trivalent inactivated vaccine and the live-attenuated influenza vaccine. These vaccines induce an immunity dominated by the response to hemagglutinin (HA) and are most effective when there is sufficient antigenic relatedness between the vaccine strain and the HA of the circulating wild-type virus. Vaccine strategies against influenza may benefit from inclusion of other viral antigens in addition to HA. Epidemiologic evidence and studies in animals and humans indicate that anti-neuraminidase (NA) immunity will provide protection against severe illness or death in the event of a significant antigenic change in the HA component of the vaccine. However, there is little NA immunity induced by trivalent inactivated vaccine and live-attenuated influenza vaccine. The quantity of NA in influenza vaccines is not standardized and varies significantly among manufacturers, production lots and tested strains. The activity and stability of the NA enzyme is influenced by concentration of divalent cations. If immunity against NA is desirable, a better understanding of how the enzymatic properties affect the immunogenicity is needed.

OBJECTIVES: The objectives of this review are to discuss the mechanisms by which respiration impacts cardiovascular function and vice versa, with an emphasis on the impact of these interactions in pediatric cardiac critical care. DATA SOURCE: A search of MEDLINE was conducted using PubMed. CONCLUSIONS: In the presence of underlying cardiac and respiratory disease, the interplay between these two systems is significant and plays a pivotal role in the pathophysiology of acute and chronic phases of a wide spectrum of diseases. An understanding of these relationships is essential to optimizing the care of critically ill patients.

Many orthodontists working on patients with cleft lip and palate (CLP) have shown great enthusiasm for presurgical infant orthopedics (PSIO) to improve surgical outcomes with minimal intervention. Even though every clinician aims to use the best treatment modality for their patients, PSIO effects can be confounded by surgical type and timing of the primary repair, as is discussed in many studies. In such cases, one should be cautious when evaluating the particular outcomes for patients with CLP since it is difficult to differentiate the sole effect of an individual surgical or orthodontic intervention. As with any treatment methodology, nasoalveolar molding (NAM) has both benefits and limitations. Commonly cited concerns with NAM, and PSIO in general, include increased cost, increased burden of care, and a negative impact on maxillary growth. However, NAM cannot be deemed as having apparent long-term negative or positive effects on skeletal or soft tissue facial growth, based on previous studies. A review of the literature suggests that NAM does not alter skeletal facial growth when compared with the samples that did not receive PSIO. Nevertheless, the published studies on NAM show evidence of benefits to the patient, caregivers, the surgeon, and society. These benefits include documented reduction in severity of the cleft deformity prior to surgery and as a consequence improved surgical outcomes, reduced burden of care on the care givers, reduction in the need for revision surgery, and consequent reduced overall cost of care to the patient and society.

Many ad hoc fasting guidelines for pre-anesthetic patients prohibit gum chewing. We find no evidence that gum chewing during pre-anesthetic fasting increases the volume or acidity of gastric juice in a manner that increases risk, nor that the occasional associated unreported swallowing of gum risks subsequent aspiration. On the contrary, there is evidence that gum chewing promotes gastrointestinal motility and physiologic gastric emptying. Recommendations against pre-anesthetic gum chewing do not withstand scrutiny and miss an opportunity to enhance comfort and sense of wellbeing for patients awaiting anesthesia. Gum chewing during the pre-anesthetic nil per os (NPO) period would also permit the development of gum-delivered premedications and should be permitted in children old enough to chew gum safely. Gum chewing should cease when sedatives are given and all patients should be instructed to remove any chewing gum from the mouth immediately prior to anesthetic induction.

Rapid-onset obesity, hypoventilation, hypothalamic dysfunction, and autonomic dysfunction is an increasingly common diagnosis in patients who are being seen at tertiary care children's hospitals. We present two cases of anesthetics from the authors' own experience in addition to a comprehensive review of the disorder and anesthetic implications.

PURPOSE: Anaplastic lymphoma kinase (ALK) aberrations have been identified in pediatric-type infant gliomas, but their occurrence across age groups, functional effects, and treatment response has not been broadly established. EXPERIMENTAL DESIGN: We performed a comprehensive analysis of ALK expression and genomic aberrations in both newly generated and retrospective data from 371 glioblastomas (156 adult, 205 infant/pediatric, and 10 congenital) with in vitro and in vivo validation of aberrations. RESULTS: ALK aberrations at the protein or genomic level were detected in 12% of gliomas (45/371) in a wide age range (0-80 years). Recurrent as well as novel ALK fusions (LRRFIP1-ALK, DCTN1-ALK, PRKD3-ALK) were present in 50% (5/10) of congenital/infant, 1.4% (3/205) of pediatric, and 1.9% (3/156) of adult GBMs. ALK fusions were present as the only candidate driver in congenital/infant GBMs and were sometimes focally amplified. In contrast, adult ALK fusions co-occurred with other oncogenic drivers. No activating ALK mutations were identified in any age group. Novel and recurrent ALK rearrangements promoted STAT3 and ERK1/2 pathways and transformation in vitro and in vivo. ALK-fused GBM cellular and mouse models were responsive to ALK inhibitors, including in patient cells derived from a congenital GBM. Relevant to the treatment of infant gliomas, we showed that ALK protein appears minimally expressed in the forebrain at perinatal stages, and no gross effects on perinatal brain development were seen in pregnant mice treated with the ALK inhibitor ceritinib. CONCLUSIONS: These findings support use of brain-penetrant ALK inhibitors in clinical trials across infant, pediatric, and adult GBMs. See related commentary by Mack and Bertrand, p. 2567.

Most inflammatory myofibroblastic tumors (IMTs) harbor ALK fusions but oncogene fusions involving ROS1, RET, NTRK, and PDGFR also occur. The recognition that most IMTs harbor receptor tyrosine kinase fusions has provided a rationale for the use of tyrosine kinase inhibitors to target these oncogenic drivers in advanced IMTs. Crizotinib has been effective in ALK and ROS1-positive IMTs but resistance eventually develops. Here we report the successful use of lorlatinib in a patient with heavily pretreated ROS1-positive IMT of the chest wall with acquired crizotinib-resistance and metastasis to the brain.

OBJECTIVE: Dental implant placement in scapular free flaps is challenging. This study examines the scapula with computed tomography to identify ideal locations for predictable implant placement during preoperative planning. METHODS: Sixty-eight adult patient chest CT scans (34 men, 34 women) captured for various medical indications, were analyzed for age, height, weight, and scapula length. The lateral border of the scapula was divided into six equal segments; the midpoints of each segment (labeled proximally to distally as 1M-6M) were analyzed in cross-section as potential recipient sites for 3.5 × 8 mm implants. Also, we present a case of a 77-year-old male with ameloblastoma of the mandible who underwent patient specific planning and received a scapular free flap with dental implant placement. RESULTS: There was greater bone availability in males with a mean depth of 8.3 ± 2.8 versus 5.1 ± 3.3 mm in females (p < .01). The proximal portion (1M) of the scapula in males and females had depths of 11.3 ± 1.5 and 9.5 ± 2.3 mm, respectively. Males had depths of 8.4 ± 3.0 in M3, 9.7 ± 1.7 in M4, and 8.9 ± 1.2 mm in M6. Depth of bone available for patients with heights ≥165 cm versus <165 cm had means of 10.4 ± 1.3 and 8.0 ± 1.6 mm (p < .01), respectively; but showed no significant differences between BMI (BMI <25 vs. ≥25) and bone availability (6.8 ± 1.7 vs. 6.8 ± 1.6, p = .07), or age (<55 years vs. ≥55 years) and bone availability (9.8 ± 1.6 vs. 9.8 ± 1.6, p = .11). In our case, the patient received 6 cm length of scapular bone with four 4.1 × 14 mm endosteal implants, which upon osseointegration was able to receive a fixed dental prosthesis. Three years after the initial surgery, the patient has had no difficulty with his prosthesis. CONCLUSION: In females the most proximal portion of the scapula will predictably accommodate a dental implant, while males have multiple sites including the proximal, middle, and distal portions.

OBJECTIVE: To characterize the use and delivery of cough and cold medicines in children younger than 6 presenting to an inner-city pediatric emergency department (PED) following 2007 FDA warnings. METHODS: A cross-sectional observational study was performed using a convenience sampling of PED patients during the fall of 2010. Caregivers were presented with 6 commonly used cough medicine preparations and were asked to demonstrate if and how they would administer these to their children. RESULTS: In all, 65 patients and their caregivers consented and participated in the study. During the demonstration, 82% (53/65) stated that they would treat with cough or cold medicines, and 72% (38/53) incorrectly dosed the medication they desired to give. CONCLUSIONS: Despite current recommendations, cough and cold medicines are still used in children younger than 6 years of age. A significant portion of caregivers report that they are still unaware of public warnings, potential side effects, and interactions with other medications.

We report a retrospective case series of six Hispanic children with tumors treated with metronomic chemotherapy. The six cases comprised one rhabdoid tumor of the kidney, one ependymoma, two medulloblastomas, one neuroblastoma, and a type II neurocytoma of the spine. Treatment included oral cyclophosphamide daily for 21 days alternating with oral etoposide daily for 21 days in a backbone of daily valproic acid and celecoxib. In one case, celecoxib was substituted with sulindac. Of the six patients, three showed complete responses, and all patients showed some response to metronomic therapy with only minor hematologic toxicity. One patient had hemorrhagic gastritis likely associated with NSAIDs while off prophylactic antacids. These data add to a growing body of evidence suggesting that continuous doses of valproic acid and celecoxib coupled with alternating metronomic chemotherapy of agents such as etoposide and cyclophosphamide can produce responses in pediatric tumors relapsing to conventional dose chemotherapy.

Congenital anomalies of the kidneys and urinary tract (CAKUT) are developmental disorders that commonly cause pediatric chronic kidney disease and mortality. We examine here rare coding variants in 248 CAKUT trios and 1742 singleton CAKUT cases and compare them to 22,258 controls. Diagnostic and candidate diagnostic variants are detected in 14.1% of cases. We find a significant enrichment of rare damaging variants in constrained genes expressed during kidney development and in genes associated with other developmental disorders, suggesting phenotype expansion. Consistent with these data, 18% of CAKUT patients with diagnostic variants have neurodevelopmental or cardiac phenotypes. We identify 40 candidate genes, including CELSR1, SSBP2, XPO1, NR6A1, and ARID3A. Two are confirmed as CAKUT genes: ARID3A and NR6A1. This study suggests that many yet-unidentified syndromes would be discoverable with larger cohorts and cross-phenotype analysis, leading to clarification of the genetic and phenotypic spectrum of developmental disorders. The authors analyze rare coding variants in 1990 individuals with congenital kidney anomalies, finding diagnostic variants in 14.1% of cases. They identify two new causal genes, ARID3A and NR6A1, along with 38 candidate genes, providing evidence for shared genetics with other developmental disorders.

Embryonal tumor with multilayered rosettes (ETMR) are rare pediatric brain tumors with increased malignant potential. Despite the advances in multimodal treatment schemes the overall 5-year event free survival rates for ETMR are not favorable. Further, therapeutic regimes are limited to a case by case basis due to the limited amount of literature and guidelines available for treating childhood ETMR. We report one patient with refractory ETMR who was successfully treated by implementing a molecular profiling approach which identified the tyrosine kinase inhibitor dasatinib as a viable therapy. Our results suggest that utilizing this precision medicine approach might prove useful in treating patients with refractory ETMR.

BACKGROUND AND OBJECTIVES: Early onset sepsis (EOS) is an important cause of neonatal morbidity and mortality. Timely administration of antibiotics is crucial in management. We initiated a quality improvement project to improve timely administration of antibiotics. METHODS: Primary drivers of change identified by the team were improving delivery of antibiotics from pharmacy and improving time to admit in the electronic medical record (EMR) in order to improve overall timeliness of antibiotics administration. Timings of antibiotics administration was tracked by using a control chart. Timings of antibiotics and outcomes of pre-intervention (December 2016) were compared with post intervention of PDSA cycles (January 2017-November 2018). RESULTS: There was statistically significant improvement in time to admission in electronic medical records over the time periods of pre-intervention, PDSA I and PDSA II (p-value < 0.05) (Table 1). Also, time to delivery of antibiotics from pharmacy was significantly reduced between PDSA cycles from 21 minutes to 9 minutes with improvement in overall workflow. An average time to infusion of antibiotics decreased from 70 minutes to 48 minutes. There was also overall improvement in number of neonates receiving antibiotics under 1 hour of decision making from 37% to 77%. CONCLUSIONS: In our study we were able to successfully implement our "antibiotics under one hour" goal. The ability to achieve this objective can be met across multi-institutions rendering care to newborns if the approach is multidisciplinary. Deleting obstructions in the process that involve admission, registration and entry into the EMR effectively reduced time.

Background: Apnea of prematurity (AOP) affects preterm neonates. AOP, combined with intermittent hypoxemic (IH) events frequently prolongs the length of stay. Caffeine is the preferred medication to treat AOP and may help improve IH events. There is lack of information on the safety of discharging preterm neonates home on caffeine for AOP in the literature. Our objective was to assess safety and benefits, if any, of discharging preterm infants home on caffeine. Methods: After IRB approval, preterm infants discharged home from the neonatal intensive care unit (NICU) on caffeine were compared with those without a discharge prescription for the period of January 2013 to December 2017. Results: A total of 297 infants were started on caffeine, and of those, 87 infants were discharged home on caffeine. There was no difference in length of stay between two groups. Duration of caffeine at home was 31 (28–42) days. The average cost of apnea monitor and caffeine at home per 30 days was USD 1326 and USD 50. There was no difference in number or reasons for emergency department (ED) visits or hospitalizations between two groups. Conclusion: AOP affects almost all preterm infants and along with intermittent hypoxemic events, and is one of the most common reasons for prolonged hospital stay. Discharging stable preterm infants home on caffeine may be safe, especially in those who are otherwise ready to be discharged and are only awaiting complete resolution of AOP/IH events.

Pelvic pyomyositis is an infection of the skeletal muscles around the hip joint. Fever, hip pain, limp, and leukocytosis are common; however, the clinical picture is often vague. MRI is the current gold-standard imaging for pyomyositis. No studies in the current literature have reported an unremarkable initial MRI in a patient with symptomatic pyomyositis. An adolescent female presented with symptomatic pelvic pyomyositis, but admission MRI was normal. A follow-up MRI indicated development of pelvic pyomyositis. The patient was successfully managed nonoperatively. The initial MRI in pyomyositis can be misleading. Patients should be admitted and MRI should be repeated, as indicated clinically.

OBJECTIVES: Inconsistent results have been reported by authors of studies of the management of pediatric patients with ovarian torsion (OT). Our objective was to identify predictors of oophorectomy in girls hospitalized throughout Texas with OT. METHODS: The Texas Public Use Data File (years 2013–2014) was queried for the records of girls under the age of 18 years who had a principal or secondary discharge diagnosis of OT (International Classification of Diseases, Ninth Revision, Clinical Modification code 620.5). Adjusted odds ratios were estimated from a logistic regression model by using Firth’s bias-reducing penalized likelihood. Variables for inclusion in the final model were identified by using a directed acyclic graph. RESULTS: A sample of 158 girls was identified with an overall risk of oophorectomy during the hospital stay of 41.1% (65 out of 158). After adjusting for the patient’s age, health insurance status, and the presence of an ovarian cyst, girls who were treated at a nonteaching hospital were more than twice as likely to undergo oophorectomy than girls who were treated at a teaching hospital (odds ratio = 2.22; 95% confidence interval: 1.05–4.69). CONCLUSIONS: Our analysis of a statewide database revealed that girls with OT who presented at nonteaching hospitals were significantly more likely to undergo oophorectomy compared with girls who presented at teaching hospitals.

Background: The ability to complete nipple feedings is one of the discharge criteria for most premature neonates. The Infant Driven Feeding (IDF) program suggests a system of objective promotion of oral feeds in premature infants. There is a lack of studies systematically studying the effects of IDF on the provision of breast milk. Methods: This was a retrospective study of all premature infants born before 33 weeks and birth weight of &lt;1,500 g admitted to a level IV neonatal intensive care unit. Infants on IDF were compared with those not on IDF. Results: A total of 46 infants in the IDF group and 52 in the non-IDF group met the inclusion criteria. A higher number of infants in the IDF group breastfed at first oral attempt (54% versus 12%). Forty-five percent of IDF mothers completed a full 72 hours of protected breastfeeding at the start of oral feeds, and IDF infants had earlier removal of nasogastric (NG) tube. There was no difference in the provision of breast milk and/or breastfeeding on discharge between the two groups. There was no difference in the length of stay between the two groups. Conclusion: The IDF program attempts to streamline the promotion of oral feeds in very low birth weight infants. Higher incidence of breastfeeding at the start of oral feeds and earlier removal of NG tube did not translate into higher provision of breast milk on discharge in very low birth weight infants in the IDF group. Prospective randomized trials are needed to validate cue-based infant driven feeding programs and their effects on the provision of breast milk.

OPINION article Front. Pediatr., 21 August 2017Sec. Children and Health Volume 5 - 2017 | https://doi.org/10.3389/fped.2017.00177

This 5-year study evaluated a virtual visitation implementation initiative in a neonatal intensive care unit. Our objectives were to (1) use the Plan-Do-Study-Act methodological framework to implement a virtual visitation program, (2) investigate whether implementation of virtual visitation could be done with no patient harm and minimal workflow disruption, (3) foster a top-down participatory structure for decision making, and (4) evaluate parent use and satisfaction. The study involved a qualitative and quantitative description of cycles and results. Routine collection of outcome data allowed problems that arose as a result of changing practices to be quickly and efficiently addressed. The study results suggested that the virtual visitation implementation initiative in a neonatal intensive care unit using Plan-Do-Study-Act cycles helped create an environment of trust and provided benefits. A steady increase in the use of virtual visitation by parents and their extended families indicated utilization. During the COVID-19 pandemic, virtual visitation helped families feel connected with each other and their neonate, despite being in separate locations.