MINOR TERATA. 7 page

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

Date: 2014-12-29; view: 582