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Journal of Pediatric Urology (2015) xx, 1.e1e1.e7
27 years of experience with the comprehensive surgical treatment of prune belly syndrome R.I. Lopes, A. Tavares, M. Srougi, F.T. De ´nes Pediatric Urology Unit, Division of Urology, Hospital das Clı´nicas, University of Sa ˜o Paulo School of Medicine, Sa ˜o Paulo, Brazil Correspondence to: Rua Dr. Eneas de Carvalho Aguiar 255 7 andar, Division of Urology, Hospital das Clinicas da FMUSP, Sa ˜o Paulo CEP 05403000, Brazil, Tel.: þ55 11 2661 8080, þ55 11 99626 8974 (mobile) [email protected] (R.I. Lopes) [email protected] (A. Tavares) [email protected] (M. Srougi) [email protected] (F.T. De´nes) Keywords Prune belly syndrome; Abdominal wall defect; Urinary tract reconstruction Received 23 December 2014 Accepted 6 May 2015 Available online xxx
Summary Introduction Prune belly syndrome (PBS) presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism. As a result, urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. Objectives To report experiences with comprehensive surgical management of prune belly syndrome. Material and methods From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. According to individual needs, treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract, and perform bilateral orchiopexy and circumcision, which were performed in one procedure. Urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation with evidence of significant stasis and/or vesicoureteral reflux was associated with recurrent urinary tract infections (UTI). Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions. The median age at surgery was 16 months and children were followed for a median of 143 months.
Results Abdominal appearance and tonus were improved in 90% of the children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children and long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Lower urinary tract reconstruction was performed in most children (95.6%); on late follow-up, continence was observed in 81% of them, while incontinence was present in 19% and usually associated with polyuria. Adequate bladder emptying was possible in most boys (82.6%), while the remaining required clean intermittent catheterization. Pre-operative UTI was present in 89.1% and urinary sepsis in 15.2%. Postoperatively, the incidence of laboratorial UTI was significantly reduced to 39.1%, while urinary sepsis was absent. Bilateral orchiopexy was performed in all children, with 85% of the testes becoming normal in size and well located in the scrotum.
Conclusions Comprehensive surgical treatment is feasible and has good long-term results. A considerable incidence of reoperations due to complications or progression of the disease was observed. The long-term results for reno-ureteral anatomy and function, bladder function, infection, testicular size and location, as well as abdominal aspect and tonus, show that comprehensive surgery is an adequate method for managing children with PBS.
http://dx.doi.org/10.1016/j.jpurol.2015.05.018 1477-5131/ª 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Introduction Prune belly syndrome (PBS) has an incidence of 1:35,000 to 1:50,000 live births and mainly occurs in boys. It presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism [1e5]. Its characteristic, but variable, abdominal flaccidity may cause physical limitations and compromised self-image for these children. The variable anomalies of the urinary tract, which are not always proportional to the abdominal flaccidity, affect the kidneys and bladder in a non-uniform fashion [1e6]. Renal dysplasia, ureterohydronephrosis, megaureter, VUR, and bladder dysfunction are associated to recurrent UTI, which when untreated or not prevented further impair renal function in most children [1e6]. Intraabdominal cryptorchidism is uniformly associated with infertility in these children [1e7]. Management of these children is controversial. Urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. Non-operative treatment of the urinary tract anomalies and the abdominal wall has been proposed, assuming that renal function will stabilise with prevention of UTI and the spontaneous improvement of the reno-ureteral anatomy [8,9]. Some studies have recommended urinary tract reconstruction (UTR) only in cases of confirmed obstruction and/or untreatable UTI [1,8,9]. This approach requires close surveillance and the long-term results have not been homogeneous [1,8,9]. Other studies have advocated UTR whenever stasis, VUR or UTI are present because these common factors put children with PBS at risk of progressive renal deterioration [10,11]. Because long-term continuous surveillance of these children is difficult in Brazil, due to social and geographical conditions, comprehensive surgical management of these children with PBS is preferred [6]. The aim of this study was to present the long-term results of this approach. It is believed that, to date, this experience is one of the largest reported in the literature.
Patient and methods At initial presentation, all children with PBS underwent clinical evaluation followed by functional evaluations with ultrasonography or intravenous urography, as well as radioisotopic renography. VCUG was performed to assess the presence of urethral athresia and megalourethra in cases with severe UTI, marked ureteral dilatation or in those who pre-operatively presented with abnormal voiding patterns or urachal fistula [12e14]. Urodynamic evaluation was only performed for older children who could not spontaneously void and/or had episodes of urinary retention. Renal function was evaluated by blood urea and creatinine levels. Urinalysis and uroculture were performed in all children. From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. Children were classified according to disease severity using the Woodard
R.I. Lopes et al. scale [1], which classifies children in three groups. All groups have the characteristic, but variable, abdominal flaccidity. In Group 1, significant renal dysplasia and pulmonary hypoplasia are present, and the children have a high rate of perinatal mortality resulting from pulmonary and/or renal insufficiency. In Group 2, despite the significant anatomical and functional abnormalities of the urinary tract, renal function is initially stable, but may progress to renal insufficiency due to recurrent obstruction or infection. In Group 3, mild urinary tract abnormalities may be present and most children present with stable renal and pulmonary function, with high survival rates without intervention. Forty children of the cohort were classified as Woodard Grade 2, while the remaining six had Grade 3 disease. The median age at surgery was 16 months (25 dayse10 years) and children were followed for a median of 143 months (15 monthse26 years). Two of the children had loop ureterostomies and one had a vesicostomy performed elsewhere, while three others had patent urachuses draining the bladder. Some children were performing CIC, while others were referred with bladder catheters. In one patient with malnutrition and a single functioning kidney associated with a severely dilated ureter and recurrent UTIs, a terminal loop ureterostomy was performed before UTR in order to improve general conditions. According to individual needs, surgical treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract and orchiopexy, preferably in one procedure. Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions.
Abdominoplasty and laparotomy Forty-four of the 46 children received abdominal wall reconstruction. The timing of this procedure was dictated by the need for other surgical procedures, particularly orchiopexy and urinary tract reconstruction. Three children were initially submitted to Erlich’s technique and two to Randolph’s repair [1], but in all 39 subsequent children, abdominoplasty was performed with the modified technique, which has previously been described [15].
Upper urinary tract reconstruction Upper urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation was associated with significant stasis or obstruction, as well as to VUR, recurrent UTI and scarring of the ipsilateral kidney (Fig. 1A). Significant stasis or obstruction was considered when progressive dilatation with worsening renal or differential renal function was observed. Reconstruction of the upper urinary tract [16] required either unilateral or bilateral distal ureterectomy and ureterocystoneostomy (Fig. 1B and C). Non-functioning units due to dysplasia or hydronephrosis were submitted to nephroureterectomy. Distal ureterectomy with ureterocystoneostomy was performed due to redundancy or dilatation, as well as VUR. Uretero-
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Figure 1 A: Pre-operative marked bilateral ureteral dilatation; B: After resection of distal ureters, proximal ureters are cannulated; C: Surgical specimens consisting of extremely enlarged distal ureters and bladder dome; D: Pre-operative excretory urography demonstrating marked bilateral dilation of ureters, with distal ureter redundancy; E: After urinary tract reconstruction, the postoperative improvement of ureteral dilation is evident. Source: authors’ own photos.
vesical reimplantation was usually performed with the Paquin technique, associated with a psoas hitch when performed unilaterally. When required, ureteral tailoring or infolding, as well as renal mobilization, were performed to ensure optimal ureteroneocystostomy. Ureteral stents were left indwelling and attached to a Foley urethral catheter or percutaneously exteriorised through the bladder and abdominal wall. Previous ureterostomies were closed or removed with the distal ureter. In four cases of associated UPJ obstruction, non-dismembered side-to-side pyeloureteral anastomoses were performed.
Lower urinary tract reconstruction At laparotomy, the bladder was detached from the abdominal wall, sectioning the urachal remnants. An individually tailored cystoplasty was performed with removal of the urachal diverticulum or fistula and part of the bladder dome, particularly in cases with significantly enlarged bladders [4,17,18]. A reduction cystoplasty was indicated for the resection of a urachal diverticulum or for excessively large bladders, as described by Woodard. Overresection of the bladder was avoided, ensuring adequate capacity after closure. Primary appendiceal Mitrofanoff
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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1.e4 channels were made in children that required continuous or intermittent bladder drainage before surgery, one of them had scaphoid megalourethra [19]. All parents were advised on the importance of regular bladder emptying and many were trained with Valsalva and Crede ´ manoeuvres to adequately empty their children’s bladders.
Orchiopexy Cryptorchid testes are usually found high in the abdomen, lying above the enlarged ureteral folds. As both the right and left colonic segments lack fixation to the abdominal wall, their retraction easily exposes the testes in the retroperitoneum. They are dissected and separated from the ureter, with preservation of the gonadal vessels, vas deferens and its vessels, and, if possible, gubernaculum. When adequate vessel length is obtained, the inguinal fascia is incised on both sides and the testes are brought down to the scrotum and fixed in subdartic pouches, completing the orchiopexy. If the testes are distant from the inguinal region and the spermatic vessels are too short, these are sectioned; the still intact paradeferential and gubernacular vessels ensure gonadal vascularisation [7]. All 46 children underwent bilateral orchiopexy. In 31 children, adequate testicular repositioning was achieved without the need to transect the vessels; while in 15 children, the vessels were sectioned either unilaterally (in seven) or bilaterally (in eight).
R.I. Lopes et al.
Upper urinary tract reconstruction All pre-operative UTI were symptomatic. All children were on prophylactic antibiotics pre-operatively. Postoperatively, children received antibiotic prophylaxis whenever they were submitted to UTR, usually for a period of 3 months. After this period, they only received antibiotics temporarily when complications such as UTI or obstruction were observed. In the study cohort, pre-operative UTI was present in 41 children (89.1%) and urinary sepsis in seven (15.2%). Postoperatively, the incidence of UTI was significantly reduced to 39.1% (18/46); most of them were asymptomatic, while urinary sepsis was absent. The McNemar test was performed to compare pre- and postoperative data regarding UTI and sepsis. There was a statistically significant decrease in UTI rate (P < 0.001/X2 Z 19.36) and sepsis (P Z 0.0233/X2 Z 5.193) postoperatively. As seen in Table 1, bilateral distal ureterectomy followed by bilateral ureteral reimplantation was performed in 23 children and unilateral distal ureterectomy followed by ureterocystoneostomy was performed in 16 children. Unilateral nephroureterectomy alone was performed in one child. Straightforward ureterocystoneostomy was performed after distal ureterectomy in 24 units, but infolding and tailoring in the proximal ureter were required for reimplantation in 38 units. A total of 62 renal units were treated in 40 children. In the late follow-up, the upper urinary tract showed significant anatomical improvement in 44 renal units of 24
Circumcision Circumcision was routinely performed in the latter 36 children at the end of the procedure, also to reduce the risk of UTI.
Results Clinical and radiological work-up was used to compare preoperative and postoperative status of the cohort and to determine treatment success or failure. Polyuria was defined as a urine output of >5 ml/kg/hour and/or hypoosmolar urine (urinary density of 1010).
Abdominoplasty and laparotomy
Table 1 Procedures performed for upper urinary tract reconstruction. Procedures
Number of patients
Bilateral UCN Bilateral UCN þ bilat. PP Bilateral UCN þ unilat. UUA Sub-total Unilateral UCN þ contralat. NU Unilateral UCN þ ipsilat. PP þ contralat. NU Unilateral UCN þ contralat. PP Unilateral UCN Sub-total Unilateral NU Total
21 1 1 23 pts. (46 units) UCN 10 2 1 3 16 pts. (16 units) UCN 1 40 pts. (62 units) UCN
Ureterocystoneostomy
The medical staff and parents subjectively evaluated abdominal flaccidity and posture. Abdominal tonus was determined by abdominal observation and palpation in the horizontal decubitus, sitting up and in the upright position. For 40 children, the abdominal appearance and significantly improved after the procedure (90%), while four (10%) had unsatisfactory results, either with persistent or recurrent flaccidity. All required secondary abdominoplasties and had a good outcome. It is noteworthy that three children had late, acute abdomen due to intestinal obstruction that required exploratory laparotomy and intestinal resection, with a mean time to reoperation of 0.7 years (0.5e0.9 years).
Distal ureterectomy Only þ Proximal infolding þ Proximal tailoring Technique Politano-Leadbetter Paquin-Psoas hitch Total
24 29 9 46 16 62 units
UCN: ureterocystoneostomy. PP: pyeloplasty. UUA: uretero-ureteroanastomosis (duplex kidney). NU: nephroureterectomy.
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Comprehensive surgery of Prune Belly Syndrome
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children (Fig. 1D and E). Sixteen children remained with 18 dilated renal units, but only seven of these evidently became obstructed, requiring surgical revision. The mean time for reoperation was 1.3 years (12 dayse6.7 years). Pre-operative VUR was evident in 26 of the 34 children (76.5%) who performed pre-operative VCUG. In the late post-operative control, 13 children repeated VCUG; four of the 26 units presented with VUR (15.4%). Three of these were cured with secondary procedures e one open and two endoscopic. The mean time for reoperation was 3.6 years (1e6.2 years). In the long run, 35 of the 40 children who underwent upper tract reconstruction had normal renal function, while two developed clinically managed chronic renal failure (CRF) and three were submitted for renal transplantation due to end-stage renal failure (ESRF). It is noteworthy that there was no difference in the pre-operative creatinine levels of these children [wC: 0.61 (0.4e1.5) for those with normal renal function versus wC: 0.73 (0.6e1.0) for those with CRF/ESRF]. On the other hand, renal dysplasia, as seen by renal ultrasonographic hyperechogenicity and/or multiple cysts in the initial image study, was present in all CRF/ ESRF children, and in 21.7% of those with normal renal function. Likewise, polyuria was more prevalent in the CRF/ESRF children (80%) than in those with normal renal function (39.3%).
Orchiopexy The postoperative size and location of the testes were evaluated and compared with age-related nomograms. In children who underwent orchiopexy without vascular section, 87% of the testes remained normal in size and were well located in the scrotum in the late follow-up, while 78% did so after section of the vessels. Considering both groups, 85% of the testes were normal in size and adequately located in the scrotum (Table 2). Three children were submitted to redo orchiopexy due to reascended testes, with a mean time of 4.5 years (3.2e5.1 years) after primary surgery. All pubertal children developed normal secondary sexual characteristics. A Fischer’s exact test was employed to compare testes that underwent vessel ligation to those that did not, in terms of outcome (atrophy vs normal size), with P Z 0.3276 (without reaching statistical significance).
Circumcision All children that were submitted to circumcision had no complications regarding this procedure.
Lower urinary tract reconstruction Lower urinary tract reconstruction was performed in 44 children, 41 of them with resection of the bladder dome and urachal remnant. Three of the 44 children who presented with evident pre-operative bladder failure with high residual volume underwent primary cystoplasty associated with appendiceal Mitrofanoff. Postoperatively, three children required Mitrofanoff diversion in a secondary procedure due to postoperative bladder failure. During the late follow-up after UTR, which included cystoplasty, continence was evaluated in 42 boys who were older than 5 years: 34 (81%) were continent, while in the group of eight incontinent children (19%), five presented with polyuria. Adequate bladder emptying (defined as absence of increased bladder residual volume on ultrasonography) could be observed in 33 boys (71.7%), while five (10.9%) required either Crede ´ or Valsava manoeuvres to accomplish it. Eight children (17.4%) required CIC, four of them (8.7%) through Mitrofanoff channels. Two other children, who also had these channels, acquired normal voiding habits and ceased CIC. The remaining four children performed CIC through the urethra. No children had urethral atresia, but three presented with megalourethra. Only one, who had a scaphoid Table 2
megalourethra associated with penile curvature, was submitted to a secondary orthophalloplasty with urethral tapering at adolescence due to significant ballooning with post-void dribbling.
Discussion Comprehensive surgery, including simultaneous urinary tract reconstruction, bilateral orchiopexy, abdominoplasty and circumcision, is feasible. It had an acceptable morbidity rate of 26% (12/46) consisting of surgical-related complications of all steps of the aforementioned procedures - necessitating surgical revision of seven obstructed reimplanted ureters, four cases of postoperative unilateral VUR, four redo abdominoplasties, and three testes that were not topic after surgery. The total reoperation rate was 36.9% (17/46). This was for children who needed additional procedures for comprehensive surgeryrelated complications and others that had disease progression (bladder failure, ESRF) or complications indirectly related to the surgical procedure itself (acute abdomen), as shown in Table 3. No mortality was observed in this cohort. Abdominoplasty has physical and physiological effects on children with PBS. Correction of the abdominal flaccidity improves abdominal strength and, as a result, it ameliorates constipation and bladder emptying [15,20]. Abdominal appearance and tonus were improved in 90% of the
Results of orchiopexy with and without vascular section.
Number of testes
Ectopic
Atrophic
Atrophic & ectopic
Normal & topic
No vascular section 69 With vascular section 23 Total 92
2 (2.9%) 0 2 (2.2%)
6 (8.7%) 5 (21.7%) 11 (11.9%)
1 (1.4%) 0 1 (1.1%)
60a (86.9%) 18b (78.3%) 78 (84.8%)
a b
2 testes submitted to redo orchiopexy. 1 testis submitted to redo orchiopexy.
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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R.I. Lopes et al. Necessary reoperation in the cohort.
Complication
N of pts.
N of procedure
Type of reintervention
Ureteral obstruction
7
10
Persistent or “de novo” VUR
4
4
Persistent cryptorchidism Acute abdomen (obstruction) Bladder failure
3 3 3
3 3 4
Persistent abdominal flaccidity End stage renal failure Total
4 3 17/46 (36.9%)
Pyelostomy, nephrostomy, ureteral reimplantation, nephroureterectomy Ureteral reimplantation, endoscopic injection Redo orchiopexy Exploratory laparotomy Vesicostomy/ appendicovesicostomy Abdominoplasty Renal Transplantation
4 3 31
children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children (90.9%) with significant dilatation or VUR associated with recurrent UTI that put them at risk of progressive renal deterioration [6,21,22]. As a result, the frequency of UTI and urinary sepsis significantly decreased in the postoperative period, becoming mostly asymptomatic. The reduced incidence of VUR, as well as the routine circumcision, probably contributed to the decrease in postoperative UTIs, which had a positive impact in the longterm follow-up of the children. Although upper tract dilatation was relatively common, even after surgery, as seen in 40% of the children, clinically proven obstruction that required reintervention was rare (17.5%). Long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Bilaterally abnormal kidneys on ultrasound or renal scan, nadir serum creatinine of >0.7 mg/dl and recurrent pyelonephritis were shown to be prognostic factors for renal failure [23,24]. In the present series, pre-operative serum creatinine levels were not associated with progression to renal failure, but renal dysplasia and polyuria were predictors of late renal failure. There was an 11% rate of ureteral reimplantation surgical revision. However, it should be noted that children with PBS frequently have massive ureteral dilatation, much more than in cases of isolated VUR or megaureter, as shown in Fig. 1. Therefore, major ureteroplasties were required and performed in the present cohort, with a high, but acceptable, rate of complications. Early treatment of ureteral obstruction did not lead to renal unit loss in any of the cases. On the other hand, the present series had a lower rate of chronic renal insufficiency in comparison to most series in the literature. In the present study, five of the 46 children (10.8%) developed chronic renal failure and three (6.5%) were submitted for renal transplantation. Seidel et al. [25] presented a much higher proportion of children that presented with chronic kidney disease (39%) and that were submitted for renal transplantation (17.4%) in their follow-up. Furthermore, although performing ureteral, bladder and abdominal procedures in
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Journal of Pediatric Urology (2015) xx, 1.e1e1.e7
27 years of experience with the comprehensive surgical treatment of prune belly syndrome R.I. Lopes, A. Tavares, M. Srougi, F.T. De ´nes Pediatric Urology Unit, Division of Urology, Hospital das Clı´nicas, University of Sa ˜o Paulo School of Medicine, Sa ˜o Paulo, Brazil Correspondence to: Rua Dr. Eneas de Carvalho Aguiar 255 7 andar, Division of Urology, Hospital das Clinicas da FMUSP, Sa ˜o Paulo CEP 05403000, Brazil, Tel.: þ55 11 2661 8080, þ55 11 99626 8974 (mobile) [email protected] (R.I. Lopes) [email protected] (A. Tavares) [email protected] (M. Srougi) [email protected] (F.T. De´nes) Keywords Prune belly syndrome; Abdominal wall defect; Urinary tract reconstruction Received 23 December 2014 Accepted 6 May 2015 Available online xxx
Summary Introduction Prune belly syndrome (PBS) presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism. As a result, urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. Objectives To report experiences with comprehensive surgical management of prune belly syndrome. Material and methods From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. According to individual needs, treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract, and perform bilateral orchiopexy and circumcision, which were performed in one procedure. Urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation with evidence of significant stasis and/or vesicoureteral reflux was associated with recurrent urinary tract infections (UTI). Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions. The median age at surgery was 16 months and children were followed for a median of 143 months.
Results Abdominal appearance and tonus were improved in 90% of the children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children and long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Lower urinary tract reconstruction was performed in most children (95.6%); on late follow-up, continence was observed in 81% of them, while incontinence was present in 19% and usually associated with polyuria. Adequate bladder emptying was possible in most boys (82.6%), while the remaining required clean intermittent catheterization. Pre-operative UTI was present in 89.1% and urinary sepsis in 15.2%. Postoperatively, the incidence of laboratorial UTI was significantly reduced to 39.1%, while urinary sepsis was absent. Bilateral orchiopexy was performed in all children, with 85% of the testes becoming normal in size and well located in the scrotum.
Conclusions Comprehensive surgical treatment is feasible and has good long-term results. A considerable incidence of reoperations due to complications or progression of the disease was observed. The long-term results for reno-ureteral anatomy and function, bladder function, infection, testicular size and location, as well as abdominal aspect and tonus, show that comprehensive surgery is an adequate method for managing children with PBS.
http://dx.doi.org/10.1016/j.jpurol.2015.05.018 1477-5131/ª 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Introduction Prune belly syndrome (PBS) has an incidence of 1:35,000 to 1:50,000 live births and mainly occurs in boys. It presents with three main features: abdominal wall flaccidity, urological abnormalities and cryptorchidism [1e5]. Its characteristic, but variable, abdominal flaccidity may cause physical limitations and compromised self-image for these children. The variable anomalies of the urinary tract, which are not always proportional to the abdominal flaccidity, affect the kidneys and bladder in a non-uniform fashion [1e6]. Renal dysplasia, ureterohydronephrosis, megaureter, VUR, and bladder dysfunction are associated to recurrent UTI, which when untreated or not prevented further impair renal function in most children [1e6]. Intraabdominal cryptorchidism is uniformly associated with infertility in these children [1e7]. Management of these children is controversial. Urologists must consider the eventual repair of the abdominal wall flaccidity and urinary tract abnormalities, and the mandatory correction of cryptorchidism, as well as decide whether to perform the procedures in a single comprehensive approach or in multiple steps. Non-operative treatment of the urinary tract anomalies and the abdominal wall has been proposed, assuming that renal function will stabilise with prevention of UTI and the spontaneous improvement of the reno-ureteral anatomy [8,9]. Some studies have recommended urinary tract reconstruction (UTR) only in cases of confirmed obstruction and/or untreatable UTI [1,8,9]. This approach requires close surveillance and the long-term results have not been homogeneous [1,8,9]. Other studies have advocated UTR whenever stasis, VUR or UTI are present because these common factors put children with PBS at risk of progressive renal deterioration [10,11]. Because long-term continuous surveillance of these children is difficult in Brazil, due to social and geographical conditions, comprehensive surgical management of these children with PBS is preferred [6]. The aim of this study was to present the long-term results of this approach. It is believed that, to date, this experience is one of the largest reported in the literature.
Patient and methods At initial presentation, all children with PBS underwent clinical evaluation followed by functional evaluations with ultrasonography or intravenous urography, as well as radioisotopic renography. VCUG was performed to assess the presence of urethral athresia and megalourethra in cases with severe UTI, marked ureteral dilatation or in those who pre-operatively presented with abnormal voiding patterns or urachal fistula [12e14]. Urodynamic evaluation was only performed for older children who could not spontaneously void and/or had episodes of urinary retention. Renal function was evaluated by blood urea and creatinine levels. Urinalysis and uroculture were performed in all children. From 1987 to 2014, 46 children with PBS were submitted for comprehensive surgical treatment. Children were classified according to disease severity using the Woodard
R.I. Lopes et al. scale [1], which classifies children in three groups. All groups have the characteristic, but variable, abdominal flaccidity. In Group 1, significant renal dysplasia and pulmonary hypoplasia are present, and the children have a high rate of perinatal mortality resulting from pulmonary and/or renal insufficiency. In Group 2, despite the significant anatomical and functional abnormalities of the urinary tract, renal function is initially stable, but may progress to renal insufficiency due to recurrent obstruction or infection. In Group 3, mild urinary tract abnormalities may be present and most children present with stable renal and pulmonary function, with high survival rates without intervention. Forty children of the cohort were classified as Woodard Grade 2, while the remaining six had Grade 3 disease. The median age at surgery was 16 months (25 dayse10 years) and children were followed for a median of 143 months (15 monthse26 years). Two of the children had loop ureterostomies and one had a vesicostomy performed elsewhere, while three others had patent urachuses draining the bladder. Some children were performing CIC, while others were referred with bladder catheters. In one patient with malnutrition and a single functioning kidney associated with a severely dilated ureter and recurrent UTIs, a terminal loop ureterostomy was performed before UTR in order to improve general conditions. According to individual needs, surgical treatment aimed to correct the abdominal flaccidity, reconstruct the urinary tract and orchiopexy, preferably in one procedure. Treatment for this cohort included: 44 abdominoplasties, 40 upper urinary tract reconstructions, 44 cystoplasties associated with three appendico-vesicostomies, 46 bilateral orchiopexies and 36 circumcisions.
Abdominoplasty and laparotomy Forty-four of the 46 children received abdominal wall reconstruction. The timing of this procedure was dictated by the need for other surgical procedures, particularly orchiopexy and urinary tract reconstruction. Three children were initially submitted to Erlich’s technique and two to Randolph’s repair [1], but in all 39 subsequent children, abdominoplasty was performed with the modified technique, which has previously been described [15].
Upper urinary tract reconstruction Upper urinary tract reconstruction was indicated whenever pyelo-ureteral dilatation was associated with significant stasis or obstruction, as well as to VUR, recurrent UTI and scarring of the ipsilateral kidney (Fig. 1A). Significant stasis or obstruction was considered when progressive dilatation with worsening renal or differential renal function was observed. Reconstruction of the upper urinary tract [16] required either unilateral or bilateral distal ureterectomy and ureterocystoneostomy (Fig. 1B and C). Non-functioning units due to dysplasia or hydronephrosis were submitted to nephroureterectomy. Distal ureterectomy with ureterocystoneostomy was performed due to redundancy or dilatation, as well as VUR. Uretero-
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Comprehensive surgery of Prune Belly Syndrome
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Figure 1 A: Pre-operative marked bilateral ureteral dilatation; B: After resection of distal ureters, proximal ureters are cannulated; C: Surgical specimens consisting of extremely enlarged distal ureters and bladder dome; D: Pre-operative excretory urography demonstrating marked bilateral dilation of ureters, with distal ureter redundancy; E: After urinary tract reconstruction, the postoperative improvement of ureteral dilation is evident. Source: authors’ own photos.
vesical reimplantation was usually performed with the Paquin technique, associated with a psoas hitch when performed unilaterally. When required, ureteral tailoring or infolding, as well as renal mobilization, were performed to ensure optimal ureteroneocystostomy. Ureteral stents were left indwelling and attached to a Foley urethral catheter or percutaneously exteriorised through the bladder and abdominal wall. Previous ureterostomies were closed or removed with the distal ureter. In four cases of associated UPJ obstruction, non-dismembered side-to-side pyeloureteral anastomoses were performed.
Lower urinary tract reconstruction At laparotomy, the bladder was detached from the abdominal wall, sectioning the urachal remnants. An individually tailored cystoplasty was performed with removal of the urachal diverticulum or fistula and part of the bladder dome, particularly in cases with significantly enlarged bladders [4,17,18]. A reduction cystoplasty was indicated for the resection of a urachal diverticulum or for excessively large bladders, as described by Woodard. Overresection of the bladder was avoided, ensuring adequate capacity after closure. Primary appendiceal Mitrofanoff
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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1.e4 channels were made in children that required continuous or intermittent bladder drainage before surgery, one of them had scaphoid megalourethra [19]. All parents were advised on the importance of regular bladder emptying and many were trained with Valsalva and Crede ´ manoeuvres to adequately empty their children’s bladders.
Orchiopexy Cryptorchid testes are usually found high in the abdomen, lying above the enlarged ureteral folds. As both the right and left colonic segments lack fixation to the abdominal wall, their retraction easily exposes the testes in the retroperitoneum. They are dissected and separated from the ureter, with preservation of the gonadal vessels, vas deferens and its vessels, and, if possible, gubernaculum. When adequate vessel length is obtained, the inguinal fascia is incised on both sides and the testes are brought down to the scrotum and fixed in subdartic pouches, completing the orchiopexy. If the testes are distant from the inguinal region and the spermatic vessels are too short, these are sectioned; the still intact paradeferential and gubernacular vessels ensure gonadal vascularisation [7]. All 46 children underwent bilateral orchiopexy. In 31 children, adequate testicular repositioning was achieved without the need to transect the vessels; while in 15 children, the vessels were sectioned either unilaterally (in seven) or bilaterally (in eight).
R.I. Lopes et al.
Upper urinary tract reconstruction All pre-operative UTI were symptomatic. All children were on prophylactic antibiotics pre-operatively. Postoperatively, children received antibiotic prophylaxis whenever they were submitted to UTR, usually for a period of 3 months. After this period, they only received antibiotics temporarily when complications such as UTI or obstruction were observed. In the study cohort, pre-operative UTI was present in 41 children (89.1%) and urinary sepsis in seven (15.2%). Postoperatively, the incidence of UTI was significantly reduced to 39.1% (18/46); most of them were asymptomatic, while urinary sepsis was absent. The McNemar test was performed to compare pre- and postoperative data regarding UTI and sepsis. There was a statistically significant decrease in UTI rate (P < 0.001/X2 Z 19.36) and sepsis (P Z 0.0233/X2 Z 5.193) postoperatively. As seen in Table 1, bilateral distal ureterectomy followed by bilateral ureteral reimplantation was performed in 23 children and unilateral distal ureterectomy followed by ureterocystoneostomy was performed in 16 children. Unilateral nephroureterectomy alone was performed in one child. Straightforward ureterocystoneostomy was performed after distal ureterectomy in 24 units, but infolding and tailoring in the proximal ureter were required for reimplantation in 38 units. A total of 62 renal units were treated in 40 children. In the late follow-up, the upper urinary tract showed significant anatomical improvement in 44 renal units of 24
Circumcision Circumcision was routinely performed in the latter 36 children at the end of the procedure, also to reduce the risk of UTI.
Results Clinical and radiological work-up was used to compare preoperative and postoperative status of the cohort and to determine treatment success or failure. Polyuria was defined as a urine output of >5 ml/kg/hour and/or hypoosmolar urine (urinary density of 1010).
Abdominoplasty and laparotomy
Table 1 Procedures performed for upper urinary tract reconstruction. Procedures
Number of patients
Bilateral UCN Bilateral UCN þ bilat. PP Bilateral UCN þ unilat. UUA Sub-total Unilateral UCN þ contralat. NU Unilateral UCN þ ipsilat. PP þ contralat. NU Unilateral UCN þ contralat. PP Unilateral UCN Sub-total Unilateral NU Total
21 1 1 23 pts. (46 units) UCN 10 2 1 3 16 pts. (16 units) UCN 1 40 pts. (62 units) UCN
Ureterocystoneostomy
The medical staff and parents subjectively evaluated abdominal flaccidity and posture. Abdominal tonus was determined by abdominal observation and palpation in the horizontal decubitus, sitting up and in the upright position. For 40 children, the abdominal appearance and significantly improved after the procedure (90%), while four (10%) had unsatisfactory results, either with persistent or recurrent flaccidity. All required secondary abdominoplasties and had a good outcome. It is noteworthy that three children had late, acute abdomen due to intestinal obstruction that required exploratory laparotomy and intestinal resection, with a mean time to reoperation of 0.7 years (0.5e0.9 years).
Distal ureterectomy Only þ Proximal infolding þ Proximal tailoring Technique Politano-Leadbetter Paquin-Psoas hitch Total
24 29 9 46 16 62 units
UCN: ureterocystoneostomy. PP: pyeloplasty. UUA: uretero-ureteroanastomosis (duplex kidney). NU: nephroureterectomy.
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Comprehensive surgery of Prune Belly Syndrome
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children (Fig. 1D and E). Sixteen children remained with 18 dilated renal units, but only seven of these evidently became obstructed, requiring surgical revision. The mean time for reoperation was 1.3 years (12 dayse6.7 years). Pre-operative VUR was evident in 26 of the 34 children (76.5%) who performed pre-operative VCUG. In the late post-operative control, 13 children repeated VCUG; four of the 26 units presented with VUR (15.4%). Three of these were cured with secondary procedures e one open and two endoscopic. The mean time for reoperation was 3.6 years (1e6.2 years). In the long run, 35 of the 40 children who underwent upper tract reconstruction had normal renal function, while two developed clinically managed chronic renal failure (CRF) and three were submitted for renal transplantation due to end-stage renal failure (ESRF). It is noteworthy that there was no difference in the pre-operative creatinine levels of these children [wC: 0.61 (0.4e1.5) for those with normal renal function versus wC: 0.73 (0.6e1.0) for those with CRF/ESRF]. On the other hand, renal dysplasia, as seen by renal ultrasonographic hyperechogenicity and/or multiple cysts in the initial image study, was present in all CRF/ ESRF children, and in 21.7% of those with normal renal function. Likewise, polyuria was more prevalent in the CRF/ESRF children (80%) than in those with normal renal function (39.3%).
Orchiopexy The postoperative size and location of the testes were evaluated and compared with age-related nomograms. In children who underwent orchiopexy without vascular section, 87% of the testes remained normal in size and were well located in the scrotum in the late follow-up, while 78% did so after section of the vessels. Considering both groups, 85% of the testes were normal in size and adequately located in the scrotum (Table 2). Three children were submitted to redo orchiopexy due to reascended testes, with a mean time of 4.5 years (3.2e5.1 years) after primary surgery. All pubertal children developed normal secondary sexual characteristics. A Fischer’s exact test was employed to compare testes that underwent vessel ligation to those that did not, in terms of outcome (atrophy vs normal size), with P Z 0.3276 (without reaching statistical significance).
Circumcision All children that were submitted to circumcision had no complications regarding this procedure.
Lower urinary tract reconstruction Lower urinary tract reconstruction was performed in 44 children, 41 of them with resection of the bladder dome and urachal remnant. Three of the 44 children who presented with evident pre-operative bladder failure with high residual volume underwent primary cystoplasty associated with appendiceal Mitrofanoff. Postoperatively, three children required Mitrofanoff diversion in a secondary procedure due to postoperative bladder failure. During the late follow-up after UTR, which included cystoplasty, continence was evaluated in 42 boys who were older than 5 years: 34 (81%) were continent, while in the group of eight incontinent children (19%), five presented with polyuria. Adequate bladder emptying (defined as absence of increased bladder residual volume on ultrasonography) could be observed in 33 boys (71.7%), while five (10.9%) required either Crede ´ or Valsava manoeuvres to accomplish it. Eight children (17.4%) required CIC, four of them (8.7%) through Mitrofanoff channels. Two other children, who also had these channels, acquired normal voiding habits and ceased CIC. The remaining four children performed CIC through the urethra. No children had urethral atresia, but three presented with megalourethra. Only one, who had a scaphoid Table 2
megalourethra associated with penile curvature, was submitted to a secondary orthophalloplasty with urethral tapering at adolescence due to significant ballooning with post-void dribbling.
Discussion Comprehensive surgery, including simultaneous urinary tract reconstruction, bilateral orchiopexy, abdominoplasty and circumcision, is feasible. It had an acceptable morbidity rate of 26% (12/46) consisting of surgical-related complications of all steps of the aforementioned procedures - necessitating surgical revision of seven obstructed reimplanted ureters, four cases of postoperative unilateral VUR, four redo abdominoplasties, and three testes that were not topic after surgery. The total reoperation rate was 36.9% (17/46). This was for children who needed additional procedures for comprehensive surgeryrelated complications and others that had disease progression (bladder failure, ESRF) or complications indirectly related to the surgical procedure itself (acute abdomen), as shown in Table 3. No mortality was observed in this cohort. Abdominoplasty has physical and physiological effects on children with PBS. Correction of the abdominal flaccidity improves abdominal strength and, as a result, it ameliorates constipation and bladder emptying [15,20]. Abdominal appearance and tonus were improved in 90% of the
Results of orchiopexy with and without vascular section.
Number of testes
Ectopic
Atrophic
Atrophic & ectopic
Normal & topic
No vascular section 69 With vascular section 23 Total 92
2 (2.9%) 0 2 (2.2%)
6 (8.7%) 5 (21.7%) 11 (11.9%)
1 (1.4%) 0 1 (1.1%)
60a (86.9%) 18b (78.3%) 78 (84.8%)
a b
2 testes submitted to redo orchiopexy. 1 testis submitted to redo orchiopexy.
Please cite this article in press as: Lopes RI, et al., 27 years of experience with the comprehensive surgical treatment of prune belly syndrome, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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1.e6 Table 3
R.I. Lopes et al. Necessary reoperation in the cohort.
Complication
N of pts.
N of procedure
Type of reintervention
Ureteral obstruction
7
10
Persistent or “de novo” VUR
4
4
Persistent cryptorchidism Acute abdomen (obstruction) Bladder failure
3 3 3
3 3 4
Persistent abdominal flaccidity End stage renal failure Total
4 3 17/46 (36.9%)
Pyelostomy, nephrostomy, ureteral reimplantation, nephroureterectomy Ureteral reimplantation, endoscopic injection Redo orchiopexy Exploratory laparotomy Vesicostomy/ appendicovesicostomy Abdominoplasty Renal Transplantation
4 3 31
children after the primary surgery and 100% after reoperation. Upper urinary tract reconstruction was performed in most children (90.9%) with significant dilatation or VUR associated with recurrent UTI that put them at risk of progressive renal deterioration [6,21,22]. As a result, the frequency of UTI and urinary sepsis significantly decreased in the postoperative period, becoming mostly asymptomatic. The reduced incidence of VUR, as well as the routine circumcision, probably contributed to the decrease in postoperative UTIs, which had a positive impact in the longterm follow-up of the children. Although upper tract dilatation was relatively common, even after surgery, as seen in 40% of the children, clinically proven obstruction that required reintervention was rare (17.5%). Long-term follow-up showed functional stabilization of the urinary tract in about 90% of the children, with progression to renal failure in 10%. Bilaterally abnormal kidneys on ultrasound or renal scan, nadir serum creatinine of >0.7 mg/dl and recurrent pyelonephritis were shown to be prognostic factors for renal failure [23,24]. In the present series, pre-operative serum creatinine levels were not associated with progression to renal failure, but renal dysplasia and polyuria were predictors of late renal failure. There was an 11% rate of ureteral reimplantation surgical revision. However, it should be noted that children with PBS frequently have massive ureteral dilatation, much more than in cases of isolated VUR or megaureter, as shown in Fig. 1. Therefore, major ureteroplasties were required and performed in the present cohort, with a high, but acceptable, rate of complications. Early treatment of ureteral obstruction did not lead to renal unit loss in any of the cases. On the other hand, the present series had a lower rate of chronic renal insufficiency in comparison to most series in the literature. In the present study, five of the 46 children (10.8%) developed chronic renal failure and three (6.5%) were submitted for renal transplantation. Seidel et al. [25] presented a much higher proportion of children that presented with chronic kidney disease (39%) and that were submitted for renal transplantation (17.4%) in their follow-up. Furthermore, although performing ureteral, bladder and abdominal procedures in
