ACCESSORY PHALLIC URETHRA IN ADULT FEMALE JACOB RAMON, M.D. JOHN A. MEKRAS, M.D. GEORGE D. WEBSTER, M.B., ER.C.S. From the Division of Urology, Duke University Medical Center, Durham, North Carolina

ABSTRACT--Duplication of the urethra with an accessory phallic urethra in females is a rare anomaly usually discovered early in life because of associated genitourinary anomalies. We report on a thirty-three-year-old female with this anomaly who presented with double urinary stream and recurrent urinary tract injections. The nature of this anomaly, its diagnosis, and management are reviewed.

Urethral duplication is a rare anomaly which occurs almost exclusively in males. It may occur in association with bladder duplication, but more commonly it is an isolated phenomenon. In the female with a single bladder, the combination of urethral duplication with an accessory phallic channel is rare, and only 17 cases have been reported of which only 2 were adults.~'2 In females, duplication of the urethra most commonly presents early in life because of the presence of associated genital anomalies, and its clinical course into adulthood is unknown. Of the previously reported adult cases, one was a sixteen-year-old patient who presented with an accessory phallic urethra, 2 and the second was a fifty-three-year-old woman who presented with an accessory channel that ran parallel and below the normal urethra. 3 Both patients suffered from recurrent urinary tract infections. Failure to recognize the nature of this anomaly in adults may lead to a management dilemma as was the case in our thirtythree-year-old female patient. Case Report A thirty-three-year-old white woman was referred for evaluation of "an unusual anatomic variation of a clitoral urethra." Since childhood she had voided predominantly through a vaginally displaced urethra, but with an accessory 280

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small urinary stream exiting from the clitoris~: Directed horizontally, this second stream would i wet her clothing. She also had known duplication of the uterus and vagina, but this had not:. prevented fertility, and she had delivered 4 children by cesarean section. She gave a history of recurrent urinary tract infections which had been evaluated by cystoscopy, demonstrating cystitis cystica, and excre~ tory urography, demonstrating normal upper urinary tracts. Her referring physician had peri formed a retrograde urethrogram through the accessory clitoral urethra demonstrating the narrowed access~u,y channel with a fusiform dii lated section in its proximal portion (Figs. 1 andi: 2). He had fulgurated the clitoral opening, andi~ this had aggravated her symptoms because o b ~ struction of the channel had led to painfuI~ engorgement during voiding, and s t a g n a t i o ~ had increased the incidence of urinary tract in~i~ fections. Following each void, a tender cystic~ lesion developed inferior to the symphysis:~ which she would evacuate by compression (pre!ii~ sumably evacuating urine back into the bladi:!~ der). On referral, the patients history was a!i~ above and included dyspareunia Examination showed a slightly protuberant," normally 10! tedaclitoris, with no visible opening. Ther~ n elongated distance of interlabial tissu! oLocY

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"Mascu phallic

"Female" orthotopic urethra

FIGURE 2. this case.

F m u a E 1. Retrograde urethrocystography and ::iVoiding cystogram demonstrating "masculine" ac" ;cessory urethra, and vaginally displaced "feminine" urethra (arrow). .~ from the base of the tissue back to the normali~ appearing but intravaginally displaced urethral m e a t u s . The accessory urethra was not palpa::ble Vaginal examination confirmed a duplicat i o n of the vagina in its proximal two-thirds. Under anesthesia, and with a plan to proceed to excision of the accessory channel, initial cyst! ;tography failed to fill the accessory system. Ret;:i:rograde cystourethroscopy revealed a normal, b u t foreshortened urethra and a normal blad:!~ der neck and bladder. The point of entry of the ~ c e s s o r y anterior urethra could not be disileerned by retrograde cystoscopy, and hence antegrade cystoscopy was performed after percuii~aneous s u p r a p u b i c i n t r o d u c t i o n of t h e ;ystoscope. This study showed a small, normalppearing accessory bladder neck directly venral to the orthotopic bladder neck. It was )atheterized with a guide wire which ob!strutted in its distal portion of the site of prior rethral obliteration. he patient in the lithotomy position, a vertical interlabial incision was made teeessory urethral channel located and

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Schematic representation of anomaly in

dissected proximally and distally. In the subpubie space the fusiformly dilated seetion was identified, dissected, and transsected at the point of communication with the bladder (at the aeeessory bladder neek). The bladder was closed primarily. The fusiformly dilated section was thick-walled but was separate from the fascial surrounds of the orthotopic urethra which remained identified by an indwelling Foley catheter during dissection. The patient's postoperative reeovery was uneventful and efficient voiding and continence was re-established and no further infections occurred. Pathologic examination of the resected speeimen confirmed urethral duplieation, and in the dilated fusiform section stratified squamous epithelial mucosa with subjacent skeletal muscle and layers of smooth muscle were found. Comment A review of the literature shows that complete urethral duplication reflects a spectrum of disease and may present with other obvious anomalies. ~-7 Bonney et al. 4 found that in the female, urethral duplication appears in two patterns. In one pattern the main urethra is vaginal or perineal and the accessory urethra is subcorporeal, and in the other pattern both urethras are perineal or vaginal. The first pattern represents a sagittal duplication, and the second pattern represents a collateral duplication. When the accessory urethra is in the parallel pattern (second pattern), the pathology is difficult to define. In I case, it was suspected because of its association with double clitoris, ~ but otherwise it may not be suspected and may be discovered usually during evaluation of recurrent urinary tract infection. This may be discovered at a young age ~ or up until the age of fifty-three. 3 Accessory phallic urethra is the rule

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in the sagittal urethral duplication, although a case of a female infant with accessory urethra exiting further dorsally than the clitoris was reported. 7 Female subjects with accessory phallic urethra share some basic common features. 1 These include a prominent clitoris with lack of ventral curvature, accessory phallic urethra with male configuration, anomalous labioscrotal elements, abnormal vaginal introitus (introital stenosis, absent vaginal vestibule, absent hymen, and posterior diplaeement), and deficient perineal body. The radiographic appearance of the accessory urethra is masculine, appearing elongated, usually with a slightly proximal dilatation resembling a prostatic fossa. The "female" component of the duplicated urethra in these patients usually enters the anterior vaginal wall high and just inside the introitus (Figs. 1 and 9.). Bellinger and Duekett, 1 in an attempt to explain this anomaly, the spectrum of associated anomalies, suggested that accessory phallic urethra is the consequence of an uncoordinated interaction between the embryonal duetal elements. 1 Abnormal descent of the miillerian duets results in posterior displacement of the vaginal introitus, allows the accessory phallic urethra to form along a male pattern from the inferior urogenital sinus, and results in an unusually prominent phallus. Associated bifid vagina or uterus is explained by failure of normal fusion of the paired mfillerian structures. The clinical presentation will depend on the type of duplication present and on the associated anomalies. Abnormal vaginal introitus, anomalous labial elements, and clitoral prominence or hypertrophy may raise the suspicion of this anomaly in female newborns. At an older age, it may present as a double urinary stream, urinary incontinence, or recurrent urinary tract infeetion.

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Investigation should include retrograde urethrography through the accessory channel, and voiding eystourethrography in an attempt to delineate the anatomy of the anomaly. Endoscopy is also useful, but may need to be performed with a flexible instrument or in an antegrade fashion in view of the anterior/vertical location of the accessory bladder neck. Treatment is only indicated in symptomatic patients, occasionally mild urinary tract infee, ::i tions being manageable by prophylactic antibaeterials. Surgical excision is indicated in cases : of urinary incontinence, problematic voiding (misdirected stream as in this case), or uneon trolled urinary tract infections. Clitoral reloea tion, etc., have been reported.1 When surgical intervention is performed, it should include excision of the accessory channel and closure of the accessory bladder neck, tab ing care to avoid injury to the orthotopic urethra. Dissection of the clitoral portion of t h e aeeessory ehannel may be avoided, reducing; the risk of damage to its innervation, and if dis: connected from the bladder this should remain asymptom atie. Box 3146 Duke University Medical Center ii Durham, North Carolina 2771 (DR. WEBSTElq Referenees i. Be]lingerMF, and Duckett JW: Aeecssoryphallicurethrai n the female patient,J Urol 127:1159 (1982). 2. Glenn JF: Editorial comment, J Urol 127:1164 (1982). 3. Susan LP, Both RB, and Kaminsky AF; Complete dupliea:; tion of urethra, Urology 5:390 (1975). 1 4. Bonney WW, Young HH, Levin D, and Goodwin WE~ Complete duplication of the urethra with vaginal stenosis, J Urol 113:132 (1975). 5. Boissonnat P Two cases of complete double functional urethra with a single bladder, Br J Urol 33:453 (1961). 6 DeNieola RR, and MeCartney RC Urethral duplication in~, a female child, J Urol 61:1065 (1949) :~i 7. Kaur M and Pleasure JR: Complete duplication of the fe;'.!{ male urethra in a neonate, Clin Pediatr 21:759 (1982).

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Accessory phallic urethra in adult female.

Duplication of the urethra with an accessory phallic urethra in females is a rare anomaly usually discovered early in life because of associated genit...
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