Chapter V: Major Terata (2)
The most celebrated monster of this type was Ritta-Christina, who was born in Sassari, in Sardinia, March 23, 1829. These twins were the result of the ninth confinement of their mother, a woman of thirty-two. Their superior extremities were double, but they joined in a common trunk at a point a little below the mammae. Below this point they had a common trunk and single lower extremities. The right one, christened Ritta, was feeble and of a sad and melancholy countenance; the left, Christina, was vigorous and of a gay and happy aspect. They suckled at different times, and sensations in the upper extremities were distinct. They expelled urine and feces simultaneously, and had the indications in common. Their parents, who were very poor, brought them to Paris for the purpose of public exhibition, which at first was accomplished clandestinely, but finally interdicted by the public authorities, who feared that it would open a door for psychologic discussion and speculation. This failure of the parents to secure public patronage increased their poverty and hastened the death of the children by unavoidable exposure in a cold room. The nervous system of the twins had little in common except in the line of union, the anus, and the sexual organs, and Christina was in good health all through Ritta's sickness; when Ritta died, her sister, who was suckling at the mother's breast, suddenly relaxed hold and expired with a sigh. At the postmortem, which was secured with some difficulty on account of the authorities ordering the bodies to be burned, the pericardium was found single, covering both hearts. The digestive organs were double and separate as far as the lower third of the ilium, and the cecum was on the left side and single, in common with the lower bowel. The livers were fused and the uterus was double. The vertebral columns, which were entirely separate above, were joined below by a rudimentary os innorminatum. There was a junction between the manubrium of each. Sir Astley Cooper saw a monster in Paris in 1792 which, by his description, must have been very similar to Ritta-Christina.
The Tocci brothers were born in 1877 in the province of Turin, Italy. They each had a well-formed head, perfect arms, and a perfect thorax to the sixth rib; they had a common abdomen, a single anus, two legs, two sacra, two vertebral columns, one penis, but three buttocks, the central one containing a rudimentary anus. The right boy was christened Giovanni-Batista, and the left Giacomo. Each individual had power over the corresponding leg on his side, but not over the other one. Walking was therefore impossible. All their sensations and emotions were distinctly individual and independent. At the time of the report, in 1882, they were in good health and showed every indication of attaining adult age. Figure 48 represents these twins as they were exhibited several years ago in Germany.
McCallum saw two female children in Montreal in 1878 named Marie-Rosa Drouin. They formed a right angle with their single trunk, which commenced at the lower part of the thorax of each. They had a single genital fissure and the external organs of generation of a female. A little over three inches from the anus was a rudimentary limb with a movable articulation; it measured five inches in length and tapered to a fine point, being furnished with a distinct nail, and it contracted strongly to irritation. Marie, the left child, was of fair complexion and more strongly developed than Rosa. The sensations of hunger and thirst were not experienced at the same time, and one might be asleep while the other was crying. The pulsations and the respiratory movements were not synchronous. They were the products of the second gestation of a mother aged twenty-six, whose abdomen was of such preternatural size during pregnancy that she was ashamed to appear in public. The order of birth was as follows: one head and body, the lower extremity, and the second body and head.
CLASS VII.--There are many instances of bicephalic monsters on record. Pare mentions and gives an illustration of a female apparently single in conformation, with the exception of having two heads and two necks. The Ephemerides, Haller, Schenck, and Archenholz cite examples, and there is an old account of a double-headed child, each of whose heads were baptized, one called Martha and the other Mary. One was of a gay and the other a sad visage, and both heads received nourishment; they only lived a couple of days. There is another similar record of a Milanese girl who had two heads, but was in all other respects single, with the exception that after death she was found to have had two stomachs. Besse mentions a Bavarian woman of twenty-six with two heads, one of which was comely and the other extremely ugly; Batemen quotes what is apparently the same case--a woman in Bavaria in 1541 with two heads, one of which was deformed, who begged from door to door, and who by reason of the influence of pregnant women was given her expenses to leave the country.
A more common occurrence of this type is that in which there is fusion of the two heads. Moreau speaks of a monster in Spain which was shown from town to town. Its heads were fused; it had two mouths and two noses; in each face an eye well conformed and placed above the nose; there was a third eye in the middle of the forehead common to both heads; the third eye was of primitive development and had two pupils. Each face was well formed and had its own chin. Buffon mentions a cat, the exact analogue of Moreau's case. Sutton speaks of a photograph sent to Sir James Paget in 1856 by William Budd of Bristol. This portrays a living child with a supernumerary head, which had mouth, nose, eyes, and a brain of its own. The eyelids were abortive, and as there was no orbital cavity the eyes stood out in the form of naked globes on the forehead. When born, the corneas of both heads were transparent, but then became opaque from exposure. The brain of the supernumerary head was quite visible from without, and was covered by a membrane beginning to slough. On the right side of the head was a rudimentary external ear. The nurse said that when the child sucked some milk regurgitated through the supernumerary mouth. The great physiologic interest in this case lies in the fact that every movement and every act of the natural face was simultaneously repeated by the supernumerary face in a perfectly consensual manner, i.e., when the natural mouth sucked, the second mouth sucked; when the natural face cried, yawned, or sneezed, the second face did likewise; and the eyes of the two heads moved in unison. The fate of the child is not known.
Home speaks of a child born in Bengal with a most peculiar fusion of the head. The ordinary head was nearly perfect and of usual volume, but fused with its vertex and reversed was a supernumerary head. Each head had its own separate vessels and brain, and each an individual sensibility, but if one had milk first the other had an abundance of saliva in its mouth. It narrowly escaped being burned to death at birth, as the midwife, greatly frightened by the monstrous appearance, threw it into the fire to destroy it, from whence it was rescued, although badly burned, the vicious conformation of the accessory head being possibly due to the accident. At the age of four it was bitten by a venomous serpent and, as a result, died. Its skull is in the possession of the Royal College of Surgeons in London.
The following well-known story of Edward Mordake, though taken from lay sources, is of sufficient notoriety and interest to be mentioned here:--
"One of the weirdest as well as most melancholy stories of human deformity is that of Edward Mordake, said to have been heir to one of the noblest peerages in England. He never claimed the title, however, and committed suicide in his twenty-third year. He lived in complete seclusion, refusing the visits even of the members of his own family. He was a young man of fine attainments, a profound scholar, and a musician of rare ability. His figure was remarkable for its grace, and his face--that is to say, his natural face--was that of an Antinous. But upon the back of his head was another face, that of a beautiful girl, 'lovely as a dream, hideous as a devil.' The female face was a mere mask, 'occupying only a small portion of the posterior part of the skull, yet exhibiting every sign of intelligence, of a malignant sort, however.' It would be seen to smile and sneer while Mordake was weeping. The eyes would follow the movements of the spectator, and the lips would 'gibber without ceasing.' No voice was audible, but Mordake avers that he was kept from his rest at night by the hateful whispers of his 'devil twin,' as he called it, 'which never sleeps, but talks to me forever of such things as they only speak of in hell. No imagination can conceive the dreadful temptations it sets before me. For some unforgiven wickedness of my forefathers I am knit to this fiend--for a fiend it surely is. I beg and beseech you to crush it out of human semblance, even if I die for it.' Such were the words of the hapless Mordake to Manvers and Treadwell, his physicians. In spite of careful watching he managed to procure poison, whereof he died, leaving a letter requesting that the 'demon face' might be destroyed before his burial, 'lest it continues its dreadful whisperings in my grave.' At his own request he was interred in a waste place, without stone or legend to mark his grave."
A most curious case was that of a Fellah woman who was delivered at Alexandria of a bicephalic monster of apparently eight months' pregnancy. This creature, which was born dead, had one head white and the other black the change of color commencing at the neck of the black head. The bizarre head was of negro conformation and fully developed, and the colored skin was found to be due to the existence of pigment similar to that found in the black race. The husband of the woman had a light brown skin, like an ordinary Fellah man, and it was ascertained that there were some negro laborers in port during the woman's pregnancy; but no definite information as to her relations with them could be established, and whether this was a case of maternal impression or superfetation can only be a matter of conjecture.
Fantastic monsters, such as acephalon, paracephalon, cyclops, pseudencephalon, and the janiceps, prosopthoracopagus, disprosopus, etc., although full of interest, will not be discussed here, as none are ever viable for any length of time, and the declared intention of this chapter is to include only those beings who have lived.
CLASS VIII.--The next class includes the parasitic terata, monsters that consist of one perfect body, complete in every respect, but from the neighborhood of whose umbilicus depends some important portion of a second body. Pare, Benivenius, and Columbus describe adults with acephalous monsters attached to them. Schenck mentions 13 cases, 3 of which were observed by him. Aldrovandus shows 3 illustrations under the name of "monstrum bicorpum monocephalon." Bustorf speaks of a case in which the nates and lower extremities of one body proceeded out of the abdomen of the other, which was otherwise perfect. Reichel and Anderson mention a living parasitic monster, the inferior trunk of one body proceeding from the pectoral region of the other.
Pare says that there was a man in Paris in 1530, quite forty years of age, who carried about a parasite without a head, which hung pendant from his belly. This individual was exhibited and drew great crowds. Pare appends an illustration, which is, perhaps, one of the most familiar in all teratology. He also gives a portrait of a man who had a parasitic head proceeding from his epigastrium, and who was born in Germany the same year that peace was made with the Swiss by King Francis. This creature lived to manhood and both heads were utilized in alimentation. Bartholinus details a history of an individual named Lazarus-Joannes Baptista Colloredo, born in Genoa in 1617, who exhibited himself all over Europe. From his epigastrium hung an imperfectly developed twin that had one thigh, hands, body, arms, and a well-formed head covered with hair, which in the normal position hung lowest. There were signs of independent existence in the parasite, movements of respiration, etc., but its eyes were closed, and, although saliva constantly dribbled from its open mouth, nothing was ever ingested. The genitals were imperfect and the arms ended in badly formed hands. Bartholinus examined this monster at twenty-two, and has given the best report, although while in Scotland in 1642 he was again examined, and accredited with being married and the father of several children who were fully and admirably developed. Moreau quotes a case of an infant similar in conformation to the foregoing monster, who was born in Switzerland in 1764, and whose supernumerary parts were amputated by means of a ligature. Winslow reported before the Academie Royale des Sciences the history of a girl of twelve who died at the Hotel-Dieu in 1733. She was of ordinary height and of fair conformation, with the exception that hanging from the left flank was the inferior half of another girl of diminutive proportions. The supernumerary body was immovable, and hung so heavily that it was said to be supported by the hands or by a sling. Urine and feces were evacuated at intervals from the parasite, and received into a diaper constantly worn for this purpose. Sensibility in the two was common, an impression applied to the parasite being felt by the girl. Winslow gives an interesting report of the dissection of this monster, and mentions that he had seen an Italian child of eight who had a small head proceeding from under the cartilage of the third left rib. Sensibility was common, pinching the ear of the parasitic head causing the child with the perfect head to cry. Each of the two heads received baptism, one being named John and the other Matthew. A curious question arose in the instance of the girl, as to whether the extreme unction should be administered to the acephalous fetus as well as to the child.
In 1742, during the Ambassadorship of the Marquis de l'Hopital at Naples, he saw in that city an aged man, well conformed, with the exception that, like the little girl of Winslow, he had the inferior extremities of a male child growing from his epigastric region. Haller and Meckel have also observed cases like this. Bordat described before the Royal Institute of France, August, 1826, a Chinaman, twenty-one years of age, who had an acephalous fetus attached to the surface of his breast (possibly "A-ke").
Dickinson describes a wonderful child five years old, who, by an extraordinary freak of nature, was an amalgamation of two children. From the body of an otherwise perfectly formed child was a supernumerary head protruding from a broad base attached to the lower lumbar and sacral region. This cephalic mass was covered with hair about four or five inches long, and showed the rudiments of an eye, nose, mouth, and chin. This child was on exhibition when Dickinson saw it. Montare and Reyes were commissioned by the Academy of Medicine of Havana to examine and report on a monstrous girl of seven months, living in Cuba. The girl was healthy and well developed, and from the middle line of her body between the xiphoid cartilage and the umbilicus, attached by a soft pedicle, was an accessory individual, irregular, of ovoid shape, the smaller end, representing the head, being upward. The parasite measured a little over 1 foot in length, 9 inches about the head, and 7 3/4 inches around the neck. The cranial bones were distinctly felt, and the top of the head was covered by a circlet of hair. There were two rudimentary eyebrows; the left eye was represented by a minute perforation encircled with hair; the right eye was traced by one end of a mucous groove which ran down to another transverse groove representing the mouth; the right third of this latter groove showed a primitive tongue and a triangular tooth, which appeared at the fifth month. There was a soft, imperforate nose, and the elements of the vertebral column could be distinguished beneath the skin; there were no legs; apparently no vascular sounds; there was separate sensation, as the parasite could be pinched without attracting the perfect infant's notice. The mouth of the parasite constantly dribbled saliva, but showed no indication of receiving aliment.
Louise L., known as "La dame a quatre jambes," was born in 1869, and had attached to her pelvis another rudimentary pelvis and two atrophied legs of a parasite, weighing 8 kilos. The attachment was effected by means of a pedicle 33 cm. in diameter, having a bony basis, and being fixed without a joint. The attachment almost obliterated the vulva and the perineum was displaced far backward. At the insertion of the parasite were two rudimentary mammae, one larger than the other. No genitalia were seen on the parasite and it exhibited no active movements, the joints of both limbs being ankylosed. The woman could localize sensations in the parasite except those of the feet. She had been married five years, and bore, in the space of three years, two well-formed daughters.
Quite recently there was exhibited in the museums of the United States an individual bearing the name "Laloo," who was born in Oudh, India, and was the second of four children. At the time of examination he was about nineteen years of age. The upper portion of a parasite was firmly attached to the lower right side of the sternum of the individual by a bony pedicle, and lower by a fleshy pedicle, and apparently contained intestines. The anus of the parasite was imperforate; a well-developed penis was found, but no testicles; there was a luxuriant growth of hair on the pubes. The penis of the parasite was said to show signs of erection at times, and urine passed through it without the knowledge of the boy. Perspiration and elevation of temperature seemed to occur simultaneously in both. To pander to the morbid curiosity of the curious, the "Dime Museum" managers at one time shrewdly clothed the parasite in female attire, calling the two brother and sister; but there is no doubt that all the traces of sex were of the male type. An analogous case was that of "A-Ke," a Chinaman, who was exhibited in London early in the century, and of whom and his parasite anatomic models are seen in our museums. Figure 58 represents an epignathus, a peculiar type parasitic monster, in which the parasite is united to the inferior maxillary bone of the autosite.
CLASS IX.--Of "Lusus naturae" none is more curious than that of duplication of the lower extremities. Pare says that on January 9, 1529, there was living in Germany a male infant having four legs and four arms. In Paris, at the Academie des Sciences, on September 6, 1830, there was presented by Madame Hen, a midwife, a living male child with four legs, the anus being nearly below the middle of the third buttock; and the scrotum between the two left thighs, the testicles not yet descended. There was a well-formed and single pelvis, and the supernumerary legs were immovable. Aldrovandus mentions several similar instances, and gives the figure of one born in Rome; he also describes several quadruped birds. Bardsley speaks of a male child with one head, four arms, four legs, and double generative organs. He gives a portrait of the child when it was a little over a year old. Heschl published in Vienna in 1878 a description of a girl of seventeen, who instead of having a duplication of the superior body, as in "Millie-Christine, the two-headed nightingale," had double parts below the second lumbar vertebra. Her head and upper body resembled a comely, delicate girl of twelve.
Wells a describes Mrs. B., aged twenty, still alive and healthy. The duplication in this case begins just above the waist, the spinal column dividing at the third lumbar vertebra, below this point everything being double. Micturition and defecation occur at different times, but menstruation occurs simultaneously. She was married at nineteen, and became pregnant a year later on the left side, but abortion was induced at the fourth month on account of persistent nausea and the expectation of impossible delivery. Whaley, in speaking of this case, said Mrs. B. utilized her outside legs for walking; he also remarks that when he informed her that she was pregnant on the left side she replied, "I think you are mistaken; if it had been on my right side I would come nearer believing it;"--and after further questioning he found, from the patient's observation, that her right genitals were almost invariably used for coitus. Bechlinger of Para, Brazil, describes a woman of twenty-five, a native of Martinique, whose father was French and mother a quadroon, who had a modified duplication of the lower body. There was a third leg attached to a continuation of the processus coceygeus of the sacrum, and in addition to well developed mammae regularly situated, there were two rudimentary ones close together above the pubes. There were two vaginae and two well-developed vulvae, both having equally developed sensations. The sexual appetite was markedly developed, and coitus was practised in both vaginae. A somewhat similar case, possibly the same, is that of Blanche Dumas, born in 1860. She had a very broad pelvis, two imperfectly developed legs, and a supernumerary limb attached to the symphysis, without a joint, but with slight passive movement. There was a duplication of bowel, bladder, and genitalia. At the junction of the rudimentary limb with the body, in front, were two rudimentary mammary glands, each containing a nipple.
Other instances of supernumerary limbs will be found in Chapter VI.
CLASS X.--The instances of diphallic terata, by their intense interest to the natural bent of the curious mind, have always elicited much discussion. To many of these cases have been attributed exaggerated function, notwithstanding the fact that modern observation almost invariably shows that the virile power diminishes in exact proportion to the extent of duplication. Taylor quotes a description of a monster, exhibited in London, with two distinct penises, but with only one distinct testicle on either side. He could exercise the function of either organ.
Schenck, Schurig, Bartholinus, Loder, and Ollsner report instances of diphallic terata; the latter case a was in a soldier of Charles VI, twenty-two years old, who applied to the surgeon for a bubonic affection, and who declared that he passed urine from the orifice of the left glans and also said that he was incapable of true coitus. Valentini mentions an instance in a boy of four, in which the two penises were superimposed. Bucchettoni speaks of a man with two penises placed side by side. There was an anonymous case described of a man of ninety-three with a penis which was for more than half its length divided into two distinct members, the right being somewhat larger than the left. From the middle of the penis up to the symphysis only the lower wall of the urethra was split. Jenisch describes a diphallic infant, the offspring of a woman of twenty-five who had been married five years. Her first child was a well-formed female, and the second, the infant in question, cried much during the night, and several times vomited dark-green matter. In lieu of one penis there were two, situated near each other, the right one of natural size and the left larger, but not furnished with a prepuce. Each penis had its own urethra, from which dribbled urine and some meconium. There was a duplication of each scrotum, but only one testicle in each, and several other minor malformations.
Gore, reported by Velpeau, has seen an infant of eight and one-half months with two penises and three lower extremities. The penises were 4 cm. apart and the scrotum divided, containing one testicle in each side. Each penis was provided with a urethra, urine being discharged from both simultaneously. In a similar case, spoken of by Geoffroy-Saint-Hilaire, the two organs were also separate, but urine and semen escaped sometimes from one, sometimes from both.
The most celebrated of all the diphallic terata was Jean Baptista dos Santos, who when but six months old was spoken of by Acton. His father and mother were healthy and had two well-formed children. He was easily born after an uneventful pregnancy. He was good-looking, well proportioned, and had two distinct penises, each as large as that of a child of six months. Urination proceeded simultaneously from both penises; he had also two scrotums. Behind and between the legs there was another limb, or rather two, united throughout their length. It was connected to the pubis by a short stem 1/2 inch long and as large as the little finger, consisting of separate bones and cartilages. There was a patella in the supernumerary limb on the anal aspect, and a joint freely movable. This compound limb had no power of motion, but was endowed with sensibility. A journal in London, after quoting Acton's description, said that the child had been exhibited in Paris, and that the surgeons advised operation. Fisher, to whom we are indebted for an exhaustive work in Teratology, received a report from Havana in July, 1865, which detailed a description of Santos at twenty-two years of age, and said that he was possessed of extraordinary animal passion, the sight of a female alone being sufficient to excite him. He was said to use both penises, after finishing with one continuing with the other; but this account of him does not agree with later descriptions, in which no excessive sexual ability had been noticed. Hart describes the adult Santos in full, and accompanies his article with an illustration. At this time he was said to have developed double genitals, and possibly a double bladder communicating by an imperfect septum. At adulthood the anus was three inches anterior to the os coceygeus. In the sitting or lying posture the supernumerary limb rested on the front of the inner surface of the lower third of his left thigh. He was in the habit of wearing this limb in a sling, or bound firmly to the right thigh, to prevent its unseemly dangling when erect. The perineum proper was absent, the entire space between the anus and the posterior edge of the scrotum being occupied by the pedicle. Santos' mental and physical functions were developed above normal, and he impressed everybody with his accomplishments. Geoffroy-Saint-Hilaire records an instance in which the conformation was similar to that of Santos. There was a third lower extremity consisting of two limbs fused into one with a single foot containing ten distinct digits. He calls the case one of arrested twin development.
Van Buren and Keyes describe a case in a man of forty-two, of good, healthy appearance. The two distinct penises of normal size were apparently well formed and were placed side by side, each attached at its root to the symphysis. Their covering of skin was common as far as the base of the glans; at this point they seemed distinct and perfect, but the meatus of the left was imperforate. The right meatus was normal, and through it most of the urine passed, though some always dribbled through an opening in the perineum at a point where the root of the scrotum should have been. On lifting the double-barreled penis this opening could be seen and was of sufficient size to admit the finger. On the right side of the aperture was an elongated and rounded prominence similar in outline to a labium majus. This prominence contained a testicle normal in shape and sensibility, but slightly undersized, and surrounded, as was evident from its mobility, by a tunica vaginalis. The left testicle lay on the tendon of the adductor longus in the left groin; it was not fully developed, but the patient had sexual desires, erections, and emissions. Both penises became erect simultaneously, the right more vigorously. The left leg was shorter than the right and congenitally smaller; the mammae were of normal dimensions.
Sangalli speaks of a man of thirty-five who had a supernumerary penis, furnished with a prepuce and capable of erection. At the apex of the glans opened a canal about 12 cm. long, through which escaped monthly a serous fluid. Smith mentions a man who had two penises and two bladders, on one of which lithotomy was performed. According to Ballantyne, Taruffi, the scholarly observer of terata, mentions a child of forty-two months and height of 80 cm. who had two penises, each furnished with a urethra and well-formed scrotal sacs which were inserted in a fold of the groin. There were two testicles felt in the right scrotum and one in the left. Fecal evacuations escaped through two anal orifices. There is also another case mentioned similar to the foregoing in a man of forty; but here there was an osseous projection in the middle line behind the bladder. This patient said that erection was simultaneous in both penises, and that he had not married because of his chagrin over his deformity. Cole speaks of a child with two well-developed male organs, one to the left and the other to the right of the median line, and about 1/4 or 1/2 inch apart at birth. The urethra bifurcated in the perineal region and sent a branch to each penis, and urine passed from each meatus. The scrotum was divided into three compartments by two raphes, and each compartment contained a testicle. The anus at birth was imperforate, but the child was successfully operated on, and at its sixtieth day weighed 17 pounds.
Lange says that an infant was brought to Karg for relief of anal atresia when fourteen days old. It was found to possess duplicate penises, which communicated each to its distinct half of the bladder as defined by a median fold. The scrotum was divided into three portions by two raphes, and each lateral compartment contained a fully formed testicle. This child died because of its anal malformation, which we notice is a frequent associate of malformations or duplicity of the penis. There is an example in an infant described in which there were two penises, each about 1/2 inch long, and a divided scrotal sac 21 inches long. Englisch speaks of a German of forty who possessed a double penis of the bifid type.
Ballantyne and his associates define diphallic terata as individuals provided with two more or less well-formed and more or less separate penises, who may show also other malformations of the adjoining parts and organs (e.g., septate bladder), but who are not possessed of more than two lower limbs. This definition excludes, therefore, the cases in which in addition to a double penis there is a supernumerary lower extremity--such a case, for example, as that of Jean Baptista dos Santos, so frequently described by teratologists. It also excludes the more evident double terata, and, of course, the cases of duplication of the female genital organs (double clitoris, vulva, vagina, and uterus). Although Schurig, Meckel, Himly, Taruffi, and others give bibliographic lists of diphallic terata, even in them erroneous references are common, and there is evidence to show that many cases have been duplicated under different names. Ballantyne and Skirving have consulted all the older original references available and eliminated duplications of reports and, adhering to their original definition, have collected and described individually 20 cases; they offer the following conclusions:--
1. Diphallus, or duplication of the penis in an otherwise apparently single individual, is a very rare anomaly, records of only 20 cases having been found in a fairly exhaustive search through teratologic literature. As a distinct and well-authenticated type it has only quite recently been recognized by teratologists.
2. It does not of itself interfere with intrauterine or extrauterine life; but the associated anomalies (e.g., atresia ani) may be sources of danger. If not noticed at birth, it is not usually discovered till adult life, and even then the discovery is commonly accidental.
3. With regard to the functions of the pelvic viscera, urine may be passed by both penises, by one only, or by neither. In the last instance it finds exit by an aperture in the perineum. There is reason to believe that semen may be passed in the same way; but in most of the recorded cases there has been sterility, if not inability to perform the sexual act.
4. All the degrees of duplication have been met with, from a fissure of the glans penis to the presence of two distinct penises inserted at some distance from each other in the inguinal regions.
5. The two penises are usually somewhat defective as regards prepuce, urethra, etc.; they may lie side by side, or more rarely may be situated anteroposteriorly; they may be equal in size, or less commonly one is distinctly larger than the other; and one or both may be perforate or imperforate.
6. The scrotum may be normal or split; the testicles, commonly two in number, may be normal or atrophic, descended or undescended; the prostate may be normal or imperfectly developed, as may also the vasa deferentia and vesiculae seminales.
7. The commonly associated defects are: More or less completely septate bladder, atresia ani, or more rarely double anus, double urethra, increased breadth of the bony pelvis with defect of the symphysis pubis, and possibly duplication of the lower end of the spine, and hernia of some of the abdominal contents into a perineal pouch. Much more rarely, duplication of the heart, lungs, stomach, and kidneys has been noted, and the lower limbs may be shorter than normal.
CLASS XI.--Cases of fetus in fetu, those strange instances in which one might almost say that a man may be pregnant with his brother or sister, or in which an infant may carry its twin without the fact being apparent, will next be discussed. The older cases were cited as being only a repetition of the process by which Eve was born of Adam. Figure 63 represents an old engraving showing the birth of Eve. Bartholinus, the Ephemerides, Otto, Paullini, Schurig, and Plot speak of instances of fetus in fetu. Ruysch describes a tumor contained in the abdomen of a man which was composed of hair, molar teeth, and other evidences of a fetus. Huxham reported to the Royal Society in 1748 the history of a child which was born with a tumor near the anus larger than the whole body of the child; this tumor contained rudiments of an embryo. Young speaks of a fetus which lay encysted between the laminae of the transverse mesocolon, and Highmore published a report of a fetus in a cyst communicating with the duodenum. Dupuytren gives an example in a boy of thirteen, in whom was found a fetus. Gaetano-Nocito, cited by Philipeaux, has the history of a taken with a great pain in the right hypochondrium, and from which issued subsequently fetal bones and a mass of macerated embryo. His mother had had several double pregnancies, and from the length of the respective tibiae one of the fetuses seemed to be of two months' and the other of three months' intrauterine life. The man died five years after the abscess had burst spontaneously.
Brodie speaks of a case in which fetal remains were taken from the abdomen of a girl of two and one-half years. Gaither describes a child of two years and nine months, supposed to be affected with ascites, who died three hours after the physician's arrival. In its abdomen was found a fetus weighing almost two pounds and connected to the child by a cord resembling an umbilical cord. This child was healthy for about nine months, and had a precocious longing for ardent spirits, and drank freely an hour before its death.
Blundell says that he knew "a boy who was literally and without evasion with child, for the fetus was contained in a sac communicating with the abdomen and was connected to the side of the cyst by a short umbilical cord; nor did the fetus make its appearance until the boy was eight or ten years old, when after much enlargement of pregnancy and subsequent flooding the boy died." The fetus, removed after death, on the whole not very imperfectly formed, was of the size of about six or seven months' gestation. Bury cites an account of a child that had a second imperfectly developed fetus in its face and scalp. There was a boy by the name of Bissieu who from the earliest age had a pain in one of his left ribs; this rib was larger than the rest and seemed to have a tumor under it. He died of phthisis at fourteen, and after death there was found in a pocket lying against the transverse colon and communicating with it all the evidences of a fetus.
At the Hopital de la Charite in Paris, Velpeau startled an audience of 500 students and many physicians by saying that he expected to find a rudimentary fetus in a scrotal tumor placed in his hands for operation. His diagnosis proved correct, and brought him resounding praise, and all wondered as to his reasons for expecting a fetal tumor. It appears that he had read with care a report by Fatti of an operation on the scrotum of a child which had increased in size as the child grew, and was found to contain the ribs, the vertebral column, the lower extremities as far as the knees, and the two orbits of a fetus; and also an account of a similar operation performed by Wendt of Breslau on a Silesian boy of seven. The left testicle in this case was so swollen that it hung almost to the knee, and the fetal remains removed weighed seven ounces.
Sulikowski relates an instance of congenital fetation in the umbilicus of a girl of fourteen, who recovered after the removal of the anomaly. Aretaeos described to the members of the medical fraternity in Athens the case of a woman of twenty-two, who bore two children after a seven months' pregnancy. One was very rudimentary and only 21 inches long, and the other had an enormous head resembling a case of hydrocephalus. On opening the head of the second fetus, another, three inches long, was found in the medulla oblongata, and in the cranial cavity with it were two additional fetuses, neither of which was perfectly formed.
Broca speaks of a fetal cyst being passed in the urine of a man of sixty-one; the cyst contained remnants of hair, bone, and cartilage. Atlee submits quite a remarkable case of congenital ventral gestation, the subject being a girl of six, who recovered after the discharge of the fetal mass from the abdomen. McIntyre speaks of a child of eleven, playing about and feeling well, but whose abdomen progressively increased in size 1 1/2 inches each day. After ten days there was a large fluctuating mass on the right side; the abdomen was opened and the mass enucleated; it was found to contain a fetal mass weighing nearly five pounds, and in addition ten pounds of fluid were removed. The child made an early recovery. Rogers mentions a fetus that was found in a man's bladder. Bouchacourt reports the successful extirpation of the remains of a fetus from the rectum of a child of six. Miner describes a successful excision of a congenital gestation.
Modern literature is full of examples, and nearly every one of the foregoing instances could be paralleled from other sources. Rodriguez is quoted as reporting that in July, 1891, several newspapers in the city of Mexico published, under the head of "A Man-mother," a wonderful story, accompanied by wood-cuts, of a young man from whose body a great surgeon had extracted a "perfectly developed fetus." One of these wood-cuts represented a tumor at the back of a man opened and containing a crying baby. In commenting upon this, after reviewing several similar cases of endocymian monsters that came under his observation in Mexico, Rodriguez tells what the case which had been so grossly exaggerated by the lay journals really was: An Indian boy, aged twenty-two, presented a tumor in the sacrococcygeal region measuring 53 cm. in circumference at the base, having a vertical diameter of 17 cm. and a transverse diameter of 13 cm. It had no pedicle and was fixed, showing unequal consistency. At birth this tumor was about the size of a pigeon's egg. A diagnosis of dermoid cyst was made and two operations were performed on the boy, death following the second. The skeleton showed interesting conditions; the rectum and pelvic organs were natural, and the contents of the cyst verified the diagnosis.
Quite similar to the cases of fetus in fetu are the instances of dermoid cysts. For many years they have been a mystery to physiologists, and their origin now is little more than hypothetic. At one time the fact of finding such a formation in the ovary of an unmarried woman was presumptive evidence that she was unchaste; but this idea was dissipated as soon as examples were reported in children, and to-day we have a well-defined difference between congenital and extrauterine pregnancy. Dermoid cysts of the ovary may consist only of a wall of connective tissue lined with epidermis and containing distinctly epidermic scales which, however, may be rolled up in firm masses of a more or less soapy consistency; this variety is called by Orth epidermoid cyst; or, according to Warren, a form of cyst made up of skin containing small and ill-defined papillae, but rich in hair follicles and sebaceous glands. Even the erector pili muscle and the sudoriparous gland are often found. The hair is partly free and rolled up into thick balls or is still attached to the walls. A large mass of sebaceous material is also found in these cysts. Thomson reports a case of dermoid cyst of the bladder containing hair, which cyst he removed. It was a pedunculated growth, and it was undoubtedly vesical and not expelled from some ovarian source through the urinary passage, as sometimes occurs.
The simpler forms of the ordinary dermoid cysts contain bone and teeth. The complicated teratoma of this class may contain, in addition to the previously mentioned structures, cartilage and glands, mucous and serous membrane, muscle, nerves, and cerebral substance, portions of eyes, fingers with nails, mammae, etc. Figure 64 represents a cyst containing long red hair that was removed from a blonde woman aged forty-four years who had given birth to six children. Cullingworth reports the history of a woman in whom both ovaries were apparently involved by dermoids, who had given birth to 12 children and had three miscarriages--the last, three months before the removal of the growths. The accompanying illustration, taken from Baldy, pictures a dermoid cyst of the complicated variety laid open and exposing the contents in situ. Mears of Philadelphia reports a case of ovarian cyst removed from a girl of six and a half by Bradford of Kentucky in 1875. From this age on to adult life many similar cases are recorded. Nearly every medical museum has preserved specimens of dermoid cysts, and almost all physicians are well acquainted with their occurrence. The curious formations and contents and the bizarre shapes are of great variety. Graves mentions a dermoid cyst containing the left side of a human face, an eye, a molar tooth, and various bones. Dermoid cysts are found also in regions of the body quite remote from the ovary. The so-called "orbital wens" are true inclusion of the skin of a congenital origin, as are the nasal dermoids and some of the cysts of the neck.
Weil reported the case of a man of twenty-two years who was born with what was supposed to be a spina bifida in the lower sacral region. According to Senn, the swelling never caused any pain or inconvenience until it inflamed, when it opened spontaneously and suppurated, discharging a large quantity of offensive pus, hair, and sebaceous material, thus proving it to have been a dermoid. The cyst was freely incised, and there were found numerous openings of sweat glands, from which drops of perspiration escaped when the patient was sweating.
Dermoid cysts of the thorax are rare. Bramann reported a case in which a dermoid cyst of small size was situated over the sternum at the junction of the manubrium with the gladiolus, and a similar cyst in the neck near the left cornu of the hyoid bone. Chitten removed a dermoid from the sternum of a female of thirty-nine, the cyst containing 11 ounces of atheromatous material. In the Museum of St. Bartholomew's Hospital in London there is a congenital tumor which was removed from the anterior mediastinum of a woman of twenty one, and contained portions of skin, fat, sebaceous material, and two pieces of bone similar to the superior maxilla, and in which several teeth were found. Dermoids are found in the palate and pharynx, and open dermoids of the conjunctiva are classified by Sutton with the moles. According to Senn, Barker collected sixteen dermoid tumors of the tongue. Bryk successfully removed a tumor of this nature the size of a fist. Wellington Gray removed an enormous lingual dermoid from the mouth of a negro. It contained 40 ounces of atheromatous material. Dermoids of the rectum are reported. Duyse reports the history of a case of labor during which a rectal dermoid was expelled. The dermoid contained a cerebral vesicle, a rudimentary eye, a canine and a molar tooth, and a piece of bone. There is little doubt that many cases of fetus in fetu reported were really dermoids of the scrotum.
Ward reports the successful removal of a dermoid cyst weighing 30 pounds from a woman of thirty-two, the mother of two children aged ten and twelve, respectively. The report is briefly as follows: "The patient has always been in good health until within the last year, during which time she has lost flesh and strength quite rapidly, and when brought to my hospital by her physician, Dr. James of Williamsburg, Kansas, was quite weak, although able to walk about the house. A tumor had been growing for a number of years, but its growth was so gradual that the patient had not considered her condition critical until quite recently. The tumor was diagnosed to be cystoma of the left ovary. Upon opening the sac with the trocar we were confronted by complications entirely unlooked for, and its use had to be abandoned entirely because the thick contents of the cyst would not flow freely, and the presence of sebaceous matter blocked the instrument. As much of the fluid as possible was removed, and the abdominal incision was enlarged to allow of the removal of the large tumor. An ovarian hematoma the size of a large orange was removed from the right side. We washed the intestines quite as one would wash linen, since some of the contents of the cyst had escaped into the abdominal cavity. The abdomen was closed without drainage, and the patient placed in bed without experiencing the least shock. Her recovery was rapid and uneventful. She returned to her home in four weeks after the operation.
"The unusual feature in this case was the nature of the contents of the sac. There was a large quantity of long straight hair growing from the cyst wall and an equal amount of loose hair in short pieces floating through the tumor-contents, a portion of which formed nuclei for what were called 'moth-balls,' of which there were about 1 1/2 gallons. These balls, or marbles, varied from the size of moth-balls, as manufactured and sold by druggists, to that of small walnuts. They seemed to be composed of sebaceous matter, and were evidently formed around the short hairs by the motion of the fluid produced by walking or riding. There was some tissue resembling true skin attached to the inner wall of the sac."
There are several cases of multiple dermoid cysts on record, and they may occur all over the body. Jamieson reports a case in which there were 250, and in Maclaren's case there were 132. According to Crocker, Hebra and Rayer also each had a case. In a case of Sangster, reported by Politzer, although most of the dermoids, as usual, were like fibroma-nodules and therefore the color of normal skin, those over the mastoid processes and clavicle were lemon-yellow, and were generally thought to be xanthoma until they were excised, and Politzer found they were typical dermoid cysts with the usual contents of degenerated epithelium and hair.
Hermaphroditism.--Some writers claim that Adam was the first hermaphrodite and support this by Scriptural evidence. We find in some of the ancient poets traces of an Egyptian legend in which the goddess of the moon was considered to be both male and female. From mythology we learn that Hermaphroditus was the son of Hermes, or Mercury, and Venus Aphrodite, and had the powers both of a father and mother. In speaking of the foregoing Ausonius writes, "Cujus erat facies in qua paterque materque cognosci possint, nomen traxit ab illis." Ovid and Virgil both refer to legendary hermaphrodites, and the knowledge of their existence was prevalent in the olden times. The ancients considered the birth of hermaphrodites bad omens, and the Athenians threw them into the sea, the Romans, into the Tiber. Livy speaks of an hermaphrodite being put to death in Umbria, and another in Etruria. Cicero, Aristotle, Strabonius, and Pliny all speak concerning this subject. Martial and Tertullian noticed this anomaly among the Romans. Aetius and Paulus Aegineta speak of females in Egypt with prolonged clitorides which made them appear like hermaphrodites. Throughout the Middle Ages we frequently find accounts, naturally exaggerated, of double-sexed creatures. Harvey, Bartholinus, Paullini, Schenck, Wolff, Wrisberg, Zacchias, Marcellus Donatus, Haller, Hufeland, de Graff, and many others discuss hermaphroditism. Many classifications have been given, as, e.g., real and apparent; masculine, feminine, or neuter; horizontal and vertical; unilateral and bilateral, etc. The anomaly in most cases consists of a malformation of the external genitalia. A prolonged clitoris, prolapsed ovaries, grossness of figure, and hirsute appearance have been accountable for many supposed instances of hermaphrodites. On the other hand, a cleft scrotum, an ill-developed penis, perhaps hypospadias or epispadias, rotundity of the mammae, and feminine contour have also provoked accounts of similar instances. Some cases have been proved by dissection to have been true hermaphrodites, portions or even entire genitalia of both sexes having been found.
Numerous accounts, many mythical, but always interesting, are given of these curious persons. They have been accredited with having performed the functions of both father and mother, notwithstanding the statements of some of the best authorities that they are always sterile. Observation has shown that the sexual appetite diminishes in proportion to the imperfections in the genitalia, and certainly many of these persons are sexually indifferent.
We give descriptions of a few of the most famous or interesting instances of hermaphroditism. Pare speaks of a woman who, besides a vulva, from which she menstruated, had a penis, but without prepuce or signs of erectility. Haller alludes to several cases in which prolonged clitorides have been the cause of the anomaly. In commenting on this form of hermaphroditism Albucasiusus describes a necessary operation for the removal of the clitoris.
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Anomalies and Curiosities of MedicineChapter V: Major Terata (2)
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