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surgery for hirschsprungs

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https://www.readbyqxmd.com/read/29778545/a-descriptive-model-for-a-multidisciplinary-unit-for-colorectal-and-pelvic-malformations
#1
Alejandra Vilanova-Sanchez, Devin R Halleran, Carlos A Reck-Burneo, Alessandra C Gasior, Laura Weaver, Meghan Fisher, Andrea Wagner, Onnalisa Nash, Kristina Booth, Kaleigh Peters, Charae Williams, Sarah Mayer Brown, Peter Lu, Molly Fuchs, Karen Diefenbach, Jeffrey R Leonard, Geri Hewitt, Kate McCracken, Carlo Di Lorenzo, Richard J Wood, Marc A Levitt
INTRODUCTION: Patients with anorectal malformations (ARM), Hirschsprung disease (HD), and colonic motility disorders often require care from specialists across a variety of fields, including colorectal surgery, urology, gynecology, and GI motility. We sought to describe the process of creating a collaborative process for the care of these complex patients. METHODS: We developed a model of a devoted center for these conditions that includes physicians, psychologists, social workers, nurses, and advanced practice nurses...
April 19, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29682507/bacterial-microbiome-dynamics-in-post-pull-through-hirschsprung-associated-enterocolitis-haec-an-experimental-study-employing-the-endothelin-receptor-b-null-mouse-model
#2
Zhi Cheng, Lifu Zhao, Deepti Dhall, Paul M Ruegger, James Borneman, Philip K Frykman
Purpose: Hirschsprung-associated enterocolitis (HAEC) is the most frequent potentially life-threatening complication in children with Hirschsprung disease (HSCR) even after definitive corrective surgery. Mounting evidence suggests that intestinal microbiota likely contribute to the etiology of enterocolitis, so the aim of this study was to use a mouse model of post pull-through HAEC to compare the fecal bacterial communities of animals which developed HAEC to those free of enterocolitis...
2018: Frontiers in Surgery
https://www.readbyqxmd.com/read/29681704/cytomegalovirus-as-a-cause-of-colonic-stricture-simulating-hirschsprung-s-disease
#3
Chiranjiv Kumar, Meera Luthra
Colonic stenosis/atresias account for only 5%-15% of all atresias. Colonic stenosis is rarer than atresia and mostly occurs in ascending and transverse colon. Gastrointestinal sequelae of cytomegalovirus (CMV) are rare, frequently requires surgical intervention for diagnosis and management. We describe a 5-month-old female child with complaints of recurrent abdominal distension, vomiting, and constipation for 2 months simulating Hirschsprung's disease. After barium enema, the baby was taken up for surgery. Intraoperatively, we found a colonic stricture in the descending colon...
April 2018: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/29605259/gene-expression-profiling-coupled-with-connectivity-map-database-mining-reveals-potential-therapeutic-drugs-for-hirschsprung-disease
#4
Shang-Jie Xiao, Xiao-Chun Zhu, Hua Deng, Wei-Ping Zhou, Wen-Yi Yang, Li-Ke Yuan, Jiang-Yu Zhang, Song Tian, Lu Xu, Liang Zhang, Hui-Min Xia
BACKGROUND: Hirschsprung disease (HD) is a congenital intestinal anomaly resulting from a failure to form enteric ganglia in the lower bowel. Surgery is the main therapeutic strategy, although neural stem cell transplantation has recently shown promise. However, HD remains a challenging disorder to treat. Our aim was to identify drugs that could counteract the dysregulated pathways in HD and could thus be potential novel therapies. METHODS: We used microarray analysis to identify genes differentially expressed in ganglionic and aganglionic bowel samples from eight children with HD...
March 4, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29467083/bilateral-hydrosalpinx-in-patients-with-hirschsprung-s-disease
#5
Pedro Palazón, Laura Saura, Irene de Haro, Oriol Martín-Solé, Asteria Albert, Xavier Tarrado, Victoria Julià
PURPOSE: Hirschsprung's disease (HD) is uncommon in females. There are very few reports on the patients' obstetric and gynecological outcome. Hydrosalpinx causes pain and infertility. It is rare in nonsexually active teenagers. It may be because of an intrinsic disease of the fallopian tubes or secondary to surgery. AIM: to describe the relationship between hydrosalpinx and HD or its surgical approach; to report the impact of bilateral hydrosalpinx on fertility in HD...
January 31, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29458231/perioperative-complications-of-transanal-pull-through-surgery-for-hirschsprung-s-disease
#6
REVIEW
Mikko Pakarinen
Transanal pull-through is currently one of the most commonly used operation to treat Hirschsprung's disease (HD) worldwide. It has undergone some technical modifications during the last decades and still contains variable surgical practices. As high quality comparative studies between different surgical approaches are missing, debate over the optimal operation for HD still continues. Transanal pull-through is associated with multiple potential complications, which may result in permanently impaired functional outcome, life-long morbidity, and significant social restrictions...
April 2018: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29419957/-risk-factors-associated-with-the-development-of-enterocolitis-in-hirschsprung-s-disease
#7
M I Romo Muñoz, A Martínez de Aragón, V Núñez Cerezo, C Udaondo, M Sellers, S Barrena, M De Ceano, M López Santamaría, L Martínez Martínez
OBJECTIVES: Hirschsprung's-associated enterocolitis (HAEC) is a live-threatening complication that remains badly understood. Our objective is to identify the risk factors related to the development of HAEC in the cohort of patients with Hirschsprung's disease (HD) treated in our center. METHODS: We reviewed the patients treated for HD between 2000 and 2016. Ninety four patients were included, and the clinical details related to the disease were evaluated. Our primary outcome measure was the development of HAEC...
February 1, 2018: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/29368076/the-socioeconomic-impact-of-a-pediatric-ostomy-in-uganda-a-pilot-study
#8
Arlene Muzira, Nasser Kakembo, Phyllis Kisa, Monica Langer, John Sekabira, Doruk Ozgediz, Tamara N Fitzgerald
INTRODUCTION: Multiple pediatric surgical conditions require ostomies in low-middle-income countries. Delayed presentations increase the numbers of ostomies. Patients may live with an ostomy for a prolonged time due to the high backlog of cases with insufficient surgical capacity. In caring for these patients in Uganda, we frequently witnessed substantial socioeconomic impact of their surgical conditions. METHODS: The operative log at the only pediatric surgery referral center in Uganda was reviewed to assess the numbers of children receiving ostomies over a 3-year period...
January 24, 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29336779/prophylactic-thyroidectomies-in-men2-syndrome-management-and-outcomes
#9
Virginie Bussières, Shreyas Roy, Johnny Deladoey, Élisabeth Rousseau, Dickens St-Vil, Nelson Piché
AIM OF THE STUDY: The aim of the study was to evaluate the outcomes of prophylactic thyroidectomies performed in an academic setting in the context of multiple endocrine neoplasia type 2 (MEN2) syndrome. METHODS: A chart review of patients <18years old who underwent prophylactic thyroidectomy for a MEN2 syndrome at a children's hospital between 2006 and 2015 was performed. MAIN RESULTS: The study included 21 patients (57% female) with a mean age of 6...
February 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29300049/hirschsprung-disease-integrating-basic-science-and-clinical-medicine-to-improve-outcomes
#10
REVIEW
Robert O Heuckeroth
Hirschsprung disease is defined by the absence of enteric neurons at the end of the bowel. The enteric nervous system (ENS) is the intrinsic nervous system of the bowel and regulates most aspects of bowel function. When the ENS is missing, there are no neurally mediated propulsive motility patterns, and the bowel remains contracted, causing functional obstruction. Symptoms of Hirschsprung disease include constipation, vomiting, abdominal distension and growth failure. Untreated disease usually causes death in childhood because bloodstream bacterial infections occur in the context of bowel inflammation (enterocolitis) or bowel perforation...
March 2018: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29280111/-choice-of-surgical-procedures-and-control-of-surgical-risks-in-chronic-constipation
#11
Jinke Sui, Wei Zhang
Surgery, as one of the methods for the treatment of chronic constipation, is the final choice after the failure of non-surgical treatment with its specific particularity. The history of surgical treatment of chronic constipation is complex and tortuous. How to select operation among many kinds of surgery, and control risk is difficult for clinician. The choice of surgical procedure depends mainly on the patient's conditions, the objective examination basis and the experience of physician teams. Based on the previous reports and the team's experience, this paper discusses the choice of surgical treatment for the following types of chronic constipation: (1) Slow transit constipation: subtotal colorectal resection plus ileorectal anastomosis or ascending colon rectum anastomosis is widely used at present in the domestic, and its efficacy is quite good...
December 25, 2017: Zhonghua Wei Chang Wai Ke za Zhi, Chinese Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/29238171/a-5-year-follow-up-study-of-neonates-with-hirschsprung-s-disease-undergoing-transanal-soave-or-swenson-surgery
#12
Jianjun Zhang, Tongsheng Ma, Yuan Peng, Guangfeng Huang, Fengli Liu
Objective: To investigate different outcomes and long-term efficacy of transanal Soave or Swenson surgery in treatment for neonates with Hirschsprung's disease (HD). Methods: In the present study, a total of 29 neonatal patients were included, with 20 patients undergoing the Soave procedure and nine patients undergoing the Swenson procedure. Data collected from the patients included basic demographics, age and weight at the time of operation, pathological typing, type of operation, operative time, blood loss, length of hospital stay, bowel function, and complications...
2017: Patient Preference and Adherence
https://www.readbyqxmd.com/read/29209677/laparoscopic-assisted-endorectal-pull-through-for-hirschsprung-s-disease-a-retrospective-study
#13
Xiaogang Li, Xiaoyun Li, Jun Cheng, Yongkang Zhang, Wei Zou, Fei Xie, Zhenggui Tao, Shaotao Tang, Mingguo Du, Xiaofeng Liao
OBJECTIVES: To summarize the efficacy of the laparoscopic-assisted transanal-endorectal pull-through procedure for Hirschsprung's disease (HD). METHODS: Between May 2006 and May 2013, 22 children with HD undergoing laparoscopic-assisted endorectal pull-through procedures were retrospectively analyzed. The operative time, pathology, intraoperative blood loss, recovery time for gastrointestinal function, postoperative hospital stay, complications, and defecation functions of the patients were analyzed...
December 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/29190183/a-long-term-follow-up-of-a-new-surgery-method-laparoscope-assisted-heart-shaped-anastomosis-for-hirschsprung-s-disease
#14
Chunlei Jiao, Donghai Yu, Dandan Li, Guo Wang, Jiexiong Feng
BACKGROUND: Laparoscopic surgery is widely used in the treatment of Hirschsprung's disease (HD). However, constipation and soiling are still the main long-term complications. A new type of anastomosis, which is characterized by a heart-shaped colorectal anastomosis after splitting the posterior rectum wall to 0.5 cm above the dentate line, has been improved by our medical center. The purpose of this study was to determine the long-term effects of laparoscope-assisted heart-shaped anastomosis (LHSA) and to compare it with a more generally applied approach, the laparoscope-assisted Soave procedure (LSP)...
November 30, 2017: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/29170900/the-challenges-of-closing-an-ileostomy-in-patients-with-total-intestinal-aganglionosis-after-small-bowel-transplant
#15
Fereshteh Salimi Jazi, Tiffany J Sinclair, Chad M Thorson, Ricardo Castillo, Andrew C Bonham, Carlos O Esquivel, Matias Bruzoni
We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung's disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were interested in closing the ileostomy and undergoing pull-through surgery. The complexity of the case includes the presence of an aganglionic rectal segment-a short root of the mesentery due to the small bowel transplant-and significant immunosuppression...
January 2018: Pediatric Surgery International
https://www.readbyqxmd.com/read/29162749/role-of-synaptophysin-in-the-intraoperative-assessment-of-quadrantic-innervation-of-the-proximal-doughnut-in-hirschsprung-disease
#16
Lokendra Yadav, M K Babu, Kanishka Das, Suravi Mohanty, P Divya, Gowri Shankar, Usha Kini
BACKGROUND: Symptoms may persist in a retained aganglionic segment of the colon after corrective (pull-through) surgery in Hirschsprung disease (HD). Thus, it is important to assess the proximal doughnut for innervation abnormalities intraoperatively by frozen sections stained with conventional haematoxylin and eosin stain and supported by rapid acetylcholinesterase (AChE) histochemistry. When the doughnut is proximal to the sigmoid colon, AChE is not useful and requires ratification by yet another rapid technique and hence this study...
July 2017: National Medical Journal of India
https://www.readbyqxmd.com/read/29127965/minimally-invasive-neonatal-surgery-hirschsprung-disease
#17
REVIEW
Atsuyuki Yamataka, Go Miyano, Masahiro Takeda
Transanal pull-through (TAPT) is the procedure of choice for treating Hirschsprung disease and should be performed with laparoscopic assistance using the anorectal line (ARL) to ensure optimum postoperative bowel function (POBF). The dentate line (DL) has traditionally been used as the landmark for commencing dissection during TAPT, but we prefer the ARL because the DL is too subjective and can be associated with risk for injury to delicate sensory innervation required for normal defecation in the anal transition zone...
December 2017: Clinics in Perinatology
https://www.readbyqxmd.com/read/29079318/transitions-in-care-from-pediatric-to-adult-general-surgery-evaluating-an-unmet-need-for-patients-with-anorectal-malformation-and-hirschsprung-disease
#18
Sarah B Cairo, Priscilla P L Chiu, Roshni Dasgupta, Karen A Diefenbach, Allan M Goldstein, Nicholas A Hamilton, Andrea Lo, Michael D Rollins, David H Rothstein
BACKGROUND: The provision of timely and comprehensive transition of care from pediatric to adult surgical providers for patients who have undergone childhood operations remains a challenge. Understanding the barriers to transition from a patient and family perspective may improve this process. METHODS: A cross-sectional survey was conducted of patients with a history of anorectal malformation (ARM) or Hirschsprung Disease (HD) and their families. The web-based survey was administered through two support groups dedicated to the needs of individuals born with these congenital abnormalities...
October 7, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28985842/increased-population-of-immature-enteric-glial-cells-in-the-resected-proximal-ganglionic-bowel-of-hirschsprung-s-disease-patients
#19
Gakuto Tani, Christian Tomuschat, Anne Marie O'Donnell, David Coyle, Prem Puri
BACKGROUND: Enteric glial cells are essential for normal gastrointestinal function. Abnormalities in glial structure, development, or function lead to disturbances in gastrointestinal physiology. Fatty acid-binding protein 7 (FABP7) is a marker of immature enteric glial cells, whereas S100 is expressed only by mature glial cells. Patients with Hirschsprung's disease (HSCR) often suffer from dysmotility and enterocolitis despite proper surgery. We designed this study to determine the distribution and expression of glial cells in patients with HSCR compared to normal controls...
October 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28983688/inflammatory-bowel-disease-in-patients-with-hirschsprung-s-disease-a-systematic-review-and-meta-analysis
#20
H Nakamura, T Lim, P Puri
AIM AND OBJECTIVES: Hirschsprung-associated enterocolitis (HAEC) continues to be an important cause of morbidity in patients with Hirschsprung's disease (HSCR). HAEC can occur at any time during the course of the disease. The reported incidence of HAEC before surgery ranges from 6 to 50%, and after surgery, it ranges from 2 to 35%. HAEC and inflammatory bowel disease (IBD) have similar clinical presentation including diarrhea, hematochezia, and abdominal pain. In recent years, isolated cases of IBD have been reported in patients who had surgical treatment for HSCR...
February 2018: Pediatric Surgery International
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