Seminars in Pediatric Surgery (2008) 17, 79-89

Imperforate anus: long- and short-term outcome

Risto J. Rintala, MD, PhD, Mikko P. Pakarinen, MD, PhD

From the Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland.

KEYWORDS Anorectal malformations are common anomalies observed in neonates. Survival of these babies is
Anorectal currently achieved in most cases and improvements in operative technique, patient care, and better
malformations; follow-up have led to improved functional results. A new, simplified classification system (Krickenbeck
Functional results; classification) and method of functional assessment has led to an improved understanding of these
Imperforate anus; anomalies and has allowed for a better comparison of outcomes. Following successful anatomical repair
Short-term outcomes; and appropriate programs of bowel care, socially acceptable continence can be achieved in a majority
Long-term outcomes of patients, especially those with an intact sacrum.
© 2008 Elsevier Inc. All rights reserved.

The outcomes of patients with anorectal malformations
have greatly improved by modern surgical techniques and
neonatal care facilities during the last decades. Early sur-
vival is currently the rule, except in some rare cases with
associated cardiac and urogenital anomalies or chromo-
somal defects that are not compatible with life. The overall
long-term functional outcome expectancy in terms of fecal
and urinary continence is relatively optimistic today. The
majority of patients reaching adolescence and adulthood are
able to maintain socially acceptable continence.
There is no generally accepted method to classify ano-
rectal malformations. The most commonly used method has
been the Wingspread International Classification for Ano-
rectal Malformations.1 Recently, a new simplified classifi-
cation, the Krickenbeck classification (Table 1), that is
based on consensus recommendations of world authorities
has emerged.2 The classification used in the present com-
munication is the Krickenbeck classfication; patients who
have no perineal fistula are grouped under the title “high
malformations,” and those with a perineal bowel opening
are included under the title “low malformations.”
Address reprint requests and correspondence: Risto J. Rintala, MD,
PhD, Hospital for Children and Adolescents, PO Box 281, FIN-00029
HUS, Finland.
E-mail: risto.rintala@hus.fi.
The present review is based mainly on pertinent litera-
ture. In addition, the author’s personal experience with 270
patients treated with posterior sagittal anorectoplasty
(PSARP) procedures for high malformations and manage-
ment of 140 low anomalies between 1984 and 2006 is used
as a basis to address specific previously unpublished issues
in the management of anorectal malformations.
Short-term outcome
Mortality
Anorectal malformations are very often a part of a malfor-
mation complex. Some associated anomalies, especially
cardiovascular malformations, may be uncorrectable.
Therefore, there is always going to be some mortality
among these patients. The mortality of patients with ano-
rectal malformations during the last few decades has been
between 10% and 20% of all cases.3,4 The mortality of
patients with high anomalies has been about three times
higher than that of patients with low anomalies, which
corresponds to the higher incidence of severe associated
anomalies. Only a minority of deaths are directly related to
the anorectal anomaly and its treatment.3-5 At Children’s
Hospital, University of Helsinki, the mortality of anorectal

1055-8586/$ -see front matter © 2008 Elsevier Inc. All rights reserved.
doi:10.1053/j.sempedsurg.2008.02.003
80
Table 1 International classification of anorectal
malformations (Krickenbeck)2
Major clinical groups
Perineal (cutaneous) fistula
Rectourethral fistula
Bulbar
Prostatic
Rectovesical fistula
Vestibular fistula
Cloaca
No fistula
Anal stenosis
Rare/regional variants
Pouch colon
Rectal atresia/stenosis
Rectovaginal fistula
H-type fistula
Others
malformations has decreased from 23% in the late 1940s
and early 1950s to 3% in the 1980s and 1990s (Figure 1).
The decrease is clearly due to improved care of severe
associated anomalies, especially cardiac defects.
Operative complications
A colostomy performed in the neonatal period carries a high
morbidity. The most common complications are colostomy
prolapse and stricture.6,7 Stoma complications may be less
common with a completely divided sigmoid colostomy.8
The reported total incidence of complications of infant co-
lostomies ranges between 17% and 68% of the cases; the
complications also include a few colostomy-related deaths.
Early complications occur following all commonly used
reconstructions for severe anorectal anomalies. The inci-
dence of major complications, such as peritonitis, retraction
or dehiscence of the pull-through segment, and recurrent
fistula, has ranged from 10% to 30% following abdomino-
perineal or sacroabdominoperineal pull-through opera-
tions.3,4,6 Severe complications are less common following
posterior sagittal anorectoplasty. In the large series by Peña
and coworkers, serious complications requiring major reop-
erative surgery occurred in 2% of the cases, mainly follow-
ing repair of a cloaca.9 In the authors’ series of 270 patients
with high anomalies who underwent posterior sagittal ano-
rectoplasty, the incidence of major early complications re-
quiring reoperation has been also 2%.
Postoperative anal complications have been common fol-
lowing traditional pull-through operations. Anal stenosis
and mucosal prolapse have been found in up to 15% to 78%
of reported patients.3,4,6,10,11 Stenosis has usually been at-
tributed to inadequate anal dilatations during the follow-up
period. On the other hand, local anal problems have been
rare following posterior sagittal anorectoplasty. Peña re-
ported very few local complications in his series of 792
patients.9 In the author’s series of 279 posterior sagittal
Seminars in Pediatric Surgery, Vol 17, No 2, May 2008
anorectoplasties, anal stenosis requiring surgery occurred in
5 patients. In addition, 3 patients, early in the series, re-
quired local operation for a minor mucosal ectopy.
Early complications including wound problems after
neonatal treatment of low anomalies are very uncommon.
Urological complications following surgery for low anorec-
tal malformations are unacceptable, and we have been for-
tunate enough to avoid them. However, local complications
may occur later and are usually caused by insufficient long-
term follow-up and care. Postoperative anal stenosis can be
prevented by appropriate dilatations and careful follow-up.
Untreated anal stenosis may cause secondary megacolon,
which may even require operative treatment.3,12 Especially
in cases with delayed diagnosis, the blind rectal pouch may
be primarily ectatic enough to cause constipation without
any associated anal stricture or stenosis.12
Before the definitive repair, it is essential to minimize the
risk of urinary tract infections and permanent damage to
kidneys caused by the rectourogenital connection if such is
present and/or urinary tract anomalies that are common in
patients with anorectal malformations.13,14 This is best ac-
complished by establishing a completely diverting colos-
tomy and by careful washout of the rectal pouch.9
Outcome during early childhood
Many patients with repaired high malformations have ab-
normal bowel function following closure of the protecting
colostomy. The most common problem is frequent bowel
movements causing perineal skin problems. In patients with
traditional pull-through operations, this stage of bowel func-
tion, which is best characterized as uncontrollable soiling,
continued for a long period of time, often for years. This is
particularly the case with operations where the terminal
rectal reservoir is resected, such as sacroabdominoperineal
and endorectal abdominoperineal pull-through.3,15
Constipation is a major problem with patients who have
had posterior sagittal anorectoplasty. Females with recto-
vestibular fistula are especially affected.3,9,12,16 Constipa-
tion may develop as a result of untreated anal stenosis but
more commonly is a consequence of disordered colonic
25
20
15
Percent of Total
10
5
0
46-53 54-63 64-73 74-83 84-98
Figure 1 Mortality rate in patients with anorectal malformations
in Helsinki (1946 –1998). (Color version of figure is available
online.)
Rintala and Pakarinen Imperforate Anus
motility.9,17 Constipation may begin early after the opera-
tion, and its severity is related to the degree of the initial
dilation of the rectal blind pouch.9,16
Constipation is the most common early functional prob-
lem in patients with low anomalies, occurring in about 40%
of the children.18-20 Constipation responds to regular laxa-
tives most of the time. Enemas are seldom needed but are
promptly prescribed if fecal impaction occurs. Severe soil-
ing, not associated with constipation, is extremely rare and
may be caused by operative sphincter damage or severe
sacral defects. The mean age at toilet training for feces and
urine as well as frequency of day- and night-time wetting is
similar when compared with age-matched controls.18-20
Long-term outcome
Evaluation of long-term functional outcome
Clinical evaluation
There is a great variation in the literature regarding func-
tional results after repair of anorectal malformations. This is
due to the fact that there is no generally agreed method to
assess the bowel function of patients with anorectal malfor-
mations. The main problem in comparing different series is
the highly variable criteria used in the evaluation of fecal
continence. Evaluation of bowel function during childhood
may be biased because the information concerning the func-
tional outcome is mainly derived from the parents. They
may not want to report unfavorable results to a surgeon who
has been responsible for the treatment of their child. The
parents may also ignore minor and moderate defects in
continence for a child whose bowel function has been de-
ficient from birth or, in the case of smaller children, may
consider the situation to be part of normal maturation of
defecation. The final outcome may not be fully assessed
until the patients have reached adulthood when as indepen-
dent individuals, patients can evaluate the social conse-
quences of possibly defective bowel control.
Scoring methods based on subjective parameters with or
without clinical examination have been designed to get
quantitative information about the bowel function. The most
commonly used classifications are the Kelly-score,6 the
Templeton score,15 Holschneider score,21,22 and Rintala
score.23 All these classifications ultimately categorize the
outcomes as good, fair, or poor. Descriptive, nonscoring
methods to assess and describe functional outcome were
advocated by the Wingspread group of pediatric surgeons.1
This method and the descriptive, nonscoring outcome clas-
sification of Peña, based on his extensive series,9 are the
basis of the new Krickenbeck outcome classification
method (Table 2).
Objective methods
Manometric assessment has been the principal method to
obtain objective data of postoperative sphincter function.
81
Table 2 Assessment of outcome (Krickenbeck)2
1. Voluntary bowel movements yes/no
Feeling of urge
Capacity to verbalize
Hold the bowel movement
2. Soiling yes/no
Grade 1: occasionally (1 to 2/week)
Grade 2: every day
Grade 3: constant, social problem
3. Constipation yes/no
Grade 1: manageable with diet
Grade 2: requires laxatives
Grade 3: resistant to diet and laxatives
However, there is no standard for manometric evaluation.
Single fluid-filled balloons in the anal canal have been used
by several authors.24,25 Balloon devices allow only static
measurements, therefore the open tip perfused catheter
method is favored by others.26,27 The quantitative pressure
recordings in static and dynamic studies are not directly
comparable; the values of manometric tracings tend to be
higher with the balloon method.
Manometric and clinical results have often been found
contradictory. Some investigators have found positive cor-
relation between clinical continence and the anal resting
pressure profile,23,25,28 whereas others have found no such
correlation but a relationship between continence and vol-
untary squeeze force,29 and still others suggest no correla-
tion at all between clinical continence and pressure profile
or squeeze force.26,30 However, a clear correlation between
the presence of inhibitory rectoanal reflex and clinical con-
tinence has been reported by several authors.23,25,26,28 De-
creased rectal sensitivity with rectal distension has been
reported to correlate with poor functional outcome.25,26,29
Of the available and relevant imaging modalities, MRI is
superior because of excellent soft tissue characterization,
multiplanar imaging, and lack of exposure to ionizing radi-
ation. Hypoplastic sphincter complex, the misplacement of
bowel in relation to sphincters, and obtuse anorectal angle
have been factors related to poor outcome.31 However,
correlation between clinical results and the findings with
MRI have not been convincing. The primary value of MRI
in patients with anorectal malformations is not in detecting
abnormalities in the sphincter complex, but in revealing
associated spinal dysraphism, which is common in these
patients.32
Long-term bowel function
A major difficulty lies in comparing functional results be-
tween reported follow-up series. The criteria used to eval-
uate long-term outcome are quite variable and mostly de-
signed for high anomalies.1,6,15,21,33 In patients with high
anomalies, a good result usually means socially acceptable
continence which is not equivalent to normal anal function.
A patient with a high anomaly and a good functional result
rarely has normal bowel function, and although socially
82
continent, they often have a minor degree of smearing or
soiling associated with physical straining or loose stools.
Although many patients with low malformations have nor-
mal bowel function at long-term evaluation, a method de-
signed to assess long-term outcome in high anomalies may
underestimate minor defects in bowel function, and these
may become significant when the patient leads a life of an
independent adult individual.
Results in high anomalies
Prognostic factors
The level of the anomaly is an important prognostic factor
in terms of bowel function. Males with a bladder neck
fistula and females with a high confluence cloaca9 have
significantly poorer prognosis than patients with a lower
urogenital connection.23,28 The obvious cause of poorer
prognosis in very high anomalies is the more marked hyp-
oplasia of the voluntary sphincter muscles, especially the
infralevator component of the muscles.9
The presence of severe sacral abnormalities is associated
with hypoplastic sphincters. If more than two sacral verte-
brae are missing or if the patient has other major sacral
deformities, such as hemivertebrae and vertebral fusions,
the functional outcome is worse than in patients with normal
sacrum or lesser degree of sacral maldevelopment.9,23
The role of the internal sphincter in anorectal malforma-
tions is a topic which has been debated for decades. Re-
cently, embryological, animal, and clinical studies have
documented the presence of the internal sphincter in the
region of the fistulous bowel termination.34-36 The function-
ing internal sphincter can be demonstrated by the presence
of rectoanal relaxation reflex in anorectal manometry. Most
patients with a low anomaly have positive rectoanal re-
flex.3,28,37 In patients with high malformations, rectoanal
relaxation reflex has traditionally been present in only a
minority of patients.28,30,37 However, when the rectouro-
genital fistulous connection has been preserved at the time
of anorectal reconstruction, the percentage of patients with
preserved functional internal sphincter has been between
40% and 80%.23,38,39 The presence of internal sphincter has
been clearly shown to correlate with favorable functional
outcome.23,25,28,38
Colonic motility disorders usually presenting as consti-
pation have been earlier reported to be a problem in patients
with low anorectal malformations and in females with a
vestibular fistula.3,9 Chronic constipation is also the main
functional complication following repair of high anomalies
by posterior sagittal anorectoplasty.9,16,40 The incidence of
constipation following PSARP procedure has varied be-
tween less than 10%41 and 73%.23 Constipation seems to be
more common when internal sphincter-preserving tech-
niques have been used.23,38 The cause of constipation is
unclear; the extensive mobilization of the anorectum may
cause partial sensory denervation of the rectum and impair
the awareness of rectal fullness. Also, rectosigmoid hypo-
Seminars in Pediatric Surgery, Vol 17, No 2, May 2008
motility and generalized colonic motility disturbance have
been suggested.17
It is likely that the surgical method of anorectal recon-
struction in high malformations is a significant prognostic
factor. However, this is very difficult to prove since ran-
domized controlled studies are unavailable. Holschneider40
reported significantly better continence outcome in 21 pa-
tients who had posterior sagittal anorectoplasty compared
with 16 patients having abdominoperineal pull-through with
or without submucosal rectal resection advocated by Reh-
bein. Mulder and coworkers42 found no difference among
patients undergoing sacroabdominoperineal operation and
those who had posterior sagittal anorectoplasty. deVries in
a literature review43 could not find conclusive evidence to
support superiority for any procedure used for anorectal
reconstruction in instances of high anomalies.
Long-term bowel function during childhood
Reports concerning long-term results for high anomalies
are highly variable. Most series grade the results as good,
fair, or poor. It must be remembered that a good outcome
does not mean that the patient has normal bowel function.
The patients with a good result have usually been consid-
ered socially continent, which implies that the defects in
bowel function do not cause significant social disability. In
the era before the posterior sagittal anorectoplasty, the re-
ported percentages of patients, evaluated by clinical criteria,
with a “good” result varied between 6% and 56%. The
percentage of poor results (which has meant more or less
total incontinence) varied between 10% and 70% of affected
patients (Table 3). It is unlikely that such a wide variation
would reflect true differences in long-term results. The op-
erative methods used in all these series were routine proce-
dures for anorectal reconstruction, and the number of pa-
tients in each series were relatively large which implies that
the reporting authors/centers had experience in the repair of
anorectal malformations. The plausible explanation for the
variation is differences in the strictness of assessment cri-
teria. The two relatively recent large series reported by
Templeton15 and Rintala3 both used a quantitative multifac-
torial evaluation for continence. Both these series identified
a lower percentage of poor results than the other series using
mainly qualitative criteria. These multifactorial quantitative
assessments seemed to grade continence higher than a qual-
Table 3 Functional outcome during childhood: high
malformations
Before the era of posterior
sagittal anorectoplasty
No. Good Fair Poor
50
Partridge, et al. 63 33% 43% 24%
Stephens and Smith6 25 56% 32% 12%
Taylor, et al.30 45 24% 20% 56%
Rintala and Pakarinen Imperforate Anus
Table 4 Functional outcome during childhood: high
malformations
Posterior sagittal
anorectoplasty
Total Significant
continence soiling Constipation
Peña9 36% 41% 43%
Rintala, et al.23 35% 30% 60%
Langemeijer, et al.41 7% 56% 5%
itative clinical assessment, probably because they are more
sensitive to the patient’s social adaptation to abnormal ano-
rectal function.22 Only Taylor and coworkers30 and Rintala3
have reported the incidence of patients with a completely
normal bowel function without soiling or staining in any
circumstances. In both series, only 7.5% of the patients had
full bowel control comparable to that of healthy children. It
is probable that this figure reflects the true incidence of
unequivocally good long-term outcome in the patients
treated with abdominoperineal and sacral approaches.
There are still only a few reports concerning long-term
functional outcome following posterior sagittal anorecto-
plasty, and the results have been contradictory (Table 4).
Some surgeons report a dismal outcome with most patients
requiring adjunctive measures to maintain social conti-
nence.41 On the other hand, Peña9 reports that approxi-
mately one-third of the patients with high or intermediate
anomalies in his personal series could be considered as
totally continent. In the series reported by Rintala and co-
workers,23 in which the bowel function of the patients were
compared with that of healthy children with similar age and
sex distribution, 35% of the patients had an age-appropriate
normal bowel function. A fair outcome with intermittent
soiling requiring frequent change of underwear or protective
aids, or poor outcome with intractable constipation or total
incontinence was found in 30% of the patients.
Many authors report an improvement in fecal continence
with increasing age in patients operated by abdominoperi-
neal procedure with or without a sacral approach.3,15,33 In
Rintala’s series, the incidence of good outcome increased
from 35% in the age group between 5 and 10 years to 58%
in patients between 11 and 15 years. The improvement in
fecal continence is more clearly shown in the series includ-
ing adolescent or adult patients.33,44 It is not clear whether
this improvement is true improvement of sphincter function
or simply adaptation to the residual dysfunction. On the
other hand, recent reports by Peña and Rintala9,23 on pa-
tients operated on by posterior sagittal anorectoplasty have
shown that patients with favorable anatomy gain normal or
near normal bowel function as early as age 3 years, provided
that the inherent functional complications related to the
procedure, especially constipation, are treated early and
vigorously. In many cases, soiling during the early years
after posterior sagittal anorectoplasty has been a conse-
83
quence of severe constipation with overflow incontinence,
rather than sphincter insufficiency. The treatment of soiling
associated with constipation is much more rewarding than
treatment of soiling related to sphincter insufficiency.
Long-term bowel function at adult age
The pediatric surgical literature has only a few reports
concerning functional outcome for high anorectal anomalies
assessed at adult age (Table 5). The functional results in
adults, however, illustrate the endpoint in the development
of bowel function.
Nixon and coworkers44 found normal bowel control in 7
(15%) of 47 adolescent and young adult patients. Twenty-
nine patients (62%) had occasional soiling and 11 (23%)
frequent soiling or a colostomy. More recently, Rintala and
coworkers45 studied 33 adult patients with a mean age of 35
years using a questionnaire-based scoring system. Healthy
adults with an age and sex distribution similar to the patients
were used as controls. None of the patients reached a score
indicating normal bowel function, and only 6 (18%) had a
good continence score. All controls had good scores and
80% a score indicating normal bowel function. Thirty-one
(94%) of the 33 patients reported some degree of fecal
soiling. Hassink and coworkers46 evaluated 58 patients with
a median age of 26 years by using similar scoring methods
as Rintala and coworkers. Also in this study none of their
patients met the criteria for normal bowel habits. About
80% of the patients had soiling. In these series, most pa-
tients had an abdominoperineal repair as a primary recon-
struction. In Nixon’s44 series, 68% of the patients had major
secondary surgery in an attempt to improve continence. In
Rintala’s45 series 30% and in Hassink’s46 series half of the
patients had secondary sphincter repairs.
According to above data, it is obvious that almost all
adult patients who have undergone repair of a high anorectal
malformation with traditional methods, such as direct peri-
neal, abdominoperineal, or sacro-abdominoperineal opera-
tions, have some form of fecal incontinence despite many
secondary sphincter reconstructions. Although many pa-
tients report being satisfied with their current level of fecal
continence,46 objective evaluation of the data concerning
bowel function provides a different picture. It is likely that
the adult patients have accepted their handicap. The patients
have developed measures to cope with unsatisfactory bowel
control, such as staying near toilets, wearing liners or dia-
Table 5 Functional outcome at adult age: high
malformations
No. Normal Good Fair Poor
46
Hassink, et al. 58 0 36% 43% 21%
Rintala, et al.45 33 0 18% 54% 27%
Nixon, et al.44 47 15 62% 23%
(good/fair)
Rintala 2007* 69 43% 36% 9% 12% (ACE)
*Unpublished data.
84
pers, having regular enemas, or having dietary restric-
tions.45-47
At adult age, defective fecal continence has significant
social consequences. The main problem is fecal soiling
which restricts social activities. In Rintala’s series,45 85% of
the adult patients reported social disability related to soiling.
Other problems especially disturbing to occupational life
were inability to hold back flatus and fecal urgency. Hassink
and coworkers47 reported that adult patients had signifi-
cantly lower educational level than expected.
There are no reports concerning the functional outcome
in adult patients who have undergone repair of their severe
anorectal malformation by posterior sagittal anorectoplasty.
In our institution, we reviewed the functional outcome in 69
patients that were older than 15 years at the time of assess-
ment (Table 5). Normal continence without any fecal soil-
ing or constipation requiring medication was found in 30
patients (43%). Twenty-five (36%) had minor problems,
such as constipation requiring medication or occasional
staining; these functional aberrations did not have any social
impact, and none of these 25 used any protective aids. Six
patients (9%) had significant continence problems causing
frequent soiling and need to use protective pads or change
of underwear. Five of these were mentally retarded. In 8
patients (12%), fecal continence was so poor that they
required a permanent appendicostomy for bowel manage-
ment with regular washouts. These preliminary data from 1
institute largely support the former reports of functional
outcome during childhood,9,44 suggesting that PSARP is
superior to the prior traditional methods of repair. In our
institution, nearly half of patients who have undergone
PSARP-procedure have normal fecal continence beyond
childhood.
Results in low anomalies
Traditionally, the long-term results in low malformations
are considered to be good in the great majority of pa-
tients.33,48-50 Poor outcomes have been related to neurolog-
ical damage and mental retardation37 or insufficient long-
term follow-up and care of the patients.4,44
More critical reviews on the long-term outcome have
clearly demonstrated a significant number of children with
functional defects, the most common of which is chronic
constipation followed by soiling. In Yeung’s series,51 15 of
the 32 children with a follow-up between 1 and 7 years had
normal bowel function. Of the remaining 17 patients, all had
constipation and 9 occasional or frequent soiling requiring
treatment. In the series of Ong and coworkers52 concerning
70 patients with low anorectal anomalies, there were 35
children under the age of 15 years, 9 of whom were clean,
14 had occasional smearing, and 12 had soiling.
Rintala and coworkers48 compared the bowel function of
40 children with low anomalies, including patients with
perineal fistula, anal stenosis, and operatively treated ste-
notic anterior perineal anus, to that of healthy children using
a scoring system. Only 52% of the reconstructed patients
Seminars in Pediatric Surgery, Vol 17, No 2, May 2008
had scores within the 90th percentile of the controls of
healthy children; constipation was found in 42% and soiling
in 10% of the patients. Four (10%) patients reported re-
stricted social life due to fecal soiling. The issue was as-
sessed further by our institution in a recent prospective
follow-up study including only boys (median age, 8.5 years;
range 4.3-13.5 years) with perineal (anocutaneous) fistula
who were compared with age- and sex-matched healthy
controls.19 The children and their caregivers were inter-
viewed by an independent third party. A total of 68% of the
patients had bowel function scores within the 90th percen-
tile of the controls; constipation occurred in 41% and soiling
in 55% of the patients. The figures for constipation and
soiling among controls were statistically and clinically sig-
nificantly lower: 8% and 24%, respectively. Soiling oc-
curred occasionally in every child with a reconstructed low
malformation, but bowel dysfunction restricted social life in
only 1 of the 22 patients.
In perineal fistula, the anus is anteriorly displaced but is
surrounded by the sphincter muscles. One may accept the
anterior displacement of the anus and perform a simple
anoplasty or perform more complex PSARP. It may be
argued that the latter surgical approach is associated with
better functional outcome. To address this issue, we per-
formed a prospective multicenter comparison of functional
outcome between these 2 surgical techniques.18 There were
24 boys treated with anoplasty and 17 boys treated with
PSARP. The groups were comparable regarding age, asso-
ciated malformations, and sacral dysplasia. The results sug-
gest that functional outcome is very similar following both
procedures without any differences in the overall bowel
function score or in the rate of constipation and soiling.18
However, the need for redo surgery was significantly in-
creased in the PSARP group.
Bowel function at adult age
There are only a few functional outcome studies of low
anorectal malformations with a follow-up extending to
adulthood. Karkowski53 reported good continence in 12
(80%) of his 15 patients with low malformations. Nixon and
coworkers4 found entirely normal bowel control in 23
(74%) of his 31 adult or adolescent patients. The remaining
patients had occasional or frequent soiling.
More recently, the large series of Ong and coworkers52
and Rintala and coworkers22 have demonstrated that a sig-
nificant percentage of these patients have abnormal anorec-
tal function at the adult age. Ong and coworkers reported 35
patients with a follow-up of more than 15 years. Although
the majority were considered to have good continence ac-
cording to commonly used clinical scoring methods, only
13 (37%) of the patients were clean at all times. Seventeen
patients (49%) had fecal smearing and 5 (14%) intermittent
soiling.52 Rintala and coworkers, using a quantitative scor-
ing method, compared the bowel function of 83 patients to
that of healthy individuals with similar age and sex distri-
bution.22 All controls had good fecal continence, 76% with
completely normal bowel function. In contrast, 60% of the
Rintala and Pakarinen Imperforate Anus 85

patients with a low anorectal anomaly had good continence, Table 6 Urinary incontinence
but completely normal bowel function was observed only in
15%. It should be noted that these findings are not fully No. Incontinence (%)
comparable with our more recent studies because the pa- Low anomalies
tients included 38 women with anovestibular fistula. Nev- Peña9 14 0
ertheless, social problems related to deficient fecal control Rintala, et al.48 40 0
were reported by 39% of the patients. In addition, 13% of Trusler, et al.49 20 10
Rintala, et al.22 83 11
the patients had difficulties in sexual functions. Other health High anomalies (before posterior
problems were reported by 52% of the patients, but only by sagittal anorectoplasty)
6% of the controls. Trusler, et al.49 15 33
Based on our experience, overall long-term bowel func- Wiener, et al.57 90 31
tion is impaired at least in one-third of children with a low Smith, et al. 18 28
anorectal malformation. The main reasons for impaired ano- Rintala, et al.45 33 33
Hassink, et al.46 58 22
rectal function are constipation and occasional soiling af- High anomalies (posterior
fecting up to half of the patients. In most patients, the nature sagittal anorectoplasty)
of constipation and soiling is modest enough not to produce Peña9 233 10
social problems or restrict social activities. Patients with Rintala, et al.58 65 8
operated low anorectal malformations require continuing
follow-up and care beyond childhood.

rior sagittal anorectoplasty requires often extensive dissec-

Long-term problems related to associated
malformations
Urinary tract problems
Urinary tract anomalies occur in more than 40% of all
patients with anorectal malformations; however, the actual
incidence of long-term urological morbidity is difficult to
assess because only a few long-term follow-up reports have
specifically addressed urological problems.
A high incidence of neurovesical dysfunction in patients
with anorectal malformations has been reported by several
authors.13,54-56 Neurovesical dysfunction is usually congen-
ital and often associated with lumbosacral or intraspinal
abnormalities.56,57 Vesicoureteral reflux in patients with
anorectal malformations is commonly associated with neu-
rovesical dysfunction, and therefore, carries a high risk of
recurrent urinary tract infection and subsequent renal dam-
age. Urinary incontinence is related to dysplastic sacrum,
urethral and bladder anomalies, and neurovesical dysfunc-
tion. Operative damage to bladder neck or urethra seem to
account for a minority of causes of urinary inconti-
nence.56,57
Urinary incontinence is uncommon in patients with low
anomalies, which probably reflects the lower incidence of
spinal anomalies or neurogenic bladder in these patients.
Rintala and coworkers44 and Peña9 reported no such cases
with urinary incontinence in their recent series. Recent re-
ports have shown that the age of toilet training for urine is
similar in patients with low anal anomalies and healthy
children.18,19,48
Urinary incontinence was common in patients with high
anomalies operated on by traditional methods in the past
(Table 6). It appears, however, that patients who have had
posterior sagittal anorectoplasty have a lower incidence of
neurogenic bladder and urinary incontinence than patients
operated on by earlier traditional methods, although poste-
tion behind the urethra and bladder neck (Table 6). Peña9
described a 10% incidence of urinary incontinence in his
233 children who had posterior sagittal anorectoplasty
for high or intermediate anomalies, including rectoves-
tibular fistulas. In Rintala’s series,58 of 65 patients with
high or intermediate anorectal malformations repaired by
internal sphincter preserving posterior sagittal anorecto-
plasty, 8% had long-term postoperative urinary inconti-
nence. Also in this series, urinary incontinence was cor-
related with severe sacral anomalies and high cloacal
deformities. A recent institutional analysis of 69 patients
older than 15 years of age showed that some kind of
urinary incontinence was present in 11% of patients with
high anomalies. In a majority of these patients, the symp-
toms were mild and caused no social disability. Two of
these 69 patients required clean intermittent catheteriza-
tion to remain dry.
Genital anomalies, fertility, and sexual problems
Females with anorectal malformations have a high inci-
dence of genital anomalies; the most common are vaginal
and uterine septation anomalies and vaginal agenesis.59
Genital tract and sexual function has been reported to be
impaired in almost half of these patients because of vaginal
scarring.59,60 Vaginal scarring may cause dyspareunia and
may interfere with child birth.60 Late gynecological prob-
lems are especially common in patients with cloaca. Ob-
struction of Mullerian structures with subsequent cystic
menstrual blood collections have been reported to develop
in a high percentage of postpubertal girls with cloaca.62
There is little information about fertility in patients with
anorectal malformations. In Rintala’s series of 83 adult
patients21 with low malformations, 47 (57%) had offspring
of their own. In the same study, 54% of the healthy controls
with similar age and sex distribution had children of their
own. On the other hand, in another study from the same
86
institution,45 concerning high malformations, only 39% of
the patients had children, which was significantly less than
healthy controls, 60% of whom had offspring. Obviously,
the low frequency of offspring in patients with high anom-
alies reflects true infertility in a significant percentage of
patients. Ejaculatory duct obstruction has been reported in
males,61 some have erectile dysfunction, weak or missing
erections, or retrograde ejaculations,45 and some females
have Mullerian structure agenesis.59 On the other hand,
some patients may avoid sexual contacts because of defec-
tive fecal continence. Rintala21,45 reported that 20% of the
patients with high anomalies and 13% of the patients with
low anomalies avoided sexual intercourse because of poor
bowel control.
Vertebral anomalies and myelodysplasias
In the literature, there are essentially no reports concerning
late problems related to vertebral anomalies in patients with
anorectal malformations. In the senior author’s consecutive
series of 270 high anorectal anomalies operated on between
the years 1984 and 2006, 5 patients have required operative
spinal stabilization because of progressive scoliosis. A re-
port from the same institution noted that 16% of adults
with anorectal malformations had spine-related symptoms,
mainly chronic back pain.21,45
Recently, much attention has been placed on the occur-
rence of myelodysplasias in patients with anorectal anom-
alies.54,63,64 The effect of spinal abnormalities, especially
tethered cord on long-term functional outcome in terms of
bladder and bowel function or neurological symptoms in the
lower extremities, is unclear, although some recent reports
suggest that worsening of neurologic function due to spinal
anomalies is possible.63 There appears to be no evidence to
support prophylactic detethering of patients who do not
have specific symptoms related to tethering.64,65 There is
also no evidence to support the concept that tethered cord
affects functional outcome in terms of fecal or urinary
continence in patients with anorectal malformations.64,66
Screening for spinal abnormalities is clearly indicated in
all patients with anorectal malformations, including patients
with low anomalies.32,63 Normal vertebral anatomy on plain
spinal radiographs does not preclude the presence of spinal
cord abnormalities.63 Screening can be performed by ultra-
sound during early infancy or by MRI at any age.
Methods to improve defective fecal continence
Secondary reconstructions
Secondary reconstructions to improve fecal continence have
been used extensively in patients with anorectal malforma-
tions. In most long-term follow-up series extending to adult-
hood, a significant proportion of patients have undergone
various types of redo-surgery.44-46 In most reports, the long-
term functional outcome is not better in patients who had
secondary surgery45 and may be worse than in those with
only primary repair.46 It is, however, possible that the pa-
Seminars in Pediatric Surgery, Vol 17, No 2, May 2008
tients with redo-operations had initially worse continence
than those with only one operation.
Gracilisplasty has been a common method for secondary
sphincter reconstruction. Several reports have shown a clear
improvement in fecal continence in the short term.67,68 The
improvement in continence is caused by somewhat increased
resting pressure67 and significantly increased squeeze pressure.
In adults who have had gracilisplasty during childhood, the
functional results are not encouraging.44-46 The fecal con-
tinence is no better and may be worse than in patients with
only primary reconstruction. Recently, continuous electrical
stimulation of the gracilis muscle has been shown to induce
a transition in muscle composition, from fatigable type II
fibers to fatigue-resistant type I fibers.69 In this report,
patients underwent gracilisplasty followed by implantation
of a muscle stimulator. After a training period, the stimu-
lator was used continuously to maintain constant anal tone.
Short-term clinical and manometric results were promis-
ing,69 but longer follow-up has, however, revealed that only
one-third of patients develop satisfactory continence.70
Levatorplasty, originally described by Kottmeier and co-
workers,71 was popularized as a secondary sphincter recon-
struction by Puri and Nixon.72 Encouraging results have
been published by several authors.44,73,74 The functional
improvement following this procedure has been thought to
be related to creation of an acute anorectal angle, because
actual resting or squeeze pressures are not changed at the
level of the anal canal. Long-term outcomes in adults are not
encouraging. There are no significant differences in fecal
continence between those who had secondary levatorplasty
and those with primary repair only.45,46
Rerouting of the pulled-through bowel has been advo-
cated for patients who have a misplaced anal canal follow-
ing primary operation.6,75 The bowel may traverse the le-
vator and not lay anterior to it. Essentially identical
procedures for rerouting and repair of the muscular anal
canal have been suggested by Stephens, Kiesewetter, and
Peña.6,75,76 The repair is performed through a posterior
sagittal sacroperineal incision and includes splitting of the
voluntary sphincter muscles in the midline as in standard
posterior sagittal anorectoplasty.
The reported outcomes in terms of improved fecal con-
tinence have been variable. Following redo posterior sagit-
tal anorectoplasty, Peña found very significant improvement
in 52% of his 62 patients, mild improvement in 18%, and no
improvement in 12%; the length of follow-up is not given.
Mulder and coworkers reported that 25% of their 20 patients
became continent following this procedure; the mean fol-
low-up period was 3.5 years. Brain and coworkers77 had a
success rate of 16% following a relatively short follow-up
period. Rintala and coworkers78 followed-up 16 patients
with redo-posterior sagittal anorectoplasty beyond child-
hood (mean follow-up period 6 years). Although the clinical
continence and manometric findings initially improved in 13 of
the 16 patients, at adult age only 4 (25%) of the patients could
be considered continent. According to the results of these
Rintala and Pakarinen Imperforate Anus
reports, the role of secondary PSARP in the treatment of fecal
incontinence after primary reconstruction of anorectal malfor-
mations remains unestablished.
Other secondary sphincter substituting methods for fecal
incontinence following reconstruction of anorectal malfor-
mations include free transplantation of palmaris longus
muscle, gluteus muscle plasty, free smooth muscle trans-
plantation, and artificial sphincters. None of these methods
has gained widespread popularity.
Sacral nerve stimulation has yielded promising results in
patients with neurogenic bladder and bowel dysfunction.79
It remains to be seen if this modality can be successfully
used in patients with anorectal anomalies. Other modern
modalities to treat fecal incontinence (bulk agents, Secca
procedure, artificial sphincters) have been used in patients
with anorectal anomalies only infrequently, and presently
no follow-up data are available.
In some patients, late fecal soiling is related to intractable
constipation.9,12,23,80 Many of these patients have an ady-
namic megarectum, which cannot be emptied with medical
management or regular enemas. Aganglionosis, although a
rare occurrence in patients with anorectal anomalies, should
be ruled out by rectal biopsies. In recalcitrant cases, resec-
tion of the dilated distal colon has given favorable re-
sults.80,81 The constipation may, however, not be resolved
permanently; recurrent constipation has been reported fol-
lowing rectosigmoid resection of a megarectum.17
The Malone antegrade colonic enema (MACE) has
proven to be a powerful modality to treat fecal incontinence
of various etiologies.82 Two-thirds to 95% of the patients
have gained full continence following this procedure. Most
treated patients have had neurogenic fecal incontinence.
Most series also include patients with anorectal malforma-
tions. In the authors’ institution, 27 patients with anorectal
malformations have had MACE procedure for fecal incoti-
nence, with excellent or good continence outcome in 80% of
the patients.
Biofeedback conditioning has been used to treat fecal
incontinence in patients with anorectal malformations.83,84
Limited results have been encouraging, but in the author’s
experience, most patients with severe incontinence never
gain full bowel control in the long-term. Minor defects in
continence are more likely to respond to biofeedback.
Conclusion
Despite significant developments in the understanding of
the pathological anatomy and physiology of anorectal mal-
formations, the results of surgical therapy remain far from
perfect. Completely normal bowel function, comparable to
that of healthy individuals, is possible, however, in a sig-
nificant proportion of patients. In low malformations, most
patients develop fecal continence by adulthood. In high
anomalies up to half of the patients who have had PSARP
procedure have excellent fecal continence beyond child-
87
hood. Secondary surgery for failed or inadequate primary
reconstruction is unlikely to provide results that are com-
parable to those following a successful primary repair.
Patients with anorectal malformations need careful fol-
low-up throughout their childhood. Functional complica-
tions, especially treatable ones such as constipation, should
be detected and treated early to achieve optimal outcome.
The treatment of defective continence should be started well
before the child reaches school age to overcome the devas-
tating social consequences of fecal soiling and to integrate
the child into the social context of his peers. Because the
management of anorectal malformations requires years of
commitment and special knowledge concerning the anatom-
ical and physiological characteristics of this complex group
of congenital malformations, these children should be
treated by experienced medical personnel in specialized
referral centers.
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