Urethral duplication is a rare phenomenon. Multiple case reports and case series have catalogued experiences with evaluation and treatment of duplications, as well as offered schema for classifications. Williams and Kenawi1 first classified sagittal versus collateral duplications. The accessory, or duplicate, urethra is typically described based on its meatal orientation in the sagittal plane as dorsal or ventral, hypospadic or epispadic. More rare is duplication in the frontal plane, described as coronal, side-by-side, or collateral. Effmann and colleagues2 stratified their series into blind incomplete (distal vs proximal), complete (double vs single meatus), and component of caudal duplication (complete or partial) based on radiographic imaging. Salle and colleagues3 added collateral duplication as a subclassification of the system described by Effmann and colleagues.2 When this coronal presentation is present, there is typically an associated midline defect or caudal duplication.
A 50-year-old African American man with stage V chronic renal insufficiency secondary to adult polycystic kidney disease (APKD) and hypertension was referred to the urology department for evaluation of gross hematuria and dysuria. Prior to our evaluation, he was empirically given multiple rounds of antibiotics despite negative urine culture results. His urinary symptoms persisted but hematuria resolved. He has no history of recurrent urinary tract infections and has never passed clots or developed urinary retention. He reports terminal dysuria that began 10 months prior to referral and associated umbilical pain. He has bothersome daytime frequency and urgency, as well as nocturia, every 1 to 2 hours. He endorses weakened stream but denies feelings of incomplete emptying. Although he has stage V chronic kidney disease, he has never required dialysis and voids normal volumes. He has never undergone urologic surgery. After examination and further questioning, he reports that, as a young child, he had two separate urinary streams and was able to ejaculate through each meatus.
He denies any family or personal history of nephrolithiasis. His father and paternal grandfather, both deceased, had prostate cancer; his mother, also deceased, had APKD requiring transplantation. He has a 10 pack-year smoking history and currently continues to smoke. He has a daughter, whom he fathered naturally.
On examination, he was noted to be hypertensive, with a blood pressure measuring 140/102 mm Hg. He was obese and well appearing, in no distress. Genitourinary examination demonstrated an uncircumcised phallus with intact foreskin. There were two meatal openings in the coronal plane (Figure 1A). The right meatus had a mucosal web with pinpoint opening, whereas the left urethral meatus appeared of normal caliber (Figure 1B). These were located at the tip of the glans bilaterally. The right testis was descended and of normal caliber without nodules or tenderness and the left testis was atrophic. His prostate was 30 g and without nodules. He had no costovertebral angle tenderness and no palpable abdominal masses. He did have a nontender, reducible umbilical hernia and rectus diastasis.
Urinalysis demonstrated proteinuria, 35 erythrocytes per high-power field, 15 leukocytes per high-power field, and 0 casts. Urine culture results were negative. Prostate specific antigen level was 0.42 ng/mL, and urine cytology results were negative for malignant cells. His creatinine level was 5.5 mg/dL with an estimated glomerular filtration rate of 13 mL/min/1.73m2.
Computed tomography renal stone protocol was available, as well as renal and bladder ultrasound. These demonstrated findings consistent with polycystic kidney disease. The kidneys were markedly enlarged, containing innumerable hypo-and hyperdensities consistent with simple cysts. There was no hydronephrosis or nephrolithiasis bilaterally. The bladder appeared normal.
His renal dysfunction precluded contrast administration, so he underwent bilateral retrograde pyelograms and cystourethroscopy to complete his hematuria work-up. In order to fully evaluate his anomalous genitourinary tract, we elected to further delineate his anatomy with observation during voiding (Figure 1C) and retrograde urethrogram. He was noted to have a strong stream from the left urethra and no voiding from the right urethra. A retrograde urethrogram was performed simultaneously with a 60-mL catheter-tipped syringe in the functional (left) urethra and 14G angiocatheter in the nonfunctional (right) urethra (Figure 2A). This demonstrated an Effmann type IIb duplication in the coronal plane. There were no filling defects in either urethra (Figure 3). Urethroscopy of the blind-ending right urethra was performed using a 6Fr flexible ureteroscope secondary to its small caliber (Figure 2B). This demonstrated normal mucosa to a sclerotic, blind-ending urethra. Urethral washings were sent for culture and cytology from the blind-ending urethra; results were negative for malignancy and infection. Rigid cystourethroscopy of the left urethra identified a ventral obliterated os that correlated with the area of blind ending of the right urethra (Figures 2C and 2D). The prostate demonstrated trilobar hypertrophy and the bladder mucosa was without any areas concerning for malignancy. Bilateral retrograde pyelograms demonstrated delicate calyces and splayed collecting system consistent with extrinsic compression of polycystic kidneys. There were no filling defects and the kidneys drained readily.
We discussed reconstruction for improved cosmesis; however, the patient elected observation as he was asymptomatic from his urethral duplication. The patient was given finasteride and tamsulosin for his lower urinary tract symptoms (LUTS), which gave him significant relief. The umbilical pain was attributed to umbilical hernia for which there is planned surgical repair. He is currently undergoing continued evaluation for renal transplantation, and although he has a continued, slow decline in his renal function, he has not required dialysis.
This case highlights the importance of identification of anatomic anomalies to ensure appropriate work-up of urologic complaints. In this case, the duplicate urethras were fully evaluated for possible contribution to hematuria.
Classification of urethral duplication was popularized by Effmann and colleagues.2 Later, a distinction for coronal duplications was first proposed by Salle and associates.3 Published cases of duplicate urethras in the coronal plane were most recently reviewed by AbouZeid and associates.4 Most reported cases are described in the pediatric population and are associated with midline defects or caudal duplication (including complete or incomplete duplication of bladder, phallus, or glans, or colorectal anomalies such as imperforate anus or colonic duplication). Only four other cases have been described with isolated urethral duplication in the coronal plane. These include a case of a 69-year-old man who presented with LUTS and double urinary stream who was asymptomatic and therefore observed,5 an 11-month-old boy with complete urethral duplication who had surgical approximation of the distal urethra to develop solitary meatus at the glans tip,6 an 8-month-old boy with two meatal openings who was observed because he was asymptomatic,7 and another asymptomatic adult.8 To our knowledge, this case of urethral duplication is the first to be described in a patient with APKD with or without other anomalies.
We describe a new association with a rare variant of the already rare phenomenon, a case of an adult with urethral duplication and APKD. This adds to a very small number of reported cases of isolated coronal duplication without other genitourinary duplication. This case highlights the importance of identification of anatomic anomalies to ensure complete evaluation of common urologic complaints, such as hematuria and LUTS.
The authors declare no real or apparent conflicts of interest.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review.
The authors thank Shannon Caruso. who provided professional photography services on behalf of Ochsner Clinic Foundation.
