MINOR TERATA. 8 page

paper Ballowitz has collected 213 cases, from which the following

extract has been made by the British Medical Journal:--

"Ballowitz (Virchow's Archiv, August 5, 1895) has collected as far as

possible all the recorded cases of congenital absence of one kidney.

Excluding cases of fused kidney and of partial atrophy of one kidney,

he finds 213 cases of complete absence of one kidney, upon which he

bases the following conclusions: Such deficiency occurs almost twice as

often in males as in females, a fact, however, which may be partly

accounted for by the greater frequency of necropsies on males. As to

age, 23 occurred in the fetus or newly born, most having some other

congenital deformity, especially imperforate anus; the rest were about

evenly distributed up to seventy years of age, after which only seven

cases occurred. Taking all cases together, the deficiency is more

common on the left than on the right side; but while in males the left

kidney is far more commonly absent than the right, in females the two

sides show the defect equally. The renal vessels were generally absent,

as also the ureter, on the abnormal side (the latter in all except 15

cases); the suprarenal was missing in 31 cases. The solitary kidney was

almost always normal in shape and position, but much enlarged.

Microscopically the enlargement would seem to be due rather to

hyperplasia than to hypertrophy. The bladder, except for absence of the

opening of one ureter, was generally normal. In a large number of cases

there were associated deformities of the organs of generation,

especially of the female organs, and these were almost invariably on

the side of the renal defect; they affected the conducting portion much

more than the glandular portion--that is, uterus, vagina, and Fallopian

tubes in the female, and vas deferens or vesiculae seminales in the

male, rather than the ovaries or testicles. Finally, he points out the

practical bearing of the subject--for example, the probability of

calculus causing sudden suppression of urine in such cases--and also

the danger of surgical interference, and suggests the possibility of

diagnosing the condition by ascertaining the absence of the opening of

one ureter in the bladder by means of the cystoscope, and also the

likelihood of its occurring where any abnormality of the genital organs

is found, especially if this be unilateral."

Green reports the case of a female child in which the right kidney and

right Fallopian tube and ovary were absent without any rudimentary

structures in their place. Guiteras and Riesman have noted the absence

of the right kidney, right ureter, and right adrenal in an old woman

who had died of chronic nephritis. The left kidney although cirrhotic

was very much enlarged.

Tompsett describes a necropsy made on a coolie child of nearly twelve

months, in which it was seen that in the place of a kidney there were

two left organs connected at the apices by a prolongation of the

cortical substance of each; the child had died of neglected malarial

fever. Sandifort speaks of a case of double kidneys and double ureters,

and cases of supernumerary kidney are not uncommon, generally being

segmentation of one of the normal kidneys. Rayer has seen three kidneys

united and formed like a horseshoe. We are quite familiar with the

ordinary "horseshoe kidney," in which two normal kidneys are connected.

There are several forms of displacement of the kidneys, the most common

being the "floating kidney," which is sometimes successfully removed or

fixed; Rayer has made an extensive study of this anomaly.

The kidney may be displaced to the pelvis, and Guinard quotes an

instance in which the left kidney was situated in the pelvis, to the

left of the rectum and back of the bladder. The ureter of the left side

was very short. The left renal artery came from the bifurcation of the

aorta and the primitive iliacs. The right kidney was situated normally,

and received from the aorta two arteries, whose volume did not surpass

the two arteries supplying the left suprarenal capsule, which was in

its ordinary place. Displacements of the kidney anteriorly are very

rare.

The ureters have been found multiple; Griffon reports the history of a

male subject in whom the ureter on the left side was double throughout

its whole length; there were two vesical orifices on the left side one

above the other; and Morestin, in the same journal, mentions ureters

double on both sides in a female subject. Molinetti speaks of six

ureters in one person. Littre in 1705 described a case of coalition of

the ureters. Allen describes an elongated kidney with two ureters.

Coeyne mentions duplication of the ureters on both sides. Lediberder

reports a case in which the ureter had double origin. Tyson cites an

instance of four ureters in an infant. Penrose mentions the absence of

the upper two-thirds of the left ureter, with a small cystic kidney,

and there are parallel cases on record.

The ureters sometimes have anomalous terminations either in the rectum,

vagina, 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 canal, 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

participated but little in the deformity, and with the exception that

the umbilicus was low and the anus 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 passed

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 sexual 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 anal pouches. Both testicles

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 illustration, 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 rectum and intestine.

Exstrophy of the bladder is not rare, and is often associated 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 Lusitanus.

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 puberty 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 puberty.

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 analogous 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 deglutition became

impossible and the patient died of malnutrition. A necropsy revealed

the presence of the subclavicular artery passing between the tracheal

artery and the esophagus, compressing this latter tube and opposing the

passage of food.

Anomalies of the Breasts.--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 passed for a female, but who was really a

male, in whom the breasts were absolutely wanting. Foerster, Froriep,

and Ried cite instances associated 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

associate 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

breasts 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 breasts 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 breasts. Anne Boleyn, the unfortunate wife of Henry VIII

of England, was reputed to have had six toes, six fingers, and three

breasts. 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 breasts; 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 breasts or nipples are due to

atavism, and that our most remote inferiorly organized ancestors had

many breasts, 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 breasts woman was

originally intended to bear two children.

Hirst says: "Supernumerary breasts 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 Mitchell 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 goddess is portrayed with numerous

breasts, 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 breasts 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

breasts, arranged parallel. She was extremely ugly, and when on all

fours, with her breasts pendulous, she resembled a beast. The authors

have seen a man with six distinct nipples, arranged as regularly as

those of a bitch 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 space

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 quantity as the others. Gardiner describes a

mulatto woman who had four mammae, two of which were near the axillae,

about four inches in circumference, with proportionate sized nipples.

She became a mother at fourteen, and gave milk from all her breasts. In

his "Dictionnaire Philosophique" Voltaire gives the history of a woman

with four well-formed and symmetrically arranged breasts; 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 puberty.

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 quantities. 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 breasts. 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 breasts, one of which was

situated on the groin and with which she occasionally suckled; her

mother had three breasts, but they were all situated on the chest.

Saunois details an account of a female who had two supernumerary

breasts 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 illustration 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 breasts 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 breasts 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 vulva may be caused by the persistence of a thick hymen, by

congenital occlusion, or by absolute absence in vulvar structure.

Bartholinus, Borellus, Ephemerides, Julius, Vallisneri, and Baux are

among the older writers who mention this anomaly, but as it is

generally associated with congenital occlusion, or complete absence of

the vagina, the two will be considered together.

Complete absence of the vagina 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 vagina. Vicq

d'Azir is accredited with having seen two females who, not having a

vagina, 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 vulva, closure of the

vagina, 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, Hancock, Coste,

Klayskens, Debrou, Braid, Watson, and others are quoted by Churchill as

having mentioned the absence of the vagina. Amussat observed a German

girl who did not have a trace of a vagina 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

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