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.

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.

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.

Recurrent Gastrointestinal Bleed Due to Small Bowel Vascular Malformation in a Patient with Turner Syndrome Diagnosed with Surgically Assisted Push Enteroscopy.

BACKGROUND Obscure gastrointestinal bleeding accounts for approximately 5% of all cases of gastrointestinal bleeding and the most frequent site is the small bowel. Turner syndrome (TS) is a genetic disorder of the second X chromosome in females. The association between gastrointestinal vascular malformations and Turner syndrome has been described in some case reports.

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case.

Background: Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief.

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.

Adjuncts to bowel management for fecal incontinence and constipation, the role of surgery; appendicostomy, cecostomy, neoappendicostomy, and colonic resection.

Constipation and fecal incontinence are common problems in children after repair of an anorectal malformation (ARM). While many children can be effectively managed with an oral laxative regimen, others require a mechanical colonic washout to achieve social continence. Appendicostomy and cecostomy are two techniques which permit antegrade access to the colon for the purpose of enema delivery, which improves compliance and quality of life for patients and families.

Measure twice and cut once: Comparing endoscopy and 3D cloacagram for the common channel and urethral measurements in patients with cloacal malformations.

Introduction: Precise and accurate measurement of the common channel and urethra is a critical determinant prior to the repair of cloacal malformations. Endoscopy and 3D reconstruction cloacagram are two common modalities utilized to help plan the surgical approach, however the consistency between these methods is unknown.

Methods: Common channel and urethral lengths obtained by endoscopy and 3D cloacagram of cloaca patients at six pediatric colorectal centers were compared.

Pediatric Inflammatory Bowel Disease: Special Considerations.

Pediatric inflammatory bowel disease may present differently than adult onset disease. It is important to consider a broader differential diagnosis in very early onset disease. Diagnostic and treatment decisions must consider the long term risks and benefits over a lifetime.

Intestinal stem cells remain viable after prolonged tissue storage.

Intestinal stem cells (ISCs) are responsible for renewal of the epithelium both during normal homeostasis and following injury. As such, they have significant therapeutic potential. However, whether ISCs can survive tissue storage is unknown.

Isolation and characterization of intestinal stem cells based on surface marker combinations and colony-formation assay.

Background & Aims: Identification of intestinal stem cells (ISCs) has relied heavily on the use of transgenic reporters in mice, but this approach is limited by mosaic expression patterns and difficult to directly apply to human tissues. We sought to identify reliable surface markers of ISCs and establish a robust functional assay to characterize ISCs from mouse and human tissues.

Methods: We used immunohistochemistry, real-time reverse-transcription polymerase chain reaction, and fluorescence-activated cell sorting (FACS) to analyze intestinal epithelial cells isolated from mouse and human intestinal tissues.

Brief report: CD24 and CD44 mark human intestinal epithelial cell populations with characteristics of active and facultative stem cells.

Recent seminal studies have rapidly advanced the understanding of intestinal epithelial stem cell (IESC) biology in murine models. However, the lack of techniques suitable for isolation and subsequent downstream analysis of IESCs from human tissue has hindered the application of these findings toward the development of novel diagnostics and therapies with direct clinical relevance. This study demonstrates that the cluster of differentiation genes CD24 and CD44 are differentially expressed across LGR5 positive "active" stem cells as well as HOPX positive "facultative" stem cells.

Intestinal crypts reproducibly expand in culture.

Background: In vitro growth techniques for intestinal crypts and single intestinal stem cells have been recently described, but several questions of translational importance remain unaddressed. The purpose of this study was to first, evaluate if intestinal crypts reproducibly expand in vitro; second, determine the impact of age and region of intestine on crypt growth in vitro; and third, determine the effects of cryopreservation on crypt growth in vitro.

Methods And Materials: Crypts were harvested from 5 cm of proximal, middle, and distal small intestine of C57BL/6J mice aged 4 wk, 6-8 wk, 12-14 wk, and 18-20 wk (n = 4-6 animals) and cultured.

Thoracic empyema, application of video-assisted thoracic surgery and its current management.

Purpose Of Review: Pneumonia in children is frequently complicated by pleural effusions, which rarely progress to empyema. Appropriate clinical management depends on correctly diagnosing the stage of the disease process. Recently, increasing use of video-assisted thoracic debridement has altered the traditional management of pleural effusions and empyema in children, resulting in decreasing reliance on thoracentesis and earlier surgical intervention.