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.
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]).
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]).
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.
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.
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.