Juvenile xanthogranuloma: diverse presentations of noncutaneous disease.

Juvenile xanthogranulomas (JXGs) are benign cutaneous lesions of childhood that often spontaneously involute. They rarely present as a noncutaneous tumors. However, JXG tumors have been described in numerous noncutaneous anatomic sites, presenting with a variety of symptoms. The severity of symptoms and accurate preoperative diagnosis of JXG should determine operative and nonoperative treatment options of these uncommon, benign, and self-limiting tumors. We report 3 cases of symptomatic, noncutaneous JXG from disparate anatomic sites all treated with aggressive surgical resection.

Effect of Quarantine and Reopening Measures on Pediatric Trauma Admissions During the 2019 SARS-CoV2 Virus Pandemic.

BACKGROUND: Several studies have reported decreased trauma admissions and increased physical abuse in children resulting from stay-at-home measures. However, these studies have focused on a limited period after the implementation of lockdown policies. The purpose of this study was to examine the effect of quarantine and reopening initiatives on admissions for varying types of injuries in pediatric patients. STUDY DESIGN: Registry data for an urban Level I pediatric trauma center were evaluated from April 1, 2018, to March 30, 2021. A timeline of local shutdown and reopening measures was established and used to partition the data into 6-month intervals. Data about demographics and injury characteristics were compared with similar intervals in 2018 and 2019 using appropriate statistical methodology for categorical, parametric, and nonparametric data. RESULTS: A total of 3,110 patients met criteria for inclusion. A total of 1,106 patients were admitted the year after the closure of schools and nonessential businesses. Decreases in overall admissions and evaluations for suspected child abuse noted early in the pandemic were not sustained during shutdown or reopening periods. However, we observed a 77% increase in all-terrain vehicle injuries, along with a 59% reduction in sports injuries (chi-square [8, N = 3,110] = 49.7; p < 0.001). Significant shifts in demographic and payor status were also noted. CONCLUSIONS: This is the first study to comprehensively examine the effects of quarantine and reopening policies on admission patterns for a pediatric trauma center in a metropolitan area. Total admissions and child abuse evaluations were not impacted. If shutdown measures are re-instituted, preventative efforts should be directed towards ATV use and recreational activities.

Inguinal prolapse of a retroperitoneal lymphovascular malformation.

Abdominal lymphovascular malformations (ALMs) are rare cystic masses that can present with nonspecific symptoms. We present a case of a 7-month-old boy who, during an uncomplicated communicating hydrocele repair, was found to have an incidental large, prolapsed mesenteric abdominal lymphovascular malformation. The case serves to highlight the variability in presentation and natural history of ALMs, and the ease with which they can be disguised by more common pathology. We further underscore the importance of individualized therapy with regards to ALMs, emphasized by our course of active surveillance allowing our patient to avoid ionizing radiation and additional surgical intervention.

Echocardiography, not chest radiography, for evaluation of cannula placement during pediatric extracorporeal membrane oxygenation.

OBJECTIVE: Optimal cannula position is essential during extracorporeal membrane oxygenation (ECMO). We hypothesize that echocardiography is superior to chest radiography in diagnosing abnormal cannula position during ECMO. DESIGN: Retrospective. SETTING: Pediatric hospital. PATIENTS: 100 pediatric patients requiring ECMO. MEASUREMENTS AND MAIN RESULTS: We reviewed the medical records of all ECMO patients (n = 100), including reports of all echocardiograms (n = 326), during the years 2002-2004. Of the 91 patients who had echocardiograms while on ECMO, 33 had at least 1 echocardiogram for cannula-position evaluation. Of the remaining 58 patients with echocardiograms for reasons other than cannula-position evaluation, 4 (7%) were found to have abnormal cannula position. These included arterial cannula (AC) within 2-4 mm of the aortic valve (n = 2), AC across the aortic valve into the left ventricle (n = 1), and venous cannula (VC) abutting the atrial septum (n = 1). Of the 33 patients with echocardiograms for evaluation of cannula position, 8 (24%) required intervention. Of those 8 patients, 4 required cannula repositioning due to VC in the coronary sinus (n = 1), VC abutting atrial septum (n = 1), AC in left subclavian artery (n = 1), and AC within 3 mm of aortic valve (n = 1). The remaining 4 with normal cannula position required upsizing of the VC (n = 2), increased circuit flow (n = 1), or intravascular volume administration (n = 1). Overall, 12 of 91 patients (13%) required intervention based on echocardiographic findings. Chest radiography did not detect abnormalities of ECMO cannula position in any of the 8 patients with this problem, nor were any additional patients with abnormal cannula position identified by chest radiography. CONCLUSIONS: Echocardiography appears to be superior to chest radiography for assessing ECMO cannula position in our institution. A prospective study, including cost analysis, comparing chest radiography and echocardiography, is needed to definitely determine the preferred diagnostic test or sequence of tests to establish ECMO cannula position.

Puestow Procedure for the Management of Pediatric Chronic Pancreatitis.

OBJECTIVE: Recurrent pancreatitis significantly impacts childhood development and quality of life. Our goal was to evaluate the efficacy of the Puestow procedure. MATERIALS AND METHODS: After obtaining the Institutional Review Board approval, we reviewed the charts of all patients who underwent lateral pancreaticojejunostomy from January 1999 to January 2014. Statistical analysis was performed using paired Student's t-test and Fisher's exact test as appropriate. RESULTS: During the 15-year study period, 13 patients underwent a lateral pancreaticojejunostomy for chronic pancreatitis. The most common causes of pancreatitis were hereditary (n = 5) or obstructive (n = 5); pancreas divisum (n = 2), one iatrogenic stricture, one idiopathic stricture, and one unresectable pancreatic head mass); two patients had idiopathic disease, and one case was drug-induced. Six patients had failed management with endoscopic retrograde cholangiopancreatography and pancreatic duct stenting. Preoperatively, the median body mass index (BMI) percentile-for-age was 61.0% (range 11.0-99.0%). Median age at operation was 12.8 years (range 7.7-16.7). There were no deaths, four patients developed postoperative ileus, and one patient developed an intra-abdominal abscess, which resolved with antibiotics. Median postoperative length of stay was 7 days (range 5-15).Two patients were lost to follow-up; median follow-up for the remaining 12 patients was 35.5 months (range 4.9-131.2). Four patients were readmitted within 90 days: three due to abdominal pain which were not recurrences of pancreatitis, and one due to complications of chemotherapy. Postoperatively, there was no change in the average BMI percentile-for-age (p = 0.64). Seven patients reported resolution or significant improvement in their abdominal pain symptoms at the time of last follow-up. Patients with obstructive causes of pancreatitis were not more likely to experience relief than those with nonobstructive causes (42.9 vs. 80.0%, p = 0.29). CONCLUSION: In our experience, lateral pancreaticojejunostomy results in durable improvement or resolution of abdominal pain symptoms in nearly 60% of patients with chronic pancreatitis regardless of etiology.

Aortoesophageal Fistula in a Child With Undiagnosed Vascular Ring: Life-Threatening or Lethal?

Exsanguinating hematemesis secondary to an aortoesophageal fistula from an impacted foreign body occurred in a patient with a vascular ring. This report describes successful resuscitation and repair in a 6-year-old boy who was transferred from an outside hospital in extremis with an aortoesophageal fistula from a foreign body in the presence of a vascular ring.

Nonoperative management of blunt liver and spleen injury in children: Evaluation of the ATOMAC guideline using GRADE.

BACKGROUND: Nonoperative management of liver and spleen injury should be achievable for more than 95% of children. Large national studies continue to show that some regions fail to meet these benchmarks. Simultaneously, current guidelines recommend hospitalization for injury grade + 2 (in days). A new treatment algorithm, the ATOMAC guideline, is in clinical use at many centers but has not been prospectively validated. METHODS: A literature review conducted through MEDLINE identified publications after the American Pediatric Surgery Association guidelines using the search terms blunt liver trauma pediatric, blunt spleen trauma pediatric, and blunt abdominal trauma pediatric. Decision points in the new algorithm generated clinical questions, and GRADE [Grading of Recommendations, Assessment, Development, and Evaluations] methodology was used to assess the evidence supporting the guideline. RESULTS: The algorithm generated 27 clinical questions. The algorithm was supported by six 1A recommendations, two 1B recommendations, one 2B recommendation, eight 2C recommendations, and ten 2D recommendations. The 1A recommendations included management based on hemodynamic status rather than grade of injury, support for an abbreviated period of bed rest, transfusion thresholds of 7.0 g/dL, exclusion of peritonitis from a guideline, accounting for local resources and concurrent injuries in the management of children failing to stabilize, as well as the use of a guideline in patients with multiple injuries. The use of more than 40 mL/kg or 4 U of blood to define end points for the guideline, and discharging stable patients before 24 hours received 1B recommendations. CONCLUSION: The original American Pediatric Surgery Association guideline for pediatric blunt solid organ injury was instrumental in improving care, but sufficient evidence now exists for an updated management guideline. LEVEL OF EVIDENCE: Expert opinion, guideline, grades I to IV.

Complex spontaneous bile duct perforation: an alternative approach to standard porta hepatis drainage therapy.

INTRODUCTION: Spontaneous perforation of the bile duct (SPBD) is a rare abnormality of the extrahepatic biliary tree in infants. Limited porta hepatis exploration and drainage of the bile-soiled peritoneum are often sufficient treatment. Here, we describe three cases of SPBD, one of which required complex multi-disciplinary interventions for restoration of biliary continuity. METHODS: Three infants with bilious ascites from spontaneous biliary perforation were reviewed. Metrics included age, presenting symptoms, diagnostic tests, interventions performed, and outcomes. RESULTS: The presenting symptoms of all three infants were bilious ascites, sepsis, lethargy, anorexia, fever and persistent emesis. SPBD was confirmed pre-operatively by HIDA scan (hepatobiliary scintigraphy) in each case. Intra-operative, trans-cholecystic cholangiogram confirmed SBDP in two cases. Two of the infants were successfully treated with placement of a cholecystostomy tube and porta hepatis drains. The third infant, having failed multiple similar drainage procedures, required percutaneous transhepatic drainage of a persistent porta hepatis biloma. Ultimately percutaneous, transhepatic cannulation of the extra-hepatic biliary tree for prolonged stenting was required to successfully treat this biliary perforation. CONCLUSION: Complex spontaneous biliary perforation may require extensive interventions if the perforation fails to resolve with standard porta hepatis drainage. Access of the biliary tree via Interventional Radiology procedures for complex biliary disease of this type is novel and presents an alternative to traditional open surgical treatment and control of spontaneous biliary perforations.

Trauma activation: are we making the right call? A 3-year experience at a Level I pediatric trauma center.

BACKGROUND: Trauma is the leading cause of death in children, accounting for half of all deaths in patients between birth and 18 years of age, and is the cause of a significant number of hospital admissions. We reviewed our experience at a Level I pediatric trauma center with a 2-level trauma activation (TA) system for mobilization of personnel over a 3-year period. The aim was to assess severity of injury of the trauma patients, resource use, and outcome. METHODS: After obtaining institutional review board approval, a retrospective analysis of all trauma patients between January 2006 and December 2008 was performed. Data analyzed included number of admissions, level of TA (STAT vs ALERT), mechanism of injury, intensive care unit (ICU) admission, injury severity score (ISS), need for operative intervention, and survival. RESULTS: In 3 years, there were 4502 patients entered. Trauma activation was initiated in 1315 patients (29.2%), divided between 211 STATs (4.7%) and 1104 ALERTs (24.5%). Mean patient age was 5.9 ± 4.1 years, 65% of the patients were boys, and blunt trauma accounted for 92% of the admissions. An ICU admission was required in 736 (16.3%) of the entire group, whereas 502 (38.2%) patients in the TA group were admitted to the ICU(1). The 154 STAT (21%) and 348 ALERT (47%) patients accounted for 68% of all ICU admissions(1). An ISS listed as severe (16-24) or very severe (>24) was found in 468 (10.4%) and 232 (5.2%) patients, respectively. An ISS listed as 16 or higher was found in 144 (68.2%) of the STATs and 264 (23.9%) of the ALERTs(1). Operative intervention was required in 2118 patients (47%). The overall mortality rate was 1.9%, and this increased to 5.8% in the TA group(1). There were 48 deaths (22.7%) in the STAT group, 29 deaths (2.6%) in the ALERT group, and 9 deaths (0.28%) in patients with no TA(1). When emergency department deaths were excluded, the remaining 60 deaths resulted in a mortality rate of 1.3%. CONCLUSIONS: Our Level I pediatric trauma center manages a large volume of patients with significant acuity and, evidenced by a TA in 29% of the patients, a severe or very severe ISS in 16% of the patients, 16% of the patients requiring ICU admission, and 47% requiring operative intervention. The TA patients had markedly higher rates of ICU admission, ISS, and mortality. Deaths in the study were lower by almost an order of magnitude comparing TA STATs with TA ALERTs and TA ALERT patients with patients without TA. The TA criteria are in many ways very helpful and is integral to a Level I trauma center. However, opportunities were identified for improvement because of areas of "overutilization" and discordance between TA and ISS.

Prehospital cardiopulmonary resuscitation in the pediatric trauma patient.

PURPOSE: Children requiring prehospital cardiopulmonary resuscitation (CPR) after traumatic injury have been shown to have poor survival. However, outcome of children still receiving CPR on-arrival by emergency medical service to the emergency department (ED) has not been demonstrated in a published clinical series. METHODS: An 11-year retrospective analysis from a level I pediatric trauma center of the outcomes of children requiring prehospital CPR after traumatic injury was undertaken. Outcome variables were stratified by survival, death, and CPR on-arrival. RESULTS: Of 169 children requiring prehospital CPR, there were 28 survivors and 141 deaths. Of 69 children requiring CPR on-arrival to the ED, there were no survivors. There were 70 females and 99 males. Mean age of survivors was 3.4 years; nonsurvivors, 8.8 years; and 4.6 years for CPR on-arrival. Thirty-nine percent of all injuries were sustained in motor vehicle collisions; 20%, motor pedestrian collisions; 19%, assaults; 7%, falls; 4%, all terrain vehicle/motorcycle/bicycle; and 4%, gunshot wounds. Forty-two percent of all patients expired in the ED, whereas 34% expired in the intensive care unit. Eighty-seven percent of CPR on-arrival patients expired in the ED. Fifty-five percent of survivors had full neurologic recovery. CONCLUSION: Although mortality was extremely high for children requiring CPR in the field After traumatic injury, it was absolute for those arriving at the ED still undergoing CPR.

Late presentation of intestinal malrotation: an argument for elective repair.

BACKGROUND: Midgut malrotation most commonly presents in young children. This diagnosis is not often entertained in the adolescent or adult with abdominal complaints. We reviewed our experience with this subset of malrotation patients. METHODS: A retrospective review of medical records from adolescent or adult patients identified with a diagnosis of anomaly of intestinal fixation or malrotation, who were treated within our health system between 1993 and 2004. RESULTS: A total of 33 patients were diagnosed with malrotation and treated with Ladd's procedure. Acute abdominal pain was present in 50%, and chronic complaints were present in the other patients. Initial work-up included computed tomography (CT) scan (28%), upper gastrointestinal (UGI) study (38%), and plain films (47%) Postoperative complications occurred more frequently in patients that were operated on emergently (60%) than in those that underwent elective surgery (22%; p = 0.04). CONCLUSIONS: This large case series of intestinal malrotation in the nonpediatric age group suggests that Ladd's procedure can be performed very safely. Moreover, the results suggest that patients with known malrotation should have Ladd's procedure performed electively rather than urgently.