Medical Alert Cards for Pediatric Patients With Antegrade Continence Enemas to Enhance Awareness.

INTRODUCTION: The antegrade continence enema (ACE) is a surgical technique that establishes a route for colonic irrigation, necessitating a detailed comprehension of the procedural methodology, including the conduit construction, the catheters selection, and the administration of irrigation solutions. It is critical to disseminate comprehensive information regarding ACE procedures to health-care professionals and caregivers to enhance their understanding and support management of patients undergoing this intervention. This study aims to evaluate the efficacy of a novel educational tool designed to augment the knowledge base of physicians and caregivers regarding ACE procedures. METHODS: Medical alert cards were conceptualized for patients managed with ACEs via an appendicostomy, neoappendicostomy, or cecostomy. These cards included essential information such as (1) contact details of the health-care team, (2) specifics of the surgical procedure, (3) criteria for emergency department referral, and (4) bespoke patient-specific information. A single-institution survey was conducted to assess the cards' role in improving awareness of ACE procedures among health-care providers and caregivers and guiding management of patients with ACEs. The study adhered to Consensus-Based Checklist for Reporting of Survey Studies guidelines. RESULTS: Twenty-seven responses were collected from 18 health-care providers and nine caregivers. A significant majority (n = 24, 88%) affirmed the cards' effectiveness in providing detailed information about individual patients. The design and layout of the card were well-received by 93% (n = 25) of respondents, with a median readability score of 8 (interquartile range 7-10). Additionally, some providers (n = 3, 17%) highlighted the card's utility in facilitating patient referrals to specialized care centers. Suggestions for improvement included adding information about different types of tubes, enhancing readability, and optimizing the visual representation of the conduit. CONCLUSIONS: Medical alert cards demonstrated a significant potential to improve understanding of ACEs among health-care providers and caregivers. The cards aid in informing stakeholders about the ACE procedure, guiding the child's management, and referring the patients to specialized care facilities when necessary.

Global Strategies for Postoperative Care and Bowel Management in Patients With Anorectal Malformations: Varied Practices and Barriers.

BACKGROUND: Anorectal malformations (ARMs) commonly result in constipation and fecal incontinence following primary surgical reconstruction. This study investigates global variations in postoperative care and resources. METHODS: A survey was distributed via the International Pediatric Endosurgery Group (IPEG) and snowball sampling. Geographically, respondents were categorized into high-income countries (HICs) and low-or middle-income countries (LMICs). RESULTS: 233 surveys were received, 64% from LMICs and 36% from HICs. Of these, 51% reported monitoring ARM patients for over a year, while 23% utilized digital technologies. Access to anesthesia for anorectal exams was available to 70% of respondents. Only 29% had established a one-week Bowel Management Program (BMP). Collaboration with urologists and gynecologists was more prevalent in HICs (59%) compared to LMICs (37%, p < 0.01). In HICs, nurses and advanced practice providers were significantly more involved in BMP (71% vs. 39% in LMICs; p < 0.01), and abdominal radiographs for regimen adjustments were used more frequently (80% vs. 69% in LMICs; p = 0.03). Treatment regimens were more varied in HICs, and quality of life tracking was more consistent (19% vs. 9% in LMICs; p = 0.02). LMICs reported significant shortages of medications and equipment (75% vs. 58% in HICs; p = 0.01), inadequate sanitation (48% vs. 24%; p < 0.01), and insufficient insurance coverage (58% vs. 44%; p = 0.04). CONCLUSIONS: There are notable global disparities in the postoperative care of ARM patients, particularly in BMP protocols and treatment regimens, with LMICs facing severe socioeconomic challenges. This emphasizes the urgent need for targeted strategies and resources to enhance outcomes for ARM patients across different regions. LEVEL OF EVIDENCE: Level III.

Evaluation and Management of Biliary Dyskinesia in Children and Adolescents: A Systematic Review From the APSA Outcomes and Evidence-Based Committee.

INTRODUCTION: The diagnosis and management of biliary dyskinesia in children and adolescents remains variable and controversial. The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee (APSA OEBP) performed a systematic review of the literature to develop evidence-based recommendations. METHODS: Through an iterative process, the membership of the APSA OEBP developed five a priori questions focused on diagnostic criteria, indications for cholecystectomy, short and long-term outcomes, predictors of success/benefit, and outcomes of medical management. A systematic review was conducted, and articles were selected for review following Preferred Reporting Items for Systematic Review and Meta-analyses (PRISMA) guidelines. Risk of bias was assessed using Methodologic Index for Non-Randomized Studies (MINORS) criteria. The Oxford Levels of Evidence and Grades of Recommendation were utilized. RESULTS: The diagnostic criteria for biliary dyskinesia in children and adolescents are not clearly defined. Cholecystectomy may provide long-term partial or complete relief in some patients; however, there are no reliable predictors of symptom relief. Some patients may experience resolution of symptoms with non-operative management. CONCLUSIONS: Pediatric biliary dyskinesia remains an ill-defined clinical entity. Pediatric-specific guidelines are necessary to better characterize the condition, guide work-up, and provide management recommendations. Prospective studies are necessary to more reliably identify patients who may benefit from cholecystectomy. LEVEL OF EVIDENCE: Level 3-4. TYPE OF STUDY: Systematic Review of Level 3-4 Studies.

Commentary- Bridging the Gap: The Impact of Bowel Management Programs on Daily Life in Pediatric Colorectal Disease.

BACKGROUND: This commentary discusses the social impact of bowel management programs (BMPs) on children with colorectal diseases, including anorectal malformations (ARM), Hirschsprung disease (HD), functional constipation (FC), and spina bifida. Previous studies focused on functional outcomes, but this study bridges the gap to daily life experiences. METHODS: The study examined children's experiences in BMPs, focusing on school participation, vacation ability, and overall patient experience. Cleanliness, defined as fewer than one stool soiling episode per week, was achieved by 70% of participants. RESULTS: Positive patient experiences were linked to achieving stool cleanliness, regardless of the management method. Invasive methods like enemas did not negatively affect experiences if cleanliness was maintained. Validated patient-reported experience measures (PREMs) and patient-reported outcomes measures (PROMs) were used, though the median age of 8.9 years posed limitations. CONCLUSION: The commentary highlights the significance of stool cleanliness in improving patient experiences and supports the effectiveness of various BMP methods. Future research should include longitudinal follow-ups to assess BMP durability and gather data from older children.

Clinical Consensus Statement on the Use of Indocyanine Green Fluorescence-guided Surgery in Pediatric Patients.

BACKGROUND AND AIMS: Indocyanine Green Fluorescence (ICG-F)- guided surgery is becoming an increasingly helpful tool in pediatric surgical care. This consensus statement investigates the utility of ICG-F in various pediatric surgical applications, primarily focusing on its evidence base, safety, indications, use across different surgical specialties and dosing strategies. The aim is to establish an international consensus for ICG-F use in pediatric surgery. METHODS: An international panel of 15 pediatric surgeons from 9 countries was assembled. The structured process consisted of a rapid scoping review, iterative discussion sessions, mixed-methods studies with key stakeholders, and voting rounds on individual statements to create draft consensus statements. RESULTS: 100 articles were identified during the review and summarized by application. Based on this condensed evidence, consensus statements were generated after 3 iterative rounds of anonymous voting. Key areas of agreement were quality of evidence, the safety of ICG, pediatric surgical indications, utilization per surgical specialty, and dosing of ICG. CONCLUSION: This consensus statement aims to guide healthcare professionals in managing ICG-F use in pediatric surgical cases based on the best available evidence, key stakeholder consultation, and expert opinions. Despite ICG-F's promising potential, the need for higher-quality evidence, prospective trials, and safety studies is underscored. The consensus also provides a framework for pediatric surgeons to utilize ICG-F effectively. LEVEL OF EVIDENCE: III.

Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group.

BACKGROUND: Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD. METHODS: The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD. RESULTS: This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations. CONCLUSIONS: A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management. LEVEL OF EVIDENCE: V.

Screening and Prophylaxis for Venous Thromboembolism in Pediatric Surgery: A Systematic Review.

OBJECTIVE: The American Pediatric Surgical Association Outcomes and Evidence-Based Practice Committee conducted a systematic review to describe the epidemiology of venous thromboembolism (VTE) in pediatric surgical and trauma patients and develop recommendations for screening and prophylaxis. METHODS: The Medline (Ovid), Embase, Cochrane, and Web of Science databases were queried from January 2000 through December 2021. Search terms addressed the following topics: incidence, ultrasound screening, and mechanical and pharmacologic prophylaxis. The Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were followed. Consensus recommendations were derived based on the best available literature. RESULTS: One hundred twenty-four studies were included. The incidence of VTE in pediatric surgical populations is 0.29% (Range = 0.1%-0.48%) and directly correlates with surgery type, transfusion, prolonged anesthesia, malignancy, congenital heart disease, inflammatory bowel disease, infection, and female sex. The incidence of VTE in pediatric trauma populations is 0.25% (Range = 0.1%-0.8%) and directly correlates with injury severity, major surgery, central line placement, body mass index, spinal cord injury, and length-of-stay. Routine ultrasound screening for VTE is not recommended. Consider sequential compression devices in at-risk nonmobile, pediatric surgical patients when an appropriate sized device is available. Consider mechanical prophylaxis alone or with pharmacologic prophylaxis in adolescents >15 y and post-pubertal children <15 y with injury severity scores >25. When utilizing pharmacologic prophylaxis, low molecular weight heparin is superior to unfractionated heparin. CONCLUSIONS: While VTE remains an infrequent complication in children, consideration of mechanical and pharmacologic prophylaxis is appropriate in certain populations. TYPE OF STUDY: Systematic Review of level 2-4 studies. LEVEL OF EVIDENCE: Level 3-4.

Classification and Surgical Management of Anorectal Malformations: A Systematic Review and Evidence-based Guideline From the APSA Outcomes and Evidence-based Practice Committee.

OBJECTIVE: Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs. These questions were related to categorization of ARMs and optimal methods and timing of surgical management. A comprehensive search strategy was performed, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to perform the systematic review to attempt to answer five questions related to surgical care of ARM. RESULTS: A total of 10,843 publications were reviewed, of which 90 were included in final recommendations, and some publications addressed more than one question (question: 1 n = 6, 2 n = 63, n = 15, 4 n = 44). Studies contained largely heterogenous groups of ARMs, making direct comparison for each subtype challenging and therefore, no specific recommendation for optimal surgical approach based on outcomes can be made. Both loop and divided colostomy may be acceptable methods of fecal diversion for patients with a diagnosis of anorectal malformation, however, loop colostomies have higher rates of prolapse in the literature reviewed. In terms of timing of repair, there did not appear to be significant differences in outcomes between early and late repair groups. Clear and uniform definitions are needed in order to ensure similar populations of patients are compared moving forward. Recommendations are provided based primarily on A-D levels of evidence. CONCLUSIONS: Evidence-based best practices for ARMs are lacking for many aspects of care. Multi-institutional registries have made progress to address some of these gaps. Further prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

A Systematic Review of Telehealth Utilization for Bowel Management Programs in Pediatric Colorectal Surgery.

Recent advancements in pediatric surgery have embraced telehealth (TH) modalities, transitioning from traditional in-person consultations to virtual care. This shift has broadened access to healthcare, potentially enhancing affordability, patient and caregiver satisfaction, and clinical outcomes. In pediatric colorectal surgery, telehealth has been effectively utilized to support Bowel Management Programs (BMPs) for children suffering from constipation and fecal incontinence. A systematic review was conducted to assess the effectiveness of virtual BMPs, analyzing studies from January 2010 to December 2023, sourced from MEDLINE (via PubMed), Embase, and the Cochrane Library, with five studies included. Remote BMPs, implemented through video or telephone consultations, reported satisfaction rates exceeding 75% among families, indicating a strong preference for virtual interactions over traditional visits. Significant findings from the studies include improvements in Vancouver and Baylor scores, reductions in the duration of multidisciplinary consultations, enhancements in pediatric quality of life and Cleveland scores, and decreased frequency of laxative treatments. The implementation of TH has facilitated patient-led care, enabling timely adjustments in treatment and efficient distribution of medical supplies. The findings suggest that virtual BMPs are a viable and effective alternative to conventional approaches, yielding high caregiver satisfaction and superior clinical outcomes while promoting patient independence.

Utilization of Indocyanine Green (ICG) Fluorescence in Patients with Pediatric Colorectal Diseases: The Current Applications and Reported Outcomes.

In pediatric colorectal surgery, achieving and visualizing adequate perfusion during complex reconstructive procedures are paramount to ensure postoperative success. However, intraoperative identification of proper perfusion remains a challeng. This review synthesizes findings from the literature spanning from January 2010 to March 2024, sourced from Medline/PubMed, EMBASE, and other databases, to evaluate the role of indocyanine green (ICG) fluorescence imaging in enhancing surgical outcomes. Specifically, it explores the use of ICG in surgeries related to Hirschsprung disease, anorectal malformations, cloacal reconstructions, vaginal agenesis, bladder augmentation, and the construction of antegrade continence channels. Preliminary evidence suggests that ICG fluorescence significantly aids in intraoperative decision-making by improving the visualization of vascular networks and assessing tissue perfusion. Despite the limited number of studies, initial findings indicate that ICG may offer advantages over traditional clinical assessments for intestinal perfusion. Its application has demonstrated a promising safety profile in pediatric patients, underscoring the need for larger, prospective studies to validate these observations, quantify benefits, and further assess its impact on clinical outcomes. The potential of ICG to enhance pediatric colorectal surgery by providing real-time, accurate perfusion data could significantly improve surgical precision and patient recovery.

Management and Outcomes of Pediatric Lymphatic Malformations: A Systematic Review From the APSA Outcomes and Evidence-Based Practice Committee.

BACKGROUND: Significant variation in management strategies for lymphatic malformations (LMs) in children persists. The goal of this systematic review is to summarize outcomes for medical therapy, sclerotherapy, and surgery, and to provide evidence-based recommendations regarding the treatment. METHODS: Three questions regarding LM management were generated according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Publicly available databases were queried to identify articles published from January 1, 1990, to December 31, 2021. A consensus statement of recommendations was generated in response to each question. RESULTS: The initial search identified 9326 abstracts, each reviewed by two authors. A total of 600 abstracts met selection criteria for full manuscript review with 202 subsequently utilized for extraction of data. Medical therapy, such as sirolimus, can be used as an adjunct with percutaneous treatments or surgery, or for extensive LM. Sclerotherapy can achieve partial or complete response in over 90% of patients and is most effective for macrocystic lesions. Depending on the size, extent, and location of the malformation, surgery can be considered. CONCLUSION: Evidence supporting best practices for the safety and effectiveness of management for LMs is currently of moderate quality. Many patients benefit from multi-modal treatment determined by the extent and type of LM. A multidisciplinary approach is recommended to determine the optimal individualized treatment for each patient.

Can Anorectal Stenosis be Managed With Dilations Alone? A PCPLC Review.

PURPOSE: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. METHODS: A retrospective database review was performed using the PCPLC registry. The patients were evaluated for demographics, co-morbidities, diagnostic work-up, surgical intervention, current bowel management, and complications. RESULTS: 64 patients with anal or rectal stenosis were identified (57 anal, 7 rectal) from a total of 14 hospital centers. 59.6% (anal) and 42.9% (rectal) were male. The median age was 3.2 (anal) and 1.9 years (rectal). 11 patients with anal stenosis also had Currarino Syndrome with 10 of the 11 patients diagnosed with a presacral mass compared to only one rectal stenosis with Currarino Syndrome and a presacral mass. 13 patients (22.8%, anal) and one (14.3%, rectal) underwent surgical correction. Nine patients (8 anal, 1 rectal) underwent PSARP. Other procedures performed were cutback anoplasty and anterior anorectoplasty. The median age at repair was 8.4 months (anal) and 10 days old (rectal). One patient had a wound complication in the anal stenosis group. Bowel management at last visit showed little differences between groups or treatment approach. CONCLUSION: The PCPLC registry demonstrated that these patients can often be managed successfully with dilations alone. PSARP is the most common surgical repair chosen for those who undergo surgical repair. LEVEL OF EVIDENCE: III.

Evaluation of Post-neonatal Intensive Care Unit Home Irrigations Prior to Pull-through: Implications for Hirschsprung Disease Management.

BACKGROUND: Pull-through procedures for Hirschsprung disease (HD) can be performed during the Neonatal Intensive Care Unit (NICU) stay or delayed until discharge following home irrigations. This study assesses the safety of a delayed pull-through as an alternative to neonatal reconstruction in infants with successful abdomen decompression with home irrigations based on Hirschsprung-associated enterocolitis (HAEC) development. METHODS: A single-institution retrospective review of neonates with HD who underwent delayed or neonatal pull-through from July 2018-July 2022. Endpoints included post-pull-through HAEC incidence, recurrence at an 18-month follow-up, time to the first HAEC episode, NICU length of stay (LOS), and HAEC-related LOS. RESULTS: Twenty-four neonates were included. Eighteen were discharged from the NICU with home irrigations. Of these, 3 (28%) developed enterocolitis preoperatively, 12 (67%) underwent a delayed pull-through. NICU LOS in the delayed cohort was 3 times shorter than in the neonatal (6 vs. 18 days, p < 0.01). The incidence of enterocolitis (82% vs. 80%), time to the first episode (43 vs. 57 days), and HAEC-related LOS (median of 3 days) were similar. CONCLUSIONS: Delayed HD pull-through is a viable neonatal reconstruction alternative that reduces NICU stay without increasing the risk of postoperative HAEC development. TYPE OF STUDY: Original Research Article. LEVEL OF EVIDENCE: III.

Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium.

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days-12 months; toddler 1 year-5 years and child >5 years). RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001). CONCLUSION: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through. LEVEL OF EVIDENCE: III.

Gastrointestinal Ostomies in Children: A Primer for the Pediatrician.

Despite the advancement of medical therapies in the care of the preterm neonate, in the management of short bowel syndrome and the control of pediatric inflammatory bowel disease, the need to create fecal ostomies remains a common, advantageous treatment option for many medically complex children.

Comparison of the PCPLC Database to NSQIP-P: A Patient Matched Comparison of Surgical Complications Following Repair of Anorectal Malformation.

BACKGROUND: Anorectal malformations (ARM) are rare and heterogenous which creates a challenge in conducting research and offering recommendations for best practice. The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) was formed in 2016 to address this challenge and created a shared national data registry to collect information about pediatric colorectal patients. There has been no external validation of the data collected. We sought to evaluate the database by performing a patient matched analysis comparing 30-day outcomes identified in the PCPLC registry with the NSQIP-P database for patients undergoing surgical repair of ARM. METHODS: Patients captured in the PCPLC database from 2016 to 2021 at institutions also participating in NSQIP-P who underwent ARM repair younger than 12 months old were reviewed for 30-day complications. These patients were matched to their NSQIP-P record using their hospital identification number, and records were compared for concordance in identified complications. RESULTS: A total of 591 patient records met inclusion criteria in the PCPLC database. Of these, 180 patients were also reviewed by NSQIP-P. One hundred and fifty-six patient records had no complications recorded. Twenty-four patient records had a complication listed in one or both databases. There was a 91 % concordance rate between databases. When excluding complications not tracked in the PCPLC registry, this agreement improved to 93 %. CONCLUSION: Including all patients evaluated for this subpopulation, a 91 % concordance rate was observed when comparing PCPLC collected complications to NSQIP-P. Future efforts can focus on further validating the data within the PCPLC for other patient populations. LEVEL OF EVIDENCE: V.

Intravaginal Mesalamine Administration: A Novel Technique for Neovaginal Colitis.

OBJECTIVE: We aim to present a novel technique for the treatment of neovaginal diversion colitis (also known as neovaginal colitis). CASE: OT is a 21-year-old cisgender female with a history of VACTERL who underwent a colonic vaginoplasty as an infant. She presented with symptoms indicative of and later diagnosed as neovaginal diversion colitis. The patient underwent a novel regimen of vaginal instillation of mesalamine followed by complete resolution of her symptoms. DISCUSSION: The following case study demonstrates a potentially effective treatment for cases of neovaginal diversion colitis.