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Erschienen in: European Journal of Pediatrics 3/2023

12.01.2023 | RESEARCH

Bowel function at preschool and early childhood age in children with long-segment Hirschsprung disease

verfasst von: Changgui Lu, Shiwen Pan, Xinyi Hua, Weiwei Jiang, Weibing Tang

Erschienen in: European Journal of Pediatrics | Ausgabe 3/2023

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Abstract

Bowel dysfunction has been proven to be the most typical complication of long-segment Hirschsprung disease (LSHD). Additionally, bowel dysfunction in preschool and early childhood should be properly assessed, because it may result in persistent bowel dysfunction, social issues, and depression in adolescence and adulthood. This study evaluated bowel function during preschool and early childhood in patients with LSHD. A cross-sectional bowel function score (BFS) questionnaire survey was conducted on 270 infants with short-segment Hirschsprung disease (SSHD) and LSHD who underwent pull-through (PT) between January 2014 and December 2019 at the Children’s Hospital of Nanjing Medical University. One hundred ninety patients who consented to the survey were asked to complete the questionnaire and were divided into two groups: the LSHD group (n = 42) and the SSHD group (n = 148). Bowel function outcomes were assessed by using a questionnaire. The total BFS score in the LSHD group was significantly lower than that in the SSHD group (15.0 [14.0, 17.0] vs. 18.0 [16.0, 19.0], p < 0.05) and did not improve with age (p > 0.05). Independent BFS items with lower scores were discovered in LSHD than in SSHD, including the capacity to hold back defecation, feeling/reporting the urge to defecate, frequency, soiling, and accidents (all p < 0.05). After subgroup analysis of follow-up age (3–5 years, 5 ~ 7 years, and > 7 years), there was no difference between the score of ability to hold back defecation over 7 years old and feeling/reporting the urge to defecate over 5 years old (all p > 0.05). The frequency and soiling scores were lower in the LSHD group than in the SSHD group over 5 years of age (all p < 0.05). Fortunately, there was no difference in constipation scores and social problems between the LSHD and SSHD groups in every sub-follow-up group (all p > 0.05).
  Conclusion: Overall, bowel function at preschool and early childhood age was poorer in LSHD than in SSHD and did not improve with age. However, the ability to hold back defecation and feeling/reporting the urge to defecate with age in LSHD may be similar to those in SSHD. Simultaneously, the frequency, soiling, and number of accidents were consistently worse in the LSHD group.
  Trial registration: This study was retrospectively registered in the ClinicalTrials database.gov (NCT05461924) in August 2022.
What is Known:
• Bowel function in SSHD is satisfactory or near-normal, and the general consensus is that the longer the aganglionic segment, the worse the bowel function after surgery.
• Bowel functional outcomes of LSHD were not defined, and the limited reports of bowel functional outcomes compared with SSHD were extensively varied. Bowel dysfunction at preschool and early childhood may lead to persistent bowel dysfunction, social problems, and depression in adolescence or adulthood.
What is New:
• Bowel function in preschool and early childhood in patients with LSHD has not been defined and should be thoroughly assessed in a larger group of patients with precise definitions of incontinence or soiling.
• The present study was primarily designed to evaluate bowel function at preschool and early childhood age in LSHD compared with SSHD in a relatively large number of HD cases using BFS.
Literatur
1.
Zurück zum Zitat Lu C, Hou G, Liu C (2017) Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates: a multicenter study. J Pediatr Surg 52(7):1102–1107CrossRefPubMed Lu C, Hou G, Liu C (2017) Single-stage transanal endorectal pull-through procedure for correction of Hirschsprung disease in neonates and nonneonates: a multicenter study. J Pediatr Surg 52(7):1102–1107CrossRefPubMed
2.
Zurück zum Zitat Fang Y, Bai J, Zhang B (2020) Laparoscopic Soave procedure for long-segment Hirschsprung’s disease - single-center experience. Videosurgery and Other Miniinvasive Techniques 15(1):234–238CrossRefPubMed Fang Y, Bai J, Zhang B (2020) Laparoscopic Soave procedure for long-segment Hirschsprung’s disease - single-center experience. Videosurgery and Other Miniinvasive Techniques 15(1):234–238CrossRefPubMed
3.
Zurück zum Zitat Kawaguchi AL, Guner YS, Somme S (2021) Management and outcomes for long-segment Hirschsprung disease: a systematic review from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg 56(9):1513–1523CrossRefPubMedPubMedCentral Kawaguchi AL, Guner YS, Somme S (2021) Management and outcomes for long-segment Hirschsprung disease: a systematic review from the APSA Outcomes and Evidence Based Practice Committee. J Pediatr Surg 56(9):1513–1523CrossRefPubMedPubMedCentral
4.
Zurück zum Zitat Kim SH, Cho YH, Kim HY (2021) Assessment of defecation function beyond infantile period for transanal single-stage endorectal pull-through in Hirschsprung disease. Annals of Surgical Treatment and Research 101(4):231–239CrossRefPubMedPubMedCentral Kim SH, Cho YH, Kim HY (2021) Assessment of defecation function beyond infantile period for transanal single-stage endorectal pull-through in Hirschsprung disease. Annals of Surgical Treatment and Research 101(4):231–239CrossRefPubMedPubMedCentral
5.
Zurück zum Zitat Bystrom C, Ostlund S, Hoff N (2021) Evaluation of bowel function, urinary tract function, and quality of life after transanal endorectal pull-through surgery for Hirschsprung’s disease. Eur J Pediatr Surg 31(1):40–48CrossRefPubMed Bystrom C, Ostlund S, Hoff N (2021) Evaluation of bowel function, urinary tract function, and quality of life after transanal endorectal pull-through surgery for Hirschsprung’s disease. Eur J Pediatr Surg 31(1):40–48CrossRefPubMed
6.
Zurück zum Zitat Verkuijl SJ, Meinds RJ, van der Steeg A (2022) Functional outcomes after surgery for total colonic, long-segment, versus rectosigmoid segment Hirschsprung disease. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION 74(3):348–354CrossRef Verkuijl SJ, Meinds RJ, van der Steeg A (2022) Functional outcomes after surgery for total colonic, long-segment, versus rectosigmoid segment Hirschsprung disease. JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION 74(3):348–354CrossRef
7.
Zurück zum Zitat Stenstrom P, Brautigam M, Borg H (2017) Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis. J Pediatr Surg 52(8):1302–1307CrossRefPubMed Stenstrom P, Brautigam M, Borg H (2017) Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis. J Pediatr Surg 52(8):1302–1307CrossRefPubMed
8.
Zurück zum Zitat Chung P, Wong K, Tam P (2018) Are all patients with short segment Hirschsprung’s disease equal? A retrospective multicenter study. Pediatr Surg Int 34(1):47–53CrossRefPubMed Chung P, Wong K, Tam P (2018) Are all patients with short segment Hirschsprung’s disease equal? A retrospective multicenter study. Pediatr Surg Int 34(1):47–53CrossRefPubMed
9.
Zurück zum Zitat Chan K, Lee KH, Wong H (2021) Long-term results of one-stage laparoscopic-assisted endorectal pull-through for rectosigmoid Hirschsprung’s disease in patients aged above 5 years. J Laparoendosc Adv Surg Tech 31(2):225–229CrossRef Chan K, Lee KH, Wong H (2021) Long-term results of one-stage laparoscopic-assisted endorectal pull-through for rectosigmoid Hirschsprung’s disease in patients aged above 5 years. J Laparoendosc Adv Surg Tech 31(2):225–229CrossRef
10.
Zurück zum Zitat Townley OG, Lindley RM, Cohen MC (2020) 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 55(2):273–277CrossRefPubMed Townley OG, Lindley RM, Cohen MC (2020) 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 55(2):273–277CrossRefPubMed
11.
Zurück zum Zitat Fosby MV, Stensrud KJ, Bjornland K (2020) Bowel function after transanal endorectal pull-through for Hirschsprung disease - does outcome improve over time? J Pediatr Surg 55(11):2375–2378CrossRefPubMed Fosby MV, Stensrud KJ, Bjornland K (2020) Bowel function after transanal endorectal pull-through for Hirschsprung disease - does outcome improve over time? J Pediatr Surg 55(11):2375–2378CrossRefPubMed
12.
Zurück zum Zitat Dai Y, Deng Y, Lin Y (2020) Long-term outcomes and quality of life of patients with Hirschsprung disease: a systematic review and meta-analysis. BMC Gastroenterol 20(1):67CrossRefPubMedPubMedCentral Dai Y, Deng Y, Lin Y (2020) Long-term outcomes and quality of life of patients with Hirschsprung disease: a systematic review and meta-analysis. BMC Gastroenterol 20(1):67CrossRefPubMedPubMedCentral
13.
Zurück zum Zitat Drissi F, Meurette G, Baayen C (2019) Long-term outcome of Hirschsprung disease: impact on quality of life and social condition at adult age. Dis Colon Rectum 62(6):727–732CrossRefPubMed Drissi F, Meurette G, Baayen C (2019) Long-term outcome of Hirschsprung disease: impact on quality of life and social condition at adult age. Dis Colon Rectum 62(6):727–732CrossRefPubMed
14.
Zurück zum Zitat Meinds RJ, van der Steeg A, Sloots C (2019) Long-term functional outcomes and quality of life in patients with Hirschsprung’s disease. Br J Surg 106(4):499–507CrossRefPubMedPubMedCentral Meinds RJ, van der Steeg A, Sloots C (2019) Long-term functional outcomes and quality of life in patients with Hirschsprung’s disease. Br J Surg 106(4):499–507CrossRefPubMedPubMedCentral
15.
Zurück zum Zitat Tran VQ, Mahler T, Dassonville M (2018) Long-term outcomes and quality of life in patients after soave pull-through operation for Hirschsprung’s disease: an observational retrospective study. Eur J Pediatr Surg 28(5):445–454CrossRefPubMed Tran VQ, Mahler T, Dassonville M (2018) Long-term outcomes and quality of life in patients after soave pull-through operation for Hirschsprung’s disease: an observational retrospective study. Eur J Pediatr Surg 28(5):445–454CrossRefPubMed
16.
Zurück zum Zitat Kyrklund K, Neuvonen MI, Pakarinen MP (2018) Social morbidity in relation to bowel functional outcomes and quality of life in anorectal malformations and Hirschsprung’s disease. Eur J Pediatr Surg 28(6):522–528CrossRefPubMed Kyrklund K, Neuvonen MI, Pakarinen MP (2018) Social morbidity in relation to bowel functional outcomes and quality of life in anorectal malformations and Hirschsprung’s disease. Eur J Pediatr Surg 28(6):522–528CrossRefPubMed
17.
Zurück zum Zitat Peng C, Tan SS, Pang W (2021) Rectourethral and rectovesical fistula as serious and rare complications after Hirschsprung disease operation: experience in seven patients. J Pediatr Surg 56(2):263–268CrossRefPubMed Peng C, Tan SS, Pang W (2021) Rectourethral and rectovesical fistula as serious and rare complications after Hirschsprung disease operation: experience in seven patients. J Pediatr Surg 56(2):263–268CrossRefPubMed
18.
Zurück zum Zitat Jiao C, Zhuansun D, He Y (2022) Transanal full-thickness pull-through approach in the treatment of anastomotic leakage after operation for Hirschsprung disease. Pediatr Surg Int 38(9):1263–1271CrossRefPubMed Jiao C, Zhuansun D, He Y (2022) Transanal full-thickness pull-through approach in the treatment of anastomotic leakage after operation for Hirschsprung disease. Pediatr Surg Int 38(9):1263–1271CrossRefPubMed
19.
Zurück zum Zitat Oyania F, Ogwal A, Nimanya S (2020) Long term bowel function after repair of anorectal malformations in Uganda. J Pediatr Surg 55(7):1400–1404CrossRefPubMed Oyania F, Ogwal A, Nimanya S (2020) Long term bowel function after repair of anorectal malformations in Uganda. J Pediatr Surg 55(7):1400–1404CrossRefPubMed
20.
Zurück zum Zitat van der Steeg H, van Rooij I, Iacobelli BD (2022) Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study. J Pediatr Surg 57(9):89–96CrossRefPubMed van der Steeg H, van Rooij I, Iacobelli BD (2022) Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula: An ARM-Net consortium study. J Pediatr Surg 57(9):89–96CrossRefPubMed
21.
Zurück zum Zitat Verkuijl SJ, Jonker JE, Trzpis M (2021) Functional outcomes of surgery for colon cancer: a systematic review and meta-analysis. EJSO 47(5):960–969CrossRefPubMed Verkuijl SJ, Jonker JE, Trzpis M (2021) Functional outcomes of surgery for colon cancer: a systematic review and meta-analysis. EJSO 47(5):960–969CrossRefPubMed
22.
Zurück zum Zitat Magdeburg J, Glatz N, Post S (2016) Long-term functional outcome of colonic resections: how much does faecal impairment influence quality of life? Colorectal Dis 18(11):O405–O413CrossRefPubMed Magdeburg J, Glatz N, Post S (2016) Long-term functional outcome of colonic resections: how much does faecal impairment influence quality of life? Colorectal Dis 18(11):O405–O413CrossRefPubMed
23.
Zurück zum Zitat Hoel AT, Tofft L, Bjornland K (2021) Reaching adulthood with Hirschsprung’s disease: patient experiences and recommendations for transitional care. J Pediatr Surg 56(2):257–262CrossRefPubMed Hoel AT, Tofft L, Bjornland K (2021) Reaching adulthood with Hirschsprung’s disease: patient experiences and recommendations for transitional care. J Pediatr Surg 56(2):257–262CrossRefPubMed
24.
Zurück zum Zitat Roorda D, Verkuij SJ, Derikx J (2022) Did age at surgery influence outcome in patients with Hirschsprung disease? A nationwide cohort study in the Netherlands. Journal Of Pediatric Gastroentology And Nutrition 75(4):431–437CrossRef Roorda D, Verkuij SJ, Derikx J (2022) Did age at surgery influence outcome in patients with Hirschsprung disease? A nationwide cohort study in the Netherlands. Journal Of Pediatric Gastroentology And Nutrition 75(4):431–437CrossRef
25.
Zurück zum Zitat Sakurai T, Tanaka H, Endo N (2021) Predictive factors for the development of postoperative Hirschsprung-associated enterocolitis in children operated during infancy. Pediatr Surg Int 37(2):275–280CrossRefPubMed Sakurai T, Tanaka H, Endo N (2021) Predictive factors for the development of postoperative Hirschsprung-associated enterocolitis in children operated during infancy. Pediatr Surg Int 37(2):275–280CrossRefPubMed
26.
27.
Zurück zum Zitat Yuan Y, Xu M, Yang H (2021) The efficacy of biofeedback therapy for the treatment of fecal incontinence after soave procedure in children for Hirschsprung’s disease. Front Pediatr 9:638120CrossRefPubMedPubMedCentral Yuan Y, Xu M, Yang H (2021) The efficacy of biofeedback therapy for the treatment of fecal incontinence after soave procedure in children for Hirschsprung’s disease. Front Pediatr 9:638120CrossRefPubMedPubMedCentral
28.
Zurück zum Zitat Kyrklund K, Koivusalo A, Rintala RJ (2012) Evaluation of bowel function and fecal continence in 594 Finnish individuals aged 4 to 26 years. Dis Colon Rectum 55(6):671–676CrossRefPubMed Kyrklund K, Koivusalo A, Rintala RJ (2012) Evaluation of bowel function and fecal continence in 594 Finnish individuals aged 4 to 26 years. Dis Colon Rectum 55(6):671–676CrossRefPubMed
30.
Zurück zum Zitat Choi BJ, Kwon W, Baek SH (2020) Single-port laparoscopic Deloyers procedure for tension-free anastomosis after extended left colectomy or subtotal colectomy: a 6-patient case series. Medicine 99(31):e21421CrossRefPubMedPubMedCentral Choi BJ, Kwon W, Baek SH (2020) Single-port laparoscopic Deloyers procedure for tension-free anastomosis after extended left colectomy or subtotal colectomy: a 6-patient case series. Medicine 99(31):e21421CrossRefPubMedPubMedCentral
31.
Zurück zum Zitat Saadai P, Trappey AF, Goldstein AM (2019) Guidelines for the management of postoperative soiling in children with Hirschsprung disease. Pediatr Surg Int 35(8):829–834CrossRefPubMed Saadai P, Trappey AF, Goldstein AM (2019) Guidelines for the management of postoperative soiling in children with Hirschsprung disease. Pediatr Surg Int 35(8):829–834CrossRefPubMed
32.
Zurück zum Zitat Saad SA, Elseed M, AbouZeid AA (2020) Histopathological perspective of the pulled-through colon in Hirschsprung disease: impact on clinical outcome. J Pediatr Surg 55(9):1829–1833CrossRefPubMed Saad SA, Elseed M, AbouZeid AA (2020) Histopathological perspective of the pulled-through colon in Hirschsprung disease: impact on clinical outcome. J Pediatr Surg 55(9):1829–1833CrossRefPubMed
33.
Zurück zum Zitat Brooks LA, Fowler KL, Veras LV (2020) Resection margin histology may predict intermediate-term outcomes in children with rectosigmoid Hirschsprung disease. Pediatr Surg Int 36(8):875–882CrossRefPubMedPubMedCentral Brooks LA, Fowler KL, Veras LV (2020) Resection margin histology may predict intermediate-term outcomes in children with rectosigmoid Hirschsprung disease. Pediatr Surg Int 36(8):875–882CrossRefPubMedPubMedCentral
34.
Zurück zum Zitat Coyle D, O’Donnell AM, Tomuschat C (2019) The extent of the transition zone in Hirschsprung disease. J Pediatr Surg 54(11):2318–2324CrossRefPubMed Coyle D, O’Donnell AM, Tomuschat C (2019) The extent of the transition zone in Hirschsprung disease. J Pediatr Surg 54(11):2318–2324CrossRefPubMed
35.
Zurück zum Zitat Kapur RP, Smith C, Ambartsumyan L (2020) Postoperative pullthrough obstruction in Hirschsprung disease: etiologies and diagnosis. Pediatric Aand Developmental Pathology 23(1):40–59CrossRef Kapur RP, Smith C, Ambartsumyan L (2020) Postoperative pullthrough obstruction in Hirschsprung disease: etiologies and diagnosis. Pediatric Aand Developmental Pathology 23(1):40–59CrossRef
36.
Zurück zum Zitat Langer JC, Rollins MD, Levitt M (2017) Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 33(5):523–526CrossRefPubMed Langer JC, Rollins MD, Levitt M (2017) Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 33(5):523–526CrossRefPubMed
37.
Zurück zum Zitat Ghosh DN, Liu Y, Cass DT (2017) Transition zone pull-through in Hirschsprung’s disease: a tertiary hospital experience. ANZ J Surg 87(10):780–783CrossRefPubMed Ghosh DN, Liu Y, Cass DT (2017) Transition zone pull-through in Hirschsprung’s disease: a tertiary hospital experience. ANZ J Surg 87(10):780–783CrossRefPubMed
38.
Zurück zum Zitat Fernandez IM, Sanchez MJ, Martinez CI (2014) Quality of life and long term results in Hirschsprung’s disease]. Cir Pediatr 27(3):117–124 Fernandez IM, Sanchez MJ, Martinez CI (2014) Quality of life and long term results in Hirschsprung’s disease]. Cir Pediatr 27(3):117–124
39.
Zurück zum Zitat Xiong X, Chen X, Wang G (2015) Long term quality of life in patients with Hirschsprung’s disease who underwent heart-shaped anastomosis during childhood: a twenty-year follow-up in China. J Pediatr Surg 50(12):2044–2047CrossRefPubMed Xiong X, Chen X, Wang G (2015) Long term quality of life in patients with Hirschsprung’s disease who underwent heart-shaped anastomosis during childhood: a twenty-year follow-up in China. J Pediatr Surg 50(12):2044–2047CrossRefPubMed
Metadaten
Titel
Bowel function at preschool and early childhood age in children with long-segment Hirschsprung disease
verfasst von
Changgui Lu
Shiwen Pan
Xinyi Hua
Weiwei Jiang
Weibing Tang
Publikationsdatum
12.01.2023
Verlag
Springer Berlin Heidelberg
Erschienen in
European Journal of Pediatrics / Ausgabe 3/2023
Print ISSN: 0340-6199
Elektronische ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-023-04814-7

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