Journal of Pediatric Urology (2017) 13, 52.e1e52.
e5
a
Department of Urology,
Katharinenhospital, Stuttgart,
Germany
b
Department of Pediatric
Urology, University Medical
Center, Regensburg, Germany
c
Department of Pediatric
Urology, Krankenhaus
Barmherzige Schwestern, Linz,
Austria
d
Department of Pediatric
Surgery, Paracelsus Medical
University, Salzburg, Austria
e
Department of Urology and
Pediatric Urology, Medical
University Ulm, Ulm, Germany
Correspondence to: F. Blasl,
Department of Urology,
Katharinenhospital,
Kriegsbergstr. 60, 70174
Stuttgart, Germany, Tel.: þ49
711 2783 3801; fax: þ49 711
2783 3809
f.blasl@klinikum-stuttgart.de
(F. Blasl)
Keywords
Cowper’s syringocele; Infraves-
ical obstruction; Urethrotomy
Received 7 October 2015
Accepted 29 August 2016
Available online 8 October 2016
http://dx.doi.org/10.1016/j.jpurol.2016.08.023
1477-5131/ª 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Cowper’s syringocele: A rare differential
diagnosis of infravesical obstruction in
boys and young adults
Felix Blasl a, Wolfgang H. Rösch b, Mark Koen c,
Mircia-Aurel Ardelean d, Anne-Karoline Ebert e
Summary
Objective and introduction
Cowper’s syringocele is a cystic dilation of the bul-
bourethral gland duct, initially defined by Maizels
et al. in 1983. Although obstructive and non-
obstructive types of this rare anomaly are described,
clinical symptoms are highly unspecific. Therefore, we
report 12 cases of children and young adults diagnosed
with Cowper’s syringocele to further clarify clinical
course, comorbidity and treatment strategies.
Study design
We retrospectively collected clinical data of 12
children and young adults from birth to 18.5 years
(median 7.2 years) who had been treated in four
different institutes during a period of 16 years. The
primary specific diagnostic work-up consisted of ul-
trasound, cystourethrography, and cystoscopy.
Results
3Older patients with a median age of 11.8 years
clinically presented with obstructive voiding pattern
or gross hematuria; infants with a median age of 0.6
years presented with febrile urinary tract infections
(UTIs). After cystoscopic confirmation in all patients,
endoscopic treatment was possible in nine; open
surgical resection was necessary in three patients.
Because of intrauterine megacystis and chronic
renal failure, one boy underwent suprapubic diver-
sion with a cystostomy soon after birth. Owing to
urological comorbidity or later complications,
Figure Prolonged, strained micturition in 11.25-year-old boy. IUCG and voiding cystourethrogram
showed a perforate, large Cowper’s syringocele.
additional procedures were necessary, for example,
resection of minor urethral valves, prophylactic
circumcision for UTI, endoscopic or open antireflux
procedures, and occasionally complex bladder re-
constructions in the long term.
Discussion
According to our data, the initial clinical symptoms of
Cowper’s syringocele were related to presenting age
rather than the previously described type of syrin-
gocele according to Maizels et al. Infants presented
with febrile UTIs; however, older boys and young
adults had mainly voiding problems or nocturnal
enuresis. Therefore, the clinical significance of the
described syringocele types must be questioned.
Eighty-three percent of our patients showed addi-
tional urological pathology such as vesicoureter-
orenal reflux, ureteropelvic junction obstruction,
megaureter, or minor urethral valves. Thus, Cowper’s
syringocele hardly seems to be an isolated pathology.
Conclusion
Although rare, Cowper’s syringoceles should be
considered in differential diagnosis of infravesical
obstruction in boys and young adults. Diagnostics are
usually justified by presenting symptoms such as UTI
or urinary flow impairment, which seem to be age
dependent. Despite modern diagnostic tools, diag-
nosis is usually made by cystourethrography and
sometimes accidentally by cystoscopy. Considerable
urological comorbidities and consecutive bladder
dysfunction need long-term follow-up.
Cowper’s syringocele in boys and young adults
Introduction
Cowper’s syringocele is a cystic dilated duct of the bul-
bourethral glands [1]. In 1983, Maizels et al. [2] described
different types of syringoceles that could be divided into
obstructive and non-obstructive pattern according to their
morphology and clinical symptoms (Fig. 1) [3]. Prevalence is
unknown [4], and the available sparse literature reports on
small patient cohorts or single case studies. Diagnosis of
Cowper’s syringocele is usually made during voiding cys-
tourethrography (VCUG) or urethrocystoscopy. Although
computed tomography scans or magnetic resonance imag-
ing may provide a wider set of anatomical details, consid-
ering cost, availability and exposure to radiation, today
only voiding urosonography seems to be an additional
method worth considering for standard work-up [5,6].
Standard treatment consists of visual endoscopic cold-knife
urethrotomy for obstructive and non-obstructive syringo-
celes, with open surgical procedures rarely needed [3]. To
further clarify the clinical course, urological comorbidity,
and treatment strategies of Cowper’s syringocele, we pre-
sent a retrospective multicenter case collection of 12
patients.
Data and methods
We retrospectively reviewed the clinical records of 12 boys
and young adults, diagnosed and clinically surveyed in four
Figure 1 Cowper’s syringocele, as described by Maizels et al. [2]: (1) simple, (2) perforate, (3) imperforate, and (4) ruptured
syringocele.
52.e2
pediatric urology centers during a 16-year period. The
anonymous files of patients were analyzed according to a
standardized evaluation form. Because of the small cohort,
statistical analysis mainly made use of descriptive methods,
including counts, means, and ranges.
The study was submitted to, reviewed, and approved by
the institutional ethics committee in Ulm, Germany.
Results
The median age at presentation was 7.2 years, ranging from
birth to 18.5 years. Follow-up of the included patients
ranged from 1 month to 16 years (median 3.2 years). One
patient was lost to follow-up, but the others underwent
routine follow-up at the participating centers.
Initial clinical symptoms of Cowper’s syringocele were
related to presenting age (Table 1). Five children, age
range 0.5e1.6 years (median 0.6 years), presented with
febrile urinary tract infection (UTI) (42%). The youngest
child with prenatally diagnosed megacystis had a supra-
pubic catheter inserted immediately after birth. After
stabilization a vesicostomy was created, which was closed
at the age of 3 years after previous endoscopic resection of
the syringocele. However, initial prenatal kidney damage or
dysplasia of the kidneys had already led to chronic renal
failure. In the older group with a median age of 13.8 years
(ranging from 5.5 to 18.5 years), five patients (42%) pre-
sented predominantly with obstructive voiding pattern,
such as a weak stream, hesitancy, and straining, and only
52.e3 F. Blasl et al.

Table 1 Patient data.

Age Signs and symptoms Urological comorbidities Diagnostics in Surgical procedures
(at presentation, addition to
in years) ultrasound
0 Intrauterine urinary Intrauterine megacystitis, VCUG Vesicostomy,
retention chronic kidney failure urethrotomy
0.25 Febrile UTI Left dilating reflux, renal VCUG renal scan Circumcision, surgical
function impairment, left resection of the urethral
kidney 30%, stone within the diverticulum
syringocele
0.25 Febrile UTI Non-refluxive and obstructive VCUG, renal scan Urethrotomy,
left megaureter, ureter fissus circumcision
0.25 Febrile UTI Left UPJ obstruction VCUG, renal scan Urethrotomy,
circumcision
0.66 Febrile UTI Minor urethral valves, bilateral VCUG, renal scan PUV resection,
non-obstructive, non refluxive urethrotomy,
megaureter circumcision
1.6 Febrile UTI Secondary left dilating reflux VCUG Surgical resection of the
urethral diverticulum
5.5 Febrile UTI, prolonged, Secondary bilateral reflux II VCUG, renal scan, Urethrotomy, bilateral
strained micturition, uroflowmetry HIT (Deflux)
nocturnal enuresis
11.25 Prolonged, strained None VCUG, Surgical resection of the
micturition uroflowmetry urethral diverticulum
12.3 Prolonged, strained Minor urethral valves VCUG, renal scan, Urethrotomy, PUV
micturition, nocturnal uroflowmetry resection
enuresis
17.25 Prolonged, micturition Scarred phimosis Uroflowmetry Urethrotomy,
circumcision
18 Prolonged, micturition, Secondary bilateral reflux I MRI of the spine First CIC, Urethrotomy,
residual urine VCUG, video Conservative bladder
urodynamics, management
neurologic work-
up
18.5 Hematuria None None Urethrotomy
CIC Z clean intermittent catheterization; HIT Z hydrodistention implantation technique; MRI Z magnetic resonance imaging;
PUV Z posterior urethral valve; UPJ Z ureteropelvic junction; UTI Z urinary tract infection; VCUG Z voiding cystourethrography.

one patient had gross hematuria (8.3%). A 5.5-year-old boy
had a febrile urinary tract infection in addition to impedi-
ment of urine outflow and nocturnal enuresis. An 18-year-
old patient presented with a severe dysfunctional bladder
emptying requiring clean intermittent catheterization and
a comprehensive neurological work-up.
Initial diagnostics started with a clinical examination
followed by renal and bladder ultrasound in all patients. In
seven cases, pathology of the upper tract was detected by
ultrasound. Further clarification by renal scans was needed
in six cases. Ten of 12 patients underwent a VCUG. In five
patients these tests were performed after a history of
febrile UTIs and with kidney abnormalities on ultrasound
according to current guidelines. Four VCUGs were per-
formed to evaluate obstructive voiding pattern and one was
done because of prenatal suspicion of posterior urethral
valves (PUVs). One young adult patient with gross hema-
turia and one older patient with phimosis, obstructive
micturition, and considerable residual urine did not have a
VCUG before cystoscopy. In three of the five infants with
UTI and in a further four patients, the syringocele was
radiologically suspected during VCUG (Fig. 2, and Summary
figure). In the other five cases, the syringocele was solely
and accidentally detected during cystoscopy. Uroflowmetry
was performed in all boys above the age of 5 years. The
flow pattern reached a plateau in four cases, with a mean
voided volume of 300 mL (range 200e400 mL), Qmax flow of
6.15 mL/s (range 5.5e6.8 mL/s), micturition time of 43.5 s
(range 38e49 s), and mean residual urine of 137.25 mL
(range 75e200 mL). Nine of our 12 patients were treated
with visual endoscopic urethrotomy (75%). Owing to the
retrospective nature of the case collection it was not
clearly stated in every single case whether this incision was
performed with a cold knife or diathermia.
The only patient with a ruptured syringocele did not
undergo a VCUG. He was diagnosed and treated cys-
toscopically by cold-knife unroofing of remnants of a
syringocele located in the bulbar part of the urethra. One 8-
month-old baby was first diagnosed and treated for minor
urethral valves. When follow-up VCUG showed persistent
Cowper’s syringocele in boys and young adults
Figure 2 One-month-old boy following an episode of a
febrile urinary tract infection with the initial suspicion of a
posterior urethral valve. Voiding cystourethrogram showed an
imperforate Cowper’s syringocele.
infravesical obstruction, a second cystoscopy revealed an
obstructive Cowper’s syringocele, which could then be
treated. Three open surgical procedures were indicated,
mainly because of the expanse of the syringocele and the
presence of a contained stone.
Additional urologic pathology was seen in 83% of the
patients. Reflux, primary or secondary, was the most
common entity (33%). Two patients showed initial unilat-
eral dilating vesicoureterorenal reflux: one in a normal
kidney and one in a 30% hypo-dysplastic kidney. Two older
patients had secondary bilateral mild vesicoureterorenal
reflux; one of them was treated with a bilateral hydro-
distention implantation technique procedure after infra-
vesical desobstruction. One child had ureteropelvic
junction obstruction (UPJO) without urodynamic relevance.
Two boys had non-refluxive non-obstructive megaureters
with a regular function on follow-up renal scans; one of
them had a left-sided ureter fissus. Two patients had minor
urethral valves in addition to the syringocele pathology.
Five circumcisions were done: one to cure a scarred phi-
mosis, the other four as a prophylactic procedure to further
reduce UTIs.
During follow-up, five infants remained without residual
signs or symptoms after infravesical desobstruction. One
boy was lost to follow-up. Five older patients at the pre-
senting ages of 5.5, 12.3, 17.25, 18, and 18.5 years (median
14.3 years) and the prenatally detected case were under
active surveillance because of ongoing dysfunctional
bladder emptying. One patient who presented at the age of
1.6 years developed a severe bladder dysfunction with
secondary bilateral vesicoureterorenal reflux. He conse-
quently needed antireflux procedures and further opera-
tions to optimize bladder storage and emptying function. In
addition to urological comorbidities, only one patient had
diabetes mellitus type 1 and an additional ventricular
septal defect.
52.e4
Discussion
Bulbourethral glands have been known since the 17th
century, when they were described by Jean Méry (1684) and
William Cowper (1699) [1]. The Cowper glands are situated
in the bulbus of the spongy body of the penis and are
connected to the male urethra via several ducts. The
secretion produced by the Cowper glands is an important
part of the seminal fluid and lubricates the male urethra
before ejaculation [7]. Maizels et al. [2] were the first in
1983 to categorize the bulbourethral glands and its ducts
and described potential pathophysiology. They identified
four different types of a dilated connecting duct and,
mainly because of their appearance, named them syringo-
cele. Syringocele is a synthesis of the Greek words that
stand for tube (syringos) and swelling (cele). Simple syrin-
goceles constitute a swelling of the duct, a perforate
syringocele presents as a swollen duct that is openly con-
nected to the urethra, an imperforate syringocele has no
connection to the urethra and therefore is similar to a
submucosal cyst, and a ruptured syringocele simply consists
of the remaining membrane after rupture of a swollen duct
[2]. These types of malformation are believed to cause
different symptoms and were clinically classified into
obstructive and non-obstructive syringoceles [3]. The term
non-obstructive syringocele describes perforate and
ruptured syringoceles that usually lead to symptoms like
post-void dribbling or episodes of hematuria. Obstructive
syringoceles are equivalent to simple and imperforate
syringoceles and usually cause prolonged and straining
micturition or perineal swelling [8]. Only one patient in the
cohort had by definition a non-obstructive syringocele,
implicating that this is a very rare entity. Cowper’s syrin-
gocele may be an adequate differential diagnosis or even
be linked to a number of other urological malformations,
such as posterior or anterior urethral valves. An appropriate
example can be found in the work of McLellan et al. [9],
where the rare entity of an anterior urethral valve was
suspected to be a remnant or distal formation of a ruptured
syringocele. Knowledge about further anomalies associated
with Cowper’s syringocele has not been provided in the
literature [4]. The pathophysiology of an infravesical
obstruction may be similar to posterior urethral valves, and
in particular an imperforated Cowper’s syringocele may
lead to neurogenization of the bladder and secondary ves-
icoureterorenal reflux disease, or in severe cases even to
intrauterine or perinatal death [10]. The current cohort was
collected randomly in the participating centers and showed
an age distribution that is similar to earlier studies [3].
Initial clinical symptoms of the Cowper’s syringocele in our
cohort were related to presenting age rather than the type
of syringocele, according to Maizels et al [2]. Infants pre-
sented with febrile UTIs; older boys and young adults had
mainly voiding problems or nocturnal enuresis. Therefore,
the clinical significance of the described syringocele types
must be questioned. Clinical presentation was almost
exclusively the obstructive type, but with a wide range of
consequences for bladder function and the upper tract,
such as VUR and megaureters. There was one severe case of
prenatal megacystis that was detected by ultrasound and
treated with primary suprapubic diversion soon after birth.
52.e5
The majority of Cowper’s syringoceles could be estab-
lished by a VCUG. However, in 42% of the cohort, syringo-
celes were an incidental finding during cystoscopy.
Clinically relevant differential diagnosis such as PUV or
minor urethral valves must be considered too, and can be
coincidental with or secondary to a syringocele. This is a
striking fact, because 83% of our patients showed addi-
tional, in some cases probably secondary, urological pa-
thology, for example vesicoureterorenal reflux, UPJO,
megaureter, or minor urethral valves. Considering the
above, and despite missing “hard” facts in literature,
Cowper’s syringocele hardly seems to be an isolated pa-
thology. Treatment is mostly endoscopic, but there are no
clear recommendations of which morphological criteria will
lead to an endoscopic or an open surgical treatment.
Pragmatically, associated pathology such as diverticula
stones or the not quantified size seem to be arguments for
an open procedure. Endoscopically, infravesical obstruction
in childhood can be managed either with the cold knife or
with the diathermy hook. In the literature, comparative
data about the outcome of both incision methods are
scarce. However, in posterior urethral valves cold knife
incision was reported to be of equal efficiency, but with a
lower postoperative stricture rate [11]. Depending on the
dimension of the syringocele, a cold knife simple incision
will usually be effective. However, there is no indication in
the literature whether unroofing or simple incision is the
method of choice for an unobstructed urinary stream and
therefore a good unrestricted outcome.
Conclusion
As a recent case report [5] clearly demonstrates, Cowper’s
syringocele is still a rare and little-known cause of infra-
vesical obstruction, but should be considered in differential
diagnosis of infravesical obstruction in boys and young
adults. Diagnostics are justified by the presenting symp-
toms such as UTI or urinary flow impairment, which are age
dependent. Despite modern diagnostic tools, diagnosis is
usually made by cystourethrography and ultimately and
sometimes accidentally via cystoscopy. Besides raising
awareness of Cowper’s syringocele, the current series
strives to underline the importance of regular follow-up.
Neurogenization of the bladder and consecutive emptying
disturbances seem to be long-term consequences of infra-
vesical obstruction caused by Cowper’s syringocele. These
conditions and considerable urological comorbidities call
F. Blasl et al.
not only for regular observation but also for possible further
medical or if needed surgical treatment.
Conflict of interest
None.
Funding
None.
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