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Urethral Duplication and Other

Urethral Anomalies
Guy A. Bogaert

DEVELOPMENT AND ANATOMY The normally developed urethra consists of the prostatic
urethra (region 1), the bulbomembranous urethra (region 2),
The female and male urethra have clearly different develop- the bulbospongious urethra (region 3), and the penile or pen-
mental paths and anatomy. Early in development, at about 3 dulous urethra (region 4) (Fig. 34-4). The bulbomembranous
to 6 weeks of gestation, the components of the musculature of urethra consists of the striated urethral sphincter and lies
the bladder neck, trigonal system, and ventral urethral wall in the urogenital diaphragm. The bulbospongious urethra
are identical in female and male fetuses. At the end of the 6th consists of the suspensory ligament, and the bulbourethral
week of gestation, the urogenital membrane retracts and the Cowper ducts typically end in this segment of the urethra. The
phase of sexual differentiation begins. The Leydig cells of the penile or pendulous urethra is characterized by the fact that it
fetal testes become hormonally active and induce a signifi- extends from the suspensory ligament to the external meatus.
cantly higher testosterone level between the 8th and the 20th It is typically surrounded by the corpus spongiosum, which
weeks of gestation. After this period, the testosterone level is functions only during sexual activity.
identical in female and male fetuses.1
Development and Anatomy of the Female Urethra
The female urethra derives from the endoderm of the uro-
Timing of the Urethral Anomalies
genital sinus. During fetal development, the posterior ends of 1. Urethral duplication: 10 to 14 weeks of gestation
the urethral folds fuse, and the remaining portion develops 2. Anterior urethral valvesurethral diverticulum: various
into the labia minora. The proximal smooth muscle part of the prenatal
female urethra inserts into the extension of the superficial tri- 3. Syringocele: various prenatal
gone and surrounds the ventral part of the urethra. The distal 4. Urethral prolapse: various postnatal
part inserts more dorsally in a way similar to that in the dorsal 5. Urethral stricture: various postnatal
part of the male membranous urethra. 6. Urethrorrhagia idiopathica posterior: puberty
The striated urethral muscle has two parts, one around the 7. Cowper duct and cysts: 10 to 14 weeks of gestation to
urethra itself and one around the urogenital sinus. The muscle postnatal
is continuous throughout the complete urethra. The caudal
part of the muscle that lies below the pelvic floor will become
attached laterally to the ischiopubic bone (Fig. 34-1).1
Duplication of the Urethra
The length of the urethra at birth is less than 1 cm, and in the Urethral duplication is a rare congenital anomaly that has been
adult it is 4 cm. The internal diameter during micturition aver- known for a very long time. The earliest descriptions go back
ages between 0.1 and 0.4 cm. However, the internal urethra can to Aristotle and Vesalius.5-7 Since then, duplication of the ure-
be normally stretched up to a Charrire number equal to the thra has been reported several times in case reports or small
age in years plus 10 (during urethral calibration).2 The urethral series.8 Urethral duplication is reported mainly in young chil-
mucosa is composed of pseudostratified columnar epithelium, dren, although it is sometimes discovered in adulthood.9
with more transitional epithelium near the bladder and strati-
fied squamous epithelium near the external meatus.3
The anatomic diversity and lack of a uniform embryologic the-
Development and Anatomy of the Male Urethra
ory have led to many classifications of urethral duplication. As
The formation of the male urethral plate and growth of the a consequence, there is a confusion in the literature concerning
genital tubercle coincide during fetal development. It is during complete versus incomplete duplication and accessory urethra
the 8th week of gestation that the genital tubercle grows over versus urethral duplication.
the urogenital membrane. As the phallus enlarges, its floor According to Ortolano and Nasrallah,6 a urethral duplication
is formed by the urogenital membrane. The urethral plate is must originate from the bladder, bladder neck, or prostatic
formed at the same time from a strip of sagittally staked endo- urethra. An accessory urethra originates distally off the prostat-
dermal cells that move into the mesodermal core of the genital ic urethra or is a blind-ending passage rising from a separate
tubercle.4 Later, the urethral plate extends further within the external opening.
mesenchyma of the phallus, and its partial degeneration forms Middleton and Melzer7 considered a complete urethral
the secondary urethral groove (Fig. 34-2).1 duplication to originate proximally from the external urethral
A different mechanism occurs in the development of the sphincter, whereas an incomplete urethral duplication originates
glandular segment of the urethra. A plug of ectoderm from the distally from that sphincter. They also recognized two channels
tip of the glans invades the mesenchyme, and, as it invades ending in one external orifice as an internal urethral duplica-
more deeply, it develops a lumen at the same time that the ure- tion. In Table 34-1, a more clinical classification elaborated
thral folds enclose more of the urethral plate. The plug of ecto- by Williams and Kenawi is outlined.10 This classification
derm correlates with the anatomic fossa navicularis (Fig. 34-3). is simple and was reported frequently in the literature.11,12