with the knife. From these few historical facts it becomes evident that the physicians of antiquity knew the more common tumors of the vulva, and their operative treatment. CHAPTER I. DEFORMITIES OF THE VULVA. THE formation of the external sexual organs begins in the fourth week of embryonic life, with the appearance of a slight elevation, in which a depression is soon observed. This depression grows deeper toward the allantois, which is still connected with the intestine, later becoming a cloaca upon the disappearance of the wall separating the depression from the allantois, and into this the intestine and urachus open. Fourteen days later an eminence, the first indication of the sexual organs, appears above this opening, and on each side of it a large fold of skin. At the end of the next fourteen days a furrow may be recognized situated between the cloaca and genital eminence. Then the wall between the intestine and allantois grows from above downward into the cloaca, thus dividing the single opening into two, and forming the perineum. In the further development the genital eminence becomes the clitoris, the first folds of skin become the labia majora, the borders of the furrow forming the nymphæ or labia minora. Into the anterior division of the cloaca the ends of the ureters open, i. e., the bladder developed from the urachus, and the ducts of the seminal glands, whence it now receives the name sinus urogenitalis. The sinus has a length of 1.5 millimeters, or about of an inch, in the embryo at four months. The further downward this wall from which the perineum is formed grows, the more the ends of Müller's ducts are drawn down, the descent of the vagina occurs, and the uro-genital sinus thus becomes shorter; the urethra is formed, and hence as early as the fourth month of embryonic life the urethra and genital canal are completely differentiated. At this time epithelial cells cause the clitoris and prepuce, as well as the glans penis and prepuce in the male, to be adherent. The orifices of the glands of Bartholin may already be distinguished, opening into the vestibule one millimeter, or of an inch, above the lower end of the uro-genital sinus, and one millimeter below the origin of the vagina and hymen. The glands themselves, which are either oval or round, and one or more millimeters in diameter, show well-formed glandular acini, and mucus may be found both in them and in their ducts, from which it is evident that they begin to furnish their peculiar secretion even at this early date.* In place of this normal development of the external sexual organs, the following malformations may occur: I. If the genital fissure is not formed the skin remains entire, and the vulva is absent, defectus vulva or atresia totalis. In this case, neither the ends of the ureters, the seminal glands, nor the intestinal canal, have an external opening; the rectum, bladder, and genital canal may communicate internally, or the intestine and bladder may remain separate, the seminal glands opening into the latter. Malformations of this character occur invariably in monstrosities with deficient vitality, several of which have been represented by Foerster, some showing not the slightest trace of a vulva, as in the acephalus sympus; § others only a small pedunculated appendage, as occurs especially in the sirenian malformation. II. If, after the formation of the cloaca, the septum between the bladder and intestine, which forms the perineum and which draws the vagina down with it, does not grow downward, the cloaca becomes permanent; then the intestine, the end of the urachus, and the seminal glands empty into a common canal. This condition has been called atresia ani vaginalis, but incorrectly, because the intestine does not end in the vagina, the latter beginning at a considerable distance above its lower opening. III. If the uro-genital sinus remains very long and narrow, not being shortened by the descent of the united ducts of Müller, the urethra remains short and occupies an abnormally high posi * R. Geigel. Atlas, plates ix. and x. † Olshausen's Case, Archiv, ii., p. 280. Plate ix., fig. 6. tion. This condition is to be considered as the beginning of hypospadia feminæ, a true hypospadias occurring when the bladder opens without the vagina into the vestibule. A case of this kind has been described by Heppner. IV. If the formation of the urethra and the closure of the anterior parts of the vulva be prevented by a delayed communication of the allantois with the external surface, or if there be a defect in the anterior wall of the bladder with a cleft of the pubic symphysis, the condition is known as epispadias. Here the clitoris and nymphæ are cleft, and the anterior wall of the urethra is absent; cases of this kind have been described by Roeser, Gosselin, Testelin, and others. The causes of all such arrests in development lie in part in the relations of the respective organs themselves, e. g., an adhesion of ducts may interfere with the passage of the contents, and the consequent abnormal distension may prevent normal union, or bring about new separation. Again, they may be caused by neighboring organs, e. g., by the omphalo-mesaraic duct, by the intestines, or from abnormalities in the larger abdominal organs, such as the liver, which, owing to its great size or unusual connections, may permanently dislocate the organs beneath it. It is certainly incorrect to speak of a congenital vulvar atresia where there is an orifice beneath the hypertrophied clitoris leading to the uro-genital sinus, through which the urinary and genital secretions pass out, for here there is no complete closure and the vulva is not imperforate. The condition which Hildebrandt represents in his work, p. 5, fig. 3, does not, therefore, show an atresia of the vulva, but hyperplasia of the clitoris, persistence of the uro-genital sinus, and an abnormal shortening of the recto-vaginal septum. V. The condition known as hermaphrodism is likewise a peculiar malformation of the external genitals. Here some parts of the vulva, especially the clitoris and labia majora, are developed to an unusual degree. The labia, uniting at a higher level, present a sort of raphe and not infrequently contain the sexual glands, increasing the resemblance to the scrotum. On the other hand, other parts have been arrested in development, particularly the nymphæ, and hence the glans is partially exposed. But usually such cases are not examples of true hermaphrodism, but of individuals of only one sex. There are, however, cases of true hermaphrodism in which testicles and ovaries may be microscopically demonstrated in the same individual, but, as a rule, the parts peculiar to one sex are tolerably well developed, while the others are rudimentary. The following combinations are possible: a. Bilateral hermaphrodism, when a testicle is found on each side. The case described by Heppner* probably belongs in this category, still it cannot be accepted without reserve that the glandular body connected with the parovarium is always a testicle, because radiating tubes passing toward the hilus have been found in it; and, moreover, there was no trace of an epididymis. b. Unilateral hermaphrodism, when an ovary or a testicle is found on one side, and on the other, both ovary and testicle. No case of this kind has been observed. c. On the other hand, numerous cases of lateral hermaphrodism have been observed, i. e., a testicle on one side and an ovary on the other; this has been proved microscopically by careful observers. The best authenticated case is that of H. Meyer,† where, in an infant, a normal testicle was found on one side, the Ovary on the other, the uterus, tubes, vagina, and uro-genital sinus being present. The penis was long and imperforate, the left half of the scrotum contained the testicle, the vas deferens of the normal testicle alone being wanting. The condition of things was similar in the case of the wellknown hermaphrodite, Catharine Holman. Menstruation had incontestably been proved to occur from the rudimentary uterus, an ovary had been found, and spermatozoids repeatedly demonstrated in the ejaculated genital fluid. Here the ovary and the testicle must have been unusually well developed. I have had the opportunity to examine another hermaphrodite, a person who had been brought up as a girl, with a smooth face, large breasts, hairy mons veneris, short penis, and two large labia; later, in an operation for hernia, a testicle and epididymis were found. * Müller's Archiv, 1870, p. 679. Virchow's Archiv, xi., p. 420, and Klebs, i., 2 A., p. 750, Path. Anat. Treatment. That the physician may be forced to operate, even in cases of monstrosities with the above-mentioned deformities, is proven by that of Olshausen.* Here, owing to the complete absence of the vulva, the bladder, uterus, tubes, and intestines were so distended with urine that it was necessary after the head was born to open the abdomen and remove part of its contents before the body could be delivered. Should the uro-genital sinus persist, the intestine opening into its lower end, the latter may be freed and made to unite with an opening in the skin at the normal position; the narrow genital canal may then be dilated. An operation is decidedly indicated if there be a defect in the superior wall of the urethra, i. e., epispadias, for these patients generally suffer much inconvenience from dribbling of urine. By forming a superior or lateral flap, and liberating the posterior wall of the urethra, and then carefully uniting the two, a normal urethra may be made. Roeser has performed this operation successfully, and K. Schroeder reports two successes where the mons veneris and clitoris were cleft, and the anterior wall of the urethra was wanting. Kleinwächter's patient, fifteen years old, persistently refused to be operated upon, notwithstanding constant dribbling of urine.§ As a matter of course no operation is admissible in hermaphrodism. The diagnosis is of most importance in early life, on account of the bringing up; later, because of the ordinary duties of the individual, and not at all infrequently on account of intended marriage. It is, however, often impossible to determine the sex, even after the most thorough examination. The final conclusion should be based not only upon inspection, the sounding of all canals, palpation and examination per rectum, but all the secretions of the individual must be examined with the microscope and reagents, all of which may require months of close observation. Success may not attend our efforts even then, as has recently been proven by Sippel's case.|| * Archiv, ii., 280. † Würtemberger Correspondenzblatt, 1881, No. 20. ? Monatsschrift, 34, p. 81. Archiv für Gynecol., xiv. |