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Intestinal malrotation of the midgut is well documented in infants and children but rare in adults. Most adults with intestinal malrotation are asymptomatic, and the anomaly is found incidentally at laparotomy. Classically, the Ladd procedure via a laparotomy is the treatment of choice for patients with symptomatic malrotation. Laparoscopic Ladd procedures have been described in the pediatric literature, but little is known of its efficacy and applicability in adults. We present the case of a symptomatic adult patient found to have malrotation of the midgut with a cocoon deformity, a rare anomaly, that was treated successfully by a laparoscopic Ladd procedure, and we discuss the role of laparoscopy in this entity. [ABSTRACT FROM AUTHOR]

Purpose: Restoration of intestinal continuity by ileal pouch-anal anastomosis (IPAA) following subtotal colectomy may not require a temporary, protective ileostomy. Diversion contributes to patient discomfort, cost, and additional operative risk at the time of subsequent reversal. We compared the outcomes of pediatric patients undergoing modified two-stage to three-stage IPAA after recovering from subtotal colectomy. Methods: We reviewed children (age <18) who underwent IPAA creation for ulcerative or indeterminate colitis from January 1, 2007 to December 31, 2017. Patient characteristics, operative details, 30-day complications, and postoperative length of stay (LOS) were abstracted. Total LOS for the three-stage group included both the IPAA and the ileostomy reversal operations. Univariate comparisons between patients undergoing modified two-stage and three-stage operations were performed. Results: A total of 43 patients underwent IPAA after subtotal colectomy; 32 (74%) underwent a three-stage approach, and 11 (26%) had a modified two-stage approach. Operative approach was laparoscopic in 33 (77%), planned open in 9 (21%), and converted to open in 1 (2%). Single-incision technique was used in 12 of 33 (36%) laparoscopic cases. Modified two-stage procedures had shorter total median LOS (7 days versus 9 days, P = .005). Incidence of postoperative leak, readmission, return to the operating room, and maximum 30-day Clavien-Dindo scores at the time of IPAA creation did not differ between modified two- and three-stage approaches (all P > .05). Conclusion: The modified two-stage approach to IPAA creation resulted in fewer hospital days compared to the three-stage approach. Considering the risks and patient burdens of diversion, further research is needed to assist in decisions regarding protective ileostomy after completion proctectomy with IPAA. [ABSTRACT FROM AUTHOR]

Background: Although single-incision laparoscopic (SIL) ileal pouch-anal anastomosis (IPAA) has been shown to be feasible and safe, outcomes have not been compared with the standard laparoscopic-assisted (LA) procedures. The purpose of this study was to compare the two techniques in children with chronic ulcerative colitis (CUC) and familial adenomatous polyposis (FAP). Subjects and Methods: Children ≤18 years of age who underwent SIL and LA IPAA between 2000 and 2013 at our institution were identified. Patient information was obtained retrospectively from patients' medical records and compared between approaches. Results for operative time and postoperative length of stay were stratified by number of stages (one, two, or three), and postoperative complications were stratified by diagnosis (CUC or FAP). Results: Children who underwent SIL IPAA ( n=19) and LA IPAA ( n=62) were not significantly different in age, gender, diagnosis, anti-tumor necrosis factor-α antibody use, staged approach, and stapled versus mucosectomy with hand-sewn anastomosis. SIL and LA IPAA had equivalent operative times for two- and three-stage procedures, but operative time for one-stage procedures was shorter with SIL (308 versus 355 minutes; P<.001). Median length of stay was shorter following SIL for all patients (4 versus 7 days; P<.001) and, specifically, for two-stage patients (4 versus 6 days; P=.009). There were no significant differences in complications between SIL and LA. Conclusions: SIL IPAA is a safe alternative to LA IPAA for children with CUC or FAP and may reduce postoperative length of stay without affecting short-term postoperative morbidity. Additional studies are needed to determine if there are long-term benefits. [ABSTRACT FROM AUTHOR]

Aim The minimally-invasive Nuss procedure has become the preferred technique for pectus excavatum repair. This procedure is still associated with significant postoperative pain, and an optimal pain-management strategy is yet to be determined. The purpose of this study was to compare the efficacy of patient-controlled analgesia ( PCA) to thoracic epidural analgesia ( TEA). Patients and Methods We retrospectively reviewed 112 charts from a single paediatric centre. Patients were grouped according to pain-management strategy: 90 patients received a PCA, and 22 patients received TEA. Outcomes included length of hospitalization and daily pain scores, operating room time and duration of Foley catheterization. Results Demographic data were similar between the two groups. The daily pain scores were not statistically different between the groups. Length of hospitalization was similar ( PCA: 4.6 days, epidural: 4.3 days, P = 0.33). The PCA group required less operating room time (2:44 vs 2:58, P = 0.04) and shorter Foley catheter duration (2.1 days vs 2.5 days, P = 0.04). Conclusion In our patient population, TEA for the Nuss procedure does not offer an advantage over PCA-centred analgesia in terms of subjective daily pain scores or length of hospital stay. The potential risks of TEA need to be carefully considered in this patient population. [ABSTRACT FROM AUTHOR]

Background: Although single-incision endoscopic splenectomy (SIES-Sp) has been shown to be feasible and safe, few have compared the SIES-Sp with multiport laparoscopic splenectomy (MPLS). The purpose of this study was to compare the two techniques in children undergoing total splenectomy. Materials and Methods: We reviewed all children (age <18 years) who underwent minimally invasive total splenectomy at a single tertiary referral center from January 1, 2000 to January 1, 2019. The primary outcome was complication rate 30 days after discharge defined by maximum Clavien-Dindo score. Secondary outcomes included conversion, operative time, hospital length of stay, postoperative pain scores, and readmission within 30 days of discharge. SIES-Sp and MPLS were compared using univariate analysis. Results: Of 48 children undergoing laparoscopic total splenectomy, 60% (n = 29) were SIES-Sp and 40% (n = 19) were MPLS. Subjects were 48% female (n = 23). Common diagnoses were idiopathic thrombocytopenic purpura (33% [n = 16]), hereditary spherocytosis (29% [n = 14]), and other congenital hemolytic anemias (23% [n = 11]). There were no differences in age, gender, or diagnosis between groups (all P > .05). One in three cases involved additional procedures. Spleens were smaller in both greatest dimension (13.0 cm versus 16.4 cm) and weight (156.5 g versus 240.0 g) in SIES-Sp compared with MPLS patients (both P < .05). Readmission and reoperation rates were similar (both P > .05). Complications occurred in 7% (n = 2) of SIES-Sp and in 11% (n = 2) of MPLS patients (P > .99). Severe complications included: cardiac arrest in 1 SIES-Sp patient and bleeding requiring reoperation in 1 MPLS patient. Conclusion: SIES-Sp is a safe alternative to the traditional MPLS for children. Additional procedures do not preclude a less invasive approach, but larger spleens may present a challenge. [ABSTRACT FROM AUTHOR]

Background: The pediatric resuscitation and trauma outcome (PRESTO) model was developed to aid comparisons of risk-adjusted mortality after injury in low- and middle-income countries (LMICs). We sought to validate PRESTO using data from a middle-income country (MIC) trauma registry and compare its performance to the Pediatric Trauma Score (PTS), Revised Trauma Score, and pediatric age-adjusted shock index (SIPA). Methods: We included children (age < 15 years) admitted to a single trauma center in South Africa from December 2012 to January 2019. We excluded patients missing variables necessary for the PRESTO model—age, systolic blood pressure, pulse, oxygen saturation, neurologic status, and airway support. Trauma scores were assigned retrospectively. PRESTO's previously high-income country (HIC)-validated optimal threshold was compared to MIC-validated threshold using area under the receiver operating characteristic curves (AUROC). Prediction of in-hospital death using trauma scoring systems was compared using ROC analysis. Results: Of 1160 injured children, 988 (85%) had complete data for calculation of PRESTO. Median age was 7 (IQR: 4, 11), and 67% were male. Mortality was 2% (n = 23). Mean predicted mortality was 0.5% (range 0–25.7%, AUROC 0.93). Using the HIC-validated threshold, PRESTO had a sensitivity of 26.1% and a specificity of 99.7%. The MIC threshold showed a sensitivity of 82.6% and specificity of 89.4%. The MIC threshold yielded superior discrimination (AUROC 0.86 [CI 0.78, 0.94]) compared to the previously established HIC threshold (0.63 [CI 0.54, 0.72], p < 0.0001). PRESTO showed superior prediction of in-hospital death compared to PTS and SIPA (all p < 0.01). Conclusion: PRESTO can be applied in MIC settings and discriminates between children at risk for in-hospital death following trauma. Further research should clarify optimal decision thresholds for quality improvement and benchmarking in LMIC settings. [ABSTRACT FROM AUTHOR]

Purpose: The objective of this study was to examine the long-term outcomes of pediatric patients who underwent surgical resection for lipoblastoma and lipoblastomatosis (LB/LBM).Methods: A single-center retrospective study of pediatric patients with LB/LBMs seen between 1991 and 2015 was conducted. A systematic review, including studies published prior to late August 2018, was performed. Using a random effect meta-analysis, pooled weighted proportions and unadjusted odds ratios (OR) with 95% confidence intervals (CI) were calculated.Results: The retrospective study included 16 patients, while the systematic review included 19 published studies consisting of 381 patients. Among 329 (82%) patients with follow-up information, the pooled recurrence rate was 16.8% (95% CI 10.9-23.5%; I2 = 59%). The reported time to recurrence ranged from < 1 to 8 years. Recurrence risk was greater for incomplete (n = 34) than complete resection (n = 150): OR 11.4 (95% CI 3.0-43.6; I2 = 43%). LBMs (n = 35) had a greater recurrence risk than LBs (n = 116): OR 5.5 (95% CI 1.9-15.9; I2 = 0%). Recurrences were higher for studies with approximately ≥ 3 years of follow-up versus studies with < 3 years of follow-up.Conclusion: Recurrences are more likely to occur with LBMs and/or incomplete resection. Follow-up beyond 3-5 years should be considered given that the recurrence risk appears to be greater in the long-term. [ABSTRACT FROM AUTHOR]

Background: Adrenalectomy for non-neuroblastic pathologies in children is rare with limited data on outcomes. We reviewed our experience of adrenalectomy in this unique population.Methods: Retrospective study of children (age ≤ 18) who underwent adrenalectomy with non-neuroblastic pathology from 1988 to 2018. Clinical and operative details of patients were abstracted. Outcomes included length of stay and 30-day postoperative morbidity.Results: Forty children underwent 50 adrenalectomies (12 right-sided, 18 left-sided, 10 bilateral). Six patients (15%) presented with an incidental adrenal mass while 4 (10%) had masses found on screening for genetic mutations or prior malignancy. The remaining 30 (75%) presented with symptoms of hormonal excess. Nineteen patients (48%) underwent genetic evaluation and 15 (38%) had genetic predispositions. Diagnoses included 9 patients (23%) with pheochromocytoma, 8 (20%) with adrenocortical adenoma, 8 (20%) with adrenocortical carcinoma, 7 (18%) with adrenal hyperplasia, 2 (5%) with metastasis, and 6 (14%) with additional benign pathologies. Of 50 adrenalectomies, twenty-five (50%) were laparoscopic. Median hospital length of stay was 3 days (range 0-11). Post-operative morbidity rate was 17% with the most severe complication being Clavien-Dindo grade II.Conclusion: Adrenalectomy for non-neuroblastic pathology can be done with low morbidity. Its frequent association with genetic mutations and syndromes requires surgeons to have knowledge of appropriate pre-operative testing and post-operative surveillance. [ABSTRACT FROM AUTHOR]

Background: Operative maneuvers to increase mesenteric length during ileal pouch-anal anastomosis (IPAA) are frequently utilized in adults, but limited data exist on the need for their use in children. Materials and Methods: A retrospective chart review of children (age <18) considered for IPAA creation at two affiliated tertiary referral centers from 2007 to 2017 was conducted, and patient factors, operative details, and 30-day postoperative complications were abstracted. Body mass index (BMI) was normalized to BMI percentile-for-age-and-sex and classified as underweight (BMI <5th percentile), healthy weight (5th ≤ BMI percentile <85th), or overweight/obese (BMI ≥85th percentile). Maneuvers were identified from operative notes. Univariate analysis and multivariable logistic regression were performed to determine independent factors associated with the use of maneuvers. Results: A total of 94 patients underwent attempt at IPAA creation, which was successful in 91 (97%). Fourteen (15%) of 91 patients were classified as overweight or obese. The 3 failures occurred secondary to inability to reach in 3 patients, with specific mention of patients' obesity in 2 and pouch ischemia in 1. Sixty (66%) patients required maneuvers to lengthen the mesentery. Overweight/obese patients required maneuvers more often than nonoverweight/obese patients (93% versus 61%, P = .03). There were no differences in 30-day maximum Clavien-Dindo scores between patients with and without maneuvers performed (P = .83). Being overweight/obese was an independent risk factor for requiring maneuvers (odds ratio: 9.3, 95% confidence interval: 1.1-82.8) after adjusting for age, sex, height, operative stage, and surgeon. Conclusion: Surgeons should be prepared to perform mesenteric lengthening maneuvers when operating on pediatric patients to ensure minimal tension on the IPAA, and more so when operating on obese children. Whether these maneuvers have an impact on long-term pouch function is undetermined. [ABSTRACT FROM AUTHOR]

Background: Despite surgical correction procedures for pectus deformities, remaining cosmetic asymmetry may have significant psychological effects. We sought to evaluate factors associated with plastic surgery (PS) consultation and procedures for these deformities at an academic institution.Methods: We reviewed patients aged 0-21 diagnosed with a pectus excavatum or carinatum deformity at our institution between January 2001 and October 2016. Pectus diagnoses were identified by ICD-9/ICD-10 codes and surgical repair by CPT codes; patients receiving PS consultation were identified by clinical note service codes. Student's t tests, Fisher's exact tests, and Chi-squared tests were utilized.Results: 2158 patients were diagnosed with a pectus deformity; 442 (20.4%) underwent surgical correction. 19/442 (4.3%) sought PS consultation, either for pectus excavatum [14/19 (73.7%)], carinatum [4/19 (21.0%)], and both [1/19 (5.3%)], (p = 0.02). Patients seeking PS consultation were more likely to be female (p < 0.01), have scoliosis (p = 0.02), or undergo an open repair (p < 0.01). The need for PS consultation did not correlate with Haller index, p = 0.78.Conclusion: PS consultation associated with pectus deformity repair was rare, occurring in < 5% of patients undergoing repair. Patients who consulted PS more commonly included females, patients with scoliosis, and those undergoing open repair. These patients would likely benefit most from multidisciplinary pre-operative discussions regarding repair of the global deformity. [ABSTRACT FROM AUTHOR]

Purpose: Prehospital transfusions are a novel yet increasingly accepted intervention in the adult population as part of remote damage control resuscitation, but prehospital transfusions remain controversial in children. Our purpose was to review our pediatric prehospital transfusion experience over 12 years to describe the safety of prehospital transfusion in appropriately triaged trauma and nontrauma patients.Methods: Children (<18 years) transfused with packed red blood cells (pRBC) or plasma during transport to a single regional academic medical center between 2002 and 2014 were identified. Admission details, in-hospital clinical course, and outcomes were analyzed.Results: 28 children were transfused during transport; median age was 8.9 ± 7 years and 15 patients were male (54%). Most patients required at least one additional unit of blood products during their hospitalization (79%), and/or required operative intervention (53%), endoscopy (7%), or died during their hospitalization (14%). Comparison of trauma patients (n = 16) and nontrauma patients (n = 12) revealed that nontrauma patients were younger, more anemic, more coagulopathy on admission, and required more ongoing transfusion in the hospital. Trauma patients were more likely to need operative intervention. No patient had a transfusion reaction.Conclusion: Remote damage control prehospital transfusions of blood products were safe in this small group of appropriately triaged pediatric patients. Further studies are needed to determine if outcomes are improved and to devise a rigorous protocol for this prehospital intervention for critically ill pediatric patients. [ABSTRACT FROM AUTHOR]

Introduction: Injured children may be transported to trauma centers by helicopter air ambulance (HAA); however, a benefit in outcomes to this expensive resource has not been consistently shown in the literature and there is concern that HAA is over-utilized. A study that adequately controls for selection biases in transport mode is needed to determine which injured children benefit from HAA. The purpose of this study was to determine if HAA impacts mortality differently in minimally and severely injured children and if there are predictors of over-triage of HAA in children that can be identified.Methods: Children ≤18 years of age transported by HAA or ground ambulance (GA) from scene to a trauma center were identified from the 2010-2011 National Trauma Data Bank. Analysis was stratified by Injury Severity Score (ISS) into low ISS (≤15) and high ISS (>15) groups. Following propensity score matching of HAA to GA patients, conditional multivariable logistic regression was performed to determine if transport mode independently impacted mortality in each stratum. Rates and predictors of over-triage of HAA were also determined.Results: Transport by HAA occurred in 8218 children (5574 low ISS, 2644 high ISS) and by GA in 35305 (30506 low ISS, 4799 high ISS). Overall mortality was greater in HAA patients (4.0 vs 1.4%, p<0.001). After propensity score matching, mortality was equivalent between HAA and GA for low ISS patients (0.2 vs 0.2%, p=0.82) but, for high ISS patients, mortality was lower in HAA (9.0 vs 11.1% p=0.014). On multivariable analysis, HAA was associated with decreased mortality in high ISS patients (OR=0.66, p=0.017) but not in low ISS patients (OR=1.13, p=0.73). Discharge within 24h of HAA transport occurred in 36.5% of low ISS patients versus 7.4% high ISS patients (p<0.001).Conclusions: Based on a national cohort adjusted for nonrandom assignment of transport mode, a survival benefit to HAA transport exists only for severely injured children with ISS >15. Many children with minor injuries are transported by helicopter despite frequent dismissal within 24h and no mortality benefit. [ABSTRACT FROM AUTHOR]

Introduction: Few studies have assessed the impact of obesity on laparoscopic cholecystectomy (LC) in pediatric patients.Materials and Methods: Children who underwent LC were identified from the 2012 to 2013 American College of Surgeons' National Surgical Quality Improvement Program Pediatrics data. Patient characteristics, operative details, and outcomes were compared. Multivariable logistic regression was utilized to identify predictors of increased operative time (OT) and duration of anesthesia (DOAn).Results: In total, 1757 patients were identified. Due to low rates of obesity in children <9 years old, analyses were limited to those 9-17 (n = 1611, 43% obese). Among obese children, 80.6% were girls. A higher proportion of obese patients had diabetes (3.0% versus 1.0%, P < .01) and contaminated or dirty/infected wounds (15.1% versus 9.4%, P < .01). Complication rates were low. The most frequent indications for surgery were cholelithiasis/biliary colic (34.3%), chronic cholecystitis (26.9%), and biliary dyskinesia (18.2%). On multivariable analysis, obesity was an independent predictor of OT >90 (odds ratio [OR] 2.02; 95% confidence interval [95% CI] 1.55-2.63), and DOAn >140 minutes (OR 1.86; 95% CI 1.42-2.43).Conclusions: Obesity is an independent risk factor for increased OT in children undergoing LC. Pediatric surgeons and anesthesiologists should be prepared for the technical and physiological challenges that obesity may pose in this patient population. [ABSTRACT FROM AUTHOR]

Background: Pectus excavatum results from dorsal deviation of the sternum causing narrowing of the anterior-posterior diameter of the chest. It can result in significant cosmetic deformities and cardiopulmonary compromise if severe. The Nuss procedure is a minimally invasive technique that involves placing a thin horizontally oriented metal bar below the dorsal sternal apex for correction of the pectus deformity.Objective: To identify the frequency and types of Nuss bar migrations, to present a new categorization of bar migrations, and to present examples of true migrations and pseudomigrations.Materials and Methods: We retrospectively reviewed the electronic medical records and all pertinent radiologic studies of 311 pediatric patients who underwent a Nuss procedure. We evaluated the frequency and type of bar migrations.Results: Bar migration was demonstrated in 23 of 311 patients (7%) and occurred within a mean period of 26 days after surgery. Bar migrations were subjectively defined as deviation of the bar from the position demonstrated on the immediate postoperative radiographs and categorized as superior, inferior, rotation, lateral or flipped using a new classification system. Sixteen of the 23 migrations required re-operation.Conclusion: Nuss bar migration can be diagnosed with careful evaluation of serial radiographs. Nuss bar migration has a wide variety of appearances and requires exclusion of pseudomigration resulting from changes in patient positioning between radiologic examinations. [ABSTRACT FROM AUTHOR]

Purpose: The incidence and etiology of empyema with fistula (EWF) in children is unknown. We analyzed a national database to define the epidemiology and diagnoses associated with this condition.Methods: Discharge data from the Kids' Inpatient Database were reviewed for EWF (ICD-9 diagnosis code 510.0) in children ≤18 years from 2000 to 2012. Patient characteristics, institutional data, and accompanying conditions were evaluated. Weighted national estimates were calculated and incidence compared across years (2000, 2003, 2006, 2009) using the Rao-Scott Chi Square.Results: From 2000 to 2012, 908 children were hospitalized with EWF. Age distribution was bimodal. Common primary diagnoses related to the hospitalization were pneumonia/pulmonary abscess (31.2 %) and EWF (19.3 %). Manipulation of the pleural space (e.g. decortication, drainage) comprised 45.0 % of procedures. Incidence rates of EWF increased (Rao Scott Adjusted Chi Square: 16.13, p < 0.01) over the study period. Although not statistically significant, median length of stay and age of diagnosis decreased and increased, respectively.Conclusion: This first, national pediatric EWF study reveals rising incidence during the years 2000-2009. Despite limitations in ICD-9 coding, concomitant primary diagnoses and procedures suggest bronchopleural fistulae likely represent the vast majority of cases in this cohort. Multi-institutional studies are needed to confirm etiology and characterize outcome of EWF. [ABSTRACT FROM AUTHOR]

Background: We sought to examine our institutional experience (1998 to 2014) with minimally invasive repairs of pectus excavatum (MIRPE).Study Design: We conducted a retrospective review and a mailed survey (quality of life assessment). Associations with reoperation due to bar migration and recurrence after bar removal were evaluated with logistic regression.Results: Three hundred and thirteen patients (79% male) underwent MIRPE at a mean ± SD age of 15 ± 3 years. Bar migration requiring reoperation occurred in 16 (5%) patients (median 26 days, interquartile range 15 to 70 days from repair). Wire fixation (hazard ratio [HR] = 3.16; p = 0.014) and bar stabilizer (HR = 4.57; p = 0.002) use were associated with increased risk of reoperation, and bilateral pericostal suture fixation (HR = 0.15; p < 0.001) and thoracoscopic assistance (78%, HR = 0.23; p < 0.001) were associated with decreased risks. Reoperations rates varied (6% to 26%) during the first 50 cases of each surgeon (n = 6), falling to ≤2% afterward. Of the 101 (32%) patients who have had their bars removed electively, 10 (10%) have required reoperation for recurrence. Patients with a recurrence after bar removal were younger (14.1 ± 3.9 years vs 18.4 ± 3.7 years; p = 0.007) and had their bars removed earlier (2.4 ± 1.2 years vs 3.8 ± 2.1 years; p = 0.036). Of survey respondents (n = 145 [47%]), most (99%) were either very happy (n = 79) or mostly happy (n = 63) with their outcomes.Conclusions: Although excellent outcomes after MIRPE can be achieved, our results highlight identified strategies that are associated with decreased risk of reoperation (eg, use of bilateral pericostal suture fixation, surgeon experience, and thoracoscopic guidance). Our results also suggest that elective bar removal should be delayed until the patient is at least 18 years old and has had the bar in for at least 4 years. [ABSTRACT FROM AUTHOR]

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal.Methods and Results: We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD.Conclusions: LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks. [ABSTRACT FROM AUTHOR]

Background--Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Blockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results--We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1- and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD. Conclusions--LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To characterize the risk factors and patterns of Injury for children Involved in snowmobile incidents. PATIENTS AND METHODS: We reviewed the medical records of patients younger than 18 years who required hospital admission for snowmobile-related incidents from 1992 to 2001. Information obtained from these records and from the trauma database included patient demographics, mechanism of Injury, injury patterns, medical care, and outcomes. RESULTS: Forty-three patients were admitted to our hospital for snowmobile-related Incidents. Snowmobile Incidents occurred most commonly in male adolescents. The 2 most common mechanisms of Injury were ejection and striking a stationary object. Twenty-seven (63%) of the patients drove the snowmobile. Only 23 patients (53%) wore a helmet. At presentation, the mean ± SEM Injury Severity Score (ISS) was 12.1±1.4. Orthopedic Injuries predominated (n=42); however, abdominal (n=12) and head (n=8) Injuries were also common. Four patients were intubated, and 15 required Intensive care unit admission. Twenty-nine patients (67%) required surgical intervention. The mean ± SEM length of hospitalization was 6.7±1.4 days. No deaths occurred; however, 7 patients (16%) had long-term disabilities. A significant improvement occurred In both Glasgow Coma Scale (GCS) score and ISS for patients using a helmet. In addition, helmet use Increased with age (P=.01). Days in the Intensive care unit were proportional to both GCS score (ç=-0.47 P=.002) and ISS (rs=0.6; P<.001). Length of hospitalization also correlated with both GCS score (rs=-.0.03; P=.008) and 155 (rs=0.54; P<.02). CONCLUSION: Snowmobiles are a significant source of multi- trauma for children. Orthopedic Injuries predominate, especially in older children, and can lead to long-term disabilities. Helmet use significantly reduces Injuries; however, vulnerable younger patients do not frequently wear helmets. [ABSTRACT FROM AUTHOR]

Accidental burial in sand is a tragically unrecognized risk associated with a popular childhood recreational activity. We describe 4 boys, aged 10 to 13 years, who were accidentally buried by sand. One boy died after his self-made tunnel in a sandbox collapsed. In a separate incident at a construction site, 1 boy died, and 2 were injured after a 30-foot sandpile collapsed as they ran down the embankment; all 3 were buried by the sand. In both incidents, play was unsupervised, and burial was sudden and complete. The calculated weight of the sand exceeded the expected maximal muscle effort of the chest, leading to traumatic asphyxiation secondary to restrictive compression of the chest. Only 15 accidental burials have been reported in the literature. To our knowledge, this is the first report describing children who died of respiratory asphyxia due to overwhelming thoracic compression after sand burial. Greater awareness by public health and safety officials at beaches, sandboxes, sandpiles, and natural play areas may prevent potentially lethal accidents. [ABSTRACT FROM AUTHOR]

A response from the author of the article "Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia: The Role of Left Cardiac Sympathetic Denervation" is presented in the 2016 issue of the journal.