Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease

Sanne J Verkuijl; Rob J Meinds; Alida F W van der Steeg; Wim G van Gemert; Ivo de Blaauw; Marieke J Witvliet; Cornelius E J Sloots; Ernst van Heurn; Karin M Vermeulen; Monika Trzpis; Paul M A Broens

doi:10.1097/MPG.0000000000003355

Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease

Sanne J Verkuijl^*, Rob J Meinds, Alida F W van der Steeg, Wim G van Gemert, Ivo de Blaauw, Marieke J Witvliet, Cornelius E J Sloots, Ernst van Heurn, Karin M Vermeulen, Monika Trzpis, Paul M A Broens

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

OBJECTIVES: Knowledge on long-term outcomes in patients with Hirschsprung's 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's 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 (OR = 6.00, 95% 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.

An infographic is available for this article at:http://links.lww.com/MPG/C598.

Original language	English
Pages (from-to)	348-354
Number of pages	7
Journal	Journal of Pediatric Gastroenterology and Nutrition
Volume	74
Issue number	3
Early online date	12 Nov 2021
DOIs	https://doi.org/10.1097/MPG.0000000000003355
Publication status	Published - Mar 2022

Keywords

ADOLESCENTS
AGANGLIONOSIS
FOLLOW-UP
HEALTH
MANAGEMENT
PULL-THROUGH
QUALITY-OF-LIFE
TERM BOWEL FUNCTION
aganglionosis
constipation
incontinence

Access to Document

10.1097/MPG.0000000000003355Licence: CC BY

Cite this

Verkuijl, S. J., Meinds, R. J., van der Steeg, A. F. W., van Gemert, W. G., de Blaauw, I., Witvliet, M. J., Sloots, C. E. J., van Heurn, E., Vermeulen, K. M., Trzpis, M., & Broens, P. M. A. (2022). Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease. Journal of Pediatric Gastroenterology and Nutrition, 74(3), 348-354. https://doi.org/10.1097/MPG.0000000000003355

@article{eafb02e05e2d4939b762a90039a1e281,

title = "Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease",

abstract = "OBJECTIVES: Knowledge on long-term outcomes in patients with Hirschsprung's 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's 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 (OR = 6.00, 95% 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.An infographic is available for this article at:http://links.lww.com/MPG/C598.",

keywords = "ADOLESCENTS, AGANGLIONOSIS, FOLLOW-UP, HEALTH, MANAGEMENT, PULL-THROUGH, QUALITY-OF-LIFE, TERM BOWEL FUNCTION, aganglionosis, constipation, incontinence",

author = "Verkuijl, {Sanne J} and Meinds, {Rob J} and {van der Steeg}, {Alida F W} and {van Gemert}, {Wim G} and {de Blaauw}, Ivo and Witvliet, {Marieke J} and Sloots, {Cornelius E J} and {van Heurn}, Ernst and Vermeulen, {Karin M} and Monika Trzpis and Broens, {Paul M A}",

note = "Copyright {\textcopyright} 2021 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology.",

year = "2022",

month = mar,

doi = "10.1097/MPG.0000000000003355",

language = "English",

volume = "74",

pages = "348--354",

journal = "Journal of Pediatric Gastroenterology and Nutrition",

issn = "0277-2116",

publisher = "John Wiley & Sons Inc.",

number = "3",

}

Verkuijl, SJ, Meinds, RJ, van der Steeg, AFW, van Gemert, WG, de Blaauw, I, Witvliet, MJ, Sloots, CEJ, van Heurn, E, Vermeulen, KM, Trzpis, M & Broens, PMA 2022, 'Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease', Journal of Pediatric Gastroenterology and Nutrition, vol. 74, no. 3, pp. 348-354. https://doi.org/10.1097/MPG.0000000000003355

TY - JOUR

T1 - Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease

AU - Verkuijl, Sanne J

AU - Meinds, Rob J

AU - van der Steeg, Alida F W

AU - van Gemert, Wim G

AU - de Blaauw, Ivo

AU - Witvliet, Marieke J

AU - Sloots, Cornelius E J

AU - van Heurn, Ernst

AU - Vermeulen, Karin M

AU - Trzpis, Monika

AU - Broens, Paul M A

PY - 2022/3

Y1 - 2022/3

N2 - OBJECTIVES: Knowledge on long-term outcomes in patients with Hirschsprung's 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's 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 (OR = 6.00, 95% 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.An infographic is available for this article at:http://links.lww.com/MPG/C598.

AB - OBJECTIVES: Knowledge on long-term outcomes in patients with Hirschsprung's 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's 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 (OR = 6.00, 95% 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.An infographic is available for this article at:http://links.lww.com/MPG/C598.

KW - ADOLESCENTS

KW - AGANGLIONOSIS

KW - FOLLOW-UP

KW - HEALTH

KW - MANAGEMENT

KW - PULL-THROUGH

KW - QUALITY-OF-LIFE

KW - TERM BOWEL FUNCTION

KW - aganglionosis

KW - constipation

KW - incontinence

U2 - 10.1097/MPG.0000000000003355

DO - 10.1097/MPG.0000000000003355

M3 - Article

C2 - 34775429

SN - 0277-2116

VL - 74

SP - 348

EP - 354

JO - Journal of Pediatric Gastroenterology and Nutrition

JF - Journal of Pediatric Gastroenterology and Nutrition

IS - 3

ER -

Functional Outcomes After Surgery for Total Colonic, Long-Segment, Versus Rectosigmoid Segment Hirschsprung's Disease

Abstract

Keywords

Access to Document

Fingerprint

Cite this