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Homan M, Thomson M, Bontems P, Saccomani MD, Dias JA, Faraci S, Furlano R, Hojsak I, Ledder O, Slae M, Narula P, Nita AF, Norsa L, Oliva S, Papadopoulou A, Romano C, Rybak A, Spyropoulou V, Tambucci R, Tzivinikos C, van Wijk M, Borrelli O. Drugs in focus: Botulinum toxin in the therapy of gastrointestinal disorders in children. J Pediatr Gastroenterol Nutr 2024; 79:1096-1105. [PMID: 39315663 PMCID: PMC11615126 DOI: 10.1002/jpn3.12376] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2024] [Revised: 08/10/2024] [Accepted: 09/05/2024] [Indexed: 09/25/2024]
Abstract
What is Known Botulinum toxin (BoNT) causes muscle relaxation by inhibiting acetylcholine release from presynaptic motor neurons at the neuromuscular junction. What is New In children with achalasia, BoNT can be considered only in patients in whom rapid weight gain is important to improve surgical outcomes. BoNT has been suggested for treating cricopharyngeal achalasia and delayed gastric emptying. Anal achalasia and constipation after Hirschsprung disease corrective surgery are very promising indications for BoNT use. In selected children with resistant type of functional constipation and chronic anal fissure, BoNT is a viable option for treating.
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Affiliation(s)
- Matjaž Homan
- Department of Gastroenterology, Hepatology, and Nutrition, University Children's Hospital, Faculty of MedicineUniversity of LjubljanaLjubljanaSlovenia
| | - Mike Thomson
- Department of Paediatric GastroenterologySheffield Children's Hospital NHS FT and University of SheffieldSheffieldUK
| | - Patrick Bontems
- Paediatric Gastroenterology, Hôpital Universitaire des Enfants Reine FabiolaUniversité Libre de BruxellesBrusselsBelgium
| | | | - Jorge Amil Dias
- Department of GastroenterologyHospital LusíadasPortoPortugal
| | - Simona Faraci
- Gastroenterology and Nutrition UnitBambino Gesù Children's Hospital, IRCCSRomeItaly
| | - Raoul Furlano
- Department of GastroenterologyUniversity Children's Hospital Basel (UKBB), University of BaselBaselSwitzerland
| | - Iva Hojsak
- Children's Hospital ZagrebUniversity of Zagreb Medical SchoolZagrebCroatia
| | - Oren Ledder
- Juliet Keidan Institute of Pediatric Gastroenterology, Shaare Zedek Medical CenterHebrew University of JerusalemJerusalemIsrael
| | - Mordechai Slae
- Pediatric Gastroenterology, Hepatology and Nutrition UnitHadassah University HospitalJerusalemIsrael
| | - Priya Narula
- Department of Paediatric GastroenterologySheffield Children's Hospital NHS FT and University of SheffieldSheffieldUK
| | | | - Lorenzo Norsa
- Pediatric Department, Children's Hospital Vittore BuzziUniversity of MilanMilanItaly
| | - Salvatore Oliva
- Pediatric Gastroenterology and Liver Unit, Maternal and Child Health DepartmentSapienza—University of RomeRomeItaly
| | - Alexandra Papadopoulou
- Division of Gastroenterology and Hepatology, First Department of PediatricsUniversity of Athens, Children's Hospital Agia SofiaAthensGreece
| | - Claudio Romano
- Claudio Romano Pediatric Gastroenterology and Cystic Fibrosis UnitUniversity of MessinaMessinaItaly
| | - Anna Rybak
- Paediatric Gastroenterology DepartmentGreat Ormond Street HospitalLondonUK
| | - Vasiliki Spyropoulou
- Division of Gastroenterology, Hepatology and NutritionUniversity Children's Hospital ZurichZurichSwitzerland
| | - Renato Tambucci
- Gastroenterology and Nutrition UnitBambino Gesù Children's Hospital, IRCCSRomeItaly
| | - Christos Tzivinikos
- Pediatric Gastroenterology Department, Al Jalila Children's Specialty HospitalMohammed Bin Rashid University of Medicine and Health SciencesDubaiUnited Arab Emirates
| | - Michiel van Wijk
- Emma Children's Hospital‐Amsterdam UMCVrije Universiteit AmsterdamAmsterdamthe Netherlands
| | - Osvaldo Borrelli
- Paediatric Gastroenterology DepartmentGreat Ormond Street HospitalLondonUK
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Rentea RM, Bokova E, Frischer JS, Gosain A, Langer JC, Levitt MA, Nandivada P, Rialon KL, Rollins MD, Segura B, Wood RJ, Lim IIP. Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group. J Pediatr Surg 2024; 59:161677. [PMID: 39217005 DOI: 10.1016/j.jpedsurg.2024.08.017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/25/2024] [Revised: 07/24/2024] [Accepted: 08/04/2024] [Indexed: 09/04/2024]
Abstract
BACKGROUND Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD. METHODS The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD. RESULTS This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations. CONCLUSIONS A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management. LEVEL OF EVIDENCE V.
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Affiliation(s)
- Rebecca M Rentea
- Comprehensive Colorectal Center, Section of Colorectal and Pelvic Reconstructive Surgery, Department of Pediatric Surgery, Children's Mercy Kansas City, Kansas City, MO, 64108, USA; Department of Surgery, University of Missouri-Kansas City, Kansas City, MO, 64108, USA.
| | - Elizaveta Bokova
- Comprehensive Colorectal Center, Section of Colorectal and Pelvic Reconstructive Surgery, Department of Pediatric Surgery, Children's Mercy Kansas City, Kansas City, MO, 64108, USA
| | - Jason S Frischer
- Colorectal Center for Children, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
| | - Ankush Gosain
- Department of Pediatric Surgery, Children's Hospital of Colorado, University of Colorado, Aurora, CO, USA
| | - Jacob C Langer
- Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
| | - Marc A Levitt
- Division of Colorectal and Pelvic Reconstruction, Children's National Medical Center, Washington, DC, 20001, USA
| | - Prathima Nandivada
- Colorectal and Pelvic Malformation Center, Department of Surgery, Boston Children's Hospital, Boston, MA, 02115, USA
| | - Kristy L Rialon
- Division of Pediatric Surgery, Department of Surgery, Texas Children's Hospital, Texas, USA
| | - Michael D Rollins
- Department of Surgery, Primary Children's Hospital, University of Utah, Salt Lake City, UT, USA
| | - Bradley Segura
- University of Minnesota, Department of Pediatric Surgery, MHealth Fairview Masonic Children's Hospital, Minneapolis, MN, USA
| | - Richard J Wood
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH, USA
| | - Irene Isabel P Lim
- Comprehensive Colorectal Center, Section of Colorectal and Pelvic Reconstructive Surgery, Department of Pediatric Surgery, Children's Mercy Kansas City, Kansas City, MO, 64108, USA; Department of Surgery, University of Missouri-Kansas City, Kansas City, MO, 64108, USA
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Wheeler JC, Short SS, Rollins MD. Anorectal Manometry in Pediatric Colorectal Surgical Care. CHILDREN (BASEL, SWITZERLAND) 2024; 11:654. [PMID: 38929233 PMCID: PMC11201792 DOI: 10.3390/children11060654] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/12/2024] [Revised: 05/14/2024] [Accepted: 05/21/2024] [Indexed: 06/28/2024]
Abstract
BACKGROUND Pediatric colorectal specialists care for patients with a variety of defecation disorders. Anorectal (AR) manometry testing is a valuable tool in the diagnosis and management of these children. This paper provides a summary of AR manometry techniques and applications as well as a review of AR manometry findings in pediatric patients with severe defecation disorders referred to a pediatric colorectal center. This is the first study describing multi-year experience using a portable AR manometry device in pediatric patients. METHODS An electronic medical record review was performed (1/2018 to 12/2023) of pediatric patients with defecation disorders who had AR manometry testing. Demographics, diagnostic findings, and outcomes are described. KEY RESULTS A total of 297 unique patients (56.9% male, n = 169) had AR manometry testing. Of these, 72% (n = 188) had dyssynergic defecation patterns, of which 67.6% (n = 127) had fecal soiling prior to treatment. Pelvic rehabilitation (PR) was administered to 35.4% (n = 105) of all patients. A total of 79.5% (n = 58) of the 73 patients that had fecal soiling at initial presentation and completed PR with physical therapy and a bowel management program were continent after therapy. AR manometry was well tolerated, with no major complications. CONCLUSIONS AR manometry is a simple test that can help guide the management of pediatric colorectal surgical patients with defecation disorders. As a secondary finding, PR is a useful treatment for patients with dyssynergic stooling.
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Affiliation(s)
- Justin C. Wheeler
- Division of Pediatric Gastroenterology, University of Utah, Salt Lake City, UT 84113, USA;
| | - Scott S. Short
- Division of Pediatric Surgery, University of Utah, Salt Lake City, UT 84112, USA
| | - Michael D. Rollins
- Division of Pediatric Surgery, University of Utah, Salt Lake City, UT 84112, USA
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Thaiwatcharamas K, Loilome W, Ho PN, Chusilp S, Tanming P, Klanrit P, Phetcharaburanin J. Children with Hirschsprung disease exhibited alterations in host-microbial co-metabolism after pull-through operation. Pediatr Surg Int 2024; 40:87. [PMID: 38512700 DOI: 10.1007/s00383-024-05667-3] [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] [Accepted: 02/23/2024] [Indexed: 03/23/2024]
Abstract
PURPOSE This study aims to compare the fecal metabolome in post pull-through HD with and without HAEC patients and healthy young children using nuclear magnetic resonance (NMR) spectroscopy. METHODS Fresh fecal samples were collected from children under 5 years of age in both post-pull-through HD patients and healthy Thai children. A total of 20 fecal samples were then analyzed using NMR spectroscopy. RESULTS Thirty-four metabolites identified among HD and healthy children younger than 5 years were compared. HD samples demonstrated a significant decrease in acetoin, phenylacetylglutamine, and N-acetylornithine (corrected p value = 0.01, 0.04, and 0.004, respectively). Succinate and xylose significantly decreased in HD with HAEC group compared to HD without HAEC group (corrected p value = 0.04 and 0.02, respectively). Moreover, glutamine and glutamate metabolism, and alanine, aspartate, and glutamate metabolism were the significant pathways involved, with pathway impact 0.42 and 0.50, respectively (corrected p value = 0.02 and 0.04, respectively). CONCLUSION Differences in class, quantity, and metabolism of protein and other metabolites in young children with HD after pull-through operation were identified. Most of the associated metabolic pathways were correlated with the amino acids metabolism, which is required to maintain intestinal integrity and function.
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Affiliation(s)
| | - Watcharin Loilome
- Department of Systems Biosciences and Computational Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
- Cholangiocarcinoma Research Institute, Khon Kaen University, Khon Kaen, Thailand
- Khon Kaen University Phenome Centre, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Phuc N Ho
- Department of Systems Biosciences and Computational Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Sinobol Chusilp
- Department of Surgery, Division of Pediatric Surgery, Khon Kaen University, Khon Kaen, Thailand
| | - Patchareeporn Tanming
- Department of Surgery, Division of Pediatric Surgery, Khon Kaen University, Khon Kaen, Thailand
| | - Poramate Klanrit
- Department of Systems Biosciences and Computational Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
- Cholangiocarcinoma Research Institute, Khon Kaen University, Khon Kaen, Thailand
- Khon Kaen University Phenome Centre, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
| | - Jutarop Phetcharaburanin
- Department of Systems Biosciences and Computational Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
- Cholangiocarcinoma Research Institute, Khon Kaen University, Khon Kaen, Thailand.
- Khon Kaen University Phenome Centre, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
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Walther M, Müller H, Weiß C, Carbon R, Diez S, Besendörfer M. A comparative observational study on intrasphincteric injections with Botulinum toxin vs. enteral neuromodulation in chronic refractory constipation. BMC Pediatr 2023; 23:448. [PMID: 37684559 PMCID: PMC10486061 DOI: 10.1186/s12887-023-04217-5] [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: 05/07/2023] [Accepted: 07/27/2023] [Indexed: 09/10/2023] Open
Abstract
PURPOSE Botulinum toxin injections in the anal sphincter apparatus (Botox) and enteral neuromodulation (ENM) are options for treatment of refractory chronic constipation. We present a retrospective comparative observational study. PATIENTS AND METHODS From 2014 to 2022, pediatric patients with chronic constipation were either treated with Botox or ENM with continuation of conservative treatment. Comparison was conducted regarding the primary outcome variables defecation frequency, stool consistency, and abdominal pain. Secondary outcomes were fecal incontinence, enuresis, change of medication and safety of treatment. RESULTS 19 Botox patients (10 boys, 9 girls, 12 patients with Hirschsprung disease (HD), 7 patients with functional constipation (FC)) were compared to 24 ENM patients (18 boys, 6 girls, 12 HD patients, 7 FC patients). Groups differed significantly in age (5.0 years (Botulinum toxin) and 6.5 years (ENM), mean values, p-value 0.008). Improvement of constipation was seen in 68% (n = 13/19) of Botox and 88% (n = 21/24) of ENM patients (p = 0.153). Influence of etiology on therapeutic effects was not observed. Complications were minor. CONCLUSIONS Botox and ENM can be considered as valuable and effective treatment options in refractory chronic constipation. Prospective, large-population studies should be designed to enable improved evidence.
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Affiliation(s)
- Mona Walther
- Department of Surgery, Section of Pediatric Surgery, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), University Hospital Erlangen, Loschgestrasse 15, 91054, Erlangen, Germany
| | - Hanna Müller
- Neonatology and Pediatric Intensive Care, Department of Pediatrics, University Hospital Marburg, 35043, Baldingerstraße, Marburg, Germany
| | - Christel Weiß
- Department of Medical Statistics, Biomathematics, and Information Processing, Medical Faculty, Mannheim of Heidelberg University, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany
| | - Roman Carbon
- Department of Surgery, Section of Pediatric Surgery, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), University Hospital Erlangen, Loschgestrasse 15, 91054, Erlangen, Germany
| | - Sonja Diez
- Department of Surgery, Section of Pediatric Surgery, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), University Hospital Erlangen, Loschgestrasse 15, 91054, Erlangen, Germany.
| | - Manuel Besendörfer
- Department of Surgery, Section of Pediatric Surgery, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), University Hospital Erlangen, Loschgestrasse 15, 91054, Erlangen, Germany
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Bokova E, Prasade N, Janumpally S, Rosen JM, Lim IIP, Levitt MA, Rentea RM. State of the Art Bowel Management for Pediatric Colorectal Problems: Hirschsprung Disease. CHILDREN (BASEL, SWITZERLAND) 2023; 10:1418. [PMID: 37628417 PMCID: PMC10453740 DOI: 10.3390/children10081418] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/30/2023] [Revised: 08/12/2023] [Accepted: 08/17/2023] [Indexed: 08/27/2023]
Abstract
After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with increased age. Children with a patulous anus due to iatrogenic damage of the anal sphincters are offered sphincter reconstruction. Hypermotility is managed with antidiarrheals and small-volume enemas. Family education is crucial for the early detection of HAEC and for performing at-home rectal irrigations.
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Affiliation(s)
- Elizaveta Bokova
- Comprehensive Colorectal Center, Department of Surgery, Children’s Mercy Kansas City, Kansas City, MO 64108, USA
| | - Ninad Prasade
- Comprehensive Colorectal Center, Department of Surgery, Children’s Mercy Kansas City, Kansas City, MO 64108, USA
| | - Sanjana Janumpally
- Comprehensive Colorectal Center, Department of Surgery, Children’s Mercy Kansas City, Kansas City, MO 64108, USA
| | - John M. Rosen
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children’s Mercy Kansas City, Kansas City, MO 64108, USA
- Department of Pediatrics, University of Missouri-Kansas City, Kansas City, MO 64108, USA
| | - Irene Isabel P. Lim
- Comprehensive Colorectal Center, Department of Surgery, Children’s Mercy Kansas City, Kansas City, MO 64108, USA
- Department of Surgery, University of Missouri-Kansas City, Kansas City, MO 64108, USA
| | - Marc A. Levitt
- Division of Colorectal and Pelvic Reconstruction, Children’s National Medical Center, Washington, DC 20001, USA
| | - Rebecca M. Rentea
- Comprehensive Colorectal Center, Department of Surgery, Children’s Mercy Kansas City, Kansas City, MO 64108, USA
- Department of Surgery, University of Missouri-Kansas City, Kansas City, MO 64108, USA
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Gershon EM, Rodriguez L, Arbizu RA. Hirschsprung's disease associated enterocolitis: A comprehensive review. World J Clin Pediatr 2023; 12:68-76. [PMID: 37342453 PMCID: PMC10278080 DOI: 10.5409/wjcp.v12.i3.68] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2022] [Revised: 02/09/2023] [Accepted: 03/21/2023] [Indexed: 06/08/2023] Open
Abstract
Hirschsprung's disease (HSCR) is a congenital disorder characterized by failure of the neural crest cells to migrate and populate the distal bowel during gestation affecting different lengths of intestine leading to a distal functional obstruction. Surgical treatment is needed to correct HSCR once the diagnosis is confirmed by demonstrating the absence of ganglion cells or aganglionosis of the affected bowel segment. Hirschsprung's disease associated enterocolitis (HAEC) is an inflammatory complication associated with HSCR that can present either in the pre- or postoperative period and associated with increased morbidity and mortality. The pathogenesis of HAEC remains poorly understood, but intestinal dysmotility, dysbiosis and impaired mucosal defense and intestinal barrier function appear to play a significant role. There is no clear definition for HAEC, but the diagnosis is primarily clinical, and treatment is guided based on severity. Here, we aim to provide a comprehensive review of the clinical presentation, etiology, pathophysiology, and current therapeutic options for HAEC.
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Affiliation(s)
- Eric M Gershon
- Section of Pediatric Gastroenterology, Yale University School of Medicine, New Haven, CT 06520, United States
| | - Leonel Rodriguez
- Section of Pediatric Gastroenterology, Yale University School of Medicine, New Haven, CT 06520, United States
| | - Ricardo A Arbizu
- Section of Pediatric Gastroenterology, Yale University School of Medicine, New Haven, CT 06520, United States
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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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Ambartsumyan L, Patel D, Kapavarapu P, Medina-Centeno RA, El-Chammas K, Khlevner J, Levitt M, Darbari A. Evaluation and Management of Postsurgical Patient With Hirschsprung Disease Neurogastroenterology & Motility Committee: Position Paper of North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN). J Pediatr Gastroenterol Nutr 2023; 76:533-546. [PMID: 36720091 DOI: 10.1097/mpg.0000000000003717] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Children with Hirschsprung disease have postoperative long-term sequelae in defecation that contribute to morbidity and mortality and significantly impact their quality of life. Pediatric patients experience ongoing long-term defecation concerns, which can include fecal incontinence (FI) and postoperative obstructive symptoms, such as constipation and Hirschsprung-associated enterocolitis. The American Pediatric Surgical Association has developed guidelines for management of these postoperative obstructive symptoms and FI. However, the evaluation and management of patients with postoperative defecation problems varies among different pediatric gastroenterology centers. This position paper from the Neurogastroenterology & Motility Committee of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition reviews the current evidence and provides suggestions for the evaluation and management of postoperative patients with Hirschsprung disease who present with persistent defecation problems.
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Affiliation(s)
- Lusine Ambartsumyan
- From the Division of Gastroenterology and Nutrition, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA
| | - Dhiren Patel
- the Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cardinal Glennon Children's Medical Center, Saint Louis University School of Medicine, St Louis, MO
| | - Prasanna Kapavarapu
- the Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Ricardo A Medina-Centeno
- the Division of Gastroenterology, Hepatology and Nutrition, Phoenix Children's, College of Medicine, University of Arizona, Tucson, AZ
| | - Khalil El-Chammas
- the Division of Gastroenterology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH
| | - Julie Khlevner
- the Division of Gastroenterology, Hepatology and Nutrition, Columbia University Vagelos College of Physicians and Surgeons, New York, NY
| | - Marc Levitt
- the Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, Washington, DC
| | - Anil Darbari
- the Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, Washington, DC
- the Division of Gastroenterology and Nutrition, Children's National Hospital, Washington, DC
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10
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de Geus A, Koppen IJN, Flint RB, Benninga MA, Tabbers MM. An Update of Pharmacological Management in Children with Functional Constipation. Paediatr Drugs 2023; 25:343-358. [PMID: 36941393 PMCID: PMC10097737 DOI: 10.1007/s40272-023-00563-0] [Citation(s) in RCA: 14] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 02/19/2023] [Indexed: 03/23/2023]
Abstract
Functional constipation is a common problem in childhood worldwide and has a great impact on social, physical, and emotional functioning of affected children and their caregivers. It is a clinical diagnosis based on the Rome IV criteria. Non-pharmacological treatment involves education, demystification, lifestyle advice, and toilet training. Pharmacological treatment consists of disimpaction, maintenance treatment, and eventually weaning if possible. Polyethylene glycol is considered as the first choice of laxative for both disimpaction and maintenance treatment. Different osmotic laxatives, stimulant laxatives, lubricants, and enemas are available as alternative pharmacological treatment options. Novel drugs are emerging but evidence to support the widespread application of these drugs in the pediatric population is often lacking and more high-quality research is needed in this field. If children remain symptomatic despite optimal pharmacological treatment, botulinum toxin injections in the anal sphincter can be considered as an alternative, more invasive treatment option. This review provides an update on currently available literature concerning the pharmacologic treatment of functional constipation in children.
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Affiliation(s)
- Anna de Geus
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital/Academic Medical Center, Meibergdreef 9, 1105, Amsterdam, AZ, The Netherlands
| | - Ilan J N Koppen
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital/Academic Medical Center, Meibergdreef 9, 1105, Amsterdam, AZ, The Netherlands
| | - Robert B Flint
- Department of Clinical Pharmacy, Erasmus MC University Medical Center, Rotterdam, The Netherlands
- Division of Neonatology, Department of Paediatrics, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands
| | - Marc A Benninga
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital/Academic Medical Center, Meibergdreef 9, 1105, Amsterdam, AZ, The Netherlands
| | - Merit M Tabbers
- Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital/Academic Medical Center, Meibergdreef 9, 1105, Amsterdam, AZ, The Netherlands.
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Tan YW, Chacon CS, Geoghegan N, Saxena A, Clarke S, Haddad M, Choudhry M. Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes. Eur J Pediatr Surg 2022; 32:512-520. [PMID: 35263774 DOI: 10.1055/s-0042-1744147] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
INTRODUCTION Late diagnosis of Hirschsprung's disease (LDHD) may carry a poor prognosis. Its definition remains unclear and its implication on HD-related core outcomes has not been fully reported. METHODS A single-center 20-year series was reviewed to include HD with follow-up of 1 year or more post pull-through (PT) and aged 5 years or older. We investigated six core outcomes derived from NETS1HD study by comparing the groups dichotomized by four time points using age at diagnosis (44-week gestation, 6 months, 1 year, and 3 years). Following establishment of definition of LDHD, the outcomes and complications were compared with timely diagnosis of HD (TDHD). RESULTS Forty-nine out of eighty-six HD were included. The definition of LDHD was found to be HD diagnosed at 1 year of age or later because 3/6 core outcomes were significantly worse than TDHD. Nine patients (18%) had LDHD-median age at diagnosis 42 months (12-89) and PT performed at 57 months (12-103), mostly Soave (73%); a covering stoma was performed in 7/9, significantly higher than TDHD in 10/40 (p = 0.001). LDHD was associated with increased unplanned surgery (78% vs. 30%, p = 0.019), fecal incontinence (100% vs. 62%, p = 0.01), and permanent stoma (33% vs. 5%, p = 0.037). Major complications (56% vs. 20%, p = 0.043) and redo PT (33% vs. 5%, p = 0.037) were also higher in LDHD. CONCLUSION LDHD could be defined as HD diagnosis at or over 1 year of age. LDHD was associated with more preoperative stoma, major postoperative complications, unplanned reoperation, and worse HD-related core outcomes.
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Affiliation(s)
- Yew-Wei Tan
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom
| | - Carmen Sofia Chacon
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom
| | - Niamh Geoghegan
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom
| | - Amulya Saxena
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom.,Department of Paediatric Surgery, Imperial College London, London, United Kingdom
| | - Simon Clarke
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom.,Department of Paediatric Surgery, Imperial College London, London, United Kingdom
| | - Munther Haddad
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom.,Department of Paediatric Surgery, Imperial College London, London, United Kingdom
| | - Muhammad Choudhry
- Department of Paediatric Surgery, Chelsea and Westminster Hospital, Chelsea, London, United Kingdom
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12
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Xie C, Yan J, Zhang Z, Kai W, Wang Z, Chen Y. Risk factors for Hirschsprung-associated enterocolitis following Soave: a retrospective study over a decade. BMC Pediatr 2022; 22:654. [PMID: 36357849 PMCID: PMC9647981 DOI: 10.1186/s12887-022-03692-6] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/23/2022] [Accepted: 10/20/2022] [Indexed: 11/11/2022] Open
Abstract
Background Hirschsprung-associated enterocolitis (HAEC), one of the most significant causes of morbidity and mortality for patients with Hirschsprung disease (HSCR), can occur before and after radical surgery. This study aims to identify the risk factors for HAEC before and after Soave. Methods A retrospective study of 145 patients with HSCR treated by transanal or combination with laparoscopic or laparotomy Soave procedure between January 2011 and June 2021 was performed. Data were retrieved from the medical records. HAEC was defined as the presence of clinical signs of bowel inflammation and requiring treatment with intravenous antibiotics and rectal irrigation for at least two days in the outpatient or inpatient department. Univariate analysis and multivariate regression models were used to identify risk factors for developing pre-and postoperative HAEC. Results The incidence of pre-and postoperative HAEC was 24.1% and 20.7%, respectively. More than 90% of the patients with the first episode of postoperative HAEC occurred within the first year after Soave. Long-segment aganglionosis was the independent risk factor for developing preoperative HAEC ([OR] 5.8, Cl 2.4–14.2, p < 0.001), while the history of preoperative HAEC was significantly associated with developing postoperative HAEC ([OR] 4.2, Cl 1.6–10.8, p = 0.003). Conclusions Long-segment aganglionosis was the independent risk factor for the development of preoperative HAEC, and the history of preoperative HAEC was strongly associated with developing HAEC after Soave. Level of Evidence Level III
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13
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Tian L, Ma C, Deng Z, Zhu T, Zhao X, He Y, Wei M, Feng J, Yu D. Treatment of postoperative intestinal dysfunction of hirschsprung's disease based on the principle of "anorectal balance". Front Surg 2022; 9:996455. [PMID: 36386515 PMCID: PMC9649898 DOI: 10.3389/fsurg.2022.996455] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2022] [Accepted: 10/06/2022] [Indexed: 09/14/2023] Open
Abstract
PURPOSE Radical surgery is the most effective treatment for Hirschsprung's disease. However, some children still have symptoms of intestinal dysfunction such as constipation, abdominal distension, and recurrent enterocolitis after operation. The purpose of this study was to evaluate treatment outcomes of postoperative intestinal dysfunction in children with Hirschsprung's disease by using the principle of "anorectal balance". METHODS The clinical data of postoperative intestinal dysfunction in children with Hirschsprung's disease in the single treatment group from July 2019 to July 2021 were retrospectively analyzed. All the enrolled children underwent botulinum toxin injection (2.5 U/kg); 3 to 6 months later, the injection was performed again; the children who had received more than two botulinum toxin injections underwent the internal sphincter myectomy. Anorectal manometry was performed routinely after operation, and abdominal distension and defecation were recorded. RESULTS A total of thirty children with postoperative intestinal dysfunction underwent radical surgery for Hirschsprung's disease were included in this study. Symptoms of constipation, abdominal distension and enterocolitis were improved after botulinum toxin injections in most children compared to before surgery (P < 0.01). After re-injection of botulinum toxin in twelve children, the frequency of defecation increased, the anal resting pressure decreased, and the clinical symptoms were relieved again (P < 0.05). Eleven children underwent internal sphincter myectomy, and the symptoms of constipation, abdominal distension and enterocolitis were significantly improved after the operation (P < 0.01). CONCLUSION Botulinum toxin injection and internal sphincter myectomy based on the principle of "anorectal balance" can effectively reduce the resting pressure of the anus and relieve intestinal dysfunction, and have satisfactory clinical effect.
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Affiliation(s)
- Li Tian
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Chensen Ma
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Zhengdong Deng
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Tianqi Zhu
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Xiang Zhao
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Ying He
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Mingfa Wei
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Jiexiong Feng
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
| | - Donghai Yu
- Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Hubei Clinical Center of Hirschsprung's Disease and Allied Disorders, Wuhan, China
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14
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Ahmad H, Rentea RM, Knaus ME, Wilbur JG, Halaweish I, Langer JC, Levitt MA, Wood RJ. Routine botulinum toxin injection one month after a Swenson pull-through does not change the incidence of Hirschsprung associated enterocolitis. J Pediatr Surg 2022; 57:1453-1457. [PMID: 34991869 DOI: 10.1016/j.jpedsurg.2021.11.029] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2021] [Revised: 10/21/2021] [Accepted: 11/11/2021] [Indexed: 12/01/2022]
Abstract
PURPOSE Patients with Hirschsprung disease (HD) are at risk of Hirschsprung associated enterocolitis (HAEC) following pull-through. The purpose of this study was to determine if routine Botulinum toxin (BT) injected one-month post pull-through decreases the incidence of HAEC. METHODS We reviewed patients who underwent a primary (not redo) pull-through operation for HD between April 2014 to December 2019. Over the most recent 18 months, BT was administered routinely one-month post-pull-through procedure; these patients were compared to the prior group that did not receive routine BT. A HAEC episode was defined as one that required initiation of treatment for obstructive symptoms in the inpatient or outpatient setting with antibiotics and irrigations. Categorical variables were compared using the nonparametric chi-square test or Fisher's exact test. Continuous variables were compared using the two-tailed Student's t-test. P-value <0.05 was determined to be statistically significant. RESULTS A total of 70 patients underwent Swenson pull-through during the study period (52% male). There were no statistically significant differences in demographics in the BT vs. non-BT group. Routine post-pull-through BT was given in 28 patients and did not significantly change HAEC incidence compared to the non-BT group (12/28, 43% vs. 16/42, 38%. P = 0.691). Of note, the BT group patients developed HAEC significantly sooner than the patients in the non-BT group (37.5 days vs. 253 days, p = 0.029). More patients in the BT group (n = 18, 64%) required at least one subsequent BT injection compared to the patients in the non-BT group (n = 11, 26%. P = 0.001). CONCLUSIONS We conclude that routine postoperative botulinum toxin injection given one month postoperatively from Swenson pull-through did not change the incidence of HAEC. A prospective controlled study is necessary to confirm these findings.
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Affiliation(s)
- Hira Ahmad
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Rebecca M Rentea
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Maria E Knaus
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Jameson G Wilbur
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Ihab Halaweish
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Jacob C Langer
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Marc A Levitt
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205
| | - Richard J Wood
- Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, 611 E. Livingston Ave., Columbus, Ohio, 43205.
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15
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Baaleman DF, Hallagan A, Halleran DR, Orsagh-Yentis DK, Levitt MA, Wood RJ, Benninga MA, Bali N, Vaz KH, Yacob D, Di Lorenzo C, Lu PL. Anal Botulinum Toxin in Children with Hirschsprung Disease and Functional Constipation: A Prospective Cohort study. Eur J Pediatr Surg 2022; 33:241-248. [PMID: 35853466 DOI: 10.1055/s-0042-1751048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
INTRODUCTION Anal sphincter botulinum toxin injections (BTIs) are used in the treatment of children with severe defecation disorders, including Hirschsprung disease (HD) and functional constipation (FC). Our objective was to evaluate the outcomes of BTI in these children. MATERIALS AND METHODS We performed a prospective cohort study of children undergoing BTI from July 2018 to December 2018. We recorded perceived effect of the BTI, including effectiveness ranging from 0 (not at all effective) to 4 (extremely effective). In addition, we recorded symptoms and the Cleveland Clinic Constipation Score (CCCS). Data were collected at baseline and at 2 weeks, 2 months, and 4 months post-injection. RESULTS Forty-two children (HD = 25, FC = 17) were included in the study (median age 4.3 years, IQR 2.4-7.2, 52% male). Twenty-two (88%) children with HD and eight (47%) children with FC had previously undergone a BTI. BTIs were perceived effective in 16 (76%) and 12 (71%) children with HD and eight (47%) and seven (47%) children with FC at 2 weeks and 2 months follow-up, respectively. Effectiveness was not rated differently between groups except at the 2-month follow-up, when patients with HD rated the BTI more effective compared to those with FC (median 2 [HD] vs. median 1 [FC], p = 0.022). Over the course of the study, 17/39 (44%) children reported self-limiting adverse effects such as fecal incontinence and pain at the injection site. CONCLUSION Anal sphincter BTIs can be effective in the treatment of constipation in both HD and FC patients.
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Affiliation(s)
- Desiree F Baaleman
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States.,Department of Pediatric Gastroenterology, Amsterdam UMC, Emma Childrens' Hospital UMC, University of Amsterdam, Amsterdam, the Netherlands
| | - Alexandra Hallagan
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States.,College of Medicine, The Ohio State University, Columbus, Ohio, United States
| | - Devin R Halleran
- Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Danielle K Orsagh-Yentis
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Marc A Levitt
- Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Richard J Wood
- Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Marc A Benninga
- Department of Pediatric Gastroenterology, Amsterdam UMC, Emma Childrens' Hospital UMC, University of Amsterdam, Amsterdam, the Netherlands
| | - Neetu Bali
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Karla H Vaz
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Desale Yacob
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Carlo Di Lorenzo
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States
| | - Peter L Lu
- Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, United States
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16
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Svetanoff WJ, Lopez JJ, Briggs KB, Fraser JA, Fraser JD, Oyetunji TA, Peter SDS, Rentea RM. Management of Hirschsprung associated enterocolitis-How different are practice strategies? An international pediatric endosurgery group (IPEG) survey. J Pediatr Surg 2022; 57:1119-1126. [PMID: 35282932 DOI: 10.1016/j.jpedsurg.2022.01.036] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2022] [Accepted: 01/25/2022] [Indexed: 02/07/2023]
Abstract
INTRODUCTION Hirschsprung's-associated enterocolitis (HAEC) is a common post-operative problem for patients with Hirschsprung disease (HSCR). However, treatment strategies remain variable among providers, institutions, and even nations. The purpose of this study was to identify differences in treatment patterns for HAEC. METHODS A questionnaire was distributed to members of the International Pediatric Endoscopic Group (IPEG) community that focused on HSCR and HAEC management strategies. Questionnaire responses were collected via the Research Electronic Data Capture (RedCap). RESULTS 178 responses were obtained: 30% from North America, 20% South America, 20% Europe, 26% Asia, and 4% from Australia. 37% had a dedicated pediatric colorectal center. After diagnosis, 53% send patients home with irrigations, while 29% perform a primary PT before discharge; the type of PT varied between Soave (50%), Swenson (25%) and Duhamel (13%). Only 29 respondents (17%) stated their institution had guidelines for HAEC management; however, inpatient treatments were fairly consistent: 95% performed rectal irrigations, 93% obtained an abdominal radiograph, and 72% held feeds; 55% taught families irrigations before discharge. Utilization of Botulinum (BT) injections was mixed: 36% never utilized BT injections, 33% only used BT if irrigations were not tolerated, and 16% only injected BT for recurrent episodes. Preventative HAEC measures were also varied and included anal dilations (44%), prophylactic antibiotics (34%), probiotics (29%), and routine home irrigations (22%). CONCLUSION There is wide variation of care in managing enterocolitis episodes in patients with Hirschsprung disease. Further research leading to consensus guidelines and standardization practices can help improve the care for these patients. LEVEL OF EVIDENCE V TYPE OF STUDY: Treatment study/ survey.
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Affiliation(s)
- Wendy Jo Svetanoff
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States.
| | - Joseph J Lopez
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States
| | - Kayla B Briggs
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States
| | - James A Fraser
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States
| | - Jason D Fraser
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States; University of Missouri-Kansas City School of Medicine, Kansas City, MO, United States
| | - Tolulope A Oyetunji
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States; University of Missouri-Kansas City School of Medicine, Kansas City, MO, United States
| | - Shawn D St Peter
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States; University of Missouri-Kansas City School of Medicine, Kansas City, MO, United States
| | - Rebecca M Rentea
- Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States; University of Missouri-Kansas City School of Medicine, Kansas City, MO, United States
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17
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Characterizing the use of botulinum toxin in patients with Hirschsprung disease treated at referral institutions for pediatric colorectal surgery. J Pediatr Surg 2022; 57:1033-1039. [PMID: 35292167 DOI: 10.1016/j.jpedsurg.2022.02.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2022] [Accepted: 02/01/2022] [Indexed: 02/07/2023]
Abstract
PURPOSE Botulinum toxin (BT) is used to treat pediatric patients with Hirschsprung disease (HD) with obstructive symptoms. We aimed to characterize use of BT in HD patients across pediatric colorectal surgery referral centers. METHODS A multicenter retrospective study of BT use in children (0-18y) with HD was performed using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) between 2017 and 2021. Sites with <10 HD patients recorded were excluded. Patterns were evaluated using Fisher's exact, Wilcoxon rank-sum, Kruskal-Wallis, and Cochran-Armitage trend test. RESULTS 494 patients at 8 centers were included. 118 (23.9%) received at least one BT injection. Among patients who required redo pullthrough procedures, 53.1% received BT compared to 22.7% of patients who only underwent one pullthrough (p<0.001). Age at pullthrough was also significantly associated (p = 0.021). A lower proportion of Hispanic patients received BT (9.6% vs. 26.3%;p = 0.006). Percentage of HD patients receiving BT varied significantly across sites (p<0.001). CONCLUSIONS Use of BT in patients with HD varies widely with greater use in patients who underwent redo surgery and in those who underwent pullthrough at an older age. Hispanic patients received less BT. These findings highlight the need to develop consensus guidelines and for further study on timing of injections and potential disparities in care. LEVEL OF EVIDENCE III.
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18
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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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19
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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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20
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Arbizu R, Freiberg B, Rodriguez L. Lower Gastrointestinal Functional and Motility Disorders in Children. Pediatr Clin North Am 2021; 68:1255-1271. [PMID: 34736588 DOI: 10.1016/j.pcl.2021.07.010] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Functional and motility gastrointestinal disorders are the most common complaints to the pediatric gastroenterologist. Disorders affecting the small intestine carry a significant morbidity and mortality due to the severe limitation of therapeutic interventions available and the complications associated with such interventions. Congenital colorectal disorders are rare but also carry significant morbidity and poor quality of life plus the social stigma associated with its complications. In this review, we summarize the clinical presentation, diagnostic evaluations, and the therapeutic interventions available for the most common and severe gastrointestinal functional and motility disorders of the small bowel, colon, and anorectum.
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Affiliation(s)
- Ricardo Arbizu
- Section of Pediatric Gastroenterology, Hepatology and Nutrition, Pediatric Gastroenterology and Hepatology, Neurogastroenterology and Motility Center, Yale School of Medicine, Yale University School of Medicine, Yale New Haven Children's Hospital, 333 Cedar Street, LMP 4093, PO Box 208064, New Haven, CT 06520, USA
| | - Ben Freiberg
- Section of Pediatric Gastroenterology, Hepatology and Nutrition, Pediatric Gastroenterology and Hepatology, Neurogastroenterology and Motility Center, Yale School of Medicine, Yale University School of Medicine, Yale New Haven Children's Hospital, 333 Cedar Street, LMP 4093, PO Box 208064, New Haven, CT 06520, USA
| | - Leonel Rodriguez
- Section of Pediatric Gastroenterology, Hepatology and Nutrition, Pediatric Gastroenterology and Hepatology, Neurogastroenterology and Motility Center, Yale School of Medicine, Yale University School of Medicine, Yale New Haven Children's Hospital, 333 Cedar Street, LMP 4093, PO Box 208064, New Haven, CT 06520, USA.
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21
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Ahmad H, Levitt MA, Yacob D, Halleran DR, Gasior AC, Di Lorenzo C, Wood RJ, Langer JC. Evaluation and Management of Persistent Problems After Surgery for Hirschsprung Disease in a Child. Curr Gastroenterol Rep 2021; 23:18. [PMID: 34633517 DOI: 10.1007/s11894-021-00819-0] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/23/2021] [Indexed: 10/20/2022]
Abstract
PURPOSE OF REVIEW Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD. RECENT FINDINGS Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.
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Affiliation(s)
- Hira Ahmad
- Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Marc A Levitt
- Children's National Colorectal Center, Division of Colorectal and Pelvic Reconstructive Surgery, Children's National Hospital, Washington, District of Columbia, USA
| | - Desale Yacob
- Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA.,Division of Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Devin R Halleran
- Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Alessandra C Gasior
- Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA.,Department of Colorectal Surgery, The Ohio State University, Columbus, Ohio, USA
| | - Carlo Di Lorenzo
- Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA.,Division of Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Richard J Wood
- Department of Pediatric Colorectal and Pelvic Reconstruction Surgery, Nationwide Children's Hospital, Columbus, Ohio, USA
| | - Jacob C Langer
- Division of General and Thoracic Surgery, Hospital for Sick Children, Department of Surgery, University of Toronto, Toronto, Canada.
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22
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Svetanoff WJ, Lopez J, Aguayo P, Hendrickson RJ, Oyetunji TA, Rentea RM. The impact of botulinum injection for hospitalized children with Hirschsprung-associated enterocolitis. Pediatr Surg Int 2021; 37:1467-1472. [PMID: 34309717 DOI: 10.1007/s00383-021-04966-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 07/12/2021] [Indexed: 02/07/2023]
Abstract
INTRODUCTION Stasis from obstruction at the level of the internal anal sphincter (IAS) can lead to Hirschsprung-associated enterocolitis (HAEC) and may be improved by botulinum toxin (BT) injections. Our aim was to determine if BT injection during HAEC episodes decreased the number of recurrent HAEC episodes and/or increased the interval between readmissions. METHODS A retrospective review was performed of patients admitted for HAEC from January 2010 to December 2019. Demographics and outcomes of patients who received BT were compared to patients who did not receive BT during their hospital stay. RESULTS A total of 120 episodes of HAEC occurred in 40 patients; 30 patients (75%) were male, 7 (18%) had Trisomy 21 and 10 (25%) had long-segment disease. On multivariate analysis, patients who received BT during their inpatient HAEC episode had a longer median time between readmissions (p = 0.04) and trending toward an association with fewer readmissions prior to a follow-up clinic visit (p = 0.08). CONCLUSION The use of BT in HD patients hospitalized for HAEC is associated with an increased time between recurrent HAEC episodes and trended toward fewer recurrent episodes. The use of BT should be considered in the management of patients admitted with HAEC.
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Affiliation(s)
- Wendy Jo Svetanoff
- Department of Surgery, Comprehensive Colorectal Center, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA
| | - Joseph Lopez
- Department of Surgery, Comprehensive Colorectal Center, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA
| | - Pablo Aguayo
- Department of Surgery, Comprehensive Colorectal Center, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.,University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA
| | - Richard J Hendrickson
- Department of Surgery, Comprehensive Colorectal Center, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.,University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA
| | - Tolulope A Oyetunji
- Department of Surgery, Comprehensive Colorectal Center, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.,University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA
| | - Rebecca M Rentea
- Department of Surgery, Comprehensive Colorectal Center, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA. .,University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA.
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23
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Roorda D, Oosterlaan J, van Heurn E, Derikx JPM. Risk factors for enterocolitis in patients with Hirschsprung disease: A retrospective observational study. J Pediatr Surg 2021; 56:1791-1798. [PMID: 34078551 DOI: 10.1016/j.jpedsurg.2021.04.020] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/14/2020] [Revised: 04/10/2021] [Accepted: 04/18/2021] [Indexed: 11/17/2022]
Abstract
INTRODUCTION Hirschsprung-associated enterocolitis (HAEC) accounts for substantial morbidity and mortality in patients with Hirschsprung disease (HD). The aim of this study was to identify incidence of pre- and postoperative HAEC in our consecutive cohort and to identify patient and clinical characteristics that are associated with developing postoperative HAEC and HAEC-free interval. MATERIAL AND METHODS A retrospective cohort study was performed with all 146 HD patients treated between 2000 and 2017. Data were retrieved from the medical records. HAEC was defined as presence of clinical signs of bowel inflammation, that required treatment with intravenous antibiotics and admittance to the hospital during at least two days. To identify risk factor for HAEC, patients with and without a history of postoperative HAEC were compared. Kaplan-Meier and Cox-regression were used to assess HAEC free intervals before and after surgery. RESULTS Out of 146 patients, 12 patients had pre-operative HAEC (8%) and 31 patients had postoperative HAEC (21%). Median preoperative HAEC free interval was 112 days (IQR 182 days). Length of hospital stay due to readmissions was longer for patients with a history of postoperative HAEC compared to patients without a history of postoperative HAEC (9.5 vs 16 days, U = 1872.5, p = 0.047). Median postoperative HAEC free interval was 226 days. Of the patients who had postoperative HAEC, 66% had their first episode within the first year after surgery and that the incidence of HAEC declined over follow-up. CONCLUSIONS HAEC incidence was relatively low in our population. No patient or clinical characteristics were associated with the risk of postoperative HAEC.
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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, The Netherlands; Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Follow Me Follow-Up Program, Department of Pediatrics, Amsterdam Reproduction and Development Research Institute, Amsterdam, The Netherlands.
| | - Jaap Oosterlaan
- Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Follow Me Follow-Up Program, Department of Pediatrics, Amsterdam Reproduction and Development Research Institute, Amsterdam, The 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, The Netherlands
| | - Joep P M 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, The Netherlands
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24
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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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25
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Chanpong A, Borrelli O, Thapar N. Hirschsprung disease and Paediatric Intestinal Pseudo-obstruction. Best Pract Res Clin Gastroenterol 2021; 56-57:101765. [PMID: 35331399 DOI: 10.1016/j.bpg.2021.101765] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2021] [Revised: 09/13/2021] [Accepted: 09/14/2021] [Indexed: 01/31/2023]
Abstract
Hirschsprung disease (HSCR) and Paediatric Intestinal Pseudo-obstruction (PIPO) comprise two of the most recognized and severe disorders of gastrointestinal (GI) motility. HSCR is a developmental disorder of the enteric nervous system invariably affecting the large intestine, whereas the majority of PIPO conditions represent congenital disorders of one or more components of the neuromusculature and more diffusely affect the GI tract. Histopathology is deemed the gold standard for the diagnosis of HSCR and, arguably, of PIPO, but, other diagnostic modalities such as manometric and genetic studies have seen recent advances that may increase their utility. Especially for PIPO, management is multidisciplinary and best performed in specialist referral centres. Surgery remains the only viable treatment for HSCR and appears essential to optimize and sustain feeding and viability of intestinal function in PIPO patients. Novel therapies such as neural stem cell transplants show promise for the future.
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Affiliation(s)
- Atchariya Chanpong
- Neurogastroenterology & Motility Unit, Gastroenterology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, United Kingdom; Division of Gastroenterology and Hepatology, Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Songkhla, 90110, Thailand; Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, WC1N 1EH, United Kingdom
| | - Osvaldo Borrelli
- Neurogastroenterology & Motility Unit, Gastroenterology Department, Great Ormond Street Hospital for Children, London, WC1N 3JH, United Kingdom
| | - Nikhil Thapar
- Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, WC1N 1EH, United Kingdom; Gastroenterology, Hepatology and Liver Transplant, Queensland Children's Hospital, Brisbane, Queensland, 4101, Australia.
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26
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Demehri FR, Dickie BH. Reoperative techniques and management in Hirschsprung disease: a narrative review. Transl Gastroenterol Hepatol 2021; 6:42. [PMID: 34423163 DOI: 10.21037/tgh-20-224] [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: 05/18/2020] [Accepted: 08/12/2020] [Indexed: 11/06/2022] Open
Abstract
The majority of children who undergo operative management for Hirschsprung disease have favorable results. A subset of patients, however, have long-term dysfunctional stooling, characterized by either frequent soiling or obstructive symptoms. The evaluation and management of a child with poor function after pull-through for Hirschsprung disease should be conducted by an experienced multidisciplinary team. A systematic workup is focused on detecting pathologic and anatomic causes of pull-through dysfunction. This includes an exam under anesthesia, pathologic confirmation including a repeat biopsy, and a contrast enema, with additional studies depending on the suspected etiology. Obstructive symptoms may be due to technique-specific types of mechanical obstruction, histopathologic obstruction, or dysmotility-each of which may benefit from reoperative surgery. The causes of soiling symptoms include loss of the dentate line and damage to the anal sphincter, which generally do not benefit from revision of the pull-through, and pseudo-incontinence, which may reveal underlying obstruction. A thorough understanding of the types of complications associated with various pull-through techniques aids in the evaluation of a child with postoperative dysfunction. Treatment is specifically tailored to the patient, guided by the etiology of the patient's symptoms, with options ranging from bowel management to redo pull-through procedure. This review details the workup and management of patients with complications after pull-through, with a focus on the perioperative management and technical considerations for those who require reoperation.
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Affiliation(s)
- Farokh R Demehri
- Department of Surgery, Boston Children's Hospital, Boston, MA, USA
| | - Belinda H Dickie
- Department of Surgery, Boston Children's Hospital, Boston, MA, USA
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27
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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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28
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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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29
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Inpatient management of Hirschsprung's associated enterocolitis treatment: the benefits of standardized care. Pediatr Surg Int 2020; 36:1413-1421. [PMID: 33001257 DOI: 10.1007/s00383-020-04747-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/16/2020] [Indexed: 12/15/2022]
Abstract
INTRODUCTION Patients with Hirschsprung's disease (HSCR) remain at risk of developing Hirschsprung-associated enterocolitis (HAEC) after surgical intervention. As inpatient management remains variable, our institution implemented an algorithm directed at standardizing treatment practices. This study aimed to compare the outcomes of patients pre- and post-algorithm. METHODS A retrospective review of patients admitted for HAEC was performed; January 2017-June 2018 encompassed the pre-implementation period, and October 2018-October 2019 was the post-implementation period. Demographics and outcomes were compared between the two groups. RESULTS Sixty-two episodes of HAEC occurred in 27 patients during the entire study period. Sixteen patients (59%) had more than one episode. The most common levels of the transition zone were the rectosigmoid (50%) and descending colon (27%). Following algorithm implementation, the median length of stay (2 vs. 7 days, p < 0.001), TPN duration (0 vs. 5.5 days, p < 0.001), and days to full enteral diet (6 days vs. 2 days, p < 0.001) decreased significantly. Readmission rates for recurrent enterocolitis were similar pre- and post-algorithm implementation. CONCLUSION The use of a standardized algorithm significantly decreases the length of stay and duration of intravenous antibiotic administration without increasing readmission rates, while still providing appropriate treatment for HAEC. LEVEL OF EVIDENCE III level. TYPE OF STUDY Retrospective comparative study.
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