27.1 Introduction
Standardized assessment of clinical outcome after re- pair of anorectal malformations (ARM) is essential for appropriate quality control in series of patients treated in single or different institutions, and for com- paring different treatment modalities. Clinical assess- ment is subjective and may be biased by the observer, who is often the surgeon treating the patient. There- fore, pediatric surgeons performing clinical research need scales and scores that provide reliable informa- tion on the condition and functional status of their patients. However, appropriate methods and instru- ments for collecting data on the outcome after repair of ARM have been a matter of debate for decades.
In 1960, Scott [30] introduced a simple score, which was based on his clinical experience and which included exclusively clinical data. Since then, approxi- mately ten scores have been introduced and used with varying frequency in patients after ARM repair. These scores are of different complexity with regard to clini- cal, functional, and other parameters. However, none
of the instruments has undergone a proper validation process. This may be the main reason why no single score has been generally accepted to date. Therefore, the reported differences in results of different series of patients with ARM undergoing different methods of repair remain difficult to interpret [10], and a gener- ally accepted score remains mandatory.
27.2 Principles of Scaling and Scoring
A scale is an instrument that is used to measure clini- cal phenomena, such as the degree of incontinence or the squeezing pressure of the anal sphincter. A score is a value on a scale in a given patient. The simplest and most complex scales have similar structures. They consist of elements and questions and their answers.
Scores in specific patients may be dichotomous (yes/
no), or rank-ordered. Thus, qualitative scores can be differentiated from numerical scores.
Principally, a score may serve three functions:
prediction, evaluation over time, or description at a certain time point [2]. Scores have to be within a reasonable range of variation, with repeated admin- istrations to the same patient by the same and differ- ent observers (reproducibility). In addition, a score has to be a valid measure of what it is supposed to be measuring (validity), and should show changes when the patient changes and no change when the patient is stable (responsiveness). Ideally, the process of en- suring reproducibility, validity, or responsiveness of a specific scale or score should not be based on the observer’s clinical knowledge and common sense, but on a structured process.
Patients with ARM have mostly been scored de- scriptively. None of the scores suggested for use in these patients has undergone a standardized valida- tion process concerning reproducibility, validity, or responsiveness, with the exception of specific quality- of-life scores. The latter were not specifically designed for patients with ARM. In addition, the problem of definition of endpoints has not been sufficiently ad-
Contents
27.1 Introduction . . . 351
27.2 Principles of Scaling and Scoring . . . 351 27.3 Specific Scores used in Patients
with ARM . . . 352 27.3.1 The Scott Score . . . 352 27.3.2 The Kelly Score . . . 352 27.3.3 The Holschneider Score . . . 352 27.3.4 The Wingspread Score . . . 353 27.3.5 The Rintala Score . . . 353 27.3.6 Peña 1995 . . . 355 27.3.7 Other Scores . . . 355
27.4 Other “Objective” Methods of Scoring . . . 355 27.5 Quality-of-Life Measurements . . . 356 27.6 Comparison of Scores and Outcomes . . . 357 27.7 Conclusions and Results of the
Krickenbeck Meeting 2005 . . . 357 References . . . 358
27 Scoring Postoperative Results
Benno M. Ure, Risto J. Rintala, and Alexander M. Holschneider
dressed. Constipation, intermittent soiling, or other symptoms are not uniformly defined.
27.3 Specific Scores used in Patients with ARM
There is consensus that fecal continence represents the most important endpoint in patients with ARM.
Therefore, specific scores for assessment of long-term results are focused on differentiating various de- grees of fecal incontinence. No consensus has been achieved on including and scoring other symptoms such as constipation, urinary incontinence, electro- manometric and endosonographic findings, or qual- ity-of-life measurements.
27.3.1 The Scott Score
In 1960, Scott [30] established a qualitative score that differentiates between “good”, “fair,” and “poor”
continence (Table 27.1). The items used are defeca- tion habits, stool control, perianal soreness, and the function of the puborectalis muscle on digital ex- amination. “Good” continence is defined as sponta- neous regular defecation with or without occasional soiling during stress situations. Patients who are scored as “fair” have spontaneous and regular def- ecations or chronic constipation. They are continent for normal, but not for liquid stool. They suffer from frequent soiling and intermittent perianal soreness.
The strength of the puborectalis sphincter is reduced.
Patients with “poor” continence suffer from frequent stools, constant soiling, and perianal soreness, and have no puborectalis sphincter tone. This score was not validated, and a clear definition of specific items such as constipation, and puborectalis sphincter pres- sure was not given. However, the score was used and modified subsequently in early series of children with ARM [17,23].
27.3.2 The Kelly Score
In the Kelly method [15], the criteria are somewhat similar to the Scott score, but continence is scored quantitatively (Table 27.2). The determination is based on leakage phenomena, on the strength of the puborectalis sphincter, and on sensitivity. Factors include the appearance of staining or smearing, ac- cidental defecation or soiling, sensitivity, the strength of the puborectal is muscle action on digital examina- tion, and “feeling of defecation”. A total of 5–6 points
is considered “good,” 3–4 points is “fair,” and 2 points is “poor.” Although not validated, the score has gained increasing popularity [6,13,19,22,29] and probably represents the most commonly used instrument for assessment of fecal incontinence today. It is not of- ten used as a single instrument, but is compared with other more objective measures, such as manometry, electromyography, and quality-of-life data.
27.3.3 The Holschneider Score
Holschneider and Metzer [11] introduced a quantita- tive clinical score, including the parameters frequency
Table 27.1 Assessment criteria for continence according to Scott et al. (1960) [30]
Evaluation Assessment
criteria Clinical result Good Defecation Spontaneous/regular
Stool control Normal/occasional soiling during stress situations Soreness perianal None
Sphincter tension Strong
Fair Defecation a) Regular
b) Chronic constipation Stool control Frequent soiling, con-
tinence restricted to normal stool consistency Soreness perianal Only with frequent soiling Sphincter tension Reduced
Poor Defecation Frequent
Stool control Constant soiling Soreness perianal Constant Sphincter tension None
Table 27.2 Kelly score (Kelly, 1972) [15]
Staining/smearing none 2
occasional 1
constant 0
Accidental defecation/soiling none 2 occasional 1
constant 0
Strength of puborectalis muscle strong 2
weak 1
none 0
of defecation, fecal consistency, soiling, rectal sensa- tion, ability to hold back, discrimination, and need of therapy. Each of these seven parameters is scored as 0–2 according to the degree of impairment. In con- trast to previous scores, items such as “frequency of stools” or “warning period” are clearly defined. A score of 10–14 points is “continent,” 5–9 points “fair,”
and 0–4 points “incontinent.” Later, the authors felt that the parameters of rectoanal sensibility were over- represented. They modified the score, reduced the clinical parameters, and included manometric data without changing the numerical scoring (Table 27.3) [8]. Both the initial and the modified scores have been used by some other authors in recent years [27].
Holschneider et al. [10] recently stated that neither a reference to the course of the anal or rectal fistula, nor a rating as “good,” “satisfactory”, or “sufficient”,
nor the current score systems are suitable for com- parative postoperative studies. The authors suggested renouncing the assessment of fecal continence, tak- ing chronic constipation into account. With regard to three subgroups, the types of partial continence, but not the degrees of continence, are differentiated. The authors used their score in 78 patients and postulated that with regard to therapeutic conclusions, the results were more evident and more correct as compared to other scores.
27.3.4 The Wingspread Score
In the Wingspread score [31,32], the grades of conti- nence are scored qualitatively. They fall into the four main categories of “clean,” “staining,” “intermittent fe- cal soiling,” and “constant fecal soiling” (Table 27.4).
Subcategories include the need of occasional or con- stant therapy. In an additional category, related com- plications concerning the anorectum, urinary, genital, or other functions are noted. The grades are scored qualitatively and the instrument has been widely used in recent years [3,18,22,25,27].
27.3.5 The Rintala Score
Rintala and Lindahl [25] established a clinical score for the evaluation of fecal continence. The score is de- rived from standardized questionnaires and physical examination is not required (Table 27.5). The score consists of seven factors, which are scored from 0 to 3, except the factor of frequency of defecation, which is scored 1–2. The maximum bowel function score is 20.
The authors used the score initially in 46 consecutive patients who had undergone surgical repair of high or intermediate ARM, and compared the results with data obtained from 70 healthy children with a similar age and sex distribution. Only 52% of the children of the control population obtained a completely normal bowel function score of 20 points. Functional aberra- tions in controls were occasional staining in 42% and constipation in 15%. The authors considered a score of 18 or higher to be normal. The score differentiated an excellent outcome with a normal score in 35% of the patients after repair. Another 35% of the patients were scored as “good,” having occasional staining and infrequent accidents; this group of patients scored 9–
16 points. Patients with “fair” results had intermittent daily soiling or staining and scored 7–11 points. Pa- tients with “poor” results scored 6–9 points and had to use daily enemas because of severe constipation or had constant soiling.
Table 27.3 Clinical evaluation of continence according to Holschneider (1983) [8]. For these scores, 14 points means normal bowel habits, 10–13 points means good (social con- tinence, few limitations in social life), 5–9 points means fair (marked limitations in social life), and 0–4 points means poor bowel habits (total incontinence)
Frequency of defecation Normal (1–2/day) 2 Often (3–5/day) 1
Very often 0
Fecal consistency Normal 2
Soft 1
Liquid 0
Soiling No 2
Stress/diarrhea 1
Constant 0
Sensitivity Normal 2
Reduced
(no discrimination) 1
Missing 0
Anorectal resting pressure profile ≥ 20–24 mmHg 2
14–19 mmHg 1
< 13 mmHg 0
Maximum pressure at maximum ≥ 30 mmHg 2
squeezing 20–29 mmHg 1
< 20 mmHg 0
Adaptation reaction Normal 2
Small amplitude,
shortened 1
Not detectable 0
There were some validation steps. The scores de- rived from the questionnaires and the clinical out- come noted in the hospital records were positively correlated. In addition, pathological findings on plain
spinal radiography or magnetic resonance imaging in 11 patients were negatively correlated with the bowel function score. Manometric findings did not differen- tiate between patients with excellent and good clini- cal outcome, but showed a significantly reduced anal resting pressure in patients with “fair” or “poor” clini- cal outcome. The authors used the score subsequently in a series of patients with low ARM [28]. Only half of these children had age-appropriate bowel function as compared to the control group. The main problem was constipation.
Table 27.4 Wingspread Score according to Stephens et al.
(1988) [32]
1. Clean
1.1 No accumulated feces 1.11 No therapy 1.12 Occasional therapy 1.13 Therapy dependent 1.2 Accumulated feces 1.21 No therapy 1.22 Occasional therapy 1.23 Therapy dependent 2. Staining
2.1 No accumulated feces 2.11 No therapy 2.12 Occasional therapy 2.13 Therapy dependent 3. Intermittent fecal soiling 3.1 No accumulated feces 3.11 No therapy 3.12 Occasional therapy 3.13 Therapy dependent 3.2 Accumulated feces 3.21 No therapy 3.22 Occasional therapy 3.33 Therapy dependent 4. Constant fecal soiling
4.1 No accumulated feces 4.11 No therapy 4.12 Occasional therapy 4.13 Therapy dependent 4.2 Accumulated feces 4.21 No therapy 4.22 Occasional therapy 4.23 Therapy dependent Re lated complications (specify)
1. Anorectal
(a) abnormal position (b) stenosis
(c) prolapse (d) fistula
(e) lack of contractility (f) abnormal length 2. Urinary
3. Genital 4. Other
Table 27.5 Evaluation of fecal continence according to Rintala and Lindahl (1995) [25]
Ability to hold back defecation Always
Problems less than 1/week Weekly problems No voluntary control
3 2 1 0 Feels/reports the urge to defecate
Always Most of the time Uncertain Absent
3 2 1 0 Frequency of defecation
Every other day to twice a day More often
Less often
2 1 1 Soiling
Never
Staining less than 1/week, no change of underwear required
Frequent staining, change of underwear often required
Daily soiling, requires protective aids
3 2 1 0 Accidents
Never
Fewer than 1/week
Weekly accidents; often requires protective aids Daily, requires protective aids during day and night
3 2 1 0 Constipation
No constipation Manageable with diet Manageable with laxatives Manageable with enemas
3 2 1 0 Social problems
No social problems Sometimes (foul odors)
Problems causing restrictions in social life Severe social and/or psychic problems
3 2 1 0
The advantages of the score are threefold. First, the questionnaires are completed by patients or parents;
the assessment is thus observer-independent. Second, a physical examination is not required. And finally, data from a control group of children with normal bowel habits are available, and a drawback is the over- lapping of scores in the different groups of continence (i.e., patients with a score of 9–11 may have either
“good” or “fair” continence), which is the result of a lack of clear cut-off points.
27.3.6 Peña 1995
Peña [24] suggested a specific methodology for evalu- ation of long-term results according to his personal experience (Table 27.6). He evaluated 387 out of 792 patients who had undergone a posterior sagittal ano- rectoplasty. At the time of evaluation, none of the pa- tients was allowed to be subjected to any type of med- ical management. Four parameters are evaluated:
1. Voluntary bowel movements, which are defined as feeling the urge to use the toilet to have a bowel movement, the capacity to verbalize it, and to hold the bowel movement.
2. Soiling is defined as involuntary leaking of small amounts of stool, which may be present with or without voluntary bowel movements. Soiling grade 1 occurs occasionally (once or twice per week).
Grade 2 refers to soiling that occurs every day, but does not cause social problems. Grade 3 represents constant soiling with social problems.
3. Constipation is defined as the incapacity to empty the rectum spontaneously without help every day (grade 1: when the patient is manageable by diet;
grade 2: when he requires laxatives; grade 3: when he requires enemas).
4. Urinary incontinence is considered grade 1 when the patient has mild dribbling and wetness of the underwear day and night, and grade 2 when he is completely incontinent. Patients with voluntary bowel movements and no soiling are considered totally continent. (These were 41% of the total se- ries.)
Peña presented a detailed analysis of the postop- erative results related to the types of fistula and mal- formation. However, to our knowledge, this score has not yet been widely used.
27.3.7 Other Scores
Other authors have suggested their own method of assessing continence and investigated small series of patients. Ditesheim and Templeton [4] introduced a
“qualitative fecocontinence score.” Points are assigned for the degree of awareness of impending stool, oc- currence of accidental defecation, need for extra un- derwear or liners, social problems related to odor, re- striction of physical activity, and presence of rashes.
The items are scored from 0 to 1; a total score of 4–5 points is “good continence”, 2–3.5 points is “fair conti- nence”, and 0–1.5 points is “poor continence.”
Kiesewetter and Chang [16] categorized conti- nence as “continent most of the time, suffering only occasionally from soiling when diarrhea or unusual physical stress were encountered,” fair as “occasional soiling or staining with a normal consistency stool, but with a socially acceptable degree of continence,”
and poor as “frank incontinence, with occasional times of control, or permanent colostomy established after a period of definitive therapy.”
27.4 Other “Objective” Methods of Scoring
Electromanometry has been used to determine the degree of incontinence since the early 1960s [11–13].
Holschneider [8] electromanometrically defined four grades of continence. These grades were derived from numerous parameters, such as anorectal pressure pro- file, fluctuations, relaxation of the internal sphincter, external sphincter contractions, puborectalis sphinc-
Table 27.6 Evaluation of Bowel Function according to Peña 1995 [24]
1. Voluntary bowel movements Feeling of urge
Capacity to verbalize Hold the bowel movement 2. Soiling
Grade 1 Occasionally (once or twice per week) Grade 2 Every day, no social problem Grade 3 Constant, social problem 3. Constipation
Grade 1 Manageable by changes in diet Grade 2 Requires laxatives
Grade 3 Requires enemas 4. Urinary incontinence
Grade 1 Mild dribbling/wetness day and night Grade 2 Complete incontinence
ter contractions, pressure tolerance, defecation reflex, adaptation, compliance, and critical volume. The cri- teria for grading continence were well established, but there remained some inconsistency due to overlap- ping of the parameters in the different groups. The author stated that electromanometric scoring reveals more unfavorable results when compared to clinical scores, but data derived from a large series of patients undergoing both clinical and electromanometric scoring are lacking. However, he suggested including selected manometric data in his clinical score for ob- taining a more objective result [8].
Diseth and Emblem [3] confirmed that anal ca- nal resting pressure and squeeze pressure correlated negatively with fecal incontinence. In a study of Hed- lund et al. [7], abnormal anal resting tone was found in 14 out of 17 patients with soiling 5–10 years after repair. However, the correlation to clinical results was incomplete and some patients without soiling had an abnormal resting tone. Other authors confirmed a lack of correlation of some manometric parameters with clinical continence. In a long-term study of 22 patients with high ARM, Rintala et al. [26] found that the only manometric parameter that correlated with the continence outcome was voluntary squeeze pres- sure.
Schuster et al. [29] recently used computerized vector manometry in 17 patients with various types of ARM. Besides computerized software supported by data on standard manometric parameters, a score as- sessing three pressure zones of the anal canal (0–16 points) was established. However, the authors found a poor correlation between quantitative manometric parameters and clinical results, which were assessed by a modified Kelly score.
Fukata et al. [6] compared endosonography and electromyography of the external anal sphincter with electromanometry and clinical data derived from the Kelly score. Endosonographic findings for the external anal sphincter corresponded well with elec- tromyographic findings, but not with manometry.
Only 15 patients were investigated. Jones et al. [14]
compared endosonography with magnetic resonance imaging after repair of ARM. The findings were com- parable in only 9 out of 14 patients. Fukuya et al. [5]
compared magnetic resonance imaging with clinical assessment on the basis of the Kelly score. The pro- portion of “fair” or “poor” developed muscles was not significantly different between the continence groups according to Kelly. Therefore, no conclusion concern- ing the correlation of endosonographic and magnetic resonance imaging findings with clinical scores can be drawn to date.
27.5 Quality-of-Life Measurements
Quality of life is a multidimensional concept, which includes, but is not limited to, the social, physical, and psychological functioning of the individual. Validated instruments are supposed to objectively measure the domains of quality of life, and to exclude observer bias. The relevance of quality-of-life assessment in children with ARM was confirmed in an early study by Ditesheim and Templeton [4], who used a ques- tionnaire scoring system that included items such as school attendance, social relationships, and physical capacities. Today it is well known that children and adolescents with fecal incontinence may suffer from emotional problems, internalizing behavior problems, and depressive symptoms. Various measures of qual- ity of life have been used for quantitative and quali- tative scoring of children and adolescents with fecal incontinence and constipation. However, the results presented below are not conclusive and none of the suggested instruments has been generally accepted.
Diseth and Emblem [3] used semistructured in- terviews and questionnaires, such as the Child As- sessment Schedule, Child Behavior Check List, and self report in 33 adolescents with ARM. Psychosocial functions were impaired in 73% of the adolescents, and 58% met the criteria for psychiatric diagnosis.
The authors found a significant correlation of the degree of flatus incontinence with the degree of psy- chosocial impairment and of continence of flatus with mental health symptom scores.
Ludman and Spitz [19] assessed the quality of life by self-report questionnaires, such as the Depression Self-Rating Scale and Self-Perception Profile, in 157 children and adolescents. In addition, the authors as- sessed parents and teachers using the Child Behavior Checklist and other instruments. The level of conti- nence, which was defined by the Kelly score, did not influence psychological adjustment, with exception of incontinent young girls. There were no significant dif- ferences between continent and incontinent children concerning the global self-worth measure. However, 29% had some psychiatric disorder and these were more likely children with incontinence. Other work confirmed that mental health or psychological prob- lems were found more frequently in 160 children and adolescents with ARM as compared to the normal population, but the incidence of these problems was similar in continent and incontinent patients [20].
Bai et al. [1] used the Achenbach’s Child Behavior Checklist in 71 children with ARM and found qual- ity of life to be significantly reduced as compared to a normal control group. The authors established tenta-
tive quality-of-life scoring criteria, including somatic assessment, social aspects, and psychological investi- gation (Table 27.7).
Quality-of-life assessment in patients with ARM is essential. However, no instruments taking the specific symptoms and problems into account have yet been established. The use of currently available instru- ments and the calculation of scores remains difficult and time consuming. Therefore, a specific instrument for scoring quality of life after ARM repair cannot be recommended for routine use yet.
27.6 Comparison of Scores and Outcomes
Studies comparing different scores in the same study population are scarce. Ong and Beasley [21] compared 4 scoring methods in 37 patients who had undergone sacroperineal rectoplasty. Continence was scored as
“good,” “fair,” and “poor” using two numerical (Kelly [15], Templeton and Ditesheim [33] and two qualita- tive scores (Kiesewetter and Chang [16], Wingspread – Stephens and Smith [31]). The Wingspread score was adapted to three categories for comparison pur-
poses. The results differed considerably. The authors found “good” continence in approximately 30–70%
of patients with high ARM, and “poor” continence in approximately 5–20%. The Templeton score assigned a higher score of fecal continence than the other three, with excellent concordance. In patients requiring re- vision after Stephens rectoplasty, the Templeton and Kelly scores, in contrast to the others, did not show incontinence in two and three patients, respectively [22].
27.7 Conclusions and Results of the Krickenbeck Meeting 2005
A clear recommendation concerning specific instru- ments cannot be derived from clinical experience or from data in the literature. To date, the Kelly score [15] has been most widely used. The Holschneider score [8] is the only one that includes an objective parameter (electromanometry) and gives more de- tailed information regarding bowel habits. The score introduced by Rintala and Lindahl [25] underwent a validation process and data from a control group of children with normal bowel habits are available.
Quality-of-life measurement reveals the most rel- evant and detailed information. However, the appro- priate instruments for quality-of-life measurement of children and adolescents with incontinence remain a matter of discussion.
At the Krickenbeck Meeting in 2005, consensus was achieved concerning the assessment of outcome after ARM repair (Table 27.8). The method comprises three parameters: voluntary bowel movements (yes/
no), soiling (yes/no, if yes grade 1–3), and constipa-
Table 27.7 Quality-of-life scoring criteria for children (aged 8–16 years) with fecal incontinence according to Bai et al.
(2000) [1]. Note that the higher the scores, the better the qual- ify of life
Item Criteria Points
Soiling Absent 4
Accidental 3
Frequent 2
Incontinence Accidental 1
Frequent 0
School absence Never 2
Accidental 1
Frequent 0
Unhappy or anxious Never 2
Accidental 1
Frequent 0
Food restriction No 2
Somewhat 1
Much 0
Peer rejection Never 2
Accidental 1
Frequent 0
Table 27.8 Method for assessment of outcome established in Krickenbeck 2005 (patient age > 3 years, no therapy; Holsch- neider et al. [9])
1. Voluntary bowel movements yes/no
Feeling of urge Capacity to verbalize Hold the bowel movement
2. Soiling yes/no
Grade 1 Occasionally (once or twice per week) Grade 2 Every day, no social problem Grade 3 Constant, social problem
3. Constipation yes/no
Grade 1 Manageable by changes in diet Grade 2 Requires laxatives
Grade 3 Resistant to diet and laxatives
tion (yes/no, if yes grade 1–3). The assessment should be performed in children more than 3 years of age who are not undergoing therapy. The surgeon, who is involved in the treatment and follow-up of the pa- tients who are to be scored, may bias the results of scoring. Therefore, the assessment and analysis of data should preferably be done by a person who is not involved in the treatment of the patient. This may bring pediatric surgeons closer to psychologists and methodologists, who are essential for establishing validated instruments for assessment of children and adolescents with ARM in the future.
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