A multidisciplinary approach to management of abdominal lymphatic malformations.

BACKGROUND/PURPOSE: Abdominal lymphatic malformations (LM) are a rare subset of vascular anomaly caused by abnormal development of the lymphatic system. They are classified as macrocystic, microcystic or combination macrocystic and microcystic. Surgical resection, percutaneous sclerotherapy, and medical therapy are all employed to treat these complex and often symptomatic lesions. No standardized treatment algorithm exists currently. The purpose of this study was to establish a multidisciplinary treatment approach to abdominal LMs. METHODS: A retrospective observational study was conducted from 2013 to 2019 on patients with abdominal LMs at a single tertiary children's hospital vascular anomalies center. Demographics, imaging, and treatment modality were recorded. Clinical and/or radiographic response to the primary treatment modality as well as complications was the outcomes measured. RESULTS: Nineteen patients (12 macrocystic, 5 microcystic and 2 combined) were identified, with a median age at diagnosis of 2.2 years (range 0.1-20.8 years). Sclerotherapy was the most common primary treatment, followed by surgical resection and sirolimus. No difference in clinical response (p = 0.58) or complications (p = 0.31) was observed based on primary treatment or subtypes. CONCLUSIONS: Based on our institutional experience, we propose an LM subtype-based treatment algorithm for abdominal LMs. It employs a multidisciplinary approach, and results in satisfactory patient outcomes with minimal complications. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Pneumonectomy for Pediatric Tumors-a Pediatric Surgical Oncology Research Collaborative Study.

OBJECTIVE: To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. SUMMARY BACKGROUND DATA: Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. METHODS: The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival. RESULTS: Thirty-eight patients (mean 12 ±â€Š6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045). CONCLUSION: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma.

Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study.

BACKGROUND: To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD). METHODS: Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected. RESULTS: Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival. CONCLUSION: This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.

Interactive Education is Associated With Lower Incidence of Pedestrian-Related Injury in Children.

BACKGROUND: Pedestrian-related injuries are a significant contributor to preventable mortality and disability in children. We hypothesized that interactive pedestrian safety education is associated with increased knowledge, safe crosswalk behaviors, and lower incidence of pedestrian-related injuries in elementary school-aged children. METHODS: An interactive street-crossing simulation was implemented at target elementary schools in Los Angeles County beginning in 2009. Mixed-methods were used to evaluate the impact of this intervention. Multiple-choice examinations were used to test pedestrian safety knowledge, anonymous observations were used to assess street-crossing behaviors, and statewide traffic records were used to report pedestrian injuries in elementary school-aged (4-11 y) children in participating school districts. Pedestrian injury incidence was compared 1 y before and after the intervention, standardized to the incidence in the entire City of Los Angeles. RESULTS: A total of 1424 and 1522 children completed the pretest and post-test, respectively. Correct answers increased for nine of ten questions (all P < 0.01). Children more frequently looked both ways before crossing the street after the intervention (10% versus 41%, P < 0.001). There were 6 reported pedestrian-related injuries in intervention school districts in the year before the intervention and 2 injuries in the year after the intervention, resulting in a significantly lower injury incidence (standardized rate ratio 0.28; 95% CI, 0.11-0.73). CONCLUSION: Pedestrian safety education at Los Angeles elementary schools was associated with increased knowledge, safe street-crossing behavior, and lower incidence of pediatric pedestrian-related injury. Formal pedestrian safety education should be considered with injury prevention efforts in similar urban communities.

Laparotomy complications on extracorporeal life support: Surgical site bleeding does not increase mortality.

PURPOSE: The risks of laparotomy during extracorporeal life support (ECLS) are poorly defined. We examined risk factors associated with bleeding and mortality after laparotomy on ECLS. METHODS: The Extracorporeal Life Support Organization (ELSO) database was queried for all pediatric patients [0-17 years] with a procedure code for laparotomy. Outcome data were analyzed to define factors contributing to laparotomy complications and mortality while on ECLS. Univariate and multivariate analyses were applied to determine independent risk factors. RESULTS: 196 patients who met inclusion criteria were identified. The mortality rate in the entire cohort was 67.3%. In both univariate and multivariate analyses, surgical site bleeding did not significantly increase the risk of mortality (OR 0.8; 95% CI 0.4-1.7). Logistic regression analysis revealed that lower gestational age, infectious complications and nonsurgical site hemorrhagic complications were independently increased mortality risk (all p < 0.05). CONCLUSION: Mortality following laparotomy on ECLS is not independently associated with surgical site bleeding, but is associated with lower gestational age, infectious and nonsurgical site hemorrhagic complications. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Non-Operative Management is More Cost-Effective than Immediate Operation in Perforated Appendicitis Patients with Seven or More Days of Symptoms.

BACKGROUND: Management of perforated appendicitis in children remains controversial. Nonoperative (NO) and immediate operative (IO) strategies are used with varying outcomes. We hypothesized that IO intervention for patients with perforated appendicitis would be more cost-effective than NO management. METHODS: A retrospective chart review of all patients with appendicitis from 2012 to 2015 was performed. Patients with perforated appendicitis were defined by evidence of perforation on imaging. We excluded patients who presented with sepsis, organ failure, and ventriculoperitoneal shunts. NO management was determined by surgeon preference. Univariate and multivariate analyses were performed. RESULTS: IO was performed on 145 patients with perforated appendicitis, whereas 83 were treated with NO management. Compared to IO patients, NO patients incurred higher overall costs, greater length of stay, more readmissions, complications, peripherally inserted central venous catheter lines, interventional radiology drains, and unplanned clinic and emergency department visits (P < 0.0001 for all). Multivariate analysis adjusting for age, days of symptoms, admission C-reactive protein and white blood cell count revealed that NO management was independently associated with increased costs (OR 1.35, 1.12-1.62, 95% CI). Cost curves demonstrated that total cost for IO surpasses that of NO management when patients present with greater than 6.3 d of symptoms (P = 0.01). CONCLUSIONS: Our data suggest that IO is more cost-effective than NO management for patients with perforated appendicitis who present with less than 6.3 d of symptoms, after which point, NO management is more cost-effective. LEVEL OF EVIDENCE: IV.

Activated Natural Killer Cells in Combination with Anti-GD2 Antibody Dinutuximab Improve Survival of Mice after Surgical Resection of Primary Neuroblastoma.

PURPOSE: Immunotherapy of neuroblastoma that remains after myeloablative chemotherapy with anti-GD2 antibody dinutuximab has increased the two-year event-free and overall survival of high-risk neuroblastoma patients; however, 40% of patients develop recurrent disease during or after this treatment. To determine the potential of such antibody-based immunotherapy earlier in treatment, a mouse model was developed in which surgical resection of the primary tumor was followed by therapy of residual disease with dinutuximab combined with ex vivo-activated human natural killer (aNK) cells. EXPERIMENTAL DESIGN: The effect of combining dinutuximab with human aNK cells was determined in vitro with cellular cytotoxicity and Matrigel invasion assays. The in vivo efficacy of dinutuximab and aNK cells against neuroblastoma was assessed following resection of primary tumors formed by two cell lines or a patient-derived xenograft (PDX) in immunodeficient NOD-scid gamma mice. RESULTS: In vitro, the combination of aNK cells and dinutuximab caused cytotoxicity and decreased invasiveness of three human neuroblastoma cell lines. Treatment of mice with dinutuximab combined with aNK cells after surgical resection of primary intrarenal tumors formed by two cell lines or a PDX decreased tumor cells in liver and bone marrow as evaluated by histopathology and bioluminescence imaging. Survival of mice after resection of these tumors was most significantly increased by treatment with dinutuximab combined with aNK cells compared with that of untreated mice. CONCLUSIONS: The combination of dinutuximab and adoptively transferred human aNK cells following surgical resection of primary neuroblastomas significantly improves survival of immunodeficient mice.

Pediatric firearm injuries in Los Angeles County: Younger children are more likely to be the victims of unintentional firearm injury.

BACKGROUND: Firearm injuries are now the third leading cause of death in children. Understanding the circumstances surrounding pediatric firearm injuries will allow for targeted injury prevention efforts. We hypothesized that younger children are more likely to be victims of unintentional firearm injury. METHODS: A multicenter, retrospective review of patients <18 years old who sustained firearm injuries in Los Angeles County from 2006 to 2015 was performed. Unintentional injuries were defined as accidental firearm discharge without violent intent. Intentional injuries were defined as firearm discharge with intent to injure (including suicide). RESULTS: After review of 304 pediatric firearm injuries, 206 had sufficient narrative to determine intent with 10% of injuries classified as unintentional. Unintentional injuries were more common in younger children, more frequently caused by a firearm from within the home, and more likely to involve friend/family (all p < 0.05). Intentional injuries were associated with more injuries and accounted for all deaths in our study cohort. CONCLUSIONS: In pediatric firearm injury, younger children are more susceptible to unintentional injuries, but intentional injuries are more common overall. Future interventions need to target both intentional violence in older children and unintentional firearm injury in young children if the frequency is to be reduced. TYPE OF STUDY: Epidemiologic study. LEVEL OF EVIDENCE: Level III.

Congenital Perirectal Dermoid Cyst: A Rare Cause of Complex, Recurrent Pediatric Fistula-in-ano.

Perianal abscess and fistula-in-ano are well-described in the pediatric population. They are most common in infants less than 1 year of age and often resolve with oral antibiotics; occasionally they require drainage or fistulotomy. The etiology is commonly associated with cryptoglandular obstruction and subsequent infection, however alternative diagnoses should be considered in cases of recurrent abscesses and fistulae that are refractory to standard treatments. In this report, we present the case of an 8-year-old boy with a complex, recurrent fistula-in-ano that resulted from a rare congenital perirectal dermoid cyst.

Preoperative Educational Intervention Decreases Unplanned Gastrostomy-Related Health Care Utilization.

Apprehension in taking independent care of children with medical devices may lead to unnecessary visits to the ED and/or acute clinic (AC). To address these concerns, our institution implemented a gastrostomy tube (GT) class in 2011 for caretakers. We hypothesized that inappropriate GT-related ED/AC visits would be lower in preoperatively educated caregivers. We performed a retrospective cohort study of all patients aged 0 to 18 who received GT (surgical or percutaneous) at our institution between 2006 and 2015 (n = 1340). Class attendance (trained vs untrained) and unscheduled GT-related ED/AC visits one year after GT placement were reviewed. Gastrostomy-related ED/AC visits were classified as appropriate (hospital-based intervention) or inappropriate (site care and education/reassurance). Occurrence of ED/AC visits was compared between trained and untrained cohorts. We found that 59 per cent of patients had an unscheduled GT-related ED/AC visit within one year of placement. The trained cohort had 27 per cent less unplanned ED/AC visits within one year (mean 1.21 (SD 1.82) vs untrained 1.65 (2.24), P < 0.001). On multivariate analysis, GT education independently decreased one-year GT-related health care utilization (Odds Ratio 0.75, 95% Confidence Interval 0.59-0.95). Formal education seems to decrease GT-related health care utilization within one year of placement and should be integrated into a comprehensive care plan to improve caregiver self-efficacy.

Pediatric Morgagni diaphragmatic hernia: a descriptive study.

INTRODUCTION: The Morgagni hernia (MH) accounts for 3-4% of congenital diaphragmatic hernias. There is a paucity of data regarding this rare defect. The purpose of this study is to describe the characteristics of children with MH, surgical approaches for repair, and patient outcomes. METHODS: Pediatric patients (ages 0-18) with a MH from 2002 to 2014 at a single, freestanding pediatric hospital were retrospectively reviewed. Patient presentation, demographics, operative methods and findings, and outcomes were evaluated. RESULTS: Twenty-six infants and children with a congenital MH were treated. There were 20 males (77%) and six females (23%) with a median age at diagnosis of 14.75 months (range 1 week to 13 years). Half were symptomatic. Sixteen hernias were repaired laparoscopically, nine by an open approach, and one laparoscopic converted to open. Colon was the most commonly herniated organ (N = 14). Hernia sacs were found in 22 patients of which, 20 were resected. Two patients underwent treatment with ECLS. There was one mortality in a patient who underwent repair on ECLS in the setting of an omphalocele and SVC obstruction. There were no recurrences in our sample. CONCLUSION: In this series, congenital MH appears to have a male predominance, frequently presents with pulmonary symptoms, and has excellent outcomes regardless of operative approach.