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surgery for hirschsprungs

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https://www.readbyqxmd.com/read/27912977/functional-outcomes-in-hirschsprung-disease-a-single-institution-s-12-year-experience
#1
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...
November 14, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27898990/european-paediatric-surgeons-association-survey-on-the-management-of-hirschsprung-disease
#2
Augusto Zani, Simon Eaton, Francesco Morini, Prem Puri, Risto Rintala, Ernest van Heurn, Marija Lukac, Pietro Bagolan, Joachim F Kuebler, Florian Friedmacher, Rene Wijnen, Juan A Tovar, Michael E Hoellwarth, Agostino Pierro
: Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%). DIAGNOSIS: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm...
November 29, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27896152/a-retrospective-cohort-study-of-total-colonic-aganglionosis-is-the-appendix-a-reliable-diagnostic-tool
#3
T O'Hare, M McDermott, M O'Sullivan, P Dicker, B Antao
BACKGROUND: Hirschsprung's disease (HD) is characterized by a lack of ganglion cells in the myenteric and submucosal plexus, associated with increased numbers of acetyl cholinesterase (AChE) positive nerve fibres. In approximately 10% of patients with HD the entire colon will be affected; a condition known as Total Colonic Aganglionosis (TCA). Aganglionosis of the appendix has long been considered to be an important finding in a patient in whom TCA is suspected, but its reliability for diagnosis has seldom been discussed...
October 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27895411/reoperation-in-an-adult-female-with-right-sided-hirschsprung-s-disease-complicated-by-refractory-hypertension-and-cough
#4
Zhi-Jian Wei, Lei Huang, A-Man Xu
Hirschsprung's disease (HD) is an intestinal malformation caused by the innate absence of ganglion cells in the neural plexus of the colorectal wall, and is most common in male infants. It is rare in adult, and is usually left-sided. Herein we reported based on the CARE guidelines a case of a 47-year-old adult female suffering from "right-sided" HD complicated by refractory hypertension and cough. The patient with a history of cesarean section and with digestive unfitness (abdominal pain, distention, and constipation) only since 20 years old had recurrence of HD after initial surgery due to the incomplete removal of the HD-affected bowel based on a diagnosis of "chronic ileus", leading to the relapse of the digestive symptoms and the emergence of some intractable circulatory and respiratory complications which could be hardly controlled by conservative treatment...
November 7, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27890312/variables-associated-with-loss-of-ileoanal-pouches-constructed-in-childhood
#5
Khaled Abdelaal, Bruce Jaffray
AIMS: To quantify the incidence of loss of an ileoanal pouch in children and to identify variables associated this event. METHODS: Logistic regression of possible explanatory variables: age, sex, indication for procto-colectomy, pre-operative continence status, use of immunosuppressive drugs, open or laparoscopic surgery, number of stages, anastomotic leak, operative complication excluding anastomotic leak, performance of revisional surgery, albumin concentration and platelet count at time of surgery, rank order in series...
November 13, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27858582/long-term-outcome-of-laparoscopic-duhamel-procedure-for-extended-hirschsprung-s-disease
#6
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)...
November 18, 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/27836370/long-term-results-of-the-duhamel-technique-are-superior-to-those-of-the-transanal-pullthrough-a-study-of-fecal-continence-and-quality-of-life
#7
Ana Cristina Aoun Tannuri, Mariana Aparecida Elisei Ferreira, Arthur Loguetti Mathias, Uenis Tannuri
BACKGROUND/PURPOSE: The Duhamel and transanal pull-through (TAPT) techniques have been commonly used for the treatment of children with Hirschsprung disease (HD). However, despite adequate treatment, some patients present with fecal incontinence that severely affects the quality of life (QoL) and lead to psychiatric disorders. The objectives of the present study were to evaluate, through previously adapted questionnaires, the incidence of fecal incontinence and the quality of life (QoL) of children with HD who underwent Duhamel or TAPT techniques...
October 19, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27830038/advances-in-minimally-invasive-neonatal-colorectal-surgery
#8
REVIEW
Ashwath S Bandi, Catherine J Bradshaw, Stefano Giuliani
Over the last two decades, advances in laparoscopic surgery and minimally invasive techniques have transformed the operative management of neonatal colorectal surgery for conditions such as anorectal malformations (ARMs) and Hirschsprung's disease. Evolution of surgical care has mainly occurred due to the use of laparoscopy, as opposed to a laparotomy, for intra-abdominal procedures and the development of trans-anal techniques. This review describes these advances and outlines the main minimally invasive techniques currently used for management of ARMs and Hirschsprung's disease...
October 27, 2016: World Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/27813711/a-scoring-system-to-predict-the-severity-of-hirschsprung-disease-at-diagnosis-and-its-correlation-with-molecular-genetics
#9
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 non-severe 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...
January 27, 2016: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/27781255/-timing-investigation-of-single-stage-definitive-surgery-for-newborn-with-hirschsprung-s-disease
#10
Shangjie Xiao, Wenyi Yang, Like Yuan, Ying Zhang, Tao Song, Lu Xu, Song Tian, Wuping Ge, Jialiang Zhou, Xiaochun Zhu
OBJECTIVE: To investigate the operation timing of newborns with rectosigmoid Hirschsprung's disease (HD). METHODS: From March 2013 to September 2015, 35 newborns diagnosed as rectosigmoid HD in our department were prospectively and randomly divided into 2 groups: less than 3 months treatment group (18 cases) and more than 3 months treatment group (17 cases, conservative treatment for 3 months). They all underwent laparoscopic-assisted transanal endorectal pull-through (LATEP) (modified Soave) procedure...
October 25, 2016: Zhonghua Wei Chang Wai Ke za Zhi, Chinese Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/27780281/surgical-management-of-functional-constipation-preliminary-report-of-a-new-approach-using-a-laparoscopic-sigmoid-resection-combined-with-a-malone-appendicostomy
#11
Alessandra Gasior, Giulia Brisighelli, Karen Diefenbach, Victoria Alison Lane, Carlos Reck, Richard J Wood, Marc Levitt
Introduction Functional constipation is a common problem in children. It usually can be managed with laxatives but a small subset of patients develop intolerable cramps and need to be temporarily treated with enemas. The senior author has previously reported: 1) open sigmoid resection as a surgical option, but this did not sufficiently reduce the laxative need, then 2) a transanal approach (with resection of rectosigmoid), but this led to a high rate of soiling due to extensive stretching of the anal canal and loss of the rectal reservoir...
October 25, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27775297/-outcome-of-posterior-anorectal-myectomy-for-the-treatment-of-idiopathic-chronic-constipation
#12
M Ortega Escudero, J M Gutiérrez Dueñas, C Hernández Díaz, C Ruiz Hierro, R Chamorro Juárez, E Ardela Díaz
OBJECIVES: The aim of this study is to assess the outcome of surgery (Lynn's myectomy) in patients with chronic persistent constipation and failure of medical treatment. MATERIAL AND METHODS: Descriptive study of patients with severe chronic constipation treated by posterior anorectal myectomy (Lynn's technique). We report data from the last 15 years, with a minimum postoperative follow-up of one year. Patients included in the study suffered from a long-term constipation refractory to medical management...
October 10, 2015: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27765267/redo-pull-through-surgery-in-hirschsprung-s-disease-short-term-clinical-outcome
#13
Ajm Dingemans, Hjj van der Steeg, R Rassouli-Kirchmeier, M W Linssen, Ialm van Rooij, I de Blaauw
INTRODUCTION: Although surgery is effective in most patients with Hirschsprung's disease (HD), some have persistent obstructive symptoms. Additional medical treatment is generally sufficient, but a small fraction of these patients needs secondary surgery. Series on redo surgery are scarce. Aim of this study is to evaluate complications and clinical outcome of patients in need of redo surgery for HD. MATERIALS AND METHODS: Sixteen patients underwent redo endorectal pull-through surgery in our center between 2007 and 2015...
September 23, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27755344/rectal-suction-biopsy-in-patients-with-previous-anorectal-surgery-for-hirschsprung-disease
#14
Zareen Vadva, Samuel Nurko, Rebecca Hehn, Sara O Vargas
OBJECTIVES: 1. To determine rates of histologically positive, negative and inconclusive rectal suction biopsies in post-pull-through patients with Hirschsprung disease evaluated for potential residual aganglionosis at our institution. 2. To determine how patients were managed after a post-pull-through rectal suction biopsy. METHODS: Thirty-nine post-pull-through suction biopsies from our institution were reviewed. Samples, stained with H&E and often acetylcholinesterase and/or calretinin, were categorized as "histologically" positive, negative or inconclusive for aganglionosis...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27733240/urological-and-sexual-outcome-in-patients-with-hirschsprung-disease-a-systematic-review
#15
Hendt P Versteegh, Navroop S Johal, Ivo de Blaauw, Michael P Stanton
PURPOSE: There is a paucity of recent evidence regarding long-term urological and sexual outcomes following surgery for Hirschsprung disease (HD). We aimed to undertake a systematic review of all HD literature to define these outcomes. MATERIALS AND METHODS: A systematic literature search was conducted on studies from 1966 to 2014. Relevant articles were assessed for urological/sexual operative complications and functional sequelae. Studies were analysed in qualitative (Rangel score) and quantitative syntheses...
September 30, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/27712891/usefulness-of-posterior-sagittal-anorectoplasty-for-redo-pull-through-in-complicated-and-recurrent-hirschsprung-disease-experience-with-a-single-surgical-group
#16
Song Sun, Gong Chen, Shan Zheng, Kuiran Dong, Xianmin Xiao
AIM: To retrospectively examine 12 patients with Hirschsprung disease (HD) who underwent posterior sagittal anorectoplasty (PSARP) for various complications. METHODS: This study included patients with HD who underwent redo pull-through (PT) via PSARP at our institute between 2005 and 2014. The type of initial procedure, clinical presentations, indications, and functional results were analyzed. Postoperative excretory function was assessed using the Krickenbeck classification...
August 31, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27682968/hirschsprung-s-disease-clinical-dysmorphology-genes-micro-rnas-and-future-perspectives
#17
Consolato Sergi, Oana Caluseriu, Hunter McColl, David D Eisenstat
On the occasion of the 100(th) anniversary of Dr. Harald Hirschsprung's death, there is a worldwide significant research effort towards identifying and understanding the role of genes and biochemical pathways involved in the pathogenesis as well as the use of new therapies for the disease harboring his name (Hirschsprung disease, HSCR). HSCR (aganglionic megacolon) is a frequent diagnostic and clinical challenge in perinatology and pediatric surgery, and a major cause of neonatal intestinal obstruction. HSCR is characterized by the absence of ganglia of the enteric nervous system, mostly in the distal gastrointestinal tract (GIT)...
September 28, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27639057/population-based-study-shows-that-hirschsprung-disease-does-not-have-a-negative-impact-on-education-and-income
#18
Anna Löf Granström, Anna Svenningsson, Agneta Nordenskjöld, Tomas Wester
AIM: Hirschsprung disease is a multifactorial disease, which is mainly treated during childhood. There is a risk of impaired bowel function for a long time after surgery, and its impact on adult life has not been well studied. This study assessed whether having Hirschsprung disease affected social parameters such as educational level and income. METHODS: This nationwide, population-based cohort study included all patients with Hirschsprung disease, registered in the Swedish National Patient Register from 1964 and 2013, who were each matched by age and sex to ten individuals randomly selected from the Swedish Population Register...
December 2016: Acta Paediatrica
https://www.readbyqxmd.com/read/27581039/transition-zone-pull-through-in-hirschsprung-s-disease-a-tertiary-hospital-experience
#19
Dhruva N Ghosh, Yingrui Liu, Daniel T Cass, Soundappan S V Soundappan
BACKGROUND: Pull-through of ganglionic bowel is essential for successful treatment of Hirschsprung's disease. We studied the incidence of transition zone pull-through in our institution and compared its outcome with ganglionic bowel pull-through. METHODS: Children who underwent Soave's pull-through for Hirschsprung's disease from January 2005 to November 2012 were studied. Patients were divided into two groups: ganglionic bowel pull-throughs (Group 1) and transition zone pull-throughs (Group 2)...
August 31, 2016: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/27567306/altered-expression-of-retinoblastoma-1-in-hirschsprung-s-disease
#20
Anne Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: The retinoblastoma 1 (RB1) tumor suppressor is a critical regulator of cell cycle progression and development, and has been widely documented to be inactivated in human cancer. A recent study using RB1 knockout mice suggested a new role for RB1 in the normal regulation of the enteric nervous system (ENS), because of knockout mice showing ENS abnormalities and severe intestinal dysmotility. The aim of our study was to investigate the expression of RB1 in the normal human colon and in Hirschsprung's disease (HD)...
November 2016: Journal of Pediatric Surgery
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