Dataset Information

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

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.

SUBMITTER: Verkuijl SJ

PROVIDER: S-EPMC8860201 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

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

Verkuijl Sanne J SJ Meinds Rob J RJ van der Steeg Alida F W AFW van Gemert Wim G WG de Blaauw Ivo I Witvliet Marieke J MJ Sloots Cornelius E J CEJ van Heurn Ernst E Vermeulen Karin M KM Trzpis Monika M Broens Paul M A PMA

Journal of pediatric gastroenterology and nutrition 20220301 3

<h4>Objectives</h4>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.<h4>Methods</h4>In this nationwide, cross-sectional study participants with proven Hirschsprung disease received the Defecation and Fecal Continence que ...[more]

PMID: 34775429

Similar Datasets

Project description:BackgroundTotal colonic aganglionosis (TCA) is a rare variant of Hirschsprung's disease occurring in 3-10% of the cases. Only few studies reported the long-term clinical and metabolic outcomes of patients with TCA. The aim of this study was to evaluate the functional and metabolic long-term outcomes of children undergoing surgical treatment for TCA.MethodsA 15-year retrospective study was performed. Blood chemistry tests and stool analysis performed at the last follow-up visit were recorded. Height and weight development were assessed using the corresponding percentiles for age. Faecal continence and quality of life were evaluated using a detailed questionnaire.ResultsEleven patients were included in the study. The median age at surgery was 6 months (range: 3-72 months). After histological confirmation, all patients underwent a total colectomy. Ileoanal anastomosis (n = 6), ileorectal anastomosis (n = 1), J-pouch (n = 1) and Duhamel procedure (n = 3) were performed. Temporary ileostomy was closed after a median of 8 weeks in 10/11 patients. After a median follow-up of 78 months (range: 27-199 months), all evaluated patients were continent. Height and weight were appropriate for age in only 5 patients. Vitamin B12 and folic acid serum levels were normal in all examined patients. Ten patients had normal hemoglobin serum levels. Seven patients had low transferrin saturation in serum. Hemoccult tests were negative in all examined patients. Despite complex postoperative courses in some cases, patients and parents showed good overall satisfaction in terms of quality of life.ConclusionThe majority of patients reported a good quality of life. This can result from the adaptation of the patients to certain disease states. The failure to thrive seems to be related with the extent of aganglionosis. The inclusion of these patients in interdisciplinary long-term follow-up care, in which pediatric surgeons, gastroenterologists, and dieticians are involved, is essential.

Project description:BackgroundPrevious studies have shown that the anesthetic technique may influence long-term outcomes after cancer surgery. However, the association between the anesthetic technique and long-term oncological outcomes after oral cancer surgery remains unclear. Therefore, we conducted this study to address this gap.MethodsWe reviewed the electronic medical records of patients who underwent elective oral cancer surgery between January 2014 and December 2015. The patients were grouped based on the anesthesia maintenance: either propofol or sevoflurane. Propensity score matching in a 1:1 ratio was performed to deal with the potential confounding effects of baseline characteristics. Univariate and multivariate Cox regression analyses were performed to compare hazard ratios (HRs) and identify the risk factors for death and recurrence. Survival analysis was performed using the Kaplan-Meier method, and survival curves were constructed from the date of surgery to death.ResultsIn total, 1347 patients were eligible for analysis, with 343 and 1004 patients in the propofol and sevoflurane groups, respectively. After propensity score matching, 302 patients remained in each group. Kaplan-Meier survival curves demonstrated the 5-year overall and recurrence-free survival rates of 59.3% and 56.0% and 62.7% and 56.5% in the propofol and sevoflurane groups, respectively. There was no significant difference in overall survival or recurrence-free survival between the groups. The multivariate Cox analysis verified this conclusion with HRs of 1.10 and 1.11 for overall survival and recurrence-free survival, respectively, in the sevoflurane group. Older age, advanced tumor-node-metastasis (TNM) stage, and American Society of Anesthesiologists class III were associated with poor overall survival. Patients with advanced TNM stage and poorly differentiated squamous cell carcinoma had a higher recurrence risk than their counterparts.ConclusionThe overall and recurrence-free survival rates were similar between propofol-based intravenous anesthesia and sevoflurane volatile anesthesia in patients who underwent oral cancer surgery.

Dataset Information

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

Objectives

Methods

Results

Conclusions

Publications

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

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets