HOMOLOGUES OF THE INTERNAL GENITALIA
Although sex is
determined at the time of fertilization, phenotypic gender is determined by a
complex tissue differentiation process that begins in the medial genital
thickening or ridges on the posterior surface of the embryonic body cavity.
During the 5th fetal week, primordial germ cells migrate from the yolk sac to
the posterior body wall and induce the formation of genital ridges on either
side of the midline. Here, these migrating cells induce the formation of
undifferentiated primitive sex cords.
Signaled
by the arrival of primordial germ cells, two sets of paired genital ducts, the
mesonephric or nephric (wolffian) ducts and the paramesonephric (müllerian)
ducts also develop. The mesonephros is a prominent excretory structure that
consists of a series of mesonephric tubules that connect with the elongating
mesonephric (wolffian) ducts as the latter extend caudally until they terminate
in the urogenital sinus on each side of the midline. The paramesonephric ducts
develop lateral to each of the mesonephric ducts and are derived from the
evagination of the coelomic epithelium. The cephalad ends open directly into
the peritoneal cavity, whereas the distal ends grow caudally, fuse in the lower
midline, form the uterovaginal primordium, and join the urogenital sinus as an
elevation, the müllerian tubercle, which separates the urogenital area from the
more posterior gut.
Under the
influence of the SRY gene in the male primitive sex cord, the mesonephric
(wolffian) ducts are maintained during development. As the developing male
Sertoli cells begin to differentiate in response to SRY, they secrete a
glycoprotein hormone, müllerianinhibiting substance (MIS) or anti-müllerian
hormone (AMH) that causes the paramesonephric (müllerian) ducts to regress
rapidly between the 8th and 10th fetal weeks. Müllerian duct remnants in the
male include the appendix testis and the prostatic utricle. In females, MIS is
not present, so müllerian ducts remain, and the mesonephric tubules and ducts
degenerate in the absence of androgens, often resulting in remnant epoöphoron
and paroöphoron cystic structures within the ovarian mesentery and Gartner duct
cysts within the anterolateral vaginal wall. These structures are clinically
important because they may develop into sizable and symptomatic cysts (see
Plates 8-13 and 9-13).
In the
male, under the influence of testosterone secreted by Leydig cells at 9 to 10 weeks, the
majority of the mesonephric ducts develop into the vas deferens and body
(corpus) and tail (cauda) of the epididymis. The mesonephric tubules nearest to
presumptive testis form the globus major or caput of the epididymis and the
efferent ductules that connect to the testis, forming ducts to transport sperm.
The more cranial mesonephric tubules develop into the vestigial appendix epididymis,
and the more caudal tubules may develop into remnants called paradidymis. The
seminal vesicles sprout from the distal ends of the mesonephric ducts, whereas
the prostate and bulbourethral glands develop from the urogenital sinus, thus revealing
different embryologic origins. In the fully developed male embryo, the distal
orifice of the mesonephric duct (ejaculatory duct) terminates in the
verumontanum on the floor of the prostatic urethra.
During
the 10th week of gestation in females, in the absence of MIS and androgens, the
primordial müllerian ducts remain separate and form the fallopian tubes
superiorly. At their caudal ends, the ducts join, fuse, and form a common
channel called the uterovaginal canal, which later develops into the uterus and proximal
four-fifths of the vagina. The remainder of the distal vagina forms from paired
thickenings on the posterior urogenital sinus called sinovaginal bulbs and the
vaginal plate, whose origin is not clear.
Intersex
disorders can result from failure of the müllerian or wolffian ducts to regress
completely. An example of this is hernia uteri inguinale or persistent
müllerian duct syndrome, in which MIS deficiency or receptor abnormalities cause
persistence of müllerian duct
structures in an otherwise phenotypically normal male. This is commonly
diagnosed during exploration for an infant hernia or undescended testicle
because the müllerian structures can tether the testis in the abdomen and
restrict normal scrotal descent. Vestigial remnants of the wolffian duct can
also exist in fully developed females. Vestiges of the male prostate may appear
as periurethral ducts in the female (see Plate 7-5). In addition, homologues of
male Cowper glands are the major vestibular glands (Bartholin glands) in the
female (see Plate 6-16).