Outcomes Following Fecal Diversion for Intractable Hirschsprung Associated Enterocolitis: A Study From the Pediatric Colorectal and Pelvic Learning Consortium.

Background: Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes.

Variation in Practice Surrounding Antegrade Colonic Enema Channel Placement.

Purpose: 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.

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

Purpose: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry.

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

Background: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown.

Evolution and Variations of the Ovine Model of Spina Bifida.

Spina bifida is the most common congenital anomaly of the central nervous system and the first non-fatal fetal lesions to be addressed by fetal intervention. While research in spina bifida has been performed in rodent, nonhuman primate, and canine models, sheep have been a model organism for the disease. This review summarizes the history of development of the ovine model of spina bifida, previous applications, and translation into clinical studies.

Rectal Prolapse Following Repair of Anorectal Malformation: Incidence, Risk Factors, and Management.

Background: The incidence and optimal management of rectal prolapse following repair of an anorectal malformation (ARM) has not been well-defined.

Methods: 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.

Prenatal Rupture of Hydrocolpos in a Cloacal Malformation.

Introduction: Cloacal dysgenesis occurs from failure of embryological division of urogenital sinus and hindgut, leading to a single common perineal opening for genitourinary and gastrointestinal tracts. The prenatal diagnosis of cloacal malformation is imprecise, but the clinical correlation of postnatal findings to prenatal history can help reveal explanations for unusual pathological findings in patients with urogenital abnormalities.

Case Presentation: A 21-year-old woman was referred after her 20-week ultrasound demonstrated anhydramnios and concern for dilated fetal bowel.

Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group.

Background/purpose: 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.

Hirschsprung Disease for the Practicing Surgical Pathologist.

Objectives: Hirschsprung disease (HD) is a congenital condition defined by the absence of ganglion cells in the distal-most portion of the gastrointestinal tract. Biopsies and resections for HD can be adrenaline inducing for the general surgical pathologist because specimens are infrequent; HD is 1 of only a few neuroanatomic diseases that general surgical pathologists diagnose; numerous preanalytic factors (eg, biopsy adequacy, surgeon sampling protocol, processing artifacts) can affect histologic interpretation; and most importantly, the diagnosis has high stakes.

Methods: We provide a comprehensive overview of the background, relevant clinical procedures, and pathologic assessment of HD.

Fecal continence disparities in patients with idiopathic constipation treated at referral institutions for pediatric colorectal surgery.

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.

Association Between Race, Gender, and Pediatric Postoperative Outcomes: An Updated Retrospective Review.

Introduction: There has not been a recent evaluation of the association between racial and gender and surgical outcomes in children. We aimed to evaluate improvements in race- and gender-related pediatric postoperative outcomes since a report utilizing the Kids' Inpatient Database data from 2003 to 2006.

Methods: Using Kids' Inpatient Database (2009, 2012, 2016), we identified 245,976 pediatric patients who underwent appendectomy for acute appendicitis (93.

Does presence of a VACTERL anomaly predict an associated gynecologic anomaly in females with anorectal malformations?: A Pediatric Colorectal and Pelvic Learning Consortium Study.

Background: 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).

Methods: This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC).

The Utility of Discharge Antibiotics in Pediatric Perforated Appendicitis Without Leukocytosis.

Introduction: Optimal management of pediatric perforated appendicitis remains a topic of active investigation. Our institutional clinical practice guidelines (CPGs) were modified to discontinue antibiotics on discharge for patients with normal white blood cell count (WBC) without left shift. We hypothesized that patients would receive fewer antibiotics without increased complications.

Recognition and management of traumatic fetal injuries.

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.

Early investigations into improving bowel and bladder function in fetal ovine myelomeningocele repair.

Introduction: 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.

Urinary continence disparities in patients with anorectal malformations.

Purpose: 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.

Surgical Management of an Obstructive Müllerian Anomaly in a Patient with Anorectal Malformation.

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 Mullerian 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.

State-level political partisanship strongly correlates with health outcomes for US children.

The Cook Partisan Voting Index (PVI) determines how strongly a state leans toward the Democratic or Republican Party in US presidential elections compared to the nation. We set out to determine the correlation between childhood health outcomes and state-level partisanship using PVI. Sixteen measures of childhood health were obtained from several US governmental agencies for 2003-2017.

Variations in Perceptions of Postoperative Opioid Need for Pediatric Surgical Patients.

This survey study evaluates the opinions of key stakeholders regarding the need and duration of postoperative opioids for pediatric surgical patients.

Diagnosing Hirschsprung disease by detecting intestinal ganglion cells using label-free hyperspectral microscopy.

Hirschsprung disease (HD) is a congenital disorder in the distal colon that is characterized by the absence of nerve ganglion cells in the diseased tissue. The primary treatment for HD is surgical intervention with resection of the aganglionic bowel. The accurate identification of the aganglionic segment depends on the histologic evaluation of multiple biopsies to determine the absence of ganglion cells in the tissue, which can be a time-consuming procedure.

Optimal management of the newborn with an anorectal malformation and evaluation of their continence potential.

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.

Is Pseudomonas infection associated with worse outcomes in pediatric perforated appendicitis?

Background: 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.

Methods: 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.

Total gastrectomy with delayed Hunt-Lawrence pouch reconstruction for neonatal gastric perforation presenting with hematemesis.

The differential for neonatal hematoma sis ranges from benign etiologies to life-threatening emergencies. Neonatal gastric perforation is a rare cause of neonatal hematoma sis but is a deadly condition, requiring prompt diagnosis and treatment. The etiology is usually related to conditions predisposing to over distension of the stomach, such as positive pressure ventilation or distal obstruction, but in some cases cannot be determined.