Open versus thoracoscopic thymectomy for juvenile myasthenia gravis.

BACKGROUND: Juvenile myasthenia gravis (JMG) is an antibody mediated autoimmune disorder that manifests as progressive voluntary muscle weakness and fatigue. In medically refractory cases, thymectomy has been shown to abrogate symptoms and reduce glucocorticoid dependence. While transcervical or transsternal incisions have been the traditional approach, adult trends now favor thoracoscopic thymectomy. Little data exist to support this approach in children. METHODS: A retrospective review of all patients younger than 20 years of age who underwent a thymectomy for JMG at two pediatric institutions between 2001 and 2018 was performed. Children were divided into either an open (transcervical or transsternal) or thoracoscopic group and baseline characteristics, perioperative, and postoperative outcomes were compared. RESULTS: Thirty-four thymectomies were performed during the 18-year study period; 18 via an open and 16 via a thoracoscopic approach. The operative time was shorter for open procedures compared thoracoscopic ones (108 ±â€¯49 and 145 ±â€¯43 min, respectively, p = 0.025). Thoracoscopic thymectomy was associated with less intraoperative blood loss (5.5 ±â€¯6.0 vs 55 ±â€¯67 ml, p = 0.007), decreased duration of postoperative intravenous narcotic use (5.0 ±â€¯1.5 vs 20 ±â€¯23 h, p = 0.018), and a shorter length of hospitalization (1.7 ±â€¯1.0 vs 2.7 ±â€¯1.1 days, p = 0.009). No perioperative complication occurred in either group. Clinical improvement was reported in 94% of children in both groups. CONCLUSIONS: Thoracoscopic thymectomy in children is a safe and effective surgical technique for the treatment of JMG. Increased acceptance of this minimally invasive approach by children, families, and referring neurologists may enable earlier surgical intervention. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: III.

Peritoneal Nodules in a Pediatric Patient with Benign Teratoma. A Case Report and Review of Literature.

BACKGROUND: Mature ovarian teratomas are common in children. These well differentiated tumors are typically confined to the ovary. In rare cases, they can rupture leading to granulomatous peritonitis that mimics carcinomatosis. Ovarian tumors with peritoneal/omental implants suggest malignant pathology with a different prognosis. CASE: A 15-year-old girl presented with 5 months of abdominal pain, and weight loss. Computed tomography (CT) imaging of the abdomen revealed a large mass filling the abdomen. Slightly elevated lactate dehydrogenase (LDH) and carcinoma antigen 125 (CA125). On laparotomy an ovarian tumor with peritoneal and omental implants was identified. Left salpingo-oophorectomy, omentectomy, and peritoneal washing were performed. Pathology revealed a benign cystic teratoma. SUMMARY AND CONCLUSION: Although ovarian teratomas are typically benign, they might mimic carcinomatosis. In patients with unexpected finding of peritoneal implants, histologic diagnosis is recommended before proceeding with a full oncologic ovarian resection.

Utility of hospital admission for pediatric intussusceptions.

PURPOSE: The standard practice in pediatric patients diagnosed with intussusception has been reduction via enema and admission for a period of nil per os and observation. Little data exists to support this practice. The objective of this study was to examine whether post-reduction admission to hospital is required. METHODS: A retrospective chart review was performed on all patients aged 0-18 years old with intussusception over a span of 20 years. Study included children treated for intussusception on first encounter with enema and subsequently admitted for observation. Study excluded those readmitted for recurrence after 48 h, patients whose intussusception did not reduce on first try, those lost to follow-up, and those who went to the operating room. Early recurrence was defined as recurrence within 48 h post-reduction. RESULTS: Out of 171 patients admitted, only one experienced an early recurrence (0.6 %). Median length of stay for all patients was 2 days. Average cost incurred per day for intussusception admission was $404. CONCLUSION: Intussusception in a child that is successfully reduced via enema has a low recurrence rate and is usually followed by prompt resolution of symptoms. An abbreviated period of observation in the emergency department post-reduction may result in healthcare savings.

Firearm injuries in the pediatric population: A tale of one city.

BACKGROUND: Firearm-related injuries are a significant cause of morbidity and mortality in children. To determine current trends and assess avenues for future interventions, we examined the epidemiology and outcome of pediatric firearm injuries managed at our region's two major pediatric trauma centers. METHODS: Following institutional review board approval, we conducted a 5-year retrospective review of all pediatric firearm victims, 16 years or younger, treated at either of the region's two Level 1 pediatric trauma centers, St. Louis Children's Hospital and Cardinal Glennon Children's Medical Center. RESULTS: There were 398 children treated during a 5-year period (2008-2013) for firearm-related injuries. Of these children, 314 (78.9%) were black. Overall, there were 20 mortalities (5%). Although most (67.6%) patients were between 14 years and 16 years of age, younger victims had a greater morbidity and mortality. The majority of injuries were categorized as assault/intentional (65%) and occurred between 6:00 pm and midnight, outside the curfew hours enforced by the city. Despite a regional decrease in the overall incidence of firearm injuries during the study period, the rate of accidental victims per year remained stable. Most accidental shootings occurred in the home (74.2%) and were self-inflicted (37.9%) or caused by a person known to the victim (40.4%). CONCLUSION: Despite a relative decrease in intentional firearm-related injuries, a constant rate of accidental shootings suggest an area for further intervention. LEVEL OF EVIDENCE: Prognostic and epidemiologic study, level IV.

Early diffuse slowing on electroencephalogram in pediatric traumatic brain injury: Impact on management and prognosis.

INTRODUCTION: We hypothesized that the finding of diffuse slowing on bedside EEG in children with moderate to severe traumatic brain injury (TBI) is associated with prolonged hospital stay and worse functional outcomes. METHODS: We reviewed the medical records of all patients admitted to a single level I pediatric trauma center with moderate or severe TBI from 1/10-12/12 (defined by GCS<10 on admission). EEG monitoring results, patient demographics, clinical characteristics, length of stay and postinjury outcomes were recorded. We compared outcomes between patients with and without diffuse slowing on EEG. Data are presented as mean±SEM; p<0.05 was considered statistically significant. RESULTS: 219 children with TBI were identified; 81 had a bedside EEG performed within 48 hours of admission. Diffuse slowing was present in 50 (mean age 5.7+0.7 years) and absent in 31 (n=31, mean age 4.2+0.9 years). Patients with diffuse slowing had a significant increase in ventilator days, ICU LOS, need for rehabilitation, and rehabilitation length of stay. CONCLUSION: The presence of diffuse slowing on EEG in children with TBI is associated with prolonged patient recovery and poor functional outcomes. This finding should prompt early consideration for rehabilitation and the need for intensive therapy.

Contrast blush in pediatric blunt splenic trauma does not warrant the routine use of angiography and embolization.

BACKGROUND: Splenic artery embolization (SAE) in the presence of contrast blush (CB) has been recommended to reduce the failure rate of nonoperative management. We hypothesized that the presence of CB on computed tomography has minimal impact on patient outcomes. METHODS: A retrospective review was conducted of all children (<18 years) with blunt splenic trauma over a 10-year period at a level 1 pediatric trauma center. Data are presented as mean ± standard error of mean. RESULTS: Seven hundred forty children sustained blunt abdominal trauma, of which 549 had an identified solid organ injury. Blunt splenic injury was diagnosed in 270 of the 740 patients. All patients were managed nonoperatively without SAE. CB was seen on computed tomography in 47 patients (17.4%). There were no significant differences in the need for blood transfusion (12.5% vs 11.1%) or length of stay (3.1 vs 3.3 days) or need for splenectomy when compared in children with or without CB. CONCLUSION: Pediatric trauma patients with blunt splenic injuries can be safely managed without SAE and physiologic response and hemodynamic stability should be the primary determinants of appropriate management.

Evaluation of laparoscopic management of recurrent gastroesophageal reflux disease and hiatal hernia: long term results and evaluation of changing trends.

INTRODUCTION: Recurrent gastro-esophageal reflux disease (GERD) following fundoplication remains a common problem. This study evaluates a long-term experience with laparoscopic management of these cases. METHODS: From January 1994 to December 2012, 252 patients with recurrent GERD underwent a laparoscopic redo Nissen (LRN) fundoplication with average age of 6.8years. Eighty-four had previous open fundoplications and 144 previous LNRs. Thirty-two had more than one previous fundoplication. RESULTS: All procedures were completed laparoscopically. The average operative time was 82min. The intra-operative complication rate was 5.1%, the most common being a gastrostomy during the mobilization. The average time to full feeds was 1.4days, and the average hospital stay was 1.6days. The post-operative complication rate was 3.6%. The wrap failure rate was 6.2%. The most common cause of wrap failure was H/H, with increasing incidence of slipped wrap during the second half. The highest recurrence rate was in patients receiving their LNR before 4months of age. CONCLUSIONS: Redo Laparoscopic Nissen fundoplication is safe and effective, with the same benefits as a primary laparoscopic Nissen, with low morbidity and quick recovery. A change in the etiology of recurrence suggests that there is a failure to adequately identify and mobilize the GE junction in laparoscopic cases.

Stearoyl-CoA desaturase plays an important role in proliferation and chemoresistance in human hepatocellular carcinoma.

BACKGROUND: Hepatocellular carcinoma (HCC) is often diagnosed at an advanced stage, when it is not amenable for aggressive therapies such as surgical resection or liver transplantation. Current therapeutic options achieve clinical responses in only a small percentage of cases. As a consequence, effective approaches for prevention and treatment are greatly needed. Altered lipid metabolism has been recently linked to HCC pathogenesis. The aims of this study were to define the cellular and molecular mechanisms linking stearoyl-CoA desaturase (SCD), the rate-limiting enzyme and an essential regulator of lipid homeostasis in liver cells, to carcinogenesis in HCC. MATERIAL AND METHODS: HCC and normal liver specimens were collected. Human HCC cell lines: HepG2, Hep3B, and PLC/PLF/5 were used for immunoblot, cell viability, proliferation, and apoptosis assays. Small interfering RNAs were used for genetic inhibition, and 10, 12 conjugated linoleic acid was used for pharmacologic SCD inhibition. RESULTS: SCD was strongly expressed in surgically resected HCC (n = 64) and various human HCC cell lines (HepG2, Hep3B, and PLC/PLF/5). The levels of SCD negatively correlated with degree of tumor differentiation (P < 0.01). Treatment of these HCC cell lines with a panel of chemotherapeutic drugs resulted in a time-dependent, phosphatidylinositol 3 kinase- and c-Jun N-terminal kinases1/2-mediated upregulation of SCD expression, which paralleled the degree of resistance to drug-induced apoptosis. Specific genetic or pharmacologic SCD suppression resulted in inhibition of cell proliferation (P < 0.001) and significantly increased sensitivity to chemotherapy-induced apoptosis. CONCLUSIONS: Our data suggest that increased SCD expression plays an important role in HCC development and resistance to chemotherapy-induced apoptosis, and this is in part mediated by phosphatidylinositol 3 kinase/c-Jun N-terminal kinases activation. Specific targeted interruption of this pathway in HCC could be a desirable approach in designing novel therapeutic strategies.

Appendicitis in children less than 5 years old: influence of age on presentation and outcome.

PURPOSE: Appendicitis is the most common emergency surgical condition of the abdomen in children. This study sought to delineate the presentation and the outcome of appendicitis in children younger than 5 years old. METHODS: A retrospective review was conducted of all children younger than 5 years of age who underwent appendectomy for acute appendicitis over a 12-year period. RESULTS: One thousand eight hundred thirty-six patients younger than 19 years of age underwent appendectomy. Two hundred eighty-one children with an age range of 6 months to 4.9 years were included in this study. Perforation rates were higher in the younger patients (86% <1 year, 74% 1-1.9 years, 60% 2-2.9 years, 64% 3-3.9 years, and 49% 4-4.9 years), but the youngest children had fewer postoperative abscesses. CONCLUSIONS: In children less than 5 years old with appendicitis, age has a direct correlation to the stage of disease. The youngest children present with more advanced appendicitis but are less likely to develop postoperative abscesses.