Long-term (> 10 years) bowel function of anorectal malformations: a retrospective single-center study.

PURPOSE: To describe the long-term bowel function of anorectal malformation (ARM) patients and explore the potential influence factors. METHODS: ARM patients with follow-up data > 10 years were included. Cases of cloaca, Currarino syndrome, and VACTERL syndrome were excluded. Rintala score and PedsQL 4.0 were used to assess bowel function score (BFS) and quality of life (QoL). Based on the results, patients were divided into satisfactory group with BFS ≥ 17 and unsatisfactory group with it < 17. Comparisons between the groups were made. RESULTS: Among the 81 patients were 44 males and 37 females. Follow-up time was 138 (126,151) months. 16 (19.75%) patients had associated anomalies. 23 (28.40%) patients had reoperations, and fistula recurrence was the most common reason. BFS of the patients was 20 (18,20). QoL score was 100 (100,100), which correlated positively with BFS (r = 0.648, P < 0.001). The satisfactory and the unsatisfactory groups had 69 and 12 cases, and their BFS were 20 (20,20) and 11 (8,15) respectively, which had statistical difference (P < 0.001). Total QoL score and psycho-social health score of the unsatisfactory group were lower (P < 0.001). Only reoperations were statistically different between the groups (P < 0.001). CONCLUSIONS: Long-term (> 10 years) bowel function of ARM patients is good in this study. Defecation problems have negative impacts on QoL and mainly affects their psycho-social health. Primary anorectoplasty is extremely important. Reoperations, which are most commonly seen in recto-urethral fistula recurrence, adversely affect the outcome.

Long-term bowel function after single-stage transanal endorectal pull-through in neonatal patients with Hirschsprung disease.

BACKGROUND: The aim of this study was to assess long-term outcomes of neonatal patients with Hirschsprung disease (HD) after single-stage transanal endorectal pull-through (TEPT) and to explore the predictive factors contributing to subnormal bowel function. METHODS: Patients aged > 3 years operated for HD with TEPT during neonatal period between 2007 and 2019 answered the bowel function score (BFS) questionnaire. The patients were retrospectively divided into two groups according to whether they had normal bowel function. The clinical variables were compared between the subnormal and normal BFS groups. Univariate and multivariable logistic regression analysis were performed to identify the predictive factors contributing to subnormal bowel function. RESULTS: A total of 160 children (71.7%) were included in this study, with mean follow-up time of 7.3 years (range 3.0-15.1 years). The level of aganglionosis were determined to be the short-segment (124/160, 77.5%), long-segment (33/160, 20.6%), and TCA (3/160, 1.9%). One hundred and thirty-four patients (83.8%) had a BFS ≥ 17, and 26 patients (16.2%) with subnormal bowel function (BFS < 17). Univariate and multivariate logistic regression analysis showed that level of aganglionosis with long-segment or TCA and postoperative hospital stay > 8.5 days were independent risk factors with OR of 3.213 (1.252, 8.246) and 3.813 (1.371, 10.606) for subnormal BFS, respectively. CONCLUSION: Most HD patients who underwent one-stage TEPT in the neonatal period have favorable long-term results, and the level of aganglionosis with long-segment or TCA and long postoperative hospital stay may be closely related to subnormal bowel function.

Comparison of clinical outcomes after total transanal and laparoscopic assisted endorectal pull-through in patients with rectosigmoid Hirschsprung disease.

BACKGROUND: Total transanal (TERPT) and laparoscopic endorectal pull-through (LERPT) are the most common procedures to treat rectosigmoid Hirschsprung's disease (HD). Since few studies have compared the two methods, we aimed to assess clinical outcomes after TERPT and LERPT in this cross-sectional study. METHODS AND PATIENTS: All patients with rectosigmoid HD operated with TERPT and LERPT between 2001 and 2018 were eligible. Peri-operative data were registered from patients' records, and bowel function was assessed according to the Krickenbeck classification. RESULTS: 91/97 (94%) patients were included; 46 operated with TERPT and 45 with LERPT. Bowel function was assessed in 80 patients at median seven (4-17) years. There was no difference in functional outcome between the procedures. Unplanned procedures under general anesthesia were frequent; 28% after TERPT and 49% after LERPT (p = 0.04). 11% of TERPT and 29% of LERPT patients got botulinum toxin injections (p = 0.03). In the TERPT group, patients operated in the neonatal period had poorer outcome (78%) than those operated later (24%) (p = 0.005). No difference in operative time, length of hospital stay, and rate of early and late complications was found between the procedures. CONCLUSION: There was no difference in long-term bowel function in patients with rectosigmoid HD operated with TERPT or LERPT. More LERPT patients had an unplanned procedure under general anesthesia, mostly due to obstructive symptoms. LEVEL OF EVIDENCE: III.

A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease.

OBJECTIVE: Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS: Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS: 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION: A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.