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Xu TO, Levitt MA, Feng C. Controversies in Hirschsprung surgery. WORLD JOURNAL OF PEDIATRIC SURGERY 2024; 7:e000887. [PMID: 39346552 PMCID: PMC11429006 DOI: 10.1136/wjps-2024-000887] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2024] [Accepted: 07/31/2024] [Indexed: 10/01/2024] Open
Abstract
The treatment of Hirschsprung disease (HSCR) is surgical resection of aganglionic bowel and subsequent pull-through of ganglionated bowel. Despite many advances since the initial description of the disease and its surgical management more than half a century ago, there remain considerable controversies regarding the history of the surgical technique, the optimal timing of the primary and multistage pull-through, the best treatment for patients with a delayed diagnosis of HSCR, and the management of post pull-through complications such as soiling due to sphincter incompetence, the presence of a transition zone, and the prevention of enterocolitis. The following review will explore each of these controversies.
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Affiliation(s)
- Thomas O Xu
- Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, Washington, District of Columbia, USA
| | - Marc A Levitt
- Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, Washington, District of Columbia, USA
| | - Christina Feng
- Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, Washington, District of Columbia, USA
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van der Zande JMJ, Koppen IJN, Yacob D, Sanchez RE, Puri NB, Vaz K, Di Lorenzo C, Benninga MA, Lu PL. Current practice in the care of children with functional constipation: What is the hold up? J Pediatr Gastroenterol Nutr 2024; 79:301-308. [PMID: 38924156 DOI: 10.1002/jpn3.12295] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/12/2024] [Revised: 04/22/2024] [Accepted: 06/05/2024] [Indexed: 06/28/2024]
Abstract
OBJECTIVES For children with intractable functional constipation (FC), there are no evidence-based guidelines for subsequent evaluation and treatment. Our objective was to assess the practice patterns of a large, international cohort of pediatric gastroenterologists. METHODS We administered a survey to physicians who attended the 2nd World Congress of Pediatric Neurogastroenterology and Motility held in Columbus, Ohio (USA) in September 2023. The survey included 29 questions on diagnostic testing, nonpharmacological and pharmacological treatment, and surgical options for children with intractable FC. RESULTS Ninety physicians from 18 countries completed the survey. For children with intractable FC, anorectal manometry was the most commonly used diagnostic test. North American responders were more likely than Europeans to use stimulant laxatives (97% vs. 77%, p = 0.032), prosecretory medications (69% vs. 8%, p < 0.001), and antegrade continence enemas (ACE; 83% vs. 46%, p = 0.009) for management. Europeans were more likely than North Americans to require colonic transit testing before surgery (85% vs. 30%, p < 0.001). We found major differences in management practices between Americans and the rest of the world, including use of prosecretory drugs (73% vs. 7%, p < 0.001), anal botulinum toxin injections (81% vs. 58%, p = 0.018), ACE (81% vs. 58% p = 0.018), diverting ileostomies (56% vs. 26%, p = 0.006), and colonic resections (42% vs. 16%, p = 0.012). No differences were found when respondents were compared by years of experience. CONCLUSIONS Practice patterns in the evaluation and treatment of children with intractable FC differ widely among pediatric gastroenterologists from around the world. A clinical guideline regarding diagnostic testing and surgical decision-making is needed.
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Affiliation(s)
- Julia M J van der Zande
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
| | - Ilan J N Koppen
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
| | - Desale Yacob
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Raul E Sanchez
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Neetu B Puri
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Karla Vaz
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Carlo Di Lorenzo
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Marc A Benninga
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
| | - Peter L Lu
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
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Beltman L, Labib H, Ahmed H, Benninga M, Roelofs J, van der Voorn P, van Schuppen J, Oosterlaan J, van Heurn E, Derikx J. Transition Zone Pull-through in Patients with Hirschsprung Disease: Is Redo Surgery Beneficial for the Long-term Outcomes? J Pediatr Surg 2023; 58:1903-1909. [PMID: 36941171 DOI: 10.1016/j.jpedsurg.2023.02.043] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Revised: 01/17/2023] [Accepted: 02/08/2023] [Indexed: 02/23/2023]
Abstract
INTRODUCTION Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients. METHODS We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up. RESULTS Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12-260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63). CONCLUSION Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.
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Affiliation(s)
- Lieke Beltman
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Surgery, Meibergdreef 9, Amsterdam, the Netherlands; Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatrics, Emma Children's Hospital Amsterdam UMC Follow-Me Program & Emma Neuroscience Group, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology and Metabolism Research Institute, Amsterdam, the Netherlands; Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherlands.
| | - Hosnieya Labib
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Surgery, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology and Metabolism Research Institute, Amsterdam, the Netherlands; Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherlands
| | - Hafsa Ahmed
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Surgery, Meibergdreef 9, Amsterdam, the Netherlands
| | - Marc Benninga
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Gastroenterology and Nutrition, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology and Metabolism Research Institute, Amsterdam, the Netherlands
| | - Joris Roelofs
- Amsterdam UMC Location University of Amsterdam, Department of Pathology, Meibergdreef 9, Amsterdam, the Netherlands
| | - Patrick van der Voorn
- Amsterdam UMC Location Vrije Universiteit Amsterdam, Department of Pathology, Boelelaan 1117, Amsterdam, the Netherlands
| | - Joost van Schuppen
- Amsterdam UMC Location University of Amsterdam, Department of Radiology and Nuclear Medicine, Meibergdreef 9, Amsterdam, the Netherlands
| | - Jaap Oosterlaan
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatrics, Emma Children's Hospital Amsterdam UMC Follow-Me Program & Emma Neuroscience Group, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherlands
| | - Ernest van Heurn
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Surgery, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology and Metabolism Research Institute, Amsterdam, the Netherlands; Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherlands
| | - Joep Derikx
- Emma Children's Hospital Amsterdam, Amsterdam UMC Location University of Amsterdam, Department of Pediatric Surgery, Meibergdreef 9, Amsterdam, the Netherlands; Amsterdam Gastroenterology Endocrinology and Metabolism Research Institute, Amsterdam, the Netherlands; Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherlands
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Pini Prato A, Faticato MG, Mazzola C, Arrigo S, Mattioli G, Arnoldi R, Mosconi M. Intrasphincteric Botox injections in Hirschsprung's disease: indications and outcome in 64 procedures over a ten-year period. Minerva Pediatr (Torino) 2023; 75:482-489. [PMID: 30419742 DOI: 10.23736/s2724-5276.18.05238-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/20/2023]
Abstract
BACKGROUND Intrasphincteric botulinum toxin (Botox) injection for symptomatic postoperative anal achalasia in Hirschsprung's disease (HSCR) has found wide application in the last twenty years. The aim of this study was to describe effectiveness and functional outcome of a series of patients treated over a 10-year period. METHODS All consecutive HSCR patients who received intrasphincteric Botox injections between January 2007 and December 2016 were included. Demographic data and clinical features were collected. A detailed questionnaire focusing on outcome in the medium and long-term was administered to all families. RESULTS In the study period 64 intrasphincteric Botox injections were performed in 31 patients. Completed questionnaires were returned by 27 out of 28 eligible patients (96%) reporting improvement or symptoms resolution in 16 (59%). The highest success rates were experienced by patients younger than 4, with long HSCR forms and with recurrent enterocolitis (75%, 100% and 100% of success rates, respectively). No major complications occurred. Minor complications were described by 7 patients (26%). CONCLUSIONS Intrasphincteric Botox injection proved to be feasible, safe and reasonably effective in children with HSCR and postoperative anal achalasia. Infants and toddlers with long HSCR forms and recurrent bouts of enterocolitis are those who would benefit most from this treatment.
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Affiliation(s)
- Alessio Pini Prato
- Unit of Pediatric Surgery, The Children Hospital, AON SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy -
| | - Maria G Faticato
- Department of Pediatric Surgery, Institute for Scientific Research, Giannina Gaslini Children's Hospital, Genoa, Italy
- Department of Neuroscience, Ophthalmology, Rehabilitation, Genetics and Maternal-Infant Science - DINOGMI, University of Genoa, Genoa, Italy
| | - Cinzia Mazzola
- Department of Pediatric Surgery, Institute for Scientific Research, Giannina Gaslini Children's Hospital, Genoa, Italy
| | - Serena Arrigo
- Unit of Pediatric Gastroenterology, Institute for Scientific Research, Giannina Gaslini Children's Hospital, Genoa, Italy
| | - Girolamo Mattioli
- Department of Pediatric Surgery, Institute for Scientific Research, Giannina Gaslini Children's Hospital, Genoa, Italy
- Department of Neuroscience, Ophthalmology, Rehabilitation, Genetics and Maternal-Infant Science - DINOGMI, University of Genoa, Genoa, Italy
| | - Rossella Arnoldi
- Unit of Pediatric Surgery, The Children Hospital, AON SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - Manuela Mosconi
- Department of Pediatric Surgery, Institute for Scientific Research, Giannina Gaslini Children's Hospital, Genoa, Italy
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Encisco EM, Lim IIP, Velazco CS, Rosen NG, Garrison AP, Rymeski B, Frischer JS. Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review. J Pediatr Surg 2023:S0022-3468(23)00250-6. [PMID: 37221126 DOI: 10.1016/j.jpedsurg.2023.04.008] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/13/2023] [Accepted: 04/17/2023] [Indexed: 05/25/2023]
Abstract
BACKGROUND Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. METHODS Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. RESULTS We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102). CONCLUSION Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Ellen M Encisco
- Colorectal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
| | | | - Cristine S Velazco
- Department of Pediatric Surgery, Arnold Palmer Hospital for Children, Orlando Health, Orlando, FL, USA
| | - Nelson G Rosen
- Colorectal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
| | - Aaron P Garrison
- Colorectal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
| | - Beth Rymeski
- Colorectal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
| | - Jason S Frischer
- Colorectal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
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Hirsch S, Nurko S, Rosen R. Author response to letter to the editor. Neurogastroenterol Motil 2022; 34:e14478. [PMID: 36210756 DOI: 10.1111/nmo.14478] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2022] [Accepted: 09/19/2022] [Indexed: 12/07/2022]
Affiliation(s)
- Suzanna Hirsch
- Aerodigestive Center, Center for Motility and Functional Gastrointestinal Disorders, Division of Gastroenterology, Hepatology & Nutrition, Boston Children's Hospital, Boston, Massachusetts, USA
| | - Samuel Nurko
- Aerodigestive Center, Center for Motility and Functional Gastrointestinal Disorders, Division of Gastroenterology, Hepatology & Nutrition, Boston Children's Hospital, Boston, Massachusetts, USA
| | - Rachel Rosen
- Aerodigestive Center, Center for Motility and Functional Gastrointestinal Disorders, Division of Gastroenterology, Hepatology & Nutrition, Boston Children's Hospital, Boston, Massachusetts, USA
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7
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Management of Anorectal Malformations and Hirschsprung Disease. Surg Clin North Am 2022; 102:695-714. [DOI: 10.1016/j.suc.2022.07.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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Abstract
Total Colonic Hirschsprung Disease (HD) can be challenging from a diagnostic and management standpoint and occurs in around 8% of cases of HD. Long term outcomes are difficult to compare due to variation in length of aganglionosis, chosen surgical techniques, and terminology utilized in the literature. In this review we highlight some of the management controversies and clinical challenges and emphasize future areas of suggested collaboration and research.
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Affiliation(s)
- Richard J Wood
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH, USA.
| | - Aaron P Garrison
- Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
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9
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The utilization of botulinum toxin for Hirschsprung disease. Semin Pediatr Surg 2022; 31:151161. [PMID: 35690464 DOI: 10.1016/j.sempedsurg.2022.151161] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Patients with Hirschsprung disease (HD) can struggle with persistent obstructive symptoms even after a successful pull-through. These symptoms lead to stasis of stool and can result in Hirschsprung associated enterocolitis (HAEC). Recurrent episodes of HAEC warrant further workup; if there are no signs of mechanical obstruction or an aganglionic pull-through, the use of botulinum toxin injections to the internal anal sphincter has been utilized to relieve these symptoms. In this review, we describe the variations in botulinum toxin injection use and describe ongoing studies to prevent obstructive symptoms and Hirschsprung-associated enterocolitis (HAEC). Botulinum toxin injection utilization has been described for obstructive symptoms after HD pull-through, in the setting of active HAEC, and has been proposed to be part of the treatment algorithm for prevention of HAEC after pull-through. Dosing utilized for the injections, along with the complications, are also described. Prospective, multi-institutional trials are needed to identify the effectiveness of botulinum toxin injections in the outpatient/prophylactic setting as current data suggest some benefits in preventing future obstructive symptoms; however, other studies have conflicting results.
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Current understanding of Hirschsprung-associated enterocolitis: Pathogenesis, diagnosis and treatment. Semin Pediatr Surg 2022; 31:151162. [PMID: 35690459 PMCID: PMC9523686 DOI: 10.1016/j.sempedsurg.2022.151162] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Hirschsprung-associated enterocolitis (HAEC) was described in 1886 by Harald Hirschsprung and is a potentially deadly complication of Hirschsprung Disease. HAEC is classically characterized by abdominal distension, fever, and diarrhea, although there can be a variety of other associated symptoms, including colicky abdominal pain, lethargy, and the passage of blood-stained stools. HAEC occurs both pre-operatively and post-operatively, is the presenting symptom of HSCR in up to 25% of infants and varies in overall incidence from 20 to 60%. This article reviews our current understanding of HAEC pathogenesis, diagnosis, and treatment with discussion of areas of ongoing research, controversy, and future investigation.
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Sun G, Trzpis M, Broens PMA. High Anal Canal Pressure and Rectal Washouts Contribute to the Decrease of Anal Basal Pressure After Botulinum Toxin Injections in Paediatric Patients With Chronic Constipation. Front Pediatr 2022; 10:819529. [PMID: 35391742 PMCID: PMC8980778 DOI: 10.3389/fped.2022.819529] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2021] [Accepted: 02/14/2022] [Indexed: 11/17/2022] Open
Abstract
INTRODUCTION Chronic constipation can be treated by injecting botulinum toxin into the anal sphincter to decrease anal basal pressure. To assess the effect of botulinum toxin, we investigated the factors that contribute to changes in anal basal pressure after injection. METHODS This was a retrospective study conducted in a tertiary hospital in the Netherlands. We included children with chronic constipation treated with botulinum toxin injections and measured anal basal pressure before and after each injection. Multivariable linear regression analyses were used. RESULTS We investigated 30 cases with idiopathic constipation. Their median age was 20.5 (7.75-53.25) months. Anal basal pressure decreased after injection in 20 cases. The mean decrease of anal basal pressure after injection was 18.17 ± 35.22 mmHg. The anal basal pressure change was linearly correlated with preinjection pressure (R 2 = 0.593, P < 0.001). A significant decrease of pressure was observed in patients with preinjection pressure > 70 mmHg. Preinjection anal basal pressure (β = -0.913, P < 0.001) and rectal washouts (β = -21.015, P = 0.007) contributed significantly to pressure changes. Changes in anal basal pressure were also significantly associated with patients' weights (β = 0.512, 95% CI, 0.011-1.013) and sex (β = 22.971, 95% CI, 9.205-36.736). CONCLUSIONS Botulinum toxin significantly decreases anal basal pressure when preinjection pressure is higher than 70 mmHg. In patients with severely elevated anal basal pressure, we recommend rectal washouts to promote the decrease of anal basal pressure.
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Affiliation(s)
- Ge Sun
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen, Netherlands
| | - Monika Trzpis
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen, Netherlands
| | - Paul M A Broens
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen, Netherlands.,Department of Surgery, Division of Pediatric Surgery, University of Groningen, University Medical Center Groningen, Groningen, Netherlands
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Svetanoff WJ, Briggs K, Fraser JA, Lopez J, Fraser JD, Juang D, Aguayo P, Hendrickson RJ, Snyder CL, Oyetunji TA, St Peter SD, Rentea RM. Outpatient Botulinum Injections for Early Obstructive Symptoms in Patients with Hirschsprung Disease. J Surg Res 2021; 269:201-206. [PMID: 34587522 DOI: 10.1016/j.jss.2021.07.017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2021] [Revised: 06/23/2021] [Accepted: 07/22/2021] [Indexed: 11/18/2022]
Abstract
INTRODUCTION Botulinum toxin (BT) injections may play a role in preventing Hirschsprung associated enterocolitis (HAEC) episodes related to internal anal sphincter (IAS dysfunction). Our aim was to determine the association of outpatient BT injections for early obstructive symptoms on the development of HAEC. METHODS A retrospective review of children who underwent definitive surgery for Hirschsprung disease (HSCR) from July 2010 - July 2020 was performed. The timing from pull-through to first HAEC episode and to first BT injection was recorded. Primary analysis focused on the rate of HAEC episodes and timing between episodes in patients who did and did not receive BT injections. RESULTS Eighty patients were included. Sixty patients (75%) were male, 15 (19%) were diagnosed with trisomy 21, and 58 (72.5%) had short-segment disease. The median time to pull-through was 150 days (IQR 16, 132). Eight patients (10%) had neither an episode of HAEC or BT injections and were not included in further analysis. Forty-six patients (64%) experienced at least one episode of HAEC, while 64 patients (89%) had at least one outpatient BT injection. Compared to patients who never received BT injections (n = 9) and those who developed HAEC prior to BT injections (n = 35), significantly fewer patients who received BT injections first (n = 28) developed enterocolitis (P < 0.001), with no patient developing more than one HAEC episode. CONCLUSION Outpatient BT is associated with decreased episodes of HAEC and increased interval between HAEC episodes requiring inpatient treatment. Scheduling outpatient BT injections to manage obstructive symptoms may be beneficial after pull-through for HSCR.
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Affiliation(s)
| | - Kayla Briggs
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri
| | - James A Fraser
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri
| | - Joseph Lopez
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri
| | - Jason D Fraser
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - David Juang
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - Pablo Aguayo
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - Richard J Hendrickson
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - Charles L Snyder
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - Tolulope A Oyetunji
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - Shawn D St Peter
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri
| | - Rebecca M Rentea
- Department of Surgery, Children's Mercy Hospital; Kansas City, Missouri; University of Missouri-Kansas City School of Medicine; Kansas City, Missouri.
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Roorda D, Oosterlaan J, van Heurn E, Derikx J. Intrasphincteric botulinum toxin injections for post-operative obstructive defecation problems in Hirschsprung disease: A retrospective observational study. J Pediatr Surg 2021; 56:1342-1348. [PMID: 33288128 DOI: 10.1016/j.jpedsurg.2020.11.025] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2020] [Revised: 11/11/2020] [Accepted: 11/21/2020] [Indexed: 01/03/2023]
Abstract
BACKGROUND Patients with Hirschsprung disease may have obstructive symptoms after resection of the aganglionic segment. Botulinum toxin (BT) injections can help improve faecal passage by relaxing the internal anal sphincter. This study assess effect of BT injections and aims to identify factors associated with receiving BT injections and favourable response to the first BT injection. METHODS A retrospective study was performed in a cohort of consecutive patients treated for Hirschsprung disease in our centre between 2003 and 2017. The indication for BT injections was obstructive defecation problems that were non-responsive to high-dose laxatives or rectal irrigation, or an episode of Hirschsprung-associated enterocolitis (HAEC). Effectiveness of BT injections was measured in terms of clinical improvement. Relationships between factors associated with receiving BT injections and with response to the first BT injection were tested with group comparison and logistic regression. RESULTS Forty-one out of 131 patients received BT injections (31%) with a median of two injections (range 1-11). All patients had obstructive defecation problems non-responsive to high-dose laxatives or rectal irrigation, two patient also had an episode of HAEC. Twenty-five out of 41 patients (61%) had clinical improvement after first injection. In 29 of the 41 patients (71%) spontaneous defecation or treatment with laxatives only was achieved. Adverse effects were seen in 12 out of 41 patients (29%) after 14 injections (16%), and consisted of anal pain, temporary loss of stools and dermatitis. Patients who received BT injections more often had long segment disease, more often required laxatives or rectal irrigation and had longer length of hospital stay, both after corrective surgery and in follow-up. None of the tested factors was associated with clinical improvement after first BT injection. CONCLUSION Our findings show that BT injections effectively treat obstructive defecation problems in the majority of patients with Hirschsprung disease with mild adverse effects. LEVEL OF EVIDENCE Level III.
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Affiliation(s)
- Daniëlle Roorda
- Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Department of Pediatric Surgery, Amsterdam Gastroenterology and Metabolism Research Institute and Amsterdam Reproduction and Development Research Institute, Amsterdam, Netherlands; Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Follow Me Aftercare Program, Department of Pediatrics, Amsterdam Reproduction and Development Research Institute, Amsterdam, Netherlands.
| | - Jaap Oosterlaan
- Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Follow Me Aftercare Program, Department of Pediatrics, Amsterdam Reproduction and Development Research Institute, Amsterdam, Netherlands
| | - Ernest van Heurn
- Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Department of Pediatric Surgery, Amsterdam Gastroenterology and Metabolism Research Institute and Amsterdam Reproduction and Development Research Institute, Amsterdam, Netherlands
| | - Joep Derikx
- Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Department of Pediatric Surgery, Amsterdam Gastroenterology and Metabolism Research Institute and Amsterdam Reproduction and Development Research Institute, Amsterdam, Netherlands
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14
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Radwan AB, Gadallah MA, Shahawy MR, Albagdady AA, Talaat AA. Can botulinum toxin help in managing children with functional constipation and obstructed defecation? J Pediatr Surg 2021; 56:750-753. [PMID: 32739102 DOI: 10.1016/j.jpedsurg.2020.06.044] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/04/2020] [Revised: 06/21/2020] [Accepted: 06/26/2020] [Indexed: 01/16/2023]
Abstract
BACKGROUND Functional constipation (FC) is a common pediatric healthcare problem worldwide. Obstructed defection syndrome (ODS) is frequently presented with an inability to coordinate the bowel movement with pelvic floor muscles. Botulinum toxin (BT) intraanal sphincteric injection can improve the obstructed defecation by relaxing the anal sphincter and reducing the required force to propel the stools. PURPOSE This study aimed to compare the changes in Rintala scores (as a mean of assessing defecatory function), in children with FC and ODS, managed by BT injection, bowel management program (BMP), and senna based laxatives (SBL), versus a control group managed by BMP and SBL only. METHODS Prospective randomized controlled study, started at December 2017 on 40 pediatric patients, divided into 2 equal groups (group A: managed by BMP and SBL, group B: like group A with once intraanal sphincteric BT injection) suffering from FC, ODS, with contrast enema showing persistence of ≤90 rectoanal angle (RAA) even with trials of defecation. Patients were excluded if they have neuromuscular abnormalities, hypothyroidism, previous colorectal or anal surgery. Rintala score was assessed before treatment, at 2 months, and 6 months after management. RESULTS The study included 18 females (45%) and 22 males (55%). Group A had equal gender distribution and mean age of 6.9 years, while group B had 12 males with mean age of 7.35 years. The mean follow up period was 11.35 months in group A and 11.6 months in group B. Mean Rintala scores of both groups at initial presentation, 2 months and 6 months follow up were: group A: 9.10, 9.40, 10.90; group B: 9.30, 10.70, 11.05 respectively, and showed no statistically significant difference (p value: 0.884, 0.294, 0.923 respectively). No complications were detected from BT injection like allergic reactions, neuromuscular urinary or lower limbs disturbances. CONCLUSION Intraanal sphincteric injection of botulinum toxin by the mentioned technique and dose, did not result in additional defecatory functional improvement (when assessed by Rintala score) over the routine protocol (using bowel management program and laxatives) of managing functional constipation with obstructed defecation. ANNOUNCEMENT: a preliminary report of this work was presented in the 34th Egyptian pediatric surgery association (EPSA) meeting in Cairo in November 2019. TYPE OF STUDY Treatment/prospective study. LEVEL OF EVIDENCE Level I.
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Affiliation(s)
- Ahmed B Radwan
- Pediatric Surgery Department, Faculty of Medicine, Ain-Shams University, Cairo, Egypt.
| | - Mohamed A Gadallah
- Pediatric Surgery Department, Faculty of Medicine, Ain-Shams University, Cairo, Egypt
| | - Mohammed R Shahawy
- General Surgery Department, Faculty of Medicine, Ain-Shams University, Cairo, Egypt
| | - Ayman A Albagdady
- Pediatric Surgery Department, Faculty of Medicine, Ain-Shams University, Cairo, Egypt
| | - Ayman A Talaat
- General Surgery Department, Faculty of Medicine, Ain-Shams University, Cairo, Egypt
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15
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Impact of Botulinum Toxin on Hirschsprung-Associated Enterocolitis After Primary Pull-Through. J Surg Res 2021; 261:95-104. [PMID: 33422904 DOI: 10.1016/j.jss.2020.12.018] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/10/2020] [Revised: 11/09/2020] [Accepted: 12/08/2020] [Indexed: 11/20/2022]
Abstract
BACKGROUND Hirschsprung-associated enterocolitis (HAEC) is a serious potential complication after primary pull-through surgery for Hirschsprung's disease (HSCR). Administration of anal botulinum toxin (BT) injection may improve obstructive symptoms at the internal anal sphincter, leading to improved fecal passage. The timing of administration and effects on delay or prevention of HAEC are unknown. We hypothesized that BT administration increased the postoperative time to HAEC and aimed to investigate whether anal BT administration after primary pull-through surgery for HSCR is associated with increased time to inpatient HAEC admission development. METHODS We performed a retrospective cohort study examining children with HSCR at US children's hospitals from 2008 to 2018 using the Pediatric Health Information System database with an associated primary pull-through operation performed before 60 d of age. The intervention assessed was the administration of BT concerning the timing of primary pull-through, and two groups were identified: PRO (received BT at or after primary pull-through, before HAEC) and NOT (never received BT, or received BT after HAEC). The primary outcome was time from pull-through to the first HAEC admission. The Cox proportional hazards model was developed to examine the BT administration effect on the primary outcome after controlling for patient-level covariates. RESULTS We examined a total of 1439 children (67 in the PRO and 1372 in the NOT groups). A total of 308 (21.4%) developed at least one episode of HAEC, including 76 (5.3%) who had two or more episodes. Between 2008 and 2018, the frequency of BT administration has increased from three to 20 hospitals with a frequency of administration between 2.2% and 16.2%. Prophylactic BT (PRO) was not associated with increased time to HAEC event on adjusted analysis. CONCLUSIONS Among children with HSCR undergoing primary pull-through surgery, prophylactic BT administration did not demonstrate increased time to first HAEC event. A better-powered study with prophylactic BT is required to determine the effect on HAEC occurrence and timing. LEVEL OF EVIDENCE Level II (retrospective cohort study).
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16
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Vilanova-Sanchez A, Levitt MA. Surgical Interventions for Functional Constipation: An Update. Eur J Pediatr Surg 2020; 30:413-419. [PMID: 32987436 DOI: 10.1055/s-0040-1716729] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Chronic idiopathic constipation, also known as functional constipation, is defined as difficult and infrequent defecation without an identifiable organic cause. Medical management with laxatives is effective for the majority of constipated children. However there is a subset of patients who may need evaluation by a surgeon. As constipation progresses, it can lead to fecal retention and rectal and sigmoid distension, which impairs normal colorectal motility. Surgical interventions are influenced by the results of: a rectal biopsy, transit studies, the presence of megacolon/megarectum on contrast enema, the degree of soiling/incontinence, anorectal manometry findings, and colonic motility evaluation. In this review, we describe the different surgical options available (intestinal diversion, antegrade enemas, sacral nerve stimulation, colonic resections, and Botulinum toxin injection) and provide guidance on how to choose the best procedure for a given patient.
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Affiliation(s)
- Alejandra Vilanova-Sanchez
- Deparment of Pediatric Surgery, Urogenital and Colorectal Unit, La Paz University Hospital Children Hospital, Madrid, Comunidad de Madrid, Spain
| | - Marc A Levitt
- Department of Pediatric Surgery, Children's National Medical Center, Washington, District of Columbia, United States
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17
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Cariati M, Chiarello MM, Cannistra' M, Lerose MA, Brisinda G. Gastrointestinal Uses of Botulinum Toxin. Handb Exp Pharmacol 2020; 263:185-226. [PMID: 32072269 DOI: 10.1007/164_2019_326] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Botulinum toxin (BT), one of the most powerful inhibitors that prevents the release of acetylcholine from nerve endings, represents an alternative therapeutic approach for "spastic" disorders of the gastrointestinal tract such as achalasia, gastroparesis, sphincter of Oddi dysfunction, chronic anal fissures, and pelvic floor dyssynergia.BT has proven to be safe and this allows it to be a valid alternative in patients at high risk of invasive procedures but long-term efficacy in many disorders has not been observed, primarily due to its relatively short duration of action. Administration of BT has a low rate of adverse reactions and complications. However, not all patients respond to BT therapy, and large randomized controlled trials are lacking for many conditions commonly treated with BT.The local injection of BT in some conditions becomes a useful tool to decide to switch to more invasive therapies. Since 1980, the toxin has rapidly transformed from lethal poison to a safe therapeutic agent, with a significant impact on the quality of life.
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Affiliation(s)
- Maria Cariati
- Department of Surgery, "San Giovanni di Dio" Hospital, Crotone, Italy
| | | | - Marco Cannistra'
- Department of Surgery, "San Giovanni di Dio" Hospital, Crotone, Italy
| | | | - Giuseppe Brisinda
- Department of Surgery, "San Giovanni di Dio" Hospital, Crotone, Italy. .,Department of Surgery, "Agostino Gemelli" Hospital, Catholic School of Medicine, Rome, Italy.
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18
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Anal sphincter botulinum toxin injection in children with functional anorectal and colonic disorders: A large institutional study and review of the literature focusing on complications. J Pediatr Surg 2019; 54:2305-2310. [PMID: 31060739 DOI: 10.1016/j.jpedsurg.2019.03.020] [Citation(s) in RCA: 31] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/12/2018] [Revised: 03/04/2019] [Accepted: 03/24/2019] [Indexed: 11/21/2022]
Abstract
BACKGROUND AND AIM Botulinum toxin (botox) is a commonly used treatment for functional anorectal and colonic disorders. Although generally regarded as safe, complications associated with botox injection into the anal sphincters in children with severe defecation disorders are not well described. We aimed to review our institutional experience and the existing literature to better understand the safety of this practice. METHODS We performed a retrospective review of pediatric patients undergoing botox administration into the anal sphincter for treatment of a variety of defecation disorders between 2014 and 2018. Additionally, we performed a review of all published literature reporting complications from botox injection in this patient population. RESULTS 881 patients ranging from 5 weeks to 19.7 years underwent a total of 1332 botox injections including our institution (332 patients/526 injections) and the reviewed series (549 patients/806 injections). Overall, complications were seen after 9 (0.7%) injections and included urinary incontinence (n = 5), pelvic muscle paresis (n = 2), perianal abscess (n = 1), pruritis ani (n = 1), and rectal prolapse (n = 1). Patient age, weight, and diagnosis were not associated with an increased rate of complication in our institutional experience. All complications were self-limited and did not require intervention. There were no episodes of systemic botulinum toxicity. CONCLUSION Botox injection into the anal sphincters is accepted practice in children with Hirschsprung disease, severe functional constipation, and internal anal sphincter achalasia and appears to be safe from this review. The precise dosing and age at which complications are more likely to arise could not be ascertained and require further study. LEVEL OF EVIDENCE IV TYPE OF STUDY: Retrospective cohort study.
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19
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Youn JK, Han JW, Oh C, Kim SY, Jung SE, Kim HY. Botulinum toxin injection for internal anal sphincter achalasia after pull-through surgery in Hirschsprung disease. Medicine (Baltimore) 2019; 98:e17855. [PMID: 31702647 PMCID: PMC6855586 DOI: 10.1097/md.0000000000017855] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Botulinum toxin (Botox) was introduced for the management of internal anal sphincter (IAS) achalasia after a pull-through procedure in Hirschsprung disease (HD). We conducted a prospective evaluation of the efficacy and safety of this Botox treatment.Our study group included 15 patients with HD (median age, 4.8 years; range, 1.7-7.4 years) who experienced persistent constipation after pull-through surgery. Rectal biopsy and colon study were performed before Botox injection to exclude agangliosis. Intersphincteric Botox injections (dose, 4 IU/kg) were performed at 3 sites, (3, 6, and 9 o'clock) under general anesthesia. Measured outcomes of efficacy included anorectal manometry, Wexner constipation score and the quality of life score for defecation, measured at baseline and at 2 weeks and 3 months after injection. The Holschneider incontinence score and an assessment of pain, bleeding, heating sensation, and swelling were also performed at follow-up as outcomes of safety.There was no significant change in measured outcomes with Botox treatment. Botox did decrease the number of patients who experienced abdominal distension at 3 months, compared to 2-weeks, post-injection. No major complications were identified, with only 2 cases of anal bleeding that resolved spontaneously. Local tenderness at the injection site was reported by 4 patients, recovering without treatment.The efficacy of Botox, injected into the IAS, for the treatment of achalasia is questionable on short-term follow-up. Larger studies with a longer follow-up period and the use of repeated injections are required to evaluate the evidence for this treatment.
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Affiliation(s)
- Joong Kee Youn
- Department of Surgery, Jeju National University Hospital, Jeju
| | - Ji-Won Han
- Department of Pediatric Surgery, Seoul National University Hospital, Seoul
| | - Chaeyoun Oh
- Department of Surgery, Korea University Ansan Hospital, Gyeonggi
| | - So-Young Kim
- Department of Pediatric Surgery, Seoul National University Hospital, Seoul
| | - Sung-Eun Jung
- Department of Pediatric Surgery, Seoul National University College of Medicine, Seoul, Korea
| | - Hyun-Young Kim
- Department of Pediatric Surgery, Seoul National University College of Medicine, Seoul, Korea
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20
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Guidelines for the management of postoperative soiling in children with Hirschsprung disease. Pediatr Surg Int 2019; 35:829-834. [PMID: 31201486 DOI: 10.1007/s00383-019-04497-y] [Citation(s) in RCA: 37] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/06/2019] [Indexed: 12/17/2022]
Abstract
Although most children with Hirschsprung disease ultimately achieve functional and comfortable stooling, some will experience a variety of problems after pull-through surgery. The most common problems include soiling, obstructive symptoms, enterocolitis, and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative soiling in children with Hirschsprung disease. The American Pediatric Surgical Association Hirschsprung Disease Interest Group engaged in a literature review and group discussions. Expert consensus was then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with soiling symptoms following pull-through for Hirschsprung disease. Causes of soiling after pull-through are broadly categorized as abnormalities in sensation, abnormalities in sphincter control, and "pseudo-incontinence." A stepwise algorithm for the diagnosis and management of soiling after a pull-through for Hirschsprung disease is presented; it is our hope that this rational approach will facilitate treatment and optimize outcomes.
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21
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Roorda D, Abeln ZAM, Oosterlaan J, van Heurn LWE, Derikx JPM. Botulinum toxin injections after surgery for Hirschsprung disease: Systematic review and meta-analysis. World J Gastroenterol 2019; 25:3268-3280. [PMID: 31333317 PMCID: PMC6626723 DOI: 10.3748/wjg.v25.i25.3268] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2019] [Revised: 05/05/2019] [Accepted: 06/01/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND A large proportion of patients with Hirschsprung disease experience persistent obstructive symptoms after corrective surgery. Persistent obstructive symptoms may result in faecal stasis that can develop into Hirschsprung-associated enterocolitis, a potential life-threatening condition. Important treatment to improve faecal passage is internal anal sphincter relaxation using botulinum toxin injections.
AIM To give an overview of all empirical evidence on the effectiveness of botulinum toxin injections in patients with Hirschsprung disease.
METHODS A systematic review and meta-analysis was done by searching PubMed, EMBASE and the Cochrane Library, using entry terms related to: (1) Hirschsprung disease; and (2) Botulinum toxin injections. 14 studies representing 278 patients met eligibility criteria. Data that were extracted were proportion of patients with improvement of obstructive symptoms or less enterocolitis after injection, proportion of patients with adverse effects and data on type botulinum toxin, mean dose, average age at first injection and patients with associated syndromes. Random-effects meta-analysis was used to aggregate effects and random-effects meta-regression was used to test for possible confounding factors.
RESULTS Botulinum toxin injections are effective in treating obstructive symptoms in on average 66% of patients [event rate (ER) = 0.66, P = 0.004, I2 = 49.5, n = 278 patients]. Type of botulinum toxin, average dose, average age at first injections and proportion of patients with associated syndromes were not predictive for this effect. Mean 7 duration of improvement after one botulinum toxin injections was 6.4 mo and patients needed on average 2.6 procedures. There was a significant higher response rate within one month after botulinum toxin injections compared to more than one month after Botulinum toxin injections (ER = 0.79, vs ER = 0.46, Q = 19.37, P < 0.001). Botulinum toxin injections were not effective in treating enterocolitis (ER 0.58, P = 0.65, I2 = 71.0, n = 52 patients). There were adverse effects in on average 17% of patients (ER = 0.17, P < 0.001, I2 = 52.1, n = 187 patients), varying from temporary incontinence to mild anal pain.
CONCLUSION Findings from this systematic review and meta-analysis indicate that botulinum toxin injections are effective in treating obstructive symptoms and that adverse effects were present, but mild and temporary.
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Affiliation(s)
- Daniëlle Roorda
- Department of Pediatric Surgery, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
| | - Zarah AM Abeln
- Department of Pediatric Surgery, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
| | - Jaap Oosterlaan
- Department of Pediatrics, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam, Emma Neuroscience Group, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
| | - Lodewijk WE van Heurn
- Department of Pediatric Surgery, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
- Department of Pediatric Surgery, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam 1105 AZ, Netherlands
| | - Joep PM Derikx
- Department of Pediatric Surgery, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Reproduction and Development, Amsterdam 1105 AZ, Netherlands
- Department of Pediatric Surgery, Emma Children’s Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam 1105 AZ, Netherlands
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22
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Neurostimulation-guided Anal Intrasphincteric Botulinum Toxin Injection in Children With Hirschsprung Disease. J Pediatr Gastroenterol Nutr 2019; 68:527-532. [PMID: 30444834 DOI: 10.1097/mpg.0000000000002204] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
OBJECTIVES In Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients. METHODS In this monocenter cohort study, 15 HD patients with postoperative functional intestinal obstructive symptoms received neurostimulation-guided anal intrasphincteric BT injections. Short-, medium-, and long-term effects were evaluated. The Bristol stool form scale was used to assess stool consistency, and the Jorge-Wexner (JW) score to assess fecal continence. RESULTS The median age at first injection was 4 years. In the short-term, a significant improvement in stool consistency was noted in 12 of 14 patients (P = 0.0001) and JW score decreased for 14 of 15 patients (P = 0.001). In the medium-term, JW score significantly decreased for all patients (P = 0.0001), with an improvement of 50% or more for 10 patients (66.7%). In the long term, 83.3% of patients had normal stool consistency and JW score was <3 for all. Recurrent enterocolitis decreased from 86.7% to 8.3%. A complete resolution of all symptoms without further medication was observed in 66.7% of patients in the long term. CONCLUSIONS Intrasphincteric BT injection was a safe, effective, and durable option for the management of postoperative functional intestinal obstructive symptoms in HD. The use of neurostimulator guidance for specific delivery of BT to muscular fibers of nonrelaxing anal sphincter complex takes into consideration the variability of patient's anatomy secondary to curative surgery.
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Prevention and management of recurrent postoperative Hirschsprung's disease obstructive symptoms and enterocolitis: Systematic review and meta-analysis. J Pediatr Surg 2018; 53:2423-2429. [PMID: 30236605 DOI: 10.1016/j.jpedsurg.2018.08.024] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/02/2018] [Accepted: 08/25/2018] [Indexed: 12/12/2022]
Abstract
BACKGROUND/PURPOSE The purpose of this study was to review the management of obstructive symptoms and enterocolitis (HAEC) following pull-through for Hirschsprung's disease. METHODS A systematic review and meta-analysis (1992-2017) was performed. Included studies were: randomized controlled trials (RCT), retrospective/prospective case-control (C-C), case-series (C-S). Random-effect model was used to produce risk ratio (RR) [95% CI]. P < 0.05 was considered significant. RESULTS Twenty-nine studies were identified. Routine postoperative dilatations (5 C-S, 2 C-C; 405 patients): no effect on stricture incidence (RR 0.3 [0.02-5.7]; p = 0.4). Routine postoperative rectal irrigations (2 C-C; 172 patients): reduced HAEC incidence (RR 0.2 [0.1-0.5]; p = 0.001). Posterior myotomy/myectomy (4 C-S; 53 patients): resolved obstructive symptoms in 79% [60.6-93.5] and HAEC in 80% [64.1-92.1]. Botulinum toxin injection (9 C-S; 166 patients): short-term response in 77.3% [68.2-85.2], long-term response in 43.0% [26.9-59.9]. Topical nitric oxide (3 C-S; 13 patients): improvement in 100% of patients. Probiotic prophylaxis (3 RCT; 160 patients): no reduction in HAEC (RR 0.6 [0.2-1.7]; p = 0.3). Anti-inflammatory drugs (1 C-S, sodium cromoglycate; 8 patients): improvement of HAEC in 75% of patients. CONCLUSIONS Several strategies with variable results are available in patients with obstructive symptoms and HAEC. Routine postoperative dilatations and prophylactic probiotics have no role in reducing the incidence of postoperative obstructive symptoms and HAEC. TYPE OF STUDY Systematic review and meta-analysis. LEVEL OF EVIDENCE Level II.
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Abstract
PURPOSE OF REVIEW To overview the current medical literature on the efficacy of botulism toxin treatment (BTX-A) for lower gastrointestinal disorders (GIT). RECENT FINDINGS BTX-A was found to have a short-term efficacy for the treatment of dyssynergic defecation. Surgical treatment was found to be more effective than BTX-A for the healing of chronic anal fissures, and BTX-A can be considered when surgery is undesirable. Data regarding the effects of BTX-A injection for the treatment of chronic anal pain is limited. Beneficial effects were observed only in a minority of patients. BTX-A treatment was found to be effective for the treatment of obstructive symptoms after surgery for Hirsprung's disease as well as for the treatment of internal anal sphincter achalasia. BTX-A treatment has a short-term efficacy and is safe. Further research is still needed in order to establish the exact place of BTX-A treatment of lower GIT disorders.
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Affiliation(s)
- Dan Carter
- Department of Gastroenterology, Chaim Sheba Medical Center, Tel Hashomer, Derech Sheba 2, Ramat Gan, Israel.,Sackler Faculty of Medicine, Tel Aviv University, P.O.B 39040, 69978, Ramat Aviv, Israel
| | - Ram Dickman
- Sackler Faculty of Medicine, Tel Aviv University, P.O.B 39040, 69978, Ramat Aviv, Israel. .,Division of Gastroenterology, Rabin Medical Center, Ze'ev Jabotinsky St 39, 4941492, Petah Tikva, Israel.
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25
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The Role of Bowel Management in Children with Bladder and Bowel Dysfunction. CURRENT BLADDER DYSFUNCTION REPORTS 2018. [DOI: 10.1007/s11884-018-0458-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022]
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Zar-Kessler C, Kuo B, Belkind-Gerson J. Botulinum toxin injection for childhood constipation is safe and can be effective regardless of anal sphincter dynamics. J Pediatr Surg 2018; 53:693-697. [PMID: 29395154 DOI: 10.1016/j.jpedsurg.2017.12.007] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/23/2016] [Revised: 12/04/2017] [Accepted: 12/17/2017] [Indexed: 12/27/2022]
Abstract
BACKGROUND Childhood constipation is common. Previously, internal anal sphincterotomy has been used for hypertensive/non-relaxing sphincters; however, recent benefit has been shown with Botulinum Toxin (BT) injections. The aim is to investigate BT, including response duration, symptom association and effectiveness in relation to sphincter dynamics. METHODS Retrospective study of 164 children receiving sphincter BT for severe constipation unresponsive to medication management. Charts reviewed for symptoms, anorectal manometry (ARM) findings and response defined by decreased pain or increased defecation. Patients were grouped: normal sphincter pressure (≤50 mmHg), elevated (>50 mmHg), normal and abnormal rectoanal inhibitory reflex (RAIR). RESULTS There were 142 analyzed and 124 completed ARMs; 98 (70%) had positive response with 57% lasting greater than 6 months. 36 had normal sphincter pressure with 24 (69%) responding. 88 had elevated pressure with 60 (68%) responding (p=0.87). 90 normal RAIRs with 64 (71%) responding. 34 abnormal RAIRs with 22 (64%) responding (p=0.41). With logistic regression, fecal incontinence prior to BT was a predictor of poor response (p= 0.02). The most common side effect was fecal incontinence typically resolving within week with equal frequency regardless of sphincter dynamics. CONCLUSIONS BT is effective for children with chronic constipation. Patients with fecal incontinence are less likely to respond. More than half had prolonged beneficial response. Those with normal and abnormal sphincter dynamics had similar responses and without differences in side effects. Therefore, injection may be considered in patients with intractable constipation unresponsive to medication, regardless of anal sphincter dynamics. LEVEL OF EVIDENCE Level III (Treatment Study: Retrospective comparative study).
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Affiliation(s)
- Claire Zar-Kessler
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Center for Neurointestinal Health, Massachusetts General Hospital for Children, 55 Fruit St., Boston, MA 02114.
| | - Braden Kuo
- Division of Gastroenterology, Center for Neurointestinal Health, Massachusetts General Hospital Gastroenterology, 55 Fruit St., Boston, MA 02114
| | - Jaime Belkind-Gerson
- Neurogastroenterology Program, Digestive Health Institute, Children's Hospital Colorado/University of Colorado, 13123 E 16th Ave, Aurora, CO 80045
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Pedrosa Carrasco AJ, Timmermann L, Pedrosa DJ. Management of constipation in patients with Parkinson's disease. NPJ Parkinsons Dis 2018; 4:6. [PMID: 29560414 PMCID: PMC5856748 DOI: 10.1038/s41531-018-0042-8] [Citation(s) in RCA: 37] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Revised: 01/30/2018] [Accepted: 02/06/2018] [Indexed: 12/13/2022] Open
Abstract
A considerable body of research has recently emerged around nonmotor symptoms in Parkinson's disease (PD) and their substantial impact on patients' well-being. A prominent example is constipation which occurs in up to two thirds of all PD-patients thereby effecting psychological and social distress and consequently reducing quality of life. Despite the significant clinical relevance of constipation, unfortunately little knowledge exists on effective treatments. Therefore this systematic review aims at providing a synopsis on clinical effects and safety of available treatment options for constipation in PD. For this purpose, three electronic databases (MEDLINE, EMBASE, PsycINFO) were searched for experimental and quasi-experimental studies investigating the efficacy/effectiveness of interventions in the management of PD-associated constipation. Besides, adverse events were analyzed as secondary outcome. In total, 18 publications were identified involving 15 different interventions, of which none can be attributed sufficient evidence to derive strong recommendations. Nevertheless, some evidence indicates that dietetic interventions with probiotics and prebiotics may reduce symptom burden while providing a very favorable side-effects profile. Furthermore, the use of lubiprostone, macrogol and in the specific case of isolated or prominent outlet obstruction constipation injections of botulinum neurotoxin A into the puborectal muscles may as well be moderately supported. In summary, too little attention has been paid to treatment options for constipation in PD leaving abundant room for further research addressing this topic.
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Affiliation(s)
- Anna J. Pedrosa Carrasco
- Sir Michael Sobell House, Oxford University Hospitals, Oxford, UK
- King’s College London, Cicely Saunders Institute, Department of Palliative Care, Policy and Rehabilitation, London, UK
- Department of Internal Medicine V, University Hospital of Gießen and Marburg, Gießen, Germany
| | - Lars Timmermann
- Department of Neurology, University Hospital of Gießen and Marburg, Marburg, Germany
| | - David J. Pedrosa
- Department of Neurology, University Hospital of Gießen and Marburg, Marburg, Germany
- Department of Psychiatry, University Hospital of Gießen and Marburg, Marburg, Germany
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Cheng LS, Goldstein AM. Surgical Management of Idiopathic Constipation in Pediatric Patients. Clin Colon Rectal Surg 2018; 31:89-98. [PMID: 29487491 DOI: 10.1055/s-0037-1609023] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Constipation is a common childhood problem, but an anatomic or physiologic cause is identified in fewer than 5% of children. By definition, idiopathic constipation is a diagnosis of exclusion. Careful clinical evaluation and thoughtful use of imaging and other testing can help exclude specific causes of constipation and guide therapy. Medical management with laxatives is effective for the majority of constipated children. For those patients unresponsive to medications, however, several surgical options can be employed, including anal procedures, antegrade colonic enemas, colorectal resection, and intestinal diversion. Judicious use of these procedures in properly selected patients and based on appropriate preoperative testing can lead to excellent outcomes. This review summarizes the surgical options available for managing refractory constipation in children and provides guidance on how to choose the best procedure for a given patient.
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Affiliation(s)
- Lily S Cheng
- Department of General Surgery, University of California San Francisco, San Francisco, California
| | - Allan M Goldstein
- Department of Surgery, Harvard Medical School, Boston, Massachusetts.,Department of Pediatric Surgery, Massachusetts General Hospital, Boston, Massachusetts.,MassGeneral Hospital for Children, Boston, Massachusetts
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Church JT, Gadepalli SK, Talishinsky T, Teitelbaum DH, Jarboe MD. Ultrasound-guided intrasphincteric botulinum toxin injection relieves obstructive defecation due to Hirschsprung's disease and internal anal sphincter achalasia. J Pediatr Surg 2017; 52:74-78. [PMID: 27836361 DOI: 10.1016/j.jpedsurg.2016.10.023] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2016] [Accepted: 10/20/2016] [Indexed: 12/17/2022]
Abstract
PURPOSE Chronic obstructive defecation can occur in patients with Hirschsprung Disease (HD) and internal anal sphincter (IAS) achalasia. Injection of Botulinum Toxin (BoTox) into the IAS can temporarily relieve obstructive defecation, but can be challenging when performed by tactile sense alone. We compared results of BoTox injections with and without ultrasound (US) guidance. METHODS We retrospectively reviewed BoTox injections into the IAS for obstructive defecation over 5years. Analyzed outcomes included short-term improvement, defined as resolution of enterocolitis, new ability to spontaneously defecate, and/or normalization of bowel movement frequency 2weeks post-operatively, as well as requirement of more definitive surgical therapy (myotomy/myomectomy, colectomy, colostomy, cecostomy/appendicostomy, and/or sacral nerve stimulator implantation). Outcomes were compared using t-test and Fisher's Exact test, with significance defined as p<0.05. RESULTS Twelve patients who underwent BoTox injection were included, including 5 patients who underwent injections both with and without ultrasound. Ten underwent an ultrasound-guided injection (13 injection procedures), 5 of whom had HD. Seven underwent an injection without ultrasound guidance (17 injection procedures), 5 of whom had HD. Procedures performed with US resulted in greater short-term improvement (76% versus 65% without ultrasound) and less requirement of a definitive procedure for obstructive defecation (p<0.05). CONCLUSIONS US-guided BoTox injection is safe and effective for obstructive defecation, and may decrease the need for a definitive operation. LEVEL OF EVIDENCE III.
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Affiliation(s)
- Joseph T Church
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Health System, Ann Arbor, USA.
| | - Samir K Gadepalli
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Health System, Ann Arbor, USA
| | - Toghrul Talishinsky
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Health System, Ann Arbor, USA
| | - Daniel H Teitelbaum
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Health System, Ann Arbor, USA
| | - Marcus D Jarboe
- Department of Surgery, Section of Pediatric Surgery, University of Michigan Health System, Ann Arbor, USA
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Brisinda G, Sivestrini N, Bianco G, Maria G. Treatment of gastrointestinal sphincters spasms with botulinum toxin A. Toxins (Basel) 2015; 7:1882-1916. [PMID: 26035487 PMCID: PMC4488680 DOI: 10.3390/toxins7061882] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2015] [Revised: 05/10/2015] [Accepted: 05/21/2015] [Indexed: 02/05/2023] Open
Abstract
Botulinum toxin A inhibits neuromuscular transmission. It has become a drug with many indications. The range of clinical applications has grown to encompass several neurological and non-neurological conditions. One of the most recent achievements in the field is the observation that botulinum toxin A provides benefit in diseases of the gastrointestinal tract. Although toxin blocks cholinergic nerve endings in the autonomic nervous system, it has also been shown that it does not block non-adrenergic non-cholinergic responses mediated by nitric oxide. This has promoted further interest in using botulinum toxin A as a treatment for overactive smooth muscles and sphincters. The introduction of this therapy has made the treatment of several clinical conditions easier, in the outpatient setting, at a lower cost and without permanent complications. This review presents current data on the use of botulinum toxin A in the treatment of pathological conditions of the gastrointestinal tract.
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Affiliation(s)
- Giuseppe Brisinda
- Department of Surgery, University Hospital "Agostino Gemelli", Largo Agostino Gemelli 8, 00168 Rome, Italy.
| | - Nicola Sivestrini
- Department of Surgery, University Hospital "Agostino Gemelli", Largo Agostino Gemelli 8, 00168 Rome, Italy.
| | - Giuseppe Bianco
- Department of Surgery, University Hospital "Agostino Gemelli", Largo Agostino Gemelli 8, 00168 Rome, Italy.
| | - Giorgio Maria
- Department of Surgery, University Hospital "Agostino Gemelli", Largo Agostino Gemelli 8, 00168 Rome, Italy.
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Arbizu RA, Rodriguez L. Use of Clostridium botulinum toxin in gastrointestinal motility disorders in children. World J Gastrointest Endosc 2015; 7:433-437. [PMID: 25992183 PMCID: PMC4436912 DOI: 10.4253/wjge.v7.i5.433] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2014] [Revised: 12/06/2014] [Accepted: 02/11/2015] [Indexed: 02/05/2023] Open
Abstract
More than a century has elapsed since the identification of Clostridia neurotoxins as the cause of paralytic diseases. Clostridium botulinum is a heterogeneous group of Gram-positive, rod-shaped, spore-forming, obligate anaerobic bacteria that produce a potent neurotoxin. Eight different Clostridium botulinum neurotoxins have been described (A-H) and 5 of those cause disease in humans. These toxins cause paralysis by blocking the presynaptic release of acetylcholine at the neuromuscular junction. Advantage can be taken of this blockade to alleviate muscle spams due to excessive neural activity of central origin or to weaken a muscle for treatment purposes. In therapeutic applications, minute quantities of botulinum neurotoxin type A are injected directly into selected muscles. The Food and Drug Administration first approved botulinum toxin (BT) type A in 1989 for the treatment of strabismus and blepharospasm associated with dystonia in patients 12 years of age or older. Ever since, therapeutic applications of BT have expanded to other systems, including the gastrointestinal tract. Although only a single fatality has been reported to our knowledge with use of BT for gastroenterological conditions, there are significant complications ranging from minor pain, rash and allergic reactions to pneumothorax, bowel perforation and significant paralysis of tissues surrounding the injection (including vocal cord paralysis and dysphagia). This editorial describes the clinical experience and evidence for the use BT in gastrointestinal motility disorders in children.
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Wester T, Granström AL. Botulinum toxin is efficient to treat obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 2015; 31:255-9. [PMID: 25616563 DOI: 10.1007/s00383-015-3665-4] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 01/13/2015] [Indexed: 12/17/2022]
Abstract
BACKGROUND Obstructive symptoms are common after pull-through for Hirschsprung disease. Botulinum toxin injection treatment may improve the bowel function if internal sphincter achalasia is the cause of obstructive symptoms. The aim of this study was to review the outcome in patients treated with intrasphincteric botulinum toxin injections after pull-through for Hirschsprung disease. METHODS The operative records were used to identify children with Hirschsprung disease who were treated with botulinum toxin injections at Karolinska University Hospital, Stockholm, Sweden, from September 2007 to November 2014. Data on age, sex, associated syndromes, length of aganglionic segment, age at pull-through, type of pull-through, age at first botulinum toxin injection, indication for botulinum toxin injection, and effect of first botulinum toxin injection were retrieved from the case records. Bowel function at last follow-up visit or telephone contact was recorded. RESULTS Nineteen patients were identified. All had biopsy-verified Hirschsprung disease. Eighteen (15 males and 3 females) children had undergone intrasphincteric botulinum toxin injection treatment for obstructive symptoms after pull-through, which was done at 127 (18-538) days of age. Four children had total colonic aganglionosis. The first botulinum toxin injection was given at 2.4 (0.53-6.9) years of age. Thirteen children (72 %) had a good response to the first injection treatment. The children underwent 3 (1-13) injection treatments. At follow-up four patients had improved and did not need treatment for obstruction, four were scheduled for further botulinum toxin injections, eight had persistent obstructive symptoms treated with laxatives or enemas, and two children had an ileostomy. CONCLUSION Botulinum toxin injection treatment improves the obstructive symptoms in children after pull-through for Hirschsprung disease. The effect is reversible and a majority of patients need repeat injections. When injection treatment is not repeated, a large proportion of children need laxatives or enemas due to recurrent symptoms.
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Affiliation(s)
- Tomas Wester
- Section of Pediatric Surgery, Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden,
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33
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Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease. J Pediatr Gastroenterol Nutr 2014; 59:604-7. [PMID: 25000353 DOI: 10.1097/mpg.0000000000000483] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
OBJECTIVES A nonrelaxing internal anal sphincter is present in a relatively large proportion of children with surgically treated Hirschsprung disease (HD) and can cause obstructive gastrointestinal symptoms. The short- and long-term outcome and adverse effects of intrasphincteric botulinum toxin (Botox) injections in children with obstruction after surgically treated HD are evaluated. METHODS The outcome of children with surgically treated HD treated with intrasphincteric Botox injections for obstructive symptoms was analyzed with a retrospective chart review between 2002 and 2013 in the University Medical Centers of Maastricht and Nijmegen. RESULTS A total of 33 patients were included. The median time of follow-up was 7.3 years (range 1-24). A median of 2 (range 1-5) injections were given. Initial improvement was achieved in 76%, with a median duration of 4.1 months (range 1.7-58.8). Proportion of children hospitalized for enterocolitis decreased after treatment from 19 to 7. A good long-term response was found in 49%. Two children experienced complications: transient pelvic muscle paresis with impairment of walking. In both children symptoms resolved within 4 months without treatment. CONCLUSIONS Intrasphincteric Botox injections in surgically treated HD are an effective long-term therapy in approximately half of our patients with obstructive symptoms. The possibility of adverse effects should be noticed.
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Basson S, Charlesworth P, Healy C, Phelps S, Cleeve S. Botulinum toxin use in paediatric colorectal surgery. Pediatr Surg Int 2014; 30:833-8. [PMID: 24997611 DOI: 10.1007/s00383-014-3536-4] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/18/2014] [Indexed: 12/15/2022]
Abstract
PURPOSE To evaluate outcomes of intrasphincteric botulinum toxin injection (ISBTI) in children with intractable constipation. METHODS Retrospective case-note review of patients ≤ 16 years of age undergoing ISBTI between January 2010 and February 2014. Data collected included patient demographics, diagnosis, complications, follow-up duration and functional outcomes. Successful outcome was defined as resolution/improvement in symptoms and failed when there was no change in symptoms. Statistical analyses were performed using PRISM (GraphPad, CA, USA). p values <0.05 were considered as significant. RESULTS 43 patients [male 29, median age 5 years 9 months (range 13 months-13 years 5 months)] underwent 86 ISBTIs. Underlying diagnoses were idiopathic constipation (67 %), Hirschsprung disease (26 %), anorectal malformation (5 %), gastrointestinal dysmotility (2 %). 72 % (31/43) reported improvement in symptoms after the first ISBTI. 39 % of patients had recurrence of symptoms at 12-month median follow-up. 10 patients non-responsive to ISBTI required an antegrade continence enema or stoma. There was no correlation between age (p = 0.3), gender (p = 0.7), diagnosis (p = 0.84), or number of ISBTIs (p = 0.17) with successful outcome. CONCLUSION Successful outcomes occurred in 72 % patients after the first ISBTI. 25 % required further surgical management of their symptoms. Further work is required to help predict which patients will benefit from ISBTI.
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Affiliation(s)
- S Basson
- Department of Paediatric Surgery, The Royal London Hospital, Whitechapel, London, E1 1BB, UK
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35
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Abstract
The prevalence of functional constipation in children is almost 10%. The etiology is multifactorial and not fully understood. In the majority of cases, there is no organic explanation for the symptoms. However, it is very important to exclude organic causes, particularly in neonates and infants. There are warning symptoms, such as delayed passage of meconium, bilious vomiting and abdominal distention that should prompt further investigations. Fecal incontinence is a very common symptom secondary to fecal impaction. The first-line treatment for both disimpaction and maintenance is use of laxatives. Parental education is extremely important. There are very limited data on surgical approaches for functional constipation.
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Abstract
"Variants of Hirschsprung's disease" are conditions that clinically resemble Hirschsprung's disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.
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37
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Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH. Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int 2013; 29:873-81. [PMID: 23913261 DOI: 10.1007/s00383-013-3353-1] [Citation(s) in RCA: 95] [Impact Index Per Article: 7.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung disease (HD). Presenting either before or after definitive surgery for HD, HAEC may manifest clinically as abdominal distension and explosive diarrhea, along with emesis, fever, lethargy, and even shock. The pathogenesis of HAEC, the subject of ongoing research, likely involves a complex interplay between a dysfunctional enteric nervous system, abnormal mucin production, insufficient immunoglobulin secretion, and unbalanced intestinal microflora. Early recognition of HAEC and preventative practices, such as rectal washouts following a pull-through, can lead to improved outcomes. Treatment strategies for acute HAEC include timely resuscitation, colonic decompression, and antibiotics. Recurrent or persistent HAEC requires evaluation for mechanical obstruction or residual aganglionosis, and may require surgical treatment with posterior myotomy/myectomy or redo pull-through. This chapter describes the incidence, pathogenesis, treatment, and preventative strategies in management of HAEC.
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Affiliation(s)
- Farokh R Demehri
- Section of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan Health System, 1540 E. Hospital Dr., SPC 4211, Ann Arbor, MI 48109-4211, USA
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Butler Tjaden NE, Trainor PA. The developmental etiology and pathogenesis of Hirschsprung disease. Transl Res 2013; 162:1-15. [PMID: 23528997 PMCID: PMC3691347 DOI: 10.1016/j.trsl.2013.03.001] [Citation(s) in RCA: 159] [Impact Index Per Article: 13.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/03/2012] [Revised: 02/25/2013] [Accepted: 03/01/2013] [Indexed: 02/08/2023]
Abstract
The enteric nervous system is the part of the autonomic nervous system that directly controls the gastrointestinal tract. Derived from a multipotent, migratory cell population called the neural crest, a complete enteric nervous system is necessary for proper gut function. Disorders that arise as a consequence of defective neural crest cell development are termed neurocristopathies. One such disorder is Hirschsprung disease (HSCR), also known as congenital megacolon or intestinal aganglionosis. HSCR occurs in 1/5000 live births and typically presents with the inability to pass meconium, along with abdominal distension and discomfort that usually requires surgical resection of the aganglionic bowel. This disorder is characterized by a congenital absence of neurons in a portion of the intestinal tract, usually the distal colon, because of a disruption of normal neural crest cell migration, proliferation, differentiation, survival, and/or apoptosis. The inheritance of HSCR disease is complex, often non-Mendelian, and characterized by variable penetrance. Extensive research has identified a number of key genes that regulate neural crest cell development in the pathogenesis of HSCR including RET, GDNF, GFRα1, NRTN, EDNRB, ET3, ZFHX1B, PHOX2b, SOX10, and SHH. However, mutations in these genes account for only ∼50% of the known cases of HSCR. Thus, other genetic mutations and combinations of genetic mutations and modifiers likely contribute to the etiology and pathogenesis of HSCR. The aims of this review are to summarize the HSCR phenotype, diagnosis, and treatment options; to discuss the major genetic causes and the mechanisms by which they disrupt normal enteric neural crest cell development; and to explore new pathways that may contribute to HSCR pathogenesis.
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Levitt MA, Dickie B, Peña A. The Hirschsprungs patient who is soiling after what was considered a "successful" pull-through. Semin Pediatr Surg 2012; 21:344-53. [PMID: 22985840 DOI: 10.1053/j.sempedsurg.2012.07.009] [Citation(s) in RCA: 55] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
After surgery for Hirschsprungs disease, most children thrive, have few if any episodes of abdominal distention and enterocolitis, and are fecally continent. However, there exists a small group of patients who do not do well. Either they suffer from persistent distension and enterocolitis or they experience soiling after their pull-through procedure. These patients can be systematically evaluated and successfully treated with a combination of bowel management, dietary changes, and laxatives, and, in certain circumstances, a reoperation.
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Affiliation(s)
- Marc A Levitt
- Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Division of Pediatric Surgery, Department of Surgery, University of Cincinnati, Cincinnati, OH 45229, USA.
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Abstract
Hirschsprung-associated enterocolitis remains the greatest cause of morbidity and mortality in children with Hirschsprung disease. This chapter details the various approaches used to treat and prevent this disease process. This includes prevention of complications, such as stricture formation, prophylaxis with rectal washouts, and identification of high-risk individuals. The chapter also details approaches to diagnose Hirschsprung-associated enterocolitis as well as to exclude other etiologies.
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Friedmacher F, Puri P. Comparison of posterior internal anal sphincter myectomy and intrasphincteric botulinum toxin injection for treatment of internal anal sphincter achalasia: a meta-analysis. Pediatr Surg Int 2012; 28:765-71. [PMID: 22806601 DOI: 10.1007/s00383-012-3123-5] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/28/2023]
Abstract
PURPOSE Internal anal sphincter (IAS) achalasia is a clinical condition with presentation similar to Hirschsprung's disease, but with the presence of ganglion cells on rectal suction biopsy (RSB). The diagnosis is made by anorectal manometry (ARM), which demonstrates the absence of the rectosphincteric reflex on rectal balloon inflation. The recommended treatment of choice is posterior IAS myectomy. Recently, intrasphincteric botulinum toxin (Botox) injection has been effectively used for treatment of IAS achalasia. The aim of this meta-analysis was to compare the efficacy of posterior IAS myectomy with intrasphincteric Botox injection for treatment of IAS achalasia. METHODS A systematic literature search for relevant articles was conducted using the following databases: MEDLINE( ® ), EMBASE(®), ISI Web of Science(SM) and the Cochrane Library. A meta-analysis was performed with the studies where IAS achalasia was diagnosed based on the results of ARM and RSB. Odds ratio (OR) with 95 % confidence intervals were calculated. RESULT Sixteen prospective and retrospective studies, published from 1973 to 2009, were identified. A total of 395 patients with IAS achalasia were included in this meta-analysis. Fifty-eight percent of patients underwent IAS myectomy and 42 % Botox injection. Regular bowel movements were significantly more frequent after IAS myectomy (OR 0.53, [95 % CI 0.29-0.99]; p = 0.04). There was no significant difference in continued use of laxatives or rectal enemas (OR 0.92, [95 % CI 0.34-2.53], p = 0.89) and in overall complication rates between both procedures (OR 0.68, [95 % CI 0.38-1.21]; p = 0.19). Looking at specific complications, the rate of transient faecal incontinence was significantly higher after Botox injection (OR 0.07, [95 % CI 0.01-0.54]; p < 0.01). Constipation and soiling were not significantly different between both procedures (OR 0.66, [95 % CI 0.30-1.48]; p = 0.31 and OR 0.24, [95 % CI 0.03-2.07]; p = 0.25). The rate of non-response was significantly higher after Botox injection (OR 0.52, [95 % CI 0.27-0.99]; p = 0.04). Subsequent surgical treatment was significantly more frequent after Botox injection (OR 0.18, [95 % CI 0.07-0.44]; p < 0.0001). Short- and long-term improvements were significantly more frequent after IAS myectomy (OR 0.56, [95 % CI 0.32-0.97]; p = 0.04 and OR 0.25, [95 % CI 0.15-0.41]; p < 0.0001). CONCLUSION This meta-analysis indicates that in patients with IAS achalasia, posterior IAS myectomy appears to be a more effective treatment option compared to intrasphincteric Botox injection. After Botox injection, the rate of transient faecal incontinence, non-response and subsequent surgical procedures were significantly higher compared to IAS myectomy.
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Affiliation(s)
- Florian Friedmacher
- National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland
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42
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Affiliation(s)
- A Gunnarsdóttir
- Section of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden
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43
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Friedmacher F, Puri P. Residual aganglionosis after pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis. Pediatr Surg Int 2011; 27:1053-7. [PMID: 21789665 DOI: 10.1007/s00383-011-2958-5] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
PURPOSE Most patients with Hirschsprung's disease (HD) have a satisfactory outcome after pull-through (PT) operation. However, some children continue to have persistent bowel symptoms after the initial operation and may require redo PT. Redo PT operation in HD is usually indicated for anastomotic strictures or residual aganglionosis (RA). We designed this meta-analysis to determine the incidence and outcome of RA among patients with HD following PT operation. METHODS A meta-analysis of redo PT operations for HD reported in the literature between 1985 and 2011 was performed. Detailed information was recorded in patients with RA and transition-zone bowel (TZB), including recurrent bowel problems, histological findings on repeat rectal biopsy, type of redo PT operation and outcome. RESULTS Twenty-nine articles reported 555 patients with redo PT operations. 193 (34.8%) patients demonstrated abnormal histological findings on rectal biopsy with 144 patients showing RA and 49 patients showing TZB. These 193 patients presented with persistent constipation (n = 135), recurrent enterocolitis (n = 45) and abnormal histology of the pulled-through bowel (n = 13). Mean age at redo PT was 4.4 years (range 4 months-17 years). Redo procedures were Duhamel (n = 57), transanal endorectal PT (n = 40), Soave (n = 35), Swenson (n = 10), posterior sagittal approach (n = 1) and not reported (n = 50). Follow-up information after redo PT was available in 134 (69.4%) patients and not available in 59 patients. Of the 134 patients, 99 (73.9%) patients had normal bowel habits, 19 patients had persistent constipation/soiling and 16 patients had recurrent enterocolitis. CONCLUSION This meta-analysis reveals that RA and TZB are the underlying causes of persistent bowel symptoms in one-third of all patients with HD requiring redo PT operation. Most patients have a satisfactory outcome after redo operation. Rectal biopsy should be performed in all patients with recurrent bowel problems after PT operation.
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Affiliation(s)
- Florian Friedmacher
- National Childrens's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland
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Hosseini SMV, Foroutan HR, Zeraatian S, Sabet B. Botulinium toxin, as bridge to transanal pullthrough in neonate with Hirschsprungs disease. J Indian Assoc Pediatr Surg 2011; 13:69-71. [PMID: 20011471 PMCID: PMC2788448 DOI: 10.4103/0971-9261.43025] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Aims and Objectives: The aim of this study is to find easier way of home care while obviating the colostomy before single stage pull through operation. Materials and Methods: From August 2005 to December 2006, eight cases of neonatal Hirschsprung disease were treated. Mean age 4.5 (2-6) day/old with absent anorectal inhibitory reflex, rectosigmiod disease in Barium enema, positive Acetylcholine esterase (Ache) staining, good response to rectal washout. They underwent botulinium toxin injection (5 unit /kg/quadrant) in four quadrant intrasphincteric. They were followed until pull through operation in 8-10 weeks post injection. Results: Four of 8 (50%) cases only needed rectal washout for three to five days post injection until pull through operation, two had decrease in number of rectal washouts /day and the remaining two underwent colostomy five days post injection because of no response. Conclusion: Botulinium toxin injection can help in palliative care in patients with Hirschsprung disease who are waiting for colostomy or definitive pullthrough. It gives an option of eaiser home care for these patients.
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Affiliation(s)
- S M V Hosseini
- Department of Pediatric Surgery, Shiraz University Medical Science, Shiraz, Iran
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Abstract
BACKGROUND AND OBJECTIVE The majority of children with Hirschsprung disease (HD) after corrective surgery (CS) develop protracted defecation disorders (DDs) such as constipation, fecal incontinence, and/or enterocolitis. The aim of this investigation was to determine the diagnoses, therapies, and long-term clinical outcomes using a systematic algorithm to address protracted DD in children with HD after CS. METHODS Retrospective review of children with HD after CS cared for using a systematic algorithm at a tertiary care center. Potential anatomic etiologies were evaluated for first. Clinical outcome was categorized into 4 groups based on symptom severity, time interval from last enterocolitis episode, laxative usage, and/or rectal therapies at the time of last follow-up. RESULTS Fifty-seven children were identified, of whom 51 (89.5%) had obstructive symptoms and/or enterocolitis and 6 (10.5%) had nonretentive fecal incontinence. Nonintractable constipation responsive to laxatives was identified in 10 (17.5%), colonic dysmotility in 4 (7.0%), nonrelaxing anal sphincter as a primary etiology in 22 (38.6%), bacterial overgrowth in 2 (3.5%), food intolerance in 2 (3.5%), and rapid transit in 2 (3.5%). Further surgical intervention was undertaken in 22 (38.6%), including 9 (15.8%) for residual aganglionosis. Mean follow-up was 41.4 ± 4.5 months. Clinical outcomes were excellent in 16 (28.1%), good in 22 (38.6%), fair in 1 (1.8%), and poor in 18 (31.6%). Children with enterocolitis were more likely to have an excellent or good clinical outcome. CONCLUSIONS The majority of children with HD and protracted DD after CS have a favorable long-term clinical outcome when following a systematic algorithm.
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Chao NS, Wong BP, Leung MW, Tang PM, Liu CS, Fan TW, Tsang TK, Lo KK, Kwok WK, Liu KK. Botulinum toxin in the treatment of refractory constipation associated with anal sphincter hypertonicity: A pilot prospective study in Chinese children (CME paper). SURGICAL PRACTICE 2011. [DOI: 10.1111/j.1744-1633.2010.00528.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Patrus B, Nasr A, Langer JC, Gerstle JT. Intrasphincteric botulinum toxin decreases the rate of hospitalization for postoperative obstructive symptoms in children with Hirschsprung disease. J Pediatr Surg 2011; 46:184-7. [PMID: 21238663 DOI: 10.1016/j.jpedsurg.2010.09.089] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/25/2010] [Accepted: 09/30/2010] [Indexed: 12/18/2022]
Abstract
BACKGROUND Although most children with Hirschsprung disease do well after pull-through surgery, some continue to have persistent obstructive symptoms. The purpose of this study was to evaluate the effect of intrasphincteric botulinum toxin in the management of these children. METHODS A retrospective review of patients with Hirschsprung disease treated over 10 years was performed. RESULTS Twenty-two patients who had previously undergone pull-through surgery received a median number of 2 botulinum toxin injections (range, 1-23). The number of hospitalizations for obstructive symptoms significantly decreased from preinjection (median, 1.5; interquartile range [IQR], 1-3) to postinjection (median, 0; IQR, 0-1) (P = .0003). The number of injections was lower in children with a rectosigmoid transition zone (median, 1 injection; IQR, 1-3.5) than in those with long-segment disease (median, 3 injections; IQR, 1-15) (P = .04). Eighty percent of patients had a good response to the first dose of botulinum toxin, and 69% of them required additional injections. There were no short-term or long-term complications related to botulinum toxin. CONCLUSIONS Intrasphincteric botulinum toxin significantly decreased the need for obstruction-related hospitalization in children who had undergone pull-through surgery for Hirschsprung disease. Botulinum toxin should be strongly considered in the management algorithm for postoperative obstructive symptoms in children with Hirschsprung disease.
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Affiliation(s)
- Bashar Patrus
- Division of General and Thoracic Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
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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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Keshtgar AS, Ward HC, Clayden GS. Transcutaneous needle-free injection of botulinum toxin: a novel treatment of childhood constipation and anal fissure. J Pediatr Surg 2009; 44:1791-8. [PMID: 19735827 DOI: 10.1016/j.jpedsurg.2009.02.056] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/04/2008] [Revised: 02/27/2009] [Accepted: 02/27/2009] [Indexed: 01/28/2023]
Abstract
PURPOSE Constipation is a common problem in children, and when it becomes chronic fecal impaction, overflow soiling and megarectum may develop. Children with chronic idiopathic constipation (IC) may not respond to conventional treatments of laxatives, enemas, and toilet training. The aims of the study were to evaluate the long-term outcome of transcutaneous needle-free injection of botulinum toxin (TNFBT) into the external anal sphincter (EAS) and to assess the extent of the toxin penetration into the sphincter. METHOD Children were recruited if symptomatic with chronic constipation, soiling, painful defecation, and withholding behavior requiring disimpaction of stool and rectal biopsy under general anesthesia. A total dose of 200 U of botulinum toxin (BT) (Dysport; Ipsen Limited, Slough, United Kingdom) was injected transcutaneously into the EAS at 3 and 9-o'clock positions using J-tip needle-free syringes (National Medical Products Inc, Irvine, Calif). The depth and width of toxin penetration was assessed by endosonography. Outcome was measured by a validated symptom severity (SS) score questionnaire. The total SS score ranged between 0 (best) and 65 (worst). The outcome was compared with 31 children in a comparable historical control group at 3 and 12-month follow-up. RESULTS Sixteen children were recruited with median age of 6.11 (range, 3-14.85) years and median duration of symptoms of 3.9 years (1.6-11.5). On endosonography, the median depth and width of BT penetration was 8 (7-10) mm and 8 (6-10) mm, respectively. At 3-month follow-up, the median SS score improved in all children after TNFBT from 32.50 (5-57) to 7.50 (0-26) (Wilcoxon's P < .0001). There were significant improvements in symptoms of constipation, soiling, painful defecation, general health and behavior, and fecal impaction of rectum (P < .05). Anal fissures healed in all 4 children. The SS score in the control group improved from 33 (12-49) to 15 (0-40) (P < .0001). At 12-month follow-up, the improvement of SS score in TNFBT group was significantly more than the control group as follows: 4 (0-25) vs 15 (0-51), respectively (Mann-Whitney U P < .002). Three patients had a second TNFBT injection for relapsed symptoms. There were no complications. The transcutaneous needle-free injection of botulinum toxin eliminates the risk of intravascular injection or needlestick injury. The transcutaneous needle-free injection of botulinum toxin also has other therapeutic applications including an alternative therapy to biofeedback training for dyssynergia of the EAS, treatment of muscle limb spasticity in cerebral palsy, and cosmetic treatment of overactive facial muscles and wrinkles and hyperhydrosis. CONCLUSION Transcutaneous needle-free injection of botulinum toxin into the external anal sphincter is a novel and safe new treatment of chronic idiopathic constipation and anal fissure in children. A second injection may be required in 20% of patients.
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Affiliation(s)
- Alireza S Keshtgar
- University Hospital Lewisham, National Health Service Trust, London, United Kingdom.
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Chumpitazi BP, Fishman SJ, Nurko S. Long-term clinical outcome after botulinum toxin injection in children with nonrelaxing internal anal sphincter. Am J Gastroenterol 2009; 104:976-83. [PMID: 19259081 DOI: 10.1038/ajg.2008.110] [Citation(s) in RCA: 52] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVES Children with surgically repaired Hirschsprung's disease (HD) and those with internal anal sphincter (IAS) achalasia may develop obstructive gastrointestinal symptoms and/or enterocolitis due to a functional obstruction caused by an inability of the IAS to relax. Anal sphincter Clostridium botulinum toxin (BoTox) injections may provide a reversible therapy. However, there is limited information regarding the long-term outcomes of children receiving this therapy. The primary aim of this study was to determine the long-term clinical outcomes of BoTox therapy in children with a nonrelaxing IAS. The secondary aim of this study was to determine prognostic factors predicting a favorable outcome following BoTox IAS injection. METHODS We conducted a retrospective review of children with nonrelaxing IAS who received anal sphincter BoTox at a tertiary medical center. Children were classified into one of four long-term clinical outcome groups (excellent, good, fair, poor). RESULTS A total of 73 children (30 HD, 43 IAS achalasia) received anal sphincter BoTox injections and had a mean follow-up of 32.1+/-2.9 (s.e.) months. A mean of 2.7+/-0.2 injections were given to each child, with 56 (76.7%) children receiving multiple injections. An initial clinical improvement was seen in 65 of 73 (89%) children after the first injection. A total of 39 (53.4%) children had an excellent or good long-term outcome that was maintained for a mean of 17.1+/-3.1 months from the time of the last BoTox injection. Hospitalization rates significantly decreased in those previously hospitalized before initial BoTox injection. Seven (9.5%) patients developed transient fecal incontinence, and one (1.3%) developed significant pain after an injection. Factors predicting a favorable long-term clinical outcome were initial short-term improvement after the first BoTox injection and having IAS achalasia rather than HD. CONCLUSIONS Anal sphincter BoTox may be an effective and safe long-term therapy for children with nonrelaxing IAS.
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Affiliation(s)
- Bruno P Chumpitazi
- Center for Motility and Functional Gastrointestinal Disorders, Children's Hospital Boston, Boston, Massachusetts 02115, USA
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