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Antegrade colonic enemas (ACE) can be an effective management option for defecation disorders and improve quality of life. Best practice regarding channel placement is unclear and variation may exist around preferred initial type of channel, age at placement, and underlying diagnoses. We aimed to describe practice patterns and patient characteristics around ACE channel placement. We conducted a multicenter retrospective study of children with an ACE channel cared for at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) from 2017 to 2022. Kruskal–Wallis test was utilized to test the age at surgery by site with significance level of 0.05. 500 patients with ACE channel were included. 293 (58.6%) patients had their ACE procedure at a PCPLC center. The median age at surgery was 7.6 [IQR 5.3–11.0] years for the overall cohort and 8.1 [IQR 5.3–11.5] years for placement at PCPLC centers. For PCPLC centers, median age at placement varied significantly across centers (p = 0.009). 371 (74.2%) patients received Malone appendicostomy, 116 (23.2%) received cecostomy, and 13 (2.6%) received Neo-Malone appendicostomy. Median age of patients by channel type was 7.7 [IQR 5.3–11.0], 7.5 [IQR 5.7–11.0], and 9.8 [IQR 4.2–11.6] years, respectively. The most common indication for cecostomy was idiopathic/refractory constipation (52.6%), whereas anorectal malformation was the most common indication for Malone (47.2%) and Neo-Malone (61.5%). Among ACE channels placed at PCPLC centers, there was variation across institutions in preferred initial channel type. The 4 highest volume centers favored Malone appendicostomy over cecostomy. There is variation in practice of ACE channel placement. At specialty pediatric colorectal centers, age at time of placement and type of channel placed varied across institutions. Further work is needed to better characterize diagnosis- and age-focused patient centered outcomes to clarify recommendations for our patients who benefit from these procedures. Retrospective comparative study. Level III. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. III. [ABSTRACT FROM AUTHOR]

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. 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). 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). 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. III [ABSTRACT FROM AUTHOR]

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. 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. 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 %. 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. V. [ABSTRACT FROM AUTHOR]

The incidence and optimal management of rectal prolapse following repair of an anorectal malformation (ARM) has not been well-defined. A retrospective cohort study was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium registry. All children with a history of ARM repair were included. Our primary outcome was rectal prolapse. Secondary outcomes included operative management of prolapse and anoplasty stricture following operative management of prolapse. Univariate analyses were performed to identify patient factors associated with our primary and secondary outcomes. A multivariable logistic regression was developed to assess the association between laparoscopic ARM repair and rectal prolapse. A total of 1140 patients met inclusion criteria; 163 (14.3%) developed rectal prolapse. On univariate analysis, prolapse was significantly associated with male sex, sacral abnormalities, ARM type, ARM complexity, and laparoscopic ARM repairs (p < 0.001). ARM types with the highest rates of prolapse included rectourethral-prostatic fistula (29.2%), rectovesical/bladder neck fistula (28.8%), and cloaca (25.0%). Of those who developed prolapse, 110 (67.5%) underwent operative management. Anoplasty strictures developed in 27 (24.5%) patients after prolapse repair. After controlling for ARM type and hospital, laparoscopic ARM repair was not significantly associated with prolapse (adjusted odds ratio (95% CI): 1.50 (0.84, 2.66), p = 0.17). Rectal prolapse develops in a significant subset of patients following ARM repair. Risk factors for prolapse include male sex, complex ARM type, and sacral abnormalities. Further research investigating the indications for operative management of prolapse and operative techniques for prolapse repair are needed to define optimal treatment. Retrospective cohort study. II. [ABSTRACT FROM AUTHOR]

A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients. Retrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale. There were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD. More than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment. Level 4 • What is currently known about this topic? HD-IBD is rare with approximately 2% incidence based on previous studies. • What new information is contained in this article? - Risk factors for HD-IBD may include long segment disease, trisomy 21, and previous episodes of HAEC. - HD-IBD can be found in patients younger than 5 years old but often occurs many years after pullthrough surgery. - Biological agents are the most effective pharmacological treatment for HD-IBD. - Almost 30% of HD-IBD required surgical treatment for IBD. [ABSTRACT FROM AUTHOR]

• What does this study add to the literature (required). • What is currently known about this topic? A large number of patients with cloacal malformation have associated gynecologic anomalies. • What new information is contained in this article? Girls with less complex ARM subtypes (such as rectoperineal and rectovestibular fistulas) have a high incidence of gynecologic anomalies. This incidence is significantly increased in the presence of a VACTERL association. VACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM). This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The 834 female patients with ARM who were enrolled in the registry by January 1, 2020 were included in this study. The relationship of VACTERL association with presence of a gynecologic anomaly was evaluated with Fisher's exact test. The relationship of each VACTERL system with presence of a gynecologic anomaly was assessed in patients with cloaca, rectovestibular fistulas and rectoperineal fistulas. P-values reported were based on a 2-sided alternative and considered significant when less than 0.05. 834 patients with ARM underwent VACTERL screening and gynecologic evaluation with the three most common subtypes being cloaca (n = 215, 25.8%), rectovestibular fistula (n = 191, 22.9%) and rectoperineal fistula (n = 194, 23.3%). A total of 223 (26.7%) patients with ARM had gynecologic anomalies. VACTERL association was seen in 380 (45.6%) of patients with ARM. Gynecologic anomalies were present in 149 (39.1%) vs. 74 (16.3%) of subjects with vs. without VACTERL association (p < 0.001). VACTERL association did not significantly increase the risk of gynecologic anomaly in patients with cloaca and VACTERL (n = 88, 61.5%) vs. cloaca without VACTERL (n = 39, 54.2% p = 0.308). VACTERL association increased the risk of gynecologic anomalies in patients with rectoperineal fistulas (n = 7, 14.9% vs n = 9, 6.1% p = 0.014) and rectovestibular fistulas (n = 19, 31.1% vs. n = 13, 10.0% p <0.001). In patients with ARM who had a VACTERL association, when one of the associated anomalies was renal, there was an even higher risk of having an associated gynecologic anomaly (n = 138, 44.2% vs. n = 85, 16.3% p <0.001). VACTERL association in patients with rectoperineal and rectovestibular fistulas correlates with an increased risk of gynecologic anomalies. The presence of VACTERL associated findings, especially renal, should prompt a thorough evaluation of the gynecologic system. III. Retrospective comparative study [ABSTRACT FROM AUTHOR]

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. 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. 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. 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 III [ABSTRACT FROM AUTHOR]

Benign mesothelial cysts are rare, and to our knowledge there are no reported cases of benign mesothelial cysts in patients with a history of gastroschisis. A 15-year-old girl with a history of complicated gastroschisis and multiple operations as a child presented to the emergency department with back pain and abdominal bloating that worsened over the course of a year. At the time of presentation, she was febrile, but her basic blood tests were within normal limits. Imaging studies including an abdominal ultrasound, a computed tomography of the abdomen, and a magnetic resonance imagining of the abdomen demonstrated a 31× 21 × 11cm cystic abdominopelvic lesion, the origin of which was unclear. Tumor markers including alpha fetoprotein, CA 125, CA 19–9, beta HCG, carcinoembryonic antigen, inhibin A/B, and lactate dehydrogenase were normal. The fluid was sampled by needle aspiration. Amber fluid with mostly macrophages (72 %) and was obtained. A pigtail catheter was placed, and 2500 ml of clear yellow/brown fluid were drained. She was sent home with the drain and was scheduled for an elective cyst excision but presented to the hospital with a bowel obstruction a few days later. She underwent an extensive laparoscopic lysis of adhesions, drain removal, and partial excision of the cyst wall. The pathology analysis was consistent with a benign mesothelial cyst. No new drains were placed. She recovered well from the operation. At 3 months of follow up she is doing well and has no recurrence of the cyst by ultrasound. She will continue to be monitored with periodic ultrasounds. While rare, benign mesothelial cysts should be on the differential diagnosis of patients with large abdominal cysts, particularly if they have a history of prior abdominal operations. [ABSTRACT FROM AUTHOR]

Sacrococcygeal teratomas (SCTs) and jejunoileal atresias are both rare (1 in 35,000–40,000 births and 1 in 5000 births, respectively). We present a case of a concomitant diagnosis of a cystic sacrococcygeal teratoma and a jejunoileal atresia. A twenty-week screening ultrasound identified a cystic pelvic mass (5.5 x 2.8 × 2.2 cm), dilated bowel, and mild polyhydramnios in an otherwise healthy G1P0 woman. Fetal magnetic resonance image showed a multiseptated presacral cystic mass suspicious for an internal sacrococcygeal teratoma and dilated bowel, thought to represent dilated ascending and transverse colon. Scheduled delivery at 39 weeks of gestation was uncomplicated. Postnatal ultrasound and MRI were consistent with prenatal findings. Given the rarity of both congenital anomalies and no known association between SCTs and jejunoileal atresia, the prenatal bowel dilation was thought to be due to partial obstruction secondary to the SCT. The baby thus underwent SCT resection on day-of-life 2. Ongoing obstruction after resection prompted a contrast enema which demonstrated a microcolon with reflux into the terminal ileum but no reflux into more dilated proximal loops concerning for intestinal atresia. On day-of-life 5, the baby underwent exploratory laparotomy which identified a type-2 ileal atresia, and a primary anastomosis was performed. The patient had an uneventful recovery and was discharged home on day-of-life 22. Prenatal findings of dilated bowel should prompt providers to have a high index of suspicion for jejunoileal atresia, even if other diagnoses exist. [ABSTRACT FROM AUTHOR]

• Myelomeningocele is associated with significant bowel and bladder dysfunction. • Fetal ovine myelomeningocele repair augmented with placental mesenchymal stem cells was associated with near-normal bowel and bladder function. Fetal myelomeningocele (MMC) repair improves lower extremity motor function. We have previously demonstrated that augmentation of fetal MMC repair with placental mesenchymal stromal cells (PMSCs) seeded on extracellular matrix (PMSC-ECM) further improves motor function in the ovine model. However, little progress has been made in improving bowel and bladder function, with many patients suffering from neurogenic bowel and bladder. We hypothesized that fetal MMC repair with PMSC-ECM would also improve bowel and bladder function. MMC defects were surgically created in twelve ovine fetuses at median gestational age (GA) 73 days, followed by defect repair at GA101 with PMSC-ECM. Fetuses were delivered at GA141. Primary bladder function outcomes were voiding posture and void volumes. Primary bowel function outcome was anorectal manometry findings including resting anal pressure and presence of rectoanal inhibitory reflex (RAIR). Secondary outcomes were anorectal and bladder detrusor muscle thickness. PMSC-ECM lambs were compared to normal lambs (n = 3). Eighty percent of PMSC-ECM lambs displayed normal voiding posture compared to 100% of normal lambs (p = 1). Void volumes were similar (PMSC-ECM 6.1 ml/kg vs. normal 8.8 ml/kg, p = 0.4). Resting mean anal pressures were similar between cohorts (27.0 mmHg PMSC-ECM vs. normal 23.5 mmHg, p = 0.57). RAIR was present in 3/5 PMSC-ECM lambs that underwent anorectal manometry and all normal lambs (p = 0.46). Thicknesses of anal sphincter complex, rectal wall muscles, and bladder detrusor muscles were similar between cohorts. Ovine fetal MMC repair augmented with PMSC-ECM results in near-normal bowel and bladder function. Further work is needed to evaluate these outcomes in human patients. [ABSTRACT FROM AUTHOR]

While fecal incontinence is a primary concern for many children with anorectal malformations (ARM), urinary incontinence is also prevalent in this population. Racial, ethnic, and socioeconomic disparities in urinary continence have been observed in other conditions, but have not been previously evaluated in ARM. We aimed to evaluate urinary continence and associated demographic and socioeconomic characteristics in individuals with ARM. We performed a multicenter retrospective study of ARM patients evaluated at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). We included all patients with ARM 3 years and older. The primary outcome was urinary continence which was categorized as complete (no accidents), daytime (accidents at night), partial (rare or occasional accidents), and none (frequent accidents or no continence). We evaluated for associations between urinary continence and race, sex, age, insurance status, and adoption status, employing Kruskal-Wallis and trend tests. Secondary outcomes included bladder management strategies such as clean intermittent catheterization and continence surgery. P-value < 0.05 was considered significant. A total of 525 patients with ARM were included. Overall, 48% reported complete urinary continence, and continence was associated with greater age. For school-aged children (age ≥ 5 years), 58% reported complete continence, while 30% reported none. Public insurance and adoption status were associated with decreased likelihood of incontinence. We observed a novel finding of disparities in urinary continence for children with ARM related to insurance and adoption status. Further investigation regarding the etiologies of these inequities is needed in order to affect clinical outcomes. [ABSTRACT FROM AUTHOR]

There is little information on the effects of Pseudomonas infection on outcomes in perforated appendicitis. As Pseudomonas is not covered by many empiric appendicitis antibiotic regiments, we hypothesized that children with Pseudomonas would have worse outcomes. Patients <18 years old undergoing appendectomy for perforated appendicitis at a tertiary children's hospital 2015–2019 were included and were stratified by presence of Pseudomonas on intraoperative culture. The primary outcome was post-operative organ-space infection (SSI). Intraoperative cultures were collected in 58.4% of patients (n = 149/255) with 22.2% (n = 33) positive for Pseudomonas. SSIs occurred in 21.2% of children with Pseudomonas compared to 20.7% of children without Pseudomonas (p = 0.9). Children with Pseudomonas had longer antibiotic duration (9.1 vs. 6.7 days, p = 0.03) and LOS (6.7 vs. 5.9 days, p = 0.03) than those without, but a similar rate of post-operative interventions (12.2% vs. 19.0%, p = 0.4), hospital costs ($28,860 vs. $23,945, p = 0.3), ED visits (9.1% vs. 19.9%, p = 0.3), and readmissions (9.1% vs. 9.5%, p = 1). Pseudomonas was identified in 22% children with perforated appendicitis and was associated with longer antibiotic durations and LOS, but similar rates of SSI, post-operative interventions, and readmissions compared to children without Pseudomonas. Empiric coverage of Pseudomonas may not be necessary. [ABSTRACT FROM AUTHOR]

Our multi-institutional university consortium implemented a gastroschisis pathway in 2015 to standardize and improve care by promoting avoidance of routine intubation and paralysis during silo placement, expeditious abdominal wall closure, discontinuation of antibiotics/narcotics within 48 h of closure, and early initiation/advancement of feeds. Adherence to the gastroschisis pathway was prospectively monitored. Outcomes for the contemporary cohort (2015–2018) were compared with a historical cohort (2007–2012). Good adherence to the pathway was observed for 70 cases of inborn uncomplicated gastroschisis. The contemporary cohort had significantly lower median mechanical ventilator days (2 versus 5; p < 0.01) and antibiotic days (5.5 versus 9; p < 0.01) as well as earlier days to initiation of feeds (12 versus 15; p < 0.01). However, no differences were observed in length of stay (28 versus 29 days; p = 0.70). A skin closure technique was performed in 66% of the patients, of which 46% were performed at bedside without intubation, the assistance of an operating-room team, or general anesthesia. In this study, adherence to a clinical pathway for gastroschisis across different facilities was feasible and led to reduction in exposure to mechanical ventilation and antibiotics. The adoption of a bedside skin closure technique appears to facilitate compliance with the pathway. Level II/III Prospective comparative study with historical cohort [ABSTRACT FROM AUTHOR]

Many management options exist for the treatment of refractory rectal prolapse (RP) in children. Our goal was to characterize current practice patterns among active members of APSA. A 23-item questionnaire assessed the management of full-thickness RP for healthy children who have failed medical management. The survey was approved by our IRB and by the APSA Outcomes committee. 236 surgeons participated. The respondents were geographically dispersed (44 states, 5 provinces). 32% of respondents had twenty or more years of clinical experience. 71% evaluated 1–5 RP patients in the last 2 years, while 5% evaluated > 10. 71% performed 0–1 procedure (operation or local therapy [LT]) for RP over 2 years. 59% would treat a 2-year-old patient differently than a 6-year-old with the same presentation, and were more likely to offer up-front surgery to a 6-year-old (26% vs 15%, p = 0.04), less likely to continue medical management indefinitely (2% vs 7%, p = 0.01), and more likely to perform resection with rectopexy (30% vs. 15%, p = 0.01). 71% perform LT as an initial intervention: injection sclerotherapy (59%), anal encirclement (8%), and sclerotherapy + anal encirclement (5%). 70% consider LT a failure after 1–3 attempts. If LT fails, surgical management consists of transabdominal rectopexy (46%), perineal proctectomy or proctosigmoidectomy (22%), transabdominal sigmoidectomy + rectopexy (22%), and posterior sagittal rectopexy (9%). There is wide variability in the surgical management of pediatric rectal prolapse. This suggests a need for development of processes to identify best practices and optimize outcomes for this condition. [ABSTRACT FROM AUTHOR]

Abstract Background Although the incidence of gastroschisis is increasing, risk factors are not clearly identified. Methods Using the Linked Birth Database from the California Office of Statewide Health Planning and Development from 1995 to 2012, patients with gastroschisis were identified by ICD-9 diagnosis/procedure code or birth certificate designation. Logistic regressions examined demographics, birth factors, and maternal exposures on risk of gastroschisis. Results The prevalence of gastroschisis was 2.7 cases per 10,000 live births. Patients with gastroschisis had no difference in fetal exposure to alcohol (p = 0.609), narcotics (p = 0.072), hallucinogenics (p = 0.239), or cocaine (p = 0.777), but had higher exposure to unspecified/other noxious substances (OR 3.27, p = 0.040; OR 2.02, p = 0.002). Gastroschisis was associated with low/very low birthweight (OR 5.08–16.21, p < 0.001) and preterm birth (OR 3.26–10.0, p < 0.001). Multivariable analysis showed lower risk in black (OR 0.44, p < 0.001), Asian/Pacific Islander (OR 0.76, p = 0.003), and Hispanic patients (OR 0.72, p < 0.001) compared to white patients. Risk was higher in rural areas (OR 1.24–1.76, p = 0.001). Compared to women age < 20, risk decreased with advancing maternal age (OR 0.49-OR 0.03, p < 0.001). Patients with gastroschisis had increased total charges ($336,270 vs. $9012, p < 0.001) and length of stay (38.1 vs. 2.9 days, p < 0.001). Mortality was 4.6%. Conclusions This is the largest population-based study summarizing current epidemiology of gastroschisis in California. Type of study Retrospective comparative cohort study. Level of evidence III. [ABSTRACT FROM AUTHOR]

Abstract A 10-year-old girl presented to her pediatrician with a history of cough and fever. A chest radiograph revealed a paraspinal mass. On cross-sectional imaging, the mass traversed the diaphragm, extending from T9 to L1 spinal levels with involvement of the T10-12 neural foramen. Vanillylmandelic and homovanillic acid levels were normal. On review of historical radiographs, the mass had increased in size. Thus, surgical resection was recommended for diagnosis and treatment. The patient was placed in left lateral decubitus position. The retroperitoneal space was accessed inferior to the twelfth rib. One 12 mm and two 5 mm ports were used. Development of the retroperitoneal space was achieved with both blunt dissection and a vessel-sealing device. The diaphragm was incised to resect the thoracic component of the mass. The tumor was adherent at the neural foramen and was resected flush with the spine. The diaphragm repaired primarily. She was discharged home on post-operative day four without complication. Pathology demonstrated a ganglioneuroma. The patient was well at her follow-up, and imaging one year post-operatively was without recurrence. No additional treatment was required. A laparoscopic retroperitoneal approach allows for a safe, minimally invasive resection of a thoracoabdominal mass without violation of the abdominal cavity. [ABSTRACT FROM AUTHOR]

An Amyand's hernia is an unusual diagnosis in children and even rarer in neonates. Perforation of the appendix inside the hernia sac is also extremely unusual. The diagnosis of an Amyand's hernia is difficult as presenting symptoms can also be attributed to an isolated inguinal hernia, noncommunicating hydrocele, or testicular torsion. We present a case of an Amyand's hernia with perforated appendix and scrotal abscess in a premature newborn. [ABSTRACT FROM AUTHOR]

Abstract: Background: Despite advances in prenatal repair, myelomeningocele (MMC) still produces devastating neurologic deficits. The amniotic membranes (AM) are a biologically active tissue that has been used anecdotally for human fetal MMC repair. This study evaluated the use of autologous AM compared to skin closure in an established fetal MMC model. Methods: Seven fetal lambs underwent surgical creation of MMC at gestational age of 75days followed by in utero repair at gestational age of 100days. Lambs were repaired with an autologous AM patch followed by skin closure (n=4) or skin closure alone (n=3). Gross necropsy and histopathology of the spinal cords were performed at term to assess neuronal preservation at the lesion. Results: An increase in preserved motor neurons and a larger area of spinal cord tissue were seen in AM-repaired lambs, as was decreased wound healing of the overlying skin. Loss of nearly all spinal cord tissue with limited motor neuron preservation was seen in skin only-repaired lambs. Conclusions: AM-repaired lambs showed increased protection of spinal cord tissue compared to skin only-repaired lambs, but the overlying skin failed to close in AM-repaired lambs. These results suggest a potential role for AM in fetal MMC repair that warrants further study. [Copyright &y& Elsevier]

Abstract: We report the successful salvage of a patient''s native liver 25 years after hepatic portocholecystostomy for biliary atresia. Our case demonstrates the effectiveness of biliary specific, high-resolution CT imaging in the diagnosis of, and operative planning for complex cases of biliary obstruction. We also report the longest-term pathologic follow-up of biliary atresia after hepatic portocholecystostomy. Life-long follow-up of patients with biliary atresia is important to prevent life-threatening complications of biliary stasis/obstruction. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Neurological function in patients with myelomeningocele (MMC) is limited even after prenatal repair. Neural crest stem cells (NCSCs) can improve neurological function in models of spinal cord injury. We aimed to evaluate the survival, integration, and differentiation of human NCSCs derived from induced pluripotent stem cells (iPSC-NCSCs) in the fetal lamb model of MMC. Methods: Human iPSCs derived from skin fibroblasts were differentiated into NCSCs in vitro, mixed with hydrogel, and seeded on nanofibrous scaffolds for surgical transplantation. Fetal lambs (n=2) underwent surgical MMC creation and repair with iPSC-NCSC seeded scaffolds. Gross necropsy and immunohistochemistry were performed at term. Results: IPSC-NCSCs expressed NCSC markers, maintained > 95% viability, and demonstrated neuronal differentiation in vitro. Immunohistochemical analysis of repaired spinal cords thirty days after transplantation demonstrated the co-localization of human nuclear mitotic apparatus protein (NuMA) and Neurofilament M subunit (NFM) in the area of spinal cord injury. No gross tumors were identified. Conclusions: Human iPSC-NCSCs survived, integrated, and differentiated into neuronal lineage in the fetal lamb model of MMC. This is the first description of human stem cell engraftment in a model of fetal MMC and supports the concept of using NCSCs to address spinal cord damage in MMC. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Congenital high airway obstructive syndrome (CHAOS) is a rare and devastating condition that is uniformly fatal without fetal intervention. We sought to describe fetal treatment and long-term outcomes of CHAOS at a single referral center. Methods: The medical records of patients with fetal CHAOS evaluated at our center between 1993 and 2011 were reviewed. Maternal history, radiographic findings, antenatal management, and postnatal outcomes were compared. Results: Twelve fetuses with CHAOS were identified. Eleven had concomitant hydrops at diagnosis. Six were electively terminated, and 2 had intra- or peripartum demise. Four patients underwent fetal intervention. Two underwent delivery via ex utero intrapartum treatment (EXIT) procedure with tracheostomy placement only, and 2 underwent fetal bronchoscopy with attempted wire tracheoplasty followed by EXIT with tracheostomy at delivery. All 4 patients who underwent EXIT were alive at last follow-up. One patient was ventilator and tracheostomy free and feeding by mouth. Conclusion: Long-term and tracheostomy-free survival is possible with appropriate fetal intervention even in the presence of hydrops. Fetal intervention earlier in pregnancy may improve long-term outcomes, but patient selection for intervention remains challenging. Magnetic resonance imaging may help select those patients for whom fetal intervention before EXIT delivery may be beneficial. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Bidirectional trafficking of cells between the mother and the fetus is routine in pregnancy and a component of maternal-fetal tolerance. Changes in fetal-to-maternal cellular trafficking have been reported in prenatal complications, but maternal-to-fetal trafficking has never been studied in the context of fetal intervention. We hypothesized that patients undergoing open fetal surgery would have altered maternal-fetal cellular trafficking. Methods: Cellular trafficking was analyzed in patients with myelomeningocele (MMC) who underwent open fetal surgical repair (n = 5), patients with MMC who had routine postnatal repair (n = 6), and healthy control healthy patients (n = 9). As an additional control for the fetal operation, trafficking was also analyzed in patients who were delivered by an ex utero intrapartum treatment procedure (n = 6). Microchimerism in maternal and cord blood was determined using quantitative real-time polymerase chain reaction for nonshared alleles. Results: Maternal-to-fetal trafficking was significantly increased in patients who underwent open fetal surgery for MMC compared with healthy controls, patients who underwent postnatal MMC repair, and patients who underwent ex utero intrapartum treatment. There were no differences in fetal-to-maternal cell trafficking among groups. Conclusion: Patients undergoing open fetal surgery for MMC have elevated levels of maternal microchimerism. These results suggest altered trafficking and/or increased proliferation of maternal cells in fetal blood and may have important implications for preterm labor. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Spinal cord damage in myelomeningocele (MMC) results from abnormal cord development and subsequent local trauma. Prenatal surgery prevents additional neural injury. However, existing damage is not reversed. Biodegradable nanofibrous scaffolds (NSs) promote regeneration of neural tissues. They mimic the microtopography of the extracellular matrix and guide tissue formation and organization. The purpose of this pilot study was to evaluate the practicality and safety of using biodegradable NS as a regenerative device in prenatal MMC repair. Methods: Two fetal lambs underwent a surgically induced MMC defect followed by open fetal repair using aligned biodegradable NS. Lambs were killed at day 138. Spinal cords were examined for inflammation or fibrosis and stained for spinal cord architecture, myelin, and neuron cell bodies. Results: Prenatal repair with NS demonstrated technical feasibility. There was no evidence of a surrounding inflammatory response or foreign-body reaction to the scaffold. Conclusion: Biodegradable NS can be used surgically for the prenatal repair of MMC in a large animal model and does not appear to elicit an inflammatory or fibrotic reaction in fetal tissue. Further studies will determine their potential for neural cell infiltration, delivery of growth factors, drugs or stem cells, and functional recovery greater than standard repair. [Copyright &y& Elsevier]

Multi-modal analgesia with a reduction in perioperative opioid use has been a goal for many institutions due to the ongoing opioid epidemic. A reduction in opioid use can also limit undesirable side effects such as nausea, respiratory depression, and ileus in the perioperative and postoperative periods. In this case report, we describe a child who had excellent pain control for colorectal surgery after receiving a novel sacral erector spinae plane block (ESPB) along with bilateral thoracic ESPB's with sustained-release liposomal bupivacaine (Exparel). While there is ample literature describing the efficacy of thoracic and lumbar ESPB's for post-operative analgesia, there is a paucity of information regarding the sacral approach to the ESPB when combined with liposomal bupivacaine and its application to perineal, perianal, and colorectal surgery. [ABSTRACT FROM AUTHOR]

Müllerian duct anomalies are rare in the general population, occurring in less than 3% of women, but much more prevalent in female patients with anorectal malformation, occurring in up to 30% of these patients. Unicornuate uterus with a rudimentary non-communicating horn is a congenital anomaly of Müllerian development which can be seen in isolation or in conjunction with other anomalies, with several case reports described in patients with VACTERL association. These anomalies may be asymptomatic until the patient develops dysmenorrhea or devastating obstetrical complications. We describe the successful surgical management of an obstructive Müllerian anomaly in a post-pubertal female patient with anorectal malformation. [ABSTRACT FROM AUTHOR]

Anorectal malformations affect 1 in 3000-5000 children, with varied incidences dependent upon geographical location. Accurate assessment, and subsequent targeted management in the newborn is critical to reducing potential morbidity and mortality. We have focused in this review upon the management of newborns with anorectal malformations, and the evaluation of the potential for long-term fecal continence. [ABSTRACT FROM AUTHOR]

The differential for neonatal hematemesis ranges from benign etiologies to life-threatening emergencies. Neonatal gastric perforation is a rare cause of neonatal hematemesis but is a deadly condition, requiring prompt diagnosis and treatment. The etiology is usually related to conditions predisposing to overdistension of the stomach, such as positive pressure ventilation or distal obstruction, but in some cases cannot be determined. Patients generally present with abdominal distension and respiratory distress. We present a case of a 1-day old term baby girl who developed sudden onset hematemesis and clinical deterioration, who was found to have a large proximal gastric perforation requiring emergent total gastrectomy with delayed reconstruction. [ABSTRACT FROM AUTHOR]

Diagnosis of a tumor in utero is a rare occurrence and poses diagnostic and therapeutic challenges. In cases of tumor-associated hydrops, there is significant risk of fetal demise, and prenatal intervention may be considered to avoid this outcome when possible. When fetal intervention is unlikely to improve survival, information can be useful for counseling families. We present a rare case of fetal diagnosis of a primary renal malignancy with widespread metastases and hydrops, with unique immunohistochemical findings consistent with malignant primitive epithelioid sarcoma with features of rhabdoid tumor. [ABSTRACT FROM AUTHOR]

Pulmonary sarcomatoid carcinoma is a rare subtype of non-small cell lung cancer typically seen in elderly male patients, accounting for 0.1–0.4% of all lung cancers [1]. We present a case report of a 3-month old boy diagnosed with pulmonary sarcomatoid carcinoma after presenting to the emergency department with respiratory distress. This is the first reported case of pulmonary sarcomatoid carcinoma presenting in an infant. [ABSTRACT FROM AUTHOR]

Trauma during pregnancy is the leading non-obstetric cause of morbidity and mortality, and accounts for five per 1000 fetal deaths. Direct fetal injury due to trauma during pregnancy is rare, and limited information is available about how to optimize fetal outcomes after injury. Early recognition and appropriate management of direct fetal trauma may improve outcomes for the fetus. There are currently no available guidelines to direct management of the injured fetus. We provide a detailed literature review of the management and outcomes of direct fetal injury following blunt and penetrating injury during pregnancy, and describe a suggested initial approach to the injured pregnant patient with a focus on evaluation for fetal injury. We identified 45 reported cases of blunt trauma resulting in direct fetal injury, with 21 surviving past the neonatal period, and 33 of penetrating trauma resulting in direct fetal injury, with 24 surviving past the neonatal period. Prenatal imaging identified fetal injury in 19 cases of blunt trauma and was used to identify bullet location relative to the fetus in 6 cases. These reports were used to develop management algorithms for the injured fetus. [ABSTRACT FROM AUTHOR]