Multifocal Insulinoma as the Unique Presenting Feature of Multiple Endocrine Neoplasia Type 1 in an Adolescent.

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder defined by the presence of two of the following endocrinopathies: primary hyperparathyroidism, anterior pituitary tumors, and duodenopancreatic neuroendocrine tumors (NETs). NETs, which can secrete hormones including insulin, gastrin, and glucagon, among others, are common in patients with MEN1 and are a major cause of morbidity and premature death. NETs are more common later in life, with very few cases described in children.

Interventional endoscopy for abdominal transplant patients.

Interventional endoscopy can play a significant role in the care and management of children pre-and post- abdominal solid organ transplantation. Such procedures primarily include endoscopic retrograde cholangiopancreatography (ERCP), endoscopic ultrasound (EUS), and balloon-assisted enteroscopy (BAE), though additional interventions are available using standard endoscopes (gastroscopes, colonoscopes) for therapeutics purposes such as endoscopic hemostasis. The availability of pediatric practitioners with the advanced training to effectively and safely perform these procedures are most often limited to large tertiary care pediatric centers.

Pneumatosis intestinalis after hematopoietic stem cell transplantation: When not doing anything is good enough.

Background/purpose: Pneumatosis intestinalis (PI) has been reported in hematopoietic stem cell transplant recipients (HSCT) since 1980s and at present there is no uniform consensus of the significance and management of this condition.

Methods: We retrospectively reviewed medical records of 990 consecutive pediatric HSCT recipients and examined data for clinical PI presentation, management and outcomes RESULTS: PI was identified in 53 patients (5.4%), mainly allogeneic HSCT recipients receiving systemic steroids.

Standardization of clinical care pathway leads to sustained decreased length of stay following Nuss pectus repair: A multidisciplinary quality improvement initiative.

Background And Purpose: Postoperatively, standardized clinical care pathways (SCCPs) help patients reach necessary milestones for discharge. The objective of this study was to achieve 90% compliance with a pectus specific SCCP within 9 months of implementation. We hypothesized that adherence to a pectus SCCP following the Nuss procedure would decrease postsurgical length of stay (LOS).

Minimally invasive surgery for abdominal and thoracic neuroblastic tumors: A systematic review by the APSA Cancer committee.

Background: Minimally invasive surgery has broad applicability to pediatric diseases, including pediatric cancer resection. Neuroblastic tumors of childhood are highly variable in presentation, and so careful selection of appropriate candidates for minimally invasive resection is paramount to achieving safe and durable surgical and oncological outcomes.

Methods: The American Pediatric Surgical Association Cancer Committee developed questions seeking to better define the role of minimally invasive surgery for neuroblastic tumors.

Topical sirolimus for the treatment of cutaneous manifestations of vascular anomalies: A case series.

Background: Vascular anomalies (VA), characterized by the abnormal development or growth of blood and/or lymphatic vessels, encompasses a spectrum of conditions with a range of symptoms and complications. VA are frequently associated with cutaneous complications that can cause significant morbidity. Systemic sirolimus has previously been shown to be effective in the treatment of complicated VA.

Thrombopoietin Contributes to Extreme Thrombocytosis After Pediatric Pancreatectomy With Islet Autotransplantation.

Objective: This study aims to explore the role of thrombopoietin (TPO) production in extreme thrombocytosis that is often observed after pancreatectomy with islet autotransplantation (IAT) and the effectiveness of hydroxyurea in thrombocytosis management.

Methods: Retrospective chart review was performed for all patients who underwent pancreatectomy with IAT at our institution between April 1, 2015, and December 31, 2016. Data evaluated included demographics, platelet counts, TPO levels, and thrombocytosis management strategies.

Congenital high airway obstruction syndrome (CHAOS): Natural history, prenatal management strategies, and outcomes at a single comprehensive fetal center.

Purpose: Congenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions.

Vomiting and Dehydration in a 2-Year-Old.

A 2-year-old girl with a past medical history of cutaneous mastocytosis and eczema presented with 1 day of yellow-green, nonbloody vomiting, bradycardia, and listlessness. She was evaluated by her pediatrician and sent to the emergency department because of concern for dehydration. In the emergency department, she improved with fluid rehydration but still had decreased energy and bradycardia.

Don't Get Stuck: A Quality Improvement Project to Reduce Perioperative Blood-Borne Pathogen Exposure.

Background: Blood-borne pathogen exposure (BBPE) represents a significant safety and resource burden, with more than 380,000 events reported annually across hospitals in the United States. The perioperative environment is a high-risk area for BBPE, and efforts to reduce exposures are not well defined. A multidisciplinary group of nurses, surgical technologists, surgeons, and employee health specialists created a BBPE prevention bundle to reduce Occupational Safety and Health Administration (OSHA) recordable cases.

Rhabdomyosarcoma and Extraosseous Ewing Sarcoma.

Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It arises from mesenchymal origin and forms part of the group of small round blue cell tumors of childhood. It has a constant annual incidence of 4.

Pediatric trauma undertriage in Ohio.

Background: Appropriate and timely triage is an essential component of a trauma system. In the state of Ohio, there are 6 verified pediatric trauma centers (PTCs) across 8 state regions. The purpose of this study was to better understand the pediatric undertriage rates in the state.

Spindle cell sarcomatoid carcinoma of the trachea: first case report of surgical resection.

Background: Primary malignant tracheal tumors are rare, accounting for approximately 0.2 % of respiratory tract tumors yearly, with squamous cell carcinomas and adenoid cystic carcinomas accounting for two-thirds of these cases. Sarcomatoid carcinomas are a group of poorly differentiated non-small cell lung carcinomas containing a component of sarcoma or sarcoma-like (spindle and/or giant cell) differentiation, categorized into five morphologic subgroups.

Surgical treatment of nonalcoholic fatty liver disease in severely obese patients.

Obesity is a multi-organ system disease with underlying metabolic abnormalities and chronic systemic inflammation. Nonalcoholic fatty liver disease (NAFLD) is a hepatic manifestation of obesity metabolic dysfunction and its associated cardiovascular- and liver-related morbidities and mortality. Our current understanding of NAFLD pathogenesis, disease characteristics, the role of insulin resistance, chronic inflammation, gut-liver and gut-brain crosstalk and the effectiveness of pharmacotherapy is still evolving.

Nonalcoholic fatty liver disease and bariatric surgery in adolescents.

Obesity is a multi-organ system disease with underlying insulin resistance and systemic chronic inflammation. Nonalcoholic fatty liver disease (NAFLD) is a hepatic manifestation of the underlying metabolic dysfunction. This review provides a highlight of the current understanding of NAFLD pathogenesis and disease characteristics, with updates on the challenges of NAFLD management in obese and severely obese (SO) patients and recommendations for the pediatric surgeons' role in the care of SO adolescents.