Adult outcomes after surgery for Hirschsprung’s disease: Evaluation of bowel function and quality of life

Granström, Anna Löf; Danielson, Johan; Husberg, Britt; Nordenskjöld, Agneta; Wester, Tomas

doi:10.1016/j.jpedsurg.2015.06.014

Cited by 56 publications

(44 citation statements)

References 141 publications

(198 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our outcome relating to QOL supports the findings of Granström et al [12] that HD patients have impaired disease-specific, but normal generic, QOL when compared to age- and sex-matched controls. However, this was in contrast with the study of Neuvonen [19] where adult HD-patients scored lower on an emotional scale than the healthy controls.…”

Section: Discussionsupporting

confidence: 91%

“…The incidence of symptoms consistent with both obstruction and fecal incontinence was higher among HD-patients compared to controls. This complies well with other recent studies of functional outcome of HD in adults compared to age- and sex-matched controls [10–12, 19]. These recent reports challenge the older view that HD has a favorable outcome in adulthood, exemplified by the Sherman report from 1989 [20].…”

Section: Discussionsupporting

confidence: 90%

“…The outcome of HD in adulthood has been reported to be impaired and include both fecal incontinence and obstructive symptoms and the current knowledge has recently been summarized in a review article [9]. How functional outcome in adulthood differs from age-matched controls has, however, been sparsely investigated with only three studies to date [10–12]. All three publications found that functional outcome was worse when compared to controls.…”

Section: Introductionmentioning

confidence: 99%

“…All three publications found that functional outcome was worse when compared to controls. Two of these studies [11, 12] also investigated how bowel function affects the patient’s quality of life (QOL) with the GIQLI-questionnaire. In the publication by Järvi et al [11], it was found that HD patients had marginally lower GIQLI-scores when compared to controls, whereas Granström et al [12] found that HD patients had significantly lower GIQLI-scores.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Controlled outcome of Hirschsprung’s disease beyond adolescence: a single center experience

2018

Self Cite

View full text Add to dashboard Cite

PurposeThe aim of this study was to assess the function and quality of life of Hirschsprung’s Disease (HD) beyond adolescence and relate it to matched controls.MethodsAll 203 patients diagnosed with HD at our department from 1961 to 1995 were identified. 21 had died, 43 had unclear diagnosis and 16 could not be traced. The remaining 123 patients were sent bowel function and SF-36 quality of life questionnaires. 69 patients (mean age 37.8, range 22–58, 13 female) responded and were matched with 138 age and sex-matched controls.ResultsFunction: HD-patients had significantly higher number of bowel movements per week, higher incidence of soiling, urgency, permanent stomas, use of laxatives, enemas and loperamide. HD-patients also scored significantly lower in their satisfaction with their bowel function. There was, however, no significant difference in Miller Incontinence score.QOL: HD-patients reported a significantly higher incidence of negative impact by their bowel function on daily life, social interaction and ability to go on vacation. There were no significant differences in SF-36-scores.ConclusionsBowel function has a lifelong negative impact on the lives of patients with HD. This strongly indicates a need for structured follow-up beyond adolescence.

show abstract

Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 90%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Controlled outcome of Hirschsprung’s disease beyond adolescence: a single center experience

2018

Self Cite

View full text Add to dashboard Cite

show abstract

“…Hypoganglionosis and intestinal neuronal dysplasia are the common allied disorders of HD, and the whole digestive tract might be affected to be disordered in motility, causing refractory anorexia and constipation[14,15], which however slightly affects the QoL in the long term[16]. The tolerance of HD is relatively good due to a usually short aganglionic bowel segment[17]. Unfortunately, the initial surgery in this case only removed most part of the dilated colon, and ignored the silent aganglionic part just adjacent to the structurally-abnormal bowel.…”

Section: Discussionmentioning

confidence: 99%

Reoperation in an adult female with "right-sided" Hirschsprung's disease complicated by refractory hypertension and cough

Wei¹,

Huang²,

Xu³

2016

WJG

View full text Add to dashboard Cite

Hirschsprung’s disease (HD) is an intestinal malformation caused by the innate absence of ganglion cells in the neural plexus of the colorectal wall, and is most common in male infants. It is rare in adult, and is usually left-sided. Herein we reported based on the CARE guidelines a case of a 47-year-old adult female suffering from “right-sided” HD complicated by refractory hypertension and cough. The patient with a history of cesarean section and with digestive unfitness (abdominal pain, distention, and constipation) only since 20 years old had recurrence of HD after initial surgery due to the incomplete removal of the HD-affected bowel based on a diagnosis of “chronic ileus”, leading to the relapse of the digestive symptoms and the emergence of some intractable circulatory and respiratory complications which could be hardly controlled by conservative treatment. During the long interval before coming to our department for help, she had been re-hospitalized for several times with various misdiagnoses and supplied merely with symptomatic treatment which could only achieve temporary symptomatic relief. At her admission to our department, the imaging examinations strongly indicated recurrent HD which was further supported by pathological examinations, and right hemi-colectomy was performed to remove the remnant aganglionic intestinal segment. Intraoperative and postoperative pathology supported the completeness of the definitive resection. Post-operation, the patient’s bowel motility significantly improved, and interestingly, the complications disappeared. For adult patients with long-term constipation combined with cough and hypertension, rare diseases like HD which requires definite surgery and which could be “right-sided” should not be overlooked. It is vital to diagnose and cure HD patients in childhood. Through the comparison of the two surgeries, it is noteworthy that for diagnosed HD, sufficient removal of the non-functional intestine confirmed by intraoperative pathology is essential.

show abstract

Premigratory neural crest stem cells generate enteric neurons populating the mouse colon and regulating peristalsis in tissue-engineered intestine

Yuan

Chen

et al. 2021

Stem Cells Translational Medicine

View full text Add to dashboard Cite

Hirschsprung's disease (HSCR) is a common congenital defect. It occurs when bowel colonization by neural crest-derived enteric nervous system (ENS) precursors is incomplete during the first trimester of pregnancy. Several sources of candidate cells have been previously studied for their capacity to regenerate the ENS, including enteric neural crest stem cells (En-NCSCs) derived from native intestine or those

show abstract

Adult outcomes after surgery for Hirschsprung’s disease: Evaluation of bowel function and quality of life

Cited by 56 publications

References 141 publications

Controlled outcome of Hirschsprung’s disease beyond adolescence: a single center experience

Controlled outcome of Hirschsprung’s disease beyond adolescence: a single center experience

Reoperation in an adult female with "right-sided" Hirschsprung's disease complicated by refractory hypertension and cough

Premigratory neural crest stem cells generate enteric neurons populating the mouse colon and regulating peristalsis in tissue-engineered intestine

Contact Info

Product

Resources

About