eMedicine Specialties > Urology > Congenital Urologic Conditions
Prune Belly Syndrome: Treatment
Updated: Oct 29, 2009
Treatment
Medical Therapy
Children with prune belly syndrome who have reflux should receive maintenance suppressive antibiotics. Some believe that surgical intervention is not warranted in these patients as long as they remain free of infection. Once infections start occurring frequently, the reflux should be corrected.
Management of the undescended testis with human chorionic gonadotropin (hCG) is not warranted in these cases. The literature clearly shows that hCG for the management of intra-abdominal testis is useless.
Surgical Therapy
Undescended testis
Management of undescended testis in a patient with prune belly syndrome should be left to the experienced pediatric urologist. In some instances, patients with undescended testis do not require any urologic work and the testis can be brought down using laparoscopic techniques. The laparoscopic technique reduces the morbidity associated with intra-abdominal surgery. The author has performed reconstructive surgery in several patients using this technique, with superior results. In other cases, the testis can be brought down during open surgery for the reconstruction of the urinary tract.
Abdominal wall reconstruction
Abdominal wall reconstruction is performed in most patients with prune belly syndrome to improve respiratory function and to improve cosmesis. Several innovations in abdominal wall reconstruction have been developed. The Monfort5 and Ehrlich6 variations of the Randolph operation have improved results, with decreased morbidity and the ability to preserve the umbilicus. Furness et al have described a new technique that allows for improved results over other techniques, without opening the abdominal cavity.7 Most recently, Franco modified this technique further to obtain even better results by using laparoscopic guidance to ensure that wall tension and cosmetic results persist postoperatively.8 These innovations have significantly reduced the morbidity of abdominal wall reconstruction.
Placement of a percutaneous nephrostomy
Obstruction at the ureteropelvic junction (UPJ) has been observed in patients with prune belly syndrome. In some cases, diagnosing this can be difficult; however, the diagnosis can be confirmed with the placement of a percutaneous nephrostomy. This procedure can be performed under ultrasound guidance with relative ease in a dilated system and provides the opportunity to perform a renal biopsy, which could help with the later management of the urinary tract.
Placement of a percutaneous nephrostomy provides the surgeon with several options. The surgeon can confirm the diagnosis of UPJ obstruction. The urinary tract can be decompressed in an ill child or in a child who is too small to safely undergo reconstructive surgery at the time. Finally, the technique also allows the surgeon to evaluate the renal function of the obstructed unit.
Standard pyeloplasty
If conditions are right and the child is stable, a standard pyeloplasty can be performed as another means of treating the obstructed UPJ. The surgeon should be meticulous in the dissection of the upper ureter. The proximal ureteral blood supply should be preserved as much as possible in case ureteral tapering or reimplantation becomes necessary later.
Recent studies indicate that the more distal lower ureter is abnormal in several ways, while the more proximal portion of the ureter is more anatomically normal. Histologically, the ureter has a smooth muscle deficiency with fibrous degeneration and a poor blood supply. In addition, a decrease in nerve plexuses is reported, with irregularity in degeneration of nonmyelinated Schwann fibers.
In patients who may have supravesical obstruction and functional vesical obstruction, cutaneous pyelostomy has been recommended as the preferred means of diverting the ureter and the pelvis for several reasons. The proximal ureter is believed to less compromised with this procedure than with a high-loop ureterostomy, as had been performed previously. In addition, by performing a pyelostomy, surgeons avoid the undesirable attachment of the ureters to the abdominal wall, thereby preventing the common problem of a prolapsed ureterostomy. When ureterostomy is performed, the distal ureter is typically used because this ureter would most likely be discarded when the reconstruction is performed.
Cutaneous pyelostomy still carries the risk of resultant scarring from excessive dissection, which may be required to take down the pyelostomy at a later stage. At the time of reconstruction, ureteral tapering or reimplantation that is necessary creates a difficult situation. Recently, these problems have been overcome with the increasing use of percutaneous nephrostomy drainage and with the use of vesicostomy as a means of draining the upper tracts. Some believe that vesicostomy can decompress the upper tracts just as well as high diversion. Subsequently, since the introduction of these 2 procedures, the use of high diversion has declined significantly over the last few years, and reconstruction in these patients has become easier.
Infravesical obstruction or obstruction at the prostatic urethra
Patients with documented obstruction can be treated with several means.
Blocksom vesicostomy performed in the early newborn period is the simplest and best treatment in patients with documented obstruction. Bringing the dome of the bladder out to the skin is essential when creating the vesicostomy; this approach prevents the resultant herniation of the bladder in an improperly created vesicostomy.
Herniation of the bladder is quite prevalent in patients with prune belly syndrome because the bladder is quite large and redundant. A patent urachus can be found frequently in patients with urethral obstruction. The patent urachus is the means by which patients are able to survive; early deaths usually are observed in patients with urethral obstruction without a patent urachus. In these patients, once a vesicostomy is performed, the surgeon should try to identify the urachus and ligate it at that time.
Some patients with prune belly syndrome have posterior urethral valves, and the valves are best managed with transurethral resection. Some advocate the use of sphincterotomy, transurethral resection of the bladder neck, or internal urethrotomy to manage the functionally obstructed system. Snyder and associates believe that judicious use of urethrotomy can lower urethral resistance and improve voiding dynamics without causing incontinence.3 Opinions are mixed regarding the use of these modalities because few actual results have been seen. Passerini-Glazel et al used soft catheter dilatation to dilate the functionally obstructed urethra in patients with prune belly syndrome.9 They left soft indwelling catheters for extended periods while they gradually increased the size of the catheter until they achieved the desired caliber. Radiologic and functional results are needed.
Ureteral reimplantation in patients with megaureters, reflux, and a ureteral transplant requires an aggressive approach; as much of the abnormal distal ureter as possible should be removed and the blood supply to the proximal ureter should be preserved. Some authors advocate shortening, tapering, and reimplanting the better of the 2 ureters with a long tunnel and psoas hitch and draining the contralateral side with a transureteroureterostomy (TUU). These authors reason that the bladder may be thick and fibrous in patients with prune belly syndrome, thereby complicating placement 2 good long reimplants. Ureters are preferentially tapered over an 8F or 10F red rubber catheter and excess tissue is excised. Most recently, Starr has reimplanted the ureter using the folding cross-reimplantation. He has not reported obstruction or reflux. This technique may prevent interruption of the critical blood supply to the distal ureter; this is not possible with traditional excisions.
In patients with megacystitis, reduction cystoplasty has met with mixed reviews. Reduction cystoplasty is generally recognized to be unnecessary as a primary procedure but may be useful and is frequently performed at the time of ureteral reimplantation and tapering.
Preoperative Details
Preoperatively, all patients should have stable respiration. This is critical because postoperative respiratory infections pose a serious risk to these patients.
The urine should be sterile prior to surgery.
In patients undergoing abdominal wall reconstruction, the presence of a peg gastrostomy precludes the safe reconstruction of the abdominal wall. The author prefers to not have a gastrostomy in place before abdominal wall reconstruction.
Intraoperative Details
The most critical factor that must be watched during surgery in patients with prune belly syndrome is airway pressure during abdominal wall reconstruction. If the abdomen is tightened too much, the patient may not be able to breathe spontaneously after surgery. Airway pressures should be noted early in treatment, and these pressures should be maintained while the abdomen is closed.
During tapering of the ureter, careful dissection of the blood supply of the ureter is essential.
During laparoscopic surgery, the trocars need to be held in place with sutures to anchor them to the abdominal wall to prevent slippage and increased carbon dioxide loss. In addition, the umbilical trocar should be placed on the superior margin of the umbilicus to prevent penetration of the bladder or the urachal remnant.
Postoperative Details
Postoperatively, these patients must be monitored closely. They can easily become fatigued and may experience respiratory arrest. Judicious use of narcotics is critical, and the use of ketorolac (Toradol) is recommended for postoperative pain control. The author has also used postoperative epidural anesthesia, with excellent results.
Follow-up
Follow-up care depends on the procedure performed.
Complications
Undescended testis
The most obvious complication of orchidopexy is incomplete descent of the testis. Atrophy of the testis is another possible complication. This can occur in some cases (as many as 50%) of Fowler-Stephens orchidopexy. For this reason, the author does not suggest performing simultaneous Fowler-Stephens orchidopexies.
Abdominal wall reconstruction
After reconstruction, persistent laxity of the abdominal wall is a concern, regardless of the technique used. Using the laparoscope to perform the abdominal wall plication significantly reduces the risk of this complication.
The abdominal wall skin can slough when the skin flaps are raised. Care should be taken to avoid incisions that cut the blood supply to the flaps.
Ureteral reimplants
Ureteral strictures are not uncommon in tapered reimplants of ureters in patients with prune belly syndrome. This can be avoided using Kaliscinski-type tapering of the ureters.
More on Prune Belly Syndrome |
| Overview: Prune Belly Syndrome |
| Workup: Prune Belly Syndrome |
 Treatment: Prune Belly Syndrome |
| Follow-up: Prune Belly Syndrome |
| Multimedia: Prune Belly Syndrome |
| References |
| « Previous Page | Next Page » |
References
Bellah RD, States LJ, Duckett JW. Pseudoprune-Belly syndrome: imaging findings and clinical outcome. AJR Am J Roentgenol. Dec 1996;167(6):1389-93. [Medline].
Waldbaum RS, Marshall VF. The prune belly syndrome: a diagnostic therapeutic plan. J Urol. May 1970;103(5):668-74. [Medline].
Snyder HM, Harrison NW, Whitfield HN, Williams INNES. Urodynamics in the prune belly syndrome. Br J Urol. 1976;48(7):663-70. [Medline].
Alhawsawi AM, Aljiffry M, Walsh MJ, Peltekian K, Molinari M. Hepatic artery aneurysm associated with prune belly syndrome: a case report and review of the literature. J Surg Educ. Jan-Feb 2009;66(1):43-7. [Medline].
Monfort G, Guys JM, Bocciardi A, Coquet M, Chevallier D. A novel technique for reconstruction of the abdominal wall in the prune belly syndrome. J Urol. Aug 1991;146(2 ( Pt 2)):639-40. [Medline].
Ehrlich RM, Lesavoy MA. Umbilicus preservation with total abdominal wall reconstruction in prune-belly syndrome. Urology. Mar 1993;41(3):231-2. [Medline].
Furness PD 3rd, Cheng EY, Franco I, Firlit CF. The prune-belly syndrome: a new and simplified technique of abdominal wall reconstruction. J Urol. Sep 1998;160(3 Pt 2):1195-7; discussion 1216. [Medline].
Franco I. Laparoscopic assisted modification of the firlit abdominal wall plication. J Urol. Jul 2005;174(1):280-3. [Medline].
Passerini-Glazel G, Araguna F, Chiozza L, Artibani W, Rabinowitz R, Firlit CF. The P.A.D.U.A. (progressive augmentation by dilating the urethra anterior) procedure for the treatment of severe urethral hypoplasia. J Urol. Nov 1988;140(5 Pt 2):1247-9. [Medline].
Woodhouse CR, Kellett MJ, Williams DI. Minimal surgical interference in the prune belly syndrome. Br J Urol. Dec 1979;51(6):475-80. [Medline].
Massad CA, Cohen MB, Kogan BA, Beckstead JH. Morphology and histochemistry of infant testes in the prune belly syndrome. J Urol. Dec 1991;146(6):1598-600. [Medline].
Woodard JR, Parrott TS. Orchiopexy in the prune belly syndrome. Br J Urol. Aug 1978;50(5):348-51. [Medline].
Smith CA, Smith EA, Parrott TS, Broecker BH, Woodard JR. Voiding function in patients with the prune-belly syndrome after Monfort abdominoplasty. J Urol. May 1998;159(5):1675-9. [Medline].
Woodard JR, Parrott TS. Reconstruction of the urinary tract in prune belly uropathy. J Urol. Jun 1978;119(6):824-8. [Medline].
Noh PH, Cooper CS, Winkler AC, Zderic SA, Snyder HM 3rd, Canning DA. Prognostic factors for long-term renal function in boys with the prune-belly syndrome. J Urol. Oct 1999;162(4):1399-401. [Medline].
Biard JM, Johnson MP, Carr MC, Wilson RD, Hedrick HL, Pavlock C, et al. Long-term outcomes in children treated by prenatal vesicoamniotic shunting for lower urinary tract obstruction. Obstet Gynecol. Sep 2005;106(3):503-8. [Medline].
Duckett J, Knutrud O, Hohenfellner R. Prune Belly Syndrome. J Urol. 1980;3:9.
Ehrlich RM. The ureteral folding technique for megaureter surgery. J Urol. Oct 1985;134(4):668-70. [Medline].
Fallat ME, Skoog SJ, Belman AB, Eng G, Randolph JG. The prune belly syndrome: a comprehensive approach to management. J Urol. Sep 1989;142(3):802-5. [Medline].
Greskovich FJ 3rd, Nyberg LM Jr. The prune belly syndrome: a review of its etiology, defects, treatment and prognosis. J Urol. Oct 1988;140(4):707-12. [Medline].
Levitt S, Franco I. Overview: surgery for prune belly Syndrome. In: Whitehead ED, ed. Current Operative Urology. Philadelphia, Pa: JB Lippincott; 1992:157.
Lindgren BW, Franco I, Blick S, Levitt SB, Brock WA, Palmer LS, et al. Laparoscopic Fowler-Stephens orchiopexy for the high abdominal testis. J Urol. Sep 1999;162(3 Pt 2):990-3; discussion 994. [Medline].
Nunn IN, Stephens FD. The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J Urol. Dec 1961;86:782-94. [Medline].
Palmtag H, Dreikorn K, Röhl L. Urodynamic studies in prune belly syndrome. A case report. Scand J Urol Nephrol. 1979;13(1):11-6. [Medline].
Perlmutter AD. Reduction cystoplasty in prune belly syndrome. J Urol. Sep 1976;116(3):356-62. [Medline].
Saxena AK, Brinkmann OA. Unique features of prune belly syndrome in laparoscopic surgery. J Am Coll Surg. Aug 2007;205(2):217-21. [Medline].
Stephens FD. Four valves in prune belly syndrome. In: Congenital Malformations of the Urinary Tract. Philadelphia, Pa: Praeger Press; 1983.
Tank ES, McCoy G. Limited surgical intervention in the prune belly syndrome. J Pediatr Surg. Dec 1983;18(6):688-91. [Medline].
Weiser A, Schoor RA, Franco I. Annual Meeting Section of Urology of American Academy of Pediatrics,. In: Health Related Quality of Life, Social and Sexual Function In Patients with Prune Belly Syndrome. Nov. 2003.
Woodard JR. The prune belly syndrome. Urol Clin North Am. Feb 1978;5(1):75-93. [Medline].
Further Reading
Keywords
prune belly syndrome, PBS, Eagle-Barrett syndrome, triad syndrome, undescended testis, undescended testes, abdominal wall reconstruction, percutaneous nephrostomy, standard pyeloplasty, infravesical obstruction, obstruction at the prostatic urethra, trisomy 18, trisomy 21, tetralogy of Fallot, TF, ventriculoseptal defect, ventricular septal defect
