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

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

3. 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

4. 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

5. Monday
Tuesday
Wednesday
Thursday
Friday
Saturday
Sunday

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

7. 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.

8. 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 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

9. 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.

10. 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.

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