MALFORMATIONS OF THE GENITAL ORGANS.
Congenital malformations are found in all parts of the genital tract.
Some of the more common forms, like arrested development of the uterus,
have been referred to in the previous pages. Others will briefly be
considered here. Reference to the method of development of the sexual
organs will elucidate this subject.
The Fallopian tubes, the uterus, and the vagina are developed from two
embryonic structures called the ducts of Müller. These ducts become
fused, first at the lower extremity, between the sixth and eighth
weeks of fetal life (Fig. 175). The early genital tract thus formed is
consequently divided throughout by a septum, which normally disappears
during fetal development, so that there results one vaginal and uterine
tract, from which the Fallopian tubes branch.
[Illustration: FIG. 175.--Diagrams showing the development of the
vagina and the uterus from Müller’s ducts.]
The most important malformations of the vagina and the uterus arise
from arrest, at any stage, of this normal developmental process.
Very rarely the uterus is completely absent, or it may be represented
by a small band of muscular and connective tissue stretched across the
pelvis. In other cases the cervix is well formed, while the body of the
uterus is but poorly developed.
We have seen that this condition is often associated with pathological
anteflexion of the uterus.
=Uterus Unicornis.=--Sometimes there is arrest in the development
of one of Müller’s ducts, so that the uterus becomes one-sided or
one-horned and presents only one formed Fallopian tube. In such a case
both ovaries may be present.
=Uterus Didelphys.=--Müller’s ducts may unite only as far as the top of
the vagina, no fusion whatever taking place in the uterine portion. In
such a case two separated uterine bodies are produced; the condition of
double uterus exists (Fig. 176).
[Illustration: FIG. 176.--Uterus didelphys and double vagina.]
=Uterus Bicornis Duplex.=--In this variety of malformation development
has proceeded a step farther than in the preceding variety. The
uterine bodies have become externally united. There is, however, no
fusion of the cavities. Two cavities are present, opening into a double
vagina.
=Uterus Bicornis Unicollis.=--Here the development of the cervix and
the lower part of the uterus is normal. The upper parts of the body of
the uterus have not become fused, and diverge sharply from each other.
The organ is two-horned (Fig. 177).
[Illustration: FIG. 177.--Uterus bicornis unicollis (Winckel).]
=Uterus Cordiformis.=--In this variety the two halves of the uterus
are united throughout. Externally on the fundus there appears a slight
depression, which, with the broad body of the uterus, demonstrates the
imperfection of development. The name is derived from the resemblance
to the conventional heart-shape.
=Uterus Septus.=--In this variety development has progressed so far
that externally the uterus presents the normal appearance. The septum
that divides the two ducts has, however, failed to disappear, and a
divided uterus results. The septum may extend throughout the body of
the uterus, or it may be less perfectly formed. Often one side of the
uterus is better developed than the other (Fig. 178).
=Malformation of the Vagina.=--Malformation of the vagina is frequently
present with malformation of the uterus. The septum that divides
Müller’s ducts may persist throughout the whole length of the vagina,
forming a double vagina; or the septum may have partly disappeared,
being present in various stages of perfection. In double vagina each
orifice may be guarded by a distinct hymen.
Sometimes one of the canals of a double vagina is much better developed
than the other. The orifice of the poorly developed canal may be closed
at its lower extremity, so that the malformation is never recognized by
the woman or physician unless the closed canal becomes distended with
blood or other secretion. A variety of vaginal cyst may be formed in
this way.
[Illustration: FIG. 178.--Uterus septus (Cruveilhier).]
_Unilateral Vagina._--In this variety of malformation one of the ducts
of Müller fails to develop at all. The condition always occurs with
uterus unicornis. The vaginal canal is smaller than normal and may be
situated to one side of the median line.
_Absence of the vagina_ rarely occurs. There may be no sign whatever
of this structure, or it may be represented by a fibrous cord. The
external genitals may also be absent, or they may be well developed.
If the uterus and ovaries are well developed, much trouble may arise
from retention of menstrual blood.
An attempt should be made, by means of a transverse incision between
the rectum and the urethra, to reach the cervix, and, if possible, to
make an artificial vagina by transposition of skin from the buttocks.
Such treatment is usually unsatisfactory, as a patulous canal cannot be
maintained. It may be necessary to remove the uterus and appendages.
Sometimes the vagina is absent in only part of its course, being open
below and represented above by a fibrous cord; or the upper and lower
portions may be developed, while the middle portion is imperforate.
[Illustration: FIG. 179.--Transverse septum of the vagina (Heyder).]
These conditions are more amenable to operative treatment than in the
case of complete absence of the vagina. The intervening septum should
be incised, and the patulous condition maintained by the passage of
bougies if necessary.
Sometimes the lumen of the vagina is obstructed by the presence of
transverse bands or crescentic folds, which have been described as
supplementary hymens (Fig. 179).
A _hematocolpos_ is produced when the vagina becomes distended with
menstrual blood above such an obstruction.
=Hermaphroditism.=--A true hermaphrodite is an individual who possesses
the organs of both sexes in a condition of perfect function. The
existence of true hermaphroditism is denied by many authorities of
the present day, though the older writers firmly believed in it. The
coexistence of testicles and ovaries has never been proved beyond doubt
in the human subject. It is doubtful if there are any cases, recorded
as true hermaphrodites, in which the demonstration of the condition
is not open to serious criticism; such individuals are in reality
pseudo-hermaphrodites. The term hermaphrodite is still, however, very
commonly applied to any individual of doubtful sex.
A _pseudo-hermaphrodite_ is possessed of a distinct sex, and has either
ovaries or testicles, though the external genitals and other secondary
sexual characteristics may present the appearance of a double sex.
In _male pseudo-hermaphroditism_ the individual has testicles, and the
external genital organs simulate those of the female.
In _female pseudo-hermaphroditism_ the individual has ovaries, and the
external genital organs simulate those of the male.
In male pseudo-hermaphroditism the condition of hypospadias is usually
present, the lower surface of the urethra and the perineum being split.
The penis may be very small and imperforate, the urethra opening at its
base. The fissure of the perineum closely resembles the vagina, and the
split scrotum may be mistaken for the labia. Cases of this kind are on
record in which the individuals, ignorant of their true sex, have for
years indulged in sexual connection with men.
In female pseudo-hermaphroditism there is hypertrophy of the clitoris
and the prepuce, with approximation of the labia majora and contraction
or occlusion of the ostium vaginæ, giving the genitals the appearance
of the masculine type.
The secondary sexual characteristics of both varieties of
pseudo-hermaphrodites--the distribution of hair, mammary development,
shape, voice, etc.--are usually of the feminine type.
It is often exceedingly difficult to determine during life the true sex
of the individual in cases of hermaphroditism. The only absolute test
of the sex is the determination of the genital glands.
The labia should be carefully palpated to determine whether or not
testicles are present. Rectal examination should be made to determine
the existence of uterus or ovaries. The sexual inclinations of the
individual should be observed. The discharge from the genitals during
sexual excitement should be examined for spermatozoa.
The presence of a uterus is not necessarily indicative of a female,
as a uterus may be associated with a perfect penis and testes; and a
periodic discharge of blood from the genitals has been found in men.
If conception occurs, of course, all doubt is removed. If the sex
cannot be definitely determined by such examination, it is best to
consider the case one of male pseudo-hermaphroditism, which is the
usual form, and to treat the individual as a male.