eMedicine Specialties > Pediatrics: Surgery > Urology

Posterior Urethral Valves: Follow-up

Author: Martin David Bomalaski, MD, FAAP, Pediatric Urologist, Alaska Southcentral Urology Specialists
Contributor Information and Disclosures

Updated: Jun 12, 2008

Follow-up

Further Outpatient Care

Posterior urethral valves (PUVs) represent a lifelong disorder that can have a profound effect on the entire urinary tract. As such, patients need periodic long-term urologic follow-up care. The status of the kidneys determines the need for additional specialty follow-up care (eg, with a pediatric nephrologist).

• Resolution of obstruction: Relief of bladder outlet obstruction is the first step in treatment. After incision of the valves, a repeat VCUG or repeat cystoscopy 1-3 months later confirms valve resolution and urethral healing. These patients may also be at risk of subsequent urethral stricture formation; repeat these studies at any point in the future if any recurrent bladder outlet obstruction is indicated.
• Urodynamics: Chronic changes, which can lead to elevated intravesical pressures, may occur in the bladder of patients with PUV. This leads to upper tract changes, urinary incontinence, and recurrent UTI. These patients may need periodic urodynamic studies to determine bladder capacity, compliance, and postvoid residual urine volumes. In older children, uroflow and bladder scanning may be a less invasive way to monitor bladder dynamics.
• Upper tract changes: Patients may have baseline renal dysplasia. Elevated bladder pressures and recurrent UTI further may compromise renal function. Obtain periodic renal sonography and serum creatinine levels. Severity of the renal and bladder dysfunction determines the frequency of these studies.
• Urinary incontinence: Approximately one third of patients with PUVs have problems with diurnal enuresis when older than 5 years. Diurnal enuresis may be caused by the bladder changes that lead to elevated storage pressures and poor emptying. Rarely, sphincteric dysfunction secondary to valve ablation can be present. Treatment includes anticholinergic medication, intermittent catheterization, and, in some patients, bladder augmentation.

Inpatient & Outpatient Medications

• Because PUV is a lifelong condition due to an anatomic anomaly, medications may be necessary for years to suppress symptoms of infection or enuresis. All of the medications listed above are intended for long-term use.

Transfer

• Newborn care
  • In the newborn with PUV, the first step in treatment is relief of bladder outlet obstruction by placement of a urethral catheter.
  • Cystoscopic valve ablation or vesicostomy can then be performed when the child is stable.
  • Rarely, a urethral catheter cannot be placed because of hypertrophy of the bladder neck (see Media file 1). These patients require cystoscopy under anesthesia for catheter placement, suprapubic tube placement, or primary vesicostomy.
  • Therefore, care of the newborn depends on having adequate instrumentation (eg, pediatric cystoscopic equipment) and expertise (eg, pediatric radiologist, pediatric urologist, pediatric anesthesiologist). If these services are unavailable, place a catheter (if possible) and transfer the child to an appropriate facility.
• Care of the older child
  • Care of the older child also requires adequate equipment and expertise.
  • Periodic radiologic and urodynamic evaluation is important to monitor the upper urinary tract and bladder changes. These evaluations occur over an extended period of time and rarely constitute an emergency.
  • These patients require a timely referral to a center where appropriate services are available.

Deterrence/Prevention

• Because PUV is a congenital anomaly of unknown origin, it is not preventable.
• Subsequent renal deterioration and bladder changes can be treated and minimized with adequate follow-up care.

Complications

• In the newborn
  • Pulmonary hypoplasia secondary to intrauterine renal dysfunction and oligohydramnios is the primary cause of patient death.
  • Other complications of PUV are generally secondary to chronic bladder changes, leading to elevated detrusor pressures.
  • This, in turn, leads to progressive renal damage, infection, and incontinence.
• Renal insufficiency
  • Historically, of those patients with adequate pulmonary function, approximately 25% died of renal insufficiency in the first year of life, 25% died later in childhood, and 50% survived to adulthood with varying degrees of renal function.
  • Today, with the advent of better techniques in the treatment of pediatric renal insufficiency, most of these children can be expected to survive.
  • The goal of treatment is to preserve the maximal obtainable renal function for each patient. This entails aggressive treatment of infections and bladder dysfunction.
• Vesicoureteral reflux
  • Vesicoureteral reflux is commonly associated with PUVs and is present in as many as one third of patients (see Media file 2).
  • Vesicoureteral reflux in most children is believed to be due to an insufficient intravesical ureter.
  • When associated with PUV, reflux is generally secondary to elevated intravesical pressures.
  • Therefore, the treatment of vesicoureteral reflux in patients with PUVs involves treatment of intravesical pressures using anticholinergics, timed voiding, double voiding, intermittent catheterization, and, at times, bladder augmentation.
• Urinary tract infections
  • Recurrent UTIs are common in patients with PUV.
  • Elevated intravesical pressures predispose patients to infection, possibly by altering urothelial blood flow.
  • Additionally, patients with PUV may have elevated postvoid residual urine volumes, leading to stasis of urine.
  • Dilated upper urinary tracts, with or without vesicoureteral reflux, further elevate UTI risk.
  • UTI management is directed at lowering bladder pressures (anticholinergic medication), lowering postvoid residual urine volume (via clean intermittent catheterization), and at times, administering prophylactic antibiotics.
• Urinary incontinence
  • The same factors that lead to vesicoureteral reflux and UTI also lead to urinary incontinence.
  • Correct management of bladder function depends on adequate bladder evaluation with urodynamic studies.
  • Lowering bladder pressure, improving bladder compliance, and minimizing postvoid residual urine volume contribute to attainment of urinary continence.
  • In some, bladder augmentation may be needed.

Prognosis

• The prognosis of children with PUV is continually improving.
• In the past, most children were found to have PUV only after presenting with urosepsis or progressive renal insufficiency. Older series demonstrated mortality rates approaching 50% by late adolescence.
• Today, most individuals with PUV are discovered when prenatal ultrasonography reveals hydronephrosis.
  • Prompt resolution of bladder obstruction, aggressive treatment of bladder dysfunction, and improved surgical techniques have lowered the neonatal mortality rate to less than 3%.
  • Approximately one third of patients progress to renal insufficiency in their lifetimes. Improved dialysis and transplantation techniques have significantly improved not only the mortality rate for these children but also their quality of life.
  • Additionally, medical and surgical management can achieve urinary continence in nearly all patients.
• An interesting group of patients are those with vesicoureteral reflux dysplasia (VURD) syndrome. In these patients, one kidney is hydronephrotic, nonfunctioning, and has high-grade vesicoureteral reflux. The high-grade reflux is thought to act as a pop-off valve, leading to reduced overall bladder pressures and preservation of contralateral renal function. In the past, these patients were thought to have a better outcome due to preserved renal function in one kidney at the sacrifice of the other. More recent data by Narasimhan et al suggests that, although short-term serum creatinine levels may be favorable, these patients may suffer long-term adverse renal function with hypertension, proteinuria, and renal failure.⁷  In the long run, VURD syndrome may not have the favorable outcome it was once thought to have.

Patient Education

• PUV is a lifelong condition that requires continued medical management. Because of this, both the physician and family must understand the potential long-term complication of renal deterioration if bladder function is not adequately treated.
• Patients and families need realistic expectations regarding continence. Although achievable in nearly all patients, continence often depends on adherence to a timed voiding schedule and intermittent catheterization.
• Patients and families must also realize that medications, such as anticholinergics and suppressive antibiotics, are for control of the symptoms of PUV and are not curative.
• For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Bladder Control Problems.

Miscellaneous

Medicolegal Pitfalls

Several publications have suggested aggressive evaluation of pediatric UTIs.

• Often, a UTI may be the only sign of significant underlying urologic pathology.
• Although most patients with posterior urethral valves (PUVs) are identified because of abnormal prenatal ultrasonography findings, a significant number present later in life with symptoms of UTI or diurnal enuresis recurring or persisting in children older than 5 years. Because of this, the author recommends that any male child older than 5 years with a documented UTI or diurnal enuresis undergo renal and bladder sonography and VCUG.

Special Concerns

• The primary special concerns involved with patients with PUV pertain to the issues of upper urinary tract preservation, UTI, and diurnal urinary incontinence, all of which are secondary to decreased bladder compliance.
• Remember that PUV is a dynamic disease that can have lifelong effects on the bladder. These patients need long-term follow-up care to monitor and treat the effects of altered bladder compliance.

• Search for CME/CE on This Topic »

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• Vesicoureteral Reflux (Radiology)
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• Kidneys and Urinary System Center
• Bladder Control Problems Symptoms
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• Pediatric Voiding Dysfunction: Current Evaluation and Management
• Pediatric Voiding Dysfunction: A Case Study