Collateral Urethral Duplication in an Adult With Adult Polycystic Kidney Disease

Case Review Collateral Urethral Duplication in an Adult With Adult Polycystic Kidney Disease Dunia Taufiq Khaled, MS, MD, Amanda Feige Saltzman, MD, Lester Joseph Prats, MD Department of Urology, Ochsner Clinic Foundation, New Orleans, LA Duplications of the lower urinary tract are rare congenital anomalies that are usually accompanied by concomitant nonurologic anomalies; they are typically diagnosed in early childhood. In more rare cases these are isolated, leading to diagnosis later. We describe a 50-year-old man who remained asymptomatic and therefore undiagnosed for five decades. His is one of fewer than 20 cases in the literature describing urethral ­duplication in the coronal (collateral) plane, the more common variant being sagittal (dorsal-ventral) duplication. He is one of only four cases reported without c­ oncomitant midline anomaly. Furthermore, he is the sole case associated with adult polycystic ­kidney disease. [Rev Urol. 2016;18(4):242-245 doi: 10.3909/riu0705] ® © 2016 MedReviews , LLC Key words Collateral duplication • Coronal duplication • Duplicate urethra • Urethral anomaly • Duplication of lower urinary tract U rethral 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 242 • Vol. 18 No. 4 • 2016 • Reviews in Urology 17_4004170006_RIU0705.indd 242 12/12/16 4:30 pm Collateral Urethral 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. Case Presentation 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 hema- 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 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 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. turia 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, c­ aliber without nodules or tenderness and the left testis was atrophic. His prostate was 30 g and without nodules. He had no costovertebral Figure 1. (A) Observed voiding, a strong stream from the left urethra. (B) Collateral duplication in the frontal plane. (C) Mucosal web obstructing n ­ onfunctional right urethra seen with narrow opening. A B C Vol. 18 No. 4 • 2016 • Reviews in Urology • 243 17_4004170006_RIU0705.indd 243 12/12/16 4:30 pm Collateral Urethral Duplication continued A B C D Figure 2. (A) Simultaneous retrograde urethrogram with 60-mL catheter-tipped syringe and 14G angiocatheter. (B) 5Fr open-ended urethral catheter within blind urethra, rigid cystoscope within functional urethra. (C) Urethroscopy performed with flexible ureteroscope within blindending urethra, 16Fr Foley within functional urethra. (D) Blind-ending os at proximal penile urethra of functional urethra. to complete his hematuria workup. 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 Rigid cystourethroscopy of the left urethra identified a ventral obliterated os that correlated with the area of blind ending of the right urethra. Figure 3. Retrograde urethrogram demonstrated a right urethra with stenotic end lateral to the left urethra, which is patent without stricture to a single bladder. 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 244 • Vol. 18 No. 4 • 2016 • Reviews in Urology 17_4004170006_RIU0705.indd 244 12/12/16 4:30 pm Collateral Urethral Duplication 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 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 Most reported cases are described in the pediatric population and are associated with midline defects or caudal duplication… 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. Discussion 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 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. Conclusions 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. References 1. 2. 3. 4. 5. 6. 7. We describe a new association with a rare variant of the already rare 8. Williams DI, Kenawi MM. Urethral duplication in the male. Eur Urol. 1975;1:209-215. Effmann EL, Lebowitz RL, Colodny AH. ­Duplication of the urethra. Radiology. 1976;119:179-185. Salle JL, Sibai H, Rosenstein D, et al. Urethral ­duplication in the male: review of 16 cases. J Urol. 2000;163:1936-1940. AbouZeid AA, Safoury HS, Mohammad SA, et al. The double urethra: revisiting the surgical classification. Ther Adv Urol. 2015;7:76-84. Urakami S, Igawa M, Shilina H, et al. Congenital ­collateral urethral duplication in the frontal plane. J Urol. 1999;162:2097-2098. Frederick L, Benson AD. A case of coronal urethral duplication with no other abnormalities. Can J Urol. 2011;18:5557-5559. Ching CB, Palmer JS. Collateral urethral duplication. Dialogues in Pediatric Urology. 2008;29:16-17. Pastor Navarro H, Carrion Lopez P, Martinez Ruiz J, et al. Collateral urethral duplication in an adult. Arch Esp Urol. 2014;67:345-349. Main Points • Duplications of the lower urinary tract are rare congenital anomalies that are usually accompanied by concomitant nonurologic anomalies, and are typically diagnosed in early childhood. • The 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-byside, or collateral. When this coronal presentation is present, there is typically an associated midline defect or caudal duplication. • Identification of anatomic anomalies is critical to ensure complete evaluation of common urologic complaints, such as hematuria and lower urinary tract symptoms. Vol. 18 No. 4 • 2016 • Reviews in Urology • 245 17_4004170006_RIU0705.indd 245 12/12/16 4:30 pm