Anomalies And Curiosities Of Medicine Part 23

Three of the patients were young women who had never borne children or suffered injury. Pregnancy existed in each case. In the first the septum was about two inches from the introitus, and contained an opening about 1/2 inch in diameter which admitted the tip of the finger. The membrane was elastic and thin and showed no signs of inflammation. Menstruation had always been regular up to the time of pregnancy. The second was a duplicate of the first, excepting that a few bands extended from the cervix to the membranous septum. In the third the lumen of the v.a.g.i.n.a, about two inches from the introitus, was distinctly narrowed by a ridge of tissue. There was uterine displacement and some endocervicitis, but no history of injury or operation and no tendency to contraction. The two remaining cases occurred in patients seen by Dr. J. F. Scott. In one the septum was about 1 3/4 inches from the entrance to the v.a.g.i.n.a and contained an orifice large enough to admit a uterine probe. During labor the septum resisted the advance of the head for several hours, until it was slit in several directions. In the other, menstruation had always been irregular, intermissions being followed by a profuse flow of black and tarry blood, which lasted sometimes for fifteen days and was accompanied by severe pain. The septum was 1 1/2 inches from the v.a.g.i.n.al orifice and contained an opening which admitted a uterine sound. It was very dense and tight and fully 1/8 inch in thickness.

Mordie reported a case of congenital deficiency of the rectov.a.g.i.n.al septum which was successfully remedied by operation.

Anomalous Openings of the v.a.g.i.n.a.--The v.a.g.i.n.a occasionally opens abnormally into the r.e.c.t.u.m, into the bladder, the urethra, or upon the abdominal parietes. Rossi reports from a hospital in Turin the case of a Piedmontese girl in whom there was an enormous tumor corresponding to the opening of the v.a.g.i.n.al orifice; no traces of a v.a.g.i.n.a could be found. The tumor was incised and proved to be a living infant. The husband of the woman said that he had coitus without difficulty by the r.e.c.t.u.m, and examination showed that the v.a.g.i.n.a opened into the r.e.c.t.u.m, by which means impregnation had been accomplished. Bonnain and Payne have observed a.n.a.logous cases of this abnormality of the v.a.g.i.n.al opening and subsequent accouchement by the a.n.u.s. Payne's case was of a woman of thirty-five, well formed, who had been in labor thirty-six hours, when the physician examined and looked in vain for a v.a.g.i.n.al opening; the finger, gliding along the perineum, came in contact with the distended a.n.u.s, in which was recognized the head of the fetus. The woman from prolongation of labor was in a complete state of prostration, which caused uterine inertia. Payne anesthetized the patient, applied the forceps, and extracted the fetus without further accident. The v.u.l.v.a of this woman five months afterward displayed all the characteristics of virginity, the v.a.g.i.n.a opened into the r.e.c.t.u.m, and menstruation had always been regular. This woman, as well as her husband, averred that they had no suspicion of the anomaly and that coitus (by the a.n.u.s) had always been satisfactory.

Opening of the v.a.g.i.n.a upon the parietes, of which Le Fort has collected a number of cases, has never been observed in connection with a viable fetus.

Absence of the l.a.b.i.a majora has been observed, especially by Pozzi, to the exclusion of all other anomalies. It is the rule in exstrophy of the bladder.

Absence of the nymphae has also been observed, particularly by Auvard and by Perchaux, and is generally a.s.sociated with imperfect development of the c.l.i.toris. Constantinedes reports absence of the external organs of generation, probably also of the uterus and its appendages, in a young lady. Van Haartman, LeFort, Magee, and Ogle cite cases of absence of the external female organs. Riolan in the early part of the seventeenth century reported a case of defective nymphae; Neubauer in 1774 offers a contrast to this case in an instance of triple nymphae.

The nymphae are sometimes enormously enlarged by hypertrophy, by varicocele, or by elephantiasis, of which latter type Rigal de Gaillac has observed a most curious case. There is also a variety of enlargement of the c.l.i.toris which seems to be constant in some races; it may be a natural hypertrophy, or perhaps produced by artificial manipulation.

The peculiar conditions under which the Chinese women are obliged to live, particularly their mode of sitting, is said to have the effect of causing unusual development of the mons veneris and the l.a.b.i.a majora.

On the other hand, some of the lower African races have been distinguished by the deficiency in development of the l.a.b.i.a majora, mons veneris, and genital hair. In this respect they present an approximation to the genitals of the anthropoid apes, among whom the orang-outang alone shows any tendency to formation of the l.a.b.i.a majora.

The l.a.b.i.al appendages of the Hottentot female have been celebrated for many years. Blumenbach and others of the earlier travelers found that the ap.r.o.n-like appearance of the genitals of the Hottentot women was due to abnormal hypertrophy of the l.a.b.i.a and nymphae. According to John Knott, the French traveler, Le Vaillant, said that the more coquettish among the Hottentot girls are excited by extreme vanity to practice artificial elongation of the nympha and l.a.b.i.a. They are said to pull and rub these parts, and even to stretch them by hanging weights to them. Some of them are said to spend several hours a day at this process, which is considered one of the important parts of the toilet of the Hottentot belle, this malformation being an attraction for the male members of the race. Merensky says that in Basutoland the elder women begin to practice l.a.b.i.al manipulation on their female children shortly after infancy, and Adams has found this custom to prevail in Dahomey; he says that the King's seraglio includes 3000 members, the elect of his female subjects, all of whom have l.a.b.i.a up to the standard of recognized length. Cameron found an a.n.a.logous practice among the women of the sh.o.r.es of Lake Tanganyika. The females of this nation manipulated the skin of the lower part of the abdomens of the female children from infancy, and at p.u.b.erty these women exhibit a cutaneous curtain over the genitals which reaches half-way down the thighs.

A corresponding development of the preputian c.l.i.torides, attaining the length of 18 mm. or even more, has been observed among the females of Bechua.n.a.land. The greatest elongation measured by Barrow was five inches, but it is quite probable that it was not possible for him to examine the longest, as the females so gifted generally occupied very high social positions.

Morgagni describes a supernumerary left nympha, and Pet.i.t is accredited with seeing a case which exhibited neither nymphae, c.l.i.toris, nor urinary meatus. Mauriceau performed nymphotomy on a woman whose nymphae were so long as to render coitus difficult. Morand quotes a case of congenital malformation of the nymphae, to which he attributed impotency.

There is sometimes coalition of the l.a.b.i.a and nymphae, which may be so firm and extensive as to obliterate the v.u.l.v.a. Debout has reported a case of absence of the v.u.l.v.a in a woman of twenty upon whom he operated, which was the result of the fusion of the l.a.b.i.a minora, and this with an enlarged c.l.i.toris gave the external appearance of an hermaphrodite.

The absence of the c.l.i.toris coincides with epispadias in the male, and in atrophy of the v.u.l.v.a it is common to find the c.l.i.toris rudimentary; but a more frequent anomaly is hypertrophy of the c.l.i.toris.

Among the older authorities quoting instances of enlarged c.l.i.torides are Bartholinus, Schenck, h.e.l.lwig, Rhodius, Riola.n.u.s, and Zacchias.

Albucasis describes an operation for enlarged c.l.i.toris, Chabert ligated one, and Riedlin gives an instance of an enlarged c.l.i.toris, in which there appeared a tumor synchronous with the menstrual epoch.

We learn from the cla.s.sics that there were certain females inhabiting the borders of the Aegean Sea who had a sentimental attachment for one another which was called "Lesbian love," and which carried them to the highest degree of frenzy. The immortal effusions of Sappho contain references to this pa.s.sion. The solution of this peculiar ardor is found in the fact that some of the females had enlarged c.l.i.torides, strong voices, robust figures, and imitated men. Their manner was imperative and authoritative to their s.e.x, who worshiped them with perverted devotion. We find in Martial mention of this perverted love, and in the time of the dissolute Greeks and Romans ridiculous jealousies for unfaithfulness between these women prevailed. Aetius said that the Egyptians practiced amputation of the c.l.i.toris, so that enlargement of this organ must have been a common vice of conformation along the Nile. It was also said that the Egyptian women practiced circ.u.mcision on their females at the age of seven or eight, the time chosen being when the Nile was in flood. Bertherand cites examples of enlarged c.l.i.torides in Arab women; Bruce testifies to this circ.u.mstance in Abyssinia, and Mungo Park has observed it in the Mandingos and the Ibbos.

Sonnini says that the women of Egypt had a natural excrescence, fleshy in consistency, quite thick and pendulous, coming from the skin of the mons veneris. Sonnini says that in a girl of eight he saw one of these caruncles which was 1/2 inch long, and another on a woman of twenty which was four inches long, and remarks that they seem peculiar only to women of distinct Egyptian origin.

Duhouset says that in circ.u.mcision the Egyptian women not only remove a great part of the body of the c.l.i.toris with the prepuce, but also adjacent portions of the nymphae; Gallieni found a similar operation customary on the upper banks of the Niger.

Otto at Breslau in 1824 reports seeing a negress with a c.l.i.toris 4 1/2 inches long and 1 1/2 inches in the transverse diameter; it projected from the v.u.l.v.a and when supine formed a complete covering for the v.a.g.i.n.al orifice. The c.l.i.toris may at times become so large as to prevent coitus, and in France has const.i.tuted a legitimate cause for divorce. This organ is very sensitive, and it is said that in cases of supposed catalepsy a woman cannot bear t.i.tillation of the c.l.i.toris without some visible movement.

Columbus cites an example of a c.l.i.toris as long as a little finger; Haller mentions one which measured seven inches, and there is a record of an enlarged c.l.i.toris which resembled the neck of a goose and which was 12 inches long. Bainbridge reports a case of enlarged c.l.i.toris in a woman of thirty-two who was confined with her first child. This organ was five inches in length and of about the diameter of a quiescent p.e.n.i.s. Figure 149 shows a well-marked case of hypertrophy of the c.l.i.toris. Rogers describes a woman of twenty-five in a reduced state of health with an enormous c.l.i.toris and warts about the a.n.u.s; there were also manifestations of tuberculosis. On questioning her, it was found that she had formerly m.a.s.t.u.r.b.a.t.ed; later she had s.e.xual intercourse several times with a young man, but after his death she commenced self-abuse again, which brought on the present enlargement. The c.l.i.toris was ligated and came away without leaving disfigurement.

Ca.s.sano and Pedretti of Naples reported an instance of monstrous c.l.i.toris in 1860 before the Academy of Medicine.

In some cases ossification of the c.l.i.toris is observed Fournier speaks of a public woman in Venice who had an osseous c.l.i.toris; it was said that men having connection with her invariably suffered great pain, followed by inflammation of the p.e.n.i.s.

There are a few instances recorded of bifid c.l.i.toris, and Arnaud cites the history of a woman who had a double c.l.i.toris. Secretain speaks of a c.l.i.toris which was in a permanent state of erection.

Complete absence of the ovaries is seldom seen, but there are instances in which one of the ovaries is missing. Hunter, Vidal, and Chaussier report in full cases of the absence of the ovaries, and Thudic.u.m has collected 21 cases of this nature. Morgagni, Pears, and Cripps have published observations in which both ovaries were said to have been absent. Cripps speaks of a young girl of eighteen who had an infantile uterus and no ovaries; she neither menstruated nor had any signs of p.u.b.erty. Lauth cites the case of a woman whose ovaries and uterus were rudimentary, and who exhibited none of the princ.i.p.al physiologic characteristics of her s.e.x; on the other hand, Ruband describes a woman with only rudimentary ovaries who was very pa.s.sionate and quite feminine in her aspect.

At one time the existence of genuine supernumerary ovaries was vigorously disputed, and the older records contain no instances, but since the researches of Beigel, Puech, Thudic.u.m, Winckler, de Sinety, and Paladino the presence of multiple ovaries is an incontestable fact.

It was originally thought that supernumerary ovaries as well as supernumerary kidneys were simply segmentations of the normal organs and connected to them by portions of the proper substance; now, however, by the recent reports we are warranted in admitting these anomalous structures as distinct organs. It has even been suggested that it is the persistence of these ovaries that causes the menstruation of which we sometimes hear as taking place after ovariotomy. Sippel records an instance of third ovary; Mangiagalli has found a supernumerary ovary in the body of a still-born child, situated to the inner side of the normal organ. Winckel discovered a large supernumerary ovary connected to the uterus by its own ovarian ligament. Klebs found two ovaries on one side, both consisting of true ovarian tissue, and connected by a band 3/5 inch long.

Doran divides supernumerary ovaries into three cla.s.ses:--

(1) The ovarium succentauriatum of Beigel.

(2) Those cases in which two ma.s.ses of ovarian tissue are separated by ligamentous bands.

(3) Entirely separate organs, as in Winckel's case.

Prolapsus or displacement of the ovaries into the culdesac of Douglas, the v.a.g.i.n.al wall, or into the r.e.c.t.u.m can be readily ascertained by the resulting sense of nausea, particularly in defecation or in coitus.

Munde, Barnes, Lentz, Madden, and Heywood Smith report instances, and Cloquet describes an instance of inguinal hernia of the ovary in which the uterus as well as the Fallopian tube were found in the inguinal ca.n.a.l. Debierre mentions that Puech has gathered 88 instances of inguinal hernia of the ovary and 14 of the crural type, and also adds that Otte cites the only instance in which crural ovarian hernia has been found on both sides. Such a condition with other a.s.sociate malformations of the genitalia might easily be mistaken for an instance of hermaphroditic t.e.s.t.i.c.l.es.

The Fallopian tubes are rarely absent on either side, although Blasius reports an instance of deficient oviducts. Blot reports a case of atrophy, or rather rudimentary state of one of the ovaries, with absence of the tube on that side, in a woman of forty.

Doran has an instance of multiple Fallopian tubes, and Richard, in 1861, says several varieties are noticed. These tubes are often found fused or adherent to the ovary or to the uterus; but Fabricius describes the symphysis of the Fallopian tube with the r.e.c.t.u.m.

Absence of the uterus is frequently reported. Lieutaud and Richerand are each said to have dissected female subjects in whom neither the uterus nor its annexed organs were found. Many authors are accredited with mentioning instances of defective or deficient uteri, among them Bosquet, Boyer, Walther, Le Fort, Calori, Pozzi, Munde, and Strauch.

Balade has reported a curious absence of the uterus and v.a.g.i.n.a in a girl of eighteen. Azem, Bastien, Bibb, Bovel, Warren, Ward, and many others report similar instances, and in several cases all the adnexa as well as the uterus and v.a.g.i.n.a were absent, and even the kidney and bladder malformed.

Phillips speaks of two sisters, both married, with congenital absence of the uterus. In his masterly article on "Heredity," Sedgwick quotes an instance of total absence of the uterus in three out of five daughters of the same family; two of the three were twice married.

Double uterus is so frequently reported that an enumeration of the cases would occupy several pages. Bicorn, bipart.i.te, duplex, and double uteruses are so called according to the extent of the duplication. The varieties range all the way from slight increase to two distinct uteruses, with separate appendages and two v.a.g.i.n.ae. Meckel, Boehmer, and Callisen are among the older writers who have observed double uterus with a.s.sociate double v.a.g.i.n.a. Figure 150 represents a transverse section of a bipart.i.te uterus with a double v.a.g.i.n.a. The so-called uterus didelphus is really a duplex uterus, or a veritable double uterus, each segment having the appearance of a complete unicorn uterus more or less joined to its neighbor. Vallisneri relates the history of a woman who was poisoned by cantharides who had two uteruses, one opening into the v.a.g.i.n.a, the other into the r.e.c.t.u.m. Morand, Bartholinus, Tiedemann, Ollivier, Blundell, and many others relate instances of double uterus in which impregnation had occurred, the fetus being retained until the full term.

Purcell of Dublin says that in the summer of 1773 he opened the body of a woman who died in the ninth month of pregnancy. He found a uterus of ordinary size and form as is usual at this period of gestation, which contained a full-grown fetus, but only one ovary attached to a single Fallopian tube. On the left side he found a second uterus, unimpregnated and of usual size, to which another ovary and tube were attached. Both of these uteruses were distinct and almost entirely separate.

Pregnancy with Double Uterus.--Hollander describes the following anomaly of the uterus which he encountered during the performance of a celiotomy:--

"There were found two uteruses, the posterior one being a normal organ with its adnexa; connected with this uterus was another one, anterior to it. The two uteruses had a common cervix; the anterior of the two organs had no adnexa, though there were lateral peritoneal ligaments; it had become pregnant." Hollander explains the anomaly by stating that probably the Mullerian ducts or one of them had grown excessively, leading to a folding off of a portion which developed into the anterior uterus.

Other cases of double uterus with pregnancy are mentioned on page 49.

When there is simultaneous pregnancy in each portion of a double uterus a complication of circ.u.mstances arises. Debierre quotes an instance of a woman who bore one child on July 16, 1870, and another on October 31st of the same year, and both at full term. She had only had three menstrual periods between the confinements. The question as to whether a case like this would be one of superfetation in a normal uterus, or whether the uterus was double, would immediately arise. There would also be the possibility that one of the children was of protracted gestation or that the other was of premature birth. Article 312 of the Civil Code of France accords a minimum of one hundred and eighty and a maximum of three hundred days for the gestation of a viable child. (See Protracted Gestation.)

Voight is accredited with having seen a triple uterus, and there are several older parallels on record. Thilow mentions a uterus which was divided into three small portions.

Of the different anomalous positions of the uterus, most of which are acquired, the only one that will be mentioned is that of complete prolapse of the uterus. In this instance the organ may hang entirely out of the body and even forbid locomotion.

Of 19 cases of hernia of the uterus quoted by Debierre 13 have been observed in the inguinal region, five on the right and seven on the left side. In the case of Roux in 1891 the hernia existed on both sides. The uterus has been found twice only in crural hernia and three times in umbilical hernia. There is one case recorded, according to Debierre, in which the uterus was one of the const.i.tuents of an obturator hernia. Sometimes its appendages are found with it. Doring, Ledesma, Rektorzick, and Scazoni have found the uterus in the sac of an inguinal hernia; Leotaud, Murray, and Hagner in an umbilical hernia.

The accompanying ill.u.s.tration represents a hernia of the gravid womb through the linea alba.

Absence of the p.e.n.i.s is an extremely rare anomaly, although it has been noted by Schenck, Borellus, Bouteiller, Nelaton, and others. Fortunatus Fidelis and Revolat describe a newly born child with absence of external genitals, with spina bifida and umbilical hernia. Nelaton describes a child of two entirely without a p.e.n.i.s, but both t.e.s.t.i.c.l.es were found in the s.c.r.o.t.u.m; the boy urinated by the r.e.c.t.u.m. Ashby and Wright mention complete absence of the p.e.n.i.s, the urethra opening at the margin of the a.n.u.s outside the external sphincter; the s.c.r.o.t.u.m and t.e.s.t.i.c.l.es were well developed. Murphy gives the description of a well-formed infant apparently without a p.e.n.i.s; the child pa.s.sed urine through an opening in the lower part of the abdomen just above the ordinary location of the p.e.n.i.s; the s.c.r.o.t.u.m was present. Incisions were made into a small swelling just below the urinary opening in the abdomen which brought into view the p.e.n.i.s, the glans being normal but the body very small. The treatment consisted of pressing out the glans daily until the wound healed; the p.e.n.i.s receded spontaneously. It is stated that the organ would doubtless be equal to any requirements demanded of it. Demarquay quotes a somewhat similar case in an infant, but it had no urinary opening until after operation.

Among the older writers speaking of deficient or absent p.e.n.i.s are Bartholinus, Bauhinus, Cattierus, the Ephemerides, Frank, Panaroli, van der Wiel, and others. Renauldin describes a man with a small p.e.n.i.s and enormous mammae. Goschler, quoted by Jacobson, speaks of a well-developed man of twenty-two, with abundant hair on his chin and suprapubic region and the s.c.r.o.t.u.m apparently perfect, with median rapine; a careful search failed to show any trace of a p.e.n.i.s; on the anterior wall of the r.e.c.t.u.m four lines above the a.n.u.s was an orifice which gave vent to urine; the right t.e.s.t.i.c.l.e and cord were normal, but there was an acute orchitis in the left. Starting from just in front of the a.n.a.l orifice was a fold of skin 1 1/2 inches long and 3/4 inch high continuous with the rapine, which seemed to be formed of erectile tissue and which swelled under excitement, the enlargement lasting several minutes with usually an emission from the r.e.c.t.u.m. It was possible to pa.s.s a sound through the opening in the r.e.c.t.u.m to the bladder through a urethra 1 1/2 inches wide; the patient had control of the bladder and urinated from every three to five hours.

Many instances of rudimentary development of the p.e.n.i.s have been recorded, most of them complicated with cryptorchism or other abnormality of the s.e.xual organs. In other instances the organ is present, but the infantile type is present all through life; sometimes the subjects are weak in intellect and in a condition similar to cretinism. Kaufmann quotes a case in a weakly boy of twelve whose p.e.n.i.s was but 3/4 inch long, about as thick as a goose-quill, and feeling as limp as a mere tube of skin; the corpora cavernosa were not entirely absent, but ran only from the ischium to the junction of the fixed portion of the p.e.n.i.s, suddenly terminating at this point. Nothing indicative of a prostate could be found. The t.e.s.t.i.c.l.es were at the entrance of the inguinal ca.n.a.l and the glans was only slightly developed.

Binet speaks of a man of fifty-three whose external genitalia were of the size of those of a boy of nine. The p.e.n.i.s was of about the size of the little finger, and contained on each side t.e.s.t.i.c.l.es not larger than a pea. There was no hair on the p.u.b.es or the face, giving the man the aspect of an old woman. The prostate was almost exterminated and the seminal vesicles were very primitive in conformation. Wilson was consulted by a gentleman of twenty-six as to his ability to perform the marital function. In size his p.e.n.i.s and t.e.s.t.i.c.l.es hardly exceeded those of a boy of eight. He had never felt desire for s.e.xual intercourse until he became acquainted with his intended wife, since when he had erections and nocturnal emissions. The patient married and became the father of a family; those parts which at twenty-six were so much smaller than usual had increased at twenty-eight to normal adult size.

There are three cases on record in the older literature of p.e.n.i.ses extremely primitive in development. They are quoted by the Ephemerides, Plater, Schenck, and Zacchias. The result in these cases was impotency.

In the Army and Medical Museum at Washington are two injected specimens of the male organ divested of skin. From the meatus to the pubis they measure 6 1/2 and 5 1/2 inches; from the extremity to the termination of either crus 9 3/4 and 8 3/4 inches, and the circ.u.mferences are 4 3/4 and 4 1/4 inches. Between these two we can strike an average of the size of the normal p.e.n.i.s.