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We evaluate the incidence, outcomes, and management of peri‑umbilical hernias after sutured or sutureless gastroschisis closure.A retrospective, longitudinal follow-up of neonates with gastroschisis who underwent closure at 11 children's hospitals from 2013 to 2016 was performed. Patient encounters were reviewed through 2019 to identify the presence of a peri‑umbilical hernia, time to spontaneous closure or repair, and associated complications.Of 397 patients, 375 had follow-up data. Sutured closure was performed in 305 (81.3%). A total of 310 (82.7%) infants had uncomplicated gastroschisis. Peri-umbilical hernia incidence after gastroschisis closure was 22.7% overall within a median follow-up of 2.5 years [IQR 1.3,3.9], and higher in those with uncomplicated gastroschisis who underwent primary vs. silo assisted closure (53.0% vs. 17.2%, p0.001). At follow-up, 50.0% of sutureless closures had a persistent hernia, while 16.4% of sutured closures had a postoperative hernia of the fascial defect (50.0% vs. 16.4%, p0.001). Spontaneous closure was observed in 38.8% of patients within a median of 17 months [9,26] and most frequently observed in those who underwent a sutureless primary closure (52.2%). Twenty-seven patients (31.8%) underwent operative repair within a median of 13 months [7,23.5]. Rate and interval of spontaneous closure or repair were similar between the sutured and sutureless closure groups, with no difference between those who underwent primary vs. silo assisted closure.Peri-umbilical hernias after sutured or sutureless gastroschisis closure may be safely observed similar to congenital umbilical hernias as spontaneous closure occurs, with minimal complications and no additional risk with either closure approach.Level II.

Purpose To report outcomes of sutured and sutureless closure for gastroschisis across a large multi-institutional cohort. Methods A retrospective study of infants with uncomplicated gastroschisis at 11 children's from 2014 to 2016 was performed. Outcomes of sutured and sutureless abdominal wall closure were compared. Results Among 315 neonates with uncomplicated gastroschisis, sutured closure was performed in 248 (79%); 212 undergoing sutured closure after silo and 36 undergoing primary sutured closure. Sutureless closure was performed in 67 (21%); 37 primary sutureless closure, 30 sutureless closure after silo placement. There was no significant difference in gestational age, gender, birth weight, total days on TPN, and time from closure to initial oral intake or goal feeds. Sutureless closure patients had less general anesthetics, ventilator use/time, time from birth to final closure, antibiotic use after closure, and surgical site/deep space infections. Subgroup analysis demonstrated primary sutureless closure had less ventilator use and anesthetics than primary sutured closure. Sutureless closure after silo led to less ventilator use/time, anesthetics, and antibiotics compared to those with sutured closure after silo. Conclusion Sutureless abdominal wall closure of neonates with gastroschisis was associated with less general anesthetics, antibiotic use, surgical site/deep space infections, and decreased ventilator time. These findings support further prospective study by our group. Level of Evidence Level III.

Introduction The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. Methods After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. Results Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1–12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6–11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). Conclusion This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients. Level of evidence Level II.

Introduction The purpose of this study was to evaluate short-term respiratory outcomes in neonates with symptomatic congenital lung malformations (CLM). Methods Consecutive newborns who underwent surgical resection of a CLM were retrospectively reviewed. Demographic, prenatal, and outcomes data were analyzed as appropriate (p Results Twenty-one neonates were managed at a median gestational age of 36.2 weeks [interquartile range (IQR), 33.8–39.0]. Endotracheal intubation was required in 14 (66.7%) for a median of 7.5 days [interquartile range (IQR), 3.0–25.8]. Three (14.3%) children underwent ex utero intrapartum treatment-to-resection, and another 14 (66.7%) had neonatal lung resections performed at a median age of 2.0 days (IQR, 0.08–19.5 days). Excluding one patient who received comfort care at birth, all neonates survived to hospital discharge with a median length of hospitalization of 36.5 days (IQR, 23.8–56.5). More than one-quarter were discharged on supplemental oxygen by nasal cannula. Based on a median follow up of 35.5 months (IQR, 19.0–80.8), CLM-related morbidity was still evident in 55.0%. Conclusion Our study suggests a high incidence of complications and chronic respiratory morbidity after neonatal lung resection for symptomatic CLMs. These data highlight the need to provide realistic expectations in perinatal counseling discussions with families and the importance of coordinating appropriate multidisciplinary follow up for these children. Level of Evidence: Level IV.

Introduction: Inguinal hernias are among the most common pediatric surgical operations. For pediatric patients a high ligation of the hernia sac is the most common type of repair, which ca...

Recent advances in ECLS technology have led to the adoption of centrifugal pumps for the majority of patients worldwide. Despite several advantages of centrifugal pumps, they remain controversial because a number of studies have shown increased rates of hemolysis. The aim of this study was to assess the impact of transitioning from roller to centrifugal pumps on hemolysis rates at our center. A retrospective analysis of all pediatric ECMO patients at a single center between 2005 and 2017 was undertaken. Hemolysis was defined as a plasma free hemoglobin >50 mg/dL. Multivariable logistic regression was performed correcting for several factors to determine risk factors for hemolysis and analyze outcomes among patients with hemolysis. Significant findings were those with p

Introduction Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol. Materials and Methods A retrospective study of neonates with uncomplicated gastroschisis at 11 children's hospitals from 2013 to 2016 was performed.Outcomes of infants fed via institutional-specific protocols were compared with those fed without a protocol. Subgroup analyses of protocol use with immediate versus delayed closure and with sutured versus sutureless closure were conducted. Results Among 315 neonates, protocol-based feeding was utilized in 204 (65%) while no feeding protocol was used in 111 (35%). There were less surgical site infections (SSI) in those fed with a protocol (7 vs. 16%, p = 0.019). There were no differences in TPN duration, time to initial oral intake, time to goal feeds, ventilator use, peripherally inserted central catheter line deep venous thromboses, or length of stay. Of those fed via protocol, less SSIs occurred in those who underwent sutured closure (9 vs. 19%, p = 0.026). Further analyses based on closure timing or closure method did not demonstrate any significant differences. Conclusion Across this multi-institutional cohort of infants with uncomplicated gastroschisis, there were more SSIs in those fed without an institutional-based feeding protocol but no differences in other outcomes.

Purpose Inguinal hernias are amongst the most common surgical conditions in children. Typically, these repairs are performed through an open or laparoscopic approach, using a high ligation of the hernia sac. The use of ultrasound has been described in identifying and evaluating hernia contents in children. Our goal was to determine if ultrasound guidance could be used to perform a high ligation of the hernia sac in pediatric patients. Methods Following IRB approval, a retrospective review of all female patients at a single center undergoing ultrasound guided inguinal hernia repair between 2017 and 2018 was performed. Pre-operative characteristics, intra-operative outcomes, and post-operative outcomes were all evaluated. Laparoscopy was used to evaluate the repair and evaluate for a contralateral hernia. Male patients did not undergo ultrasound inguinal hernia repair to avoid damage to the vas deferens and vessels. Results A total of 10 patients with 13 hernias total were found during the study period. A total of one patient was converted to a laparoscopic repair. No patients were found to have an inappropriate repair or a missed contralateral hernia, and there were no vascular injuries or injuries to surrounding structures. No patients had a hernia recurrence during the study period. Conclusion This study demonstrates the safety and feasibility of ultrasound guided inguinal hernia repairs in female pediatric patients. Further study is needed to compare these repairs to existing techniques, evaluate for recurrences over time, and evaluate if these repairs can be performed without general anesthetic in some patients.

To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling. A retrospective study of 394 neonates with gastroschisis at 11 children’s hospitals from January 2013 to March 2017 was performed. Analysis by Fisher’s exact tests and Wilcoxon rank sum tests were performed. Outcomes of complex and simple gastroschisis are reported. There were 315 (80%) SG and 79 (20%) CG. CG had increased time from birth to closure (6 vs 4.4 days), closure to goal feeds (69 vs 23 days), ventilator use (90% vs 73%), SSIs (31% vs 11%), NEC (14% vs 6%), PN use (71 vs 24 days), LOS (104.5 vs 33 days), and mortality (11% vs 0%). This study provides generalizable estimates for expected outcomes of patients with both SG and CG that can be utilized during counseling. CG has significantly worse in-hospital outcomes.

The advent of laparoscopy has revolutionized surgical practice within the last 30 years. Conversion to open surgery, however, remains necessary at times, even for the most experienced laparoscopic surgeon.The kids' inpatient database was analyzed for 2006, 2009, and 2012 for patients who underwent laparoscopic appendectomy and conversion to open (CPT 470.1 and V64.41, respectively). Variables included in multivariable analysis were determined based on those variables found to have significance on univariate analysis.A total of 104,865 patients, ages 0-17 years, underwent laparoscopic appendectomy during the three study periods. Of these, 2370 (2.2%) laparoscopic surgeries were converted to open appendectomy. Multivariable logistic regression showed significantly higher rates of conversion amongst patients with peritonitis (OR 6.7, p 0.001) or abscess (OR 14.3, p 0.001), obesity (OR 2.02, p 0.001), age 13 years (OR 1.53 for ages 13-15, OR 1.77 for ages 16-17, p 0.001 for both), or cared for at rural hospitals (OR 1.55, p = 0.002). Rates of conversion decreased over time for children at adult hospitals and at urban hospitals, regardless of teaching status (p 0.001 for both).Risk factors for conversion from laparoscopic to open appendectomy included abscess, peritonitis, increased age, obesity, male gender, socioeconomic status and treatment at a non-pediatric-specific hospital, and the overall rate is decreasing over time.

Purpose For the last seven years, our institution has repaired infants with CDH that require ECMO early after cannulation. Prior to that, we attempted to decannulate before repair, but repaired on ECMO if we were unable to wean after two weeks. This study compares those strategies. Methods From 2002 to 2016, 65 infants with CDH required ECMO. 67.7% were repaired on ECMO, and 27.7% were repaired after decannulation. Data were compared between patients repaired ≤5days after cannulation ("early protocol", n=30) and >5days after cannulation or after de-cannulation ("late protocol", n=35). We used Cox regression to assess differences in outcomes between groups. Results Survival for the early and late protocol groups was 43.3% and 68.8%, respectively (p=0.0485). For patients that were successfully decannulated before repair, survival was 94.4%. Moreover, the early repair protocol was associated with prolongation of ECMO (16.8±7.4 vs. 12.6±6.8days, p =0.0216). After multivariate regression, the early repair protocol was an independent predictor of both mortality (HR=3.48, 95% CI=1.28–9.45, p =0.015) and days on ECMO (IRR=1.39, 95% CI=1.07–1.79, p =0.012). All bleeding occurred in patients repaired on ECMO (29.5%, 13/44). Conclusions Our data suggest that protocolized CDH repair early after ECMO cannulation may be associated with increased mortality and prolongation of ECMO. However, early repair is not necessarily harmful for those patients who would otherwise be unable to wean from ECMO before repair. Further work is needed to better move towards individualized patient care. Type of study Treatment Study. Level of evidence Level III.

Guidelines for diagnosis and treatment of adrenal insufficiency (AI) in newborns with congenital diaphragmatic hernia (CDH) are poorly defined. From 2002 to 2016, 155 infants were treated for CDH at our institution. Patients with shock refractory to vasopressors (clinically diagnosed AI) were treated with hydrocortisone (HC). When available, random cortisol levels

The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium.After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015).Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0).Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion.Level III.

Airway foreign bodies (FB) are a common medical emergency within the pediatric population. While deaths are not uncommon, the in-hospital mortality rates and correlation with anatomic location of the airway foreign body have not been previously reported. The KID database was reviewed for 2003, 2006, 2009, and 2012 for pediatric patients with a discharge diagnosis of airway foreign body using ICD-9 codes (933.1, 934.x). 11,793 patients, ages 0–17, were found to have an airway FB. Of patients admitted for airway FB 21.2 % required mechanical ventilation during their hospitalization, and the overall mortality rate was 2.5 %. Location of the airway FB was dependent on age (p

This study aimed to evaluate postoperative outcomes after minimally invasive repair of pectus excavatum (Nuss procedure) using video-assisted intercostal nerve cryoablation (INC) compared to thoracic epidural (TE).We performed a single center retrospective review of pediatric patients who underwent Nuss procedure with INC (n = 19) or TE (n = 13) from April 2015 to August 2017. Preoperative, intraoperative, and postoperative characteristics were collected. The primary outcome was length of stay (LOS) and secondary outcomes were intravenous and oral opioid use, pain scores, and complications. Opioids were converted to oral morphine milligram equivalents per kilogram (oral morphine equivalent [OME]/kg). Mann-Whitney U test was used for continuous and chi-squared analysis for categorical variables.There were no significant differences in patient characteristics, except Haller Index (INC: median [interquartile range] 4.3 [3.6-4.9]; TE: 3.2 [2.8-4.0]; P = 0.03). LOS was shorter with INC (INC: 3 [3-4] days; TE: 6 [5-7] days; P 0.001). Opioid use was higher intraoperatively (INC: 1.08 [0.87-1.37] OME/kg; TE: 0.46 [0.37-0.67] OME/kg; P = 0.002) and unchanged postoperatively (INC: 1.78 [1.26-3.77] OME/kg; TE: 1.82 [1.05-3.37] OME/kg; P = 0.80), and prescription doses were lower at discharge in INC (INC: 30 [30-40] doses; TE: 42 [40-60] doses; P = 0.005). There was no significant difference in postoperative complications (INC: 42.1%; TE: 53.9%; P = 0.51).INC during Nuss procedure reduced LOS, shifting postoperative opioid use earlier during admission. This may reflect the need for improved early pain control until INC takes effect. Prospective evaluation after INC is needed to characterize long-term pain medication requirements.

Background Neurologic complications are common, and amongst the most devastating complications in pediatric patients undergoing extracorporeal life support (ECLS). Carotid artery cannulation (CAN) has been associated with an increase in these complications, thereby shaping practices to avoid this approach in most pediatric patients in which other cannulation approaches are viable. Methods A retrospective review of children (0–18years) in the ELSO database was undertaken from 1989 through 2013. Multivariate logistic regression analysis of rates of stroke and other neurologic complications based on cannulation technique was undertaken, adjusting for patient factors including age, underlying disease process, and severity of illness. Results A total of 30,282 ECLS runs were found in the database. CAN was associated with higher rates of stroke (5.15% vs 3.74%) and overall neurologic complications. However, when correcting for patient factors, including age, underlying disease process, and support type, CAN was not associated with an increased rate of neurologic complications or stroke (p>0.05 for both). Conclusion When correcting for patient related factors CAN is not associated with an increase in stroke or neurologic compilcations. CAN should be re-examined as a cannulation technique for older pediatric patients. Level of evidence III.

Jejunal diverticulosis is a rare condition that is usually found incidentally. It is most often asymptomatic but presenting symptoms are nonspecific and include abdominal pain, nausea, diarrhea, malabsorption, bleeding, obstruction, and/or perforation. A retrospective review of medical records between 1999 and 2012 at a tertiary referral center was conducted to identify patients requiring emergency management of complicated jejunal diverticulosis. Complications were defined as those that presented with inflammation, bleeding, obstruction, or perforation. Eighteen patients presented to the emergency department with acute complications of jejunal diverticulosis. Ages ranged from 47 to 86 years (mean, 72 years). Seven patients presented with evidence of free bowel perforation. Six had either diverticulitis or a contained perforation. The remaining five were found to have gastrointestinal bleeding. Fourteen of the patients underwent surgical management. Four patients were successfully managed nonoperatively. As a result of the variety of presentations, complications of jejunal diverticulosis present a diagnostic and therapeutic challenge for the acute care surgeon. Although nonoperative management can be successful, most patients should undergo surgical intervention. Traditional management dictates laparotomy and segmental jejunal resection. Diverticulectomy is not recommended as a result of the risk of staple line breakdown. The entire involved portion of jejunum should be resected when bowel length permits.

Tailgut cysts are uncommon lesions that usually occur within the presacral space. The relative rarity and nonspecific complaints associated with these lesions often lead to misdiagnosis or unnecessary procedures before the correct diagnosis is made. We describe a case of a 16-year-old female who presented with pelvic pain. She had previously undergone several procedures at an outside institution for recurrent perianal fistula and perirectal abscess. Subsequent evaluation under anesthesia revealed a presacral cystic mass with a well-developed tract within the anorectal ring in the posterior midline. This mass was surgically removed using a combined transanal and posterior sagittal excision technique and was found to be a tailgut cyst upon pathologic evaluation. Tailgut cysts and other presacral masses should be included in the differential for patients with recurrent abscess in the presacral space or fistula within the anal canal. A variety of surgical approaches are available depending on the anatomy of the lesion.

Management of inflammatory bowel disease in children is complex with multiple treatment modalities, including surgery. The use of laparoscopic surgery in the treatment of these disease processes has many advantages over standard laparotomy incisions, including faster recovery and making future operations safer and less complex due to less scarring. Described here are the evaluation, operative techniques, and outcomes related to the use of laparoscopy in the treatment of inflammatory bowel disease in the pediatric population.

Hypertrophic pyloric stenosis is a commonly encountered pediatric surgical issue. Initially treated with open surgical techniques, many pediatric surgeons have adopted the minimally invasive approach using laparoscopy. However, some concerns exist that the rate of incomplete pyloromyotomy is elevated in laparoscopy. We propose a new technique to assess the adequacy of laparoscopic pyloromyotomy.Adequacy of laparoscopic pyloromyotomy was assessed by confirming that the top of the serosa on one side of the pylorus has adequate freedom to reach the bottom of the muscle on other side. A retrospective review of patients undergoing laparoscopic pyloromyotomy confirmed by this method from March 2012 to January 2016 was conducted. Demographics, laboratory values on admission, and postoperative outcomes were collected. Descriptive statistics was utilized.Thirty-three patients were included. Median age was 30 days (interquartile range [IQR]: 24, 47). Median pylorus length and thickness were 19 mm (IQR 17.3, 21) and 4.5 mm (IQR: 4.0, 4.8), respectively. Median time to first full feed was 8.5 hours (IQR: 6.6, 15.6). Twenty-three (69%) patients had postoperative emesis. Median length of stay postoperation was 26.5 hours (IQR: 21.1, 44.7). There were no reoperations for incomplete pyloromyotomy and no infections. On follow-up, 1 patient had prolonged postoperative emesis that resolved without further intervention and 1 patient on peritoneal dialysis before surgery had an incisional hernia that required operation in the setting of bilateral inguinal hernias.In a small series, the top to bottom assessment appears to confirm adequacy of pyloromyotomy.

BACKGROUND Acute small bowel obstruction (SBO) is a common condition encountered by the on-call emergency surgeon. The role of laparoscopy in the management of SBO continues to be defined. This modality can be limited by dilated bowel and inadequate assessment of compromised tissue. This review was undertaken to determine the reliability of laparoscopic evaluation and the subsequent need for bowel resection. METHODS A retrospective review of all patients surgically managed for acute SBO between July 2005 and September 2010 was conducted. The clinical presentation, computed tomographic findings, indications for surgery, type of intervention, need for reoperation, length of stay (LOS), and outcomes were all abstracted. RESULTS A total of 119 patients were surgically managed for acute SBO during this period, 63 with initial laparoscopy and 56 with an open procedure. Twenty-five (40%) of the laparoscopy patients were converted to open, leaving 38 completed laparoscopically. Of the completed group, three patients underwent bowel resection compared with 16 in the converted group (8% vs. 64%, p < 0.0001). No patients in the completed group required a subsequent procedure for bowel resection. Twenty-three (41%) patients in the open cohort required a resection. LOS was significantly reduced in the completed group (7.7 days) compared with the converted (11.0 days, p = 0.01) and open groups (11.4 days, p = 0.002). CONCLUSIONS Overall, 32% of acute SBOs were managed solely with laparoscopy. No patients requiring a bowel resection were missed using this method of evaluation. Laparoscopic management should be considered as safe and effective initial therapy in most cases of acute SBO.

Minimally invasive repair of pectus excavatum (MIRPE) is performed by placement of substernal metal bars. Visualization across the mediastinum is compromised in severe defects, and cardiac puncture and aortic injury have been described. In adults with less chest wall flexibility, the bars require more force to rotate and intercostal muscle stripping may result, leading to bar malpositioning. We present a technique to elevate the sternum and facilitate adult MIRPE.

Background Although fetal ultrasound, fetal MRI and postnatal CT are now widely used in the evaluation of congenital lung malformations (CLM), their diagnostic accuracy remains undefined. Objective To correlate prenatal and postnatal imaging studies with pathological data after CLM resection. Design Retrospective, descriptive case series study. Setting A North American tertiary care centre. Patients One hundred and three consecutive lung resections for a suspected CLM between 1 January 2005 and 31 December 2015. Main outcome measures Diagnostic accuracy of imaging diagnosis compared with pathological evaluation. Results Pathological diagnoses included congenital pulmonary airway malformation ((CPAM) n=45, 44%), bronchopulmonary sequestration (BPS; n=25, 24%), CPAM/BPS hybrid lesions (n=22, 21%) and pleuropulmonary blastoma (n=2, 2%). Fetal ultrasound detected 85 (82.5%) lesions and correctly diagnosed whether or not a lesion was a CPAM in 75% of cases (sensitivity 93%, specificity 32%). Fetal MRI had a similar concordance rate (73%) but was superior in correctly determining whether a systemic feeding vessel was present in 80% of cases (sensitivity 71%, specificity 88%) compared with an ultrasound accuracy rate of 72% (sensitivity 49%, specificity 93%). By comparison, postnatal CT correctly diagnosed whether a CPAM was present in 84% of cases (sensitivity 86%, specificity 77%) and whether a systemic feeding vessel was present in 90% of cases (sensitivity 92%, specificity 88%). Conclusions Fetal ultrasound remains an important tool in the detection and evaluation of congenital lung malformations. However, it does not correctly predict histology in approximately 25% of prenatally detected CLMs and remains limited by relatively poor sensitivity for systemic feeding vessels pathognomic for a bronchopulmonary sequestration. These data suggest the importance of obtaining additional cross-sectional imaging, preferably a postnatal CT scan, in all patients to help counsel families and to guide in the optimal management of these lesions.

The aim of this study is to compare liver function and cholangitis episodes during the first year postoperatively between patients who undergo hepatic portocholecystostomy (HPC) and patients who undergo hepatic portoenterostomy (HPE). Records of six patients who underwent HPC for biliary atresia (BA) and 27 patients who underwent HPE for BA were reviewed retrospectively Comparison was done of the patient's total bilirubin, albumin, and international normalized ratio values preoperatively and at 3 months, 6 months, and 1 year postoperatively. Comparison was also done of the occurrence of ascending cholangitis during the first year postoperatively and in rates of transplant and mortality during long-term follow-up. Preoperative laboratory values between the two groups were not significantly different. At 6 months, the patients who underwent HPC had significantly lower total bilirubin levels compared with those who underwent HPE (HPC 0.8 ± 0.96, n = 4; HPE 4.93 ± 7.73, n = 21; P < 0.05). No other laboratory values or rates of ascending cholangitis, transplant, or mortality showed a significant difference. Those patients who underwent HPC had significantly lower total bilirubin levels at 6 months postoperatively. This may suggest that HPC may be a superior operative technique for patients who are candidates for the operation.

Inflammatory myofibroblastic tumors are rare tumors characterized as low-to-intermediate grade sarcomas. This is a case of a 7-year-old male with a 5-cm lung mass, which recurred 11 months after complete resection. The recurrence manifested as multifocal metastatic disease involving the ipsilateral parietal and visceral pleura. A novel chemotherapeutic regimen, which included vincristine, ifosfamide, doxorubicin, and celecoxib was utilized for the disease recurrence. The patient had complete and durable remission of the disease and has been disease-free for >4 years. This novel regimen including a cyclooxygenase 2 inhibitor may be an effective regimen for metastatic inflammatory myofibroblastic tumors.

Background Successful repair of recurrent pectus excavatum (PE) after failed open procedure has been reported using minimally invasive repair (MIRPE) and open approaches. Neither approach alone may be adequate for some patients. A hybrid technique for repair is presented for revision of recurrent PE. Methods A retrospective review of adults undergoing repair for recurrent PE after prior open repair from January 2010 to June 2014 was performed. Results Seventy-three adult patients underwent repair for recurrent PE, with 48 patients (65.8%) undergoing repair for recurrence after at least one prior open PE repair. Mean patient age was 34.5 years (range, 19 to 54 years); mean Haller index was 4.7 (range, 2.8 to 14.7). Fourteen (29%) recurrences with adequate chest wall pliability and no malunion were repaired with MIRPE alone; 34 patients (71%) underwent a hybrid procedure for repair (20 for PE recurrence alone; 14 for PE with acquired thoracic dystrophy). All had at least two support bars placed, and 11 patients (23%) had three bars placed. Mean hospitalization for MIRPE was 5 days, for hybrid was 7 days, and for hybrid because of acquired thoracic dystrophy was 10 days. One patient died of unexpected out-of-hospital arrest; there was one emergent conversion to open sternotomy for bleeding. Conclusions Most recurrent PE may be repaired with excellent results and minimal complications. Those with adequate chest pliability and no malunion are candidates for MIPRE alone. A hybrid procedure with thoracoscopic support bars combined with sternal elevation, multiple open osteotomies, and chest wall fixation is appropriate for recurrences associated with malunion or fixation of the anterior chest and failure to lift with MIRPE.

Jejunal diverticulosis is a rare condition that is usually found incidentally. It is most often asymptomatic but presenting symptoms are nonspecific and include abdominal pain, nausea, diarrhea, malabsorption, bleeding, obstruction, and/or perforation. A retrospective review of medical records between 1999 and 2012 at a tertiary referral center was conducted to identify patients requiring emergency management of complicated jejunal diverticulosis. Complications were defined as those that presented with inflammation, bleeding, obstruction, or perforation. Eighteen patients presented to the emergency department with acute complications of jejunal diverticulosis. Ages ranged from 47 to 86 years (mean, 72 years). Seven patients presented with evidence of free bowel perforation. Six had either diverticulitis or a contained perforation. The remaining five were found to have gastrointestinal bleeding. Fourteen of the patients underwent surgical management. Four patients were successfully managed nonoperatively. As a result of the variety of presentations, complications of jejunal diverticulosis present a diagnostic and therapeutic challenge for the acute care surgeon. Although nonoperative management can be successful, most patients should undergo surgical intervention. Traditional management dictates laparotomy and segmental jejunal resection. Diverticulectomy is not recommended as a result of the risk of staple line breakdown. The entire involved portion of jejunum should be resected when bowel length permits.

Background In young children, acquired thoracic dystrophy (ATD) is associated with extensive resection of cartilage, often during open pectus excavatum (PE) repair. Progressive dyspnea or exercise intolerance may develop in these patients secondary to cardiac compression or restrictive pulmonary function. Surgical treatment of ATD by attempting to increase the overall thoracic volume has been controversial. We describe our experience with adults presenting for surgical correction of ATD. Methods A retrospective medical record review was performed for all patients with ATD presenting for surgical evaluation from December 2010 through February 2013. Results Ten adult male patients were evaluated for treatment of ATD after an open Ravitch procedure for PE. Nine patients, whose mean age was 34 years (range, 21-42 years), elected to proceed with surgical treatment. The mean age of the initial repair was 3.7 years. Extensive reconstruction, chest wall expansion, and placement of stainless steel support bars and titanium plating were performed in all patients. Eight patients had minor complications, and major complications occurred in 3 patients. Respiratory failure with prolonged ventilator support occurred in 3 patients. There were no reoperations or deaths. At mean follow-up of 16 months (range, 6-31 months), all patients subjectively reported improvement in their ability to exercise and in their symptoms, including dyspnea with exertion. Conclusions ATD may be associated with early childhood Ravitch repair. Adults may present with disabling symptoms related to cardiac compression and restrictive pulmonary function. Reconstruction with sternal elevation and expansion of the anterior chest subjectively improves symptoms.

Introduction Alcohol use is a risk factor for adult trauma. Alcohol may significantly influence pediatric trauma risk, but literature is sparse. The aim of this study was to examine the impact of alcohol use screening in pediatric trauma patients. Methods A retrospective review was performed of all trauma patients to identify those undergoing CRAFFT alcohol screening assessment between July 1, 2009, and January 31, 2011. Inclusion criteria involved screening of level 1 or 2 trauma activations for patients greater than 12years. Results During the study period, 232 patients were eligible for screening, of which 51% (n=118) were screened. Among the patients screened, 21 (18%) had a positive screen (mean age 14.6years, range 13–16). Twenty patients were referred for further counseling. Sixteen males and 5 females screened positive during the study. The most common mechanism of injury in the positive screen patients was motor vehicle or ATV accident (n=9), followed by assault (n=6), and motor versus pedestrian collision (n=2). Of the 21 patients who screened positive, 10 had positive blood alcohol content (BAC) or urine drug screen (UDS) at the time of injury. No patients with a positive screen returned during the study as a trauma patient. Conclusion Alcohol and drug screening for injured pediatric trauma patients is frequently omitted despite policy-required screening. Of those patients screened, 18% admitted to risky alcohol or drug-related behaviors or had positive BAL or UDS at presentation. Pediatric trauma screening for risky alcohol use identifies a significant number of children. Alcohol and drug screening in pediatric trauma appears over age 13 years to have a yield which justifies continued screening. Alcohol related trauma recidivism, however, does not seem common.

We present a case of life-threatening hemorrhage occurring during Nuss bar removal without obvious cardiac or major vascular injury. A 19-year-old woman with marfanoid features had undergone a Nuss procedure 3 years earlier for a pectus index of 7.2. A lateral chest radiograph revealed erosion of the upper bar into the sternum. During surgery, a 3.5-L blood loss occurred after removal of the eroded bar. This case provided many opportunities to improve preparedness for bar removal.

Background Minimally invasive repair of pectus excavatum has become an established method for repair of pectus excavatum. Bar displacement or rotation remains the most common complication of this repair requiring return to the operating room. Methods Retrospective review of all patients at a single institution who underwent repair of pectus excavatum using FiberWire for bar stabilization between December 2009 and March 2013 was undertaken. Results 93 patients underwent minimally invasive pectus repair using FiberWire during the study period. The patients included 73 males and 20 females, with an average age of 14.6years (range 7–21years). Mean operative time was 102minutes (range 56–198minutes). No patients developed wound complications, two patients developed pain because of bar migration and required return to the OR, and no patients had recurrence of their pectus defect because of bar migration during the study period. Median length of follow-up was 17months (range 3–36months). Conclusion Stabilization of pectus bars using circumferential rib fixation with FiberWire at multiple points on both sides of the bar appears to be effective in preventing bar rotation and displacement, and requires minimal change to the operation as it has been previously described. Early experience shows a low rate of complications.

For more than 50 years, surgeons used traditional open surgical methods to correct pectus excavatum deformities. These techniques have undergone multiple modifications but involve resection of costal cartilages and mobilization of the sternum to an anterior position. Long-term postoperative complications are rarely published. Recurrence with lung herniation presents unique challenges. We report a technique to repair this condition in a patient with massive chest wall defect and residual excavatum deformity after open repair of his excavatum deformity.

Malignant peripheral nerve sheath tumors (MPNST) are a rare form of soft tissue sarcoma with few studies reporting on patient outcomes and prognostic variables.A retrospective review of 175 patients diagnosed with MPNST from 1985 to 2010 was performed. Patient, tumor, and treatment characteristics were evaluated to identify prognostic variables.The median age of our study population was 44 years, and 51% were female. Median tumor size was 6 cm, and 61% of patients had high-grade tumors. Tumors were most commonly located on the extremities (45%), then trunk (34%) and head/neck (19%). The majority of patients underwent surgical resection (95%) and adjuvant treatment with chemotherapy (6%), radiation (42%) or both (22%). Margin status was R0 in 69%, R1 in 2%, R2 in 9%, and unknown in 20%. The local recurrence rate was 22%, and 5- and 10-year disease-specific survival (DSS) were 60% and 45%, respectively. On univariate analysis, no predictors for local recurrence were identified. Tumor size ≥ 5 cm, high tumor grade, tumor location, presence of neurofibromatosis type 1, local recurrence, and adjuvant chemotherapy were all associated with DSS. On multivariate analysis, size ≥ 5 cm [hazard ratio (HR)= 6.1, 95% confidence interval (CI) 1.5-25.0], local recurrence (HR = 4.4, 95% CI 1.7-11.4), high tumor grade (HR = 3.8, 95% CI 1.1-13.2), and truncal location (HR = 3.7, 95% CI 1.1-12.7) were poor prognostic indicators for DSS.High tumor grade and tumor size ≥ 5 cm predict adverse DSS for MPNST. In the context of a multidisciplinary treatment regimen, local recurrence and survival outcomes at 5 and 10 years were better than previously reported for MPNST.

The aim of this study is to compare liver function and cholangitis episodes during the first year postoperatively between patients who undergo hepatic portocholecystostomy (HPC) and patients who undergo hepatic portoenterostomy (HPE). Records of six patients who underwent HPC for biliary atresia (BA) and 27 patients who underwent HPE for BA were reviewed retrospectively. Comparison was done of the patient's total bilirubin, albumin, and international normalized ratio values preoperatively and at 3 months, 6 months, and 1 year postoperatively. Comparison was also done of the occurrence of ascending cholangitis during the first year postoperatively and in rates of transplant and mortality during long-term follow-up. Preoperative laboratory values between the two groups were not significantly different. At 6 months, the patients who underwent HPC had significantly lower total bilirubin levels compared with those who underwent HPE (HPC 0.8 +/- 0.96, n = 4; HPE 4.93 +/- 7.73, n = 21; P0.05). No other laboratory values or rates of ascending cholangitis, transplant, or mortality showed a significant difference. Those patients who underwent HPC had significantly lower total bilirubin levels at 6 months postoperatively. This may suggest that HPC may be a superior operative technique for patients who are candidates for the operation.

Hydrocortisone-21-lysinate was synthesized as an amino acid prodrug of hydrocortisone to serve as a substrate for brush border aminopeptidases. This strategy was developed to demonstrate that an improvement in oral absorption could be obtained through reconversion in vivo. The aqueous stability of hydrocortisone-21-lysinate was studied over the pH range 3-8 at 25 degrees C. Reversible acyl migration of the lysine group between the 21- and 17-position hydroxyl groups was observed as well as hydrolysis. The observed half-life for direct hydrolysis of hydrocortisone-21-lysinate is 40 d at pH 3 and 30 min at pH 7. The relative instability at pH 7 is probably due to electrostatic stabilization of the negatively charged tetrahedral intermediate by the protonated amino groups.