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Davidson JR, Curry J. Obstacles to an Effective Transition to Adult Services for Patients with Hirschsprung Disease. CHILDREN (BASEL, SWITZERLAND) 2024; 11:1237. [PMID: 39457202 PMCID: PMC11506805 DOI: 10.3390/children11101237] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/21/2024] [Revised: 10/07/2024] [Accepted: 10/12/2024] [Indexed: 10/28/2024]
Abstract
A growing number of patients with Hirschsprung disease are reaching adulthood, of whom a significant minority will require ongoing input from healthcare providers. In order to ensure patients receive the best care possible, it is essential to transition patients appropriately to adult services. This article describes the unmet need and some of the obstacles to this process and explores potential solutions, drawing on model examples for transitional care.
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Affiliation(s)
- Joseph R. Davidson
- UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK
- Evelina London Children’s Hospital, London SE1 7EH, UK
| | - Joe Curry
- Great Ormond Street Hospital for Children, London WC1N 3JH, UK;
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Liu Z, Zhang Y, Li S, Zhao J, Yang T, Huang J. Long-term bowel function after single-stage transanal endorectal pull-through in neonatal patients with Hirschsprung disease. Pediatr Surg Int 2023; 39:255. [PMID: 37642732 DOI: 10.1007/s00383-023-05517-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 07/06/2023] [Indexed: 08/31/2023]
Abstract
BACKGROUND The aim of this study was to assess long-term outcomes of neonatal patients with Hirschsprung disease (HD) after single-stage transanal endorectal pull-through (TEPT) and to explore the predictive factors contributing to subnormal bowel function. METHODS Patients aged > 3 years operated for HD with TEPT during neonatal period between 2007 and 2019 answered the bowel function score (BFS) questionnaire. The patients were retrospectively divided into two groups according to whether they had normal bowel function. The clinical variables were compared between the subnormal and normal BFS groups. Univariate and multivariable logistic regression analysis were performed to identify the predictive factors contributing to subnormal bowel function. RESULTS A total of 160 children (71.7%) were included in this study, with mean follow-up time of 7.3 years (range 3.0-15.1 years). The level of aganglionosis were determined to be the short-segment (124/160, 77.5%), long-segment (33/160, 20.6%), and TCA (3/160, 1.9%). One hundred and thirty-four patients (83.8%) had a BFS ≥ 17, and 26 patients (16.2%) with subnormal bowel function (BFS < 17). Univariate and multivariate logistic regression analysis showed that level of aganglionosis with long-segment or TCA and postoperative hospital stay > 8.5 days were independent risk factors with OR of 3.213 (1.252, 8.246) and 3.813 (1.371, 10.606) for subnormal BFS, respectively. CONCLUSION Most HD patients who underwent one-stage TEPT in the neonatal period have favorable long-term results, and the level of aganglionosis with long-segment or TCA and long postoperative hospital stay may be closely related to subnormal bowel function.
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Affiliation(s)
- Zhaozhou Liu
- Department of Neonatal Surgery, Beijing Children Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Beijing, 100045, China
| | - Yanan Zhang
- Department of Neonatal Surgery, Beijing Children Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Beijing, 100045, China
| | - Shuangshuang Li
- Department of Neonatal Surgery, Beijing Children Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Beijing, 100045, China
| | - Jiawei Zhao
- Department of Neonatal Surgery, Beijing Children Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Beijing, 100045, China
| | - Ting Yang
- Department of Neonatal Surgery, Beijing Children Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Beijing, 100045, China
| | - Jinshi Huang
- Department of Neonatal Surgery, Beijing Children Hospital, Capital Medical University, National Center for Children's Health, 56 Nanlishi Road, Beijing, 100045, China.
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Zhang Z, Li Q, Li B, Alganabi M, Li L. Long-term Bowel function and pediatric health-related quality of life after transanal rectal mucosectomy and partial internal anal sphincterectomy pull-through for Hirschsprung Disease. Front Pediatr 2023; 11:1099606. [PMID: 36865694 PMCID: PMC9971990 DOI: 10.3389/fped.2023.1099606] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Accepted: 01/16/2023] [Indexed: 02/16/2023] Open
Abstract
OBJECTIVE The aim of this study was to define controlled outcomes for bowel function and quality of life (QoL) after transanal rectal mucosectomy and partial internal anal sphincterectomy pull-through (TRM-PIAS, A modified Swenson procedure) for Hirschsprung disease (HD). BACKGROUND We have previously shown that a novel modification of transanal rectal mucosectomy and partial internal anal sphincterectomy (TRM-PIAS, A modified procedure) for Hirschsprung disease have the advantage of low postoperative Hirschsprung associated enterocolitis. The controlled long-term follow-up studies evaluating Bowel Function Score (BFS) and Pediatric Quality of Life Inventory (PedsQoL, age <18 years) remain unclear. METHODS Between Jan 2006 and Jan 2016, 243 Patients underwent TRM-PIAS older than 4 years were included, while experienced redo surgery because of complication were excluded. Patients were compared with age- and gender-matched 244 healthy children each randomly selected from the 405 general population. The enrollee was investigated for questionnaires on BFS and PedsQoL. RESULTS One hundred and ninety-nine (81.9%) patients' representatives for the entire study population responded. The mean age of patients was 84.4 months (48-214 months). Compared with controls, patients reported impairment of hold back defecation, fecal soiling, and the urge to defecate (P < 0.05), and no significantly different in fecal accidents, constipation and social problems. With advancing age, the total BFS of HD patients improved, with a tendency close to the normal level beyond 10 years old. But, after grouped according to presence or absence of HAEC, the non-HAEC group experienced more dramatic improvement with age increasing. CONCLUSIONS Compared with matched peers, significant impairment of fecal control prevails after TRM-PIAS in HD patients, but bowel function improve with age and recovery faster than conventional procedure. It should be emphasized that post-enterocolitis is a high-risk factor for delayed recovery.
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Affiliation(s)
- Zhen Zhang
- Department of General Surgery, Capital Institute of Pediatrics, Beijing, China.,Translational Medicine Program, Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada
| | - Qi Li
- Department of General Surgery, Capital Institute of Pediatrics, Beijing, China
| | - Bo Li
- Translational Medicine Program, Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada
| | - Mashriq Alganabi
- Translational Medicine Program, Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada
| | - Long Li
- Department of General Surgery, Capital Institute of Pediatrics, Beijing, China
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Rintala RJ. Long-term outcomes in newborn surgery. Pediatr Surg Int 2022; 39:57. [PMID: 36542182 DOI: 10.1007/s00383-022-05325-6] [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] [Accepted: 11/24/2022] [Indexed: 12/24/2022]
Abstract
This article describes the common methods to study long-term outcomes in patients who have undergone major surgery in newborn period. It also sums up today's knowledge on the long-term outcome of some classic newborn surgical conditions. The analysis of long-term outcomes is important to pediatric surgeons. Knowledge of long-term outcome can guide the patient's management and principles of the follow-up throughout the patient's childhood. It also aims to give the parents of the patient a realistic picture on the development of their child. Recent data have shown that many patients who have undergone major surgery during early childhood have significant functional aberrations at adult age. Some of these have a profound influence on the quality of life of these patients.
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Affiliation(s)
- Risto J Rintala
- Department of Pediatric Surgery, Children's Hospital, Helsinki University Central Hospital, Helsinki, Finland.
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Inpatient Care Utilization and Epidemiology of Hirschsprung Disease: Analysis of the National Inpatient Sample. J Pediatr Gastroenterol Nutr 2022; 75:3-9. [PMID: 35622006 DOI: 10.1097/mpg.0000000000003449] [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: 12/10/2022]
Abstract
BACKGROUND/OBJECTIVES Hirschsprung disease (HD) is associated with significant morbidities including long-term bowel dysfunction. The aim of this study was to update national and regional trends in the inpatient care utilization and epidemiology of HD in the United States between 2009 and 2014 using the National Inpatient Sample (NIS) database. METHODS We identified all pediatric admissions with a diagnosis of HD within the NIS from 2009 through 2014. We analyzed HD discharges with respect to various demographic and clinical factors, specifically trends and group differences in inflation-adjusted cost of hospitalization, procedures, co-morbidities, hospital mortality, and length of stay (LOS). A modified Cochrane-Armitage trend test was used to analyze trends for dichotomous outcome variables, and regression analyses were conducted for continuous and binary variables. RESULTS National estimates of HD-discharges showed no significant trend between 2009 and 2014 ( P = 0.27), with estimated relative incidence ranging from 46 to 70 per 100,000 pediatric discharges. Inflation-adjusted cost of hospitalization increased by $1137 (SE $326) per year ( P = 0.0005). Pull-through procedures in neonatal age group increased from 33.0% in 2009 to 36.5% in 2014 ( P = 0.003). Hospital mortality has remained stable between 0.4% and 1.0% ( P = 0.598). LOS decreased by 0.23 days per year ( P = 0.036). CONCLUSION Increasing cost of HD-related hospitalization despite decreasing LOS was observed in this cohort. Stable rate of hospitalizations with increasing proportions of pull-through procedures among neonates was noted. Future studies and development of protocols to standardize patient care could improve outcomes and healthcare spending.
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Verkuijl SJ, Meinds RJ, van der Steeg AF, van Gemert WG, de Blaauw I, Witvliet MJ, Sloots CE, van Heurn E, Vermeulen KM, Trzpis M, Broens PM. Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung Disease. J Pediatr Gastroenterol Nutr 2022; 74:348-354. [PMID: 34775429 PMCID: PMC8860201 DOI: 10.1097/mpg.0000000000003355] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2021] [Accepted: 10/10/2021] [Indexed: 12/14/2022]
Abstract
OBJECTIVES Knowledge on long-term outcomes in patients with Hirschsprung disease is progressing. Nevertheless, differences in outcomes according to aganglionic lengths are unclear. We compared long-term bowel function and generic quality of life in Hirschsprung patients with total colonic or long-segment versus rectosigmoid aganglionosis. METHODS In this nationwide, cross-sectional study participants with proven Hirschsprung disease received the Defecation and Fecal Continence questionnaire, and the Child Health Questionnaire Child Form-87, or the WHO Quality of Life-100. We excluded deceased patients, patients who were younger than 8 years, lived abroad, had a permanent enterostomy, or were intellectually impaired. RESULTS The study population (n = 334) was operated for rectosigmoid (83.9%), long-segment (8.7%), or total colonic aganglionosis (7.5%). Fecal incontinence in general was not significantly different between the three groups, but liquid fecal incontinence was significantly associated with total colonic aganglionosis (odds ratio [OR] = 6.00, 95% confidence interval [CI] 2.07-17.38, P = 0.001). Regarding constipation, patients with total colonic or long-segment aganglionosis were less likely to suffer from constipation than the rectosigmoid group (OR = 0.21, 95% CI, 0.05-0.91, P = 0.038 and OR = 0.11, 95% CI, 0.01-0.83, P = 0.032). Quality of life was comparable between the three groups, except for a lower physical score in children with total colonic aganglionosis (P = 0.016). CONCLUSIONS Over time Hirschsprung patients with total colonic or long-segment aganglionosis do not suffer from worse fecal incontinence in general. A difference in stool consistency may underlie the association between liquid fecal incontinence and total colonic aganglionosis and constipation in patients with rectosigmoid aganglionosis. Despite these differences, generic quality of life is comparable on reaching adulthood.
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Affiliation(s)
- Sanne J. Verkuijl
- Department of Surgery, Division of Pediatric Surgery
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen
| | - Rob J. Meinds
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen
- Department of Gastroenterology and Hepatology, Medisch Spectrum Twente, Enschede
| | | | - Wim G. van Gemert
- Department of Pediatric Surgery, University Medical Centre Maastricht, University of Maastricht, Maastricht
| | - Ivo de Blaauw
- Department of Surgery, Division of Pediatric Surgery, Radboudumc–Amalia Children's Hospital, Nijmegen
| | - Marieke J. Witvliet
- Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht
| | - Cornelius E.J. Sloots
- Department of Pediatric Surgery, Erasmus Medical Centre, Sophia Children's Hospital, Rotterdam
| | - Ernst van Heurn
- Department of Pediatric Surgery, Emma Children's Hospital, Academic Medical Centre and VU University Medical Centre, Amsterdam
| | - Karin M. Vermeulen
- Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
| | - Monika Trzpis
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen
| | - Paul M.A. Broens
- Department of Surgery, Division of Pediatric Surgery
- Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen
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Davidson JR, Kyrklund K, Eaton S, Pakarinen MP, Thompson DS, Cross K, Blackburn SC, De Coppi P, Curry J. Long-term surgical and patient-reported outcomes of Hirschsprung Disease. J Pediatr Surg 2021; 56:1502-1511. [PMID: 33706942 DOI: 10.1016/j.jpedsurg.2021.01.043] [Citation(s) in RCA: 43] [Impact Index Per Article: 10.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/13/2020] [Revised: 01/07/2021] [Accepted: 01/26/2021] [Indexed: 12/29/2022]
Abstract
BACKGROUND Information is needed regarding the complex relationships between long-term functional outcomes and health-related quality of life (HRQoL) in Hirschsprung's Disease (HSCR). We describe long-term outcomes across multiple domains, completing a core outcome set through to adulthood. METHODS HSCR patients operated at a single center over a 35-year period (1978-2013) were studied. Patients completed detailed questionnaires on bowel and urologic function, and HRQOL. Patients with learning disability (LD) were excluded. Outcomes were compared to normative data. Data are reported as median [IQR] or mean (SD). RESULTS 186 patients (median age 28 [18-32] years; 135 males) completed surveys. Bowel function was reduced (BFS 17 [14-19] vs. 19 [19-20], p < 0•0001;η2 = 0•22). Prevalence and severity of fecal soiling and fecal awareness improved with age (p < 0•05 for both). Urinary incontinence was more frequent than controls, most of all in 13-26y females (65% vs. 31%,p = 0•003). In adults, this correlated independently with constipation symptoms (OR 3.18 [1.4-7.5],p = 0.008). HRQoL outcomes strongly correlated with functional outcome: 42% of children demonstrated clinically significant reductions in overall PedsQL score, and poor bowel outcome was strongly associated with impaired QOL (B = 22•7 [12•7-32•7],p < 0•001). In adults, GIQLI scores were more often impacted in patients with extended segment disease. SF-36 scores were reduced relative to population level data in most domains, with large effect sizes noted for females in General Health (g = 1.19) and Social Wellbeing (g = 0.8). CONCLUSION Functional impairment is common after pull-through, but bowel function improves with age. Clustering of poor functional outcomes across multiple domains identifies a need for early recognition and long-term support for these patients.
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Affiliation(s)
- Joseph R Davidson
- Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK; Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, UK.
| | - Kristiina Kyrklund
- Department of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Finland
| | - Simon Eaton
- Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, UK
| | - Mikko P Pakarinen
- Department of Paediatric Surgery, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Finland
| | - David S Thompson
- Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK
| | - Kate Cross
- Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK
| | - Simon C Blackburn
- Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK
| | - Paolo De Coppi
- Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK; Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, UK
| | - Joe Curry
- Specialist Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK.
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Kastenberg ZJ, Taylor MA, Durham MM, Calkins CM, Rentea RM, Wood RJ, Avansino JR, Levitt MA, van Leeuwen KD, Lewis KE, Reeder RW, Rollins MD. Perioperative and long-term functional outcomes of neonatal versus delayed primary endorectal pull-through for children with Hirschsprung disease: A pediatric colorectal and pelvic learning consortium study. J Pediatr Surg 2021; 56:1465-1469. [PMID: 34052005 DOI: 10.1016/j.jpedsurg.2021.04.024] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/21/2021] [Accepted: 04/22/2021] [Indexed: 12/29/2022]
Abstract
BACKGROUND/PURPOSE the timing of endorectal pull-through for Hirschsprung disease (HD) is controversial. Neonatal primary endorectal pull-through theoretically prevents preoperative enterocolitis. Delayed primary endorectal pull-through offers the surgeon the benefit of more robust perineal anatomy and allows primary caregivers the time to emotionally process the diagnosis and to gain experience with rectal irrigations. We hypothesized that delayed primary endorectal pull-through would be associated with equivalent perioperative morbidity compared to the neonatal repair and would lead to improved long-term functional outcomes. METHODS we analyzed all patients in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry who had a primary endorectal pull-through for HD and at least three-and-one-half years of follow up in a specialty colorectal clinic. We evaluated patient demographics, operative outcomes, perioperative episodes of enterocolitis, and long-term functional outcomes for the neonatal (<31 days) and delayed (≥31 days) pull-through cohorts. RESULTS eighty-two patients were identified of whom 49 were operated upon in the neonatal period and 33 in a delayed fashion. The median age at operation was 11 days [IQR 7 - 19 days] for the neonatal cohort and 98 days [IQR 61 - 188 days] for the delayed cohort. Thirty-four (69.4%) of the neonatal and 22 (66.7%) of the delayed cohort patients had rectosigmoid transition zones. Four of 49 patients (8.1%) in the neonatal cohort were diagnosed with enterocolitis preoperatively compared to two of 33 (6.0%) in the delayed cohort (p = 0.89). Eighteen of 49 patients (36.7%) in the neonatal cohort and 16 of 33 (48.5%) in the delayed cohort had at least one postoperative episode of enterocolitis (p = 0.38). Fifteen of 49 patients (30.6%) in the neonatal cohort were receiving bowel management for fecal incontinence at most recent follow up compared to five of 33 (15.2%) in the delayed cohort (p = 0.13). CONCLUSION delayed primary endorectal pull-through offers a safe alternative to operation in the neonatal period and appears to have, at least, equivalent functional outcomes. LEVEL OF EVIDENCE Level III, retrospective comparative study.
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Affiliation(s)
- Zachary J Kastenberg
- Division of Pediatric Surgery, Department of Surgery, University of Utah School of Medicine, Primary Children's Hospital, 100N. Mario Cappecchi Dr., Suite 3800, Salt Lake City, UT 84113, United States.
| | - Mark A Taylor
- Division of Pediatric Surgery, Department of Surgery, University of Utah School of Medicine, Primary Children's Hospital, 100N. Mario Cappecchi Dr., Suite 3800, Salt Lake City, UT 84113, United States
| | - Megan M Durham
- Division of Pediatric Surgery, Department of Surgery, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, GA, United States
| | - Casey M Calkins
- Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI, United States
| | - Rebecca M Rentea
- Division of Pediatric Surgery, Department of Surgery, Children's Mercy Kansas City, Kansas City, MO, United States
| | - Richard J Wood
- Division of Pediatric Surgery, Department of Surgery, Ohio State College of Medicine, Nationwide Children's Hospital, Columbus, OH, United States
| | - Jeffrey R Avansino
- Division of Pediatric General and Thoracic Surgery, Department of Surgery, University of Washington School of Medicine, Seattle Children's, WA, United States
| | - Marc A Levitt
- Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, Washington DC, United States
| | | | - Katelyn E Lewis
- Division of Pediatric Critical Care, Department of Pediatrics, University of Utah School of Medicine, Primary Children's Hospital, Salt Lake City, UT, United States
| | - Ron W Reeder
- Division of Pediatric Critical Care, Department of Pediatrics, University of Utah School of Medicine, Primary Children's Hospital, Salt Lake City, UT, United States
| | - Michael D Rollins
- Division of Pediatric Surgery, Department of Surgery, University of Utah School of Medicine, Primary Children's Hospital, 100N. Mario Cappecchi Dr., Suite 3800, Salt Lake City, UT 84113, United States
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Apfeld JC, Wood RJ, Halleran DR, Deans KJ, Minneci PC, Cooper JN. Relationships Between Hospital and Surgeon Operative Volumes and Surgical Outcomes in Hirschsprung's Disease. J Surg Res 2021; 257:379-388. [DOI: 10.1016/j.jss.2020.08.014] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2020] [Revised: 07/14/2020] [Accepted: 08/02/2020] [Indexed: 12/20/2022]
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Dai Y, Deng Y, Lin Y, Ouyang R, Li L. Long-term outcomes and quality of life of patients with Hirschsprung disease: a systematic review and meta-analysis. BMC Gastroenterol 2020; 20:67. [PMID: 32164539 PMCID: PMC7066788 DOI: 10.1186/s12876-020-01208-z] [Citation(s) in RCA: 41] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2019] [Accepted: 02/25/2020] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Advances in surgical techniques and perioperative care have improved the short- and mid-term postoperative outcomes of patients with Hirschsprung disease (HD). However, the long-term outcomes of these patients (older than 10 years) have not been fully investigated. The aim of this systematic review is to clarify the prevalence of long-term outcomes and the quality of life of these patients. METHODS PubMed, AMED, Cochrane Library, CINAHL and PsycINFO databases were searched from inception to October 2018, following the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guideline. Original studies reporting the outcomes of patients older than ten years with HD were selected and reviewed. The overall prevalence of fecal incontinence, constipation, bowel function score, bladder dysfunction symptoms, and patients' quality of life were extracted from the included studies and pooled through the random-effects meta-analysis model. The heterogeneity and variation in the pooled estimations were evaluated by Cochrane's Q test and the I2 test. The sensitivity analysis was conducted by the sequential omission of individual studies. Publication bias was evaluated by Egger's linear regression test. The whole procedure was conducted with Stata (version 14). RESULTS In total, 3406 articles were identified from the literature search, among which twelve studies, including 625 patients, were included for analysis. The pooled prevalences of fecal incontinence, constipation, and bladder dysfunction symptoms and good to excellent bowel function scores were 0.20 (95% CI 0.13-0.28), 0.14 (95% CI 0.06-0.25), 0.07 (95% CI 0.04-0.12), and 0.95 (95% CI: 0.91-0.97), respectively; the pooled mean score of gastrointestinal-related quality of life was 118 (95% CI: 112.56-123.44). CONCLUSIONS HD patients older than ten years old have an overall high prevalence of fecal incontinence and a low quality of life. Targeted and evidence-based follow-up procedures and transitional care are essential to meet these patients' long-term care needs. Prospective and multicenter research that focuses on the attributes and predictors of the long-term prognosis of patients with HD are necessary.
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Affiliation(s)
- Ying Dai
- Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, No. 9 Jinsui Road, Guangzhou, Guangdong Province China
| | - Yongfang Deng
- Department of Obstetrics and Gynecology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Yan Lin
- Department of Nursing, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, China
| | - Runxian Ouyang
- Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, No. 9 Jinsui Road, Guangzhou, Guangdong Province China
| | - Le Li
- Department of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, No. 9 Jinsui Road, Guangzhou, Guangdong Province China
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Oh C, Youn JK, Han JW, Yang HB, Kim HY, Jung SE. The Patients with Hirschsprung’s Disease Who Underwent Pull-Through at Age Less than 1 Year: Longitudinal Bowel Function. World J Surg 2020; 44:2426-2439. [DOI: 10.1007/s00268-020-05474-6] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Functional outcome, quality of life, and 'failures' following pull-through surgery for hirschsprung's disease: A review of practice at a single-center. J Pediatr Surg 2020; 55:273-277. [PMID: 31759654 DOI: 10.1016/j.jpedsurg.2019.10.042] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/09/2019] [Accepted: 10/26/2019] [Indexed: 12/13/2022]
Abstract
AIMS The purpose of this study was to assess bowel function and quality of life (QoL) in patients with Hirschsprung's disease (HD) and identify patients who have 'failed' treatment. METHODS A review of a single-center HD cohort treated with pull-through surgery from 2004 to 2017 was completed. Bowel function of patients five years and above and QoL of all patients were assessed using validated questionnaires. Patients who 'failed' treatment were defined as above five years with one or more of: a) long-term stoma, b) needing an antegrade continence enema or transanal irrigation, c) severe soiling, or d) severe constipation. Statistical analysis was performed with P < 0.05 deemed significant. Data are given as mean [range]. RESULTS Seventy-one patients presented with HD within the study period. Mean follow-up was 5.4 years [0.7-13.3]. Of 38 eligible patients, bowel function was assessed in 24 patients (nine had a stoma, five lost to follow-up). The mean incontinence score was 17 [0-28)], and the mean constipation score was 17 [5-25]. Incontinence and constipation scores were worse than healthy controls (P < 0.001 and P = 0.001, respectively) and did not improve with age. Fifty-six patients had QoL assessed with no difference between our cohort (81 [25-100]) and healthy controls (81 [unknown]); (P = 0.85). Thirty-three patients were assessed for 'failure' (bowel function score n = 24; stoma n = 9). Thirty of 33 (91%) children older than five years can be considered to have 'failed' treatment. CONCLUSIONS Patients have worse bowel function than healthy children, which does not improve with age. QoL is comparable to healthy controls. A significant proportion of patients have poor outcomes and have 'failed' treatment. LEVEL OF EVIDENCE Level III.
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Schmitteckert S, Mederer T, Röth R, Günther P, Holland-Cunz S, Metzger M, Samstag Y, Schröder-Braunstein J, Wabnitz G, Kurzhals S, Scheuerer J, Beretta CA, Lasitschka F, Rappold GA, Romero P, Niesler B. Postnatal human enteric neurospheres show a remarkable molecular complexity. Neurogastroenterol Motil 2019; 31:e13674. [PMID: 31318473 DOI: 10.1111/nmo.13674] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/07/2019] [Revised: 06/26/2019] [Accepted: 06/26/2019] [Indexed: 01/30/2023]
Abstract
BACKGROUND The enteric nervous system (ENS), a complex network of neurons and glial cells, coordinates major gastrointestinal functions. Impaired development or secondary aberrations cause severe enteric neuropathies. Neural crest-derived stem cells as well as enteric neuronal progenitor cells, which form enteric neurospheres, represent a promising tool to unravel molecular pathomechanisms and to develop novel therapy options. However, so far little is known about the detailed cellular composition and the proportional distribution of enteric neurospheres. Comprehensive knowledge will not only be essential for basic research but also for prospective cell replacement therapies to restore or to improve enteric neuronal dysfunction. METHODS Human enteric neurospheres were generated from three individuals with varying age. For detailed molecular characterization, nCounter target gene expression analyses focusing on stem, progenitor, neuronal, glial, muscular, and epithelial cell markers were performed. Corresponding archived paraffin-embedded individuals' specimens were analyzed accordingly. KEY RESULTS Our data revealed a remarkable molecular complexity of enteric neurospheres and archived specimens. Amongst the expression of multipotent stem cell, progenitor cell, neuronal, glial, muscle and epithelial cell markers, moderate levels for the pluripotency marker POU5F1 were observed. Furthermore, besides the interindividual variability, we identified highly distinct intraindividual expression profiles. CONCLUSIONS & INFERENCES Our results emphasize the assessment of molecular signatures to be essential for standardized use, optimization of experimental approaches, and elimination of potential risk factors, as the formation of tumors. Our study pipeline may serve as a blueprint implemented into the characterization procedure of enteric neurospheres for various future applications.
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Affiliation(s)
- Stefanie Schmitteckert
- Department of Human Molecular Genetics, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany
| | - Tanja Mederer
- Department of Human Molecular Genetics, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany
| | - Ralph Röth
- Department of Human Molecular Genetics, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany.,nCounter Core Facility, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany
| | - Patrick Günther
- Division of Pediatric Surgery, Department of Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | - Stefan Holland-Cunz
- Division of Pediatric Surgery, Department of Surgery, Heidelberg University Hospital, Heidelberg, Germany.,Pediatric Surgery, University Children's Hospital Basel, Basel, Switzerland
| | - Marco Metzger
- Fraunhofer Institute for Silicate Research (ISC), Translational Centre Regenerative Therapies (TLC-RT) Wuerzburg, Wuerzburg, Germany
| | - Yvonne Samstag
- Institute of Immunology, Heidelberg University Hospital, Heidelberg, Germany
| | | | - Guido Wabnitz
- Institute of Immunology, Heidelberg University Hospital, Heidelberg, Germany
| | - Stefan Kurzhals
- Institute of Immunology, Heidelberg University Hospital, Heidelberg, Germany
| | - Jutta Scheuerer
- Institute of Pathology, Heidelberg University Hospital, Heidelberg, Germany
| | - Carlo A Beretta
- CellNetworks Math-Clinic Core Facility, Bioquant, Heidelberg University, Heidelberg, Germany
| | - Felix Lasitschka
- Institute of Pathology, Heidelberg University Hospital, Heidelberg, Germany
| | - Gudrun A Rappold
- Department of Human Molecular Genetics, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany.,Interdisciplinary Center for Neurosciences (IZN), Heidelberg University, Heidelberg, Germany
| | - Philipp Romero
- Division of Pediatric Surgery, Department of Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | - Beate Niesler
- Department of Human Molecular Genetics, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany.,nCounter Core Facility, Institute of Human Genetics, Heidelberg University Hospital, Heidelberg, Germany.,Interdisciplinary Center for Neurosciences (IZN), Heidelberg University, Heidelberg, Germany
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14
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Veras LV, Chotai PN, Tumen AZ, Gosain A. Impaired growth outcomes in children with congenital colorectal diseases. J Surg Res 2018; 229:102-107. [PMID: 29936975 DOI: 10.1016/j.jss.2018.03.069] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2017] [Revised: 03/02/2018] [Accepted: 03/29/2018] [Indexed: 12/20/2022]
Abstract
BACKGROUND Cloaca, Hirschsprung disease, and anorectal malformations (CHARM) are congenital anomalies of the hindgut. Small series have suggested that children suffering from one of these anomalies may be at risk for growth impairment. We sought to expand on these findings in a comprehensive cohort, hypothesizing that patients with Medicaid insurance or African-American (AA) race would be at higher risk for poor growth. METHODS Following Institutional Review Board (IRB) approval, single-institution retrospective review of children with CHARM anomalies was performed (2009-2016). Body mass index (BMI) value Z-scores were obtained using the 2006 World Health Organization (age 0-24 mo) and 2000 Centers for Disease Control (CDC) (age >2 y) growth charts and calculators (statistical analysis system). Patient factors and BMI Z-scores were analyzed with descriptive statistics and Fisher's exact test. RESULTS One hundred sixty-six patients (Cloaca n = 16, Hirschsprung disease [HD] n = 71, anorectal malformation [ARM] n = 79) were identified. The BMI Z-score distribution for the entire CHARM cohort was lower than controls (P < 0.0001). HD and ARM BMI Z-scores were also lower versus controls (P < 0.0007, P < 0.0037). Requiring more or less than the average number of surgeries did not impact BMI Z-score [P = non-significant (NS)]. Patients with Medicaid had lower Z-scores versus private or commercial insurance (P < 0.0001). AA race BMI Z-score distribution was lower than controls (P < 0.0002), but there was no statistical difference in BMI Z-scores when comparing AA versus non-AA CHARM patients (P = NS). CONCLUSIONS Patients born with CHARM anomalies are at risk for impaired growth. Furthermore study is warranted to identify modifiable risk factors contributing to this impairment. Longitudinal follow-up should include interventions to mitigate these risks.
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Affiliation(s)
- Laura V Veras
- Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee
| | - Pranit N Chotai
- Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee
| | - Andrew Z Tumen
- Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee
| | - Ankush Gosain
- Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee; Children's Foundation Research Institute, Le Bonheur Children's Hospital, Memphis, Tennessee.
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15
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Abstract
Hirschsprung disease is a developmental defect of the enteric nervous system characterized by lack of enteric neurons in the distal hindgut. There are numerous reports on short-term outcomes indicating that impaired bowel function is common. Recently, several controlled studies show that bowel function outcomes are affected beyond childhood, in adolescents and adults, compared with healthy control subjects. Constipation and fecal incontinence are common. The impaired bowel function appears to have a negative impact on quality of life, although, a majority of patients have adapted to their symptoms. On the other hand, Hirschsprung disease seems to have limited impact on education and occupation in adult life. The aim of this review was to summarize current knowledge of bowel function outcome beyond childhood in patients with Hirschsprung disease.
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Affiliation(s)
- Tomas Wester
- Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden.
| | - Anna Löf Granström
- Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden; Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
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16
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Bjørnland K, Pakarinen MP, Stenstrøm P, Stensrud KJ, Neuvonen M, Granström AL, Graneli C, Pripp AH, Arnbjörnsson E, Emblem R, Wester T, Rintala RJ. A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease. J Pediatr Surg 2017; 52:1458-1464. [PMID: 28094015 DOI: 10.1016/j.jpedsurg.2017.01.001] [Citation(s) in RCA: 41] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/04/2016] [Revised: 12/07/2016] [Accepted: 01/03/2017] [Indexed: 12/17/2022]
Abstract
OBJECTIVE Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.
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Affiliation(s)
- Kristin Bjørnland
- Department of Paediatric Surgery, Oslo University Hospital-Rikshospitalet and University of Oslo, Oslo, Norway.
| | - Mikko P Pakarinen
- Department of Paediatric Surgery, Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Pernilla Stenstrøm
- Department of Paediatric Surgery, Skåne University Hospital, Lund, Sweden
| | - Kjetil J Stensrud
- Department of Paediatric Surgery, Oslo University Hospital-Rikshospitalet and University of Oslo, Oslo, Norway
| | - Malla Neuvonen
- Department of Paediatric Surgery, Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Anna L Granström
- Department of Paediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
| | - Christina Graneli
- Department of Paediatric Surgery, Skåne University Hospital, Lund, Sweden
| | - Are H Pripp
- Department of Paediatric Surgery, Oslo Centre of Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital, Oslo, Norway
| | - Einar Arnbjörnsson
- Department of Paediatric Surgery, Skåne University Hospital, Lund, Sweden
| | - Ragnhild Emblem
- Department of Paediatric Surgery, Oslo University Hospital-Rikshospitalet and University of Oslo, Oslo, Norway
| | - Tomas Wester
- Department of Paediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
| | - Risto J Rintala
- Department of Paediatric Surgery, Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
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Wang Y, Liang H, Wu Q, Zheng H, Liu G, Wen Z, Lan M, Yu J, Zhu D, Liang J, Zhang J, Xu X, Xia H. Bowel management program for pediatric postoperative fecal incontinence in China: A surgeon's experience. Medicine (Baltimore) 2017; 96:e7078. [PMID: 28562577 PMCID: PMC5459742 DOI: 10.1097/md.0000000000007078] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
The purpose of this article is to report the status of the efficacy of and long-term adherence to the Bowel Management Program (BMP) for fecal incontinence (FI) postoperation in China.Children over 3 years of age with FI postoperation referred to our medical center were included in the study. Evaluations were performed before and 2 years after their clinic visit. The cost of bowel care, improvement in incontinence, health-related quality of life, and family functioning with the BMP were analyzed.A total of 48 children with FI were included in our study, of whom 38 were boys. The median treatment fee was 660.1 dollars. The complications included abdominal pain (4 patients, 8%), occasional vomiting (2 patients, 4%), and hypoglycemia (1 patient, 2%). The incontinence status and health-related quality of life improved significantly after the BMP. Despite the good outcome of the BMP, half of the patients discontinued the program.The BMP is an effective approach to manage FI and improve the patients' quality of life. Poor long-term adherence is currently the main challenge affecting the BMP application in China.
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Affiliation(s)
- Yong Wang
- Southern Medical University
- Department of Pediatric Surgery
| | - Huiying Liang
- Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong, China
| | | | - Haiqing Zheng
- Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong, China
| | - Guangjian Liu
- Institute of Pediatrics, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong, China
| | - Zhe Wen
- Department of Pediatric Surgery
| | | | | | | | | | | | | | - Huimin Xia
- Southern Medical University
- Department of Pediatric Surgery
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18
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Thakkar HS, Bassett C, Hsu A, Manuele R, Kufeji D, Richards CA, Agrawal M, Keshtgar AS. Functional outcomes in Hirschsprung disease: A single institution's 12-year experience. J Pediatr Surg 2017; 52:277-280. [PMID: 27912977 DOI: 10.1016/j.jpedsurg.2016.11.023] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/04/2016] [Accepted: 11/08/2016] [Indexed: 01/06/2023]
Abstract
AIMS Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period. MATERIALS AND METHODS We conducted a retrospective review of all children operated for HD between 2002 and 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0-20, 20=best score). We assessed hospital admissions, complications including Hirschsprung associated enterocolitis (HAEC) and the need for further surgical procedures. RESULTS 72 (52 male) patients were studied, of whom, 6 (8%) had a positive family history, 5 (7%) had Trisomy 21 and 5 (7%) had total colonic HD. The median age at diagnosis was 6.5days (2 days-6.7 years) and median follow-up was 6years (1-12years). All patients except two underwent a Duhamel pull-through procedure. The median age at surgery was 4months (6days-90months). 37 (51%) procedures were performed single-stage and 7 (10%) were laparoscopically assisted. Our early complication rate was 15%; 11 (15%) patients were treated for HAEC and 43 (60%) did not require any further surgery. 12 (17%) underwent injection of botulinum toxin, 7 (10%) needed residual spur division and 4 (5%) required an unplanned, post pull-through enterostomy for obstructive defecation symptoms and HAEC. Two (3%) patients underwent an Antegrade Colonic Enema (ACE) stoma. The median BFS was 17 (5-20). There were two deaths both out of hospital. CONCLUSIONS Long-term functional outcomes following Duhamel Pull-Through surgery are satisfactory although 40% of patients needed some form of further surgical intervention. The management of anal sphincter achalasia has improved with the use of botulinum toxin and we advocate aggressive and early management of this condition for symptoms of obstructive defecation and HAEC. LEVEL OF EVIDENCE III.
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Affiliation(s)
- Hemanshoo S Thakkar
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Christopher Bassett
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Andy Hsu
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Riccardo Manuele
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Dorothy Kufeji
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Catherine A Richards
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Meena Agrawal
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH
| | - Alireza S Keshtgar
- Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
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19
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Wei ZJ, Huang L, Xu AM. Reoperation in an adult female with "right-sided" Hirschsprung's disease complicated by refractory hypertension and cough. World J Gastroenterol 2016; 22:9235-9241. [PMID: 27895411 PMCID: PMC5107605 DOI: 10.3748/wjg.v22.i41.9235] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/27/2016] [Revised: 08/17/2016] [Accepted: 09/06/2016] [Indexed: 02/06/2023] Open
Abstract
Hirschsprung's disease (HD) is an intestinal malformation caused by the innate absence of ganglion cells in the neural plexus of the colorectal wall, and is most common in male infants. It is rare in adult, and is usually left-sided. Herein we reported based on the CARE guidelines a case of a 47-year-old adult female suffering from "right-sided" HD complicated by refractory hypertension and cough. The patient with a history of cesarean section and with digestive unfitness (abdominal pain, distention, and constipation) only since 20 years old had recurrence of HD after initial surgery due to the incomplete removal of the HD-affected bowel based on a diagnosis of "chronic ileus", leading to the relapse of the digestive symptoms and the emergence of some intractable circulatory and respiratory complications which could be hardly controlled by conservative treatment. During the long interval before coming to our department for help, she had been re-hospitalized for several times with various misdiagnoses and supplied merely with symptomatic treatment which could only achieve temporary symptomatic relief. At her admission to our department, the imaging examinations strongly indicated recurrent HD which was further supported by pathological examinations, and right hemi-colectomy was performed to remove the remnant aganglionic intestinal segment. Intraoperative and postoperative pathology supported the completeness of the definitive resection. Post-operation, the patient's bowel motility significantly improved, and interestingly, the complications disappeared. For adult patients with long-term constipation combined with cough and hypertension, rare diseases like HD which requires definite surgery and which could be "right-sided" should not be overlooked. It is vital to diagnose and cure HD patients in childhood. Through the comparison of the two surgeries, it is noteworthy that for diagnosed HD, sufficient removal of the non-functional intestine confirmed by intraoperative pathology is essential.
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20
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Bandi AS, Bradshaw CJ, Giuliani S. Advances in minimally invasive neonatal colorectal surgery. World J Gastrointest Surg 2016; 8:670-678. [PMID: 27830038 PMCID: PMC5081548 DOI: 10.4240/wjgs.v8.i10.670] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/06/2016] [Revised: 07/27/2016] [Accepted: 08/18/2016] [Indexed: 02/06/2023] Open
Abstract
Over the last two decades, advances in laparoscopic surgery and minimally invasive techniques have transformed the operative management of neonatal colorectal surgery for conditions such as anorectal malformations (ARMs) and Hirschsprung’s disease. Evolution of surgical care has mainly occurred due to the use of laparoscopy, as opposed to a laparotomy, for intra-abdominal procedures and the development of trans-anal techniques. This review describes these advances and outlines the main minimally invasive techniques currently used for management of ARMs and Hirschsprung’s disease. There does still remain significant variation in the procedures used and this review aims to report the current literature comparing techniques with an emphasis on the short- and long-term clinical outcomes.
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21
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Zimmer J, Tomuschat C, Puri P. Long-term results of transanal pull-through for Hirschsprung's disease: a meta-analysis. Pediatr Surg Int 2016; 32:743-9. [PMID: 27385111 DOI: 10.1007/s00383-016-3908-z] [Citation(s) in RCA: 60] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 06/18/2016] [Indexed: 12/13/2022]
Abstract
INTRODUCTION Several operative techniques have been developed for the treatment of Hirschsprung's disease (HD) in the past decades. Since one-stage transanal pull-through (TAPT) was first performed in 1998, multiple studies have shown favourable short-and midterm results compared to other techniques with shorter operation length, shorter hospital stay and lower complication rates. The aim of this meta-analysis was to determine the longterm results following TAPT for HD. METHODS A systematic literature search for relevant articles was performed in four databases using the following terms "Hirschsprung/Hirschsprung's disease", "aganglionosis", "transanal", "pullthrough/pull-through", "longterm/long-term" "results", "follow-up" and "outcome". A meta-analysis was conducted for relevant articles for one-stage transanal pull-through for HD with a minimal follow-up of median 36 months regarding constipation, incontinence/soiling, enterocolitis and secondary operations. Odds ratio (OR) with 95 % confidence intervals (CI) were calculated. RESULTS Six studies with 316 patients matched the set criteria and were included in this analysis. Overall 45 (14.2 %) patients had disturbances of bowel function (OR 0.05, 95 % CI 0.03-0.07, p < 0.00001). Of these, 24 (53.3 %) patients experienced constipation, 8 (17.8 %) incontinence/soiling and 13 (28.9 %) enterocolitis. 10 (3.2 %) patients developed complications requiring secondary surgery. Most patients had a daily defecation frequency of 1-3 bowel movements 3 years postoperatively, resembling the stooling patterns of healthy controls. CONCLUSION Nearly 15 % of all patients operated with TAPT for HD continue to experience persistent bowel symptoms with constipation as the main problem. Further studies on the long-term outcome of children operated with this technique for HD are necessary to evaluate stooling patterns, urinary and sexual function as well as general quality of life during adolescence and adulthood.
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Affiliation(s)
- J Zimmer
- National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland
| | - C Tomuschat
- National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland
| | - P Puri
- National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland.
- School of Medicine and Medical Science and Conway Institute of Biomolecular and Biomedical Research, University College Dublin, Dublin, Ireland.
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22
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Burns AJ, Goldstein AM, Newgreen DF, Stamp L, Schäfer KH, Metzger M, Hotta R, Young HM, Andrews PW, Thapar N, Belkind-Gerson J, Bondurand N, Bornstein JC, Chan WY, Cheah K, Gershon MD, Heuckeroth RO, Hofstra RMW, Just L, Kapur RP, King SK, McCann CJ, Nagy N, Ngan E, Obermayr F, Pachnis V, Pasricha PJ, Sham MH, Tam P, Vanden Berghe P. White paper on guidelines concerning enteric nervous system stem cell therapy for enteric neuropathies. Dev Biol 2016; 417:229-51. [PMID: 27059883 DOI: 10.1016/j.ydbio.2016.04.001] [Citation(s) in RCA: 101] [Impact Index Per Article: 11.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2016] [Revised: 03/29/2016] [Accepted: 04/02/2016] [Indexed: 12/22/2022]
Abstract
Over the last 20 years, there has been increasing focus on the development of novel stem cell based therapies for the treatment of disorders and diseases affecting the enteric nervous system (ENS) of the gastrointestinal tract (so-called enteric neuropathies). Here, the idea is that ENS progenitor/stem cells could be transplanted into the gut wall to replace the damaged or absent neurons and glia of the ENS. This White Paper sets out experts' views on the commonly used methods and approaches to identify, isolate, purify, expand and optimize ENS stem cells, transplant them into the bowel, and assess transplant success, including restoration of gut function. We also highlight obstacles that must be overcome in order to progress from successful preclinical studies in animal models to ENS stem cell therapies in the clinic.
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Affiliation(s)
- Alan J Burns
- Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, UK; Department of Clinical Genetics, Erasmus Medical Center, Rotterdam, The Netherlands.
| | - Allan M Goldstein
- Department of Pediatric Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
| | - Donald F Newgreen
- Murdoch Childrens Research Institute, Royal Children's Hospital, Parkville 3052, Victoria, Australia
| | - Lincon Stamp
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, Victoria 3010, Australia
| | - Karl-Herbert Schäfer
- University of Applied Sciences, Kaiserlautern, Germany; Clinic of Pediatric Surgery, University Hospital Mannheim, University Heidelberg, Germany
| | - Marco Metzger
- Fraunhofer-Institute Interfacial Engineering and Biotechnology IGB Translational Centre - Würzburg branch and University Hospital Würzburg - Tissue Engineering and Regenerative Medicine (TERM), Würzburg, Germany
| | - Ryo Hotta
- Department of Pediatric Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA
| | - Heather M Young
- Department of Anatomy and Neuroscience, University of Melbourne, Parkville, Victoria 3010, Australia
| | - Peter W Andrews
- Centre for Stem Cell Biology, Department of Biomedical Science, University of Sheffield, Sheffield, UK
| | - Nikhil Thapar
- Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, UK
| | - Jaime Belkind-Gerson
- Division of Gastroenterology, Hepatology and Nutrition, Massachusetts General Hospital for Children, Harvard Medical School, Boston, USA
| | - Nadege Bondurand
- INSERM U955, 51 Avenue du Maréchal de Lattre de Tassigny, F-94000 Créteil, France; Université Paris-Est, UPEC, F-94000 Créteil, France
| | - Joel C Bornstein
- Department of Physiology, University of Melbourne, Parkville, Victoria 3010, Australia
| | - Wood Yee Chan
- School of Biomedical Sciences, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong
| | - Kathryn Cheah
- School of Biomedical Sciences, The University of Hong Kong, Hong Kong
| | - Michael D Gershon
- Department of Pathology and Cell Biology, Columbia University, New York 10032, USA
| | - Robert O Heuckeroth
- Department of Pediatrics, The Children's Hospital of Philadelphia Research Institute, Philadelphia, PA 19104, USA; Perelman School of Medicine at the University of Pennsylvania, Abramson Research Center, Philadelphia, PA 19104, USA
| | - Robert M W Hofstra
- Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, UK; Department of Clinical Genetics, Erasmus Medical Center, Rotterdam, The Netherlands
| | - Lothar Just
- Institute of Clinical Anatomy and Cell Analysis, University of Tübingen, Germany
| | - Raj P Kapur
- Department of Pathology, University of Washington and Seattle Children's Hospital, Seattle, WA, USA
| | - Sebastian K King
- Department of Paediatric and Neonatal Surgery, The Royal Children's Hospital, Melbourne, Australia
| | - Conor J McCann
- Stem Cells and Regenerative Medicine, UCL Great Ormond Street Institute of Child Health, London, UK
| | - Nandor Nagy
- Department of Anatomy, Histology and Embryology, Faculty of Medicine, Semmelweis University, Budapest, Hungary
| | - Elly Ngan
- Department of Surgery, The University of Hong Kong, Hong Kong
| | - Florian Obermayr
- Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital Tübingen, D-72076 Tübingen, Germany
| | | | | | - Mai Har Sham
- Department of Biochemistry, The University of Hong Kong, Hong Kong
| | - Paul Tam
- Department of Surgery, The University of Hong Kong, Hong Kong
| | - Pieter Vanden Berghe
- Laboratory for Enteric NeuroScience (LENS), TARGID, University of Leuven, Belgium
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Granström AL, Danielson J, Husberg B, Nordenskjöld A, Wester T. Adult outcomes after surgery for Hirschsprung's disease: Evaluation of bowel function and quality of life. J Pediatr Surg 2015; 50:1865-9. [PMID: 26164226 DOI: 10.1016/j.jpedsurg.2015.06.014] [Citation(s) in RCA: 46] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/27/2015] [Revised: 04/29/2015] [Accepted: 06/10/2015] [Indexed: 12/13/2022]
Abstract
BACKGROUND AND AIMS Hirschsprung's disease (HSCR) is treated surgically. There is a risk for faecal incontinence and constipation postoperatively. The long-term bowel functional outcome in adults and quality of life are sparsely studied. The aim of this study was to assess bowel function and quality of life in patients who had undergone surgery for HSCR during childhood. METHODS All patients treated between 1969 and 1994 at St. Göran's Children's Hospital in Stockholm were invited to participate in the study. After consent, the patients received questionnaires containing general questions, validated questions on bowel function, questions about urinary function, SF-36 health survey (SF-36) and the Gastrointestinal Quality of Life Index (GIQLI). Clinical data were extracted from the case records. Controls matched for sex and age were randomly selected from the National Swedish Population Register. RESULTS 48 of 60 (80%) invited patients responded to the questionnaires. Nine patients were excluded since the HSCR diagnosis could not be confirmed. The median age of the included patients was 28 (20-43) years. Most patients had undergone Soave's operation (73.4%) and two patients had a stoma at the time of follow-up. The bowel function was impaired in the HSCR group compared to controls, especially problems with flatulence, need to strain at defecation and several defecations for emptying. Patients in the HSCR group also had significantly more problems with faecal incontinence than controls. Quality of life according to SF-36 did not differ significantly between patients and controls, but the GIQLI score showed a significantly worse outcome in the HSCR group compared to the controls. CONCLUSION General quality of life in adults treated for HSCR during childhood is comparable to controls. However, they have impaired bowel function and gastrointestinal quality of life.
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Affiliation(s)
- Anna Löf Granström
- Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
| | - Johan Danielson
- Institution of Women's and Children's Health, Uppsala University, Uppsala, Sweden
| | - Britt Husberg
- Department of CLINTEC, Karolinska Institutet, Stockholm, Sweden
| | - Agneta Nordenskjöld
- Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden
| | - Tomas Wester
- Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Division of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden
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Enteric neurospheres are not specific to neural crest cultures: implications for neural stem cell therapies. PLoS One 2015; 10:e0119467. [PMID: 25799576 PMCID: PMC4370605 DOI: 10.1371/journal.pone.0119467] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2014] [Accepted: 01/27/2015] [Indexed: 12/15/2022] Open
Abstract
Objectives Enteric neural stem cells provide hope of curative treatment for enteric neuropathies. Current protocols for their harvesting from humans focus on the generation of ‘neurospheres’ from cultures of dissociated gut tissue. The study aims to better understand the derivation, generation and composition of enteric neurospheres. Design Gut tissue was obtained from Wnt1-Cre;Rosa26Yfp/Yfp transgenic mice (constitutively labeled neural crest cells) and paediatric patients. Gut cells were cultured either unsorted (mixed neural crest/non-neural crest), or following FACS selection into neural crest (murine-YFP+ve/human-p75+ve) or non-neural crest (YFP-ve/p75-ve) populations. Cultures and resultant neurospheres were characterized using immunolabelling in vitro and following transplantation in vivo. Results Cultures of (i) unsorted, (ii) neural crest, and (iii) non-neural crest cell populations generated neurospheres similar in numbers, size and morphology. Unsorted neurospheres were highly heterogeneous for neural crest content. Neural crest-derived (YFP+ve/p75+ve) neurospheres contained only neural derivatives (neurons and glia) and were devoid of non-neural cells (i.e. negative for SMA, c-Kit), with the converse true for non-neural crest-derived (YFP-ve/p75-ve) ‘neurospheres’. Under differentiation conditions only YFP+ve cells gave rise to neural derivatives. Both YFP+ve and YFP-ve cells displayed proliferation and spread upon transplantation in vivo, but YFP-ve cells did not locate or integrate within the host ENS. Conclusions Spherical accumulations of cells, so-called ‘neurospheres’ forming in cultures of dissociated gut contain variable proportions of neural crest-derived cells. If they are to be used for ENS cell replacement therapy then improved protocols for their generation, including cell selection, should be sought in order to avoid inadvertent transplantation of non-therapeutic, non-ENS cells.
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Han Y, Lin MB, Zhang YJ, Yin L. Total laparoscopic modified Duhamel operation in combination with transanal endoscopic microsurgery. JSLS 2014; 18:128-31. [PMID: 24680156 PMCID: PMC3939328 DOI: 10.4293/108680813x13693422520288] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Abstract
INTRODUCTION Laparoscopic-assisted colonic resection has been well described for multiple surgical indications and typically requires an abdominal incision for specimen removal that is associated with most of the postoperative pain. We report the total laparoscopic modified Duhamel operation for megacolon in combination with transanal endoscopic microsurgery for transanal specimen retrieval and anastomosis to avoid the additional abdominal extraction incision. CASE DESCRIPTION Two cases are presented: case 1 was a 15-year-old boy who presented with intermittent abdominal distention, pain, and constipation for 3 years' duration and was diagnosed with Hirschsprung disease, and case 2 was a 60-year-old man who presented with repeated attacks of incomplete intestinal obstruction for 2 years' duration and was diagnosed with adult megacolon. They were treated by the total laparoscopic modified Duhamel operation without an abdominal extraction incision in combination with transanal endoscopic microsurgery. The operations were successfully accomplished without conversion to open surgery. The patients tolerated the procedure well, complained of minimal postoperative pain, and did not require narcotics beyond the day of the operation. No surgical complications occurred. Discharge from the hospital occurred on the ninth postoperative day in case 1 and the 13th postoperative day in case 2. DISCUSSION The total laparoscopic modified Duhamel operation in combination with transanal endoscopic microsurgery is a feasible and minimally invasive technique for idiopathic megacolon and adult megacolon. This advanced surgical technique was developed by combining laparoscopy with the concept of natural orifice transluminal endoscopic surgery.
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Affiliation(s)
- Yi Han
- Department of General Surgery, RuiJin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Mou-Bin Lin
- Department of General Surgery, RuiJin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Ya-Jie Zhang
- Department of General Surgery, RuiJin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
| | - Lu Yin
- Department of General Surgery, RuiJin Hospital, Shanghai Jiaotong University School of Medicine, No. 197, Ruijin No. 2 Road, Shanghai, China.
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Long-term outcomes and quality of life after subtotal colectomy combined with modified Duhamel procedure for adult Hirschsprung's disease. Pediatr Surg Int 2014; 30:55-61. [PMID: 24232173 DOI: 10.1007/s00383-013-3423-4] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/14/2013] [Indexed: 12/14/2022]
Abstract
BACKGROUND Subtotal colectomy combined with modified Duhamel procedure (Jinling procedure) was used in patients with adult Hirschsprung's disease (AHD) at Jinling Hospital in the last decade. The aim of this study is to evaluate the safety, effectiveness and quality of life of Jinling procedure for AHD. MATERIALS AND METHODS All the data are from the database of the Jinling Hospital Constipation Registry System. Primary outcomes, including safety (morbidity and adverse events), effectiveness (satisfaction rate, Wexner constipation scale (WCS) and bowel function score (BFS)) and gastrointestinal quality of life index (GIQLI), were all evaluated. RESULTS Fifty-nine patients were available for this study and the mean postoperative period was 44 months. Seventeen major complications were found in 11 patients and most of the complications could be managed conservatively. A significant improvement in WCS, BFS and GIQLI at 6 months postoperatively was obtained. CONCLUSION Jinling procedure is safe and effective for AHD.
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Granström AL, Husberg B, Nordenskjöld A, Svensson PJ, Wester T. Laparoscopic-assisted pull-through for Hirschsprung's disease, a prospective repeated evaluation of functional outcome. J Pediatr Surg 2013; 48:2536-9. [PMID: 24314199 DOI: 10.1016/j.jpedsurg.2013.07.017] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/18/2013] [Revised: 07/04/2013] [Accepted: 07/12/2013] [Indexed: 10/26/2022]
Abstract
AIM OF THE STUDY To evaluate the functional outcome of laparoscopic-assisted endorectal pull-through (LAP) for Hirschsprung's disease (HSCR) over time. METHODS Thirty-five children with HSCR underwent laparoscopic-assisted pull-through at our institution between 1998 and 2009. The diagnosis was histologically confirmed in all cases. Clinical data was extracted from the case records. A prospective assessment of the functional outcome was performed in 2009 and 2012. Exclusion criteria were a follow-up of less than 6 months after treatment (1 case) and total colonic aganglionosis (1 case). An independent examiner, not involved in the clinical care of the patients, performed interviews using a semi-structured questionnaire. Four patients could not be traced for the first interview. Two cases were lost for the second interview. Altogether twenty-seven patients completed the study. Data from the two interviews were compared. The regional ethical review board approved the study. MAIN RESULTS The median patient age was 4 years old (range 2-16) at the time of the first interview and 7 years old (range 5-19) at the time of the second interview . There were 23 males and 4 females in the study group. The median age at laparoscopic-assisted pull-through was 104 days old (range 29 days-8 years). In the first interview 11 patients reported constipation, 18 patients reported soiling more frequently than once per week when they had loose stools and 16 patients when they had solid stools. Laxatives or irrigations were used by 13 of the patients. In the second interview 4 patients reported constipation, 16 patients reported soiling when they had loose stools and 15 patients reported soiling when they had solid stools. Eight patients used laxatives or irrigations. The decrease in constipation was statistically significant (p=0,023). CONCLUSIONS Our study shows a statistically significant reduction of constipation over time. There is a high risk of incontinence after laparoscopic-assisted pull-through, with few signs of short-term improvement.
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Affiliation(s)
- Anna Löf Granström
- Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Unit of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
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Abstract
Despite significant developments in the understanding of the pathologic anatomy and physiology of Hirschsprung's disease (HD), the results of surgical therapy remain far from perfect. The functional defects and psychosocial difficulties that occur commonly in children with HD are passed on to adulthood in a significant proportion of patients. Recent prospective and adequately controlled cross-sectional studies reveal that constipation and fecal soiling are common late sequelae in adulthood. HD patients show uniformly lower scores of overall bowel function than healthy control subjects. Functional outcome and quality of life may be interrelated and deteriorate with increasing age. Despite these shortcomings, at adult age, most of the HD patients appear to be able to function as normal members of the society in terms of psychosocial, occupational, and recreational activities. Patients are at risk of developing related conditions, such as cancer, that require planning of specific screening programs. Many of the long-term problems that these patients experience are specific to HD. The follow-up of HD patients should be performed by medical personnel familiar with the disease, preferably in a specialized referral center, and the follow-up should continue beyond childhood.
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Affiliation(s)
- Risto J Rintala
- Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Finland.
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Affiliation(s)
- A Gunnarsdóttir
- Section of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden
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Bowel function and gastrointestinal quality of life among adults operated for Hirschsprung disease during childhood: a population-based study. Ann Surg 2010; 252:977-81. [PMID: 21107107 DOI: 10.1097/sla.0b013e3182018542] [Citation(s) in RCA: 111] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVE To assess bowel function and gastrointestinal quality of life among adults with operated Hirschsprung's disease (HD). SUMMARY BACKGROUND DATA Outcomes of HD extending to adulthood are unclear; bowel function and quality of life may deteriorate by aging. METHODS Bowel function and gastrointestinal quality of life were cross-sectionally assessed in a population-based manner among adults operated for HD during childhood between 1950 and 1986. Patients were interviewed during their outpatient visit. Controls matched for age and sex were randomly chosen from the Population Register Centre of Finland. RESULTS Ninety-two (64%) patients representative for the entire study population responded. The mean age of patients (79% male) was 43 (interquartile range [IQR], 35-48) years. Most (78%) had undergone Duhamel operation, and 94% had aganglionosis confined to the rectosigmoid. The mean overall bowel function score was decreased among patients (17.1 ± 2.8 vs 19.1 ± 1.2; P < 0.0001). They reported increased incidence of inability to hold back defecation (40% vs 17%), fecal soiling (48% vs 22%), constipation (30% vs 9%) and social problems related to bowel function (29% vs 11%; P < 0.05 for all). Gastrointestinal quality of life was only marginally lower among patients (121 ± 15.3 vs 125 ± 13.1; P = 0.0578) mainly because of significantly lower scores in questions assessing disease-specific factors such as bowel function and continence. Age was the only predictor of poor bowel function (OR 1.07, 95% CI 1.00-1.14, P = 0.049), which weakly predicted gastrointestinal quality of life (OR 0.81, 95% CI 0.66-1.01, P = 0.055). CONCLUSIONS Although bowel function deteriorates with increasing age after operated HD, it is associated with only slightly decreased gastrointestinal quality of life.
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Rintala RJ, Pakarinen MP. Outcome of anorectal malformations and Hirschsprung's disease beyond childhood. Semin Pediatr Surg 2010; 19:160-7. [PMID: 20307853 DOI: 10.1053/j.sempedsurg.2009.11.021] [Citation(s) in RCA: 72] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Anorectal malformations (ARMs) and Hirschsprung disease (HD) are the most common congenital colorectal defects in the newborn. The outcomes of HD and ARMs have improved significantly because of improved understanding of the pathologic anatomy and physiology of these defects and of the modern surgical techniques. Still, many patients suffer from defective bowel control even as adults. Some of these also have problems with urinary control and sexual functions. The functional problems are more pronounced in patients with ARMs. Compared with healthy people, both patients with ARMs and those with HD have limitations in their quality of life. Inferior quality of life is more common in patients with ARMs. There are very few published data on long-term outcome of adults with ARMs and HD. The effect of aging on the functional outcome and quality of life remains unclear, although some preliminary data suggest that the bowel function and quality of life may deteriorate with aging.
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Affiliation(s)
- Risto J Rintala
- Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, FIN-00029Helsinki, Finland.
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Hotta R, Natarajan D, Thapar N. Potential of cell therapy to treat pediatric motility disorders. Semin Pediatr Surg 2009; 18:263-73. [PMID: 19782309 DOI: 10.1053/j.sempedsurg.2009.07.008] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
Gut motility disorders represent a significant challenge in clinical management with current palliative approaches failing to overcome disease and treatment-related morbidity. The recent progress with stem cells to restore missing or defective elements of the gut neuromusculature offers new hope for potential cure. Focusing on enteric neuropathies such as Hirschsprung's disease, the review discusses the progress that has been made in the sourcing of putative stem cells and the studies into their biology and therapeutic potential. It also explores the practical challenges that must be overcome before stem cell-based therapies can be applied in the clinical arena. Although many obstacles remain, the speed of advancement of the enteric stem cell field suggests that such therapies are on the horizon.
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Affiliation(s)
- Ryo Hotta
- Department of Anatomy & Cell Biology, University of Melbourne, Victoria, Australia
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Metzger M, Caldwell C, Barlow AJ, Burns AJ, Thapar N. Enteric nervous system stem cells derived from human gut mucosa for the treatment of aganglionic gut disorders. Gastroenterology 2009; 136:2214-25.e1-3. [PMID: 19505425 DOI: 10.1053/j.gastro.2009.02.048] [Citation(s) in RCA: 149] [Impact Index Per Article: 9.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2008] [Revised: 01/13/2009] [Accepted: 02/10/2009] [Indexed: 12/02/2022]
Abstract
BACKGROUND & AIMS Enteric nervous system stem cells (ENSSCs) provide potential therapeutic tools to replenish absent ganglia in Hirschsprung's disease. Although full-thickness human postnatal gut tissue can be used to generate ENSSCs, reliance on its harvesting from surgical resection poses significant practical limitations. This study aimed to explore whether gut tissue obtained utilizing minimally invasive routine endoscopy techniques could be used to generate ENSSCs and whether such cells retain the potential to generate an ENS upon transplantation into aganglionic gut. METHODS Postnatal human gut mucosal tissue obtained from children undergoing gastrointestinal endoscopy was used to generate cell cultures in which ENSSCs were contained within neurosphere-like bodies (NLBs). These NLBs were characterized by immunostaining, and their potential to generate components of the ENS, in vitro and upon transplantation into models of aganglionic gut, was examined. RESULTS Gut mucosal biopsy specimens were obtained from 75 children (age, 9 months-17 years). The biopsy specimens contained neural cells and ENSSCs and, on culturing, generated characteristic NLBs at all ages examined. Postnatal mucosa-derived NLBs contained cells that, akin to their embryonic counterparts, were proliferating, expressed ENSSC markers, were bipotent, and capable of generating large colonies in clonogenic cultures and multiple ENS neuronal subtypes. Upon transplantation, cells from NLBs colonized cultured recipient aganglionic chick and human hindgut to generate ganglia-like structures and enteric neurons and glia. CONCLUSIONS The results represent a significant practical advance toward the development of definitive cell replenishment therapies for ENS disorders such as Hirschsprung's disease.
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Affiliation(s)
- Marco Metzger
- Gastroenterology, Institute of Child Health, University College London, London, United Kingdom
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