MISCELLANEOUS SURGICAL ANOMALIES. 10 page

accompanying illustration shows the condition of the limb of a girl of

twenty-one, the subject of lymphedema, five years after the inception

of the disease. The changes in the limb were as yet moderate. The

photograph from which the cut was made was taken in 1875 At the present

time (seventeen years later) the case presents the typical condition of

the worst form of elephantiasis. Repeated attacks of lymphangitis have

occurred during this period, each producing an aggravation of the

previous condition. The leg below the knee has become enormously

deformed by the production of the elephantoid masses; the outer side of

the thigh remains healthy, but the skin of the inner side has developed

so as to form a very large and pendant lobulated mass. A similar

condition has begun to develop in the other leg, which is row about in

the condition of the first, as shown in the figure. Figure 273

represents this disease in its most aggravated form, a condition rarely

observed in this country. As an example of the change in the weight of

a person after the inception of this disease, we cite a case reported

by Griffiths. The patient was a woman of fifty-two who, five years

previous, weighed 148 pounds. The elephantoid change was below the

waist, yet at the time of report the woman weighed 387 pounds. There

was little thickening of the skin. The circumference of the calf was 28

inches; of the thigh, 38 inches; and of the abdomen, 80 inches; while

that of the arm was only 15 inches.

The condition commonly known as "Barbadoes leg" is a form of

elephantiasis deriving its name from its relative frequency in

Barbadoes.

Figure 275 represents a well-known exhibitionist who, from all

appearances, is suffering from an elephantoid hypertrophy of the lower

extremities, due to a lymphedema. Quite a number of similar

exhibitionists have been shown in recent years, the most celebrated of

whom was Falmy Mills, one of whose feet alone was extensively involved,

and was perhaps the largest foot ever seen.

Elephantiasis seldom attacks the upper extremities. Of the older cases

Rayer reports four collected by Alard. In one case the hard and

permanent swelling of the arm occurred after the application of a

blister; in another the arm increased so that it weighed more than 200

Genoese pounds, 40 of which consisted of serum. The swellings of the

arm and forearm resembled a distended bladder. The arteries, veins,

and nerves had not undergone any alteration, but the lymphatics were

very much dilated and loaded with lymph.

The third case was from Fabricius Hildanus, and the fourth from Hendy.

Figure 276 represents a remarkable elephantoid change in the hand of an

elderly German woman. Unfortunately there is no medical description of

the case on record, but the photograph is deemed worthy of reproduction.

Terry describes a French mulatto girl of eleven whose left hand was

enormously increased in weight and consistency, the chief enlargement

being in the middle finger, which was 6 1/2 inches long, and 5 1/2

inches about the nail, and 8 1/2 around the base of the finger. The

index finger was two inches thick and four inches long, twisted and

drawn, while the other fingers were dwarfed. The elephantiasis in this

case slowly and gradually increased in size until the hand weighed 3

1/2 pounds. The skin of the affected finger, contrary to the general

appearance of a part affected with elephantiasis, was of normal color,

smooth, shiny, showed no sensibility, and the muscles had undergone

fatty degeneration. It was successfully amputated in August, 1894. The

accompanying illustration shows a dorsal view of the affected hand.

Magalhaes of Rio Janeiro reports a very interesting case of

elephantiasis of the scalp, representing dermatolysis, in which the

fold of hypertrophied skin fell over the face like the hide of an

elephant, somewhat similar in appearance to the "elephant-man." Figure

279 represents a somewhat similar hypertrophic condition of the scalp

and face reported in the Photographic Review of Medicine and Surgery,

1870.

Elephantiasis of the face sometimes only attacks it on one side. Such

a case was reported by Alard, in which the elephantiasis seems to have

been complicated with eczema of the ear. Willier, also quoted by Alard,

describes a remarkable case of elephantiasis of the face. After a

debauch this patient experienced violent pain in the left cheek below

the zygomatic arch; this soon extended under the chin, and the

submaxillary glands enlarged and became painful; the face swelled and

became erythematous, and the patient experienced nausea and slight

chills. At the end of six months there was another attack, after which

the patient perceived that the face continued puffed. This attack was

followed by several others, the face growing larger and larger. In

similar cases tumefaction assumes enormous proportions, and Schenck

speaks of a man whose head exceeded that of an ox in size, the lower

part of the face being entirely covered with the nose, which had to be

raised to enable its unhappy owner to breathe.

Rayer cites two instances in which elephantiasis of the breast enlarged

these organs to such a degree that they hung to the knees. Salmuth

speaks of a woman whose breasts increased to such a size that they hung

down to her knees. At the same time she had in both axillae glandular

tumors as large as the head of a fetus. Borellus also quotes the case

of a woman whose breasts became so large that it was necessary to

support them by straps, which passed over the shoulders and neck.

Elephantiasis is occasionally seen in the genital regions of the

female, but more often in the scrotum of the male, in which location it

produces enormous tumors, which sometimes reach to the ground and

become so heavy as to prevent locomotion. This condition is curious in

the fact that these immense tumors have been successfully removed, the

testicles and penis, which had long since ceased to be distinguished,

saved, and their function restored. Alibert mentions a patient who was

operated upon by Clot-Bey, whose scrotum when removed weighed 110

pounds; the man had two children after the disease had continued for

thirteen years, but before it had obtained its monstrous development--a

proof that the functions of the testicles had not been affected by the

disease.

There are several old accounts of scrotal tumors which have evidently

been elephantoid in conformation. In the Ephemerides in 1692 there was

mentioned a tumor of the scrotum weighing 200 pounds. In the West

Indies it was reported that rats have been known to feed on these

enormous tumors, while the deserted subjects lay in a most helpless

condition. Larrey mentioned a case of elephantiasis of the scrotum in

which the tumor weighed over 200 pounds. Sir Astley Cooper removed a

tumor of 56 pounds weight from a Chinese laborer. It extended from

beneath the umbilicus to the anterior border of the anus; it had begun

in the prepuce ten years previously. Clot-Bey removed an elephantoid

tumor of the scrotum weighing 80 pounds, performing castration at the

same time. Alleyne reports a case of elephantiasis, in which he

successfully removed a tumor of the integuments of the scrotum and

penis weighing 134 pounds.

Bicet mentions a curious instance of elephantiasis of the penis and

scrotum which had existed for five years. The subject was in great

mental misery and alarm at his unsightly condition. The parts of

generation were completely buried in the huge mass. An operation was

performed in which all of the diseased structures that had totally

unmanned him were removed, the true organs of generation escaping

inviolate. Thebaud mentions a tumor of the scrotum, the result of

elephantiasis, which weighed 63 1/2 pounds. The weight was ascertained

by placing the tumor on the scales, and directing the patient to squat

over them without resting any weight of the body on the scales. This

man could readily feel his penis, although his surgeons could not do

so. The bladder was under perfect control, the urine flowing over a

channel on the exterior of the scrotum, extending 18 inches from the

meatus. Despite his infirmity this patient had perfect sexual desire,

and occasional erections and emissions. A very interesting operation

was performed with a good recovery.

Partridge reports an enormous scrotal tumor which was removed from a

Hindoo of fifty-five, with subsequent recovery of the subject. The

tumor weighed 111 1/2 pounds. The ingenious technic of this operation

is well worth perusal by those interested. Goodman successfully

removed an elephantiasis of the scrotum from a native Fiji of

forty-five. The tumor weighed 42 pounds, without taking into

consideration the weight of the fluid which escaped in abundance during

the operation and also after the operation, but before it was weighed.

Van Buren and Keyes mention a tumor of the scrotum of this nature

weighing 165 pounds. Quoted by Russell, Hendy describes the case of a

negro who had successive attacks of glandular swelling of the scrotum,

until finally the scrotum was two feet long and six feet in

circumference. It is mentioned that mortification of the part caused

this patient's ultimate death.

Figure 281 is taken from a photograph loaned to the authors by Dr.

James Thorington. The patient was a native of Fiji, and was

successfully operated on, with preservation of the testes. The tumor,

on removal, weighed 120 pounds.

W. R. Browne, Surgeon-General, reports from the Madras General Hospital

an operation on a patient of thirty-five with elephantoid scrotum of

six years' duration. The proportions of the scrotum were as follows:

Horizontally the circumference was six feet 6 1/2 inches, and

vertically the circumference was six feet ten inches. The penis was

wholly hidden, and the urine passed from an opening two feet 5 1/2

inches from the pubis. The man had complete control of his bladder, but

was unable to walk. The operation for removal occupied one hour and

twenty minutes, and the tumor removed weighed 124 3/4 pounds. Little

blood was lost on account of an elastic cord tied about the neck of the

tumor, and secured by successful removal of a scrotal tumor weighing 56

pounds.

Fenger describes a case of the foregoing nature in a German of

twenty-three, a resident of Chicago. The growth had commenced eight

years previously, and had progressively increased. There was no pain or

active inflammation, and although the patient had to have especially

constructed trousers he never ceased his occupation as a driver. The

scrotum was represented by a hairless tumor weighing 22 pounds, and

hanging one inch below the knees. No testicles or penis could be made

out. Fenger removed the tumor, and the man was greatly improved in

health. There was still swelling of the inguinal glands on both sides,

but otherwise the operation was very successful. The man's mental

condition also greatly improved. Fenger also calls especial attention

to the importance of preserving the penis and testes in the operation,

as although these parts may apparently be obliterated their functions

are undisturbed.

The statistics of this major operation show a surprisingly small

mortality. Fayrer operated on 28 patients with 22 recoveries and six

deaths, one from shock and five from pyemia The same surgeon collected

193 cases, and found the general mortality to be 18 per cent. According

to Ashhurst, Turner, who practiced as a medical missionary in the

Samoan Islands, claims to have operated 136 times with only two deaths.

McLeod, Fayrer's successor in India, reported 129 cases with 23 deaths.

Early in this century Rayer described a case of elephantiasis in a boy

of seventeen who, after several attacks of erysipelas, showed marked

diminution of the elephantoid change; the fact shows the antagonism of

the streptococcus erysipelatis to hypertrophic and malignant processes.

Acromegaly is a term introduced by Marie, and signifies large

extremities. It is characterized by an abnormally large development of

the extremities and of the features of the face,--the bony as well as

the soft parts. In a well-marked case the hands and feet are greatly

enlarged, but not otherwise deformed, and the normal functions are not

disturbed. The hypertrophy involves all the tissues, giving a curious

spade-like appearance to the hands. The feet are similarly enlarged,

although the big toe may be relatively much larger. The nails also

become broad and large. The face increases in volume and becomes

elongated, in consequence of the hypertrophy of the superior and

inferior maxillary bones. The latter often projects beyond the upper

teeth. The teeth become separated, and the soft parts increase in size.

The nose is large and broad, and the skin of the eyelids and ears is

enormously hypertrophied. The tongue is greatly hypertrophied. The

disease is of long duration, and late in the history the bones of the

spine and thorax may acquire great deformity. As we know little of the

influences and sources governing nutrition, the pathology and etiology

of acromegaly are obscure. Marie regards the disease as a systemic

dystrophy analogous to myxedema, due to a morbid condition of the

pituitary body, just as myxedema is due to disease of the thyroid. In

several of the cases reported the squint and optic atrophy and the

amblyopia have pointed to the pituitary body as the seat of a new

growth of hypertrophy. Pershing shows a case of this nature. The

enlargement of the face and extremities was characteristic, and the

cerebral and ocular symptoms pointed to the pituitary body as the seat

of the lesion. Unverricht, Thomas, and Ransom report cases in which the

ocular lesions, indicative of pituitary trouble, were quite prominent.

Of 22 cases collected by Tamburini 19 showed some change in the

pituitary body, and in the remaining three cases either the diagnosis

was uncertain or the disease was of very short duration. Linsmayer

reported a case in which there was a softened adenoma in the pituitary

body, and the thymus was absent.

Hersman reports an interesting case of progressive enlargement of the

hands in a clergyman of fifty. Since youth he had suffered with pains

in the joints. About three years before the time of report he noticed

enlargement of the phalangeal joint of the third finger of the right

hand. A short time later the whole hand became gradually involved and

the skin assumed a darker hue. Sensation and temperature remained

normal in both hands; acromegaly was excluded on account of the absence

of similar changes elsewhere. Hersman remarks that the change was

probably due to increase in growth of the fibrous elements of the

subcutaneous lesions about the tendons, caused by rheumatic poison.

Figure 283 shows the palmer and dorsal surfaces of both hands.

Chiromegaly is a term that has been applied by Charcot and Brissaud to

the pseudoacromegaly that sometimes occurs in syringomyelia. Most of

the cases that have been reported as a combination of these two

diseases are now thought to be only a syringomyelia. A recent case is

reported by Marie. In this connection it is interesting to notice a

case of what might be called acute symptomatic transitory

pseudoacromegaly, reported by Potovski: In an insane woman, and without

ascertainable cause, there appeared an enlargement of the ankles,

wrists, and shoulders, and later of the muscles, with superficial

trophic disturbances that gradually disappeared. The author excludes

syphilis, tuberculosis, rheumatism, gout, hemophilia, etc., and

considers it to have been a trophic affection of cerebral origin.

Cases of pneumonia osteoarthropathy simulating acromegaly have been

reported by Korn and Murray.

Megalocephaly, or as it was called by Virchow, leontiasis ossea, is due

to a hypertrophic process in the bones of the cranium. The cases

studied by Virchow were diffuse hyperostoses of the cranium. Starr

describes what he supposes to be a case of this disease, and proposes

the title megalocephaly as preferable to Virchow's term, because the

soft parts are also included in the hypertrophic process. A woman of

fifty-two, married but having no children, and of negative family

history, six years before the time of report showed the first symptoms

of the affection, which began with formication in the finger-tips. This

gradually extended to the shoulders, and was attended with some

uncertainty of tactile sense and clumsiness of movement, but actual

anesthesia had never been demonstrated. This numbness had not invaded

the trunk or lower extremities, although there was slight uncertainty

in the gait. There had been a slowly progressing enlargement of the

head, face, and neck, affecting the bone, skin, and subcutaneous

tissues, the first to the greatest degree. The circumference of the

neck was 16 inches; the horizontal circumference of the head was 24

inches; from ear to ear, over the vertex, 16 inches; and from the root

of the nose to the occipital protuberance, 16 inches. The cervical

vertebrae were involved, and the woman had lost five inches in height.

It may be mentioned here that Brissaud and Meige noticed the same loss

in height, only more pronounced, in a case of gigantism, the loss being

more than 15 inches. In Starr's case the tongue was normal and there

was no swelling of the thyroid.

Cretinism is an endemic disease among mountainous people who drink

largely of lime water, and is characterized by a condition of physical,

physiologic, and mental degeneracy and nondevelopment, and possibly

goiter. The subjects of this disease seldom reach five feet in height,

and usually not more than four. The word cretin is derived from the

Latin creatura. They are found all over the world. In Switzerland it is

estimated that in some cantons there is one cretin to every 25

inhabitants. In Styria, the Tyrol, and along the Rhine cretins are

quite common, and not long since cases existed in Derbyshire. These

creatures have been allowed to marry and generate, and thus extend

their species. In "Le Medicin de Campagne," Balzac has given a vivid

picture of the awe and respect in which they were held and the way in

which they were allowed to propagate. Speaking of the endemic cretins,

Beaupre says: "I see a head of unusual form and size, a squat and

bloated figure, a stupid look, bleared, hollow, and heavy eyes, thick,

projecting eyelids, and a flat nose. His face is of a leaden hue, his

skin dirty, flabby, covered with tetters, and his thick tongue hangs

down over his moist, livid lips; his mouth, always open and full of

saliva, shows teeth going to decay. His chest is narrow, his back

curved, his breath asthmatic, his limbs short, misshapen, without

power. The knees are thick and inclined inward, the feet flat. The

large head droops listlessly on the breast; the abdomen is like a bag."

The cretin is generally deaf and dumb, or only able to give a hoarse

cry. He is indifferent to heat and cold, and even to the most revolting

odors. The general opinion has always been that the sexual desire and

genital organs are fully developed.

A quotation under our observation credits Colonel Sykes with the

following statistics of cretinism, which show how in some locations it

may be a decided factor of population. In December, 1845, in a

population of 2,558,349 souls (the locality not mentioned), there were

18,462 people with simple goiter. Of the cretins without goiter there

were 2089. Of cretins with goiter there were 3909; and cretins in which

goiter was not stated 962, making a total of 6960. Of these 2185 had

mere animal instincts; 3531 possessed very small intellectual

faculties; 196 were almost without any; 1048 not classified. Of this

number 2483 were born of healthy and sane fathers; 2285 from healthy

mothers; 961 from goitrous fathers; 1267 from goitrous mothers; 49 from

cretin fathers; 41 from cretin mothers; 106 from cretin fathers with

goiter; 66 from cretin mothers with goiter; 438 fathers and 405 mothers

were not specified.

Sporadic cretinism, or congenital myzedema, is characterized by a

congenital absence of the thyroid, diminutiveness of size, thickness of

neck, shortness of arms and legs, prominence of the abdomen, large size

of the face, thickness of the lips, large and protruding tongue, and

imbecility or idiocy. It is popularly believed that coitus during

intoxication is the cause of this condition. Osler was able to collect

11 or 12 cases in this country. The diagnosis is all-important, as the

treatment by the thyroid extract produces the most noteworthy results.

There are several remarkable recoveries on record, but possibly the

most wonderful is the case of J. P. West of Bellaire, Ohio, the

portraits of which are reproduced in Plate 11. At seventeen months the

child presented the typical appearance of a sporadic cretin. The

astonishing results of six months' treatment with thyroid extract are

shown in the second figure. After a year's treatment the child presents

the appearance of a healthy and well-nourished little girl.

Myxedema proper is a constitutional condition due to the loss of the

function of the thyroid gland. The disease was first described by Sir

William Gull as a cretinoid change, and later by William Ord of London,

who suggested the name. It is characterized clinically by a

myxedematous condition of the subcutaneous tissues and mental failure,

and anatomically by atrophy of the thyroid gland. The symptoms of

myxedema, as given by Ord, are marked increase in the general bulk of

the body, a firm, inelastic swelling of the skin, which does not pit on

pressure; dryness and roughness which tend, with swelling, to

obliterate the lines of expression in the face; imperfect nutrition of

the hair; local tumefaction of the skin and subcutaneous tissues,

particularly in the supraclavicular region. The physiognomy is

remarkably altered; the features are coarse and broad, the lips thick,

the nostrils broad and thick, and the mouth enlarged. There is a

striking slowness of thought and of movement; the memory fails, and

conditions leading to incipient dementia intervene. The functions of

the thoracic and abdominal organs seem to be normal, and death is

generally due to some intercurrent disease, possibly tuberculosis. A

condition akin to myxedema occurs after operative removal of the

thyroid gland.

In a most interesting lecture Brissaud shows the intimate relation

between myxedema, endemic cretinism, sporadic cretinism, or

myxedematous idiocy, and infantilism. He considers that they are all

dependent upon an inherited or acquired deficiency or disease of the

thyroid gland, and he presents cases illustrating each affection.

Figure 285 shows a case of myxedema, one of myxedema in a case of

arrested development--a transition case between myxedema of the adult

and sporadic cretinism--and a typical case of sporadic cretinism.

Cagots are an outcast race or clan of dwarfs in the region of the

Pyrenees, and formerly in Brittany, whose existence has been a

scientific problem since the sixteenth century, at which period they

were known as Cagots, Gahets, Gafets, Agotacs, in France; Agotes or

Gafos, in Spain; and Cacous, in Lower Brittany. Cagot meant the dog of

a Goth; they were of supposed Gothic origin by some, and of Tartar

origin by others. These people were formerly supposed to have been the

descendants of lepers, or to have been the victims of leprosy

themselves. From the descriptions there is a decided difference between

the Cagots and the cretins. In a recent issue of Cosmos a writer

describes Cagots as follows:--

"They inhabit the valley of the Ribas in the northwestern part of the

Spanish province now called Gerona. They never exceed 51 1/2 inches in

height, and have short, ill-formed legs, great bellies, small eyes,

flat noses, and pale, unwholesome complexions. They are usually stupid,

often to the verge of idiocy, and much subject to goiter and scrofulous

affections. The chief town of the Ribas Valley is Ribas, a place of

1500 inhabitants, about 800 feet above sea-level. The mountains rise

about the town to a height of from 6000 to 8000 feet, and command an

amazingly beautiful panorama of mountain, plain, and river, with

Spanish cities visible upon the one side and French upon the other. The

region is rich, both agriculturally and minerally, and is famous for

its medicinal springs. In this paradise dwell the dwarfs, perhaps as

degraded a race of men and women as may be found in any civilized

community. They are almost without education, and inhabit wretched huts

when they have any shelter. The most intelligent are employed as

shepherds, and in summer they live for months at an elevation of more

than 6000 feet without shelter. Here they see no human creature save

some of their own kind, often idiots, who are sent up every fifteen or

twenty days with a supply of food.

"It is said that formal marriage is almost unknown among them. The

women in some instances are employed in the village of Ribas as nurses

for children, and as such are found tender and faithful. Before

communication throughout the region was as easy as it is now, it was

thought lucky to have one of these dwarfs in a family, and the dwarfs

were hired out and even sold to be used in beggary in neighboring

cities. There are somewhat similar dwarfs in other valleys of the

Pyrenees, but the number is decreasing, and those of the Ribas Valley

are reduced to a few individuals."

Hiccough is a symptom due to intermittent, sudden contraction of the

diaphragm. Obstinate cases are most peculiar, and sometimes exhaust the

physician's skill. Symes divides these cases into four groups:--

(1) Inflammatory, seen particularly in inflammatory diseases of the

viscera or abdominal membranes, and in severe cases of typhoid fever.

(2) Irritative, as in direct stimulus of the diaphragm in swallowing

some very hot substance; local disease of the esophagus near the

diaphragm, and in many conditions of gastric and intestinal disorder,

more particularly those associated with flatus.

(3) Specific or idiopathic, in which there are no evident causes

present; it is sometimes seen in cases of nephritis and diabetes.

(4) Neurotic, in which the primary cause is in the nervous

system,--hysteria, epilepsy, shock, or cerebral tumors.

The obstinacy of continued hiccough has long been discussed. Osler

Date: 2014-12-29; view: 601