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previously visited MULLERIAN REMNANT Random Case
Authored By: Edward Richer, Resident, Emory University, Georgia, USA.
Patient: 6 year 1 month old male
History: 6 year old boy with a palpable, usually painless mass between the testicles for past 6-9 months, recently getting bigger.  Urologist was able to express brown fluid from urethra after squeezing mass.
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Fig. 1: VCUG
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Fig. 2: VCUG

Fig. 3: VCUG

Fig. 4: VCUG

Fig. 5: US

Fig. 6: US

Fig. 7: US

Fig. 8: US
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Findings: Ultrasound: The inferior midline scrotum has a lobulated, mass lesion with a thick, echogenic rim and large central echogenic septation.  It appeared to have complex fluid properties  with some swirling and dependent internal debris.  The periphery of the 
mass and the septation contained color Doppler flow, but no definite 
internal flow was identified.  Dimensions were 4.4 cm craniocaudad x 1.6 cm anteroposterior x 2.2 cm transverse.

VCUG: An elongated outpouching was noted from the bulbous  urethra, which appeared to extend into the scrotum where the outpouching  was blind-ended.  The proximal portion of this outpouching was rounded in  appearance.  A septation was then noted, as seen with ultrasound, and a  widened neck was then filled.  The blind end of this outpouching is 
rounded in appearance.
DDx: Mullerian remnant, urethral diverticulum, syringocele, prostatic utricle, hydrocele, lymphangioma, chronic hematoma,  chronic abscess, atypical spermatocele, extratesticular dermoid, teratomatous lesion.
Diagnosis: Urethral diverticulum arising from the bulbous urethra.  Patient has been referred to urology and pathology is pending.
Discussion: Anterior urethral diverticulum, although uncommon, is the second most common cause of congenital urethral obstruction in boys. A diverticulum of the anterior urethra develops on the ventral surface of the penile urethra as a result of either incomplete development of the corpus spongiosum focally or incomplete fusion of a segment of the urethral plate. A lip of tissue may be seen around the diverticulum. As the diverticulum distends, the lip of tissue is pressed against the urethral wall and results in a valvelike obstruction. Congenital urethral diverticula have a wide spectrum of clinical manifestations, although most children are diagnosed in infancy with dribbling-type micturation or infection. The dribbling may be due to emptying of the diverticulum or to overflow incontinence. If the obstruction is distal, ballooning of the urethra may occur with voiding. VCUG is the key to diagnosis. During VCUG, the typical saccular diverticulum of the anterior urethra fills with contrast material and appears as an oval structure on the ventral aspect of the anterior urethra.

An uncommon diverticulum occurs with retention cysts of the Cowper ducts. The Cowper glands are paired paraurethral glands located in the urogenital diaphragm near the bulbous urethra. They are involved in the immune defense of the genitourinary tract, play a role in infertility, and secrete many glycoproteins. They can be visualized as a ductlike structure parallel to the urethra, sometimes associated with opacification of the gland at IVU. This finding can be differentiated from a fistula, contrast material extravasation, urethral duplication, or artifact by the course parallel to the urethra and the position of the orifice. A retention cyst of a Cowper gland is seen as a filling defect on the floor of the bulbous urethra during VCUG. The cyst can erode into the bulbous urethra, either spontaneously or after surgery, leading to filling of the cyst during voiding and a resulting diverticulum. Tubular or cystic dilatation of the Cowper gland duct has been called a syringocele.


The prostatic utricle is a short, blind-ending pouch located on the verumontanum (ie, the floor of the prostatic urethra) that represents a mesodermal remnant of the Müller tubercle formed by the fused, paired distal müllerian ducts. In males, the müllerian ducts regress under the influence of müllerian inhibiting factor produced by the fetal testis, leaving the prostatic utricle as a vestige. Because regression of the utricle is androgen mediated, utricular cysts are found with increased frequency in boys with other disorders, such as hypospadias or prune-belly syndrome. An opacified prostatic utricle is usually well demonstrated at lateral VCUG, appearing as a posterior urethral diverticulum. Occasionally, urethral diverticula may be gigantic.

References: Anomalies of the Distal Ureter, Bladder, and Urethra in Children: Embryologic, Radiologic, and Pathologic Features1

Teresa Berrocal, MD, PhD, Pedro López-Pereira, MD, Antonia Arjonilla, MD and Julia Gutiérrez, MD

http://radiographics.rsnajnls.org/cgi/content/full/22/5/1139#F22
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Additional Details:

Case Number: 9858148Last Updated: 10-30-2009
Rating:

4 ratings
Anatomy: Genitourinary (GU)   Pathology: Congenital
Modality: GU, USAccess Level: Readable by all users
Contained in: Featured Cases
Case has been viewed 1266 times.

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