A giant congenital posterior urethral diverticulum associated with renal dysplasia.

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Case Report
247 Urology Journal Vol 3 No 4 Autumn 2006
A Giant Congenital Posterior Urethral Diverticulum
Associated with Renal Dysplasia
Shahram Mousavi, Abdolrasoul Mehrsai, Mohammadreza Nikoobakht, Amir Reza
Abedi, Sepehr Salem, Gholamreza Pourmand
Urol J (Tehran). 2006;4:247-9.
www.uj.unrc.ir
Keywords: congenital, urethra,
diverticulum, mullerian ducts, renal
dysplasia
Urology Research Center, Sina
Hospital, Tehran University of
Medical Sciences, Tehran, Iran
Corresponding Author:
Shahram Mousavi, MD
Urology Research Center,
Sina Hospital, Hasan-Abad sq,
Tehran, Iran
Tel: +98 21 6671 7447
Fax: +98 21 6671 7447
E-mail: shahram_moosavi1353@yahoo.com
Received January 2006
Accepted July 2006
INTRODUCTION
There is a paucity of information
about congenital posterior urethral
diverticulum (PUD). In the English
literature, only 3 cases have been
reported.(1-3) We report a unique case
of PUD in association with renal
dysplasia.
CASE REPORT
A 19-year-old man with a history
of recurrent urinary tract infection
(UTI) due to Escherichia coli, chronic
urinary obstructive symptoms,
and incomplete bladder emptying
since childhood presented to
Sina Hospital. The patient did
not mention any history of the
urethral trauma, instrumentation,
or surgical intervention.
Physical examination revealed
a massive bladder distention up
to the umbilicus. Examination
of the external genitalia was
unremarkable. Serum creatinine
level was 1.3 mg/dL and other
biochemistry results were within
the reference ranges.
In the evaluations performed
by ultrasonography and renal
scintigraphy, the right kidney was
absent and the left one showed
compensatory hypertrophy. A large
bladder and a large mass in the left
side of the pelvis with multiple
hyperechoic masses were seen. On
CT scan, there was a large cystic
mass beginning from the lower pole
of the right kidney extending into
the pelvis (Figure 1). There was
another cystic mass, posterior to
the first one, which was at a lower
level in the pelvis. The left kidney
was normal with significant ureteral
dilatation. Voiding cystourethrography
(VCUG) showed a giant diverticulum,
communicated with the urethra,
shifting the bladder to the right side.
Also, left vesicoureteral reflux was
noted (Figure 2).
On urethrocystoscopy, an orifice was
seen adjacent to the verumontanum.
The ureteroscope was advanced into
a capacious pouch full of debris.
Its volume was approximately 500
milliliters. There was a long prostatic
urethra (approximately 4 cm) and
an elevated bladder neck. A huge
moderately trabeculated bladder with a
fully developed trigone was observed.
Although the left ureteral orifice was
Figure 1. A slice from the abdominopelvic CT scan. B indicates
the bladder; D, diverticulum; and U, the ureter.
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Congenital Posterior Urethral Diverticulum—Mousavi et al
248Urology Journal Vol 3 No 4 Autumn 2006
prominent, our effort to find the right ureteral orifice
was not successful. Posterior urethral valve (PUV)
was not detected in our evaluation.
A Foley catheter was inserted into the diverticulum.
The large diverticulum was dissected from the
surrounding tissues and the urinary bladder through
a lower midline incision; its communication with the
posterior part of the urethra was identified and the
diverticulum was resected completely (Figures 3 and
4). The right kidney was absent. The right ureter
was dissected completely. Reduction cystoplasty was
also performed. Foley catheters were placed into the
bladder and the perivesical space. The pathologic
evaluation of the tip of the resected ureter indicated
features of a dysplastic kidney. The wall of the
diverticulum consisted of the muscle cells and
squamous epithelium.
After the catheter removal, the patient experienced
a transient period of stress incontinence which was
spontaneously relieved after 2 months. Vesicoureteral
reflux disappeared on the follow-up VCUG. On
retrograde urethrography, there was no stricture. No
episode of UTI was reported during the 6 months’
follow-up. The only complication reported was
meatal stenosis that was corrected by meatotomy.
During the follow-up period, serum creatinine levels
were within the normal range with an average of 1.2
mg/dL (range, 0.9 mg/dL to 1.7 mg/dL).
DISCUSSION
Urethral diverticulum is an epithelium-lined
pouch that is formed because of either distention
Figure 3. Retrograde urethrography. Left, Preoperative image. Right, Postoperative image.
Figure 2. Lateral view of the bladder, the diverticulum, and the left
ureter. B indicates the bladder; D, diverticulum; and U, the ureter.
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to the urethra, they usually enter the midline of
verumontanum.(1)
Congenital Posterior Urethral Diverticulum—Mousavi et al
Urology Journal Vol 3 No 4 Autumn 2006
249
of a segment of the urethra or the attachment
of a structure to the urethra by a narrow neck (ie,
a Mullerian remnant).(4) This condition, especially
in men, is extremely rare and may be congenital or
acquired. An acquired diverticulum usually forms
due to infection, urethral stricture, and/or trauma.(5)
Majority of the cases with PUD are of the Mullerian
origin. The remainders are formed as a result of an
aborted urethral duplication. The Mullerian remnants
may be prostatic utricles or Mullerian duct cysts.(1)
Prostatic utricles do not usually require any treatment,
unless they become very large causing recurrent
UTIs or other complications.(4) Mullerian duct cysts
are cystic dilatations in the remnants of the distal
ends of the fused Mullerian ducts. They rarely
communicate with the urethra. If they are connected
Esposito and colleagues reported a giant congenital
PUD in a 4-year-old boy with an enlarged utricle.(2)
The 41-year-old patient described by Plank and
Scholen was a case of congenital PUD simulating
Mullerian duct cyst.(3) These authors concluded that
the detected cases of PUD were not of Mullerian
origin in neither of the articles mentioned. Similar
to Ng’s report,(1) all features of our case including its
midline location, well developed external genitalia,
and the absence of communication with genital tract
imply that this is a Mullerian duct cyst.
The method of treatment depends on the size of
the diverticulum and the degree of the obstruction.
Small and asymptomatic lesions may just be followed
up.(6) Excision of the symptomatic lesions is often a
surgical challenge. There are different approaches for
the symptomatic lesions. Classically, they are excised
through suprapubic, retorovesical, or transvesical
approaches(7,8); however, while some authors have
used this approach with an acceptable success rate,
others have shown a lower rate of complete excision
in their cases.(9) A posterior approach has been
advocated for the better maintenance of the erectile
response.(10) Perineal approach affords more direct
access for diverticula originating at the level of the
urogenital diaphragm.(3) Our case is unique due to
the existence of both renal dysplasia and congenital
PUD.
CONFLICT OF INTEREST
None declared.
REFERENCES
1. Ng WT. Congenital posterior urethral diverticulum.
Aust N Z J Surg. 1996;66:717-9.
2. Esposito G, Savanelli A, Tenore A, Tamburrini
O, Palescandolo P, Di Tuoro A. Congenital giant
diverticulum of the posterior urethra in a 4-year-old
boy. Z Kinderchir. 1986;41:244-5.
3. Plank LE, Schoen WA Jr. Congenital prostatic urethral
diverticulum simulating mullerian duct cyst. J Urol.
1960;84:144-6.
4. Jordan GH, Schlossberg SM. Surgery of the penis and
urethra. In: Walsh PC, Retik AB, Vaughan ED Jr, et al,
editors. Campbell’s urology. 8th ed. Philadelphia: WB
Saunders; 2002. p. 3903-4.
5. Laungani RG, Angermeier KW, Montague DK. Giant
urethral diverticulum in an adult male: a complication
of the artificial urinary sphincter. J Urol. 2003;170:
1307-8.
6. Mohan V, Gupta SK, Cherian J, Tripathi VN, Sharma
BB. Urethral diverticulum in male subjects: report of 5
cases. J Urol. 1980;123:592-4.
7. Smith JA Jr, Middleton RG. Surgical approach to large
mullerian duct cysts. Urology. 1979;14:44-6.
8. Monfort G. Transvesical approach to utricular cysts. J
Pediatr Surg. 1982;17:406-9.
9. Schuhrke TD, Kaplan GW. Prostatic utricle cysts
(mullerian duct cysts). J Urol. 1978;119:765-7.
10. Wesson MB. Cysts of the prostate and urethra. J Urol.
1925;13:605-29.
Figure 4. Postoperative voiding cyctourethrography
demonstrated no remained diverticula.
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