Pediatr Surg Int (2014) 30:773–781
DOI 10.1007/s00383-014-3535-5
ORIGINAL ARTICLE
The influence of anorectal malformations on fertility: a systematic
review
E. C. P. Huibregtse • J. M. Th. Draaisma •
M. J. Hofmeester • K. Kluivers • I. A. L. M. van Rooij
I. de Blaauw
Accepted: 18 June 2014 / Published online: 27 June 2014
Ó Springer-Verlag Berlin Heidelberg 2014
Abstract
Purpose This systematic review aims to give an overview
of available evidence concerning the influence of anorectal
malformations (ARM) on fertility.
Methods We conducted a search in PubMed, EMBASE
and Cochrane Library conformed to the PRISMA stan-
dards. All studies reporting on fertility and ARM were
included.
Results 2,905 studies were identified. Based on title,
abstract and full text, nine articles on 429 patients remained
to answer the research question. Childbirth rate was the
only reported outcome parameter to describe fertility. An
overall childbirth rate of 27 % (range 0–57 %) was found.
Mean age at time of study ranged from 23 to 35 years.
There was no statistical significant difference in childbirth
rate between female and male patients, based on seven
E. C. P. Huibregtse
M. J. Hofmeester
I. de Blaauw (&)
Department of Pediatric Surgery, Radboud University Medical
Center, Geert Grooteplein 10, 6525 GA Nijmegen,
The Netherlands
e-mail: Ivo.deBlaauw@radboudumc.nl
J. M. Th. Draaisma
Department of Pediatrics, Radboud University Medical Center,
Nijmegen, The Netherlands
K. Kluivers
Department of Gynecology and Obstetrics, Radboud University
Medical Center, Nijmegen, The Netherlands
I. A. L. M. van Rooij
Department for Health Evidence, Radboud University Medical
Center, Nijmegen, The Netherlands
I. de Blaauw
Department of Pediatric Surgery, Erasmus MC-Sophia
Children’s Hospital, Rotterdam, The Netherlands
•
studies (p = 0.45). Patients with a more complex type of
ARM (imperforated anus without fistula, rectourethral
bulbar and prostatic fistulas, rectobladderneck fistulas and
cloacal malformations) had a lower childbirth rate com-
pared to healthy controls, whereas in patients with a less
complex ARM (rectoperineal or rectovestibular fistula) the
childbirth rate was similar to healthy controls. Patients with
a more complex type of ARM had a significant lower
childbirth rate than patients with a less complex type of
ARM (18 vs 47 %, respectively) (p = 0.0001). When
further dividing these patients by gender, this difference
was only seen in female patients (p = 0.04).
Conclusion In patients with a more complex type of
ARM a lower childbirth rate was found compared to
healthy controls and patients with a less complex type of
ARM. The latter was only seen in female patients. How-
ever, conclusions concerning fertility in ARM patients
have to be taken with caution due to limited quality of the
studies. Further investigation is recommended.
Keywords Anorectal malformations
Fertility

Childbirth
Introduction
Anorectal malformations (ARM) comprise a wide spec-
trum of congenital conditions, characterized by an atresia
or narrowing of the anorectal canal, with or without a fis-
tula to neighboring organs. The malformations vary
between minor defects with an excellent functional prog-
nosis and complex defects with poor functional prognosis.
ARM are associated in 40–70 % with other congenital
malformations [1–4], such as those present in the VACT-
ERL association (vertebral, anal, cardiac, trachea-
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esophageal, renal, limb anomalies) or other genital and
sacral malformations, but may also occur isolated [1–3].
The incidence of ARM is approximately 2–6 births per
10,000 births worldwide [5].
The initial therapy to obtain the function of the rectum is
by surgery, which most often takes place in the first 2 years
of life. Improvement in medical care has led to decreased
morbidity and mortality, but complete functionality may
still not be obtained, often leading to some degree of
physical and social morbidity. The vast majority of patients
with an ARM reaches adulthood nowadays. In this phase of
life the influence of ARM on fertility plays a role. It is
known that persons with all kinds of congenital malfor-
mations have lower childbirth rates than those without [6,
7]. The term fertility is defined as the capacity to conceive
or to induce conception and to produce offspring. Fertility
is influenced by many factors, including different topics
ranging from the quality of the sperm cells and oocyts to
the maintenance of a pregnancy. Fecundability or time-to-
pregnancy is the best term to describe fertility.
The reasons for impaired fertility are complex and
multifactorial in ARM patients. These reasons may be
related to the associated congenital malformations of the
genital organ, spine and spinal cord, the surgery performed
in the small pelvis, as well as the psychosocial problems
caused by fecal incontinence, constipation, urinary incon-
tinence and impaired sexual function.
Although fertility is a very important issue in life, as has
been indicated by parents’ societies [8], literature on this
topic is not abundant. Therefore, it is relevant to juxtapose
evidence and draw (preliminary) conclusions, which can be
used to inform patients, parents and health care workers.
The aim of our study was therefore to conduct a systematic
review on the influence of ARM on fertility.
Methods
Search strategy
A literature search was performed in September 2013 in
PubMed, the Cochrane Library and EMBASE to obtain all
publications on studies that reported on ARM and fertility.
The PubMed search used the Medical Subject Headings
(MeSH) terms ‘‘Congenital abnormalities’’, ‘‘Anal Canal’’,
‘‘Anal’’, ‘‘Rectum’’, ‘‘Fistula’’, ‘‘Perineum’’, ‘‘Cloaca’’ and
the text words ‘‘Anorectal malformation*’’, ‘‘Abnormali-
ties’’, ‘‘Malformations’’, ‘‘Anus’’, ‘‘Anal’’, ‘‘Rectum’’,
‘‘Rectal’’, ‘‘Anorectal’’, ‘‘Fistula’’, ‘‘Perine*’’, ‘‘Vestibu*’’,
‘‘Rectoperineal’’, ‘‘Rectovestibular’’, ‘‘Rectourethral’’,
‘‘Rectovesical’’, ‘‘Rectobladderneck’’ and ‘‘Cloaca’’ com-
bined with the MeSH terms ‘‘Fertility’’, ‘‘Infertility’’,
‘‘Pregnancy rate’’, ‘‘Pregnancy’’, ‘‘Reproductive
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Pediatr Surg Int (2014) 30:773–781
techniques’’, ‘‘Quality of life’’ and the text words ‘‘Fertil-
ity’’, ‘‘Infertility’’, ‘‘Subfertility’’, ‘‘Time-to-pregnancy’’,
‘‘Pregnancy rate’’, ‘‘Pregnancy’’, ‘‘Fecundity’’, ‘‘Fecunda-
bility’’, ‘‘Reproductive techniques’’, ‘‘Quality of life’’ and
‘‘QoL’’.
The Cochrane search used the same MeSH terms and
text words.
The EMBASE search used the EMTREE terms ‘‘Ano-
rectal malformation’’, ‘‘Anus’’, ‘‘Rectum’’, ‘‘Anal canal’’,
‘‘Anus fistula’’, ‘‘Rectum fistula’’, ‘‘Rectovaginal fistula’’,
‘‘Perineum’’, ‘‘Fistula’’, ‘‘Cloaca’’ and the text words
‘‘Anorectal malformation*’’, ‘‘Abnormalities’’, ‘‘Malfor-
mation*’’, ‘‘Rectum’’, ‘‘Rectal’’, ‘‘Anus’’, ‘‘Anal’’, ‘‘Anal
canal’’, ‘‘Fistula’’, ‘‘Perine*’’, ‘‘Vestibu*’’, ‘‘Rectoperi-
neal’’, ‘‘Rectovestibular’’, ‘‘Rectourethral’’, ‘‘Rectovesi-
cal’’, ‘‘Rectobladderneck’’ and ‘‘Cloaca’’, combined with
the EMTREE terms ‘‘Fertility’’, ‘‘Infertility’’, ‘‘Reproduc-
tion’’, ‘‘Childbirth’’, ‘‘Conception’’ ‘‘Fertilization’’, ‘‘Par-
ity’’, ‘‘Pregnancy’’, ‘‘Pregnancy outcome’’, ‘‘Pregnancy
rate’’, ‘‘Infertility therapy’’, ‘‘Quality of life’’, ‘‘Birth rate’’
and the text words ‘‘Fertility’’, ‘‘Infertility’’, ‘‘Subfertility’’,
‘‘Pregnancy outcome’’, ‘‘Pregnancy rate’’, ‘‘Birth rate’’,
‘‘Pregnancy’’, ‘‘Fecundity’’, ‘‘Fecundability’’, ‘‘Child-
birth’’, ‘‘Infertility therapy’’, ‘‘Quality of life’’ and ‘‘Time-
to-pregnancy’’.
Limits were set to articles on humans.
We conformed to the PRISMA statement to achieve the
highest standard in reporting items for a systematic review
[9, 10].
Eligibility criteria
All studies that reported on any outcome measure regard-
ing fertility in patients with an ARM were included.
Studies on cloacal exstrophy were excluded, because of the
complexity of the malformation with involvement of the
bladder. Furthermore, case reports, studies without access
to the full text and studies not written in English were
excluded. The references of included studies were
reviewed to include useful studies that might have been
missed with the initial search (cross check).
Study selection
The study selection was dependent on four different steps;
search strategy, study screening, study eligibility and study
inclusion. They were all performed by one researcher (EH),
under supervision of two medical experts (IdB, JD).
Data extraction
A predefined checklist was used. Data on study design,
number and type of population, definition used to describe
Pediatr Surg Int (2014) 30:773–781
fertility, percentage of childbirth and described subtypes of
ARM were collected. The Krickenbeck classification was
used to define the subgroups of ARM patients, based on
fistula type and the clinical use [11]. It was designed to
make comparison of follow-up studies possible. However,
the Wingspread classification was the most frequently used
classification of ARM in literature. It classified different
types of ARM in high, low and intermediate and contained
a separate group for cloacal and rare malformations [12].
We therefore reclassified patients from the Wingspread
classification, into the Krickenbeck classification as
described in Table 1. ‘The more complex ARM group’
consisted of imperforated anus without fistula, rectoure-
thral bulbar and prostatic fistulas, rectobladderneck fistulas
and cloacal malformations, whereas ‘the less complex
ARM group’ consisted of rectoperineal and rectovestibular
fistulas.
Statistical analysis
Descriptive analyses were performed. Childbirth rate was
calculated by cumulating the number of patients with
children of their own divided by the total patients inclu-
ded in the studies, when possible. 95 % confidence
intervals were calculated around the mean. Using the
Fisher’s exact test, we statistically tested differences in
childbirth rate between males and females and between
the less complex ARM group and the more complex
ARM group.
Table 1 Translation of the Wingspread classification to the Krickenbeck classification
Type of classification Krickenbeck classification
Males Rectoperineal fistula
Rectourethral bulbar fistula
Rectourethral prostatic fistula
Rectobladderneck fistula
Imperforated anus without fistula
Complex and unusual defects
Female Rectoperineal fistula
Rectovestibular fistula
Cloaca with short common channel (\3 cm)
Cloaca with long common channel ([3 cm)
Imperforated anus without fistula
Complex and unusual defects Cloacal exstrophy, covered cloacal exstrophy
Posterior cloaca
Associated to presacral mass
Rectal atresia
775
Results
The search resulted in 1,213 abstracts in PubMed, 23
abstracts in the Cochrane library and 1,669 abstracts in
EMBASE. After exclusion of duplicates, 2,291 abstracts
remained. Based on title, 2,145 abstracts were excluded.
Based on abstract, another 111 abstracts were excluded.
Reasons for exclusion were that articles were not reporting
on ARM (n = 27), not reporting on fertility (n = 71),
reporting on cloacal exstrophy (n = 5), concerning case
reports (n = 18) or not written in English or no full text
was available (n = 17). One study was included by cross
checking references. Eventually, nine articles were left to
answer our research question (Fig. 1).
These nine studies were published between 1992 and
2013 in developed countries. Two out of the nine studies
made a comparison with healthy controls [13, 14], the
others studies were case series from a certain hospital or
country. The total number of patients included ranged from
21 to 83 per study, in total 163 females and 120 males with
ARM. The mean age at time of the study ranged from 23 to
35 years. All studies presented a differentiation in subtypes
of ARM, but there was variation in reported classifications.
Only one study used the Krickenbeck classification [8].
Childbirth rate was used to describe fertility in all studies
and was defined as the percentage of persons who have
children of their own. One study also mentioned the fer-
tility rate (number of children per woman) [15]. Informa-
tion on the number of patients who intended to conceive
was available in two studies [16, 17]. Table 2 shows the
Wingspread classification
High Intermediate Low Cloaca
X
X
X
X
X
X
X
X (intermediate)
X (high)
X
X
X
X
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776
Fig. 1 Flow chart of study
selection
study characteristics and Table 3 provides an overview of
the results.
Hundred and fifteen of the 429 patients with ARM had
children of their own. This makes the overall childbirth rate
27 % [8, 13–20]. The two studies comparing patients with
healthy controls showed that in patients with a less com-
plex ARM, childbirth rate was similar, whereas in patients
with a more complex ARM, childbirth rate was lower
compared to controls [13, 14]. No differentiation was made
between female and male patients.
In seven out of nine studies, childbirth rate could be
distracted by gender. There was no statistical significant
difference in childbirth rate between female (19 %) and
male patients (15 %) (p = 0.45). Childbirth in the more
complex ARM group could be separated from the less
complex ARM group, also in seven out of nine studies.
This resulted in more childbirth in patients with a less
complex ARM [57 of the 122 patients had children (47 %)]
[13, 15, 19] than in patients with a more complex ARM [31
of the 176 patients had children (18 %)] (p = 0.0001) [14,
15, 17–20]. When dividing these patients by gender, a
difference in childbirth rate in male patients with a less
(10 %) and more (11 %) complex ARM was not found
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Pediatr Surg Int (2014) 30:773–781
(p = 1.00). However, female patients with a more complex
ARM did have a lower childbirth rate than female patients
with a less complex ARM (14 vs 39 %, respectively). This
difference was statistical significant (p = 0.04).
Cloacal malformation was the only subtypes of ARM
that could be studied as a separate group [8, 15, 17–20]. Of
the 79 included patients with a cloaca, 12 had offspring of
their own (15 %). Mean age of the patients studied ranged
from 23 to 26 years.
Discussion
There is a lower childbirth rate in female patients with
more complex ARM, compared to female patients with less
complex ARM. This result may be explained by the fact
that the incidence of genital malformations is significantly
increasing with an increased complexity of the ARM [21–
23]. In patients with a rectoperineal fistula, genital mal-
formations are rare (0–3 %) [21, 23], whereas genital
malformations are by definition associated with patients
with cloacal malformations, the most complex malforma-
tions in females. In these patients, 30 % present with a
 Table 2 Study characteristics
References, country Definition used for fertility Study design and method of data Number of Patients with children of Mean age at time of Type of ARM included
collection patients their own, no. (%) study (years)

Rintala [13], Childbirth rate Case-control study using 53 F Cases: 48 (57 %) 35.2 (SD 4.1) 8 Anal stenosis
Finland questionnaires Controls: 45 (57 %) 3 Anocutaneous fistula
38 Anovestibular/vulvar fistula
4 N-type rectovestibular fistula
30 M 16 Anal stenosis
14 Anocutaneous fistula
Pediatr Surg Int (2014) 30:773–781

Rintala [14], Childbirth rate Case-control study using 8F Cases: 13 (39 %) 35.3 (SD 4.8) 4 Rectovaginal fisula
Finland questionnaires Controls: 21 (60 %) 3 Cloaca
1 Cloacal extrophy
25 M 19 Rectourethral fistula
2 Rectovesical fistula
2 Anal agenesis
1 Atresia Recti
1 Rectobulbar fistula
Schmidt [8], Childbirth rate Case series using personal 23 F 5 (22 %) 23 (range 18-56) 6 Perineal
Germany interviews 32 M 3 (9 %) 19 Rectouretral
5 Rectovesical
10 Vestibular
6 Cloaca (1/6 got children)
3 Imperforated anus
0 Anal stenosis
4 Others
2 Unknown
Hassink [18], The Childbirth rate Case series using questionnaires 19 F 3 (16 %) 26.0 (range High
Netherlands 39 M 5 (13 %) 18.1–56.9)
Davies [16], United Childbirth rate Case series using questionnaires 44 F 7 (16 %) 25.6 (SD 0.6) 9 High
Kingdom 15 Cloaca
17 Low
3 Unknown
30 M 11 (37 %) 6 High
16 Low
8 Unknow
777

123
 Table 2 continued
778

References, country Definition used for fertility Study design and method of data Number of Patients with children of Mean age at time of Type of ARM included
collection patients their own, no. (%) study (years)

123
Mantoo [15], Fertility rate (children/ Case series using questionnaires 21 F 6 (29 %) 28 (SD 9) 6 High (0/6 got children)
France woman). Childbirth rate 14 Low (5/14 got children)
1 Unknown (1/1 got children)
31 M 4 (13 %) 12 High (2/12 got children)
18 Low (2/18 got children)
1 Unknown (0/1 got children)
Iwai [19], Japan Childbirth rate Case series using questionnaires 11 F 4 (36 %) 29.3 3 High (2/3 got children)
or personal interviews 4 Intermediate (0/4 got children)
4 Low (2/4 got children)
18 M 0 (0 %) 25.8 10 High (0/10 got children)
5 Intermediate (0/5 got children)
3 Low (0/3 got children)
Hendren [20], Childbirth rate Case series using hospital charts 24 F 6 (25 %) X Cloaca
Massachusetts,
US
Warne [17], United Childbirth rate (pregnan- Case series using hospital charts 21 F 0 (0 %) 24 Cloaca
Kingdom cies)
F female, M male, X not reported, SD standard deviation
Pediatr Surg Int (2014) 30:773–781
Pediatr Surg Int (2014) 30:773–781
Table 3 Childbirth rate in female and male patients with an ARM, divided by type of ARM
% of Type of ARM
childbirth
Rectoperineal Recto- Rectourethral Imperforated
fistula vestibular bulbar fistula anus without
fistula fistula
Female 39 % (95 % CI: 16–61 %)
patients [15, 19]
Male 10 % (95 %
patients CI: 0–22 %)
[15, 19]
Female 47 % (95 % CI: 38–56 %) 18 % (95 % CI: 12–23 %) [14, 15, 17–20]
and [13, 15, 19]
male
patients
hydrocolpos in the newborn period, which can lead to
infection and vaginal scarring and reduce fertility [24].
Furthermore, 40 % of the cloaca patients will have a
double Müllerian system [24]. In another study, Warne
et al. found a normal uterine anatomy in only 12 of the 41
patients with a cloaca (29 %), the other patients had a
unicornuate, bicornuate, double or absent uterus. All
Müllerian abnormalities are associated with increased rates
of pregnancy loss and preterm delivery [25]. 29 % of
patients with a cloaca presented themselves for gyneco-
logical examination with an obstructed uterus due to ste-
nosis of persistent urogenital sinus or cervical stenosis [26].
The inadequate outflow of menstrual blood can result in
secondary damage, e.g., endometriosis, leading to further
infertility [25, 26].
In male ARM patients, cryptorchidism and hypospadias
are the most common genital malformations. In patients
with hypospadia, no convincing evidence has been
described for impaired fertility [27]. Cryptorchidism is
described in 19 % of the boys with an ARM [28]. The
higher the level of ARM, the higher the incidence [28].
Paternity rates have been found reduced in one-third of the
patients with bilateral cryptorchidism [29]. If the incidence
of bilateral cryptorchidism increases with an increased
complexity of ARM, a decreased childbirth rate is expected
in patients with more complex ARM. Apparently, genital
malformations in male patients have less influence on
childbirth rate than in female patients, because difference
in childbirth rate between less and more complex ARM in
male patients was not seen in our study.
In spite of the evolution in surgical management of
ARM [30], a second possible reason for a decreased
childbirth rate is the possibility of iatrogenic damage. Since
the mid-1980s, most patients are operated by the posterior
779
Rectourethral Rectobladder- Cloaca Overall
prostatic neck fistula
fistula
15 % (95 % 19 % (95 % CI:
CI: 7–23 %) 13–25 %) [8,
[15, 17–20] 15–20]
11 % (95 % CI: 3–19 %) [15, 15 % (95 % CI:
18, 19] 9–21 %) [8,
15, 16, 18, 19]
28 % (95 % CI:
24–33 %) [8,
13–20]
sagittal anorectoplasty (PSARP). This technique allows a
direct visualization of the anatomy of the muscle complex
and nerves of the pelvic floor are spared as much as pos-
sible [31]. In female patients with less complex ARM,
complications such as scarring of the perineum or vagina
may occur, but are seldom described. Further damage to
the reproductive tract is even less likely. Female patients
with more complex ARM, such as cloacas, require a more
challenging operation, with the risk of complications such
as ischemic vaginal atresia, stenosis, recurrent fistulas or
injury to the Müllerian structures. In 30 % an additional
abdominal approach will be necessary [32]. Long-term
outcome data on the effect of these complications are,
however, limited.
In male patients, surgery of the most frequent recto-
perineal fistula, does normally not involve the exposure of
the reproductive tract and will therefore not affect fertility
rates. Surgical exposure during PSARP performed in the
other types of ARM is closer to the ejaculatory ducts,
seminal vesicals, prostate, vas deference and nerves,
responsible for sexual function. Iatrogenic complication
rate of the reproductive tract was however only 1.7 % (10
out of 572 patients) in a study by Hong et al. [33]. Long-
term outcomes regarding fertility in these patients are not
available.
Another reason for a decreased childbirth rate in more
complex ARM may be related to psychosocial aspects of
functional disorders such as fecal incontinence, constipa-
tion, urinary incontinence and impaired sexual function.
These disorders are more often present in patients with
more complex ARM. Psychosocial consequences may
influence sexual activity and therefore indirectly have an
impact on childbirth, but high quality studies are lacking on
this subject.
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780
Comparing results of fertility in patients with ARM
remains difficult, because of the several limitations of the
studies included. Fertility in ARM patients should be
properly assessed and compared with a sex-, age- and
social-matched control group. This adequate comparison
was performed in only two studies. Small patient groups
were used that could have caused the wide variation in
childbirth rate. The mean age of patients in the cohorts of
our review was relatively young, whereas the mean age to
become a parent is usually higher. This obviously leads to
a lower childbirth rate, independent from ARM. Another
limitation of most studies is the use of childbirth rate as
measurements for fertility. Questionnaires, personal
interviews and hospital charts were used to count child-
birth in patients with ARM, but only two studies reported
the number of patients who had actively been trying to
conceive [16, 17]. In a study by Davies et al. [16], 7 out
of 44 women had children; the other women had not yet
tried to conceive. In a study by Warne et al. [17], no
children were born, but only four patients had tried to
conceive. When childbirth was measured only in patients
who tried to have children, the childbirth rate would
probably have been much higher. At last, the type of
ARM and additional anomalies should be well docu-
mented in all patients to relate this with the outcome of
fertility.
We recommend further research and studies with a
preferable prospective design, with larger (multicenter)
patient groups, higher mean age, well documented type of
ARM and additional anomalies, time-to-pregnancy or fe-
cundability as outcome measurement and healthy controls,
matched for age, gender, and social status as comparison
group.
Conclusion
The aim of our study was to investigate the influence of
ARM on fertility. The more complex ARM types seem to
have a lower childbirth rate than healthy controls and
patients with less complex ARM. The latter was only seen
in female patients. Possible explanations for these findings
could be found in the additional genital malformations
often seen in female patients, iatrogenic damage of surgery
and psychosocial consequences of ARM related to sexual
activity.
Overall, caution should be taken with interpreting these
results. Due to limited quality of the studies, it remains
difficult to make an unequivocal statement on fertility in
patients with ARM and the underlying causes. Further
investigation is recommended.
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Pediatr Surg Int (2014) 30:773–781
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