Long-term surgical and patient-reported outcomes of Hirschsprung Disease

Davidson, Joseph; Kyrklund, Kristiina; Eaton, Simon; Pakarinen, Mikko P.; Thompson, David; Cross, Kate; Blackburn, Simon; Coppi, Paolo De; Curry, Joe

doi:10.1016/j.jpedsurg.2021.01.043

Cited by 43 publications

(23 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Numbers within each section represent n (%). Numbers with poor outcome include patients with stoma/ACE who are not charted as they are not scored with BFS patients with LD are nearly 10 times more likely to have a poor functional outcome compared to cognitively normal patients according to a multivariate model controlling for age (which we have shown to be factors associated with poorer outcome in the cognitively normal patient population [2]). We also found a high incidence of moderate/severe problems withholding defecation, fecal accidents, soiling, constipation, and HAEC.…”

Section: Discussionmentioning

confidence: 86%

“…The breakdown of the COS and the corresponding instruments are listed in Table 1. The responses of patients with LD were compared to our previously collected data on cognitively normal HSCR patients from the same institution over the same time period [2]. Since the age ranges were wide and age at assessment is a known factor in continence outcomes in both healthy population and HSCR, we described bowel function and urinary function separately for patients ≥ 18 years and < 18 years of age.…”

Section: Methodsmentioning

confidence: 99%

“…cognitively normal patients [2] and included age, sex, redo pullthrough, and segment length: rectosigmoid vs. extended segment (long segment (LS) and total colonic aganglionosis +/− small bowel involvement (TCA)). An additional variable of constipation (requiring laxatives, enema, or ACE) was added to the multivariate analysis for frequent urinary incontinence (weekly or daily symptoms).…”

Section: Methodsmentioning

confidence: 99%

“…Study inclusion flowchart and dropout analysis is shown in Fig. 1 and Table 2; there were no differences between those eligible patients with LD who took part (n = 32) and those who did not respond or declined to take part (n = 18); the dropout analysis for the control group of cognitively normal patients has already been published [2]; however as is illustrated in Tables 2 and 3, most patients in the cohort underwent a Duhamel pull-through, and most had rectosigmoid disease (although this proportion was lower in patients with LD). Incomplete datasets (5/32) were included but accounted in the denominator of the relevant analyses.…”

Section: Outcomes Assessmentmentioning

confidence: 99%

“…Although inferior bowel functional outcomes are recognized associations of learning disability (LD) [1], these have not been specifically quantified in studies of long-term outcomes in HSCR to date. Herein, we describe a continuous cohort of adult and pediatric HSCR patients with LD with reference to cognitively normal HSCR patients [2], completing a core outcome set for these individuals [3]. These novel results provide a basis for counselling patients and families on the likely functional outcomes in this group of HSCR patients into adulthood.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Outcomes in Hirschsprung’s disease with coexisting learning disability

et al. 2021

Self Cite

View full text Add to dashboard Cite

This study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung’s disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala’s BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half of patients surveyed (44–60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0–23]).Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. What is Known:• Hirschsprung’s disease is commonly associated with syndromes or other anomalies with resultant cognitive impairments.• The outcomes for these patients specifically have been poorly described in the literature. What is New:• Objective functional and quality of life surveys demonstrate significant differences from patients without cognitive impairment.• Patients with learning disability Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.

show abstract

Section: Discussionmentioning

confidence: 86%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Outcomes Assessmentmentioning

confidence: 99%