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Comprehensive surgical treatment of prune belly syndrome: 17 years' experience with 32 patients
PEDIATRIC UROLOGY
COMPREHENSIVE SURGICAL TREATMENT OF PRUNE BELLY SYNDROME: 17 YEARS’ EXPERIENCE WITH 32 PATIENTS FRANCISCO T. DÉNES, MARCO A. ARAP, AMILCAR MARTINS GIRON, FREDERICO A. Q. SILVA, AND SAMI ARAP
ABSTRACT Objectives. To describe the results of comprehensive surgical management of prune belly syndrome. Methods. Thirty-two patients were evaluated and treated for abdominal and genitourinary abnormalities. The surgical procedure, including simultaneous abdominoplasty, bilateral orchiopexy, and urinary tract reconstruction, was performed according to individual needs. At follow-up, the urinary tract, abdominal, and genital anatomy and function were evaluated. Results. The upper urinary tract anatomy and function were stabilized in 30 patients, and no vesicoureteral reflux was found postoperatively. The bladder drainage was adequate in 29 patients, and 4 presented with recurrent bacteriuria. Abdominoplasty improved flaccidity in 29 patients. Adequate testicular position and consistency were obtained in 54 testes. Complications included acute tubular necrosis (1 patient) and ureterovesical obstruction (3 patients). Conclusions. Individualized comprehensive surgical management of prune belly syndrome is effective, with an acceptable incidence of complications and excellent long-term results. UROLOGY 64: 789–794, 2004. © 2004 Elsevier Inc.
P
rune belly syndrome (PBS) has an incidence of 1:35,000 to 1:50,000 live births and presents with three main features: abdominal wall flaccidity, bilateral intra-abdominal cryptorchidism, and urologic abnormalities.1 The abdominal flaccidity may cause several conditions, including lordosis and respiratory infections owing to a lack of abdominal support.1– 4 The testes, located inside the abdomen, are indistinguishable from cryptorchid testes of nonsyndromic patients.5 When untreated, despite adequate testosterone levels, the testes disclose a lack of spermatogenesis after puberty.6 The urinary tract presents with varying degrees of renal dysplasia and ureteropelvic dilation and redundancy, particularly in the distal ureter. Large-capacity bladders are generally present, in association with urachal diverticulum or fistula, and with large postvoid residual volumes.3,7 In a few cases, urethral hypoplasia or atresia further impairs bladder emptyFrom the Division of Urology, University of São Paulo Medical School Hospital, São Paulo, Brazil Reprint requests: Francisco Tibor Dénes, M.D., Division of Urology, University of São Paulo Medical School Hospital, Rua Barata Ribeiro, 414 cj 135, São Paulo 01308-000, Brazil Submitted: February 11, 2004, accepted (with revisions): May 6, 2004 © 2004 ELSEVIER INC. ALL RIGHTS RESERVED
ing.8 Vesicoureteral reflux (VUR) occurs in two thirds of patients.9 The prognosis may depend on the degree of urinary tract stasis or obstruction and infection that impairs renal function later in life.4,10 According to Woodard, patients with PBS are classified into three groups.4 In group 1 (20% of cases), significant renal dysplasia and pulmonary hypoplasia is present, and most patients are stillborn or die shortly after birth. In group 2 (40%), despite many anatomic and functional abnormalities, adequate renal function is present at birth, but may become compromised by obstruction and infection. One third of untreated patients in group 2 die within the first 2 years of renal failure or sepsis. Patients in group 3 (40%) present with mild urinary tract abnormalities, ensuring normal function and survival in most patients.4,11,12 The late prognosis of patients in groups 2 and 3 depends on the occurrence of urinary infection and obstruction. Some investigators recommend nonoperative treatment, assuming that renal function will stabilize with spontaneous improvement of the urinary tract.2,9,13,14 This approach, however, demands close surveillance, and its long-term results have not been homogeneous. Furthermore, it does not address the bilateral cryptorchidism and abdominal flaccidity. 0090-4295/04/$30.00 doi:10.1016/j.urology.2004.05.053 789
Because stabilization is impossible in many patients, other investigators believe that urinary reconstruction that eliminates stasis, corrects VUR, and improves bladder drainage is beneficial.11,15,16 Nevertheless, it is not clear at what age this surgery is appropriate, nor whether it can be performed together with orchiopexy and abdominoplasty. Our therapeutic approach entails a comprehensive procedure that includes individually tailored urinary tract reconstruction, bilateral orchiopexy, and abdominoplasty.12 In this report, we present our long-term results. MATERIAL AND METHODS From 1985 to May 2002, 32 children were prospectively evaluated and treated in our institution. They were classified as having Woodard’s group 2 (16 patients) and 3 (16 patients) PBS. All 32 children had abdominal flaccidity, which was classified as severe in 15, mild in 11, and slight in 6. Of the 32 patients, 27 presented with significant urinary tract infection, which was treated before surgery. Four patients had undergone previous operations elsewhere: three urinary diversions (one bilateral ureterostomy and two vesicostomies) and one failed bilateral orchiopexy. Two patients presented with urachal fistulas and one with megalourethra. All patients underwent functional preoperative and postoperative evaluations with ultrasonography, intravenous urography, radioisotopic renography, and voiding cystourethrography. In addition, patients older than 3 years also underwent urodynamic studies, completing the functional/anatomic evaluation. Renal function was evaluated by the blood urea and creatinine levels. The results were used to compare the patients preoperatively and postoperatively and, therefore, to define treatment failure and success. The postoperative size and location of the testes were examined and compared with age-related nomograms. Because objective criteria to evaluate cosmetic results are rare and difficult to apply, especially when patients present in a wide age range, the abdominal flaccidity and posture were subjectively evaluated by the medical team, the patients, and their parents. Abdominal tonus was determined by observing patients in the upright position, palpating the abdominal wall, and head lifting from the horizontal decubitus.
SURGICAL PROCEDURE An individually tailored longitudinal ellipsoid figure was drawn on the anterior abdomen, according to its flaccidity and redundancy. The skin and subcutaneous layers of this area were removed, with the exception of the umbilicus, which was previously demarcated. The aponeurotic fascia was exposed (Fig. 1A,B). The fascia was then incised using only one unilateral curved paramedian incision in the most redundant side, extending from the upper to the lower extremities of the skin incision. A large retractor was inserted, allowing unobstructed view of the abdominal cavity. The bladder was separated from the abdominal wall, removing the urachal ligament or fistula. The testes, found between the ureteral folds, were dissected, preserving the spermatic vessels, vas deferens and its vessels, and, if possible, the gubernaculum. When an adequate length was obtained, they were left resting (Fig. 1C) until later in the operation. Urinary tract reconstruction was performed as described by Woodard.11 Unilateral nonfunctioning kidneys were treated with nephroureterectomy. In cases of severe ureteral dilation 790
FIGURE 1. Surgical procedure. (A) Demarcation of skin incision and area of abdominoplasty; (B) excision of skin and subcutaneous tissue, preserving aponeurotic fascia and umbilicus; (C) bladder mobilization and dissection of testes and ureters; (D) aponeurotic suture; and (E) closure of abdominal wall, with overlap of aponeurotic flaps. and tortuosity or VUR, the redundant distal ureter was removed and proximal ureter straightened with careful preservation of its blood supply. Previous ureterostomies were closed or removed with the distal ureter. If pyeloureteral obstruction remained, a nondismembered side-to-side pyeloureteral anastomosis could be performed. The vesical dome was then removed with the urachal diverticulum. Over-resection of the bladder must be avoided to ensure adequate capacity after closure. The proximal ureters were reimplanted using tension-free Politano-Leadbetter (bilateral reimplantation) or Paquin-psoas hitch (unilateral reimplantation) techniques. When necessary, ureteral tailoring or infolding, as well as renal mobilization, was performed to ensure optimal ureterocystoneostomy. Percutaneous ureteral stents were left indwelling, exteriorized through the bladder. Orchiopexy was then completed. The inguinal fascia was incised on both sides, and the testes were brought to the scrotum, and fixed in subdartic pouches. If the spermatic vessels UROLOGY 64 (4), 2004
were short, they were sectioned if the paradeferential and gubernacular vessels were still intact. The abdomen was closed by overlapping and suturing the two leaves of the musculoaponeurotic fascia to each other, in a “vest-over” fashion. The intact umbilicus, left in the larger posterior fascia, was exposed through a “buttonhole” in the smaller anterior fascia, where it was sutured (Fig. 1D,E). The skin was approximated according to the initial demarcation. Circumcision was incorporated into the procedure in the last 14 patients. The details of the surgical procedures are presented in Table I.
RESULTS The mean age at surgery was 23 months (range 1 month to 6 years). Preoperatively, 27 patients had clinically significant recurrent urinary tract infection. At surgery, 7 patients had increased urea and creatinine levels, and 7 presented with unilateral nonfunctioning kidneys, with normal contralateral function. Significant ureteral dilation and redundancy were present in 24 patients (Fig. 2A), and 15 presented with massive unilateral or bilateral pyelocaliceal dilation. Twenty-one patients presented with VUR; it was unilateral in 10 and bilateral in 11 (26 units with VUR grade III and IV and 6 with grade I and II). The duration of the reconstructive surgery averaged 255 minutes (range 210 to 345). No intraoperative complications occurred. The mean postoperative follow-up was 60 months (range 13 to 212). Six patients presented with early postoperative complications: urinary tract infection in three, pneumonia in one, acute tubular necrosis (ATN) in one, and increased vesical residue in one. Late complications included two ureterovesical obstructions and three hypotonic bladders. Ten patients underwent 13 repeat operations (Table I). Complications of the primary surgery required repeated operation in 5 patients. In 3 patients, ureterovesical obstruction occurred after Leadbetter-Politano reimplantation without tailoring or infolding; 1 patient underwent a unilateral nephrostomy and later nephroureterectomy, and 1 underwent secondary unilateral ureterocystoneostomy. The third patient presented with anuria after bilateral stent removal and underwent emergency bilateral pyelostomy. After 3 weeks, he resumed spontaneous voiding, and pyelography documented normal ureterovesical flow (the pyelostomies were closed later). In 1 patient with oliguria, exploratory lumbotomy was performed because of suspected urinary extravasation that was not confirmed; renal biopsy revealed ATN that normalized after 2 weeks. Another patient with recurrent vesical retention 1.5 years after the first reconstruction, who was unable to undergo additional clean intermittent catheterization owing to social reasons, underwent secondary vesicostomy. Six elective reoperations, not related to complications, were performed in 6 patients. One patient who had undergone unilateral nephroureterecUROLOGY 64 (4), 2004
tomy in the first operation, developed contralateral VUR 2 years later, and right ureterocystoneostomy was performed. Five years later, penile curvature and megalourethra were also corrected. One patient presented with a right inguinal hernia 4 years after the first procedure, and 3 patients who had not undergone abdominoplasty during the first procedure underwent it later. All patients were alive at last follow-up. Radiographic and ultrasound evaluations revealed a decreased prevalence of ureterohydronephrosis, which was observed in only 8 patients. No postoperative VUR was observed in any patient. Postoperative bladder emptying was normal in 20 patients as verified by ultrasonography and urodynamic studies, including 4 who had not undergone reductive cystoplasty. Nine patients had daytime overflow incontinence but remained dry at night. Urodynamic studies showed decreased bladder sensation with increased capacity and normal compliance. The decreased urinary flow was due to a hypocontractile detrusor, but bladder emptying improved with Credé and Valsalva maneuvers. These patients presented with no upper urinary tract deterioration and were treated with scheduled voiding. Only 3 patients, who had undergone reductive cystoplasty, had persistent large postvoid residual urine volumes; 2 were using CIC at last follow-up, and 1 underwent secondary vesicostomy. Recurrent asymptomatic bacteriuria was present in 4 children, including 2 using CIC; all 4 children received antibiotic prophylaxis. Renal function remained stable or improved in 30 patients, as verified by normal blood urea and creatinine levels late in the follow-up period, but deteriorated in 2. One of these 2 patients, who had increased creatinine levels before surgery, had only transitory postoperative improvement. This patient developed renal insufficiency 7 years postoperatively and began peritoneal dialysis 11 years after the primary procedure. Another patient had increased blood urea and/or creatinine levels after secondary unilateral ureterocystoneostomy and was using CIC 12 years after the primary surgery. Abdominoplasty resulted in immediate functional and cosmetic improvement compared with the preoperative status (Fig. 2C), with decreased flaccidity and better orthostatic posture in older patients (Fig. 2D). Parental satisfaction in all cases was significant. No wound infection occurred in this series, although 3 patients presented with transient umbilical ischemia. Additional spontaneous gain of abdominal tonus was noted in most patients, but two had recurrent flaccidity; secondary abdominoplasty was performed in one of them. Of the 64 testes taken down to the scrotum, 5 (of 791
TABLE I. Surgical procedures performed in 32 patients with prune belly syndrome Primary Surgery
(n)
Secondary Surgery
(n)
Abdomen Abdominoplasty
28
Primary abdominoplasty Secondary abdominoplasty
3 1
27 19 8 7 3 1
Bilateral pyelostomy Unilateral nephrostomy Ureterocystoneostomy Lumbotomy ⫹ renal biopsy Vesicostomy
1 1 2 1 1
Urinary tract Reductive ureteroplasty Bilateral Unilateral Unilateral nephroureterectomy Unilateral pyeloureteroplasty Ipsilateral ureteral-ureteral anastomosis (duplication) Testes Bilateral orchiopexy No vascular section Unilateral vascular section Bilateral vascular section Other Circumcision
Tertiary Surgery
(n)
Bilateral pyelostomy closure Nephrectomy
1 1
Phalloplasty ⫹ urethroplasty
1
32 26 2 4 14
beral patients developed normal secondary sexual characteristics. COMMENT
FIGURE 2. (A,C) Preoperative and (B,D) postoperative intravenous urography and abdominal aspect after bilateral reductive ureteroplasty with ureterovesical reimplantation and abdominoplasty.
5 patients) atrophied and 5 (of 3 patients) were positioned inguinally. Of those that atrophied, only three had undergone vascular ligature. All pu792
Owing to the relative rarity of PBS, no reports have been published comparing surgical and conservative treatment. Our previous experience with clinical treatment of patients with PBS was disappointing, with frequent urologic complications and a significantly greater rate of renal insufficiency.7 The present work shows that comprehensive surgery, with simultaneous urinary tract reconstruction, orchiopexy, and abdominoplasty, is feasible, even in infants and children with previous diversions, with an acceptable morbidity rate (18.7% early and 15.6% late complications) and no mortality. The reoperation rate owing to these complications was 15.6%. Preoperative unilateral or bilateral hydronephrosis was present in 46.9%, ureteral dilation in 75%, and increased creatinine levels in 21.9% of the patients. Furthermore, 65.6% had VUR and 84.4% presented with recurrent or chronic urinary infection. After the initial surgery and treatment of the complications, 12.5% patients presented with significant pyeloureteral dilation and 9.4% with ureterovesical obstruction (with one kidney lost because of this complication). No patient presented with VUR, and only 12.5% had asymptomatic bacteriuria, which was treated with continuous antibiotic prophylaxis. Late follow-up showed functional and anatomic stabilization of the urinary tract in 93.7% of the patients, with significant improvement in their quality of life; progression to renal failure occurred in only 6.3%. After reductive cystoplasty, some bladders showed UROLOGY 64 (4), 2004
recurrent hypotonicity and low sensitivity, resulting in overflow incontinence and postvoid residual urine volumes. Most of these cases were successfully managed with regular voiding plus Credé and Valsalva maneuvers; only 9.4% of the patients required some form of bladder drainage. Abdominoplasty improved not only the corporal image and self-esteem, but also abdominal strength, with good results in 93.5% of the patients. Upright body posture also improved in most patients, and we believe this was due not only to reinforcement of the abdominal tone but also to a decrease in bladder volume. Although it is difficult to evaluate the cosmetic improvement objectively, this was substantiated by patient and parental satisfaction. With the exception of 1 patient who presented with postoperative pneumonia, no other pulmonary complications occurred in our series. Although Monfort’s technique is an excellent reconstructive technique, our modified abdominoplasty also offers very good cosmetic results, with the added advantage of unobstructed exposure of the abdominal cavity.16
7. Arap S, Dénes FT, Silva FAQ, et al: Le syndrome d’aplasie des muscles de la paroi abdominale antérieure. J Urol Nephrol 3: 161–171, 1978. 8. Snyder HM, Harrison NW, Whitfield HN, et al: Urodynamics in the prune belly syndrome. Br J Urol 48: 663– 670, 1976. 9. Williams DI, and Burkgholder GV: The prune-belly syndrome. J Urol 98: 224 –251, 1967. 10. Reinberg Y, Manivel JC, Pettinato G, et al: Development of renal failure in children with the prune belly syndrome. J Urol 145: 1017–1019, 1991. 11. Woodard JR, and Parrot TS: Reconstruction of the urinary tract in prune belly uropathy. J Urol 119: 824 – 830, 1978. 12. Randolph J: Total surgical reconstruction for patients with abdominal muscular deficiency (“prune-belly”) syndrome. J Pediatr Surg 12: 1033–1043, 1977. 13. Tank ES, and McCoy G: Limited surgical intervention in the prune belly syndrome. J Pediatr Surg 18: 688 – 691, 1983. 14. Duckett JW Jr: Cutaneous vesicostomy in childhood. Urol Clin North Am 1: 485– 495, 1974. 15. Hendren WH: Restoration of function in the severely decompensated ureter, in Johnston JH, and Scholtmejer RJ (Eds): Problems in Paediatric Urology. Amsterdam, Excerpta Medica, 1972, pp 1–56. 16. Monfort G, Guys JM, Bocciardi A, et al: A novel technique for reconstruction of the abdominal wall in the prune belly syndrome. J Urol 145: 639 – 640, 1991.
CONCLUSIONS Early simultaneous correction of PBS abnormalities with individualized urinary reconstruction, abdominoplasty, and orchiopexy is feasible and provides excellent long-term results. Anatomic and functional stabilization of the urinary tract can be achieved with a decreased incidence of urinary tract infection. Early orchiopexy, with or without vascular ligature, provided adequate testicular size and location in most cases. Associated abdominoplasty improved the cosmetic appearance, flaccidity, and posture. In our hands, comprehensive surgery simplified the treatment and decreased the need for long-term follow-up of patients with PBS. REFERENCES 1. Greskovich FJ, and Nyberg LM: The prune belly syndrome: a review of its etiology, defects, treatment and prognosis. J Urol 140: 707–712, 1988. 2. Duckett JW Jr: Prune belly syndrome, in Welch KJ, Randolph JG, and Ravitch MM (Eds): Pediatric Surgery. Chicago, Year Book Medical, 1986, pp 1193–1203. 3. Saldanha LB, Dénes FT, Arap S, et al: Agenesia da musculatura abdominal com atresia uretral completa: estudo necroscópico. Rev Hosp Clin Fac Med S Paulo 32: 186 –189, 1977. 4. Smith EA, and Woodard JR: Prune-belly syndrome, in Walsh PC, Retik AB, Vaughan ED Jr, et al (Eds): Campbell’s Urology. Philadelphia, WB Saunders, 2002, pp 2117–2135. 5. Orvis BR, Bottles K, and Kogan BA: Testicular histology in fetuses with prune belly syndrome and posterior urethral valves. J Urol 139: 335–337, 1988. 6. Woodhouse CR, and Snyder HM III: Testicular and sexual function in adults with prune belly syndrome. J Urol 133: 607– 609, 1985. UROLOGY 64 (4), 2004
EDITORIAL COMMENT The appropriate treatment of patients with PBS has produced controversy between those who advocate early surgical rehabilitation and those who believe that surgical remodeling of the urinary tract should be considered only when required by the patient’s deteriorating clinical course. The resolution of this controversy has been confounded by the rarity and broad variation in the severity of this disorder. Comparisons of the relative merits of these approaches remain based on patient series in which one or the other method has been applied. This report describes the management of a diverse group of patients and cannot fully resolve this debate. However, this large patient series does confirm that a comprehensive surgical approach can yield very good long-term results. Improved upper tract drainage and elimination of reflux in this series translated into less urinary stasis and a lower incidence of urinary tract infection. Of the 32 patients, 30 demonstrated stable or improved renal function at a mean follow-up of 5 years. Of note, these results were achieved only after several patients had undergone multiple procedures. The authors’ approach may have been more aggressive than that applied by others in that 16 of these patients were considered to display category III features. Most often these patients still require orchiopexy but are less likely to require urinary tract reconstruction. Reflux, particularly when associated with urinary tract infection, progressive dilation of the urinary tract, or worsening renal function, would represent the usual indication for intervention and may have emerged in this group. One could argue that all these patients had undergone some interval of observation whether by the authors’ choice or by the age of the referral (mean age at surgery was 23 months). Measures taken to protect against infection before surgery were not defined, although 27 patients were reported to have had significant urinary tract infections. Observation in patients with PBS must take a proactive stance. Circumcision was performed at reconstructive surgery in 14 patients, but might have lowered the incidence of urinary tract infection if applied during early infancy. Antibiotic prophylaxis should 793
COMPREHENSIVE SURGICAL TREATMENT OF PRUNE BELLY SYNDROME: 17 YEARS’ EXPERIENCE WITH 32 PATIENTS FRANCISCO T. DÉNES, MARCO A. ARAP, AMILCAR MARTINS GIRON, FREDERICO A. Q. SILVA, AND SAMI ARAP
ABSTRACT Objectives. To describe the results of comprehensive surgical management of prune belly syndrome. Methods. Thirty-two patients were evaluated and treated for abdominal and genitourinary abnormalities. The surgical procedure, including simultaneous abdominoplasty, bilateral orchiopexy, and urinary tract reconstruction, was performed according to individual needs. At follow-up, the urinary tract, abdominal, and genital anatomy and function were evaluated. Results. The upper urinary tract anatomy and function were stabilized in 30 patients, and no vesicoureteral reflux was found postoperatively. The bladder drainage was adequate in 29 patients, and 4 presented with recurrent bacteriuria. Abdominoplasty improved flaccidity in 29 patients. Adequate testicular position and consistency were obtained in 54 testes. Complications included acute tubular necrosis (1 patient) and ureterovesical obstruction (3 patients). Conclusions. Individualized comprehensive surgical management of prune belly syndrome is effective, with an acceptable incidence of complications and excellent long-term results. UROLOGY 64: 789–794, 2004. © 2004 Elsevier Inc.
P
rune belly syndrome (PBS) has an incidence of 1:35,000 to 1:50,000 live births and presents with three main features: abdominal wall flaccidity, bilateral intra-abdominal cryptorchidism, and urologic abnormalities.1 The abdominal flaccidity may cause several conditions, including lordosis and respiratory infections owing to a lack of abdominal support.1– 4 The testes, located inside the abdomen, are indistinguishable from cryptorchid testes of nonsyndromic patients.5 When untreated, despite adequate testosterone levels, the testes disclose a lack of spermatogenesis after puberty.6 The urinary tract presents with varying degrees of renal dysplasia and ureteropelvic dilation and redundancy, particularly in the distal ureter. Large-capacity bladders are generally present, in association with urachal diverticulum or fistula, and with large postvoid residual volumes.3,7 In a few cases, urethral hypoplasia or atresia further impairs bladder emptyFrom the Division of Urology, University of São Paulo Medical School Hospital, São Paulo, Brazil Reprint requests: Francisco Tibor Dénes, M.D., Division of Urology, University of São Paulo Medical School Hospital, Rua Barata Ribeiro, 414 cj 135, São Paulo 01308-000, Brazil Submitted: February 11, 2004, accepted (with revisions): May 6, 2004 © 2004 ELSEVIER INC. ALL RIGHTS RESERVED
ing.8 Vesicoureteral reflux (VUR) occurs in two thirds of patients.9 The prognosis may depend on the degree of urinary tract stasis or obstruction and infection that impairs renal function later in life.4,10 According to Woodard, patients with PBS are classified into three groups.4 In group 1 (20% of cases), significant renal dysplasia and pulmonary hypoplasia is present, and most patients are stillborn or die shortly after birth. In group 2 (40%), despite many anatomic and functional abnormalities, adequate renal function is present at birth, but may become compromised by obstruction and infection. One third of untreated patients in group 2 die within the first 2 years of renal failure or sepsis. Patients in group 3 (40%) present with mild urinary tract abnormalities, ensuring normal function and survival in most patients.4,11,12 The late prognosis of patients in groups 2 and 3 depends on the occurrence of urinary infection and obstruction. Some investigators recommend nonoperative treatment, assuming that renal function will stabilize with spontaneous improvement of the urinary tract.2,9,13,14 This approach, however, demands close surveillance, and its long-term results have not been homogeneous. Furthermore, it does not address the bilateral cryptorchidism and abdominal flaccidity. 0090-4295/04/$30.00 doi:10.1016/j.urology.2004.05.053 789
Because stabilization is impossible in many patients, other investigators believe that urinary reconstruction that eliminates stasis, corrects VUR, and improves bladder drainage is beneficial.11,15,16 Nevertheless, it is not clear at what age this surgery is appropriate, nor whether it can be performed together with orchiopexy and abdominoplasty. Our therapeutic approach entails a comprehensive procedure that includes individually tailored urinary tract reconstruction, bilateral orchiopexy, and abdominoplasty.12 In this report, we present our long-term results. MATERIAL AND METHODS From 1985 to May 2002, 32 children were prospectively evaluated and treated in our institution. They were classified as having Woodard’s group 2 (16 patients) and 3 (16 patients) PBS. All 32 children had abdominal flaccidity, which was classified as severe in 15, mild in 11, and slight in 6. Of the 32 patients, 27 presented with significant urinary tract infection, which was treated before surgery. Four patients had undergone previous operations elsewhere: three urinary diversions (one bilateral ureterostomy and two vesicostomies) and one failed bilateral orchiopexy. Two patients presented with urachal fistulas and one with megalourethra. All patients underwent functional preoperative and postoperative evaluations with ultrasonography, intravenous urography, radioisotopic renography, and voiding cystourethrography. In addition, patients older than 3 years also underwent urodynamic studies, completing the functional/anatomic evaluation. Renal function was evaluated by the blood urea and creatinine levels. The results were used to compare the patients preoperatively and postoperatively and, therefore, to define treatment failure and success. The postoperative size and location of the testes were examined and compared with age-related nomograms. Because objective criteria to evaluate cosmetic results are rare and difficult to apply, especially when patients present in a wide age range, the abdominal flaccidity and posture were subjectively evaluated by the medical team, the patients, and their parents. Abdominal tonus was determined by observing patients in the upright position, palpating the abdominal wall, and head lifting from the horizontal decubitus.
SURGICAL PROCEDURE An individually tailored longitudinal ellipsoid figure was drawn on the anterior abdomen, according to its flaccidity and redundancy. The skin and subcutaneous layers of this area were removed, with the exception of the umbilicus, which was previously demarcated. The aponeurotic fascia was exposed (Fig. 1A,B). The fascia was then incised using only one unilateral curved paramedian incision in the most redundant side, extending from the upper to the lower extremities of the skin incision. A large retractor was inserted, allowing unobstructed view of the abdominal cavity. The bladder was separated from the abdominal wall, removing the urachal ligament or fistula. The testes, found between the ureteral folds, were dissected, preserving the spermatic vessels, vas deferens and its vessels, and, if possible, the gubernaculum. When an adequate length was obtained, they were left resting (Fig. 1C) until later in the operation. Urinary tract reconstruction was performed as described by Woodard.11 Unilateral nonfunctioning kidneys were treated with nephroureterectomy. In cases of severe ureteral dilation 790
FIGURE 1. Surgical procedure. (A) Demarcation of skin incision and area of abdominoplasty; (B) excision of skin and subcutaneous tissue, preserving aponeurotic fascia and umbilicus; (C) bladder mobilization and dissection of testes and ureters; (D) aponeurotic suture; and (E) closure of abdominal wall, with overlap of aponeurotic flaps. and tortuosity or VUR, the redundant distal ureter was removed and proximal ureter straightened with careful preservation of its blood supply. Previous ureterostomies were closed or removed with the distal ureter. If pyeloureteral obstruction remained, a nondismembered side-to-side pyeloureteral anastomosis could be performed. The vesical dome was then removed with the urachal diverticulum. Over-resection of the bladder must be avoided to ensure adequate capacity after closure. The proximal ureters were reimplanted using tension-free Politano-Leadbetter (bilateral reimplantation) or Paquin-psoas hitch (unilateral reimplantation) techniques. When necessary, ureteral tailoring or infolding, as well as renal mobilization, was performed to ensure optimal ureterocystoneostomy. Percutaneous ureteral stents were left indwelling, exteriorized through the bladder. Orchiopexy was then completed. The inguinal fascia was incised on both sides, and the testes were brought to the scrotum, and fixed in subdartic pouches. If the spermatic vessels UROLOGY 64 (4), 2004
were short, they were sectioned if the paradeferential and gubernacular vessels were still intact. The abdomen was closed by overlapping and suturing the two leaves of the musculoaponeurotic fascia to each other, in a “vest-over” fashion. The intact umbilicus, left in the larger posterior fascia, was exposed through a “buttonhole” in the smaller anterior fascia, where it was sutured (Fig. 1D,E). The skin was approximated according to the initial demarcation. Circumcision was incorporated into the procedure in the last 14 patients. The details of the surgical procedures are presented in Table I.
RESULTS The mean age at surgery was 23 months (range 1 month to 6 years). Preoperatively, 27 patients had clinically significant recurrent urinary tract infection. At surgery, 7 patients had increased urea and creatinine levels, and 7 presented with unilateral nonfunctioning kidneys, with normal contralateral function. Significant ureteral dilation and redundancy were present in 24 patients (Fig. 2A), and 15 presented with massive unilateral or bilateral pyelocaliceal dilation. Twenty-one patients presented with VUR; it was unilateral in 10 and bilateral in 11 (26 units with VUR grade III and IV and 6 with grade I and II). The duration of the reconstructive surgery averaged 255 minutes (range 210 to 345). No intraoperative complications occurred. The mean postoperative follow-up was 60 months (range 13 to 212). Six patients presented with early postoperative complications: urinary tract infection in three, pneumonia in one, acute tubular necrosis (ATN) in one, and increased vesical residue in one. Late complications included two ureterovesical obstructions and three hypotonic bladders. Ten patients underwent 13 repeat operations (Table I). Complications of the primary surgery required repeated operation in 5 patients. In 3 patients, ureterovesical obstruction occurred after Leadbetter-Politano reimplantation without tailoring or infolding; 1 patient underwent a unilateral nephrostomy and later nephroureterectomy, and 1 underwent secondary unilateral ureterocystoneostomy. The third patient presented with anuria after bilateral stent removal and underwent emergency bilateral pyelostomy. After 3 weeks, he resumed spontaneous voiding, and pyelography documented normal ureterovesical flow (the pyelostomies were closed later). In 1 patient with oliguria, exploratory lumbotomy was performed because of suspected urinary extravasation that was not confirmed; renal biopsy revealed ATN that normalized after 2 weeks. Another patient with recurrent vesical retention 1.5 years after the first reconstruction, who was unable to undergo additional clean intermittent catheterization owing to social reasons, underwent secondary vesicostomy. Six elective reoperations, not related to complications, were performed in 6 patients. One patient who had undergone unilateral nephroureterecUROLOGY 64 (4), 2004
tomy in the first operation, developed contralateral VUR 2 years later, and right ureterocystoneostomy was performed. Five years later, penile curvature and megalourethra were also corrected. One patient presented with a right inguinal hernia 4 years after the first procedure, and 3 patients who had not undergone abdominoplasty during the first procedure underwent it later. All patients were alive at last follow-up. Radiographic and ultrasound evaluations revealed a decreased prevalence of ureterohydronephrosis, which was observed in only 8 patients. No postoperative VUR was observed in any patient. Postoperative bladder emptying was normal in 20 patients as verified by ultrasonography and urodynamic studies, including 4 who had not undergone reductive cystoplasty. Nine patients had daytime overflow incontinence but remained dry at night. Urodynamic studies showed decreased bladder sensation with increased capacity and normal compliance. The decreased urinary flow was due to a hypocontractile detrusor, but bladder emptying improved with Credé and Valsalva maneuvers. These patients presented with no upper urinary tract deterioration and were treated with scheduled voiding. Only 3 patients, who had undergone reductive cystoplasty, had persistent large postvoid residual urine volumes; 2 were using CIC at last follow-up, and 1 underwent secondary vesicostomy. Recurrent asymptomatic bacteriuria was present in 4 children, including 2 using CIC; all 4 children received antibiotic prophylaxis. Renal function remained stable or improved in 30 patients, as verified by normal blood urea and creatinine levels late in the follow-up period, but deteriorated in 2. One of these 2 patients, who had increased creatinine levels before surgery, had only transitory postoperative improvement. This patient developed renal insufficiency 7 years postoperatively and began peritoneal dialysis 11 years after the primary procedure. Another patient had increased blood urea and/or creatinine levels after secondary unilateral ureterocystoneostomy and was using CIC 12 years after the primary surgery. Abdominoplasty resulted in immediate functional and cosmetic improvement compared with the preoperative status (Fig. 2C), with decreased flaccidity and better orthostatic posture in older patients (Fig. 2D). Parental satisfaction in all cases was significant. No wound infection occurred in this series, although 3 patients presented with transient umbilical ischemia. Additional spontaneous gain of abdominal tonus was noted in most patients, but two had recurrent flaccidity; secondary abdominoplasty was performed in one of them. Of the 64 testes taken down to the scrotum, 5 (of 791
TABLE I. Surgical procedures performed in 32 patients with prune belly syndrome Primary Surgery
(n)
Secondary Surgery
(n)
Abdomen Abdominoplasty
28
Primary abdominoplasty Secondary abdominoplasty
3 1
27 19 8 7 3 1
Bilateral pyelostomy Unilateral nephrostomy Ureterocystoneostomy Lumbotomy ⫹ renal biopsy Vesicostomy
1 1 2 1 1
Urinary tract Reductive ureteroplasty Bilateral Unilateral Unilateral nephroureterectomy Unilateral pyeloureteroplasty Ipsilateral ureteral-ureteral anastomosis (duplication) Testes Bilateral orchiopexy No vascular section Unilateral vascular section Bilateral vascular section Other Circumcision
Tertiary Surgery
(n)
Bilateral pyelostomy closure Nephrectomy
1 1
Phalloplasty ⫹ urethroplasty
1
32 26 2 4 14
beral patients developed normal secondary sexual characteristics. COMMENT
FIGURE 2. (A,C) Preoperative and (B,D) postoperative intravenous urography and abdominal aspect after bilateral reductive ureteroplasty with ureterovesical reimplantation and abdominoplasty.
5 patients) atrophied and 5 (of 3 patients) were positioned inguinally. Of those that atrophied, only three had undergone vascular ligature. All pu792
Owing to the relative rarity of PBS, no reports have been published comparing surgical and conservative treatment. Our previous experience with clinical treatment of patients with PBS was disappointing, with frequent urologic complications and a significantly greater rate of renal insufficiency.7 The present work shows that comprehensive surgery, with simultaneous urinary tract reconstruction, orchiopexy, and abdominoplasty, is feasible, even in infants and children with previous diversions, with an acceptable morbidity rate (18.7% early and 15.6% late complications) and no mortality. The reoperation rate owing to these complications was 15.6%. Preoperative unilateral or bilateral hydronephrosis was present in 46.9%, ureteral dilation in 75%, and increased creatinine levels in 21.9% of the patients. Furthermore, 65.6% had VUR and 84.4% presented with recurrent or chronic urinary infection. After the initial surgery and treatment of the complications, 12.5% patients presented with significant pyeloureteral dilation and 9.4% with ureterovesical obstruction (with one kidney lost because of this complication). No patient presented with VUR, and only 12.5% had asymptomatic bacteriuria, which was treated with continuous antibiotic prophylaxis. Late follow-up showed functional and anatomic stabilization of the urinary tract in 93.7% of the patients, with significant improvement in their quality of life; progression to renal failure occurred in only 6.3%. After reductive cystoplasty, some bladders showed UROLOGY 64 (4), 2004
recurrent hypotonicity and low sensitivity, resulting in overflow incontinence and postvoid residual urine volumes. Most of these cases were successfully managed with regular voiding plus Credé and Valsalva maneuvers; only 9.4% of the patients required some form of bladder drainage. Abdominoplasty improved not only the corporal image and self-esteem, but also abdominal strength, with good results in 93.5% of the patients. Upright body posture also improved in most patients, and we believe this was due not only to reinforcement of the abdominal tone but also to a decrease in bladder volume. Although it is difficult to evaluate the cosmetic improvement objectively, this was substantiated by patient and parental satisfaction. With the exception of 1 patient who presented with postoperative pneumonia, no other pulmonary complications occurred in our series. Although Monfort’s technique is an excellent reconstructive technique, our modified abdominoplasty also offers very good cosmetic results, with the added advantage of unobstructed exposure of the abdominal cavity.16
7. Arap S, Dénes FT, Silva FAQ, et al: Le syndrome d’aplasie des muscles de la paroi abdominale antérieure. J Urol Nephrol 3: 161–171, 1978. 8. Snyder HM, Harrison NW, Whitfield HN, et al: Urodynamics in the prune belly syndrome. Br J Urol 48: 663– 670, 1976. 9. Williams DI, and Burkgholder GV: The prune-belly syndrome. J Urol 98: 224 –251, 1967. 10. Reinberg Y, Manivel JC, Pettinato G, et al: Development of renal failure in children with the prune belly syndrome. J Urol 145: 1017–1019, 1991. 11. Woodard JR, and Parrot TS: Reconstruction of the urinary tract in prune belly uropathy. J Urol 119: 824 – 830, 1978. 12. Randolph J: Total surgical reconstruction for patients with abdominal muscular deficiency (“prune-belly”) syndrome. J Pediatr Surg 12: 1033–1043, 1977. 13. Tank ES, and McCoy G: Limited surgical intervention in the prune belly syndrome. J Pediatr Surg 18: 688 – 691, 1983. 14. Duckett JW Jr: Cutaneous vesicostomy in childhood. Urol Clin North Am 1: 485– 495, 1974. 15. Hendren WH: Restoration of function in the severely decompensated ureter, in Johnston JH, and Scholtmejer RJ (Eds): Problems in Paediatric Urology. Amsterdam, Excerpta Medica, 1972, pp 1–56. 16. Monfort G, Guys JM, Bocciardi A, et al: A novel technique for reconstruction of the abdominal wall in the prune belly syndrome. J Urol 145: 639 – 640, 1991.
CONCLUSIONS Early simultaneous correction of PBS abnormalities with individualized urinary reconstruction, abdominoplasty, and orchiopexy is feasible and provides excellent long-term results. Anatomic and functional stabilization of the urinary tract can be achieved with a decreased incidence of urinary tract infection. Early orchiopexy, with or without vascular ligature, provided adequate testicular size and location in most cases. Associated abdominoplasty improved the cosmetic appearance, flaccidity, and posture. In our hands, comprehensive surgery simplified the treatment and decreased the need for long-term follow-up of patients with PBS. REFERENCES 1. Greskovich FJ, and Nyberg LM: The prune belly syndrome: a review of its etiology, defects, treatment and prognosis. J Urol 140: 707–712, 1988. 2. Duckett JW Jr: Prune belly syndrome, in Welch KJ, Randolph JG, and Ravitch MM (Eds): Pediatric Surgery. Chicago, Year Book Medical, 1986, pp 1193–1203. 3. Saldanha LB, Dénes FT, Arap S, et al: Agenesia da musculatura abdominal com atresia uretral completa: estudo necroscópico. Rev Hosp Clin Fac Med S Paulo 32: 186 –189, 1977. 4. Smith EA, and Woodard JR: Prune-belly syndrome, in Walsh PC, Retik AB, Vaughan ED Jr, et al (Eds): Campbell’s Urology. Philadelphia, WB Saunders, 2002, pp 2117–2135. 5. Orvis BR, Bottles K, and Kogan BA: Testicular histology in fetuses with prune belly syndrome and posterior urethral valves. J Urol 139: 335–337, 1988. 6. Woodhouse CR, and Snyder HM III: Testicular and sexual function in adults with prune belly syndrome. J Urol 133: 607– 609, 1985. UROLOGY 64 (4), 2004
EDITORIAL COMMENT The appropriate treatment of patients with PBS has produced controversy between those who advocate early surgical rehabilitation and those who believe that surgical remodeling of the urinary tract should be considered only when required by the patient’s deteriorating clinical course. The resolution of this controversy has been confounded by the rarity and broad variation in the severity of this disorder. Comparisons of the relative merits of these approaches remain based on patient series in which one or the other method has been applied. This report describes the management of a diverse group of patients and cannot fully resolve this debate. However, this large patient series does confirm that a comprehensive surgical approach can yield very good long-term results. Improved upper tract drainage and elimination of reflux in this series translated into less urinary stasis and a lower incidence of urinary tract infection. Of the 32 patients, 30 demonstrated stable or improved renal function at a mean follow-up of 5 years. Of note, these results were achieved only after several patients had undergone multiple procedures. The authors’ approach may have been more aggressive than that applied by others in that 16 of these patients were considered to display category III features. Most often these patients still require orchiopexy but are less likely to require urinary tract reconstruction. Reflux, particularly when associated with urinary tract infection, progressive dilation of the urinary tract, or worsening renal function, would represent the usual indication for intervention and may have emerged in this group. One could argue that all these patients had undergone some interval of observation whether by the authors’ choice or by the age of the referral (mean age at surgery was 23 months). Measures taken to protect against infection before surgery were not defined, although 27 patients were reported to have had significant urinary tract infections. Observation in patients with PBS must take a proactive stance. Circumcision was performed at reconstructive surgery in 14 patients, but might have lowered the incidence of urinary tract infection if applied during early infancy. Antibiotic prophylaxis should 793