Outcome in anorectal malformation type rectovesical fistula: A nationwide cohort study in the Netherlands H.J.J. van der Steeg, S.M.B.I. Botden, C.E.J. Sloots, A.F.W. van der Steeg, P.M.A. Broens, L.W.E. van Heurn, D.V. Travassos, I.A.L.M. van Rooij, I. de Blaauw PII: DOI: Reference:

S0022-3468(16)00065-8 doi: 10.1016/j.jpedsurg.2016.02.002 YJPSU 57541

To appear in:

Journal of Pediatric Surgery

Received date: Revised date: Accepted date:

28 October 2015 2 February 2016 2 February 2016

Please cite this article as: van der Steeg HJJ, Botden SMBI, Sloots CEJ, van der Steeg AFW, Broens PMA, van Heurn LWE, Travassos DV, van Rooij IALM, de Blaauw I, Outcome in anorectal malformation type rectovesical fistula: A nationwide cohort study in the Netherlands, Journal of Pediatric Surgery (2016), doi: 10.1016/j.jpedsurg.2016.02.002

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ACCEPTED MANUSCRIPT Outcome in anorectal malformation type rectovesical fistula: A nationwide cohort study in the Netherlands

van Heurn3,5, D.V. Travassos6, I.A.L.M. van Rooij7, I. de Blaauw1

of Surgery-Pediatric Surgery, Radboudumc-Amalia Children’s Hospital, Nijmegen, The

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1Department

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Netherlands 2Department

of Pediatric Surgery, Erasmus-MC Sophia Children’s Hospital, Rotterdam, The

Netherlands

of Pediatric Surgery, Emma Children's Hospital, AMC and VU University Medical Center,

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3Department

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H.J.J. van der Steeg1, S.M.B.I. Botden1, C.E.J. Sloots2, A.F.W. van der Steeg3 , P.M.A. Broens4, L.W.E.

Amsterdam,

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The Netherlands

of Pediatric Surgery, University Medical Center, Groningen, The Netherlands

5Department

of Pediatric Surgery, Maastricht University Medical Center, Maastricht, The Netherlands

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4Department

6Department

of Pediatric Surgery, Wilhelmina Children’s Hospital, University Medical Center,

Utrecht, The Netherlands 7Department

for Health Evidence, Radboud Institute for Health Sciences, Radboud university medical

center, Nijmegen, The Netherlands Corresponding author H.J.J. van der Steeg Radboud University Medical Center Nijmegen, the Netherlands

ACCEPTED MANUSCRIPT Department of pediatric surgery [email protected]

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tel. +31 24 3619761

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fax: +31 24 3613547

ACCEPTED MANUSCRIPT Abstract Purpose

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Outcomes of patients with an ARM type rectovesical fistula are scarcely reported in medical literature. This study evaluates associated congenital anomalies and long-term colorectal and

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urological outcome in this group of ARM-patients.

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Methods

A retrospective Dutch cohort study on patients treated between 1983 and 2014 was performed. Associated congenital anomalies were documented, and colorectal and urological outcome recorded

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at five and ten years of follow-up. Results

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Eighteen patients were included, with a mean follow-up of 10,8 years. Associated congenital

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anomalies were observed in 89% of the patients, 61% considered a VACTERL association. Total sacral

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agenesis was present in 17% of our patients. At five and ten years follow-up voluntary bowel movements were described in 80% and 50%, constipation in 80% and 87%, and soiling in 42% and 63% of the patients, respectively. Bowel management 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

ACCEPTED MANUSCRIPT 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

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achieved in the majority of patients, and end-stage kidney failure is rare. Keywords:

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Anorectal malformation (ARM) - rectovesical fistula - bladder neck fistula - colorectal outcome -

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urological outcome - long-term follow up

ACCEPTED MANUSCRIPT Introduction Anorectal malformations (ARM) represent a complex group of congenital anatomical anomalies of the anorectum, characterized by the absence of a normal anus at its anatomic position central in the

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sphincter complex [1,2]. The estimated prevalence ranges between 1:2000-5000 live births [2,3].

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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

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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].

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A rectovesical or bladder neck fistula is an anorectal malformation, 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 contradictory [8,9]. This type of anorectal malformation is a severe type of ARM occurring exclusively in males, in approximately 10% of the patients [10]. It is further associated with hypoplasia of the buttocks and external sphincter complex,

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contributing to the poor functional outcome for these patients. Data on patients with a rectovesical fistula (RVF) are typically reported as part of larger cohorts of the full spectrum, or of ‘high’

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malformations, which also include recto-urethral-prostatic fistulas [2,11-13]. Until now, ARMpatients with a rectovesical fistula have not been described before as a single cohort. Therefore, a

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retrospective nation-wide study was conducted to evaluate associated anomalies and long-term

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functional colorectal and urological outcome for this rare group of patients.

ACCEPTED MANUSCRIPT Patients and Methods

A retrospective cohort study was performed in the six pediatric surgical centers in the Netherlands.

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Charts of all patients with an ARM type rectovesical fistula treated between 1983 and 2014 were

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reviewed and data on associated congenital anomalies, type of surgery and its timing and complications were collected. Concerning the work-up on associated (VACTERL) anomalies, all patients in the Netherlands are screened for the presence of tethered cord by ultrasound in the first

nowadays performed within the first year of life.

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week of life [14]. In case of presence of a tethered cord or doubt on its presence, a spinal MRI is

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For the patients with a minimum follow up period of 48 months, the long-term outcome was assessed, considering the fact that in the general 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, respectively, and was graded on the Krickenbeck scoring scale for obstipation and soiling [16].

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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 due to loss to follow-up or discharge from follow-

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up, and therefore not analyzed.

The urological and renal function outcomes (spontaneous voiding, continence, clean intermittent

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catheterization, urinary diversions or other urological surgery, recurrent urinary tract infections, end-

Statistics

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stage kidney failure) after 5 years were also documented in this study.

Frequencies of nominal variables are presented including percentages. 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 is provided. Numbers are too small to perform statistical significant tests.

ACCEPTED MANUSCRIPT Results

Eighteen patients were identified and included. Mean follow up was 10,8 years (SD: 5 years, range: 2-

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19 years). Table 1 shows the associated congenital anomalies observed in 16 patients (89%). Eleven

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patients met the criteria of a VACTERL association (61%). Most prevalent associated anomalies were sacral vertebral in 12 patients (67%), being hemivertebrae, sacral fusion, partial sacral agenesis and coccygeal agenesis. Three patients had a total sacral agenesis. No patient was diagnosed with

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Currarino-syndrome. Anatomical kidney anomalies were present in 11 patients (61%). Of this group there were three patients with a single kidney, three presented with a horseshoe kidney, two had

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ureteropelvic junction (UPJ) stenosis, one dysplastic kidney, another a dysfunctional kidney and one had multicystic kidney disease.

All patients needed a colostomy in the neonatal period, with a complication 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.

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In the majority of patients (83%), reconstructive surgery was performed using a posterior sagittal anorectoplasty (PSARP). For the abdominal phase a laparotomy was performed in 10 and a

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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

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last patients eventually had a definitive colostomy. In two patients the data on the surgical treatment were missing. The median age at reconstructive surgery was six months (range: 2-26 months). To

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prevent anal stenosis, anal dilatations according to the Pena guidelines were standard post-operative care in all pediatric surgical centres [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, respectively. Twelve patients (80%) had voluntary bowel movements after five years and five patients (50%) after ten years.

ACCEPTED MANUSCRIPT 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 socially disabling, was

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present in 17% and 13% of the patients after five and ten years respectively.

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After ten years follow-up, bowel management was needed in 90% of the patients (9 out of 10) for the treatment of incontinence and constipation. 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

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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

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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-ureteral reflux (VUR) at or shortly after birth (2 Grade I, 2 Grade II, 2 Grade III and 1 Grade IV). The 3 patients with a VUR Grade III-IV all had ureterreimplantation (43% of all VUR-patients). No reliable data on possible prophylactic antibiotics could be collected after 5 years. Nine patients (56%) voided spontaneously. Thirteen patients (81%) were

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considered continent for urine either by spontaneous voiding or clean intermittent catheterization (CIC). Five patients (28%) needed a total of nine additional urological surgical procedures. Six patients

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(38%) suffered from recurrent urinary tract infections. Of these six, all had anatomical renal anomalies (two horseshoe kidney, two UPJ-stenosis, one dysplastic kidney and one single kidney),

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and two had VUR (both Grade III). No end-stage renal failure, represented by kidney transplantation,

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was seen in any of the patients during the follow up.

ACCEPTED MANUSCRIPT Discussion Anorectal malformation with rectovesical fistula is the most complex and therefore the most challenging colorectal anatomical anomaly in males. To date, this nationwide study is the largest to

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report the outcome of this specific group of patients.

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Associated congenital anomalies were reported in 89% of the patients in our series. Other studies in patients with ARM show associated anomalies in 40-70% of the patients, increasing with the complexity of the ARM to 80% [2,4-7,18-20]. The VACTERL-association was present in 61% of the

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patients, which is relatively high compared to the mentioned 15-37% in previous studies [5,7,21]. Additionally, sacral anomalies were present in the majority of cases, including 17% sacral agenesis.

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Thus, this cohort confirms the high prevalence of associated congenital anomalies in patients with ARM with a rectovesical fistula, including those anomalies that have a negative effect on colorectal and urological function.

The median age at reconstructive surgery was six months which seems relatively late. Nowadays, most pediatric colorectal centers perform definitive surgery for all ARM at the age of 2-3 months. As this is a multicenter and retrospective study, it is unclear whether this delay was because of

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associated anomalies that needed prior attention, or if this actually reflects the practice of the individual center/surgeon involved in the most complex ARM in males.

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Voluntary bowel movements were reported in 80% after five years follow-up and 50% after ten years. As a single variable voluntary bowel movement does not imply continence. In our group after a

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follow-up of 10 years, all patients but one, having a definitive colostomy, were fecally incontinent despite having voluntary bowel movements.

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Incontinence and soiling are considered to be the major functional 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 shows that the majority (87%) of the patients with a RVF also suffer from constipation and possible (overflow) incontinence. Constipation and incontinence are thus co-existing in these patients and difficult to differentiate, as there is considerable overlap. Bowel management is an essential tool to realize social (pseudo)continence, as it treats both constipation and (overflow)incontinence simultaneously. Another explanation for the good outcome in soiling, may be that the definition of grade 2 in the Krickenbeck classification (daily soiling without social problems) is easily mistaken for grade 3 soiling due to poor validation of the scoring system and the retrospective nature of our study. Significant soiling was reported in 30-56% of the patients in other studies [1,22,23], although in these series the results on functional outcome were contradictory and therefore the results on soiling should be interpreted with caution.

ACCEPTED MANUSCRIPT Results considering fecal continence in literature vary considerably. Peña [1] reported that approximately one-third of the patients with high or intermediate anomalies operated on with a PSARP in his series could be considered totally continent. Earlier series of Taylor [24] and Rintala [25]

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showed worse outcome of a mere 7.5% of the patients with high malformations having full bowel

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control. Surgical reconstructive techniques varied between studies, as these patients were primarily operated on with a (sacro)abdominoperineal (AP) approach, and therefore data are not completely comparable. It is reported that fecal incontinence can improve with increasing age [25,26]. It is

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unclear whether this improvement is an actual functional improvement of sphincteric function, or a gradual adjustment to residual dysfunction [27]. Our study clearly did not show any significant

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improvement in fecal continence with increasing age, but follow-up was till 10 years of age. It is interesting to know if and how continence develops in puberty and young adulthood. In recent data, PSARP is reported to give better short-term [26,27], as well as long-term results [28,29] regarding continence, than the prior used AP approaches. Overall, AP procedures seem to result in less constipation, but more incontinence than PSARP [29]. Nevertheless, even with the currently used operational techniques, the complete functional colorectal prognosis (voluntary

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bowel movement, constipation, soiling and continence) for patients with a RVF compared to the overall ARM-patients still remains poor [1], as this study underlines. Since sacral anomalies are found

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in 67% of the patients, and sacral agenesis in 17%, it remains unknown to what extent the poor innervation of the pelvic floor contributes to the incontinence.

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Although constipation is generally considered a common problem in lower type and less complex ARM (e.g. perineal fistulas) [2], it has been described as a functional complication after PSARP

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procedures for “high malformations” as well [1,8,30]. In the present study, a large proportion of patients suffered from constipation at both five and ten years follow-up. The etiology of this constipation is unclear, particularly considering poor pelvic muscle quality with poor sphincter complex function, which usually leads to incontinence rather than constipation. Possibly the surgical mobilization of the rectum or an (congenital) impairment of the distal colon and rectum (rectosigmoid hypomotility and generalized colonic motility disturbances) explains the high prevalence of constipation in these patients [31]. Additionally, preserving the distal fistulous termination as the neo-anus with histopathological, architectural abnormalities might lead to constipation [9]. Recent data, however, 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 reported 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

ACCEPTED MANUSCRIPT 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.

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However, a high percentage of patients in this study voids spontaneously, and supported by clean

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intermittent catheterization (CIC), the majority is continent. Although more objective urological data (e.g. urodynamic data) specifically 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

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spontaneous voiding and 76% was continent for urine [34].

Chronic renal failure is a devastating complication of ARM associated with urological comorbidity.

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However, it is rarely encountered in patients 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

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developing chronic renal insufficiency [34,35]. In our study, 38% suffered from recurrent UTI, which is comparable to 31% in recto-urethral fistula type ARM [36], but lower than the 53% in cloacal

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malformation group [34]. Numbers are, however, too small to draw any firm conclusions. Currently, no standard monitoring of patients with a rectovesical fistula for the presence of UTI is

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available. In children with neurogenic bladder a monitoring program for the presence of UTI has proven valuable [37]. It can be concluded that scheduled urological follow up in this subgroup of

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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 eventually 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 fistula 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 possibly allow for earlier adequate management of

ACCEPTED MANUSCRIPT 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 sacral anomalies, will prevent unnecessary frustration and negative influence on quality of life due to unsuccessful efforts in

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achieving continence.

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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

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kidney-transplantation.

ACCEPTED MANUSCRIPT References 1. Peña A. Anorectal malformations. Semin Pediatr Surg 1995;4:35-47

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2. Levitt MA, Peña A. Anorectal malformations. Orphanet J Rare Dis 2007;2:33. Review. 3. International Clearing House for Birth Defects Surveillance and Research. Annual report

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2011: with data for 2009.

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http://www.icbdsr.org/filebank/documents/ar2005/Report2011.pdf 4. Hassink EA, Rieu PN, Hamel BC et al. Additional congenital defects in anorectal malformations. Eur J Pediatr 1996;155:477-82

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5. Cuschieri A, EUROCAT Working Group. Anorectal anomalies associated with or as part of other anomalies. Am J Med Genet 2002;110:122-130

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6. Stoll C, Alembik Y, Dott B, Roth MP. Associated malformations in patients with anorectal

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anomalies. Eur J Med Genet 2007; 50:281-90

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7. Moore SW. Associations of anorectal malformations and related syndromes. Pediatr Surg Int 2013;29:665-676

8. Rintala R, Lindahl H, Marttinen E, Sariola H. Constipation is a major functional complication after internal sphincter-saving posterior sagittal anorectplasty for high and intermediate anorectal malformations. J Ped Surg 1993;8:1054-1058 9. Lombardi L, Bruder E, Caravaggi F et al. Abnormalities in “low” anorectal malformations (ARMs) and functional results resecting the distal 3 cm. J Ped Surg 2013;48:1294-1300 10. Levitt MA, Peña A. Operative management of anomalies in males. In: Holschneider AM, Hutson JM (eds.) Anorectal malformations in children, Springer-Verlag, New York; 2006, p.

ACCEPTED MANUSCRIPT 295-302 11. Bischoff A, Peña A, Levitt MA. Laparoscopic-assisted PSARP - the advantages of combining

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both techniques for the treatment of anorectal malformations with recto-bladderneck or high prostatic fistulas. J Ped Surg 2013;48:367-371

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12. Shawyer AC, Livingston MH, Cook DJ, Braga LH. Laparoscopic versus open repair of rectobladderneck

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and recto-prostatic anorectal malformations: a systematic review and metaanalysis. Ped Surg Int 2015;31:17-30

13. Tong Q-S, Tang S-T, Mao Y-Z et al. Laparoscopically assisted anorectal pull-through for high

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imperforate anus in infants: intermediate results. J Ped Surg 2011;46:1578-1586

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14. Van Heurn E, de Blaauw I, Heij H et al. Quality measurement in neonatal surgical disorders:

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development of clinical indicators. Eur J Pediatr Surg 2015;25:526-531 15. Burgers R, Benninga MA. Functional nonretentive fecal incontinence in children: a frustrating

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and long-lasting clinical entity. J Pediatr Gastroenterol Nutr 2009;48 Suppl 2:S98-S100 16. Holschneider A, Hutson J, Peña A et al. Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Ped Surg 2005;40(10):1521-1526 17. Midrio P, Mosiello G, Ausili E et al. Peristeen® Trans Anal Irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study. Colorectal Dis. 2016;18(1):86-93. 18. Cuschieri A, EUROCAT Working Group. Descriptive epidemiology of isolated anal anomalies: a

ACCEPTED MANUSCRIPT survey of 4.6 million births in Europe. Am J Med Genet 2001;103:207-215 19. Boocock GR, Donnai D. Anorectal malformation: familial aspects and associated anomalies.

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Arch Dis Child 1987;62:576-579 20. Metts JC, Kotkin L, Kasper S et al. Genital malformations and coexistent urinary tract or spinal

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anomalies in patients with imperforate anus. J Urol 1997;158:1298-1300

Indian J Pedaitr 2004;71:509-514

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21. Mittal A, Airon RK, Magu S et al. Associated anomalies with anorectal malformation (ARM).

22. Rintala R, Lindahl H. Is normal bowel function possible after repair of intermediate and high

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anorectal malformations? J Ped Surg 1995;30:491-494

Surg 1991;26:587-590

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23. Langemeijer RATM, Molenaar JC. Continence after posterior sagittal anorectoplasty. J Ped

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24. Taylor I, Duthie HL, Zachary RB. Anal continence following surgery for imperforate anus. J Ped

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Surg 1973;8:497-503

25. Rintala R, Lindahl H, Louhimo I. Anorectal malformations - results of treatment and long term follow-up of 208 patients. Pediatr Surg Int 1991;6:36-41 26. Kyrklund K, Pakarinen MP, Koivusalo A, Rintala RJ. Long-term bowel functional outcomes in recturethral fistula treated with PSARP: controlled results after 4-29 years of follow-up. A single-institution, cross-sectional study. J Ped Surg 2014;49:1635-1642 27. Rintala RJ, Pakarinen MP. Imperforate anus: long- and short-term outcome. Semin Pediatr Surg 2008;17:79-89 28. Danielson J, Karlbom U, Graf W et al. Posterior sagittal anorectoplasty results in better bowel

ACCEPTED MANUSCRIPT function and quality of life in adulthood than pull-through procedures. J Ped Surg 2015;50:1556-1559

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29. Rintala RJ, Lindahl HG. PSARP is superior to sacroperineal-sacroabdominoperineal pullthrough: a long term follow-up study in males with anorectal anomalies. J Ped Surg

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1999;34(2):334-337

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30. Holschneider AM, Pfrommer W, Gerresheim B. Results in the treatment of anorectal malformations with special regard to the histology of the rectal pouch. Eur J Ped Surg 1994;4:303-309

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31. Rintala RJ, Marttinen E, Virkola K et al. Segmental colonic motility in patients with anorectal malformations. J Ped Surg 1997;32:453-456

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32. Rintala RJ, Lindahl HG. Fecal continence in patients having undergone PSARP procedures for

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high anorectal malformations improves at adolescence as constipation disappears. J Ped Surg

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2001;36:1218-1221

33. Goossens WJH, de Blaauw I, Wijnen MH et al. Urological anomalies in anorectal malformations in The Netherlands: effects of screening all patients on long-term outcome. Pediatr Surg Int 2011;27:1091-1097 34. Versteegh HP, Sloots CE, Wolffenbuttel KP et al. Urogenital function after cloacal reconstruction, two techniques evaluated. J Pediatr Urol 2014;10:1160-1164 35. Ganesan I, Rajah S. Urological anomalies and chronic kidney disease in children with anorectal malformations. Pediatr Nephrol 2012;27:1125-1130

ACCEPTED MANUSCRIPT Table 1. Associated congenital anomalies in 18 patients with ARM and a rectovesical fistula. N (%) (28) (6) (22) (67) (11) (61) (11)

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5 1 4 12 2 11 2

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Cardiac Oesophageal Spinal cord Sacral Vertebral* Renal Limb * 1 missing value

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Type of anomaly

ACCEPTED MANUSCRIPT Table 2. Complications after reconstructive surgery

anorectal anal stenosis

10 4

anal prolaps mechanical bowel obstruction megacolon stenosis of colostomy

2 2 1 1

urologic urinary tract infection urinary retention

2 1 1

TOTAL complications

12

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1 redo-PSARP, 3 repetitive anal dilatations under anesthesia unknown unknown twice plications in a single patient unknown

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* patients can have multiple complications ** N is number of patients

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Surgical intervention

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N = 17** 7 (41%)

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Complication* none

ACCEPTED MANUSCRIPT Table 3. Long-term functional colorectal outcome of patients with ARM and a rectovesical fistula.

10 years (N=10)

no 1 2 3

7 (out of 12) 2 1 2

(58%) (17%) (8%) (17%)

3 (out of 8) 1 3 1

Constipation (grade) no 1 2 3

3 (out of 15) 0 5 7

(20%) (0%) (33%) (47%)

13 (out of 15) (79%) 10 (out of 15) (67%)

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Bowel management Laxative treatment

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Soiling (grade)

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5 years (N=15)

1 (out of 8) 1 2 4

(37%) (13%) (37%) (13%) (13%) (13%) (25%) (50%)

9 (out of 10) (90%) 5 (out of 10) (50%)

ACCEPTED MANUSCRIPT Table 4. Long-term urological and renal outcomes (after 5 years) in patients with an ARM and rectovesical fistula.

Urological interventions Vesicostomy Bladder augmentation Ureteral reimplantation Urinary diversion Pyeloplasty (multiple) Nephrectomy

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1 (6) 1 (6) 3 (19) 1 (6) 1 (6) 2 (13)

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9 (56) 13 (81) 4 (25) 7 (44) 6 (38) 0 (0)

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Spontaneous voiding Continence (dry for urine) Clean intermittent catheterization Awaiting continence Recurrent urinary tract infections End-stage kidney failure

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N = 16 (%)