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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.

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.

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.

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.

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.

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.

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.