Journal of Pediatric Surgery 51 (2016) 1229–1233

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Journal of Pediatric Surgery

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Original Articles

Outcome in anorectal malformation type rectovesical fistula: a

nationwide cohort study in The Netherlands
H.J.J. van der Steeg a,⁎, S.M.B.I. Botden a, C.E.J. Sloots b, A.F.W. van der Steeg c, P.M.A. Broens d,
L.W.E. van Heurn c,e, D.V. Travassos f, I.A.L.M. van Rooij g, I. de Blaauw a
a
Department of Surgery-Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands
b
Department of Pediatric Surgery, Erasmus-MC Sophia Children's Hospital, Rotterdam, The Netherlands
c
Department of Pediatric Surgery, Emma Children's Hospital, AMC and VU University Medical Center, Amsterdam, The Netherlands
d
Department of Pediatric Surgery, University Medical Center, Groningen, The Netherlands
e
Department of Pediatric Surgery, Maastricht University Medical Center, Maastricht, The Netherlands
f
Department of Pediatric Surgery, Wilhelmina Children's Hospital, University Medical Center, Utrecht, The Netherlands
g
Department for Health Evidence, Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen, The Netherlands

a r t i c l e i n f o a b s t r a c t

Article history: Purpose: Outcomes of patients with an ARM-type rectovesical fistula are scarcely reported in medical literature.
Received 28 October 2015 This study evaluates associated congenital anomalies and long-term colorectal and urological outcome in this
Received in revised form 2 February 2016 group of ARM-patients.
Accepted 2 February 2016 Methods: A retrospective Dutch cohort study on patients treated between 1983 and 2014 was performed. Asso-
ciated congenital anomalies were documented, and colorectal and urological outcome recorded at five and ten
Key words:
years of follow-up.
Anorectal malformation (ARM)
Rectovesical fistula
Results: Eighteen patients were included, with a mean follow-up of 10.8 years. Associated congenital anomalies
Bladder neck fistula were observed in 89% of the patients, 61% considered a VACTERL-association. Total sacral agenesis was present
Colorectal outcome in 17% of our patients. At five and ten years follow-up voluntary bowel movements were described in 80% and
Urological outcome 50%, constipation in 80% and 87%, and soiling in 42% and 63% of the patients, respectively. Bowel management
Long-term follow-up was needed in 90% and one patient had a definitive colostomy. PSARP was the surgical reconstructive procedure
in 83%. Urological outcome showed 14 patients (81%) to be continent. No kidney transplantations were needed.
Conclusion: In our national cohort of ARM-patients type rectovesical fistula that included a significant proportion
of patients with major sacral anomalies, the vast majority remained reliant on bowel management to be clean
after ten years follow-up, despite “modern” PSARP-repair. Continence for urine is achieved in the majority of
patients, and end-stage kidney failure is rare.
© 2016 Elsevier Inc. All rights reserved.

Anorectal malformations (ARM) represent a complex group of con-
genital anatomical anomalies of the anorectum, characterized by the ab-
sence of a normal anus at its anatomic position central in the sphincter
complex [1,2]. The estimated prevalence ranges between 1:2000–5000
live births [2,3]. There is a variety of clinical presentations, ranging from
mild to complex ARM, and associated congenital anomalies are present
in 40–70% [4–6]. Most anomalies involved are those that are also part of
the VACTERL-association (vertebral, cardiac, tracheo-esophageal, renal
and limb). Although syndromes are encountered in approximately
10% of the patients (e.g. Townes-Brocks, Currarino syndrome, trisomy
21), the majority of ARM is non-syndromic [2,7].
⁎ Corresponding author at: Radboud University Medical Center, Nijmegen, the
Netherlands, Department of pediatric surgery. Tel.: + 31 24 3619761; fax: + 31 24
3613547.
E-mail address: herjan.vandersteeg@radboudumc.nl (H.J.J. van der Steeg).
An anorectal malformation type rectovesical or bladder neck fistula
is characterized by a fistulous termination of the rectum to the bladder,
typically the bladder neck. Rudimentary elements of the internal anal
sphincter are present in this fistulous termination. When preserved,
they might improve continence in ARM-patients, although data are con-
tradictory [8,9]. This type of anorectal malformation is a severe type of
ARM occurring exclusively in males, in approximately 10% of the pa-
tients [10]. It is further associated with hypoplasia of the buttocks and
external sphincter complex, contributing to the poor functional out-
come for these patients. Data on patients with a rectovesical fistula
(RVF) are typically reported as part of larger cohorts of the full spec-
trum, or of ‘high’ malformations, which also include recto-urethral-
prostatic fistulas [2,11–13]. Until now, ARM-patients with a rectovesical
fistula have not been described as a single cohort. Therefore, a retro-
spective nationwide study was conducted to evaluate associated anom-
alies and long-term functional colorectal and urological outcome for this
rare group of patients.

http://dx.doi.org/10.1016/j.jpedsurg.2016.02.002
0022-3468/© 2016 Elsevier Inc. All rights reserved.

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1230 H.J.J. van der Steeg et al. / Journal of Pediatric Surgery 51 (2016) 1229–1233
1. Patients and methods
A retrospective cohort study was performed in the six pediatric sur-
gical centers in The Netherlands. Charts of all patients with an ARM type
rectovesical fistula treated between 1983 and 2014 were reviewed and
data on associated congenital anomalies, type of surgery and its timing
and complications were collected. Concerning the work-up on associat-
ed (VACTERL) anomalies, all patients in The Netherlands are screened
for the presence of tethered cord by ultrasound in the first week of life
[14]. In case of presence of a tethered cord or doubt on its presence, a
spinal MRI is nowadays performed within the first year of life.
For the patients with a minimum follow up period of 48 months, the
long-term outcome was assessed, considering the fact that in the gener-
al Dutch population children are supposed to be toilet trained by the age
of four [15].
The functional colorectal outcome (voluntary bowel movement,
constipation, soiling and continence) was documented based on chart
notes by the treating surgeon at 4–5 years and 10 years of follow up, re-
spectively, and was graded on the Krickenbeck scoring scale for
obstipation and soiling [16]. Voluntary bowel movement is defined by
the ability to feel urge with the capacity to verbalize and hold. Data on
longer follow-up was scarce, partly because of loss to follow-up or dis-
charge from follow-up, and therefore not analyzed.
The urological and renal function outcomes (spontaneous voiding,
continence, clean intermittent catheterization, urinary diversions or
other urological surgery, recurrent urinary tract infections, end-stage
kidney failure) after 5 years were also documented in this study.
1.1. Statistics
Frequencies of nominal variables are presented including percent-
ages. Continuous variables with a normal distribution are presented
with a mean and standard deviation. In some instances also a range is
shown for extra information. For continuous variables with a skewed
distribution the median and range are provided. Numbers are too
small to perform statistical significant tests.
2. Results
Eighteen patients were identified and included. Mean follow up was
10.8 years (SD: 5 years, range: 2–19 years). Table 1 shows the associated
congenital anomalies observed in 16 patients (89%). Eleven patients
met the criteria of a VACTERL-association (61%). Most prevalent associ-
ated anomalies were sacral vertebral in 12 patients (67%), being
hemivertebrae, sacral fusion, partial sacral agenesis and coccygeal agen-
esis. Three patients had a total sacral agenesis. No patient was diagnosed
with Currarino syndrome. Anatomical kidney anomalies were present
in 11 patients (61%). Of this group there were three patients with a sin-
gle kidney, three presented with a horseshoe kidney, two had
ureteropelvic junction (UPJ) stenosis, one dysplastic kidney, another a
dysfunctional kidney and one had multicystic kidney disease.
Table 1
Associated congenital anomalies in 18 patients with ARM and a rectovesical fistula.
Type of anomaly
Cardiac
Oesophageal
Spinal cord
Sacral
Vertebral⁎
Renal
Limb
⁎ 1 missing value.
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All patients needed a colostomy in the neonatal period, with a com-
plication rate of 17% (3 of 18 patients, in one patient data were missing).
The colostomy was closed at a mean age of 11 months (SD: 5 months,
range: 3–24 months). One patient had a definitive colostomy at the
age of 5, after multiple laparotomies and ischemic complications.
In the majority of patients (83%), reconstructive surgery was per-
formed using a posterior sagittal anorectoplasty (PSARP). For the ab-
dominal phase a laparotomy was performed in 10 and a laparoscopy
in 6 patients (62% and 19% respectively). Of these 6 laparoscopy-
assisted procedures, 3 were combined with a PSARP and 3 had a
laparoscopy-assisted pull through procedure. One of these last patients
eventually had a definitive colostomy. In two patients the data on the
surgical treatment were missing. The median age at reconstructive sur-
gery was six months (range: 2–26 months). To prevent anal stenosis,
anal dilatations according to the Peña guidelines were standard post-
operative care in all pediatric surgical centers [2].
Table 2 shows the number of complications (12) after reconstructive
surgery, which occurred in 59% of the patients. They are divided in
anorectal (10) and urological (2). Of the anorectal complications, two
were considered major (anal stenosis and megacolon), as they have
lead to major redo-surgery. All other anorectal complications can be
considered mild, although the consequences were multiple additional
surgical procedures in three patients. Data on possible reoperations
were missing in the other patients. The urological complications, being
urinary tract infection and a urinary retention, were considered mild.
A mean of 5.9 surgical procedures per patient (SD: 2.8, range: 2–12),
being reconstructive and/or urological, was needed for definitive repair.
Table 3 shows functional colorectal outcome analyses after five and
ten years according to the Krickenbeck criteria in 15 and 10 patients, re-
spectively. Twelve patients (80%) had voluntary bowel movements
after five years and five patients (50%) after ten years.
Constipation was documented in 12 out of 15 patients after five
years, and 7 out of 8 at ten years follow-up (80 and 87%, respectively).
Soiling was reported in 42% of 12 patients after five years, and 63% of
eight patients after ten years follow-up. Grade 3 soiling, defined as so-
cially disabling, was present in 17% and 13% of the patients after five
and ten years respectively.
After ten years follow-up, bowel management was needed in 90% of
the patients (9 out of 10) for the treatment of incontinence and consti-
pation. This was mostly done by administering rectal enemas without
the need for an ACE-conduit [17]. There were no reliable data on the
timing of commencement of bowel management. Fifty percent of the
patients used laxatives, initiated by the individual surgeon not based
on any predefined criteria, and occasionally on request by the patient
and/or his parents. One patient (10%) had a definitive colostomy.
Urological outcome on 16 patients could only be evaluated at 5 years
follow-up (Table 4). Seven of the included 18 patients (39%) had vesico-
Table 2
Complications after reconstructive surgery.
Complication⁎ N = 17⁎⁎ Surgical intervention
None 7 (41%)
Anorectal 10
Anal stenosis 4 1 redo-PSARP, 3 repetitive anal
dilatations under anesthesia
Anal prolaps 2 Unknown
N (%)
Mechanical bowel obstruction 2 Unknown
5 (28) Megacolon 1 Twice plication in a single patient
1 (6) Stenosis of colostomy 1 Unknown
4 (22) urologic 2
12 (67) Urinary tract infection 1
2 (11) Urinary retention 1
11 (61) Total complications 12
2 (11)
⁎ Patients can have multiple complications.
⁎⁎ N is number of patients.
 H.J.J. van der Steeg et al. / Journal of Pediatric Surgery 51 (2016) 1229–1233 1231

Table 3 perform definitive surgery for all ARM at the age of 2–3 months. As
Long-term functional colorectal outcome of patients with ARM and a rectovesical fistula. this is a multicenter and retrospective study, it is unclear whether this
5 years (N = 15) 10 years (N = 10) delay was because of associated anomalies that needed prior attention,
Soiling (grade) no 7 (out of 12) (58%) 3 (out of 8) (37%)
or if this actually reflects the practice of the individual center/surgeon
1 2 (17%) 1 (13%) involved in the most complex ARM in males.
2 1 (8%) 3 (37%) Voluntary bowel movements were reported in 80% after five years
3 2 (17%) 1 (13%) follow-up and 50% after ten years. As a single variable voluntary
Constipation (grade) no 3 (out of 15) (20%) 1 (out of 8) (13%)
bowel movement does not imply continence. In our group after a
1 0 (0%) 1 (13%)
2 5 (33%) 2 (25%) follow-up of 10 years, all patients but one, having a definitive colostomy,
3 7 (47%) 4 (50%) were fecally incontinent despite having voluntary bowel movements.
Bowel management 13 (out of 15) (79%) 9 (out of 10) (90%) Incontinence and soiling are considered to be the major functional
Laxative treatment 10 (out of 15) (67%) 5 (out of 10) (50%) problems in ARM type RVF [2]. Bowel function was registered at 5 and
10 years follow-up. The relatively good outcome in soiling in this
study (13% grade 3 after ten years of follow up) can be explained by
the high percentage of patients on bowel management. Our study also
ureteral reflux (VUR) at or shortly after birth (2 grade I, 2 grade II, 2 shows that the majority (87%) of the patients with an RVF also suffer
grade III and 1 grade IV). The 3 patients with a VUR grade III-IV all had from constipation and possible (overflow) incontinence. Constipation
ureter reimplantation (43% of all VUR-patients). No reliable data on pos- and incontinence are thus co-existing in these patients and difficult to
sible prophylactic antibiotics could be collected after 5 years. Nine pa- differentiate, as there is considerable overlap. Bowel management is
tients (56%) voided spontaneously. Thirteen patients (81%) were an essential tool to realize social (pseudo)continence, as it treats both
considered continent for urine either by spontaneous voiding or clean constipation and (overflow) incontinence simultaneously. Another ex-
intermittent catheterization (CIC). Five patients (28%) needed a total planation for the good outcome in soiling, may be that the definition
of nine additional urological surgical procedures. Six patients (38%) suf- of grade 2 in the Krickenbeck classification (daily soiling without social
fered from recurrent urinary tract infections. Of these six, all had ana- problems) is easily mistaken for grade 3 soiling because of poor valida-
tomical renal anomalies (two horseshoe kidney, two UPJ-stenosis, one tion of the scoring system and the retrospective nature of our study. Sig-
dysplastic kidney and one single kidney), and two had VUR (both nificant soiling was reported in 30–56% of the patients in other studies
Grade III). No end-stage renal failure, represented by kidney transplan- [1,22,23], although in these series the results on functional outcome
tation, was seen in any of the patients during the follow up. were contradictory and therefore the results on soiling should be
interpreted with caution.
Results considering fecal continence in literature vary considerably.
3. Discussion Peña [1] reported that approximately one-third of the patients with
high or intermediate anomalies operated on with a PSARP in his series
Anorectal malformation type rectovesical fistula is the most complex could be considered totally continent. Earlier series of Taylor [24] and
and therefore the most challenging colorectal anatomical anomaly in Rintala [25] showed worse outcome of a mere 7.5% of the patients with
males. To date, this nationwide study is the largest to report the out- high malformations having full bowel control. Surgical reconstructive
come of this specific group of patients. techniques varied between studies, as these patients were primarily oper-
Associated congenital anomalies were reported in 89% of the pa- ated on with a (sacro)abdominoperineal (AP) approach, and therefore
tients in our series. Other studies in patients with ARM show associated data are not completely comparable. It is reported that fecal incontinence
anomalies in 40–70% of the patients, increasing with the complexity of can improve with increasing age [25,26]. It is unclear whether this im-
the ARM to 80% [2,4–7,18–20]. VACTERL-association was present in provement is an actual functional improvement of sphincteric function,
61% of the patients, which is relatively high compared to the mentioned or a gradual adjustment to residual dysfunction [27]. Our study clearly
15–37% in previous studies [5,7,21]. Additionally, sacral anomalies were did not show any significant improvement in fecal continence with in-
present in the majority of cases, including 17% sacral agenesis. Thus, this creasing age, but follow-up was till 10 years of age. It is interesting to
cohort confirms the high prevalence of associated congenital anomalies know if and how continence develops in puberty and young adulthood.
in patients with ARM rectovesical fistula, including those anomalies that In recent data, PSARP is reported to give better short-term [26,27], as
have a negative effect on colorectal and urological function. well as long-term results [28,29] regarding continence, than the prior
The median age at reconstructive surgery was six months which used AP approaches. Overall, AP procedures seem to result in less con-
seems relatively late. Nowadays, most pediatric colorectal centers stipation, but more incontinence than PSARP [29]. Nevertheless, even
with the currently used operational techniques, the complete functional
colorectal prognosis (voluntary bowel movement, constipation, soiling
and continence) for patients with an RVF compared to the overall
Table 4
Long-term urological and renal outcomes (after 5 years) in patients with an ARM and ARM-patients still remains poor [1], as this study underlines. Since sa-
rectovesical fistula. cral anomalies are found in 67% of the patients, including sacral agenesis
in 17%, it remains unknown to what extent the poor innervation of the
N = 16 (%)
pelvic floor contributes to the incontinence.
Spontaneous voiding 9 (56) Although constipation is generally considered a common problem in
Continence (dry for urine) 13 (81)
Clean intermittent catheterization 4 (25)
lower type and less complex ARM (e.g. perineal fistulas) [2], it has been
Awaiting continence 7 (44) described as a functional complication after PSARP procedures for “high
Recurrent urinary tract infections 6 (38) malformations” as well [1,8,30]. In the present study, a large proportion
End-stage kidney failure 0 (0) of patients suffered from constipation at both five and ten years follow-
Urological interventions
up. The etiology of this constipation is unclear, particularly considering
Vesicostomy 1 (6)
Bladder augmentation 1 (6) poor pelvic muscle quality with poor sphincter complex function, which
Ureteral reimplantation 3 (19) usually leads to incontinence rather than constipation. Possibly the sur-
Urinary diversion 1 (6) gical mobilization of the rectum or an (congenital) impairment of the
Pyeloplasty (multiple) 1 (6) distal colon and rectum (rectosigmoid hypomotility and generalized co-
Nephrectomy 2 (13)
lonic motility disturbances) explains the high prevalence of

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1232 H.J.J. van der Steeg et al. / Journal of Pediatric Surgery 51 (2016) 1229–1233
constipation in these patients [31]. Additionally, preserving the distal
fistulous termination as the neo-anus with histopathological, architec-
tural abnormalities might lead to constipation [9]. Recent data, howev-
er, suggest that constipation experienced during childhood, improves
over time [28,32].
Vesico-ureteral reflux was encountered at or shortly after birth in
39% of the patients. Treatment and follow-up regimes have not been re-
ported reliably in our data. In general, as was published recently by one
of the involved centers of this study, 60% of patients with a complex
ARM receive prophylactic antibiotics [33]. In our study, 43% of VUR-
patients had ureter-reimplantation performed, indicating the need for
a continuous urological follow-up in these patients. Because of the
rectovesical fistula and possible urethral stenosis, combined with the
extensive surgery in the bladder neck, voiding problems are expected
in this type of anorectal malformation patients. However, a high per-
centage of patients in this study voids spontaneously, and supported
by clean intermittent catheterization (CIC), the majority is continent. Al-
though more objective urological data (e.g. urodynamic data) specifical-
ly on rectovesical fistula is lacking, our data correspond well with the
study by Versteegh et al. in cloacal malformation patients, in which
69% of the patients had spontaneous voiding and 76% was continent
for urine [34].
Chronic renal failure is a devastating complication of ARM associated
with urological comorbidity. However, it is rarely encountered in pa-
tients with ARM, even in the most complex malformations. This study
did not show any patients with end-stage renal failure and consequent
renal transplantation, although follow-up time was limited to 5 years. A
previous study showed an incidence of 0.8%, although this was reported
in all ARM-patients combined [35]. In the female most complex ARM,
cloacal malformations, a transplantation incidence of 7% was reported
in The Netherlands [34]. Recurrent urinary tract infections (UTI) were
described to be a risk factor for developing chronic renal insufficiency
[34,35]. In our study, 38% suffered from recurrent UTI, which is compa-
rable to 31% in recto-urethral fistula type ARM [36], but lower than the
53% in cloacal malformation patients [34]. Numbers are, however, too
small to draw any firm conclusions.
Currently, no standard monitoring of patients with a rectovesical fis-
tula for the presence of UTI is available. In children with neurogenic
bladder a monitoring program for the presence of UTI has proven valu-
able [37]. It can be concluded that scheduled urological follow-up in this
subgroup of patients should be developed and evaluated.
The major limitation of this study is its retrospective design giving
bias to the collection of data. Another limitation is the large timeframe
(32 years) in which the patients were treated. Although 83% was even-
tually treated with a PSARP, other treatment regimes evolved over time,
including neonatal, pediatric and surgical perioperative care, which all
may influence the reported results.
In conclusion, the results of this multi-center study over a 32 year
period support the notion that the outcomes in ARM-patients with a
rectovesical fistula are less optimistic than in other types of urethral fis-
tula in males. Although the majority of patients have had a PSARP as a
“modern” definitive repair, this study shows that, nevertheless, after
10 years follow-up, all patients are dependent on bowel management.
This leads us to recommend early institution of bowel management,
possibly as early as the start of elementary school (4–5 years of age).
This will alleviate the social burden of incontinence and soiling, and pos-
sibly allow for earlier adequate management of this severe type of ARM.
Additionally, an early understanding by the parents and patient of the
poor prognosis of continence, particularly in the presence of major sa-
cral anomalies, will prevent unnecessary frustration and negative influ-
ence on quality of life because of unsuccessful efforts in achieving
continence.
Regarding urological outcome, most patients are dry for urine,
and although end-stage kidney failure is rare, regular urological
follow-up is recommended to prevent this and its subsequent need of
kidney transplantation.
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