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Virtual Pediatric Hospital: Paediapaedia: Posterior Urethral Valves (PUV) Paediapaedia: Genitourinary Diseases

Posterior Urethral Valves (PUV)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
The diagnosis is now often made on prenatal ultrasound with an enlarged posterior urethra, thickened bladder walls, and hydronephros. Clinically, early presentations include renal or bladder enlargement and failure to void. Later presentations may have symptoms of dysuria, hematuria, and straining to void that gradually become more prominent with a diminished urinary stream. This is the nost common cause of urethral obstruction in males during infancy and childhood.

Etiology/Pathophysiology:
A congenital malformation of the male urethra that results from the anterior fusion of the normal plicae collicularis which extend distally from the verumontanum to the posterior urethral wall, forming circumferentially obstructing valves below the verumontanum. This obstruction leads to vesicoureteral reflux which can lead to renal dysplasia. The reflux can also lead to a calyceal leak of urine which can cause a perinephric urinoma or a urinary ascites.

Pathology:
Not applicable

Imaging Findings:
On VCUG see a sharply defined lucent defect in the prostatic urethra lumen distal to the verumontanum causing a fusiform dilation of the posterior urethra. A diminished urinary stream is often seen. The bladder wall is often thickened and trabeculated. Vesicoureteral reflux is present in 35-40% of patients.

US and IVP will show bilateral hydroureteronephrosis.

DDX:

References:
See References Chapter.

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