Part 43

Syphilis of the Spleen.--Disease of this organ in inherited syphilis is especially important from two points of view. It is a valuable aid to diagnosis, and by its size and the degree of persistence of the swelling gives an approximate indication of the severity of the case.

Attention was first called to the frequency and importance of enlargement of the spleen in early hereditary syphilis by Gee in a paper read before the Royal Medical and Chirurgical Society in 1867. He gave the histories of thirteen children in support of the statement that such enlargement occurred in almost one-fourth of all cases of hereditary syphilis, sometimes with, sometimes without, enlargement of the liver and lymphatic glands. According to him, the degree of splenic enlargement may be taken as a sort of index of the severity of the cachexia; the majority of cases with great enlargement die, but sometimes such children survive, the spleen gradually diminishing in size as the health improves--not diminishing, however, pari passu with such improvement, but remaining for a long time "a monument of past cachexia."

Barlow, ten years later, thought Gee had rather understated the proportion of cases in which splenic enlargement occurs, he having found it in 22 out of 28 children with definite hereditary syphilis. Birch-Hirschfeld, Eisenschitz, and Tepel corroborate these observations, finding that the enlargement is almost invariable and that the spleen is often double its normal size.

Mr. W. J. Tyson has reported a cure of a child born of syphilitic parents, in whom, at two years of age, the spleen extended downward three and a half inches, reaching the crest of the ilium and approaching closely to the umbilicus. The liver was not enlarged; the urine was not albuminous. He ordered mercury with chalk, one grain every morning and evening, and one grain of iodide of potassium, with ten minims of syrup of iodide of iron to an ounce of water, three times a day. Sixteen months later the spleen had become imperceptible, and three years afterward the child was in excellent health.

The diminution of the liver under treatment appears to take place before there is any diminution in the size of the spleen. This persistence of the latter renders it, as has been stated, a valuable diagnostic sign. In the paper already quoted from, Macnamara and Barlow allude to this as follows: Enlargement of the liver, although it ought to be noted because it is often present in hereditary syphilis, has but little value as a confirmatory symptom--first, because the liver is proportionally large in infancy, and it is difficult to state the limit of what is actually normal; and, secondly, because other causes besides congenital syphilis lead to its enlargement.

With regard to enlargement of the spleen the case is different. Gee's observation, that in the early stage of infantile syphilis some enlargement of the spleen occurs in a large number of cases, has been abundantly {306} confirmed. Although with the subsidence of the other symptoms this enlargement often disappears, so that on post-mortem examination two or three months after there may be no trace of it, yet in a few cases it persists, and indeed sometimes increases, so as to be considerable when the other signs have quite vanished. The importance of this sign is greatest when noted early; as, for example, when the child is from two to three months old, for at that period the enlargement of the spleen due to rickets can hardly come into question.

The condition of the spleen during this period of enlargement seems to be simply that of hyperæmia, or at the most of hyperplasia, Gee's, Barlow's, and Birch-Hirschfeld's autopsies showing no evidence of new growth or of amyloid or other changes.

The cause seems to me to be in all probability the well-known effect of syphilis on the glandular system in general, and the lymphatic system in particular, to which I believe the spleen is now generally assigned. The analogy between this slow, persistent, painless enlargement preceding the cutaneous symptoms, unaccompanied by inflammatory symptoms, unattended by any breaking down of tissue, subsiding slowly but evenly under specific treatment, and the behavior of the buboes of acquired syphilis, is certainly very striking.

In most cases of hereditary syphilis there are evidences of disturbance of the gastro-intestinal tract. Vomiting, diarrhoea, colic, anorexia, and emaciation are well-known, but of course not at all characteristic, symptoms.

It has been supposed that the mucous membrane of the entire tract was probably, during the early period at least, and coincidently with the cutaneous eruption, in a condition of hyperæmia and irritation comparable to that of the skin. Whether this be so, or whether it is due to associated involvement of the glandular apparatus, has not yet been determined.

Förster has found fibroid degeneration of Peyer's patches in a syphilitic infant who died six days after birth, the glandular structure having been replaced by elevated grayish-red masses of nuclei, cells, and connective-tissue fibre. Ulcers of the intestines have been described, but appear to have had no specific characters.

The pancreas has been most extensively studied by Birch-Hirschfeld, who examined seventy-three syphilitic foetuses. In thirteen of them he {307} found enlargement of the pancreas with increased weight and density, proliferation of connective tissue, and in some cases compression--almost obliteration--of the lobules, with atrophy and fatty degeneration of their epithelium.

The peritoneum has been described by Simpson, Von Baerensprung, and others as occasionally invaded in early hereditary syphilis. There seems to be no evidence that it is ever directly affected, the cases in which death occurs from peritonitis being due usually to trouble connected with the liver or spleen.

Syphilis of the lung, originally described by Depaul and Virchow, has been carefully studied also by Förster, Robin, Lorain, and Cornil, from whose description of the pathology of the condition I condense the following: In the syphilitic foetus born before term, in the syphilitic child born dead at full term, and in the syphilitic children who live a few days, there are found at the autopsy, in the lungs, nodules or tumors, usually superficial, sometimes deep, hard, isolated or in groups, pink, gray, or red in color, with scattered whitish or yellowish points. Their size varies from a pea to a small walnut. They represent a portion of the lung more or less considerable in a state of special lobular hepatization. Sometimes a whole lobe is involved. The affected portion is very dense and covered with thickened inflamed pleura. The lesions are those of chronic pneumonia. The interlobular connective tissue enters into proliferation and presents a large quantity of embryonal cells; the alveolar walls are thick, while the narrow alveoli are livid, and even filled by epithelial cells, which are of the pavement form in contact with the walls, round in the centre of the alveoli. As the process progresses the epithelial cells become fatty, degenerated, and subsequently broken down and absorbed, while the embryonal interalveolar tissue rapidly organizes into fibrous tissue. Thus results a small fibrous tumor, in which a gumma may ultimately develop.

It is impossible to confound this syphilitic pneumonia with tuberculosis. The granulations of tubercle are never congenital.

The fact that in syphilitic interstitial hyperplasia the change begins in the interlobular connective tissue and around the interlobular vessels, at first consisting of small spindle-shaped and roundish cells which quickly develop into connective tissue, and the fact that blood-vessels are freely produced among the fibres of this new tissue, seemed, in the estimation of those pathologists who took part in the discussion on visceral syphilis in 1877, to constitute its most distinctive feature.

Gummata in the lungs of children suffering from inherited syphilis have been described by a number of writers. They appear likewise to begin in the walls of the blood-vessels or the bronchioles. They differ from tubercular nodules in being few in number--not more than half a dozen usually--and are generally confined to one lung.

The condition of the arteries in the few cases in which they have been noticed as affected by inherited syphilis was precisely similar to that found by Heubner in the arteries of adults.

{308} The symptoms are very variable; new-born children often die asphyxiated in a few days. If they live longer, the disease develops into a more generalized broncho-pneumonia.

Syphilis of the Larynx.--The hoarse cry of the new-born infant so characteristic of hereditary syphilis depends upon the presence of hyperæmia, of mucous patches, or even of extensive ulceration. I am inclined to think that the first is the more common, as if it were otherwise cases of death from oedema glottidis or other forms of laryngeal obstruction would be oftener met with. When ulceration does exist it is generally, but not invariably, secondary to pharyngeal ulcers.

Bronchial catarrh, giving rise to cough, and sometimes to considerable embarrassment of respiration, is a not infrequent complication of laryngeal syphilis.

Later troubles of the larynx in connection with inherited syphilis have not yet been carefully enough studied to warrant us in drawing any distinction between them and the usual symptoms seen in the acquired disease.

Syphilis of the testicles has been studied by Henoch, Cornil, Parrot, Hutinel, North, Bryant, and others. It is found to consist of a true interstitial orchitis, very closely resembling that seen in the syphilitic testicles of adults. Hutinel's investigations, based on ten cases, showed the testicles slightly enlarged and harder than normal, the scrotum pendent, the epididymis normal, the tension of the tunica vaginalis and tunica albuginea slight. The basis of the lesion is in a collection of small round embryonal cells resembling lymph-cells, arranged in the connective tissue around the arterioles which come from the tunica albuginea. This may be accompanied by a more or less marked diffused interstitial orchitis, or there may be only a thickening from the new formation of small round cells on the connective tissues of the testicles. Cornil found the seminal ducts separated by numerous round or fusiform cells. The disease usually occurs at from two months to three years of age; both testicles are generally involved, and are enlarged, hard, inelastic, and frequently nodulated. Mercurial treatment generally causes a marked improvement unless the inflammation has already resulted in the development of a new fibroid formation, in which case it would be likely to remain unaffected by any form of treatment. Inunctions with diluted mercurial ointment, iodoform, etc. are useful adjuvants.

The kidneys are not infrequently involved in inherited syphilis. Parrot reports the pathological change to consist of a proliferation of small round cells in the intertubular connective tissue, followed by contraction, obliteration of the tubules, and degeneration of their epithelium.

Bradley has reported the case of a child aged four months in whom a well-marked syphilitic eruption and an attack of acute Bright's disease were coincident. Mercurial treatment for three weeks cured both.

Coupland has reported two cases of parenchymatous nephritis associated with inherited syphilis, but advances no proof that it was not an accident. {309} Gummata have been from time to time found in the kidneys of very young children who have died from their effects and from other visceral lesions due to syphilis. Cases of enlargement, of fibroid, fatty, and gelatinous degenerations of the suprarenal capsules, have been recorded.

The thymus gland is occasionally found in syphilis to have undergone alterations claimed by Dubois, Depaul, and others to be syphilitic in their nature, but ascribed by Parrot simply to degenerative changes due to malnutrition. The gland does not appear to undergo any marked alteration in size, color, or consistency, but is found after death to contain a small quantity of purulent matter.

The tendency of syphilis is certainly not, as a rule, to the formation of pus. Nearly all the lesions we have studied with the exception of breaking-down gummata have consisted in various forms of cell-proliferation or accumulation, and not in the formation of abscesses, and it is not probable that this is an exception. I doubt very much the syphilitic character of these changes.