Anomalies And Curiosities Of Medicine Part 22

The ureters sometimes have anomalous terminations either in the r.e.c.t.u.m, v.a.g.i.n.a, or directly in the urethra. This latter disposition is realized normally in a number of animals and causes the incessant flow of urine, resulting in a serious inconvenience. Flajani speaks of the termination of the ureters in the pelvis; Nebel has seen them appear just beneath the umbilicus; and Lieutaud describes a man who died at thirty-five, from another cause, whose ureters, as large as intestines, terminated in the urethral ca.n.a.l, causing him to urinate frequently; the bladder was absent. In the early part of this century there was a young girl examined in New York whose ureters emptied into a reddish carnosity on the mons veneris. The urine dribbled continuously, and if the child cried or made any exertion it came in jets. The genital organs partic.i.p.ated but little in the deformity, and with the exception that the umbilicus was low and the a.n.u.s more anterior than natural, the child was well formed and its health good. Colzi reports a case in which the left ureter opened externally at the left side of the hymen a little below the normal meatus urinarius. There is a case described of a man who evidently suffered from a patent urachus, as the urine pa.s.sed in jets as if controlled by a sphincter from his umbilicus. Littre mentions a patent urachus in a boy of eighteen. Congenital dilatation of the ureters is occasionally seen in the new-born. Shattuck describes a male fetus showing reptilian characters in the s.e.xual ducts. There was ectopia vesicae and prolapse of the intestine at the umbilicus; the right kidney was elongated; the right vas deferens opened into the ureter. There was persistence in a separate condition of the two Mullerian ducts which opened externally inferiorly, and there were two ducts near the openings which represented a.n.a.l pouches. Both t.e.s.t.i.c.l.es were in the abdomen. Ord describes a man in whom one of the Mullerian ducts was persistent.

Anomalies of the Bladder.--Blanchard, Blasius, Haller, Nebel, and Rhodius mention cases in which the bladder has been found absent and we have already mentioned some cases, but the instances in which the bladder has been duplex are much more frequent. Bourienne, Oberteuffer, Ruysch, Bartholinus, Morgagni, and Franck speak of vesical duplication. There is a description of a man who had two bladders, each receiving a ureter. Bussiere describes a triple bladder, and Scibelli of Naples mentions an instance in a subject who died at fifty-seven with symptoms of retention of urine. In the ill.u.s.tration, B represents the normal bladder, A and C the supplementary bladders, with D and E their respective points of entrance into B. As will be noticed, the ureters terminate in the supplementary bladders. Fantoni and Malgetti cite instances of quintuple bladders.

The Ephemerides speaks of a case of coalition of the bladder with the os pubis and another case of coalition with the omentum. Prochaska mentions vesical fusion with the uterus, and we have already described union with the r.e.c.t.u.m and intestine.

Exstrophy of the bladder is not rare, and is often a.s.sociated with hypospadias, epispadias, and other malformations of the genitourinary tract. It consists of a deficiency of the abdominal wall in the hypogastric region, in which is seen the denuded bladder. It is remedied by many different and ingenious plastic operations.

In an occasional instance in which there is occlusion at the umbilicus and again at the neck of the bladder this organ becomes so distended as to produce a most curious deformity in the fetus. Figure 143 shows such a case.

The Heart.--Absence of the heart has never been recorded in human beings except in the case of monsters, as, for example, the omphalosites, although there was a case reported and firmly believed by the ancient authors,--a Roman soldier in whom Telasius said he could discover no vestige of a heart.

The absence of one ventricle has been recorded. Schenck has seen the left ventricle deficient, and the Ephemerides, Behr, and Kerckring speak of a single ventricle only in the heart. Riolan mentions a heart in which both ventricles were absent. Jurgens reported in Berlin, February 1, 1882, an autopsy on a child who had lived some days after birth, in which the left ventricle of the heart was found completely absent. Playfair showed the heart of a child which had lived nine months in which one ventricle was absent. In King's College Hospital in London there is a heart of a boy of thirteen in which the cavities consist of a single ventricle and a single auricle.

Duplication of the heart, notwithstanding the number of cases reported, has been admitted with the greatest reserve by Geoffroy-Saint-Hilaire and by a number of authors. Among the celebrated anatomists who describe duplex heart are Littre, Meckel, Collomb, Panum, Behr, Paullini, Rhodins, Winslow, and Zacutus Lusita.n.u.s.

The Ephemerides cites an instance of triple heart, and Johnston has seen a triple heart in a goose.

The phenomenon of "blue-disease," or congenital cyanosis, is due to the patency of the foremen ovale, which, instead of closing at birth, persists sometimes to adult life.

Perhaps the most unique collection of congenital malformations of the heart from persons who have reached the age of p.u.b.erty was to be seen in London in 1895. In this collection there was an adult heart in which the foremen ovale remained open until the age of thirty-seven; there were but two pulmonary valves; there was another heart showing a large patent foramen ovale from a man of forty-six; and there was a septum ventriculorum of an adult heart from a woman of sixty-three, who died of carcinoma of the breast, in which the foremen ovale was still open and would admit the fore-finger. This woman had shown no symptoms of the malformation. There were also hearts in which the interventricular septum was deficient, the ductus arteriosus patent, or some valvular malformation present. All these persons had reached p.u.b.erty.

Displacements of the heart are quite numerous. Deschamps of Laval made an autopsy on an old soldier which justified the expression, "He had a heart in his belly." This organ was found in the left lumbar region; it had, with its vessels, traversed an anomalous opening in the diaphragm.

Franck observed in the Hospital of Colmar a woman with the heart in the epigastric region. Ramel and Vetter speak of the heart under the diaphragm.

Inversion of the heart is quite frequent, and we often find reports of cases of this anomaly. Fournier describes a soldier of thirty years, of middle height, well proportioned and healthy, who was killed in a duel by receiving a wound in the abdomen; postmortem, the heart was found in the position of the right lung; the two lungs were joined and occupied the left chest.

The anomalies of the vascular system are so numerous that we shall dismiss them with a slight mention. Malacarne in Torino in 1784 described a double aorta, and Hommelius mentions an a.n.a.logous case. The following case is quite an interesting anatomic anomaly: A woman since infancy had difficulty in swallowing, which was augmented at the epoch of menstruation and after exercise; bleeding relieved her momentarily, but the difficulty always returned. At last deglut.i.tion became impossible and the patient died of malnutrition. A necropsy revealed the presence of the subclavicular artery pa.s.sing between the tracheal artery and the esophagus, compressing this latter tube and opposing the pa.s.sage of food.

Anomalies of the b.r.e.a.s.t.s.--The first of the anomalies of the generative apparatus to be discussed, although not distinctly belonging under this head, will be those of the mammae.

Amazia, or complete absence of the breast, is seldom seen. Pilcher describes an individual who pa.s.sed for a female, but who was really a male, in whom the b.r.e.a.s.t.s were absolutely wanting. Foerster, Froriep, and Ried cite instances a.s.sociated with thoracic malformation. Greenhow reports a case in which the mammae were absent, although there were depressed rudimentary nipples and areolae. There were no ovaries and the uterus was congenitally imperfect.

There was a negress spoken of in 1842 in whom the right breast was missing, and there are cases of but one breast, mentioned by King, Paull, and others. Scanzoni has observed absence of the left mamma with absence of the left ovary.

Micromazia is not so rare, and is generally seen in females with a.s.sociate genital troubles. Excessive development of the mammae, generally being a pathologic phenomenon, will be mentioned in another chapter. However, among some of the indigenous negroes the female b.r.e.a.s.t.s are naturally very large and pendulous. This is well shown in Figure 144, which represents a woman of the Bushman tribe nursing an infant. The b.r.e.a.s.t.s are sufficiently pendulous and loose to be easily thrown over the shoulder.

Polymazia is of much more frequent occurrence than is supposed. Julia, the mother of Alexander Severus, was surnamed "Mammea" because she had supernumerary b.r.e.a.s.t.s. Anne Boleyn, the unfortunate wife of Henry VIII of England, was reputed to have had six toes, six fingers, and three b.r.e.a.s.t.s. Lynceus says that in his time there existed a Roman woman with four mammae, very beautiful in contour, arranged in two lines, regularly, one above the other, and all giving milk in abundance.

Rubens has pictured a woman with four b.r.e.a.s.t.s; the painting may be seen in the Louvre in Paris.

There was a young and wealthy heiress who addressed herself to the ancient faculty at Tubingen, asking, as she displayed four mammary, whether, should she marry, she would have three or four children at a birth. This was a belief with which some of her elder matron friends had inspired her, and which she held as a hindrance to marriage.

Leichtenstern, who has collected 70 cases of polymazia in females and 22 in males, thinks that accessory b.r.e.a.s.t.s or nipples are due to atavism, and that our most remote inferiorly organized ancestors had many b.r.e.a.s.t.s, but that by constantly bearing but one child, from being polymastic, females have gradually become bimastic. Some of the older philosophers contended that by the presence of two b.r.e.a.s.t.s woman was originally intended to bear two children.

Hirst says: "Supernumerary b.r.e.a.s.t.s and nipples are more common than is generally supposed. Bruce found 60 instances in 3956 persons examined (1.56 per cent). Leichtenstern places the frequency at one in 500. Both observers declare that men present the anomaly about twice as frequently as women. It is impossible to account for the accessory glands on the theory of reversion, as they occur with no regularity in situation, but may develop at odd places on the body. The most frequent position is on the pectoral surface below the true mammae and somewhat nearer the middle line, but an accessory gland has been observed on the left shoulder over the prominence of the deltoid, on the abdominal surface below the costal cartilages, above the umbilicus, in the axilla, in the groin, on the dorsal surface, on the labium majus, and on the outer aspect of the left thigh. Ahlfeld explains the presence of mammae on odd parts of the body by the theory that portions of the embryonal material entering into the composition of the mammary gland are carried to and implanted upon any portion of the exterior of the body by means of the amnion."

Possibly the greatest number of accessory mammae reported is that of Neugebauer in 1886, who found ten in one person. Peuch in 1876 collected 77 cases, and since then Hamy, Quinqusud, Whiteford, Engstrom, and Mitch.e.l.l Bruce have collected cases. Polymazia must have been known in the olden times, and we still have before us the old images of Diana, in which this G.o.ddess is portrayed with numerous b.r.e.a.s.t.s, indicating her ability to look after the growing child. Figure 145 shows an ancient Oriental statue of Artemisia or Diana now at Naples.

Bartholinus has observed a Danish woman with three mammae, two ordinarily formed and a third forming a triangle with the others and resembling the b.r.e.a.s.t.s of a fat man. In the village of Phullendorf in Germany early in this century there was an old woman who sought alms from place to place, exhibiting to the curious four symmetrical b.r.e.a.s.t.s, arranged parallel. She was extremely ugly, and when on all fours, with her b.r.e.a.s.t.s pendulous, she resembled a beast. The authors have seen a man with six distinct nipples, arranged as regularly as those of a b.i.t.c.h or sow. The two lower were quite small. This man's body was covered with heavy, long hair, making him a very conspicuous object when seen naked during bathing. The hair was absent for a s.p.a.ce of nearly an inch about the nipples. Borellus speaks of a woman with three mammae, two as ordinarily, the third to the left side, which gave milk, but not the same quant.i.ty as the others. Gardiner describes a mulatto woman who had four mammae, two of which were near the axillae, about four inches in circ.u.mference, with proportionate sized nipples.

She became a mother at fourteen, and gave milk from all her b.r.e.a.s.t.s. In his "Dictionnaire Philosophique" Voltaire gives the history of a woman with four well-formed and symmetrically arranged b.r.e.a.s.t.s; she also exhibited an excrescence, covered with a nap-like hair, looking like a cow-tail. Percy thought the excrescence a prolongation of the coccyx, and said that similar instances were seen in savage men of Borneo.

Percy says that among some prisoners taken in Austria was found a woman of Valachia, near Roumania, exceedingly fatigued, and suffering intensely from the cold. It was January, and the ground was covered with three feet of snow. She had been exposed with her two infants, who had been born twenty days, to this freezing temperature, and died on the next day. An examination of her body revealed five mammae, of which four projected as ordinarily, while the fifth was about the size of that of a girl at p.u.b.erty.

They all had an intense dark ring about them; the fifth was situated about five inches above the umbilicus. Percy injected the subject and dissected and described the mammary blood-supply. Hirst mentions a negress of nineteen who had nine mammae, all told, and as many nipples.

The two normal glands were very large. Two accessory glands and nipples below them were small and did not excrete milk. All the other glands and nipples gave milk in large quant.i.ties. There were five nipples on the left and four on the right side. The patient's mother had an accessory mamma on the abdomen that secreted milk during the period of lactation.

Charpentier has observed in his clinic a woman with two supplementary axillary mammae with nipples. They gave milk as the ordinary mammae.

Robert saw a woman who nourished an infant by a mamma on the thigh.

Until the time of pregnancy this mamma was taken for an ordinary nevus, but with pregnancy it began to develop and acquired the size of a citron. Figure 147 is from an old wood-cut showing a child suckling at a supernumerary mamma on its mother's thigh while its brother is at the natural breast. Jenner speaks of a breast on the outer side of the thigh four inches below the great trochanter. Hare describes a woman of thirty-seven who secreted normal milk from her axillae. Lee mentions a woman of thirty-five with four mammae and four nipples; she suckled with the pectoral and not the axillary b.r.e.a.s.t.s. McGillicudy describes a pair of rudimentary abdominal mammae, and there is another similar case recorded. Hartung mentions a woman of thirty who while suckling had a mamma on the left labium majus. It was excised, and microscopic examination showed its structure to be that of a rudimentary nipple and mammary gland. Leichtenstern cites a case of a mamma on the left shoulder nearly under the insertion of the deltoid, and Klob speaks of an acromial accessory mamma situated on the shoulder over the greatest prominence of the deltoid. Hall reports the case of a functionally active supernumerary mamma over the costal cartilage of the 8th rib.

Jussieu speaks of a woman who had three b.r.e.a.s.t.s, one of which was situated on the groin and with which she occasionally suckled; her mother had three b.r.e.a.s.t.s, but they were all situated on the chest.

Saunois details an account of a female who had two supernumerary b.r.e.a.s.t.s on the back. Bartholinus (quoted by Meckel) and Manget also mention mammae on the back, but Geoffroy-Saint-Hilaire questions their existence. Martin gives a very clear ill.u.s.tration of a woman with a supernumerary breast below the natural organ. Sneddon, who has collected quite a number of cases of polymazia, quotes the case of a woman who had two swellings in each axilla in which gland-structure was made out, but with no external openings, and which had no anatomic connection with the mammary glands proper. Shortly after birth they varied in size and proportion, as the b.r.e.a.s.t.s were full or empty, and in five weeks all traces of them were lost. Her only married sister had similar enlargements at her third confinement.

Polymazia sometimes seems to be hereditary. Robert saw a daughter whose mother was polymastic, and Woodman saw a mother and eldest daughter who each had three nipples. Lousier mentions a woman wanting a mamma who transmitted this vice of conformation to her daughter. Handyside says he knew two brothers in both of whom b.r.e.a.s.t.s were wanting.

Supernumerary nipples alone are also seen, as many as five having been found on the same breast. Neugebauer reports eight supernumerary nipples in one case. Hollerus has seen a woman who had two nipples on the same breast which gave milk with the same regularity and the same abundance as the single nipple. The Ephemerides contains a description of a triple nipple. Barth describes "mamma erratica" on the face in front of the right ear which enlarged during menstruation.

Cases of deficiency of the nipples have been reported by the Ephemerides, Lentilius, Severinus, and Werckardus.

Cases of functional male mammae will be discussed in Chapter IX.

Complete absence of the hymen is very rare, if we may accept the statements of Devilliers, Tardieu, and Brouardel, as they have never seen an example in the numerous young girls they have examined from a medico-legal point of view.

Duplication or biperforation of the hymen is also a very rare anomaly of this membrane. In this instance the hymen generally presents two lateral orifices, more or less irregular and separated by a membranous band, which gives the appearance of duplicity. Roze reported from Strasburg in 1866 a case of this kind, and Delens has observed two examples of biperforate hymen, which show very well that this disposition of the membrane is due to a vice of conformation. The first was in a girl of eleven, in which the membrane was of the usual size and thickness, but was duplicated on either side. In her sister of nine the hymen was normally conformed. The second case was in a girl under treatment by Cornil in 1876 for vaginitis. Her brother had accused a young man of eighteen of having violated her, and on examination the hymen showed a biperforate conformation; there were two oval orifices, their greatest diameter being in the vertical plane; the openings were situated on each side of the median line, about five mm. apart; the dividing band did not appear to be cicatricial, but presented the same roseate coloration as the rest of the hymen. Since this report quite a number of cases have been recorded.

The different varieties of the hymen will be left to the works on obstetrics. As has already been observed, labor is frequently seriously complicated by a persistent and tough hymen.

Deficient v.u.l.v.a may be caused by the persistence of a thick hymen, by congenital occlusion, or by absolute absence in v.u.l.v.ar structure.

Bartholinus, Borellus, Ephemerides, Julius, Vallisneri, and Baux are among the older writers who mention this anomaly, but as it is generally a.s.sociated with congenital occlusion, or complete absence of the v.a.g.i.n.a, the two will be considered together.

Complete absence of the v.a.g.i.n.a is quite rare. Baux a reports a case of a girl of fourteen in whom "there was no trace of fundament or of genital organs." Oberteuffer speaks of a case of absent v.a.g.i.n.a. Vicq d'Azir is accredited with having seen two females who, not having a v.a.g.i.n.a, copulated all through life by the urethra, and Fournier sagely remarks that the extra large urethra may have been a special dispensation of nature. Bosquet describes a young girl of twenty with a triple vice of conformation--an obliterated v.u.l.v.a, closure of the v.a.g.i.n.a, and absence of the uterus. Menstrual hemorrhage took place from the gums. Clarke has studied a similar case which was authenticated by an autopsy.

O'Ferral of Dublin, Gooch, Davies, Boyd, Tyler Smith, Hanc.o.c.k, Coste, Klayskens, Debrou, Braid, Watson, and others are quoted by Churchill as having mentioned the absence of the v.a.g.i.n.a. Amussat observed a German girl who did not have a trace of a v.a.g.i.n.a and who menstruated regularly. Griffith describes a specimen in the Museum of St.

Bartholomew's Hospital, London, in which the ovaries lay on the surface of the pelvic peritoneum and there was neither uterus nor v.a.g.i.n.a; the pelvis had some of the characteristics of the male type. Matthews Duncan has observed a somewhat similar case, the v.a.g.i.n.a not measuring more than an inch in length. Ferguson describes a prost.i.tute of eighteen who had never menstruated. The l.a.b.i.a were found well developed, but there was no v.a.g.i.n.a, uterus, or ovaries. Coitus had been through the urethra, which was considerably distended, though not causing incontinence of urine. Hulke reports a case of congenital atresia of the v.a.g.i.n.a in a brunette of twenty, menstruation occurring through the urethra. He also mentions the instance of congenital atresia of the v.a.g.i.n.a with hernia of both ovaries into the left groin in a servant of twenty, and the case of an imperforate v.a.g.i.n.a in a girl of nineteen with an undeveloped uterus.

Brodhurst reports an instance of absence of the v.a.g.i.n.a and uterus in a girl of sixteen who at four years of age showed signs of approaching p.u.b.erty. At this early age the mons was covered with hair, and at ten the c.l.i.toris was three inches long and two inches in circ.u.mference. The mammae were well developed. The l.a.b.i.a descended laterally and expanded into folds, resembling the s.c.r.o.t.u.m.

Azema reports an instance of complete absence of the v.a.g.i.n.a and impermeability and probable absence of the col uterinus. The deficiencies were remedied by operation. Berard mentions a similar deformity and operation in a girl of eighteen. Gooding cites an instance of absent v.a.g.i.n.a in a married woman, the uterus discharging the functions. Gosselin reports a case in which a voluminous tumor was formed by the retained menstrual fluid in a woman without a v.a.g.i.n.a. An artificial v.a.g.i.n.a was created, but the patient died from extravasation of blood into the peritoneal cavity. Carter, Polaillon, Martin, Curtis, Worthington, Hall, Hicks, Moliere, Patry, Dolbeau, Desormeaux, and Gratigny also record instances of absence of the v.a.g.i.n.a.

There are some cases reported in extramedical literature which might be cited. Bussy Rabutin in his Memoires in 1639 speaks of an instance. The celebrated Madame Recamier was called by the younger Dumas an involuntary virgin; and in this connection could be cited the malicious and piquant sonnet--

Chateaubriand et Madame Recamier.

"Juliette et Rene s'aimaient d'amour si tendre Que Dien, sans les punir, a pu leur pardonner: Il n'avait pas voulu que l'une put donner Ce que l'autre ne pouvait prendre."

Duplex v.a.g.i.n.a has been observed by Bartholinus, Malacarne, Asch, Meckel, Osiander, Purcell, and other older writers. In more modern times reports of this anomaly are quite frequent. Hunter reports a case of labor at the seventh month in a woman with a double v.a.g.i.n.a, and delivery through the r.e.c.t.u.m. Atthill and Watts speak of double v.a.g.i.n.a with single uterus.

Robb of Johns Hopkins Hospital reports a case of double v.a.g.i.n.a in a patient of twenty suffering from dyspareunia. The v.a.g.i.n.al orifice was contracted; the urethra was dilated and had evidently been used for coitus. A membrane divided the v.a.g.i.n.a into two ca.n.a.ls, the cervix lying in the right half; the septum was also divided. Both the thumbs of the patient were so short that their tips could scarcely meet those of the little fingers. Double v.a.g.i.n.a is also reported by Anway, Moulton, Freeman, Frazer, Haynes, Lemaistre, Boardman, d.i.c.kson, Dunoyer, and Rossignol. This anomaly is usually a.s.sociated with bipart.i.te or double uterus. Wilc.o.x mentions a primipara, three months pregnant, with a double v.a.g.i.n.a and a bicornate uterus, who was safely delivered of several children. Haller and Borellus have seen double v.a.g.i.n.a, double uterus, and double ovarian supply; in the latter case there was also a double v.u.l.v.a. Sanger speaks of a supernumerary v.a.g.i.n.a connecting with the other v.a.g.i.n.a by a fistulous opening, and remarks that this was not a case of patent Gartner's duct.

Cullingworth cites two cases in which there were transverse septa of the v.a.g.i.n.a. Stone reports five cases of transverse septa of the v.a.g.i.n.a.