Anterior urethral valves - a rare but challenging congenital pathology
Kunj R. Sheth, MD1, Jeffrey T. White, MD2, Aylin N. Bilgutay, MD3, Abhishek Seth, MD1, Angela G. Mittal, MD1.
1Texas Children's Hospital - Baylor College of Medicine, Houston, TX, USA, 2Norton Children's Hospital, Louisville, KY, USA, 3Emory University/Children's Healthcare of Atlanta, Atlanta, GA, USA.
Background: Anterior urethral valves (AUV) and associated anterior urethral diverticuli (AUD) are a rare cause of congenital lower urinary tract obstruction. The occur 25-30 times less frequently than posterior urethral valves (PUV) and historically tend to have a less aggressive presentation or long-term effect. However, due to the low incidence, less is known about management and long-term outcomes for patients with AUVs when compared to patients with PUVs. We hypothesize that AUV patients have a higher failure rate after initial valve ablation compared to PUV patients.
Methods: In this IRB-approved study, we retrospectively identified all patients from 2002 to 2017 undergoing transurethral resection (TUR) valves using CPT code 54200. Charts were then manually reviewed to identify patients with a postoperative diagnosis of AUV. For these patients we evaluate 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.
Results: Over the 16-year study period, 8 patients with anterior urethral valves were identified. Only three (37.5%) patients were diagnosed prenatally, one of which underwent a vesicoamniotic shunt at 29 weeks of age. One additional patient was diagnosed after birth due to a weak/stream dribbling and two (25%) patients presented with febrile UTIs. The remaining two patients were diagnosed at age 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, 3 cautery), but 50% had residual valves and or diverticula identified on routine postoperative imaging in one patient and due to development of clinic symptoms in three patients. All four patients with recurrences underwent repeat TUR-AUV, and thereafter two patients required reconstruction with urethroplasty. In contrast, only 15% (16/104) of PUV patients required re-TUR. At an average follow-up time of 4.24 years (range 0.6-11.4 years), 50% of patients had no evidence of CKD, and only one patients (12.5%) had progressed to CKD of at least stage IIIA. In comparison, for our PUV cohort, 20.2% (21/104) had progressed to CKD of IIIA and 8.6% (9/104) had progressed to ESRD at a mean follow up of 2.3 years.
Conclusions: The overall incidence of AUV is low, making it difficult to characterize these patients definitively. However, it does appear that patients with AUV, even though the majority having a milder phenotype and presented later, do require more aggressive surgical treatment for resolution of the AUV completely. Therefore, close urologic follow-up is essential in this group.
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