0022-5347/97/1583-1030$03.00/0 THEJ o r a . ; ~OF ~ UROLO(:Y Copyright 0 1997 by iLMERlCiLv UROL€K:IChL ASSIKL~TIOS, IN(..
Vol. 158, 1030-1032. September 1997 Printed rn U.S.A.
AN ALGORITHM FOR THE MANAGEMENT OF ANTERIOR URETHRAL
VALVES JOHN G. VAN SAVAGE, ANTOINE E. KHOURY; GORDON A. McLORIE
DARIUS J. BAGLI
From the Division of Urologl. Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Purpose: We p r e s e n t o u r management algorithm for patients with anterior urethral valves seen in the last 2 decades w i t h and without the benefit of p r e n a t a l sonography. Materials and Methods: A case series design w a s used to s t u d y 17 cases of a n t e r i o r urethral valves. Five p a t i e n t s presented w i t h prenatal hydronephrosis from 1984 t o 1993 and 12 presented w i t h predominant voiding symptoms between 1975 and 1996 at a m e a n age of 6 years. Results: T r e a t m e n t included supravesical diversion in 1case, vesicostomy in 5, u r e t h r o p l a s t y in 5 and transurethral fulguration in 6. Four of the 5 patients with a p r e n a t a l diagnosis of hydronephrosis had moderate t o severe hydronephrosis compared t o 3 of the 12 w h o did n o t undergo p r e n a t a l sonography. All 17 patients were continent and infection-free, and had little o r n o hydronephrosis at a m e a n followup of 5 years. Conclusions: We recommend vesicostomy i n infants with high grade bilateral vesicoureteral reflux and poor emptying of the u r i n a r y tract, transurethral fulguration if the urethra has sufficient caliber and support, and urethroplasty in other patients. Based o n o u r m a n a g e m e n t algorithm patients w i t h anterior u r e t h r a l valves do not have sequelae due to distal obstruction of the urinary tract. KEY WORDS: urethra, urethral obstruction Anterior urethral valves and congenital urethral diverticula are rare entities generally described in case reports and small series.l-8 The largest series of 17 patients was reported in 1969 by Williams and Retik before the era of prenatal sonography.8 We were interested in whether prenatal sonography had made a difference in the diagnosis and treatment of anterior urethral valves. We discuss the diagnosis of and our management algorithm for anterior urethral valves in a retrospective review of 17 cases, and comment on the impact of prenatal sonography on the disease. PATIENTS
We retrospectively identified 17 patients with anterior urethral valves in this series. Patients with the prune-belly syndrome were excluded from study. Five of the 17 patients who presented between 1984 and 1993 with prenatal hydronephrosis were designated the prenatal group and 12 who presented between 1975 and 1996 at ages 1 day to 17 years (mean 6.2 years) with predominant voiding symptoms were termed the postnatal group (see table). No patient presented with a urinary tract infection. Each patient was evaluated with voiding cystourethrography and renal sonography or excretory urography as appropriate. Serum creatinine was determined when bilateral hydronephrosis was detected. Urine flow and post-void residual were assessed when the child was toilet trained. Patients underwent cystourethoscopy at the time of the operation. We used a management algorithm for treating neonates with anterior urethral valves during evaluation for prenatal hydronephrosis or suspected signs and symptoms (see figure). This algorithm was also used when anterior urethral valves were suspected in older children (see figure). Only neonates and infants were considered for vesicostomy and no patient presented with severe obstructive uropathy later in childhood. No patient has required pyelostomy for anterior urethral valves in the last 20 years at our facility. However, * Requests for reprints: Division of Urology, Hospital for Sick Children, 555 University Ave., Toronto, Ontario, Canada M5G 1x8.
patient 12 underwent supravesical diversion elsewhere secondary to massive hydroureteronephrosis on day 2 of life, when nadir creatinine was not yet established (see table). Vesicostomy was performed when bilateral high grade vesicoureteral reflux was present. Our rationale in these cases was to ensure emptying of the whole urinary tract, which would not occur even when the bladder emptied efficiently since refluxing urine from the ureters filled the bladder after voiding. Vesicostomy was also performed in neonates or infants with persistent azotemia and severe hydroureteronephrosis despite Foley catheter drainage. Two-stage urethroplasty in neonates and infants with a massive anterior urethral diverticulum involved open resection of the valve and marsupialization of the anterior urethral diverticulum or perineal urethrostomy as stage 1 and urethroplasty as stage 2. Stage 2 was done when the phallus was adequately grown and the diverticulum had decompressed. One-stage urethroplasty was indicated when the urethra did not fulfill the criteria for transurethral fulguration and was not associated with high grade bilateral vesicoureteral reflux. Indications for transurethral fulguration of anterior urethral valves included an adequate urethra with a caliber distal to the valve large enough to permit safe instrumentation, and sufficient corpus spongiosum and periurethral tissue to avoid subcutaneous extravasation or a urethrocutaneous fistula. RESULTS
The table shows radiographic abnormalities or azotemia at presentation. Cystoscopy revealed a n associated urethral diverticulum in all cases. Followup included uroflowmetry when the child was toilet trained, post-void residual and voiding cystourethrography. Serum creatinine was obtained in patients with bilateral abnormalities in upper tract imaging studies. All 5 patients in the prenatal group were continent, able to empty the bladder and urethra, and free of urinary tract infections a t a mean followup of 3 years. Hydronephrosis was
ALGORITHM FOR MANAGEMENT OF ANTERIOR URETHRAL VALVES Characteristics of 17 patients with anterior urethral v a h e s at presentation ~~
2 3 4 5 6 I 8 9
10 11 12 13 14
15 16 17
Sims + SvmDtoms
Associated anomaly Voiding symptoms Voiding symptoms Voiding symptoms Hematuria Voiding symptoms Voiding symptoms Voiding symptoms Penile ballooning Penile ballooning Respiratory distress Penile ballooning Prenatal hydronephrosis Prenatal hydronephrosis Prenatal hydronephrosis Prenatal hydronephrosis Prenatal hydronephrosis
Bulbar Penile Bulbar Bulbar Penile Penoscrotal Bulbar Penoscrotal Bulbar Bulbar Penile
Mild Moderate Mild Moderate Mild Moderate Moderate Mild Moderate Moderate Severe
Not determined None None None None None Mild None None Moderate, azotemia Moderate. azotemia
Primary Treatment lagel Transurethral resection ( 4 yrs.) Transurethral resection ('7 yrs.) Transurethral resection (8yrs.) Transurethral resection (12 yrs.1 Transurethral resection (17 yrs.1 1-Stage urethroplasty (3 y r s . ) 1-Stage urethroplasty ( 3 y r s . ) 1-Stage urethroplasty (6 yrs.) 2-Stage urethroplasty ( 2 + 16 yrs.) Vesicostomy (9days) Vesicostomy ( 3 wks.)
Ureterostomy ( 2 days) Transurethral resection ( 4 wks.) Stage 1 urethroplasty (10 days) Vesicostomy (6 days)
Vesicostomy (2 wks.)
mild a t followup and there were no postoperative complications. Azotemia resolved in 3 of 4 patients. However, patient 17 with bilateral small echogenic kidneys and mild hydronephrosis subsequently had progressive renal failure and preemptive live related donor renal transplantation was performed. Pathological evaluation of a nephroureterectomy specimen from this patient revealed renal dysplasia. Antireflux surgery was required in only 1 patient despite initial high grade reflux in 3 others. Reflux has resolved or decreased to grade 1or 2 and it is being followed conservatively. All 12 patients in the postnatal group were continent of urine, able to empty the bladder and urethra, and free of urinary tract infections at a mean followup of 5 years. There were 2 postoperative complications, including urinary extravasation after endoscopic valve ablation and a urethrocutaneous fistula after stage 2 of a 2-stage urethroplasty. In these 2 cases the skin, subcutaneous tissue and corpus spongiosum were attenuated over the urethra. In the former patient a scrota1 urinoma developed after catheter removal several days after anterior urethral valve ablation at the bulbar urethra. Mild to moderate hydronephrosis in 2 patients a t presentation decreased to minimal or mild at followup. The neonate who had severe hydroureteronephrosis now has bilateral mild hydronephrosis and normal diuretic renogram parameters after 15 years of followup. He is 1 of the 2 patients in this series who required antireflux surgery. All other patients had no or low grade vesicoureteral reflux
after the relief of infravesical obstruction. Patient 5 underwent renal transplantation for glomerulonephritis and had no evidence of hydronephrosis. DISCUSSION
The spectrum of urinary tract involvement in patients with anterior urethral valves is broad, ranging from mild urethral dilatation to bilateral hydroureteronephrosis with azotemia. Nearly half of our patients underwent simple transurethral fulguration, consistent with the recent series of Firlit e t al.2 We believe that the classification of anterior urethral valves as a spectrum of urinary tract obstruction with anterior urethral valves alone, urethral diverticula, bladder distension and hydroureteronephrosis, in increasing order of severity,2 is useful because clinical decision making can be based on whether vesicostomy is needed. We prefer to include azotemia and high grade bilateral vesicoureteral reflux in our schema, since it allows patients in the worst prognostic group to be treated most effectively. None of our patients had progressive azotemia from obstructive uropathy due to anterior urethral valves, which conflicts with the results of others.18.104.22.168 Possible reasons for this difference may include more liberal use of voiding cystourethrography, which is the study of choice to diagnose anterior urethral valves, a larger group with prenatally detected hydronephrosis, and standard inclusion of the anterior urethra in our evaluation of urinary tract pathology. A high index of suspicion is required in cases of obstructive bladder changes and no definable uropathy, since they are frequently associated with silent deterioration of the upper tracts and lack of high quality Obstructive uropathy suggested by antenatal hydronephrosis, urethrography. Cystoscopy may be less accurate, because it voiding symptoms or penile ballooning with voiding. is easy to miss the flap valve if it is not looked for specifically. 3. Our management algorithm for anterior urethral valves Renal sonogram and VCUG considers timing of presentation, severity of obstruction and No-mild hydronephrosis Moderate-severe diverticular versus phallic size. The neonate generally reI bilateral hydronephrosis quires Foley catheter decompression of the bladder for inJI fravesical obstruction and serum creatinine monitoring. Check serum creatinine Since high grade bilateral vesicoureteral reflux results in continuous poor emptying of the urinary tract, we recomNormal creatinine Foley catheter if azotemic mend vesicostomy in these neonates and infants, so that the Recheck creatinine bladder and upper tracts have low pressure drainage. A bladder with poor emptying, low capacity and poor compliValve fulguration or single-stage urethroplasty if urethra adequate ance is also amenable to vesicostomy, although we have not Staged urethroplasty for massive urethral diverticulum yet observed this in patients with anterior urethral valves. In Vesicostomy for high grade bilateral reflux or persistent azotemia older children the valves can usually be fulgurated without a Management algorithm for anterior urethral valves. VCUG, void- urinary catheter depending on cystography and cystoscop~ findings in the bladder. ing cystourethrogram.
ALGORITHM FOR MANAGEMENT OF ANTERIOR URETHRAL VALVES
The type of urethroplasty depends on anterior urethral diverticular size in relation to phallic size in infants and children. Since t h e only complications of surgical treatment in our series were related to attenuation of urethral and periurethral tissue, we a r e particularly aware of this situation and we do not overextend t h e indication for transurethral fulguration or 1-stage urethroplasty in this setting. Despite the rarity of this condition, which is 10-fold less common t h a n posterior urethral valves, urologists who t r e a t such patients can use this algorithm to diagnose and manage anterior urethral valves. The presentation of infravesical obstruction due to anterior urethral valves in our series does not differ markedly from t h a t of posterior urethral valves. Anterior urethral valves present with voiding symptoms in a third of the patients or due to evaluation of prenatal hydronephrosis in a third. Most of t h e remainder of those with anterior urethral valves present with a visible urethral diverticulum on the ventral shaft or bulb of t h e penis. Posterior urethral valves most often present with a palpably enlarged bladder, urinary tract infection or t h e effects of azotemia.' A weak urinary stream is present in a third of the cases, and half of those with anterior urethral valves have associated hydronephrosis compared to 90% with posterior urethral valves.9 However, t h e effects of infravesical obstruction due to anterior urethral valves a r e less severe t h a n those due to posterior urethral valves. The severity of hydronephrosis in anterior urethral valves i s much less than i n posterior urethral valves. Anterior urethral valves cause few long lasting upper tract radiographic changes a n d they are associated with chronic renal failure i n less t h a n 5%' of t h e patients (none i n our series) compared to some degree of chronic renal failure in 30%of those with posterior urethral valves.1-*,1" Bilateral vesicoureteral reflux is not a poor prognostic factor i n anterior urethral valves as it frequently i s in posterior urethral valves, which may be why vesicostomy is so successful and pyelostomy has not been required for anterior urethral valves at our institution. Patients with anterior urethral valves in our series are continent of urine, a n d free of urinary tract infections and obstructive symptoms at long-term followup. Posterior urethral valves cause continence problems in 14 to 38%)of t h e patients as well as a significant incidence of urinary tract infections and other voiding symptoms at long-term followup.11.*2 In a third of our patients anterior urethral valves were diagnosed after detection of prenatal hydronephrosis. Although t h e detection of prenatal hydronephrosis is thought to have a beneficial effect on hydronephrosis associated with posterior urethral valves, t h e number of patients in t h e largest recent study of anterior urethral valves is too small to prove such a n effect.'" However, several observations i n t h e prenatal group a r e worthy of mention. The proximal effect on t h e urinary tract from obstruction due to anterior urethral
valves is more severe t h a n when t h e diagnosis is made postnatally, comprising nearly all cases of azotemia. Despite this difference in severity a t presentation, t h e 2 groups in our series had similar and minimal long-term sequelae of anterior urethral valves on urinary tract function. Complications are fewer and minimal in patients in t h e prenatal group. Because this condition involves fixed mechanical obstruction of varying degrees, one would expect a better response in patients who were treated earlier. C'ON(:LUSIONS
Our large series of patients with anterior urethral valves indicates t h a t treatment may be addressed with a n algorithm based on urinary tract emptying and refluxing ureters, and t h e caliber and adequacy of t h e urethra a n d periurethral tissue. Specific recommendations for t h e treatment of patients with anterior urethral valves include transurethral fulguration when t h e urethra has sufficient caliber and support, vesicostomy i n infants with a poorly emptying urinary tract, and 1 or %stage urethroplasty depending on t h e size of t h e anterior urethral diverticulum in other patients. REFERENCES
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