Role of 3D high resolution anorectal manometry compared to conventional technique in management of constipation and fecal incontinence in children.

BACKGROUND: Anorectal manometry is a valuable tool for objective assessment of motility motor and sensory function of the anorectum. The aim of this study was to investigate role of water-perfused (WP) three dimensional high-resolution anorectal manometry (3D-HRARM) compared to WP conventional manometry (CM) in the management of chronic idiopathic constipation (CIC) and faecal incontinence (FI) in children. METHODS: This was a retrospective review of 122 consecutive children, who had WP 8-channel CM or 24 channel 3D-HRARM and endosonography under ketamine anaesthesia from September 2012 to February 2019. All patients had a validated symptom severity score questionnaire ranging from 0 (best) to 65 (worst). Mann-Whitney-U test and Spearman rank test were used and p<0.05 was considered significant. RESULTS: Subjects were divided according to investigation: CM group (n = 75) and 3D-HRAM (n = 47), who were otherwise comparable. Median anal resting pressure was 50 mmHg and rectoanal inhibitory reflex (RAIR) threshold volume was 10mls across the entire cohort. There were no significant differences in resting pressure or RAIR threshold when using conventional or 3D-HRARM. Rectal capacity was significantly higher in the CM group (p = 0.002). Rectal capacity and internal anal sphincter (IAS) thickness positively correlated with symptom severity, duration and patient age. 3D-HRARM provided a more detailed depiction of the anorectal pressure profile. Botulinum toxin was injected into the external anal sphincter (EAS) muscle (n = 75, 61%) and an anterograde colonic enema (ACE) stoma was needed subsequently in 19 (16%) to treat recurrent constipation and soiling symptoms. Rectal biopsy was done in 43(35%) patients, if RAIR was absent or inconclusive. CONCLUSION: CM and 3D-HRARM are comparable in terms of resting pressure and RAIR threshold. 3D-HRAM is safe and provides detailed functional morphology of anal sphincters and it is useful to understand underlying pathophysiology of constipation and faecal incontinence in children and plan further treatment. LEVEL OF EVIDENCE: Level I.

Long term outcome of antegrade colonic enema (ACE) stoma for treatment of constipation and fecal incontinence in children.

AIM: To determine the long term outcome of antegrade colonic enema (ACE) stoma for treatment of chronic constipation and soiling in children. METHOD: We reviewed patients prospectively who underwent formation of ACE stoma after failure of maximum medical treatment of chronic constipation and soiling from September 2008 to October 2020. All patients had anorectal manometry, endosonography and colonic transit study. A validated symptom severity (SS) score questionnaire was used pre operatively and during follow up. The sum of SS score ranged between 0 (best) and 65 (worst). Data was expressed as median(range) and Wilcoxon rank sum test was used and p value < 0.05 was considered significant. RESULTS: 58 patients had formation of ACE stoma, laparoscopic appendicostomy 56, cecostomy 1, and open procedure 1. The main indications for the ACE stoma were chronic functional constipation (FC) 50(86%), anorectal malformations (ARM) 6(11%) and Hirschsprung disease (HD) 2(3%). 41(71%) of patients had previous intrasphincteric botulinum toxin (BT) treatment. Age at operation was 11(range 4-15) years and follow up was 36(3-84) months. 47(81%) patients were continent of stool within three months postoperatively. The preoperative SS score improved from 34/65 (26-47) to 8/65 (4-12) at 12 months and 5/65 (2-11) at 24 months follow up, p ≤ 0.05. OVERALL: complication rate was 15(26%), wound infection 5, granulation tissue 4, stoma prolapse 4, retraction 1, abscess formation and fistula 1. 15 (26%) adolescents had transition of their care to adult services. CONCLUSION: There is a significant improvement of constipation, soiling and general health of patients following formation of ACE stoma. Adolescents benefits an orderly transition of their care to adult services. LEVEL OF EVIDENCE: Level 2, Therapeutic study.

Functional outcomes in Hirschsprung disease: A single institution's 12-year experience.

AIMS: Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period. MATERIALS AND METHODS: We conducted a retrospective review of all children operated for HD between 2002 and 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0-20, 20=best score). We assessed hospital admissions, complications including Hirschsprung associated enterocolitis (HAEC) and the need for further surgical procedures. RESULTS: 72 (52 male) patients were studied, of whom, 6 (8%) had a positive family history, 5 (7%) had Trisomy 21 and 5 (7%) had total colonic HD. The median age at diagnosis was 6.5days (2 days-6.7 years) and median follow-up was 6years (1-12years). All patients except two underwent a Duhamel pull-through procedure. The median age at surgery was 4months (6days-90months). 37 (51%) procedures were performed single-stage and 7 (10%) were laparoscopically assisted. Our early complication rate was 15%; 11 (15%) patients were treated for HAEC and 43 (60%) did not require any further surgery. 12 (17%) underwent injection of botulinum toxin, 7 (10%) needed residual spur division and 4 (5%) required an unplanned, post pull-through enterostomy for obstructive defecation symptoms and HAEC. Two (3%) patients underwent an Antegrade Colonic Enema (ACE) stoma. The median BFS was 17 (5-20). There were two deaths both out of hospital. CONCLUSIONS: Long-term functional outcomes following Duhamel Pull-Through surgery are satisfactory although 40% of patients needed some form of further surgical intervention. The management of anal sphincter achalasia has improved with the use of botulinum toxin and we advocate aggressive and early management of this condition for symptoms of obstructive defecation and HAEC. LEVEL OF EVIDENCE: III.

Outcome of excision of megarectum in children with anorectal malformation.

BACKGROUND/PURPOSE: Megarectum in association with anorectal malformation contributes to chronic constipation and fecal incontinence. Resection of megarectum in anorectal malformation improves bowel function, but neuropathy and poor sphincter quality may affect the outcome of fecal continence adversely. The aim of this study was to evaluate the benefits of resection of megarectum in anorectal malformation and to ascertain the impact of anal sphincter quality and neuropathy on the outcome. METHODS: We studied 62 children with intractable fecal incontinence after repair of anorectal malformation between January 1991 and January 2005. All patients were investigated with anorectal manometry and anal endosonography under ketamine anesthesia. On endosonography, an intact or scarred internal anal sphincter (IAS) was classified as good and a fragmented or absent IAS as poor. On manometry, a resting anal sphincter pressure equal to or more than 30 mm Hg was classified as good and a lower pressure as poor. Functional assessment of fecal continence was done before and after excision of megarectum using a modified Wingfield scores. RESULTS: Sixteen children had excision of megarectum with median age of 9 years (range, 2-15 years) and postoperative follow-up of 5 years (range, 1-10 years). Seven had formation of antegrade continent enema stoma before excision of megarectum. Children were classified into three groups of anomalies: low (n = 6), intermediate (n = 4), and high (n = 6). All children were incontinent of feces. After excision of megarectum, of the 9 children with good IAS and no neuropathy, 7 became continent of feces. Of the remaining 7 children, 4 had poor IAS and 3 had neuropathy, 5 of whom required an antegrade continent enema stoma to be clean. CONCLUSION: Excision of megarectum in children who had previous repair of anorectal malformation results in fecal continence in the presence of a good IAS and absence of neuropathy. Patients with a poor IAS or neuropathy will often require artificial means of fecal continence.