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surgery for hirschsprungs

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
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, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
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
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
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
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
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
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...
September 17, 2016: Acta Paediatrica
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
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
Meng Jiang, Kang Li, Shuai Li, Li Yang, Dehua Yang, Xi Zhang, Mijing Fang, Guoqing Cao, Yong Wang, Weibin Chen, Shaotao Tang
Evaluation of rectal suction biopsies for the ganglion cells and neural hypertrophy is the basic modality for the diagnosis of Hirschsprung's disease (HD). However, the traditional hematoxylin and eosin staining coupled with acetylcholinesterase histochemistry remain challenging, especially in newborns. Thus we conducted a prospective study to evaluate the usefulness of calretinin combined with S100 and protein gene product 9.5 (PGP9.5) immunostaining of rectal suction biopsies for the diagnosis of HD. A total of 195 patients were enrolled in our study...
2016: American Journal of Translational Research
Ahmed Hosni Morsi, Hossam Rashad Omar, Ahmed Osama, Ahmed Refaat Khodary
BACKGROUND: Neonatal pneumoperitoneum is attributed, in most of the reported cases, to necrotising enterocolitis (NEC). There are also other causes leading to free intraperitoneal air. The aim of this study is to describe the clinical spectrum, causes, management and outcome of neonates admitted with pneumoperitoneum in the paediatric surgery unit of a university hospital. SUBJECTS AND METHODS: This retrospective study included neonates having radiographic evidences of pneumoperitoneum from 2012 to the end of 2014...
July 2016: African Journal of Paediatric Surgery: AJPS
Bin Quan, Qiyi Chen, Jun Jiang, Ling Ni, Rongrong Tang, Yu Huang, Yifang Shi, Ning Li
OBJECTIVE: To investigate the safety, efficacy and long-term outcomes of Jinling procedure in the treatment of adult Hirschsprung disease. METHODS: Clinical and follow-up data of 125 patients with adult Hirschsprung disease undergoing Jinling procedure at the Department of General Surgery between January 2000 and January 2013 were summarized. All the patients were diagnosed by CT, barium enema, anorectal pressure detection and pathology examination. Abdominal symptoms, gastrointestinal quality of life index(GIQLI, the lower score, the worse quality of life), Wexner constipation score (higher score indicated worse symptom), defecography (evaluation included rectocele, mucosal prolapse, intramucosal intussusception, perineal prolapse) and other operative complications were compared before and after operation...
July 2016: Zhonghua Wei Chang Wai Ke za Zhi, Chinese Journal of Gastrointestinal Surgery
Harbi Khalayleh, Benjamin Z Koplewitz, Vadim Kapuller, Yaron Armon, Sinan Abu-Leil, Dan Arbell
BACKGROUND: Neonatal sigmoid volvulus is a rare entity. It is associated with Hirschsprung's disease. Presentation is acute abdominal distention, vomiting and obstipation. Abdominal radiograph will show the "coffee bean" sign, but this is frequently missed and the diagnosis requires a high index of suspicion. Treatment options include contrast enema, colonoscopy or laparotomy, depending on the condition of the baby and local availability. POPULATION AND RESULTS: During the last 6years, 6 infants with sigmoid volvulus were treated in our department...
November 2016: Journal of Pediatric Surgery
Anne Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: NEDD4-like ubiquitin protein ligase 2 (NEDL2) plays an important role in many physiological and pathological processes. NEDL2 is a positive regulator of GDNF/Ret signaling during enteric neurogenesis. Mice lacking NEDL2 exhibit decreased numbers of enteric neurons, progressive bowel dysmotility and intestinal hypoganglionosis. We designed this study to investigate the expression of NEDL2 in the normal human colon and in HSCR. METHODS: HSCR tissue specimens (n=10) were collected at the time of pull-through surgery and divided into aganglionic and ganglionic segments...
July 5, 2016: Journal of Pediatric Surgery
Lefteris Stathopoulos, Sebastian K King, Bridget R Southwell, John M Hutson
BACKGROUND/PURPOSE: Long-term problems with faecal incontinence occur in up to 50 % of patients after pull-through for Hirschsprung disease (HSCR). The cause often remains unknown, leading to empirical treatments. Using nuclear transit study, we found some patients surprisingly had rapid proximal colonic transit, suspicious of occult diarrhoea. We aimed to assess whether these patients had unrecognized adverse reactions to food. METHODS: Patients (n = 10, all males, 9...
August 2016: Pediatric Surgery International
J Zimmer, C Tomuschat, P Puri
INTRODUCTION: Several operative techniques have been developed for the treatment of Hirschsprung's disease (HD) in the past decades. Since one-stage transanal pull-through (TAPT) was first performed in 1998, multiple studies have shown favourable short-and midterm results compared to other techniques with shorter operation length, shorter hospital stay and lower complication rates. The aim of this meta-analysis was to determine the longterm results following TAPT for HD. METHODS: A systematic literature search for relevant articles was performed in four databases using the following terms "Hirschsprung/Hirschsprung's disease", "aganglionosis", "transanal", "pullthrough/pull-through", "longterm/long-term" "results", "follow-up" and "outcome"...
August 2016: Pediatric Surgery International
C Tomuschat, J Zimmer, P Puri
PURPOSE: In the last two decades, laparoscopic-assisted pull-through (LAPT) has gained much popularity in the treatment of Hirschsprung's disease. The aim of this meta-analysis was to determine the long-term outcome of patients treated laparoscopically. METHODS: A systematic literature-based search for relevant cohorts was performed using the terms "Hirschsprung's disease and Laparoscopy", "Laparoscopic-assisted pull-through outcome", "Laparoscopic-assisted Soave pull-through" "Laparoscopic-assisted Swenson pull-through" and Laparoscopic-assisted Duhamel pull-through...
August 2016: Pediatric Surgery International
Raj P Kapur
BACKGROUND: Submucosal nerve hypertrophy is a feature of the transition zone in Hirschsprung disease and has been used as a primary diagnostic feature of transition zone pull-through for patients with persistent obstructive symptoms after their initial surgery. Most published criteria for identification of hypertrophy rely on a nerve diameter of greater than 40μm, based primarily on data from a relatively small number of infants with Hirschsprung disease and controls. The validity of these objective measures has not been validated in appropriate controls for post-pull-through patients...
October 2016: Journal of Pediatric Surgery
Patrycja Sosnowska, Michał Błaszczyński, Sebastian Moryciński, Witold Porzucek, Przemysław Mańkowski
INTRODUCTION: Surgical treatment of Hirschsprung's disease may be performed in a single step, or in stages with a temporary stoma. The therapy depends on the clinical condition of the patient and the severity of symptoms. Planned multistage treatment is carried out in two or three steps. AIM: To analyse our 15 years of experience with multistage surgery for the treatment of Hirschsprung's disease, to identify multistage-related factors influencing the course of surgery and hospitalisation, and to evaluate the probability of complications during multistage treatment...
2016: Przegla̜d Gastroenterologiczny
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