Long-term outcomes of Hirschsprung's disease

2012 ◽  
Vol 21 (4) ◽  
pp. 336-343 ◽  
Author(s):  
Risto J. Rintala ◽  
Mikko P. Pakarinen
2017 ◽  
Vol 28 (05) ◽  
pp. 445-454 ◽  
Author(s):  
Tania Mahler ◽  
Martine Dassonville ◽  
Dinh Truong ◽  
Annie Robert ◽  
Philippe Goyens ◽  
...  

Introduction Patients after pull-through operation for Hirschsprung's disease (HD) are at high risk of defecation disorders. This study aimed at investigating their long-term outcomes and quality of life (QoL) in comparison with controls. Patients and Methods Patients older than 5 years operated on for HD were interviewed to complete detailed questionnaires on bowel function. Patients without neurologic impairment were enrolled in a QoL survey to compare with controls matched for sex and age and selected randomly from the general population using sampling set in a ratio of four controls to one case of HD. Results In total, 53 operated patients were enrolled. Mean age of the patients was 16 ± 8 years, with 68% boys. Rectosigmoid aganglionosis was the most seen form of HD in 38 (72%) cases. Open Soave was performed in 40 (75.5%) cases, and minimally invasive surgery Soave (MIS Soave) in 13 (24.5%) cases. At investigation, prevalence of fecal incontinence and constipation were 22.6 and 13.2%, respectively. Regarding QoL survey, 45 patients and 180 controls were enrolled, excluding 8 patients with neurologic impairment. Thirty-seven (82.2%) patients were classified as having a good QoL (score ≥ 9 points); whereas six had a fair QoL (5–8 points) and two had a poor QoL (< 5 points). QoL score in the cases and the controls were 10.2 ± 2.5 and 11.9 ± 0.4 points, respectively. Long aganglionosis form of HD was significantly associated with a low QoL (score < 8 points), adjusted odds ratio = 9, 95% confidence interval [1.3; 64.1] (p < 0.05). In subscales analyses, the prevalence of each dimension including fecal continence, school absenteeism, unhappiness or anxiety, food restriction, and peer rejection was significantly higher in operated patients than in controls (p <0.001). Conclusion Although the QoL of patients operated on for HD in general was with good outcomes, fecal incontinence and constipation still are problematic issues and challenges in a high percentage of patients. Therefore, a long-term and multidisciplinary follow-up is essentially required for these patients.


2005 ◽  
Vol 22 (2) ◽  
pp. 179-181 ◽  
Author(s):  
Antonino Morabito ◽  
Anupam Lall ◽  
Sobbia Gull ◽  
Amar Mohee ◽  
Adrian Bianchi

2019 ◽  
Vol 229 (4) ◽  
pp. e170
Author(s):  
Shun Onishi ◽  
Kazuhiko Nakame ◽  
Masakazu Murakami ◽  
Toshio Harumatsu ◽  
Seiro Machigashira ◽  
...  

Endoscopy ◽  
2020 ◽  
Author(s):  
Amol Bapaye ◽  
Parag Dashatwar ◽  
Vishnu Biradar ◽  
Shital Biradar ◽  
Rajendra Pujari

Introduction Hirschsprung’s disease (HSCR) is congenital aganglionosis affecting hindgut and presents with constipation. Surgical pull-through is current standard but has morbidity. Per-rectal endoscopic myotomy (PREM) is a novel third space endoscopy technique for treating SS-HSCR. Methods Retrospective series of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, anorectal manometry and treated by PREM. Aganglionic segment mapped pre-PREM. PREM performed using third space endoscopy principles. Objective – to compare stool frequency and unit laxative (UL) usage pre and post-PREM. Results N = 9; duration 4-years. Mean age – 7.5 (± 5.2) years; 7 males. Mean aganglionic segment length – 6.3 (± 4.4) cm. Mean procedure time – 96.1 (±37.9) minutes. Mean LOS – 2.5 (±0.7) days. Median follow-up –17 months (9 – 58). Stool frequency – pre – 1/4.4 (±1.5) vs. post – 1/1.2 (±0.4) days (p = 0.0004). Mean UL usage – pre – 5.4 (±4.9) vs. post – 0.4 (±0.7) units (p = 0.0002). No laxatives in 6 (66%). Single AE (anal stenosis) – dilatation. Conclusions PREM is a safe and effective minimally invasive procedure for SS-HSCR and provides long-term response.


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