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surgery for hirschsprungs

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https://www.readbyqxmd.com/read/28328696/enterocolitis-in-a-child-with-hirschsprung-disease
#1
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/28285706/radiologist-performance-in-the-interpretation-of-contrast-enemas-performed-for-hirschsprung-s-disease-in-children-1-year-of-age
#2
T J Hwang, S Servaes, P Mattei, S A Anupindi
AIM: To evaluate the diagnostic performance of contrast enemas (CEs) for the diagnosis of Hirschsprung's disease (HD). METHODS AND MATERIALS: CE studies performed as part of an HD workup in patients 1-18 years of age over a 10-year period were identified. All abnormal CE studies and an equal number of age-matched controls were included in the final study group. Two radiologists independently and blindly reviewed all CE studies for quality (scale of 0-3) and the presence of large colon calibre, colon redundancy, transition zone, rectosigmoid ratio, and abnormal contractions...
March 9, 2017: Clinical Radiology
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
#3
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
#4
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/28242026/surgical-approach-for-fecal-incontinence-with-a-patulous-anus-after-transanal-pull-through-for-hirschsprung-disease
#5
Yoshitomo Yasui, Syoichi Nishida, Tsubasa Shironomae, Miwa Satomi, Tsuyoshi Kuwahara, Miyuki Kohno
BACKGROUND: We have performed transanal pull-through (TAPT) for Hirschsprung disease since 1998. Some of our patients after TAPT showed a patulous anus and suffered from severe true fecal incontinence. We performed anal canal plasty for these patients and evaluated its efficacy in restoring anorectal function. METHODS: Thirty-one patients who were ≥5years old were previously operated on for Hirschsprung disease, and seven (22.5%) of these were indicated for this procedure...
February 12, 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
#6
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])...
February 2, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28180937/guidelines-for-the-management-of-postoperative-obstructive-symptoms-in-children-with-hirschsprung-disease
#7
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...
February 8, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28154902/guidelines-for-the-diagnosis-and-management-of-hirschsprung-associated-enterocolitis
#8
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...
February 2, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28099057/a-provisional-experience-with-robot-assisted-soave-procedure-for-older-children-with-hirschsprung-disease-back-to-the-future
#9
Girolamo Mattioli, Luca Pio, Lorenzo Leonelli, Barbara Razore, Nicola Disma, Giovanni Montobbio, Vincenzo Jasonni, Paolo Petralia, Alessio Pini Prato
Hirschsprung disease is a congenital disease characterized by intestinal aganglionosis of various extents. Most patients are younger than 1 year of age. Though, a minority of cases can be older or even adult. Older the patient the more difficult and prolonged is the endorectal dissection required for the pull-through procedure. Longer surgery leads to longer anal dilatation and trauma with subsequent higher likelihood of continence impairment. The article aims at describing the first case series of robot-assisted Soave procedure, which was adopted as an alternative minimally invasive approach to older patients with Hirschsprung disease...
January 18, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/28094015/a-nordic-multicenter-survey-of-long-term-bowel-function-after-transanal-endorectal-pull-through-in-200-patients-with-rectosigmoid-hirschsprung-disease
#10
Kristin Bjørnland, Mikko P Pakarinen, Pernilla Stenstrøm, Kjetil J Stensrud, Malla Neuvonen, Anna L Granström, Christina Graneli, Are H Pripp, Einar Arnbjörnsson, Ragnhild Emblem, Tomas Wester, Risto J Rintala
OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records...
January 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28040830/redo-pullthrough-for-hirschsprung-disease
#11
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/28032565/surgical-site-infection-after-stoma-closure-in-children-outcomes-and-predictors
#12
Dani O Gonzalez, Erica Ambeba, Peter C Minneci, Katherine J Deans, Benedict C Nwomeh
BACKGROUND: Surgical site infection (SSI) is a burdensome complication following intestinal stoma closure, with reported rates ranging from 0% to 40%. We aimed to identify risk factors for SSI in children undergoing stoma closure. MATERIALS AND METHODS: Using 2012-2014 NSQIP Pediatric data, we identified patients aged 0-18 years undergoing stoma closure. Demographic, clinical, and 30-day outcome characteristics between children with and without SSI were compared...
November 4, 2016: Journal of Surgical Research
https://www.readbyqxmd.com/read/28003043/genetic-impact-on-the-treatment-management-of-hirschsprung-disease
#13
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/27989362/complications-in-colorectal-surgery
#14
REVIEW
Jason S Frischer, Beth Rymeski
Colorectal pediatric surgery is a diverse field that encompasses many different procedures. The pullthrough for Hirschsprung disease, the posterior sagittal anorectoplasty for anorectal malformations including complex cloaca reconstructions and the ileal pouch anal anastomosis for ulcerative colitis and familial adenomatous polyposis present some of the most technically challenging procedures pediatric surgeons undertake. Many children prevail successfully following these surgical interventions, however, a small number of patients suffer from complications following these procedures...
December 2016: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/27916369/expression-of-connexin-26-and-connexin-43-is-reduced-in-hirschsprung-s-disease
#15
David Coyle, Brian Doyle, Justin M Murphy, Anne Marie O'Donnell, John Gillick, Prem Puri
BACKGROUND: Despite having an optimal pull-through operation, some children with Hirschsprung's disease (HSCR) continue to experience persistent bowel symptoms. Coordinated colonic electrical activity depends on intercellular communication between the enteric nerves, interstitial cells of Cajal (ICCs), smooth muscle cells, and fibroblast-like (platelet-derived growth factor receptor-alpha-positive) cells. Gap junctions are low-resistance channels composed of connexin (Cx) proteins which couple cells electrically and chemically...
November 2016: Journal of Surgical Research
https://www.readbyqxmd.com/read/27912977/functional-outcomes-in-hirschsprung-disease-a-single-institution-s-12-year-experience
#16
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/27898990/european-paediatric-surgeons-association-survey-on-the-management-of-hirschsprung-disease
#17
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...
February 2017: 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
#18
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
#19
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
#20
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...
February 2017: Journal of Pediatric Surgery
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