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The megacystis-megaureter syndrome
Insights
Megacystis-megaureter syndrome involves a large bladder and massive reflux. Antireflux surgery alone effectively treats this condition in children, preserving renal function.
Area of Science:
- Pediatric Urology
- Radiology
- Nephrology
Background:
- Megacystis-megaureter syndrome is characterized by a large, thin-walled bladder and significant primary vesicoureteral reflux.
- The condition involves continuous recycling of large urine volumes, leading to massively dilated ureters and bladder.
Observation:
- 29 children (1 day to 13 years) with megacystis-megaureter were treated.
- 74% of cases presented with urinary tract infections.
- Children treated before 1975 were often misdiagnosed with bladder outlet obstruction, leading to inappropriate interventions and renal damage.
Findings:
- 15 children treated after 1975 with antireflux surgery alone showed stable renal function and minimal residual urine.
- This indicates that correction of massive reflux is the primary therapeutic goal.
Implications:
- Early and accurate diagnosis of megacystis-megaureter is crucial.
- Antireflux surgery is the preferred treatment, avoiding interventions for presumed bladder outlet obstruction or bladder capacity reduction.
- Effective management preserves long-term renal health in affected children.
Abstract:
The term megacystis-megaureter describes the radiologic appearance of a large capacity thin-walled bladder and massive primary vesicoureteral reflux. The pathophysiology of these massively dilated ureters and the large capacity bladder is the constant recycling of large volumes of refluxed urine. We treated 29 children between 1 day and 13 years old with this syndrome. Urinary tract infection was present in 74 per cent of the cases. Of these children who presented before 1975, 14 were misdiagnosed primarily as having bladder outlet obstruction, which resulted in inappropriate treatment in 13 and renal deterioration in 5. The remaining 15 children treated after 1975 by means of antireflux surgery alone have remained well with stable renal function and virtual elimination of residual urine. Initial therapy should be aimed at correction of the massive reflux, rather than surgical reduction of bladder capacity or relief of presumed bladder outlet obstruction.