State of the Art Bowel Management for Pediatric Colorectal Problems: Anorectal Malformations.

Up to 79% of patients with anorectal malformations (ARMs) experience constipation and/or soiling after a primary posterior sagittal anoplasty (PSARP) and are referred to a bowel management program. We aim to report the recent updates in evaluating and managing these patients as part of the manuscript series on the current bowel management protocols for patients with colorectal diseases (ARMs, Hirschsprung disease, functional constipation, and spinal anomalies). The unique anatomic features of ARM patients, such as maldeveloped sphincter complex, impaired anal sensation, and associated spine and sacrum anomalies, indicate their bowel management plan. The evaluation includes an examination under anesthesia and a contrast study to exclude anatomic causes of poor bowel function. The potential for bowel control is discussed with the families based on the ARM index calculated from the quality of the spine and sacrum. The bowel management options include laxatives, rectal enemas, transanal irrigations, and antegrade continence enemas. In ARM patients, stool softeners should be avoided as they can worsen soiling.

Unique Evaluation and Management Considerations for Adolescents with Late Gynecologic and Colorectal Issues in the Setting of Anorectal Malformations.

STUDY OBJECTIVE: There is little guidance for managing pubertally identified Mullerian anomalies in patients with anorectal malformations (ARMs). We sought to assess these unique issues. DESIGN: Retrospective review SETTING: Single-institution study PARTICIPANTS: Natal female patients aged 10-25, with an ARM, cloaca, or exstrophy, who presented from 2009 to 2019 with a gynecologic concern were included. INTERVENTION: Data collection was performed and included the presenting problem, psychological evaluation, fertility and sexuality concerns, and management strategies for these problems. MAIN OUTCOME MEASURES: The main outcome was unique needs that had to be addressed in the young adult population and the type of colorectal and gynecological procedures needed on representation. RESULTS: Twelve patients were identified; all had gynecologic concerns. Ten had ARMs, including cloaca (n = 3) and cloacal exstrophy (n = 5). Median age at representation was 14.6 years (IQR = 12.7, 15.3). Colorectal revisions included posterior sagittal anorectoplasty (n = 1), resection of bowel attached to urogenital sinus (n = 1), and appendicostomy revision (n = 1). Gynecologic issues included dysmenorrhea (n = 8), obstructed Mullerian anomaly (n = 6), and introital stenosis (n = 5). Behavioral health concerns (n = 9) and fertility/sexuality concerns (n = 4) were identified. Median time from first visit to reconstruction was 1.5 years (IQR = 0.5, 1.5), providing multiple visits to achieve consensus among patients and providers before intervention, including vaginal or introital repair (n = 5) and hysterectomy of obstructed uterine horns (n = 3). CONCLUSIONS: Goal-directed follow-up is required before surgical management to identify psychological and reproductive issues in patients with ARMs who have gynecologic concerns. Patient input and psychologic consultation are helpful for patients requiring staged reconstruction.

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.

Single institution review of Mini-ACE® low-profile appendicostomy button for antegrade continence enema administration.

PURPOSE: Malone antegrade continence enemas (MACE) provide a conduit in which the patient can achieve improved continence, be clean of stool, and gain independence in maintaining bowel function. The Mini-ACE® is a low-profile balloon button that is used to facilitate the administration of antegrade enemas. We sought to describe our practice and short-term outcomes. METHODS: This work is a retrospective review of the Mini-ACE® appendicostomy button from April 2019 to March 2021, with follow-up concluding in October 2021. Patient demographics, colorectal diagnoses, and outcomes were examined. RESULTS: Forty-three patients underwent Mini-ACE® placement; 22 (51%) were male. The average age at Mini-ACE® insertion was 9.2 years (range 3-20 years). The most common diagnoses were functional constipation in 19 (44%), anorectal malformation in 15 (35%), and Hirschsprung disease in 3 (7%), spinal differences 3 (7%). There were no intra-operative complications, but 5 (12%) required prolapse resection. The median length of stay was two days (IQR 1, 4). Patients achieved self-catheterization at 4.5 [3,7] months from MACE creation, with 38 children (88%) reporting excellent success in remaining clean of stool. CONCLUSION: The Mini-ACE® appears to be a safe and low-profile option for antegrade continence enema access. Further research is needed directly comparing complications and patient satisfaction rates between different MACE devices and overall quality of life. LEVEL OF EVIDENCE: Level IV.

Leveraging Collaboration in Pediatric Multidisciplinary Colorectal Care Using a Telehealth Platform.

PURPOSE: Pediatric colorectal problems often require complex multidisciplinary care (MDC), which has been affected by the SARS-CoV-2-2019 (COVID-19) pandemic. We describe our utilization and implementation of telehealth (TH) for pediatric colorectal surgery MDC visits and collate patient satisfaction using TH compared to in-person (IP) visits. METHODS: Implementation of a single-institution MDC TH platform to perform patient visits on February 1, 2020 was studied. Following 6 months of implementation, TH visits' characteristics were compared with IP visits in the 3 months before implementation by patient volume, length of clinic visits, and patient satisfaction survey results. RESULTS: Before implementation, 152 (100%) of clinic visits were IP. During the implementation, 87 (37.7%) were TH visits. Seventy-four (49%) were MDC visits, 17 (23%) of these using the TH platform. Each TH visit's median length was 25 minutes (IQR 15-30), while the median length of IP visits was 45 minutes (IQR 30-45). Pre-implementation satisfaction scores were 88.6% positive, while satisfaction scores after implementation were 96.8% positive. None of the patients who utilized the TH platform had an unplanned hospital admission within 24 hours of being seen. CONCLUSION: Our experience demonstrates that the TH platform can provide an efficient avenue for established patients and families to receive highly complex multidisciplinary follow-up care. High levels of patient satisfaction indicated that TH should become part of the routine care plan for patients who require long-term or consistent follow-up.

Psychosocial factors affecting quality of life in patients with anorectal malformation and Hirschsprung disease-a qualitative systematic review.

INTRODUCTION: Little is known about psychosocial and behavioral factors that impact the quality of life of patient's with anorectal malformations (ARM) and Hirschsprung disease (HSCR). We aimed to highlight the psychosocial, emotional, and behavioral themes that affect these patients. METHODS: A qualitative literature review of articles published between 1980 and 2019 was performed. Articles that reported quality of life (QoL) measures not directly related to bowel function and incorporated data on patients aged 0-21 years old were included. Data were separated based on distinct developmental time points. RESULTS: In the neonatal period, parents relayed uncertainty about the future and feeling overwhelmed by lack of social support. Difficulties with anxiety, peer rejection, and behavioral problems were noted in primary grades, while adolescents experienced low self-confidence, poor body image, and depression. Young adults expressed hesitancy to engage in romantic relationships or sexual activity. Lack of long-term follow-up, an incomplete transition to adult healthcare, and lack of psychology services leave young adults without guidance to manage a chronic condition. CONCLUSION: Multiple psychosocial stressors are present in the lives of ARM and HSCR patients. Provision of developmentally matched medical, psychological, and community-based supports for ARM and HSCR patients and their families can lead to improved QoL.

Development of a multi-institutional registry for children with operative congenital lung malformations.

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.

Treatment of Facial Dog Bite Injuries in the Emergency Department Compared to the Operating Room.

OBJECTIVE: To assess factors associated with repair of facial dog bites in the emergency department (ED) versus the operating room (OR) and to compare rates of surgical site infection and reoperation for each venue. STUDY DESIGN: Case series with chart review. SETTING: Single institution. SUBJECTS AND METHODS: All patients younger than 18 years of age who underwent surgical repair by a consulting surgical service within 24 hours of presentation for facial dog bites between 2010 and 2013 were included. Demographics, site of injury, associated evidence of complex injury, surgical site infections within 30 days, and reoperation within 2 years were compared between patients undergoing surgical repair in the ED versus the OR. RESULTS: One hundred sixty-five patients were evaluated; 75 patients underwent repair in the ED, and 90 patients were treated in the OR. Patients treated in the ED underwent surgery more promptly than patients treated in the OR (median time from arrival to procedure start 3.3 vs 6.8 hours, P < .001). Patients treated in the OR were more likely to have longer lacerations (3.0 cm vs 7.8 cm, P < .001), lacerations of the eyelid (17% vs 42%, P = .001), involvement of multiple regions of the face (11% vs 22%, P = .039), and multiple indicators of severe injury (3% vs 12%, P = .024). There were no differences in surgical site infections (1% vs 1%, P = .721) or reoperation rates (5% vs 13%, P = .071). CONCLUSIONS: Surgical management of pediatric facial dog bites may be successfully performed in both the ED and OR settings. Severity of the injury should dictate the choice for management.

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium.

PURPOSE: 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. METHODS: 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). RESULTS: 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). CONCLUSIONS: 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 OF EVIDENCE: Level III.

Ultrasound Elastography as a Non-Invasive Method to Monitor Liver Disease in Children with Short Bowel Syndrome: Updated Results.

PURPOSE: The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS). METHODS: A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests. Receiver operating characteristic curves (ROC) were generated and the area under the curves (AUC) estimated in order to assess the accuracy of SWS measurements to discriminate between stages of fibrosis. RESULTS: Thirty-seven paired elastography and biopsy samples from 31 patients were included. The median age was 0.6 years, and 61% were male. There was a significant positive correlation between stage of fibrosis and mean SWS (ß=0.16 m/s increase per stage, p=<0.001). ROC analysis revealed that mean SWS had good accuracy for discriminating between mild liver fibrosis (F0-F1) and moderate to severe fibrosis (F2-F4) (AUC=0.80, 95% CI 0.65-0.95). In addition, ROC analysis demonstrated that mean SWS can also accurately discriminate between mild to moderate fibrosis (F0-F2) and more severe fibrosis (F3-F4) (AUC=0.84, 95% CI 0.71-0.96). CONCLUSION: ARFI elastography is an accurate, non-invasive method to monitor liver disease in children with SBS. TYPE OF STUDY: Retrospective Cohort Study LEVEL OF EVIDENCE: II.

Recurrence of Pilonidal Disease: Our Best is Not Good Enough.

BACKGROUND: Recurrence rates of pilonidal disease have been reported to be high as 30%. Patients with recurrent pilonidal disease often develop chronic wounds and draining sinuses that incur long-term morbidity, disability, and decreased quality of life. The aim of this study was to characterize rates of recurrence in patients with pilonidal disease treated by pediatric surgeons. METHODS: A single center retrospective review of patients with pilonidal disease evaluated by pediatric surgeons from 2010 to 2015 was performed. Recurrence of pilonidal disease was defined as an episode of active pilonidal disease that required medical or surgical intervention >30 days from the preceding treatment. Repeated events proportional hazards regression modeling was performed to identify factors associated with time to recurrence. RESULTS: Among 307 patients treated for pilonidal disease, nearly 50% were male, and the median age at initial evaluation was 16 years (IQR 15-17). Approximately 45% were obese (BMI ≥ 95th percentile). The initial treatment during the study period was surgical excision in two-thirds and incision and drainage and/or antibiotics in one-third. The overall recurrence rate was 33%, with the majority of recurrences (80%) occurring within the first year. On multivariable analysis, obese BMI was the only factor independently associated with time to disease recurrence. CONCLUSIONS: Pilonidal disease has a substantial recurrence rate even after surgical excision. Future studies investigating treatments that can prevent disease recurrence are needed.

High Rate of Major Morbidity after Surgical Excision for Pilonidal Disease.

BACKGROUND: Recurrent pilonidal disease has been reported to occur in up to 30% of patients after their initial infection. Surgical resection is often performed to prevent recurrence of disease, however, morbidity after surgical excision from incision complications and disease recurrence is common. The aim of this study was to quantify major morbidity after initial pilonidal excision. PATIENTS AND METHODS: Patients with pilonidal disease who had initial excision procedures between 2011-2013 at hospitals reporting data to the Pediatric Health Information System (PHIS) were included. Predictors of the composite outcome of major surgical site complication or surgical re-excision within one year were evaluated using multivariable logistic regression models. Kaplan-Meier analysis was used to examine time to surgical re-excision. RESULTS: Of the 1,932 patients included, 4.7% (n = 138) had a major surgical site complication, 8.0% (n = 154) had a surgical re-excision, and 8.7% experienced either event within one year of their initial excision. The majority of re-excisions for recurrent disease occurred during the first two years after the initial excision. Risk factors associated independently with a greater risk of the composite outcome included older age (odds ratio [OR] 1.04 [95% confidence interval {CI} 1.00-1.07), p = 0.03), male gender (OR 1.49 [95% CI 1.09-2.08), p = 0.01), and the presence of a complex chronic gastrointestinal condition (OR 4.33 [95% CI 1.96-9.59], p < 0.001). CONCLUSIONS: Surgical excision of pilonidal disease is often complicated by site complications and nearly 1 of 10 patients develop recurrent disease requiring re-excision within two years after their initial excision. Future research into alternative therapies to treat pilonidal disease is warranted.

Sarcopenia in children with perforated appendicitis.

BACKGROUND: Decreased skeletal muscle mass, or sarcopenia, has been shown to be associated with worse postoperative recovery and a higher risk of complications in adult surgical patients. We hypothesized that pediatric patients with complicated appendicitis may experience sarcopenic changes over the course of their treatment. METHODS: The medical records and computed tomography scans of 36 pediatric complex appendicitis patients who had both preoperative and postoperative computerized tomography scans at our hospital were reviewed. Changes in psoas muscle area were examined using linear mixed models with random patient-level intercept and time effects. RESULTS: The median change in body mass index among all patients from admission to discharge was -0.8 kg/m2 (interquartile range: -1.3 to -0.2). The mean percentage change in psoas muscle area per day over the course of appendicitis-related treatment was -0.81% (95% confidence interval: -1.12 to -0.50) (P < 0.001). The relative decrease in psoas muscle area per day did not vary by initial body mass index, gender, or race (P > 0.10 for all interactions). CONCLUSIONS: Our data suggest that pediatric patients with complex appendicitis experience sarcopenic changes during their hospital admission. Given previous reports that sarcopenia is a significant predictor of worse surgical outcomes, more investigation is warranted to assess whether these changes are associated with postsurgical complications and to evaluate potential interventions that may prevent these changes.

Safety and Tolerability of Laser Hair Depilation in Pilonidal Disease: A Pilot Study.

BACKGROUND: Pilonidal disease is a common and painful disorder that can be challenging to manage. Recurrent surgical treatment is often warranted and may result in significant morbidity, with reported wound complication rates as high as 30%. Laser hair depilation of the natal cleft may decrease the incidence of recurrence. The purpose of this study was to assess the safety and tolerability of laser hair depilation in adolescents with pilonidal disease. METHODS: We performed a prospective, single arm, pilot trial of laser hair depilation to the natal cleft in 13 patients with pilonidal disease. Each patient received an outpatient laser depilation treatment every four weeks with a goal of five total treatments. Follow-up tolerability was measured after each treatment by obtaining Likert scale, patient-reported, pain scores immediately after laser treatment and every six hours post-treatment, for the first 24 hours. Consistent pain scores <4 were used as a proxy for tolerability. The primary end point was tolerability and safety, defined as pain scores consistently <4 and no deep second-degree burns during the 24-hour post-treatment period. The secondary end point was disease recurrence at one year. RESULTS: Thirteen patients were enrolled with 12 patients completing five laser depilation treatment sessions and one patient completing only four. There was 100% tolerability of treatments with no occurrence of second-degree burns. No patient was unable to complete a treatment session because of discomfort. Significantly diminished hair growth was noted after three treatments. All 13 patients were recurrence-free at a median follow-up of 13 months post-treatment initiation. CONCLUSIONS: Laser hair depilation is safe and well tolerated in adolescents with pilonidal disease and may be effective at decreasing pilonidal disease recurrence. A prospective randomized controlled trial is planned to determine effectiveness of laser hair depilation compared with chemical/mechanical depilation methods in preventing pilonidal disease recurrence.

Sclerotherapy for splenic cysts in children.

BACKGROUND: Sclerotherapy has been described as a treatment option for nonparasitic pediatric splenic cysts; however, there are limited data on its long-term effectiveness. METHODS: We performed a retrospective review and prospective follow-up imaging study of children treated for nonparasitic splenic cysts at our institution during 2006-2015. Included patients had International Classification of Disease, Ninth Revision, Clinical Modification diagnosis code 289.59 or 759.0 and underwent either sclerotherapy or a partial splenic operation (partial splenectomy, cyst excision, or marsupialization). Charts were reviewed for demographics, imaging, treatments, and complications. Identified patients were contacted and asked to return for splenic ultrasonography. RESULTS: Six surgical patients and 19 sclerotherapy patients were identified. Sclerotherapy patients underwent a median of four treatments with a decrease in the size of the cyst or complete ablation in 89.5% (17/19). Of the two patients who underwent unsuccessful sclerotherapy, one patient had sclerotherapy with sotradecol and ethanol for eight treatments, developed infection, and underwent total splenectomy. The other patient did not improve with sclerotherapy and underwent partial splenectomy. Nine patients (eight patients treated with sclerotherapy and one patient treated with partial splenectomy) were successfully contacted and underwent follow-up imaging at a median follow-up of 2.4 y since last treatment. Of the eight patients treated with sclerotherapy, four patients remained cyst-free, three had small residual asymptomatic cysts stable in size, and one developed a recurrent small asymptomatic cyst; the patient treated with surgery had no recurrence. CONCLUSIONS: Sclerotherapy appears to be an effective and durable alternative treatment for children with splenic cysts.

Impact of functional status and medical comorbidities on tracheostomy decannulation in pediatric patients.

PURPOSE: To determine if medical or functional factors influence the ability of a pediatric patient with a tracheostomy to tolerate decannulation. Retrospective evaluation of patients at a tertiary Children's Hospital undergoing evaluation with capped tracheostomy polysomnogram (cPSG) for possible tracheostomy decannulation. Charts were reviewed for demographic information, functional status, cPSG characteristics, and success or failure of decannulation. Statistical analysis was performed to determine which patient factors were predictive of successful decannulation. A total of 139 sleep studies were analyzed in 104 unique children followed for at least 1 year after a cPSG was performed to determine readiness for decannulation. At 1 year after most recent PSG, 79.8% of children were decannulated. There was no significant association between any single comorbid condition and the ability to decannulate. There was no association between individual or total functional status score and successful decannulation. Patients with at least 3 comorbid conditions investigated and a total functional score less than 7 were less likely to be decannulated successfully than other patients (71% vs. 93%, p= 0.04). Functional status and comorbid conditions do not independently predict successful decannulation. Regular multi-disciplinary team reevaluation is indicated in patients with lower functional status, as removal of tracheostomy tube may be successfully accomplished.

Nonoperative management of appendicitis in children.

PURPOSE OF REVIEW: The aim of this review is to summarize the recent literature investigating nonoperative management of uncomplicated and complicated appendicitis and highlight recent data establishing its safety and efficacy. RECENT FINDINGS: Recent studies and clinical trials have demonstrated the efficacy of nonoperative treatment of both uncomplicated and complicated appendicitis, defined as perforated appendicitis with or without formed abscess or phlegmon. Nonoperative management of uncomplicated appendicitis has been reported to be effective in approximately 71-94% of cases. In complicated appendicitis, treatment with antibiotics alone or antibiotics with interval appendectomy has been shown to be a well tolerated and reasonable treatment alternative. SUMMARY: Appendicitis is one of the most common surgical diagnoses in children. The standard of care for many years has been surgical appendectomy; however, it carries with it risks including bleeding, wound complications, injury to surrounding structures, and the potential need for reoperation. Nonoperative management of both uncomplicated and complicated appendicitis in children is well tolerated and efficacious in select populations.

Comparing laparoscopic versus open Ladd's procedure in pediatric patients.

BACKGROUND/PURPOSE: The objective of this study was to perform a comparative analysis of laparoscopic versus open Ladd's procedure on 30-day postoperative outcomes. METHODS: All elective Ladd's procedures performed on patients with intestinal malrotation in the 2013-2014 National Surgical Quality Improvement Program Pediatric were identified. A propensity score-matched analysis was used to account for baseline differences between groups, and generalized estimating equations (GEEs) were used to compare 30-day outcomes between open versus laparoscopic groups. RESULTS: Fifty-eight (18.6%) patients underwent laparoscopic Ladd's while 253 (81.4%) underwent an open technique. After propensity score matching, 53 laparoscopic cases (38.1%) and 86 open cases (61.9%) were identified and compared for outcomes. Total length of stay was shorter for the laparoscopic group compared to the open group (6 vs. 4days, p<0.001). Postoperative length of stay was shorter for the laparoscopic group as well (5 vs. 4days, p<0.001). Postoperative complications occurred in 5 laparoscopic cases (9.4%) and in 18 open cases (20.9%), but did not meet statistical significance (p=0.08). One laparoscopic patient (1.9%) and 8 open patients (9.3%) required hospitalization beyond 30days, but this also did not meet significance (p=0.08). CONCLUSIONS: In a matched analysis, laparoscopic Ladd's led to shorter hospital stays than open Ladd's in the initial 30-day postoperative period. Short-term benefits of laparoscopic Ladd's lend support for using additional resources to perform multi-institutional studies to compare differences in long-term outcomes between laparoscopic and open Ladd's. TYPE OF STUDY: Therapeutic LEVEL OF EVIDENCE: III.

Morbidity and mortality in patients with esophageal atresia.

BACKGROUND: This study reports national estimates of population characteristics and outcomes for patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and evaluates the relationships between hospital volume and outcomes. METHODS: Patients admitted within 30 days of life who had International Classification of Diseases, 9th Edition, Clinical Modification diagnosis and procedure codes relevant to EA/TEF during 1999-2012 were identified with the Pediatric Health Information System database. Baseline demographics, comorbidities, and postoperative outcomes, including predictors of in-hospital mortality, were examined up to 2 years after EA/TEF repair. RESULTS: We identified 3,479 patients with EA/TEF treated at 43 children's hospitals; 37% were premature and 83.5% had ≥1 additional congenital anomaly, with cardiac anomalies (69.6%) being the most prevalent. Within 2 years of discharge, 54.7% were readmitted, 5.2% had a repeat TEF ligation, 11.4% had a repeat operation for their esophageal reconstruction, and 11.7% underwent fundoplication. In-hospital mortality was 5.4%. Independent predictors of mortality included lower birth weight, congenital heart disease, other congenital anomalies, and preoperative mechanical ventilation. There was no relationship between hospital volume and mortality or repeat TEF ligation. CONCLUSION: This study describes population characteristics and outcomes, including predictors of in-hospital mortality, in EA/TEF patients treated at children's hospitals across the United States. Across these hospitals, rates of mortality or repeat TEF ligation were not dependent on hospital volume.

Protein kinase C phosphomimetics alter thin filament Ca2+ binding properties.

Adrenergic stimulation modulates cardiac function by altering the phosphorylation status of several cardiac proteins. The Troponin complex, which is the Ca(2+) sensor for cardiac contraction, is a hot spot for adrenergic phosphorylation. While the effect of ß-adrenergic related PKA phosphorylation of troponin I at Ser23/24 is well established, the effects of α-adrenergic induced PKC phosphorylation on multiple sites of TnI (Ser43/45, Thr144) and TnT (Thr194, Ser198, Thr203 and Thr284) are much less clear. By utilizing an IAANS labeled fluorescent troponin C, TnC(IAANS)(T53C), we systematically examined the site specific effects of PKC phosphomimetic mutants of TnI and TnT on TnC's Ca(2+) binding properties in the Tn complex and reconstituted thin filament. The majority of the phosphomemetics had little effect on the Ca(2+) binding properties of the isolated Tn complex. However, when incorporated into the thin filament, the phosphomimetics typically altered thin filament Ca(2+) sensitivity in a way consistent with their respective effects on Ca(2+) sensitivity of skinned muscle preparations. The altered Ca(2+) sensitivity could be generally explained by a change in Ca(2+) dissociation rates. Within TnI, phosphomimetic Asp and Glu did not always behave similar, nor were Ala mutations (used to mimic non-phosphorylatable states) benign to Ca(2+) binding. Our results suggest that Troponin may act as a hub on the thin filament, sensing physiological stimuli to modulate the contractile performance of the heart.