The structural connectome in children: basic concepts, how to build it, and synopsis of challenges for the developing pediatric brain.

PURPOSE: The structural connectome is a comprehensive structural description of the network of elements and connections forming the brain. In recent years, this framework has progressively been used to investigate the pediatric brain. METHODS: We discuss the different steps and emphasize key technical aspects required for the successful reconstruction, analysis, and visualization of the pediatric structural connectome using current state-of-the-art neuroimaging and post-processing techniques. RESULTS: The two key components of structural connectome are a node (a cortical region obtained with high-resolution anatomical imaging) and an edge (structural association between cortical regions, defined with tractography). After delineation of nodes and edges, an association matrix can be generated by compiling all pairwise associations between nodes and applying a threshold to produce a binary adjacency matrix. Several measures can be used to characterize the topological architecture of the brain's networks. Finally, we provide an overview of various visualization methods of the structural connectome in children. CONCLUSION: The human connectome is the culmination of more than a century of conceptual and methodological innovation. Biological substrates of brain development such as cortical gyration and myelination challenge the acquisition, reconstruction, and analysis of structural connectome in children and require specific considerations compared to adults.

The impact of surgical strategies on outcomes for pediatric chronic pancreatitis.

PURPOSE: To review our institutional experience in the surgical treatment of pediatric chronic pancreatitis (CP) and evaluate predictors of long-term pain relief. METHODS: Outcomes of patients ≤21 years surgically treated for CP in a single institution from 1995 to 2014 were evaluated. RESULTS: Twenty patients underwent surgery for CP at a median of 16.6 years (IQR 10.7-20.6 years). The most common etiology was pancreas divisum (n = 7; 35%). Therapeutic endoscopy was the first-line treatment in 17 cases (85%). Surgical procedures included: longitudinal pancreaticojejunostomy (n = 4, 20%), pancreatectomy (n = 9, 45%), total pancreatectomy with islet autotransplantation (n = 2; 10%), sphincteroplasty (n = 2, 10%) and pseudocyst drainage (n = 3, 15%). At a median follow-up of 5.3 years (IQR 4.2-5.3), twelve patients (63.2%) were pain free and five (26.3%) were insulin dependent. In univariate analysis, previous surgical procedure or >5 endoscopic treatments were associated with a lower likelihood of pain relief (OR 0.06; 95% CI 0.006-0.57; OR 0.07; 95%, CI 0.01-0.89). However, these associations were not present in multivariate analysis. CONCLUSION: In children with CP, the step-up practice including a limited trial of endoscopic interventions followed by surgery tailored to anatomical abnormalities and gene mutation status is effective in ensuring long-term pain relief and preserving pancreatic function.

Laparoscopic versus open inguinal hernia repair in children ≤3: a randomized controlled trial.

PURPOSE: Laparoscopy is being increasingly applied to pediatric inguinal hernia repair. In younger children, however, open repair remains preferred due to concerns related to anesthesia and technical challenges. We sought to assess outcomes after laparoscopic and open inguinal hernia repair in children less than or equal to 3 years. METHODS: A prospective, single-blind, parallel group randomized controlled trial was conducted at three clinical sites. Children ≤3 years of age with reducible unilateral or bilateral inguinal hernias were randomized to laparoscopic herniorrhaphy (LH) or open herniorrhaphy (OH). The primary outcome was the number of acetaminophen doses. Secondary outcomes included operative time, complications, and parent/caregiver satisfaction scores. RESULTS: Forty-one patients were randomized to unilateral OH (n = 10), unilateral LH (n = 17), bilateral OH (n = 5) and bilateral LH (n = 9). Acetaminophen doses, LOS, complications, and parent/caregiver scores did not differ among groups. Laparoscopic unilateral hernia repair demonstrated shorter operative time, a consistent finding for overall laparoscopic repair in univariate (p = 0.003) and multivariate (p = 0.010) analysis. No cases of testicular atrophy were documented at 2 (SD = 2.7) years. CONCLUSION: Children ≤3 years of age in our cohort safely underwent LH with similar pain scores, complications, and recurrence as OH. Parents and caregivers report high satisfaction with both techniques.

Pancreatic surgery for tumors in children and adolescents.

PURPOSE: Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies. METHODS: We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent surgery at a single institution between 1995 and 2015. RESULTS: Nineteen patients were identified with a median age at operation of 16.6 years (IQR 13.5-18.9). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 13), followed by pancreatic neuroendocrine tumor (n = 3), serous cystadenoma (n = 2) and pancreatoblastoma (n = 1). Operative procedures included formal pancreatectomy (n = 17), enucleation (n = 1) and central pancreatectomy (n = 1). SPNs were noninvasive in all but one case with perineural, vascular and lymph node involvement. Seventeen patients (89.5 %) are currently alive and disease free at a median follow-up of 5.7 (IQR 3.7-10.9) years. Two patients died: one with metastatic insulinoma and another with SPN who developed peritoneal carcinomatosis secondary to a concurrent rectal adenocarcinoma. CONCLUSIONS: Pediatric pancreatic tumors are a heterogeneous group of neoplastic lesions for which surgery can be curative. SPN is the most common histology, is characterized by low malignant potential and in selected cases can be safely and effectively treated with a tissue-sparing resection and minimally invasive approach.

Safety of outpatient laparoscopic cholecystectomy in children: analysis of 2050 elective ACS NSQIP-pediatric cases.

PURPOSE: Limited data exists evaluating the extent of utilization and safety of outpatient laparoscopic cholecystectomy (LC) in children. The aim of this study was to investigate the safety of outpatient LC in the pediatric population utilizing a national surgical quality improvement database. METHODS: The National Surgical Quality Improvement Program-Pediatric (NSQIP-P) databases from 2012 and 2013 were queried to identify pediatric patients who underwent elective LC. Patients who underwent outpatient LC were compared with those who underwent inpatient LC. Outcomes of interest included 30-day overall morbidity, readmission, and reoperation. RESULTS: A total of 2,050 LC were identified, 995 (48.5 %) were performed as an outpatient procedure and 1055 (51.5 %) as inpatient. Patients who underwent outpatient LC were more often white (79.6 vs. 69.2 %; p = <0.0001). Choledocholithiasis was more often treated in inpatient setting (12.5 vs. 1.7 %; p < 0.0001), while biliary dyskinesia was performed in outpatient setting (26.1 v. 12.6 %; p = 0.0001). Overall 30-day morbidity was greater in the inpatient group (2.5 vs. 0.8 %; p = 0.03). There were no differences in term of 30-day readmission rate and related reoperations (0.9 vs 0.3 % respectively; p = 0.09). CONCLUSION: This analysis of a large multicenter dataset demonstrates that pediatric patients without significant associated comorbidities can safely undergo laparoscopic cholecystectomy as an outpatient procedure.

Patient Satisfaction After Minimally Invasive Repair of Pectus Excavatum in Adults: Long-Term Results of Nuss Procedure in Adults.

BACKGROUND: Extensive literature has proved that the Nuss procedure leads to permanent remodeling of the chest wall in pediatric patients with pectus excavatum (PE). However, limited long-term follow-up data are available for adults. Herein, we report a single-institution experience in the management of adult PE with the Nuss procedure, evaluating long-term outcomes and overall patient satisfaction after bar removal. METHODS: Adult patients who underwent PE repair with a modified Nuss procedure between January 1998 and June 2011 were retrospectively identified. Outcomes of interest were postoperative pain, recurrence, and patient satisfaction. A modified single-step Nuss questionnaire was administered to evaluate patient satisfaction and quality-of-life improvement after PE repair. RESULTS: Ninety-eight patients with a median age of 30.9 years (range, 21.8 to 55.1 years) at the time of repair were identified. One bar was placed in most patients (89.7%). Four patients (4.1%) required reoperation for bar displacement. Results after bar removal were overall satisfactory in 94.4% of patients; 2 patients required reoperation for recurrence. Thirty-nine patients participated in the survey. Satisfaction with chest appearance was reported by 89.7% of responders. Seven patients reported dissatisfaction with the overall results; the most common complaints were severe postoperative chest pain and dissatisfaction with surgical scars. CONCLUSIONS: Favorable long-term results can be achieved with the Nuss procedure in adults. However, postoperative pain may require a more aggressive analgesic regimen, and it may be the overriding factor in the patient's perception of the quality of the postoperative course.

The impact of obesity on laparoscopic appendectomy: Results from the ACS National Surgical Quality Improvement Program pediatric database.

BACKGROUND: Childhood obesity is a worsening epidemic. Little is known about the impact of elevated BMI on perioperative and postoperative complications in children who undergo laparoscopic surgery. The purpose of this study was to examine the effects of obesity on surgical outcomes in children using laparoscopic appendectomy as a model for the broader field of laparoscopic surgery. STUDY DESIGN: Using the Pediatric National Surgical Quality Improvement Program (NSQIP) data from 2012, patients aged 2-18years old with acute uncomplicated and complicated appendicitis who underwent laparoscopic appendectomy were identified. Children with a body mass index (BMI)≥95th percentile for their age and gender were considered obese. Primary outcomes, including overall morbidity and wound complications, were compared between nonobese and obese children. Multivariate regression analysis was conducted to identify the impact of obesity on outcome. RESULTS: A total of 2812 children with acute appendicitis who underwent appendectomy were included in the analysis; 22% were obese. Obese children had longer operative times but did not suffer increased postoperative complications when controlling for confounders (OR 1.3, 95% CI: 0.83-0.072 for overall complications, OR 1.3, 95% CI: 0.84-1.95 for wound complications). CONCLUSIONS: Obesity is not an independent risk factor for postoperative complications following laparoscopic appendectomy. Although operative times are increased in obese children, obesity does not increase the likelihood of 30-day postoperative complications.

Contemporary management of recurrent pectus excavatum.

BACKGROUND: Optimal management of recurrent pectus excavatum (PE) has not been established. Here, we review our institutional experience in managing recurrent PE to evaluate long-term outcomes and propose an anatomic classification of recurrences, and a decision-making algorithm. METHODS: Clinical records of patients undergoing repair of recurrent PE (1996-2011) were reviewed. Univariate and multivariate logistic regression analyses were employed to examine patient characteristics as potential predictors for re-recurrence. RESULTS: Eighty-five patients with recurrent PE were identified during the study period. The initial operation was a Ravitch procedure in 85% of cases. Revision procedures were most frequently Nuss repairs (N=73, 86%), with remaining cases managed via open approach. Overall cosmetic and functional results were satisfactory in 67 patients (91.8%) managed with Nuss and in 7 (58%) patients managed with other techniques. Seven (8%) patients required additional surgical revision. Multivariate analysis identified no statistically significant patient or procedural factors predictive of re-recurrence. CONCLUSION: This study demonstrates that the Nuss procedure can be an effective intervention for recurrent pectus excavatum, regardless of the initial repair technique. However, open repair remains valuable when managing severe cases with abnormalities of the sternocostal junction and cartilage regrowth under the sternum.

Z-type pattern pectus excavatum/carinatum in a case of Noonan syndrome.

Noonan syndrome is a genetic condition that can present with complex thoracic defects, the management of which often presents a surgical challenge. We present the surgical approach applied to a severe combined excavatum/carinatum deformity that had resulted in a Z-type configuration of the chest in a 9-year-old girl with Noonan syndrome.

Laparoscopic versus open appendectomy in children: the effect of surgical technique on healthcare costs.

BACKGROUND: Reducing healthcare costs while maintaining quality of care is one of the challenges of the current healthcare system. The purpose of this study was to compare the hospital charges accrued following laparoscopic (LA) and open (OA) appendectomies in the pediatric population. METHODS: We retrospectively reviewed all pediatric appendectomies (n = 264) performed from 2007 to 2013 at a single academic center. Subgroup analysis on charges and costs was performed on perforated and nonperforated LA and OA. RESULTS: A total of 195 (73.9%) appendectomies were performed laparoscopically. LA in both perforated and nonperforated groups was associated with higher surgical supply, operating room, and total hospital charges compared with OA. Surgical supply costs to the facility were higher by an average of $1,000 for both nonperforated and perforated appendicitis in the LA group. Length of stay and postoperative complications were comparable within all groups. CONCLUSIONS: In this study, LA is associated with significantly higher surgical costs and charges than OA without improvement in outcomes. Investigation into cost reduction strategies of laparoscopy should be a component of future clinical appendicitis research.

Minimally invasive repair of pectus excavatum: analyzing contemporary practice in 50 ACS NSQIP-pediatric institutions.

BACKGROUND: Minimally invasive repair of pectus excavatum (MIRPE) is a well-established procedure. However, morbidity rate varies widely among institutions, and the incidence of major complications remains unknown. STUDY DESIGN: The American College of Surgeons 2012 National Surgical Quality Improvement Program-Pediatric (NSQIP-P) participant user file was utilized to identify patients who underwent MIRPE at 50 participant institutions. Outcomes of interest were overall 30-day morbidity, hospital readmission, and reoperation. RESULTS: Chest wall repair designated MIRPE accounted for 0.6% (n = 264) of all surgical cases included in the NSQIP-P database in 2012. The median age at surgical repair was 15.2 years. Thoracoscopy was used in 83.7% of cases. No mediastinal injuries or perioperative blood transfusions were identified. The 30-day readmission rate was 3.8%. Three patients (1.1%) required re-operation due to the following complications: superficial site infection, bar displacement and pneumothorax. The overall morbidity was 3.8% with no incidences of mortality. CONCLUSIONS: This analysis of a large prospective multicenter dataset demonstrates that major complications following MIRPE are uncommon in contemporary practice. Wound infection is the most common complication and the main cause of hospital readmission. Targeted quality improvement initiative should be focused on perioperative strategy to further reduce wound occurrences and hospital readmission.

Diagnostic imaging for acute appendicitis: interfacility differences in practice patterns.

PURPOSE: To evaluate trends and factors associated with interfacility differences in imaging modality selection in the diagnosis and management of children with suspected acute appendicitis. METHODS: We conducted a retrospective review of diagnostic imaging selection and outcomes in patients <20 years of age who underwent appendectomy at a single Children's Hospital from June 2008 to June 2013. These results were then compared with those of referring hospitals. RESULTS: A total of 232 children underwent appendectomy during the study period. Imaging results contributed to diagnostic and management decisions in 95.3 % of cases. CT scan was utilized as first-line imaging in 50 % of cases. CTs were preferentially performed at referring institutions (78 vs. 46 %, p < 0.001). Children were five times more likely to undergo CT at referring institutions (OR = 5.5, CI 3.0-10.2). Adjusting for demographics and Alvarado score, diagnostic imaging choice was independent of patient's clinical status. CONCLUSION: This study demonstrates that initial presentation to a referring hospital independently predicts the use of CT scan for suspected acute appendicitis. Further efforts should be undertaken to develop a clinical pathway that minimizes radiation exposure in the diagnosis of acute appendicitis, with focus on access to pediatric abdominal ultrasound.

The "weekend effect" in pediatric surgery - increased mortality for children undergoing urgent surgery during the weekend.

BACKGROUND: For a number of pediatric and adult conditions, morbidity and mortality are increased when patients present to the hospital on a weekend compared to weekdays. The objective of this study was to compare pediatric surgical outcomes following weekend versus weekday procedures. METHODS: Using the Nationwide Inpatient Sample and the Kids' Inpatient Database, we identified 439,457 pediatric (<18 years old) admissions from 1988 to 2010 that required a selected index surgical procedure (abscess drainage, appendectomy, inguinal hernia repair, open fracture reduction with internal fixation, or placement/revision of ventricular shunt) on the same day of admission. Outcome metrics were compared using logistic regression models that adjusted for patient and hospital characteristics as well as procedure performed. RESULTS: Patient characteristics of those admitted on the weekend (n=112,064) and weekday (n=327,393) were similar, though patients admitted on the weekend were more likely to be coded as emergent (61% versus 53%). After multivariate adjustment and regression, patients undergoing a weekend procedure were more likely to die (OR 1.63, 95% CI 1.21-2.20), receive a blood transfusion despite similar rates of intraoperative hemorrhage (OR 1.15, 95% CI 1.01-1.26), and suffer from procedural complications (OR 1.40, 95% CI 1.14-1.74). CONCLUSION: Pediatric patients undergoing common urgent surgical procedures during a weekend admission have a higher adjusted risk of death, blood transfusion, and procedural complications. While the exact etiology of these findings is not clear, the timing of surgical procedures should be considered in the context of systems-based deficiencies that may be detrimental to pediatric surgical care.

Perioperative strategies and technical modifications to the Nuss repair for pectus excavatum in pediatric patients: a large volume, single institution experience.

BACKGROUND: The safety and efficacy of minimally invasive pectus excavatum repair have been demonstrated over the last twenty years. However, technical details and perioperative management strategies continue to be debated. The aim of the present study is to review a large single-institution experience with the modified Nuss procedure. METHODS: A retrospective review was performed of patients who underwent primary pectus excavatum repair at a single tertiary hospital via a modified Nuss procedure that included: no thoracoscopy, retrosternal dissection achieved via a left-to-right thoracic approach, four-point stabilization of the bar, and no routine epidural analgesia. Data collected included demographics, preoperative symptoms, operative characteristics, hospital charges and postoperative outcomes. RESULTS: A total of 336 pediatric patients were identified. No cardiac perforations occurred and the rate of pericarditis was 0.6%. Contemporary rates of bar displacement have fallen to 1.2%. Routine use of chlorhexidine scrub reduced superficial site infections to 0.7%. Two patients (0.6%) with severe recurrence required reoperation. Bars were removed after an average period of 31.7(SD 13.2) months, with satisfactory cosmetic and functional results in 94.9% of cases. CONCLUSIONS: We report here a single-institution large volume experience, including modifications to the Nuss procedure that make the technique simpler and safer, improve results, and minimize hospital charges.

Operative management of acquired Jeune's syndrome.

BACKGROUND: Acquired Jeune's syndrome is a severe iatrogenic deformity of the thoracic wall following a premature and aggressive open pectus excavatum repair. We report herein our technique and experience with this rare condition. METHODS: From 1996 to 2011, nineteen patients with acquired Jeune's syndrome were retrospectively identified in a tertiary referral center. The technique used to expand and reconstruct the thoracic wall consisted of 1) release of the sternum from fibrous scar tissue, 2) multiple osteotomies along the lateral aspect of the ribs with anterior advancement of costal-cartilages to protect the heart, 3) stabilization of the thorax by placing a curved bar for retrosternal support and, 4) restoration of the sterno-costal junction by wiring the lower cartilages to the edge of the sternum. RESULTS: Major complications observed in this series were: bar displacement (seven cases), postoperative death from cardiac arrest following bronchoscopy (one case), late cardiac tamponade from migration of wire suture fragment (one case), and need for multiple reoperations (one case). Long-term cosmetic results and improvement in daily quality of life were reported as positive in the majority of cases. CONCLUSIONS: Anterior chest wall reconstruction successfully treated our series of patients with acquired Jeune's syndrome. This multifaceted technique is an effective procedure that allows expansion of the thoracic cavity and improvement of aerobic activity.

Use of risk stratification indices to predict mortality in critically ill children.

UNLABELLED: The complexity and high cost of neonatal and pediatric intensive care has generated increasing interest in developing measures to quantify the severity of patient illness. While these indices may help improve health care quality and benchmark mortality across hospitals, comprehensive understanding of the purpose and the factors that influenced the performance of risk stratification indices is important so that they can be compared fairly and used most appropriately. In this review, we examined 19 indices of risk stratification used to predict mortality in critically ill children and critically analyzed their design, limitations, and purposes. Some pediatric and neonatal models appear well-suited for institutional benchmarking purposes, with relatively brief data acquisition times, limited potential for treatment-related bias, and reliance on diagnostic variables that permit adjustment for case mix. Other models are more suitable for use in clinical trials, as they rely on physiologic variables collected over an extended period, to better capture the interaction between organ systems function and specific therapeutic interventions in acutely ill patients. Irrespective of their clinical or research applications, risk stratification indices must be periodically recalibrated to adjust for changes in clinical practice in order to remain valid outcome predictors in pediatric intensive care units. Longitudinal auditing, education, training, and guidelines development are also critical to ensure fidelity and reproducibility in data reporting. CONCLUSION: Risk stratification indices are valid tools to describe intensive care unit population and explain differences in mortality.

Increasing age at time of pectus excavatum repair in children: emerging consensus?

BACKGROUND: Advances in surgical technique for pectus excavatum repair continue to change practice patterns. The present study examines trends in operative age in a nationwide administrative database. METHODS: A cross-sectional descriptive analysis was performed using the Nationwide Inpatient Sample (NIS) and Kids' Inpatient Database (KID) data from 1998 to 2009. Pediatric discharges involving surgical repair of pectus excavatum were selected. Patients were sub-grouped by age at operation and calendar year of repair for further comparison. RESULTS: A total of 5830 elective admissions were identified that met inclusion criteria. Mean age at operation was 13.5 years, and this increased from 11.8 years to 14.4 years over the period studied and was accompanied by narrowing of the interquartile range. Examined over groups of four calendar years, patient age at the time of repair was significantly higher in more recent years in both unadjusted and multivariate analyses (P < .001). CONCLUSIONS: The age at operation in this sample has steadily increased, with an accompanying decrease in variability. This is consistent with previous findings and with overall trends in patient selection reported in the literature. This selection pattern may reflect evolving consensus regarding optimal management of pectus excavatum and provide clinical guidance regarding appropriate referral and intervention.

Modified nuss procedure in concurrent repair of pectus excavatum and open heart surgery.

BACKGROUND: Pectus excavatum (PE) can be associated with congenital and acquired cardiac disorders that also require surgical repair. The timing and specific surgical technique for repair of PE remains controversial. The present study reports the experience of combined repair of PE and open heart surgery at Johns Hopkins Hospital. METHODS: A retrospective case review was conducted of all patients who presented for repair of PE deformity while undergoing concurrent open heart surgery from 1998 through 2011. RESULTS: A total of 9 patients met inclusion criteria. All patients had a connective tissue disorder. Repair of PE was performed by modified Nuss technique after completion of the cardiac procedure, performed through a median sternotomy. Open heart procedures were either aortic root replacement or mitral valvuloplasty. Eight patients had bar removal after an average period of 30.3 months. No PE recurrence, bar displacement, or upper sternal depression was reported in 7 patients. Postoperatively, 1 patient exhibited pectus carinatum after a separate spinal fusion surgery for scoliosis. One patient died of unrelated cardiac complications before bar removal. CONCLUSIONS: Simultaneous repair of PE and open heart surgery is safe and effective. We recommend that the decision to perform a single-stage versus a multistage procedure should be reserved until after the cardiac procedure has been completed. In such cases, the Nuss technique allows for correction of the pectus deformity with good long-term cosmetic and functional results.

Natural history of vesicoureteral reflux associated with kidney anomalies.

OBJECTIVES: To evaluate the cystographic follow-up of patients with multicystic dysplastic kidney (MCDK), renal agenesis, and renal ectopia with associated primary vesicoureteral reflux (VUR). METHODS: Patients with primary associated VUR (grade 2 or more) and with a minimal follow-up of 24 months were included in this study. RESULTS: Of the children with renal agenesis, 24% had VUR. The median grade of VUR was significantly greater in the boys than in the girls (4 versus 2, respectively; P < 0.05). All girls and 34% of the boys experienced spontaneous resolution 1 year after diagnosis; 66% of the boys required operative treatment. Of the patients with MCDK, 16% had VUR. The median grade of VUR in the kidney contralateral to the MCDK was greater in the boys than in the girls (3.5 versus 2, respectively; P = 0.06). All girls and 60% of the boys had spontaneous resolution 1 year after diagnosis; 40% of the boys underwent operative treatment. Of the children with renal ectopia, 30% had VUR. The median grade of VUR for the refluxing unit was significantly greater in the girls than in the boys (3 versus 2, respectively; P < 0.05). Spontaneous resolution of VUR was observed in all boys (66% at 1 year) and in 40% of the girls; 60% of the girls required operative treatment. CONCLUSIONS: Spontaneous resolution of VUR can be anticipated in girls with MCDK or renal agenesis and in boys with renal ectopia. These data can be used in planning the proper follow-up schedule for babies with a kidney anomaly detected by ultrasonography.