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Morera C, Nurko S. Hirschsprung's disease. Management. Aliment Pharmacol Ther 2024; 60 Suppl 1:S66-S76. [PMID: 38922939 DOI: 10.1111/apt.18068] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/16/2024] [Revised: 04/08/2024] [Accepted: 05/15/2024] [Indexed: 06/28/2024]
Abstract
BACKGROUND Hirschsprung's disease (HD) is a rare congenital disease that is characterised by the absence of ganglion cells in the myenteric plexus starting in the distal bowel. This results in distal functional obstruction and may lead to complications like enterocolitis. The treatment is surgical and requires the resection of the aganglionic segment, and the pull-through of normal intestine into the anal opening. However, even after successful surgery, patients may continue to have symptoms. AIM Discuss current surgical techniques and management strategies for patients with postoperative symptoms after surgical correction of Hirschsprung's disease. METHODS A review of the literature was done through PubMed, with a focus on clinical management and approach. RESULTS We describe the clinical problems that can occur after surgical correction. These include obstructive symptoms, enterocolitis, or faecal incontinence. A systematic approach for the evaluation of these patients includes the exclusion of anatomic, inflammatory, behavioural or motility related factors. Depending on the severity of the symptoms, the evaluation includes examination under anaesthesia, the performance of contrast studies, endoscopic studies, measurement of anal sphincter function and colonic motility studies. The treatment is focused towards addressing the different pathophysiological mechanisms, and may include medical management, botulinum toxin to the anal sphincter or rarely redo-operation. CONCLUSIONS Patients with Hirschsprung's disease need to have surgical correction, and their postoperative long-term management is complex given a variety of associated problems that can occur after surgery. A systematic evaluation is necessary to provide appropriate therapy.
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Affiliation(s)
- Claudio Morera
- Center for Motility and Functional Gastrointestinal Disorders, Children's Hospital Boston, Boston, Massachusetts, USA
| | - Samuel Nurko
- Center for Motility and Functional Gastrointestinal Disorders, Children's Hospital Boston, Boston, Massachusetts, USA
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Montalva L, Cheng LS, Kapur R, Langer JC, Berrebi D, Kyrklund K, Pakarinen M, de Blaauw I, Bonnard A, Gosain A. Hirschsprung disease. Nat Rev Dis Primers 2023; 9:54. [PMID: 37828049 DOI: 10.1038/s41572-023-00465-y] [Citation(s) in RCA: 25] [Impact Index Per Article: 12.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/12/2023] [Indexed: 10/14/2023]
Abstract
Hirschsprung disease (HSCR) is a rare congenital intestinal disease that occurs in 1 in 5,000 live births. HSCR is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Most patients present during the neonatal period with the first meconium passage delayed beyond 24 h, abdominal distension and vomiting. Syndromes associated with HSCR include trisomy 21, Mowat-Wilson syndrome, congenital central hypoventilation syndrome, Shah-Waardenburg syndrome and cartilage-hair hypoplasia. Multiple putative genes are involved in familial and isolated HSCR, of which the most common are the RET proto-oncogene and EDNRB. Diagnosis consists of visualization of a transition zone on contrast enema and confirmation via rectal biopsy. HSCR is typically managed by surgical removal of the aganglionic bowel and reconstruction of the intestinal tract by connecting the normally innervated bowel down to the anus while preserving normal sphincter function. Several procedures, namely Swenson, Soave and Duhamel procedures, can be undertaken and may include a laparoscopically assisted approach. Short-term and long-term comorbidities include persistent obstructive symptoms, enterocolitis and soiling. Continued research and innovation to better understand disease mechanisms holds promise for developing novel techniques for diagnosis and therapy, and improving outcomes in patients.
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Affiliation(s)
- Louise Montalva
- Department of Paediatric Surgery, Robert-Debré Children's University Hospital, Paris, France.
- Faculty of Health, Paris-Cité University, Paris, France.
- NeuroDiderot, INSERM UMR1141, Paris, France.
| | - Lily S Cheng
- Division of Paediatric Surgery, Texas Children's Hospital, Houston, TX, USA
- Division of Paediatric Surgery, University of Virginia, Charlottesville, VA, USA
| | - Raj Kapur
- Department of Pathology, Seattle Children's Hospital, Seattle, WA, USA
| | - Jacob C Langer
- Division of Paediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
| | - Dominique Berrebi
- Department of Pathology, Robert-Debré and Necker Children's University Hospital, Paris, France
| | - Kristiina Kyrklund
- Department of Paediatric Surgery, Helsinki University Central Hospital, Helsinki, Finland
| | - Mikko Pakarinen
- Department of Paediatric Surgery, Helsinki University Central Hospital, Helsinki, Finland
| | - Ivo de Blaauw
- Department of Surgery, Division of Paediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, Netherlands
| | - Arnaud Bonnard
- Department of Paediatric Surgery, Robert-Debré Children's University Hospital, Paris, France
- Faculty of Health, Paris-Cité University, Paris, France
- NeuroDiderot, INSERM UMR1141, Paris, France
| | - Ankush Gosain
- Department of Paediatric Surgery, Children's Hospital Colorado, Aurora, CO, USA.
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Huerta CT, Ramsey WA, Davis JK, Saberi RA, Gilna GP, Parreco JP, Sola JE, Perez EA, Thorson CM. Nationwide Outcomes of Immediate Versus Staged Surgery for Newborns with Rectosigmoid Hirschsprung Disease. J Pediatr Surg 2023; 58:1101-1106. [PMID: 36959060 DOI: 10.1016/j.jpedsurg.2023.02.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2023] [Accepted: 02/09/2023] [Indexed: 02/20/2023]
Abstract
PURPOSE Debate exists on whether patients with Hirschsprung Disease (HD) should undergo immediate resection during their newborn hospitalization or undergo a staged procedure. This study sought to compare postoperative outcomes among newborns receiving immediate versus staged surgery for rectosigmoid HD. METHODS The Nationwide Readmission Database was queried (2016-2018) for newborns with HD who underwent surgical resection during their newborn hospitalization (immediate) versus planned readmission (staged). Those who did not receive rectal biopsy or had long-segment or total colonic HD were excluded. A propensity score-matched analysis (PSMA) of patients receiving either surgery was constructed utilizing >70 comorbidities. Outcomes were analyzed using standard statistical tests. RESULTS 1,048 newborns with HD were identified (56% immediate vs. 44% staged). Staged resection was associated with higher total hospitalization cost ($56,642 vs. $50,166 immediate), p = 0.014. After PSMA, the staged cohort was more likely to require home healthcare at discharge and experience unplanned readmission (40% vs. 23%). These patients experienced more gastrointestinal complications (40% vs. 22%) on readmission, especially Hirschsprung-associated enterocolitis (35% vs. 20%). CONCLUSION Newborns receiving staged procedures for HD experience higher rates of unplanned readmission complications and incur higher hospitalization costs. This information should be utilized to defray healthcare utilization costs for newborns with HD. TYPE OF STUDY Retrospective Comparative. LEVEL OF EVIDENCE III.
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Affiliation(s)
- Carlos Theodore Huerta
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Walter A Ramsey
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Jenna K Davis
- University of Miami Miller School of Medicine, Miami, FL, USA
| | - Rebecca A Saberi
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Gareth P Gilna
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Joshua P Parreco
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Juan E Sola
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Eduardo A Perez
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA
| | - Chad M Thorson
- DeWitt Daughtry Family Department of Surgery, Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
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Systematic appraisal of the guidelines for the diagnosis and treatment of Hirschsprung's disease. Pediatr Surg Int 2022; 38:1197-1208. [PMID: 35867125 DOI: 10.1007/s00383-022-05151-w] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/04/2022] [Indexed: 10/17/2022]
Abstract
BACKGROUND Some guidelines for management of Hirschsprung's disease (HSCR, HD) have been developed, but their quality is vague. This study will systematically assess the quality of guidelines and analyze the key recommendations and the best evidence for guidelines. METHODS Applicable guidelines were retrieved using a systematic search of databases. The Appraisal of Guidelines for Research and Evaluation II (AGREE II) tool was used to assess the quality of the guidelines. Then, the recommendations and evidence for the included guidelines were extracted and compared. RESULTS A total of nine guidelines were included in this study, and only one had an overall standardized score of more than 60%, indicating that it is worthy of recommendation. The problems identified included ambiguous and low-quality evidence; obvious distributional heterogeneity among the recommendations; a lack of in-depth discussion on the interpretation of staging, diagnostic methods, conservative treatment, and surgical staging of disease. CONCLUSION The quality of guidelines varies widely, and there is a lack of high-quality professional opinions and supporting evidence for the main recommendations. At present, only comprehensive guidelines can be considered high-quality and there is still room for improvement.
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Surgical approach to Hirschsprung disease. Semin Pediatr Surg 2022; 31:151156. [PMID: 35690469 DOI: 10.1016/j.sempedsurg.2022.151156] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
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Manzoor A, Talat N, Adnan HM, Zia MW, Aziz MA, Ahmed E. Contrast Enema: Solving Diagnostic Dilemmas in Neonates With Lower Intestinal Obstruction. Cureus 2022; 14:e23458. [PMID: 35495017 PMCID: PMC9038504 DOI: 10.7759/cureus.23458] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/24/2022] [Indexed: 11/05/2022] Open
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Freedman-Weiss MR, Chiu AS, Caty MG, Solomon DG. Delay in operation for Hirschsprung Disease is associated with decreased length of stay: a 5-Year NSQIP-Peds analysis. J Perinatol 2019; 39:1105-1110. [PMID: 31209278 DOI: 10.1038/s41372-019-0405-y] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2018] [Revised: 05/02/2019] [Accepted: 05/03/2019] [Indexed: 12/21/2022]
Abstract
OBJECTIVE The optimal timing of a pull-through procedure for Hirschsprung Disease is unknown. We, therefore, compared outcomes of pull-throughs performed in the first 30 days of age to 31-120 days. STUDY DESIGN Retrospective review of 282 patients in the NSQIP-Peds database from 2012-2016 of infants ≤120-days old and >36-weeks gestational age with Hirschsprung Disease who underwent primary pull-through. Primary outcome was postoperative and total length of stay (LOS). Operative morbidity and readmissions were also compared. RESULTS Postoperative LOS in <31-day group was 8.3 days (SD- 8.3) vs. 4.3 days (SD- 5.5) in 31-120-day group (p < 0.001). This finding was maintained on multivariate linear regression. Complication and readmission rates did not differ between groups (readmission: 15.6 vs 13% p = 0.51; complication: 5.5 vs 10% p = 0.16). CONCLUSION For appropriately selected patients with Hirschsprung Disease, delaying pull-through until the second month of life is associated with lower total and postoperative stays without increased readmissions or complications.
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Affiliation(s)
| | - Alexander S Chiu
- Department of Surgery, Yale School of Medicine, New Haven, CT, USA
| | - Michael G Caty
- Department of Surgery, Yale School of Medicine, New Haven, CT, USA
| | - Daniel G Solomon
- Department of Surgery, Yale School of Medicine, New Haven, CT, USA.
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Jiao CL, Chen XY, Feng JX. Novel Insights into the Pathogenesis of Hirschsprung's-associated Enterocolitis. Chin Med J (Engl) 2017; 129:1491-7. [PMID: 27270548 PMCID: PMC4910376 DOI: 10.4103/0366-6999.183433] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Objective: To systematically summary the updated results about the pathogenesis of Hirschsprung's-associated enterocolitis (HAEC). Besides, we discussed the research key and direction based on these results. Data Sources: Our data cited in this review were obtained mainly from PubMed from 1975 to 2015, with keywords “Hirschsprung enterocolitis”, “Hirschsprung's enterocolitis”, “Hirschsprung's-associated enterocolitis”, “Hirschsprung-associated enterocolitis”, “HAEC”, and “EC”. Study Selection: Articles regarding the pathogenesis of HAEC were selected, and the articles mainly regarding the diagnosis, surgical approach, treatment, and follow-up were excluded. Results: Several factors, mainly including mucus barrier, intestinal microbiota, and immune function, as well as some other factors such as genetic variations and surgical reasons, have been found to be related to the pathogenesis of HAEC. Changed quantity and barrier property of mucus, different composition of microbiota, and an abnormal immune state work together or separately trigger HAEC. Conclusions: The maintenance of intestinal homeostasis is due to a well cooperation of microbiota, mucus barrier, and immune system. If any part presents abnormal, intestinal homeostasis will be broken. Meanwhile, for patients with Hirschsprung's disease or HAEC, dysfunction of these parts has been found. Thus, the happening of HAEC may be mainly attributed to the disorders of intestinal microbiota, mucus barrier, and immune system.
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Affiliation(s)
- Chun-Lei Jiao
- Department of Pediatric Surgery, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei 430000, China
| | - Xu-Yong Chen
- Department of Pediatric Surgery, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei 430000, China
| | - Jie-Xiong Feng
- Department of Pediatric Surgery, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei 430000, China
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Bandi AS, Bradshaw CJ, Giuliani S. Advances in minimally invasive neonatal colorectal surgery. World J Gastrointest Surg 2016; 8:670-678. [PMID: 27830038 PMCID: PMC5081548 DOI: 10.4240/wjgs.v8.i10.670] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/06/2016] [Revised: 07/27/2016] [Accepted: 08/18/2016] [Indexed: 02/06/2023] Open
Abstract
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. There does still remain significant variation in the procedures used and this review aims to report the current literature comparing techniques with an emphasis on the short- and long-term clinical outcomes.
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Abstract
BACKGROUND How to manage a late diagnosed Hirschsprung's disease (HD) and how to avoid calibre discrepancy? SUBJECTS AND METHODS A retrospective study of all patients diagnosed with HD over 2 years in our hospital from January 2009 to December 2012. Data were analysed for clinical presentations, investigations, surgical procedures and post-operative outcome. RESULTS Fifteen patients, operated by one single surgeon, were included in this study. The mean age was 6 years (2-16 years). Patients had an ultra-short segment type in 4 cases, rectosigmoid type in 9 cases and descending colonic aganglionosis in 2 cases. Rectal wash out was effective in 12 patients. A blowhole transverse colostomy was performed in 2 patients. Twelve patients underwent one single stage endorectal pull-through. Anastomosis incongruence was avoided by a plication procedure never described before. The assessment of post-operative outcomes by the paediatric incontinence and constipation scoring system revealed a normal continence function in all our patients, but 3 patients suffered from soiling secondary to constipation. CONCLUSION One single stage pull-through can be safe and effective in children with late diagnosed HD. Routine rectal washout is a good way to prepare the colon. In some cases, blowhole colostomy can be an option. Anastomosis incongruence is a challenge; we describe a plication procedure to avoid it.
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Affiliation(s)
- Mohamed Ouladsaiad
- Department of Pediatric Surgery, School of Medicine, Mohammed VI University Hospital, Cadi Ayyad University, Marrakesh, Morocco
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Almetaher HA, Hassan HS, Elhalaby EA. Current management of Hirschsprung’s disease in Egypt. ANNALS OF PEDIATRIC SURGERY 2015. [DOI: 10.1097/01.xps.0000472751.68453.56] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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Stamp LA, Obermayr F, Pontell L, Young HM, Xie D, Croaker DH, Song ZM, Furness JB. Surgical Intervention to Rescue Hirschsprung Disease in a Rat Model. J Neurogastroenterol Motil 2015; 21:552-9. [PMID: 26424040 PMCID: PMC4622138 DOI: 10.5056/jnm15079] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2015] [Revised: 06/17/2015] [Accepted: 06/30/2015] [Indexed: 01/11/2023] Open
Abstract
Background/Aims Rats with a spontaneous null mutation in endothelin receptor type B or Ednrb (sl/sl; spotting lethal) lack enteric neurons in the distal bowel and usually die within the first week after birth. This early postnatal lethality limits their use for examining the potential of cell therapy to treat Hirschsprung disease, and for studies of the influence of EDNRB on the mature CNS and vascular systems. Methods We have developed a surgical intervention to prolong the life of the spotting lethal sl/sl rat, in which we perform a colostomy on postnatal (P) day 4–6 rats to avoid the fatal obstruction caused by the lack of colonic enteric neurons. Results The stomas remained patent and functional and the rats matured normally following surgery. Weight gains were comparable between control and Hirschsprung phenotype (sl/sl) rats, which were followed until 4 weeks after surgery (5 weeks old). We confirmed the absence of enteric neurons in the distal colon of rats whose lives were saved by the surgical intervention. Conclusions This study provides a novel approach for studying EDNRB signalling in multiple organ systems in mature rats, including an animal model to study the efficacy of cell therapy to treat Hirschsprung disease.
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Affiliation(s)
- Lincon A Stamp
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, VIC, Australia
| | - Florian Obermayr
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, VIC, Australia.,Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital Tuebingen, Tuebingen, Germany
| | - Louise Pontell
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, VIC, Australia
| | - Heather M Young
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, VIC, Australia
| | - Dan Xie
- Eccles Institute of Neuroscience, John Curtin School of Medical Research and Medical School, Australian National University, Canberra, ACT, Australia
| | - David H Croaker
- Department of Pediatrics and Child Health, Canberra Hospital, Canberra, Australia
| | - Zan-Min Song
- Eccles Institute of Neuroscience, John Curtin School of Medical Research and Medical School, Australian National University, Canberra, ACT, Australia
| | - John B Furness
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, VIC, Australia
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Obermayr F, Hotta R, Enomoto H, Young HM. Development and developmental disorders of the enteric nervous system. Nat Rev Gastroenterol Hepatol 2013; 10:43-57. [PMID: 23229326 DOI: 10.1038/nrgastro.2012.234] [Citation(s) in RCA: 148] [Impact Index Per Article: 12.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
The enteric nervous system (ENS) arises from neural crest-derived cells that migrate into and along the gut, leading to the formation of a complex network of neurons and glial cells that regulates motility, secretion and blood flow. This Review summarizes the progress made in the past 5 years in our understanding of ENS development, including the migratory pathways of neural crest-derived cells as they colonize the gut. The importance of interactions between neural crest-derived cells, between signalling pathways and between developmental processes (such as proliferation and migration) in ensuring the correct development of the ENS is also presented. The signalling pathways involved in ENS development that were determined using animal models are also described, as is the evidence for the involvement of the genes encoding these molecules in Hirschsprung disease-the best characterized paediatric enteric neuropathy. Finally, the aetiology and treatment of Hirschsprung disease in the clinic and the potential involvement of defects in ENS development in other paediatric motility disorders are outlined.
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Affiliation(s)
- Florian Obermayr
- Department of Pediatric Surgery, University Children's Hospital, University of Tübingen, Hoppe-Seyler Straße 3, Tübingen 72076, Germany
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Abstract
The surgical correction of Hirschsprung disease has undergone a complete evolution in the past decade. Refinements in the performance of both transanal and laparoscopic procedures have tremendously facilitated the advancement of these surgeries. This chapter presents the history of these procedures, and then discusses the various approaches and details of these techniques.
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Affiliation(s)
- Jacob C Langer
- Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
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Stensrud KJ, Emblem R, Bjørnland K. Late diagnosis of Hirschsprung disease--patient characteristics and results. J Pediatr Surg 2012; 47:1874-9. [PMID: 23084200 DOI: 10.1016/j.jpedsurg.2012.04.022] [Citation(s) in RCA: 34] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/22/2011] [Revised: 03/26/2012] [Accepted: 04/28/2012] [Indexed: 10/27/2022]
Abstract
PURPOSE The aim of the present study was to describe the characteristics and the postoperative results of children diagnosed as having Hirschsprung disease (HD) after the age of 3 years. METHODS All patients with HD diagnosed after the age of 3 years in our hospital from 1998 to 2011 were included. Patient characteristics and postoperative results were prospectively registered. RESULTS Eleven children were included. Age at diagnosis was 3.0 to 9.6 years. Ten patients had rectosigmoid disease, whereas 1 had total colonic aganglionosis. Three children were given a diverting ileostomy before the pull-through procedure, and all 3 had ileostomy-related complications. Early postoperative complications were seen in 5 children, of whom 2 had anastomotic leakage. At final follow-up, with a median of 3 years postoperatively, 7 had normal bowel function, 1 had frequent loose stools, and 3 were soiling. CONCLUSIONS Early postoperative complications, especially anastomotic leakage, occurred frequently in children with late-diagnosed HD. Therefore, a diverting stoma should be considered in these patients. The long-term functional results were comparable with those seen in children operated on as neonates.
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One-stage transanal endorectal pull-through for treatment of hirschsprung's disease in adolescents and adults. J Gastrointest Surg 2011; 15:2246-50. [PMID: 21909845 DOI: 10.1007/s11605-011-1662-z] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/31/2011] [Accepted: 08/09/2011] [Indexed: 01/31/2023]
Abstract
BACKGROUND One-stage pull-through operation has become increasingly popular for treatment of Hirschsprung's disease. The one-stage transanal pull-through was introduced in the late 1990s and has rapidly replaced traditional procedures in infants and young children in many surgical centers. OBJECTIVE The aim of this study is to determine feasibility and safety of transanal primary repair in adolescent and adults. METHODS Fifteen patients who underwent transanal endorectal pull-through were prospectively studied. All patients presented by chronic refractory constipation with the age ranged from 11 to 22 years. The patients were followed up for a mean of 18 months. Anal continence and postoperative complication were evaluated. RESULTS Incomplete continence in the form of soiling occurred in four patients (26.6%) and improved gradually with conservative management. No patients suffered from complete incontinence. Anastomotic strictures occurred in two patients and were successfully treated with regular dilatations. One patient had continued outlet obstruction and revision was considered for him. One patient complicated with low perianal fistula which needed fistulectomy. There was no impotence in adults. CONCLUSION These findings indicate that one-stage transanal endorectal pull-through operation in adolescent and adults is feasible and safe.
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Bradnock TJ, Walker GM. Evolution in the management of Hirschsprung's disease in the UK and Ireland: a national survey of practice revisited. Ann R Coll Surg Engl 2010; 93:34-8. [PMID: 20738896 DOI: 10.1308/003588410x12771863936846] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
INTRODUCTION The management of Hirschsprung's disease continues to evolve. This questionnaire survey aimed to determine current surgical management strategies for Hirschsprung's disease in Britain. SUBJECTS AND METHODS The survey was sent electronically to all British paediatric surgeons. Initial questions explored individual experience and regional service provision. Additional questions, reserved for surgeons who perform definitive Hirschsprung's disease surgery, addressed specific clinical scenarios. RESULTS Surveys were sent to 142 surgeons yielding 85 responses. After exclusions, 64 surveys from 21 centres were analysed. Forty-seven respondents worked in centres with designated 'Hirschsprung's disease surgeons'. Forty respondents perform definitive Hirschsprung's disease surgery. In a well neonate with left-sided Hirschsprung's disease, 34 of 40 surgeons favour primary pull-through following bowel decompression with rectal washouts; 35 of 40 surgeons aim to perform definitive surgery at less than 3 months of age, with 17 favouring laparoscopic-assisted Soave-Boley and 15 favouring an open Duhamel pull-through. Of the 40 surgeons, 36 use a staged approach to right-sided/total colonic Hirschsprung's disease with 23 favouring a Duhamel or Long Duhamel pull-through. CONCLUSIONS The primary pull-through, using an open Duhamel or laparoscopic-assisted Soave-Boley technique, during the first 3 months of life, has become the operative strategy of choice in rectosigmoid Hirschsprung's disease in Britain. Marked variation in practice remains for right-sided Hirschsprung's disease.
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Affiliation(s)
- T J Bradnock
- Department of Paediatric Surgery, Royal Hospital for Sick Children, Glasgow, UK.
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De La Torre L, Langer JC. Transanal endorectal pull-through for Hirschsprung disease: technique, controversies, pearls, pitfalls, and an organized approach to the management of postoperative obstructive symptoms. Semin Pediatr Surg 2010; 19:96-106. [PMID: 20307846 DOI: 10.1053/j.sempedsurg.2009.11.016] [Citation(s) in RCA: 100] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2023]
Abstract
The transanal endorectal pull-through emerged in the late 1990s as the most recent step in the evolution of the surgical correction of Hirschsprung disease. This operation provides the advantages of a minimal access approach with shorter hospital stay, shorter time to full feeding, less pain, and improved cosmesis with excellent outcomes. This article will review the technical principles of the transanal endorectal pull-through, and will address ongoing controversies in the application of this technique. We will also discuss an organized approach to the problem of obstructive symptoms that may affect a subgroup of patients after the transanal pull-through.
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Affiliation(s)
- Luis De La Torre
- Universidad Nacional Autónoma de México, Department of Surgery, Hospital para el Niño Poblano and Hospital Angeles Puebla, Puebla 72190, Mexico.
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Initial experience with Soave's transabdominal pull-through: An observational study. Int J Surg 2010; 8:225-8. [DOI: 10.1016/j.ijsu.2009.11.016] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2009] [Accepted: 11/22/2009] [Indexed: 11/20/2022]
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Abstract
Hirschsprung disease is a relatively common condition managed by pediatric surgeons. Significant advances have been made in understanding its etiologies in the last decade, especially with the explosion of molecular genetic techniques and early diagnosis. The surgical management has progressed from a two- or three-stage procedure to a primary operation. More recently, definitive surgery for Hirschsprung disease through minimally invasive techniques has gained popularity. In neonates, the advancement of treatment strategies for Hirschsprung disease continues with reduced patient morbidity and improved outcomes.
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Affiliation(s)
- Ramanath N Haricharan
- Division of Pediatric Surgery, Department of General Surgery, University of Alabama at Birmingham, Birmingham, Alabama 35233, USA
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Vick LR, Gosche JR, Boulanger SC, Islam S. Primary laparoscopic repair of high imperforate anus in neonatal males. J Pediatr Surg 2007; 42:1877-81. [PMID: 18022439 DOI: 10.1016/j.jpedsurg.2007.07.014] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
PURPOSE The standard approach to males with high imperforate anus has been a staged procedure starting with a descending colostomy, then posterior sagittal anorectoplasty with colostomy closure after 3 months. Recently, a minimally invasive approach to the repair of high imperforate anus has been described in infants after colostomy. We describe 6 newborn males with high imperforate anus successfully repaired laparoscopically as a primary, single-stage procedure. METHODS A retrospective chart review was performed on all patients with imperforate anus from October 2003 to October 2006. RESULTS We evaluated 9 newborn males with high imperforate anus. Of these patients, 6 underwent primary laparoscopic repair on day 1 to day 2 of life. Of these 6 patients, 3 were found to have bladder neck fistulas, whereas the other 3 had prostatic urethra fistulas. All patients passed stool within the first 72 hours postoperatively. One patient has required a procedure for a mild rectal prolapse. Follow-up ranges from 2 to 30 months in the single-stage group. CONCLUSION Our early results using primary laparoscopic repair appear encouraging. Laparoscopy allows excellent visualization and assessment of the fistula and repair of high imperforate anus without need for colostomy. Long-term follow-up will be needed to assess outcomes and continence rates.
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Affiliation(s)
- Laura R Vick
- Division of Pediatric Surgery, Department of Surgery, University of Mississippi Medical Center, Jackson, MS 39216, USA
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