Surgery for Pediatric Bladder Anomalies Treatment & Management

Updated: May 08, 2018
  • Author: Bartley G Cilento, Jr, MD; Chief Editor: Marc Cendron, MD  more...
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Treatment

Medical Care

With bladder diverticula, bladder outlet obstruction should be corrected if present because many bladder diverticula resolve spontaneously after relief of obstruction. Congenital diverticula are usually removed surgically.

Urachal sinus can be observed in the first 4-8 weeks of life. For small asymptomatic urachal cysts discovered incidentally, observation with serial ultrasonography (US) may be appropriate. A patent urachus can generally be observed in the first few months of life because some cases may resolve spontaneously. Persistence after 2 months warrants surgical correction. If bladder outlet obstruction is present, it should be corrected first because it may be the cause of the persistent urachal patency. Most patients with a vericourachal diverticulum do not require treatment.

No treatment is necessary for bladder ears, because nearly all cases resolve spontaneously.

Bladder agenesis must be treated by means of urinary diversion and subsequent reconstruction (eg, continent urinary reservoir).

With megacystis, clean intermittent catheterization (CIC) may be considered in patients who have incomplete bladder emptying or frequent urinary tract infections (UTIs). In children in whom CIC is instituted, urodynamic studies should be obtained. (See Surgical Care.)

Incomplete bladder duplication often need not be treated when the upper urinary tracts are normal and the bladder empties completely. If other associated anomalies are present, they should be addressed individually. (See Surgical Care.)

For bladder septation, depending on the anatomy, no treatment may be appropriate; an example would be a small incomplete septation with normal upper tracts and complete bladder emptying.

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Surgical Care

Patients with bladder diverticula generally require surgery when the diverticula are causing obstruction, recurrent UTIs, vesicoureteral reflux (VUR), or stone formation. [19, 20] If diverticula have arisen secondary to obstruction, the outlet obstruction should be corrected first because some of these diverticula spontaneously resolve with relief of the obstruction.

Surgical diverticulectomy for congenital diverticula may be approached extravesically or intravesically. The surgical approach varies, depending on lesion size and location, associated anomalies (eg, VUR), and individual surgeon preferences. In very large paraureteral diverticula, care must be taken to avoid injuring adjacent structures (eg, ureter or vas deferens).

A robot-assisted laparoscopic approach to bladder diverticulectomy has been described. [21, 6]  Marte et al, in a series of 16 male children (age range, 4-12 years) with symptomatic bladder diverticula, found vesicoscopic diverticulectomy to be safe and effective. [22]

In the presence of ureteral obstruction, renal function dictates management. If the associated kidney has little or no function, a nephrectomy with bladder diverticulectomy is performed. If kidney function is adequate, diverticulectomy and ureteral reimplantation are indicated.

Surgical correction of a urachal sinus involves complete removal of the urachus, from the umbilicus to the dome of the bladder. In an infant or child, this can be approached easily via a Pfannenstiel incision. In an infant, the distance from the dome of the bladder to the base of the umbilicus is very short. Complete removal eliminates any further problems. Surgical removal should be planned after adequate treatment of the infection because intraperitoneal structures may adhere to the urachus in the inflammatory process. [23]

After adequate treatment of an infected urachal cyst, surgical removal of the entire urachus is warranted. A Pfannenstiel incision can be used in an infant or child. Again, in an infant, the distance from the dome of the bladder to the base of the umbilicus is very short. Complete removal eliminates any further problems. [24, 25]

As is the case with a urachal cyst, complete removal of a patent urachus using the described technique eliminates any further problems.

Surgical removal of a vesicourachal diverticulum is reserved for large symptomatic diverticula causing recurrent UTIs, stones, or poor emptying.

Patients with bladder ears do not require surgical treatment, because nearly all cases resolve spontaneously.

Initial treatment of bladder agenesis consists of urinary diversion. Complex urinary reconstruction (eg, creation of a continent urinary reservoir) may be undertaken later in life.

In patients with megacystis, the massive VUR is corrected by ureteral reimplantation, which usually involves tapering the ureters at the time of reimplantation. Correction of megacystis by means of reduction cystoplasty often is not necessary; correction of the reflux alone may suffice. If recurrent UTIs or incomplete bladder emptying occur after reimplantation, reduction cystoplasty may be considered. In rare cases, vesicostomy can be performed as a temporary measure in children with megacystis and severe VUR until a more formal reconstruction can be performed.

Complete bladder duplication has a much higher incidence of associated anomalies necessitating surgical correction, such as fistulas between the urethra and adjacent structures. The variable anatomy of each case dictates the surgical approach. A combined robotic-open approach was described by Bowen et al in a patient with bladder duplication and urethral triplication. [26]

Surgical treatment of bladder septation is dictated by the individual patient's anatomy. The goals are to relieve bladder and upper-tract obstruction.

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Complications

Complications are related to persistent obstruction or recurrent UTIs—namely, renal insufficiency or failure. If the obstruction is corrected and urinary drainage is unimpeded, long-term complications are minimal.

In the case of bladder agenesis, the long-term complications are related to those specific to urinary diversion, continent reservoir, or both. These include ureteral stricture at the junction of the ureter and reservoir, reservoir stones, recurrent UTI, stomal stenosis of the catheterizable segment, and metabolic disturbances relative to the intestinal segment used.

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Diet

Generally, no dietary restrictions are needed for the various bladder anomalies. In cases of significant renal insufficiency, the nephrologist may impose certain dietary restrictions, such as limited protein intake.

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Consultations

For all of the bladder anomalies, consultation with other professionals is dictated primarily by the presence of the associated anomalies. In situations of renal insufficiency with the various obstructive entities (eg, posterior urethral valves, neurogenic bladder), consultation with a nephrologist is advisable.

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Long-Term Monitoring

For bladder diverticula, no follow-up care is required after surgical removal. Small diverticula that are treated by observation alone may be annually monitored with renal and bladder US.

No follow-up care is required after surgical removal of a urachal sinus, a urachal cyst, or a patent urachus. If a small urachal cyst is to be monitored, yearly US studies are indicated. No follow-up care is necessary for a vesicourachal diverticulum.

No follow-up care is necessary for bladder ears.

For bladder agenesis, frequent outpatient follow-up visits are necessary after urinary diversion. Initially, outpatient follow-up care may occur as often as monthly. Once stability has been achieved, follow-up visits usually are annual. Evaluation with serial renal US is performed to assess renal growth and the presence or absence of hydronephrosis. Conduit US can detect the presence of stones. Serum electrolyte and creatinine determinations help detect metabolic disturbances and renal insufficiency.

For megacystis, yearly evaluation with renal and bladder US may be appropriate until just after the toilet training period to monitor the upper tracts and ensure adequate bladder emptying. Similar evaluation is also appropriate for bladder duplication and bladder septation.

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