A system of practical medicine. By American authors. Vol. 2

Chapter XVIII: Front Matter (18)

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The affections of the middle ear and Eustachian tube are said to be 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}

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).]

[Footnote 119: These conditions may all result in a child the subject of acquired syphilis, but are apt to be milder, to involve fewer bones, and to yield more readily to treatment. This would of course be expected, inasmuch as the same difference in favor of the acquired form, as compared with that which is inherited, extends to all the lesions. As Diday succinctly expresses it: "In the one case the poison vitiates only the elements of nutrition; in the other it vitiates at the same time those of formation and those of nutrition." It would exceed the limits of the present article to describe acquired syphilis in children.]

The most important differential diagnosis to be made in these cases is between the rachitis of young children and the form of syphilis in question. Much difference of opinion still exists as to the relation between these diseases, syphilis being claimed, on the one hand, as having in the majority of cases a definite causative influence, while, on the other, the existence of this relation is denied. When we come to contrast the pathology of the two diseases, we can readily understand why they should be confounded, the minuter changes which occur being essentially the same--viz. cell-proliferation and accumulation, with subsequent inflammatory changes, associated with irregular deposits of lime salts.

Compare, for example, the description of the pathology of bone diseases in inherited syphilis already given (pp. 287, 288) with the following terse summary of the changes which take place in rickets in cases where no suspicion of syphilis exists, either ancestral or acquired: "The changes are more distinctly noticed at the epiphyses than in the diaphyses. Instead of the regular stages and distinct boundaries observed in the normal development of bone, there is a singular disorderly commingling of the exaggerated cartilage-proliferation and transition substance, with calcification. The cartilage-cells, stimulated to excessive multiplication, are transformed, some into bone-corpuscles, some into medullary cells, {290} and others into connective-tissue forms. The same process is in active operation in the deep periosteal layers, the material accumulating to such a degree as to add much to the thickness of the shaft."[120]

[Footnote 120: Agnew's _Surgery_, vol. i. p. 1030.]

The points of resemblance are manifest, just as they are between a syphilitic and a variolous pustule, but they end in both cases when we come to study the evolution of the phenomena either from an anatomical or from a clinical standpoint. They may be expressed as follows in tabular form:

[Footnote 121: See p. 313.]

[Footnote 122: This table is founded on one which I added to the translation of Cornil made by Dr. Simes and myself, and is compiled chiefly from the excellent work of Dr. Taylor already alluded to.]

The diagnosis of the bone lesions of hereditary exostosis can readily be recognized in a short time by noting the fact that they are stationary, {291} even if their later appearance, larger size, the absence of syphilitic history or symptoms, and the resistance to specific treatment left us in doubt.

The diagnosis from accidental separation of the epiphysis, or from fractures, may be made from the history of the case.

In cases of separation of the epiphysis, complicated with suppuration, sinuses, etc., the trouble may be mistaken for a similar condition due to non-specific inflammation. In all the recorded instances, however, the latter has occurred much later in life, is attended with much more acute inflammatory symptoms, lymphangitis, etc., and is of course without concomitant symptoms of syphilis. In both these cases there is a decided osteo-periostitis, and as so much depends on the early and vigorous use of specific treatment, it may be worth while to contrast the two forms of the disease.

[Footnote 123: Cornil, _op. cit._, p. 274.]

Syphilitic dactylitis in the inherited variety of the disease, as in the acquired, consists of two varieties. The one of these which usually appears earlier involves chiefly the periosteum and the fibrous and integumentary structures surrounding a joint, usually a metacarpo- or metatarso-phalangeal articulation, involving a phalanx, and is characterized by slow, almost painless, swelling and discoloration of the affected member. (Fig. 9.) This is due to a gummous infiltration which, after absorption under proper treatment, leaves the toe or finger temporarily stiff, but not permanently disabled.

The second form is a specific osteo-myelitis, with periostitis, coming on later, and often destroying the bone or the articulation involved. (Fig. 10.)

The absence of acute inflammatory symptoms in the first variety distinguishes it from paronychia, whitlow, and gout. Rheumatoid arthritis begins in the joints, is associated with other symptoms; deformity of the fingers comes early in the disease, and there is a teno-synovitis with contraction.

The second variety might be taken for enchondroma or exostosis, but these growths increase much more slowly, involve only a limited portion of the bone, are of greater density, and are much more strictly circumscribed.

As a rule, especially in cases which are recognized early and treated {292} actively, the prognosis is good. Iodide of potassium should be used in combination with mercury.

Syphilis of the Teeth.--Syphilis of the teeth has its chief interest to the general practitioner from its very important bearing on diagnosis. As manifesting itself at an age when the child is not apt to present the active and unmistakable cutaneous and mucous lesions of the disease, and when, consequently, its recognition is often extremely difficult, this diagnostic importance is greatly increased.

The teeth of the first dentition, although exhibiting the usual signs of interference with nutrition in their irregular development, opaque and chalky enamel deficient in quantity and unevenly distributed, soft and friable dentine, incongruity of size individually and relatively, and proneness to decay, do not often display any distinctive evidence of syphilis. {293} The same conditions may, and often do, depend on other causes, and are commonly associated with various cachexiæ--the strumous, gouty, rheumatic, rachitic, etc.--and even with other slighter ailments tending to produce imperfect assimilation and malnutrition.

In the permanent teeth, likewise, the same condition may be due to the same causes. Stomatitis, however produced--by mercury, by gastro-intestinal derangements, by local irritation of any kind--is apt to result in imperfectly organized dental structures. Mercurial teeth, for example, are usually irregularly aligned, horizontally seamed, honeycombed, craggy, malformed, of an unhealthy dirty yellow color, separated too widely, and deficient in enamel.[124] The diseases of childhood, especially the eruptive fevers, eclampsia, typhoid fever, etc., by temporarily arresting or greatly interfering with nutrition during the developmental period of the teeth, often cause horizontal furrows across their crowns, which are, of course, persistent throughout life, and mark indelibly the influence of such disorders on all the formative processes.

[Footnote 124: The latter defect is particularly noticeable on the cusps of the sixth-year molars. (See note on "Syphilis of the Teeth," by Dr. James W. White, in Am. ed. of Cornil, pp. 287-290.) The discussion as to the effect of mercury in producing the condition of the teeth known as honeycombed is still going on, but the evidence seems to point clearly to a direct connection with the administration of mercury in infancy, either for syphilis or in excessive doses as a purge, or in some of the teething powders, which often consist of calomel and opium. Mr. Hutchinson, at a meeting of the Odontological Society (see _Proceedings_ for 1877, p. 249), gave an interesting description of the way in which the supposition was arrived at. Lamellar cataract is a disease which affects the eyes of children who have suffered from convulsions: it was noticed that in cases of that form of catarrh there were also honeycombed teeth, and it was thought that the convulsions, the cataract, and the honeycombed teeth were all due to the same unknown cause. At last a few exceptions were found--patients with cataract, but with good teeth, and then some who had had convulsions only, and yet had honeycombed teeth; lastly, it was noticed that most of the patients had been treated with mercury. So it came to be recognized that the honeycombed teeth were only accidentally associated with the cataract, and that they were, in fact, the result of the mercury which had been given to cure the convulsions. The same gentleman figures a case of this disease in his _Illustrations of Clinical Surgery_ (London, 1875), and thus describes it (p. 55): "The present state of his permanent teeth is so characteristic as to deserve more detailed description. The change about to be mentioned affects all the incisors, canines, and first molars of both upper and lower jaws, the bicuspid being scarcely implicated at all; the second molars are also quite healthy. In the first molars the alterations consist of deficiency of enamel on the upper surface of the crown and the presence of spines of uncovered dentine. In the case of the incisors a considerable portion of the crown of each tooth is totally devoid of enamel, and its dentine is also deficient to some extent, so that the teeth are thin, sharp-edged, and of a dirty yellowish color. The transition from the enamel-covered to the diseased part occurs suddenly in a horizontal line at some little distance from the crown of each tooth; the position of this line or step being in each tooth nearly at the same distance from the gum. The general effect when all the teeth are seen together is as if a string had been tied around them when soft and the distal part had withered."]

None of these conditions, however, are in the least degree characteristic of syphilis, the special expression of which in the mouth is to be found only in the permanent upper median incisors. For the recognition and description of the peculiarities of these teeth in the subjects of inherited syphilis we are indebted, as we are for so much else of inestimable value in the study of the disease, to Mr. Hutchinson. In 1863, in a memoir on _Syphilitic Diseases of the Eye and Ear_, he wrote as follows[125] {294} concerning the symptoms which, in a suspected case, would aid in determining the diagnosis: "By far the most reliable amongst the objective symptoms is the state of the permanent teeth if the patient be of age to show them. Although the temporary teeth often, indeed usually, present some peculiarities in syphilitic children of which a trained observer may avail himself, yet they show nothing which is pathognomonic, and nothing which I dare describe as worthy of general reliance.[126] _The central upper incisors of the second set are the test teeth_, and the surgeon not thoroughly conversant with the various and very common forms of dental malformation will avoid much risk of error if he restricts his attention to this pair. In syphilitic patients these teeth are usually short and narrow, with a broad vertical notch in their edges and their corners rounded off. Horizontal notches or furrows are often seen, but they, as a rule, have nothing to do with syphilis. If the question be put, Are teeth of the type described pathognomonic of syphilis? I answer unreservedly that when well characterized I believe they are. I have met with many cases in which the type in question was so slightly marked that it served only to suggest suspicion, and by no means to remove doubt; but I have never seen it well characterized without having reason to believe that the inference to which it pointed was well founded."

[Footnote 125: Chapter on "The Means of Recognition of the Subjects of Hereditary Syphilis during the Tertiary Stage," p. 204. Before this, however, he had called attention to the same peculiarities in a paper on "The Means of Recognizing the Subjects of Inherited Syphilis in Adult Life," _Medical Times and Gazette_, London, Sept. 11, 1858, p. 265.]

[Footnote 126: So far as I know, the only recorded instances by reliable observers of the temporary teeth presenting the peculiar characteristics of syphilis are as follows: In the _Transactions_ of the Odontological Society of Great Britain, vol. ix., 1877, p. 258, Mr. Oakley Coles described a case--without, unfortunately, giving details--in which the "peg-shaped temporary teeth were very characteristic of syphilis." Mr. Coles's abilities are well known, and the fact that at the previous meeting the society had been addressed by Mr. Hutchinson on this very subject would seem to indicate that he was quite familiar with the importance of his statement.

M. Fournier calls attention (_Archives de Derm. et Syph._, Sept. 25, 1883) to a cast (No. 48) to be found in the collection of M. Parrot in the museum of the Foundling Hospital at Paris. This displays the dentition of a child twenty-seven months old, in which the two upper median incisors are typical Hutchinson teeth. M. Fournier adds that later researches show unmistakably that the temporary teeth may be affected by hereditary syphilis in the same manner as those of the second dentition, although not more than one case of the former is recognized to fifteen or twenty of the latter. He thinks, however, that this proportion would be greatly modified if in the autopsies of young children the alveoli were opened to examine the embryonic teeth. M. Parrot by this means claims to have often discovered lesions of the milk teeth.]

As a matter of course, so positive a statement in a matter of such gravity and importance excited considerable criticism, and the views of Mr. Hutchinson have never been without earnest and often able opponents; but it is safe to say that time has only served to place them on a surer foundation and to enhance their value in the eyes of the profession. That they have undergone no material change in the mind of their distinguished author is shown by his expressions of opinion during the debate on syphilis in the London Pathological Society in 1876,[127] and still later by the following memoranda which he gives as a guide in diagnosis:[128]

"1. No special peculiarities are to be looked for in the first set of teeth.

"2. There can be no more serious blunder than to imagine that bad teeth in proportion to their badness of form are to be suspected of syphilis.

{295} "3. The upper central incisors are the only teeth which are positively characteristic. The others may afford corroborative testimony, but are not to be relied upon alone.

"4. The chief peculiarity is a general dwarfing of the tooth, which is both too short and too narrow, and, from its sides slanting together, presents a tendency to become pointed. This tendency to pointing is always defeated by the cutting off of the end, the truncation being usually effected in a line curved upward, so as to produce a single shallow notch. At the bottom of this notch the enamel is deficient and the dentine exposed, but there is no irregular pitting, as in stomatitis teeth.

"5. The malformations are unusually symmetrical and affect pairs of teeth. The two central incisors resemble each other, and the two laterals are also alike. If any defect passes horizontally across all the incisors at the same level, and affects them all alike, it is probably not due to syphilis.

"6. In syphilis the lateral incisors usually show little or no malformation.

"7. The occurrence of the peculiarities due to syphilis and those due to mercury in the same mouth are exceedingly common."

[Footnote 127: _London Lancet_, 1876, pp. 56 and 535.]

[Footnote 128: _Illustrations of Clinical Surgery_, fasciculus xi., London, 1878.]

The great importance of the subject seems to me to justify one more quotation, as showing the opinion at a very recent date of men well qualified to judge of the correctness or inaccuracy of these statements. Mr. C. Macnamara and Dr. Thomas Barlow[129] say: "The characters of the teeth are so valuable when present that it is important to have them clearly noted--the more so that, in spite of Mr. Hutchinson's clear description, they have been much misrepresented. It may be pointed out--(1) That only the upper median permanent incisors are characteristic, and sometimes only one of them is typical, of the disease; (2) that these teeth are generally a little apart, instead of being in apposition, and are more or less dwarfed; (3) that in a typical specimen the width of the cutting edge is narrower than the width of the tooth as it emerges from the gum; (4) that a typical syphilitic tooth presents a single notch, not a serrated margin; and that occasionally, if the notch has not been actually scooped out, there is a little lunula-shaped area which may readily become a notch; (5) finally, that although such teeth, when present, are absolutely pathognomonic, the existence of normal permanent upper median incisors by no means excludes the existence of hereditary syphilis."

[Footnote 129: On behalf of the Collective Investigation Committee, who have issued a circular designed to elicit information as to the effect of syphilis on the civil population of Great Britain. This circular has been sent to physicians, and contains queries as to various points relating to the symptoms of inherited syphilis; among others as to the existence in any given case of "notched, dwarfed upper median incisors," which, with or without other symptoms, would establish the diagnosis of that case. The observations above quoted are explanatory of this question (_The British Medical Journal_, Dec. 16, 1882).]

I believe this may fairly be taken to represent the general belief at the present day among those best qualified to pronounce upon the merits of the case; and I may say that it is unqualifiedly my own opinion, arrived at after some experience and considerable investigation into the literature of the subject. It is not uncommon, however, to hear doubts expressed as to the value of this sign in the diagnosis of syphilis, and at intervals articles are written or papers read to prove that it is not of the uniform and distinctively conclusive significance that has been attributed to it.[130]

[Footnote 130: "I cannot say more in favor of the diagnostic values of these teeth than that, when {296} present in typical form, they have a certain weight in favor of the existence of hereditary syphilis in the given subject" (Van Harlingen, article "Syphilis" in _Internat. Encyc. of Surgery_, vol. ii. p. 565). "It has been the custom from time to time since Mr. Hutchinson made his observation to question the validity of his views, both as to the fact of interstitial keratitis being due to hereditary syphilis, and as to the diagnostic values of the so-called characteristic teeth. Thus, it has been asserted, not only in England, but on the Continent, and especially in Germany, that the disease may be the result of malnutrition in scrofulous and rickety subjects; and it has been maintained that the malformation of the teeth is the simple arrest of development in a perverted constitution from other causes than syphilis" (Bumstead and Taylor, _op. cit._, p. 701). Garretson says (_Oral Surgery_, p. 316): "Observations will be found to greatly vary concerning the existence of any constancy in phenomenal expressions of the teeth in this relation."

M. Magitot, who has for some years been supposed to pay especial attention to this subject, has recently written an elaborate paper (_Gazette des Hôpitaux_, Sept. 29, Oct. 4, 11, and 18, 1881) to prove (1st) that dental erosions, as he calls them, are not due to syphilis; and (2d) that they are due to, or are almost invariably associated with, infantile convulsions. He has collected a number of interesting facts, but a very careful study of his article and inspection of his diagrams have convinced me that he does not recognize at all the special peculiarities of the Hutchinson teeth, but includes under his title of "erosion" a variety of widely-differing conditions. He has altogether misinterpreted Mr. Hutchinson's views as stated in his _Memoirs on Certain Diseases of the Eye and Ear due to Inherited Syphilis_, and represents him as at that time (1863) believing that the cause of the change in the incisors was a mercurial stomatitis. The quotation on p. 294 sufficiently refutes this absurdity. Of course the paper as an argument against the syphilitic origin of these teeth is without the slightest value.]

Corroborative evidence--which, to my mind, is very strong as coming from men whose opportunities for observation of dental peculiarities are almost unlimited--is found in the writings of those gentlemen who have devoted themselves to dental and oral surgery exclusively. Mr. Henry Moon[131] says:[132] "My observations on this subject extend over some twelve years, and include some hundreds of cases; and although, in some details as to the manner of causation, I may differ from the view published by Mr. Hutchinson (before knowledge on tooth-development was advanced as it is at present), yet I must coincide entirely with his general conclusions." "The question really is this: Is there one peculiar conformation of the teeth due to inherited syphilis and not produced by any other cause? The evidence in favor of an affirmative answer to this question appears to me to be so strong that I think the onus of disproof rests with the sceptics."[133] These views were coincided in by the majority of the gentlemen to whom they were addressed, all of them dentists and surgeons of experience and repute, and who included men so well known to the profession as Mr. Oakley Coles, Mr. Samuel Cartwright, Mr. Charles Tomes, and others.

[Footnote 131: Author of the section on "Surgery of the Teeth" incorporated in Bryant's _Surgery_.]

[Footnote 132: _Proceedings of the Odontological Society of Great Britain_, vol. ix., 1877, pp. 238, 239. In the same journal for 1875, vol. vii. p. 17, Langdon Down says that whenever he has discovered syphilitic teeth he has "never failed to find confirmatory evidence of the syphilitic history of the case."]

[Footnote 133: He says elsewhere (Bryant's _Surgery_, 3d Am. ed., p. 429) that the most characteristic change in these teeth is "the lessened breadth of the cutting edge as compared with that of the neck, the vertical groove on their anterior face being often absent, and the notch on their cutting edge not being an absolutely constant feature, and being also subject to obliteration through wear."]

It may be considered as well established, then, that these peculiar teeth--stunted, abnormally narrow at the cutting edge, crescentically rounded with the convexity upward, and the surface inclined upward and forward instead of backward as in normal teeth, widely separated, but converging at their lower edges--are pathognomonic of hereditary syphilis.[134] They are {297} often described as pegged, having been likened to a row of pegs stuck in the gums. This appearance is due to the fact that they are shortened, often projecting not more than half the normal distance from the gum, and are also widely separated; which abnormalities often affect the adjoining teeth as well, and sometimes the entire dentine. It has been asserted that other specific peculiarities are to be found associated with those of the incisors, and Mr. Moon describes as characteristic, and figures[135] small dome-shaped first molars with suppressed angles and absence of enamel from the masticatory surfaces. He believes also that when the upper incisors are typical it is exceedingly rare for the lower incisors to be altogether unaffected.

[Footnote 134: Mr. Hutchinson stated in 1877 that in spite of the fact that many years previously he had challenged any one to bring forward a patient with well-marked syphilitic teeth in whose history no evidence of syphilis could be found, none had come.]

[Footnote 135: _Proc. of Odont. Soc. of Great Brit._, vol. ix. pp. 241, 242; Bryant's _Surgery_, Am. ed., p. 429.]

A mistake which I know, from observation, to be frequently made is the confusing of the normal serrations of the cutting edges of recently-erupted normal incisors with the peculiar crescentic edges of the syphilitic teeth. It seems worth while to call especial attention to this, on account of the unpleasant consequences which often follow injudicious questioning based on such supposed syphilitic phenomena. Indeed, the space which has been devoted to this subject of syphilis of the teeth is well warranted, I am sure, by the fact that not only do diagnosis, prognosis, and treatment in cases of great severity, and in both children and parents, often depend upon a recognition of these peculiarities, but that in addition to the clinical and therapeutic problems there are others the solution of which is dependent upon the same knowledge on the part of the practitioner, and which may involve reputation, marital relations, and personal honor and happiness.

The approximate cause of these peculiarities in the incisors can hardly be said to have been demonstrated. Mr. Hutchinson thought at one time[136] that they were due to a stomatitis or an alveolar periostitis, but he has since changed his mind as to that point, believing now[137] that the syphilitic tooth is the result of an arrest of development in the central or first-formed portion of the dentine. The incisors being made up of these lobes or denticles, and dwarfing of the middle one taking place, the two lateral ones fall together. This accounts at once for the small size of the tooth, its shape of an inverted truncated cone, and its crescentic edge.[138] If it were due to stomatitis, it would be more likely to be equally {298} distributed, syphilis in its late manifestations being notably unsymmetrical; there would be no rational explanation of the involvement of one or two teeth while those on either side so frequently escaped; if it were mercurial stomatitis, the enamel too would be involved, as is not usually the case in the syphilitic teeth. It is possible that the central incisors are chiefly affected because they, with the first molars--also affected according to Mr. Moon--and the lower incisors--not infrequently involved--are the first-formed teeth.

[Footnote 136: "The physiognomonical, dental, and other peculiarities by which we recognize the subject of inherited taint when advanced beyond the period of infancy are all of them the direct consequences of special inflammations from which the patient has suffered at former periods; _e.g._ the synechiæ and lustreless iris of iritis; the malformed teeth of periostitis of the alveolus and dental sacs; the protuberant forehead of hydrocephalus; the flattened nose of snuffles; the pale, earthy, opaque skin of cutaneous inflammation and eruption" (_Aphorisms respecting Constitutional Syphilis_, 1863).]

[Footnote 137: _Proc. of Odont. Soc. of Great Britain_, vol. ix., p. 248. See also _ibid._, pp. 241, 242, remarks of Mr. Moon; also _Monthly Review of Dental Surgery_, June 15, 1877.]

[Footnote 138: The denticle theory of formation is not necessarily opposed by the fact that there is only a single undivided pulp-cavity in these incisors. Instances of the separate formation of processes of dentinal pulp while others are being used and worn away, all of them finally to unite in a common pulp-chamber, have been observed in lower animals, as in the molar of the elephant.]

The most elaborate article upon syphilitic teeth which has appeared since Mr. Hutchinson's original memoir is one by Fournier,[139] in which, after a very broad and comprehensive consideration of the subject, he arrives at the following conclusions: The hereditary influence of syphilis shows itself in the dental system in two ways, very unequal in point of diagnostic value--viz. first, by a retardation of evolution; second, by the arrest of growth and modifications of structure. The phenomena belonging to the second class may be grouped as follows: First, _dental erosion_. This is due to imperfect formation of the tooth, the result of a temporary stoppage in its development; but as it produces an appearance like that of worm-eaten wood, it has been called erosion, though in so far as the word conveys the idea of the wearing of a surface which has been previously normal, it is incorrect. The tooth affected with syphilitic erosion has never been normal. The different forms of erosion can be subdivided into groups according as they affect the face or the free edge or grinding surface of the tooth. Of those involving the face there are four types: Erosions _en cupule_, consisting of small excavations or cups in the surface of the crown; erosions _en facettes_, in which the surface presents a series of small planes, as though they had been filed; erosions _en sillon_ when there is a linear excavation in the crown of the tooth in the shape of a transverse groove; and erosions _en nappe_, in which the whole surface is discolored, disorganized, and honeycombed.

[Footnote 139: _Archives de Derm. et Syph._, Sept. 25, Oct. 2, Oct. 9, 1883. A translation made by the writer may be found in the _Dental Cosmos_ for January and February, 1884.]

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A system of practical medicine. By American authors. Vol. 2Chapter XVIII: Front Matter (18)

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