MINOR TERATA. 9 page

of the pelvic peritoneum and there was neither uterus nor vagina; the

pelvis had some of the characteristics of the male type. Matthews

Duncan has observed a somewhat similar case, the vagina not measuring

more than an inch in length. Ferguson describes a prostitute of

eighteen who had never menstruated. The labia were found well

developed, but there was no vagina, 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 vagina in a brunette of twenty, menstruation occurring

through the urethra. He also mentions the instance of congenital

atresia of the vagina with hernia of both ovaries into the left groin

in a servant of twenty, and the case of an imperforate vagina in a girl

of nineteen with an undeveloped uterus.

Brodhurst reports an instance of absence of the vagina and uterus in a

girl of sixteen who at four years of age showed signs of approaching

puberty. At this early age the mons was covered with hair, and at ten

the clitoris was three inches long and two inches in circumference. The

mammae were well developed. The labia descended laterally and expanded

into folds, resembling the scrotum.

Azema reports an instance of complete absence of the vagina 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 vagina 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 vagina. An

artificial vagina 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 vagina.

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 vagina 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 vagina, and

delivery through the rectum. Atthill and Watts speak of double vagina

with single uterus.

Robb of Johns Hopkins Hospital reports a case of double vagina in a

patient of twenty suffering from dyspareunia. The vaginal orifice was

contracted; the urethra was dilated and had evidently been used for

coitus. A membrane divided the vagina into two canals, 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 vagina is also reported by Anway, Moulton,

Freeman, Frazer, Haynes, Lemaistre, Boardman, Dickson, Dunoyer, and

Rossignol. This anomaly is usually associated with bipartite or double

uterus. Wilcox mentions a primipara, three months pregnant, with a

double vagina and a bicornate uterus, who was safely delivered of

several children. Haller and Borellus have seen double vagina, double

uterus, and double ovarian supply; in the latter case there was also a

double vulva. Sanger speaks of a supernumerary vagina connecting with

the other vagina 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 vagina. Stone reports five cases of transverse septa of the vagina.

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 vagina, 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 vagina 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

vaginal 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 rectovaginal

septum which was successfully remedied by operation.

Anomalous Openings of the Vagina.--The vagina occasionally opens

abnormally into the rectum, 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 vaginal orifice; no traces of a vagina 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

rectum, and examination showed that the vagina opened into the rectum,

by which means impregnation had been accomplished. Bonnain and Payne

have observed analogous cases of this abnormality of the vaginal

opening and subsequent accouchement by the anus. 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 vaginal

opening; the finger, gliding along the perineum, came in contact with

the distended anus, 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 vulva of this woman five months afterward displayed all

the characteristics of virginity, the vagina opened into the rectum,

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 anus) had always been satisfactory.

Opening of the vagina 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 labia 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 associated with imperfect development

of the clitoris. 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 clitoris 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 labia majora.

On the other hand, some of the lower African races have been

distinguished by the deficiency in development of the labia 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 labia majora.

The labial appendages of the Hottentot female have been celebrated for

many years. Blumenbach and others of the earlier travelers found that

the apron-like appearance of the genitals of the Hottentot women was

due to abnormal hypertrophy of the labia 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 labia. 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 labial 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 labia up to the standard

of recognized length. Cameron found an analogous practice among the

women of the shores 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 puberty these women exhibit a cutaneous

curtain over the genitals which reaches half-way down the thighs.

A corresponding development of the preputian clitorides, attaining the

length of 18 mm. or even more, has been observed among the females of

Bechuanaland. 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 Petit is accredited

with seeing a case which exhibited neither nymphae, clitoris, 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 labia and nymphae, which may be so

firm and extensive as to obliterate the vulva. Debout has reported a

case of absence of the vulva in a woman of twenty upon whom he

operated, which was the result of the fusion of the labia minora, and

this with an enlarged clitoris gave the external appearance of an

hermaphrodite.

The absence of the clitoris coincides with epispadias in the male, and

in atrophy of the vulva it is common to find the clitoris rudimentary;

but a more frequent anomaly is hypertrophy of the clitoris.

Among the older authorities quoting instances of enlarged clitorides

are Bartholinus, Schenck, Hellwig, Rhodius, Riolanus, and Zacchias.

Albucasis describes an operation for enlarged clitoris, Chabert ligated

one, and Riedlin gives an instance of an enlarged clitoris, in which

there appeared a tumor synchronous with the menstrual epoch.

We learn from the classics 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 passion. The solution of this peculiar ardor is

found in the fact that some of the females had enlarged clitorides,

strong voices, robust figures, and imitated men. Their manner was

imperative and authoritative to their sex, 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 clitoris, 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

circumcision 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 clitorides in Arab women; Bruce testifies to this circumstance

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 circumcision the Egyptian women not only remove a

great part of the body of the clitoris 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 clitoris 4 1/2

inches long and 1 1/2 inches in the transverse diameter; it projected

from the vulva and when supine formed a complete covering for the

vaginal orifice. The clitoris may at times become so large as to

prevent coitus, and in France has constituted a legitimate cause for

divorce. This organ is very sensitive, and it is said that in cases of

supposed catalepsy a woman cannot bear titillation of the clitoris

without some visible movement.

Columbus cites an example of a clitoris as long as a little finger;

Haller mentions one which measured seven inches, and there is a record

of an enlarged clitoris which resembled the neck of a goose and which

was 12 inches long. Bainbridge reports a case of enlarged clitoris 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

penis. Figure 149 shows a well-marked case of hypertrophy of the

clitoris. Rogers describes a woman of twenty-five in a reduced state of

health with an enormous clitoris and warts about the anus; there were

also manifestations of tuberculosis. On questioning her, it was found

that she had formerly masturbated; later she had sexual intercourse

several times with a young man, but after his death she commenced

self-abuse again, which brought on the present enlargement. The

clitoris was ligated and came away without leaving disfigurement.

Cassano and Pedretti of Naples reported an instance of monstrous

clitoris in 1860 before the Academy of Medicine.

In some cases ossification of the clitoris is observed Fournier speaks

of a public woman in Venice who had an osseous clitoris; it was said

that men having connection with her invariably suffered great pain,

followed by inflammation of the penis.

There are a few instances recorded of bifid clitoris, and Arnaud cites

the history of a woman who had a double clitoris. Secretain speaks of a

clitoris 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 Thudicum 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

puberty. Lauth cites the case of a woman whose ovaries and uterus were

rudimentary, and who exhibited none of the principal physiologic

characteristics of her sex; on the other hand, Ruband describes a woman

with only rudimentary ovaries who was very passionate 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, Thudicum, 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 classes:--

(1) The ovarium succentauriatum of Beigel.

(2) Those cases in which two masses 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 vaginal wall, or into the rectum 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

canal. 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 associate

malformations of the genitalia might easily be mistaken for an instance

of hermaphroditic testicles.

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 rectum.

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 vagina 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 vagina 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, bipartite, 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 vaginae. Meckel, Boehmer,

and Callisen are among the older writers who have observed double

uterus with associate double vagina. Figure 150 represents a transverse

section of a bipartite uterus with a double vagina. 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 vagina, the other into the rectum. 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 circumstances 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 constituents 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 illustration represents a hernia of the gravid womb

through the linea alba.

Absence of the penis 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 penis, but both testicles

were found in the scrotum; the boy urinated by the rectum. Ashby and

Wright mention complete absence of the penis, the urethra opening at

the margin of the anus outside the external sphincter; the scrotum and

testicles were well developed. Murphy gives the description of a

well-formed infant apparently without a penis; the child passed urine

through an opening in the lower part of the abdomen just above the

ordinary location of the penis; the scrotum was present. Incisions were

made into a small swelling just below the urinary opening in the

abdomen which brought into view the penis, the glans being normal but

the body very small. The treatment consisted of pressing out the glans

daily until the wound healed; the penis 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 penis are

Bartholinus, Bauhinus, Cattierus, the Ephemerides, Frank, Panaroli, van

der Wiel, and others. Renauldin describes a man with a small penis and

Date: 2014-12-29; view: 728