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.

Surgical management of complicated Crohn's disease.

Surgical management of pediatric Crohn's disease is fundamentally palliative, aiming to treat the sequalae of complicated disease while preserving intestinal length. Multidisciplinary discussion of risk factors and quality of life should take place prior to operative intervention. Though the surgical management of pediatric Crohn's disease is largely based on the adult literature, there are considerations specific to the pediatric population - notably disease and treatment effects on growth and development.

Sacral Nerve Stimulation in Children with Medically Refractory Fecal Incontinence or Severe Constipation.

Introduction: The goal of this study was to evaluate outcomes in patients treated with sacral nerve stimulation (SNS) for medically refractory fecal incontinence or severe constipation.

Methods: We performed a retrospective cohort study of all patients treated with SNS after failed medical management at a single center between 9/1/2015 and 6/30/2022. Demographic and clinical data was extracted from the electronic medical record.

Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review.

Background: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence.

Methods: Patients with HD seen at our institution between 2005 and 2019 were reviewed.

Long-Term Male Sexual Function and Fecal Incontinence Outcomes for Adult Patients with Hirschsprung Disease or Anorectal Malformation.

Background: This study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD).

Methods: We conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email.

Surgical history of Hirschsprung disease.

Harald Hirschsprung presented the first comprehensive and detailed description of clinical outcomes in patients with congenital megacolon. In the century following his discovery, we have seen realization of the etiology, diagnosis, and treatment of Hirschsprung disease. In this article we describe the surgical management of Hirschsprung disease starting with Orvar Swenson, who pioneered the field with the first full thickness transrectal dissection followed by several additional innovative surgeons who have contributed to the modifications that have brought us to the modern surgical management of Hirschsprung Disease.

Hemorrhage after on-ECMO repair of CDH is equivalent for muscle flap and prosthetic patch.

Background/purpose: Prosthetic patch (patch) and muscle flap (flap) techniques are utilized for severe congenital diaphragmatic hernia (CDH) repair; however, when performed on extracorporeal membrane oxygenation (ECMO), the risk of hemorrhage increases. We sought to compare bleeding complications between repair types.

Methods: We retrospectively reviewed 2010-2016 on-ECMO CDH repairs.

A comparison of surgical complications after appendicostomy and neoappendicostomy in pediatric patients.

Purpose: There are limited data on neoappendicostomy complications owing to small patient populations. This study compares appendicostomy and neoappendicostomy procedures with an emphasis on major postoperative complications requiring either a surgical or interventional radiology procedure.

Method: A single-institution retrospective review included all patients with complete medical charts in the Cincinnati Children's Colorectal Database who underwent either an appendicostomy or neoappendicostomy from August 2005 through December 2016.

Initial development and validation of a fecal incontinence-specific quality of life measure.

Background/purpose: Fecal incontinence is a prevalent pediatric condition with psychosocial impacts on both children and their caregivers. We sought to develop and validate the Cincinnati Fecal Incontinence Scale (CINCY-FIS) as a psychometrically valid measure to assess the quality of life and caregiver impacts of pediatric fecal incontinence.

Methods: Items were generated through review of previous measures, expert consensus, and pilot testing with feedback from 8 families.

Surgical outcomes, bowel habits and quality of life in young patients after ileoanal anastomosis for ulcerative colitis.

Purpose: We aim to investigate the postoperative outcomes, bowel habits and quality of life (QoL) of younger pediatric ulcerative colitis (UC) patients following surgical intervention compared to an older pediatric population.

Methods: Medical records of UC patients after colectomy with ileoanal reconstruction (2002-2013) at our institution were reviewed. Patients/parents completed a QoL, bowel habits and disease course questionnaire.

The association of the severity of anorectal malformations and intestinal malrotation.

Introduction: Intestinal malrotation is a known association of anorectal malformations (ARM). Exact incidence, prognosis and surgical implications related to ARM are unknown. The aim of this study was to identify relevant associations between ARM and the presence of malrotation.

Murine colitis treated with multitargeted tyrosine kinase inhibitors.

Background: Angiogenesis, a known pathogenic component of neoplastic and nonneoplastic diseases, serves as a therapeutic target. Vascular endothelial growth factor (VEGF) and angiogenesis are clinically elevated in inflammatory bowel disease. By targeting vascular endothelial growth factor receptor (VEGFR) and platelet-derived growth factor receptor (PDGFR) with receptor tyrosine kinase inhibitors in a murine model of colitis, we hypothesize that angiogenesis will be suppressed and disease severity improved.

Mouse strain influences angiogenic response to dextran sodium sulfate-induced colitis.

Background: Angiogenesis is a known pathologic factor in chronic inflammatory diseases. Regarding the murine dextran sodium sulfate (DSS) colitis model, different mouse strains produce variable clinical and inflammatory responses. We hypothesize that DSS colitis applied to diverse mouse strains will similarly elevate colonic microvessel density in parallel with inflammation, but will do so with different angiogenic profiles.