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 The present review is based mainly on pertinent litera-
have greatly improved by modern surgical techniques and ture. In addition, the author’s personal experience with 270
neonatal care facilities during the last decades. Early sur- patients treated with posterior sagittal anorectoplasty
vival is currently the rule, except in some rare cases with (PSARP) procedures for high malformations and manage-
associated cardiac and urogenital anomalies or chromo- ment of 140 low anomalies between 1984 and 2006 is used
somal defects that are not compatible with life. The overall as a basis to address specific previously unpublished issues
long-term functional outcome expectancy in terms of fecal in the management of anorectal malformations.
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- Short-term outcome
rectal malformations. The most commonly used method has
been the Wingspread International Classification for Ano- Mortality
rectal Malformations.1 Recently, a new simplified classifi-
cation, the Krickenbeck classification (Table 1), that is Anorectal malformations are very often a part of a malfor-
based on consensus recommendations of world authorities mation complex. Some associated anomalies, especially
has emerged.2 The classification used in the present com- cardiovascular malformations, may be uncorrectable.
munication is the Krickenbeck classfication; patients who Therefore, there is always going to be some mortality
have no perineal fistula are grouped under the title “high among these patients. The mortality of patients with ano-
malformations,” and those with a perineal bowel opening rectal malformations during the last few decades has been
are included under the title “low malformations.” 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
Address reprint requests and correspondence: Risto J. Rintala, MD,
PhD, Hospital for Children and Adolescents, PO Box 281, FIN-00029 anomalies. Only a minority of deaths are directly related to
HUS, Finland. the anorectal anomaly and its treatment.3-5 At Children’s
E-mail: [email protected]. 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 Seminars in Pediatric Surgery, Vol 17, No 2, May 2008

Table 1 International classification of anorectal

anorectoplasties, anal stenosis requiring surgery occurred in
malformations (Krickenbeck)2 5 patients. In addition, 3 patients, early in the series, re-
quired local operation for a minor mucosal ectopy.
Major clinical groups Early complications including wound problems after
Perineal (cutaneous) fistula neonatal treatment of low anomalies are very uncommon.
Rectourethral fistula
Bulbar Urological complications following surgery for low anorec-
Prostatic tal malformations are unacceptable, and we have been for-
Rectovesical fistula tunate enough to avoid them. However, local complications
Vestibular fistula may occur later and are usually caused by insufficient long-
Cloaca term follow-up and care. Postoperative anal stenosis can be
No fistula
Anal stenosis prevented by appropriate dilatations and careful follow-up.
Rare/regional variants Untreated anal stenosis may cause secondary megacolon,
Pouch colon which may even require operative treatment.3,12 Especially
Rectal atresia/stenosis in cases with delayed diagnosis, the blind rectal pouch may
Rectovaginal fistula be primarily ectatic enough to cause constipation without
H-type fistula
Others 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
malformations has decreased from 23% in the late 1940s present and/or urinary tract anomalies that are common in
and early 1950s to 3% in the 1980s and 1990s (Figure 1). patients with anorectal malformations.13,14 This is best ac-
The decrease is clearly due to improved care of severe complished by establishing a completely diverting colos-
associated anomalies, especially cardiac defects. tomy and by careful washout of the rectal pouch.9

Outcome during early childhood

Operative complications
Many patients with repaired high malformations have ab-
A colostomy performed in the neonatal period carries a high normal bowel function following closure of the protecting
morbidity. The most common complications are colostomy colostomy. The most common problem is frequent bowel
prolapse and stricture.6,7 Stoma complications may be less movements causing perineal skin problems. In patients with
common with a completely divided sigmoid colostomy.8 traditional pull-through operations, this stage of bowel func-
The reported total incidence of complications of infant co- tion, which is best characterized as uncontrollable soiling,
lostomies ranges between 17% and 68% of the cases; the continued for a long period of time, often for years. This is
complications also include a few colostomy-related deaths. particularly the case with operations where the terminal
Early complications occur following all commonly used rectal reservoir is resected, such as sacroabdominoperineal
reconstructions for severe anorectal anomalies. The inci- and endorectal abdominoperineal pull-through.3,15
dence of major complications, such as peritonitis, retraction Constipation is a major problem with patients who have
or dehiscence of the pull-through segment, and recurrent had posterior sagittal anorectoplasty. Females with recto-
fistula, has ranged from 10% to 30% following abdomino- vestibular fistula are especially affected.3,9,12,16 Constipa-
perineal or sacroabdominoperineal pull-through opera- tion may develop as a result of untreated anal stenosis but
tions.3,4,6 Severe complications are less common following more commonly is a consequence of disordered colonic
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-
25
ing repair of a cloaca.9 In the authors’ series of 270 patients
with high anomalies who underwent posterior sagittal ano- 20
rectoplasty, the incidence of major early complications re-
quiring reoperation has been also 2%. 15
Percent of Total
Postoperative anal complications have been common fol- 10
lowing traditional pull-through operations. Anal stenosis
and mucosal prolapse have been found in up to 15% to 78% 5
of reported patients.3,4,6,10,11 Stenosis has usually been at-
0
tributed to inadequate anal dilatations during the follow-up 46-53 54-63 64-73 74-83 84-98
period. On the other hand, local anal problems have been
rare following posterior sagittal anorectoplasty. Peña re- Figure 1 Mortality rate in patients with anorectal malformations
ported very few local complications in his series of 792 in Helsinki (1946 –1998). (Color version of figure is available
patients.9 In the author’s series of 279 posterior sagittal online.)
Rintala and Pakarinen Imperforate Anus 81

motility.9,17 Constipation may begin early after the opera- Table 2 Assessment of outcome (Krickenbeck)2
tion, and its severity is related to the degree of the initial
dilation of the rectal blind pouch.9,16 1. Voluntary bowel movements yes/no
Constipation is the most common early functional prob- Feeling of urge
Capacity to verbalize
lem in patients with low anomalies, occurring in about 40% Hold the bowel movement
of the children.18-20 Constipation responds to regular laxa- 2. Soiling yes/no
tives most of the time. Enemas are seldom needed but are Grade 1: occasionally (1 to 2/week)
promptly prescribed if fecal impaction occurs. Severe soil- Grade 2: every day
ing, not associated with constipation, is extremely rare and Grade 3: constant, social problem
3. Constipation yes/no
may be caused by operative sphincter damage or severe Grade 1: manageable with diet
sacral defects. The mean age at toilet training for feces and Grade 2: requires laxatives
urine as well as frequency of day- and night-time wetting is Grade 3: resistant to diet and laxatives
similar when compared with age-matched controls.18-20

However, there is no standard for manometric evaluation.

Long-term outcome Single fluid-filled balloons in the anal canal have been used
by several authors.24,25 Balloon devices allow only static
Evaluation of long-term functional outcome measurements, therefore the open tip perfused catheter
method is favored by others.26,27 The quantitative pressure
Clinical evaluation recordings in static and dynamic studies are not directly
There is a great variation in the literature regarding func- comparable; the values of manometric tracings tend to be
tional results after repair of anorectal malformations. This is higher with the balloon method.
due to the fact that there is no generally agreed method to Manometric and clinical results have often been found
assess the bowel function of patients with anorectal malfor- contradictory. Some investigators have found positive cor-
mations. The main problem in comparing different series is relation between clinical continence and the anal resting
the highly variable criteria used in the evaluation of fecal pressure profile,23,25,28 whereas others have found no such
continence. Evaluation of bowel function during childhood correlation but a relationship between continence and vol-
may be biased because the information concerning the func- untary squeeze force,29 and still others suggest no correla-
tional outcome is mainly derived from the parents. They tion at all between clinical continence and pressure profile
may not want to report unfavorable results to a surgeon who or squeeze force.26,30 However, a clear correlation between
has been responsible for the treatment of their child. The the presence of inhibitory rectoanal reflex and clinical con-
parents may also ignore minor and moderate defects in tinence has been reported by several authors.23,25,26,28 De-
continence for a child whose bowel function has been de- creased rectal sensitivity with rectal distension has been
ficient from birth or, in the case of smaller children, may reported to correlate with poor functional outcome.25,26,29
consider the situation to be part of normal maturation of Of the available and relevant imaging modalities, MRI is
defecation. The final outcome may not be fully assessed superior because of excellent soft tissue characterization,
until the patients have reached adulthood when as indepen- multiplanar imaging, and lack of exposure to ionizing radi-
dent individuals, patients can evaluate the social conse- ation. Hypoplastic sphincter complex, the misplacement of
quences of possibly defective bowel control. bowel in relation to sphincters, and obtuse anorectal angle
Scoring methods based on subjective parameters with or have been factors related to poor outcome.31 However,
without clinical examination have been designed to get correlation between clinical results and the findings with
quantitative information about the bowel function. The most MRI have not been convincing. The primary value of MRI
commonly used classifications are the Kelly-score,6 the in patients with anorectal malformations is not in detecting
Templeton score,15 Holschneider score,21,22 and Rintala abnormalities in the sphincter complex, but in revealing
score.23 All these classifications ultimately categorize the associated spinal dysraphism, which is common in these
outcomes as good, fair, or poor. Descriptive, nonscoring patients.32
methods to assess and describe functional outcome were
advocated by the Wingspread group of pediatric surgeons.1 Long-term bowel function
This method and the descriptive, nonscoring outcome clas- A major difficulty lies in comparing functional results be-
sification of Peña, based on his extensive series,9 are the tween reported follow-up series. The criteria used to eval-
basis of the new Krickenbeck outcome classification uate long-term outcome are quite variable and mostly de-
method (Table 2). signed for high anomalies.1,6,15,21,33 In patients with high
anomalies, a good result usually means socially acceptable
Objective methods continence which is not equivalent to normal anal function.
Manometric assessment has been the principal method to A patient with a high anomaly and a good functional result
obtain objective data of postoperative sphincter function. rarely has normal bowel function, and although socially
82 Seminars in Pediatric Surgery, Vol 17, No 2, May 2008

continent, they often have a minor degree of smearing or motility and generalized colonic motility disturbance have
soiling associated with physical straining or loose stools. been suggested.17
Although many patients with low malformations have nor- It is likely that the surgical method of anorectal recon-
mal bowel function at long-term evaluation, a method de- struction in high malformations is a significant prognostic
signed to assess long-term outcome in high anomalies may factor. However, this is very difficult to prove since ran-
underestimate minor defects in bowel function, and these domized controlled studies are unavailable. Holschneider40
may become significant when the patient leads a life of an reported significantly better continence outcome in 21 pa-
independent adult individual. tients who had posterior sagittal anorectoplasty compared
with 16 patients having abdominoperineal pull-through with
or without submucosal rectal resection advocated by Reh-
Results in high anomalies
bein. Mulder and coworkers42 found no difference among
patients undergoing sacroabdominoperineal operation and
Prognostic factors those who had posterior sagittal anorectoplasty. deVries in
The level of the anomaly is an important prognostic factor a literature review43 could not find conclusive evidence to
in terms of bowel function. Males with a bladder neck support superiority for any procedure used for anorectal
fistula and females with a high confluence cloaca9 have reconstruction in instances of high anomalies.
significantly poorer prognosis than patients with a lower
urogenital connection.23,28 The obvious cause of poorer
Long-term bowel function during childhood
prognosis in very high anomalies is the more marked hyp-
Reports concerning long-term results for high anomalies
oplasia of the voluntary sphincter muscles, especially the
are highly variable. Most series grade the results as good,
infralevator component of the muscles.9
fair, or poor. It must be remembered that a good outcome
The presence of severe sacral abnormalities is associated
does not mean that the patient has normal bowel function.
with hypoplastic sphincters. If more than two sacral verte-
The patients with a good result have usually been consid-
brae are missing or if the patient has other major sacral
ered socially continent, which implies that the defects in
deformities, such as hemivertebrae and vertebral fusions,
bowel function do not cause significant social disability. In
the functional outcome is worse than in patients with normal
the era before the posterior sagittal anorectoplasty, the re-
sacrum or lesser degree of sacral maldevelopment.9,23
ported percentages of patients, evaluated by clinical criteria,
The role of the internal sphincter in anorectal malforma-
with a “good” result varied between 6% and 56%. The
tions is a topic which has been debated for decades. Re-
percentage of poor results (which has meant more or less
cently, embryological, animal, and clinical studies have
total incontinence) varied between 10% and 70% of affected
documented the presence of the internal sphincter in the
patients (Table 3). It is unlikely that such a wide variation
region of the fistulous bowel termination.34-36 The function-
would reflect true differences in long-term results. The op-
ing internal sphincter can be demonstrated by the presence
erative methods used in all these series were routine proce-
of rectoanal relaxation reflex in anorectal manometry. Most
dures for anorectal reconstruction, and the number of pa-
patients with a low anomaly have positive rectoanal re-
tients in each series were relatively large which implies that
flex.3,28,37 In patients with high malformations, rectoanal
the reporting authors/centers had experience in the repair of
relaxation reflex has traditionally been present in only a
anorectal malformations. The plausible explanation for the
minority of patients.28,30,37 However, when the rectouro-
variation is differences in the strictness of assessment cri-
genital fistulous connection has been preserved at the time
teria. The two relatively recent large series reported by
of anorectal reconstruction, the percentage of patients with
Templeton15 and Rintala3 both used a quantitative multifac-
preserved functional internal sphincter has been between
torial evaluation for continence. Both these series identified
40% and 80%.23,38,39 The presence of internal sphincter has
a lower percentage of poor results than the other series using
been clearly shown to correlate with favorable functional
mainly qualitative criteria. These multifactorial quantitative
outcome.23,25,28,38
assessments seemed to grade continence higher than a qual-
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 Table 3 Functional outcome during childhood: high
functional complication following repair of high anomalies malformations
by posterior sagittal anorectoplasty.9,16,40 The incidence of Before the era of posterior
constipation following PSARP procedure has varied be- sagittal anorectoplasty
tween less than 10%41 and 73%.23 Constipation seems to be
more common when internal sphincter-preserving tech- No. Good Fair Poor
niques have been used.23,38 The cause of constipation is 50
Partridge, et al. 63 33% 43% 24%
unclear; the extensive mobilization of the anorectum may Stephens and Smith6 25 56% 32% 12%
cause partial sensory denervation of the rectum and impair Taylor, et al.30 45 24% 20% 56%
the awareness of rectal fullness. Also, rectosigmoid hypo-
Rintala and Pakarinen Imperforate Anus 83

Table 4 Functional outcome during childhood: high

quence of severe constipation with overflow incontinence,
malformations rather than sphincter insufficiency. The treatment of soiling
associated with constipation is much more rewarding than
Posterior sagittal treatment of soiling related to sphincter insufficiency.
anorectoplasty
Long-term bowel function at adult age
Total Significant
continence soiling Constipation The pediatric surgical literature has only a few reports
concerning functional outcome for high anorectal anomalies
Peña9 36% 41% 43% assessed at adult age (Table 5). The functional results in
Rintala, et al.23 35% 30% 60%
Langemeijer, et al.41 7% 56% 5% 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-
itative clinical assessment, probably because they are more nine patients (62%) had occasional soiling and 11 (23%)
sensitive to the patient’s social adaptation to abnormal ano- frequent soiling or a colostomy. More recently, Rintala and
rectal function.22 Only Taylor and coworkers30 and Rintala3 coworkers45 studied 33 adult patients with a mean age of 35
have reported the incidence of patients with a completely years using a questionnaire-based scoring system. Healthy
normal bowel function without soiling or staining in any adults with an age and sex distribution similar to the patients
circumstances. In both series, only 7.5% of the patients had were used as controls. None of the patients reached a score
full bowel control comparable to that of healthy children. It indicating normal bowel function, and only 6 (18%) had a
is probable that this figure reflects the true incidence of good continence score. All controls had good scores and
unequivocally good long-term outcome in the patients 80% a score indicating normal bowel function. Thirty-one
treated with abdominoperineal and sacral approaches. (94%) of the 33 patients reported some degree of fecal
There are still only a few reports concerning long-term soiling. Hassink and coworkers46 evaluated 58 patients with
functional outcome following posterior sagittal anorecto- a median age of 26 years by using similar scoring methods
plasty, and the results have been contradictory (Table 4). as Rintala and coworkers. Also in this study none of their
Some surgeons report a dismal outcome with most patients patients met the criteria for normal bowel habits. About
requiring adjunctive measures to maintain social conti- 80% of the patients had soiling. In these series, most pa-
nence.41 On the other hand, Peña9 reports that approxi- tients had an abdominoperineal repair as a primary recon-
mately one-third of the patients with high or intermediate struction. In Nixon’s44 series, 68% of the patients had major
anomalies in his personal series could be considered as secondary surgery in an attempt to improve continence. In
totally continent. In the series reported by Rintala and co- Rintala’s45 series 30% and in Hassink’s46 series half of the
workers,23 in which the bowel function of the patients were patients had secondary sphincter repairs.
compared with that of healthy children with similar age and According to above data, it is obvious that almost all
sex distribution, 35% of the patients had an age-appropriate adult patients who have undergone repair of a high anorectal
normal bowel function. A fair outcome with intermittent malformation with traditional methods, such as direct peri-
soiling requiring frequent change of underwear or protective neal, abdominoperineal, or sacro-abdominoperineal opera-
aids, or poor outcome with intractable constipation or total tions, have some form of fecal incontinence despite many
incontinence was found in 30% of the patients. secondary sphincter reconstructions. Although many pa-
Many authors report an improvement in fecal continence tients report being satisfied with their current level of fecal
with increasing age in patients operated by abdominoperi- continence,46 objective evaluation of the data concerning
neal procedure with or without a sacral approach.3,15,33 In bowel function provides a different picture. It is likely that
Rintala’s series, the incidence of good outcome increased the adult patients have accepted their handicap. The patients
from 35% in the age group between 5 and 10 years to 58% have developed measures to cope with unsatisfactory bowel
in patients between 11 and 15 years. The improvement in control, such as staying near toilets, wearing liners or dia-
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 Table 5 Functional outcome at adult age: high
or simply adaptation to the residual dysfunction. On the malformations
other hand, recent reports by Peña and Rintala9,23 on pa-
No. Normal Good Fair Poor
tients operated on by posterior sagittal anorectoplasty have
46
shown that patients with favorable anatomy gain normal or Hassink, et al. 58 0 36% 43% 21%
near normal bowel function as early as age 3 years, provided Rintala, et al.45 33 0 18% 54% 27%
Nixon, et al.44 47 15 62% 23%
that the inherent functional complications related to the (good/fair)
procedure, especially constipation, are treated early and Rintala 2007* 69 43% 36% 9% 12% (ACE)
vigorously. In many cases, soiling during the early years
*Unpublished data.
after posterior sagittal anorectoplasty has been a conse-
84 Seminars in Pediatric Surgery, Vol 17, No 2, May 2008

pers, having regular enemas, or having dietary restric- had scores within the 90th percentile of the controls of
tions.45-47 healthy children; constipation was found in 42% and soiling
At adult age, defective fecal continence has significant in 10% of the patients. Four (10%) patients reported re-
social consequences. The main problem is fecal soiling stricted social life due to fecal soiling. The issue was as-
which restricts social activities. In Rintala’s series,45 85% of sessed further by our institution in a recent prospective
the adult patients reported social disability related to soiling. follow-up study including only boys (median age, 8.5 years;
Other problems especially disturbing to occupational life range 4.3-13.5 years) with perineal (anocutaneous) fistula
were inability to hold back flatus and fecal urgency. Hassink who were compared with age- and sex-matched healthy
and coworkers47 reported that adult patients had signifi- controls.19 The children and their caregivers were inter-
cantly lower educational level than expected. viewed by an independent third party. A total of 68% of the
There are no reports concerning the functional outcome patients had bowel function scores within the 90th percen-
in adult patients who have undergone repair of their severe tile of the controls; constipation occurred in 41% and soiling
anorectal malformation by posterior sagittal anorectoplasty. in 55% of the patients. The figures for constipation and
In our institution, we reviewed the functional outcome in 69 soiling among controls were statistically and clinically sig-
patients that were older than 15 years at the time of assess- nificantly lower: 8% and 24%, respectively. Soiling oc-
ment (Table 5). Normal continence without any fecal soil- curred occasionally in every child with a reconstructed low
ing or constipation requiring medication was found in 30 malformation, but bowel dysfunction restricted social life in
patients (43%). Twenty-five (36%) had minor problems, only 1 of the 22 patients.
such as constipation requiring medication or occasional In perineal fistula, the anus is anteriorly displaced but is
staining; these functional aberrations did not have any social surrounded by the sphincter muscles. One may accept the
impact, and none of these 25 used any protective aids. Six anterior displacement of the anus and perform a simple
patients (9%) had significant continence problems causing anoplasty or perform more complex PSARP. It may be
frequent soiling and need to use protective pads or change argued that the latter surgical approach is associated with
of underwear. Five of these were mentally retarded. In 8 better functional outcome. To address this issue, we per-
patients (12%), fecal continence was so poor that they formed a prospective multicenter comparison of functional
required a permanent appendicostomy for bowel manage- outcome between these 2 surgical techniques.18 There were
ment with regular washouts. These preliminary data from 1 24 boys treated with anoplasty and 17 boys treated with
institute largely support the former reports of functional PSARP. The groups were comparable regarding age, asso-
outcome during childhood,9,44 suggesting that PSARP is ciated malformations, and sacral dysplasia. The results sug-
superior to the prior traditional methods of repair. In our gest that functional outcome is very similar following both
institution, nearly half of patients who have undergone procedures without any differences in the overall bowel
PSARP-procedure have normal fecal continence beyond function score or in the rate of constipation and soiling.18
childhood. However, the need for redo surgery was significantly in-
creased in the PSARP group.
Results in low anomalies
Bowel function at adult age
Traditionally, the long-term results in low malformations There are only a few functional outcome studies of low
are considered to be good in the great majority of pa- anorectal malformations with a follow-up extending to
tients.33,48-50 Poor outcomes have been related to neurolog- adulthood. Karkowski53 reported good continence in 12
ical damage and mental retardation37 or insufficient long- (80%) of his 15 patients with low malformations. Nixon and
term follow-up and care of the patients.4,44 coworkers4 found entirely normal bowel control in 23
More critical reviews on the long-term outcome have (74%) of his 31 adult or adolescent patients. The remaining
clearly demonstrated a significant number of children with patients had occasional or frequent soiling.
functional defects, the most common of which is chronic More recently, the large series of Ong and coworkers52
constipation followed by soiling. In Yeung’s series,51 15 of and Rintala and coworkers22 have demonstrated that a sig-
the 32 children with a follow-up between 1 and 7 years had nificant percentage of these patients have abnormal anorec-
normal bowel function. Of the remaining 17 patients, all had tal function at the adult age. Ong and coworkers reported 35
constipation and 9 occasional or frequent soiling requiring patients with a follow-up of more than 15 years. Although
treatment. In the series of Ong and coworkers52 concerning the majority were considered to have good continence ac-
70 patients with low anorectal anomalies, there were 35 cording to commonly used clinical scoring methods, only
children under the age of 15 years, 9 of whom were clean, 13 (37%) of the patients were clean at all times. Seventeen
14 had occasional smearing, and 12 had soiling. patients (49%) had fecal smearing and 5 (14%) intermittent
Rintala and coworkers48 compared the bowel function of soiling.52 Rintala and coworkers, using a quantitative scor-
40 children with low anomalies, including patients with ing method, compared the bowel function of 83 patients to
perineal fistula, anal stenosis, and operatively treated ste- that of healthy individuals with similar age and sex distri-
notic anterior perineal anus, to that of healthy children using bution.22 All controls had good fecal continence, 76% with
a scoring system. Only 52% of the reconstructed patients 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 tion behind the urethra and bladder neck (Table 6). Peña9
malformations described a 10% incidence of urinary incontinence in his
233 children who had posterior sagittal anorectoplasty
Urinary tract problems for high or intermediate anomalies, including rectoves-
Urinary tract anomalies occur in more than 40% of all tibular fistulas. In Rintala’s series,58 of 65 patients with
patients with anorectal malformations; however, the actual high or intermediate anorectal malformations repaired by
incidence of long-term urological morbidity is difficult to internal sphincter preserving posterior sagittal anorecto-
assess because only a few long-term follow-up reports have plasty, 8% had long-term postoperative urinary inconti-
specifically addressed urological problems. nence. Also in this series, urinary incontinence was cor-
A high incidence of neurovesical dysfunction in patients related with severe sacral anomalies and high cloacal
with anorectal malformations has been reported by several deformities. A recent institutional analysis of 69 patients
authors.13,54-56 Neurovesical dysfunction is usually congen- older than 15 years of age showed that some kind of
ital and often associated with lumbosacral or intraspinal urinary incontinence was present in 11% of patients with
abnormalities.56,57 Vesicoureteral reflux in patients with high anomalies. In a majority of these patients, the symp-
anorectal malformations is commonly associated with neu- toms were mild and caused no social disability. Two of
rovesical dysfunction, and therefore, carries a high risk of these 69 patients required clean intermittent catheteriza-
recurrent urinary tract infection and subsequent renal dam- tion to remain dry.
age. Urinary incontinence is related to dysplastic sacrum,
urethral and bladder anomalies, and neurovesical dysfunc- Genital anomalies, fertility, and sexual problems
tion. Operative damage to bladder neck or urethra seem to Females with anorectal malformations have a high inci-
account for a minority of causes of urinary inconti- dence of genital anomalies; the most common are vaginal
nence.56,57 and uterine septation anomalies and vaginal agenesis.59
Urinary incontinence is uncommon in patients with low Genital tract and sexual function has been reported to be
anomalies, which probably reflects the lower incidence of impaired in almost half of these patients because of vaginal
spinal anomalies or neurogenic bladder in these patients. scarring.59,60 Vaginal scarring may cause dyspareunia and
Rintala and coworkers44 and Peña9 reported no such cases may interfere with child birth.60 Late gynecological prob-
with urinary incontinence in their recent series. Recent re- lems are especially common in patients with cloaca. Ob-
ports have shown that the age of toilet training for urine is struction of Mullerian structures with subsequent cystic
similar in patients with low anal anomalies and healthy menstrual blood collections have been reported to develop
children.18,19,48 in a high percentage of postpubertal girls with cloaca.62
Urinary incontinence was common in patients with high There is little information about fertility in patients with
anomalies operated on by traditional methods in the past anorectal malformations. In Rintala’s series of 83 adult
(Table 6). It appears, however, that patients who have had patients21 with low malformations, 47 (57%) had offspring
posterior sagittal anorectoplasty have a lower incidence of of their own. In the same study, 54% of the healthy controls
neurogenic bladder and urinary incontinence than patients with similar age and sex distribution had children of their
operated on by earlier traditional methods, although poste- own. On the other hand, in another study from the same
86 Seminars in Pediatric Surgery, Vol 17, No 2, May 2008

institution,45 concerning high malformations, only 39% of tients with redo-operations had initially worse continence
the patients had children, which was significantly less than than those with only one operation.
healthy controls, 60% of whom had offspring. Obviously, Gracilisplasty has been a common method for secondary
the low frequency of offspring in patients with high anom- sphincter reconstruction. Several reports have shown a clear
alies reflects true infertility in a significant percentage of improvement in fecal continence in the short term.67,68 The
patients. Ejaculatory duct obstruction has been reported in improvement in continence is caused by somewhat increased
males,61 some have erectile dysfunction, weak or missing resting pressure67 and significantly increased squeeze pressure.
erections, or retrograde ejaculations,45 and some females In adults who have had gracilisplasty during childhood, the
have Mullerian structure agenesis.59 On the other hand, functional results are not encouraging.44-46 The fecal con-
some patients may avoid sexual contacts because of defec- tinence is no better and may be worse than in patients with
tive fecal continence. Rintala21,45 reported that 20% of the only primary reconstruction. Recently, continuous electrical
patients with high anomalies and 13% of the patients with stimulation of the gracilis muscle has been shown to induce
low anomalies avoided sexual intercourse because of poor a transition in muscle composition, from fatigable type II
bowel control. fibers to fatigue-resistant type I fibers.69 In this report,
patients underwent gracilisplasty followed by implantation
Vertebral anomalies and myelodysplasias of a muscle stimulator. After a training period, the stimu-
In the literature, there are essentially no reports concerning lator was used continuously to maintain constant anal tone.
late problems related to vertebral anomalies in patients with Short-term clinical and manometric results were promis-
anorectal malformations. In the senior author’s consecutive ing,69 but longer follow-up has, however, revealed that only
series of 270 high anorectal anomalies operated on between one-third of patients develop satisfactory continence.70
the years 1984 and 2006, 5 patients have required operative Levatorplasty, originally described by Kottmeier and co-
spinal stabilization because of progressive scoliosis. A re- workers,71 was popularized as a secondary sphincter recon-
port from the same institution noted that 16% of adults struction by Puri and Nixon.72 Encouraging results have
with anorectal malformations had spine-related symptoms, been published by several authors.44,73,74 The functional
mainly chronic back pain.21,45 improvement following this procedure has been thought to
Recently, much attention has been placed on the occur- be related to creation of an acute anorectal angle, because
rence of myelodysplasias in patients with anorectal anom- actual resting or squeeze pressures are not changed at the
alies.54,63,64 The effect of spinal abnormalities, especially level of the anal canal. Long-term outcomes in adults are not
tethered cord on long-term functional outcome in terms of encouraging. There are no significant differences in fecal
bladder and bowel function or neurological symptoms in the continence between those who had secondary levatorplasty
lower extremities, is unclear, although some recent reports and those with primary repair only.45,46
suggest that worsening of neurologic function due to spinal Rerouting of the pulled-through bowel has been advo-
anomalies is possible.63 There appears to be no evidence to cated for patients who have a misplaced anal canal follow-
support prophylactic detethering of patients who do not ing primary operation.6,75 The bowel may traverse the le-
have specific symptoms related to tethering.64,65 There is vator and not lay anterior to it. Essentially identical
also no evidence to support the concept that tethered cord procedures for rerouting and repair of the muscular anal
affects functional outcome in terms of fecal or urinary canal have been suggested by Stephens, Kiesewetter, and
continence in patients with anorectal malformations.64,66 Peña.6,75,76 The repair is performed through a posterior
Screening for spinal abnormalities is clearly indicated in sagittal sacroperineal incision and includes splitting of the
all patients with anorectal malformations, including patients
voluntary sphincter muscles in the midline as in standard
with low anomalies.32,63 Normal vertebral anatomy on plain
posterior sagittal anorectoplasty.
spinal radiographs does not preclude the presence of spinal
The reported outcomes in terms of improved fecal con-
cord abnormalities.63 Screening can be performed by ultra-
tinence have been variable. Following redo posterior sagit-
sound during early infancy or by MRI at any age.
tal anorectoplasty, Peña found very significant improvement
in 52% of his 62 patients, mild improvement in 18%, and no
Methods to improve defective fecal continence improvement in 12%; the length of follow-up is not given.
Mulder and coworkers reported that 25% of their 20 patients
Secondary reconstructions became continent following this procedure; the mean fol-
Secondary reconstructions to improve fecal continence have low-up period was 3.5 years. Brain and coworkers77 had a
been used extensively in patients with anorectal malforma- success rate of 16% following a relatively short follow-up
tions. In most long-term follow-up series extending to adult- period. Rintala and coworkers78 followed-up 16 patients
hood, a significant proportion of patients have undergone with redo-posterior sagittal anorectoplasty beyond child-
various types of redo-surgery.44-46 In most reports, the long- hood (mean follow-up period 6 years). Although the clinical
term functional outcome is not better in patients who had continence and manometric findings initially improved in 13 of
secondary surgery45 and may be worse than in those with the 16 patients, at adult age only 4 (25%) of the patients could
only primary repair.46 It is, however, possible that the pa- be considered continent. According to the results of these
Rintala and Pakarinen Imperforate Anus 87

reports, the role of secondary PSARP in the treatment of fecal hood. Secondary surgery for failed or inadequate primary
incontinence after primary reconstruction of anorectal malfor- reconstruction is unlikely to provide results that are com-
mations remains unestablished. parable to those following a successful primary repair.
Other secondary sphincter substituting methods for fecal Patients with anorectal malformations need careful fol-
incontinence following reconstruction of anorectal malfor- low-up throughout their childhood. Functional complica-
mations include free transplantation of palmaris longus tions, especially treatable ones such as constipation, should
muscle, gluteus muscle plasty, free smooth muscle trans- be detected and treated early to achieve optimal outcome.
plantation, and artificial sphincters. None of these methods The treatment of defective continence should be started well
has gained widespread popularity. before the child reaches school age to overcome the devas-
Sacral nerve stimulation has yielded promising results in tating social consequences of fecal soiling and to integrate
patients with neurogenic bladder and bowel dysfunction.79 the child into the social context of his peers. Because the
It remains to be seen if this modality can be successfully management of anorectal malformations requires years of
used in patients with anorectal anomalies. Other modern commitment and special knowledge concerning the anatom-
modalities to treat fecal incontinence (bulk agents, Secca ical and physiological characteristics of this complex group
procedure, artificial sphincters) have been used in patients of congenital malformations, these children should be
with anorectal anomalies only infrequently, and presently treated by experienced medical personnel in specialized
no follow-up data are available. referral centers.
In some patients, late fecal soiling is related to intractable
constipation.9,12,23,80 Many of these patients have an ady-
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