[Non-coordinated micturition syndrome mimicking posterior urethral valves in a male neonate].
High-grade of vesicoureteral reflux (VUR) has been reported in association with prenatal diagnosis of abnormal bladder function in a male neonate with postpatally no anatomical urethral obstruction and bladder dysfunction. This study was designed to describe clinical presentation of this entity in male neonates, the urodynamic pattern, prognosis in terms of renal function and therapeutic management since birth, in our experience with four cases.


METHODS
We reviewed the records of 4 male neonates diagnosed at birth of bilateral high-grade VUR (grade > or = 4) with prenatally diagnosed hydronephrosis; thus 8 refluxing renal units (RRU) were studied. All cases had renal failure at birth. In all cases dimercaptosuccinic acid (DMSA) renography was performed. All RRU had reflux nephropathy; 5 RRU had moderate impairment of renal function (20-40%). In 3 RUU was demonstrated by a severe decrease in renal function (10-20% in 2 RUU, and less than 10% in 1 RUU). Micturating cystouretrography (MCUG) excluded the presence of posterior urethral valves; however, in all cases a tightened bladder neck ("bladder neck impression") was present. Endoscopy was performed in one baby excluding posterior urethral valves. Follow up ranged from 2 to 5 years (mean 3.5).


RESULTS
In all 4 cases underwent transient urinary diversion during their first month of life. Urodynamic study revealed a high-risk bladder with low compliance, a reduced functional bladder capacity and a high residual urine volume in all cases. All the patients was placed on antibiotic prophylaxis and oral oxybutinin chloride. Three patients underwent bilateral Cohen ureteral reimplantation. The other boy underwent left nephrectomy, right Politano ureteral reimplantation and ureterocystoplasty. In the 7 RRU postoperative MCUG revealed cessation of reflux. Currently therapy includes antibiotic prophylaxis in 4, oral oxybutinin in 4 and intermittent catheterisation in 2 patients. 3 patients have normal renal function. The other boy underwent re-diversion because of renal failure.


CONCLUSIONS
Fetal severe bilateral reflux nephropathy is a clinical entity almost exclusively in male neonate mimicking hyper-pressure syndrome due to urethral obstruction, in terms of evolution (to chronification and renal failure) and treatment (that requires primordial management of bladder dysfunction). Absence of anatomical urethral obstruction and urodynamic pattern suggest functional obstruction of bladder neck-periurethral sphincter complex in fetal life as a cause for this syndrome. For this reason we consider it as a clinical presentation of fetal non-coordinated voiding in male or "posterior urethral valves like syndrome".
Martín-Crespo Izquierdo
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Servicio de Cirugía Pediátrica, Hospital Sanatorio Nuestra Señora del Rosario, C/Príncipe de Vergara, 53, 28006 Madrid. rmartinc@telefonica.net
Luque Mialdea
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D003937:Diagnosis, Differential; D006801:Humans; D006869:Hydronephrosis / Q000150:complications / Q000175:diagnosis; D007231:Infant, Newborn; D008297:Male; D051437:Renal Insufficiency / Q000150:complications; D012189:Retrospective Studies; D012720:Severity of Illness Index; D001745:Urinary Bladder Diseases / Q000175:diagnosis* / Q000503:physiopathology; D014563:Urodynamics / Q000502:physiology; D013521:Urologic Surgical Procedures, Male; D014718:Vesico-Ureteral Reflux / Q000150:complications / Q000175:diagnosis* / Q000601:surgery