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CONTRIBUTIONS TO EMBRYOLOGY, No. 22


INTEGUMENT.


The dome of the head is narrowed and flattened, and is covered with lightbrown hair, 1 cm. in length. Just above and behind the ears the hair is 2 cm. long
and is ({uite thick. Across the middle of the forehead at the hair margin is a narrow
raised ridge of puckered skin, 2 cm. long. A section through this area shows the
structures of the skin to be well develoi)ed and similar to the adjoining noi-mal
skin, except that where the surface is raised the jiapilla' are somewhat flattened
underneath. The ridge is probably the result of rough handling before fixation.


STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH ENCEPHALOCELES AND OTHER ABNORMALITIES.  
From the back of the head protrude three encephaloccles in a horizontal line,
extending from a point 6 cm. behind the tip of the left ear to a point 2 cm.
behind the tip of the right ear. These are best dis])laved in figures 1 and 3. They
nu'iisure 13 cm. horizontally along their superior margin, and the vertical diameter
is 4.3 cm. The midline of the specimen comes between the left and middle encephaloccles. In the midline their vertictU diameter extends from a point 3.6 cm. below
the vertex to within 6 cm. (»f the amis. The middle swelling is the largest of the






Bv Theodora Wheeler.
ENCEPHALOCELES AND OTHER ABNORMALITIES. 93
 
 
 
With four plates.
 
 
 
STUDY OF HUMAN SPINA BIFIDA MONSTER WITH ENCEPHALOCELES AND OTHER ABNORMALITIES.  


three and is cone-shaped, whereas the other two are smaller and hemispherical.
All are of soft consistency. The large coneUke swelling measures 6 cm. from the
superior margin to its tip and 2.5 cm. from tip to inferior margin, its base being
circular and measuring 4.3 cm. in diameter. This cone lies pendant over the back;
the proximal half of its superior surface is covered with scalp and fine brown hair
2.5 cm. long. A strip of coarser hair of the same length follows the median margin
of the sweUing to its lowest point. The covering of the distal half of the superior
surface and the entire inferior surface resembles smooth, fine-grained leather. No
hairs are present, but it is dotted with minute pores which on microscopical examination are seen to be the mouths of sweat-ducts. The wall of the sac is 3 mm.
thick, a section through which shows an extremely thin layer of epidermis h'ing
immediately over a vascular connective tissue, containing the sweat-glands mentioned above; but no hair follicles. There are two oval naevi near the tip of the
sac, which in the gross resemble scars. These lie in the same long axis directed
laterall}' through the tip of the sac. The smaller of the two is 5 mm. to the left
of the tip and measures 7 by 3 mm. The larger lies 10 mm. to the right of the tip
and measures 25 by 17 mm. The color of these areas is lighter than the surrounding tissue and the surfaces are stiff, smooth, and slightly raised. In their neighborhood the thickness of the sac wall is increased to 9 mm. Histologically the
epitheUum is lacking here and a very vascular connective tissue forms the raised
surface. The lining of the upper part of the sac is smooth fibrous tissue continuous with the dura of the main cerebro-spinal cavit5^ Near the tip, however, it
is made up of shaggy strands of blood-vessels whose compUcated, interweaving
pattern is like the early capillary plexus of the dura, as described by Streeter (1915).
This suggests that the irregular vascularization in this region may be due to arrested
development of the vascular system. There is marked engorgement of these vessels
and congestion in all the tissues. Part of this extreme engorgement was probably
caused by birth trauma.


The left encephalocele measures 5 by 4 cm. and protrudes 1 cm. from the
surface. Its upper half is covered with fine hairs and the lower half with normalappearing skin. The wall is 1 to 2 cm. thick, composed chiefly of a layer of subcutaneous fat. On its left lower border there is a rounded bleb of porous, wrinkled
skin 1 cm. in diameter, over which are scattered a few hairs 2 cm. long, and which
contains around its depressed circumference a much thicker growth of similar hairs.
A section through the wrinkled skin shows that it lies over a funnel-shaped canal,
the wider mouth of which extends down through 1.5 cm. of subcutaneous fat to
the subdural space, where the canal becomes narrow. This canal is filled with fluid
and contains a few blood-vessels supported by loose connective-tissue septa. Its
walls are formed of rather dense connective tissue.


By Theodora Wheeler.  
The right encephalocele is 4 cm. in diameter at its base, and the surface, which
is covered with, hairy scalp, is but slightly raised above the adjoining structures. Its
wall, consisting of epidermis, connective tissue, and fat, is but 3 mm. thick.  


A summing up of the integument findings shows that both normal skin and
scalp are found over the areas adjoining the encephaloceles and over parts of the




The specimen described in this study is a human female monster with spina
bifida, in which there is total subcutaneous involvement of the spine and a defective
occiput. The thoracic and cervical regions of the spine are much shortened,
and encephaloceles and numerous other abnormalities are present. The type,
though a rather unusual variety of spina bifida, occurs frequently enough to have
been recognized and grouped by itself for some time past, and to this group the
term iniencephaly has been appUed. Because of the striking appearance of these
specimens, one or more are usualh^ to be found in any museum of pathology. In the
embryological collection of over 1,600 specimens belonging to the Department of
Embryologj' of the Carnegie Institution of Washington, the onh^ example is the
one presented in this paper. No. 862a. It was through the courtesy of the Bridgeport General Hospital that this specimen was obtained.


Only a short review of the Hterature on spina bifida will be given here. More
94 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH
complete historical accounts with extensive bibhographies are to be found in articles
by Kermauner and b}' Ernst in Schwalbe's IMorphologie der Missbildung (1909),
and also in a chapter on spina bifida by Tillmann, in his volume of Deutsche
Chirurgie, v. 62a (1905). The earliest references to many teratological conditions
are supposed to be found in folklore and in mythological tales of centaurs,
Cyclops, mermaids, and such creatures, and it has been suggested that among such
stories may Ukewise be found the first record of spina bifida. Possible' the hairj^
and cloven-hoofed satyr was originally a fairly normal individual with spina bifida,
hypertrichosis, and club feet, whose abnormalities gradually developed through
excited hearsay into the hind-quarters of a beast. Even in recent times, in
connection with scientific work on this condition, such inaccuracies have only
too frequently been paralleled by superficial observations and indefinite speculations. However, it is not surprising that a good deal of vagueness has existed in
regard to spina bifida, as the subject-matter includes widely dissimilar and very
comphcated conditions.


As described among human forms, two chief types are distinguished : the flatspine type (rachischisis, spina bifida aperta) and the subcutaneous type (cystic,  
enccphaloccles as well. In addition to this, in the adjoining areas there are hypertrichosis and thickened subcutaneous fat, varying from 5 to 25 mm. in thickness.
occulta). In both of these forms the greatest variations exist as to location and  
The wall of the large encephalocele varies from 3 to 9 mm. in thickne.ss and is formed
amount of spinal involvement. In some instances only a single segment is affected ;
by angiomatous tissue covered with a thin layer of epidermis penetrated by sweatglands. In two places near the tip of the sac naevi are formed by the vascular
in others the whole spinal column, together with the cranium, may be involved.  
tissue extending to the surface. The walls of the small sacs vary from 3 to 20 mm.  
Combinations of these two forms are to be found, and also conditions whei:e the  
and are formed chiefly by subcutaneous fat covered with scalp. On the left a pore
two varieties merge into one another. Associated with every type of the condition are found innumerable other abnormalities.  
and canal pierce through to the subdural space.  


Owing to this mass of complicated material and to the widely different nomenclatures used by the large number of investigators who have worked on the problem,  
Spietscka in 1894 collected the various forms of skin changes associated with
the literature is enormous and rather confused. The classification is still very
spina bifida. Besides the varieties here found, he described pigment blotches and  
sui)erficial. In teratology, as in general pathology, the trend has been to supi^lant
such a marked increase of fatty tissue as to amount to lipomata. In an article on
classifications based on regional distribution by those having an etiological basis.
skin anomalies by Bettmann, in Schwalbe's Morphologic der Missbildungen, naevi
There exists still in the literature on spina bifida a great deal of the former
are noted as among the most frequent anomalies.  
method. This is due to the fact that until quite recently study has been of external
form alone, from which method only a crude regional classification can result.
By the application of the more penetrating methods of modern anatomy,
embryology, and experimental biology, progress has been made toward etiological
classification.  


In 1881 Koch assembled a number of different forms of spina bifida. He
METHODS.  
pointed out the distinction between the flat-spine form, in which the spinal cord is
uncovered (spina bifida aperta), and the cystic or subcutaneous form, in which the
soft parts have joined but the bony arches remain ununited. He attributed a
later formative period to the subcutaneous than to the open form. In 188G von
Recklinhausen presented over 30 specimens of spina bifida and focussed attention
esjjecially upon the pathological anatomy of the central nervous system and its
membranes in the fetal and older forms. By thoroughly analyzing the conditions
met with and applying the conception of arrested development, he was able to offer
reasonable interpretations for much of the developmental mechanism which up to
that time had not been understood. Contemporaneously with these two writers,
and since their time, many aspects of the subject have been studied. The surgical
treatment of spina bifida has been taken up by many, notably Bayer, Hildebrand.
and Muscatello. Other authors have described special types of the abnormality
Among these may be mentioned Lewis's paper on iniencephaly. He collected
23 cases similar to the one herein described, which show some of the variations
jiresented by this special form. In the literature are to be found fairly nvunerous
descriptions of young specimens with sj^ina bifida. In "A study of the causes
underlying the origin of human monsters" (1908), Mall describes 12 from his collection and cites several others from the literature. An interesting 8 mm. ferret
embryo with localized cervical hydromyeUa was described by Good in 1912.  


Experimental studies on the lower animals have formed a very important
A sagittal section was made of the specimen under discussion (see fig. 9).  
source of information with regard to the open variety of spina bifida. In Mall's
The spinal column shoAvs extreme lordosis, undeveloped arches throughout, and
pai)er a review of the literature on the subject uj) to 1908 is given. The work of
.shortening and fusion of the upper vertebrae. The central nervous system is very
Ilertwig and Morgan has attracted especial attention, the former showing that
much disturbed, a large part of it having slipped down below the cranium, through  
external agents causing delay in the closure of the blastopore can bring about
a much enlarged foramen magnum. This portion lies on the thoracic and lumbar
embryological si)ina bifida. The work of the latter author has been interpreted as
vertebrae and protrudes into the sacs already described. The brain and cord were  
pointing toward NaCl as the definite etiological agent, as he was able to produce
removed, and a clay impression was made of the entire space occupied by the central
a delayed closure of the blastojior(> in frogs' eggs through the use of a 0.6 per cent
nervous system. This was then cast in wax and photographed, as shown in figures
solution of XaCl. Eml)ryological s])ina bifida has also been produced occasionally
4, 5, 6, and 7. By the help of this model the general shape taken ])y the central
in chicks by overheating and various other methods. Working with frogs' eggs,
nervous system was demonstrated and the study of its internal arrangement and
by ultra-violet-ray exposures Baldwin (1915) obtained a condition of doubli'd
relation to other structures was facilitated. The consideration of these will be
and closed neural canal aiul sometimes doubled cord. His specimens were usually
taken up later in this paper.  
 
 
 
ENCEPHALOCELES AND OTHER ABNORMALITIES. 91
 
two-tailed. He referred to them a.s spina bifida and gave a clear explanation of  
the mechanics of the process producing- them. However, the relation of this type
of spina bifida to the more ordinary condition of a single open neural canal is not
altogether plain, and his suggestion that " imperfect oxidation " causes spina bifida
does not further claiify the question.  


The earhest picture of the subcutaneous type of spina bifida with which
In the sagittal section, thick subcutaneous pads of fat are seen in the imdifferentiated region of the neck between chin and thorax, above the sj'mphysis, and
we are familiar is that encountered in embiyos around the 10 mm. stage of development, in which the neural tube is everywhere closed, showing, however, a
over the sacral region. Dissection shows this sul)cutaneous fat to be likewise
greater or less area of enlargement. Such a state has not as yet been experimentally
particularly abundant over the back and shoulders. There is also found an extreme
produced. Several explanations have been advanced to account for it, none of
grade of undeveloped or | split soft palate, associated with which is a bilateral
which are satisfying, nor substantiated bj^ evidence. One suggestion is that the
anlage of the uvula, that on the left side being shown in figure 9a. Consideration
enlargement of the neural tube is due to the fact that dorsally it remains
of the normal development of the .soft palate will help to indicate how this defect
attached to the ectoderm (non-separation of the membrana reuniens). On the
originated. It is generally agreed that at a very early date the tongue occupies
other hand, it has been suggested that the neural tube becomes enlarged because
the area which is later occupied by the septum and palate. The normal rearrangement of these parts to their final po.sitions is acconi])li,'<hed by medial growth of the  
of increased pressure from the contained fluid. In this connection it would seem
palate and downward growth of the sejjtum, associated with independent shifting
that when the affected areas are limited in extent they are in some way connected
of the tongue. If for any reason the tongue can not withdraw, the palate remains
with the curvatures of the body, since such areas usuallj^ occur in the neck or
sjilit to a greater or less degree. That such a cause was operative in this specimen
sacral region, where, in the embryo, the curves are most pronounced. The process
seems lik(!ly; the distorted position of the cervical spine might easily have caused a  
is supposed to be one of subsequent pressure of the h3alromyelia on the surrounding parts, thus inhibiting the development of cartilage and bone. With our
crowding in the adjoining pharyngeal region and so prevented the tongue from
advance in knowledge regarding- the circulation of the cerebro-spinal fluid, some
receding.  
of the most puzzling features presented by subcutaneous spina bifida will probably be satisfactorily exjilained. The work of Weed on the normal cerebro-spinal
fluid circulation is most helpful, supplying as it does for the first time an adequately'
correlated picture of the formation and extension of the cerebro-spinal fluid with  
the difi"erentiation of the perimedullar}' mesenchyme to form the meninges. In
the meantime, an}' discussion concerning the etiology of subcutaneous spina bifida
is entirely theoretical. Suggestions have been made that it may arise directly
from the open spina bifida form; again, that it may be the result of some entirely
different process, or that both forms may-be prodiiced by the same pathological
agent acting at a different stage of development. While it has been generally
assumed that the open and subcutaneous forms of spina bifida are related, this  
has never been proved. A more definite picture of each process must be obtained
before we can know the nature of the relation, or whether there is such a relation,
existing between the two. The hterature on the subject gives the impression that,
although sound facts and more or less sound theories regarding spina bifida have
multiplied, there is much that is not clear and that must be understood before we
can have a comprehensive insight into the processes producing it. That this
information may be gained through a closer embryological study seems probable.  


The study of the specimen dealt with in this paper has been made chiefly along
morphological lines. Only a meager clinical history regarding it was obtainable;
the child was illegitimate, was born spontaneously at full term, and lived only a
few hours. Its external form is shown in figures 1, 2, and 3, and various measurements are given in table 2. Externally, the most marked abnormaUty is the




ENCEPHALOCELES AND OTHER ABNORMALITIES. 95


92 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH
SKELETON.


extreme dorsal flexion and shortening of the trunk. The head is drawn back close
A dissection of the skeleton was made, the vertebrae and ribs being left connected bj' their ligaments, so that the specimen could be easily mounted. To
to the sacral region. The chest and abdomen are unusually prominent. The arms
facilitate handling, two transverse cuts in the skeleton were made at the level of  
and legs are symmetrical and well developed, but the shoulders are hunched up and  
the first thoracic and first lumbar vertebra?. A study of the skeleton shows marked
lie far forward, close to the cheeks. The face is directed upward, which throws the
maldevelopment and distortion, as may be seen in figures 10, 11, and 12. The  
top of the head back so that the vertex lies level with the raised shoulders. The
axial skeleton is most affected, the arches of all the vertebrae being defective; these
neck is obhterated and the chin and chest lie in one plane. The features are well
are open posteriorly in the midline and are flattened outward, forming wide anterior
formed with the exception of lack of prominence of the chin and deformity of the  
support for the central nervous system. In the cervical and thoracic regions the  
ears. Figures 1 and 8 show the right ear. The deformity of the left ear is similar.
bodies of the vertebrae are fused, shortened, and dorsally flexed, so that the spine
The anthelix is pushed outward so as to be unusually prominent; the tragus is
is bent almost double. The occiput actually rests on the gaping vertebral arches
shifted medially and uj^ward, so that it lies opposite the concha; the antitragus
and fuses with them.  
lies below it, pressed against the cheek. Darwin's tubercle is present. The external auditory meatus is patent and the parts of the middle and inner ear prove on
dissection to be well developed. The whole external ear is considerably narrowed,  
as is indicated by as low a physiognomical index as 48.5. The average physiognomical index of the right ear of 14 white infants under 3 weeks of age, in the  
obstetrical ward of the Johns Hopkins Hospital, was found to be 69.1, varying
between 62.5 and 78.7. Measurements made by Dr. A. H. Schultz of 4 dead
white infants not older than 1 month showed the physiognomical index of the
right ear to be 65.0, with a variation between 60.0 and 73.1. Though the physiognomical index shows a rather wide variation due to the great flexibility of the  
ear cartilage in infants, nowhere in the small group of available normal cases is  
it nearly so low as in his specimen. The ear deformity is apparently caused by
pressure upon and twisting of the external parts of the ear during their development by the backward-bent head and the shoulders which lie close on either side.
Marx describes a deformed ear which he designates as "Wildermiithsche Ohr, " in
which the anthelix is very prominent. From the base of each ear a crease in the  
skin extends for 3 cm. medially under the chin to within 2 cm. of the midline, as
is seen in figure 2.
 
INTEGUMENT.  


The dome of the head is narrowed and flattened, and is covered with lightbrown hair, 1 cm. in length. Just above and behind the ears the hair is 2 cm. long
Viewing the occiput in figures 13 and 14, the inferior and medial two-thirds of
and is ({uite thick. Across the middle of the forehead at the hair margin is a narrow
the squama occipitahs is seen to be defective. A bilateral bony excrescence on
raised ridge of puckered skin, 2 cm. long. A section through this area shows the  
its dorsal surface, near the defective medial margin of the squama and close to its
structures of the skin to be well develoi)ed and similar to the adjoining noi-mal
junction with the partes laterales joins it to the everted arches of the second lumbar
skin, except that where the surface is raised the jiapilla' are somewhat flattened
vertebra on the left side and to the first lumbar vertebra on the right. The defect
underneath. The ridge is probably the result of rough handling before fixation.
of the squama in the midline, together with a widening of the angles formed by the  
 
junction of the pars basahs with the partes laterales, has greatly increased the size
From the back of the head protrude three encephaloccles in a horizontal line,  
of the foramen magnum. This is oval in shape and measures 4.5 bj' 3.7 cm. The
extending from a point 6 cm. behind the tip of the left ear to a point 2 cm.
long diameter is antero-posterior, and posteriorly it slants slightly to the left.
behind the tip of the right ear. These are best dis])laved in figures 1 and 3. They
For purposes of comparison the size of a normal foramen is indicated in figure 13
nu'iisure 13 cm. horizontally along their superior margin, and the vertical diameter
by means of dotted lines. The large foramen resembles that of the chondrocraniumat a very early stage of development. The participation of both the squama
is 4.3 cm. The midline of the specimen comes between the left and middle encephaloccles. In the midline their vertictU diameter extends from a point 3.6 cm. below
occipitalis and the vertebral arches in the midline defect, as it exists here, has been
the vertex to within 6 cm. (»f the amis. The middle swelling is the largest of the
regarded as teratological evidence of the homology of these parts, and probably
has been a factor in advancing the opinion, which has slowlj' gained ground, that
some cranial defects, even when existing alone, belong in the same category with
certain vertebral abnormalities.  


The two partes laterales are well formed and but slightly asymmetrical. The
left jugular process is more marked than the right. On the left inferior surface
directly under the jugular process there is a cartilaginous prominence which meets
the tip of the transverse process of the underlying atlas. The hypoglossal foramen
on the left side is a single canal, and while the right hypoglossal foramen has a single
perforation on the medial surface of the pars lateralis, it has a double exit on the
outer surface of the bone. A small rod of bone divides it into a smaller anterior
and a larger posterior foramen, as is demonstrated in figure 11. A division similar
to this has been observ-ed frequently in embryological studies and appears on the
left side in a skull of a human fetus modeled by MackUn. The condition is of rather
frequent occurrence. Lillie gives a ratio of 14 per cent complete division and
36 per cent indicated division, out of 305 left and right canals examined by him.
The explanation generally offered is that it is persisting tissue from primitive
cranial divisions which usually disappear at a very early stage.
96 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH
In our specimen the pars basalis of the occiput is oval and asymmetrical along
its mferior margin, as shown in figure 11, and measures 18 by 14 mm. Its sphenoidal margin is 6 mm. thick, and the thickness of the bone elsewhere is 3 mm. Its
I)()sterior surface is slightly concave, there being a rather deeper depression immediately under the sphenoidal articulation than elsewhere. The anterior surface of
the pars basalis is nearly flat. The inferior margin has a notch near the midline
and on either side of this the bone projects downward, 2 mm. on the right and 4
mm. on the left. A slit-like foramen 2.7 mm. wide directed forward and upward
pierces the pars basalis near its center. On each temporal bone the eminentia
arcuata is very prominent and the fossa subarcuata deeply depressed below. The
ear ossicles are well developed and the other relations of the bone are normal.
With the exception of the small size of the cranial vault the rest of the skull is well
formed.


The bodies of all the cervical and thoracic vertebrae and the dorsal surface of
the first lumbar vertebra are fused together in a bent and irregular central plate
of cartilage containing irregular ossification centers. The roots of the arches and
the ribs project from this plate. The relations of the various parts are shown in
figures 10, 11, and 15. At the superior end of this plate the foveal surfaces of the
atlas and its transverse processes are distinguishable, but, as may be seen in figure
10, both posterior and anterior arches are lost. The fovese are shifted to the right
in relation to their transverse processes, as may be seen in figure 11. This shifting causes the right atlantal transverse process to lie immediately under the fovea.
The left is uncovered by the fovea on that side, but is fused at its tip with the left
pars lateralis.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 93
Viewed from the side in figure 12, the cervical and lower thoracic jjortions of  
 
the central vertebral plate form the two arms of a wide-mouthed U, while the  
three and is cone-shaped, whereas the other two are smaller and hemispherical.
bent base of the U occurs in the plate from the level of the first to the sixth ribs.  
All are of soft consistency. The large coneUke swelling measures 6 cm. from the  
Besides this marked lordosis, there is a very slight lateral bend which shows in
superior margin to its tip and 2.5 cm. from tip to inferior margin, its base being
the dorsal view of the skeleton (fig. 10), giving the vertebral plate a slightly curved
circular and measuring 4.3 cm. in diameter. This cone lies pendant over the back;
S-shaf)e. (This condition of scolisosis and lordosis in varying degrees is very
the proximal half of its superior surface is covered with scalp and fine brown hair
frcHjuently noted in the extreme forms of si^ina bifida.) The concavity at the right
2.5 cm. long. A strip of coarser hair of the same length follows the median margin
margin of the vertebral plate is opjjosite the first rib and at the left opposite the
of the sweUing to its lowest point. The covering of the distal half of the superior
sixth rib. From the central plate of this specimen throughout its extent the radices
surface and the entire inferior surface resembles smooth, fine-grained leather. No
project outward on both sides and formed between them are two uneven rows of  
hairs are present, but it is dotted with minute pores which on microscopical examination are seen to be the mouths of sweat-ducts. The wall of the sac is 3 mm.
intervertebral foramina. The processes are tiny spicules of bone in the cervical
thick, a section through which shows an extremely thin layer of epidermis h'ing
and U[)per thoracic region, becoming larger in the lower ])art of the colunm. In
immediately over a vascular connective tissue, containing the sweat-glands mentioned above; but no hair follicles. There are two oval naevi near the tip of the
the cervical region 7 radices are distitict and 12 in the thoracic region. The former
sac, which in the gross resemble scars. These lie in the same long axis directed
could not all be shown in the drawings.
laterall}' through the tip of the sac. The smaller of the two is 5 mm. to the left
of the tip and measures 7 by 3 mm. The larger lies 10 mm. to the right of the tip
and measures 25 by 17 mm. The color of these areas is lighter than the surrounding tissue and the surfaces are stiff, smooth, and slightly raised. In their neighborhood the thickness of the sac wall is increased to 9 mm. Histologically the
epitheUum is lacking here and a very vascular connective tissue forms the raised
surface. The lining of the upper part of the sac is smooth fibrous tissue continuous with the dura of the main cerebro-spinal cavit5^ Near the tip, however, it
is made up of shaggy strands of blood-vessels whose compUcated, interweaving
pattern is like the early capillary plexus of the dura, as described by Streeter (1915).  
This suggests that the irregular vascularization in this region may be due to arrested
development of the vascular system. There is marked engorgement of these vessels
and congestion in all the tissues. Part of this extreme engorgement was probably
caused by birth trauma.  
 
The left encephalocele measures 5 by 4 cm. and protrudes 1 cm. from the  
surface. Its upper half is covered with fine hairs and the lower half with normalappearing skin. The wall is 1 to 2 cm. thick, composed chiefly of a layer of subcutaneous fat. On its left lower border there is a rounded bleb of porous, wrinkled
skin 1 cm. in diameter, over which are scattered a few hairs 2 cm. long, and which
contains around its depressed circumference a much thicker growth of similar hairs.  
A section through the wrinkled skin shows that it lies over a funnel-shaped canal,
the wider mouth of which extends down through 1.5 cm. of subcutaneous fat to
the subdural space, where the canal becomes narrow. This canal is filled with fluid
and contains a few blood-vessels supported by loose connective-tissue septa. Its
walls are formed of rather dense connective tissue.  
 
The right encephalocele is 4 cm. in diameter at its base, and the surface, which
is covered with, hairy scalp, is but slightly raised above the adjoining structures. Its
wall, consisting of epidermis, connective tissue, and fat, is but 3 mm. thick.  
 
A summing up of the integument findings shows that both normal skin and
scalp are found over the areas adjoining the encephaloceles and over parts of the  
 


The arches of both cervical and thoracic regions are everted and fused. This
formation, together with the antero-posterior bend of the plate, makes a rather
deep pocket of bone which contains parts of the much disturbed central nervous
system. The lumbar and sacral vertebral colunm is much less afTected than the
upper part. The dorsal part of the first lumbar vertebral body is fused with the
thoracic vertebra?, its ventral surface, however, being distinct. The four lower


94 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH


enccphaloccles as well. In addition to this, in the adjoining areas there are hypertrichosis and thickened subcutaneous fat, varying from 5 to 25 mm. in thickness.
The wall of the large encephalocele varies from 3 to 9 mm. in thickne.ss and is formed
by angiomatous tissue covered with a thin layer of epidermis penetrated by sweatglands. In two places near the tip of the sac naevi are formed by the vascular
tissue extending to the surface. The walls of the small sacs vary from 3 to 20 mm.
and are formed chiefly by subcutaneous fat covered with scalp. On the left a pore
and canal pierce through to the subdural space.


Spietscka in 1894 collected the various forms of skin changes associated with
ENCEPHALOCELES AND OTHER ABNORMALITIES.
spina bifida. Besides the varieties here found, he described pigment blotches and
 
such a marked increase of fatty tissue as to amount to lipomata. In an article on
 
skin anomalies by Bettmann, in Schwalbe's Morphologic der Missbildungen, naevi
 
are noted as among the most frequent anomalies.
97


METHODS.


A sagittal section was made of the specimen under discussion (see fig. 9).
The spinal column shoAvs extreme lordosis, undeveloped arches throughout, and
.shortening and fusion of the upper vertebrae. The central nervous system is very
much disturbed, a large part of it having slipped down below the cranium, through
a much enlarged foramen magnum. This portion lies on the thoracic and lumbar
vertebrae and protrudes into the sacs already described. The brain and cord were
removed, and a clay impression was made of the entire space occupied by the central
nervous system. This was then cast in wax and photographed, as shown in figures
4, 5, 6, and 7. By the help of this model the general shape taken ])y the central
nervous system was demonstrated and the study of its internal arrangement and
relation to other structures was facilitated. The consideration of these will be
taken up later in this paper.


In the sagittal section, thick subcutaneous pads of fat are seen in the imdifferentiated region of the neck between chin and thorax, above the sj'mphysis, and
lumbar and the five sacral vertebral bodies are well formed, as are the transverse
over the sacral region. Dissection shows this sul)cutaneous fat to be likewise
processes of all the lumbar vertebra? and the partes laterales of the sacrum (figs.  
particularly abundant over the back and shoulders. There is also found an extreme
10 and 12). The first four lumbar arches are everted, as are the thoracic arches,
grade of undeveloped or | split soft palate, associated with which is a bilateral
though individually they are distinct and not fused. The fifth lumbar and the five
anlage of the uvula, that on the left side being shown in figure 9a. Consideration
sacral arches are incomplete, but project medially toward one another and arc not
of the normal development of the .soft palate will help to indicate how this defect
everted. The lumbar column is 4.3 cm. long and the sacrum 3 cm. in length. The
originated. It is generally agreed that at a very early date the tongue occupies
coccyx is composed of four segments, which measure 1.6 cm. and are bent to the
the area which is later occupied by the septum and palate. The normal rearrangement of these parts to their final po.sitions is acconi])li,'<hed by medial growth of the
left. In studying the proportions of the vertebral column, Aeby 's tables of relations
palate and downward growth of the sejjtum, associated with independent shifting
in normal vertebral columns in the new-born were used, with the results shown in
of the tongue. If for any reason the tongue can not withdraw, the palate remains
table 1.  
sjilit to a greater or less degree. That such a cause was operative in this specimen  
 
seems lik(!ly; the distorted position of the cervical spine might easily have caused a
Tabi,e 1. — Comparison in miUimelcrs of the vertebral lengllis of specimen with those
crowding in the adjoining pharyngeal region and so prevented the tongue from
given by Aeby for normal neio-bom.
receding.
 
 






ENCEPHALOCELES AND OTHER ABNORMALITIES. 95
Total.  


SKELETON.


A dissection of the skeleton was made, the vertebrae and ribs being left connected bj' their ligaments, so that the specimen could be easily mounted. To
Cervical.  
facilitate handling, two transverse cuts in the skeleton were made at the level of
 
the first thoracic and first lumbar vertebra?. A study of the skeleton shows marked
maldevelopment and distortion, as may be seen in figures 10, 11, and 12. The
axial skeleton is most affected, the arches of all the vertebrae being defective; these
are open posteriorly in the midline and are flattened outward, forming wide anterior
support for the central nervous system. In the cervical and thoracic regions the
bodies of the vertebrae are fused, shortened, and dorsally flexed, so that the spine
is bent almost double. The occiput actually rests on the gaping vertebral arches
and fuses with them.


Viewing the occiput in figures 13 and 14, the inferior and medial two-thirds of
Thoracic.  
the squama occipitahs is seen to be defective. A bilateral bony excrescence on
its dorsal surface, near the defective medial margin of the squama and close to its
junction with the partes laterales joins it to the everted arches of the second lumbar
vertebra on the left side and to the first lumbar vertebra on the right. The defect
of the squama in the midline, together with a widening of the angles formed by the
junction of the pars basahs with the partes laterales, has greatly increased the size
of the foramen magnum. This is oval in shape and measures 4.5 bj' 3.7 cm. The
long diameter is antero-posterior, and posteriorly it slants slightly to the left.
For purposes of comparison the size of a normal foramen is indicated in figure 13
by means of dotted lines. The large foramen resembles that of the chondrocraniumat a very early stage of development. The participation of both the squama
occipitalis and the vertebral arches in the midline defect, as it exists here, has been
regarded as teratological evidence of the homology of these parts, and probably
has been a factor in advancing the opinion, which has slowlj' gained ground, that
some cranial defects, even when existing alone, belong in the same category with
certain vertebral abnormalities.  


The two partes laterales are well formed and but slightly asymmetrical. The
 
left jugular process is more marked than the right. On the left inferior surface
Lumbar.  
directly under the jugular process there is a cartilaginous prominence which meets
 
the tip of the transverse process of the underlying atlas. The hypoglossal foramen
 
on the left side is a single canal, and while the right hypoglossal foramen has a single
Aeby (normal) ....  
perforation on the medial surface of the pars lateralis, it has a double exit on the
No. 862a
outer surface of the bone. A small rod of bone divides it into a smaller anterior
and a larger posterior foramen, as is demonstrated in figure 11. A division similar
to this has been observ-ed frequently in embryological studies and appears on the
left side in a skull of a human fetus modeled by MackUn. The condition is of rather
frequent occurrence. Lillie gives a ratio of 14 per cent complete division and
36 per cent indicated division, out of 305 left and right canals examined by him.
The explanation generally offered is that it is persisting tissue from primitive
cranial divisions which usually disappear at a very early stage.  




176.5
105.0


96 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH


In our specimen the pars basalis of the occiput is oval and asymmetrical along
45.1
its mferior margin, as shown in figure 11, and measures 18 by 14 mm. Its sphenoidal margin is 6 mm. thick, and the thickness of the bone elsewhere is 3 mm. Its
15.0
I)()sterior surface is slightly concave, there being a rather deeper depression immediately under the sphenoidal articulation than elsewhere. The anterior surface of
 
the pars basalis is nearly flat. The inferior margin has a notch near the midline
 
and on either side of this the bone projects downward, 2 mm. on the right and 4
83.9
mm. on the left. A slit-like foramen 2.7 mm. wide directed forward and upward
45.0
pierces the pars basalis near its center. On each temporal bone the eminentia
arcuata is very prominent and the fossa subarcuata deeply depressed below. The
ear ossicles are well developed and the other relations of the bone are normal.
With the exception of the small size of the cranial vault the rest of the skull is well
formed.  


The bodies of all the cervical and thoracic vertebrae and the dorsal surface of
the first lumbar vertebra are fused together in a bent and irregular central plate
of cartilage containing irregular ossification centers. The roots of the arches and
the ribs project from this plate. The relations of the various parts are shown in
figures 10, 11, and 15. At the superior end of this plate the foveal surfaces of the
atlas and its transverse processes are distinguishable, but, as may be seen in figure
10, both posterior and anterior arches are lost. The fovese are shifted to the right
in relation to their transverse processes, as may be seen in figure 11. This shifting causes the right atlantal transverse process to lie immediately under the fovea.
The left is uncovered by the fovea on that side, but is fused at its tip with the left
pars lateralis.


Viewed from the side in figure 12, the cervical and lower thoracic jjortions of
47.5
the central vertebral plate form the two arms of a wide-mouthed U, while the
43.0
bent base of the U occurs in the plate from the level of the first to the sixth ribs.
Besides this marked lordosis, there is a very slight lateral bend which shows in
the dorsal view of the skeleton (fig. 10), giving the vertebral plate a slightly curved
S-shaf)e. (This condition of scolisosis and lordosis in varying degrees is very
frcHjuently noted in the extreme forms of si^ina bifida.) The concavity at the right
margin of the vertebral plate is opjjosite the first rib and at the left opposite the
sixth rib. From the central plate of this specimen throughout its extent the radices
project outward on both sides and formed between them are two uneven rows of
intervertebral foramina. The processes are tiny spicules of bone in the cervical
and U[)per thoracic region, becoming larger in the lower ])art of the colunm. In
the cervical region 7 radices are distitict and 12 in the thoracic region. The former
could not all be shown in the drawings.  


The arches of both cervical and thoracic regions are everted and fused. This
formation, together with the antero-posterior bend of the plate, makes a rather
deep pocket of bone which contains parts of the much disturbed central nervous
system. The lumbar and sacral vertebral colunm is much less afTected than the
upper part. The dorsal part of the first lumbar vertebral body is fused with the
thoracic vertebra?, its ventral surface, however, being distinct. The four lower




A comparison of these vertebral lengths shows the lumbar portion of this
specimen to be within the limits of normal, though near the minimal margin. The
cervical portion is less than half, and the thoracic portion a trifle more than half
the length of the normal. Aebj' gives 26.4 mm. for the transverse diameter of the
atlas, 12.2 mm. for the width of the body of the sixth thoracic vertebra, and 17.5
mm. for that of the fifth lumbar. In this specimen the lateral limits of the foveae
are 31.0 mm. and the width of the transverse processes of the atlas 38.0 mm. The
width of the vertebral plate in the midthoracic region is 23 mm. and the width of
the fifth lumbar vertebra is 21 mm. These differences show an irregular widening
process to have taken place in the vertebral bodies themselves, the change being
most marked in the thoracic and cervical regions. The absence of lateral pressure
from ununited arches must have been an important factor in this broadening
process.


ENCEPHALOCELES AND OTHER ABNORMALITIES.  
There are twelve ribs on each side which ha^•e undergone considerable disturbance. On the right, the first sL\ are fused near their bases (figs. 10, 11, and 15).
 
The second rib terminates at the end of its proximal third in a plate of bone by which
 
it is joined to the first and third ribs. On the left, the fifth to nmth ribs are crowded
together in their proximal half (figs. 10, 12, and 15). The fifth and sixth have but
one costal cartilage between them. The sLxth and seventh ribs are fused for a few
milUmeters just proximal to their termination. Further fusion occurs in pairs
at the bases of the follo\\ing ribs: on the right, between seventh and eighth, ninth
and tenth; on the left, between the first and second, third and fourth. This shows
on the ventral surface in figure 15.


97
The sternum, as seen in figure 16, has four ossification centers near the median
line at the level of the first costal cartilage and of the first, second, and third left
intercostal spaces. There are six costal cartilage connections on each side. The
last on each side, however, belongs to the seventh rib. The discrepancy occurs
on the right side through the aborted second rib and on the left side through the






lumbar and the five sacral vertebral bodies are well formed, as are the transverse
98
processes of all the lumbar vertebra? and the partes laterales of the sacrum (figs.
10 and 12). The first four lumbar arches are everted, as are the thoracic arches,
though individually they are distinct and not fused. The fifth lumbar and the five
sacral arches are incomplete, but project medially toward one another and arc not
everted. The lumbar column is 4.3 cm. long and the sacrum 3 cm. in length. The
coccyx is composed of four segments, which measure 1.6 cm. and are bent to the
left. In studying the proportions of the vertebral column, Aeby 's tables of relations
in normal vertebral columns in the new-born were used, with the results shown in
table 1.


Tabi,e 1. — Comparison in miUimelcrs of the vertebral lengllis of specimen with those
given by Aeby for normal neio-bom.




STUDY OF A HUMAN SPINA BIGIDA MONSTEK WITH






Total.  
fifth and sixth, having but one cartilage between them. The first and seventh
costal cartilages of the two sides are opposite each other. The arrangement of
the other cartilages is such that the third to the fifth on the left side are from 0.5
to 1 cm. lower than the corresponding cartilages on the right, yet not quite opposite
the succeeding one. A small cartilaginous knob (2 by 5 by 3 mm.) above the manubrium is a persistent episternum. The measurements of the sternum are given
in table 2.  


Table 2. — Dimensions in centimeters.


Cervical.




Thoracic.  
Body lengths: cm.  


Vertex-anus 14 .0


Lumbar.  
umbilicus 32 .0


Lower hair border-anus (length of back) 10.0
Head:


Aeby (normal) ....
Circumference of head 32 .5
No. 862a


Biparietal diameter 8.5


176.5  
Anterior fontanel 2.7 cm. transverse by 2 .5  
105.0


Posterior fontanel 1.6 cm. " by 1 .7


45.1
Face, vertical length (border of hair to chin). . 9 .5
15.0


Clear breadth (from free edges of tragi) 9.2


83.9
Eyes apart 1-8
45.0


Nose across 2.3


47.5
Mouth across 2.0  
43.0  


Trunk:


Circumference at umbilicus, passing around the


A comparison of these vertebral lengths shows the lumbar portion of this
back at base of middle sac 33 .0
specimen to be within the limits of normal, though near the minimal margin. The
 
cervical portion is less than half, and the thoracic portion a trifle more than half
Distance across shoulders 12.0  
the length of the normal. Aebj' gives 26.4 mm. for the transverse diameter of the
atlas, 12.2 mm. for the width of the body of the sixth thoracic vertebra, and 17.5
mm. for that of the fifth lumbar. In this specimen the lateral limits of the foveae
are 31.0 mm. and the width of the transverse processes of the atlas 38.0 mm. The
width of the vertebral plate in the midthoracic region is 23 mm. and the width of
the fifth lumbar vertebra is 21 mm. These differences show an irregular widening
process to have taken place in the vertebral bodies themselves, the change being
most marked in the thoracic and cervical regions. The absence of lateral pressure
from ununited arches must have been an important factor in this broadening
process.


There are twelve ribs on each side which ha^•e undergone considerable disturbance. On the right, the first sL\ are fused near their bases (figs. 10, 11, and 15).
Nipples apart 6.4
The second rib terminates at the end of its proximal third in a plate of bone by which
it is joined to the first and third ribs. On the left, the fifth to nmth ribs are crowded
together in their proximal half (figs. 10, 12, and 15). The fifth and sixth have but
one costal cartilage between them. The sLxth and seventh ribs are fused for a few
milUmeters just proximal to their termination. Further fusion occurs in pairs
at the bases of the follo\\ing ribs: on the right, between seventh and eighth, ninth
and tenth; on the left, between the first and second, third and fourth. This shows
on the ventral surface in figure 15.  


The sternum, as seen in figure 16, has four ossification centers near the median
line at the level of the first costal cartilage and of the first, second, and third left
intercostal spaces. There are six costal cartilage connections on each side. The
last on each side, however, belongs to the seventh rib. The discrepancy occurs
on the right side through the aborted second rib and on the left side through the




Trunk — Continued. cm.


98
Diameter of right nipple .9


left nipple 7


Sternum, episternum, and zyphoid:


STUDY OF A HUMAN SPINA BIGIDA MONSTEK WITH
Length of sternum with episternum and 6.9


zyphoid sternum alone 6.0


Width of sternvmi 1.0


fifth and sixth, having but one cartilage between them. The first and seventh
Thickness of sternum 3  
costal cartilages of the two sides are opposite each other. The arrangement of
the other cartilages is such that the third to the fifth on the left side are from 0.5
to 1 cm. lower than the corresponding cartilages on the right, yet not quite opposite
the succeeding one. A small cartilaginous knob (2 by 5 by 3 mm.) above the manubrium is a persistent episternum. The measurements of the sternum are given
in table 2.


Table 2. — Dimensions in centimeters.
Length of episternum 3


Length of zyphoid 9.6


E.xtremities:


Body lengths: cm.
Upper arm (circumference of both left and


Vertex-anus 14 .0  
right) 9.0  


umbilicus 32 .0
Lower arm (circumference of both left and


Lower hair border-anus (length of back) 10.0  
right) 7.0  


Head:
Hand with middle finger 6.0


Circumference of head 32 .5
Right trochanter— heel 20 .9


Biparietal diameter 8.5
Right foot 7.5  
 
Anterior fontanel 2.7 cm. transverse by 2 .5  


Posterior fontanel 1.6 cm. " by 1 .7


Face, vertical length (border of hair to chin). . 9 .5


Clear breadth (from free edges of tragi) 9.2
The two scapulae which are shown in figures 18 and 20 are distorted, as will
 
be seen by comparison with figures 17 and 19, representing normal left and right
Eyes apart 1-8
scapulae. In both the pathological^ bones the supraspinous portions are poorly
 
formed and the inferior vertebral margins are concave. Graves designates a concavity of the vetebral margin of the scapula as scapula scaphoidea. He notes that
Nose across 2.3
it is of fairly frequent occurrence and claims that it is associated with syphilis in
the i)arents. He gives as his figures, however, no definite rate of occurrence. Here
it m:iy l)e mentioned that the Levaditi stain done on the tissues of this specimen
showed no spirocha^tes. On the right scapula the vertebral margin i^asses as
a straight line from the medial termination of the spinous process to the incisura
next the glenoid process. The vertical diameter of the right scapula measures
36 mm. from the tip of the cartilaginous process at the inferior angle to the
superior margin near the incisura. Its transverse diameter along the base of
the spinous process, near the termination of the latter, to the center of the
glenoid fossa is 26 mm. The subscapular angle is 128°, the infraspinous angle
is 122°, and the supraspinous angle is 110°. On the left scapula, the vertebral margin above the spinous process projects at a fairly sharp angle near its
middle. The vertical diameter taken from the tip of the inferior angle to the end
of the projecting point of the supraspinata is 29 mm. The horizontal diameter
of the left scai)ula, measured similarly as the right, is 36.5 mm. The vertebral
margin of the left scapula at the termination of the sjiinous i)roccss is elongated by
a bony and cartilaginous knob, which is attached to a curved rod of bone 10 mm.  


Mouth across 2.0


Trunk:


Circumference at umbilicus, passing around the
ENCEPHALOCELES AND OTHER ABNORMALITIES.


back at base of middle sac 33 .0


Distance across shoulders 12.0


Nipples apart 6.4
99






Trunk — Continued. cm.  
long and 2 mm. in diameter. This rod is joined at its other end to the everted
 
arches of the vertebrae underlying it. On the left scapula the subscapular angle
Diameter of right nipple .9
is 117°, the supraspinous angle is 109.5° and the infraspinous angle is 133.5°. The
left scapula shows a rather interesting condition, presenting three out of four features
often associated with Sprengel's deformity (congenital elevation of the shoulders).
These are, according to Horwitz: (1) changed relations of the diameters to each
other; (2) bending forward of the supraspinous process; (3) prolongation or rounding of superior median angle; (4) presence of exostoses and articulations with the
vertebral column.  


left nipple 7
In this case the exception to the above conditions is the superior median angle,
which can hardly be called prolonged. Scajnilar measurements of the new-born
could not be foimd in the literature, but two supposedly normal sets were obtained
from mounted skeletons belonging to the Obstetrical Department of the Johns
Hopkins Hospital, and the measurements of several other scapulse were available
through the courtesy of Dr. A. H. Schultz.


Sternum, episternum, and zyphoid:
Table 3. — Comparison in miUiiiwtcrs of the scapular measurements of specimen with those
of several normal new-born.


Length of sternum with episternum and 6.9


zyphoid sternum alone 6.0


Width of sternvmi 1.0
Specimen.  


Thickness of sternum 3


Length of episternum 3


Length of zyphoid 9.6
Schultz, No. 3 .
Schultz, No. 4 .  


E.xtremities:


Upper arm (circumference of both left and


right) 9.0
Obstetrical Department skeleton.  
 
Do
 
No. 862 a
 


Lower arm (circumference of both left and


right) 7.0
Vertebral
length.  


Hand with middle finger 6.0


Right trochanter— heel 20 .9


Right foot 7.5
165
151


169


172


The two scapulae which are shown in figures 18 and 20 are distorted, as will
be seen by comparison with figures 17 and 19, representing normal left and right
scapulae. In both the pathological^ bones the supraspinous portions are poorly
formed and the inferior vertebral margins are concave. Graves designates a concavity of the vetebral margin of the scapula as scapula scaphoidea. He notes that
it is of fairly frequent occurrence and claims that it is associated with syphilis in
the i)arents. He gives as his figures, however, no definite rate of occurrence. Here
it m:iy l)e mentioned that the Levaditi stain done on the tissues of this specimen
showed no spirocha^tes. On the right scapula the vertebral margin i^asses as
a straight line from the medial termination of the spinous process to the incisura
next the glenoid process. The vertical diameter of the right scapula measures
36 mm. from the tip of the cartilaginous process at the inferior angle to the
superior margin near the incisura. Its transverse diameter along the base of
the spinous process, near the termination of the latter, to the center of the
glenoid fossa is 26 mm. The subscapular angle is 128°, the infraspinous angle
is 122°, and the supraspinous angle is 110°. On the left scapula, the vertebral margin above the spinous process projects at a fairly sharp angle near its
middle. The vertical diameter taken from the tip of the inferior angle to the end
of the projecting point of the supraspinata is 29 mm. The horizontal diameter
of the left scai)ula, measured similarly as the right, is 36.5 mm. The vertebral
margin of the left scapula at the termination of the sjiinous i)roccss is elongated by
a bony and cartilaginous knob, which is attached to a curved rod of bone 10 mm.




Transverse
diameter.


ENCEPHALOCELES AND OTHER ABNORMALITIES.




R. 24
/ L. 24
\ R. 29
( L. 30
\ R. 27
/ L. 27
I R. 26


99
L. 31






long and 2 mm. in diameter. This rod is joined at its other end to the everted
Vertical
arches of the vertebrae underlying it. On the left scapula the subscapular angle
diameter.  
is 117°, the supraspinous angle is 109.5° and the infraspinous angle is 133.5°. The
left scapula shows a rather interesting condition, presenting three out of four features
often associated with Sprengel's deformity (congenital elevation of the shoulders).
These are, according to Horwitz: (1) changed relations of the diameters to each
other; (2) bending forward of the supraspinous process; (3) prolongation or rounding of superior median angle; (4) presence of exostoses and articulations with the
vertebral column.  


In this case the exception to the above conditions is the superior median angle,
which can hardly be called prolonged. Scajnilar measurements of the new-born
could not be foimd in the literature, but two supposedly normal sets were obtained
from mounted skeletons belonging to the Obstetrical Department of the Johns
Hopkins Hospital, and the measurements of several other scapulse were available
through the courtesy of Dr. A. H. Schultz.


Table 3. — Comparison in miUiiiwtcrs of the scapular measurements of specimen with those
of several normal new-born.


R. 33
L. 31
R. 38
L. 38
R. 36
L. 35
R. 36
L. 29




Specimen.


Morphological
index.




Schultz, No. 3 .
Schultz, No. 4 .


72.7
77.4
76.3
78.7
75.0
77.1
72.2
106.8




Obstetrical Department skeleton.


Do
Table 3 shows that the ratio of the diameters of the right scapula of 862 o is
 
near those of the supposedly normal bones. The left scapula, on the other hand,
No. 862 a  
has the relations of its diameters reversed. The transverse diameter exceeds the
 
vertical. Thus its morphological index is 106.8, while none of the norrnal indices
 
exceeds 80. The subscapular angle on the left side is somewhat smaller than on
 
the right. The bony articulation joining the left scapula to the vertebral column
Vertebral
is attached in the upper third of the vertebral scapular margin as in most of the
length.  
Sprengel deformity cases. Some interest is attached to this abnormal bony spicule
and various suggestions have been made concerning it. The opinion seems to be
generally accepted that it arises from its scapular end. Cases are recorded in
which other anomalous bones are joined only to the scapula, and their occurrence
substantiates this view. Case xvi in von Recklinhausen's paper is a monster very
like No. 862a. In it there is "ein 1 cm. langer knocherner, rippenartiger, am oberen
Winkel des knorpeligen Schulterblatts articulierender Korper." Gruber gives a
case found in an adult male cadaver of a "fortsatzartigen, cylindrischen Hocker an
der Vorderflache des Angulus superior der Scapula." No satisfactory hypothesis
has been advanced to further explain the origin of these bones. The length of
the right clavicle is 39 mm. while the left clavicle measures 34 mm. and is shghtly






165
100 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH
151


169
more bent at its distal end than the right. This shortening of the clavicle on the
side of the abnormal scapula is frccjuent in Sprengel's deformity. The hunched
position of the shoulders, so ]irominent externally in this case, may be seen to be
due to the defective cervical and upper thoracic vertebrae, which lie crumpled to
half their normal length under the scajjulae, their normal relations to these bones
being quite changed.


172
MUSCLES.  
 
 
 
Transverse
diameter.  


The region of abnormal musculature corresponds, as would be supposed, to
the skeleton derangements. This is limited to the neighborhood of the axial
skeleton, where the affected muscles are both under and intermingled with an unusually large amount of fascia. On superficial dissection, the topmost layer of
muscles is well formed, except for the trapezius, which is represented similarlj' on
the two sides by thin strap-like bands of muscle, 3 by 1 cm. The fibers run parallel
with the long diameter, from the origin of the muscle, situated in fascia lying over
the everted and crumpled cervical and thoracic vertebral arches, to their insertion
on the acromial extremity of the clavicle, the acromion, and spine of the scapula.
Those fibers which insert on the scapular spine have become folded under the others,
owing to the contracted and lowered origin of all the fibers. A condition of the
trapezius similar to this has been noted in a case of total rachischisis given by
Kermauner, in which case, also, lordosis and marked shortening of the spine were
the underlying skeletal conditions. As Kermauner says, the association of this
variety of muscle and bone defect is only natural, "for, with the marked shortening
of the trunk, there necessarily exists a reduction in the cranio-caudal diameter of
the muscles of this region."


Upon further dissection, the condition of the underlying muscles was determined. The rhomboidei are represented bilaterally by very thin and short muscles,
onlj'^ 3 mm. in length. They arise from the connective tissue over the fused and
everted arches of the thoracic vertebra, and are inserted in fascia along the inferior
vertebral borders of the scapula?. (In Le Double's work a reduction in the thickness of these muscles is recorded.) The two levator scapulae are present. They
arise from the fused transverse processes of the upper cervical vertebrae and are
inserted in fascia along the superior vertebral margins of the scapulae. There is
no reduction in the size of either muscle. They are directed horizontally out
instead of slanting downwards as usual. This is due to the scapuhe lying directly
over the cervical vertebr:e. A cross-section of the left muscle at its origin is shown
in figure 21. Figure 18 shows the left scapula and the rhomboideus and levator
scapulae muscles inserted in fascia which forms a sheet between the irregular projections of the vertebral margins of the bone. The abnormal spicule of bone is
attached to the scapula at the mcflian angle between the insertions of the levator
scapulae and the rhomboidcnis.


R. 24
On each side most of the dorsal inusck's consist of ;ui irregular longitudinal
/ L. 24
bundle which extends along the sides of the vertebrae from sacrum to occiput and
\ R. 29
which sends scattered projections on to the ribs. Under this bundle in the lumbar
( L. 30
region the quadratus lumborum and psoas muscles lie undisturbed. On the left
\ R. 27
/ L. 27
I R. 26


L. 31




ENCEPHALOCELES AND OTHER ABNORMALITIES. 101


Vertical
side this bundle is shown somewhat diagrammatically in figure 21 and labeled
diameter.  
sacrospinalis. In the lumbar region it is cjdindrical and measures 1.5 cm. in diameter. It grows flatter and broader as it nears the upper part of the spine, this
formation being due to a state of arrested development of the sacrospinalis and
short back-muscles. The early condition of dorsal musculature which it simulates
is strikingly illustrated in Bardeen and Lewis's model of an 11 mm. embryo (1901),
where a bundle distinct from the ventral-lateral muscles lies bilaterally in the trough
formed at the sides of the vertebrae. In another model given in the same paper of
a 20 mm. embryo, the bundle may still be seen lying under the connective tissue of
the region, and this divided condition of the back-muscles persists normally until
about the 60 mm. stage.  


The serati posterior inferior are shown by projections from the dorsal bundle
which on both sides cover the proximal half of the three lowest ribs. In the upper
thoracic region, lying on the surface of the bundle on each side, is a thin strip of muscle
near the base of the ribs. These strips extend cranio-caudally and measure 20 by 3
mm. On the left the strip lies over the third to eighth rib ; on the right side it extends
over the first to the sixth rib. The serati posterior superior are not identifiable.


The direction of the muscles of the anterior cervical region, as well as of those
attached to the skull, is distorted vvith the underlying skeleton, but the muscles
are well developed and not defective. Both sterno-cleido-mastoid muscles have
normal origins and insertions. The two splenii arise bilaterally from fascia
under the scapulae and are inserted normally on the mastoid process under the
sterno-cleido-mastoid, and posterior to this on the occipital bone. The longissimi
can be traced arising from the fascia over the cervical vertebral region and inserted
on the mastoid proces.ses. The semi-spinalis capitis muscles, arising from the upper
ribs near their origin, are inserted on the occipital bone and are next to the deepest
layer of musculature. The latter on each side consists of short fibers, rudiments
of the short neck-muscles, the recti, and obliqui. More anteriorly most of the neck
muscles are recognizable. The digaster, stylohyoid, omohyoid, and sternohyoid
muscles are well developed. The longiis capitis and colli are represented by a few
strands along the anterior surfaces of the vertebral plate. The scaleni medii and
posteriores are present as flattened bands of muscle arising in this region and
inserted in the first and second ribs near their bases. The scaleni anteriores are
symmetrical. They arise from the lateral processes of the superior cervical vertebrae and insert on the first rib near its center. The nerve trunks of the cervical
and brachial plexus pass under these muscles and are tightly bound down bj^ them.


R. 33
Of the more anterior thoracic muscles, the pectorales are not disturbed. Both
L. 31
serati anterior muscles are defective and difTer in their defects. On the right side
R. 38
there is more complete development. Here slips of the muscle arising from the
L. 38
distal portions of the first four ribs and from the eighth to the tenth ribs converge
R. 36
and are inserted around the inferior angle of the scapula. A few strands of muscle
L. 35
on the chest wall between the pectoraUs minor and the seratus are present, which
R. 36
might be remnants of th(! latter. They are shaped like half a crescent, with fibers
L. 29
running longitudinally, and extend from the first rib, where they are 3 mm. broad,
 
to the fifth rib, where they are 15 mm.  




Morphological
index.


102 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH


On the loft side the seratus anterior is very imperfect. It is represented l)y a
thin sheet of fascia, which originates from the first three ribs and is inserted in the
scapula along the vertebral margin near the medial angle. A few scattered musclefibers, which also probably represent remnants of the serati, arise over the fourth
rib near its base and are inserted into the inferior angle of the scapula. The origin
of the fascia and these muscle-fibers is shown in figure 21 by dotted lines. Some
other muscles on the left chest wall, consisting of irregular projections from the
dorsal bundle which covers the proximal part of the first seven ribs, may be serati
fibers which remained in their embryonic position close to the axis. Fibers which
jjrobably represent intercostal muscles pushed to the outer surface of the ribs are
arranged along the lower border of the fourth rib. These extend onto the lower
adjoining ribs. At the outer end they are 2 mm. across and near the base of the
ribs they measure 20 mm. (See fig. 21.) Three small muscle bundles are situated
at the distal end of the above-mentioned fibers.


72.7  
The lateral and anterior abdominal muscles are well developed. Each rectus
77.4  
is 7.4 cm. by 3.2 cm. The right rectus has two inscriptiones tendinae in its upper
76.3  
one-third opposite the sixth and seventh ribs.  
78.7
75.0
77.1
72.2
106.8
 


To summarize : Those muscles which have undergone most disturliance are the
trapezei, the rhomboidei, the serati posteriores superiores, the serati anteriores, and
the sacrosi)inalis and short back muscles. The location of these muscular abnormalities, situated near the chief skeletal abnormalities, demonstrates still further
that the ijathological process is a rather sharply circumscribed one, limited to the
neighborhood of the axis. The inclusion of the anterior serati in this group does
not contradict the statement, as the early anlage of the serati is very near the axis.


Table 3 shows that the ratio of the diameters of the right scapula of 862 o is
The muscle disturbances of "monsters" have been but little investigated or
near those of the supposedly normal bones. The left scapula, on the other hand,
recorded. From the scattered observations at present obtainable, any correlation
has the relations of its diameters reversed. The transverse diameter exceeds the
is impossible.  
vertical. Thus its morphological index is 106.8, while none of the norrnal indices
exceeds 80. The subscapular angle on the left side is somewhat smaller than on
the right. The bony articulation joining the left scapula to the vertebral column
is attached in the upper third of the vertebral scapular margin as in most of the
Sprengel deformity cases. Some interest is attached to this abnormal bony spicule
and various suggestions have been made concerning it. The opinion seems to be
generally accepted that it arises from its scapular end. Cases are recorded in
which other anomalous bones are joined only to the scapula, and their occurrence
substantiates this view. Case xvi in von Recklinhausen's paper is a monster very
like No. 862a. In it there is "ein 1 cm. langer knocherner, rippenartiger, am oberen
Winkel des knorpeligen Schulterblatts articulierender Korper." Gruber gives a
case found in an adult male cadaver of a "fortsatzartigen, cylindrischen Hocker an
der Vorderflache des Angulus superior der Scapula." No satisfactory hypothesis
has been advanced to further explain the origin of these bones. The length of
the right clavicle is 39 mm. while the left clavicle measures 34 mm. and is shghtly


VISCERA.


On dissection, the viscera are found crowded and somewhat distorted, but,
with the excejition of the right lung, are well developed. The thyroid is bilobed
and measures 1.5 by 1 cm., the thymus measures 6 by 2.8 by 1.1 cm. The esophagus measures 4 cm. from epiglottis to cardia. The lesser curvature of the stomach
is 1 cm. and its greater curvature 8 cm. The intestines are well formed. The
api)endix measures 8.5 cm. The cok)n is much bent upon itself. Because of unskillful handling the positions asssunied l)y the rest of the intestinal tract were not
ascertained. The pericardium, ])leura, and diapluagm are intact. The heart is
well developed. Sagittal section sliows it cut through the right ventricle and left
auricle. The valves are well formed. The ductus arteriosus is patent. The left
lung is approximately normal; its lateral surface is shown in figure 23; it consists
of two lobes and measures 3.9 cm. antero-posteriorly by 3.1 cm. perpendicularly
by 1.7 cm. in its thickest niedio-lateral diameter near the hilum. The right lung,
side view of which is shown in figure 22, about ('(juals the left in volunu^ and is
roughly a flattened cone-shape with apex directed anteriorly. Its corresi)onding
measurements are 5.1 by 3.2 by 2.9 cm. it is formed of only one lobe. Along tlie
margins four short fissures exist, directed toward tlu' center; one 12 mm. in length


100 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH


more bent at its distal end than the right. This shortening of the clavicle on the
side of the abnormal scapula is frccjuent in Sprengel's deformity. The hunched
position of the shoulders, so ]irominent externally in this case, may be seen to be
due to the defective cervical and upper thoracic vertebrae, which lie crumpled to
half their normal length under the scajjulae, their normal relations to these bones
being quite changed.


MUSCLES.  
ENCEPHALOCELES AND OTHER ABNORMALITIES. 103


The region of abnormal musculature corresponds, as would be supposed, to
is situated on the posterior margin at the junction of the superior third with the  
the skeleton derangements. This is limited to the neighborhood of the axial
middle third; on the inferior margin near its middle a similar fissure is situated,  
skeleton, where the affected muscles are both under and intermingled with an unusually large amount of fascia. On superficial dissection, the topmost layer of
and halfway between it and the anterior end of the lung a shorter fissure 3.5 mm.  
muscles is well formed, except for the trapezius, which is represented similarlj' on
long exists; on the superior margin another, 3.5 mm. in length, is present slightly
the two sides by thin strap-like bands of muscle, 3 by 1 cm. The fibers run parallel
anterior to the middle. These fissures are very superficial and extend for only a
with the long diameter, from the origin of the muscle, situated in fascia lying over
few millimeters on the medial surface of the lung. The relations of the bronchial
the everted and crumpled cervical and thoracic vertebral arches, to their insertion
tree were not determined.  
on the acromial extremity of the clavicle, the acromion, and spine of the scapula.  
Those fibers which insert on the scapular spine have become folded under the others,  
owing to the contracted and lowered origin of all the fibers. A condition of the
trapezius similar to this has been noted in a case of total rachischisis given by
Kermauner, in which case, also, lordosis and marked shortening of the spine were
the underlying skeletal conditions. As Kermauner says, the association of this
variety of muscle and bone defect is only natural, "for, with the marked shortening
of the trunk, there necessarily exists a reduction in the cranio-caudal diameter of
the muscles of this region."


Upon further dissection, the condition of the underlying muscles was determined. The rhomboidei are represented bilaterally by very thin and short muscles,
The liver is flattened out horizontally and shaped like an L with the angle
onlj'^ 3 mm. in length. They arise from the connective tissue over the fused and
projecting anteriorly, the gall-bladder, which is 4.1 cm. in length, being situated
everted arches of the thoracic vertebra, and are inserted in fascia along the inferior  
on the inferior surface of the long arm of the L. The closed end of the gallbladder lies near the tip of the angle and its long axis is directed diagonally toward
vertebral borders of the scapula?. (In Le Double's work a reduction in the thickness of these muscles is recorded.) The two levator scapulae are present. They
the upper end of the latter. The spleen is 2.2 by 1.3 by 1 cm. The presence of
arise from the fused transverse processes of the upper cervical vertebrae and are
the pancreas is determined histologically. It lies embedded in ti.ssue near the vertebral column. Both kidneys and adrenals are somewhat compressed and distorted,
inserted in fascia along the superior vertebral margins of the scapulae. There is  
the left much more than the right. The right kidney is somewhat flattened from side
no reduction in the size of either muscle. They are directed horizontally out
to side and at its upper end, and measures 4.5 by 1.2 by 3.5 cm. The right adrenal
instead of slanting downwards as usual. This is due to the scapuhe lying directly
lies above it and measures 3 by 2 bj^ 0.5 cm. The left kidney is bent upon itself and
over the cervical vertebr:e. A cross-section of the left muscle at its origin is shown
folded in with its closely adherent adrenal, so that together they form a rounded
in figure 21. Figure 18 shows the left scapula and the rhomboideus and levator
mass measuring 4.7 by 3.4 by 2.4 cm. The greater distortion of the left kidney
scapulae muscles inserted in fascia which forms a sheet between the irregular projections of the vertebral margins of the bone. The abnormal spicule of bone is  
and adrenal is very probably due to crowding, a result of the left-sided concave
attached to the scapula at the mcflian angle between the insertions of the levator
bending of the vertebral column in this region and fusion of the lower ribs on that
scapulae and the rhomboidcnis.  
side. The ureters and bladder are well formed. The uterus, tubes, and ovaries
are well developed. Blocks of tissue of heart, kidney, liver, and adrenal were run
through by the Levaditi method for spirochaetes by Dr. Bullard, with negative
findings. It is to be noted that the tissues had been kept in carbolic, which is not
the fixation recommended for this method.  


On each side most of the dorsal inusck's consist of ;ui irregular longitudinal
The developmental anomalies of the soft palate and the right lung are the most
bundle which extends along the sides of the vertebrae from sacrum to occiput and
marked changes which have taken place in the soft tissues anterior to the vertebral
which sends scattered projections on to the ribs. Under this bundle in the lumbar
column. They are both examples of arrested development and are secondary to,
region the quadratus lumborum and psoas muscles lie undisturbed. On the left
and probably the mechanical result of, 'the deformit.y of the vertebrae.


CRANIAL CAVITY AND CENTRAL NERVOUS SYSTEM.
The shape assumed by the cerebro-spinal cavity or subdural space is shown by
figures 4, 5, 6, and 7 of the wax model. In figures 12 and 21 the model is given in
its relation to the skeleton. The space consists of a shallow dome which contained
the frontal and part of the parietal cerebral lobes. Below this dome a relatively
slight constriction in the model denotes the enlarged foramen magnum (figs. 4, 5, 7,
and 12). Under the foramen three rounded encephaloceles project posteriorly, and
below these the pointed termination of the spinal canal may be seen. Situated
ventral to the encephaloceles and continuous with them and with the base of the
dome and the spinal canal is a blunt, wedge-shaped mass marked iv in figures 4, 5,
and 6. This portion fits into the pocket of bone formed by the thoracic and vertebral plate. With the exception of the inside of the large encephalocele, the space
occupied by the central nervous system is lined with a continuous sheet of smooth
dura. At the foramen magnum and in between the eminentiae arcuatae of the tem




ENCEPHALOCELES AND OTHER ABNORMALITIES. 101
104 STUDY OP A HUMAN SPINA BIFIDA MONSTER WITH


side this bundle is shown somewhat diagrammatically in figure 21 and labeled
ponil bones and the sella turcica it is drawn into numerous folds. On the inside
sacrospinalis. In the lumbar region it is cjdindrical and measures 1.5 cm. in diameter. It grows flatter and broader as it nears the upper part of the spine, this
of tlio large encephalocele the smooth dural surface changes to a tissue composed
formation being due to a state of arrested development of the sacrospinalis and
of many blood-vessels, fibrin, and extravasated blood, as described in the beginning
short back-muscles. The early condition of dorsal musculature which it simulates
of the paper. The fabc cerebri lies well over on the left side in its anterior and  
is strikingly illustrated in Bardeen and Lewis's model of an 11 mm. embryo (1901),
middle portions; posteriori}' it ends in a single fold about the center of the superior
where a bundle distinct from the ventral-lateral muscles lies bilaterally in the trough
margin of the occipital bone. The tentorium cerebelli is absent. The ventral
formed at the sides of the vertebrae. In another model given in the same paper of  
surface of the subdural space is pierced by two rows of cranial and spinal nerves.
a 20 mm. embryo, the bundle may still be seen lying under the connective tissue of
These number 43 in all, 12 cranial and 31 spinal, the latter distributed as follows:
the region, and this divided condition of the back-muscles persists normally until
8 cervical, 12 thoracic, 5 lumbar, 5 sacral, and 1 coccygeal. Both the anterior
about the 60 mm. stage.  
roots and the posterior roots with their ganglia are identified. In the cervical
and thoracic regions they are extremely crowded.  


The serati posterior inferior are shown by projections from the dorsal bundle
The arrangement of the central nervous system is very complicated. The
which on both sides cover the proximal half of the three lowest ribs. In the upper
cerel)rum lies part above and part below the foramen magnum. The cerebellum lies
thoracic region, lying on the surface of the bundle on each side, is a thin strip of muscle
entirely below it. The medulla and cord lie bent and crumpled ventral to the other
near the base of the ribs. These strips extend cranio-caudally and measure 20 by 3
structures below the foramen. The surface of that part of the cerebrum which  
mm. On the left the strip lies over the third to eighth rib ; on the right side it extends
lies above the foramen shows definite fissure and sulcus formation. Below the
over the first to the sixth rib. The serati posterior superior are not identifiable.  
foramen a large part of the contents of the encephaloceles consists of a hemorrhagic
mass and much of the surface of this is covered with caked blood, so that only in
a few [)laces can typical cerebral surface markings be identified, flections of these
regions, however, show definite though rather undifferentiated cortical lamination.
 
From a brief studj' of sections of other parts of the left cerebrum, made by Dr.  
Charles Bagley, the following points are determined : The cortical structure for the
most part is composed of a very wide granular zone, which is characteristic of the  
early and undifferentiated stage of lamination. There is, however, a prominent vertical fissure lying at tlie junction of the middle and posterior two-thirds of the cranial
dome, which can be identified as the central fissure of Rolando; the ventral termination of this fissure rests on the eminentia arcuata of the temporal bone. The
cortical tissue anterior to this fissure shows a definite lamination. There is a welldefuied first layer; a broad and poorl}' differentiated second layer; the third laj^er
is of medium width and is filled with small pyramidal cells; the fourth layer is
inconsi)icuous, suggesting the agranular naotor tyi)e; while the fifth layer is represented l\y large pyramidal cells, ])r()l)ably Betz cells. These cells are at least
three times as large as an}' other cells which could l)e found in the cortex and it is
owing to their presence and to the very narrow gramilar layer that this area is identified as motor cortex. Immediately posterior to this fissure there is a sharp change
in lamination types. Thc^ large cells are lost and the fifth layer is represented by  
definitely sha])ed pyramidal cells of not more than one-third the size of those cells
de.signated as Jietz cells. Above this layer there is present a very heavy granular
layer which is (|uite a contrast to the narrow granular layer of the area just described. It may be said, therefore, with a fair degre(> of certainty, that the tissue
posterior to the fissure represents sensory cortex.
 
From the rather limited amount of material studied the only other localization
that could be determined is that the cortical tissue i)ushetl down on the side of the
wedge-shaped mass (11' in the model) represents subiculum. Here, passing from a
cortex of (|iiilc deep cxlcnl, it becomes suddenly shallow and consists of atypical


The direction of the muscles of the anterior cervical region, as well as of those
attached to the skull, is distorted vvith the underlying skeleton, but the muscles
are well developed and not defective. Both sterno-cleido-mastoid muscles have
normal origins and insertions. The two splenii arise bilaterally from fascia
under the scapulae and are inserted normally on the mastoid process under the
sterno-cleido-mastoid, and posterior to this on the occipital bone. The longissimi
can be traced arising from the fascia over the cervical vertebral region and inserted
on the mastoid proces.ses. The semi-spinalis capitis muscles, arising from the upper
ribs near their origin, are inserted on the occipital bone and are next to the deepest
layer of musculature. The latter on each side consists of short fibers, rudiments
of the short neck-muscles, the recti, and obliqui. More anteriorly most of the neck
muscles are recognizable. The digaster, stylohyoid, omohyoid, and sternohyoid
muscles are well developed. The longiis capitis and colli are represented by a few
strands along the anterior surfaces of the vertebral plate. The scaleni medii and
posteriores are present as flattened bands of muscle arising in this region and
inserted in the first and second ribs near their bases. The scaleni anteriores are
symmetrical. They arise from the lateral processes of the superior cervical vertebrae and insert on the first rib near its center. The nerve trunks of the cervical
and brachial plexus pass under these muscles and are tightly bound down bj^ them.


Of the more anterior thoracic muscles, the pectorales are not disturbed. Both
serati anterior muscles are defective and difTer in their defects. On the right side
there is more complete development. Here slips of the muscle arising from the
distal portions of the first four ribs and from the eighth to the tenth ribs converge
and are inserted around the inferior angle of the scapula. A few strands of muscle
on the chest wall between the pectoraUs minor and the seratus are present, which
might be remnants of th(! latter. They are shaped like half a crescent, with fibers
running longitudinally, and extend from the first rib, where they are 3 mm. broad,
to the fifth rib, where they are 15 mm.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 105


first-zone layer containing few cells and a well-marked second layer with only a
narrow zone of undifferentiated cortex beneath it. No calcarine type of cortex was
identified, but the occipital lobe was probably included in the hemorrhagic mass
in the encephaloceles, which was in such a bad state of preservation that no sections
could be made.


102 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH
On the right side of the cerebrum the fissure corresponding to the left central
fissure of Rolando is situated slightly more anteriorly than on the left. The frontal
lobes thus occupy most of the shallow vault and rest in the anterior and in the
medial cranial fossae as well, which latter normally hold the temporal lobes. The
sulci of the frontal lobes are changed considerably from their normal positions.
The anterior ends of the superior medial and inferior frontal sulci are bent very
sharply downward. They are all situated nearer the base of the brain than usual,
as if the tissue had been pulled down on the lateral surfaces. Thus the superior
sulci lie halfway down the sides, with the medial and inferior sulci correspondingly
below them. With the exception of part of the parietal lobes on each side, the rest
of the cerebrum lies below the foramen magnum. The left-sided position of the
falx cerebri allows more room in the cranial cavity proper for the right cerebral
hemisphere than for the left. This results in a larger portion of the parietal lobe
on the right side lying above the foramen magnum than on the left side, and correspondingly a larger amount of parietal surface lying below the foramen on the
left than on the right side.


On the loft side the seratus anterior is very imperfect. It is represented l)y a
The cerebral tissue which lies below the foramen is partly contained in the  
thin sheet of fascia, which originates from the first three ribs and is inserted in the  
large bony pocket formed bj' the upper vertebral plate, and partly in the middle
scapula along the vertebral margin near the medial angle. A few scattered musclefibers, which also probably represent remnants of the serati, arise over the fourth
and left encephalocele. Sulci are present over its surface, but are so distorted
rib near its base and are inserted into the inferior angle of the scapula. The origin
that they can not be identified, nor can the identity of the lobes be determined.  
of the fascia and these muscle-fibers is shown in figure 21 by dotted lines. Some
In the contents of the middle and left sacs one can easily discern cerebral gyri,  
other muscles on the left chest wall, consisting of irregular projections from the
and the general histological structure of these is similar to that of the cerebral tissue
dorsal bundle which covers the proximal part of the first seven ribs, may be serati
situated above the foramen. In the middle sac there is a large amount of clot. The
fibers which remained in their embryonic position close to the axis. Fibers which
cerebral tissue which lies in the cervical and thoracic vertebral pocket is pressed out
jjrobably represent intercostal muscles pushed to the outer surface of the ribs are
into a thin shell, and hes next the dura, being limited anteriorly by the emerging
arranged along the lower border of the fourth rib. These extend onto the lower
cranial nerves. On the sides and back it is continuous with the cerebral tissue
adjoining ribs. At the outer end they are 2 mm. across and near the base of the  
lying in the cranial vault and with that pressed out into the encephaloceles.  
ribs they measure 20 mm. (See fig. 21.) Three small muscle bundles are situated
at the distal end of the above-mentioned fibers.  


The lateral and anterior abdominal muscles are well developed. Each rectus
In the interior of the brain the optic thalami may be identified, h'ing above the
is 7.4 cm. by 3.2 cm. The right rectus has two inscriptiones tendinae in its upper
foramen. A small space representing the third ventricle, greatly compressed, lies
one-third opposite the sixth and seventh ribs.  
between the thalami. Choroid plexus tissue is present. Its relations, however,
to the adjoining structures could not be determined. The cerebral peduncles may
be seen as flattened bundles lying central to the shell of the cerebral cortex. The
optic nerves are present. The hypophysis lies embedded in the well-formed sella
turcica. No other structures in this region or below can be identified until, in the
pocket of bone formed by the thoracic vertebrae, the inverted floor of the fourth
ventricle is recognized.
 
The midbrain wiih attached fourth nerves, the colliculi, and the aqueduct of
Sylvius were not identified. The fourth nerves, however, were found at their
dural exit. Judging from the position of the fourth ventricle floor, a sharp bend


To summarize : Those muscles which have undergone most disturliance are the
trapezei, the rhomboidei, the serati posteriores superiores, the serati anteriores, and
the sacrosi)inalis and short back muscles. The location of these muscular abnormalities, situated near the chief skeletal abnormalities, demonstrates still further
that the ijathological process is a rather sharply circumscribed one, limited to the
neighborhood of the axis. The inclusion of the anterior serati in this group does
not contradict the statement, as the early anlage of the serati is very near the axis.


The muscle disturbances of "monsters" have been but little investigated or
recorded. From the scattered observations at present obtainable, any correlation
is impossible.


VISCERA.
106 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH


On dissection, the viscera are found crowded and somewhat distorted, but,
with the angle directed posteriorly must have occurred in the midbrain region.
with the excejition of the right lung, are well developed. The thyroid is bilobed
At the beginning of the spinal cord a bend in the opposite direction is present.  
and measures 1.5 by 1 cm., the thymus measures 6 by 2.8 by 1.1 cm. The esophagus measures 4 cm. from epiglottis to cardia. The lesser curvature of the stomach
This bending of the brain stem and cord must have been in lateral outline shaped
is 1 cm. and its greater curvature 8 cm. The intestines are well formed. The  
like a crudely drawn letter Z, as shown in figure 24, which is a diagram of various
api)endix measures 8.5 cm. The cok)n is much bent upon itself. Because of unskillful handling the positions asssunied l)y the rest of the intestinal tract were not
structures in the central nervous system near the midline. The surface of the  
ascertained. The pericardium, ])leura, and diapluagm are intact. The heart is
inverted fourth ventricle floor is shaped like an isosceles triangle with its tip,
well developed. Sagittal section sliows it cut through the right ventricle and left
which is its normal anterior end, directed backward toward the encephaloceles.  
auricle. The valves are well formed. The ductus arteriosus is patent. The left
The median sulcus is well defined. The tissue next to the median sulcus on both
lung is approximately normal; its lateral surface is shown in figure 23; it consists
sides is slightly raised. The rest of the surface is flat. For estimating roughly
of two lobes and measures 3.9 cm. antero-posteriorly by 3.1 cm. perpendicularly
the amount of distortion this fourth ventricle floor had undergone, a comparison
by 1.7 cm. in its thickest niedio-lateral diameter near the hilum. The right lung,
of it with the fourth ventricle floor of three normal full-term fetuses was made.  
side view of which is shown in figure 22, about ('(juals the left in volunu^ and is
Each of the three showed a similar longitudinal ridge to be the extent of their surface
roughly a flattened cone-shape with apex directed anteriorly. Its corresi)onding
markings. The main difference which this specimen showed seemed to be in a
measurements are 5.1 by 3.2 by 2.9 cm. it is formed of only one lobe. Along tlie
blunting of the posterior end which forms the base of the triangle already referred to.  
margins four short fissures exist, directed toward tlu' center; one 12 mm. in length


From both lateral margins of the fourth ventricle floor cortical tissue resembling
the flocculus is drawn backward, downward, and to the right, joining the cerebellar
cortex contained in the right sac. It must be noted that while the flocculus is
directed toward the posterior end of the specimen as a whole, it is drawn toward
the end of the ventricle floor normally anterior. There is a much disturbed choroid
plexus folded in with the cerebellar tissue. A fairly large amount of cerebellar
tissue is present; part of this is drawn out into a sheet which is continuous with the
flocculus and which passes posteriorly and to the right into the right encephalocele,
where it lies next to a rounded mass of cerebellar cortex. Bands of tissue connecting the cerebellum and cerebrum i^robably represent the brachium conjunctivum. There are smaller flat bands of tissue passing backward near the flocculus
to the rounded cerebellar cortex .which may have been remnants of the inferior
cerebellar peduncles. There is no pontine enlargement.


From the tissue superior to and continuous with the floor of the fourth ventricle, the third and the fifth to the twelfth paired cranial nerves pass forward to .
their normal exits from the subdural space. They are elongated to between 20
and 30 mm. Their origin from the brain-stem lies ()i)i)osite the first thoracic vertebra. In this region the beginning of the flattened cord can be made out, which is
bent double upon itself. Some interest is attached to this Z bend of the brain-stem
and cord. It seems to have been brought about in this case through traction
on these parts by the major portions of the central nervous system slipping
through the enlarged foramen magnum. \'arying degrees of such kinking have been
described. The condition in its milder forms has received the name of Chiari
deformity, from a case described by Chiari in which the medulla is l)ent l)ack over
the cord for only a short extent. In Nageli's case of cyclopia there is a marked
degree of such bending associated with splitting of the cord.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 103
Caudal to the bend as a flat band the .spinal cord extends to the level of the
lumbar vertebra, where it terminates in a cauda etiuina. From its ventral surface
the spinal nerves extend into the dura. At the level of the twelfth thoracic verte])ra
the spur in the vertebral plate has left an indentation on the flat cord and on the
right anterior third of the inverted floor of the fourth ventricle.  


is situated on the posterior margin at the junction of the superior third with the
middle third; on the inferior margin near its middle a similar fissure is situated,
and halfway between it and the anterior end of the lung a shorter fissure 3.5 mm.
long exists; on the superior margin another, 3.5 mm. in length, is present slightly
anterior to the middle. These fissures are very superficial and extend for only a
few millimeters on the medial surface of the lung. The relations of the bronchial
tree were not determined.


The liver is flattened out horizontally and shaped like an L with the angle
projecting anteriorly, the gall-bladder, which is 4.1 cm. in length, being situated
on the inferior surface of the long arm of the L. The closed end of the gallbladder lies near the tip of the angle and its long axis is directed diagonally toward
the upper end of the latter. The spleen is 2.2 by 1.3 by 1 cm. The presence of
the pancreas is determined histologically. It lies embedded in ti.ssue near the vertebral column. Both kidneys and adrenals are somewhat compressed and distorted,
the left much more than the right. The right kidney is somewhat flattened from side
to side and at its upper end, and measures 4.5 by 1.2 by 3.5 cm. The right adrenal
lies above it and measures 3 by 2 bj^ 0.5 cm. The left kidney is bent upon itself and
folded in with its closely adherent adrenal, so that together they form a rounded
mass measuring 4.7 by 3.4 by 2.4 cm. The greater distortion of the left kidney
and adrenal is very probably due to crowding, a result of the left-sided concave
bending of the vertebral column in this region and fusion of the lower ribs on that
side. The ureters and bladder are well formed. The uterus, tubes, and ovaries
are well developed. Blocks of tissue of heart, kidney, liver, and adrenal were run
through by the Levaditi method for spirochaetes by Dr. Bullard, with negative
findings. It is to be noted that the tissues had been kept in carbolic, which is not
the fixation recommended for this method.


The developmental anomalies of the soft palate and the right lung are the most
ENCEPHALOCELES AND OTHER ABNORMALITIES. 107
marked changes which have taken place in the soft tissues anterior to the vertebral
 
column. They are both examples of arrested development and are secondary to,
The central canal, as such, is absent. Throughout the extent of the cord it
and probably the mechanical result of, 'the deformit.y of the vertebrae.  
is changed to a flat space following the contour of the vertebral column, whose floor
is the cord and whose roof is partly the same cord inverted, partly the inverted
floor of the fourth ventricle, and partly cerebellar tissue.
 
This fragmentary description of the central nervous system leaves much to  
be desired. It would have been especially desirable had we been able to present
a clear picture of the relationships of the meninges. The main conclusion which
can be drawn from its study is that the chief disturbance here evidenced is primarily
one of distortion, rather than of absence or real lack of development of nerve-tissue.  
 
CONCLUSION


CRANIAL CAVITY AND CENTRAL NERVOUS SYSTEM.
The exterior alone of such a specimen as this certainly presents striking evidence that an organism can undergo most serious disturbances and yet maintain
The shape assumed by the cerebro-spinal cavity or subdural space is shown by
a definite though limited growth balance ; but in order to ascertain in detail exactly
figures 4, 5, 6, and 7 of the wax model. In figures 12 and 21 the model is given in  
what constitutes the limitations of this equilibrium more intensive study is  
its relation to the skeleton. The space consists of a shallow dome which contained
necessary. A rather intereisting series of anomalies is the result of such a study in  
the frontal and part of the parietal cerebral lobes. Below this dome a relatively
this ca.se. It may be noted that these anomahes are centered about the axis. The  
slight constriction in the model denotes the enlarged foramen magnum (figs. 4, 5, 7,  
bony parts, the central nervous S3^^tem, certain adjacent muscles, and overlying
and 12). Under the foramen three rounded encephaloceles project posteriorly, and
areas of integument share profoundly in this disturbance. Subsidiary disturbances
below these the pointed termination of the spinal canal may be seen. Situated
of development are evidenced in a split soft palate and a one-lobed right lung.
ventral to the encephaloceles and continuous with them and with the base of the
These facts, in addition to supplying a clearer knowledge regarding the individual
dome and the spinal canal is a blunt, wedge-shaped mass marked iv in figures 4, 5,
specimen, contribute their small share in providing data for the better understanding
and 6. This portion fits into the pocket of bone formed by the thoracic and vertebral plate. With the exception of the inside of the large encephalocele, the space
of certain general problems of development. Classifications and analyses included
occupied by the central nervous system is lined with a continuous sheet of smooth
in such subjects as osteolog}^, myology, and organology can not be regarded as
dura. At the foramen magnum and in between the eminentiae arcuatae of the tem
complete until they contain a comprehensive picture of teratological phenomena.
This is almost entirely lacking at present. The teratological material has been so
scanty that any satisfactory correlation of it has been impossible.  
 
Up to f airh" recent times teratology was considered an isolated science ; it was
thought that the laws applying to most jiatural phenomena were not applicable to
its conditions, that it could not learn from or contribute to the normal sciences.
Studies of the past half century have entirely reversed this view. Teratology today has for its basis the same fundamental sciences of chemistry, biology, and  
physics as has those sciences whose subject-matter deals with normal phenomena.
It is constantly learning from these latter sciences, and in turn has been able to contribute suggestions on points of analysis or exposition regarding puzzling phases of
normal development.  
 
The necessity of furthering our knowledge regarding the etiological factors of  
specific abnormal conditions has been^considered. Material at such an advanced
stage of development as this specimen can contribute but Uttle along this line. We
can not determine by means of it the primary defect, nor again, except in a very
general way, a chronological picture of the early processes. We must turn to embryological material and to other than morphological methods to obtain such
knowledge.  




104 STUDY OP A HUMAN SPINA BIFIDA MONSTER WITH
ponil bones and the sella turcica it is drawn into numerous folds. On the inside
of tlio large encephalocele the smooth dural surface changes to a tissue composed
of many blood-vessels, fibrin, and extravasated blood, as described in the beginning
of the paper. The fabc cerebri lies well over on the left side in its anterior and
middle portions; posteriori}' it ends in a single fold about the center of the superior
margin of the occipital bone. The tentorium cerebelli is absent. The ventral
surface of the subdural space is pierced by two rows of cranial and spinal nerves.
These number 43 in all, 12 cranial and 31 spinal, the latter distributed as follows:
8 cervical, 12 thoracic, 5 lumbar, 5 sacral, and 1 coccygeal. Both the anterior
roots and the posterior roots with their ganglia are identified. In the cervical
and thoracic regions they are extremely crowded.


The arrangement of the central nervous system is very complicated. The
BIBLIOGRAPHY.  
cerel)rum lies part above and part below the foramen magnum. The cerebellum lies
entirely below it. The medulla and cord lie bent and crumpled ventral to the other
structures below the foramen. The surface of that part of the cerebrum which
lies above the foramen shows definite fissure and sulcus formation. Below the
foramen a large part of the contents of the encephaloceles consists of a hemorrhagic
mass and much of the surface of this is covered with caked blood, so that only in
a few [)laces can typical cerebral surface markings be identified, flections of these
regions, however, show definite though rather undifferentiated cortical lamination.  


From a brief studj' of sections of other parts of the left cerebrum, made by Dr.
Charles Bagley, the following points are determined : The cortical structure for the
most part is composed of a very wide granular zone, which is characteristic of the
early and undifferentiated stage of lamination. There is, however, a prominent vertical fissure lying at tlie junction of the middle and posterior two-thirds of the cranial
dome, which can be identified as the central fissure of Rolando; the ventral termination of this fissure rests on the eminentia arcuata of the temporal bone. The
cortical tissue anterior to this fissure shows a definite lamination. There is a welldefuied first layer; a broad and poorl}' differentiated second layer; the third laj^er
is of medium width and is filled with small pyramidal cells; the fourth layer is
inconsi)icuous, suggesting the agranular naotor tyi)e; while the fifth layer is represented l\y large pyramidal cells, ])r()l)ably Betz cells. These cells are at least
three times as large as an}' other cells which could l)e found in the cortex and it is
owing to their presence and to the very narrow gramilar layer that this area is identified as motor cortex. Immediately posterior to this fissure there is a sharp change
in lamination types. Thc^ large cells are lost and the fifth layer is represented by
definitely sha])ed pyramidal cells of not more than one-third the size of those cells
de.signated as Jietz cells. Above this layer there is present a very heavy granular
layer which is (|uite a contrast to the narrow granular layer of the area just described. It may be said, therefore, with a fair degre(> of certainty, that the tissue
posterior to the fissure represents sensory cortex.


From the rather limited amount of material studied the only other localization
that could be determined is that the cortical tissue i)ushetl down on the side of the
wedge-shaped mass (11' in the model) represents subiculum. Here, passing from a
cortex of (|iiilc deep cxlcnl, it becomes suddenly shallow and consists of atypical


Aebt, Chb., 1879. Die Altersverschiedenheiten der
menschlichen Wirbelsaule. Arch. f. Anat. u.
Physiol., Anat. Abt., pp. 95, 109.


Baldwin, \V. M., 191.5. The action of ultra-violet rays
upon the frog's egg. Anat. Record, i.\, SG.^-SSl.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 105
Ballantyne, J. VV., 1904. The embryo. Antenatal
pathology and hygiene, Edinburgh, 272, 285.
 
Bardeen, C. R., and W. H. Lewis, 1901. Development
of the limb.s, body-wall, and back in man. Amer.
Journ. Anat., l, 1-35.  


first-zone layer containing few cells and a well-marked second layer with only a
Bayer, C, 1889, 1890, 1892. Zur Chirurgio der Ruckenmarksbriiche. Prager med. Wochenschr., xiv,
narrow zone of undifferentiated cortex beneath it. No calcarine type of cortex was
227-230; xv, 48; xvii, 317, 332, 345.  
identified, but the occipital lobe was probably included in the hemorrhagic mass
in the encephaloceles, which was in such a bad state of preservation that no sections
could be made.  


On the right side of the cerebrum the fissure corresponding to the left central
Bettmann, 1912. Die Missbildung der Haut. In E.  
fissure of Rolando is situated slightly more anteriorly than on the left. The frontal
Schwalbe, Die Morphologie der Missbildungen,
lobes thus occupy most of the shallow vault and rest in the anterior and in the
III Teil, VII Lieferung, 2. Abt., 7. Kap. Jena.  
medial cranial fossae as well, which latter normally hold the temporal lobes. The
sulci of the frontal lobes are changed considerably from their normal positions.  
The anterior ends of the superior medial and inferior frontal sulci are bent very
sharply downward. They are all situated nearer the base of the brain than usual,  
as if the tissue had been pulled down on the lateral surfaces. Thus the superior
sulci lie halfway down the sides, with the medial and inferior sulci correspondingly
below them. With the exception of part of the parietal lobes on each side, the rest
of the cerebrum lies below the foramen magnum. The left-sided position of the
falx cerebri allows more room in the cranial cavity proper for the right cerebral
hemisphere than for the left. This results in a larger portion of the parietal lobe
on the right side lying above the foramen magnum than on the left side, and correspondingly a larger amount of parietal surface lying below the foramen on the
left than on the right side.  


The cerebral tissue which lies below the foramen is partly contained in the
Binder, 1899. Das Morelsche Ohr. Arch. f. Psychiat.,
large bony pocket formed bj' the upper vertebral plate, and partly in the middle
XX, 514-564.  
and left encephalocele. Sulci are present over its surface, but are so distorted
that they can not be identified, nor can the identity of the lobes be determined.  
In the contents of the middle and left sacs one can easily discern cerebral gyri,
and the general histological structure of these is similar to that of the cerebral tissue
situated above the foramen. In the middle sac there is a large amount of clot. The
cerebral tissue which lies in the cervical and thoracic vertebral pocket is pressed out
into a thin shell, and hes next the dura, being limited anteriorly by the emerging
cranial nerves. On the sides and back it is continuous with the cerebral tissue
lying in the cranial vault and with that pressed out into the encephaloceles.  


In the interior of the brain the optic thalami may be identified, h'ing above the
Brodman, K., 1910. Feinere Anatomic des Gros.shims,  
foramen. A small space representing the third ventricle, greatly compressed, lies
p. 206. In M. Lewandowsky, Handbuch der Neurologic. Erster Teil, ii. .\natomie. Berlin.
between the thalami. Choroid plexus tissue is present. Its relations, however,  
 
to the adjoining structures could not be determined. The cerebral peduncles may
ChiaRI, 1891. Veriinderungen des Kleinhirns in Folge
be seen as flattened bundles lying central to the shell of the cerebral cortex. The
von Hydrocephalic des Grosshirns. Deutsche
optic nerves are present. The hypophysis lies embedded in the well-formed sella
med. VVochensclir., xvii, 1172-1 175.  
turcica. No other structures in this region or below can be identified until, in the
pocket of bone formed by the thoracic vertebrae, the inverted floor of the fourth
ventricle is recognized.  


The midbrain wiih attached fourth nerves, the colliculi, and the aqueduct of
Ernbt, Paul, 1909. Missbildungen des Nervensystems.
Sylvius were not identified. The fourth nerves, however, were found at their
I. Storungen des Verschlusses in der hinteren
dural exit. Judging from the position of the fourth ventricle floor, a sharp bend
Mittellinie des Korpers. In E. Schwalbe, Morphologie der Missbildungen, III Teil, I Lieferung,  


II Abt., II Kap., I. p. 72.


, 1909. Mis.sbildungen des Nervensystems, X.


106 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH
Entwickelungsstorungen des Kleinhirns. In E.


with the angle directed posteriorly must have occurred in the midbrain region.
Schwalbe, Morphologie der Missbildungen, III
At the beginning of the spinal cord a bend in the opposite direction is present.
This bending of the brain stem and cord must have been in lateral outline shaped
like a crudely drawn letter Z, as shown in figure 24, which is a diagram of various
structures in the central nervous system near the midline. The surface of the
inverted fourth ventricle floor is shaped like an isosceles triangle with its tip,
which is its normal anterior end, directed backward toward the encephaloceles.
The median sulcus is well defined. The tissue next to the median sulcus on both
sides is slightly raised. The rest of the surface is flat. For estimating roughly
the amount of distortion this fourth ventricle floor had undergone, a comparison
of it with the fourth ventricle floor of three normal full-term fetuses was made.
Each of the three showed a similar longitudinal ridge to be the extent of their surface
markings. The main difference which this specimen showed seemed to be in a
blunting of the posterior end which forms the base of the triangle already referred to.


From both lateral margins of the fourth ventricle floor cortical tissue resembling
Teil, I Lieferung, II Abt., II 2. Kap., (X) p. 223
the flocculus is drawn backward, downward, and to the right, joining the cerebellar
GiLLASPiE, C, L. I. Miller, and M. Baskin, 1916.  
cortex contained in the right sac. It must be noted that while the flocculus is
directed toward the posterior end of the specimen as a whole, it is drawn toward
the end of the ventricle floor normally anterior. There is a much disturbed choroid
plexus folded in with the cerebellar tissue. A fairly large amount of cerebellar
tissue is present; part of this is drawn out into a sheet which is continuous with the
flocculus and which passes posteriorly and to the right into the right encephalocele,  
where it lies next to a rounded mass of cerebellar cortex. Bands of tissue connecting the cerebellum and cerebrum i^robably represent the brachium conjunctivum. There are smaller flat bands of tissue passing backward near the flocculus
to the rounded cerebellar cortex .which may have been remnants of the inferior
cerebellar peduncles. There is no pontine enlargement.  


From the tissue superior to and continuous with the floor of the fourth ventricle, the third and the fifth to the twelfth paired cranial nerves pass forward to .  
Anomalies in lobation of lungs. .Vnat. Rec, xi,  
their normal exits from the subdural space. They are elongated to between 20
 
and 30 mm. Their origin from the brain-stem lies ()i)i)osite the first thoracic vertebra. In this region the beginning of the flattened cord can be made out, which is
65-75.  
bent double upon itself. Some interest is attached to this Z bend of the brain-stem
Good, J. Percy, 1912. Spina bifida in the neck region of  
and cord. It seems to have been brought about in this case through traction
on these parts by the major portions of the central nervous system slipping
through the enlarged foramen magnum. \'arying degrees of such kinking have been
described. The condition in its milder forms has received the name of Chiari
deformity, from a case described by Chiari in which the medulla is l)ent l)ack over
the cord for only a short extent. In Nageli's case of cyclopia there is a marked
degree of such bending associated with splitting of the cord.


Caudal to the bend as a flat band the .spinal cord extends to the level of the
a ferret embryo, 8 mm. Journ. Anat. and Physiol.,
lumbar vertebra, where it terminates in a cauda etiuina. From its ventral surface
the spinal nerves extend into the dura. At the level of the twelfth thoracic verte])ra
the spur in the vertebral plate has left an indentation on the flat cord and on the
right anterior third of the inverted floor of the fourth ventricle.  


XLVi, 391-399.
Graves, Wm. W., Scapula scaphoidea, eine hiiufig


vorkonunende .\nomalie des SiOiulterblattes. Ihr.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 107
Zusammcnhang mit Syphilis in der Ascondens.  


The central canal, as such, is absent. Throughout the extent of the cord it
Med. Klin., 1911, No. S, p. 298.  
is changed to a flat space following the contour of the vertebral column, whose floor
Gruber, Wenzel, 1872. Ueber einen fortsatzartigen
is the cord and whose roof is partly the same cord inverted, partly the inverted
 
floor of the fourth ventricle, and partly cerebellar tissue.  
cylindrischen Hocker an der Vorderfliiche des
 
Angulus superior der Scapula. Virchow's Arch.  


This fragmentary description of the central nervous system leaves much to
f. path. Anat., Lvi, 425.
be desired. It would have been especially desirable had we been able to present
GrUnbero, Karl, 1913. Die Mi.sslMldungen des Kop
a clear picture of the relationships of the meninges. The main conclusion which
fes. Die Spalte des weichen (laurneiis. In K.  
can be drawn from its study is that the chief disturbance here evidenced is primarily
one of distortion, rather than of absence or real lack of development of nerve-tissue.  


CONCLUSION
Schwalbe, Die MorphoIogi(' der Mis.sl)ildungen.


The exterior alone of such a specimen as this certainly presents striking evidence that an organism can undergo most serious disturbances and yet maintain
III Teil, IX Lieferung, 1 Abt. 4. Kap.  
a definite though limited growth balance ; but in order to ascertain in detail exactly
Gudden, II., 1898. Fall von Knickung der Medulla
what constitutes the limitations of this equilibrium more intensive study is
necessary. A rather intereisting series of anomalies is the result of such a study in
this ca.se. It may be noted that these anomahes are centered about the axis. The
bony parts, the central nervous S3^^tem, certain adjacent muscles, and overlying
areas of integument share profoundly in this disturbance. Subsidiary disturbances
of development are evidenced in a split soft palate and a one-lobed right lung.  
These facts, in addition to supplying a clearer knowledge regarding the individual
specimen, contribute their small share in providing data for the better understanding
of certain general problems of development. Classifications and analyses included
in such subjects as osteolog}^, myology, and organology can not be regarded as
complete until they contain a comprehensive picture of teratological phenomena.
This is almost entirely lacking at present. The teratological material has been so
scanty that any satisfactory correlation of it has been impossible.  


Up to f airh" recent times teratology was considered an isolated science ; it was
oblongata und Theilung des Riickenmarks.  
thought that the laws applying to most jiatural phenomena were not applicable to
its conditions, that it could not learn from or contribute to the normal sciences.
Studies of the past half century have entirely reversed this view. Teratology today has for its basis the same fundamental sciences of chemistry, biology, and
physics as has those sciences whose subject-matter deals with normal phenomena.
It is constantly learning from these latter sciences, and in turn has been able to contribute suggestions on points of analysis or exposition regarding puzzling phases of
normal development.  


The necessity of furthering our knowledge regarding the etiological factors of
Arch. f. Psychiat., XXX, 866-876.
specific abnormal conditions has been^considered. Material at such an advanced
Hebtwio, O., 1892. IJrmund und Spina bifida. .\rch.  
stage of development as this specimen can contribute but Uttle along this line. We
can not determine by means of it the primary defect, nor again, except in a very
general way, a chronological picture of the early processes. We must turn to embryological material and to other than morphological methods to obtain such
knowledge.  


f. mikr. Anat., xxxix, 353-530.
HiLDEBRAND, 1893. Ucber Spina bifi<la. Verhandl.


d. deiitschen Gesellsch. f. Chir., xxil, 41-43.


BIBLIOGRAPHY.




HoRwiTZ, A. E., 1908. Congenital elevation of the


Aebt, Chb., 1879. Die Altersverschiedenheiten der
scapula, Sprengel's Deformity. Amer. Journ.  
menschlichen Wirbelsaule. Arch. f. Anat. u.
Physiol., Anat. Abt., pp. 95, 109.  


Baldwin, \V. M., 191.5. The action of ultra-violet rays
Orthop. Surg., vi, 260-311.  
upon the frog's egg. Anat. Record, i.\, SG.^-SSl.  
Kermauner, 1909. Missbildungen des Rumpfes. In


Ballantyne, J. VV., 1904. The embryo. Antenatal
E. Schwalbe, Morphologie der Missbildungen, III
pathology and hygiene, Edinburgh, 272, 285.


Bardeen, C. R., and W. H. Lewis, 1901. Development
Teil, I Lieferung, I .\bt., 3. Kap., p. 86.  
of the limb.s, body-wall, and back in man. Amer.  
Koch, Wilhelm, 1881. Mittheilungen iiber Fragen der
Journ. Anat., l, 1-35.  


Bayer, C, 1889, 1890, 1892. Zur Chirurgio der Ruckenmarksbriiche. Prager med. Wochenschr., xiv,
wissenschaftUchen Medicin. 1. Heft. Beitrage
227-230; xv, 48; xvii, 317, 332, 345.  


Bettmann, 1912. Die Missbildung der Haut. In E.  
zur Lehre von der Spina bifida. Kassel.  
Schwalbe, Die Morphologie der Missbildungen,
Lewis, H. F., 1897. Iniencephalus. Amer. Journ.  
III Teil, VII Lieferung, 2. Abt., 7. Kap. Jena.  


Binder, 1899. Das Morelsche Ohr. Arch. f. Psychiat.,  
Obst., xxxv, 11-53.  
XX, 514-564.  
Lewis, W. H. (See Bardeen and Lewis.)
LiLLiE, Ralph D., 1917. Variations of canalis hypo
glossi. Anat. Record, xiii, 131-144.
Macklin, C. C., 1914. The skull of a human fetus of


Brodman, K., 1910. Feinere Anatomic des Gros.shims,  
40 mm. Amer. Journ. Anat., xvi, 317, 387.  
p. 206. In M. Lewandowsky, Handbuch der Neurologic. Erster Teil, ii. .\natomie. Berlin.  
Mall, F. P., 1908. A study of the causes underlying the


ChiaRI, 1891. Veriinderungen des Kleinhirns in Folge
origin of human monsters. Wistar Institute
von Hydrocephalic des Grosshirns. Deutsche
med. VVochensclir., xvii, 1172-1 175.  


Ernbt, Paul, 1909. Missbildungen des Nervensystems.  
of Anatomy and Biology. Philadelphia. (Journ.  
I. Storungen des Verschlusses in der hinteren
Mittellinie des Korpers. In E. Schwalbe, Morphologie der Missbildungen, III Teil, I Lieferung,


II Abt., II Kap., I. p. 72.  
Morphol., XIX, 1-367.)
Marchand, 1899. Spina bifida. Missbildungen. Real


, 1909. Mis.sbildungen des Nervensystems, X.  
Encyclopadie der gesamraten Heilkunde, von A.  


Entwickelungsstorungen des Kleinhirns. In E.  
Eulenburg. 3 Aufl., 1899, xxii, 578-601.
Martin, Rudolf, 1914. Lehrbuch der Anthropologic.  


Schwalbe, Morphologie der Missbildungen, III
Jena.
Marx, Hermann, 1911. Die Missbildungen des Ohres.


Teil, I Lieferung, II Abt., II 2. Kap., (X) p. 223
In E. Schwalbe, Morphologie der MLssbildungen,  
GiLLASPiE, C, L. I. Miller, and M. Baskin, 1916.


Anomalies in lobation of lungs. .Vnat. Rec, xi,  
III Teil, V Lieferung, II Abt., 6. Kap. Jena.  
Mdscatello, 1894. L^eber die angeborenen Spalten des


65-75.  
Schadels und der Wirbelsaule. Arch. f. klin.  
Good, J. Percy, 1912. Spina bifida in the neck region of


a ferret embryo, 8 mm. Journ. Anat. and Physiol.,
Chir., XLVii, 162, 257.  
Naeqeli, O., 1897. Ueber eine neue mit Cyclopie ver
kniipfte Missbildung des Contralnervensystems.  


XLVi, 391-399.  
Arch. f. Entwickelungsmechanik, v, 168-218.  
Graves, Wm. W., Scapula scaphoidea, eine hiiufig
VON Recklinghausen, F., 1886. Untersuchung ilber


vorkonunende .\nomalie des SiOiulterblattes. Ihr.  
die Spina bifida. Virchow's Arch. f. path. .4nat.,


Zusammcnhang mit Syphilis in der Ascondens.  
cv., 243, 373.
Schneider, 1912. Die Missbildungen der .\tmung9or
gane. In E. Schwalbe; Die Morphologic iler Missbildungen, III Teil, VIII Lieferung, II Abt., 8.  


Med. Klin., 1911, No. S, p. 298.  
Kap.  
Gruber, Wenzel, 1872. Ueber einen fortsatzartigen
Spietschka, T., 1894. Hautveriinderiing bei Spina


cylindrischen Hocker an der Vorderfliiche des  
bifida. Prager med. Wochenschr., xix, 117, 131.
Sprenqel, 1891. Die angeborene Ver.schiebung des  


Angulus superior der Scapula. Virchow's Arch.  
Schulterblattes nach oben. Arch. f. klin. Chir.,


f. path. Anat., Lvi, 425.  
XLii, 54.5-549.  
GrUnbero, Karl, 1913. Die Mi.sslMldungen des Kop
Streeter, G. L., 1903. Anatomy of floor of the fourth
fes. Die Spalte des weichen (laurneiis. In K.  


Schwalbe, Die MorphoIogi(' der Mis.sl)ildungen.  
ventricle. Amer. Journ. Anat., ii, 299-313.  
, 1915. Development of the venous sinuses of


III Teil, IX Lieferung, 1 Abt. 4. Kap.
the dura mater in the human embryo. Amer.  
Gudden, II., 1898. Fall von Knickung der Medulla


oblongata und Theilung des Riickenmarks.  
Journ. Anat., xviii, 145-178.
TiLLMANNS, H., 1905. Die Verletzungen und chirur
gischen Krankheiten des Beckens. Deutsche


Arch. f. Psychiat., XXX, 866-876.  
Chirurgic, Lieferung 62a, p. 497.  
Hebtwio, O., 1892. IJrmund und Spina bifida. .\rch.  
WHed, Lewis H., 1916. The development of the
 
cerebro-spinal spaces in pig and in man. Contributions to Embryology, v. 5, No. 14. Carnegie


f. mikr. Anat., xxxix, 353-530.  
Inst. Wash. Pub. No. 226.  
HiLDEBRAND, 1893. Ucber Spina bifi<la. Verhandl.  


d. deiitschen Gesellsch. f. Chir., xxil, 41-43.




108


HoRwiTZ, A. E., 1908. Congenital elevation of the


scapula, Sprengel's Deformity. Amer. Journ.


Orthop. Surg., vi, 260-311.  
EXPLANATION OF PLATES.  
Kermauner, 1909. Missbildungen des Rumpfes. In
ABBREVIATIONS.  


E. Schwalbe, Morphologie der Missbildungen, III


Teil, I Lieferung, I .\bt., 3. Kap., p. 86.
Koch, Wilhelm, 1881. Mittheilungen iiber Fragen der


wissenschaftUchen Medicin. 1. Heft. Beitrage
anth.,


zur Lehre von der Spina bifida. Kassel.
Lewis, H. F., 1897. Iniencephalus. Amer. Journ.


Obst., xxxv, 11-53.
anthelix.  
Lewis, W. H. (See Bardeen and Lewis.)
LiLLiE, Ralph D., 1917. Variations of canalis hypo
glossi. Anat. Record, xiii, 131-144.
Macklin, C. C., 1914. The skull of a human fetus of


40 mm. Amer. Journ. Anat., xvi, 317, 387.
Mall, F. P., 1908. A study of the causes underlying the


origin of human monsters. Wistar Institute
m. obi. ext..  


of Anatomy and Biology. Philadelphia. (Journ.


Morphol., XIX, 1-367.)
antitr.,  
Marchand, 1899. Spina bifida. Missbildungen. Real


Encyclopadie der gesamraten Heilkunde, von A.


Eulenburg. 3 Aufl., 1899, xxii, 578-601.
antitragus.  
Martin, Rudolf, 1914. Lehrbuch der Anthropologic.  


Jena.
Marx, Hermann, 1911. Die Missbildungen des Ohres.


In E. Schwalbe, Morphologie der MLssbildungen,
m. pect. maj..  


III Teil, V Lieferung, II Abt., 6. Kap. Jena.
Mdscatello, 1894. L^eber die angeborenen Spalten des


Schadels und der Wirbelsaule. Arch. f. klin.  
b. occ,


Chir., XLVii, 162, 257.
Naeqeli, O., 1897. Ueber eine neue mit Cyclopie ver
kniipfte Missbildung des Contralnervensystems.


Arch. f. Entwickelungsmechanik, v, 168-218.  
basioccipitales.  
VON Recklinghausen, F., 1886. Untersuchung ilber
 


die Spina bifida. Virchow's Arch. f. path. .4nat.,  
m. pect. min.,  


cv., 243, 373.
Schneider, 1912. Die Missbildungen der .\tmung9or
gane. In E. Schwalbe; Die Morphologic iler Missbildungen, III Teil, VIII Lieferung, II Abt., 8.


Kap.  
c. i.,  
Spietschka, T., 1894. Hautveriinderiing bei Spina


bifida. Prager med. Wochenschr., xix, 117, 131.
Sprenqel, 1891. Die angeborene Ver.schiebung des


Schulterblattes nach oben. Arch. f. klin. Chir.,
first coccygeal segment.  


XLii, 54.5-549.
Streeter, G. L., 1903. Anatomy of floor of the fourth


ventricle. Amer. Journ. Anat., ii, 299-313.  
m. quad, lumb..  
, 1915. Development of the venous sinuses of


the dura mater in the human embryo. Amer.


Journ. Anat., xviii, 145-178.
c. c. 7,  
TiLLMANNS, H., 1905. Die Verletzungen und chirur
gischen Krankheiten des Beckens. Deutsche


Chirurgic, Lieferung 62a, p. 497.
WHed, Lewis H., 1916. The development of the


cerebro-spinal spaces in pig and in man. Contributions to Embryology, v. 5, No. 14. Carnegie
costal cartilage of seventh rib.  


Inst. Wash. Pub. No. 226.


m. rect..




108
c. eq.,




Cauda equina.


EXPLANATION OF PLATES.
ABBREVIATIONS.


m. rhomb.,


cer.,


anth.,


cerebrum.


anthelix.


m. sacraspin.


m. obi. ext..


cereb.,


antitr.,


cerebellum.


antitragus.


m. ser. ant..


m. pect. maj..


cr. 2,


b. occ,


second cranial nerve.


basioccipitales.


m. ser. post, inf


m. pect. min.,


cr. 5,


c. i.,


fifth cranial nerve.


first coccygeal segment.


naev.,


m. quad, lumb..


dep. em.,


c. c. 7,


depression made by eminenlia arcuata


costal cartilage of seventh rib.


r. 1 (rib).


m. rect..




c. eq.,


of temporal bone.


Cauda equina.


r. hyp.,


m. rhomb.,


cer.,  
epist.,  




cerebrum.  
episternum.  




m. sacraspin.  
rt. en..  




cereb.,  
eust. t.,  




cerebellum.  
eu.stachian-tube orifice.  




m. ser. ant..  
s. 1,




cr. 2,  
exos.,  




second cranial nerve.  
exostosis.  




m. ser. post, inf
sp. 1,  




cr. 5,  
fak.,  




fifth cranial nerve.  
falx cerebri.  




naev.,
sq. occ.  




dep. em.,  
fl. IV,  




depression made by eminenlia arcuata
floor of fourth ventricle.




r. 1 (rib).  
St.,




for. mag.,




of temporal bone.  
foramen magnum.  




r. hyp.,  
tr.,  




epist.,  
1. 1,  




episternum.  
first lumbar segment.  




rt. en..  
uv.,




eust. t.,  
1.2,  




eu.stachian-tube orifice.
second lumbar segment




s. 1,
V'l




exos.,  
1. 1 rib,  




exostosis.  
left first rib.  




sp. 1,
V. pi..  




fak.,  
1. en.,  




falx cerebri.  
left encephalocele.  




sq. occ.  
w..  




fl. IV,
1. pars lat..  




floor of fourth ventricle.  
left pars laterahs.  




St.,




for. mag.,
mid. en..  




foramen magnum.  
middle encephalocele.  




tr.,  
X.,  




1. 1,
m. lat. dors..  




first lumbar segment.  
m. latissimus dorsi (origin).  




uv.,
xyph..  




1.2,  
m. lev. scap.,  




second lumbar segment
m. levator scapulae (cross-section, fig.




V'l
iii.,




1. 1 rib,




left first rib.  
17, insertion fig. 14).  




V. pi..
*




1. en.,


m.obliquuscxternus abdominis (origin),
m. pectoralis major (origin),
m. pectoralis minor (origin),
m. quadratus lumborum.
m. rectus abdominis (origin),
m. rhomboideus (insertion),
m. .sacrospinaUs (insertion),
m. seratus anterior (insertion).
, m. seratus posterior inferior,
naevus.


left encephalocele.  
right first rib.
 
right hypoglossal canal,
 
right encephalocele.  
w..  
first sacral segment,
first spinal nerve,
squama occipitaUs.  
sternum,
tragus,
uvula,
vertex.  


vertebral plate,
central nervous system occupying bony


1. pars lat..
vertebral pocket,  
 
anomalous bone and its insertion,  
 
xyphoid.  
left pars laterahs.
third ventricle,  
 
absence of soft palate.
 
 
 
mid. en..
 
 
middle encephalocele.
 
 
X.,
 
 
m. lat. dors..
 
 
m. latissimus dorsi (origin).
 
 
xyph..
 
 
m. lev. scap.,
 
 
m. levator scapulae (cross-section, fig.
 
 
iii.,
 
 
 
 
17, insertion fig. 14).
 
 
*
 
 
 
m.obliquuscxternus abdominis (origin),
m. pectoralis major (origin),
m. pectoralis minor (origin),
m. quadratus lumborum.
m. rectus abdominis (origin),
m. rhomboideus (insertion),
m. .sacrospinaUs (insertion),
m. seratus anterior (insertion).
, m. seratus posterior inferior,
naevus.
 
right first rib.
right hypoglossal canal,
right encephalocele.
first sacral segment,
first spinal nerve,
squama occipitaUs.
sternum,
tragus,
uvula,
vertex.
 
vertebral plate,
central nervous system occupying bony
 
vertebral pocket,  
anomalous bone and its insertion,  
xyphoid.  
third ventricle,  
absence of soft palate.  
 
 
 
Plate 1.
 
 
 
Fig. 1. Right lateral view of specimen shows extreme dorsal flex-ion with vertex level with shoulders. Middle and
right encephaloceles show in this view. The distorted right ear here seen is drawn in detail in figure 8. ( x j)
 
Fig. 2. Superior view of the specimen looks directly at the face. Measurements given in table 1 . Transversely across
the forehead at the hair line an artefact puckering e.xtends horizontally for 2 cm. ( x 5) •
 
Fig. 3. The dorsal view shows the shortened trunk, superior surface of head, and encephaloceles. ( x j).
 
Fig. 4. Right lateral surface of subdural cast, showing middle and right encephalocele. (xl)
Fig. 5. Left lateral surface of subdural cast, showing middle and left encephalocele. (xi)>
 
Fig. 6. Ventral surface of subdural cast. ( x j)
 
Fig. 7. Dorsal view of subdural cast. The falx is seen to be to the left of midline, (xi)
Plate 2.
 
Fig. 8. Sketch of right ear (natural size), showing the anthelix unusually prominent. The tragus lies relatively
higher than normal, over rather than horizontally opposite the antitragus. The whole ear very narrow.
 
Fig. 9. Sagittal section. Main outUnes were geometrically projected and detail drawn free-hand. The \ascera
retain approximately their normal position, .\bsence of the soft palate is shown. The tip of the tongue
lies over the left anlage of the spht uvula. The vertebral column is bent and shortened and irregularly fused
in its upper part. The arches of all the vertebrse are lacking. A fibrous band lies over the upper sacral
vertebra, joining the opposing defective arches in that region and forming a short spinal canal. The .section
passes to the left of the sella turcica. The falx cerebri is seen well over on left side. The outhne of the central
nervous system, as is here shown, is used reversed for a diagram in figure 24. The section passes near the
median margin of the left sac. (xD
Fig. 9a. Gives left side of bilateral anlage of uvula and orifice of eustachian tube. (Natural size).
 
Fig. 10. Shows a dorsal view of the mounted skeleton, with scapulae in place. Varying degrees of gaping vertebral arches are shown at different levels of the spinal column. In the cervical and thoracic regions defective vertebral arches are fused together and markedly everted. In the upper lumbar region they are individually distinct, but still widely everted, while in the lower lumbar and sacral regions they are distinct and
bent toward one another. The lumbar transverse processes and the sacral lateral processes are well developed
and the coccyx of four segments is seen bent well to the left. In the lower thoracic region a cartilaginous
spur projects dorsa'vards from the vertebral bodies. All the thoracic and cervical vertebral bodies are
fused together in a single plate. A sUght lateral bending in this plate is present. The foveal surfaces of the
atlas face the reader. The intervertebral foramina show large spaces in the lumbar region, which are a sharp
contrast to the tiny areas of the contracted thoracic intervertebral foramina. On the right, the rough
 
109
 
 
 
no EXPLANATION OF PLATES.
 
surface of tho tip of the first lumbar arch is shown, which joins the occiput; and on I lie left, the second lumbar
arch, whicli does the same. Crowding of the base of the ribs may be seen, including the first to the sixth on
the right and the fifth to the ninth on the left. The sternum is considerably to the left of the milline.
A persistent epistermmi is i)resent as a small cartilaginous knob, surrounding the manubrium. The irregular vertebral and superior margins of the scapula; are shown. On the left side the spicule of bone passes
from the thoracic and cervical arches to the scapula. (Natural size).
Fig. U. The superior view of the thoracic skeleton and the anterior surface of the cervical vertebral iilate and of the
occiput. In the cervical part, no vertebral bodies are clistiiut, but irregular radicular, and transverse processes project laterally from the central plate. The abnorin.-d s|iicule of bone on the left side inav be seen
passing from the fused transverse processes to the left scapula. This view .shows how the foveal surfaces
of the atlas are shifted to the right in relation to their underlying transverse processes. The right fovea
almost overhes the tip of the right transverse process, while the left fovea leaves the left transverse process
uncovered. The left transverse i)rocess is bent u)) and joins the pars lateralis, thus forming a rather large
foramen. The anterior surface of the occiput shows an asymmetrical oval outline pierced by a foramen, in
its center. The double exit of the right hyi)oglos.sal canal shows. The irregular superior margins of the
scapute are seen. The episternum and the aborted second rib are demonstrated. (Natural size).
 
Plate 3.
IG. 12. The left-hand view of the axial skeleton with subdural cast in place and median outline of specimen given
This figure shows the extreme dorsoflexion of the vertebral colunm. The occiput is in position and its
squamosal junction on the left with the second lumbar arch is shown. The origin' of the spicule of bone
which projects out from the transverse process in the thoracic region is visible. The crowding and irregular
arrangement of the fifth to the ninth ribs is shown, (xi)
Fig. 13. The superior view of the occipital bone shown with its enlarged foramen maginim. A normal-sized foramen
is designated by a dotted line. The left jugular process is prominent when compared with the right, which
seems to have been twisted over to the side. The anterior outlet to the right hypoglossal canal is shown with
the tiny rod of bone which divides the exit of the hypoglossal foramen on the right side immediately under it.
 
Fig, 14. The inferior surface of the occipital bone shows the large foramen. On the squamosal surface the exostoies
which join the lumbar vertebra show. On the partes laterales the condylar surfaces and on the left side the
cartilaginous process w^hich joins the transverse process of the atlas may be seen. The notched basal margin
of the basiocciput also is visible. (Natural size).
 
Fig. 15. This shows schematically the ventral surface of the thoracic vertebral i)late with i)airing of the origin of the
seventh and eighth and ninth and tenth ribs on the right side, and of the first and second, and the third and
fourth on the left.
 
Fig. 16. This shows schematically the sternum with six costal cartilage attachments on each side. The last attachment
on both sides is that of the seventh rib. The discrepancy occurs through the second rib becoming aborted on
the right side, and the si.xth being aborted on the left side. There are four centers of ossification on the midUne of the sternum. The upper two arc opposite the first costal cartilage and resemble an exclamation
mark. The lower two are oval (4 by 3 mm.), with long diameter perpendicular. One is about at the middle
point of the sternum and the other 1 cm. below it. .\n episternum surmounts the sternum and the xyphoid
process projects at its inferior end.
 
Plate 4.
 
Fig. 17. Dorsal superior view of a normal left scapula of a new-born.
 
Fig. 18. .Same view o! left scapula of specimen 862 o shows the irregular vertebral and superior margins with the
abnormal spicule of bone attached. It also shows the sheets of fascia attached to the vertebral and median
margins of the scapula and the insertions of the rhomboideus and levator scajjula? nmscles on this fascia.
(Natural size).
 
Fio. 19. Dorsal superior view of a normal right scapula of a new-born.
 
Fig. 20. .Same view of right scapula of this specimen, showing irregular vertebral margin. (Natural size).
 
Fig. 21. Dia,gram of left thoracic anil d.Tp dorsal musculature on (he left sidi> of the mounted axial .skeleton. The
occiput and model of cerebrfj-spinal cavity are in i)lace. The median outline of the .specimen is also given in
relation to these structures. Those nuiscles approximately normal arc either sectioned or oidy drawn at
their origin or insertion. They are the m. pectoralis major and minor, the rectus, the external oblique, the
latissimuB dorsi, the quadratus lumborum, and ( he levator scapulie. The abnormal muscles are .shown entire,
except for the serratus anterior, whose origin is indicated by broken lines. The largest mass of abnormal
muscles consists of a longitudinal bundle extending from the .sacrum to the occii)ut and labeled in. sacrospin.
From about the center of this bun<lle the serratus posterior inferior projects onto the lower three ribs. The
muscles at the upper end of the bundle are quite irregular. .Vlong the fourth and fifth ribs a mass of muscle
extends nearly to their costal cartilages. At the distal termination of these fibers lie .several small irregul;irly placed bundles. In the upper thoracic region is a narrow band of muscle overlving the others. (X J).
 
Fig. 22. Lateral view of abnormal right lung formed of but one lobe. (XJ).
 
Fio. 23. Lateral view of normal two-lobed left lung. (XJ).
 
Fig. 24. Diagram of those structures of the central nervous system which lie near the midline and which can be
identified. The outline of the subdural sji.ace used was obtained from the .sagittal section. Posteriorly this
passes near to the median margin of the left eix'ephaloccle. The cerebrum designated by a dotted lielrl is
shown protruding below the foramen magnum into the encephalocele. A small portion of the cerebellum,
represented by line-hatching, is seen to lie v<Ty nmch flattened on top of the cord. The brain-stem and
cord, much bent, arc shown in solid bhick. Those cranial nerves which were identified are shown by lines.
Only the first spinal nerve is shown. The floor of the fourth ventricle lies inverted on top of a flat cord.
 
 
 
na
 
 
 
 
^Aidl
 
 
 
J. F. OWusch (ei
 
 
 
 
J. F. Dl<*usch (eclt
 
 
 
4 Hoen A CoMfh.
 
 
 
F. Didusch fee;
 
 
 
 
\ HoftnA Co Ut
 
 
 
 
L.Lung.
 
 
 
J- F. DWusch (ecit
 
 
 
CONTRIBUTIONS TO EMBRYOLOGY, No. 23.
 
 
 
A HUMAN EMBRYO BEFORE THE APPEARANCE OF THE MYOTOMES.
 
 
 
By N. William Ingalls.
 
 
 
With four plates and five text-figures.
 
 
 
A HUMAN EMBRYO BEFORE THE APPEARANCE OF THE MYOTOMES.
 
 
 
By N. William Ingalls.
 
 
 
The specimen which forms the subject of this paper came into my possession
some time ago through the kindness of Dr. E. Peterka, of Cleveland. In the
collections of embryology and teratology of the Department of Anatomy of Western Reserve University it is listed as embryo No. 1. On account of the very
interesting and important stage of human development which it illustrates, a
detailed investigation of its more essential features, esiDecially as regards the
embryo proper, has been undertaken. The extra-embryonic structures, chorion,
body-stalk, and yolk-sac, and the evidence they offer on early blood and bloodvessel formation, will not be dealt with in detail at this time.
 
The intact ovum, when it came into my hands, had been for about a month in
alcohol of unknown strength, but was, on account of its small size, quite well preserved. The following brief history accompanied the specimen:
 
April 2. Intercourse (also about two weeks before?).
April 8. Period expected; regular 24 to 26 days.
April 14. Bleeding commenced, gradually increasing.
April 17. Ovum cast off.
 
Before entering upon a discussion of the anatomical findings, something may
be said as to the probable age of the specimen. Following the example of Bryce and
Teacher (1908), which has been adopted so frequently, one can set up a similar
table for the embryo in question :
 
 
 
Dimensions in millimeters.
 
 
Days elapsed from —
 
 
Age
 
in days.
 
 
Remarks.
 
 
Ovum
 
 
Embryo
(amniotic
cavity).
 
 
Last period.
 
 
Lapsed
period.
 
 
Beginning.
 
 
End.
 
 
Beginning.
 
 
External.
 
 
Internal.
 
 
9.1X8.2
X6-6.5
 
 
ca. 1 —
1.5 less
 
 
ca. 2.0
 
 
34
 
 
(?)
 
 
9
 
 
(17 to 18)
 
 
Abortion 15 (29) days
after intercourse.
 
 
 
The estimated age of 17 to 18 days was put in parenthesis in the above table
because we could not bring ourselves to look upon it with any very great degree of
confidence. The figures were obtained by comparison with embryos which were
obviously in a stage of development either more or less advanced and by reference
to the recent estimates of Triepel (1914) and Grosser (1914). Triepel's suggestion
of subtracting about 18 days from 34 in this case would give an age of about 16 days.
Embryo No. 1 is far in advance of both that described by Fetzer (1910) and the v.
Herfi" embryo of Graf Spee (1896), the ages of which have been given as 15 and 17 to
18 days respectively. On the other hand, it is distinctly less advanced than Frassi's

Latest revision as of 08:48, 16 February 2011


INTEGUMENT.

The dome of the head is narrowed and flattened, and is covered with lightbrown hair, 1 cm. in length. Just above and behind the ears the hair is 2 cm. long and is ({uite thick. Across the middle of the forehead at the hair margin is a narrow raised ridge of puckered skin, 2 cm. long. A section through this area shows the structures of the skin to be well develoi)ed and similar to the adjoining noi-mal skin, except that where the surface is raised the jiapilla' are somewhat flattened underneath. The ridge is probably the result of rough handling before fixation.

From the back of the head protrude three encephaloccles in a horizontal line, extending from a point 6 cm. behind the tip of the left ear to a point 2 cm. behind the tip of the right ear. These are best dis])laved in figures 1 and 3. They nu'iisure 13 cm. horizontally along their superior margin, and the vertical diameter is 4.3 cm. The midline of the specimen comes between the left and middle encephaloccles. In the midline their vertictU diameter extends from a point 3.6 cm. below the vertex to within 6 cm. (»f the amis. The middle swelling is the largest of the


ENCEPHALOCELES AND OTHER ABNORMALITIES. 93

three and is cone-shaped, whereas the other two are smaller and hemispherical. All are of soft consistency. The large coneUke swelling measures 6 cm. from the superior margin to its tip and 2.5 cm. from tip to inferior margin, its base being circular and measuring 4.3 cm. in diameter. This cone lies pendant over the back; the proximal half of its superior surface is covered with scalp and fine brown hair 2.5 cm. long. A strip of coarser hair of the same length follows the median margin of the sweUing to its lowest point. The covering of the distal half of the superior surface and the entire inferior surface resembles smooth, fine-grained leather. No hairs are present, but it is dotted with minute pores which on microscopical examination are seen to be the mouths of sweat-ducts. The wall of the sac is 3 mm. thick, a section through which shows an extremely thin layer of epidermis h'ing immediately over a vascular connective tissue, containing the sweat-glands mentioned above; but no hair follicles. There are two oval naevi near the tip of the sac, which in the gross resemble scars. These lie in the same long axis directed laterall}' through the tip of the sac. The smaller of the two is 5 mm. to the left of the tip and measures 7 by 3 mm. The larger lies 10 mm. to the right of the tip and measures 25 by 17 mm. The color of these areas is lighter than the surrounding tissue and the surfaces are stiff, smooth, and slightly raised. In their neighborhood the thickness of the sac wall is increased to 9 mm. Histologically the epitheUum is lacking here and a very vascular connective tissue forms the raised surface. The lining of the upper part of the sac is smooth fibrous tissue continuous with the dura of the main cerebro-spinal cavit5^ Near the tip, however, it is made up of shaggy strands of blood-vessels whose compUcated, interweaving pattern is like the early capillary plexus of the dura, as described by Streeter (1915). This suggests that the irregular vascularization in this region may be due to arrested development of the vascular system. There is marked engorgement of these vessels and congestion in all the tissues. Part of this extreme engorgement was probably caused by birth trauma.

The left encephalocele measures 5 by 4 cm. and protrudes 1 cm. from the surface. Its upper half is covered with fine hairs and the lower half with normalappearing skin. The wall is 1 to 2 cm. thick, composed chiefly of a layer of subcutaneous fat. On its left lower border there is a rounded bleb of porous, wrinkled skin 1 cm. in diameter, over which are scattered a few hairs 2 cm. long, and which contains around its depressed circumference a much thicker growth of similar hairs. A section through the wrinkled skin shows that it lies over a funnel-shaped canal, the wider mouth of which extends down through 1.5 cm. of subcutaneous fat to the subdural space, where the canal becomes narrow. This canal is filled with fluid and contains a few blood-vessels supported by loose connective-tissue septa. Its walls are formed of rather dense connective tissue.

The right encephalocele is 4 cm. in diameter at its base, and the surface, which is covered with, hairy scalp, is but slightly raised above the adjoining structures. Its wall, consisting of epidermis, connective tissue, and fat, is but 3 mm. thick.

A summing up of the integument findings shows that both normal skin and scalp are found over the areas adjoining the encephaloceles and over parts of the


94 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH

enccphaloccles as well. In addition to this, in the adjoining areas there are hypertrichosis and thickened subcutaneous fat, varying from 5 to 25 mm. in thickness. The wall of the large encephalocele varies from 3 to 9 mm. in thickne.ss and is formed by angiomatous tissue covered with a thin layer of epidermis penetrated by sweatglands. In two places near the tip of the sac naevi are formed by the vascular tissue extending to the surface. The walls of the small sacs vary from 3 to 20 mm. and are formed chiefly by subcutaneous fat covered with scalp. On the left a pore and canal pierce through to the subdural space.

Spietscka in 1894 collected the various forms of skin changes associated with spina bifida. Besides the varieties here found, he described pigment blotches and such a marked increase of fatty tissue as to amount to lipomata. In an article on skin anomalies by Bettmann, in Schwalbe's Morphologic der Missbildungen, naevi are noted as among the most frequent anomalies.

METHODS.

A sagittal section was made of the specimen under discussion (see fig. 9). The spinal column shoAvs extreme lordosis, undeveloped arches throughout, and .shortening and fusion of the upper vertebrae. The central nervous system is very much disturbed, a large part of it having slipped down below the cranium, through a much enlarged foramen magnum. This portion lies on the thoracic and lumbar vertebrae and protrudes into the sacs already described. The brain and cord were removed, and a clay impression was made of the entire space occupied by the central nervous system. This was then cast in wax and photographed, as shown in figures 4, 5, 6, and 7. By the help of this model the general shape taken ])y the central nervous system was demonstrated and the study of its internal arrangement and relation to other structures was facilitated. The consideration of these will be taken up later in this paper.

In the sagittal section, thick subcutaneous pads of fat are seen in the imdifferentiated region of the neck between chin and thorax, above the sj'mphysis, and over the sacral region. Dissection shows this sul)cutaneous fat to be likewise particularly abundant over the back and shoulders. There is also found an extreme grade of undeveloped or | split soft palate, associated with which is a bilateral anlage of the uvula, that on the left side being shown in figure 9a. Consideration of the normal development of the .soft palate will help to indicate how this defect originated. It is generally agreed that at a very early date the tongue occupies the area which is later occupied by the septum and palate. The normal rearrangement of these parts to their final po.sitions is acconi])li,'<hed by medial growth of the palate and downward growth of the sejjtum, associated with independent shifting of the tongue. If for any reason the tongue can not withdraw, the palate remains sjilit to a greater or less degree. That such a cause was operative in this specimen seems lik(!ly; the distorted position of the cervical spine might easily have caused a crowding in the adjoining pharyngeal region and so prevented the tongue from receding.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 95

SKELETON.

A dissection of the skeleton was made, the vertebrae and ribs being left connected bj' their ligaments, so that the specimen could be easily mounted. To facilitate handling, two transverse cuts in the skeleton were made at the level of the first thoracic and first lumbar vertebra?. A study of the skeleton shows marked maldevelopment and distortion, as may be seen in figures 10, 11, and 12. The axial skeleton is most affected, the arches of all the vertebrae being defective; these are open posteriorly in the midline and are flattened outward, forming wide anterior support for the central nervous system. In the cervical and thoracic regions the bodies of the vertebrae are fused, shortened, and dorsally flexed, so that the spine is bent almost double. The occiput actually rests on the gaping vertebral arches and fuses with them.

Viewing the occiput in figures 13 and 14, the inferior and medial two-thirds of the squama occipitahs is seen to be defective. A bilateral bony excrescence on its dorsal surface, near the defective medial margin of the squama and close to its junction with the partes laterales joins it to the everted arches of the second lumbar vertebra on the left side and to the first lumbar vertebra on the right. The defect of the squama in the midline, together with a widening of the angles formed by the junction of the pars basahs with the partes laterales, has greatly increased the size of the foramen magnum. This is oval in shape and measures 4.5 bj' 3.7 cm. The long diameter is antero-posterior, and posteriorly it slants slightly to the left. For purposes of comparison the size of a normal foramen is indicated in figure 13 by means of dotted lines. The large foramen resembles that of the chondrocraniumat a very early stage of development. The participation of both the squama occipitalis and the vertebral arches in the midline defect, as it exists here, has been regarded as teratological evidence of the homology of these parts, and probably has been a factor in advancing the opinion, which has slowlj' gained ground, that some cranial defects, even when existing alone, belong in the same category with certain vertebral abnormalities.

The two partes laterales are well formed and but slightly asymmetrical. The left jugular process is more marked than the right. On the left inferior surface directly under the jugular process there is a cartilaginous prominence which meets the tip of the transverse process of the underlying atlas. The hypoglossal foramen on the left side is a single canal, and while the right hypoglossal foramen has a single perforation on the medial surface of the pars lateralis, it has a double exit on the outer surface of the bone. A small rod of bone divides it into a smaller anterior and a larger posterior foramen, as is demonstrated in figure 11. A division similar to this has been observ-ed frequently in embryological studies and appears on the left side in a skull of a human fetus modeled by MackUn. The condition is of rather frequent occurrence. Lillie gives a ratio of 14 per cent complete division and 36 per cent indicated division, out of 305 left and right canals examined by him. The explanation generally offered is that it is persisting tissue from primitive cranial divisions which usually disappear at a very early stage.


96 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH

In our specimen the pars basalis of the occiput is oval and asymmetrical along its mferior margin, as shown in figure 11, and measures 18 by 14 mm. Its sphenoidal margin is 6 mm. thick, and the thickness of the bone elsewhere is 3 mm. Its I)()sterior surface is slightly concave, there being a rather deeper depression immediately under the sphenoidal articulation than elsewhere. The anterior surface of the pars basalis is nearly flat. The inferior margin has a notch near the midline and on either side of this the bone projects downward, 2 mm. on the right and 4 mm. on the left. A slit-like foramen 2.7 mm. wide directed forward and upward pierces the pars basalis near its center. On each temporal bone the eminentia arcuata is very prominent and the fossa subarcuata deeply depressed below. The ear ossicles are well developed and the other relations of the bone are normal. With the exception of the small size of the cranial vault the rest of the skull is well formed.

The bodies of all the cervical and thoracic vertebrae and the dorsal surface of the first lumbar vertebra are fused together in a bent and irregular central plate of cartilage containing irregular ossification centers. The roots of the arches and the ribs project from this plate. The relations of the various parts are shown in figures 10, 11, and 15. At the superior end of this plate the foveal surfaces of the atlas and its transverse processes are distinguishable, but, as may be seen in figure 10, both posterior and anterior arches are lost. The fovese are shifted to the right in relation to their transverse processes, as may be seen in figure 11. This shifting causes the right atlantal transverse process to lie immediately under the fovea. The left is uncovered by the fovea on that side, but is fused at its tip with the left pars lateralis.

Viewed from the side in figure 12, the cervical and lower thoracic jjortions of the central vertebral plate form the two arms of a wide-mouthed U, while the bent base of the U occurs in the plate from the level of the first to the sixth ribs. Besides this marked lordosis, there is a very slight lateral bend which shows in the dorsal view of the skeleton (fig. 10), giving the vertebral plate a slightly curved S-shaf)e. (This condition of scolisosis and lordosis in varying degrees is very frcHjuently noted in the extreme forms of si^ina bifida.) The concavity at the right margin of the vertebral plate is opjjosite the first rib and at the left opposite the sixth rib. From the central plate of this specimen throughout its extent the radices project outward on both sides and formed between them are two uneven rows of intervertebral foramina. The processes are tiny spicules of bone in the cervical and U[)per thoracic region, becoming larger in the lower ])art of the colunm. In the cervical region 7 radices are distitict and 12 in the thoracic region. The former could not all be shown in the drawings.

The arches of both cervical and thoracic regions are everted and fused. This formation, together with the antero-posterior bend of the plate, makes a rather deep pocket of bone which contains parts of the much disturbed central nervous system. The lumbar and sacral vertebral colunm is much less afTected than the upper part. The dorsal part of the first lumbar vertebral body is fused with the thoracic vertebra?, its ventral surface, however, being distinct. The four lower


ENCEPHALOCELES AND OTHER ABNORMALITIES.


97


lumbar and the five sacral vertebral bodies are well formed, as are the transverse processes of all the lumbar vertebra? and the partes laterales of the sacrum (figs. 10 and 12). The first four lumbar arches are everted, as are the thoracic arches, though individually they are distinct and not fused. The fifth lumbar and the five sacral arches are incomplete, but project medially toward one another and arc not everted. The lumbar column is 4.3 cm. long and the sacrum 3 cm. in length. The coccyx is composed of four segments, which measure 1.6 cm. and are bent to the left. In studying the proportions of the vertebral column, Aeby 's tables of relations in normal vertebral columns in the new-born were used, with the results shown in table 1.

Tabi,e 1. — Comparison in miUimelcrs of the vertebral lengllis of specimen with those given by Aeby for normal neio-bom.



Total.


Cervical.


Thoracic.


Lumbar.


Aeby (normal) .... No. 862a


176.5 105.0


45.1 15.0


83.9 45.0


47.5 43.0


A comparison of these vertebral lengths shows the lumbar portion of this specimen to be within the limits of normal, though near the minimal margin. The cervical portion is less than half, and the thoracic portion a trifle more than half the length of the normal. Aebj' gives 26.4 mm. for the transverse diameter of the atlas, 12.2 mm. for the width of the body of the sixth thoracic vertebra, and 17.5 mm. for that of the fifth lumbar. In this specimen the lateral limits of the foveae are 31.0 mm. and the width of the transverse processes of the atlas 38.0 mm. The width of the vertebral plate in the midthoracic region is 23 mm. and the width of the fifth lumbar vertebra is 21 mm. These differences show an irregular widening process to have taken place in the vertebral bodies themselves, the change being most marked in the thoracic and cervical regions. The absence of lateral pressure from ununited arches must have been an important factor in this broadening process.

There are twelve ribs on each side which ha^•e undergone considerable disturbance. On the right, the first sL\ are fused near their bases (figs. 10, 11, and 15). The second rib terminates at the end of its proximal third in a plate of bone by which it is joined to the first and third ribs. On the left, the fifth to nmth ribs are crowded together in their proximal half (figs. 10, 12, and 15). The fifth and sixth have but one costal cartilage between them. The sLxth and seventh ribs are fused for a few milUmeters just proximal to their termination. Further fusion occurs in pairs at the bases of the follo\\ing ribs: on the right, between seventh and eighth, ninth and tenth; on the left, between the first and second, third and fourth. This shows on the ventral surface in figure 15.

The sternum, as seen in figure 16, has four ossification centers near the median line at the level of the first costal cartilage and of the first, second, and third left intercostal spaces. There are six costal cartilage connections on each side. The last on each side, however, belongs to the seventh rib. The discrepancy occurs on the right side through the aborted second rib and on the left side through the


98


STUDY OF A HUMAN SPINA BIGIDA MONSTEK WITH


fifth and sixth, having but one cartilage between them. The first and seventh costal cartilages of the two sides are opposite each other. The arrangement of the other cartilages is such that the third to the fifth on the left side are from 0.5 to 1 cm. lower than the corresponding cartilages on the right, yet not quite opposite the succeeding one. A small cartilaginous knob (2 by 5 by 3 mm.) above the manubrium is a persistent episternum. The measurements of the sternum are given in table 2.

Table 2. — Dimensions in centimeters.


Body lengths: cm.

Vertex-anus 14 .0

umbilicus 32 .0

Lower hair border-anus (length of back) 10.0

Head:

Circumference of head 32 .5

Biparietal diameter 8.5

Anterior fontanel 2.7 cm. transverse by 2 .5

Posterior fontanel 1.6 cm. " by 1 .7

Face, vertical length (border of hair to chin). . 9 .5

Clear breadth (from free edges of tragi) 9.2

Eyes apart 1-8

Nose across 2.3

Mouth across 2.0

Trunk:

Circumference at umbilicus, passing around the

back at base of middle sac 33 .0

Distance across shoulders 12.0

Nipples apart 6.4


Trunk — Continued. cm.

Diameter of right nipple .9

left nipple 7

Sternum, episternum, and zyphoid:

Length of sternum with episternum and 6.9

zyphoid sternum alone 6.0

Width of sternvmi 1.0

Thickness of sternum 3

Length of episternum 3

Length of zyphoid 9.6

E.xtremities:

Upper arm (circumference of both left and

right) 9.0

Lower arm (circumference of both left and

right) 7.0

Hand with middle finger 6.0

Right trochanter— heel 20 .9

Right foot 7.5


The two scapulae which are shown in figures 18 and 20 are distorted, as will be seen by comparison with figures 17 and 19, representing normal left and right scapulae. In both the pathological^ bones the supraspinous portions are poorly formed and the inferior vertebral margins are concave. Graves designates a concavity of the vetebral margin of the scapula as scapula scaphoidea. He notes that it is of fairly frequent occurrence and claims that it is associated with syphilis in the i)arents. He gives as his figures, however, no definite rate of occurrence. Here it m:iy l)e mentioned that the Levaditi stain done on the tissues of this specimen showed no spirocha^tes. On the right scapula the vertebral margin i^asses as a straight line from the medial termination of the spinous process to the incisura next the glenoid process. The vertical diameter of the right scapula measures 36 mm. from the tip of the cartilaginous process at the inferior angle to the superior margin near the incisura. Its transverse diameter along the base of the spinous process, near the termination of the latter, to the center of the glenoid fossa is 26 mm. The subscapular angle is 128°, the infraspinous angle is 122°, and the supraspinous angle is 110°. On the left scapula, the vertebral margin above the spinous process projects at a fairly sharp angle near its middle. The vertical diameter taken from the tip of the inferior angle to the end of the projecting point of the supraspinata is 29 mm. The horizontal diameter of the left scai)ula, measured similarly as the right, is 36.5 mm. The vertebral margin of the left scapula at the termination of the sjiinous i)roccss is elongated by a bony and cartilaginous knob, which is attached to a curved rod of bone 10 mm.


ENCEPHALOCELES AND OTHER ABNORMALITIES.


99


long and 2 mm. in diameter. This rod is joined at its other end to the everted arches of the vertebrae underlying it. On the left scapula the subscapular angle is 117°, the supraspinous angle is 109.5° and the infraspinous angle is 133.5°. The left scapula shows a rather interesting condition, presenting three out of four features often associated with Sprengel's deformity (congenital elevation of the shoulders). These are, according to Horwitz: (1) changed relations of the diameters to each other; (2) bending forward of the supraspinous process; (3) prolongation or rounding of superior median angle; (4) presence of exostoses and articulations with the vertebral column.

In this case the exception to the above conditions is the superior median angle, which can hardly be called prolonged. Scajnilar measurements of the new-born could not be foimd in the literature, but two supposedly normal sets were obtained from mounted skeletons belonging to the Obstetrical Department of the Johns Hopkins Hospital, and the measurements of several other scapulse were available through the courtesy of Dr. A. H. Schultz.

Table 3. — Comparison in miUiiiwtcrs of the scapular measurements of specimen with those of several normal new-born.


Specimen.


Schultz, No. 3 . Schultz, No. 4 .


Obstetrical Department skeleton.

Do

No. 862 a


Vertebral length.


165 151

169

172


Transverse diameter.


R. 24 / L. 24 \ R. 29 ( L. 30 \ R. 27 / L. 27 I R. 26

L. 31


Vertical diameter.


R. 33 L. 31 R. 38 L. 38 R. 36 L. 35 R. 36 L. 29


Morphological index.


72.7 77.4 76.3 78.7 75.0 77.1 72.2 106.8


Table 3 shows that the ratio of the diameters of the right scapula of 862 o is near those of the supposedly normal bones. The left scapula, on the other hand, has the relations of its diameters reversed. The transverse diameter exceeds the vertical. Thus its morphological index is 106.8, while none of the norrnal indices exceeds 80. The subscapular angle on the left side is somewhat smaller than on the right. The bony articulation joining the left scapula to the vertebral column is attached in the upper third of the vertebral scapular margin as in most of the Sprengel deformity cases. Some interest is attached to this abnormal bony spicule and various suggestions have been made concerning it. The opinion seems to be generally accepted that it arises from its scapular end. Cases are recorded in which other anomalous bones are joined only to the scapula, and their occurrence substantiates this view. Case xvi in von Recklinhausen's paper is a monster very like No. 862a. In it there is "ein 1 cm. langer knocherner, rippenartiger, am oberen Winkel des knorpeligen Schulterblatts articulierender Korper." Gruber gives a case found in an adult male cadaver of a "fortsatzartigen, cylindrischen Hocker an der Vorderflache des Angulus superior der Scapula." No satisfactory hypothesis has been advanced to further explain the origin of these bones. The length of the right clavicle is 39 mm. while the left clavicle measures 34 mm. and is shghtly


100 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH

more bent at its distal end than the right. This shortening of the clavicle on the side of the abnormal scapula is frccjuent in Sprengel's deformity. The hunched position of the shoulders, so ]irominent externally in this case, may be seen to be due to the defective cervical and upper thoracic vertebrae, which lie crumpled to half their normal length under the scajjulae, their normal relations to these bones being quite changed.

MUSCLES.

The region of abnormal musculature corresponds, as would be supposed, to the skeleton derangements. This is limited to the neighborhood of the axial skeleton, where the affected muscles are both under and intermingled with an unusually large amount of fascia. On superficial dissection, the topmost layer of muscles is well formed, except for the trapezius, which is represented similarlj' on the two sides by thin strap-like bands of muscle, 3 by 1 cm. The fibers run parallel with the long diameter, from the origin of the muscle, situated in fascia lying over the everted and crumpled cervical and thoracic vertebral arches, to their insertion on the acromial extremity of the clavicle, the acromion, and spine of the scapula. Those fibers which insert on the scapular spine have become folded under the others, owing to the contracted and lowered origin of all the fibers. A condition of the trapezius similar to this has been noted in a case of total rachischisis given by Kermauner, in which case, also, lordosis and marked shortening of the spine were the underlying skeletal conditions. As Kermauner says, the association of this variety of muscle and bone defect is only natural, "for, with the marked shortening of the trunk, there necessarily exists a reduction in the cranio-caudal diameter of the muscles of this region."

Upon further dissection, the condition of the underlying muscles was determined. The rhomboidei are represented bilaterally by very thin and short muscles, onlj'^ 3 mm. in length. They arise from the connective tissue over the fused and everted arches of the thoracic vertebra, and are inserted in fascia along the inferior vertebral borders of the scapula?. (In Le Double's work a reduction in the thickness of these muscles is recorded.) The two levator scapulae are present. They arise from the fused transverse processes of the upper cervical vertebrae and are inserted in fascia along the superior vertebral margins of the scapulae. There is no reduction in the size of either muscle. They are directed horizontally out instead of slanting downwards as usual. This is due to the scapuhe lying directly over the cervical vertebr:e. A cross-section of the left muscle at its origin is shown in figure 21. Figure 18 shows the left scapula and the rhomboideus and levator scapulae muscles inserted in fascia which forms a sheet between the irregular projections of the vertebral margins of the bone. The abnormal spicule of bone is attached to the scapula at the mcflian angle between the insertions of the levator scapulae and the rhomboidcnis.

On each side most of the dorsal inusck's consist of ;ui irregular longitudinal bundle which extends along the sides of the vertebrae from sacrum to occiput and which sends scattered projections on to the ribs. Under this bundle in the lumbar region the quadratus lumborum and psoas muscles lie undisturbed. On the left


ENCEPHALOCELES AND OTHER ABNORMALITIES. 101

side this bundle is shown somewhat diagrammatically in figure 21 and labeled sacrospinalis. In the lumbar region it is cjdindrical and measures 1.5 cm. in diameter. It grows flatter and broader as it nears the upper part of the spine, this formation being due to a state of arrested development of the sacrospinalis and short back-muscles. The early condition of dorsal musculature which it simulates is strikingly illustrated in Bardeen and Lewis's model of an 11 mm. embryo (1901), where a bundle distinct from the ventral-lateral muscles lies bilaterally in the trough formed at the sides of the vertebrae. In another model given in the same paper of a 20 mm. embryo, the bundle may still be seen lying under the connective tissue of the region, and this divided condition of the back-muscles persists normally until about the 60 mm. stage.

The serati posterior inferior are shown by projections from the dorsal bundle which on both sides cover the proximal half of the three lowest ribs. In the upper thoracic region, lying on the surface of the bundle on each side, is a thin strip of muscle near the base of the ribs. These strips extend cranio-caudally and measure 20 by 3 mm. On the left the strip lies over the third to eighth rib ; on the right side it extends over the first to the sixth rib. The serati posterior superior are not identifiable.

The direction of the muscles of the anterior cervical region, as well as of those attached to the skull, is distorted vvith the underlying skeleton, but the muscles are well developed and not defective. Both sterno-cleido-mastoid muscles have normal origins and insertions. The two splenii arise bilaterally from fascia under the scapulae and are inserted normally on the mastoid process under the sterno-cleido-mastoid, and posterior to this on the occipital bone. The longissimi can be traced arising from the fascia over the cervical vertebral region and inserted on the mastoid proces.ses. The semi-spinalis capitis muscles, arising from the upper ribs near their origin, are inserted on the occipital bone and are next to the deepest layer of musculature. The latter on each side consists of short fibers, rudiments of the short neck-muscles, the recti, and obliqui. More anteriorly most of the neck muscles are recognizable. The digaster, stylohyoid, omohyoid, and sternohyoid muscles are well developed. The longiis capitis and colli are represented by a few strands along the anterior surfaces of the vertebral plate. The scaleni medii and posteriores are present as flattened bands of muscle arising in this region and inserted in the first and second ribs near their bases. The scaleni anteriores are symmetrical. They arise from the lateral processes of the superior cervical vertebrae and insert on the first rib near its center. The nerve trunks of the cervical and brachial plexus pass under these muscles and are tightly bound down bj^ them.

Of the more anterior thoracic muscles, the pectorales are not disturbed. Both serati anterior muscles are defective and difTer in their defects. On the right side there is more complete development. Here slips of the muscle arising from the distal portions of the first four ribs and from the eighth to the tenth ribs converge and are inserted around the inferior angle of the scapula. A few strands of muscle on the chest wall between the pectoraUs minor and the seratus are present, which might be remnants of th(! latter. They are shaped like half a crescent, with fibers running longitudinally, and extend from the first rib, where they are 3 mm. broad, to the fifth rib, where they are 15 mm.


102 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH

On the loft side the seratus anterior is very imperfect. It is represented l)y a thin sheet of fascia, which originates from the first three ribs and is inserted in the scapula along the vertebral margin near the medial angle. A few scattered musclefibers, which also probably represent remnants of the serati, arise over the fourth rib near its base and are inserted into the inferior angle of the scapula. The origin of the fascia and these muscle-fibers is shown in figure 21 by dotted lines. Some other muscles on the left chest wall, consisting of irregular projections from the dorsal bundle which covers the proximal part of the first seven ribs, may be serati fibers which remained in their embryonic position close to the axis. Fibers which jjrobably represent intercostal muscles pushed to the outer surface of the ribs are arranged along the lower border of the fourth rib. These extend onto the lower adjoining ribs. At the outer end they are 2 mm. across and near the base of the ribs they measure 20 mm. (See fig. 21.) Three small muscle bundles are situated at the distal end of the above-mentioned fibers.

The lateral and anterior abdominal muscles are well developed. Each rectus is 7.4 cm. by 3.2 cm. The right rectus has two inscriptiones tendinae in its upper one-third opposite the sixth and seventh ribs.

To summarize : Those muscles which have undergone most disturliance are the trapezei, the rhomboidei, the serati posteriores superiores, the serati anteriores, and the sacrosi)inalis and short back muscles. The location of these muscular abnormalities, situated near the chief skeletal abnormalities, demonstrates still further that the ijathological process is a rather sharply circumscribed one, limited to the neighborhood of the axis. The inclusion of the anterior serati in this group does not contradict the statement, as the early anlage of the serati is very near the axis.

The muscle disturbances of "monsters" have been but little investigated or recorded. From the scattered observations at present obtainable, any correlation is impossible.

VISCERA.

On dissection, the viscera are found crowded and somewhat distorted, but, with the excejition of the right lung, are well developed. The thyroid is bilobed and measures 1.5 by 1 cm., the thymus measures 6 by 2.8 by 1.1 cm. The esophagus measures 4 cm. from epiglottis to cardia. The lesser curvature of the stomach is 1 cm. and its greater curvature 8 cm. The intestines are well formed. The api)endix measures 8.5 cm. The cok)n is much bent upon itself. Because of unskillful handling the positions asssunied l)y the rest of the intestinal tract were not ascertained. The pericardium, ])leura, and diapluagm are intact. The heart is well developed. Sagittal section sliows it cut through the right ventricle and left auricle. The valves are well formed. The ductus arteriosus is patent. The left lung is approximately normal; its lateral surface is shown in figure 23; it consists of two lobes and measures 3.9 cm. antero-posteriorly by 3.1 cm. perpendicularly by 1.7 cm. in its thickest niedio-lateral diameter near the hilum. The right lung, side view of which is shown in figure 22, about ('(juals the left in volunu^ and is roughly a flattened cone-shape with apex directed anteriorly. Its corresi)onding measurements are 5.1 by 3.2 by 2.9 cm. it is formed of only one lobe. Along tlie margins four short fissures exist, directed toward tlu' center; one 12 mm. in length


ENCEPHALOCELES AND OTHER ABNORMALITIES. 103

is situated on the posterior margin at the junction of the superior third with the middle third; on the inferior margin near its middle a similar fissure is situated, and halfway between it and the anterior end of the lung a shorter fissure 3.5 mm. long exists; on the superior margin another, 3.5 mm. in length, is present slightly anterior to the middle. These fissures are very superficial and extend for only a few millimeters on the medial surface of the lung. The relations of the bronchial tree were not determined.

The liver is flattened out horizontally and shaped like an L with the angle projecting anteriorly, the gall-bladder, which is 4.1 cm. in length, being situated on the inferior surface of the long arm of the L. The closed end of the gallbladder lies near the tip of the angle and its long axis is directed diagonally toward the upper end of the latter. The spleen is 2.2 by 1.3 by 1 cm. The presence of the pancreas is determined histologically. It lies embedded in ti.ssue near the vertebral column. Both kidneys and adrenals are somewhat compressed and distorted, the left much more than the right. The right kidney is somewhat flattened from side to side and at its upper end, and measures 4.5 by 1.2 by 3.5 cm. The right adrenal lies above it and measures 3 by 2 bj^ 0.5 cm. The left kidney is bent upon itself and folded in with its closely adherent adrenal, so that together they form a rounded mass measuring 4.7 by 3.4 by 2.4 cm. The greater distortion of the left kidney and adrenal is very probably due to crowding, a result of the left-sided concave bending of the vertebral column in this region and fusion of the lower ribs on that side. The ureters and bladder are well formed. The uterus, tubes, and ovaries are well developed. Blocks of tissue of heart, kidney, liver, and adrenal were run through by the Levaditi method for spirochaetes by Dr. Bullard, with negative findings. It is to be noted that the tissues had been kept in carbolic, which is not the fixation recommended for this method.

The developmental anomalies of the soft palate and the right lung are the most marked changes which have taken place in the soft tissues anterior to the vertebral column. They are both examples of arrested development and are secondary to, and probably the mechanical result of, 'the deformit.y of the vertebrae.

CRANIAL CAVITY AND CENTRAL NERVOUS SYSTEM. The shape assumed by the cerebro-spinal cavity or subdural space is shown by figures 4, 5, 6, and 7 of the wax model. In figures 12 and 21 the model is given in its relation to the skeleton. The space consists of a shallow dome which contained the frontal and part of the parietal cerebral lobes. Below this dome a relatively slight constriction in the model denotes the enlarged foramen magnum (figs. 4, 5, 7, and 12). Under the foramen three rounded encephaloceles project posteriorly, and below these the pointed termination of the spinal canal may be seen. Situated ventral to the encephaloceles and continuous with them and with the base of the dome and the spinal canal is a blunt, wedge-shaped mass marked iv in figures 4, 5, and 6. This portion fits into the pocket of bone formed by the thoracic and vertebral plate. With the exception of the inside of the large encephalocele, the space occupied by the central nervous system is lined with a continuous sheet of smooth dura. At the foramen magnum and in between the eminentiae arcuatae of the tem


104 STUDY OP A HUMAN SPINA BIFIDA MONSTER WITH

ponil bones and the sella turcica it is drawn into numerous folds. On the inside of tlio large encephalocele the smooth dural surface changes to a tissue composed of many blood-vessels, fibrin, and extravasated blood, as described in the beginning of the paper. The fabc cerebri lies well over on the left side in its anterior and middle portions; posteriori}' it ends in a single fold about the center of the superior margin of the occipital bone. The tentorium cerebelli is absent. The ventral surface of the subdural space is pierced by two rows of cranial and spinal nerves. These number 43 in all, 12 cranial and 31 spinal, the latter distributed as follows: 8 cervical, 12 thoracic, 5 lumbar, 5 sacral, and 1 coccygeal. Both the anterior roots and the posterior roots with their ganglia are identified. In the cervical and thoracic regions they are extremely crowded.

The arrangement of the central nervous system is very complicated. The cerel)rum lies part above and part below the foramen magnum. The cerebellum lies entirely below it. The medulla and cord lie bent and crumpled ventral to the other structures below the foramen. The surface of that part of the cerebrum which lies above the foramen shows definite fissure and sulcus formation. Below the foramen a large part of the contents of the encephaloceles consists of a hemorrhagic mass and much of the surface of this is covered with caked blood, so that only in a few [)laces can typical cerebral surface markings be identified, flections of these regions, however, show definite though rather undifferentiated cortical lamination.

From a brief studj' of sections of other parts of the left cerebrum, made by Dr. Charles Bagley, the following points are determined : The cortical structure for the most part is composed of a very wide granular zone, which is characteristic of the early and undifferentiated stage of lamination. There is, however, a prominent vertical fissure lying at tlie junction of the middle and posterior two-thirds of the cranial dome, which can be identified as the central fissure of Rolando; the ventral termination of this fissure rests on the eminentia arcuata of the temporal bone. The cortical tissue anterior to this fissure shows a definite lamination. There is a welldefuied first layer; a broad and poorl}' differentiated second layer; the third laj^er is of medium width and is filled with small pyramidal cells; the fourth layer is inconsi)icuous, suggesting the agranular naotor tyi)e; while the fifth layer is represented l\y large pyramidal cells, ])r()l)ably Betz cells. These cells are at least three times as large as an}' other cells which could l)e found in the cortex and it is owing to their presence and to the very narrow gramilar layer that this area is identified as motor cortex. Immediately posterior to this fissure there is a sharp change in lamination types. Thc^ large cells are lost and the fifth layer is represented by definitely sha])ed pyramidal cells of not more than one-third the size of those cells de.signated as Jietz cells. Above this layer there is present a very heavy granular layer which is (|uite a contrast to the narrow granular layer of the area just described. It may be said, therefore, with a fair degre(> of certainty, that the tissue posterior to the fissure represents sensory cortex.

From the rather limited amount of material studied the only other localization that could be determined is that the cortical tissue i)ushetl down on the side of the wedge-shaped mass (11' in the model) represents subiculum. Here, passing from a cortex of (|iiilc deep cxlcnl, it becomes suddenly shallow and consists of atypical


ENCEPHALOCELES AND OTHER ABNORMALITIES. 105

first-zone layer containing few cells and a well-marked second layer with only a narrow zone of undifferentiated cortex beneath it. No calcarine type of cortex was identified, but the occipital lobe was probably included in the hemorrhagic mass in the encephaloceles, which was in such a bad state of preservation that no sections could be made.

On the right side of the cerebrum the fissure corresponding to the left central fissure of Rolando is situated slightly more anteriorly than on the left. The frontal lobes thus occupy most of the shallow vault and rest in the anterior and in the medial cranial fossae as well, which latter normally hold the temporal lobes. The sulci of the frontal lobes are changed considerably from their normal positions. The anterior ends of the superior medial and inferior frontal sulci are bent very sharply downward. They are all situated nearer the base of the brain than usual, as if the tissue had been pulled down on the lateral surfaces. Thus the superior sulci lie halfway down the sides, with the medial and inferior sulci correspondingly below them. With the exception of part of the parietal lobes on each side, the rest of the cerebrum lies below the foramen magnum. The left-sided position of the falx cerebri allows more room in the cranial cavity proper for the right cerebral hemisphere than for the left. This results in a larger portion of the parietal lobe on the right side lying above the foramen magnum than on the left side, and correspondingly a larger amount of parietal surface lying below the foramen on the left than on the right side.

The cerebral tissue which lies below the foramen is partly contained in the large bony pocket formed bj' the upper vertebral plate, and partly in the middle and left encephalocele. Sulci are present over its surface, but are so distorted that they can not be identified, nor can the identity of the lobes be determined. In the contents of the middle and left sacs one can easily discern cerebral gyri, and the general histological structure of these is similar to that of the cerebral tissue situated above the foramen. In the middle sac there is a large amount of clot. The cerebral tissue which lies in the cervical and thoracic vertebral pocket is pressed out into a thin shell, and hes next the dura, being limited anteriorly by the emerging cranial nerves. On the sides and back it is continuous with the cerebral tissue lying in the cranial vault and with that pressed out into the encephaloceles.

In the interior of the brain the optic thalami may be identified, h'ing above the foramen. A small space representing the third ventricle, greatly compressed, lies between the thalami. Choroid plexus tissue is present. Its relations, however, to the adjoining structures could not be determined. The cerebral peduncles may be seen as flattened bundles lying central to the shell of the cerebral cortex. The optic nerves are present. The hypophysis lies embedded in the well-formed sella turcica. No other structures in this region or below can be identified until, in the pocket of bone formed by the thoracic vertebrae, the inverted floor of the fourth ventricle is recognized.

The midbrain wiih attached fourth nerves, the colliculi, and the aqueduct of Sylvius were not identified. The fourth nerves, however, were found at their dural exit. Judging from the position of the fourth ventricle floor, a sharp bend


106 STUDY OF A HUMAN SPINA BIFIDA MONSTER WITH

with the angle directed posteriorly must have occurred in the midbrain region. At the beginning of the spinal cord a bend in the opposite direction is present. This bending of the brain stem and cord must have been in lateral outline shaped like a crudely drawn letter Z, as shown in figure 24, which is a diagram of various structures in the central nervous system near the midline. The surface of the inverted fourth ventricle floor is shaped like an isosceles triangle with its tip, which is its normal anterior end, directed backward toward the encephaloceles. The median sulcus is well defined. The tissue next to the median sulcus on both sides is slightly raised. The rest of the surface is flat. For estimating roughly the amount of distortion this fourth ventricle floor had undergone, a comparison of it with the fourth ventricle floor of three normal full-term fetuses was made. Each of the three showed a similar longitudinal ridge to be the extent of their surface markings. The main difference which this specimen showed seemed to be in a blunting of the posterior end which forms the base of the triangle already referred to.

From both lateral margins of the fourth ventricle floor cortical tissue resembling the flocculus is drawn backward, downward, and to the right, joining the cerebellar cortex contained in the right sac. It must be noted that while the flocculus is directed toward the posterior end of the specimen as a whole, it is drawn toward the end of the ventricle floor normally anterior. There is a much disturbed choroid plexus folded in with the cerebellar tissue. A fairly large amount of cerebellar tissue is present; part of this is drawn out into a sheet which is continuous with the flocculus and which passes posteriorly and to the right into the right encephalocele, where it lies next to a rounded mass of cerebellar cortex. Bands of tissue connecting the cerebellum and cerebrum i^robably represent the brachium conjunctivum. There are smaller flat bands of tissue passing backward near the flocculus to the rounded cerebellar cortex .which may have been remnants of the inferior cerebellar peduncles. There is no pontine enlargement.

From the tissue superior to and continuous with the floor of the fourth ventricle, the third and the fifth to the twelfth paired cranial nerves pass forward to . their normal exits from the subdural space. They are elongated to between 20 and 30 mm. Their origin from the brain-stem lies ()i)i)osite the first thoracic vertebra. In this region the beginning of the flattened cord can be made out, which is bent double upon itself. Some interest is attached to this Z bend of the brain-stem and cord. It seems to have been brought about in this case through traction on these parts by the major portions of the central nervous system slipping through the enlarged foramen magnum. \'arying degrees of such kinking have been described. The condition in its milder forms has received the name of Chiari deformity, from a case described by Chiari in which the medulla is l)ent l)ack over the cord for only a short extent. In Nageli's case of cyclopia there is a marked degree of such bending associated with splitting of the cord.

Caudal to the bend as a flat band the .spinal cord extends to the level of the lumbar vertebra, where it terminates in a cauda etiuina. From its ventral surface the spinal nerves extend into the dura. At the level of the twelfth thoracic verte])ra the spur in the vertebral plate has left an indentation on the flat cord and on the right anterior third of the inverted floor of the fourth ventricle.


ENCEPHALOCELES AND OTHER ABNORMALITIES. 107

The central canal, as such, is absent. Throughout the extent of the cord it is changed to a flat space following the contour of the vertebral column, whose floor is the cord and whose roof is partly the same cord inverted, partly the inverted floor of the fourth ventricle, and partly cerebellar tissue.

This fragmentary description of the central nervous system leaves much to be desired. It would have been especially desirable had we been able to present a clear picture of the relationships of the meninges. The main conclusion which can be drawn from its study is that the chief disturbance here evidenced is primarily one of distortion, rather than of absence or real lack of development of nerve-tissue.

CONCLUSION

The exterior alone of such a specimen as this certainly presents striking evidence that an organism can undergo most serious disturbances and yet maintain a definite though limited growth balance ; but in order to ascertain in detail exactly what constitutes the limitations of this equilibrium more intensive study is necessary. A rather intereisting series of anomalies is the result of such a study in this ca.se. It may be noted that these anomahes are centered about the axis. The bony parts, the central nervous S3^^tem, certain adjacent muscles, and overlying areas of integument share profoundly in this disturbance. Subsidiary disturbances of development are evidenced in a split soft palate and a one-lobed right lung. These facts, in addition to supplying a clearer knowledge regarding the individual specimen, contribute their small share in providing data for the better understanding of certain general problems of development. Classifications and analyses included in such subjects as osteolog}^, myology, and organology can not be regarded as complete until they contain a comprehensive picture of teratological phenomena. This is almost entirely lacking at present. The teratological material has been so scanty that any satisfactory correlation of it has been impossible.

Up to f airh" recent times teratology was considered an isolated science ; it was thought that the laws applying to most jiatural phenomena were not applicable to its conditions, that it could not learn from or contribute to the normal sciences. Studies of the past half century have entirely reversed this view. Teratology today has for its basis the same fundamental sciences of chemistry, biology, and physics as has those sciences whose subject-matter deals with normal phenomena. It is constantly learning from these latter sciences, and in turn has been able to contribute suggestions on points of analysis or exposition regarding puzzling phases of normal development.

The necessity of furthering our knowledge regarding the etiological factors of specific abnormal conditions has been^considered. Material at such an advanced stage of development as this specimen can contribute but Uttle along this line. We can not determine by means of it the primary defect, nor again, except in a very general way, a chronological picture of the early processes. We must turn to embryological material and to other than morphological methods to obtain such knowledge.


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108


EXPLANATION OF PLATES. ABBREVIATIONS.


anth.,


anthelix.


m. obi. ext..


antitr.,


antitragus.


m. pect. maj..


b. occ,


basioccipitales.


m. pect. min.,


c. i.,


first coccygeal segment.


m. quad, lumb..


c. c. 7,


costal cartilage of seventh rib.


m. rect..


c. eq.,


Cauda equina.


m. rhomb.,

cer.,


cerebrum.


m. sacraspin.


cereb.,


cerebellum.


m. ser. ant..


cr. 2,


second cranial nerve.


m. ser. post, inf


cr. 5,


fifth cranial nerve.


naev.,


dep. em.,


depression made by eminenlia arcuata


r. 1 (rib).



of temporal bone.


r. hyp.,


epist.,


episternum.


rt. en..


eust. t.,


eu.stachian-tube orifice.


s. 1,


exos.,


exostosis.


sp. 1,


fak.,


falx cerebri.


sq. occ.


fl. IV,


floor of fourth ventricle.


St.,


for. mag.,


foramen magnum.


tr.,


1. 1,


first lumbar segment.


uv.,


1.2,


second lumbar segment


V'l


1. 1 rib,


left first rib.


V. pi..


1. en.,


left encephalocele.


w..


1. pars lat..


left pars laterahs.



mid. en..


middle encephalocele.


X.,


m. lat. dors..


m. latissimus dorsi (origin).


xyph..


m. lev. scap.,


m. levator scapulae (cross-section, fig.


iii.,



17, insertion fig. 14).



m.obliquuscxternus abdominis (origin), m. pectoralis major (origin), m. pectoralis minor (origin), m. quadratus lumborum. m. rectus abdominis (origin), m. rhomboideus (insertion), m. .sacrospinaUs (insertion), m. seratus anterior (insertion). , m. seratus posterior inferior, naevus.

right first rib. right hypoglossal canal, right encephalocele. first sacral segment, first spinal nerve, squama occipitaUs. sternum, tragus, uvula, vertex.

vertebral plate, central nervous system occupying bony

vertebral pocket, anomalous bone and its insertion, xyphoid. third ventricle, absence of soft palate.