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Background: Evaluation of response to blood transfusion after blunt splenic injury (BSI) may prevent the need for splenectomy. The aim of this study was to evaluate factors associated with splenectomy in pediatric patients with isolated BSI who presented with hemodynamic instability with a focus on timing of transfusion., Methods: The 2021 Trauma Quality Improvement Project database was queried for children ≤18 years with BSI who arrived with a shock index>1.1. Interfacility transfer patients and those with additional intra-abdominal injuries were excluded. Demographic, injury characteristic and timing, transfusion, operative, and outcome data were collected. A sub-analysis of patients without brain injury was also performed., Results: 516 patients met inclusion criteria; 60.1% were male, with mean age 12.3 ± 5.5 years. Initial mean shock index was 1.4 ± 0.4, ISS was 31.7 ± 15.1, and GCS was 10.7 ± 5. Splenectomy occurred in 27% of patients. Among splenectomy patients, 26.2% did not receive blood prior to splenectomy. While treatment at a pediatric trauma center showed an increased OR of splenectomy in univariable analysis, when controlling for lack of transfusion, no differences in splenectomy persisted. Patient Age (aOR-1.26, p < 0.001), BSI grade (aOR-2.30, P < 0.001), male gender, (aOR-2.2, p = 0.003), being non-white (aOR-2.0) ISS (aOR-1.03, p = 0.003), and GCS (aOR-0.95, p = 0.034) were associated with splenectomy., Conclusion: More than 26% of patients undergoing splenectomy did not receive blood prior to surgery. Differences in risk of splenectomy by center type seen on univariable analysis were not seen when controlling for transfusion. Evaluating response to blood transfusion may be an opportunity to reduce the frequency of splenectomy., Level of Evidence: Treatment Study Level III., (Copyright © 2024. Published by Elsevier Inc.)

Background: Women have historically been underrepresented in surgical leadership and in specialty organizations. This study longitudinally examines representation of women across committee leadership within the American Pediatric Surgical Association (APSA)., Methods: Annual APSA committee chair and vice-chair rosters (2003-2023) were obtained. If not self-reported in the membership roster, gender was classified based upon review of publicly available data. Proportions of women who served as chairs and vice-chairs were quantified by committee and by year., Results: Overall, the proportion of women serving as APSA committee chairs and vice-chairs increased from 11% to 48% during the study period (p = 0.001). In examining each position, the proportion of women chairs increased from 12% to 40% (p = 0.042), while women vice-chairs increased from 10% to 58% (p = 0.009). The committees with the highest cumulative proportion of women chairs were Benjy Brooks (100%), Diversity Equity & Inclusion (100%), Finance (100%), Global Pediatric Surgery (67%), and Wellness (67%). Four committees- Trauma, Access to Surgery for Kids, Practice, and Professional Development - were not led by a woman chair in the entire study period. Additionally, five committees that traditionally have had significant impact on organizational workflow and agendas all had cumulative proportions of women chairs of less than 50% - Education (33%), Publications (28.6%), Outcomes (19%), Surgical Quality & Safety (18.8%) and Program (9.5%)., Conclusion: These results demonstrate encouraging trends in the gender diversity of APSA leadership. However, this progress does not appear to be evenly distributed; leadership of key committees continues to lack substantial women's representation., Level of Evidence: III., Competing Interests: Conflicts of interest The authors have no disclosures or competing interests., (Copyright © 2024 Elsevier Inc. All rights reserved.)

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., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: There is wide variation in the language used to describe Mullerian structures. To standardize terminology, the American Society of Reproductive Medicine (ASRM) created the Mullerian Anomalies Classification (MAC) in 2021. The objective of this study was to evaluate the applicability of the MAC nomenclature to pediatric patients with cloaca., Methods: A retrospective review of all patients with cloaca was performed at a single institution. Descriptions of Mullerian structures were evaluated and compared to the ASRM MAC categories. Descriptive statistics were used to report findings., Results: 36 patients with cloaca were identified, 13 (36%) of whom had congenital Mullerian structures that could not be adequately described by the MAC terminology. All 13 patients had two hemiuteri that were not connected in the midline and were not accurately described as uterus didelphys. Additionally, 5 of these 13 patients had reproductive anatomy that was connected by a fistula or ectopic connection to other pelvic structures., Conclusion: Despite the ASRM expansion of the Mullerian anomalies nomenclature, more than a third of our patients with cloaca could not have their Mullerian structures accurately described. Describing anatomy with accurate and consistent language can improve communication between healthcare providers and may allow patients and families to better anticipate fertility options., Study Type: Retrospective., Level of Evidence: IV., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population., Methods: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher's exact tests., Results: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005)., Conclusion: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE., Level of Evidence: III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: Heterotaxy syndrome (HS) affects right-left anatomical development in 3% of children with congenital heart disease. Commonly, these patients have intestinal rotation abnormalities (IRA) that differ from typical malrotation. In this prospective study, we examine the development of a management pathway, imaging findings, and clinical course of patients with HS and IRA., Methods: After literature review, a multispecialty focus group designed a pathway for HS. Participants from a single institution were prospectively enrolled from 2016 to 2019. They underwent an abdominal ultrasound and UGI, however timing was variable based on symptoms. Symptomatic IRA was defined as feeding intolerance, bilious or non-bilious vomiting, bloating, severe reflux, and intermittent pain or abdominal distention. Screening for symptoms occurred every three months for the first two years and then annually., Results: 18 participants were followed for a median of 5.0 years. Three clinical categories were identified: 1) asymptomatic, not requiring intestinal surgery, 72.2%, 2) symptomatic with feeding intolerance or failure to thrive requiring gastrostomy placement and diagnostic laparoscopy with Ladd procedure, 16.7%, and 3) symptomatic requiring urgent exploration for suspicion of volvulus, 11.1%. Need for surgery did not necessarily correlate with US and/or UGI findings., Conclusions: In children with HS and IRA, postnatal imaging did not inform the need for intestinal surgery. Although rare, volvulus or other forms of proximal obstruction can be anticipated, and symptomatic patients should be offered surgical intervention. A multidisciplinary care pathway for patients with HS and IRA can coordinate care and help counsel families on the likelihood of needing intestinal surgery for IRA., Level of Evidence: Level II., Type of Study: Prospective Cohort Study., Competing Interests: Conflict of interest The authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: The objective of this study is to assess the postoperative outcomes of single-stage repair of anorectal malformations with vestibular (VF) or perineal fistula (PF) and early initiation of postoperative feeding., Methods: A retrospective review of patients undergoing single-stage repair of isolated low anorectal malformations (VF and PF) from 2017 to 2020 was conducted. All patients underwent an anterior anoplasty with complete mobilization of the rectal fistula, or posterior sagittal anorectoplasty (PSARP), without protective colostomy. The variables examined include age, timing of postoperative feeding initiation, length of stay (LOS), and complications., Results: Nineteen patients with VF or PF underwent a single-stage repair. 12/19 (63%) patients were female. All 7 males and 9/12 females had a PF. The range of age at surgery was 2 days to 3 years with median age of 92 days [IQR 1,3: 9,193]. The median postoperative day for initiation of feeds was day 0 [IQR 1,3: 0,1] and median LOS was 1 day [IQR 1,3: 1,4.5]. 18/19 (95%) patients were evaluated in follow-up and there were no wound infections, wound dehiscences, or recurrent fistulas. Within 90 days postoperatively, no patients were seen in the emergency department for postoperative issues. Within 6 months, 2/19 (11%) patients required an unplanned return to the operating room for anal dilation., Conclusion: In single-stage repair of isolated low anorectal malformations, VF and PF, early initiation of postoperative feeding is safe, results in a short length of stay, and does not lead to increased wound complications. Early enteral feeding eliminates the need for parenteral nutrition and central venous access, and their associated complications., Level of Evidence: Level IV., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2022. Published by Elsevier Inc.)

Purpose: Fecal continence is a concern for many patients with idiopathic constipation and can significantly impact quality of life. It is unknown whether racial, ethnic, and socioeconomic disparities are seen in fecal continence within the idiopathic constipation population. We aimed to evaluate fecal continence and associated demographic characteristics in children with idiopathic constipation referred for surgical evaluation., Methods: A multicenter retrospective study of children with idiopathic constipation was performed at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). All patients >3y of age with idiopathic constipation diagnosis were included. The primary outcome was fecal continence, categorized as complete (no accidents ever), daytime (no accidents during the day), partial (occasional incontinence day/night), and none (incontinent). We evaluated for associations between fecal continence and race, sex, age, insurance status, and other patient-level factors, employing Kruskal-Wallis and trend tests., Results: 458 patients with idiopathic constipation from 12 sites were included. The median age of diagnosis was 4.1 years. Only 25% of patients referred for surgical evaluation were completely continent. Age at the visit was significantly associated with fecal continence level (p = 0.002). In addition, patients with public and mixed public and private insurance had lower levels of continence (p<0.001). Patients with developmental delay were also more likely to have lower continence levels (p = 0.009) while diagnoses such as anxiety, ADD/ADHD, autism, depression, obsessive-compulsive disorder were not associated. Approximately 30% of patients had an ACE operation (antegrade continence enema) at a median age of 9.2 years at operation. Black patients were significantly less likely to undergo ACE operation (p = 0.016) when compared to white patients., Conclusion: We observed data that suggest differences in fecal incontinence rates based on payor status. Further investigation is needed to characterize these potential areas of disparate care., Level of Evidence: Level III., (Published by Elsevier Inc.)

Introduction: Children with severe constipation and a neurodiverse disorder (Autism and/or developmental delay) represent a challenging bowel management group. Treatment outcomes with laxative or enema therapy remain limited and are often complicated by patient/caregiver compliance. We hypothesized that children with neurodiverse disorders and severe constipation would benefit from a bowel management program (BMP) that includes early use of antegrade enemas., Materials and Methods: Children requiring BMP in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry with diagnosis of constipation and/or constipation with pseudo-incontinence were reviewed. Those with Hirschsprung disease and/or Anorectal Malformation were excluded. BMP needs in patients with a neurodiverse diagnosis were compared to those without to evaluate differences in BMP's., Results: 372 patients requiring a BMP were identified. 95 patients (58 autism spectrum, 54 developmental delay) were neurodiverse, and 277 patients were not. Neurodiverse patients had a higher prevalence of enema therapy 62.1% (59/95) vs. 54.9% (152/273) and use of antegrade enema therapy 33.7% (32/95) vs. 21.2% (58/273), p = 0.126. Neurodiverse patients were older 37.9% (36/95) > 12 years vs. 23.1% (63/273), p = 0.001 and 47.6% (10/21) were changed from laxative to enema therapy over time. 80% (8/10) of those changed from laxatives to enemas used antegrade therapy. 67.3% (35/52) of neurodiverse patients followed over time were on enema therapy at the most recent visit with 80% (28/35) requiring antegrade therapy., Conclusion: A large portion of patients with a neurodiverse disorder who fail laxative therapy use antegrade enemas to achieve effective bowel management. Early consideration of an antegrade conduit may simplify treatment in this group of children., Level of Evidence: III., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background/purpose: Primary pull-through via a perineal approach (PA) has recently been reported for Hirschsprung's disease. One criticism of this approach is that it requires a large amount of retraction on the anal sphincters. Additionally, because the procedure is new, most patients undergoing a PA are too young to assess long-term continence rates. This study examined early stooling patterns, anal sphincteric pressures, and number of enterocolitic episodes in infants who underwent a PA. Results were compared with a conventional combined transabdominal and perineal approach (TA)., Methods: Over 2 years, 26 pull-through procedures were performed. Nine of those were PA, and 17 were TA. Twelve of the 26 patients had formal manometric studies postoperatively. Results are expressed as mean +/- SD; unpaired t test and Chi;(2) were used for statistical analysis., Results: Mean follow-up post-pull-through was 23 +/- 2.3 months for the TA and 14 +/- 1.9 months for the PA. Manometric resting sphincter pressure in the TA group averaged 79 +/- 17 mm Hg compared with 76 +/- 21 mm Hg in the PA group (P =.78). Number of stools per day was 3.3 +/- 0.6 in the TA group compared with 2.2 +/- 0.3 in the PA group (P =.17). Post-pull-through enterocolitis was experienced by 53% of the TA group (mean, 1.5 +/- 0.6 episodes) and 56% of the PA group (mean 0.9 +/- 0.4 episodes) for a P =.08 by Chi;(2) analysis., Conclusions: Manometric sphincter pressure and enterocolitic episodes after a PA for Hirschsprung's disease appear to be similar to results obtained with a conventional TA. Evaluation of early stooling frequency shows a comparable frequency after a PA. This suggests that both methods are safe, and the PA does not appear to compromise sphincter integrity., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Background/purpose: The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome., Methods: From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-through at another hospital and 2 patients with an unsuccessful pull-through from C.S. Mott. All redo pull-throughs (n = 19) were performed in these patients, and their clinical courses are reviewed., Results: Twelve patients required reoperation secondary to a mechanical problem with their first pull-through. The other 7 patients had evidence of residual segments of dilated colon leading to functional failure of their initial operation including 5 patients with documented aganglionic bowel present at the second pull-through. Ten of the patients requiring reoperation initially had an endorectal pull-through (ERPT), 5 had a Duhamel procedure, 3 had a Swenson procedure, and 1 had a Rehbein procedure. Choice of revision was an ERPT in 8 patients in whom an adequate rectal cuff could be developed. Additional redo procedures included a Duhamel in 8 patients and a Swenson in 3 patients. Follow-up ranges from 3 months to 23 years (mean, 13.8 years). There were no deaths in the series, and 1 patient required a third pull-through. All patients who are not neurologically impaired and are over age 3 are continent except one (94%). Stools per day range from 1 to 10 (mean, 3.2)., Conclusions: Redo pull-through operations for Hirschsprung's disease appear to be as effective as primary procedures in terms of continence and stooling frequency. Distinct from other series, we found an ERPT to be the procedure of choice if an adequate rectal cuff was present.

Background/purpose: Regression of a cystic adenomatoid malformation (CAM) in a fetus is well described. Little, however, is known about the postnatal course of these infants. This study attempts to correlate the prenatal course of CAMs with postnatal symptoms, radiological manifestations, and need for surgery., Methods: The clinical course of patients with a CAM diagnosed prenatally were retrospectively reviewed. Inclusion in the study required a prenatal ultrasound scan documenting a CAM., Results: Over 10 years, 14 patients with a CAM were diagnosed prenatally. Six (43%) showed a partial in utero regression. Four patients were symptomatic at birth and underwent a resection as newborns. Ten patients were asymptomatic at birth, and eight of these had normal chest x-rays. Elective resection has been performed in 3 of these 10, and two additional children are scheduled to undergo an excision near 1 year of age. The remaining five patients have undergone follow-up nonoperatively for a mean of 36 +/- 15 months. Of the seven asymptomatic patients not undergoing immediate surgery, only one has shown a slight postnatal regression, despite five of these showing regression in utero. None have become symptomatic., Conclusions: The results suggest that regression of a CAM on prenatal ultrasound scan is common, but this process does not continue after birth. A normal chest x-ray does not indicate complete regression of a CAM; a computed tomography (CT) scan is required to evaluate such patients, and will generally demonstrate a CAM. Asymptomatic patients with a CAM may be followed up nonoperatively with no apparent adverse effects. The decision and timing of an excision in an asymptomatic patient remains controversial among pediatric surgeons.