Old enough for physiotherapy? Zahra Van Veldhoven Lizelotte Vandenplas Ellen Vanderhenst UZ Leuven Octobre 2016

Overview Normal defecation Physiotherapy and pelvic floor training Hirschsprung disease and postoperative outcome Evidence Follow up

Defecation Peristalsis colon Rectal compliance RAIR Rectal and anal sensation Urge to defecate Inhibition Contraction PR and EAS Decreasing intrarectal pressure Defecation Relaxation PR and EAS Increasing intra-abdominal and intrarectal pressure Stoelgang in rectum => rectale en pelviene distensie => receptoren => relaxatie interne sfincter => stoelgang naar anale kanaal => contractie EAS => geschikte moment en plaats: relaxatie IAS en EAS en verhoogde IAP => defecatie RAIR (ontspanning IAS en contractie EAS) = rectoanal inhibition reflex RAIR afwezig bij HD

Physiotherapy Multidisciplinary approach Education: dietary advice and activity Toilet training Stool diary Pelvic floor training Selected group Age Relationship child-therapist Dietary: eten en drinken

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Pelvic floor muscles IAS EAS PR Resting tone 70% Autonomic innervation Ano-rectal angle Rest: 90° Relaxation: 120°

Physiotherapy PELVIC FLOOR TRAINING Awareness Body Pelvic floor Pelvic floor exercises Biofeedback Contraction Relaxation Coordination Electrostimulation Sensibility training bioFB includes aspects: the strength of the sphincter training, rectum and pelvic floor sensation training and sphincter coordination training BioFB by dyssynergic defaecation: relax pelvic floor muscles while applying a downward intra abdominal pressure to generate a propulsive force toward the anus.

HD and postoperative outcome Indications for physiotherapy Constipation Dyssynergic defecation Fecal urgency Fecal incontinence/soiling Enlargement of rectum Enterocolitis QOL Neuvonen 2016, Meinds 2014 Dyssynergic defecation = a disorder characterized by a paradoxical involuntary contraction of the EAS which leads to a functional neuromuscular obstruction. Preoperative obstipation and postoperative pain in the anal canal Unable to relax IAS + EAS unvoluntary contracts during defecation Meinds et al: Cross sectional study => a detailed analysis of the long-term bowel functional outcomes and QOL among patients operated for HD with TEPT in relation to matched controls using validated questionnaires Most postoperative HD patients have good clinical outcomes, substantial number of patients seem to struggle with ongoing severe constipation. Most constipation complaints in HD are attributed to the disease itself. It is possible that some HD patients have never mastered the act of defecation properly owing to preoperative obstipation and postoperative pain in the anal canal causing them to suffer from dyssynergic defecation as a result. Thus besides being unable to relax their internal anal sphincter, HD patients might have the additional problem that their external anal sphincter contracts involuntary during defecation. Retrospective review of 10 patients with severe persistent constipation: all diagnosed with dyssynergic defecation. Several reasons for onset described: Faulty learning of proper defecation Pain in rectum after surgery and washouts lead to avoiding bowel movement Postoperative complications or enterocolitis could make patients more prone to develop dyssenergic defecation 10 patients (1 female, 9 male), mean age 12 y with surgery HD with severe constipation Anal pressure profile: increased anal sphincter resting pressure and squeezing pressure RAIR (rectoanal inhibition reflex) was absent 4/10 failed to expel the ballon (defecometry), or more volume or more time Increased pressures in the rectum and in the anal sphincter Diagnosis dyssynergic defaection => increased pressures in the rectum and the anal sphincter + sufficient propulsive force Proper coordination of defecation absent in 10/10 patients Increased volumes required to sense the balloon (normal values 30 – 50 ml) > 150 ml Enlargement of rectum in 8/10 Persistent constipation owing to dyssynergic defecation was shown to have a negative influence on overall quality of life as it significantly impairs social life, sex life, work life and family relationships (Rao 2004) HD= birth defect characterized by aganglionosis of the distal colon. Typisch: failure to pass meconium during the first 24-48 hours after birth. Incidence 1/5000 (male > female) Most common involved segment = rectosigmoid colon CHILDREN Chiarioni et al. 2006: Biofeedback requires cognitive processing and sustained attention

Follow up Psychosexual wellbeing Neuvonen et al, 2016 van den Hondel et al, 2015 Psychosexual wellbeing 1 in 6 males and 1 in 2 women report problems Erectile dysfunction Vaginal stenosis Dyspareunia Need for education Information improve coping skills and QOL Neuvonen 2016 TEPT (transanal endorectal pull through) => bowel mobilization and dissection through the anus has the potential to interfere with the anal sphincter integrity and rectal sensation. HD 57 patients / controls 171 (age and gender matched controls) mean age 15 y Questionnaires: Bowel function score Enquiry of enterocolitis GIQLI and SF36 health > 18 y / PedsQL 4-17 y Results: Patients reported highter rates and more severe forms of impairment in all aspects of fecal control except for constipation With advancing age all aspects of fecal control (urgency, rectal sensation, ability to hold back defecation, fecal soiling and fecal accidents) improved to levels that were no longer significantly different from controls beyond age of 18 y. However abnormal stool frequency 50% patients Patient gender, level of aganglionosis or type of TEPT did not significantly influence the overall outcome 29% reported social problems due to functional bowel symptoms The tendency for more soiling and fecal accidents among patients with persistent or recurrent enterocolitis, suggested by our data could relate to altered stool composition to microbiotic disturbances. The absence of a significant association above the age of 12 y is consistent with reducing enterocolitis over time and overall improving bowel function.

12 children with fecal soiling problems after surgery for ARM Author Sample Size Treatment Control Intervention Results Leung et al. 2006 12 children with fecal soiling problems after surgery for ARM Age > 5 y 6 months supervised BIOFB and ES and 6 months home based exercises Bowel management / 12 months Significant improvement of soiling frequency and sphincter muscle EMG Sun et al. 2009 55 patients with fecal IC 29 ARM 16 HD Mean age 9,4 y BF training 2 weeks 2x/day 30 min Self training at home Monthly evaluation 1 year Anal continence improvement in 14 ARM and 16 HD Koh et al. 2012 226 patients with functional constipation Mean age 48 y 4 sessions Exercises at home Stool chart 1 year follow up GIQLI: improve scores significant except for social function and medication 24 incontinent children after surgery for HD BIOFB 2 weeks in the hospital Home based exercises No further treatment Resting anal pressure and squeeze pressure improved significantly and clinical outcomes improved BioFB = effective treatment for patients with dyssergic defaecation Patcharatrakul 2011: IBS: treating dyssynergic defecation patients with IBS by biofeedback therapy improved both constipation and IBS symptoms Fecal IC have serious psychologic and social impact on children Fecal IC common after surgery for Hirschsprung’s disease BioFB strengthen the peripheral anal muscle (FIC resulting from sfincter dysfunction) Sun: patients with simple anal sphincter damage withe relatively normal rectal sensation, no abnormal anal position 6 tot 14 jaar Anale manometrie initial anal canal resting pressures were decreased in all patients and squeeze pressures were decreased in ARM / all cases with HD had normal anal squeeze pressure 1 jaar: anal continence improvement was satisfactory in 30 cases, including 16 HD bioFB includes aspects: the strength of the sphincter training, rectum and pelvic floor sensation training and sphincter coordination training Good results of FB and improvement ~ anal squeeze pressure > 80 mmHG better anal continence < 80 mmHG poor results, wel verbetering Koh Dyssnergic defaecation = maladaptive behaviour (as there is no neurological or anatomical defect in these patients), failure in relax the puborectalis and external anal sphincter during defeacation BioFB: relax pelvic floor muscles while applying a downward intra abdominal pressure to generate a propulsive force toward the anus. Inclusion: patients with dyssynergic defaecation Puborectalis paradoxus or anismus (anale manometry) More than 20% of total markers in the pelvic on colonic transit marker study (= exclusion slow transit) History of excessive straining on defaection with normal bowel frequency Absence of secondary causes of dyssynergic defaection such as rectocoele and rectal prolapse Absence of colonic pathology mimicking constipation by colonic imaging such as cancer Constipation despite dietary fibre and laxatives therapy GastroIntestinal Quality of Life Index: 71% improvement sustained at 1 year follow up In female and male improvement Chiarioni Outlet dysfunction can be divided into those with structural causes for obstructed defecation (ex HD) and patients with a functional defecation disorder (dyssynergic defecaetion and inadequate defecatory propulsion) Incomplete emptying of the rectum The negative outcomes reported in controlled studies have been in the pediatric population. BioFB requires complex cognitive processing and sustained attention that may be beyond the abilities of younger children. The quality of the therapist-relationship and the skills and experience of the therapist seem to influence the success of behavior therapy. Leung Because the training programme required cooperation of the patients, only children above 5 y old with no learning difficulties were recruited. 2x/week eerste 3 m, 1x/week 4e maand, 1x/2 weken 5e maand, 6 maanden thuisoefeningen Bowel management = dietary control, toilet training, medications, enemas, … Enemas = lavementen 6 months supervised, 6 months home based exercised - Before pelvic floor muscle training: bowel management including toilet training, dietary advice, medications, enemas were started. Also continued throughout the programme - outcome: soiling frequency rank, rintala continence score, sphincter muscle EMG, anorectal manometry: before and after the programme ES and biofb of pelvic floor muscle is an effective adjunct for the treatment of feacal incontinence in children following surgery for AM Tanti Case report Sun The damage of internal anal sphincter might be one of the causes of fecal incontinence after Soave procedure. The damage of internal anal sphincter could be caused by lower level of dissection, vigorous anal dilation, and excessive anal canal traction during operation. Pelvic floor muscle training is one procedure of choice to treat this complaint.

Old enough for physiotherapy? Age 6 year Cognition Indications Constipation Fecal urgency and incontinence Dyssynergic defecation Physiotherapy Pelvic floor exercises Biofeedback