Outcomes at five to eight years of age for children with Hirschsprung’s disease

Abstract

ObjectiveThis study describes core outcomes of Hirschsprung’s disease (HD) in a UK-wide cohort of primary school-aged children.DesignA prospective cohort study conducted from 1 October 2010 to 30 September 2012. Outcomes data were collected from parents and clinicians when children were 5–8 years of age, and combined with data collected at birth, and 28 days and 1 year post diagnosis.SettingAll 28 UK and Irish paediatric surgical centres.ParticipantsChildren with histologically proven HD diagnosed at <6 months of age.Main outcome measuresNETS1HD core outcomes.ResultsData were returned for 239 (78%) of 305 children. Twelve children (5%) died prior to 5 years of age.Of the 227 surviving children, 30 (13%) had a stoma and 21 (9%) were incontinent of urine. Of the 197 children without a stoma, 155 (79%) maintained bowel movements without enemas/washouts, while 124 (63%) reported faecal incontinence. Of the 214 surviving children who had undergone a pull-through operation, 95 (44%) underwent ≥1 unplanned reoperation. 89 unplanned reoperations (27%) were major/complex.Of the 83 children with returned PedsQL scores, 37 (49%) had quality of life scores, and 31 (42%) had psychological well-being scores, that were ≥1 SD lower than the reference population mean for children without HD.ConclusionThis study gives a realistic picture of population outcomes of HD in primary school-aged children in the UK/Ireland. The high rates of faecal incontinence, unplanned procedures and low quality of life scores are sobering. Ensuring clinicians address the bladder, bowel and psychological problems experienced by children should be a priority.

Late Diagnosis of Hirschsprung's Disease: Definition and Implication on Core Outcomes

“…Only one-third of LDHD had occasional (once or twice a week, grade I) fecal incontinence and none achieved full continence. This contrasted the Norwegian report of 27% incontinence rate in a cohort comparable in demographics and operative approaches (mainly Soave) as ours, except for significantly shorter follow-up in their group with a median age of 3 years (5 months-12 years) 6 compared with ours with a median age of 5.6 years (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) in this study. This might be enough to make a difference but remains untested.…”

“…Our group contributed data to the British Association of Pediatric Surgeons Congenital Anomaly Surveillance System (BAPS-CASS) which reported the NETS 1HD study to determine core outcomes of school-going children with HD. 9 Our institutional management of any newly diagnosed HD involved immediate surgical consult followed by rectal washout if a defunctioning stoma was not in situ. Older children who could not tolerate rectal washout would be offered aggressive laxative therapy in the form of stool softeners, stimulant, and enema to maintain effective daily bowel opening.…”

Using stated-preferences methods to develop a summary metric to determine successful treatment of children with a surgical condition: a study protocol

“…These recruitment strategies have been successfully used in previous quantitative and qualitative studies. 8 10–12 41 …”

“…First, we reviewed the epidemiological data available for each of the attributes to guide the range that could be presented to participants. Existing large-scale cohort studies describing the outcomes for children with a range of conditions treated under the specialised surgery in children commissioning, and affecting different regions of the body, modes of presentation and urgencies of intervention were reviewed 2 4–6 8 25–33. A researcher (BA) extracted point estimates and associated measures of uncertainty for each of the attributes.…”

“…Many of the conditions falling under the remit of specialised surgery in children commissioning affect only a few hundred children in England and Scotland each year, and what little is known about these children’s long-term health and well-being suggests that even after treatment, they have significant ongoing healthcare needs 2–8. Widespread variation in the management of children with these conditions currently occurs,2–7 but due to the rarity of the conditions, it has not to date been possible to identify how much of this variation is unwarranted or associated with variation in outcome.…”