contemporaneous with the keratitis which appears in the neighborhood of
puberty,[89] while those of the nerve are somewhat later in point of
time, and are almost always conjoined with retinitis, choroiditis, and
optic neuritis. As usual when investigating or describing any subject
relating to syphilis, Mr. Hutchinson's opinion and observation must be
detailed. In 1863 he wrote[90] that it was only recently that he had
thought of specially investigating the disorders of hearing in
reference to hereditary taint, having had his attention called to a
peculiar form of deafness, usually symmetrical, passing rapidly through
its different stages and {283} unaccompanied by any marked degree of
pain or any external disease. He then reported eighteen cases of which
he had notes. The oldest of these patients was twenty-seven, the
youngest eight--the average time of development of the deafness from
twelve to fifteen. Although the membrana tympani was in no instance
quite normal, in none were there found adequate changes to account for
the deafness. In all the Eustachian tubes were pervious. In nearly all
the disease was symmetrical. This fact, together with the absence of
discoverable lesions of the external or middle ear, seems to point
conclusively to disease of the nerves themselves, or at least to a
central cause.[91] He adds: "With regard to the prognosis of
heredito-syphilitic deafness, I believe that it is very unfavorable.
When the disease was progressive I have rarely witnessed any permanent
improvement or arrest. In most it has gone on to total loss of hearing,
and this in several instances in spite of the cautious use of specific
remedies almost from the beginning. From six months to a year would
appear to be the usual time required for the completion of the process
and the entire abolition of the function."[92]
[Footnote 89: Purves, _Guy's Hospital Reports_, 1875, p. 564;
Pritchard, _British Medical Journal_, April 21, 1877.]
[Footnote 90: _Clinical Memoirs on Certain Diseases of the Eye and Ear
consequent on Inherited Syphilis_, London, 1863, pp. 182, 183.]
[Footnote 91: In the _Lancet_ for Jan. 16, 1875, he reports a case of
total deafness in a young woman of seventeen which had come on in ten
months without pain or otorrhoea. He believes the disease of the organ
of hearing to be parallel with those cases of choroiditis disseminata
or of optic neuritis in which blindness is produced without pain or any
external evidence of inflammation, and which are distinctly and
positively associated with inherited syphilis.]
[Footnote 92: Mr. Hinton, in his edition of Toynbee's work on _Diseases
of the Ear_, states that at Guy's Hospital, of his aural patients, one
in twenty is affected with deafness due to heredito-syphilis; that it
usually makes its appearance between the tenth and sixteenth years; and
that the great majority of the cases which he has seen have been
females. He adds: "Patients suffering from this disease may, as a rule,
at least when young, be at once distinguished by the amount of deafness
which they exhibit. I know no other affection except fever which in a
person under twenty brings on a deafness so rapidly and so nearly
complete. In the course of a few weeks a girl previously hearing well
will, without pain or known cause, become unable to distinguish words."
In one of Dalby's cases total deafness came on in three weeks, previous
to which hearing was normal. According to Pierce, the deafness is most
apt to manifest itself between eleven and eighteen years of age.
Troeltsch says that "l'audition du diapason par le vertex" is lost at
an early date after the beginning of the disease, and that there are
also often concomitant affections of the nose and pharynx.]
Dalby[93] is said to regard syphilis as, next to scarlatina, the most
fruitful cause of deaf-mutism as it occurs in children born with good
hearing powers. "The patient usually becomes deaf in early
childhood--after he begins to talk--or between this period and
puberty."[94]
[Footnote 93: _The Lancet_, Jan. 22, 1876.]
[Footnote 94: Bumstead, _op. cit._, p. 734.]
Syphilis of the Liver.--In 1852, Gubler published an account of the
general appearances in syphilitic disease of the liver in new-born
children, which was distinguished especially by increase in size and
weight. This increase depended, as might be expected in this
stage--that of general cell-proliferation--upon a proliferation of
cells from the connective tissue between the acini, or from the
adventitia of the interlobular vessels, this growth becoming
transformed into connective tissue.[95] The change is quite analogous
to what is taking place at the same time in the skin, the mucous
membranes, and other tissues. Wilks has also described[96] a form of
syphilitic disease of the liver which corresponds to that of Gubler,
and in which the whole organ is infiltrated by a new fibrous tissue,
producing a uniform and general hardening.
[Footnote 95: Bäumler, _op. cit._, p. 186.]
[Footnote 96: _Trans. Path. Soc._, vol. xvii., 1866.]
{284} [Illustration: FIG. 8. Section of an old gumma of the liver. _a_,
_a_. Central caseous tissue of the gumma. _v'_, _v'_. Its vessels. _l_.
Boundary between the central portion and fibrous zone; this line of
demarcation is marked in places by an opening or cleft. _t_, _t_.
Connective tissue of the fibrous zone which entirely surrounds the
central part. _v_, _v_. Small vessels of this zone. _c_. An arteriole
of the fibrous zone. _f_, _f_. Quite large biliary vessels included in
the fibrous zone. _t'_. Fasciculi of connective-tissue fibres running
parallel with the surface of the caseous part. At _b_ and _d_ the
fasciculi of fibres of the fibrous zone penetrate into the central
caseous part. _e_, _e_. Tissue of hepatic cells interrupted by bands of
fibrous tissue, _m_, _m_. X 12.]
As described by Gubler,[97] the liver in such children is
hypertrophied; hard, resistant to pressure, so that it cannot be
indented; elastic, so that it rebounds; creaks, but does not bleed,
when it is cut into, and presents the yellow color and the
semi-transparence of flint. There are seen on a yellowish ground a
number of small white granulations like grains of wheat, which a
histological examination shows to be formed by an accumulation of
embryonic cells in the spaces which separate the hepatic acini.
Injections reveal the fact that the vascular network has become almost
impenetrable, the capillaries obliterated, the larger vessels
diminished in calibre. Fibro-plastic matter is found throughout the
organ in large quantity. In consequence of these conditions--the
compression of the hepatic cells and the destruction of the
vessels--the secretion of bile is stopped, and the gall-bladder is
found after death to contain a pale-yellow liquid consisting of bile
mixed with an excess of mucus. This form of hepatitis has thus far been
observed almost exclusively in infants. Cornil {285} says[98] that he
has had frequent occasion to examine such cases of hepatic syphilis,
and describes them as follows: "The hepatic acini, in the normal state,
are in contact except at the prismatic spaces which are formed by their
union--spaces in which the capsule of Glisson forms an envelope to the
afferent portal vessels of the lobuli. It is in these spaces that the
round lymph-cells form and collect into small nodules representing
microscopic gummata. The cells at the centre of the new formation are
sometimes granular. This neoplasm is seated about the ramifications of
the portal veins, which in consequence also present thickened walls
with newly-formed cells in their external tissues. The small granules
above mentioned are not always visible to the naked eye, and in their
places are only seen, about the perilobular capillaries of the portal
vein, an excessive number of embryonic cells." In addition to this
interstitial sclerosis or interstitial infiltrating hepatitis there is
an inflammation of the liver depending upon the presence of
gummata--gummous hepatitis--which occurs in two forms: one in which
very small and very numerous nodules are present, situated along the
course of the fibrous seams, the prolongation of the capsule, and
another in which there are two or three large circumscribed tumors.
This form of hepatitis is always accompanied by the interstitial form,
although the latter may be only slightly developed.[99] The gummata,
though not infrequently found in the liver of new-born children, are
more likely to develop later, at from about the eighth to the twelfth
year.
[Footnote 97: _Mémoires sur une nouvelle Affection de Foie_, and _Gaz.
Méd. de Paris_, 1852.]
[Footnote 98: _Op. cit._, Am. ed., p. 370.]
[Footnote 99: It does not differ essentially, either pathologically or
clinically, from the same lesion in adults.]
Rochebonne[100] describes the following symptoms of syphilitic
hepatitis in infants: A deep wine-colored venous stain and oedema of
the lower extremities, often accompanied by pemphigus; ascites due to
mechanical obstruction of the circulation, as in cirrhosis; a more or
less pronounced chloro-anæmic appearance of the face; and the presence
in the urine of albumen and hæmato-globulin. Vomiting may occur, and
constipation alternating with diarrhoea has been observed. Icterus,
symptomatic of the affection, has not been observed.
[Footnote 100: Quoted by Bumstead and Taylor, p. 758.]
Bäumler says:[101] Implication of the peritoneal coating of the liver
may be recognized by the pain in the hepatic region. In new-born
children--unless, possibly, there may be some enlargement of the
liver--the only local symptoms, often, are those due to
peritonitis--screaming, drawing up of the legs, vomiting. In those
cases it is not rare for the peritonitis to become diffuse.[102]
[Footnote 101: _Op. cit._, p. 194.]
[Footnote 102: In an article on "Inherited Syphilis" in the _British
and For. Medico-Chirurgical Review_, 1875, p. 28, it is said: "Of the
liver the lesion consists in enlargement and induration of the organ in
whole or in part, due to the development of fibro-plastic material
between the cells of the acini, with obliteration of the vessels and
interference with the secretion of bile. This condition is generally
doubtful during uterine life, and is rapidly fatal. The symptoms are
vomiting, diarrhoea, and tympanitis, but, strange to say, no jaundice.
The enlarged and indurated organ may be felt by palpation. It is
probably in this connection that the peritonitis described by Simpson
as occurring in inherited syphilis is found."]
Hill says:[103] "The symptoms are mainly those of functional
derangement of the organ, with alteration of its bulk."
[Footnote 103: _Op. cit._, p. 163.]
Hutchinson[104] has described cases in which in young persons the
subjects {286} of hereditary syphilis there has been great hepatic
enlargement which has subsequently wholly disappeared. He finds it
difficult to believe that there is any kind of gummous growth in such
cases, and feels obliged rather to fall back upon the hypothesis of
mere vascular turgescence. In one such case the liver occasionally was
so large as to be visible as the patient lay on his back in bed.[105]
[Footnote 104: _Path. Transactions_, 1877, p. 309.]
[Footnote 105: Illustrative cases of this condition may be found in the
_Med. Times and Gazette_, Dec. 22, 1877.]
It seems much more likely that the enlargement is due to an
exceptionally active cell-proliferation, which does not, however, go on
to organization, but may be just as susceptible of absorption and
resolution as are the papules or maculæ of the skin. A portion of the
enlargement may be due to a passive congestion caused by the presence
of this cell-accumulation.[106]
[Footnote 106: Barlow (_Path. Trans._, 1877, p. 355) has suggested that
the engorgement is only a preliminary stage of the fibrous thickening,
and may disappear either with or without leaving permanent contractions
or adhesions in its wake.]
As to the diagnosis of hepatic syphilis in infants, I am disposed to
agree with Cornil, who says:[107] "The symptoms are null, or they are
identical with those of local and general troubles so often observed in
children who have poor or insufficient nourishment. The only physical
sign which properly belongs to hepatic syphilis is, when it exists at
all, increase in the size of the liver."
[Footnote 107: _Op. cit._]
Syphilis of the Bones.--Until the publication in 1870 of the researches
of S. Wegner,[108] an assistant of Prof. Virchow, diseases of the
osseous system due to hereditary syphilis were either ignored or denied
by the various writers upon this subject.[109] Valleix, Bargione,
Ranvier, and Guéniot had indeed recorded cases of bone disease
occurring at the points of junction between the epiphyses and diaphyses
and in the costal cartilages, but it remained for Wegner first fully to
describe the pathological changes which occurred there, and to
differentiate them from those due to rickets or scrofula. His memoirs
recognized three stages of alteration in the long bones:[110] 1st.
While in the normal state the boundary of the hyaline cartilage is
distinctly marked by a line which indicates the direct transformation
of the cartilaginous tissue into a spongy tissue, the unaided eye being
unable to distinguish a spongio-calcareous layer, in new-born
syphilitic children, on the contrary, the bones are seen to have a
spongio-calcareous layer interposed between the bone and cartilage,
measuring two millimeters in thickness. This is a zone of calcifying
cartilaginous material more extensive than in the normal state. 2d.
These same changes become more distinct and more extensive. The
unnaturally thick layer of calcareous material continues to grow. There
is proliferation of the cartilaginous trabeculæ, abundant calcification
of the cartilage, too early and irregular ossification of the
intercellular substance {287} of the cartilage, and at the same time an
arrest of the normal formation of bone which should be going on from
the epiphysial cartilage. 3d. There is now added, by extension of these
processes, a thickening of the perichondrium and periosteum at the
extremities of the long bones and at the junction of the ribs with the
costal cartilages. In consequence of the interference with nutrition
occasioned by these changes atrophy and fatty degeneration of the
cartilage-cells occur, and they form between the epiphysis and
diaphysis a necrosed mass which irritates the living bone. This causes
osteo-myelitis, which frequently results in a separation of the
epiphyses. Occasionally pus is produced in such quantity as to
perforate the periosteum, escape into the surrounding tissues, and
become superficial. He terms the entire process an osteo-chondritis.
[Footnote 108: _Virchow's Archiv_, 1870, B. 50, S. 305: "Ueber
hereditäre knochen Syphilis bei jungen Kindern."]
[Footnote 109: Diday says: "Affections of the bones are so rare in
children with inherited syphilis that the annals of medicine scarcely
offer five or six well-authenticated cases of caries or periostitis"
(_op. cit._, p. 83). Referring to this statement, Mr. Hutchinson
remarks: "So different has been my own experience from this that I may
say that we are scarcely ever without a severe example of it in the
wards of the London Hospital" (_Illustrations of Clinical Surgery_,
London, 1875, p. 47).]
[Footnote 110: Cornil, _op. cit._, p. 282 _et seq._]
Waldeyer and Köhner,[111] after examining twelve cases, confirm in the
main these investigations of Wegner, but interpret the changes as
arising rather from the formation of a gummous tissue between the
epiphysis and diaphysis than from an osteo-chondritis. The tissue-death
which occurs later, the atrophy of the cells, etc., they compare with
the same modifications observed in syphilomata.
[Footnote 111: "Beiträge zur Kenntwiss der hereditäre knochen
Syphilis," _Virchow's Archiv_, B. 55, S. 367.]
Parrot[112] in a number of exceedingly valuable papers has repeated and
greatly extended these observations. He places especial importance upon
the formation of osteophytes, which, he says, in the first stage
envelop the diaphyses of the long bones, especially at their inferior
extremities. In the succeeding stage the new bony layers are more
porous; a gelatinous degeneration affects the epiphysial cartilage and
the spongy bones at a point where they are in contact; the epiphyses
tend to separate from the diaphyses. This solution of continuity
results in a characteristic pseudo-paralysis, with curvatures, abnormal
twistings, and preternatural mobility of the bones, with loss of the
power of locomotion. Then the osteophytes increase in size by the
formation of several layers, thus enlarging the inferior extremities of
the long bones. He describes the general process as consisting, first,
of a periosteo-genesis--a formation of osseous tissue from the
periosteum; next of a chondro-calcosis--a calcareous incrustation of
cartilage; and finally of a gelatiniform degeneration and softening of
the bone, with diaphyso-epiphysial disjunction.[113]
[Footnote 112: _Société de Biologie_, June 1, 1872; _Société
anatomique_, 1873, p. 92; _Archives de Physiologie_, 1876, vol. iii.
pp. 138, 139; _Revue mensuelle de Médecine et de Chirurgie_, 1877;
_Pathological Trans._, 1871, vol. xxx. p. 339, etc., etc.]
[Footnote 113: Cornil (_op. cit._) coincides in the main with this
description.]
Taylor[114] sums up the results of his observations as follows: "In the
first stage we have a simple hyperplasia of cells with irregular
deposition of lime salts; in the second, an intensification of this
condition; and in the third, a new element--namely, the abnormal
proliferation of all the elements of the tissues, with an infiltration
of granulation-tissue into the medullary spaces following the
vessels."[115]
[Footnote 114: _Syphilitic Lesions of the Osseous System in Infants and
Young Children_, New York, 1875, p. 134.]
[Footnote 115: Verraguth (_Archiv für Path. Anat._) describes the first
step as an excessive formation of vessels in the cartilage and a
corresponding overgrowth of the cellular elements. This becomes
inflammatory, and constitutes a primary syphilitic chondritis, the
changes in the medulla of the bone being degenerative and secondary to
the affection of the cartilage. Still other observers have described
the process, each with minor modifications; but as they are of no
clinical importance, it does not seem worth while to quote them.]
{288} We see, then, that, setting aside minor points of difference,
these observers all coincide in describing this condition as one
essentially of the nature of syphilitic bone troubles with which we are
familiar in the acquired form of the disease, consisting primarily and
throughout of an unnatural accumulation of cell-elements, which in the
later stages by their pressure produce various degenerations of
surrounding structures, and which, as they occur during the process of
bone-formation, are accompanied by irregular and abnormal deposition of
lime salts. They especially affect the regions mentioned--the junctions
of the epiphyses and diaphyses--because at that time those points are
the seat of great physiological activity. Syphilis, indeed, throughout
its entire course is notably subject to similar influences, as one
example of which I may instance the preference displayed by the
periostitis which results in nodes or in caries for the subcutaneous
bones, the tibia, clavicle, cranium, etc.; or, in other words, for
those which are subject to frequent traumatisms--trifling, perhaps, but
sufficient to determine a slight hyperæmia, which is followed by
abnormal cell-proliferation or accumulation.
The symptoms which obtain in this condition of syphilitic
osteo-chondritis are as follows: The child may be attacked during
intra-uterine life, and in that event the osseous lesions will probably
be coincident with other syphilomata and with placental disease of
sufficient gravity to destroy life.[116] If the child is born alive,
the first development of the disease will probably be noticed as a
swelling at the diaphyso-epiphysial junction of one of the long bones,
which in the emaciated subjects of hereditary syphilis is often
visible, and can always be discovered by palpation. The bones most
frequently attacked are the humerus, radius and ulna, tibia and femur,
but the clavicle, ribs, sternum, and bones of the metatarsus and
metacarpus are also often involved, and much more rarely the frontal
and parietal. The more pronounced the syphilis of the parents, or the
nearer the date of conception to the time at which their infection
occurred, the more probable is it that several bones will be affected,
and the more unfavorable the prognosis as respects the life of the
child. Indeed, it has been noticed that "in stillborn infants and in
those dying soon after birth the majority, or even all, of the long
bones are affected."[117]
[Footnote 116: Pollnow found osteo-chondritis in 35 out of 50
syphilitic foetuses (_Der Hydrops Sanguinolentes foetus_, Berlin, 1874,
quoted by Hill and Cooper, _op. cit._, p. 352).]
[Footnote 117: Bumstead and Taylor, _op. cit._, p. 767.]
The swelling is found to consist of a ring or collar which more or less
completely surrounds the bone, is apt to be smooth rather than
irregular, and when two bones situated near to each other are
simultaneously affected may conjoin them. This condition persists
during the first stage of pathologists, and passes with greater or less
rapidity into the second stage, in which the swelling, the
cell-proliferation, reaches its height. This may take, in cases
uninfluenced by treatment, several weeks or even months. Under the use
of mercurials and iodide of potassium they usually subside rapidly.
During this second stage, however, owing to the proximity of the
swellings to the joints, a moderate amount of synovitis is often
present. This affects chiefly the elbow and the knee, but may appear in
any joint. It is also readily influenced by specific treatment and
well-regulated pressure.
{289} When the third stage is reached, or that of the formation of
granulation-tissue, with degenerative changes of the cartilages and of
the bones themselves, deformity often becomes more marked. There are
unnatural curves or angles in the bones, with more or less complete
separation at the point of junction. Where many bones are affected in
this way, the resulting deformity is extreme and the patient may be
absolutely powerless, a condition of pseudo-paralysis supervening in
which the limbs lie motionless or swing about like the arms or legs of
a doll when the child is carried.
When the swelling does not undergo absorption, the superjacent tissues
sometimes become involved, abscesses form and make their appearance
externally, extensive necrosis of the shaft of the affected bone takes
place, and the little patient usually dies of hectic, pyæmia, or
exhaustion. When the cranial bones are involved, the disease is apt to
limit itself chiefly to the stage of osteophytic formation, the
immovability of the bones probably favoring the organization of the new
cell-growth rather than the production in it of inflammatory changes.
The growths are met with chiefly in older children than those affected
with the form of osteo-chondritis just described; they affect the
periphery of the liver, and are found most usually around the anterior
fontanel, and later on the parietal and frontal eminences. The sutures
are sometimes completely soldered together.[118] The osteophytes vary
in thickness from a quarter of an inch to an inch, or are even
larger.[119]
[Footnote 118: In a case reported by Barlow it was not possible at the
autopsy to discover the point of union (_Path. Transactions_, 1879, p.
339).]