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- Case Report -
Congenital agenesis of seminal vesicle
Hong-Fei Wu, Di Qiao, Li-Xin Qian, Ning-Hong Song, Ning-Han Feng, Li-Xin Hua, Wei Zhang
Department of Urology, First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China
Abstract
Congenital agenesis of the seminal vesicle (CASV) is frequently associated with congenital absence of the vas
deferens (CAVD) or ipsilateral congenital vasoureteral communication. We reported two cases of a rare condition that the
vas deferens open ectopically into Mullerian duct cyst associated with agenesis of the ipsilateral seminal vesicle. The
diagnosis was confirmed by vasography. Transurethral unroofing of the Mullerian duct cyst was performed in both
patients with favourable results, however, assisted reproductive technology (ART) was still necessary for them to
father children. (Asian J Androl 2005 Dec; 7: 449-452)
Keywords: seminal vesicle; vas deferens; congenital abnormalities; vasography
Corresponence to: Prof. Hong-Fei Wu, Department of Urology,
First Affiliated Hospital of Nanjing Medical University, Nanjing
210029, China.
Tel: +86-25-8371-4511-6603, Fax: +86-25-8378-0117
E-mail: wuhf@njmu.edu.cn
Received 2004-11-08 Accepted 2005-05-23
DOI: 10.1111/j.1745-7262.2005.00058.x
1 Case report
The seminal vesicles can be congenitally absent at
one or both sides [1]. The incidence of unilateral
agenesis of the seminal vesicle is 0.6 %-1 % [2]; while the
incidence of bilateral agenesis is unclear. It is known
that congenital agenesis of the seminal vesicle (CASV)
may occur in patients with congenital absence of the vas
deferens (CAVD) or congenital vasoureteral communication [1]. CAVD is frequently found in association with
mutation(s) of the cystic fibrosis transmembrane
regulator (CFTR)gene [2-6]. While ectopia of the vas into
ureter is caused by incomplete absorption of the
common Wolffian duct [7], it is safe to say that CASV is
associated with CFTR gene mutations and defects in the
mesonephric duct. Herein we presented two cases of
CASV associated with ipsilateral ectopic vas into the
Mullerian duct cyst, which may complement what are
already known on CASV.
2 Case reports
2.1 Case 1
A 37-year-old man presented for infertility. Physical
examination revealed the right vas deferens was absent
with only a 5-cm-long rudiment ended in a node
approximate to the epididymis cauda, and the whole epididymis
was enlarged. The right testis and the left scrotal
contents were normal. Digital rectal examination was
unremarkable. Serum testosterone (T),
follicle-stimulating hormone (FSH) and luteinizing hormone (LH) were
normal. Semen analysis yielded azoospermia with low
seminal volume of 1.2 mL, low pH of 6.5 and a few
ery-throcytes. Semen fructose test was negative.
Ultrasonography and computed tomography (CT) demonstrated that the kidney, ureter, and bladder were normal,
except for a cyst inside the prostate on the midline (Figure
1 A), with a diameter of about 1.0 cm; both seminal
vesicles were absent. Bilateral testicular biopsy revealed
normal spermatogenesis. Left vasography showed the
vas deferens terminated ectopically into a cyst located at
the prostatic midline, as well as an absent left seminal
vesicle (Figure 1 B). Regarding the midline location of
the cyst, we considered it as Mullerian origin with
respect to the X-ray appearances of Mullerian duct cyst
proposed by Hendry and Pryor [8]. Transurethral
unroofing of the Mullerian duct cyst was performed. On
histological examination the cyst wall was lined with low
hyperplasic columnar and cuboidal epithelial cells.
Semen analysis 2 months after the operation demonstrated
asthenospermia (sperm motility of 20 %, and very poor
forward progression) with seminal volume of 2.0 mL,
pH of 6.4 and sperm density of
45.48 × 106/mL, with no erythrocytes could be found. Semen fructose test was
still negative.
2.2 Case 2
A 28-year-old man complained of hematospermia and
infertility. Physical examination was unremarkable.
Serum T, FSH and LH were normal. Semen analysis yielded
azoospermia and hematospermia. Semen fructose test
was equivocal. Ultrasonography and CT demonstrated
the kidney, ureter and bladder were normal, except for a
cyst inside the prostate on the midline (Figure 2 A), with
a diameter of about 1.5 cm. The right seminal vesicle
was absent and the left, hypotrophic. Bilateral
vasography revealed an ectopic right vas deferens ended in a
Mullerian duct cyst associated with the absence of
ipsilateral seminal vesicle, the left vas deferens crossed over
the midline, emerged with a hypotrophic seminal vesicle
and the ejaculatory duct terminated into the Mullerian
duct cyst (Figure 2 B). Transurethral unroofing of the
Mullerian duct cyst was performed and histological
examination was consistent with the diagnosis. Antibiotic
therapy was applied pre- and postoperatively. One month
later, the symptom of hematospermia disappeared.
Semen analysis 6 months after the operation revealed
asthenospermia (complete absence of sperm motility)
with seminal volume of 1.6 mL, pH of 7.4 and sperm
density of 36.73 × 106/mL. Semen fructose test was
mildly positive.
3 Discussion
Anomalies of the seminal vesicles can be categorized
into abnormalities of number (agenesis, fusion,
du-plication), maturation (hypoplastic), position (ectopia) and
structure (diverticulum, cyst, communication with the
ureter) [1]. Their significances lie in their frequent
associations with mal-development of other mesonephric
derivatives, such as the vas deferens, ureter and kidney.
Among these abnormalities, agenesis of the seminal
vesicle is perhaps the commonest. It was widely
accepted that if an embryological insult occurs before 7
weeks¡¯ gestation when the ureteral bud separates from
the mesonephric duct, the seminal vesicle anomaly may
be associated with renal malformation. If the insult
occurs after 7 weeks¡¯ gestation, the seminal vesicle
agenesis will not be associated with renal agenesis [2].
The etiology of CASV was not known, but it was often found in patients with CAVD [2-6] or ipsilateral
ectopia of the vas deferens [1, 7, 9-13]. Therefore,
defects in the mesonephric duct and mutations of the
CFTR gene are probably the main causes of CASV.
Although it was said that CASV might occur in patients
with renal agenesis [1], no cases of CASV have been
found with normal developed vasa deferentia.
Previous studies suggested that in patients with
congenital bilateral absence of the vas deferens (CBAVD),
the incidence of bilateral CASV was 23 %-43 %
[3-6, 14] and the incidence of unilateral CASV was 27 %-50 %
[3-6, 14]. In patients with congenital unilateral absence
of the vas deferens (CUAVD), the incidence of ipsilateral
CASV was 71 %-90 % [4, 6, 15] and contralateral CASV,
20 % [4]. In patients with ectopic vas deferens,
agenesis of ipsilateral seminal vesicle would be inevitable [7,
9-13]. However, crossed ectopia of the vas may occur
leaving the ipsilateral seminal vesicle at normal position
[16]. The second patient in this report was found to
have a crossed ectopic left vas emerging with a hypotrophic
seminal vesicle, semen fructose test was mildly positive
postoperatively, suggesting an under-functioning
seminal vesicle. CASV may be associated with renal
agenesis or other anomalies [1, 2], but the incidence was
unclear. Although the patients may be in the condition of
sterility, the testicular spermatogenesis is usually intact
[5, 12], as in case 1.
Seminal vesicle secretion may promote sperm motility,
increase stability of sperm chromatin and suppress the
immune activity in the female reproductive tract to avoid
rejection of spermatozoa and embryos that have
antigens foreign to women [17]. No specific symptoms or
signs are directly suggesting the diagnosis of seminal
vesicle agenesis. CASV is frequently associated with
CAVD or ectopia of the vas deferens, and patients with
CASV may present with infertility, yet few cases can be
found during routine ultrasonography or pelvic CT.
Patients with ectopic vas into the Mullerian duct cyst can
also manifest hematospermia, as the second case in our
study. If the cyst is large enough, perineal and
intrascrotal pain, dysuria, epididymitis, or urinary tract
infection may be presented[10]. Digital rectal examination
may reveal a cystic lesion in the prostate gland in patients with ectopic vas into the Mullerian duct cyst. In
patients with CAVD, the scrotal vas is nonpalpable, and
for most cases, the distal 2/3 segment of epididymis is
absent leaving an enlarged and firm caput. There is still
another kind of ectopia of the vas deferens where the
vas terminated ectopically into the urinary tract,
including bladder, posterior urethra, ureter or even kidney [7,
11-13]. Most cases of this condition were described in
children and were frequently associated with congenital
anorectal and other urogenital anomalies [7, 11, 13]. Our
patients fell within a rare subgroup of people with
ectopic vas-adults with infertility but without congenital
renal, ureteral, or anorectal malformations.
CASV is not an independent condition. As stated
before, it was always associated with CAVD or
ipsilateral ectopia of the vas deferens. The clinical importance
is usually underestimated and the diagnosis has been
frequently neglected. Herein we recommended that in
patients with infertility, vasal agenesis, low ejaculate volume,
low semen pH and low semen fructose, it was mandatory to further inspect the appearance of the seminal
vesicles. In patients with CAVD, vasography cannot be
performed owing to ductal agenesis [14]. Transrectal
ultrasonography (TRUS), CT and magnetic resonance imaging (MRI) were frequently used to detect seminal
vesicle anomalies, but these imaging tools are incapable
of diagnosing ectopia of the vas deferens. In patients
with vasal ectopia, the invasive vasography is still
necessary for confirmation. Abdominal ultrasonography, CT
scans or excretory urography are helpful to determine
the renal anomalies.
Congenital agenesis of the seminal vesicle is unreco-
nstructable and requires no treatment in the subgroup of
unilateral CASV with a potent contralateral ductal system.
In patients with infertility, hematospermia, recurrent
epididymitis, urogenital abnormalities, strategies
concerning CAVD and ectopic vas deferens must be taken into
consideration. Hall and Oates [15]have done well in
treating the subgroup of male infertility patients who
presented with CUAVD and contralateral ductal abnormalities.
Corrective surgical options in this subset include
vasoepi-didymostomy, transurethral resection of the ejaculatory
duct and microscopic epididymal/testicular/vasal sperm
aspiration [15]. The first case was within this subset, a
transurethral unroofing of the Mullerian duct cyst was
performed and semen analysis improved postoperatively.
The principles of treating ectopic vas deferens are to
prevent epididymitis and urinary tract infection, to pre
serve fertility and to release the outlet obstruction [9].
Treatment of infertility due to vasal ectopia must be
individualized [12]. Takahashi et al. [10] have reported a case with vasal ectopia into the Mullerian duct cyst. They
stated that open excision of the Mullerian duct cyst was
preferred if the symptoms were severe or if other modes
of therapy such as antibiotics had failed. The incisions
include transabdominal, perineal and posterior approach
[10]. We performed transurethral unroofing of the
Mullerian duct cyst in two cases, because an extensive
operation was not necessary. After operation, semen
analysis improved in both cases and the symptoms of
hemato-spermia in case 2 subsided.
Nowadays, assisted reproductive technology (ART)
is available for patients with unreconstructable ductal
obstruction. Microsurgical epididymal sperm aspiration
or testicular sperm extraction combined with
intracytoplasmic sperm injection (ICSI) have provided a viable
treatment strategy for affected individuals [18-20].
Because testicular spermatogenesis is rarely impaired in
patients with CASV, it is reasonable for them to undergo
microsurgical epididymal sperm aspiration or testicular
sperm extraction to obtain spermatozoa for ICSI.
Although ductal obstruction was eliminated and
semen analysis was improved in our patients after operation,
they remained infertility owing to the lack of functional
seminal vesicles, sperm harvesting from epididymis or
testis for ICSI is still necessary for them to start families.
Both of the patients are recommended to our Hospital
Center of Reproductive Medicine to receive ICSI.
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