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      Prognostic factors for persistent obstructive symptoms in patients with Hirschsprung disease following pull-through

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          Abstract

          Background

          Although most patients with Hirschsprung disease (HSCR) improve after pull-through, some patients still have persistent obstructive symptoms. Most previous studies reported persistent obstructive symptoms after pull-through in HSCR patients from developed countries. Our study determined the prognostic factors of persistent obstructive symptoms in patients with HSCR following pull-through from a particular developing country.

          Methods

          A cross-sectional study was conducted using medical records of patients with HSCR at our institution from January 2017 to January 2022.

          Results

          We ascertained 114 patients with HSCR: 79 males and 35 females. Most of them (90.4%) showed a short aganglionosis and underwent transanal endorectal pull-through (55.3%). Twenty-two percent of patients showed persistent obstructive symptoms following pull-through. Operative technique and age at definitive surgery were significantly associated with the persistent obstructive symptoms after pull-through ( p = 0.011 and 0.019, respectively), while sex, aganglionic segment length, presence of global developmental delay, and Down syndrome were not ( p = 0.873, 0.525, 0.647, and 0.301, respectively). Multivariate analysis revealed that age at pull-through was a significant independent factor for persistent obstructive symptoms after pull-through, with an odds ratio of 3.41 (95% CI = 1.18–9.91; p = 0.02).

          Conclusions

          Our study shows a moderate frequency of persistent obstructive symptoms after pull-through in our institution. In addition, patients who underwent pull-throughs at a younger age might have persistent obstructive symptoms following a definitive surgery. Our study provides new data on persistent obstructive symptoms after pull-through from a particular population that might be beneficial for pediatric surgeons’ consideration before performing definitive surgery on patients with HSCR.

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          Most cited references24

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          Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention.

          Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung disease (HD). Presenting either before or after definitive surgery for HD, HAEC may manifest clinically as abdominal distension and explosive diarrhea, along with emesis, fever, lethargy, and even shock. The pathogenesis of HAEC, the subject of ongoing research, likely involves a complex interplay between a dysfunctional enteric nervous system, abnormal mucin production, insufficient immunoglobulin secretion, and unbalanced intestinal microflora. Early recognition of HAEC and preventative practices, such as rectal washouts following a pull-through, can lead to improved outcomes. Treatment strategies for acute HAEC include timely resuscitation, colonic decompression, and antibiotics. Recurrent or persistent HAEC requires evaluation for mechanical obstruction or residual aganglionosis, and may require surgical treatment with posterior myotomy/myectomy or redo pull-through. This chapter describes the incidence, pathogenesis, treatment, and preventative strategies in management of HAEC.
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            Hirschsprung's disease: A bridge for science and surgery

            Paul Tam (2016)
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              Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease.

              Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment. Level of evidence V.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: Methodology
                Role: Data curationRole: Writing – original draftRole: Writing – review & editing
                Role: Data curationRole: Writing – original draftRole: Writing – review & editing
                Role: ConceptualizationRole: SupervisionRole: Writing – review & editing
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: InvestigationRole: ResourcesRole: SupervisionRole: Writing – original draftRole: Writing – review & editing
                Role: Editor
                Journal
                PLoS One
                PLoS One
                plos
                PLOS ONE
                Public Library of Science (San Francisco, CA USA )
                1932-6203
                8 September 2023
                2023
                : 18
                : 9
                : e0290430
                Affiliations
                [001] Faculty of Medicine, Department of Surgery, Pediatric Surgery Division, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, Indonesia
                Macau University of Science and Technology, HONG KONG
                Author notes

                Competing Interests: The authors have declared that no competing interests exist.

                Author information
                https://orcid.org/0000-0002-4707-6526
                Article
                PONE-D-23-13236
                10.1371/journal.pone.0290430
                10490837
                37682877
                27a944aa-2402-4a27-ab2d-994dd50ab797
                © 2023 Balela et al

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 11 May 2023
                : 9 August 2023
                Page count
                Figures: 0, Tables: 3, Pages: 9
                Funding
                The author(s) received no specific funding for this work.
                Categories
                Research Article
                Medicine and Health Sciences
                Gastroenterology and Hepatology
                Pediatric Gastroenterology
                Hirschsprung Disease
                Medicine and Health Sciences
                Pediatrics
                Pediatric Gastroenterology
                Hirschsprung Disease
                Medicine and Health Sciences
                Surgical and Invasive Medical Procedures
                Biology and Life Sciences
                Anatomy
                Digestive System
                Gastrointestinal Tract
                Colon
                Medicine and Health Sciences
                Anatomy
                Digestive System
                Gastrointestinal Tract
                Colon
                Medicine and Health Sciences
                Surgical and Invasive Medical Procedures
                Pediatric Surgery
                Medicine and Health Sciences
                Clinical Genetics
                Chromosomal Disorders
                Down Syndrome
                Biology and Life Sciences
                Anatomy
                Digestive System
                Gastrointestinal Tract
                Rectum
                Medicine and Health Sciences
                Anatomy
                Digestive System
                Gastrointestinal Tract
                Rectum
                Research and Analysis Methods
                Mathematical and Statistical Techniques
                Statistical Methods
                Multivariate Analysis
                Physical Sciences
                Mathematics
                Statistics
                Statistical Methods
                Multivariate Analysis
                Medicine and Health Sciences
                Health Care
                Health Care Providers
                Physicians
                Surgeons
                People and Places
                Population Groupings
                Professions
                Medical Personnel
                Physicians
                Surgeons
                Custom metadata
                All relevant data are within the paper and its Supporting Information files.

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