BACKGROUND: There is limited data regarding long-term results and associated complications in patients with anterior urethral valve (AUV) and diverticulum (AUD). We retrospectively reviewed AUV/AUD cases managed by us between the year 2002-2020.
METHODS: Presentation, investigations, management, concomitant posterior urethral valves (PUV) and pre-operative characteristics predisposing to long-term poor renal outcome were assessed.
RESULTS: There were 27 patients [AUV (n = 11); AUD (n = 16)] with 5 having concomitant PUV. All presented with poor urinary stream and dribbling at a median age of 1-year (5 days-12 years). More patients with AUD (9 of 16, 56.3%) especially with concomitant PUV presented at ≤1-year-age as compared to those with AUV (4 of 11, 36.4%). Concomitant PUV and AUD cases (n = 3) had characteristic micturating cystourethrography (MCUG) features. (Fig. 1A) Retrograde urethrography delineated the valve and distal urethra dimensions better (Fig. 1B, C). Urethral hypoplasia distal to the valve (n = 4) urethral duplication (n = 1) were associated (Fig. 1D). Syringocele was ruled out in proximal AUD by absence of filling defect on MCUG and appearance of urethral walls on urethrocystoscopy. Serum creatinine > 1 mg/dL (n = 5), trabeculated bladder (n = 12), and vesicoureteral reflux (VUR) (n = 12) was noted at presentation. Diverticulum/valve excision (n = 13, 48.2%), fulguration alone (n = 12, 44.4%) and primary urinary diversion (n = 2, 7.4%) were performed. Follow up (range:3 mo-19 years), showed deranged renal function tests (n = 6), VUR (n = 4), impaired renal function on scans (n = 8), and lower urinary tract dysfunction (n = 7). Outcome with and without associated PUV was similar. Long term results were better in AUV compared to AUD. Pre-operative raised serum creatinine (>1 mg/dL), trabeculated bladder, non-dilated posterior urethra on MCUG and bilateral impaired renal function on scans had significant association with follow up eGFR less than 60 ml/kg/min.
CONCLUSIONS: Co-existing PUV and AUD present earlier and have specific imaging findings. In proximal AUD, possibility of syringocele should be kept in mind, as they have similar presentation and imaging. Concomitant PUV did not alter prognosis. Secondary effects on bladder and renal function were more with AUD. Follow up eGFR less than 60 ml/kg/min was associated with pre-operative elevated serum creatinine, trabeculated bladder, non-dilated posterior urethra, and bilateral impaired renal function on scans.

Anterior urethral valves (AUV) are a rare cause of lower urinary tract obstruction which could progress to renal damage, Clinical presentation varies according with patient\'s age and severity of obstruction, but, in most cases, diagnosis is based on voiding cysto-urethrogram (VCUG). To date, the treatment of choice is endoscopic ablation even if approved guidelines about the overall management of AUVs, including the recognition and treatment of residual valves, are not available.
We describe our protocol for AUV treatment based on primary endoscopic valve ablation followed by check cystoscopy 15 days later.
Medical records of 5 patients with AUVs admitted from 2008 to 2018 to our Pediatric Urology Unit were retrospectively reviewed. Blood tests, urinalysis, renal US and VCUG were performed in all children, while urodynamic evaluation was performed in the 3/5 patients who could void spontaneously. All patients underwent endoscopic valves ablation and after 15 days after a second look cystoscopy was performed. Follow up was based on clinical and radiological evaluation with US, urinalysis and blood tests. Postoperative non-invasive urodynamic studies were performed in the 3/5 patient toilet-trained patients and VCUG was performed in 1/5 patient.
and Discussion: At primary endoscopic ablation cystoscopy revealed AUVs in the penile urethra in three patients, in the penoscrotal urethra in one case, in the bulbar urethra in another case. In 3/5 patients check cystoscopy found residual valves and a second endoscopic ablation was performed. All patients achieved symptoms release and improved urodynamic parameters. No intra or post-operative complication were reported. The assessment of residual valves is variable in literature and it is usually described for posterior urethral valves (PUVs). Few series report the use of VCUG within the first week after valve ablation, our experience instead suggests that performing a second look cystoscopy, is very effective to evaluate the presence of residual AUVs and eventually proceed with further ablation.
Endoscopic ablation is the gold standard treatment for AUV, but residual valves management is not clearly defined. According to our experience, a check cystoscopy 15 days after primary ablation allows to identify and treat possible residual valves showing good results in terms of safety and efficacy.

Anterior urethral valves (AUV) and associated anterior urethral diverticula (AUD) are a rare cause of congenital lower urinary tract obstruction. They occur 25-30 times less frequently than posterior urethral valves (PUV) and historically tend to have a less aggressive presentation and outcome. However, due to the low incidence, little is known about management and long-term prognosis.
We aim to evaluate the outcomes after AUV valve ablation and compare this group to a previously studied PUV cohort.
In this IRB-approved study, we retrospectively identified all patients from 2002 to 2017 undergoing valve ablation using CPT code 52400. Charts were manually reviewed to identify AUV patients, their presenting symptoms, timing of diagnosis, pre and postnatal imaging findings, age at presentation and valve ablation, creatinine, recurrences, additional surgeries and follow-up. The AUV group was then compared to a previously studied PUV cohort of 104 patients from our institution.
Nine AUV patients were identified. Only four (44%) patients were diagnosed prenatally. Three (33%) patients were diagnosed neonatally, one for weak stream/dribbling and two for febrile UTIs. The remaining two patients were diagnosed at ages 3 and 4 due to dysuria and penile swelling with micturition. Patients presenting later in life had no evidence of renal dysfunction or VUR. All patients underwent primary TUR-AUV (1 laser, 4 cold knife, 4 cautery), but 55% had residual valves and/or diverticula identified on routine postoperative imaging in two patients and due to development of clinic symptoms in three patients. All five patients with recurrences underwent repeat endoscopic evaluation, and thereafter two patients required urethral reconstruction. In contrast, only 15% (16/104) of PUV patients required re-TUR (p = 0.01). At an average follow-up time of 4.24 years (range 0.6-11.4 years), 44% of patients had no evidence of CKD, and two patients (22%) had progressed to CKD of at least stage IIIA, compared to 21.4% in the PUV cohort at a mean follow-up of 2.3 years.
The overall incidence of AUV is low, making it difficult to characterize these patients definitively. However, despite a milder phenotype and later presentation in most AUV patients, they do require more aggressive surgical treatment for complete resolution of the AUV. Furthermore, the long-term renal outcomes appear more severe than previously reported.
The poorer outcomes of AUV patients both with respect to recurrence and long-term CKD indicate that close urologic follow-up is essential in this group.

OBJECTIVE: We present our experience in the diagnosis and management of anterior urethral valves (AUV) and anterior urethral diverticula (AUD) as well as review of the bibliography.
METHODS: We retrospectively evaluated all the cases of the AUV and AUD treated in our hospital during the last 10 years. The clinical exploration, renal function study and renal and bladder ultrasound were evaluated in all the children. The diagnosis was completed with voiding cystography (VCUG) and cystoscopy as well as nuclear study in the relevant patients.
RESULTS: Four patients have been treated in our center. AUV was suspected in those children with narrowing of the anterior urethra and thickened bladder with trabeculations at the VCUG. These findings were noticed in 50% of the patients, which also had a neonatal presentation. The diagnosis was confirmed by cystoscopy that allowed the endoscopic resection at the same procedure. The boys with AUD were managed by excision of the diverticulum with urethroplasty. On the follow up, one patient who had AUV, presented renal involvement in the nuclear scans with normal renal function.
CONCLUSIONS: In our experience, the AUV and AUD behave as two different entities in terms of clinical presentation and treatment. The AUV have been effectively treated with endoscopic surgery and the AUD have pointed out open surgery, as described in the literature.
OBJECTIVE: Presentamos nuestra experiencia en el diagnóstico y manejo de los divertículos de uretra anterior (DUA) y de las válvulas de uretra anterior (VUA), junto con revisión de la bibliografía.
METHODS: Se ha realizado un estudio retrospectivo de casos con DUA o VUA tratados en los últimos 10 años en nuestro servicio. En todos los casos se efectuó exploración física, estudio de función renal y ecografía renal. El diagnóstico se completó con cistografía miccional y cistoscopia, así como estudio nuclear, en los casos subsidiarios.
RESULTS: Han sido diagnosticados 4 niños. Se sospechó la presencia de VUA en aquellos pacientes en los que se objetivó en la cistografía un cambio de calibre de la uretra anterior con imagen sugestiva de vejiga de lucha. Estos hallazgos, observados en la mitad de los casos, coincidieron con los niños con presentación neonatal. El diagnóstico se confirmó mediante cistoscopia, que permitió la resección endoscópica simultánea. Los pacientes con DUA fueron tratados quirúrgicamente mediante resección del divertículo y uretroplastia. Solo un caso, el cual presentaba VUA, ha tenido afectación renal en el estudio nuclear, sin repercusión en la filtración glomerular.
CONCLUSIONS: En nuestra experiencia, las VUA y DUA se comportan como dos entidades diferentes en cuanto a clínica y necesidad de tratamiento. Las VUA han sido tratadas eficazmente con cirugía endoscópica y los DUA aislados han precisado de cirugía abierta, al igual que se describe en la literatura.

OBJECTIVE: The purpose of this study was to evaluate the prenatal findings and postnatal outcomes in fetuses with congenital megalourethra.
METHODS: This retrospective study reviewed our experience and the literature between 1989 and 2014. Prenatal findings were evaluated and compared with postnatal findings, including neonatal mortality and abnormal renal function (need for dialysis or renal transplantation).
RESULTS: Fifty fetuses with congenital megalourethra were analyzed, including 6 cases diagnosed in our centers. Most cases (n = 43 [86.0%]) were diagnosed in the second trimester. Only 1 case was diagnosed in the first trimester, whereas 6 cases (12.0%) were diagnosed in the third trimester. Thirty-five fetuses (70.0%) survived. Bilateral hydroureters were associated with perinatal death (P= .024). Among the survivors, 41.9% of the neonates had renal impairment. The following factors were associated with postnatal renal impairment: presence of severe oligohydramnios/anhydramnios (P = .033), bilateral hydronephrosis (P = .008), and earlier gestational age at delivery (P = .022).
CONCLUSIONS: In fetal megalourethra, bilateral hydroureters, bilateral hydronephrosis, and severe oligohydramnios/anhydramnios are associated with neonatal mortality and renal impairment.

OBJECTIVE: Anterior urethral valves (AUVs) is an unusual cause of congenital obstruction of the male urethra, being 15-30 times less common than posterior urethral valves (PUVs). It has been suggested that patients with congenital anterior urethral obstruction have a better prognosis than those with PUV, with less hydronephrosis, and a lower incidence of chronic renal insufficiency (5 vs. 30%). The long-term prognosis of AUVs is not clear in the literature. In this report we describe our experience and long-term follow up of patients with anterior urethral valve.
METHODS: We retrospectively identified 13 patients who presented with the diagnosis of AUVs in our institutions between January 1994 and June 2012. Two patients were excluded: one patient had no follow up after intervention; the other had a follow up <1 year. From the 11 patients included, we evaluated the gestational age, prenatal and postnatal ultrasound findings, voiding cystourethrogram findings, age upon valve ablation, micturition pattern, creatinine, and clinical follow up.
RESULTS: Between 1994 and 2012 we evaluated 150 patients with the diagnosis of urethral valves. Of this group, 11 patients (7.3%) had AUVs and an adequate follow up. Mean follow up is 6.3 years (2.5-12 years). Five (45.4%) patients had prenatal diagnosis of AUV. The most common prenatal ultrasonographic finding was bilateral hydronephrosis and distended bladder. One patient showed a large perineal cystic mass, which was confirmed to be a dilated anterior urethra. The mean gestational age was 37.6 weeks (27-40 WGA). Postnatally, 90% had trabeculated bladder, 80% hydronephrosis, and 40% renal dysplasia. The most common clinical presentation was urinary tract infection in five patients (45.4%), followed by weak urinary stream found in four patients (36.3%). The age at initial surgical intervention ranged between 7 days and 13 years. Seven (63.6%) patients had primary transurethral valve resection or laser ablation and three patients (27.2%) had primary vesicostomies. One boy (9.1%) had penile urethrostomy with excision of urethral diverticulum. Two (18.2%) patients developed end-stage renal disease.
CONCLUSIONS: Anterior urethral valve is a rare congenital entity affecting the genitourinary system in males. Early urinary tract obstruction resulted in end-stage renal disease in 18% of our patient population. In our series, the complication rate and the evolution to renal failure are high and similar to patients with PUV. In patients with AUVs we recommend long-term follow up and close evaluation of patient\'s bladder and renal function.