Evaluating the risk of peri-umbilical hernia after sutured or sutureless gastroschisis closure.

INTRODUCTION: We evaluate the incidence, outcomes, and management of peri­umbilical hernias after sutured or sutureless gastroschisis closure. METHODS: 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 peri­umbilical hernia, time to spontaneous closure or repair, and associated complications. RESULTS: 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%, p< 0.001). 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%, p< 0.001). 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. CONCLUSION: 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. LEVELS OF EVIDENCE: Level II.

Management of Hirschsprung associated enterocolitis-How different are practice strategies? An international pediatric endosurgery group (IPEG) survey.

INTRODUCTION: Hirschsprung's-associated enterocolitis (HAEC) is a common post-operative problem for patients with Hirschsprung disease (HSCR). However, treatment strategies remain variable among providers, institutions, and even nations. The purpose of this study was to identify differences in treatment patterns for HAEC. METHODS: A questionnaire was distributed to members of the International Pediatric Endoscopic Group (IPEG) community that focused on HSCR and HAEC management strategies. Questionnaire responses were collected via the Research Electronic Data Capture (RedCap). RESULTS: 178 responses were obtained: 30% from North America, 20% South America, 20% Europe, 26% Asia, and 4% from Australia. 37% had a dedicated pediatric colorectal center. After diagnosis, 53% send patients home with irrigations, while 29% perform a primary PT before discharge; the type of PT varied between Soave (50%), Swenson (25%) and Duhamel (13%). Only 29 respondents (17%) stated their institution had guidelines for HAEC management; however, inpatient treatments were fairly consistent: 95% performed rectal irrigations, 93% obtained an abdominal radiograph, and 72% held feeds; 55% taught families irrigations before discharge. Utilization of Botulinum (BT) injections was mixed: 36% never utilized BT injections, 33% only used BT if irrigations were not tolerated, and 16% only injected BT for recurrent episodes. Preventative HAEC measures were also varied and included anal dilations (44%), prophylactic antibiotics (34%), probiotics (29%), and routine home irrigations (22%). CONCLUSION: There is wide variation of care in managing enterocolitis episodes in patients with Hirschsprung disease. Further research leading to consensus guidelines and standardization practices can help improve the care for these patients. LEVEL OF EVIDENCE: V TYPE OF STUDY: Treatment study/ survey.

Fetal Risk Stratification and Outcomes in Children with Prenatally Diagnosed Lung Malformations: Results from a Multi-Institutional Research Collaborative.

OBJECTIVE: The aim of this study was to assess current clinical outcomes in children with prenatally diagnosed congenital lung malformations (CLMs) and to identify prenatal characteristics associated with adverse outcomes. SUMMARY BACKGROUND DATA: Despite a wide spectrum of clinical disease, the identification of fetal CLM subgroups at increased risk for hydrops and respiratory compromise at delivery has not been well defined. METHODS: A retrospective cohort study was conducted using an operative database of prenatally diagnosed CLMs managed at 11 children's hospitals from 2009 to 2016. Statistical analyses were performed using nonparametric bivariate or multivariable logistic regression. RESULTS: Three hundred forty-four children were analyzed. Fifteen (5.5%) fetuses were managed with maternal steroids in the setting of hydrops, and prenatal surgical intervention was uncommon (1.7%). Seventy-five (21.8%) had respiratory symptoms at birth, and 34 (10.0%) required neonatal lung resection. Congenital pulmonary airway malformation volume ratio (CVR) measurements were recorded in 169 (49.1%) cases and were significantly associated with perinatal outcome, including hydrops, respiratory distress at birth, need for supplemental oxygen, neonatal ventilator use, and neonatal resection ( P < 0.001). An initial CVR ≤1.4 was significantly correlated with a reduced risk for hydrops [area under the curve (AUC), 0.93; 95% confidence interval (CI), 0.87-1.00]. A maximum CVR <0.9 (AUC, 0.72; 95% CI, 0.67-0.85) was associated with a low risk for respiratory symptoms at birth. CONCLUSIONS: In this large, multi-institutional study, an initial CVR ≤ 1.4 identifies fetuses at very low risk for hydrops, and a maximum CVR < 0.9 is associated with asymptomatic disease at birth. These findings represent an opportunity for standardization and quality improvement for prenatal counseling and delivery planning.

Thoracoscopic versus open lobectomy in infants with congenital lung malformations: A multi-institutional propensity score analysis.

PURPOSE: The impact of thoracoscopic surgery on outcomes in children with congenital lung malformations (CLM) remains controversial. The purpose of this study was to determine the effect of operative approach on perioperative outcomes in infants undergoing lobectomy for an asymptomatic CLM. METHODS: After IRB approval, a retrospective cohort study was conducted on 506 children with a CLM resected at one of eleven children's hospitals over a seven-year period. Infants undergoing elective lobectomy were identified, and covariates were balanced based on operative approach using propensity scores with full matching. Outcomes were analyzed based on intention to treat with weighted conditional regression. RESULTS: One hundred seventy-five infants met inclusion criteria. There were 67 (38.3%) open, 89 (50.9%) thoracoscopic, and 19 (10.9%) thoracoscopic-converted-to-open lobectomies. Thoracoscopic lobectomy was associated with significantly longer operative times (26 min, 95% CI 6-47 min, p = 0.012) but used less epidural anesthesia (OR 0.02, 95% CI 0.004-0.11, p<0.001) when compared to open lobectomy. There were no significant differences in intraoperative blood loss, postoperative complications, chest tube duration, or length of stay. CONCLUSIONS: Thoracoscopy has become the most common operative approach for elective lobectomy in infants with asymptomatic CLMs. The non-inferiority of thoracoscopic lobectomy in postoperative outcomes supports its continued use as an alternative to open lobectomy. LEVEL OF EVIDENCE: Treatment study, Level III.

Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD. METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed. RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder. CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

Ethics of randomized trials in pediatric surgery.

To provide the best evidence-based treatment for children, and timely evaluation of innovations, the role of the pediatric surgeon's participation in randomized controlled trials (RCTs) and prospective comparative studies is required. The ethical considerations of pediatric surgical RCTs pose unique practical difficulties in the design and performance of clinical trials in children. There are several ethical issues unique to pediatric surgical RCTs: diseases with low volume, an inability to conduct Phase 1 and 2 trials, parental emotional involvement, difficulty with recruitment in surgical trials, volume, and modified study design, issues with permission vs. assent and investigator bias. This article reviews the ethical aspects unique to pediatric surgical RCTs and prospective comparative studies.Level of evidence: Level 3.

Epidural versus PCA Pain Management after Pectus Excavatum Repair: A Multi-Institutional Prospective Randomized Trial.

INTRODUCTION: Postoperative pain control remains the primary reason for inpatient stay after minimally invasive repair of pectus excavatum. In a previous study, our group reported that early pain control was better in patients managed with a thoracic epidural, while late pain control was better in patients managed with patient-controlled analgesia (PCA). After revising our epidural transition and modifying the PCA protocol, we conducted a multi-institutional prospective randomized trial to evaluate these two pain control strategies. MATERIALS AND METHODS: Patients were randomized to epidural or PCA following minimally invasive repair of pectus excavatum with standard protocols for each arm. Primary outcome was length of stay with secondary variables including mean patient pain scores, complications, and parental satisfaction. Scores were pooled for the two groups and reported as means with standard deviation. Results were compared using t-tests and one-way analysis of variance with p-value < 0.05 determining significance. RESULTS: Sixty-five patients were enrolled, 32 epidural and 33 PCA. Enrollment was stopped early when we developed an alternative strategy for controlling these patients' pain. There was no difference in length of stay in hours between the two arms; epidural 111.3 ± 18.5 versus PCA 111.4 ± 51.4, p = 0.98. Longer operative time was found in the epidural group. Nine patients in the epidural group (28%) required a PCA in addition to epidural for adequate pain control. Mean pain scores were lower on postoperative day 0 in the epidural group compared with the PCA groups, but were otherwise similar. CONCLUSION: In our prospective randomized trial, PCA is just as effective as thoracic epidural in decreasing early postoperative pain scores after minimally invasive repair of pectus excavatum.

Repetitive Imaging following Minimally Invasive Repair of Pectus Excavatum Is Unnecessary.

INTRODUCTION: The objective of this study was to evaluate the necessity of repeat imaging after an initial chest radiograph (CXR) following minimally invasive repair of pectus excavatum (MIRPE). MATERIALS AND METHODS: A retrospective review was performed on patients who underwent MIRPE from January 2012 to July 2016 at two academic children's hospitals. Data collected included demographics, severity of pectus defect (Haller index [HI]), utilization of CXRs, outpatient follow-up, and clinical outcomes. RESULTS: A total of 360 patients (171 at Hospital 1 and 189 at Hospital 2) underwent MIRPE. Median age was 15.6 years and 84% were males. The median HI was 4.0. Median postoperative hospital length of stay was 4.2 days and median time to bar removal was 34 months. There was significant variation in postoperative imaging between the hospitals, including frequency of immediate postoperative CXR, total number of CXRs during hospitalization, and number of postoperative outpatient CXRs prior to bar removal. However, there was no significant difference in outcomes between the hospitals, including postoperative pneumothorax, postoperative chest tube placement, and complications. CONCLUSION: These data suggest that increased repetitive imaging after an initial postoperative CXR does not affect clinical outcomes and may not be necessary after MIRPE.

Non-accidental trauma: A national survey on management.

INTRODUCTION: Non-accidental trauma (NAT) has significant societal and health care implications. Standardized care has been shown to improve outcomes. The purpose of our study was to survey trauma centers and elucidate the continued variable management of NAT. METHODS: After institutional review board approval, an email survey was sent to Level 1 and 2 ACS verified trauma centers along with general and pediatric surgery training programs. Trauma hospital characteristics and NAT management were analyzed. RESULTS: A total of 493 emails were sent and 91 responses (18%) were received. There were 74 (81%) pediatric surgeons who responded and 15(17%) adult general surgeons. The most common location of respondents were children's hospitals within academic/community hospitals (58%) followed by stand-alone children hospitals (42%), and adult only hospitals (9%). 51 (57%) providers reported using a screening tool; most commonly used by the emergency department (52%). 75% of providers reported utilizing management protocols in which 71% were initiated by trauma surgery. The most common consulting and admitting service for NAT was trauma surgery (86% and 84%). When comparing stand-alone and affiliated children hospitals, there was no difference in the use of a screening tool (54% vs. 59%; p = 0.84), and management protocol (70% vs. 85%; p = 0.19). However, those providers from pediatric trauma centers used a management protocol more often than providers from adult trauma centers (78% vs. 38%; p = 0.04). No providers from adult trauma centers had intentions to initiate a management protocol in the future. CONCLUSION: Screening and management of non-accidental trauma continues to vary across the country. Future studies focusing on standardization and outreach/education to adult trauma centers is warranted.

Simple diverticulectomy is adequate for management of bleeding Meckel diverticulum.

PURPOSE: It is unclear whether simple diverticulectomy, rather than segmental bowel resection (SBR), is adequate treatment for gastrointestinal bleeding (GIB) secondary to Meckel diverticulum (MD). There is concern that ulcers in the adjacent bowel may continue to bleed if only the diverticulum is removed. This study seeks to determine if diverticulectomy is satisfactory treatment for bleeding MD. METHODS: A multi-institution, retrospective review was performed for patients with a diagnosis of MD and GIB who underwent simple diverticulectomy or small bowel resection. Exclusion criteria were comorbid surgical conditions and other causes of GIB. The primary outcome was post-operative bleeding during the initial hospitalization. Secondary outcomes were bleeding after discharge, transfusion or additional procedure requirement, re-admission, and overall complications. RESULTS: There were 59 patients who met study criteria (42 diverticulectomy, 17 SBR). One patient in the SBR group had early post-operative bleeding (p = 0.288). There was one re-admission (p = 0.288) and three total complications in the SBR group (p = 0.021). There were no cases of bleeding or other complications in the diverticulectomy group. CONCLUSION: This study suggests that simple diverticulectomy is adequate for treatment of GIB caused by MD. Furthermore, diverticulectomy appears to have a lower overall complication rate.

The impact of morbid obesity on solid organ injury in children using the ATOMAC protocol at a pediatric level I trauma center.

INTRODUCTION: Obesity is an epidemic in the pediatric population. Childhood obesity in trauma has been associated with increased incidence of long-bone fractures, longer ICU stays, and decreased closed head injuries. We investigated for differences in the likelihood of failure of non-operative management (NOM), and injury grade using a subset of a multi-institutional, prospective database of pediatric patients with solid organ injury (SOI). METHODS: We prospectively collected data on all pediatric patients (<18years) admitted for liver or splenic injury from September 2013 to January 2016. SOI was managed based upon the ATOMAC protocol. Obesity status was derived using CDC definitions; patients were categorized as non-obese (BMI <95th percentile) or obese (BMI ≥95th percentile). The ISS, injury grade, and NOM failure rate were calculated among other data points. RESULTS: Of 1012 patients enrolled, 117 were identified as having data regarding BMI. Eighty-four percent of patients were non-obese; 16% were obese. The groups did not differ by age, sex, mechanism of injury, or associated injuries. There was no significant difference in the rate of failure of non-operative management (8.2% versus 5.3%). Obesity was associated with higher likelihood of severe (grade 4 or 5) hepatic injury (36.8% versus 15.3%, P=0.048) but not a significant difference in likelihood of severe (grade 4 or 5) splenic injury (15.3% versus 10.5%, P=0.736). Obese patients had a higher mean ISS (22.5 versus 16.1, P=0.021) and mean abdominal AIS (3.5 versus 2.9, P=0.024). CONCLUSION: Obesity is a risk factor for more severe abdominal injury, specifically liver injury, but without an associated increase in failure of NOM. This may be explained by the presence of hepatic steatosis making the liver more vulnerable to injury. A protocol based upon physiologic parameters was associated with a low rate of failure regardless of the pediatric obesity status. LEVEL OF EVIDENCE: Level II prognosis.

Pediatric appendicitis: state of the art review.

Appendicitis is a common cause of abdominal pain in children. The diagnosis and treatment of the disease have undergone major changes in the past two decades, primarily as a result of the application of an evidence-based approach. Data from several randomized controlled trials, large database studies, and meta-analyses have fundamentally affected patient care. The best diagnostic approach is a standardized clinical pathway with a scoring system and selective imaging. Non-operative management of simple appendicitis is a reasonable option in selected cases, with the caveat that data in children remain limited. A minimally invasive (laparoscopic) appendectomy is the current standard in US and European children's hospitals. This article reviews the current 'state of the art' in the evaluation and management of pediatric appendicitis.