33.1 Introduction
Most children born with anorectal malformations (ARM) suffer from defecation problems during child- hood. For a majority of patients constipation disap- pears in adolescence and fecal incontinence also im- proves at that time [1]. Although problems tend to be worse in young children [2], even some adolescents and adults continue to have difficulties with defeca- tion [3–5].
Chronic defecation problems have a negative im- pact on the child’s somatic and psychosocial develop- ment. Chronic constipation leads to eating problems, chronic fatigue, inactivity, and urinary problems [6].
Incontinence leads to social and emotional problems like a negative self-image, lack of self-confidence, behavioral difficulties, and parent–child interaction problems [7–10].
Possible conservative treatments for fecal inconti- nence include dietary, medical, and behavioral treat- ment, all of which are often effective [11]. They are directed to prevent as much suffering as possible in young children and their parents. Long-term follow- up with adequate toilet training is required for all pa- tients with an ARM [12].
We can discern the strict medical therapies, includ- ing the bowel management program, from the mul- tidisciplinary behavioral treatment and the dietary contribution, both of which are too often neglected.
An abnormality of the anorectal area does not mean it can never function properly and therefore automati- cally lead to a life with daily washouts. Proper func-
tional rehabilitation with behavioral therapy can im- prove the incontinence or constipation.
As we try to avoid all unpleasant and often painful rectal interventions, we start with behavioral treat- ment if a child is still incontinent at 4 years of age.
When a child has severe constipation and simple oral and consistently given medication therapy and di- etary advice does not result in improvement, we start at an earlier age.
33.2 Multidisciplinary Behavioral Treatment
In the Nijmegen behavioral treatment protocol, de- veloped at the Radboud University Nijmegen Medi- cal Centre, defecation problems are considered to be the result of a dysfunctional interaction between the organ, which is impaired by the ARM, and behavioral factors [13]. To have a bowel movement, there has to be rectal filling. In healthy children this will lead to a sense of urge, which induces a defecation reflex and results in an empty bowel. For children to become continent, there has to be an interaction between the organ and the behavior of the child. The child has to perceive a sense of urge and to learn to react by with- holding defecation for a short while, to go to the toilet or potty for voluntary evacuation. This will not only lead to an empty bowel, but also to empty diapers or trousers. In this learning process, several things can go wrong. For instance, a child who experiences pain with defecation will react to a sense of urge by with- holding instead of relaxation. This will lead to incom- plete defecation, which causes constipation. Children who have been constipated for a long time no longer feel any sense of urge, and in response will not do anything to have defecation, which results in persis- tence of the problems.
These processes do not only take place in healthy children, but also in children with ARM who are al- ready more vulnerable because of their impairment.
Contents
33.1 Introduction . . . 421
33.2 Multidisciplinary Behavioral Treatment . . . 421 References . . . 425
33 Postoperative Treatment: Multidisciplinary Behavioral Treatment. The Nijmegen Experience
René Severijnen, Maaike van Kuyk, Agnes Brugman-Boezeman, and Marlou Essink
In the multidisciplinary treatment, a learning process is started in the child, with the parents as co- therapist, aimed at resolving the chronic defecation problems by teaching the child bowel self-control, primarily by training optimal defecation skills and subsequently toilet behavior. The therapeutic team consists of a pediatric surgeon, a child psychologist, and a pediatric physiotherapist, who work closely to- gether. The pediatric surgeon determines whether the child’s somatic condition is sufficient to reach some form of self-control over defecation. He/she facili- tates the learning process by prescribing oral medica- tion to allow sufficient emptying of the bowel. After disimpaction by enema or a rectal washout (if neces- sary), prescribing oral laxatives prevents new impac- tion. This treatment is continued as long as the child is withholding defecation or has not yet mastered enough self-control. For some children, medication will be necessary for a very long period of time.
The child psychologist educates the child an par- ents concerning defecation and manages the learning process, looking after the defecation and /or toilet be- havior; he or she will establish whether the problems are caused by a lack of knowledge, defecation and/or toilet anxiety, or whether there is a motivational prob- lem. Parental skills and cooperation in general, and
in particular concerning the defecation problems, are investigated and attended to if necessary.
The pediatric physiotherapist assists in managing the learning process by teaching the child to use the lower part of the body in a pleasant way by playing motor games. If necessary, the child is taught to evac- uate feces voluntarily even without a feeling of urge by teaching a correct straining technique. The child is instructed how to build up pressure in the right di- rection and to tense the abdominal wall muscles and relax the muscles of the pelvic floor selectively, and to combine both to defecate in the correct way [9].
The multidisciplinary treatment is carried out in accordance with a protocol. The different steps of the Nijmegen multidisciplinary behavioral treatment pro- tocol are described in Table 33.1. This treatment pro- tocol was studied in a prospective, controlled study with follow-up. Compared with a waiting-list control group, the experimental treatment group scored sig- nificantly better on two important measures (Temple- ton and percentages of feces in toilet; Table 33.2). Al- though young children had poorer scores than older children before treatment, no significant differences in the favorable outcome of treatment were found be- tween both groups after treatment (Table 33.3). Nor was there any effect of type of ARM on treatment.
Table 33.1 The Nijmegen Behavioral Treatment protocol. ARM Anorectal malformations
Knowing • Child and parents are educated about defecation in general and in ARM, how children become continent normally, how defecation problems develop
• They are educated about the working of and compliance to medication
• Parents and child both learn what they can do to change the prob- lems and the child learns skills of self-control
Daring • Extinction of reactions of fear and aversion relating to urge sensations and defecation avoidance behavior like squeezing and withholding feces and avoiding the toilet. For example, by using desensitization techniques, by prescribing oral instead of anal medication, and by stopping invasive treatment Wanting • The child is motivated by taking small, reachable steps, being given direct
rewards, and by having their effort rewarded at first and the result later on Being able • Sufficient emptying of the bowel by oral medication
• Being able to defecate by learning an adequate straining technique Doing • Learning to try to defecate regularly and to empty their bowel 2–3 times
a day after the meal by straining adequately 5–10 times each time
• Learning to think about it themselves
• Learning to react to an external sign first and, when possible, to an internal sign
• Adjustment of exercises to the defecation rhythm
• Compliance in taking medication
Continue doing • Consolidation of behavior discipline in child and parents
• Finishing medication if possible
• Prevention of relapses
The results of multidisciplinary behavioral treatment remained stable over a mean follow-up period of 7 months (Table 33.4) [14].
This treatment is suitable for young children, thus preventing chronic problems in vulnerable children.
Children can be treated individually as well as in groups. Group treatment seems to be very effective.
Parents and children profit from each other. Groups are formed with six children of the same developmen- tal age. The group meets six times for 2 hours each time, with a follow-up session after 6 months.
For children who do not profit from multidisci- plinary behavioral treatment (for instance because of their anatomic limitations or because of psychiatric comorbidity or severe parent–child interaction prob- lems), bowel management can be a good solution of their defecation problems. In our clinic, children start with behavioral treatment when possible, and if this is
not effective enough in managing the problems, chil- dren begin bowel management.
With the new Krickenbeck classification [15], it has become easier to evaluate and compare the post- operative results after ARM repair, but it remains difficult to choose the best therapy for children with continence problems. By treating the children with multidisciplinary behavioral treatment, not only can further surgery for improving continence be avoided, but also the quality of life of these children can be im- proved, as they stop suffering from chronic inconti- nence and constipation. It is therefore a valuable ad- junct to standard medical treatment of children with surgically repaired ARM.
Table 33.2 Means and standard deviations (SD) of children in the experimental treatment and waiting-list control group on first and second assessment based on intention-to-treat analysis, and analysis of variance (ANOVA) on change of scores between both assessments, controlled for pretreatment scores. Reproduced with permission from van Kuyk et al. [14]
Experimental treatment
group n = 13 Waiting list control
group n = 11 Significance
ANOVAa First assessment Second assessment First assessment Second assessment
Templeton 1 = good 2 = fair 3 = poor
2.2 (0.63) - 9 (69%) 4 (31%)
1.6 (0.76) 7 (54%) 4 (31%) 2 (15%)
2.0 (0.59) 1 (9%) 8 (73%) 2 (18%)
2.0 (0.72) 2 (18%) 6 (55%) 3 (27%)
0.03*
Wingspread 1 = clean 2 = staining 3 = intermit- tent soiling 4 = constant soiling
2.7 (0.88) 1 (8%) 3 (23%) 5 (38%) 4 (31%)
2.3 (0.95) 2 (15%) 6 (47%) 3 (23%) 2 (15%)
2.9 (0.86) - 3 (27%) 5 (46%) 3 (27%)
2.6 (1.1) 3 (27%) - 6 (55%) 2 (18%)
0.53
Wingspread constipation 1 = no 2 = sometimes 3 = yes
1.8 (0.69) 4 (31%) 7 (54%) 2 (15%)
1.3 (0.60) 10 (77%) 2 (15%) 1 (8%)
1.8 (0.72) 3 (27%) 6 (55%) 2 (18%)
1.4 (0.49) 6 (55%) 5 (45%) -
0.52
Percentage of
feces in toilet 41.5 (30.9) 57.9 (35.0) 43.5 (28.0) 38.5 (25.6) 0.049*
Number of days
without soiling 3.7 (4.9) 5.8 (5.5) 3.7 (4.2) 2.8 (4.3) 0.09
Parental Judgment
Incontinence Scale 17.5 (5.7) 13.2 (5.3)
two missing 18.1 (6.1) 16.1 (7.3) 0.26
*p ≤ 0.05
aANOVA on change of scores (scores on the second assessment minus scores on the first assessment) between both groups, with scores at the first assessment as covariate
Table 33.3 Means and SD of young and older children at pre- and posttreatment, and Student’s t-test; also ANOVA on change of scores between young and older children, controlled for pretreatment scores. Reproduced with permission from van Kuyk et al. [14] PretreatmentPosttreatment Young children 2–4 years n = 10Older children 5–12 years n = 14Significance Student’s t-testaYoung children 2–4 years n = 10Older children 5–12 years n = 14Significance Student’s t-testbSignificance ANOVAc Templeton 1 = good 2 = f air 3 = poor
2.4 (0.52) - 6 (60%) 4 (40%)
1.9 (0.57) 1 (7%) 11 (79%) 2 (14%) 0.03*1.7 (0.82) 5 (50%) 3 (30%) 2 (20%)
1.4 (0.62) 8 (57%) 5 (36%) 1 (7%)
0.460.78 Wingspread 1 = clean 2 = staining 3 = intermittent soiling 4 = constant soiling
3.2 (0.71) - 1 (10%) 5 (50%) 4 (40%)
2.5 (0.89) 1 (7%) 5 (36%) 5 (36%) 3 (21%)
0.082.5 (0.98) 1 (10%) 4 (40%) 3 (30%) 2 (20%)
2.0 (0.87) 4 (29%) 6 (43%) 3 (21%) 1 (7%)
0.210.89 Wingspread constipation 1 = no 2 = sometimes 3 = yes
2.0 (0.83) 3 (30%) 4 (40%) 3 (30%)
1.7 (0.58) 4 (29%) 9 (64%) 1 (7%)
0.421.3 (0.63) 7 (70%) 2 (20%) 1 (10%)
1.1 (0.31) 11 (79%) 3 (21%) -
0.430.58 Percentage of feces in toilet27.8 (22.3)52.9 (29.3)0.03*54.4 (30.4)63.1 (33.2)0.520.25 Number of days without soiling1.1 (1.2)5.6 (5.1)0.01*4.5 (5.0)6.8 (5.8)0.380.47 Parental Judgment Incontinence Scale21.2 (4.2)15.3 (5.5)0.01*16.2 (6.2)11.8 (5.2) two missing0.090.77 * p ≤ 0.05 a Student’s t-test on pretreatment scores of young and older children b Student’s t-test on posttreatment scores of young and older children c ANOVA on change of scores (posttreatment scores minus pretreatment scores) between both groups, with pretreatment scores as covariate
References
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Table 33.4 Means and SD of all completers at pretreatment and follow-up, and paired samples t-test. Reproduced with permission from van Kuyk et al. [14]
First assessment
(baseline) n = 19 Last assessment
(follow-up) n = 19 Paired samples t-testa Templeton
1 = good 2 = fair 3 = poor
1.9 (0.55) 1 (5%) 15 (79%) 3 (16%)
1.3 (0.48) 13 (68%) 6 (32%) -
0.00*
Wingspread 1 = clean 2 = staining
3 = intermittent soiling 4 = constant soiling
2.6 (0.81) 1 (5%) 6 (32%) 8 (42%) 4 (21%)
2.1 (0.71) 4 (21%) 10 (53%) 5 (26%) -
0.00*
Wingspread constipation 1 = no
2 = sometimes 3 = yes
1.9 (0.66) 5 (26%) 11 (58%) 3 (16%)
1.3 (0.48) 13 (68%) 6 (32%) -
0.00*
Percentage of feces in toilet 48.8 (27.4) 77.9 (16.8) 0.00*
Number of days without soiling 4.5 (4.8) 8.2 (5.0) 0.01*
Parental Judgment
Incontinence Scale 16.7 (5.6) 12.7 (5.2) 0.00*
* P ≤ 0.05
a Paired samples t-test on scores of all children on baseline and on last assessment
