Sex in Relation to Society
1888. A little girl under Birkett's care in Guy's Hospital more than
22801 words | Chapter 19
answered to Macbeth's requisition, "Had I three ears I'd hear thee!"
since she possessed two superfluous ones at the sides of the neck,
somewhat lower than the angle of the jaw, which were well developed as
to their external contour and made up of fibrocartilage. There is
mentioned the case of a boy of six months on the left side of whose
neck, over the middle anterior border of the sternocleidomastoid
muscle, was a nipple-like projection 1/2 inch in length; a rod of
cartilage was prolonged into it from a thin plate, which was freely
movable in the subcutaneous tissue, forming a striking analogue to an
auricle. Moxhay cites the instance of a mother who was frightened by
the sight of a boy with hideous contractions in the neck, and who gave
birth to a child with two perfect ears and three rudimentary auricles
on the right side, and on the left side two rudimentary auricles.
In some people there is an excessive development of the auricular
muscles, enabling them to move their ears in a manner similar to that
of the lower animals. Of the celebrated instances the Abbe de Marolles,
says Vigneul-Marville, bears witness in his "Memoires" that the Regent
Crassot could easily move his ears. Saint Augustine mentions this
anomaly.
Double tympanitic membrane is spoken of by Loeseke. There is sometimes
natural perforation of the tympanum in an otherwise perfect ear, which
explains how some people can blow tobacco-smoke from the ear. Fournier
has seen several Spaniards and Germans who could perform this feat, and
knew one man who could smoke a whole cigar without losing any smoke,
since he made it leave either by his mouth, his ears, or in both ways.
Fournier in the same article mentions that he has seen a woman with
ears over four inches long.
Strange to say, there have been reports of cases in which the ossicles
were deficient without causing any imperfection of hearing. Caldani
mentions a case with the incus and malleus deficient, and Scarpa and
Torreau quote instances of deficient ossicles. Thomka in 1895 reported
a case of supernumerary tympanic ossicle, the nature of which was
unknown, although it was neither an inflammatory product nor a remnant
of Meckel's cartilage.
Absence of the Limbs.--Those persons born without limbs are either the
subjects of intrauterine amputation or of embryonic malformation.
Probably the most celebrated of this class was Marc Cazotte, otherwise
known as "Pepin," who died in Paris in the last century at the age of
sixty-two of a chronic intestinal disorder. He had no arms, legs, or
scrotum, but from very jutting shoulders on each side were well-formed
hands. His abdomen ended in a flattened buttock with badly-formed feet
attached. He was exhibited before the public and was celebrated for his
dexterity. He performed nearly all the necessary actions, exhibited
skilfulness in all his movements, and was credited with the ability of
coitus. He was quite intellectual, being able to write in several
languages. His skeleton is preserved in the Musee Dupuytren. Flachsland
speaks of a woman who three times had borne children without arms and
legs. Hastings describes a living child born without any traces of arms
or legs. Garlick has seen a child with neither upper nor lower
extremities. In place of them were short stumps three or four inches
long, closely resembling the ordinary stumps after amputation. The
head, chest, body, and male genitals were well formed, and the child
survived. Hutchinson reports the history of a child born without
extremities, probably the result of intrauterine amputation. The flaps
were healed at the deltoid insertion and just below the groin. Pare
says he saw in Paris a man without arms, who by means of his head and
neck could crack a whip or hold an axe. He ate by means of his feet,
dealt and played cards, and threw dice with the same members,
exhibiting such dexterity that finally his companions refused to play
with him. He was proved to be a thief and a murderer and was finally
hanged at Gueldres. Pare also relates having seen a woman in Paris who
sewed, embroidered, and did other things with her feet. Jansen speaks
of a man in Spain, born without arms, who could use his feet as well as
most people use their arms. Schenck and Lotichius give descriptions of
armless people.
Hulke describes a child of four whose upper limbs were absent, a small
dimple only being in their place. He had free movement of the shoulders
in every direction and could grasp objects between his cheeks and his
acromian process; the prehensile power of the toes was well developed,
as he could pick up a coin thrown to him. A monster of the same
conformation was the celebrated painter, Ducornet, who was born at
Lille on the 10th of January, 1806. He was completely deprived of arms,
but the rest of the body was well formed with the exception of the
feet, of which the second toe was faulty. The deformity of the feet,
however, had the happiest result, as the space between the great toe
and its neighbor was much larger than ordinary and the toes much more
mobile. He became so skilful in his adopted profession that he finally
painted a picture eleven feet in height (representing Mary Magdalene at
the feet of Christ after the resurrection), which was purchased by the
Government and given to the city of Lille. Broca describes James
Leedgwood, who was deprived of his arms and had only one leg. He
exhibited great dexterity with his single foot, wrote, discharged a
pistol, etc.; he was said to have been able to pick up a sewing-needle
on a slippery surface with his eyes blindfolded. Capitan described to
the Societe d'anthropologie de Paris a young man without arms, who was
said to play a violin and cornet with his feet. He was able to take a
kerchief from his pocket and to blow his nose; he could make a
cigarette, light it, and put it in his mouth, play cards, drink from a
glass, and eat with a fork by the aid of his dexterous toes. There was
a creature exhibited some time since in the principal cities of France,
who was called the "l'homme tronc." He was totally deprived of all his
members. Curran describes a Hindoo, a prostitute of forty, with
congenital absence of both upper extremities. A slight fleshy
protuberance depended from the cicatrix of the humerus and
shoulder-joint of the left side, and until the age of ten there was one
on the right side. She performed many tricks with her toes. Caldani
speaks of a monster without arms, Davis mentions one, and Smith
describes a boy of four with his upper limbs entirely absent. Breschet
has seen a child of nine with only portions of the upper arms and
deformity of lower extremities and pelvis. Pare says that he saw in
Paris in 1573, at the gate of St. Andrew des Arts, a boy of nine, a
native of a small village near Guise, who had no legs and whose left
foot was represented by a fleshy body hanging from the trunk; he had
but two fingers hanging on his right hand, and had between his legs
what resembled a virile penis. Pare attributes this anomaly to a
default in the quantity of semen.
The figure and skeleton of Harvey Leach, called "Hervio Nono," is in
the museum of the University College in London. The pelvis was
comparatively weak, the femurs hardly to be recognized, and the right
tibia and foot defective; the left foot was better developed, although
far from being in due proportion to the trunk above. He was one of the
most remarkable gymnasts of his day, and notwithstanding the distortion
of his lower limbs had marvelous power and agility in them. As an
arena-horseman, either standing or sitting, he was scarcely excelled.
He walked and even ran quite well, and his power of leaping, partly
with his feet and partly with his hands, was unusual. His lower limbs
were so short that, erect, he touched the floor with his fingers, but
he earned his livelihood as much with his lower as with his upper
limbs. In his skeleton his left lower limb, between the hip and heel,
measured 16 inches, while the right, between the same points, measured
nine inches. Hare mentions a boy of five and a half whose head and
trunk were the same as in any other child of like age. He was 22 1/2
inches high, had no spinal curvature, but was absolutely devoid of
lower extremities. The right arm was two inches long and the left 2
1/4. Each contained the head and a small adjoining portion of the
humerus. The legs were represented by masses of cellular tissue and fat
covered by skin which projected about an inch. He was intelligent, had
a good memory, and exhibited considerable activity. He seemed to have
had more than usual mobility and power of flexion of the lower lumbar
region. When on his back he was unable to rise up, but resting on the
lower part of the pelvis he was able to maintain himself erect. He
usually picked up objects with his teeth, and could hold a coin in the
axilla as he rolled from place to place. His rolling was accomplished
by a peculiar twisting of the thorax and bending of the pelvis. There
was no history of maternal impression during pregnancy, no injury, and
no hereditary disposition to anomalous members. Figure 112 represents a
boy with congenital deficiency of the lower extremities who was
exhibited a few years ago in Philadelphia. In Figure 113, which
represents a similar case in a girl whose photograph is deposited in
the Mutter Museum of the College of Physicians, Philadelphia, we see
how cleverly the congenital defect may be remedied by mechanical
contrivance. With her crutches and artificial legs this girl was said
to have moved about easily.
Parvin describes a "turtle-man" as an ectromelian, almost entering the
class of phocomelians or seal-like monsters; the former term signifies
abortive or imperfect formation of the members. The hands and feet were
normally developed, but the arms, forearms, and legs are much shortened.
The "turtle-woman" of Demerara was so called because her mother when
pregnant was frightened by a turtle, and also from the child's fancied
resemblance to a turtle. The femur was six inches long, the woman had a
foot of six bones, four being toes, viz., the first and second
phalanges of the first and second toes. She had an acetabulum, capsule,
and ligamentum teres, but no tibia or fibula; she also had a defective
right forearm. She was never the victim of rachitis or like disease,
but died of syphilis in the Colonial Hospital. In her twenty-second
year she was delivered of a full-grown child free of deformity.
There was a woman living in Bavaria, under the observation of Buhl, who
had congenital absence of both femurs and both fibulas. Almost all the
muscles of the thigh existed, and the main attachment to the pelvis was
by a large capsular articulation. Charpentier gives the portrait of a
woman in whom there was a uniform diminution in the size of the limbs.
Debout portrays a young man with almost complete absence of the thigh
and leg, from whose right hip there depended a foot. Accrell describes
a peasant of twenty-six, born without a hip, thigh, or leg on the right
side. The external genital organs were in their usual place, but there
was only one testicle in the scrotum. The man was virile. The rectum
instead of opening outward and underneath was deflected to the right.
Supernumerary Limbs.--Haller reports several cases of supernumerary
extremities. Plancus speaks of an infant with a complete third leg, and
Dumeril cites a similar instance. Geoffroy-Saint-Hilaire presented to
the Academie des Sciences in 1830 a child with four legs and feet who
was in good health. Amman saw a girl with a large thigh attached to
her nates. Below the thigh was a single leg made by the fusion of two
legs. No patella was found and the knee was anchylosed. One of the feet
of the supernumerary limb had six toes, while the other, which was
merely an outgrowth, had two toes on it.
According to Jules Guerin, the child named Gustav Evrard was born with
a thigh ending in two legs and two imperfect feet depending from the
left nates.
Tucker describes a baby born in the Sloane Maternity in New York,
October 1, 1894, who had a third leg hanging from a bony and fleshy
union attached to the dorsal spine. The supernumerary leg was well
formed and had a left foot attached to it. Larkin and Jones mention the
removal of a meningocele and a supernumerary limb from an infant of
four months. This limb contained three fingers only, one of which did
not have a bony skeleton.
Pare says that on the day the Venetians and the Genevois made peace a
monster was born in Italy which had four legs of equal proportions, and
besides had two supernumerary arms from the elbows of the normal limbs.
This creature lived and was baptized.
Anomalies of the Feet.--Hatte has seen a woman who bore a child that
had three feet. Bull gives a description of a female infant with the
left foot double or cloven. There was only one heel, but the anterior
portion consisted of an anterior and a posterior part. The anterior
foot presented a great toe and four smaller ones, but deformed like an
example of talipes equinovarus. Continuous with the outer edge of the
anterior part and curving beneath it was a posterior part, looking not
unlike a second foot, containing six well-formed toes situated directly
beneath the other five. The eleven toes were all perfect and none of
them were webbed.
There is a class of monsters called "Sirens" on account of their
resemblance to the fabulous creatures of mythology of that name. Under
the influence of compression exercised in the uterus during the early
period of gestation fusion of the inferior extremities is effected. The
accompanying illustration shows the appearance of these monsters, which
are thought to resemble the enchantresses celebrated by Homer.
Anomalies of the Hand.--Blumenbach speaks of an officer who, having
lost his right hand, was subsequently presented by his wife with
infants of both sexes showing the same deformity. Murray cites the
instance of a woman of thirty-eight, well developed, healthy, and the
mother of normal children, who had a double hand. The left arm was
abnormal, the flexion of the elbow imperfect, and the forearm
terminated in a double hand with only rudimentary thumbs. In working as
a charwoman she leaned on the back of the flexed carpus. The double
hand could grasp firmly, though the maximum power was not so great as
that of the right hand. Sensation was equally acute in all three of the
hands. The middle and ring fingers of the supernumerary hand were
webbed as far as the proximal joints, and the movements of this hand
were stiff and imperfect. No single finger of the two hands could be
extended while the other seven were flexed. Giraldes saw an infant in
1864 with somewhat the same deformity, but in which the disposition of
the muscles and tendons permitted the ordinary movements.
Absence of Digits.--Maygrier describes a woman of twenty-four who
instead of having a hand on each arm had only one finger, and each foot
had but two toes. She was delivered of two female children in 1827 and
one in 1829, each having exactly the same deformities. Her mother was
perfectly formed, but the father had but one toe on his foot and one
finger on his left hand.
Kohler gives photographs of quite a remarkable case of suppression and
deformity of the digits of both the fingers and toes.
Figure 123 shows a man who was recently exhibited in Philadelphia. He
had but two fingers on each hand and two toes on each foot, and
resembles Kohler's case in the anomalous digital conformation.
Figure 124 represents an exhibitionist with congenital suppression of
four digits on each hand.
Tubby has seen a boy of three in whom the first, second, and third toes
of each foot were suppressed, the great toe and the little toe being so
overgrown that they could be opposed. In this family for four
generations 15 individuals out of 22 presented this defect of the lower
extremity. The patient's brothers and a sister had exactly the same
deformity, which has been called "lobster-claw foot."
Falla of Jedburgh speaks of an infant who was born without forearms or
hands; at the elbow there was a single finger attached by a thin string
of tissue. This was the sixth child, and it presented no other
deformity. Falla also says that instances of intrauterine digital
amputation are occasionally seen.
According to Annandale, supernumerary digits may be classified as
follows:--
(1) A deficient organ, loosely attached by a narrow pedicle to the hand
or foot (or to another digit).
(2) A more or less developed organ, free at its extremity, and
articulating with the head or sides of a metacarpal, metatarsal, or
phalangeal bone.
(3) A fully developed separate digit.
(4) A digit intimately united along its whole length with another
digit, and having either an additional metacarpal or metatarsal bone of
its own, or articulating with the head of one which is common to it and
another digit.
Superstitions relative to supernumerary fingers have long been
prevalent. In the days of the ancient Chaldeans it was for those of
royal birth especially that divinations relative to extra digits were
cast. Among the ancients we also occasionally see illustrations
emblematic of wisdom in an individual with many fingers, or rather
double hands, on each arm.
Hutchinson, in his comments on a short-limbed, polydactylous dwarf
which was dissected by Ruysch, the celebrated Amsterdam anatomist,
writes as follows.--
"This quaint figure is copied from Theodore Kerckring's 'Spicilegium
Anatomicum,' published in Amsterdam in 1670. The description states
that the body was that of an infant found drowned in the river on
October 16, 1668. It was dissected by the renowned Ruysch. A detailed
description of the skeleton is given. My reason for now reproducing
the plate is that it offers an important item of evidence in reference
to the development of short-limbed dwarfs. Although we must not place
too much reliance on the accuracy of the draughtsman, since he has
figured some superfluous lumbar vertebrae, yet there can be no doubt
that the limbs are much too short for the trunk and head. This remark
especially applies to the lower limbs and pelvis. These are exactly
like those of the Norwich dwarf and of the skeleton in the Heidelberg
Museum which I described in a recent number of the 'Archives.' The
point of extreme interest in the present case is that this dwarfing of
the limbs is associated with polydactylism. Both the hands have seven
digits. The right foot has eight and the left nine. The conditions are
not exactly symmetrical, since in some instances a metacarpal or
metatarsal bone is wanting; or, to put it otherwise, two are welded
together. It will be seen that the upper extremities are so short that
the tips of the digits will only just touch the iliac crests.
"This occurrence of short limbs with polydactylism seems to prove
conclusively that the condition may be due to a modification of
development of a totally different nature from rickets. It is probable
that the infant was not at full term. Among the points which the author
has noticed in his description are that the fontanelle was double its
usual size; that the orbits were somewhat deformed; that the two halves
of the lower jaw were already united; and that the ribs were short and
badly formed. He also, of course, draws attention to the shortness of
the limbs, the stoutness of the long bones, and the supernumerary
digits. I find no statement that the skeleton was deposited in any
museum, but it is very possible that it is still in existence in
Amsterdam, and if so it is very desirable that it should be more
exactly described."
In Figure 126, A represents division of thumb after Guyot-Daubes, shows
a typical case of supernumerary fingers, and C pictures Morand's case
of duplication of several toes.
Forster gives a sketch of a hand with nine fingers and a foot with nine
toes. Voight records an instance of 13 fingers on each hand and 12 toes
on each foot. Saviard saw an infant at the Hotel-Dieu in Paris in 1687
which had 40 digits, ten on each member. Annandale relates the history
of a woman who had six fingers and two thumbs on each hand, and another
who had eight toes on one foot.
Meckel tells of a case in which a man had 12 fingers and 12 toes, all
well formed, and whose children and grandchildren inherited the
deformity. Mason has seen nine toes on the left foot. There is recorded
the account of a child who had 12 toes and six fingers on each hand,
one fractured. Braid describes talipes varus in a child of a few months
who had ten toes. There is also on record a collection of cases of from
seven to ten fingers on each hand and from seven to ten toes on each
foot. Scherer gives an illustration of a female infant, otherwise
normally formed, with seven fingers on each hand, all united and
bearing claw-like nails. On each foot there was a double halux and five
other digits, some of which were webbed.
The influence of heredity on this anomaly is well demonstrated.
Reaumur was one of the first to prove this, as shown by the Kelleia
family of Malta, and there have been many corroboratory instances
reported; it is shown to last for three, four, and even five
generations; intermarriage with normal persons finally eradicates it.
It is particularly in places where consanguineous marriages are
prevalent that supernumerary digits persist in a family. The family of
Foldi in the tribe of Hyabites living in Arabia are very numerous and
confine their marriages to their tribe. They all have 24 digits, and
infants born with the normal number are sacrificed as being the
offspring of adultery. The inhabitants of the village of Eycaux in
France, at the end of the last century, had nearly all supernumerary
digits either on the hands or feet. Being isolated in an inaccessible
and mountainous region, they had for many years intermarried and thus
perpetuated the anomaly. Communication being opened, they emigrated or
married strangers and the sexdigitism vanished. Maupertuis recalls the
history of a family living in Berlin whose members had 24 digits for
many generations. One of them being presented with a normal infant
refused to acknowledge it. There is an instance in the Western United
States in which supernumerary digits have lasted through five
generations. Cameron speaks of two children in the same family who were
polydactylic, though not having the same number of supernumerary
fingers.
Smith and Norwell report the case of a boy of fifteen both of whose
hands showed webbing of the middle and ring fingers and accessory
nodules of bone between the metacarpals, and six toes on each foot. The
boy's father showed similar malformations, and in five generations 21
out of 28 individuals were thus malformed, ten females and 11 males.
The deformity was especially transmitted in the female line.
Instances of supernumerary thumbs are cited by Panaroli, Ephemerides,
Munconys, as well as in numerous journals since. This anomaly is not
confined to man alone; apes, dogs, and other lower animals possess it.
Bucephalus, the celebrated horse of Alexander, and the horse of Caesar
were said to have been cloven-hoofed.
Hypertrophy of the digits is the result of many different processes,
and true hypertrophy or gigantism must be differentiated from
acromegaly, elephantiasis, leontiasis, and arthritis deformans, for
which distinction the reader is referred to an article by Park. Park
also calls attention to the difference between acquired gigantism,
particularly of the finger and toes, and another condition of
congenital gigantism, in which either after or before birth there is a
relatively disproportionate, sometimes enormous, overgrowth of perhaps
one finger or two, perhaps of a limited portion of a hand or foot, or
possibly of a part of one of the limbs. The best collection of this
kind of specimens is in the College of Surgeons in London.
Curling quotes a most peculiar instance of hypertrophy of the fingers
in a sickly girl. The middle and ring fingers of the right hand were of
unusual size, the middle finger measuring 5 1/2 inches in length four
inches in circumference. On the left hand the thumb and middle fingers
were hypertrophied and the index finger was as long as the middle one
of the right hand. The middle finger had a lateral curvature outward,
due to a displacement of the extensor tendon. This affection resembled
acromegaly. Curling cites similar cases, one in a Spanish gentleman,
Governor of Luzon, in the Philippine Islands, in 1850, who had an
extraordinary middle finger, which he concealed by carrying it in the
breast of his coat.
Hutchinson exhibited a photograph showing the absence of the radius and
thumb, with shortening of the forearm. Conditions more or less
approaching this had occurred in several members of the same family. In
some they were associated with defects of development in the lower
extremities also.
The varieties of club-foot--talipes varus, valgus, equinus,
equino-varus, etc.--are so well known that they will be passed with
mention only of a few persons who have been noted for their activity
despite their deformity. Tyrtee, Parini, Byron, and Scott are among the
poets who were club-footed; some writers say that Shakespeare suffered
in a slight degree from this deformity. Agesilas, Genserie, Robert II,
Duke of Normandy, Henry II, Emperor of the West, Otto II, Duke of
Brunswick, Charles II, King of Naples, and Tamerlane were victims of
deformed feet. Mlle. Valliere, the mistress of Louis XIV, was supposed
to have both club-foot and hip-disease. Genu valgum and genu varum are
ordinary deformities and quite common in all classes.
Transpositions of the character of the vertebrae are sometimes seen. In
man the lumbar vertebrae have sometimes assumed the character of the
sacral vertebrae, the sacral vertebrae presenting the aspect of lumbar
vertebrae, etc. It is quite common to see the first lumbar vertebra
presenting certain characteristics of the dorsal.
Numerical anomalies of the vertebrae are quite common, generally in the
lumbar and dorsal regions, being quite rare in the cervical, although
there have been instances of six or eight cervical vertebrae. In the
lower animals the vertebrae are prolonged into a tail, which, however,
is sometimes absent, particularly when hereditary influence exists. It
has been noticed in the class of dogs whose tails are habitually
amputated to improve their appearance that the tail gradually decreases
in length. Some breeders deny this fact.
Human Tails.--The prolongation of the coccyx sometimes takes the shape
of a caudal extremity in man. Broca and others claim that the sacrum
and the coccyx represent the normal tail of man, but examples are not
infrequent in which there has been a fleshy or bony tail appended to
the coccygeal region. Traditions of tailed men are old and widespread,
and tailed races were supposed to reside in almost every country. There
was at one time an ancient belief that all Cornishmen had tails, and
certain men of Kent were said to have been afflicted with tails in
retribution for their insults to Thomas a Becket. Struys, a Dutch
traveler in Formosa in the seventeenth century, describes a wild man
caught and tied for execution who had a tail more than a foot long,
which was covered with red hair like that of a cow.
The Niam Niams of Central Africa are reported to have tails smooth and
hairy and from two to ten inches long. Hubsch of Constantinople remarks
that both men and women of this tribe have tails. Carpus, or
Berengarius Carpensis, as he is called, in one of his Commentaries said
that there were some people in Hibernia with long tails, but whether
they were fleshy or cartilaginous could not be known, as the people
could not be approached. Certain supposed tailed races which have been
described by sea-captains and voyagers are really only examples of
people who wear artificial appendages about the waists, such as
palm-leaves and hair. A certain Wesleyan missionary, George Brown, in
1876 spoke of a formal breeding of a tailed race in Kali, off the coast
of New Britain. Tailless children were slain at once, as they would be
exposed to public ridicule. The tailed men of Borneo are people
afflicted with hereditary malformation analogous to sexdigitism. A
tailed race of princes have ruled Rajoopootana, and are fond of their
ancestral mark. There are fabulous stories told of canoes in the East
Indies which have holes in their benches made for the tails of the
rowers. At one time in the East the presence of tails was taken as a
sign of brute force.
There was reported from Caracas the discovery of a tribe of Indians in
Paraguay who were provided with tails. The narrative reads somewhat
after this manner: One day a number of workmen belonging to Tacura Tuyn
while engaged in cutting grass had their mules attacked by some
Guayacuyan Indians. The workmen pursued the Indians but only succeeded
in capturing a boy of eight. He was taken to the house of Senor
Francisco Galeochoa at Posedas, and was there discovered to have a tail
ten inches long. On interrogation the boy stated that he had a brother
who had a tail as long as his own, and that all the tribe had tails.
Aetius, Bartholinus, Falk, Harvey, Kolping, Hesse, Paulinus, Strauss,
and Wolff give descriptions of tails. Blanchard says he saw a tail
fully a span in length: and there is a description in 1690 of a man by
the name of Emanuel Konig, a son of a doctor of laws who had a tail
half a span long, which grew directly downward from the coccyx and was
coiled on the perineum, causing much discomfort. Jacob describes a
pouch of skin resembling a tail which hung from the tip of the coccyx
to the length of six inches. It was removed and was found to be thicker
than the thumb, consisted of distinctly jointed portions with synovial
capsules. Gosselin saw at his clinic a caudal appendix in an infant
which measured about ten cm. Lissner says that in 1872 he assisted in
the delivery of a young girl who had a tail consisting of a coccyx
prolonged and covered with skin, and in 1884 he saw the same girl, at
this time the tail measuring nearly 13 cm.
Virchow received for examination a tail three inches long amputated
from a boy of eight weeks. Ornstein, chief physician of the Greek army,
describes a Greek of twenty-six who had a hairless, conical tail, free
only at the tip, two inches long and containing three vertebrae. He
also remarks that other instances have been observed in recruits. Thirk
of Broussa in 1820 described the tail of a Kurd of twenty-two which
contained four vertebrae. Belinovski gives an account of a hip-joint
amputation and extirpation of a fatty caudal extremity, the only one he
had ever observed.
Before the Berlin Anthropological Society there were presented two
adult male Papuans, in good health and spirits, who had been brought
from New Guinea; their coccygeal bones projected 1 1/2 inches. Oliver
Wendell Holmes in the Atlantic Monthly, June, 1890, says that he saw in
London a photograph of a boy with a considerable tail. The "Moi Boy"
was a lad of twelve, who was found in Cochin China, with a tail a foot
long which was simply a mass of flesh. Miller tells of a West Point
student who had an elongation of the coccyx, forming a protuberance
which bulged very visibly under the skin. Exercise at the riding school
always gave him great distress, and the protuberance would often chafe
until the skin was broken, the blood trickling into his boots.
Bartels presents a very complete article in which he describes 21
persons born with tails, most of the tails being merely fleshy
protuberances. Darwin speaks of a person with a fleshy tail and refers
to a French article on human tails.
Science contains a description of a negro child born near Louisville,
eight weeks old, with a pedunculated tail 2 1/2 inches long, with a
base 1 1/4 inches in circumference. The tail resembled in shape a pig's
tail and had grown 1/4 inch since birth. It showed no signs of
cartilage or bone, and had its origin from a point slightly to the left
of the median line and about an inch above the end of the spinal column.
Dickinson recently reported the birth of a child with a tail. It was a
well-developed female between 5 1/2 and six pounds in weight. The
coccyx was covered with the skin on both the anterior and posterior
surfaces. It thus formed a tail of the size of the nail of the little
finger, with a length of nearly 3/16 inch on the inner surface and 3/8
inch on the rear surface. This little tip could be raised from the body
and it slowly sank back.
In addition to the familiar caudal projection of the human fetus,
Dickinson mentions a group of other vestigial remains of a former state
of things. Briefly these are:--
(1) The plica semilunaris as a vestige of the nictitating membrane of
certain birds.
(2) The pointed ear, or the turned-down tip of the ears of many men.
(3) The atrophied muscles, such as those that move the ear, that are
well developed in certain people, or that shift the scalp, resembling
the action of a horse in ridding itself of flies.
(4) The supracondyloid foremen of the humerus.
(5) The vermiform appendix.
(6) The location and direction of the hair on the trunk and limbs.
(7) The dwindling wisdom-teeth.
(8) The feet of the fetus strongly deflected inward, as in the apes,
and persisting in the early months of life, together with great
mobility and a distinct projection of the great toe at an angle from
the side of the foot.
(9) The remarkable grasping power of the hand at birth and for a few
weeks thereafter, that permits young babies to suspend their whole
weight on a cane for a period varying from half a minute to two minutes.
Horrocks ascribes to these anal tags a pathologic importance. He claims
that they may be productive of fistula in ano, superficial ulcerations,
fecal concretions, fissure in ano, and that they may hypertrophy and
set up tenesmus and other troubles. The presence of human tails has
given rise to discussion between friends and opponents of the Darwinian
theory. By some it is considered a reversion to the lower species,
while others deny this and claim it to be simply a pathologic appendix.
Anomalies of the Spinal Canal and Contents.--When there is a default in
the spinal column, the vice of conformation is called spina bifida.
This is of two classes: first, a simple opening in the vertebral canal,
and, second, a large cleft sufficient to allow the egress of spinal
membranes and substance. Figure 130 represents a large congenital
sacral tumor.
Achard speaks of partial duplication of the central canal of the spinal
cord. De Cecco reports a singular case of duplication of the lumbar
segment of the spinal cord. Wagner speaks of duplication of a portion
of the spinal cord.
Foot records a case of amyelia, or absence of the spinal cord, in a
fetus with hernia cerebri and complete fissure of the spinal column.
Nicoll and Arnold describe an anencephalous fetus with absence of
spinal marrow; and Smith also records the birth of an amyelitic fetus.
In some persons there are exaggerated curvatures of the spine. The
first of these curvatures is called kyphosis, in which the curvature is
posterior; second, lordosis, in which the curvature is anterior; third,
scoliosis, in which it is lateral, to the right or left.
Kyphosis is the most common of the deviations in man and is most often
found in the dorsal region, although it may be in the lumbar region.
Congenital kyphosis is very rare in man, is generally seen in monsters,
and when it does exist is usually accompanied by lordosis or spine
bifida. We sometimes observe a condition of anterior curvature of the
lumbar and sacral regions, which might be taken for a congenital
lordosis, but this is really a deformity produced after birth by the
physiologic weight of the body. Figure 131 represents a case of
lordosis caused by paralysis of the spinal muscles.
Analogous to this is what the accoucheurs call spondylolisthesis.
Scoliosis may be a cervicodorsal, dorsolumbar, or lumbosacral curve,
and the inclination of the vertebral column may be to the right or
left. The pathologists divide scoliosis into a myopathic variety, in
which the trouble is a physiologic antagonism of the muscles; or
osteopathic, ordinarily associated with rachitis, which latter variety
is generally accountable for congenital scoliosis. In some cases the
diameter of the chest is shortened to an almost incredible degree, but
may yet be compatible with life. Glover speaks of an extraordinary
deformity of the chest with lateral curvature of the spine, in which
the diameter from the pit of the stomach to the spinal integument was
only 5 1/2 inches.
Supernumerary ribs are not at all uncommon in man, nearly every medical
museum having some examples. Cervical ribs are not rare. Gordon
describes a young man of seventeen in whom there was a pair of
supernumerary ribs attached to the cervical vertebrae. Bernhardt
mentions an instance in which cervical ribs caused motor and sensory
disturbances. Dumerin of Lyons showed an infant of eight days which had
an arrested development of the 2d, 3d, 4th, and 5th ribs. Cases of
deficient ribs are occasionally met. Wistar in 1818 gives an account
of a person in whom one side of the thorax was at rest while the other
performed the movements of breathing in the usual manner.
In some cases we see fissure of the sternum, caused either by deficient
union or absence of one of its constituent parts. In the most
exaggerated cases these fissures permit the exit of the heart, and as a
general rule ectopies of the heart are thus caused. Pavy has given a
most remarkable case of sternal fissure in a young man of twenty-five,
a native of Hamburg. He exhibited himself in one medical clinic after
another all over Europe, and was always viewed with the greatest
interest. In the median line, corresponding to the absence of sternum,
was a longitudinal groove bounded on either side by a continuous hard
ridge which articulated with the costal cartilages. The skin passed
naturally over the chest from one side to another, but was raised at
one part of the groove by a pulsatile swelling which occupied the
position of the right auricle. The clavicle and the two margins of the
sternum had no connections whatever, and below the groove was a hard
substance corresponding to the ensiform cartilage, which, however, was
very elastic, and allowed the patient, under the influence of the
pectoral muscles, when the upper extremity was fixed, to open the
groove to nearly the extent of three inches, which was more than twice
its natural width. By approximating his arms he made the ends of his
clavicles overlap. When he coughed, the right lung suddenly protruded
from the chest through the groove and ascended a considerable distance
above the clavicle into the neck. Between the clavicles another
pulsatile swelling was easily felt but hardly seen, which was doubtless
the arch of the aorta, as by putting the fingers on it one could feel a
double shock, synchronous with distention and recoil of a vessel or
opening and closing of the semilunar valves.
Madden pictures (Figs. 134 and 135) a Swede of forty with congenital
absence of osseous structure in the middle line of the sternum, leaving
a fissure 5 3/8 X 1 3/16 X 2 inches, the longest diameter being
vertical. Madden also mentions several analogous instances on record.
Groux's case was in a person of forty-five, and the fissure had the
vertical length of four inches. Hodgen of St. Louis reports a case in
which there was exstrophy of the heart through the fissure. Slocum
reports the occurrence of a sternal fissure 3 X 1 1/2 inches in an
Irishman of twenty-five. Madden also cites the case of Abbott in an
adult negress and a mother. Obermeier mentions several cases. Gibson
and Malet describe a presternal fissure uncovering the base of the
heart. Ziemssen, Wrany, and Williams also record congenital fissures
of the sternum.
Thomson has collected 86 cases of thoracic defects and summarizes his
paper by saying that the structures deficient are generally the hair in
the mammary and axillary regions, the subcutaneous fat over the
muscles, nipples, and breasts, the pectorals and adjacent muscles, the
costal cartilages and anterior ends of ribs, the hand and forearm; he
also adds that there may be a hernia of the lung, not hereditary, but
probably due to the pressure of the arm against the chest. De Marque
gives a curious instance in which the chin and chest were congenitally
fastened together. Muirhead cites an instance in which a firm, broad
strip of cartilage resembling sternomastoid extended from below the
left ear to the left upper corner of the sternum, being entirely
separate from the jaw.
Some preliminary knowledge of embryology is essential to understand the
formation of branchial fissures, and we refer the reader to any of the
standard works on embryology for this information. Dzondi was one of
the first to recognize and classify congenital fistulas of the neck.
The proper classification is into lateral and median fissures. In a
case studied by Fevrier the exploration of a lateral pharyngeal fistula
produced by the introduction of the sound violent reflex phenomena,
such as pallor of the face and irregular, violent beating of the heart.
The rarest of the lateral class is the preauricular fissure, which has
been observed by Fevrier, Le Dentu, Marchand, Peyrot, and Routier.
The median congenital fissures of the neck are probably caused by
defective union of the branchial arches, although Arndt thinks that he
sees in these median fistulas a persistence of the hypobranchial furrow
which exists normally in the amphioxus. They are less frequent than the
preceding variety.
The most typical form of malformation of the esophagus is imperforation
or obliteration. Van Cuyck of Brussels in 1824 delivered a child which
died on the third day from malnutrition. Postmortem it was found that
the inferior extremity of the esophagus to the extent of about two
inches was converted into a ligamentous cord. Porro describes a case of
congenital obliteration of the esophagus which ended in a cecal pouch
about one inch below the inferior portion of the glottidean aperture
and from this point to the stomach only measured an inch; there was
also tracheal communication. The child was noticed to take to the
breast with avidity, but after a little suckling it would cough, become
livid, and reject most of the milk through the nose, in this way almost
suffocating at each paroxysm; it died on the third day.
In some cases the esophagus is divided, one portion opening into the
bronchial or other thoracic organs. Brentano describes an infant dying
ten days after birth whose esophagus was divided into two portions, one
terminating in a culdesac, the other opening into the bronchi; the left
kidney was also displaced downward. Blasius describes an anomalous case
of duplication of the esophagus. Grashuys, and subsequently Vicq
d'Azir, saw a dilatation of the esophagus resembling the crop of a bird.
Anomalies of the Lungs.--Carper describes a fetus of thirty-seven weeks
in whose thorax he found a very voluminous thymus gland but no lungs.
These organs were simply represented by two little oval bodies having
no lobes, with the color of the tissue of the liver. The heart had only
one cavity but all the other organs were perfectly formed. This case
seems to be unique. Tichomiroff records the case of a woman of
twenty-four who died of pneumonia in whom the left lung was entirely
missing. No traces of a left bronchus existed. The subject was very
poorly developed physically. Tichomiroff finds four other cases in
literature, in all of which the left lung was absent. Theremin and
Tyson record cases of the absence of the left lung.
Supplementary pulmonary lobes are occasionally seen in man and are
taken by some authorities to be examples of retrogressive anomalies
tending to prove that the derivation of the human race is from the
quadrupeds which show analogous pulmonary malformation. Eckley reports
an instance of supernumerary lobe of the right lung in close connection
with the vena azygos major. Collins mentions a similar case. Bonnet
and Edwards speak of instances of four lobes in the right lung. Testut
and Marcondes report a description of a lung with six lobes.
Anomalies of the Diaphragm.--Diemerbroeck is said to have dissected a
human subject in whom the diaphragm and mediastinum were apparently
missing, but such cases must be very rare, although we frequently find
marked deficiency of this organ. Bouchand reports an instance of
absence of the right half of the diaphragm in an infant born at term.
Lawrence mentions congenital deficiency of the muscular fibers of the
left half of the diaphragm with displacement of the stomach. The
patient died of double pneumonia. Carruthers, McClintock, Polaillon,
and van Geison also record instances of congenital deficiency of part
of the diaphragm. Recently Dittel reported unilateral defect in the
diaphragm of an infant that died soon after birth. The stomach, small
intestines, and part of the large omentum lay in the left pleural
cavity; both the phrenic nerves were normal. Many similar cases of
diaphragmatic hernia have been observed. In such cases the opening may
be large enough to allow a great part of the visceral constituents to
pass into the thorax, sometimes seriously interfering with respiration
and circulation by the pressure which ensues. Alderson reports a fatal
case of diaphragmatic hernia with symptoms of pneumothorax. The
stomach, spleen, omentum, and transverse colon were found lying in the
left pleura. Berchon mentions double perforation of the diaphragm with
hernia of the epiploon. The most extensive paper on this subject was
contributed by Bodwitch, who, besides reporting an instance in the
Massachusetts General Hospital, gives a numerical analysis of all the
cases of this affection found recorded in the writings of medical
authors between the years 1610 and 1846. Hillier speaks of an instance
of congenital diaphragmatic hernia in which nearly all the small
intestines and two-thirds of the large passed into the right side of
the thorax. Macnab reports an instance in which three years after the
cure of empyema the whole stomach constituted the hernia. Recently Joly
described congenital hernia of the stomach in a man of thirty-seven,
who died from collapse following lymphangitis, persistent vomiting, and
diarrhea. At the postmortem there was found a defect in the diaphragm
on the left side, permitting herniation of the stomach and first part
of the duodenum into the left pleural cavity. There was no history of
traumatism to account for strangulation. Longworth cites an instance
of inversion of the diaphragm in a human subject. Bartholinus mentions
coalition of the diaphragm and liver; and similar cases are spoken of
by Morgagni and the Ephemerides. Hoffman describes diaphragmatic
junction with the lung.
Anomalies of the Stomach.--The Ephemerides contains the account of a
dissection in which the stomach was found wanting, and also speaks of
two instances of duplex stomach. Bartholinus, Heister, Hufeland,
Morgagni, Riolan, and Sandifort cite examples of duplex stomach. Bonet
speaks of a case of vomiting which was caused by a double stomach.
Struthers reports two cases in which there were two cavities to the
stomach. Struthers also mentions that Morgagni, Home, Monro, Palmer,
Larry, Blasius, Hufeland, and Walther also record instances in which
there was contraction in the middle of the stomach, accounting for
their instances of duplex stomach. Musser reports an instance of
hour-glass contraction of the stomach. Hart dissected the stomach of a
woman of thirty which resembled the stomach of a predaceous bird, with
patches of tendon on its surface. The right extremity instead of
continuously contracting ended in a culdesac one-half as large as the
greater end of the stomach. The duodenum proceeded from the depression
marking the lesser arch of the organ midway between the cardiac orifice
and the right extremity. Crooks speaks of a case in which the stomach
of an infant terminated in a culdesac.
Hernia of the stomach is not uncommon, especially in diaphragmatic or
umbilical deficiency. There are many cases on record, some terminating
fatally from strangulation or exposure to traumatism. Paterson reports
a case of congenital hernia of the stomach into the left portion of the
thoracic cavity. It was covered with fat and occupied the whole left
half of the thoracic cavity. The spleen, pancreas, and transverse colon
were also superior to the diaphragm. Death was caused by a well-defined
round perforation at the cardiac curvature the size of a sixpence.
Anomalies of the Intestines.--The Ephemerides contains the account of
an example of double cecum, and Alexander speaks of a double colon, and
there are other cases of duplication of the bowel recorded. There is an
instance of coalition of the jejunum with the liver, and Treuner
parallels this case. Aubery, Charrier Poelman, and others speak of
congenital division of the intestinal canal. Congenital occlusion is
quite frequently reported.
Dilatation of the colon frequently occurs as a transient affection, and
by its action in pushing up the diaphragm may so seriously interfere
with the action of the heart and lungs as to occasionally cause
heart-failure. Fenwick has mentioned an instance of this nature.
According to Osler there is a chronic form of dilatation of the colon
in which the gut may reach an enormous size. The coats may be
hypertrophied without evidence of any special organic change in the
mucosa. The most remarkable instance has been reported by Formad. The
patient, known as the "balloon-man," aged twenty-three at the time of
his death, had had a distended abdomen from infancy. Postmortem the
colon was found as large as that of an ox, the circumference ranging
from 15 to 30 inches. The weight of the contents was 47 pounds. Cases
are not uncommon in children. Osler reports three well-marked cases
under his care. Chapman mentions a case in which the liver was
displaced by dilatation of the sigmoid flexure. Mya reports two cases
of congenital dilatation and hypertrophy of the colon (megacolon
congenito). Hirsohsprung, Genersich, Faralli, Walker, and Griffiths all
record similar instances, and in all these cases the clinical features
were obstinate constipation and marked meteorismus.
Imperforate Anus.--Cases in which the anus is imperforate or the rectum
ends in a blind pouch are occasionally seen. In some instances the
rectum is entirely absent, the colon being the termination of the
intestinal tract. There are cases on record in which the rectum
communicated with the anus solely by a fibromuscular cord. Anorectal
atresia is the ordinary imperforation of the anus, in which the rectum
terminates in the middle of the sacral cavity. The rectum may be
deficient from the superior third of the sacrum, and in this position
is quite inaccessible for operation.
A compensatory coalition of the bowel with the bladder or urethra is
sometimes present, and in these cases the feces are voided by the
urinary passages. Huxham mentions the fusion of the rectum and colon
with the bladder, and similar instances are reported by Dumas and
Baillie. Zacutus Lusitanus describes an infant with an imperforate
membrane over its anus who voided feces through the urethra for three
months. After puncture of the membrane, the discharge came through the
natural passage and the child lived; Morgagni mentions a somewhat
similar case in a little girl living in Bologna, and other modern
instances have been reported. The rectum may terminate in the vagina.
Masters has seen a child who lived nine days in whom the sigmoid
flexure of the colon terminated in the fundus of the bladder. Guinard
pictures a case in which there was communication between the rectum and
the bladder. In Figure 140 a represents the rectum; b the bladder; c
the point of communication; g shows the cellular tissue of the scrotum.
There is a description of a girl of fourteen, otherwise well
constituted and healthy, who had neither external genital organs nor
anus. There was a plain dermal covering over the genital and anal
region. She ate regularly, but every three days she experienced pain in
the umbilicus and much intestinal irritation, followed by severe
vomiting of stercoraceous matter; the pains then ceased and she
cleansed her mouth with aromatic washes, remaining well until the
following third day. Some of the urine was evacuated by the mammae. The
examiners displayed much desire to see her after puberty to note the
disposition of the menstrual flow, but no further observation of her
case can be found.
Fournier narrates that he was called by three students, who had been
trying to deliver a woman for five days. He found a well-constituted
woman of twenty-two in horrible agony, who they said had not had a
passage of the bowels for eight days, so he prescribed an enema. The
student who was directed to give the enema found to his surprise that
there was no anus, but by putting his finger in the vagina he could
discern the floating end of the rectum, which was full of feces. There
was an opening in this suspended rectum about the size of an
undistended anus. Lavage was practiced by a cannula introduced through
the opening, and a great number of cherry stones agglutinated with
feces followed the water, and labor was soon terminated. The woman
afterward confessed that she was perfectly aware of her deformity, but
was ashamed to disclose it before. There was an analogue of this case
found by Mercurialis in a child of a Jew called Teutonicus.
Gerster reports a rare form of imperforate anus, with malposition of
the left ureter, obliteration of the ostia of both ureters, with
consequent hydronephrosis of a confluent kidney. There was a minute
opening into the bladder, which allowed the passage of meconium through
the urethra. Burge mentions the case of what he calls "sexless child,"
in which there was an imperforate anus and no pubic arch; the ureters
discharged upon a tumor the size of a teacup extending from the
umbilicus to the pubes. A postmortem examination confirmed the
diagnosis of sexless child.
The Liver.--The Ephemerides, Frankenau, von Home, Molinetti, Schenok,
and others speak of deficient or absent liver. Zacutus Lusitanus says
that he once found a mass of flesh in place of the liver. Lieutaud is
quoted as describing a postmortem examination of an adult who had died
of hydropsy, in whom the liver and spleen were entirely missing. The
portal vein discharged immediately into the vena cava; this case is
probably unique, as no authentic parallel could be found.
Laget reports an instance of supernumerary lobe in the liver. Van Buren
describes a supernumerary liver. Sometimes there is rotation, real or
apparent, caused by transposition of the characteristics of the liver.
Handy mentions such a case. Kirmisson reports a singular anomaly of
the liver which he calls double displacement by interversion and
rotation on the vertical axis. Actual displacements of the liver as
well as what is known as wandering liver are not uncommon. The
operation for floating liver will be spoken of later.
Hawkins reports a case of congenital obliteration of the ductus
communis choledochus in a male infant which died at the age of four and
a half months. Jaundice appeared on the eighth day and lasted through
the short life. The hepatic and cystic ducts were pervious and the
hepatic duct obliterated. There were signs of hepatic cirrhosis and in
addition an inguinal hernia.
The Gall-Bladder.--Harle mentions the case of a man of fifty, in whom
he could find no gall-bladder; Patterson has seen a similar instance in
a men of twenty-five. Purser describes a double gall-bladder.
The spleen has been found deficient or wanting by Lebby, Ramsay, and
others, but more frequently it is seen doubled. Cabrolius, Morgagni,
and others have found two spleens in one subject; Cheselden and
Fallopius report three; Fantoni mentions four found in one subject;
Guy-Patin has seen five, none as large as the ordinary organ;
Hollerius, Kerckringius, and others have remarked on multiple spleens.
There is a possibility that in some of the cases of multiple spleens
reported the organ is really single but divided into several lobes.
Albrecht mentions a case shown at a meeting of the Vienna Medical
Society of a very large number of spleens found in the mesogastrium,
peritoneum, on the mesentery and transverse mesocolon, in Douglas'
pouch, etc. There was a spleen "the size of a walnut" in the usual
position, with the splenic artery and vein in their normal position.
Every one of these spleens had a capsule, was covered by peritoneum,
and exhibited the histologic appearance of splenic tissue. According to
the review of this article, Toldt explains the case by assuming that
other parts of the celomic epithelium, besides that of the
mesogastrium, are capable of forming splenic tissue. Jameson reports a
case of double spleen and kidneys. Bainbrigge mentions a case of
supernumerary spleen causing death from the patient being placed in the
supine position in consequence of fracture of the thigh. Peevor
mentions an instance of second spleen. Beclard and Guy-Patin have seen
the spleen congenitally misplaced on the right side and the liver on
the left; Borellus and Bartholinus with others have observed
misplacement of the spleen.
The Pancreas.--Lieutaud has seen the pancreas missing and speaks of a
double pancreatic duct that he found in a man who died from starvation;
Bonet speaks of a case similar to this last.
There are several cases of complete transposition of the viscera on
record. This bizarre anomaly was probably observed first in 1650 by
Riolanus, but the most celebrated case was that of Morand in 1660, and
Mery described the instance later which was the subject of the
following quatrain:--
"La nature, peu sage et sans douse en debauche Placa le foie au cote
gauche, Et de meme, vice versa Le coeur a le droite placa."
Young cites an example in a woman of eighty-five who died at
Hammersmith, London. She was found dead in bed, and in a postmortem
examination, ordered to discover if possible the cause of death, there
was seen complete transposition of the viscera. The heart lay with its
base toward the left, its apex toward the right, reaching the lower
border of the 4th rib, under the right mamma. The vena cava was on the
left side and passed into the pulmonary cavity of the heart, which was
also on the left side, the aorta and systemic ventricle being on the
right. The left splenic vein was lying on the superior vena cava, the
liver under the left ribs, and the spleen on the right side underneath
the heart. The esophagus was on the right of the aorta, and the
location of the two ends of the stomach was reversed; the sigmoid
flexure was on the right side. Davis describes a similar instance in a
man.
Herrick mentions transposition of viscera in a man of twenty-five.
Barbieux cites a case of transposition of viscera in a man who was
wounded in a duel. The liver was to the left and the spleen and heart
to the right etc. Albers, Baron, Beclard, Boyer, Bull, Mackensie,
Hutchinson, Hunt, Murray, Dareste, Curran, Duchesne, Musser, Sabatier,
Shrady, Vulpian, Wilson, and Wehn are among others reporting instances
of transposition and inversion of the viscera.
Congenital extroversion or eventration is the result of some congenital
deficiency in the abdominal wall; instances are not uncommon, and some
patients live as long as do cases of umbilical hernia proper. Ramsey
speaks of entire want of development of the abdominal parietes.
Robertson, Rizzoli, Tait, Hamilton, Brodie, Denis, Dickie, Goyrand, and
many others mention extroversion of viscera from parietal defects. The
different forms of hernia will be considered in another chapter.
There seem to be no authentic cases of complete absence of the kidney
except in the lowest grades of monstrosities. Becker, Blasius, Rhodius,
Baillie, Portal, Sandifort, Meckel, Schenck, and Stoll are among the
older writers who have observed the absence of one kidney. In a recent
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
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
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 scrotum apparently perfect, with median
rapine; a careful search failed to show any trace of a penis; on the
anterior wall of the rectum four lines above the anus was an orifice
which gave vent to urine; the right testicle and cord were normal, but
there was an acute orchitis in the left. Starting from just in front of
the anal 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 rectum. It was
possible to pass a sound through the opening in the rectum 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 penis have been
recorded, most of them complicated with cryptorchism or other
abnormality of the sexual 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 penis
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 penis, suddenly terminating at this point. Nothing
indicative of a prostate could be found. The testicles were at the
entrance of the inguinal canal 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 penis was of about the size of
the little finger, and contained on each side testicles not larger than
a pea. There was no hair on the pubes 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 penis and testicles hardly exceeded those
of a boy of eight. He had never felt desire for sexual 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 penises
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 circumferences are 4 3/4
and 4 1/4 inches. Between these two we can strike an average of the
size of the normal penis.
In some instances the penis is so large as to forbid coitus and even
inconvenience its possessor, measuring as much as ten or even more
inches in length. Extraordinary cases of large penis are reported by
Albinus (who mentions it as a cause for sterility), Bartholinus,
Fabricius Hildanus, Paullini, Peyer, Plater, Schurig, Sinibaldus, and
Zacchias. Several cases of enormous penises in the new-born have been
observed by Wolff and others.
The penis palme, or suture de la verge of the French, is the name given
to those examples of single cutaneous envelope for both the testicles
and penis; the penis is adherent to the scrotum by its inferior face;
the glans only is free and erection is impossible. Chretien cites an
instance in a man of twenty-five, and Schrumpf of Wesserling describes
an example of this rare anomaly. The penis and testes were inclosed in
a common sac, a slight projection not over 1/4 inch long being seen
from the upper part of this curious scrotum. When the child was a year
old a plastic operation was performed on this anomalous member with a
very satisfactory result. Petit describes an instance in which the
penis was slightly fused with the scrotum.
There are many varieties of torsion of the penis. The glans itself may
be inclined laterally, the curvature may be total, or there may be a
veritable rotation, bringing the inferior face above and the superior
face below. Gay describes a child with epispadias whose penis had
undergone such torsion on its axis that its inferior surface looked
upward to the left, and the child passed urine toward the left
shoulder. Follin mentions a similar instance in a boy of twelve with
complete epispadias, and Verneuil and Guerlin also record cases, both
complicated with associate maldevelopment. Caddy mentions a youth of
eighteen who had congenital torsion of the penis with out hypospadias
or epispadias. There was a complete half-turn to the left, so that the
slit-like urinary meatus was reversed and the frenum was above. Among
the older writers who describe incurvation or torsion of the penis are
Arantius, the Ephemerides, Haenel, Petit, Schurig, Tulpius, and
Zacchias.
Zacutus Lusitans speaks of torsion of the penis from freezing.
Paullini mentions a case the result of masturbation, and Hunter speaks
of torsion of the penis associated with arthritis.
Ossification of the Penis.--MacClellann speaks of a man of fifty-two
whose penis was curved and distorted in such a manner that urine could
not be passed without pain and coitus was impossible. A bony mass was
discovered in the septum between the corpora cavernosa; this was
dissected out with much hemorrhage and the upward curvature was
removed, but there resulted a slight inclination in the opposite
direction. The formation of bone and cartilage in the penis is quite
rare. Velpeau, Kauffmann, Lenhoseck, and Duploy are quoted by Jacobson
as having seen this anomaly. There is an excellent preparation in
Vienna figured by Demarquay, but no description is given. The
Ephemerides and Paullini describe osseous penises.
The complete absence of the frenum and prepuce has been observed in
animals but is very rare in man. The incomplete or irregular
development is more frequent, but most common is excessive development
of the prepuce, constituting phimosis, when there is abnormal adherence
with the glans. Instances of phimosis, being quite common, will be
passed without special mention. Deficient or absent prepuce has been
observed by Blasius, Marcellus Donatus, and Gilibert. Partial
deficiency is described by Petit Severinus, and others.
There may be imperforation or congenital occlusion of some portion of
the urethra, causing enormous accumulation of urine in the bladder, but
fortunately there is generally in such cases some anomalous opening of
the urethra giving vent to the excretions. Tulpius mentions a case of
deficient urethra. In the Ephemerides there is an account of a man who
had a constant flow of semen from an abnormal opening in the abdomen.
La Peyroma describes a case of impotence due to ejaculation of the
spermatic ducts into the bladder instead of into the urethra, but
remarks that there was a cicatrix of a wound of the neighboring parts.
There are a number of instances in which the urethra has terminated in
the rectum. Congenital dilatation of the urethral canal is very rare,
and generally accompanied by other malformation.
Duplication of the urethra or the existence of two permeable canals is
not accepted by all the authors, some of whom contend that one of the
canals either terminates in a culdesac or is not separate in itself.
Verneuil has published an article clearly exposing a number of cases,
showing that it is possible for the urethra to have two or more canals
which are distinct and have separate functions. Fabricius Hildanus
speaks of a double aperture to the urethra; Marcellus Donatus describes
duplicity of the urethra, one of the apertures being in the testicle;
and there is another case on record in which there was a urethral
aperture in the groin. A case of double urethra in a man of twenty-five
living in Styria who was under treatment for gonorrhea is described,
the supernumerary urethra opening above the natural one and receiving a
sound to the depth of 17 cm. There was purulent gonorrhea in both
urethrae. Vesalius has an account of a double urethral aperture, one of
which was supposed to give spermatic fluid and the other urine.
Borellus, Testa, and Cruveilhier have reported similar instances.
Instances of double penis have been discussed under the head of
diphallic terata, page 194.
Hypospadias and epispadias are names given to malformations of the
urethra in which the wall of the canal is deficient either above or
below. These anomalies are particularly interesting, as they are nearly
always found in male hermaphrodites, the fissure giving the appearance
of a vulva, as the scrotum is sometimes included, and even the perineum
may be fissured in continuity with the other parts, thus exaggerating
the deception. There seems to be an element of heredity in this
malformation, and this allegation is exemplified by Sedgwick, who
quotes a case from Heuremann in which a family of females had for
generations given birth to males with hypospadias. Belloc mentions a
man whose urethra terminated at the base of the frenum who had four
sons with the same deformity. Picardat mentions a father and son, both
of whom had double urethral orifices, one above the other, from one of
which issued urine and from the other semen--a fact that shows the
possibility of inheritance of this malformation. Patients in whom the
urethra opens at the root of the penis, the meatus being imperforate,
are not necessarily impotent; as, for instance, Fournier knew of a man
whose urethra opened posteriorly who was the father of four children.
Fournier supposed that the semen ejaculated vigorously and followed the
fissure on the back of the penis to the uterus, the membrane of the
vagina supplanting the deficient wall of the urethra. The penis was
short, but about as thick as ordinary.
Gray mentions a curious case in a man afflicted with hypospadias who,
suffering with delusions, was confined in the insane asylum at Utica.
When he determined to get married, fully appreciating his physical
defect, he resolved to imitate nature, and being of a very ingenious
turn of mind, he busied himself with the construction of an artificial
penis. While so engaged he had seized every opportunity to study the
conformation of this organ, and finally prepared a body formed of
cotton, six inches in length, and shaped like a penis, minus a prepuce.
He sheathed it in pig's gut and gave it a slight vermilion hue. To the
touch it felt elastic, and its shape was maintained by a piece of
gutta-percha tubing, around which the cotton was firmly wound. It was
fastened to the waist-band by means of straps, a central and an upper
one being so arranged that the penis could be thrown into an erect
position and so maintained. He had constructed a flesh-colored covering
which completely concealed the straps. With this artificial member he
was enabled to deceive his wife for fifteen months, and was only
discovered when; she undressed him while he was in a state of
intoxication. To further the deception he had told his wife immediately
after their marriage that it was quite indecent for a husband to
undress in the presence of his wife, and therefore she had always
retired first and turned out the light. Partly from fear that his
virile power would be questioned and partly from ignorance, the
duration of actual coitus would approach an hour. When the discovery
was made, his wife hid the instrument with which he had perpetrated a
most successful fraud upon her, and the patient subsequently attempted
coitus by contact with unsuccessful results, although both parties had
incomplete orgasms. Shortly afterward evidences of mental derangement
appeared and the man became the subject of exalted delusions. His wife,
at the time of report, had filed application for divorce. Haslam
reports a case in which loss of the penis was compensated for by the
use of an ivory succedaneum. Parallel instances of this kind have been
recorded by Ammann and Jonston.
Entire absence of the male sexual apparatus is extremely rare, but
Blondin and Velpeau have reported cases.
Complete absence of the testicles, or anorchism, is a comparatively
rare anomaly, and it is very difficult to distinguish between anorchism
and arrest of development, or simple atrophy, which is much more
common. Fisher of Boston describes the case of a man of forty-five, who
died of pneumonia. From the age of puberty to twenty-five, and even to
the day of death, his voice had never changed and his manners were
decidedly effeminate. He always sang soprano in concert with females.
After the age of twenty-five, however, his voice became more grave and
he could not accompany females with such ease. He had no beard, had
never shaved, and had never exhibited amorous propensities or desire
for female society. When about twenty-one he became associated with a
gay company of men and was addicted to the cup, but would never visit
houses of ill-fame. On dissection no trace of testicles could be found;
the scrotum was soft and flabby. The cerebellum was the exact size of
that of a female child.
Individuals with one testicle are called monorchids, and may be divided
into three varieties:--
(1) A solitary testicle divided in the middle by a deep fissure, the
two lobes being each provided with a spermatic cord on the same side as
the lobe.
(2) Testicles of the same origin, but with coalescence more general.
(3) A single testicle and two cords.
Gruber of St. Petersburg held a postmortem on a man in January, 1867,
in whom the right half of the scrotum, the right testicle, epididymis,
and the scrotal and inguinal parts of the right vas deferens were
absent. Gruber examined the literature for thirty years up to the time
of his report, and found 30 recorded postmortem examinations in which
there was absence of the testicle, and in eight of these both testicles
were missing. As a rule, natural eunuchs have feeble bodies, are
mentally dull, and live only a short time. The penis is ordinarily
defective and there is sometimes another associate malformation. They
are not always disinclined toward the opposite sex.
Polyorchids are persons who have more than two testicles. For a long
time the abnormality was not believed to exist, and some of the
observers denied the proof by postmortem examination of any of the
cases so diagnosed, but there is at present no doubt of the
fact,--three, four, and five testicles having been found at autopsies.
Russell, one of the older writers on the testicle, mentions a monk who
was a triorchid, and was so salacious that his indomitable passion
prevented him from keeping his vows of chastity. The amorous
propensities and generative faculties of polyorchids have always been
supposed greater than ordinary. Russell reports another case of a man
with a similar peculiarity, who was prescribed a concubine as a
reasonable allowance to a man thus endowed.
Morgagni and Meckel say that they never discovered a third testicle in
dissections of reputed triorchids, and though Haller has collected
records of a great number of triorchids, he has never been able to
verify the presence of the third testicle on dissection. Some authors,
including Haller, have demonstrated heredity in examples of
polyorchism. There is an old instance in which two testicles, one above
the other, were found on the right side and one on the left. Macann
describes a recruit of twenty, whose scrotum seemed to be much larger
on the right than on the left side, although it was not pendulous. On
dissection a right and left testicle were found in their normal
positions, but situated on the right side between the groin and the
normal testicle was a supernumerary organ, not in contact, and having a
separate and short cord. Prankard also describes a man with three
testicles. Three cases of triorchidism were found in recruits in the
British Army. Lane reports a supernumerary testis found in the right
half of the scrotum of a boy of fifteen. In a necropsy held on a man
killed in battle, Hohlberg discovered three fully developed testicles,
two on the right side placed one above the other. The London Medical
Record of 1884 quotes Jdanoff of St. Petersburg in mentioning a
soldier of twenty-one who had a supernumerary testicle erroneously
diagnosed as inguinal hernia. Quoted by the same reference, Bulatoff
mentions a soldier who had a third testicle, which diagnosis was
confirmed by several of his confreres. They recommended dismissal of
the man from the service, as the third testicle, usually resting in
some portion of the inguinal canal, caused extra exposure to traumatic
influence.
Venette gives an instance of four testicles, and Scharff, in the
Ephemerides, mentions five; Blasius mentions more than three testicles,
and, without citing proof, Buffon admits the possibility of such
occurrence and adds that such men are generally more vigorous.
Russell mentions four, five, and even six testicles in one individual;
all were not verified on dissection. He cites an instance of six
testicles four of which were of usual size and two smaller than
ordinary.
Baillie, the Ephemerides, and Schurig mention fusion of the testicles,
or synorchidism, somewhat after the manner of the normal disposition of
the batrachians and also the kangaroos, in the former of which the
fusion is abdominal and in the latter scrotal. Kerckring has a
description of an individual in whom the scrotum was absent.
In those cases in which the testicles are still in the abdominal cavity
the individuals are termed cryptorchids. Johnson has collected the
results of postmortem examinations of 89 supposed cryptorchids. In
eight of this number no testicles were found postmortem, the number
found in the abdomen was uncertain, but in 18 instances both testicles
were found in the inguinal canal, and in eight only one was found in
the inguinal canal, the other not appearing. The number in which the
semen was examined microscopically was 16, and in three spermatozoa
were found in the semen; one case was dubious, spermatozoa being found
two weeks afterward on a boy's shirt. The number having children was
ten. In one case a monorchid generated a cryptorchid child. Some of the
cryptorchids were effeminate, although others were manly with good
evidences of a beard. The morbid, hypochondriac, the voluptuous, and
the imbecile all found a place in Johnson's statistics; and although
there are evidences of the possession of the generative function,
still, we are compelled to say that the chances are against fecundity
of human cryptorchids. In this connection might be quoted the curious
case mentioned by Geoffroy-Saint-Hilaire, of a soldier who was hung for
rape. It was alleged that no traces of testicles were found externally
or internally yet semen containing spermatozoa was found in the seminal
vesicles. Spermatozoa have been found days and weeks after castration,
and the individuals during this period were capable of impregnation,
but in these cases the reservoirs were not empty, although the spring
had ceased to flow. Beigel, in Virchow's Archives, mentions a
cryptorchid of twenty-two who had nocturnal emissions containing
spermatozoa and who indulged in sexual congress. Partridge describes a
man of twenty-four who, notwithstanding his condition, gave evidences
of virile seminal flow.
In some cases there is anomalous position of the testicle. Hough
mentions an instance in which, from the great pain and sudden
appearance, a small tumor lying against the right pubic bone was
supposed to be a strangulated hernia. There were two well-developed
testicles in the scrotum, and the hernia proved to be a third. McElmail
describes a soldier of twenty-nine, who two or three months before
examination felt a pricking and slight burning pain near the internal
aperture of the internal inguinal canal, succeeded by a swelling until
the tumor passed into the scrotum. It was found in the upper part of
the scrotum above the original testicle, but not in contact, and was
about half the size of the normal testicle; its cord and epididymis
could be distinctly felt and caused the same sensation as pressure on
the other testicle did.
Marshall mentions a boy of sixteen in whom the right half of the
scrotum was empty, although the left was of normal size and contained a
testicle. On close examination another testicle was found in the
perineum; the boy said that while running he fell down, four years
before, and on getting up suffered great pain in the groin, and this
pain recurred after exertion. This testicle was removed successfully to
the scrotum. Horsley collected 20 instances of operators who made a
similar attempt, Annandale being the first one; his success was likely
due to antisepsis, as previously the testicles had always sloughed.
There is a record of a dog remarkable for its salacity who had two
testicles in the scrotum and one in the abdomen; some of the older
authors often indulged in playful humor on this subject.
Brown describes a child with a swelling in the perineum both painful
and elastic to the touch. The child cried if pressure was applied to
the tumor and there was every evidence that the tumor was a testicle.
Hutcheson, quoted by Russell, has given a curious case in an English
seaman who, as was the custom at that time, was impressed into service
by H.M.S. Druid in 1807 from a trading ship off the coast of Africa.
The man said he had been examined by dozens of ship-surgeons, but was
invariably rejected on account of rupture in both groins. The scrotum
was found to be an empty bag, and close examination showed that the
testicles occupied the seats of the supposed rupture. As soon as the
discovery was made the man became unnerved and agitated, and on
re-examining the parts the testicles were found in the scrotum. When
he found that there was no chance for escape he acknowledged that he
was an impostor and gave an exhibition in which, with incredible
facility, he pulled both testes up from the bottom of the scrotum to
the external abdominal ring. At the word of command he could pull up
one testicle, then another, and let them drop simultaneously; he
performed other like feats so rapidly that the movements could not be
distinguished.
In this connection Russell speaks of a man whose testicle was elevated
every time the east wind blew, which caused him a sense of languor and
relaxation; the same author describes a man whose testicles ascended
into the inguinal canal every time he was in the company of women.
Inversion of the testicle is of several varieties and quite rare, it
has been recognized by Sir Astley Cooper, Boyer, Maisonneuve, Royet,
and other writers.
The anomalies of the vas deferens and seminal vesicles are of little
interest and will be passed with mention of the case of Weber, who
found the seminal vesicles double; a similar conformation has been seen
in hermaphrodites.
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