fracture with depression. After twenty-four hours' unconsciousness he
slowly recovered. At the site of injury tliere gradually formed a
tumor wliich tinnlly attained the size of a hen's egg and was bluish col-
ored. It increased in size when he lay do«n, diminished when he held
the head erect; respiration had no influence upon it. The overlying
skin was abnormally thin ; there was evident de])rcssion of bone. The
tumor fluctuated, and could be caused to almost (lisaj)pear on pressure,
which, iiowever, caused liim ]iain and vertigo. The tumor did not pul-
sate nor were any nuirnuirs heard in it. He died of erysipelas. Upon
autopsy there was found just outside the bone a blood-sac covered with
periosteum and skin. In the underlying depressed bone were numerous
small ojienings whicli permitted the passage of veins into the supci'ior
longitudinal sinus. The inner side of the bone was normal.' Numerous
similar cases iiave been reported.

Most of tliese cases have sliown alteration in t!ie size of the tumor,
depending on the position of the head. In two cases it was shown that
pressure around the periphery of the tumor did not prevent its filling;
in another, that compression on the jugular vein produced the greatest
distention of the sac. In such cases as these there can be n() douljt that
one has to do with a sacculated collection of venous blood as a result of

' Mem. (leW Aead. clelle Se. rkW Tst. di Bologna, 1873 ; Schmidt's Jahrbvch, Bd. 168, p. 216.
' See paper of Mastin (foot-note, p. 531). â– ' Heineke, /. c, p. 69.

Vol. XL— 34



530 DLSL'ASES AXIJ ISJCUIES OF THE HEAD.

injiirv by which tlic periosteum is se]>arate(l fniin the hone, ami which is
fed witii veins tiiat eoiiiieet direetiy or iiitlireetly with tlie sinus. There
is a ])ossil)ility of sucli result after fracture with laceration of the sinus
or after separation of tiie periosteum at this point, where well-known
emissaries pass through the bone. The depression of bone which is
usually a concomitant feature may be duo to injury or to absorption.
This condition has been called by Dufour " fistule osteo-vasculare ;" by
Stromcyer, "sinus perieranii;" by Hecker, " varix s))urius vensB di})lo-
etici."

Finally, there is a class of blood-tumors conceruinji' whose uature one
cannot be (piite certain. These are the so-called hernial dilatations of
the superior longitudinal sinus. Cases of this kind have been reported
by Beikert, Flint, Busch, Ogle, Chassaignac, and others. Beikert's case
was known only as an anatomical specimen. In the other cases the
tumor was either congenital or developed early in childhood. These
tumors usually prest'Ut through oj)enings in the bone. Pressure upon
them causes vertigo and, perhaps, crying, while by compression on the
jugular veins they are made more jirominent and blue. In a case of
Demme's the tumor dilated witli each inspiration. In a case of Glat-
tiiuer's it ])ulsateil and niurniurs were heard within it. In this latter
case choked disk and sudden blindness seemed to indicate a sudden exag-
geration of intracranial pressure. Of 7 cases the tumor was 6 times in
the occipital region ; in 1 of these the tumor reached the size of a robin's
egg. In Demme's case the sac of the tumor was found to be of the same
structure as the sinus-wall. In the case of Foueteau there were found
two accessory sacs containing serous fluid. The opening into the siiuis
was as large as the little finger. By Heineke' tliese tumors are regardt'd
as a peculiar form of eneephalocele, which, he reminds us, is often con-
nected with hydrocephalus ; and its complications are foinid in the cases
reported by Glattauer and Ogle.

So far as the diagnosis of these venous tumors goes, we linil, for the
most ]iart, that they are easily compressible, tliat their size depends in a
measure upon the position of the head, and that some of them are influ-
enced l)y respiration ; that compression of their periphery does not hinder
their tilling ; that when comjiressed such pressure oi'dinarily increases in-
tracranial pressure; and that there is corresponding increase of pulsation.
They may be distinguished from simple varix by the fact that pressure
upon the periphery seems not to influence them, which also distinguishes
them from racemose varix. Various spurious foi-mations and eireum-
scribed tumors lie usually in bony depressions, but are less influenced
by ])rcssure.

There are other conditions quite likely to lead to confusion. We
have to distinguish between the aneurysms, aneurysmal varices, and
eneephalocele. Most of the aneurysms are fed from the superficial
arteries. Pressure ujmiu them can be made significant ; so, too, with
aneurysmal varices. There can almost always be found a communicat-
ing artery, jjressure u])on which will stop the jnilsation in the tumor.
As between these venous tumors and encephaloceles diagnt)sis may pos-
sibly be difficult. It will be easier, however, by noticing the bluish
tint of the venous tumor, which cannot be mistaken for the more bluish

' L. c, p. 63.



NON-INFLAMMATOKY CONDITIONS OF THE SKULL. 531

skin over an encophalocele wlicn its veins are filled. Tiic rciliieibility
of the tumor upon pressure niay be quite similar, as well as its consist-
ence ; but an encephaloeele will not till fuller and expand by compression
of the jusjnlar vein, as will be the case with a venous tumor.

Treatment of these venous tumors, so far as the deeper forms are
concerned, is not very satisfactory. In 3 cases tumors have been incised.
In Flint's case it made a very serious heniorrliaye. In a case reported
by Pelletan the hemorrhatic was so serious as to call for styptics. As a
result of their use there may set in a purulent meningitis, which may
carry off the child. Azam made an incision through the traumatic
varix, which was followed by no serious trouble, and later healed,
Mersscmann healed one such case by continuous compression. Bar-
deleben succeeded in healing one communicating tumor by electro
puncture; but in most of the cases no attempt has been made, because
of anticipated danger.'

Non-inflammatory Conditions of the Skull.

incomplete formation of bone ; aplasia cranii ; osteogenesis

cranii imperfecta.

The skulls of the new-born are sometimes found to be incomplete,
the bone being more or less deficient. At other times the bones of the
vertex are found unduly thin and flexible, and almost transparent in
spots, with lines running along them, marking them like the ribs of a
leaf. In certain instances also there will be found complete defects, so
that tlu' bone ajtjicars to have been perforated. For instance, Boecker
found in the skull of a child thirty-three days old that but a small jior-
tion of the occijiital bone and of the petrosal was composed of really
osseous tissue. These defects, however, are more commonly met with in
the region of the frontal and temporal bout's. These are sometimes as
thin as ])aper, or the osteo-cartilaginous tissues will be found extending
outward in radial stripes, or isolated jiatches of bony tissue will be found
which serve to separate the ]xeriosteum and the dura in small patches.
In cases of moderate aplasia there are often other disturbances of devel-
opment, particidarly in the osseous system, which appear during the early
months of childhood. The ossification may come on gradually with
some delay, or it may proceed from various centres, which are in this
sense abnormal, since they do not accord with the general jilan of ossifi-
cation. The fragments of bone thus produced freely blend together, and
may eventually make a skull ])ractically as good as a normal one.
Under these circumstances individual bones may be developed which are
more than mere Wormian bones and yet receive no definite names. Ogle
and Paget have described interesting cases of this "hind. Probably the

' A very interesting monosrapli by W. M. Mastin on " Venons Blood-tumors of the
C'raniuiii" was published in 1885 in the Annalf! of Suri/ery, .iml continued in the Jouni. of
the Am. Meil. Axm'n, 1886, September and Octolier. He calls attention to the fact that
these tumors have been known by various names aside from those above given ; as,
"osteovascular fistuUe," "erectile tumors of the sknll," "sanguineous tumors of the
vault," "sanguineous herniic," "varicoceles of the skull," "subpericranial venous tumors,"
" reducible sanguineous tumors of the vault." etc. ; and he classifies them as, a, congeni-
tal ; i, spontaneous; and e, traumatic. For further information with regard to these
surgical curiosities the reader must be referred to his paper.



532



DISEASES AND IMirillES OF THE HEAD.



most perfect s])eciiii('n of its kind, liowcver, lias been described by Vro-
lik : ' The skull consisted of ;i great number of cranial bones, for the

Fig. 398.




Aplasia cranii: incomplete ossificutiun (Bruns).



most part united, leaving a few osseous defects. A similar case, found
in the Wiirzburg Collection, lias been described by Bidder. Some of



Fig. 399.




Aplasia cranii: incomplete ossification (Bnms).



the children born witii these peculiar skulls are in other respects well
formed ; others are defective. In some of them there has been such

' Tabnhn ad iUimt. embrj/ogen, etc., Anistelodam, 1849.



N0y-INFLA3nHT0RY CONDITIONS OF THE SKULL.



533



complete failure of true l)oiic-develo])nK'iit as to cause them to be
regarded as cases of fa?tal rhachitis. ^Vluâ– u tlie history has been secui'ed



Vm:. 400.




Same as i â–  â– - i â–  i'

it has almost always been found that the parents were badly nourished,
particularly the mother, while in some of them a history of syphilis
plays an important part, as it certainly did in A^rolik's case. These con-
ditions are more essential tiian are those dbtainini;' in onlinary hydro-
cephalus, since in the latter case it is the increasing pressure which pre-
vents the proper ossification of the bones.



Fig. 401.







Incomplete ossification of dift'erent type (Bruns).

Aplasia seems to be at times a unilateral defect, and under these cir-
cumstances may contrilmte toward the formation of meningocele. He-



534



DISEA^IX AXD IXJUEIES OF THE HEAD.



reditary influences may be concerned in these cases ; and Frank has
rec<)r(U'd the case of a woman wiio bore three eliikh-en successively, all
of whom sliowed lateral defects in the frontal re<i'ion. In this case, how-
ever, he found in tiic mother a tumor involving tiie promontory of the
sacrum, and tliis it was which probably interfered witii ossification of the
fcetal skulls. Again, the presence of cystic and dermoid tumors has been
known to prevent ossificatit)n. It is quite probable that many cases of
apparent defect of ossification in adults are due to the ])resence at some
time of such tumors rather tiian to a primary or original aplasia.

In many of these instances it may be p.ossible to j)rotcet the growing
skull by means of some material moulded on a cast, which shall be pro-
tective both from within and from without. Celluloid or gutta-percha
would probaljly answer this purpose.



Disappearance of the Cranial Bones,
atrophia seu anosto.sis craxii.



In the skull complete disappearance
frequently observed. It may come on as



of l)one-sul)stance is not in-
an interstitial or as an excen-



FiG. 402




Abf^orpti



W'ouil Museum).



trie ancstosis. Interstitial atrophy may happen at any point, and resem-
bles atrophy of any other part of the bony skeleton. Excentric atrophy
begins at one or more points and jiroceeds in a centrifugal direction, the



RHACHITIS OF THE SKULL. 535

bone disappearing along tlic margin of the o])ening already resnlting
from the ])roce.ss. This is qnite eonimon in the skulls of the aged, and
by it large bones have been redneed to the thinness of paper. Complete
perforation may even result in this way, and the Dpening thus made may
have eonsiderable extent. It is jiractieally nothing else in these eases
than the senile atrophy whieh is met with in the other parts of the body.
^Mien it begins with the diploe, the bone becomes more spongy and por-
ous and the marrow more vascular and dark. This condition is usually
tliffuse, but sometimes circumscribed. In hypenemia of the diploe it
often presents an almost inHammatory appearance, the cause being
unknown. This is accomj)anie<l l)y no signs, anil may not be suspected
during life. It seems to be almost of the same character as osteomalacia.

of more practical importance is excentric atrophy beginning on the
outside. The shape and size of the opening thus produced may vary
verv nuich. It may be produced by pressure of a tumor and in many
other wavs. The most connuon causes of pressure are the so-called
"Pacchionian granulations." The depressions in which these lie are
evervwhere recognized ; around them we meet, sometimes, formation of
new bone, so that cpiite a cavity may be formed. Sarcoma of the dura
will produce the same effect, and even pressure of blood-vessels within
the dura seems to jirodnce growths by atrophic processes. There is
another form of excentric atrophy by which the external table disap-
]>ears, at least in patches, leaving a very thin internal table. It is met
with most often in the parietal region in the skulls of the elderly. It
was first described by Lobstein and Rokitansky. It may be possible to
recognize this condition in the living.

Even fever or an increasing hydrocephalus may jjroduce an internal
excentric anostosis. One cause which is supposed to lead to this condi-
tion is sjiontaneons separation of sutures, wliich can occur as the result
of pressure in hydn)cephalns. As a rule, it does not occur after the
fifth year of life. In the literature of surgery there may be found
numerous instances in which a spontaneous dehiscence of sutures has
occurred. About its cause really nothing is known, although it would
seem that it must be produced by increasing pressure of some kiud.

Rhachitis of the Skull ; Craniotabes.

Rickets is universally recognized as a constitutional disturbance
largely affecting the growth of the bone. It was Elsaesser' who in
184;} first ade([uately described rhachitis of the cranial liones, and who
iutroduceil the name " <'rani(itabes." As a result of careful study it has
been shown that when this condition is present the bone becomes unduly
thick and its shape is altered. As a consequence of this and the inal>ility
to expand easily we have a flattened vertex with delayed ossification.
The shape and size of the fontanelles are also characteristically altered.
As the children grow the sutures become more and more firndy fastened
together, while of late there has been a great deal of investigation con-
cerning craniotabes as a cause of defective brain-development. Rela-
tively speaking, the skull of the young is abnormally developed. So
long as children do not leave the cot or are carried in the nurse's arms

' Archie J. Phjswlog. Ueilkundc, 1S4S, vii. iS)o.



536



DISEASES A. VI) IS.IVRIES OF THE HEAD.



the pressure or weif>;ht of tlie liead is borne upon tlie o('('i|)ital region.
Valentine lias reekoneil tlie weigiit of the ne\v-l)orn iiead as seven-
tvventy-tiftiis t)f the entire bo(ly-\\eight. The weight of the entire head,
resting, therefore, continuously upon the occiput, must have a decided
influence uj)on the s]iaj)e of tiie growing skull.

Tiie thickness of rickety cranial hones is frequently excessive, meas-
uriTig 5 to 10 niiliimetres or more. Many rhachitic skulls show a
number of small openings on cither side of the middle line where the
pericranium and dura come very close together, between them being

Fig. 403.



~~H^'



X







\



'\






; /
» '» '^■^ I/'



"^



/



Craniotalx.^ dharliiiis) (Bruns).

found a tine network of fibrous tissues. Irregular thickening may also
occur at any point. Should doubt exist as to the nature of a given
lesion, it may possibly be settled by comparing the anterior and pos-
terior parts of the skull, as well as the appearance of the same. On
account of continuous pressure of the brain on the posterior portion,
marked signs of the convolution may be found on the inner side of the
bone, while the effect upon tlie bones themselves is to press them dcejter
and make the posterior aspect of the skull much broader than the ante-
rior. Similar changes are met with often at the base of the rhachitic
skull. Irregular thickening, combined with atrophic areas and impres-
sions or depressions made in the bone by the brain, are there met with.
Perforation of the roof of the tympanum is occasionally seen. Kiessel-
bach has described a peculiar (U'pression in the external table of the
posterior temporal bone, and even in the external wall of the mastoid
antrum, as a result of rhachitis.

The characteristic a])pearances of the facial bones are more easily
recognized even than those of the skull, the former being relatively
imperfectly developed. The (picstion has been raised in many of these
cases M'hether the influence secretly at work was not n^ally sy])hilis
instead of craniotabes. That the two may be combined there is no



LEONTIASIS. 537

reason to tloulit, but that it is necessary to invoke the theory of syphilis
to explain the ordinary rhachitic alterations of the skull is certainly not
the case, although that hereditary syphilis may influence nutrition is
well known.

An expanded vertex or hack of the head is particularly seen in rha-
chitic children during; the first or second years of life, ^\']^en carefully
examined it will be found that this abnormal arrangement is to l)e
exjilained by reasons already given. A face of relatively normal mag-
nitude may seem small when set off against an unnaturally enlarged
skull posteriorly. In the normal skull the longitudinal diameter is
only one-fifth to one-fourtii greater than the transvei'sc, while in the
rhachitic skull this proportion is greatly altered. The symptoms of
cranial rhachitis arc the relative disprojiortions described above, slow
development, the preponderance of the longitudinal diameter, slow clos-
ure of the large fontanelles, and softening of the ])osterior bones of the
skull. The well-known systolic murnuir heard over the large fmta-
nelles, to whicii many have aseriluMl undue importance, can be heard
in almost any child whose fontanelles are open. Along with these signs
of craniotabes will almost always be found unmistakable signs of rha-
chitis in other parts of the body, particularly the bony skeleton.

Leontiasis.

This condition seems to have been first described by Tarin.' In his
preface he states that Herodotus seems to have appreciated this condition
among the Egyptians, while iVveado has reported in his (rrcncral HiMory
of India that certain of the inhabitants had skulls so thick that the
ordinary sabre would not penetrate them, but would be broken to pieces
uj)on them. Cardamusta refers to the same condition among the in-
habitants of the island of St. Thomas and other eipiatorial regions.
There seems to have been a general suspicion that the heat of these re-
gions made the cranial ixines thick. Jonas found among these countries
a skull so thick that he could not saw it. Welchius discovered skulls
without sutures, whose bones were as thick as the little finger ; and
Keyslerus reports also specimens in the museum at Dresden in wiiicli
the bone was as thick as a thuml). INIalpighi was the first author who
gave a complete description of hyperostosis of the skull. In 1799,
Jadelot described a cranium of this character found at Rheims. Bojanus
published a description of a similar cranium. In 1<S58, Huschke pub-
lished in Jena an elaborate memoir on cranial and facial hyperostosis,
with dcscrijition of specimens.' One of the finest specimens in exist-
ence is that described by Gaddi,-' to which Yirchow has referred in
his large work. To this condition lie gave the name of "leontiasis,"
tracing a parallel between this condition of the bone and elephantiasis
of the skin and connective tissue ; and he speaks of the frequency of
erv.sipelas in each form of disease. In 1879, Le Dentu * classified the
cases previously recorded into three groups :

â–  Osleographie, Paris, 1753.

^ Ueber Omnioselerofsis 2'otalis Rhachilica, Jena, 185S.
'' Iperostosi Scrofal. Cerebr.-vertebral, ModJna, 1864.
* La Bevue mensuelte de Med. et de Chit:, 1879.



o;58 VISEASI^S AXn INJURIES OF THE HEAD.

A. Those in wliicli the cranium alone is affected ;

B. Tliose in wiiicii tlie face alone is affected ; and

C. Tliose in wiiich hoth ari' involved.

He rejected tlic name of " hyperostosis " for that of " diffuse periostosis."
The best nionogi'a])h on the subject is that by Baumgarten,' who has
added a description of live more or less interesting specimens and the
case-reports of thirteen j)atients whose cases were observed during life.

Etiology. — It is most interesting that with scarcely an exception the
trouble extended over ti\)m twelve to sixteen years, there being only two
known exceptions to this, one of which seems to have been rather a case
of akromegaly, and the other to be an example of double osteoma of the
upper jaws. Neither predisposing causes nor anything in the family
history seems to have any bearing upon the known cases.

Signs and Symptoms. — Tiic essential sign is deformity of the head
as a I'esult of thickening of the bones of tiie skull and irregidarities
produced on their surface by osseous deposits. This " leonine aspect,"
Avhich led Virchow to give it the name by which it is generally known,
is, in fact, a very excellent deseri])tion of most of these cases, particu-
larly of the cranium figured by (iaddi. The features and the appear-
ance of the up])er part of the head may be almost cnmically distorted,
or the upper jaw may l)e so greatly liypertropiiied as to give an unj)leas-
ant and repulsive ap})earance to the lower part of the face. In one or
two instances the jaws have been so enormously developed as to cause
the nose to almost sink out of sight. A\'hen the lower jaw also is involved
tiie aspect given to the fjice is one of gigantic ]n-ognathism, and it is possi-
ble for the ])alatine vault to be consideral)ly distorted. In the case of
Biekcrsteth the deformity of the mouth was the cause of inanition and
final death from starvation. When the affection extends, as it may
possibly do, to the vertebral column, the upper portion of the body is
given a gigantic appearance, which is almost indescriliable, because the
head becomes too heavy to be properly suj)ported and the vertebral
column yields gradually inider the pressure. Some jiatients have suf-
fered violent reflex headaches during the course of the disease, and occa-
sionally epileptic crises have followed the violent headaches. These are
confined rather to the earlier stages of the disease. The organs of special
sense are more or less affected according as pressure is made on special
nerves. This pressure may be also made upon the internal organs as
well as upon the nerves — c. r/. by obliteration of the orbit. Paralyses,
epileptiform convulsions, mental disturbance, and jiaralytic dementia
have been observed toward the conclusion of life in some of these indi-
viduals. These are accidents rather than usual results. Hyjierostosis
of the cranium seems to take place upward rather th.an inward ; hence
there is, as a rule, little or no cerebral eom]>ression, and the brain-symp-
toms are to be explained rather by disturbance of circulation than by
permanent intracranial lesions. A condition of fair general health is
quite com])atil)le with the serious lesions aliove mentioned, and death is
caused rather by gi-adual obliteration of the cavities of the face and of
canals for vessels and nerves than by any direct internal pressure.

The progress of the disease is slow, but almost constant. Occa-
sionally it is complicated by finictional disturbance, sucli as amaurosis,

' La Leontiasis Osfica, Paris, 1892.



LEOXTTASIS.



539



epileptiform attacks, etc. It is extremely slow, and death may not result
for twenty or thirty years. There is reason to think that in some cases
tiiere has been a spontaneous arrest of the process.

Diagnosis. — Lcontiasis may be confounded with the deforming ossifio
lesions described by Paget, usually known as " Paget 's disease," as well
as with akromegaly. Paget's <liscase is rare about the skull or face, and
affects almost exclusively the long bones, especially at their joint-ends.
It is practically never met with previous to the fortieth year of life,
while lcontiasis is an affection bcgiiniing in adolescence. Akromegaly