Delphi Consensus on Diagnostic Criteria for LUMBAR Syndrome.

OBJECTIVE: To develop consensus on diagnostic criteria for LUMBAR syndrome, the association of segmental infantile hemangiomas that affect the Lower body with Urogenital anomalies, Ulceration, spinal cord Malformations, Bony defects, Anorectal malformations, Arterial anomalies and/or Renal anomalies. STUDY DESIGN: These diagnostic criteria were developed by an expert multidisciplinary and multi-institutional team based on analysis of peer-reviewed data, followed by electronic-Delphi consensus of a panel of 61 international pediatric specialists. RESULTS: After 2 Delphi rounds, a 92% or higher level of agreement was reached for each Delphi statement. 98% of panelists agreed with the diagnostic criteria, and 100% agreed the criteria would be useful in clinical practice. The diagnosis of LUMBAR requires the presence of a segmental, or patterned, infantile hemangioma of the lumbosacral, sacrococcygeal, or pelvic cutaneous regions plus one additional criterion of the urogenital, spinal, bony, anorectal, arterial, or renal organ systems. CONCLUSIONS: These diagnostic criteria will enhance clinical care by improving screening, detection, and overall awareness of this poorly understood neurocutaneous disorder. The criteria can be utilized by a wide variety of pediatric subspecialists. In addition, formal criteria will improve phenotypic uniformity among LUMBAR syndrome cohorts and a patient registry, allowing investigators to assess clinical features, long-term outcomes, and results of genetic sequencing in a standardized manner. Finally, these criteria will serve as a starting point for prospective studies to establish formal screening and management guidelines.

Ex Utero Intrapartum Treatment for Prenatally Diagnosed Cervicofacial Lymphatic Malformations.

INTRODUCTION: Cervicofacial lymphatic malformations (cf-LM) may be identified on prenatal ultrasound, prompting consideration of ex utero intrapartum treatment (EXIT) to secure the fetal airway. Furthermore, the recent shift in postnatal management of cf-LM from resection alone toward a multimodal approach including sirolimus and sclerotherapy may impact the neonatal outcomes of cf-LM. This study aims to characterize the neonatal outcomes of patients with prenatally diagnosed cf-LM who underwent EXIT-to-airway. METHODS: Retrospective, single-center review of all patients who underwent EXIT-to-airway for cf-LM (2011-2020) was performed. Demographics, prenatal imaging, intraoperative details, and outcomes were analyzed using descriptive statistics (median [interquartile range]). RESULTS: Six patients with prenatally diagnosed cf-LM underwent EXIT-to-airway at a median gestational age of 36 (33.8-36.9) wk. The median volume on fetal magnetic resonance imaging was 187.5 mL (142.3-237.8) and median tracheoesophageal displacement index was 11 mL (9.25-15). All were successfully intubated on placental support with a median duration of 25 (15.25-91) d. There was one fatality at day of life 10 due to necrotizing enterocolitis totalis. Among survivors, 2 of 5 underwent tracheostomy placement, 4 of 5 underwent gastrostomy tubes placement, and all 5 received sirolimus at day of life of 9 [8-10] d. Four patients underwent debulking or excision of their cf-LM during the initial hospitalization. Patients had a median length of stay of 68 (45-129) d. One patient experi enced a pneumothorax with evidence of barotrauma following EXIT-to-airway requiring chest tube placement (duration 8 d). CONCLUSIONS: EXIT-to-airway procedure remains a feasible strategy for mitigating neonatal hypoxia in cases of prenatally diagnosed cervicofacial lymphatic malformations. However, postnatal outcomes are variable with potential long-term aerodigestive sequelae.

An infant with LUMBAR syndrome complicated by severe perineal ulceration managed with diverting colostomy.

Infantile hemangiomas are the most common childhood vascular lesions. LUMBAR syndrome (lower body hemangioma, urogenital abnormalities/ulceration, myelopathy, bony deformities, anorectal malformations/arterial anomalies, and rectal anomalies) warrants special treatment considerations. Here we describe a case of an infant with LUMBAR syndrome who presented with severe perineal ulceration refractory to standard medical therapy and was managed with a temporary diverting sigmoid colostomy. This case demonstrates that adjunctive surgical management can be considered in infants with aggressive perineal wounds refractory to standard medical therapy.

Institutional Management of Abdominal Lymphatic Malformations: Evolution of Treatment Over a Decade.

INTRODUCTION: Abdominal lymphatic malformations (LM) have been historically managed with surgical resection; however, sclerotherapy and sirolimus have emerged as effective therapies. The purpose of our study is to evaluate our institutional change in management and outcomes for abdominal LM over the past decade. METHODS: A retrospective cohort study was performed for all children with an abdominal LM managed at our multidisciplinary Vascular Anomalies Center from 2011 to 2020. Patient demographics, symptoms, treatment, treatment response, and complications were analyzed with descriptive statistics. RESULTS: Twenty-nine patients with abdominal LM were identified with a median age at treatment of 6 y (interquartile range 3-14). A majority of lesions were identified as macrocystic (n = 18, 62%). The most common intervention was surgery alone (n = 14, 48%) followed by sirolimus alone (n = 4, 14%), and sclerotherapy + sirolimus (n = 4, 14%). Five patients were observed due to lack of symptoms at presentation. Prior to 2017, 91% (10/11) of LM were treated with surgery alone. Following 2017, only 31% (4/13) were treated with surgery alone. Sixty-seven percent (16/24) of treated patients had >95% reduction in LM maximum diameter. A majority of patients (23/24) who received treatment had improvement or resolution of symptoms at median 9-mo follow-up. Only three patients had post-treatment complications, including a drain site infection, small bowel obstruction, and an aspiration event. Complications only occurred after sclerotherapy sessions. CONCLUSIONS: Over the study period, our institution has transitioned to initial management of symptomatic abdominal LM with sclerotherapy and/or sirolimus with almost all treated patients having excellent or satisfactory treatment response. Post-treatment complications were rare.

The role of sonography for depiction of a whirlpool sign unrelated to midgut malrotation in neonates.

BACKGROUND: The sonographic whirlpool sign of volvulus due to midgut malrotation is well recognized. However, variations of the whirlpool sign may be seen in other conditions, but this observation has received little attention in the literature. OBJECTIVE: This study presents a series of neonates with a variety of causes of congenital intestinal obstruction, all associated with a whirlpool sign (unrelated to midgut volvulus), which was correctly recognized preoperatively on sonography. We also emphasize the pivotal role of sonography in managing congenital obstruction of the intestinal tract in neonates. MATERIALS AND METHODS: This is a retrospective analysis of clinical, imaging and surgical findings in 11 neonates with congenital intestinal obstruction associated with a whirlpool sign (unrelated to midgut volvulus) that was recognized preoperatively on sonography and in whom the cause for the whirlpool was documented at surgery. RESULTS: Eleven neonates (eight male, three female) had clinical and radiographic evidence of intestinal obstruction in whom sonography depicted a whirlpool sign, which was recognized on the initial sonogram in nine and on a repeat sonogram in two. The whirlpool was located in the upper abdomen in only two, mid-abdomen in five and right lower quadrant in four. The whirlpool was only 1-2 cm in diameter. An upper gastrointestinal series in three neonates failed to depict the cause of obstruction. Contrast enema in three cases had findings suggesting the site of obstruction was in the ileum but none depicted the exact cause of the obstruction. At surgery, the whirlpool sign correlated with a segmental volvulus of the small intestine in eight neonates and with the coiled distal small intestine associated with apple-peel atresia in the other three. CONCLUSION: This study illustrates neonates in whom a whirlpool sign (unrelated to midgut volvulus) was correctly recognized on sonography before surgery. Sonography proved more useful than an upper gastrointestinal series or contrast enema in depicting the exact cause of the obstruction. Pediatric radiologists must make the effort to search throughout the entire abdomen and pelvis for a small whirlpool sign on sonography, even in the absence of midgut malrotation, in neonates with congenital intestinal obstruction. Its recognition preoperatively will facilitate a rapid diagnosis and will obviate the necessity for contrast examinations of the gastrointestinal tract, which require ionizing radiation.

Comparing oncologic outcomes after minimally invasive and open surgery for pediatric neuroblastoma and Wilms tumor.

BACKGROUND: Minimally invasive surgery (MIS) has been widely adopted for common operations in pediatric surgery; however, its role in childhood tumors is limited by concerns about oncologic outcomes. We compared open and MIS approaches for pediatric neuroblastoma and Wilms tumor (WT) using a national database. METHODS: The National Cancer Data Base from 2010 to 2012 was queried for cases of neuroblastoma and WT in children ≤21 years old. Children were classified as receiving open or MIS surgery for definitive resection, with clinical outcomes compared using a propensity matching methodology (two open:one MIS). RESULTS: For children with neuroblastoma, 17% (98 of 579) underwent MIS, while only 5% of children with WT (35 of 695) had an MIS approach for tumor resection. After propensity matching, there was no difference between open and MIS surgery for either tumor for 30-day mortality, readmissions, surgical margin status, and 1- and 3-year survival. However, in both tumors, open surgery more often evaluated lymph nodes and had larger lymph node harvest. CONCLUSION: Our retrospective review suggests that the use of MIS appears to be a safe method of oncologic resection for select children with neuroblastoma and WT. Further research should clarify which children are the optimal candidates for this approach.

Clinical Acuity Shorthand System: a standardized classification tool to facilitate handoffs.

BACKGROUND: The handoff of medical information from one provider to another can be inefficient and error prone, potentially undermining patient safety. Although several tools for structuring handoffs exist, none provide a brief, standardized framework for ensuring that patient acuity is efficiently and reliably communicated. We aim to introduce and perform initial testing of the Clinical Acuity Shorthand System (CLASS) (Copyright 2015, Duke University. All rights reserved.) for surgery, a patient classification tool intended to facilitate efficient communication of key patient information during handoffs. MATERIALS AND METHODS: Surgical trainees at a single center were asked to perform an exercise involving application of CLASS to 10 theoretical patient scenarios and to then complete a brief survey. Responses were scored based on similarity to target answers. Performance was evaluated overall and between groups of trainees. Time required to complete the exercise was also determined and perceived utility of the system was assessed based on survey responses. RESULTS: The study task was completed by 17 participants. Mean time to task completion was 10.3 ± 8.4 min. Accuracy was not decreased, and was in fact superior, in junior trainees. Most respondents indicated that such a system would be feasible and could prevent medical errors. CONCLUSIONS: CLASS is a novel system that can be learned quickly and implemented readily by trainees and can be used to convey patient information concisely and with acceptable fidelity regardless of level of training. Further study examining application of this system on clinical surgical services is warranted.

US-Guided, Direct Puncture Retrograde Thoracic Duct Access, Lymphangiography, and Embolization: Feasibility and Efficacy.

PURPOSE: To describe technical details, success rate, and advantages of direct puncture of the thoracic duct (TD) under direct ultrasound (US) guidance at venous insertion in the left neck. MATERIALS AND METHODS: All patients who underwent attempted thoracic duct embolization (TDE) via US-guided retrograde TD access in the left neck were retrospectively reviewed. Indications for lymphangiography were iatrogenic chyle leak, pulmonary lymphangiectasia, and plastic bronchitis. Ten patients with mean age 41.4 years (range, 21 d to 72 y) underwent US-guided TD access via the left neck. Technical details, procedural times, and clinical outcomes were evaluated. TD access time was defined as time from start of procedure to successful access of TD, and total procedural time was defined from start of procedure until TDE. RESULTS: All attempts at TD access via the neck were successful. Technical and clinical success of TDE was 60%. There were no complications. Mean TD access time was 17 minutes (range, 2-47 min), and mean total procedure time was 49 minutes (range, 25-69 min). Mean follow-up time was 5.4 months (range, 3-10 months). CONCLUSIONS: TDE via US-guided access in the left neck is technically feasible and safe with a potential decrease in procedure time and elimination of oil-based contrast material.

Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA.

OBJECTIVES: To test the hypothesis that somatic phosphatidylinositol-4,5-bisphospate 3-kinase, catalytic subunit alpha (PIK3CA) mutations would be found in patients with more common disorders including isolated lymphatic malformation (LM) and Klippel-Trenaunay syndrome (KTS). STUDY DESIGN: We used next generation sequencing, droplet digital polymerase chain reaction, and single molecule molecular inversion probes to search for somatic PIK3CA mutations in affected tissue from patients seen at Boston Children's Hospital who had an isolated LM (n = 17), KTS (n = 21), fibro-adipose vascular anomaly (n = 8), or congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (n = 33), the disorder for which we first identified somatic PIK3CA mutations. We also screened 5 of the more common PIK3CA mutations in a second cohort of patients with LM (n = 31) from Seattle Children's Hospital. RESULTS: Most individuals from Boston Children's Hospital who had isolated LM (16/17) or LM as part of a syndrome, such as KTS (19/21), fibro-adipose vascular anomaly (5/8), and congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (31/33) were somatic mosaic for PIK3CA mutations, with 5 specific PIK3CA mutations accounting for ∼ 80% of cases. Seventy-four percent of patients with LM from Seattle Children's Hospital also were somatic mosaic for 1 of 5 specific PIK3CA mutations. Many affected tissue specimens from both cohorts contained fewer than 10% mutant cells. CONCLUSIONS: Somatic PIK3CA mutations are the most common cause of isolated LMs and disorders in which LM is a component feature. Five PIK3CA mutations account for most cases. The search for causal mutations requires sampling of affected tissues and techniques that are capable of detecting low-level somatic mosaicism because the abundance of mutant cells in a malformed tissue can be low.

Outcomes following splenectomy in patients with myeloid neoplasms.

BACKGROUND AND OBJECTIVES: Myeloid neoplasms are classified into five major categories. These patients may develop splenomegaly and require splenectomy to alleviate mechanical symptoms, to ameliorate transfusion-dependent cytopenias, or to enhance stem cell transplantation. The objective of this study was to determine which clinical variables significantly impacted morbidity, mortality, and survival in patients with myeloid neoplasms undergoing splenectomy, and to determine if operative outcomes have improved over time. METHODS: The records of all patients with myeloid neoplasms undergoing splenectomy from 1993 to 2010 were retrospectively reviewed. RESULTS: Eighty-nine patients (n = 89) underwent splenectomy for myeloid neoplasms. Over half of patients who had symptoms preoperatively had resolution of their symptoms post-splenectomy. The morbidity rate was 38%, with the most common complications being bleeding (14%) or infection (20%). Thirty-day mortality rate was 18% and median survival after splenectomy was 278 days. Decreased survival was associated with a diagnosis of myelodysplastic syndrome/myeloproliferative neoplasm, anemia, abnormal white blood cell count, and hypoalbuminemia. Patients who underwent stem cell transplantation did not show an increased risk for morbidity or mortality. CONCLUSIONS: Patients with myeloid neoplasms have a poor prognosis after splenectomy and the decision to operate is a difficult one, associated with high morbidity and mortality.

Value of surgical resection in children with high-risk neuroblastoma.

BACKGROUND: The value of gross total resection (GTR) for children with high-risk neuroblastoma (NB) is controversial. We hypothesized that patients undergoing GTR would demonstrate improved overall survival (OS) compared those having 90% resection compared to <90% resection (P = 0.008). Multivariable Cox models confirmed these findings with improved survival in children undergoing >90% vs. <90% resection but no difference in GTR vs. 90% resection is associated with improved OS compared to less than 90% resection.

Impact of Screening for Hepatic Hemangiomas in Patients with Multiple Cutaneous Infantile Hemangiomas.

OBJECTIVES: Hepatic hemangiomas are often found in association with multiple cutaneous infantile hemangiomas. Screening abdominal ultrasonography has been recommended for patients with five or more cutaneous lesions. We sought to determine whether hemangiomas found through screening had improved clinical outcomes. METHODS: Patients entered into our hepatic hemangioma registry between 1995 and 2012 were reviewed. RESULTS: Seventy-two patients with multiple cutaneous and hepatic hemangiomas were identified; 43 (60%) were detected through screening. The median age at diagnosis was 41 days for screened patients and 53 days for those not screened. Screening detected 40 (93%) multifocal and 3 (7%) diffuse hemangiomas, compared to 18 (62%) and 11 (38%), respectively, in the nonscreened group. Patients identified by screening had lower incidences of congestive heart failure and hypothyroidism and were less likely to receive treatment for their hemangiomas. The mortality rate in the children not screened was 28% (n = 8). None of the patients found by screening died (p < 0.001). Multivariate analysis of treated patients demonstrated that screening was a significant predictor of reduced mortality (p = 0.04). CONCLUSION: Hepatic hemangiomas found through screening ultrasonography are less likely to develop serious clinical sequelae. Although the reasons for this may include detection of hemangiomas that are less likely to progress to symptomatic disease, it appears that it also allows for earlier intervention for more concerning (e.g. diffuse) subtypes. Screening may allow for closer surveillance and earlier treatment before life-threatening progression in a subset of infants with liver hemangiomas, preventing complications and reducing mortality.

The impact of gestational age on targeted amniotic cell profiling in experimental neural tube defects.

PURPOSE: The proportions of select stem cells in term amniotic fluid have been shown to correlate with the type and size of experimental neural tube defects (NTDs). We sought to determine the impact of gestational age upon this form of targeted amniotic cell profiling. METHODS: Sprague-Dawley fetuses with retinoic acid-induced NTDs (n = 110) underwent amniotic fluid procurement at four time points in gestation. Samples were analyzed by flow cytometry for the presence of cells concomitantly expressing Nestin and Sox-2 (neural stem cells, aNSCs) and cells concomitantly expressing CD29 and CD44 (mesenchymal stem cells, aMSCs). Statistical analysis was by nonparametric Kruskal-Wallis ANOVA (p < 0.05). RESULTS: There was a statistically significant impact of gestational age on the proportions of both aMSCs (p = 0.01) and aNSCs (p < 0.01) in fetuses with isolated spina bifida. No such impact was noted in normal fetuses (p > 0.10 for both cells), in isolated exencephaly (p > 0.10 for both cells), or in combination defects (p > 0.10 for both cells). Gestational age had no effect on aNSC/aMSC ratios. CONCLUSIONS: Targeted quantitative amniotic cell profiling varies with gestational age in experimental isolated spina bifida. This finding should be considered prior to the eventual translation of this diagnostic adjunct into the prenatal evaluation of these anomalies. © 2014 S. Karger AG, Basel.

Pregnancy in Medical Training: A Survey of Physician Moms on Their Experiences During Residency and Fellowship.

OBJECTIVE: Resident physicians undergo physically and emotionally rigorous training; this is particularly difficult for the pregnant resident and affects their unborn child. This study aims to elucidate pregnant residents' perspectives regarding their prenatal and postnatal experiences, across all specialties, with a focus on pregnancy complications, postpartum health, and policy execution. DESIGN: This is a nationwide cross-sectional survey study developed to characterize resident and fellow perceptions about work schedules while pregnant, perceived discrimination, complications during pregnancy, lactation and lactation support, marital distress, parental leave policy, and overall satisfaction with the parental leave period. Descriptive statistics were used to characterize survey responses. SETTING/PARTICIPANTS: The experiences of physician mothers in online Facebook support groups: Physician Mom Group, Surgeon Mom Group, and Dr Mothers Interested in Lactation Knowledge, were queried by an electronic survey distributed using Qualtrics XM. Physicians who had children during their U.S. residency training were eligible to participate and 1,690 physician mothers from all specialties completed the survey. RESULTS: One thousand six hundred and ninety responses from members of the Facebook support groups were analyzed. Most surveyed physicians (1353/1519, 89.1%) were required to work until delivery and 63.6% (993/1561) of women took in-house calls during the last month of pregnancy. Half (820/1560, 52.6%) thought that the physical demands of their jobs compromised their own health and safety, or that of their child, and 1259 complications were reported among 1690 respondents, an average of three complications for every four respondents. Twenty-nine percent (442/1519, 29.1%) of physician mothers suffered from postpartum depression. Ninety-two percent (1479/1602, 92.3%) of respondents breastfed, but only one-third (483/1456, 33.2%) breastfed for more than 12 months and 52.7% (769/1458) would have liked to breastfeed longer. Marital distress was reported by nearly half (756/1650, 45.8%) of respondents during pregnancy and/or the first year of their child's life due to parental leave policies. The majority (957/1688, 56.7%) did not have a parental leave policy at their institution. Nearly two-thirds (946/1518, 62.3%) of respondents took 6 or fewer weeks off, and 79.7% (1211/1520) felt their duration of time off was inadequate. Nearly 30% (457/1593, 28.7%) stated they would recommend against a female medical student going into their field of medicine based upon their own experiences during pregnancy. CONCLUSIONS: Many mothers experienced discrimination from colleagues and worked until delivery despite concerns about the health and safety of themselves or their unborn children, and many reported experiencing a pregnancy-related complication. Most did not have a parental leave policy, which likely contributed to the disproportionately higher rates of postpartum depression among physician mothers compared to the general public. Residency training parental leave policies should be more accommodating to improve mental health, career satisfaction, and retention of the next generation of physician mothers.

Hospital Variation in Mortality and Failure to Rescue after Surgery for High-Risk Neonatal Diagnoses.

INTRODUCTION: A large proportion of postoperative mortality after pediatric surgery occurs among neonates with specific high-risk diagnoses. The extent to which there is hospital-level mortality variation among patients with these diagnoses and whether this variation is associated with differences in failure to rescue (FTR) is unclear. METHODS: The Pediatric Health Information System® database (2012-2020) was used to identify patients who underwent surgery for eight high-risk neonatal diagnoses: gastroschisis; volvulus; necrotizing enterocolitis; intestinal atresia; meconium peritonitis; tracheoesophageal fistula; congenital diaphragmatic hernia; and perinatal intestinal perforation. Hospitals were stratified into tertiles of reliability-adjusted inpatient mortality rates (lower than average mortality - tertile 1 [T1]; higher than average mortality - tertile 3 [T3]). Multivariable hierarchical regression was used to evaluate the association between hospital-level, reliability-adjusted mortality and FTR. RESULTS: Overall, 20,838 infants were identified across 48 academic, pediatric hospitals. Adjusted hospital mortality rates ranged from 4.0% (95% CI, 0.0-8.2) to 16.3% (12.2-20.4). Median case volume (range, 80-1,238) and number of NICU beds (range, 24-126) were not significantly different across hospital tertiles. Compared to the hospitals with the lowest postoperative mortality (T1), the odds of FTR were significantly higher in hospitals with the highest (T3) postoperative mortality (odds ratio 1.97 [1.50-2.59]). CONCLUSIONS: Significant variation in neonatal hospital mortality for high-risk diagnoses does not appear to be explained by hospital structural characteristics. Rather, difference in FTR suggests quality improvement interventions targeting early recognition and management of postoperative complications could improve surgical quality and safety for high-risk neonatal care.

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.

Management of Gastroschisis: Timing of Delivery, Antibiotic Usage, and Closure Considerations (A Systematic Review From the American Pediatric Surgical Association Outcomes & Evidence Based Practice Committee).

BACKGROUND: No consensus exists for the initial management of infants with gastroschisis. METHODS: The American Pediatric Surgical Association (APSA) Outcomes and Evidenced-based Practice Committee (OEBPC) developed three a priori questions about gastroschisis for a qualitative systematic review. We reviewed English-language publications between January 1, 1970, and December 31, 2019. This project describes the findings of a systematic review of the three questions regarding: 1) optimal delivery timing, 2) antibiotic use, and 3) closure considerations. RESULTS: 1339 articles were screened for eligibility; 92 manuscripts were selected and reviewed. The included studies had a Level of Evidence that ranged from 2 to 4 and recommendation Grades B-D. Twenty-eight addressed optimal timing of delivery, 5 pertained to antibiotic use, and 59 discussed closure considerations (Figure 1). Delivery after 37 weeks post-conceptual age is considered optimal. Prophylactic antibiotics covering skin flora are adequate to reduce infection risk until definitive closure. Studies support primary fascial repair, without staged silo reduction, when abdominal domain and hemodynamics permit. A sutureless repair is safe, effective, and does not delay feeding or extend length of stay. Sedation and intubation are not routinely required for a sutureless closure. CONCLUSIONS: Despite the large number of studies addressing the above-mentioned facets of gastroschisis management, the data quality is poor. A wide variation in gastroschisis management was documented, indicating a need for high quality RCTs to provide an evidence-based approach when caring for these infants. TYPE OF STUDY: Qualitative systematic review of Level 1-4 studies.

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.

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.

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.