**Long Term Outcome After Surgery for Anorectal Malformation**

Anthony G. Catto-Smith, Misel Trajanovska and Russell Taylor

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/57072

#### **1. Introduction**

[20] Brazelli M, Griffiths P. Behavioural and cognitive interventions with or without other treatments for the management of fecal incontinence in children. *Cochrane Database of*

[21] Nixon HH. Operation for incontinence following anorectal anomalies: Levatorplasty and gracilis transplantation. Rob and Smith's Operative Surgery colon, rectum and

[22] Madoff RD, Rosen HR, Baeten CG, LaFontaine LJ, Cavina E, Devesa M. Safety and efficacy of dynamic muscle plasty for anal incontinence: lessons from a prospective,

[23] Thornton MJ, Kennedy ML, Lubowski DZ, King DW. Long-term follow-up of dy‐

[24] Wexner SD, Gonzalez-Padron A, Teoh TA, Moon HK. The stimulated gracilis neo‐ sphincter for fecal incontinence: a new use for an old concept. *Plast Reconsr Surg* 98;

[25] Wright JJr, Gehrich AP, Albright TS. Mangement of Anal Incontinence. *J Pelvic Med*

namic graciloplasty for fecal incontinence. *Colorectal Dis* 2004; 6: 470-476.

*Systematic Reviews* 2006; 19: CD002240.

160 Fecal Incontinence - Causes, Management and Outcome

1996: 693-699

*Surg* 12; 2006: 125-140

anus. 5th ed. Chapman and Hill; 1993: 460-461.

multicenter trial. *Gastroenterology* 1999;116: 549-556.

Anorectal malformations (ARM) are a spectrum of congenital caudal end defects affecting the normal development of the anus and rectum that are usually easily detected after birth by physical examination. They are relatively common, occurring in approximately 1 in 5,000 live births, and vary markedly in severity. ARM is frequently associated with other congenital anomalies that may have a much more severe impact on long-term prognosis [1]. ARM are classified into low, intermediate and high subtypes depending on the relationship of the defect to the pelvic floor musculature [2]. A variety of surgical procedures have been developed for their correction, but despite this faecal incontinence is a well-recognized long-term complica‐ tion that is said to occur in between 10% to 33% or more of patients with ARM [3-6].

Most reviews of outcome after surgical repair of ARM grade continence into broad classifica‐ tions of "good", "fair" or "poor", based on scoring systems developed by Kelly [7], Templeton [5], Kiesewetter [8] or Wingspread [9]. Faecal incontinence has a significant impact on quality of life in both children and adults [10]. These classification systems have been crucially important in providing statistical information about incontinence but provide little help in understanding how and when incontinence occurs, and its impact at an individual level.

The aims of our study were to define the extent, severity and types of faecal incontinence in children after surgical repair of anorectal malformations, as well as its impact on health care usage, medication, dietary intake, and quality of life. We also wished to relate these outcomes to the level of abnormality, whether "high", "intermediate" or "low".

© 2014 Catto-Smith et al.; licensee InTech. This is a paper distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

### **2. Methods**

#### **2.1. Ethical approval**

Ethical approval for this study was obtained from the Ethics in Human Research Committee of the Royal Children's Hospital (Approval number EHRC 21118B)

("light" and "heavy") using the amount and frequency of episode. "Heavy" soiling was classified as being larger than a streak (able to be scraped from underclothing) and occurring at least once a month. "Light" soiling was classified as being no larger than a streak (unable to be scraped from underclothing) and occurring less than once a month. Functional testing,

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072 163

Statistical analysis was performed using SPSS version 12.0 (Statistical Package for the Social Sciences). Where appropriate, we used t-test, One-Way ANOVA, Chi-square or Fisher's Exact test, Mann-Whitney U Test or Kruskal-Wallis Test. Results were considered significant if p<0.05. Data are reported as mean ± standard deviation. Patients who were still in nappies because they were too young to be toilet trained were excluded from some analyses. Missing

353 patients were identified from ICD coding. We excluded 21 patients for whom we could not obtain permission from the treating surgeon. This was primarily because these surgeons were no longer in practice and had the effect of reducing the number of older patients. A further 34 patients were excluded who were deceased, 2 from overseas, 2 who needed an interpreter and 8 with inadequate contact details. 286 recruitment packs were mailed. Of these, 67 were returned by the post office with "wrong address" and no forwarding details, and no responses were obtained from a further 110 patients. 35 respondents declined or were unable to partic‐ ipate in the study. 74 respondents agreed to participate in the study, of whom 67 were interviewed. Two of the interviewed patients were later excluded because of an incorrect diagnosis. 57 were interviewed over the phone, 7 face to face and 1 via mailed questionnaires.

The average age of the 62 interviewed patients without Down's was 11.4 ± 6.4yr; 41 were male and 21 female. The average age of the patients who were presumed to be alive but not interviewed was 18.2 ± 8.4yr of whom 428 were male and 275 female. Interviewed patients were younger (p<0.001) but gender proportions were similar (p=0.59). Four patients who wore nappies were excluded from some analyses. Two wore nappies only overnight (mean age 5.2 ± 1.6yr) and two were too young to be toilet trained, wearing nappies both day and night (mean

There were 28 patients with high/intermediate and 34 with low anorectal malformations. Both groups were similar in age, occupational prestige and family status. Eight patients who had

12 patients underwent anorectal manometry and electromyography.

including anorectal manometry and electromyography, was offered [15].

Data from patients who had Down's syndrome was analysed separately.

**2.4. Statistics**

**3. Results**

age 2.4 ± 0.1yr).

*Clinical Data (n=62) (Table 1)*

data are reflected in *n* values.

Three patients had Down's syndrome.

#### **2.2. Subjects**

We planned to recruit all patients who had presented for treatment of anorectal malformations to the Royal Children's Hospital, Melbourne between 1974 and 2001. Subjects were identified through ICD coding of medical records. We included patients coded with the diagnoses of anorectal agenesis, rectal atresia, anal agenesis, anorectal stenosis (1970-80; ICD 751.30-33); colonic atresia and stenosis, imperforate anus, anorectal agenesis, anal agenesis (1981-88; ICD 751.20-23); atresia and stenosis of large intestine, rectum and anal canal (1989-2002; ICD-10- AM, 751.2, q42.1-3, q42.18, q 43.6).

We excluded those who were deceased, or non-English speaking, or permanently resident overseas. Clinical information was obtained from medical records. Patients were sub-divided into either a combined high and intermediate category or a low malformation category based on coding information. We also inspected operative notes and separately confirmed correct classification of the level of malformation using the Wingspread Conference criteria [11]. Most patients had the same classification irrespective of the method used. Discrepancies between the two were resolved after inspection of the notes by a paediatric surgeon (RGT).

Recruitment packs were mailed to the most current address recorded in the hospital records. Each recruitment pack contained a detailed information sheet about the study and an initial consent form to allow further discussion of the project. Upon receipt of the signed consent form, we contacted each subject or parent by phone to provide further detailed information about the project and to schedule a detailed interview to administer a structured questionnaire.

#### **2.3. Questionnaire and diary**

The 70-item questionnaire was administered either in person, by phone or by mail. The questionnaire had been modified from previous studies on outcome after surgical repair of Hirschsprung disease [12-14]. The revised questionnaire incorporated more detailed informa‐ tion related to bowel function, including amount and awareness of soiling as well as consti‐ pation. Further questions were included which related to physical and social functioning, current provision of health care and general health status. The questionnaire was further modified to enable it to be completed by patients as well as parents.

The revised questionnaire was piloted on 21 patients with Hirschsprung's disease. No significant further modifications were required.

Following the interview, respondents were asked to complete a four-week diary detailing bowel habit, faecal soiling, abdominal pain, enuresis and any medical therapy. This was also based on the diary used for the previous study [12]. Soiling was classified into two groups ("light" and "heavy") using the amount and frequency of episode. "Heavy" soiling was classified as being larger than a streak (able to be scraped from underclothing) and occurring at least once a month. "Light" soiling was classified as being no larger than a streak (unable to be scraped from underclothing) and occurring less than once a month. Functional testing, including anorectal manometry and electromyography, was offered [15].

Data from patients who had Down's syndrome was analysed separately.

#### **2.4. Statistics**

**2. Methods**

**2.2. Subjects**

**2.1. Ethical approval**

162 Fecal Incontinence - Causes, Management and Outcome

AM, 751.2, q42.1-3, q42.18, q 43.6).

**2.3. Questionnaire and diary**

Ethical approval for this study was obtained from the Ethics in Human Research Committee

We planned to recruit all patients who had presented for treatment of anorectal malformations to the Royal Children's Hospital, Melbourne between 1974 and 2001. Subjects were identified through ICD coding of medical records. We included patients coded with the diagnoses of anorectal agenesis, rectal atresia, anal agenesis, anorectal stenosis (1970-80; ICD 751.30-33); colonic atresia and stenosis, imperforate anus, anorectal agenesis, anal agenesis (1981-88; ICD 751.20-23); atresia and stenosis of large intestine, rectum and anal canal (1989-2002; ICD-10-

We excluded those who were deceased, or non-English speaking, or permanently resident overseas. Clinical information was obtained from medical records. Patients were sub-divided into either a combined high and intermediate category or a low malformation category based on coding information. We also inspected operative notes and separately confirmed correct classification of the level of malformation using the Wingspread Conference criteria [11]. Most patients had the same classification irrespective of the method used. Discrepancies between

Recruitment packs were mailed to the most current address recorded in the hospital records. Each recruitment pack contained a detailed information sheet about the study and an initial consent form to allow further discussion of the project. Upon receipt of the signed consent form, we contacted each subject or parent by phone to provide further detailed information about the project and to schedule a detailed interview to administer a structured questionnaire.

The 70-item questionnaire was administered either in person, by phone or by mail. The questionnaire had been modified from previous studies on outcome after surgical repair of Hirschsprung disease [12-14]. The revised questionnaire incorporated more detailed informa‐ tion related to bowel function, including amount and awareness of soiling as well as consti‐ pation. Further questions were included which related to physical and social functioning, current provision of health care and general health status. The questionnaire was further

The revised questionnaire was piloted on 21 patients with Hirschsprung's disease. No

Following the interview, respondents were asked to complete a four-week diary detailing bowel habit, faecal soiling, abdominal pain, enuresis and any medical therapy. This was also based on the diary used for the previous study [12]. Soiling was classified into two groups

modified to enable it to be completed by patients as well as parents.

significant further modifications were required.

the two were resolved after inspection of the notes by a paediatric surgeon (RGT).

of the Royal Children's Hospital (Approval number EHRC 21118B)

Statistical analysis was performed using SPSS version 12.0 (Statistical Package for the Social Sciences). Where appropriate, we used t-test, One-Way ANOVA, Chi-square or Fisher's Exact test, Mann-Whitney U Test or Kruskal-Wallis Test. Results were considered significant if p<0.05. Data are reported as mean ± standard deviation. Patients who were still in nappies because they were too young to be toilet trained were excluded from some analyses. Missing data are reflected in *n* values.

#### **3. Results**

353 patients were identified from ICD coding. We excluded 21 patients for whom we could not obtain permission from the treating surgeon. This was primarily because these surgeons were no longer in practice and had the effect of reducing the number of older patients. A further 34 patients were excluded who were deceased, 2 from overseas, 2 who needed an interpreter and 8 with inadequate contact details. 286 recruitment packs were mailed. Of these, 67 were returned by the post office with "wrong address" and no forwarding details, and no responses were obtained from a further 110 patients. 35 respondents declined or were unable to partic‐ ipate in the study. 74 respondents agreed to participate in the study, of whom 67 were interviewed. Two of the interviewed patients were later excluded because of an incorrect diagnosis. 57 were interviewed over the phone, 7 face to face and 1 via mailed questionnaires. Three patients had Down's syndrome.

The average age of the 62 interviewed patients without Down's was 11.4 ± 6.4yr; 41 were male and 21 female. The average age of the patients who were presumed to be alive but not interviewed was 18.2 ± 8.4yr of whom 428 were male and 275 female. Interviewed patients were younger (p<0.001) but gender proportions were similar (p=0.59). Four patients who wore nappies were excluded from some analyses. Two wore nappies only overnight (mean age 5.2 ± 1.6yr) and two were too young to be toilet trained, wearing nappies both day and night (mean age 2.4 ± 0.1yr).

12 patients underwent anorectal manometry and electromyography.

#### *Clinical Data (n=62) (Table 1)*

There were 28 patients with high/intermediate and 34 with low anorectal malformations. Both groups were similar in age, occupational prestige and family status. Eight patients who had developed significant faecal incontinence had undergone redo posterior sagittal ano-recto‐ plasty (PSARP) [16]. Of these, seven had high/intermediate malformations. Patients with high/ intermediate malformations were more likely to have co-morbidity.

more likely to have had episodes of faecal soiling during the last year when they also reported problems with sensation, holding back stool, urgency and stool discrimination. (Impaired sensation of impending stool: soiling 17/35 vs no soiling 3/26, p<0.05; inability to hold back stool: soiling 22/38 vs no soiling 0/15, p<0.001; faecal urgency: soiling 34/42 vs no soiling 8/15, p<0.05; or problems with stool discrimination: soiling 14/30 vs no soiling 1/14, p<0.05).

(n=53) Never/Rarely/Sometimes 15 (62.5) 5 (17.2) 20 (37.7)

(n=36) Rarely/Sometimes 9 (69.2) 12 (52.2) 21 (58.3)

(n=55) Never/Rarely/Sometimes 14 (53.8) 8 (27.6) 22 (40)

(n=34) Formed & liquid 0 (-) 1 (5.3) 1 (2.9)

(n=29) Never/Rarely/Sometimes 7 (50) 8 (53.3) 0.86 15 (51.7)

Most patients (44/60, 73%) had soiling accidents at least once in the past year and this happened "often or always" in 22. 24 soiled only during the day and 20 soiled both during the day and

Sensation impending stool

Faecal urgency – frequency

Ability to hold back stool

If able to hold back stool – how long? (n=44)

Discriminates stool consistencies (n=45)

Stool discrimination type

Uncontrolled flatus

**Table 3.** Continence data for interviewed patients.

Faecal urgency (n=57)

**High/Intermediate n (%)**

Often/Always 9 (37.5) 24 (82.8) 0.00 33 (62.3)

Yes 17 (65.4) 25 (80.6) 0.19 42 (73.7)

Often/Always 4 (30.8) 11 (47.8) 0.32 15 (41.7)

Often/Always 12 (46.2) 21 (72.4) 0.05 33 (60)

Seconds 4 (21.0) 2 (8) 6 (13.6)

Couple minutes 12 (63.2) 16 (64) 28 (63.6) Greater than 30 mins 3 (15.8) 7 (28) 0.38 10 (22.7)

Never/Sometimes 9 (45) 6 (24) 15 (33.3)

Always 11 (55) 19 (76) 0.14 30 (66.7)

Formed & gaseous 12 (80) 11 (57.9) 23 (67.7) Formed & liquid & gaseous 3 (20) 7 (36.8) 0.36 10 (29.4)

Often / Always 7 (50) 7 (46.7) 14 (48.3)

**Low n (%) <sup>p</sup>**

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072

> **Total n (%)**

165


**Table 1.** Clinical Data for interviewed patients.

#### *Bowel Habit (n=60) (Table 2)*

Patients with a past history of either high/intermediate or low malformations passed a similar number of bowel actions each week (5.5 ± 1.8). Patients with a history of PSARP had more bowel actions each week, although this just failed to reach statistical significance (PSARP 6.6 ± 1.1 vs no PSARP 5.3 ± 1.8, p=0.052). Patients with high/intermediate malformations were more likely to have a looser (liquid or pasty) stool consistency.


**Table 2.** Bowel habit data for interviewed patients.

#### *Faecal Continence (n=57) (Table 3)*

Impairment in sensation of impending stool was common (38%), as was faecal urgency (74%), difficulty in holding back stool (40%) or controlling flatus (48%) and discriminating stool type (33%).

Patients with high/intermediate malformations were more likely to have difficulty sensing impending stool and holding it back. Younger patients were more likely to experience episodes of faecal urgency (urgency: young 0-11yr 30/36 vs old 12-33yr 12/21, p<0.05). Patients were more likely to have had episodes of faecal soiling during the last year when they also reported problems with sensation, holding back stool, urgency and stool discrimination. (Impaired sensation of impending stool: soiling 17/35 vs no soiling 3/26, p<0.05; inability to hold back stool: soiling 22/38 vs no soiling 0/15, p<0.001; faecal urgency: soiling 34/42 vs no soiling 8/15, p<0.05; or problems with stool discrimination: soiling 14/30 vs no soiling 1/14, p<0.05).


**Table 3.** Continence data for interviewed patients.

developed significant faecal incontinence had undergone redo posterior sagittal ano-recto‐ plasty (PSARP) [16]. Of these, seven had high/intermediate malformations. Patients with high/

Age (years) 11.8 ± 6.2 11.1 ± 6.7 0.65 11.4 ± 6.4 Gender 21M:7F 20M:14F 0.18 41M:21F Daniel Occupational Prestige Score 4.4 ± 1.0 4.3 ± 0.9 0.81 4.4 ± 1.0 Single Parent Family n (%) n=62 7 (11.5) 7 (11.5) 0.68 14 (23) Co-morbidities n (%) n=62 21 (34) 15 (24) 0.01 36 (58) Subsequent PSARP 7 (87.5) 1 (12.5) 0.02 8 (100)

Patients with a past history of either high/intermediate or low malformations passed a similar number of bowel actions each week (5.5 ± 1.8). Patients with a history of PSARP had more bowel actions each week, although this just failed to reach statistical significance (PSARP 6.6 ± 1.1 vs no PSARP 5.3 ± 1.8, p=0.052). Patients with high/intermediate malformations were

Bowel actions per week 5.9 ± 1.6 5.2 ± 1.9 0.13 5.5 ± 1.9

Impairment in sensation of impending stool was common (38%), as was faecal urgency (74%), difficulty in holding back stool (40%) or controlling flatus (48%) and discriminating stool type

Patients with high/intermediate malformations were more likely to have difficulty sensing impending stool and holding it back. Younger patients were more likely to experience episodes of faecal urgency (urgency: young 0-11yr 30/36 vs old 12-33yr 12/21, p<0.05). Patients were

**High / Intermediate Low p Total**

Liquid 3 (10.7) 1 (3.1) 4 (6.7) Pasty 11 (39.3) 6 (18.8) 17 (28.3) Formed 7 (25) 21 (65.6) 28 (46.7) Variable 7 (25) 4 (12.5) 0.01 11 (18.3)

**HD High/Intermediate (n=28) Low (n=34) p Total**

intermediate malformations were more likely to have co-morbidity.

more likely to have a looser (liquid or pasty) stool consistency.

**Table 1.** Clinical Data for interviewed patients.

164 Fecal Incontinence - Causes, Management and Outcome

*Bowel Habit (n=60) (Table 2)*

Stool consistency n (%)

**Table 2.** Bowel habit data for interviewed patients.

*Faecal Continence (n=57) (Table 3)*

(33%).

Most patients (44/60, 73%) had soiling accidents at least once in the past year and this happened "often or always" in 22. 24 soiled only during the day and 20 soiled both during the day and overnight. There were none who soiled only at night. A higher proportion of females reported soiling episodes (female 19/21 vs male 25/39, p<0.05). 43 patients were able to quantify the usual size of soiling episodes that occurred during the daytime. This was medium to large in 26 (60%). Of the 18 who were able to describe the consistency of daytime soiling, 9 (50%) indicated that it was pasty. Eleven had episodes of daytime soiling but were usually unaware that these were occurring (more than 75% of the time). Nocturnal soiling was of pasty consistency in 60% (3/5) patients and medium to large amount in 43% (6/14).

Patients with a past history of high/intermediate malformations were more likely to "often or always" soil during the daytime than those with low malformations (soiling often/always: high/intermediate 15/28 vs low 7/31, p<0.05). Despite their revision, those with a PSARP still soiled more frequently (soiling often/always: PSARP 7/8 vs no PSARP 15/51, p<0.01). They were also more likely to soil at night (soiling often/always: PSARP 2/7 vs no PSARP 1/48, p<0.05).

Most patients could be classified as having either heavy or light soiling based upon frequency and amount of episodes. Nine patients (daytime soiling 1, nocturnal soiling 9) were unable to accurately define the amount or frequency of their soiling episodes. A third (12/33) of patients had "heavy" soiling. "Heavy" soiling was more likely to occur in patients with a high/ intermediate malformation ("heavy"soiling: high/intermediate 10/16 vs low 2/17, p<0.01). It was also more common in males ("heavy" soiling: male 11/20 vs female 1/13, p<0.01) and patients who had undergone a PSARP ("heavy"soiling: PSARP 4/6, no PSARP 8/27, p<0.05).

#### *Constipation (n=61) (Table 4)*

We defined constipation as fewer than 3 stools per week or straining at stool for more than 25% of the time. Only 13% reported constipation occurring more than once a week. However, episodic constipation did occur. Twelve reported infrequent stools, 34 had hard stools and 39 had difficulty evacuating stools during the past year.

Patients with a low malformation were more likely to have had episodes of infrequent stooling during the past year (p<0.01).

#### *Diarrhoea and rectal prolapse (n=57) (Table 5)*

12% (7/57) of patients reported that episodes of diarrhoea occurred more than once a week. Patients with high/intermediate malformations were more likely to experience frequent episodes of diarrhoea.

*Enuresis (n=56)*

Constipation

Infrequent stool (in past year) n (%) (n=60)

If infrequent stools, number of episodes in last 3 months (n=9)

Hard stools (in past year) n (%)(n=60)

If hard stools, number of episodes in last 3 months (n=26)

Difficulty in evacuating stool (in past year)

n (%) (n=60) If difficulty in evacuating stool, number of episodes in last 3 months (n=20)

last week.

9.0 ± 3.7yr vs no enuresis 13.1 ± 6.6yr, p<0.05).

**Table 4.** Constipation data for interviewed patients.

36% (20/56) of patients had enuresis at least once in the last 3 months. Nine had daytime enuresis, 8 had nocturnal enuresis and 3 had enuresis both during the day and at night. 16% of patients with daytime enuresis and 13% with night-time enuresis had an episode within the

**High/Intermediate n (%)**

Often/Always 4 (14.8) 4(11.8) 1.00 8(13.1)

Yes 1 (3.7) 11 (34.4) 0.00 12 (20.3)

Yes 15 (53.6) 19 (59.4) 0.58 34 (56.7)

Yes 20 (71.4) 19 (59.4) 0.33 39 (65)

(n=61) Never/Rarely/Sometimes 23 (85.2) 30(88.2) 53(86.9)

**Low n (%) <sup>p</sup>**

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072

3.0 ± (-) 2.8±0.9 0.91 2.9±0.9

5.5 ± 6.5 3.5 ± 3.3 0.35 4.5 ± 5.1

7.0 ± 7.7 5.4 ± 7.1 0.63 6.1 ± 7.2

**Total n (%)** 167

Patients with either daytime or nocturnal enuresis were younger than those who did not have enuresis (daytime enuresis 7.9 ± 5.0yr vs no enuresis 12.7 ± 6.3yr, p<0.05; nocturnal enuresis

Three patients (6% of 52) had constant anal excoriation and one had a rectal prolapse during the past year. Patients with a high/intermediate malformation were more likely to have perianal excoriation. Patients who had undergone a PSARP were also more likely to have perianal excoriation (excoriation: PSARP 3/8 vs no PSARP 6/44, p<0.05).

70% (42/60) of patients had episodic abdominal pain lasting more than two minutes. Of these, 10 reported abdominal pain 1 to 3 times per week and 9 reported pain 1 to 2 times per month. This was equally frequent in patients with a past history of either high/intermediate or low malformations.


**Table 4.** Constipation data for interviewed patients.

#### *Enuresis (n=56)*

overnight. There were none who soiled only at night. A higher proportion of females reported soiling episodes (female 19/21 vs male 25/39, p<0.05). 43 patients were able to quantify the usual size of soiling episodes that occurred during the daytime. This was medium to large in 26 (60%). Of the 18 who were able to describe the consistency of daytime soiling, 9 (50%) indicated that it was pasty. Eleven had episodes of daytime soiling but were usually unaware that these were occurring (more than 75% of the time). Nocturnal soiling was of pasty

Patients with a past history of high/intermediate malformations were more likely to "often or always" soil during the daytime than those with low malformations (soiling often/always: high/intermediate 15/28 vs low 7/31, p<0.05). Despite their revision, those with a PSARP still soiled more frequently (soiling often/always: PSARP 7/8 vs no PSARP 15/51, p<0.01). They were also more likely to soil at night (soiling often/always: PSARP 2/7 vs no PSARP 1/48,

Most patients could be classified as having either heavy or light soiling based upon frequency and amount of episodes. Nine patients (daytime soiling 1, nocturnal soiling 9) were unable to accurately define the amount or frequency of their soiling episodes. A third (12/33) of patients had "heavy" soiling. "Heavy" soiling was more likely to occur in patients with a high/ intermediate malformation ("heavy"soiling: high/intermediate 10/16 vs low 2/17, p<0.01). It was also more common in males ("heavy" soiling: male 11/20 vs female 1/13, p<0.01) and patients who had undergone a PSARP ("heavy"soiling: PSARP 4/6, no PSARP 8/27, p<0.05).

We defined constipation as fewer than 3 stools per week or straining at stool for more than 25% of the time. Only 13% reported constipation occurring more than once a week. However, episodic constipation did occur. Twelve reported infrequent stools, 34 had hard stools and 39

Patients with a low malformation were more likely to have had episodes of infrequent stooling

12% (7/57) of patients reported that episodes of diarrhoea occurred more than once a week. Patients with high/intermediate malformations were more likely to experience frequent

Three patients (6% of 52) had constant anal excoriation and one had a rectal prolapse during the past year. Patients with a high/intermediate malformation were more likely to have perianal excoriation. Patients who had undergone a PSARP were also more likely to have

70% (42/60) of patients had episodic abdominal pain lasting more than two minutes. Of these, 10 reported abdominal pain 1 to 3 times per week and 9 reported pain 1 to 2 times per month. This was equally frequent in patients with a past history of either high/intermediate or low

perianal excoriation (excoriation: PSARP 3/8 vs no PSARP 6/44, p<0.05).

consistency in 60% (3/5) patients and medium to large amount in 43% (6/14).

p<0.05).

*Constipation (n=61) (Table 4)*

166 Fecal Incontinence - Causes, Management and Outcome

during the past year (p<0.01).

episodes of diarrhoea.

malformations.

*Diarrhoea and rectal prolapse (n=57) (Table 5)*

had difficulty evacuating stools during the past year.

36% (20/56) of patients had enuresis at least once in the last 3 months. Nine had daytime enuresis, 8 had nocturnal enuresis and 3 had enuresis both during the day and at night. 16% of patients with daytime enuresis and 13% with night-time enuresis had an episode within the last week.

Patients with either daytime or nocturnal enuresis were younger than those who did not have enuresis (daytime enuresis 7.9 ± 5.0yr vs no enuresis 12.7 ± 6.3yr, p<0.05; nocturnal enuresis 9.0 ± 3.7yr vs no enuresis 13.1 ± 6.6yr, p<0.05).


54% (33/61) of patients either partially or totally restricted some foods from their diet because of these adverse effects. The most frequently restricted food groups were dairy (22%), fatty or

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072 169

Over half (34/62) of patients had used a medication or other treatment for bowel related problems in the past year. 29 patients used laxatives, 2 used anti-diarrhoeals and 10 used bowel washouts. Most (27/34) patients were using some form of treatment at the time of interview. Medication-users at the time of interview were significantly younger than those who did not use medication (current medication use 9.2 ± 4.7yr vs no medication 10.4 ± 5.3yr, p<0.05). Of those who were using medication, 12 were using more than one type of laxative. Those with a high/intermediate malformation were more likely to be using a medication (high/intermedi‐ ate 20/28 vs low 14/34, p<0.05). Patients with soiling were also more likely to be using medi‐ cation (soiling 27/44 vs no soiling 5/16, p<0.05). Those who used laxatives were significantly younger than those who used anti-diarrhoeals (mean age: laxative 8.9 ± 4.3yr vs anti-diarrhoeal

6% (4/62) of patients had limited their physical activities because of soiling or odour.

Soiling interfered with social activities of 11 (19%) patients. Of these, 9 had some parental or self-imposed restrictions and 2 had extreme limitations. As expected, patients with soiling were more likely to have social limitations than patients without soiling (social limitation: soiling 11/40 vs no soiling 0/16, p<0.05). Odour from soiling was also responsible for interfer‐ ence with social activities in 14% (8/58) of patients. Of these, 5 patients were rarely affected

When questioned on the level of dependency on toilet facilities, 3.5% (2/57) of patients had used toilet facilities at least once every 30 minutes, and 5% (3/57) had to use toilet facilities at

On average, patients had been absent from school 0.8 ± 2.4 days in the past two school terms. Two of the 9 patients who were in part-time or full-time work reported some limitations to

58% (34/59) patients had not had a follow-up visit for their bowel in over a year. Of these, 15 had not had a follow-up visit in the past 5 years. Patients who had follow-up visits within the last year were younger (<1yr follow-up: young 0-11yr 20/39 vs old 12-33yr 5/20, p<0.001) and have a high/intermediate malformation (p<0.05). Patients with soiling were more likely to have had a follow-up visit in the past year (<1yr follow-up: soiling 19/41 vs no soiling 4/16, p<0.05). 12% (6/49) of patients who attended school had an integration aid for bowel related problems

37% (23/62) of patients received financial assistance from the Commonwealth Government because of health issues (Health Care card). Patients with high/intermediate malformations

fast foods (21%), fruit (17%) and vegetables (16%).

*Medication (n=63)*

19.9 ± 2.4yr, p<0.05).

*Physical and Social Aspects*

and 3 were frequently affected.

their employment because of bowel dysfunction.

least once every hour.

*Health Care (n=62)*

(ie. cleaning after soiling).

**Table 5.** Diarrhoea data for interviewed patients.

#### *Continence Aids (n=58)*

29% (17/58) of patients used continence aids for soiling (6/17), enuresis (6/17) or both (5/17). Aids were used more frequently at night (12/58) than during the day (9/58). Daytime use was primarily for soiling, however nocturnal use was primarily for enuresis.

Patients with high/intermediate malformations were more likely to use aids at night (high/ intermediate 8/26 vs low 4/32, p<0.05). Those who used aids overnight were younger than those who did not use them (continence aid use 7.7 ±3.6yr vs no use 12.7 ± 6.7yr, p<0.05).

#### *Diet (n=61)*

Over half (35/61) of patients reported adverse effects after some foods. Of these, 14 (40%) reported problems with fruit, 11 (31%) with vegetables, 16 (46%) with dairy, 12 (34%) with grains/breads/cereals, 13 (37%) with fatty or fast foods, 1 (3%) with meat, and 8 (23%) with other foods.

Overall, the most commonly reported adverse effects were diarrhoea (60%), constipation (28%) and abdominal discomfort (5%). Symptoms after fruit included diarrhoea (9/14, 64%), consti‐ pation (4/14, 29%) and peri-anal discomfort (2/14, 14%). Ingestion of vegetables was associated with diarrhoea (9/11, 82%) and constipation (1/11, 9%). Symptoms after dairy included diarrhoea (6/16, 38%), constipation (8/16, 50%) and abdominal discomfort (2/16, 13%). Grains/ breads/cereals induced diarrhoea (7/12, 58%), constipation (3/12, 25%) and abdominal dis‐ comfort (1/12, 8%). Symptoms after fatty or fast foods included diarrhoea (7/13, 54%) and constipation (5/13, 38%). One patient experienced diarrhoea after meat.

54% (33/61) of patients either partially or totally restricted some foods from their diet because of these adverse effects. The most frequently restricted food groups were dairy (22%), fatty or fast foods (21%), fruit (17%) and vegetables (16%).

#### *Medication (n=63)*

Over half (34/62) of patients had used a medication or other treatment for bowel related problems in the past year. 29 patients used laxatives, 2 used anti-diarrhoeals and 10 used bowel washouts. Most (27/34) patients were using some form of treatment at the time of interview. Medication-users at the time of interview were significantly younger than those who did not use medication (current medication use 9.2 ± 4.7yr vs no medication 10.4 ± 5.3yr, p<0.05). Of those who were using medication, 12 were using more than one type of laxative. Those with a high/intermediate malformation were more likely to be using a medication (high/intermedi‐ ate 20/28 vs low 14/34, p<0.05). Patients with soiling were also more likely to be using medi‐ cation (soiling 27/44 vs no soiling 5/16, p<0.05). Those who used laxatives were significantly younger than those who used anti-diarrhoeals (mean age: laxative 8.9 ± 4.3yr vs anti-diarrhoeal 19.9 ± 2.4yr, p<0.05).

#### *Physical and Social Aspects*

6% (4/62) of patients had limited their physical activities because of soiling or odour.

Soiling interfered with social activities of 11 (19%) patients. Of these, 9 had some parental or self-imposed restrictions and 2 had extreme limitations. As expected, patients with soiling were more likely to have social limitations than patients without soiling (social limitation: soiling 11/40 vs no soiling 0/16, p<0.05). Odour from soiling was also responsible for interfer‐ ence with social activities in 14% (8/58) of patients. Of these, 5 patients were rarely affected and 3 were frequently affected.

When questioned on the level of dependency on toilet facilities, 3.5% (2/57) of patients had used toilet facilities at least once every 30 minutes, and 5% (3/57) had to use toilet facilities at least once every hour.

On average, patients had been absent from school 0.8 ± 2.4 days in the past two school terms.

Two of the 9 patients who were in part-time or full-time work reported some limitations to their employment because of bowel dysfunction.

#### *Health Care (n=62)*

*Continence Aids (n=58)*

**Table 5.** Diarrhoea data for interviewed patients.

168 Fecal Incontinence - Causes, Management and Outcome

Abdominal pain

*Diet (n=61)*

other foods.

29% (17/58) of patients used continence aids for soiling (6/17), enuresis (6/17) or both (5/17). Aids were used more frequently at night (12/58) than during the day (9/58). Daytime use was

Diarrhoea (n=57) Never/Rarely/Sometimes 20(76.9) 30(96.8) 50(87.7)

Excoriation (n=52) Never 17(68) 26(96.3) 43(82.7)

Rectal prolapse (n=59) Yes 1(3.7) 0(-) 0.46 1(1.7) Abdominal pain (n=60) Yes 21(75) 21(65.6) 0.43 42(70)

(frequency) (n=32) 1-6 times in past yr 6(37.4) 7(43.8) 13(40.6)

**High/Intermediate n (%)**

Often / Always 6(23.1) 1(3.2) 0.04 7(12.3)

Sometimes 5(20) 1(3.7) 6(11.5) Always 3(12) 0(-) 0.02 3(5.8)

1-2 times in past month 5(31.3) 4(25) 9(28.1) 1-3 times in past wk 5(31.3) 5(31.2) 1.00 10(31.3)

**Low n (%) <sup>p</sup>** **Total n (%)**

Patients with high/intermediate malformations were more likely to use aids at night (high/ intermediate 8/26 vs low 4/32, p<0.05). Those who used aids overnight were younger than those

Over half (35/61) of patients reported adverse effects after some foods. Of these, 14 (40%) reported problems with fruit, 11 (31%) with vegetables, 16 (46%) with dairy, 12 (34%) with grains/breads/cereals, 13 (37%) with fatty or fast foods, 1 (3%) with meat, and 8 (23%) with

Overall, the most commonly reported adverse effects were diarrhoea (60%), constipation (28%) and abdominal discomfort (5%). Symptoms after fruit included diarrhoea (9/14, 64%), consti‐ pation (4/14, 29%) and peri-anal discomfort (2/14, 14%). Ingestion of vegetables was associated with diarrhoea (9/11, 82%) and constipation (1/11, 9%). Symptoms after dairy included diarrhoea (6/16, 38%), constipation (8/16, 50%) and abdominal discomfort (2/16, 13%). Grains/ breads/cereals induced diarrhoea (7/12, 58%), constipation (3/12, 25%) and abdominal dis‐ comfort (1/12, 8%). Symptoms after fatty or fast foods included diarrhoea (7/13, 54%) and

who did not use them (continence aid use 7.7 ±3.6yr vs no use 12.7 ± 6.7yr, p<0.05).

primarily for soiling, however nocturnal use was primarily for enuresis.

constipation (5/13, 38%). One patient experienced diarrhoea after meat.

58% (34/59) patients had not had a follow-up visit for their bowel in over a year. Of these, 15 had not had a follow-up visit in the past 5 years. Patients who had follow-up visits within the last year were younger (<1yr follow-up: young 0-11yr 20/39 vs old 12-33yr 5/20, p<0.001) and have a high/intermediate malformation (p<0.05). Patients with soiling were more likely to have had a follow-up visit in the past year (<1yr follow-up: soiling 19/41 vs no soiling 4/16, p<0.05).

12% (6/49) of patients who attended school had an integration aid for bowel related problems (ie. cleaning after soiling).

37% (23/62) of patients received financial assistance from the Commonwealth Government because of health issues (Health Care card). Patients with high/intermediate malformations (Health Care card: high/intermediate 15/28 vs low 8/34, p<0.05) and patients with soiling were more likely to have a health care card (Health Care card: soiling 21/44 vs no soiling 2/16, p<0.05) for bowel related problems.

27% (17/62) of patients received financial assistance in the form of a Disability allowance or their families received Carer's allowances. Patients with soiling were more likely to have received Disability allowances (Disability allowance: soiling 16/54 vs no soiling 1/16, p<0.05) for bowel related problems.

#### *Continence and Quality of Life Scores*

Templeton continence scores could only be determined for 23% (14/62) of patients. Based on the Templeton classification, 3 patients had "poor", 6 "fair" and 5 had "good" continence.

The requirement for digital anal examination meant that Holschneider classification of continence was able only used in 23% of patients. Four were classified as having "fair" continence, 7 "good" and 3 "normal".

Classification of quality of life using the Ditesheim and Templeton scale was valid for 85% (53/62) of patients. Five patients had "fair" quality of life and 48 "good".

#### *Manometry (n=12) (Table 6)*

Baseline anal sphincter pressures were significantly less than normal reference ranges, but there was little difference between those who did and did not soil. Recto-anal inhibitory reflexes were present in 5 patients, but the threshold volume for eliciting the reflex was significantly greater than normal. Sensation to rectal distension was also blunted. Most were unable to evacuate a water filled rectal balloon normally.

#### *Down's Syndrome Group (n=3)*

The three patients with Down's syndrome and high/intermediate anorectal malformations were 12 ± 3.4 years old. Two were male and one female. Two had a redo PSARP operation. Comorbidities included cardiac, gastrointestinal (non colorectal), renal and respiratory.

No patient had any limitations to their physical activities but one did limit social activities because of odour from soiling, and one needed access to toilet facilities every 30 minutes.

Sensation to distension (mL) 69±79.9 90±65.4 0.62 80.5±69.4 14±7 <0.01

Only two patients had a follow-up for their bowel in the past 12 months. One was using laxatives at the time of interview for bowel control and another patient had used laxatives in

All three had adverse effects from foods. These foods included fruit, vegetables and fatty or fast foods. The effects from these foods included diarrhoea and abdominal discomfort.

**•** One parent felt as though diagnosis (of ARM) could have been made sooner if they (parents) had been more assertive. It took a day to diagnose although the Mother knew that something

was not right with her son after birth, despite the nurses saying that he was fine.

All patients had a health care card and received disability allowance.

**Soiling Never//Sometimes**

• Normal 1 1 2 • Present but abnormal 1 2 3 • Not present 1 4 0.64 5

• Relaxation 2 1 3 • No change 2 2 4

50mL 1 2 3 30mL 0 0 0 Unable 2 4 6

• Anismus 0 2 0.33 2 10%

100mL 1 1 2 100%

Baseline sphincter pressure

Recto-anal inhibitory reflex

Recto-anal inhibitory reflex (if present) – threshold (mL)

Ability to evacuate water-

(mean±SD)

Co-ordination

filled balloon

**Soiling**

**Often/Always <sup>p</sup> Total**

38.1±20.8 37.9±20.8 0.99 38±19.4 53±12 <0.01

35±7.1 32.5±15 0.84 33.3±12.1 16±7 <0.01

**Historical normal values <sup>p</sup>** 171

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072

Social impact of incontinence – parental free comments (overall group of 62)

the past year but had ceased 4 months prior.

**Table 6.** Manometry results from 12 patients.

Earlier diagnosis:

All three had a bowel action each day. Stool consistency was formed in two and pasty in one patient. Two patients could hold back stool more than 75% of the time.

All three had soiled within the past year. Three "often" had daytime soiling episodes. Daytime soiling varied between a pasty to formed consistency and streak to large amount. One "often" soiled nocturnally and two wore nappies at night.

Only one patient had constipation and abdominal pain. The other two had episodic diarrhoea, and one also had anal excoriation.

One patient had both day and night enuresis and one only had daytime enuresis in the last week.

One patient used incontinence aids day and night for enuresis and soiling and another also used incontinence aids for enuresis and soiling but only at night.


**Table 6.** Manometry results from 12 patients.

No patient had any limitations to their physical activities but one did limit social activities because of odour from soiling, and one needed access to toilet facilities every 30 minutes.

All patients had a health care card and received disability allowance.

Only two patients had a follow-up for their bowel in the past 12 months. One was using laxatives at the time of interview for bowel control and another patient had used laxatives in the past year but had ceased 4 months prior.

All three had adverse effects from foods. These foods included fruit, vegetables and fatty or fast foods. The effects from these foods included diarrhoea and abdominal discomfort.

Social impact of incontinence – parental free comments (overall group of 62)

Earlier diagnosis:

(Health Care card: high/intermediate 15/28 vs low 8/34, p<0.05) and patients with soiling were more likely to have a health care card (Health Care card: soiling 21/44 vs no soiling 2/16, p<0.05)

27% (17/62) of patients received financial assistance in the form of a Disability allowance or their families received Carer's allowances. Patients with soiling were more likely to have received Disability allowances (Disability allowance: soiling 16/54 vs no soiling 1/16, p<0.05)

Templeton continence scores could only be determined for 23% (14/62) of patients. Based on the Templeton classification, 3 patients had "poor", 6 "fair" and 5 had "good" continence.

The requirement for digital anal examination meant that Holschneider classification of continence was able only used in 23% of patients. Four were classified as having "fair"

Classification of quality of life using the Ditesheim and Templeton scale was valid for 85%

Baseline anal sphincter pressures were significantly less than normal reference ranges, but there was little difference between those who did and did not soil. Recto-anal inhibitory reflexes were present in 5 patients, but the threshold volume for eliciting the reflex was significantly greater than normal. Sensation to rectal distension was also blunted. Most were

The three patients with Down's syndrome and high/intermediate anorectal malformations were 12 ± 3.4 years old. Two were male and one female. Two had a redo PSARP operation. Co-

All three had a bowel action each day. Stool consistency was formed in two and pasty in one

All three had soiled within the past year. Three "often" had daytime soiling episodes. Daytime soiling varied between a pasty to formed consistency and streak to large amount. One "often"

Only one patient had constipation and abdominal pain. The other two had episodic diarrhoea,

One patient had both day and night enuresis and one only had daytime enuresis in the last

One patient used incontinence aids day and night for enuresis and soiling and another also

morbidities included cardiac, gastrointestinal (non colorectal), renal and respiratory.

patient. Two patients could hold back stool more than 75% of the time.

used incontinence aids for enuresis and soiling but only at night.

(53/62) of patients. Five patients had "fair" quality of life and 48 "good".

unable to evacuate a water filled rectal balloon normally.

soiled nocturnally and two wore nappies at night.

and one also had anal excoriation.

week.

for bowel related problems.

170 Fecal Incontinence - Causes, Management and Outcome

for bowel related problems.

*Manometry (n=12) (Table 6)*

*Down's Syndrome Group (n=3)*

*Continence and Quality of Life Scores*

continence, 7 "good" and 3 "normal".

**•** One parent felt as though diagnosis (of ARM) could have been made sooner if they (parents) had been more assertive. It took a day to diagnose although the Mother knew that something was not right with her son after birth, despite the nurses saying that he was fine.

#### Genetics:

**•** Two mothers thought there might be a genetic link. One mother was concerned about her future pregnancies. Two mothers were also concerned about the chance of their children's children having malformations.

**4. Discussion**

children.

and quality of life [18-21].

fully case-controlled trials [29].

or have high/intermediate malformations.

This review reaffirms the importance of problems with bowel control in the long term outcome after surgical repair of anorectal malformation. Over two thirds of patients had faecal incon‐ tinence over the past year and half of these had soiling which occurred either often or always. Half of all the patients soiled both during the day and at night. As expected, patients with high/ intermediate malformations were more severely affected. The most severe "heavy" soiling occurred in approximately a fifth of the patients. These patients were more likely to be male

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072 173

There was evidence that bowel control improved with age, but faecal urgency was still a problem in half of those aged 12 years and over. Children with low malformations tended to be less severely affected but there was also a suggestion of a slightly different symptom complex. Constipation was more common in patients with low malformation and diarrhoea more common in patients with high/intermediate malformations. Those with Down syndrome also had a poor outcome but we had no direct comparison group for this small number of

Only one quarter of potentially eligible patients in our study were surveyed. It is possible that this may have skewed the results toward a poorer outcome. Certainly, some studies do show rather better outcomes, with only 20% having faecal incontinence using some scoring systems [17]. However, other studies have shown broadly similar long term outcomes for continence

The long term functional impact in adults upon continence and quality of life can be devas‐ tating. Rintala et al. examined the outcome for adults (mean age 35 years) in terms of quality of life following surgical repair high or intermediate anomalies compared to low anomalies [22, 23]. This pair of controlled studies determined that good continence was present in only 18% of patients with high or intermediate anomalies and in 60% of those with low anomalies. Pelvic dysfunction extended into other areas. Sexual dysfunction was present in 30% of those

Further operations have been offered and there is some evidence supporting a role for posterior sagittal anorectoplasty (PSARP) with or without provision of an antegrade continent enema in selected patients with persistent and unresponsive soiling [24]. However, in our study, soiling persisted even in those patients who had received a PSARP, suggesting that it may

Other therapies have been offered. Biofeedback has been suggested in uncontrolled studies to provide substantial improvement in continence after surgical repair of ARM [25-28]. This should be evaluated critically as biofeedback has shown similar promise in uncontrolled studies of childhood encopresis which disappeared when evaluated within the framework of

Enuresis was also a problem in many patients. Some of these children may have also had urinary system anomalies. Where they occur, it has been suggested that they are at least as

with high or intermediate anomalies and 13% of those with low anomalies.

improve function it does not usually return bowel function to normal.

Social limitations:

**•** One patient was teased at school because she wore a nappy and this would really upset her. Two patients were limited in their social activities because they were embarrassed to sleep over at friends or did not go to camps because of inadequate toilet facilities. School was difficult for one boy because he had to go to the toilet quite frequently during class.

#### Coping:


#### Associated disabilities:

**•** There were some questions by both parents and patients surrounding physical ability and if this was the reason for soiling.

#### Medical interactions:

**•** A few parents wanted more follow-up visits to occur and at more frequent times so they could monitor their child's progress. One parent did not feel that there was a need to see her doctor because he did not offer her any new information.

#### **4. Discussion**

Genetics:

Coping:

Social limitations:

just go nuts".

really helpless.

be in so much pain. Associated disabilities:

Medical interactions:

if this was the reason for soiling.

children having malformations.

172 Fecal Incontinence - Causes, Management and Outcome

**•** Two mothers thought there might be a genetic link. One mother was concerned about her future pregnancies. Two mothers were also concerned about the chance of their children's

**•** One patient was teased at school because she wore a nappy and this would really upset her. Two patients were limited in their social activities because they were embarrassed to sleep over at friends or did not go to camps because of inadequate toilet facilities. School was difficult for one boy because he had to go to the toilet quite frequently during class.

**•** Parents and patients coped with the disease in various ways. Three parents said that you just have to deal with it, with one adding that that was her role as a mother. One family put the disease into perspective for their child by saying that it could be a lot worse. One parent felt that being open and not making a big deal would hopefully decrease the chance of problems in the future. One mother said that you "have to have a sense of humour or you

**•** One parent said that she had to block out how bad the anal dilatations were in order for her to perform them. Two patients said that they had learnt to deal with it, with one stating that there was no need to dwell on it and another saying that you would cope with whatever happened when it came. One mother said that at times her daughter was quite upset with her malformations and procedures, however she understands their need. One patient felt that there is nothing he can do so he just deals with it. One patient always asks his parents why it has happened to him. One patient used to ask why this happened to her and feel

**•** One parent said that the disease affects the whole family, not just the patient. Three parents were quite distressed about having to give their children treatment (medication, bowel washouts or anal dilatations). One parent said that it was upsetting to see their child go through it all. One mother said that it was "mentally destroying" to hear her baby crying all the time when he was passing bowel actions. She said that she did not know how she coped without any help. One patient developed a fear of going to the toilet because he would

**•** There were some questions by both parents and patients surrounding physical ability and

**•** A few parents wanted more follow-up visits to occur and at more frequent times so they could monitor their child's progress. One parent did not feel that there was a need to see

her doctor because he did not offer her any new information.

This review reaffirms the importance of problems with bowel control in the long term outcome after surgical repair of anorectal malformation. Over two thirds of patients had faecal incon‐ tinence over the past year and half of these had soiling which occurred either often or always. Half of all the patients soiled both during the day and at night. As expected, patients with high/ intermediate malformations were more severely affected. The most severe "heavy" soiling occurred in approximately a fifth of the patients. These patients were more likely to be male or have high/intermediate malformations.

There was evidence that bowel control improved with age, but faecal urgency was still a problem in half of those aged 12 years and over. Children with low malformations tended to be less severely affected but there was also a suggestion of a slightly different symptom complex. Constipation was more common in patients with low malformation and diarrhoea more common in patients with high/intermediate malformations. Those with Down syndrome also had a poor outcome but we had no direct comparison group for this small number of children.

Only one quarter of potentially eligible patients in our study were surveyed. It is possible that this may have skewed the results toward a poorer outcome. Certainly, some studies do show rather better outcomes, with only 20% having faecal incontinence using some scoring systems [17]. However, other studies have shown broadly similar long term outcomes for continence and quality of life [18-21].

The long term functional impact in adults upon continence and quality of life can be devas‐ tating. Rintala et al. examined the outcome for adults (mean age 35 years) in terms of quality of life following surgical repair high or intermediate anomalies compared to low anomalies [22, 23]. This pair of controlled studies determined that good continence was present in only 18% of patients with high or intermediate anomalies and in 60% of those with low anomalies. Pelvic dysfunction extended into other areas. Sexual dysfunction was present in 30% of those with high or intermediate anomalies and 13% of those with low anomalies.

Further operations have been offered and there is some evidence supporting a role for posterior sagittal anorectoplasty (PSARP) with or without provision of an antegrade continent enema in selected patients with persistent and unresponsive soiling [24]. However, in our study, soiling persisted even in those patients who had received a PSARP, suggesting that it may improve function it does not usually return bowel function to normal.

Other therapies have been offered. Biofeedback has been suggested in uncontrolled studies to provide substantial improvement in continence after surgical repair of ARM [25-28]. This should be evaluated critically as biofeedback has shown similar promise in uncontrolled studies of childhood encopresis which disappeared when evaluated within the framework of fully case-controlled trials [29].

Enuresis was also a problem in many patients. Some of these children may have also had urinary system anomalies. Where they occur, it has been suggested that they are at least as serious and complex as gastrointestinal anomalies, and contribute substantially to long term morbidity [17].

**Author details**

**References**

Anthony G. Catto-Smith1,3\*, Misel Trajanovska1,4 and Russell Taylor2

1 Dept of Gastroenterology and Clinical Nutrition, Royal Children's Hospital, Australia

Anorectal Malformations and Continence http://dx.doi.org/10.5772/57072 175

[1] Cho S, Moore SP, Fangman T. One Hundred Three Consecutive Patients With Ano‐ rectal Malformations and Their Associated Anomalies. Arch Pediatr Adolesc Med.

[2] Shaul DB, Harrison EA. Classification of anorectal malformations--initial approach, diagnostic tests, and colostomy. Semin Pediatr Surg. 1997 11/1997;6(4):187-95.

[3] Ong NT, Beasley SW. Long-term continence in patients with high and intermediate anorectal anomalies treated by sacroperineal (Stephens) rectoplasty. J Pediatr Surg.

[4] Langemeijer RA, Molenaar JC. Continence after posterior sagittal anorectoplasty. J

[5] Templeton JM, Jr., Ditesheim JA. High imperforate anus--quantitative results of long-

[6] Holschneider AM, Pfrommer W, Gerresheim B. Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch. Eur J Pediatr

[7] Kelly JH. The clinical and radiological assessment of anal continence in childhood.

[8] Kiesewetter WB, Chang JH. Imperforate Anus: a five to thirty year follow-up per‐

[9] Stephens FD, Durham-Smith E. Classification, identification, and assessment of sur‐

gical treatment of anorectal anomalies. Pediatr Surg Int. 1986 1986;1:200-5.

term fecal continence. J Pediatr Surg. 1985 12/1985;20(6):645-52.

\*Address all correspondence to: tony.cattosmith@rch.org.au

2 Dept of Surgery, Royal Children's Hospital, Australia

3 Dept of Paediatrics, University of Melbourne, Australia

2001 5/2001;155(5):587-91.

1991 1/1991;26(1):44-8.

Surg. 1994 Oct;4(5):303-9.

Pediatr Surg. 1991 5/1991;26(5):587-90.

Aust N Z J Surg. 1972 8/1972;42(1):62-3.

spective. ProgPediatrSurg. 1977 1977;10:111-20.

4 Murdoch Childrens Research Institute, Melbourne, Australia

The social impacts of chronic gastrointestinal and urinary dysfunction are substantial. Approximately a quarter of the respondents in our study were on some form of financial health care assistance. Some of the comments made by parents would seem to go against the relatively optimistic outcomes on quality of life as determined by the Ditesheim and Templeton scale in our study, which suggested over three-quarters had a "good" quality of life. Quality of life measures can be extremely difficult to establish and there may be a case for looking more carefully at developing more specific and sensitive parameters [10].

An interesting observation was the impact of diet on bowel dysfunction. There is relatively little information on this area in the literature although an earlier study by the same authors in children with Hirschsprung disease identified similar findings in that group [14]. Many of the food groups which were problematic included either fibre-containing products (vegeta‐ bles), lactose (dairy) or grains. There has certainly been a great deal of recent interest in the impact of rapidly fermentable, short-chain carbohydrates on functional bowel disease [30]. These have been given the acronym "FODMAPS", and found to have a substantial impact on functional abdominal pain, bloating and diarrhoea which are symptoms of irritable bowel syndrome [30]. Why this should be a particular problem in children after anorectal surgery such as Hirschsprung or anorectal malformation is not clear but may relate to the loss of a "rectal brake".

The assessment of residual function can be difficult. Several clinical-based protocols have been developed, but are poorly standardized with poor inter-scale correlation [31]. Anorectal manometry can identify a range of dysfunctional parameters which might be thought likely to contribute to incontinence [32]. In our study, a number of indices were markedly abnormal. Sphincter pressures were low. Sensory thresholds to rectal distension were extremely blunted. The rectoanal inhibitory reflex was often impaired, or only elicited with very high rectal distending volumes. Incoordinate defaecation was common, as was inability to evacuate a water filled balloon. What is interesting is that there was very little statistical difference between those who soiled heavily and those who had little soiling in terms of anorectal manometric parameters. It is likely that other pathophysiological "triggers" tip the balance in favour of heavy soiling.

Rapid colonic transit may be one such trigger. Ingestion of rapidly fermentable, short-chain carbohydrates is quite likely to lead to more rapid movement of luminal content with the potential for overcoming rectal control. There are varying abilities to tolerate fermentable loads, and these depend (amongst other factors) on rate of ingestion, concomitant nutrients, small intestinal processing, and colonic flora together with their metabolic activities. There is substantially more diversity in colonic flora than previously thought, with close interactions between environment, host genotype and the metabolic functioning gut microbiome [33]. If gut microbiome does play a role in faecal incontinence in this group, however minor, it represents an opportunity for therapeutic intervention.

#### **Author details**

serious and complex as gastrointestinal anomalies, and contribute substantially to long term

The social impacts of chronic gastrointestinal and urinary dysfunction are substantial. Approximately a quarter of the respondents in our study were on some form of financial health care assistance. Some of the comments made by parents would seem to go against the relatively optimistic outcomes on quality of life as determined by the Ditesheim and Templeton scale in our study, which suggested over three-quarters had a "good" quality of life. Quality of life measures can be extremely difficult to establish and there may be a case for looking more

An interesting observation was the impact of diet on bowel dysfunction. There is relatively little information on this area in the literature although an earlier study by the same authors in children with Hirschsprung disease identified similar findings in that group [14]. Many of the food groups which were problematic included either fibre-containing products (vegeta‐ bles), lactose (dairy) or grains. There has certainly been a great deal of recent interest in the impact of rapidly fermentable, short-chain carbohydrates on functional bowel disease [30]. These have been given the acronym "FODMAPS", and found to have a substantial impact on functional abdominal pain, bloating and diarrhoea which are symptoms of irritable bowel syndrome [30]. Why this should be a particular problem in children after anorectal surgery such as Hirschsprung or anorectal malformation is not clear but may relate to the loss of a

The assessment of residual function can be difficult. Several clinical-based protocols have been developed, but are poorly standardized with poor inter-scale correlation [31]. Anorectal manometry can identify a range of dysfunctional parameters which might be thought likely to contribute to incontinence [32]. In our study, a number of indices were markedly abnormal. Sphincter pressures were low. Sensory thresholds to rectal distension were extremely blunted. The rectoanal inhibitory reflex was often impaired, or only elicited with very high rectal distending volumes. Incoordinate defaecation was common, as was inability to evacuate a water filled balloon. What is interesting is that there was very little statistical difference between those who soiled heavily and those who had little soiling in terms of anorectal manometric parameters. It is likely that other pathophysiological "triggers" tip the balance in

Rapid colonic transit may be one such trigger. Ingestion of rapidly fermentable, short-chain carbohydrates is quite likely to lead to more rapid movement of luminal content with the potential for overcoming rectal control. There are varying abilities to tolerate fermentable loads, and these depend (amongst other factors) on rate of ingestion, concomitant nutrients, small intestinal processing, and colonic flora together with their metabolic activities. There is substantially more diversity in colonic flora than previously thought, with close interactions between environment, host genotype and the metabolic functioning gut microbiome [33]. If gut microbiome does play a role in faecal incontinence in this group, however minor, it

carefully at developing more specific and sensitive parameters [10].

morbidity [17].

174 Fecal Incontinence - Causes, Management and Outcome

"rectal brake".

favour of heavy soiling.

represents an opportunity for therapeutic intervention.

Anthony G. Catto-Smith1,3\*, Misel Trajanovska1,4 and Russell Taylor2


#### **References**


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## *Edited by Anthony G. Catto-Smith*

Fecal incontinence is a common and disabling condition that unfortunately remains an "orphan" in terms of medical research and effective therapies. This book provides a brief review of the pathophysiology of fecal incontinence with specific focus on women and children. Authorship is drawn internationally, with a strong surgical input. Contributions from the authors provide critical reviews of the evaluation of function, with illustrations of a range of surgical interventions which might be applied should medical therapies fail.

Fecal Incontinence - Causes, Management and Outcome

Fecal Incontinence

Causes, Management and Outcome

*Edited by Anthony G. Catto-Smith*

Photo by Vrabelpeter1 / iStock