Hermaphrodism in the Daily Practice of
Medicine Being Information upon
Hermaphrodism Indispensable to the Practitioner.
By Dr. med. Franz von Neugebauer.
Director of the Gyencological Section of the Evangelical Hospital in Warsaw; Fellow of the Briticsh Gynecological Society, etc.
British Gynaecological Journal 19:226-263, 1903.
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In view of the grave consequences of an erroneous determination of sex, and the frequency, of such mistakes in ordinary practice, it seems desirable to glance at the cases of this anomaly in the human being recorded in contemporaneous literature, in order to ascertain the circumstances under which a medical man may have to deal with an hermaphrodite, the various questions that may need solution in various cases, and the precautions that should be taken to avoid the risk of serious mistakes.
In the course of the last few years I have been able to collect no less than 930 observations of hermaphrodism in human beings; 38 of these were cases which had come under my own observation, and the rest I found dispersed in ancient and modern literature. This collection contains a great number of surprising facts, in regard to diagnosis and therapeutics, and is full of information for all who practise medicine.
The genital organs of the human foetus consist of glands and canals; but while the foetal organisation of the canals is always bisexual, every embryo possessing the two ducts of Wolff as well as the two ducts of Mueller, the organisation of the glands is, in the earlier stage of evolution indifferent, later monosexual. Although theoretically we must admit the possibility that a foetus might have two testicles and two ovaries, or one testicle and two ovaries, or two testicles and one ovary, or one testicle and one ovary, that is to say, that it might really be a true hermaphrodite, and though there have been plenty of cases in which it has been alleged that testicular tissue has been found in one or two genital glands and in the same individual, ovarian tissue also in one or two other genital glands, up to the present time not a single one of these observations has stood the test of microscopic examination of the anatomical specimens; even in the one observed by Heppner, Slaviansky was quite unable to discover any Graafian follicles in the supposed ovaries. The supposed ovotestis of Blacker and Lawrence, a genital gland in which these authors believed they had found a mixed histological structure, testicular and ovarian, did not resist microscopical examination by Nagele. A true ovotestis has been proved microscopically in two instances only, reported respectively by Salen and Garre. [See also Simon's case (British Gynecological Journal, 1903, August, p. 106, and for Salens infra Summary).]
As yet, therefore, no one has ever found testicle and ovary in the same individual and, still less, has the essential postulate of the hermaphrodism ever been realised, namely, that the same individual should be capable of coition as a male and of becoming pregnant, save in the fables and myths of antiquity and the middle ages. One may therefore abandon once for all the ancient classification of hermaphrodism as lateral, unilateral and bilateral, since the condition is merely one of pseudohermaphrodism, an apparent hermaphrodism in which the genital canals, the external genital organs, and the secondary sexual characteristics do not completely correspond with the sexual character of the genital glands, but are developed after the contrary type, heterologous instead of homologous ; in which the masculine and feminine genital canals, the ducts of Wolff and those of Mueller, have developed simultaneously, while the external genital organs and the secondary sexual characteristics bear the appearance of one sex or the other, and appear to be either masculine or feminine.
But when there is simultaneous development of the ducts of Wolff and those of Mueller in an individual with testes, or one with ovaries, such development rarely attains the same degree in both; but generally a majus in the development of Wolff's ducts coincides with a minus in Mueller's, and vice versa, that is to say, an excess in one pair of ducts and a deficiency in the other.
The development of the genital glands is very often arrested in such a rudimentary stage, that one can find nothing in them but fibrous tissiie and blood-vessels, and even microscopic examination is quite insufficient to determine whether the specimen corresponds to a rudimentary testicle or to an ovary of arrested development. A testicle, the development of which has been arrested, to the naked eye often resembles an ovary.
Even a very inintite histological examination of a genital gland, either removed by the surgeon's knife or obtained from the cadaver, is often insufficient to determine the question of doubtful sex, it is therefore easy to understand that the practitioner, whether a surgeon, obstetrician, or gynecologist, should still more frequently abstain from giving a precise diagnosis in a case of doubtful sex, especially in the case of a new-born child.
According to Waldeyer, the primitive organisation of the genital organs of the human embryo is bi-sexual; according to Benda it is feminine, the masculine type being merely a modification of the feminine by progressive development.
Siegenbeck von Heukelom suggested a novel classification of hermaphrodism; he wished to distinguish glandular hermaphrodism, in which the individual was provided with genital glands of both the masculine and feminine type (a form of hermaphrodism that we have said has never been as yet found in human beings), from tubular hermaphrodism, in which there has been either a simultaneous development of the ducts of Wolff and of those of Mueller, or else merely the development of the ducts heterologous to the existing glands, that is to say, of Mueller's ducts, where testicles are present, or of the Wolffian ducts along with ovaries. According to this classification, the external genital organs in tubular hermaphrodism might be developed after either the masculine or feminine type, the latter representing merely an arrest in the development of the former.
But for practical reasons it is convenient to adhere to the classification Klebs has made of apparent or pseudohermaphrodism, the only form we shall have to consider.
PSEUDOHERMAPHRODISM - I. Feminine: Gynandroid, Gynander, Virago (Benda suggests pseudo-arrheny for male traits in a woman). II. Masculine: Androgynoid (Benda suggests pseudo-thely for feminine traits in a man). In each of these categories there are three varieties : (a) internal, (b) external and (c) complete.
I. Feminine Pseudohermaphrodism.-(a) Internal: External genital organs feminine; simultaneous development of the ducts of Wolff and those of Mueller, but to different degrees. (b) External: External genital organs apparently masculine ; adherent labia majora resembling a scrotum., hypertrophied clitoris resembling a penis with hypospadias, viz., perforated by the urethra, and like a normal penis; Ectopia of the ovaries in the labia simulating testicles in a scrotum; internal genital organs feminine. (c) Complete: External genital organs approaching the masculine type, internal genital organs feminine, more or less developed, with simultaneous development of the Wolffian ducts to a certain degree.
II. Masculine Hermaphrodism. -(a) Internal: External genital organs masculine, with masculine internal sexual organs; more or less development of Mueller's ducts (uterus, oviducts, vagina, broad and round ligaments) (b) External: External genital organs feminine in appearance, in consequence of penoscrotal hypospadias, with or without cryptorchism; internal genital organs masculine, more of less developed, with simultaneous development of mueller's ducts.
Penoscrotal hypospadias with cryptorchidism and the development of a more or less rudimentary vagina simulates the presence of a vulva; hypertrophy of the clitoris simulates hypospadias of a penis; labial ectopia of the ovaries, the testicles; adherence of the labia majora, the scrotum; non-adherant labia, a divided scrotum.
As already stated, an excessive evolution of the genital ducts of one type will be associated with deficient evolution in those of the other, if simultaneous evolution goes on at all.
Moreover, we have to consider: The general external appearance of the individual, sometimes homologous, sometimes heterologous to the genital glands; the evolution, homologous or heterologous, of the secondary sexual characteristics, the form of the bones and skeleton, the evolution of the muscular system, of the adipose subcutaneous pannicle which, when well developed, gives the characteristic roundness to the outlines of the female form, and when scanty allows, as in the man, the contour of the muscles to appear; the shape and size of the larynx; the abdominal or costal type of respiration; the pitch of the voice; the condition of the mammae; the general development of hair on the surface of the body, more especially upon the face (upper lip and cliin), the chest, abdomen, limbs, pubic and peri-anal region; moreover, the sexual feeling of the individual, sometimes homologous, corresponding to the sexual glands, and therefore directed towards the other sex; sometimes contrary, or heterologous, in favor of the sex of the individual, homosexual, must be taken into account.
All the variations in the different forms of hermaphrodism in the human being, when examined from the embryological point of view, are easily explained by arrest or excess of development in the two parts of the primitive bisexual organisation; but if we look for the ultimate cause of this abnormal development, heterosexual and opposed to the character of the sexual glands as it is, we have to face an enigma of nature still obscure and unexplained - As to the ultimate cause of the heterosexual or amphoteric evolution of the genital ducts, and that of the evolution of the external genital organs being incomplete (masculine) or excessive (feminine), we are still in the shades of vague hypothesis, the more so because every attempt to refer these malformations to mechanical causes has invariably proved futile. It would seem that the principal role in the etiology of these anomalies in development is that of the anatomical disposition of the arteries -- that is to say, depends on the supply of arterial blood to the parts affected. Morache gives the following scheme of the genital organs --- Deep Organs: nourished by the ovarian or spermatic branch from the aorta: Male -- Testicle; Female ---Ovary
Intermediate Organs: nourished by the internal branch of the hypogastric artery: Male --- Vesicule seminalis; Vemale --- Oviducts, Uterus, Superior vagina.
External Organs: nourished by the external branch of the hypogastric artery: Male --- Penis scrotum; Female --- Inferior vagina, Vulva, Labia
As each of these three series of deep, intermediate, and external organs is supplied by a special circulation, each may continue, or be arrested, in its development independently of either of the others. As a matter of fact, anomalies in the arteries have been found in anatomical specimens of pseudohermaphrodism; their default, for instance, coinciding with anomalous development in the corresponding series; but the question is a new one, and has not been much studied. On the other hand, the possibility of a psychic influence oii the part of the mother, and the factor of heredity, cannot be excluded in face of the instances in which several members of the same family, sometimes belonging to different generations, have suffered from the same malformation of the genital organs. It is, for instance, an incontestable fact that hypospadias, balanic, penile, or scrotal, is often found affecting father, grandfather and son, or several brothers; I know a number of instances of mistakes in sex in which two brothers-males with liypospadias-were brought tip as girls, and several families in which a thorough examination proved that three, and in one instance four, supposed sisters were males. I remember that an observation published by Lingard records that hypospadias occurred in males of six consecutive generations. The widow of one of the affected persons made a second marriage with a man without malformation of any kind, several sons and grandsons descending from this union had hypospadias. Lingard says that this remarkable observation supports the theory of indirect atavism, thus stated by Sedgwick:-- "All breeders of cattle and other animals are familiar with the fact of females throwing back, that is reproducing, after impregnation by a second male, the peculiarities of some other male by whom they had been previously impregnated. That is not impossible.in the human female."
The urogenital deformation is not merely a local deformation, but is essentially one of the phenomena of a general anomaly of evolution.
The sexual instinct in pseudohermaphrodites is most uncertain. Sometimes their sexual life is quite normal, sometimes it has little or no existence, while in other cases the inclinations are perverse and homosexual. There are even instances in which pseudohermaphrodites have carried onsexual commerce with men and also with women. Sexual inclinations at first normal towards the opposite sex often change, and afterwards become homosexual or vice versa. Krafft-Ebbing offers the following hypothetical explanation for this paradoxical inversion of desire; he looks upon the genital system as composed of three elements:-- (a) the genital glands, with their excretory ducts (the ducts of Wolff and Mueller) and the organs designed for sexual intercourse; (b) the spinal centres, which act in exciting, controlling, and directing the processes of nutrition, secretion, hypeaemisation, erection, ejaculation, &C., &C.; (c) the cerebral centres, from which the complicated psychosomatic processes originate, all that is, implied in the terms sexual life, genetic and sexual feeling, &c. These three elements, intimately united by the nervous. system, are in constant reciprocal functional correlation. Their organisation is bisexual, the psychosexual centre only developing at the age of puberty, and the evolution of the sexual glands proceeding quite independently of that centre. As a rule in the human being only one half of the bisexual organisation is developed, the other remaining in a latent condition of arrest. But under certain pathological conditions, especially when the development of the genital glands is merely rudimentary, the corresponding psychosexual centre does not develop at all, while the opposite one, which should lie dormant, and has done so till then, undergoes evolution instead of the one corresponding to the glands; that is to say, the feminine psychosexual centre develops in an individual provided with testicles, or vice versa.
In the evolution of the psychosexual centre of the genetic life of any individual, education, example and suggestion play, in my experience, a very important part. The male or female character of the genetic sense of pseudohermaphrodites depends very often on the sort of environ- ment in which they are brought up, that is to say, upon whether they are educated as boys or girls; it must be set down entirely to the influence of suggestion if a male hermaphrodite, owing to mistaken sex brought up as a girl, afterwards shows a feminine genetic sense, seeks to attract men and betrays perverse homosexual inclinations, and if when the mistake in sex is discovered he energetically opposes every attempt to make him abandon girls' petticoats, their way of life, and his feminine predilections and occupations, and if he declines to assume male attire and change his social position, and appear in future as a man. Such homosexual inclinations acquired by suggestion have in some cases been only temporary, and the male, though brought up by mistake as a female, has, sooner or later, recongnised his virility, and has not hesitated to demand his social and sexual rights sometimes somewhat abruptly. There, have been instances in which a male person, recognisiiig that his true sexual position had been misunderstood, has adopted male attire without consulting anyone, and without giving notice of the fact to the magistrate or any other authority; one such person found a mistress whom he put in the family way, and only demanded the adjustment of his social position on the evidence of that pregnancy --an incontestable proof of his manhood. In other cases the genetic sense with homosexual desire has persisted during the whole life of an hermaphrodite, whose true sex has been misunderstood; there have even been instances in which hermaphrodites of the male sex brought up as girls, have, then, too late, their true sex has been recognised, with all possible insistence demanded castration.
In very many instances the genetic sense of pseudohermaphrodites remains undecided, inactive, or altogether absent during all their life, or it may change its character once or oftener -- being homosexual at one time, heterosexual at another. But while some pseudo-hermaphrodites never show the least sign of sexual desire, but remain indifferent to men and women, many others have been extremely libidinous, and have even broken down from the consequences of frequent sexual debauch. Some pseudohermaphrodites have carried on sexual commerce with men and with women, and others have practised sodomy. Many a mistaken male brought up as a girl and married as a wife, has had his own mistresses out of doors; one widowed after thirty-five years' sexual intercourse with a husband, after the death of that husband engaged in most passionate love passages with her former maid servant. In twenty-eight instances public prostitutes have. been found to be of the male sex.
In regard to the retarded evolution of the genetic sense, I may refer to an observation of Berthold's, of Koenigsberg: A young woman, Marie S., aged 22, consulted him for hoarseness, and he found on examination with the laryngoscope that the vocal cords were as large as those of a man and, noticing that the patient was in the habit of shaving, concluded that the case was one of mistaken sex, the first instance in which such a diagnosis was due to the use of the laryngoscope. The young person was merely a male in whom hypospadias was complicated by an inguinal hernia; and when informed by Berthold of his discovery, blushed to her forehead and in outraged modesty would not believe the Professor's statement. She left the hospital at once and never returned, though Berthold had begged her to come next day and be presented to Professor Virchow. He made a fruitless appeal to her parents, who merely said that they could not force their child to visit him against her will. Berthold had forgotten the case when seven years afterwards he received a letter begging him to assist his former patient in rectifying his social position, in order that he might marry a young girl who was his mistress. And the patient, formerly timid and shamefaced as a girl, at the latter period betrayed all the aplomb and assurance of a man, and appeared naked and not ashamed before a company of medical men.
As in this case so in many other hermaphrodite males brought up as girls, the truth is brought out by their claiming their rights of their own accord, and frequently by their demanding rectification of their social position in order to marry young girls they have got with child.
The mental condition of pseudohermaphrodites may be absolutely normal, but is very often altered and pathological. There is reason to believe that in certain cases it direct relation exists between the abnormal psychic condition and the malformation of the genital organs. Of the serious influence that such a malformation may lian,e on the mental condition, the following case is a proof: A cryptorchid student having heard the celebrated Professor Cowper declare in a lecture that crvptorchids were not apt to cause conception, immediately left the lecture room and committed suicide.
The consciousness of being neither man nor woman, the constant and shameful fear that the malformation, though concealed with the utmost care, may some day betray itself and leave the sufferer to be the scorn and derision of those about him, are perpetually upsetting the mental balance and psychoticc repose of the unfortunate pseudohermaphrodite, who racks his brain demanding why he should be so afflicted, and seeking some way out of his miserable social position. Not daring to confide in anyone the poor hybrid passes his days and nights dwelling upon his lot; feeling excluded from the society of either men or women he sultivates solitude and avoids intimacy of any kind with anyone; he passes his nights in agony and tears; his health gives way, and he becomes suspicious, distrustful, shy, savage, irritable, irascible, vindictive, violent, and impulsive to an extent that may drive him to crime, or he becomes moody, apathetic, and melancholy, till at last he ends his days in self-destruction. I have found four instances of suicide in male pseudohermaplirodites brought up as girls owing to mistake in their sex, three of these unfortunates succeeded in self-murder; the fourth survived, but his brother was accidentally poisoned by strychnine taken at the same time.
I have collected twenty-two instances in which psychic anomalies have been associated with such malformations, and it is my firm conviction that without any hereditary psychopathic taint, an individual affected with malformation of the genital organs, especially when there has been it mistake as to sex, may, as soon as after puberty he becomes conscious of his abnormal organisation and his false social position, become melancholy and psychopathic. Certainly there are cases in which the mental anomaly is hereditary and independent of the condition of the genital organs. Very often the father or the mother has been a psychopath; in their hereditary anamnesis one comes across mania, general progressive paralysis, epilepsy, hysteria, alcoholism, &c. Not infrequently the pseudohermaphrodites end their days in the madhouse.
The importance of genital malformations in the development of mental affections has been pointed out by Christian, Legrand du Saulle, Magnan, and Debierre; Raffegeau counsels alienists adnld forensic experts to look upon and deal with all subjects of such anomalies as degenerates, and Debierre says: "Admitted to be degenerates, they may quite easily become irresponsible impulsives. The chain is not broken; the neuropath begets the hysterical of fugitive and bizarre tastes, habits, and propensities; or gives birth to the choreics or epileptics, whose progeny become insane." Many of the French alienists insist that every psychopathic person ought to be examined as to the condition of their genital organs.
Matzner recently reported the following case: A man was accused of having violated a woman, Mtrie G., aged 41, in a wood. Medical examination proved that Marie G. was a male with hypospadias, brought up as a girl owing to mistaken sex. The law had already some years pre- viously placed Marie G. under guardianship for imbecility and deprived the said G. of the right of dealing with any property. Owing to this certificate of imbecility, and to the accusation of rape being based merely on the word of an imbecile in the eye of the law, the court put a stop to the prosecution of the accused.
The statistics Of 930 cases of pseudohermaphrodism, with 33 personal observations which I have brought together, clearly show what disastrous consequences may follow an erroneous declaration of sex, and that not merely for the individuals immediately concerned, their family and connections, but for others beside. That collection includes 68 marriages between persons of the same sex, in consequence of such erroneous declarations! In 59 instances a wife or widow was discovered to be a male (with penoscrotal hypospadias, sometimes with cryptorchidism, and with or without the presence of a vagina). In five cases the necropsy upon a husband proved that iindividual to have been a female pseudohermaplirodite, brought up, owing to mistaken sex, as a male. The clitoris resembled a penis in shape, and was perforated by the urethra as in a male; the labia majora were adherent, and by their thin union simulated a scrottim; the vagina passing through a prostate, ended in a male urethra; the uterus and ovaries were found in the small pelvis.
The most extraordinary case was that of Charles Menniken, who had led a married life from the age of 27 to that of 57. The necropsy disclosed the feminine sex of the cadaver, and that the cause of death was uterine cancer. For thirty years this female pseudohermaphrodite had copulated with a woman.
In six instances in which there was no post-mortem evidence,) the sex of a married individual was suspected to be erroneous, but could not be decided by the judges.
The collection also contains many actions for divorce on the ground of mistaken sex. In a case recorded by Otto, it was the third husband of the person concerned who first recognised the mistake in the sex of his spouse, and demanded the relief of divorce. In 1885, a case published by Badaloni, the divorce of Maura Faustina, attracted much attention for two reasons; the marriage was dissolved not merely because Maura Faustina was a male of mistaken sex, with hypospadias, and marital relations were impossible, but because the husband complained that his supposed wife lay with other women, and made him a subject of ridicule to his acquaintances. After the divorce Maura Faustina in his right as of the masculine sex, demanded that his brother should give up to him half the property of their deceased father; the brother refused, and brought forward the counter-charge that his ci-deviant sister had seduced his wife.
A necropsy on the widow Christine Bockfleisch, who died at the age of 82 of cancer of the bladder, revealed an error of sex the body was that of a male with hypospadias. A midwife of the name of Maerker, formerly married, was charged with violating a young pregnant woman under the pretence that it was necessary to correct the position of a misplaced foetus. After the prosecution had begun numerous charges of the same kind were brought against the midwife. Examination disclosed an error of sex; Maerker was a male with hypospadias, and the charge of unnatural crime was withdrawn; but the midwife lost the right of practising that vocation.
In eighteen instances betrothals have been annulled at the last moment before marriage, in view of the fact that the intended bride was a male pseudohermaphrodite; in Worbe's case she had been betrothed twice previously.
Pseudohermaplirodites have in numerous instances been in difficulties with the various authorities-ecclesiastical and judicial tribunals, magistrates, police or gendarmerie, and even with the directors of schools. Before the ecclesiastical courts they have figured in numerous cases for divorce; before the civil law the most common accusations against them have been for rape, seduction followed by pregnancy, unnatural crimes, and even sodomy. There have been also cases of murder committed by pseudohermaphrodites, and in many instances they have been the victims of crimes committed by others.
In one case the directress, of a boarding school for young girls, having observed sexual relations between two of her pupils whom she had surprised in the apparently unnatural act of passionate intercourse, immediately summoned her medical man, who ascertained that the sex of one of the two pupils had been mistaken, and the youth up to that time brought up as a girl had forthwith to leave the institution in order to prevent a public scandal. In 1894 a governess named Wilhemina Moeller was condemned to death at Copenhagen; she had abused a little boy, one of her pupils, by sexual acts, and to avoid denunciation had afterwards poisoned him with chloral. Expert examination proved that Wilhemina Moeller was a male with hypospadias, brought up owing to mistaken sex as a girl. Marie Chupin also, who was accused of having thrown a child into a well to drown it, was also a male hypospadiac. In one of my own personnel observations a young girl of 18 administered strychnine to three persons at the same time. She and her mother were saved but a brother, aged 9, died from the poison. The girl declared that she had only intended suicide, and that the poison had accidentally fallen into the soup tureen before their family dinner. I found that the accused had normal sperma and declared him to be a male cryptorchid with hypospadias, the victim of an error of sex. As the judges decided that suicide only and not murder had been in- tended, the accused, after rectification of his social position, was merely condemned to an ecclesiastical penance, and placed under parental care.
But the pseudohermaphrodite has sometimes come into conflict with the authorities or police and been arrested, although no crime has been committed. On December 2, 1891, a gendarme arrested a young girl of 19 on the platform of the railway station at Pilsen, on the suspicion of being a man disguised as a woman. It was in vain that the prisoner showed her personal papers, in which she was described as Marie Karfiol, born on such a day, at such place, and of such parents. In spite of her protestations, she was taken to the mayor's court, where medical evidence proved that there had been an error of sex, and that Marie K. was a male hypospadiac. She then admitted that at the time of her birth there had been some difficulty in determining her sex, but she had been brought up as a girl. At the time of her puberty suspicions as to the real state of the case had led to her being taken to see the mayor of her village and the priest; but no further action had been taken. Later on she abandoned herself to her fate, being ashamed to speak to anyone of her doubts. Her pretty hair was cut off and she was dressed in men's clothes; but in her novel attire she had a very timid and wild appearance.
Of the crimes of which pseudohermaphrodites have been victims I will quote only two. Some years ago in Japan, a man fled from justice after assassinating his wife. Examination of the cadaver proved that the supposed wife had been a male hypospadiac, from error of sex brought up as a girl.
In a case published by Bellin, a young girl was the victim of rape by two men. She lost consciousness from a fisticuff on the head, and when she recovered was suffering from violent pain in the anus, from which blood was flowing. She laid complaints against the two men, and on medical examination it was found that she had no vagina, and that the anus had been torn in three places by anal coition. The young person thus violated was a male hypospadiac, and it was entirely owing to the crime of which. he had been the victim that his true sex was recognised and his social position rectified. Those two men in violating one they took for a woman had unawares committed an unnatural offence.
We have said that there are many cases in which even a very detailed microscopical examination of the genital glands of an adult, whether obtained by castration during life or from the cadaver, is altogether insufficient to determine the true sex of the individual. This is the case in those not uncommon instances in which the development of the gland is so rudimentary and atrophic that it is merely a sclerous ganglion composed of fibrous tissue and a few vessels, without any histological element whatever characteristic of either testicle or ovary. It is easy, therefore to understand the difficulty that may arise in determining the sex in doubtful cases in the adult, and still more so in the newborns If in an adult we cannot ascertain that either ejaculation of semen or menstruation has taken place, the examination of the genital glands alone can solve the question of sex. In the male hypospadiac if we find well-developed testicles, epididymes and spermatic cords in the scrotum, the solution is an easy one; but it is a very different matter where there is cryptorchism with or without arrested development. Similarly it may be extremely difficult to distinguish a female pseudohermaphrodite or gynander, from a male cryptorchid with hypospadias, or even from one without hypospadias, or even from merely balanic balanic hypospadias, when an hypertrophied clitoris traversed by the urethra simulates an erectile penis, as in the celebrated Charles Menniken, a woman married as a man, in Crocchio's case of Josephine (or Joseph) Marzo, or in a case I operated upon in October, 1903.
In the majority of cases the true sex, even when indeterminate at birth, declares itself spontaneously at puberty. For instance, the midwife in a case of doubtful sex may, after some hesitation, have advised that the child should be brought up as a girl, because the external genital organs, save for some hypertrophy of the clitoris, offered more resemblance to the female vulva than to the male organs. She saw a clitoris and vulva where there was merely penoscrotal hypospadias and bilateral cryptorchism, and the mistake was the easier to make because below the urethral orifice there was to be seen the opening of a vagina furnished with a hymen. At about the age of 12 years, let us say, the child begins to complain of pain about the groin; a diagnosis of inguinal hernia is made, and a truss applied, which, however, causes such acute pain that the child refuses to wear it. The following year a diagnosis is made of a similar hernia on the other side, and a double truss is prescribed. When about 16 the young person notices erections of the clitoris, and complains to her mother of emissions of viscous fluid. The voice changes, and the mother wonders that the menses do not appear, while at school fellow-pupils remark the masculine down on the upper lip, and make mock of the moustaches, the masculine voice, and the flat bosom and the unfeminine figure. All in contact with the youth are struck by the appearance presented, which is rather that of a boy disguised in petticoats than that of a real girl. The parents for many years have had doubts as to the sex of their child, and now in view of her unusual appearance and her development of masculine tastes, at last recognise the error of sex. Or under other circumstances, unbalanced by the effect of voluptuous dreams, the youth may give way to evil practices, and may finally attempt sexual intercourse as a woman; and so, the sexual instinct gradually awaked, may cause the disclosure of the error of sex. Or again, all doubt may be dissipated by a female friend already versed in sexual life, or by a doctor consulted by the girl herself, or by the mother, in order to know why at the age of 18 the catamenia have not appeared, and whether the girl is fit to marry and bear children, &c. Often enough the doctor will find that such inguinal hernias are merely the delayed descent of the testicles into the scrotum, and by the aid of the microscope will be able to decide whether the fluid ejaculated is semen with or without spermatozoa, or otherwise.
But though in the majority of cases the true sex can be determined at puberty, in a certain number the task before the medical man is a much more difficult one. I have myself now before me a pseudohermaphrodite nearly 20 years of age, who has been under my observation for eleven years, and whom I have examined many times since the age of 8; though I suspected that there had been an error of sex, I was unable to decide the question till in September, 1903, one testicle descended and ejaculation took place. In regard to a new-born child of doubtful sex it is better to reserve one's decision than to risk mistake, which may be followed by disastrous consequence. As to the registration of the sex and the civil rights o the new-born, the Government should admit as a new rubric, "sex undetermined"; the child should have to be presented for renewed examination at the age of 1 year, at that of puberty, and at certain intervals of time subsequently, until a decision could be arrived at.
But how should such a child be brought up? As the great majority of pseudohermaphrodites are masculine, Ahlfeld recommended that every child of doubtful sex should be educated as a boy; on the other hand, Lawson Tait advised that it should be treated as a girl on the ground that it is far easier to protect a child from the disagreeable results of a genital malformation in the social position of a girl than in that of a boy. This advice of Lawson Tait's deserves consideration, but with some reserve. It is well to remember that a male hypospadiac brought up owing to error of sex as a girl, and restored to his proper social position at puberty, will suffer far less from necessary changes in his mode of life than a female pseudohermaphrodite brought up as a boy would under corresponding conditions. There is, however, a danger that must not be lost sight of. Suppose that owing to an error of sex, an infant of doubtful character is brought up as a girl, according to Lawson Tait's advice, and attains the age of puberty. The supposed young girl of 16 then has her sexual instincts aroused by reading romances; sharing the dormitory of her fellow pupils, she watches every night and morning these maidens at their toilet, and without any restriction has opportunities of learning to admire the bodily charms of persons of the opposite sex. Would not this be enough to awake in him, however innocent hitherto in regard to sexual matters, desires that might easily lead him to abuse the situation, in a way which might be followed by consequences even more serious perhaps for the companions sacrificed to his masculine desire than for himself ? An example of the kind, which occurred at Paris, has come to my knowledge. A male pseudohermaphrodite, brought up from error in sex as a girl, was employed as forewoman in a manufactory in which only women were employed; but was not long in that position before he had behaved like a wolf in a sheepfold.
Such an individual, then, as we are considering ought to be watched with careful anxiety, not only as to his mental condition, but also with regard to his behaviour, in order to prevent any misfortune, any unexpected injury to his associates, and therefore all medical men, midwives, the clergy, masters and mistresses of schools, ought to be informed upon the practical bearings of pseudohermaphrodism and error of sex, in order that they should take warning in time, and consult a doctor of experience in these matters, directly their suspicions as to an error in sex were aroused.
The disastrous consequences of an erroneous declaration of sex upon the individuals who are victims of such mistakes, in regard to their mental condition, could not be more authoritatively exposed than they have been in the autobiography (Souvenirs et impressions) of the unfortunate Alexina B., for the publication of which we are indebted to Professor Tardieu. After being brought up as a girl, at the age of 22 she became a governess, but was discovered to be a man, and restored to her proper sex; her unhappy life, which had she been brought up as a boy might have turned out so differently, ended in suicide.
I will not here enter upon the question of embryological development, and the way in which hermaphrodism, male or female, comes about. The whole subject is completely treated in a work of mine, published in Polish, with sixty-eight illustrations, in the Proceedings of th Medical Society of Warsaw for 1899.
The number of cases in which the surgeon is confronts by pseudohermaphrodism is much larger than is generally supposed. The most common are those in which he is asked by the parents to determine the doubtful sex of a child with malformed genital organs. As the feminine appearance of the external genitalia is chiefly due to penoscrotal hypospadias, his task, in order to recognise a masculine individual, will be to discover the genital glands, the testicles with their adnexa, if they lie in the divided scrotum or in the inguinal canals. If he succeeds in finding the testicles with their adnexa, on one or both sides, the individual is certainly masculine, even though the urethral meatus opens inside the orifice of a vagina and labia minora, which are merely the ununited borders of the corpus cavernosum of the male urethra, are present. In the newborn we cannot explore the rectum for the prostate or uterus, and these organs, even if found, would not at all decide the sex of the infant, seeing that the male pseudohermaphrodite may have a uterus and the female a prostate.
Moreover, in a new-born male, it is not easy to elicit the reflex of the cremaster muscles; one may fail to do so even by rolling the spermatic cord under the finger upon the os pubis.
Klebs insisted that the presence of the labia minora was a sufficient proof of the feminine sex, but this is not so, for there have been numerous male pseudohermaphrodites whose external genitals were exactly like a normal vulva; I may instance among many others an observation by Pozzi, in which the appearance of the vulva would not have excited the slightest suspicion of an error of sex, while surgical intervention by castration demonstrated that the patient was a male. Pozzi on performing herniotomy on a maidservant, aged 33, found, in a hernia, a bicorned uterus, or rather one horn of a bicorned uterus and a testicle. The vulva of that patient was absolutely normal in appearance, like that of a well-developed girl, and there was not the least trace of hypertrophy of the clitoris to awaken a suspicion of an error of sex.
The determination of the sex of a male new-born pseudohermaphrodite becomes even more difficult if cryptorchism is associated with penoscrotal hypospadias, and in that case it is better to defer one's decision even until puberty, than to risk any mistake.
I have an anatomical specimen taken from an infant, in which at 19 days old the sex was absolutely doubtful. Three of us, a surgeon, a paediatric specialist, and a gynaecologist, had concurred in declaring the sex male. The child dying proved to be a girl, with enormous hypertrophy of the clitoris. There was a marked prominence of the clitoris and labia majora in front of the bony anterior wall of the pelvis, a more remarkable deformity than I have ever seen in male hypospadiacs.
Pseudohermaphrodism is often associated with other malformations, such as pelvis fissa, vesical exstrophia, epispadias, uterus bicornis, spina bifida, hydrorrhachis, polydactylism, sympodia, digital synechia, pes varus, palatocheiloschisis, atresia ani vel urethrae, and the like. In a great many instances it has been complicated by congenital inguinal hernia, hydrocele, or malformation of the kidneys. Many of these foetus with multiple deformities, are not born alive, but sometimes one is, and may be saved by surgical intervention; cases have, for instance, been successfully operated upon for atresia ani.
It is noteworthy that in several instances there has been much difficulty in the recognition of the sex where there has been vesical exstrophia and epispadias. The reason may perhaps be because the mother has found it much easier to keep the child comparatively clean in chemise and petticoats than if it had been put into trousers. One such hermaphrodite, owing to error of sex brought up as a girl, was placed in a lunatic asylum, and after rectification of his civil position, was put into men's clothes; but the very next day he was found to have divided the perineal seam of his trousers with a pair of scissors, because they prevented him from making water comfortably like a woman, as he had been accustomed to do all the time he had been in woman's clothes.
The chief and most frequent reason for a pseudohermaphrodite being brought before a surgeon is the wish of the parents that the doubt as to sex should be settled. The next, is to ask him to cure a hernia in a young girl who proves to be merely a male hypospadiac with delayed descent of one or both testicles. Nothing could be more absurd than to order a truss in a case of the kind, and it is not surprising that if one is ordered the child. cannot be induced to wear the instrument, the pressure of which upon the testicle gives him pain; another example of the disagreeable and even dangerous consequences of a mistake in sex to the person concerned.
Nevertheless this mistake is being still made. Assuredly it is by no means always easy, especially in an infant, to decide whether the case is one of the delayed descent of a testicle or of hernial ectopia of an ovary, and while it is true that inguinal intestinal hernia is very common among pseudohermaphrodites, it must not be forgotten that the contents of a hernia may include omentum, or a diverticulum of the bladder together with a uterus, possibly bicorned, an oviduct, or a tumour of the tube, ovary, or parovarium. Moreover, it must be remembered that in a man with quite normal external genitalia, or in a male hypospadiac, a uterus more or less developed, with its tubes and even with part of the vagina, may be found in a hernia along with one or two testicles.
Saenger, in a herniotomy on an unmarried governess, aged 32, who had never menstruated, found that the hernia contained the uterus, one tube, a parovarian cyst and a genital gland, which he took to be an ectopic ovary. As the vagina ended at a length of 8 cm. in a cul-de-sac, and no solid body could be found by the rectum, Saenger, before the operation, thought that he had to do with a male pseudohermaphrodite, with inguino-labial (-scrotal) hydrocele on the left side. During the operation he abandoned this provisional diagnosis, as he believed the genital gland found in the hernia to be an ovary. What was his astonishment when the microscope proved that the gland, extirpated with the uterus during the herniotomy, was a well-formed testicle. In that operation there were three surprises each more startling than the former. In another patient, a maid-servant, aged 23, Saenger diagnosed labial ectopia of an ovary and performed herniotomy in order to return it into the peritoneal cavity, but, as the processus vaginalis was obliterated he removed the gland. The microscope showed that he had extirpated a testicle.
I have collected forty-two herniotomies in which errors of sex were discovered, and such facts ought to be known to every surgeon: in three, operations were performed on married women for hernia of the ovaries, and the glands removed were testicles. In thirty young girls or married women, in performing herniotomy the existence of one or two testicles has been ascertained, and once the operation demonstrated the femininity of a pseudohermaphrodite brought up as a boy. In four instances the operation was undertaken simply to determine the character of the genital glands in the labia majora of young girls, and, testicles being found, the wound was closed without removing the glands.
Porro was the first to perform an exploratory incision of the labia majora simply to determine the sex, till then doubtful, of a young woman of 23.
On some of these unfortunates hemiotomy has been performed more than once, on one side after the other, or for the recurrence of the hernia. In one instance a swollen gland was removed from the groin of a little girl, aged 11, and seven years afterwards the extirpated gland, misunderstood even after its removal, was found on microscopic examination to be a testicle. In two cases recorded by Pech and Swiencicki, errors of sex were disclosed by the evacuation of inguinolabial hydrocele in women, for after the evacuation a testicle with its adnexa was found in the sac. In other operations a uterus with a tube and a testicle have been found in one hernia in persons supposed to be women, and these individuals have proved to be male pseudohermaphrodites.
On the other hand a uterus has been found, in other cases, in the hernial sac of a man, externally quite normal. Derveau performed herniotomy on a man, aged 67, the father of six children; the hernia contained a uterus with two tubes, the testicles occupied the place of the ovaries and a vagina terminated in the urethra. Similar cases have been met with by Billroth, Boeckel, Cartle, Faustino, Guldenarni, Thiersch, Stonham, Rydygier and Winckler, who came upon internal masculine pseudohermaphrodism in herniotomies performed upon male adults in whom there had been no error of sex. Bruehl, however, found a uterus and two ovaries in the hernia of a woman, aged 36, of masculine appearance with a beard, and having a clitoris 11 cm. long when erect.
Merkel quotes sixteen observations of the development of a uterus in a man, similar to that of Derveau, but mostly derived from necropsies. My personal researches would lead me to suppose internal masculine pseudohermaphrodism to be much more frequent.
In regard to the diagnosis of the contents of the labia majora in cases of doubtful sex, I have already mentioned the very variable contents of inguinolabial hernias. I have said that an ectopic ovary has often been taken for a testicle, or a testicle delayed in its descent for an ovary. I have spoken of male and female hydrocele; but I must also draw attention to the derivatives from the processus vaginalis peritonei, the diverticula of Nuck.
In the case of Claire Hacker, published by Litten, some of the consultants took the bodies contained in the labia majora of that young woman to be testicles, and suspected an error of sex; Virchow, however, dissented, as he was unable to detect the epididymes by palpation, and con- sidered them to be rather ovaries in labial ectopia and that Claire Hacker was really a female.
The necropsy proved that Virchow was right in regard to sex, that Claire Hacker was not a male hypospadiac, but a feminine pseudohermaphrodite. The cause of her death was a myxosarcoma of the right ovary, the left being normal and both being in the abdominal cavity. The bodies in the labia majora which Virchow had been the more inclined to take for the ovaries because they swelled during the catamenia, proved to be an haematocele, and a hydrocele of the processus vaginalis peritonei.
In addition to these herniotomies with unexpected results, I have collected thirty-one laparotomies performed on pseudohermaphrodites. On four occasions the abdomen was opened in order to find and extirpate the genital glands, as microscopical examination alone could determine the question of sex. Krabbel and Pozzi each extirpated in men large abdominal tumours, which proved to be ovarian; each in fact performed an ovariotomy on a patient who, owing to error of sex, had passed for a man. In another case an abdominal tumour in a woman diagnosed as a haematometra, was attacked by the vagina; in the paracentesis a vessel was wounded and the woman died. The necropsy disclosed an error of sex; the tumour was a sarcoma of the testicle in a male hypospadiac with bilateral cryptorchism, brought up by mistake as a girl. In twenty-eight coeliotomies there were no less than fifteen in which there had been an error of sex. In the case of a young woman in whom, after appearing, the catamenia ceased, Fehling diagnosed a haematometra; after paracentesis with a negative result, he came to the conclusion that the tumour must be ovarian. The young woman had a clitoris 5 cm. long; in the right labium majus there was a body which was taken to be the misplaced right ovary. Coeliotomy confirmed the amended diagnosis; a myxomatous cyst of the left ovary was removed and a hernia of the right ovary discovered.
A most singular case is recorded by Paton, an operation for Pyosalpinx on an internal masculine pseudohermaphrodite. Paton performed a coeliotomy on a man, aged 21, in whom a large abscess at one side of the hypogastrium had been opened through the abdominal wall one year previously; a pyosalpinx had in fact been incised without being recognized as such. Paton found that there was a uterus behind the bladder with two tubes; on one side at the seat of the cicatrix of the old incision the tube was adherent to the abdominal wall; the other tube, a pyosalpinx, was removed with the genital gland, a testicle, which lay in the normal position of the ovary. That man was a male pseudohermaphrodite with penoscrotal hypospadias. The opening which had been taken for that of the urethra led into a vagina in which the meatus urethrae proper was situated. The man had demanded relief for dysuria with pyuria; the catheter never entered the bladder but only the vagina, through which the pus contained in the tubes had found its way. A tumour noticed before the operation, but which had disappeared when the abdomen was opened, had been merely the bladder distended with urine. This case is quite exceptional, but one with which one ought to be acquainted before resorting to operation.
I will now refer to the cases in which the mother asks the surgeon whether her daughter may marry and have children, and why, although she is nearly twenty years old, her catamenia have not appeared. This last question, more frequently than any other, has led to the discovery of an error of sex, and the practitioner, whenever he is confronted with a case of absolute amenorrhcea, should always remember the possibility of such a mistake. Under such circumstances to omit an examination of the genital organs out of consideration for the timid modesty of a young girl, may be a matter of great regret.
It is dangerous to content oneself with the idea that such a state is not altogether unusual; that it is merely a case of hypoplasia of the genitalia; only a case of chlorosis, and that, under treatment with baths and iron, the menses will not fail to appear. A medical work has recently been published under the title "Cavete Hymeni," but it is quite possible to conduct an examination without lacerating the hymen. If one would avoid the risk of a serious mistake it is essential to demand an examination.
Steglehner has published an instance in which the mother demanded an autopsy on the body of 'her daughter to ascertain why the girl had never menstruated, and the section proved that there had been an error of sex; probably the mother had herself suspected the mistake. Gallay gives a similar case, in which a husband consented, at the request of the doctor, to a post-mortem examination of the body of his deceased wife. Just as the medical man is consulted by young unmarried pseudohermaphrodites on account of their malformation, by married persons of the same category his advice is sought on account of sterility or dyspareunia. In feminine pseudohermaphrodism there is an hypertrophy of the clitoris more or less considerable, so that when the member is traversed by a urethra it may closely resemble a normal penis; there is also a coalescence of the labia majora simulating an empty scrotum, and as there is neither vulva nor vagina cohabitation as a woman is impossible. In other cases less uncommon, the clitoris is hypertrophic and erectile, and the labia are abnormally united to each other, save at the spot where there is the urethral orifice; it was in such a case that in a married woman Huguier sepa- rated the labia by the sweep of a bistoury, and revealed the orifice of the vagina, and she was thus enabled to fulfil the duties of wedlock, and afterwards became pregnant. Similar operations have been performed by Sonnenburg and by von Mars, and I propose to do one myself this week upon a girl aged 25, after a preliminary coeliotomy and amputation of a hypertrophied clitoris. The celebrated Marie Madeleine Lefort was a subject of the same sort of external feminine pseudohermaphrodism. It was Beclard who recognised her true condition, when most of the medical men who had examined her supposed her to be a cryptorchid with hypospadias; Beclard succeeded in passing a sound into the vagina through an orifice below the hypertrophied clitoris, and in determining its presence there and the existence of a uterus by rectal examination. The simple procedure adopted by Huguier would have made Lefort capable of wife and motherhood. Beclard's diagnosis of femininity was made in 18I5, when Lefort was 16 years old, and he then suggested the discission of the labia majora. The autopsy thirty-nine years later proved that he was right. Marie Madeleine Lefort had menstruated.
In masculine pseudohermaphrodism one finds penoscrotal liypospadias with or without cryptorchism on one or both sides. The first thing is to find the testicles, and if that cannot be done to examine the fluid ejaculated one should also look for the cremaster reflex. By the vagina, if there be one, otherwise by the rectum, one may explore for the prostate, uterus, seminal and genital glands, although the diagnosis depends exclusively on the determination of the testicles and their product the semen, or of the ovaries or menstruation.
Maude records a case of error of sex that is almost unique. In a girl, aged 13«, he recognised the masculine sex; there was a normal penis, but below its base the scrotum was divided into two parts, each containing a testicle. There was a navicular fossa and an opening into a vagina admitted the finger; no uterus or hymen. Here the hypospadias was limited to the scrotum, the penis not being involved, one of the rarest varieties of this anomaly. That individual after puberty would have been as well capable of intromission and impregnation with his normal penis as of submitting to the embraces of a man.
When with penoscrotal hypospadias there is also a vagina, and that vagina is considerably dilated by frequent cohabitation, as in the case of certain prostitutes, who have been masculine pseudohermaphrodites, an error of sex may easily be made. Polaillon, who examined an individual of this kind repeatedly, found the vagina longer and wider on every occasion until, after some years, he was able to introduce a large cylindrical speculum. After the death of this person the autopsy proved to the general astonishment that he was a male hypospadiac, that. there was not any vagina at all, and that the deep canal, which had admitted a large speculum up to the end, was merely an artificial cutaneous depression, the result of many years' sexual intercourse with men. Ricord, in the case of a gay Parisienne, who had demanded a permissive card from the police, certified an error of sex and that the individual was a male, and the card was refused.
An error of sex in a male hypospadiac brought up as a girl, reveals itself comparatively frequently by seminal ejaculations; but one is very seldom able to find the openings of the vasa deferentia alongside the lumen of the vagina. In the thirty-eight personal observations of my own I have only once succeeded in finding and demonstrating those openings during life. There have been cases in which they were visible to the naked eye, but it would be necessary to witness the ejaculation to find both orifices.
In a-certain number of cases the question of sex, even in an adult, cannot be determined; but when the mistake is evident, ought one in every instance -- if, for example, it concerns a wife living in complete accord with her husband -- to reveal one's discovery and enlighten the wedded pair on the true state of affairs? The question is a new one; up to the present time the doctor has hesitated to interrupt the happiness of such couples, and has held his tongue; but in case of a demand for divorce on account of dyspareunia or sterility, he undoubtedly ought to speak. Hitherto the law has taken no account of these cases; but even though the wedded pair are entirely ignorant of the error of sex affecting one of them, and do not therefore demand divorce on account of identity of sex, the marriage is still null according to all laws, civil and ecclesiastical. If in ignorance of the error of sex, such a pair do not demand divorce, can the law insist upon it? That question has not been decided. In actual life such unions are often undisturbed, though there have been cases in which divorce has been demanded and obtained.
In differential diagnosis some stress has been laid on erection. Charles Menniken was married to a woman for a long time; he had an erectile penis traversed by a urethra, and carried on sexual intercourse. Nevertheless that married man, as was proved by necropsy, was really a woman with uterus and ovaries, and even a vagina ending in a male urethra. In the same way an individual described by Crecchio, although possessed of a penis traversed by a urethra, was a woman. In several known instances the hypertrophied erectile clitoris of a feminine pseudo- hermaphrodite has enabled her to copulate as a man, by introducing that organ into the vagina of a woman. It has, however, been necessary for the woman to take an unusual position. As a matter of fact erection has no diagnostic value at all.
Menstruation is, on the other hand, characteristic. There have, however, been exceptional cases in which a sort of pseudomenstruation, periodic genital hemorrhages, have been observed in masculine hermaphrodites, as for example in the celebrated Catherine Hohmann, who subsequently under the name of Charles, after his true sex (semen) had been disclosed, married a woman in New York.
It is not, though generally supposed to be, the case that masculine hermaphrodites with hypospadias are impotent and sterile on that account. There may be more difficulty in the intromission of the organ on account of its malformation, and therefore more difficulty in impregnation, but many hypospadiacs have had several children, as is well proved by the instances in which that deformity has been hereditary, and transmitted for one, two, three, four, five, or, as in the case recounted by Lingard, even six generations. Sterility in masculine pseudohermaphrodites does not depend upon hypospadias but rather upon the more or less rudimentary development of their testicles, especially when there is cryptorchism.
Certain sexual characteristics have been called secondary; the height of the stature, the form of the skeleton, especially of the thorax, pelvis and long bones, the development of the muscular system, that of the subcutaneous pannicle of fat, and of the hairy system generally and locally (beard, moustache, mons veneris, genitalia, perineum and limbs); also the shape and size of the larynx, the more or less accentuated ossification of the thyroid cartilage, the quality of the voice and the presence or default of certain glands; but though useful as confirmatory evidence, none of these points are decisively diagnostic. The same may be said of the existence of a hymen, vagina I or uterus with its tubes and ligaments, of the labia minora and prostate, as.may be easily understood, since the ducts of Wolff and those of Mueller exist in every embryo.
For the sake of completeness I must mention that in twenty-eight instances of pseudohermaphrodism I have found tumours, benign or malignant new growths, generally affecting some part of the urogenital system; simple ovarian cysts; sarcoma, carcinoma, myxoma or teratoma of the genital glands, especially in cryptorchids; myofibroma or carcinoma of the womb; or carcinoma of the bladder or rectum, &c.
The case of Charles Menniken recorded by Engelhardt and already alluded to, is unique; a man who married as a woman, died from cancer of a uterus the vagina of which ended in the male urethra. This is not the place to discuss whether there is any causal connection between carcinoma and congenital malformation of the genital organs; the partisans of the parasitic theory of carcinoma say there is no connection of the kind; I, who am not a partisan at all, may, however, say that I think such a connection not impossible, especially considering the effect of diminished nutrition on the organs affected by these malformations, and also the frequency of malignant degenerations in cryptorchism. Sarcoma and carcinoma too frequently affect a testicle retained in the peritoneal cavity or inguinal canal, for us to deny that such ectopia is associated with a predisposition to malignant disease. I need not insist on the carcinoma, of the bladder in the male hypospadiac. (Christine Bockfleisch whose urethra and bladder had for many years of married life fulfilled the office of a vagina.) Such facts offer much for discussion.
More difficult to understand than any of the cases of errors of sex to which we have referred, is the fact that a woman with prolapsus uteri was supposed to be an hermaphrodite until Saviard explained the true nature of the organ; the only possible explanation is that this occurred in the middle ages.
As curiosities I may mention the following exceptional cases from among those I have collected. There have been two instances in which a solider has proved to be a woman; in one, menstruation betrayed the fact, and the individual had to leave the army and adopt female attire; the other gave birth to a child; a similar case gave rise to the wellknown phrase: "Mas mulier, monachus, mundi mirabile monstrum." Again, a youth, servant in a monastery, was, for having stolen a silver cup, condemned to be whipped, naked, before the whole community, but begged not to be stripped and put to shame as he knew he was a girl; this was the case, and the true sex and existence of menstruation having been ascertained, not only was the punishment remitted, but the young person was provided with female clothes and a dowry, and her true sex having, thanks to the accident of her theft, been recognised, she was married to a wine seller, by whom she had several children. Morache, in his recent work, "Le mariage," relates that for several years he was at college with a fellow pupil, an excellent comrade, extremely intelligent, a moderate worker, taking an excellent position in the classes, who, menstruation having appeared and revealed an error of sex, was suddenly removed by her parents; she afterwards became a beautiful woman and an excellent mother. There is even an instance told of a male pseudohermaphrodite brought up mistakenly as a woman, who became abbess of a convent and died in that dignified position at an advanced age.
The Canoness Magdalena Mugnoz, after seven years in a cloister, was expelled because she was found to be of the male sex: and at the Convent des Filles de Dieu de Chartres, the Canoness Angelique de la Motte d'Aspremont, was accused of having played the man with her religious sisters, and the woman in her nocturnal escapades from the institution, and was deprived of her dignity as canoness and imprisoned.
Two other questions that may bring a pseudohermaphrodite before a surgeon must be considered: first, the parents, or the individual concerned, may demand the amputation of an hypertrophied clitoris, or of a hypospadiac penis, and although such requests are not common, it is well to know that they may be made. In a case in which Hector le Nu detected an error of sex, the parents were convinced that their child was a girl, and desired the operation done; but he refused to perform it as not urgent, but in reality one of complicity, for the youth was merely a male with hypospadias, brought up as a girl from error of sex. But the operation has been performed. Aveling amputated an hypertrophied clitoris; Dr. Berendes did as he thought the same thing in a case afterwards determined as masculine by Landau. Tauber performed it on a young person of 23, although he was aware that two years previously in a bilateral herniotomy, two normal testicles had been removed from the same patient. Quite recently (November, 4, 1902) Hartmann reported to the Surgical Society of Paris that he had in 1892 removed an hypertrophied clitoris from a little girl, aged 7, to prevent self-abuse; fe saw the patient ten years later, and finding a uterus and vagina, judged her to be really a girl. In my opinion Hartmann gave no positive proof that that person was feminine, and it seems more probable that he merely mutilated a male hypospadiac. The mistake, if it was one, was not very serious; but one ought to hold to the principle, "Primum non nocere." The operations of Berendes and Tauber seem quite unjustifiable. What reason could there be for amputating a hypospadiac penis from a man? It is worth remembering that a well-known French surgeon was assassinated, from vengeance, by a man from whom he had taken the testicles in order to cure a variocele.
But the male hypospadiac may demand surgical relief for other reasons: for the pain caused by the erection of the penis, owing to its being bound down in a curve by a cicatricial band. A plastic operation to divide such a band and unite the edges of the incision, so as to heal longitudinally, is certainly then justifiable, and has sometimes been performed, though with very uncertain results, as the cicatrix nearly always contracts in the end. The surgeon's proper field of action is when a male pseudohermaphrodite, a penoscrotal hypospadiac, desires the plastic repair of his penis and scrotum, and operation then is much more efficacious. Thiersch, uncertain that the results would be satisfactory, would not volunteer this proceeding, and only undertook it at the express wish of the interested person. But since his time surgical art has made such advances in technique, that Castellana of Palermo, succeeded by a series of operations in conferring on Carmela, afterwards Carmelo Caponetto, the ability to make water like a man, in an upright position. The supposed girl was a penoscrotal hypospadiac with a double vagina, and contracted gonorrhoea at the age of 15. Castellana succeeded in uniting the two sides of the divided scrotum and in reconstructing a urethra split almost as far as the balanic orifice of the canal. The patient had his male civil rights established, and was so charmed at being able to make water standing up without wetting his trousers, that "ex voto," he deposited the long beautiful hair he had worn as a girl in one of the chapels of the Church of the AIadonna della Pietraperzia at Palermo.
The extreme value of such a plastic operation may not at once strike even medical men -- it is apparently such a simple matter. But consider the difficulties that a male hypospadiac has in relieving himself when he wants to micturate. Of course he cannot make use of the public conveniences for men, still less would he dare to enter those for women. The consequence of this difficulty are evident, and for him this surgical relief, successfully given, is one of the greatest possible interest.
As a surgical curiosity, I may here mention a case of Nitze's, published by Kapsammer. Nitze, at an operation on a man, removed a phosphatic urinary calculus, weighing 162 grammes, which had formed in a utriculus rhasculinus, communicating with the prostatic portion of the urethra, by a narrow orifice situated in the middle of the verumontanum.
Finally, before concluding this review of the practical aspects of pseudohermaphrodism, I must mention that, in several cases, a doubtful-sex has been in the first instance declared to be feminine, later on masculine , and again later feminine, that is to say, that the opinion as to the sex of the same individual has been changed several times. For example, Anne Grandjean was baptised and up to the age of 14, brought up as a girl; afterwards, on the advice of her confessor, as a boy, and called jean. He married but had no children. Another confessor forbade his wifeto continue to consider Jean as a man on the ground that his sex was feminine. The marriage was annulled, and Jean and his wife were condemned for profanation of the Sacrament. At last after various episodes, this cruel verdict was annulled; but Jean was forced to resume female attire, and was forever interdicted from attempting sexual relations with any woman. In a case related by Otto, a peasant, Kaluza, the third husband of his wife, sought a divorce, and the medical experts decided that the wife was really a woman, and that the dyspareunia depended on the organisation of the husband himself. On appeal a new commission decided that the wife was a male hypospadiac. It is remarkable that neither of the previous husbands had remarked anything unusual about that person. Again Josephine Alarzo was baptised as a girl, and at the age of 14 was declared to be a boy; but the necropsy at an advanced age proved that she was a woman. In a case already referred to Saenger several times changed his opinion as to the sex of his patient.
The enormous frequency of these mistakes prescribes an extreme caution in determining the sex in any doubtful case. It is far better to abstain and defer any decision to a more advanced age than to risk an error of which the results might be disastrous. It is therefore with great show of reason that two French and one Italian medical men have demanded that the rubric "sex indeterminate," should be admitted in registering a birth. As the child develops the sex can be declared either later, when it records itself or by means of expert medical examination, or even by a diagnostic operation. As yet no legislation has consented to that demand and sanctioned three rubrics, that is to say, S.M., S.F., and S.I., instead of two for the description of the sex of new-born infants. Indeed, as regards pseudohermaphrodites, cases of doubtful sex, and errors of sex, the laws vary very much in different countries.
Warsaw, November 1903. [A list of the autlior's different published works relating to pseudohermaphrodism will be found ante pp. 175, 176. To those who have reason to take especial interest in the subject, "Interessante Beobactungen aus dem Gebiete des Scheinzwitterthumes" and "Chirurgische Ueberraschungen auf dem Gebiete des Scheinzwitterthumes," reprints from the Jahrbuch fuer sexuelle Zwischenstufen, 1902 and 1903, will prove particularly interesting. Some of the earlier works were noticed in this journal, ante vol. xvi. p. 104. A recent observation by Simon (p. 106. August, 1903), and a case of Sir Hector Cameron's appear not to have been known to the author at the time of writing. -- ED.]
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Organisation Intersex International
Hermaphrodism and the
Medical Community
