108 Journal of Indian Association of Pediatric Surgeons / Jul-Sep 2011 / Vol 16 / Issue 3

An ultrasonogram of abdomen confirmed a patent

urachal tract from the umbilicus to the bladder with
normal kidneys. A retrograde urethrogram (RGU) was
done by injecting contrast (Urograffin) through the two
urethral openings. The two tracts for urinary outflow
were made out in the RGU as shown in Figure 2. The
dorsal urethra ending at the tip of penis was very
narrow, irregular and stenosed at few sites with some
dilatation near the bladder neck. The ventral urethra
opening at the penoscrotal junction was of good caliber
and relatively smooth. Both the urethrae were having
INTRODUCTION
Congenital patent urachus associated with urethral
duplication is a very rare association. There are only two
such cases in the English literature. We are reporting
one such case, in which the patient also developed
testicular teratoma during follow up.
CASE REPORT
A seven day old male baby born at 39 weeks of gestation
to a primi mother with a birth weight 2.45 Kg was brought
for not passing urine since birth. After the umbilical
cord stump had fallen, on the 5
th
day the mother noticed
soakage of clothes and flow of urine from umbilical
region for two days. On examination, an intermittent
stream of urine was coming from the inferior margin of
the umbilicus suggestive of patent urachus. On palpation,
abdomen was soft with no mass palpable. Genital
examination showed normal size penis and scrotum with
descended testes. There were two urethral openings - one
at the tip of penis and another at the penoscrotal junction
without associated chordee. The urethral meatus at the
tip of penis (dorsal) was admitting a 3F ureteric catheter
which could be passed only upto 5 cm length whereas
the meatus at the penoscrotal junction (ventral and
hypospadiac) was admitting a 6 size infant feeding tube
and giving access to the bladder as shown in Figure 1. An
improvement in the urinary stream from the hypospadiac
ventral opening and a reduction in urine leak from patent
urachus were noticed after the urethral calibration.
Case Report
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DOI: 10.4103/0971-9261.83495
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ABSTRACT
In this paper, we aim to highlight a case report of a rare association of anomalies
comprising patent urachus, double urethra and development of testicular teratoma.
KEY WORDS: Hypospadias, patent urachus, teratoma of testis, urethral duplication
Patent urachus with double urethra and testicular teratoma:
A rare association
Munisamy Ragavan, Haripriya Uppalu, Shani Prem
1
, Janarthanam Sarvavinothini
2
Departments of Pediatric Surgery,
1
Radio Diagnosis,
2
Anesthesia, Narayana Medical College and Super-Speciality
Hospital, Nellore, Andhra Pradesh, India
Address for correspondence: Dr. M. Ragavan, Department of Pediatric Surgery, Narayana Medical College and Super-Speciality
Hospital, Nellore, Andhra Pradesh - 524 002, India. E-mail: dr_ ragavan_2001@rediffmail.com
Figure 1: Catheters passed into the bladder through two urethral
openings and patent urachus. (The urethral meatus at the tip of penis
(dorsal) was admitting a 3F ureteric catheter whereas the meatus at
the penoscrotal junction (ventral and hypospadiac) was admitting a
6 size infant feeding tube giving access to the bladder)
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109 Journal of Indian Association of Pediatric Surgeons / Jul-Sep 2011 / Vol 16 / Issue 3
Ragavan, et al.: Patent urachus with double urethra
separate origin from the bladder, which was confirmed
by an isolated imaging study for individual urethra.
Thus, diagnosis of a patent urachus associated with a
complete and hypospadiac urethral duplication with
normal kidneys and ureters was made. Cystoscopy
could not be done as we were lacking such a small
sized cystoscope. A good urinary stream through the
hypospadiac ventral urethra, confirmed by saline
infusion via the patent urachus, prompted us to close
the patent urachus.
The abdomen was opened under general anesthesia
through a small Pfannenstiel incision. The patent
urachus was identified with the guidance of in situ
feeding tube, transfixed and excised. Post-operatively,
the bladder was drained through the hypospadiac
opening for 7 days. There were no postoperative
complications. Urethral reconstruction was planned for
the baby at the age of 2 years. At follow up, the baby
was passing few drops of urine from the tip of penis
and a good stream from ventral urethra.
Three months after the surgery, he developed left side
hard testicular mass measuring 1.5 cm 1.5 cm arising
from the lower pole. An ultrasonogram showed mixed
echogenic heterogeneous mass. Serum Alpha feto
protein and beta human chorionic gonadotrophic (HCG)
hormone were normal. Left side high orchiectomy and
testicular prosthesis insertion was done. The biopsy of
the testis showed features of teratoma.
In two years follow-up he was doing well and hence
submitted for urethral reconstruction. Cystoscopy
done via hyposapadiac ventral urethra showed normal
caliber urethra with no communication with the other
urethra. The verumontanum was not present. The
dorsal urethra was admitting only 3 F ureteric stent up
to the penoscrotal junction and the 9.5 F cystoscope
could not be passed even after dilatation. Since the
dorsal urethra was good caliber at least from penoscrotal
junction to tip of penis and its course towards bladder
was irregular and stenotic, we planned for end to side
urethro-urethrostomy of the external end of ventral
hypospadiac urethra to the side of the ventral urethra.
The ventral urethra was mobilized and the corpus
spongiosum and dorsal urethra was slit opened in the
midline at penoscrotal junction. The end of ventral
urethra was anastomosed to the side of dorsal urethra
by interrupted stitches with 6-0 PDS suture with a 3 F
ureteric stent in situ across the anastomosis. Thus a Y
shaped urethra was created which will be having good
urine flow from the bladder via the ventral urethra and
exiting via the dorsal urethral meatus at the tip of penis.
The stent was removed on 7
th
post operative day and
there was no fistula. He is passing urine in good stream
and continent.
DISCUSSION
Urethral duplication is a rare congenital anomaly.
[1]

Patent urachus is a communication between the dome
of the bladder and the umbilicus. It results when there
is a persistence of allantoic remnant which normally
gets obliterated during embryological development. It
is often associated with lower urinary tract obstruction
like posterior urethral valve, urethral atresia etc.
[2]
There
are only two case reports of urethral duplication with
an associated patent urachus in the English literature.
There is no case report of such a case developing
testicular teratoma.
In urethral duplication, one urethra will have a
normal course and the other remains as an accessory.
Embryology of urethral duplication is unclear and
many hypotheses have been proposed which include
ischemia, abnormal Mullerian duct termination,
growth failure of the urogenital sinus etc.
[1,3]
Widely
accepted classification for male urethral duplication is
by Effman.
[4]
Blind incomplete urethral duplication is
encountered more frequently than other types
of urethral duplication in clinical practice. A similar
case of urethral duplication with posterior urethral
valve involving the ventral urethra is described by
Ramanujam et al.
[5]
The dilated part or abnormal
angulation at the origin of the urethrae from the
bladder neck may theoretically cause urinary outflow
obstruction which can lead to patent urachus.
The embryological background for the coexistence of
urethral duplication with patent urachus is difficult to
explain. We believe the hypotheses of Vaos
[6]
that both
anomalies are due to early insult to cloaca development
needs further confirmation. Patent urachus may be
due to lower urinary obstruction,
[7]
and the double
urethra may be a separate embryological error.
Differential diagnosis includes acquired fistulous tracts
Figure 2: Retrograde urethrogram showing two separate urethra
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110 Journal of Indian Association of Pediatric Surgeons / Jul-Sep 2011 / Vol 16 / Issue 3
Ragavan, et al.: Patent urachus with double urethra
(post infective and post-instrumentation), urethral
diverticula and dilated Cowpers gland. Indications for
surgery in double urethra include annoying symptoms
such as a double stream, urinary incontinence,
urinary obstruction or associated genitourinary
anomalies. Treatment for urethral duplication should
be individually tailored. An endoscopic urethrotomy
and a distal septotomy are advised. For Y-type
duplication with normal and continent dorsal urethra,
perineal excision alone can be successful,
[3,8,9]
and if the
dominant urethra is ventral, progressive augmentation
by dilatation of the urethra can be done; and, if still
unsuccessful a multistage urethral reconstruction is
necessary like posterior hypospadias. Double urethra
cases with one urethra ending in seminal vesicles or
vas deference can develop epididymo-orchitis which
can mimic testicular tumor.
To conclude, double urethra is a very rare anomaly with
varied presentation and management. An associated
anomaly needs to be addressed and treatment is
tailored to the individual. Double urethra with patent
urachus may be isolated anomalies. Its association with
testicular teratoma is not yet described.
REFERENCES
1. Podesta ML, Medel R, Castera R, Ruarte AC. Urethral
duplication in children: Surgical treatment and results. J Urol
1998;160:1830-2.
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A. Persistent patent urachus with allantoic cyst: A case report.
Ultrasound Obstet Gynecol 1997;10:366-8.
3. Haleblian G, Kraklau D, Wilcox D, Duffy P, Ransley P, Mushtaq I.
Y-type urethral duplication in the male. BJU Int 2006;97:597-602.
4. Effmann EL, Lebowitz RL, Colodny AH. Duplication of
urethra. Radiology 1976;119:179-85.
5. Ramanujam TM, Sergius A, Usha V, Ramanathan S. Incomplete
hypospadiac urethral duplication with posterior urethral valves.
Pediatr Surg Int 1998;14:134-7.
6. Vaos G, Zavras N. Congenital patent urachus associated with
incomplete urethral duplication: A rare association. Eur J Pediatr
Surg 2006;16:130-2.
7. Cudaa PS, Vanasupaa BP, Sutherland RS. Nonoperative
management of a patent urachus. J Pediatr Surg 1998;33:1441-2.
8. Wagner JR, Carr MC, Bauer SB, Colodny AH, Retik AB,
Hendren WB. Congenital posterior urethral perineal fistulae. A
unique form of urethral duplication. Urology 1996;48:277-80.
9. Bates DG, Lebowitz RL. Congenital urethroperineal fistula.
Radiology 1995;194:501-4.
Cite this article as: Ragavan M, Uppalu H, Prem S, Sarvavinothini
J. Patent urachus with double urethra and testicular teratoma: A rare
association. J Indian Assoc Pediatr Surg 2011;16:108-10.
Source of Support: Nil, Conict of Interest: None declared.
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