Posterior urethral valves in Eastern Ontario - a 30 year perspective.
BACKGROUND
Posterior urethral valves (PUV) are the most common cause of male pediatric obstructive uropathy. Recent advancements in antenatal ultrasound and endoscopy have altered the presentation and management of PUV. Herein we describe the presentation, management and outcome of PUV patients in Eastern Ontario/Western Quebec over the last 3 decades. A comparison analysis of those cases identified pre and post widespread utilization of antenatal ultrasound diagnosis was performed to discern the clinical evolution of PUV with respect to long-term outcome.


METHODS
Retrospective systematic chart review of all PUV cases diagnosed and treated at the Children's Hospital of Eastern Ontario over the last 3 decades. Charts were reviewed for initial presentation, method of diagnosis, radiological and clinical findings at diagnosis, initial management, and long-term clinical outcome. The evolution of PUV was interpreted by dividing the cohort into two groups chronologically delineated by the first case detected by antenatal ultrasound in the mid-1980s. These pre- and post- antenatal ultrasound eras were compared with respect to the parameters outlined above.


RESULTS
Fifty-three cases were reviewed - 21 prior to widespread antenatal ultrasound screening in the mid-1980s and 32 after. There were 13/53 cases (32%) discovered by prenatal ultrasound evidence of hydronephrosis, none prior to 1985. VCUG confirmed the diagnosis in all cases. Mean age at presentation in the remaining post-natally diagnosed patients was 33 months. Of the cases diagnosed post-natally, ultrasound investigation complemented VCUG findings in 19/40 cases (47%), whereas IVP was utilized in 14/40 (35%). IVP has not been utilized for this purpose since 1987. Overall, 26/53 cases (49%) had documented VUR - 16/26 (62%) bilateral; 42/53 (79%) had hydronephrosis on ultrasound - 37/42 (88%) bilateral; 26/53 (49%) had radiological evidence of renal parenchymal damage at diagnosis; 41/53 (77%) cases had a thickened bladder wall on ultrasound at diagnosis, and 23/53 (43%) had at least one bladder diverticulum. Techniques of initial management comprised: valve ablation 32/53, vesicostomy 11/53, and high diversion 10/53. Clinically significant bladder dysfunction was found in 31% of cases, ranging from bladder instability to myogenic failure. Globally impaired renal function, as determined by significantly elevated serum creatinine levels, reduced GFR, or both, was found in 12/53 (23%). 6/53 (11%) progressed to ESRD, of which 4 received transplants. Two patients died - one from complications related to renal failure. Of the six cases of myogenic bladder failure identified, three (50%) had concurrently significant renal impairment. Average length of follow-up was 8.3 years, varying between 1 month and 18 years.


CONCLUSIONS
The presentation of PUV is variable, and currently antenatal detection is the most common mode. Despite this, it still does not make up the majority of diagnoses. Complete radiological work up should include abdominal and pelvic U/S in conjunction with VCUG. Concurrent VUR in 50% of boys mandates suppressive antibiotic use. Primary valve ablation remains the gold standard for treatment of PUV, with vesicostomy reserved for selected cases. Long-term bladder and renal dysfunction is common in this population, and mandates long-term urological and nephrological follow-up.
Warren
Jeff
J
Division of Pediatric Urology, Children's Hospital of Eastern Ontario, University of Ottawa, Ontario, Canada.
Pike
John G
JG
Leonard
Michael P
MP
D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007674:Kidney Diseases / Q000209:etiology; D008297:Male; D009864:Ontario; D011247:Pregnancy; D011859:Radiography; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal; D014521:Urethra / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D001743:Urinary Bladder / Q000000981:diagnostic imaging; D014547:Urinary Diversion