Idiopathic Disorders of Fecal Continence in Children

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Idiopathic

Disorders

of Fecal

Continence

in

Children

Pierre

Arhan,

Ghislain

Devroede,

Bertrand

Jehannin,

Claude

Faverdin,

Yann

R#{233}vilIon,Daniel

Lefevre,

and

Denys

Pellerin

From the Clinique Chirurgicale Infantile, HOpital des Enfants-Malades, Paris; Laboratoire de Physiologie, Facult#{233}Necker-Enfants-Malades, Paris; and Departement de Chirurgie g#{233}neraleet Centre de Recherches en Physiopathologie digestive, Facuft#{233}de Medecine,

UniversIt#{233}de Sherbrooke, Quebec

L

ABSTRACT.

A

group of 176 patients aged 2to 15 years

was investigated for idiopathic disorders of bowel

func-tion other than Hirschsprung’s disease. Anorectal motil-ity, as well as colorectal transit of radiopaque markers,

were

investigated. Before the end of the first month of life, 70 of the patients were constipated. Resting pressure

was more unstable (P < 0.001) and higher than normal

in the rectal ampulla and upper anal canal (P < 0.01).

Retardation of markers occurred in the proximal and/or

distal large bowel of 61% of the patients. The existence

of functional abnormalities was demonstrated in the ma-jority of children with idiopathic disorders of fecal

con-tinence. Pediatrics 1983;71:774-779; constipation, incon-tinence, colonic motility, anorectal pressures.

I1 many children, constipation is associated with encopresis. Aganglionosis of the distal bowel may

be ruled

out by clinical evaluation, radiography, biopsy, and/or anorectal manometry. The under-lying pathophysiology

in

the remaining patients is poorly understood, and often, constipation of

psy-chogenic origin is then postulated.

The aim of this study is to describe the clinical presentation of children with idiopathic disorders of fecal continence and to demonstrate that they have functional abnormalities of large-bowel motil-ity.

MATERIALS

AND

METhODS

In this study, 176 consecutive patients, aged 2 to

Received for publication June 5, 1981; accepted July 23, 1982.

Reprint requests to (P.A.) Facult#{233} Necker-Enfants-Malades,

D#{233}partement de Physiologie, 156, rue de Vaugerard, 75730 Paris,

France.

American Academy of Pediatrics.

774

PEDIATRICS Vol. 71 No. 5 May1983

15 years (64% boys, 36% girls), were admitted

be-tween 1970 and 1978 to the H#{244}pitaldes Enfants-Malades, Paris, with one of the following criteria: (1) a history of less than three spontaneous stools per week; (2) evidence of a fecaloma (stools of

harder consistency than those passed sponta-neously) at rectal examination; (3) presence of fecal material in the entire descending colon or fecaloma

in the rectosigmoid area diagnosed radiologically. A detailed history was taken and a physical exam-ination was performed. A plain film of the abdomen

was taken to ascertain the presence of fecal

mate-rial. Functional studies were performed when the

rectum was free of stool, either spontaneously or as

the result of cleansing enemas.

Anorectal Motility

Studies

Anorectal motility was studied with a probe’

con-nected to pressure transducers (PMG Statham),

amplifiers (1008 Statham or Gould Brusch DC

am-plifiers), and a recorder (SP 2000 Statham and

six-channel Gould recorder). If the child was restless,

mild sedation was given either by mouth (alime-mazine tartrate, 2 mg), or by intramuscular injec-tion (diazepam, 0.25 mg/kg) if the child was less than 3 years old. These drugs never changed base-line pressure levels. In fact, by abolishing the

inter-ference due to restlessness, the drugs permitted us

to record clearly the slow waves that are

character-istic of the upper part of the anal canal and to

confirm the proper location of the recording bal-loons. Anorectal activity was recorded at three 1ev-els: rectal ampulla, upper part of the anal canal, and anal margin. After a 20-minute period of

equi-libration, mean resting (before any stimulation)

pressure was calculated from 40 measurements

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30

20

10’

0 ,/ I I I

lHn

I I

ll

I

I 1 2 3 4 5 6 7 8

H

DAY MONTH

Fig 1. Age distribution at onset of symptoms and at first consultation.

14 15

AGE (YEAR)

ARTICLES 775

taken ever 5 seconds. Standard deviations of these

values were used to express a “coefficient of

activ-ity.” The rectal ampulla was then distended in

random order for periods of 3 seconds with different

volumes (2 to 100 mL). In all cases, this induced a

rectoanal inhibitory reflex (an upper anal canal

temporary relaxation), thereby ruling out

Hirsch-sprung’s disease.28 Residual pressure during

relax-ation was plotted against the logarithm of the

dis-tending volume,9’0 and a regression line was

cal-culated. From the intersection between this line and the calculated resting mean pressure of the upper anal canal, the threshold distending volume

was obtained.1’ It is the lowest level from which a

rectoanal inhibitory reflex can be elicited. This method is more objective and reliable than simply

observing recordings at varying sensitivity settings

of the amplifiers. Data were compared with those

established in 90 normal children.

Colorectal Motility Studies

At 9 AM, 24 hours after beginning a diet contain-ing 0.5 g/kg of crude fibers, each child ingested 30 radiopaque markers. At 9 AM and 9 PM each day,

for five days, plain films of the abdomen were taken.

For the next three days, or until markers were all

passed, a film was taken at 9 AM only. Markers were counted on each film divided in three areas.’#{176}

A vertical midline drawn along the spinal processes

of the vertebrae separated descending from ascend-ing colon. From the lumbosacral end of this vertical

line, two other lines, tangent to the greater sciatic notches, were drawn. They circumscribed the rectal

area. A large caecum sometimes slightly overlapped

the rectal area: markers were considered to be still

in the caecum if a clear bowel outline of the as-cending colon was seen and no markers had yet

gone through the descending colon and rectum. Mean transit time of one radiopaque marker was

calculated with the following formula:

1

T=nzt

where

T

=

mean transit time of one marker; N

=

total number of ingested markers; n

=

number of

markers in a given segment on film taken at time

t; and L\

=

mean transit time between two films,

estimated by the formula (t2 - T,)/2 (t2

=

time of

the next film after time t; t,

=

time of previous

film. Time of ingestion was taken as t,

=

0).

This method of calculating mean transit time in different segments of the large intestine has been fully explained previously.’2 Data obtained in

chil-dren with constipation were compared with those collected in 23 children with no intestinal

abnor-malities who had to undergo a radiography of the abdomen for medical reasons.

The results are reported as means and standard errors when the distribution of data is gaussian.

Ranges are given when the distribution of data is asymmetric (log normal).

RESULTS

Clinical Data

Age at onset was known in 167 children. Of note,

constipation (less than three stools per week)

oc-curred before the end of the first month of life in 70 patients. In contrast, the peak was later in life

(6 years of age) when time of consultation was

considered (Fig 1). A significant negative

correla-lllllIlhllhlllllIlIAGE AT FRS1 SYMPTOMS

I I AGEATFIRST CLINICAL EXAMINATION

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tion was found between age at first symptom of constipation and time elapsed between this time and first clinical examination (r

=

-.49; P < .01): the older the patient at time of onset, the earlier the consultation.

Referral diagnosis was aganglionosis in 34 pa-tients (19%), idiopathic constipation in 115 (65%),

and encopresis in 14 (8%). Thirteen patients (8%) had surgical symptoms: six had intestinal obstruc-tion and seven were thought to have an abdominal tumor.

Eighty nine patients (50%) had less than three stools per week, 54 (30%) had a fecaloma at rectal examination, and 33 (19%) had fecal material in the entire descending colon at roentgenographic examination; 121 children suffered from encopresis (68%). This was in fact the only clue to constipation in 16 patients (9%). Enuresis was also present in 16 children (9%). It was not significantly related to the presence or absence of encopresis

(x2

Yates

=

3.56; P < .5).

Finding from clinical examination of the abdo-men were normal in 35 patients (20%); abdominal distension was present in 71 patients (43%); and a solid doughy abdominal tumor was found in seven

(4%). A clinical pattern of acute intestinal

obstruc-tion was present in six patients (3%). The rectal

ampulla was empty in 12% of patients; it was filled with normal stools in 37%; and a fecaloma was

present in 50%. Stools were more often present in

the rectum of older children (.03 < P < .04). Anal

fissure was present in six patients (3%).

Radiologic

Evaluation

In plain films, the abdomen never appeared

nor-mal. In 139 patients (80%), the colon was full of

stools. Fecaloma was shown in the descending colon in 59 cases (31%). Marked air distension of the bowel was seen in five cases (3%). Air-fluid levels were present in six patients (3%). Spina bifida occulta was found in 56 patients (31%): 17 at L-5, 25 at S-i, seven at L-5 and S-i, and seven at S-i, S-2, and S-3. The incidence of spina bifida occulta was similar in 150 matched nonconstipated

sub-jects, aged 2 to 14 years (32%; P

=

.87).

Anorectal Motility Studies (Table 1)

In the rectal ampulla and upper anal canal, rest-ing pressure was significantly greater in constipated patients than in normal control subjects.

Involun-tary fluctuations in rectal pressure were present in

47% of constipated subjects (Fig 2). At the anal

TABLE 1. Resting P ressures in Anorectum of Children with Idiopathic Constipation

Control Subject? Constipated Subjects* value

Rectum 14.7 (9.8-24.4) 16.6 (5-23) <.01

Upper anal canal 54.0 (31.1-93.9) 60.9 (30.3-122.6) <.01

Anal margin 50.4 (20.9-126.6) 50.1 (20.3-122.6) NS

* Values are mean resting pressures (centimeters of H,O); range is shown in parentheses.

rectal ampulla I

12cmHO[

1os.c

-0

rectal ampulla

l2cmH2O

lOsec

-0

anal canal

l2cmH2OL

lOs.c

anal canal

3OcmH2O lOsec

anal margin

l2cmH2OL..

anal margin

30cmH0

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ARTICLES

777

margin, resting pressures were statistically similar

in both groups. No correlation was found between age and pressure in either group.

Spontaneous variations of resting pressure (slow

waves)’ were observed more often in the upper anal canal of constipated patients (52%) than con-trol subjects (18%)

(x2

=

3.93; P < .05). The fre-quency of these waves was not significantly differ-ent between control subjects (i3 ± i cycles per minute) and constipated children (i2.7 ± 0.8) (P

=

NS) and their amplitude was also similar (normal

subjects 3.5 ± 0.6 cm H2O; constipated subjects 3.9

± 0.8 cm H2O; P

=

NS). The occurrence of ultraslow waves was equal in both groups (16% in normal

children; 13% in constipated children;

x2

=

0.2; P

> .5). Their frequency was the same in both groups (normal, 1.4 ± 0.3; constipated, 1.7 ± 0.2; P > .05). The “activity coefficient” (standard deviation of mean resting value) of constipated subjects was greater than normal in the upper anal canal (15.6

± 2.6 v 9.7 ± 2.2; P < .001). It was not different in

patients with or without spina bifida occulta rec-ognized on plain films of the abdomen (16.2 ± 3.6

V 15.2 ± 2.4). The greater the activity coefficient in the upper anal canal, the higher the variations of

pressure at rest in the rectum (r

=

.19; P < .05).

Indeed, there were spontaneous antagonisms

be-tween peaks of pressure in the rectum and simul-taneous relaxation of the upper anal canal, as if a rectoanal inhibitory reflex was induced by rectal distension (Fig 2).

The rectoanal inhibitory reflex was present in all

patients, and this confirmed the fact that no child with Hirschsprung’s disease (in which this reflex is absent) was included in this study. Mean threshold theoretical distending volume was 0.8 mL in normal

subjects (range 0.05 to 14). In constipated children,

it was higher and very variable (mean 4 mL; range 0.07 to 233; P < .05). The activity coefficient was not related to the threshold distending volume in

normal subjects or in patients with constipation (normal subjects; r

=

.29; P

=

.1; constipated sub-jects: r

=

-.08; P > .1).

Colorectal

Motility

The values for segmental transit times are shown

in Table 2. In the stomach and small intestine,

values were similar in constipated children and normal control subjects. However, in the colon and rectum, transit time was prolonged in constipated patients. Transit time was the same in constipated

children with or without spina bifida occulta (P> .05). Transit time appeared to be normal in all parts of the intestinal tract in 39% patients, although they were hospitalized for constipation. It was de-layed in the ascending colon in only 5.4% of pa-tients, in the descending colon in 7%, and in the rectum in 21.9%. It was abnormal in both the

ascending and descending colon in 2.7%, in the

descending colon and rectum in 13.6%, in the as-cending colon and rectum in 5.4%, and in the entire large bowel in 5.4%. In children who had a normal transit time in all parts of the digestive tract, 50%

had at least one abnormal measurement at

anorec-tal manometry: 18% had an increased anal activity coefficient; 3% had increased rectal pressure; 14.3%

had increased anal pressure; 3% had increased

mar-ginal

pressure; and 18% had increased threshold distending volume. These abnormalities were

iso-lated or associated.

DISCUSSION

This study demonstrates the existence of

func-tional abnormalities in the colon, rectum, and anus of most constipated children not suffering from

Hirschsprung’s disease. These abnormalities are not necessarily related to personality disorders.

Patients in this study were selected on the basis of either stool frequency or of findings at rectal or

TABLE 2. Transit Time of One Radiopaque Marker*

Site Normal Constipated Constipated Constipated

Children Children Children with Children

Spina Bifida without

Spina Bifida

Stomach and small 6h 24mm 4h 50mm 5h 37mm 4h 36mm

intestine ± lb 10mm ± 36mm ± lh 1mm ± 44mm

Ascending colon 7h 10mm

± lh 4mm

13h 24mint

± lh

5mm

12h l5mint ± lh 36mm

14h 6mint ± lh 20mm

Descending colon 7h 37mm

± lh 3mm

13h 49mint

± lh

37mm

14h 30mint ± 3h 36mm

13h 25mint ± lh 41mm

Rectum llh 4mm

± lh 5mm

30h 22mint ± 2h 42mm

25h 8mint

± 3h

34mm

32h 58mint

± 3h

45mm

* Values are mean transit time ± SE.

1P < .05 as compared with normal children. There is no difference in constipated children

related to presence or absence of a spina bifida.

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778

IDIOPATHIC DISORDERS OF FECAL CONTINENCE

-

-,-radiologic examination. An objective evaluation of constipation was made by studying transit time of radiopaque markers through the large bowel. Water-filled balloons rather than perfused cathe-ters were introduced into the anal canal. Despite these differences from a previous study’4 in criteria for inclusion and in methodologies, two similar conclusions emerge: the anus is hypertonic in chronically constipated children, and the threshold for a rectoanal inhibitory reflex is raised. A third abnormality was recognized in this study, namely the instability and antagonism of pressures in the rectum and anal canal. This is often found in chil-then with meningomyelocele’5 and may be related to reflex hyperactivity as it is in patients with a hypertonic bladder. It explains our fruitless search for a difference in motility pattern of constipated children, with or without spina bifida. Finally, a fourth abnormality in children with chronic idi-opathic constipation is a decreased rectal sensitiv-ity, as demonstrated by a raised conscious thresh-old.’4

Idiopathic chronic constipation is not related to excessive mucosal absorption.’6 Delayed transit through the large bowel may be due to colonic inertia (the entire bowel fails to propel contents), hindgut dysfunction (the ascending colon is normal

but the distal large bowel is not), and outlet obstruc-tion (the colon is normal, the rectum fails to

empty).’7 The hypertonicity of the anal canal and of the rectum as demonstrated in this study might conceivably lead to outlet obstruction. However, the rectum may also fail to empty, if its elastic

properties are decreased, leading to a greater

dis-tensibility and higher threshold to trigger a rec-toanal inhibitory reflex.’4 This would be the reverse of the mechanism of Hirschsprung’s disease, in which the rectum resists distension because it is more elastic than normal.’8

What is the relationship between psychological factors and motor disturbances in children with idiopathic disorders of fecal incontinence? The di-agnosis of psychogenic constipation, even if one is careful about the definition of constipation, is an exclusion diagnosis. Nothing is known about its

underlying physiologic mechanism and no

long-term follow-up study after cessation of treatment has been published. In the early part ofthis century,

Georg Groddeck,’9 the founding father of psycho-somatic medicine, speculated that the child may store his feces, either in anticipation of defecation pleasure, or to please or displease the demanding parent. Blunting of rectal sensation through chronic distension has then been proposed, again in a purely speculative manner, as the underlying mechanism of psychogenic constipation. Children

do indeed store stools in the rectum a proportionally

longer time than do adults.’2 Should this lead to blunted sensation, however, one would not expect storage to occur at a higher level in adulthood.’2 The present study is an attempt to obtain some quantitative findings about idiopathic constipation in children. From our findings, some comments can be made about the relationship between personality disorders and bowel habits.

First, irrespective of when the symptoms became marked enough for referral, many children with severe chronic idiopathic constipation had experi-enced a very early onset of their problem. Whatever

the underlying cause, constipation was found to

begin before the training period, in nearly half of the patients. Thus early onset of constipation does

not necessarily imply a diagnosis of Hirschsprung’s

disease, which was excluded for our patients, who

all had a rectoanal inhibitory reflex.

Second, approximately 40% of children had a normal transit time. These children were not con-stipated during hospitalization when their diet was

rich in crude fibers. Their decrease in stool

fre-quency was either related to a low-residue diet or to psychological factors not operant in the hospital environment.

Third, the increase in tonicity of the anal canal

was not due to excessive striated muscle activity,

inasmuch as anal margin pressure, which assesses activity of the external sphincter, was normal.

Fourth, when markers lagged in the ascending and descending colon, blunting of rectal sensation

was not a mechanism by which psychogenic factors could produce constipation. Indeed, colonic muscle

activity at the rectosigmoid junction and above is not under conscious control.

Fifth, when constipation began at a later age,

parents sought medical attention considerably

quicker. It is interesting to speculate that this may reflect the development of personality conflicts

with the parents.

Finally, does impaired defecation alter the

per-sonality or does the personality alter defecation?

This study, retrospective in this regard, does not provide any answer to this crucial question. It dem-onstrates clearly, however, that patients with idi-opathic disorders of fecal continence do have an abnormal colorectal and anal motility, a cause for which must be found. Children with chronic idi-opathic constipation who remain constipated in a

hospital environment while receiving a high-residue

diet would be the best subjects for such a study.

ACKNOWLEDGMENTS

This work was supported in part by the Institut

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ARTICLES 779

tional de la Sante et de la Recherche

M#{233}dicale(IN-SERM), CRL No. 80-700, grant MT-3511 from the

CRM, and by the French Canadian subcommission for

health matters.

We thank Drs Bernard Perey and Victor Marchessault for reviewing this manuscript.

REFERENCES

1. Arhan P, Faverdin C: Une sonde

a

ballonnets pour l’#{233}tude

de Ia m#{233}canique rectale. Pathol Biol 1972;20:191-194 2. Schnaufer L, Talbert JL, Haller JA, et al: Differential

sphincteric studies in the diagnosis of anorectal disorders of childhood. J Pediatr Surg 1967;2:538-543

3. Lawson JON, Nixon HH: Anal canal pressures in the diag-nosis of Hirschsprung’s disease. J Pedicztr Surg 1967;2:544-552

4. Schuster MM: Motor action of rectum and anal sphincters in continence and defecation, in Handbook of Physiology: A Critical, Comprehensive Presentation of Physiological

Knowl-edge and Concepts. Section 6: Alimentary Canal, vol 4,

Motility; Code CF (section ed). Washington, DC, American Physiological Society, 1968, pp 2121-2140

5. Meunier P, Marechal JM, Mollard P: Accuracy of the

man-ometric diagnosis of Hirschsprung’s disease. J Pediatr Surg

1978;13:411-415

6. Meunier P, Marechal JM, Mollard P: Accuracy of the man-ometric diagnosis of Hirschsprung’s disease. J Pediatr Surg

1978;13:41 1-415

7. Faverdin C: Technique d’exploration fonctionnelle de

l’appar-eu rectoanal de l’enfant: application au diagnostic de Ia

ma-ladie de Hirschsprung, thesis. Paris, 1979

8. Faverdin C, Dornic C, Arhan P, et al: Quantitative analysis

of anorectal pressures in Hirschsprung’s disease. Dis Colon

Rectum 1981;24:422-427

9. Arhan P, Faverdin C, Thouvenot J: Anorectal motility in sick children. Scand J Gastroenterol 1972;7:309-314

10. Martelli H, Devroede G, Arhan P, et a!: Some parameters of large bowel motility in normal man. Gastroenterology 1978;75:612-618

11. Arhan P, Devroede G, Persoz B, et al: Response of the anal

canal to repeated distension of the rectum. Clin Invest Med

1979;2:82-88

12. Arhan P, Devroede G, Jehannin B, et al: Segmental colonic transit time. Dis Colon Rectum 1981;24:625-629

13. Kerremans R: Morphological and Physiological Aspects of

Anal Continence and Defecation. Brussels, Arscia, 1969

14. Meunier P, Marechal JM, Jaubert de Beaujeu M: Rectoanal pressures and rectal sensitivity studies in chronic childhood constipation. Gastroenterology 1979;77:330-336

15. Arhan P, Faverdin C, Thouvenot J: Antagonisme in vivo de

l’activit#{233}m#{233}canique spontan#{233}e du rectum et du sphincter lisse de l’anus. C R Soc Biol 1972;166:259-262

16. Devroede G, Soffie M: Colonie absorption in idiopathic constipation. Gastroenterology 1973;64:552-561

17. Martelli H, Devroede G, Arhan P, et al: Mechanisms of

idiopathic constipation: Outlet obstruction. Gastroenterology 1978;75:623-631

18. Arhan P, Devroede G, Danis K, et al: Viscoelastic properties

of the rectal wall in Hirschsprung’s disease. J Clin Invest

1978;62:82-87

19. Groddeck G: La maladie, l’art et le symbole (partly

trans-lated from: Verstopfung als typus des Widerstands. L’Arche

no. 8-9; Aug 2-17, 1926). Paris, Gallimard, 1969

STIGMATIZED

PARENTS

The birth of a handicapped child stigmatises its parents, who were probably quite ‘normal’ previously. An abnormality that may seem trivial to them as adults (looking at someone else’s child) may assume massive proportions for

them as parents; this may be seen as a particular example of “spread.” So

profound may be the effects of their child’s stigma that parents may come to

view themselves in a distorted fashion. If they accept responsibility for their child they have no choice but to be responsible for its stigma also. In all these

ways such parents differ appreciably from those who choose to associate with

the stigmatised, through marriage for example, and thereby adopt what Goffman

calls a “courtesy” stigma. ...

It is no coincidence that the same issues-of what to tell and when, how to

handle the infant, and so on-also concern the professional attendants of the family. They too are vulnerable to the effects of stigma. Their responses should serve as valuable role models for the parents affected, but they often fall far short of this. They may even collude with parents to avoid addressing these

problems altogether.

Submitted by Student

From Sensky T: Family stigma in congenital physical handicap (Br Med J 1982;285:1033).

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1983;71;774

Pediatrics

Daniel Lefevre and Denys Pellerin

Pierre Arhan, Ghislain Devroede, Bertrand Jehannin, Claude Faverdin, Yann Révillon,

Idiopathic Disorders of Fecal Continence in Children

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