Prune Belly Syndrome Treatment & Management

Updated: Jan 17, 2020
  • Author: Israel Franco, MD, FAAP, FACS; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Treatment

Approach Considerations

Urethral obstruction should be addressed as soon as it is recognized. Progressive urethral dilation, as described by Passerini-Glazel et al, [17] is the preferred method of treatment. Vesicostomy can also be an effective method of temporary diversion.

The literature contains little controversy regarding management of undescended testes in these children. [12, 18, 19] If controversy or confusion arises, it concerns the optimal timing for the orchidopexy and which type of orchidopexy should be performed.

The management of the abdominal wall abnormalities is somewhat controversial. Some prefer no surgical reconstruction of the abdominal wall, while others advocate surgical reconstruction. [20, 21, 22, 23, 24, 25] The group that advocates surgical reconstruction is divided into 2 separate factions that support different techniques of abdominal wall reconstruction. The use of a rectus muscle transfer flap to the abdominal wall combined with an abdominal wall plication procedure can help ameliorate some of the back strain in many of these patients.

Reconstruction of the urinary tract also is controversial; some advocate prompt reconstruction, while others advocate a more passive approach and operate when necessary. Prune belly syndrome is characterized by elongated, dilated, and tortuous megaureters, which affect 81% of patients. Vesicoureteral reflux is common in these patients. Advocates of conservative treatment argue that with current medical therapy, intervention is not necessary in patients with a nonobstructed system. Those who argue for aggressive therapy in patients with prune belly syndrome point to Waldbaum and Marshall's review of 56 patients, in which 70% died and 16% were gravely ill. [26] Waldbaum and Marshall also argue for aggressive therapy when they point out that, "the purpose of the upper tract is to transport urine, not to store it."

Even with aggressive antibiotic therapy, urinary stasis and repeat infections may lead to inevitable loss of renal function. Regardless, ureteral reimplantation in patients with prune belly syndrome can be difficult and is frequently fraught with complications. To attest to this, the author has seen several patients who have previously undergone reimplantation but then developed ureteral strictures of the distal reimplanted ureters and required another reimplantation. Furthermore, reports in the literature have documented ureteral stenosis rates of up to 40% in tapered ureters.

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Medical Therapy

Children with prune belly syndrome who have vesicoureteral reflux should receive maintenance suppressive antibiotics. Some urologists 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. [27]

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Surgical Therapy

Surgical treatment for prune belly syndrome includes repair of the abdominal wall and urinary tract abnormalities and correction of cryptorchidism. These procedures can be performed in a single comprehensive approach or in multiple steps. A review by Lopes et al concluded that comprehensive surgical treatment is feasible and has good long-term results. However, many patients require reoperations because of complications or progression of disease. [28]

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 that significantly reduced the morbidity of abdominal wall reconstruction. [25]  Those include the following:

  • The Monfort [20] and Ehrlich [21] variations of the Randolph operation provided decreased morbidity and the ability to preserve the umbilicus.
  • Furness et al described a technique that allows for improved results over other techniques, without opening the abdominal cavity. [22]
  • Franco modified the technique further to obtain even better results by using laparoscopic guidance to ensure that wall tension and cosmetic results persist postoperatively. [23]  
  • Smith et al further modified the Monfort technique to address variable patterns of abdominal wall weakness. [24]   

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 kidney biopsy, which could help with the later management of the urinary tract.

Placement of a percutaneous nephrostomy allows the surgeon to do the following: 

  • Confirm the diagnosis of UPJ obstruction
  • Decompress the urinary tract in a child who is too ill or too small to safely undergo reconstructive surgery at the time.
  • Evaluate the function of the obstructed kidney

Standard pyeloplasty

If conditions are right and the child is stable, a standard pyeloplasty can be performed as another means of treating UPJ obstruction. 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.

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. However, 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. Since the introduction of these 2 procedures, the use of high diversion has declined significantly, 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 techniques.

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. [7] 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. [17] 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 of 2 good long reimplants. Ureters are preferentially tapered over an 8F or 10F red rubber catheter and excess tissue is excised.

In the Starr technique of ureteral imbrication, the excess ureteral tissue is folded over a 10F or 12F catheter as a guide. This technique may prevent interruption of the critical blood supply to the distal ureter, which is not possible with traditional excisions.

In patients with megacystitis, reduction cystoplasty has met with mixed reviews. Some authors posit that the shape of the bladder in patients with prune belly syndrome can lead to unbalanced voiding and that reduction cystoplasty could improve detrusor force, because a decreased bladder capacity would lead to increased detrusor efficiency based on Laplace's law. 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.

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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 percutaneous endoscopic gastrostomy (PEG) precludes the safe reconstruction of the abdominal wall. The author prefers to not have a gastrostomy in place before abdominal wall reconstruction.

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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.

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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; instead, the use of ketorolac (Toradol) is recommended for postoperative pain control. The author has also used postoperative epidural anesthesia with excellent results.

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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. [18, 19]

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. [25]

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 Kalicinski-type tapering of the ureters.

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Future and Controversies

Further advances in laparoscopic surgery may continue to improve the outlook for patients with prune belly syndrome. As more is learned about the natural history of obstructive uropathy, treatment of this condition will be adjusted to offer the most benefit with the least amount of intervention. Whether to institute aggressive early intervention or no intervention will continue to be debated until the true pathophysiology of prune belly syndrome is completely understood.

The fact that a decreasing number of patients are being born with prune belly syndrome indicates an ever-increasing ability to diagnose the disease in utero. Prenatal intervention with vesicoamniotic shunting has had little effect in reducing the incidence of renal failure and the need for transplantation in this group of patients. [29]  

Expertise in treatment will eventually be limited to only a small number of experts. In the future, parents of patients with prune belly syndrome will need to consider obtaining care from such specialized centers.

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