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hirschsprung's enterocolitis

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https://www.readbyqxmd.com/read/28737964/laparoscopic-duhamel-procedure-with-ex-anal-rectal-transection-for-right-sided-hirschsprung-s-disease
#1
Xi Zhang, Li Yang, Shao-Tao Tang, Guo-Qing Cao, Shuai Li, Meng Jiang, Meng Xiong, De-Hua Yang, Xiao-Pan Chang, Kang Li, Ya-Zhen Ma
BACKGROUND: Increased defecation frequency and soiling are common complications of surgery for right-sided Hirschsprung's disease (HD). Though the laparoscopic Duhamel procedure is a favorable option in right-sided HD, the conventional laparoscopic technique is time consuming and has complications that are associated with the reservoir. In this study, we described a modified laparoscopic Duhamel technique with ex-anal rectal transection combined with the Deloyer's procedure for right-sided HD...
July 24, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28705638/laparoscopic-assisted-duhamel-procedure-with-ex-anal-rectal-transection-for-total-colonic-aganglionosis
#2
Xi Zhang, Guo-Qing Cao, Shao-Tao Tang, Xiao-Pan Chang, Shuai Li, Li Yang, Kang Li, Ying Zhou, De-Hua Yang
PURPOSE: Laparoscopic-assisted Duhamel procedure has a larger anastomosis and a reservoir which allows early recovery of defecation frequency, but concerns have been raised regarding the long operative time, high incidence of pouchitis and Hirschsprung associated enterocolitis (HAEC). The purpose of this study was to evaluate the postoperative complications and functional outcomes for patients with TCA undergoing modified laparoscopic-assisted Duhamel procedure (MLDP) with ex-abdominal partial colectomy and ex-anal rectal transection...
June 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28689885/reduction-of-hydrogen-sulfide-synthesis-enzymes-cystathionine-%C3%AE-synthase-and-cystathionine-%C3%AE-lyase-in-the-colon-of-patients-with-hirschsprungs-s-disease
#3
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Hirschsprung's associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's Disease (HSCR). The pathogenesis of HAEC is poorly understood. In recent years, there is increasing evidence that a compromised intestinal barrier function plays a major role in the pathogenesis of HAEC. Hydrogen sulfide, synthesized from L-cysteine by two key enzymes, cystathionine-β-synthase (CBS) and cystathionine-γ-lysase (CSE) is reported to play a key role in regulating gastrointestinal motility and promoting resolution of inflammation...
June 23, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28455798/internal-anal-sphincter-achalasia-data-from-a-nationwide-survey-of-allied-disorders-of-hirschsprung-s-disease-in-japan
#4
Satoshi Obata, Suguru Fukahori, Minoru Yagi, Makoto Suzuki, Shigeru Ueno, Kosuke Ushijima, Tomoaki Taguchi
PURPOSE: To investigate the incidence and treatment of internal anal sphincter achalasia (IASA) in Japan based on an analysis of data from a nationwide retrospective cohort study of the allied disorders of Hirschsprung's disease. METHODS: Five cases of definitive IASA were collected from a nationwide retrospective cohort study conducted from 2001 to 2010 and a search of the Japanese literature. RESULTS: Symptoms developed during the neonatal period in two patients, during early childhood in two, and at school age in one...
April 28, 2017: Surgery Today
https://www.readbyqxmd.com/read/28385428/pathologically-assessed-grade-of-hirschsprung-associated-enterocolitis-in-resected-colon-in-children-with-hirschsprung-s-disease-predicts-postoperative-bowel-function
#5
Siyang Cheng, Jun Wang, Weihua Pan, Wenbo Yan, Jia Shi, Wenbin Guan, Yang Wang, Wei Cai
PURPOSE: The aim of this study was to investigate the relationship between the grade of enterocolitis on pathological assessment of resected colon and postoperative bowel function in children with Hirschsprung's disease (HD). METHODS: Children with HD who were seen at a large tertiary center from January 2012 to December 2013 were enrolled into this study. Resected colon was assessed using the histopathologic grade scoring system for Hirschsprung-associated enterocolitis (HAEC), and the relationship of these scores to postoperative bowel function was assessed...
March 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28328696/enterocolitis-in-a-child-with-hirschsprung-disease
#6
Alexander W D Guillaume, Andrew C Miller, Michael C Nguyen
Hirschsprung disease is a congenital abnormality that can be surgically corrected. However, Hirschsprung-associated enterocolitis can be a life-threatening sequela. Very little has been published in the emergency medicine literature about the risk of enterocolitis and shock in patients with a history of Hirschsprung disease. We describe the case of a 6-month-old male infant with a history of multiple surgeries for Hirschsprung disease who presented to the emergency department with a seemingly benign viral gastrointestinal illness...
March 21, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28283986/continuous-transanal-decompression-for-infants-with-long-and-total-type-hirschsprung-s-diseases-as-a-bridge-to-curative-surgery-a-single-center-experience
#7
Kyoko Mochizuki, Masato Shinkai, Norihiko Kitagawa, Hiroshi Take, Hidehito Usui, Takashi Hosokawa, Kaori Yamoto
BACKGROUND: The purpose of this study is to assess the usefulness of continuous bowel decompression using an indwelling transanal tube (ITT) for preoperative management in infants with long-segment (L)- or total (T)-type Hirschsprung's disease (HD). CASE PRESENTATION: Between 2012 and 2015, seven patients with L- or T-type HD underwent preoperative bowel management by continuous bowel decompression using an ITT during waiting period for curative surgery. Continuous bowel decompression was done using an ITT, a 10-12F flexible dual lumen tube placed through the rectum up to the dilated colon under fluoroscopic guidance and secured to the bilateral buttocks...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28276298/a-scoring-system-to-predict-the-severity-of-hirschsprung-disease-at-diagnosis-and-its-correlation-with-molecular-genetics
#8
Raquel Núñez-Ramos, Raquel M Fernández, Miguel González-Velasco, Jesús Ruiz-Contreras, Enrique Galán-Gómez, Ramón Núñez-Núñez, Salud Borrego
Objectives Hirschsprung disease (HSCR) has a wide range of severity. There are nonsevere forms treated conservatively until surgery and severe forms that require an early stoma and prolonged hospitalization. Our objective was to establish a clinical scoring system to predict the severity of HSCR and to evaluate the possible existence of a clinical-genetic correlation. Methods We carried out a retrospective observational study including all HSCR cases treated in our hospital. The sample was divided into severe and nonsevere disease according to the number of surgical procedures, hospitalization time, and episodes of enterocolitis...
January 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28209955/-the-complications-of-intestinal-stoma-in-children
#9
S V Minaev, N I Bykov, A V Isaeva, A V Kachanov, E A Tovkan, N V Filip'yeva, I N Gerasimenko
AIM: To investigate the complications of intestinal stoma in children and to develop measures for decrease of their incidence. MATERIAL AND METHODS: The study included 152 children with congenital and acquired gastrointestinal pathology requiring surgical treatment with the imposition of intestinal stoma. Atresia of intestinal tube was observed in 28 (18.4%) children, meconium ileus - in 10 (6.6%) cases, Hirschsprung's disease - in 11 (7.2%)cases, anorectal malformations - in 39 (25...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28208866/fatal-toxic-megacolon-in-a-child-of-hirschsprung-disease
#10
Shiwani R Garg, Pragati A Sathe, Annapurna C Taware, Ketaki M Surve
Hirschsprung disease (HD) in late childhood is uncommon and often undiagnosed or misdiagnosed. However, in a patient with Hirschsprung disease, of greater significance is the occurrence of life threatening enterocolitis. In its more severe form, this is associated with gross dilatation of the colon and profound toxaemia, the combination being termed toxic megacolon. Because of its relative rarity, we report a case of 10-year-old child who had a history of chronic constipation for nine years. He later developed complications and presented to the emergency department with toxic megacolon, a rare occurrence due to neglected constipation...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28196663/nos-interacting-protein-nosip-is-increased-in-the-colon-of-patients-with-hirschsprungs-s-disease
#11
Christian Tomuschat, Anne-Marie O'Donnell, David Coyle, Nickolas Dreher, Danielle Kelly, Prem Puri
PURPOSE: Hirschsprung's associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Nitric oxide (NO) mediates intestinal homoeostasis and is inhibited by NOSIP, a modulator of NO production. We designed this study to investigate the expression of NOSIP in the colon of patients with HSCR. METHODS: We investigated NOSIP, endothelial NO synthase, and neuronal NO synthase expression in both the aganglionic and ganglionic regions of HSCR patients (n=10) versus normal control colon (n=10)...
May 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28185631/single-stage-transanal-endorectal-pull-through-procedure-for-correction-of-hirschsprung-disease-in-neonates-and-nonneonates-a-multicenter-study
#12
Changgui Lu, Guangjun Hou, Chunyi Liu, Qiming Geng, Xiaoqun Xu, Jie Zhang, Huan Chen, Weibing Tang
PURPOSE: The outcomes of single-stage transanal endorectal pull-through (SSTEPT) for Hirschsprung disease (HSCR) in young patients are favorable; however, reports have shown that diagnosis and surgery at young ages increase the risk for postoperative enterocolitis and slows postoperative recovery. The present study was primarily designed to evaluate the outcomes of SSTEPT in a multi-institutional cohort of neonates and nonneonates with HSCR. METHODS: Between August 2005 and May 2012, a total of 650 children with HSCR were divided into the following two groups: group A (neonatal group, operative age<28days [n=186]); and group B (nonneonatal group, operative age>28days [n=464])...
July 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28180937/guidelines-for-the-management-of-postoperative-obstructive-symptoms-in-children-with-hirschsprung-disease
#13
REVIEW
J C Langer, M D Rollins, M Levitt, A Gosain, L de la Torre, R P Kapur, R A Cowles, J Horton, D H Rothstein, A M Goldstein
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...
May 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28154902/guidelines-for-the-diagnosis-and-management-of-hirschsprung-associated-enterocolitis
#14
REVIEW
Ankush Gosain, Philip K Frykman, Robert A Cowles, John Horton, Marc Levitt, David H Rothstein, Jacob C Langer, Allan M Goldstein
BACKGROUND: Patients with Hirschsprung disease are at risk for Hirschsprung-associated enterocolitis (HAEC), an inflammatory disorder of the bowel that represents the leading cause of serious morbidity and death in these patients. The diagnosis of HAEC is made based on clinical signs and symptoms which are often non-specific, making it difficult to establish a definitive diagnosis in many patients. The purpose of this guideline is to present a rational, expert-based approach to the diagnosis and management of HAEC...
May 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28058486/current-status-of-hirschsprung-s-disease-based-on-a-nationwide-survey-of-japan
#15
Tomoaki Taguchi, Satoshi Obata, Satoshi Ieiri
PURPOSE: The diagnosis and surgical treatments of Hirschsprung's disease (HD) have undergone various changes in the last few decades because of establishment of laparoscopic procedures. A retrospective nationwide survey for 4 decades was performed to study the changing profile of HD in Japan. METHODS: The patient data were collected in 4 phases: Group 1, between 1978 and 1982; Group 2, between 1988 and 1992; Group 3, between 1998 and 2002; and Group 4, between 2008 and 2012...
April 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28040830/redo-pullthrough-for-hirschsprung-disease
#16
REVIEW
Matthew W Ralls, Arnold G Coran, Daniel H Teitelbaum
Pullthrough procedures for Hirschsprung diseases typically have favorable results. However, some children experience long-term postoperative complications comprising stooling disorders, such as intermittent enterocolitis, severe stool retention, intestinal obstruction, as well as incontinence. Reoperative Hirschsprung Disease surgery is complex. This begins with the workup after the initial presentation following primary pullthrough, continues with the definitive surgical correction with redo pullthrough, and ends with long-term follow-up of individuals...
April 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28003043/genetic-impact-on-the-treatment-management-of-hirschsprung-disease
#17
REVIEW
Sam W Moore
BACKGROUND: The identification of Hirschsprung's disease (HD) as a genetic condition has been a major step forward in understanding the development of the enteric nervous system and conditions arising from ganglion cell maldevelopment. METHOD: A study of the role of genetics in HD was carried out based on previously published findings from more than 400 cases of HD. RESULTS: There are at least 7 pertinent clinical questions related to HD which were further investigated...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27912977/functional-outcomes-in-hirschsprung-disease-a-single-institution-s-12-year-experience
#18
Hemanshoo S Thakkar, Christopher Bassett, Andy Hsu, Riccardo Manuele, Dorothy Kufeji, Catherine A Richards, Meena Agrawal, Alireza S Keshtgar
AIMS: Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period. MATERIALS AND METHODS: We conducted a retrospective review of all children operated for HD between 2002 and 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0-20, 20=best score). We assessed hospital admissions, complications including Hirschsprung associated enterocolitis (HAEC) and the need for further surgical procedures...
February 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27858582/long-term-outcome-of-laparoscopic-duhamel-procedure-for-extended-hirschsprung-s-disease
#19
Cécile O Muller, Guillaume Rossignol, Louise Montalva, Jerome Viala, Christine Martinez-Vinson, Alexis Mosca, Dominique Berrebi, Arnaud Bonnard
BACKGROUND: The aim of our study was to report our experience in extended Hirschsprung's disease (HD) in children operated on by laparoscopy. PATIENTS AND METHODS: Retrospective data collection from a single center from 1991 to 2013 concerned extended forms of HD operated on by laparoscopic Duhamel procedure and included extension of aganglionosis, comorbidities, short and late postoperative outcome, and results of endoscopy when performed. RESULTS: Thirty patients presented an extended form of aganglionosis: 5 involving the transverse colon, 10 the right colon, and 15 the ileum (median length = 15 cm, range 1-60)...
December 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/27853811/altered-expression-of-il36%C3%AE-and-il36-receptor-il1rl2-in-the-colon-of-patients-with-hirschsprung-s-disease
#20
Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Hirschsprung's disease associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in Hirschsprung's disease (HSCR). Altered intestinal epithelial barrier function and abnormal microbiota are implicated in the pathogenesis of HAEC. IL-36γ, a member of the IL-1 superfamily, is involved in host defense and contributes to proinflammatory responses and development of inflammatory diseases. The IL36 receptor (IL1RL2) is an important mediator molecule in the inflammatory response...
February 2017: Pediatric Surgery International
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