tumors—in the region of the vertebral column, usually discovered at
birth, but occasionally not noticed until a later period. Thus,
Lancisi mentions a case in which the tumor first made its appearance
in a hydrocephalic child at the age of five years, and J. L. Apin one
in which the patient reached the age of twenty before any swelling was
manifested. If these records are accepted as authentic, the only
satisfactory explanation is that of Itard—viz. that the bony
deficiency existed from the time of birth, and that the dropsical
protrusion did not occur until afterward: this seems to me more
reasonable than the suggestion of the younger Andral, that the
vertebræ underwent a process of secondary softening and absorption.
The part most frequently affected is the lumbar region, but cases are
not wanting of the occurrence of spina bifida in the cervical, dorsal,
and sacral regions, and even, in at least one case (Genga's), in the
coccyx. The tumor almost invariably occupies the median line, and
projects directly backward: in at least four cases, however, recorded
by Bryant, Emmet, and Thomas, the tumor protruded anteriorly from
deficiency of the vertebral bodies.
The size of a spina bifida varies from that of a walnut to that of a
child's head, or even larger: in some rare instances, such as those
recorded by Lezelius and Richard, the whole spinal column has been
cleft, and the tumor has extended from nucha to sacrum; but cases of
this kind seldom come under the surgeon's observation, as children
with such extensive deformity usually perish shortly after birth. The
shape of the swelling is rounded, or more often oval; it is commonly
sessile, but occasionally pedunculated; and it is sometimes lobulated,
the lobules being separated by more or less distinct sulci. The skin
covering the tumor is in some cases normal, with more or less fat in
the subcutaneous connective tissue, but more often thin, tightly
stretched, red, shining, and occasionally ulcerated; more rarely it is
thickened and leathery, as in a case referred to by Sir Prescott
Hewett. Sometimes a navel-like depression is found at some part of the
surface, corresponding, as pointed out by Follin and Duplay, and by
Erb, to the place of attachment, on the interior of the sac, of the
terminal extremity of the spinal cord. The cutaneous investment is
sometimes altogether wanting, the spinal dura mater itself forming the
external covering of the tumor. It is in these cases especially that
ulceration and rupture are apt to occur.
Spinæ bifidæ are usually soft and fluctuating, and occasionally
partially reducible; their tension and elasticity are increased when
the child is in the upright position or during the acts of expiration
and crying, and are diminished during inspiration or when the child is
laid upon its face. When the part is relaxed a bony prominence can be
felt on either side, and the aperture in the vertebral column can be
more or less distinctly {759} outlined. The swelling is apt to be
painful on pressure. If the tumor is of moderate size and covered with
healthy skin, there are usually no constitutional symptoms, and, even
where ulceration and rupture occur, the opening may heal and a
spontaneous cure may possibly ensue. More often, as the tumor
increases in bulk—or at any time if pressure be made upon it—various
nervous symptoms are observed: drowsiness, muscular twitchings,
convulsions, and often paraplegia and paralysis of the sphincters.
Rupture is usually followed by the development of spinal meningitis,
or may prove directly the cause of death by the loss of cerebro-spinal
fluid which it causes; in other instances, however, if the rupture be
a small one, healing may occur (as already mentioned) or a fistulous
opening may persist; in rare cases the fluid may escape by a process
of transudation without rupture, as in an example recorded by Laborie.
As may be inferred from what has already been said, the course of
spina bifida is usually rapid and toward a fatal termination. In some
instances, however, as in cases observed by Holmes, and more recently
by Lithgow, spontaneous recovery has followed the obliteration of the
channel which unites the sac with the cavity of the spinal membranes;
and in other instances, without a cure having been effected, life has
been prolonged for very many years. Thus, Behrend reports a case in
which a patient with spina bifida lived to the age of fifty, and
Holmes refers to another in which death resulted from an independent
disease at the age of forty-three. But a still more remarkable case
was recorded by Callender, the patient in this instance having reached
the age of seventy-four.
PATHOLOGY AND MORBID ANATOMY.—The most important points for
consideration in respect to the anatomy of spina bifida are the
relations which the spinal cord and spinal nerves bear to the sac, and
the nature of the contained fluid. It is ordinarily said that the
spinal cord itself commonly enters the sac of a spina bifida—the
report of the London Clinical Society's committee gives the proportion
of cases in which it does so at 63 per cent.—and Holmes figures a
specimen from the museum of St. George's Hospital, London, in which
this condition is obviously present; on the other hand, Mayo-Robson in
eight operations only once found the cord implicated; and the late
John B. S. Jackson of Boston—whose name will be recognized as one of
high authority in regard to all questions of morbid anatomy—once
assured me that he had made very many dissections of spinæ bifidæ, and
that he had invariably found that the cord stopped short of the sac,
and that only the nerves entered the latter: this, as negative
evidence, cannot of course contravene such positive evidence as that
of the specimen referred to by Holmes, but it would seem to show that
the condition was a less common one than is generally supposed, and
that in at least a fair proportion of cases the cord itself did not
form part of the sac contents. This remark applies especially to those
cases in which the fluid is accumulated in the subarachnoid space, and
in which, as pointed out by Sir P. Hewett, the cauda equina or spinal
nerves are pushed by the vis a tergo into the sac; but when the
dropsical effusion occupies the central canal of the cord, this is apt
to be flattened and spread out like a thin lining to the sac, just as
the brain is spread over the inner wall of the skull in cases of
internal hydrocephalus; and, on the other hand, when the accumulated
fluid fills the cavity of the arachnoid the cord is apt to be pushed
forward, and the {760} sac may be entirely devoid of all
nerve-structures. Such, too, according to Giraldès, is the case in
spina bifida of the cervico-dorsal region.
The committee of the London Clinical Society classify cases of spina
bifida in three divisions: (1) Those in which the membranes only
protrude (spinal meningocele); (2) those in which the protrusion
involves both the cord and membranes (meningo-myelocele); and (3)
those in which the central canal of the cord itself forms the cavity
of the sac (syringo-myelocele). The last variety is the rarest, as the
second is the most common. In meningo-myeloceles the spinal cord with
its central canal is continued within the median, vertical portion of
the sac, and at this part there is no covering of true skin; the
nerve-roots which traverse the sac arise from this intramural portion
of the central nervous system.
Humphry of Cambridge, England, makes a somewhat similar classification
of spinæ bifidæ, recognizing as the most common variety the
hydrorachis externa anterior, in which the fluid occupies the
subarachnoid space in front of the spinal cord, and in which “the cord
and the nerves are stretched backwards and outwards upon the sac, and
are there confluent, together with the arachnoid, pia mater, and dura
mater, or their representatives, in the thin membrane which forms the
hindmost part of the wall of the sac;” rarer forms are the
hydro-meningocele, or hydrorachis externa posterior, in which the
fluid accumulates behind the cord, which does not enter the sac, and
the hydro-myelocele or hydrorachis interna, in which the fluid
occupies the central spinal canal.
The theory which, according to the Clinical Society's committee, best
explains the pathological anatomy of spina bifida is that which
assumes a primary defect of development of the mesoblast from which
the structures closing in the vertebral furrow are developed.
The fluid of spina bifida appears to be identical in character with
the cerebro-spinal fluid. Bostock found that it was very slightly
clouded by the application of heat, and that it contained, in 100
parts, 97.8 of water, 1.0 of chloride of sodium, 0.5 of albumen, 0.5
of mucus, 0.2 of gelatin, and some traces of lime. Five specimens more
recently analyzed by Hoppe-Seyler gave an average of 98.8 parts water,
0.15 parts proteids, and 1.06 extractives and salts. Turner found a
reaction somewhat similar to that of grape-sugar, as had been
previously found by Bussy and Deschamps in cerebro-spinal fluid
itself, but in two specimens analyzed by Noad for Holmes it was at
least very doubtful whether sugar was actually present. “The first
specimen was found to be completely neutral; its specific gravity was
1.0077; it contained phosphates, but no reaction could be obtained
resembling that of sugar. The second specimen ... did give a reaction
with copper like that of sugar, but no trace of fermentation could be
obtained.” Three analyses, however, made by Halliburton for the London
Clinical Society's committee showed uniformly a decided trace of
sugar, with a diminution in the quantity of proteids, which appeared
to consist entirely of globulin.
In some cases the sac of a spina bifida contains, besides
nerve-structures and cerebro-spinal fluid, both fibrous and fatty
tissues.
DIAGNOSIS.—Ordinary fatty tumors have been mistaken for spinæ bifidæ,
but such an error could only be committed through carelessness: more
difficult is the diagnosis from certain forms of congenital cyst,
occupying the median line of the back, and still more difficult the
{761} diagnosis from the several affections known as false spina
bifida. In the former case the distinction might be made by noting the
irreducibility, constant shape, and unchanging tenseness of the cyst,
and perhaps, as suggested by T. Smith, by tracing the line of spinous
processes beneath it; some information, too, might perhaps be gained
by chemical examination of the fluid obtained by puncture. Under the
name of false spina bifida Holmes includes three distinct conditions:
these are—(1) a true spina bifida, in which the connection with the
spinal membranes has in some way been obliterated, the sac then
communicating with the vertebral canal, but not with the theca; (2) a
congenital, cystic, or fatty tumor, taking its origin within the
vertebral canal, and projecting through an opening caused by a gap in
the laminæ; and (3) a tumor containing fœtal remains, and properly
regarded as an example of included fœtation. In any of these cases a
positive diagnosis might be impossible, and the probable nature of the
tumor could only be inferred by noting the absence of one or more of
the characteristic symptoms of true spina bifida.
PROGNOSIS.—The prognosis of spina bifida is without question
unfavorable; at the same time the affection is by no means to be
considered, as it was formerly, one of an invariably fatal character,
for, apart from the fact already mentioned that several cases are on
record in which patients with untreated spina bifida have reached
adult life, and even old age, the modern method of treatment has
proved so much more successful than those formerly employed that in
favorable cases surgical interference affords at least a reasonable
prospect of recovery. The circumstances which especially furnish
grounds for an unfavorable prognosis are the rapid growth of the
tumor, the thinning or ulceration of its coverings, and the occurrence
of nervous symptoms, and particularly of paralysis or convulsions.
TREATMENT.—This may be either palliative or radical. If the tumor be
not increasing materially in size, the surgeon may properly content
himself with palliative measures—applying equable support and perhaps
slight pressure by the use of a well-padded cap of gutta-percha or
leather, an air-pad, or, as advised by Treves, a simple pad of cotton
smeared with vaseline, and a bandage; if the tumor be small and
covered with healthy integument, painting its surface with collodion
may be of service through the contractile property of that substance.
Radical measures are only indicated when the child, otherwise healthy,
seems to have his life threatened by the rapid growth of the tumor,
causing risk of ulceration and rupture, or giving rise to convulsions
or paralysis. The principal modes of treatment to be considered under
this heading are—(1) simple tapping or paracentesis; (2) injection of
iodine; (3) ligation of the neck of the sac; and (4) excision.
(1) Paracentesis, a simple evacuation of the fluid contents of the
sac, is the only operation ordinarily justifiable in cases attended by
paralysis or other grave nervous manifestations. The puncture should
be made in the lower part of the sac and at a distance from the median
line (in the course of which the nerve-structures are particularly
distributed), and only a small quantity of fluid—a few drachms or at
most one or two ounces—should be removed at a time, the instrument
being at once withdrawn if convulsions follow, and the wound being
instantly closed with lint dipped in compound tincture of benzoin.
Either an aspirator-tube or a {762} small trocar may be used, and the
operation may be repeated if necessary, pressure being maintained
during the intervals. Paracentesis has occasionally though not often
proved curative.
(2) Injection of Iodine.—This mode of treatment was introduced about
the same time by Velpeau and by Brainard of Chicago. The latter
surgeon's method consists in injecting, after only partially emptying
the tumor, a solution of iodine with iodide of potassium (iodine, 5
grs.; iodide of potassium, 15 grs.; water, 1 fl. oz.), allowing it to
flow out again, washing out the sac with water, and finally
reinjecting a portion of the cerebro-spinal fluid originally
evacuated; and the former's, in completely evacuating the tumor, and
then injecting a mixture of iodine and iodide of potassium, each one
part to ten parts of water. Each of these plans has met with a fair
measure of success;[1] but the modification introduced by James Morton
of Glasgow is a great improvement, and affords what is actually the
most successful mode yet devised for dealing with spina bifida. Morton
employs a solution of iodine in glycerin (iodine, 10 grs.; iodide of
potassium, 30 grs.; glycerin, 1 fl. oz.), which has the advantage of
being less diffusible than the aqueous solution, and therefore less
liable to enter the spinal canal. The tumor is about half emptied, and
a small quantity—from fluid drachm ss to fluid drachm iij of the
solution—is then slowly injected, and allowed to remain. The operation
is repeated after a few days if necessary. Of 50 cases known to Morton
as having been treated in this way up to May, 1885, 41 were regarded
as successful; but of 71 cases collected by the London Clinical
Society's committee, only 39 had been benefited by the operation.
[Footnote 1: In a case recently recorded by Woltering, however, iodine
injection was followed by death within half an hour.]
The introduction of iodine into the sac of a spina bifida is,
according to Morton, only justifiable in cases unattended by
paralysis; under opposite circumstances I should be disposed to try a
plan recently employed with success by Noble Smith in a case of
meningocele—viz. injecting the iodo-glycerin solution into the
coverings of the sac, and as close to it as possible without
perforating it.
(3) and (4). Ligation and excision have each occasionally effected a
cure, but more often have but helped to precipitate a fatal issue. A
successful case of ligation followed by excision has been recently
recorded by Löbker. The elastic ligature, applied around the neck of
the sac (if this be pedunculated), has been employed by Laroyenne,
Ball, Colognese, Baldossare, Mouchet, and other surgeons, and of 6
cases collected by Mouchet, 3 ended in recovery. Another success has
been recently recorded by Turretta. The écraseur has been used, but
its employment cannot be recommended. Compression by clamps is advised
by Schatz of Rostock. Ligation is best adapted to cases occurring in
the cervical and dorsal regions, in which, as pointed out by Giraldès,
nerve-elements are less likely to be involved. Excision, supplemented
by transplantation of a strip of periosteum from a rabbit, has been
successfully resorted to by Mayo-Robson and by R. T. Hayes of
Rochester, N. Y.; and simple excision, with suture in separate lines
of the sac and integuments, by Mayo-Robson, Atkinson, and Jessop. The
Clinical Society's committee in all refers to 23 cases treated by
excision, 16 of which are said to have been successful.
{763}
ANÆMIA AND HYPERÆMIA OF THE BRAIN AND SPINAL CORD.
BY E. C. SPITZKA, M.D.
Cerebral Hyperæmia.
Up to within a few years it was a favorite mode of explaining the
results of the administration of certain narcotic and stimulant drugs,
and certain of the active symptoms of mental derangement, to attribute
them to an increased blood-supply of the nerve-centres. This view
seemed to harmonize so thoroughly with the physiological dictum that
functional activity depends on the supply of oxygenated blood that the
first attempts at questioning it were treated as heresies. To-day,
however, few authorities can be found to adhere unreservedly to this
once-popular and easy explanation. The drift of physiological and
medical opinion is in the direction of regarding some subtle molecular
and dynamic state of the nerve-elements as the essential factor in
intoxications as well as in maniacal and other forms of insane
excitement: if they be complicated by active or passive congestions,
this is probably a secondary occurrence of modifying but not of
intrinsic determining power. While this change in our views is the
natural result of progress in experimental pharmacology and pathology,
it does not justify the extreme assertion that there is no disorder of
the brain functions deserving the name of congestion and hyperæmia.
This assertion seems to have been provoked by the careless manner in
which these terms have been employed to designate conditions which are
in reality the most different in nature that can be well conceived. No
one familiar with the extent to which the term “congestion of the base
of the brain” has been abused in this country will marvel that the
reaction provoked by it has overstepped the boundaries of cautious
criticism. That there are physiological hyperæmias of the brain is now
universally admitted; the most recent experimental observations,
indeed, conform most closely to the claims of the older investigators.
It naturally follows that pathological hyperæmias are both possible
and probable, and even if the observations in the dead-house do not
strongly sustain the existence of pathological hyperæmias and
congestions independently of gross disease, clinical analysis and the
gratifying results of appropriate treatment justify us in retaining
these designations in our nomenclature with the limitation here
implied.
One great difficulty in determining the precise nature of the
disorders which the physician is called upon to treat on the theory of
hyperæmia lies in the number of factors which may contribute to or
modify its {764} development. If, for example, the action of the heart
be increased through hypertrophy, the result to the cerebral
circulation will obviously be different in a plethoric and in an
anæmic person; it will be also very much different in the event of the
stimulation of the centres which contract the calibre of the cerebral
blood-vessels from what it would be if there were a state of vascular
relaxation. Should the cardiac hypertrophy be associated with renal
disease, other disturbing elements may be introduced, such as
arterio-fibrosis, or the presence in the blood of certain toxic
substances having direct effects of their own on the nerve-elements.
The picture may be still more complicated by variations in the
intracranial pressure. It is impossible to prove, either by direct or
indirect evidence, that there is such a pathological state as a simple
cerebral hyperæmia; indeed, there is one fact which militates strongly
against, if it be not fatal to, such an assumption. Were a
physiological hyperæmia to become intensified to a pathological
degree, we should have corresponding clinical phenomena. In other
words, the culmination of the morbid process should be preceded by an
exaggerated physiological excitation similar to that observed with
intoxications. But this is rarely the case, and we accordingly find
that the more cautious writers, like Nothnagel,[1] do not commit
themselves to the view that the results of mental overstrain[2] are to
be classed as simple cerebral hyperæmia. Similar restrictions are to
be made regarding the established congestive states, such as those
following sunstroke. It has been usually supposed that insolation
directly produces cerebral hyperæmia, even to the degree of
engorgement, and that the after symptoms in persons who recover are
due to the non-return of the meningeal and cerebral blood-vessels to
their normal calibre, and to other more remote results of vascular
stasis. The latter half of this proposition is in part correct; the
former is contradicted by numerous pathological observations. Thus,
Arndt,[3] who had the opportunity of studying over one hundred cases
occurring in the course of a forced march of a division of infantry
from Berlin to Pankow, many of which terminated fatally, found almost
uniformly a pale brain, with peculiar color-changes, denoting rather
structural than circulatory trouble.[4] The {765} whole list of causes
of what is commonly designated cerebral hyperæmia, congestion, and
engorgement may be gone through with and similar modifying statements
be found to apply to them. The nearest approach to an ideal cerebral
congestion is that found with acute alcoholic intoxication. This is at
first accompanied by cerebral hyperæmia, which, with the comatose
climax, becomes an engorgement; accordingly, many of the results of
acute alcoholic intoxication are attributed to the circulatory
condition alone. The congestive troubles due to alcoholism which come
to the special ken of the physician, however, are those found with the
chronic form, and here a more complex pathological condition is found
to underlie it; the organization of the brain is altered, the vascular
channels more or less diseased, and the vaso-motor mechanism
continuously deranged. This disorder, as well as the apoplectiform
states attributed to vascular stasis, and the active and passive
hyperæmias associated with tumors, meningitic and other gross diseases
of the brain, as well as with the status epilepticus, are usually
included in the discussion of cerebral congestion, and serve to swell
up the chapters devoted to it. They will be found discussed in more
appropriate situations in this volume. In this place it is proposed to
consider only those congestive states which present themselves to the
physician, independently of conditions which, if associated,
preponderate in clinical and therapeutic importance.[5]
[Footnote 1: _Ziemssen's Cyclopædia_, “Nervous System,” i. p. 39, 2d
German ed.]