Faculty Bibliography

BACKGROUND:Management of infant perianal disease, including perianal abscess and fistula-in- ano (FIA), remains controversial. There is lack of consensus regarding the risks and benefits of operative and non-operative approaches. METHODS:The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee created a priori consensus-based questions regarding the various approaches to management of perianal abscess and FIA in infants. A comprehensive search strategy was created, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to identify and review relevant articles and answer the established questions. RESULTS:Over 2460 titles and abstracts were screened to identify 31 manuscripts describing the expected course of perianal disease when managed with nonoperative management (NOM) strategies (such as hygiene, sitz baths, and systemic antibiotics), operative intervention for abscess only (incision and drainage (I&D)) or direct surgical management of the FIA (e.g. upfront fistulotomy). Initial failure of NOM is approximately 37.5 %. Recurrence rate after initial success with NOM or I&D is approximately 21 % and 24 %, respectively. The rate of FIA development is approximately 21 % after NOM and 20 % after I&D. Recurrence after operative management of FIA, regardless of approach, is 7 %. Time to complete resolution varies widely and does not appear to differ based on treatment strategy. CONCLUSIONS:Both operative and non-operative approaches can be safely used in the management of perianal abscess and FIA but recurrence is common regardless of approach. Risks and benefits of each approach should be considered on a case-by-case basis. TYPE OF STUDY/METHODS:Systematic Review of level 3-4 studies. LEVEL OF EVIDENCE/METHODS:Level 4.

OBJECTIVE:Consensus on functional outcomes for anorectal malformations (ARM) is hindered by the heterogeneity of the available literature. Optimal patient counseling includes discussion of short- and long-term outcomes for bowel and urinary continence, sexual and psychosocial function, transitional care, and quality of life. This systematic review examines and summarizes the current literature available related to functional outcomes for children with ARM. METHODS:The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted consensus-based questions regarding anorectal malformations. Pertinent articles from 1985 to 2021 were reviewed. RESULTS:More than 10,843 publications were reviewed with 109 being included in the final recommendations. Recommendations are primarily based on C-D levels of evidence. Continence and constipation rates were higher in patients with perineal fistula and rectovestibular fistula, although symptoms tended to improve as patients got older. Urological anomalies are common and longer term urologic surveillance protocols for patients with ARM need to be further outlined. Sexual and psychosocial issues are common, but ARM patients can have a good quality of life when gastrointestinal symptoms are minimized. Many of the problems associated with ARM can persist into adulthood, supporting structured care plans as patients transition to adult care. CONCLUSIONS:Evidence to support best practices and achieve optimal outcomes for patients with ARM is lacking for many aspects of care. Multi-institutional registries have begun to address management and prognosis for these patients. Prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

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/CONCLUSIONS: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.

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.

INTRODUCTION/BACKGROUND: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/METHODS:Level 3-4. TYPE OF STUDY/METHODS:Systematic Review of Level 3-4 Studies.

BACKGROUND:Adolescent obesity is multifactorial, but parental history is the most significant risk factor. Laparoscopic sleeve gastrectomy (LSG) is part of the multidisciplinary approach to adolescent weight loss. OBJECTIVE:We aimed to evaluate the effects of parental history of bariatric surgery, as well as age at time of operation, on adolescents who underwent LSG at our institution. METHODS:We performed a retrospective review of patients, aged 10 to 19 years, who underwent LSG from January 2010 to December 2019. The adolescent bariatric surgical dataset maintained by our group was used to obtain patient demographics, weight, body mass index (BMI), and parental history of bariatric surgery. RESULTS:Among 328 patients, 76 (23.2%) had parents who had previously undergone bariatric surgery. These patients were significantly heavier by weight (p = 0.012) at the time of operation but had no difference in postoperative weight loss. When all patients were compared by age at operation (< 16 years, n = 102, ≥ 16 years, n = 226), there were few differences in outcomes. CONCLUSIONS:LSG is an effective approach to surgical weight loss in adolescents. Patient age should not be a barrier to weight loss surgery, especially among patients with a parental history of obesity. By intervening at a younger age, the metabolic sequelae of obesity may be reduced.

INTRODUCTION/BACKGROUND:. Shock-index (SI) and systolic blood pressure (SBP) are metrics for identifying children and adults with hemodynamic instability following injury. The purpose of this systematic review was to assess the quality of these metrics as predictors of outcomes following pediatric injury. MATERIALS AND METHODS/METHODS:We conducted a literature search in Pubmed, SCOPUS, and CINAHL to identify studies describing the association between shock metrics on the morbidity and mortality of injured children and adolescents. We used the data presented in the studies to calculate the sensitivity and specificity for each metric. This study was registered with Prospero, protocol CRD42020162971. RESULTS:Fifteen articles met the inclusion criteria. seven studies evaluated SI or SIPA score, an age-corrected version of SI, as predictors of outcomes following pediatric trauma, with one study comparing SIPA score and SBP and one study comparing SI and SBP. The remaining eight studies evaluated SBP as the primary indicator of shock. The median sensitivity for predicting mortality and need for blood transfusion was highest for SI, followed by SIPA, and then SBP. The median specificity for predicting these outcomes was highest for SBP, followed by SIPA, and then SI. CONCLUSIONS:Common conclusions were that high SIPA scores were more specific than SI and more sensitive than SBP. SIPA score had better discrimination for severely injured children compared to SI and SBP. An elevated SIPA was associated with a greater need for blood transfusion and higher in-hospital mortality. SIPA is specific enough to exclude most patients who do not require a blood transfusion.

PURPOSE/OBJECTIVE:Adnexal torsion is a gynecologic emergency, requiring intervention for tissue preservation. At our institution, torsion is managed by pediatric surgeons or gynecologists. We evaluated differences between specialties to streamline evaluation for children with gynecological emergencies, develop a clinical pathway, and prevent care delays. METHODS:A retrospective review of adolescents undergoing intervention for adnexal torsion from 2004-2018 was performed. Differences in time to intervention, operation duration, the procedure performed, and length of stay (LOS) between the specialties were analyzed. RESULTS:Eighty-six patients underwent 94 operations for presumed adnexal torsion with 87 positive cases. Pediatric surgeons performed 60 operations and 34 cases were performed by gynecologists. Preservation of fertility was the goal in both cohorts and the rate of oophoropexy, cystectomy, and oophorectomy were similar between the cohorts (p = 0.14, p = 1.0, p = 0.39, respectively). There was no difference in intra-operative time (p = 0.69). LOS was shorter in the gynecology cohort (median 1 day [1-2] vs. 2 days [2-3], p > 0.001). CONCLUSIONS:Adnexal torsion is a time-sensitive diagnosis requiring prompt intervention for ovarian or fallopian tube preservation. A multidisciplinary institutional care pathway should be developed and implemented.

Achalasia is a rare condition affecting esophageal motility in children. In a manner similar to the disease found in the adult population, children experience symptoms of dysphagia, regurgitation, and chest pain due to a failure of relaxation of the lower esophageal sphincter. Standard diagnostic approaches include upper endoscopy and esophageal manometry. New developments in diagnosis include high-resolution esophageal manometry and the endoscopic functional lumen imaging probe. Therapies available include endoscopic balloon dilations and botulinum toxin injections into the lower esophageal sphincter, as well as surgical interventions. The Heller myotomy was first described in 1913; since then, there have been many modifications to the procedure to improve outcomes and lower morbidity. Currently, the most commonly performed surgical procedure is the laparoscopic Heller myotomy, in which the sphincter muscle is divided using longitudinal incisions with or without a partial fundoplication procedure. In recent years, per oral endoscopic myotomy (POEM) is gaining support as a viable natural orifice therapy for achalasia. Complications of POEM occur at a relatively low rate, and outcomes following the procedure have been promising. The treatment of end-stage achalasia however, may include partial or total esophagectomy with reconstruction if possible. Future research is focused primarily on increasing the efficacy, and lowering complications, of existing therapeutic modalities.