Part 44

THE DIAGNOSIS AND PROGNOSIS OF INHERITED SYPHILIS.--In reviewing the general course of a case of inherited syphilis it seems evident that the differences between it and the acquired disease which have been so much dwelt upon are apparent rather than real. The primary stage is of course missing, and on any theory of the essential nature of syphilis this is readily comprehensible. Whether the chancre is the first symptom of a constitutional disease, or, as I believe to be the case, is the simple accumulation at the point of original inoculation of the cells which constitute the syphilitic virus--or are at any rate its carriers--it would naturally be in the first case undiscoverable, in the second nonexistent.

"It is not true that the diversity of symptoms presented by infants authorizes us to admit a congenital and an hereditary syphilis. Whatever the mode of infection, it is impossible to make this distinction" (Ricord, note to John Hunter's Works, 1883).]

The secondary stage, characterized in the acquired form chiefly by {310} lymphatic engorgement and symmetrical, widely-spread, polymorphic cutaneous and mucous eruptions, and pathologically by a marked tendency to the proliferation of certain new small round nucleated cells, upon the presence of which depend all the manifestations of the disease, is in inherited syphilis strictly analogous. Eruptions of the same character make their appearance, differing only in minor points, as in a greater tendency to become moist or ulcerated, due to the more delicate texture of the infantile epidermis. To the same cause must be assigned the macroscopic peculiarities of the only syphiloderm said to be peculiar to infantile syphilis--pemphigus--which has been shown, however, to have a papular basis, and in that way to conform to all the other secondary eruptions.

The lymphatic engorgement either exists in the infant as in the adult or has its analogue in the enlargement of the spleen and liver--especially the former, which is almost as constant a phenomenon as is general glandular enlargement in acquired syphilis. The same pathological changes occur, the same infiltration of cells producing, according to their situation, papular, pustular, or mucous patches, or inflammation of such structures as the iris, choroid, or retina.

The tertiary stage, except in the fact that its phenomena may appear unusually early and may be commingled with those of the secondary period, does not widely differ in the hereditary from that of the acquired disease. It affects the same tissues, results in the same pathological formations, and is preceded by the same period of latency or quiescence of variable duration. There is no reliable evidence with which I am familiar to show that in this stage inherited syphilis is either contagious or transmissible--another point of close resemblance between the two varieties under consideration.

In considering the question of diagnosis, therefore, we have an excellent guide in the fact that the disease conforms in most respects to the general laws of acquired syphilis, and that our knowledge of the latter affection will be a valuable aid to recognition of the former.

The chief elements of diagnosis and prognosis of inherited syphilis in its various stages may then be summarized as follows:

A history of syphilis in either parent is important just in proportion to the shortness of the interval between the time of infection and the date of conception. In other words, the shorter that interval the more likely (a) that the child will have syphilis, (b) that it will have it in a severe or fatal form. If the mother has been syphilitic and the father healthy--which is rare--it is perhaps more likely that the child will be diseased than when the reverse is the case. If both parents were syphilitic at or before the time of conception, the probability that the disease will be transmitted, and in a severe form, is much increased. There is no evidence to show that inheritance from one parent results in a graver variety of the disease than when it is derived from the other.

A history of abortion or miscarriage on the part of the mother should have weight in the determination of any given case, and if such accidents {311} have been very frequent their diagnostic importance is greatly increased. The loss of elder brothers or sisters and the causes of death, with the precedent symptoms, should be carefully inquired into. The nearer either of these occurrences--abortion or death of elder children, if there is a fair presumption that they were due to syphilis--has been to the birth of the patient in question, the greater the likelihood that the latter has been infected.

Upon examining the product of abortion or stillbirth the most easily observable symptoms will be those of the skin. Maceration and elevation of the epidermis into bullæ are in themselves hardly characteristic, though they may--especially the latter--be regarded as suspicious. If the cutaneous lesions are, however, distinctly papular or pustular or ulcerative, or if the bullæ have all the characteristics of syphilitic pemphigus, the diagnosis is assured.

"It is impossible to demonstrate the existence of syphilitic lesions in foetuses expelled during the first months of pregnancy. Later, the signs which have the greatest value are the lesions of the epiphyses of the long bones. When the foetus has nearly arrived at full term, and is not macerated, visceral and cutaneous lesions may be observed. According to Mewis, the skin eruptions cannot be seen before the eighth month, and are only recognizable on foetuses whose death has been very recent or who are born living. Pulmonary lesions may be determined at the end of the sixth month. Those of the pancreas are met with in about half the foetuses which perish a little before or a little after birth. The lesions of the liver, the spleen, and the bones may be recognized even in macerated foetuses, this frequency increasing from month to month" (Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 864).]

The most distinctive symptom--one which may really be considered as pathognomonic, is, however, the inflammation of the diaphyso-epiphysial articulations, with or without their disjunction. Distinct enlargement of the spleen or liver, and arachnitis with hydrocephalus, are valuable diagnostic points, and the presence of gummata--not very infrequent--would of course be conclusive.

At birth the syphilitic child may be small, stunted, emaciated, weazened, senile in appearance; this would properly give rise to suspicion, but may be associated with any disorder of nutrition on the part of child or mother. It may also disclose cutaneous or mucous eruptions evidently specific in character. The most common of these at this early date is the bullous eruption affecting the palms and soles, sometimes distributed over the whole body, and, as it indicates a feeble resistance of the tissues to the tendency to exudation and cell-growth, is usually a precursor of an early and fatal termination. In any event, marked symptoms at time of birth render the prognosis highly unfavorable.

It is quite as common, however--perhaps more so--for the subject of hereditary syphilis to give no evidence of the disease at birth, but even to appear healthy and well-nourished. In such cases the first symptoms of the disease appear, on an average, in from six weeks to two or three months, and consist principally of coryza (snuffles), hoarseness of voice, and syphilodermata. The latter may be macular, papular, pustular, or bullous. They are usually polymorphous, irregular in shape, dark coppery-red in color, with sometimes a glazed or crusted, but oftener a moist or ulcerating, surface, with a strong tendency to coalesce into large patches, or to form irregular serpiginous ulcers, or to take on hypertrophic growth {312} and develop into condylomata. Eruptions which are squamous and are situated about the mouth and chin and on the body, the legs, or the soles of the feet, though exceptional, are of more value than those on the nates, where the results of irritation from urine and feces may closely simulate syphilodermata.

Mucous patches on the tongue, cheeks, tonsils, and pharynx are common, often extending to the larynx, increasing the hoarseness, and to the nasal cavities, aggravating the snuffles. Both of these occurrences, by interfering with the respiration of the child and rendering its nursing interrupted and insufficient, greatly add to the gravity of the case. Enlargement of the spleen (common), enlargement of the liver (less so), and iritis (rare), may be mentioned among the phenomena of this stage, often associated with the skin eruptions.

About the time of the subsidence of the rash there may be developed the specific inflammation at the junction of epiphyses and diaphyses which produces a swelling of the long bones near their ends. The child will be noticed to cry a little when, for example, the wrist or elbow on one side is washed, and not to use these joints as much as the corresponding ones on the other side. The parts are not hot, only slightly tender, and as yet there is but little swelling. Later, the droop and the disuse of the affected limb become more noticeable and simulate infantile paralysis. There is, however, no wasting, no alteration of reaction by faradism, no real loss of power, so that the term pseudo-paralysis is an appropriate one. In a week or two similar symptoms will occur in the bone on the opposite side, and finally the ends of all the long bones may be affected; ordinarily the elbows, wrists, knees, and shoulders are the joints involved. Suppuration is rare, disjunction of the epiphysis from the diaphysis common. Recovery is apt to take place spontaneously within a month. The associated changes are chiefly endosteal at the junction of the shaft with the epiphysis, but there is also a little periostitis or perichondritis, which is the principal cause of the external swelling. Moderate deformity may ensue.

Similar changes occurring in the cranial bones give rise to what has been called the natiform skull. During the first year it is very common for syphilitic children to develop a number of lenticular swellings on the cranium, which appear symmetrically around the anterior fontanel, but at a little distance from it; i.e. one on each frontal and one on each parietal bone. These are said to be "bossed." They are at first circumscribed, and in a child nine or ten months old often measure three-quarters of an inch to an inch in diameter. They are at first circular, afterward more irregular, and finally tend to organize, becoming diffused and massive and causing a permanent thickening of the skull.

These symptoms which have been described are the prominent ones occurring during the first six or eight or twelve months of life. If they do not manifest themselves before the eighth month, it is highly probable, even in a case with a syphilitic parental history, that the child will either escape altogether or that the secondary stage has been very slight and altogether intra-uterine and unattended with noticeable phenomena. If during this first year the child's cachexia is marked, if there are any intercurrent diseases, if the symptoms show themselves early, if the nasal or laryngeal affection is severe, if the eruptions are markedly bullar or {313} pustular or ulcerative, if the enlargement of the spleen is great or the osseous lesions precocious or grave, and if, especially, there is any intermingling of tertiary symptoms, gummata, nodes, etc.,--the prognosis will be unfavorable.

From adolescence on through adult life the diagnosis of inherited syphilis will depend on the following points: First, of course, the history of parental or of infantile syphilis, or of both. Then a group of physical and physiognomical peculiarities, which are not definitely characteristic, and are of little value when taken separately, but are of considerable importance when all or a majority are present in any given case. These are low stature or puny development proportionate to the severity of the intra-uterine and infantile symptoms; a pasty, leaden, or earthy complexion, a relic of previous syphilodermata, probably also a result of malnutrition; a prominent forehead, bulging in the middle line at and within the frontal eminence, and due either to thickening of the skull or to a previous arachnitis and hydrocephalus before the ossification of the fontanels; a flat, sunken bridge to the nose, due to the coryza of infancy extending to the periosteum of the delicate nasal bones, and either interfering with their nutrition or partially destroying them; dryness and thinness of the hair, with brittleness and splitting of the nails; synechiæ and dulness of the iris (rare); ulcerations of the hard palate; and periosteal thickenings or enlargements of the shafts of the long bones near the ends, or slight angular deformity, results of the osteo-chondritis of infancy.

A much more valuable group of symptoms, however, are the following, which are mentioned in the order of their importance, any one of the first three being almost or quite conclusive:

Dwarfed permanent median upper incisors, broader at the top than at the cutting edge, which is crescentically notched, separated by an undue interval and converging toward each other.

Evidence of past or present interstitial keratitis--a dusky and thin sclerotic in the ciliary region and slight clouds here and there in the corneal substance, there being no scars on its surface--or of disseminated choroiditis; patches of absorption especially around the periphery.

{314} A radiating series of narrow cicatricial scars extending right across the mucous membrane of the lips, or a network of linear cicatrices on the upper lip and around the nostrils, as well as at the corners of the mouth and on the lower lip.

Periosteal nodes on one or many of the long bones; sudden, symmetrical, and complete deafness, without otorrhoea and unattended by pain or other subjective symptoms.

Late or tardy hereditary syphilis is rarely dangerous to life. The prognosis is almost unvaryingly favorable unless some grave visceral complication, such as interstitial pneumonia, gummata of the brain, liver, or kidney, or meningeal and periosteal inflammation within the cranium, should occur.

TREATMENT.--The prophylactic treatment, or that directed to the health and sexual relations of the parents previous to conception, has already been sufficiently considered. That of the mother during pregnancy, after having conceived from a syphilitic husband, or having had antecedent syphilis, or having contracted it by direct contagion subsequent to impregnation, is simply that of acquired syphilis in either adult or child. Mercury in its full physiological dose is the drug indicated. It may not be amiss to combine with it iodide of potassium in moderate doses, but the practice of employing the latter to the exclusion of the former is both theoretically and clinically unsound. Care should especially be taken to give it in such a manner, either by inunction or vaporization or so guarded with opium, that it will not produce any irritating effect on the intestinal canal, the sympathy between which and the uterus may, in the event of a strong purgative action being set up, lead to an abortion.

As we have seen that the pathology, the stages, and the general course of hereditary syphilis are all closely related to or identical with the same phenomena in the acquired disease, and so know that they both depend upon the same ultimate cause, whatever that may be--a virus, a fungus, or a degraded cell--it follows that the same principles should govern us in the treatment of the one as in that of the other.

We know from clinical experience that mercury exercises an almost {315} controlling influence over the secondary manifestations of acquired syphilis, whether by acting as a true antidote or as a tonic, or by virtue of its property of hastening destructive metamorphosis and thereby facilitating the absorption or elimination of new cell-growths. We know also that iodide of potassium, probably by virtue of its powerful stimulating influence on the lymphatic system, has an equal power over the tertiary growths, which by their pressure upon or situation in important tissues or organs may be so destructive. There is no reason, therefore, by analogy why these drugs should not, comparatively speaking, be equally beneficial in hereditary syphilis; and such is, indeed, found to be the case. In the latter affection, however, there are two elements which should modify the treatment somewhat, and must be taken into consideration. These are--1st. The existence of a more or less profound cachexia influencing all the nutritive and formative processes, and in itself, entirely apart from any definite specific involvement of vital organs, threatening life. 2d. The not infrequent occurrence during the secondary period of symptoms--notably gummata--belonging to the tertiary stage.