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.

The Source of Nasal Rustle (Nasal Turbulence): An Overview of Current Evidence.

Objective: (also called ) refers to a loud distracting sound that sometimes occurs with audible nasal emission (ANE) during the production of pressure-sensitive consonants in patients with velopharyngeal insufficiency (VPI). This article examines evidence for two hypotheses of causality: vibration of the soft palate () and periodic motion of mucus above the velopharyngeal port ().

Conclusion: A review of the relevant literature shows inconclusive evidence to support velar flutter as a cause of nasal rustle.

Anorectal Manometry in Pediatric Colorectal Surgical Care.

Background: Pediatric colorectal specialists care for patients with a variety of defecation disorders. Anorectal (AR) manometry testing is a valuable tool in the diagnosis and management of these children. This paper provides a summary of AR manometry techniques and applications as well as a review of AR manometry findings in pediatric patients with severe defecation disorders referred to a pediatric colorectal center.

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.

Prevention of Parenteral Nutrition-associated Cholestasis Using Reduced Dose Soybean Lipid Emulsion: A Multicenter Randomized Trial.

Introduction: Reducing soybean lipid emulsion (SLE) dose may prevent parenteral nutrition-associated cholestasis (PNAC) but effects on growth and neurodevelopment are unknown. The purpose of this study was to evaluate the effect of reduced dose SLE on growth and neurodevelopment.

Methods: Surgical neonates at 4 centers were randomized to standard SLE (3 g/kg/day) or reduced SLE (1 g/kg/day) over a 12-week period.

Outcomes From Colonic Pull-Through for Cloacal Exstrophy Differ by Colon Length: A Multi-Institutional Study.

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.

Antegrade Continence Enema Alone for the Management of Functional Constipation and Segmental Colonic Dysmotility (ACE-FC): A Pediatric Colorectal and Pelvic Learning Consortium Study.

Objective:  The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility.

Methods:  A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium.

Multi-Institutional Review From the Pediatric Colorectal and Pelvic Learning Consortium of Minor Spinal Cord Dysraphism in the Setting of Anorectal Malformations: Diagnosis, Treatment, and Outcomes.

Background/rationale: Anorectal malformations (ARM) are associated with congenital anomalies of the spine, but the impact of a minor spinal cord dysraphism (mSCD) on fecal continence in the setting of ARM remains unclear.

Materials/methods: A retrospective review was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. The patient cohort was reviewed for ARM type, mSCD screening/incidence/neurosurgical intervention and age-based BMP utilization.

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.

Fecal continence outcomes and potential disparities for patients with anorectal malformations treated at referral institutions for pediatric colorectal surgery.

Purpose: Fecal incontinence is a problem for many patients born with an anorectal malformation (ARM) that can impact quality of life. It is unknown if racial, ethnic, and socioeconomic disparities relate to fecal continence in these children. We sought to examine outcomes and potential disparities in care.

Decreased Incidence of Hirschsprung-Associated Enterocolitis During COVID-19 Across United States Children's Hospitals.

Background: The Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC).

Methods: Using the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days.

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.

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).

Hirschsprung disease outcomes.

Hirschsprung disease (HD) is a complex surgical and medical problem that appears to have varied health and social outcomes with the age and neurodevelopmental state of patients. In general, long-term outcomes are thought to be good for the majority of patients despite recognized problems with constipation and/or fecal incontinence. However, there are no universally accepted pathways regarding post-operative bowel management programs nor clearly defined follow-up pathways making the current outcome measures difficult to interpret.

The presence of a neurodiverse disorder is associated with increased use of antegrade enema therapy in children with severe constipation: A study from the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC).

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.

Health-related quality of life in children with Hirschsprung disease and children with functional constipation: Parent-child variability.

Background: Health-related quality of life (HRQOL) is an important outcome among children with Hirschsprung Disease (HD), but there are challenges in interpreting findings in previous studies owing to the choice of a comparator group and informant. We compared parent-proxy versus child self-report HRQOL in children with HD to children with functional constipation (FC) and examined predictors of HRQOL.

Methods: Data of 126 children (5-18 years, 60.

Characterizing the use of botulinum toxin in patients with Hirschsprung disease treated at referral institutions for pediatric colorectal surgery.

Purpose: Botulinum toxin (BT) is used to treat pediatric patients with Hirschsprung disease (HD) with obstructive symptoms. We aimed to characterize use of BT in HD patients across pediatric colorectal surgery referral centers.

Methods: A multicenter retrospective study of BT use in children (0-18y) with HD was performed using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) between 2017 and 2021.

Most children experience resolution of idiopathic pediatric rectal prolapse with bowel management alone.

Background: Recent studies in children with idiopathic rectal prolapse report up to 48% require surgical intervention to manage refractory disease. We sought to examine outcomes of our non-surgical approach to managing rectal prolapse using a bowel management program.

Methods: A retrospective review was performed for all children with the diagnosis of rectal prolapse between 2011 and 2020.

Method for Fabricating Transparent Patient-Specific Vocal Tract Replicas.

Introduction: Transparent, patient-specific vocal tract replicas are helpful in research and educational endeavors but challenging to procure. An accessible method for fabricating these models, improving on previously suggested processes, would make them more widely available.

Method: Detailed instructions for fabricating a transparent, patient-specific vocal tract model were addressed.