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Posterior Urethral Valve (PUV)

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Posterior Urethral Valve (PUV)

A posterior urethral valve is an obstructing membrane in the posterior male urethra as a result of abnormal in utero development.

The congenital obstructive lesions in the bulbar urethra, Type III Valves (Young’s classification) COBB’S COLLAR OR MOORMAN’S RING.

It can lead to ;

  • Bladder dysfunction,
  • Hydro-Uretero-nephrosis, Vesico-ureteric Reflux
  • Urinary Tract Infection , Pyelonephritis to
  • Kidney damage leading to CKD & ESRD.

Hence, long term monitoring and close follow up is required.

It includes regular investigations, growth and BP monitoring, medicines and surgery.

It is the most common cause of bladder outlet obstruction in male newborns. The disorder varies in degree, with mild cases presenting late due to milder symptoms.

More severe cases can have renal and respiratory failure from lung underdevelopment as result of low amniotic fluid volumes, requiring intensive care and close monitoring. It occurs in about one in 8,000 babies.

It occurs in about one in 8,000 babies.

Classification

Posterior urethral obstruction was first classified by H. H. Young in 1919.

  • Type I – Most common type; due to anterior fusing of the plicae colliculi, mucosal fins extending from the bottom of the verumontanum distally along the prostatic and membranous urethra[6]
  • Type II – Least common variant; vertical or longitudinal folds between the verumontanum and proximal prostatic urethra and bladder neck
  • Type III – Less common variant; a disc of tissue distal to verumontanum, also theorized to be a developmental anomaly of congenital urogenital remnants in the bulbar urethra
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Presentation

Pregnancy: PUV can be diagnosed before birth, or even at birth when the ultrasound during pregnancy shows that the male baby has a hydronephrosis, hydro-ureteronephrosis, thickened bladder wall and posterior urethra, oligohydramnios (AFI).

Birth: presentation immediately after birth can be a painful or forceful micturition, dribbling, poor stream of urine, retention of urine scrotal swelling.

Babies with oligohydramnios due to the urinary obstruction may have respiratory distress. It is an emergency. Baby may need Oxygen support, fluid and electrolyte therapy and urinary diversion.This is followed by definitive surgery when stable.

Later in Chilhood : Poor stream of urine, urinary tract infection, diurnal enuresis, abdominal distension, scrotal swelling in severe cases.

Diagnosis

Postvesicular obstruction due to urethral valves.

  • ULTRASOUND (Abdomen with KUB)
  • (VCUG /MCU) Voiding cystourethrogram is more specific for the diagnosis PUV and VUR. Vesicoureteral reflux (VUR) is also seen in more than 50% of cases. Very often the posterior urethra may be dilated thus making the abrupt narrowing more obvious. the bladder wall may show trabeculations or sacculations or even diverticuli.
  • CYSTOSCOPY
  • Urodynamic Study: During late childhood cases.
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Treatment

THE STANDARD TREATMENT IS PRIMARY (TRANSURETHRAL) ABLATION OF THE VALVES.

It is done by CYSTO-URETHROSCOPY using Pediatric Cystoscope or Resectoscope with
LASER OR ELECROCUATERY with fine bugbee electrodes (please see our operative videos).

If suspected during Pregnancy (antenatally), a consultation with paediatric urologist maybe indicated to evaluate the risk and consider treatment options.

There are three specific endoscopic treatments of posterior urethral valves:[citation needed]

  • VESICOSTOMY FOLLOWED BY VALVE ABLATION
  • PYELOSTOMY
  • Ureterostomy
  • Primary (transurethral) valve ablation – the valve is removed through the urethra without creation of a stoma

FOLLOW-UP: till transition to Adolescent age

Following surgery, the follow-up in patients with posterior urethral valve syndrome is long term, and often requires a multidisciplinary effort between  paediatric urologists, paediatric nephrologists, pulmonologists, neonatologists, radiologists and the family of the patient.

Care must be taken to promote proper bladder compliance and renal function, as well as to monitor and treat the significant lung underdevelopment that can accompany the disorder. Definitive treatment may also be indicated for the vesico-ureteral reflux.

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