Reaching consensus for unified medical language in Fontan care.

Aims: The Fontan operation has resulted in improved survival in patients with single-ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co-morbidities have been increasingly recognized in this population due to the unique features of the Fontan circulation.

Antiseizure Medication Withdrawal Following Epilepsy Surgery.

As pediatric epilepsy surgery cases increase in number and complexity, there remains a paucity of data regarding how, when, and in whom to discontinue antiseizure medications postoperatively. The "TimeToStop" data has been influential to clinical practice, revealing that while early discontinuation of antiseizure medications may reveal surgical failures earlier, it does not ultimately lead to a change in long-term seizure outcomes. The authors of other studies have also shown cognitive improvements in children for whom medications were discontinued postoperatively.

The Interdisciplinary Stem Cell Institute's Use of Food and Drug Administration-Expanded Access Guidelines to Provide Experimental Cell Therapy to Patients With Rare Serious Diseases.

The U.S. Food and Drug Administration (FDA) provides guidance for expanded access to experimental therapies, which in turn plays an important role in the Twenty-first Century Cures Act mandate to advance cell-based therapy.

Epidemiology of Acute Kidney Injury After Neonatal Cardiac Surgery: A Report From the Multicenter Neonatal and Pediatric Heart and Renal Outcomes Network.

Objectives: Cardiac surgery-associated acute kidney injury occurs commonly following congenital heart surgery and is associated with adverse outcomes. This study represents the first multicenter study of neonatal cardiac surgery-associated acute kidney injury. We aimed to describe the epidemiology, including perioperative predictors and associated outcomes of this important complication.

State of the science and future research directions in palliative and end-of-life care in paediatric cardiology: a report from the Harvard Radcliffe Accelerator Workshop.

Workshop proceedings, priorities, and recommendations from the "State of the Science and Future Directions in Palliative and End-of-Life Care in Pediatric Cardiology," a Harvard Radcliffe Accelerator Workshop, are detailed. Eight priorities for research were identified, including patient and family decision making, communication, patient and family experience, patient symptom measurement and management, training and curriculum development, teamwork, family hardships and bereavement, and ethical considerations. Barriers to research in this area were also identified: lack of outcome/measurement tools, lack of research funding, small population sizes, lack of effort/protected time for research, undervalued research topic by field and colleagues, and heterogeneous research participant diversity.

Single-stage restorative proctocolectomy for ulcerative colitis in pediatric patients: a safe alternative.

Background: Surgical management for refractory ulcerative colitis (UC) has been restorative proctocolectomy (RP) with ileal-pouch-anal-anastomosis (IPAA) done as one to three stages, with safety and effectiveness of a single-stage operation unclear.

Methods: Pediatric UC patients from 2004 to 2019 who underwent RP/IPAA in the initial operation were retrospectively reviewed. 1-stage operations were matched 1:2 to 2-stage operations using age, duration of disease, and disease severity.

Current status of subspecialization in pediatric surgery: A focus on fetal surgery.

Background/purpose: Fetal surgery is a growing field within pediatric surgery. We sought to understand practice patterns of pediatric surgeons who subspecialize in fetal surgery.

Methods: A survey was sent to all active non-trainee surgeons within the American Pediatric Surgical Association.

A Rare Case of an Intramedullary Spinal Cord Abscess Due to Escherichia coli in a Pediatric Patient.

Spinal cord abscess is a rare entity, particularly in the pediatric population. Spinal cord abscesses can be located in extradural, subdural and intradural (intramedullary or extramedullary) regions of the cord. Among these locations, intramedullary is extremely uncommon.

Advanced Therapies for Severe Tracheobronchomalacia: A Review of the Use of 3D-Printed, Patient-Specific, Externally Implanted, Bioresorbable Airway Splints.

Tracheobronchomalacia is a condition of dynamic collapse of the trachea and mainstem bronchi. The clinical significance of tracheobronchomalacia depends on its severity. Mild cases may be medically managed with limited symptomology, while severe cases require advanced therapies, lengthy hospital stays, and carry significant morbidity and mortality.

Evolving Cost-Quality Relationship in Pediatric Heart Surgery.

Background: For the more than 40,000 children in the United States undergoing congenital heart surgery annually, the relationship between hospital quality and costs remains unclear. Prior studies report conflicting results and clinical outcomes have continued to improve over time. We examined a large contemporary cohort, aiming to better inform ongoing initiatives seeking to optimize health care value in this population.

Characteristics of patients ≥10 years of age with diffuse intrinsic pontine glioma: a report from the International DIPG/DMG Registry.

Background: Diffuse intrinsic pontine gliomas (DIPG) generally occur in young school-age children, although can occur in adolescents and young adults. The purpose of this study was to describe clinical, radiological, pathologic, and molecular characteristics in patients ≥10 years of age with DIPG enrolled in the International DIPG Registry (IDIPGR).

Methods: Patients ≥10 years of age at diagnosis enrolled in the IDIPGR with imaging confirmed DIPG diagnosis were included.

Indications for Lumbar Fusion in the Skeletally Mature Adolescent: How to Address Oblique Takeoff and Limb Length Discrepancy.

Background: Indications for posterior spinal fusion (PSF) with segmental spinal instrumentation (SSI) of a scoliotic deformity in a skeletally mature individual are based on the balance between the anticipated benefit of stopping future curve progression and the potential downside of loss of spinal mobility. The dilemma regarding PSF with SSI in the adolescent population is exacerbated by the patient's participation in athletics requiring flexibility and motion of the spine, the location of the curve, the presence of pelvic obliquity, and the impact of a limb length discrepancy. The purpose of this review is to discuss the potential advantages and disadvantages of PSF with SSI in a hypothetical skeletally mature adolescent with a 45-degree lumbar curve, pelvic obliquity, and limb length discrepancy.

Ruptured sinus of Valsalva aneurysm: An uncommon presentation of shock to the pediatric emergency department.

This case report describes a rare etiology of cardiogenic shock, particularly in the pediatric population. A healthy 17 year old male presents from an outside hospital in undifferentiated shock requiring vasopressor support. Ruptured sinus of Valsalva aneurysm was diagnosed by echocardiogram and the patient went emergently to the operating room for surgical repair.

Characterisation of neurodevelopmental and psychological outcomes in CHD: a research agenda and recommendations from the cardiac neurodevelopmental outcome collaborative.

The Neurodevelopmental and Psychological Outcomes Working Group of the Cardiac Neurodevelopmental Outcome Collaborative was formed in 2018 through support from an R13 grant from the National Heart, Lung, and Blood Institute with the goals of identifying knowledge gaps regarding the neurodevelopmental and psychological outcomes of individuals with CHD and investigations needed to advance science, policy, clinical care, and patient/family outcomes. Accurate characterisation of neurodevelopmental and psychological outcomes in children with CHD will drive improvements in patient and family outcomes through targeted intervention. Decades of research have produced a generalised perspective about neurodevelopmental and psychological outcomes in this heterogeneous population.

Neurodevelopmental and psychosocial interventions for individuals with CHD: a research agenda and recommendations from the Cardiac Neurodevelopmental Outcome Collaborative.

In 2018, the Neurodevelopmental and Psychosocial Interventions Working Group of the Cardiac Neurodevelopmental Outcome Collaborative convened through support from an R13 grant from the National Heart, Lung, and Blood Institute to survey the state of neurodevelopmental and psychosocial intervention research in CHD and to propose a slate of critical questions and investigations required to improve outcomes for this growing population of survivors and their families. Prior research, although limited, suggests that individualised developmental care interventions delivered early in life are beneficial for improving a range of outcomes including feeding, motor and cognitive development, and physiological regulation. Interventions to address self-regulatory, cognitive, and social-emotional challenges have shown promise in other medical populations, yet their applicability and effectiveness for use in individuals with CHD have not been examined.

Elastic Intramedullary Nailing of Pediatric Both-Bone Forearm Fractures.

Background: Elastic intramedullary nailing of both-bone pediatric forearm fractures is used to treat unstable fractures that fail conservative management with closed reduction and casting, open injuries, or injuries with neurovascular compromise.

Description: Small incisions are used to enter the medullary canal of the distal end of the radius and proximal part of the ulna, avoiding injury to the adjacent physis. The elastic nail is advanced to the fracture site under fluoroscopic guidance.

Characterization of Post-Operative Hemodynamics Following the Norwood Procedure Using Population Data and Multi-Scale Modeling.

Children with hypoplastic left heart syndrome (HLHS) must undergo multiple surgical stages to reconstruct the anatomy to a sustainable single ventricle system. Stage I palliation, or the Norwood procedure, provides circulation to both pulmonary and systemic vasculature. The aorta is reconstructed and attached to the right ventricle and a fraction of systemic flow is redirected to the pulmonary arteries (PAs) through a systemic-to-PA shunt.

30-day postoperative outcomes of neonatal versus delayed anoplasty for perineal and vestibular fistulas.

Purpose: The purpose of this study was to compare the postoperative outcomes of neonatal versus delayed repair of rectoperineal and rectovestibular fistulae using a multi-center pediatric colorectal specific database. We hypothesized that the incidence of 30-day postoperative complications is not significantly different between these two surgical treatment strategies.

Methods: We performed a retrospective, observational study of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database.

Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis.

Purpose: The impact of thoracoscopic surgery on outcomes in children with congenital lung malformations (CLM) remains controversial. The purpose of this study was to determine the effect of operative approach on perioperative outcomes in infants undergoing lobectomy for an asymptomatic CLM.

Methods: After IRB approval, a retrospective cohort study was conducted on 506 children with a CLM resected at one of eleven children's hospitals over a seven-year period.

Investigations of Staphylococcal contamination on environmental surfaces of a neonatal intensive care unit of a children's hospital.

Staphylococci species are known to cause healthcare-associated infections in neonatal intensive care (NICU) unit patients. Little is known about Staphylococcal contamination of NICU environments. Swabs from 25 of 46 (54%) surfaces sampled in a NICU had viable Staphylococcal contamination, with 11% contaminated by methicillin resistant Staphylococcus aureus [MRSA].

Implementing and Evaluating a Standardized New Diagnosis Education Checklist: A Report From the Children's Oncology Group.

When a child is newly diagnosed with cancer, parents report feeling overwhelmed with the amount of information that they must process in order to safely care for their child at home. The Children's Oncology Group (COG) Nursing Discipline has focused on examining current practices for educating families of children newly diagnosed with cancer, and developing tools to enhance the process of patient/family education at the time of diagnosis, including development of a COG Standardized Education Checklist, which classifies education into primary, secondary, and tertiary topics. The COG Nursing Discipline awarded nursing fellowships to two doctorally prepared nurses practicing at two distinct COG institutions to evaluate the checklist implementation.