A Contribution to the Study 
of Congenital Syphilis. 
BY 
JOHN N. MACKENZIE, M. D., 
BALTIMORE 
REPRINTED FROM 
2Thr "Nrto STorft .journal 
for May 3h 1884. Reprinted from the New York Medical Journal for May 31, 1881f. 
A CONTRIBUTION TO 
THE STUDY OF CONGENITAL SYPHILIS. 
JOHN N. MACKENZIE, M. D., Baltimore. 
I HAVE selected the case, whose essential features are given below, 
from my note-book, not only on account of its peculiar interest, 
but also to serve as'the text for some remarks on the manifestations 
of congenital syphilis in the throat, and their behavior under the in- 
fluence of acute disease. 
Agnes 0., aged eight, the child of syphilitic parents, was brought to 
the London Hospital for Diseases of the Throat and Chest in the latter 
part of September, 1879. Her history was as follows: Healthy at birth. 
When five weeks old, lost power in the right upper extremity. This 
lasted for five weeks, during which time she had no control over the limb. 
W'hen eight weeks old, contracted a diarrhoea which lasted uninterrupt- 
edly for nine months. With the diarrhoea, round, irregular, elevated 
patches of a red color appeared on the back and buttocks, which were 
associated later on with an eruption around the anus and genitals. These 
lesions disappeared some months afterward with an attack of measles. 
When an infant, would cry out in the night, clinch her thumbs, and bur- 
row her head in the pillow. 
From her first to her third year, an interval of perfect health. At 
the age of three, inflammation, with a cloudy condition of the right eye, 
appeared, followed in two weeks by implication of the left. The eyes 
remained inflamed for two years. (The patient’s letter from the Royal 
Ophthalmic Hospital states the condition to have been keratitis.) At the 
expiration of this time both upper eyelids became completely paralyzed, 
so that she had no power over them whatever. She could lift them 
up with her fingers, and frequently did so; but the muscular control of 
the lids was gone. There was no inflammation of the eyes beyond some 
remains of tbe keratitis at the time, and their exposure to light gave rise 
to no irritation. The mother noticed nothing wrong about the eyeballs 
during this period. With the ptosis the child complained of intense occi- 
pital cephalalgia; convulsive movements occurred during sleep, during 
which she would contract her brows and rub her head with her hands, 
* Read before the American Laryngological Association, May 12, 1884. 2 
A CONTRIBUTION TO THE 
leading the mother to suspect trouble with the brain. These symptoms 
lasted for over a month, at the end of which time, together with the para- 
lytic condition of the lids, they completely disappeared. (During this 
period she was under intelligent medical supervision, hut 1 am unable 
to discover what treatment was adopted.) 
When they had passed away, the mother noticed for the first time that 
the right eyeball was turned inward toward the nose, and that she had 
become deaf in the right ear, from which there issued a slight but con- 
stant discharge. Shortly afterward a little white spot appeared on the 
left tonsil, which slowly ulcerated, the ulceration involving the whole 
of the back portion of the throat. At the same time she complained of 
great difficulty in deglutition and pain in the bones, the cervical glands 
became enlarged, she spoke in a nasal tone of voice, and had a profuse 
discharge from the nose. During the period of sore throat there was 
suppuration of the right lachrymal sac, which opened, and continued to 
discharge for eighteen months. The left sac then became inflamed, sup- 
purated, and ruptured spontaneously, and the discharge has been gradu- 
ally diminishing in quantity since. The ulceration of the throat healed 
slowly; would constantly break out afresh ; in fact, it never completely 
cicatrized. 
In August, 1879, the child became suddenly aphonic from exposure 
to cold, and, in the latter part of September, was brought to the hos- 
pital suffering from dysphagia and dyspnoea of several weeks’ duration. 
Condition on application at the Clinic.—Large head, prominent fron- 
tal eminences; cloudy corneae, opacity of the right lens; right internal 
strabismus; marked flattening of the nasal bones and prominence of the 
lower jaw; the remains of a dacrocystitis of the leftside. The pegged 
condition of the teeth is absent. Muco-purulent catarrh of nose and 
middle ear. On looking into the mouth, the whole of the hard palate is 
seen to be in a state of active ulceration, and there is necrosis with per- 
foration of its central portion. The palatal destruction extends over the 
anterior arch of the alveolar ridge, involving the bone to a limited extent. 
The soft palate, uvula, and anterior faucial pillars are completely de- 
stroyed, and the posterior wall of the pharynx is the seat of a large, 
angry-looking ulcer which extends into the naso-pharynx as far upward 
as the view extends. The patient has to keep her handkerchief con- 
stantly to her mouth to absorb the discharge, which accumulates with 
great rapidity. 
Laryngoscopic examination is not possible; but there is absolute 
aphonia, with dyspnoea, and the glands at the cornua of the hyoid bone 
are hard and swollen. 
Liquids and solids, which are not finely divided, return through the 
nose, and the little patient complains of cough, pain in the head, and 
night sweats. 
The ulceration continued to advance, in spite of tonic and anti-syph- 
ilitic treatment, until the 18th of October, when the patient was seized STUDY OF CONGENITAL SYPHILIS. 
3 
witli scarlet fever of a mild type. With the appearance of the exanthem 
the ulceration in the throat began at once to heal, and, when the stage 
of desquamation was reached, cicatrization was complete, the dyspnoea 
and dysphagia mitigated, and the voice restored. 
Shortly after convalescence the ulceration became again active, and 
in the early part of the following December a severe, acute pneumonia 
supervened. The pulmonary inflammation, with which she was confined 
to the house for four weeks, seemed to exercise no appreciable effect 
upon the palato-pharyngeal ulceration, which only cicatrized some weeks 
after resolution had taken place. 
The throat then presented the following appearances: The hard palate 
was covered with cicatrices, and there was a small point of incipient 
ulceration at its posterior portion. The palate was continuous behind 
with a dense mass of cicatricial tissue which completely filled the fauces 
and pharynx, and in which all trace of the original anatomy of these parts 
was lost. In its center a small, oval depression existed, which communi- 
cated with the parts below by an aperture, through which the tip of the 
little finger could not be passed. From this central sulcus, which repre- 
sented the remains of the pharyngeal cavity, a small contracted passage 
led into the naso-pharynx which would just admit an ordinary probe. 
The child breathed entirely through her mouth, upon closing which, the 
respiration had a loud, roaring sound. By forcible expiration she could 
drive a faint column of air through the nose. 
Before passing to the consideration of the subject to which I 
desire to call special attention, there are two features in the above 
case that deserve particular notice. 
While deep ulceration of the pharynx (I refer to the lower pha- 
rynx) is not rare in congenital syphilis, such a degree of resulting 
stenosis is excessively uncommon. I know of but two similar cases. 
In the one,* a mere slit formed the only communication with the 
structures beneath, and in the other f the pharynx was so constricted 
as to scarcely admit the point of the little finger. 
Of great interest, too, is the paralysis of both upper eyelids. The 
history of the case would point to a cerebral lesion as its probable 
cause, possibly a gummatous deposit in the course of the oculo- 
motorius nerve. The subsequent paralysis of the abducent and the 
symptoms referable to the organ of hearing on the same side may 
also be susceptible of a similar explanation. Any reasonable doubt 
as to the syphilitic nature of the paralysis would be dispelled by the 
easy exclusion of other causes and the curative effect of (presumedly 
anti-syphilitic) treatment. The case, then, adds one to the short list 
* Catti, “Wiener med. Presse,” No. 18, May 2, 1875. 
f Levis, “Med. and Surg. Reporter,” Philadelphia, May 3, 1879, p. 381. 4 
A CONTRIBUTION TO THE 
of affections of the cranial nerves in hereditary syphilis observed by 
Graefe, * Barlow,j- Dowse, J and Galezowski.* 
In the “American Journal of the Medical Sciences” for October,! 
1880, I called attention to the frequency with which the throat is 
involved in congenital syphilis, and gave a systematic description of 
the lesions which are found in the pharyngo-bronchial tract and 
oesophagus during the course of that disease. In opposition to the 
then generally received doctrine, I ventured to maintain, as the result 
of careful investigation of the subject, that, so far from being rare, 
as was generally supposed, laryngeal affections in congenital syphilis 
are among the most common and characteristic of its pathological 
phenomena, and that the invasion of the larynx may be looked for 
with the same confidence in the congenital as in the acquired form 
of the disease. Further experience and the study of cases recorded 
by others have only served to strengthen the positions taken in the 
paper referred to and the views there expressed, for which I must 
refer to the original. 
Among other things, the following conclusions were reached in 
reference to deep, destructive ulceration of the oro-pharyngeal cavi- 
ties: 1. That deep ulceration may invade the palate, pharynx, and 
naso-pharynx at any period of life from the first week up to the age 
of puberty. Thus, of thirty cases analyzed with reference to the 
period of invasion, fourteen occurred within the first year, and ten 
within the first six months of life, the remainder occurring at periods 
more or less advanced toward puberty. 2. When the eruption of 
inherited syphilis is apparently delayed until the latter period, that 
lesions of the palate and pharynx are found with a peculiar constancy, 
and often first attract attention to the existence of a diathesis of 
which they are the sole pathological expression. 3. That females 
are attacked more frequently than males. Thus, out of 69 cases of 
pharyngeal ulceration, 41 occurred in the former sex. 4. That ul- 
ceration may occur in any situation ; but its most frequent seat is the 
palate, for which it exhibits the closest elective affinity. 5. That, 
when situated at the posterior portion of the hard palate, the ten- 
dency is to involve the soft palate and velum, and thence to invade 
the naso-pharynx and nose; while, situated more anteriorly, it seeks 
* Virchow’s “ Archiv,” Bd. lxxxiv, S. 269. 
| “Trans, of the Pathological Society of London,” 1877. 
\ “The Brain and its Diseases,” vol. i, p. 76. 
# “Recueil d’ophthahn.,” Aug., 1879, p. 464. 
| “Congenital Syphilis of the Throat; based on the Study of 160 Cases,” N. 
clx, p. 321. STUDY OF CONGENITAL SYPHILIS. 
5 
a more direct pathway to the latter, which is established by perfora- 
tion of the bone. 6. That the next most common seats of ulceration 
in order of frequency are the fauces, nasopharynx, posterior pharyn- 
geal wall, nasal fossa and septum, tongue, and gums. 7. That ulcera- 
tion, especially that of the palate, shows a disposition to centrality 
of position, together with a special tendency to caries and necrosis of 
the bone, a fact probably explicable by the great vascularity of the 
periosteum and medullary membrane in youth. 8. That the tendency 
to necrosis exists at all periods of life, but especially in early youth, 
when it is more destructive and less amenable to treatment. 9. 
That while deep pharyngeal ulceration generally precedes or co-exists 
with similar affections of the larynx, the latter occurs too without evi- 
dence of pre-existing pharyngeal lesions. 10. That laryngeal ulcera- 
tion does not commonly" follow the pharyngeal destruction of so- 
called latent syphilis, those palato-pharyngeal ulcerations found in 
tardy' congenital syphilis having little tendency to invasion of the 
larynx, but rather to implication of the nasal pharynx and nose. 
11. That simultaneous or consecutive ulceration of the palate, pha- 
rynx, and nose seems to be characteristic of syphilis, or at least occurs 
more frequently in this than in any other disease. 
1 bring these facts again into prominence because they differ 
from commonly accepted views, and because they possess at least a 
certain value, by reason of the method by which they were obtained. 
I desire also to reiterate what was said in connection with the 
confusion of these lesions with so-called “ scrofulous ” ulceration. 
Without entering into a discussion of the subject, suffice it to say 
that there is no just ground for belief in an ulcerative scrofulide of 
the throat. It needs only the most superficial review of the writ- 
ings of those who maintain its separate existence to show the utter 
confusion which prevails as the result of erroneous views, handed 
down among the traditions of an obsolete pathology. 
It is obviously a point of great practical importance that this fact 
should be recognized, and especially in view of the rapidly destruc- 
tive tendency of inherited syphilitic ulceration in the oro-pharyngeal 
cavities and larynx. 
The throat ulceration of congenital syphilis not only exhibits a 
special tendency to rapid invasion of the deeper tissues; it often 
possesses an inherent virulence which places it apparently beyond 
the reach of therapeutic control. This is markedly true of the ulcera- 
tion which occurs in the earlier years of life. Cases are now and then 
encountered where the ulcers stubbornly refuse to cicatrize, or do so 
sluggishly and imperfectly, healing at one point and becoming simul- 6 
A CONTRIBUTION TO THE 
taneously active at others. Under such circumstances, when remedial 
measures are apparently of little or no avail, they sometimes cicatrize, 
as if by magic, on the accession of an acute disease. It is to this that 
I wish to direct particular attention. 
The clinical study of the cases upon the analysis of which the 
report referred to was based disclosed certain striking facts in con- 
nection w ith the influence of some of the ordinary infectious diseases 
of childhood upon the progress of the inherited syphilitic affection. 
From the historical narratives furnished by this particular group of 
cases, it would appear (1) that, while congenital syphilis affords no 
absolute protection against certain acute infectious diseases, its exist- 
ence in the individual seems often, other things being equal, to mitigate 
their severity and exert a favorable influence on their course ; (2) that 
certain acute diseases, accompanied by an exanthem, favor the dissi- 
pation, at least temporarily, of the throat and other manifestations of 
the disease; (3) that while at no period of the disease is the child 
exempt from these affections, they are more liable to be contracted 
during the period of latency—that curious interval of apparent health 
in congenital syphilis which Cazenave has poetically called the sleep of 
the virus. 
These remarks are limited to scarlet fever, measles, and chicken- 
pox, but they could doubtless be extended to embrace others of the 
exanthemata. 
They do not apply, for obvious reasons, in the case of excessive 
virulence of the syphilitic cachexia or malignant epidemic influence 
of the intercurrent disease. 
Of special interest is the effect produced by acute febrile disease 
upon the throat lesions of congenital syphilis. Chronic inflamma- 
tory conditions and ulceration of the larynx, pharynx, and nasal pas- 
sages are often influenced in a remarkable manner through the pres- 
ence in the individual of an intercurrent febrile affection. This is, 
moreover, eminently true of those acute blood diseases with special ten- 
dency to local manifestations in the throat, such as scarlet fever, 
measles, diphtheria, etc. According to personal experience, scarlet 
fever and measles exert, as a rule, a favorable influence on the course of 
the throat affection, their supervention being of itself sufficient to cause 
its complete disappearance. The poisons of the two diseases in their 
circulation in these regions appear to be mutually destructive, and 
the throat escapes by virtue of such reciprocal antagonism.* The 
* It is quite possible that this may also be true of other mucous surfaces of 
the body. STUDY OF CONGENITAL SYPHILIS. 
7 
cure here may be permanent, or relapses of the inflammatory or ulcera- 
tive process may follow the removal of the antagonistic influence of the 
intercurrent disease. 
These remarks do not apply to diphtheria. When this affection 
supervenes during the existence of lesions in the throat, the patients 
rapidly succumb to the disease. The existence of syphilis in the 
child apparently increases the tendency to membranous formation; 
indeed, in some instances, apart from the presence of the diphtheritic 
process, there seems to be a special tendency to fibrinous formation 
in the nose and retro-nasal space. 
The influence of acute disease upon the manifestations of consti- 
tutional syphilis is a subject which has received some attention at the 
hands of syphilographers, especially certain of the French school ; 
but very little is known as yet, beyond the empirical fact, that the 
lesions of that disease, and especially the cutaneous syphilides, are 
often modified by the introduction into the blood of the virus of an 
intercurrent febrile affection. This modification may consist either 
in the permanent or temporary dissipation of existing syphilitic 
lesions, or in the exaggeration or intensification of the morbid pro- 
cess. Thus, for example, various syphilitic affections, such as skin 
eruptions, exostoses, etc., have been observed to disappear during the 
course of erysipelas,* acute rheumatism,f cholerine,J variola,* febrile 
furunculosis,! etc. LasegueA has recorded a case of ulceration of 
the pharynx and tonsils which disappeared during an attack of ery- 
sipelas, while in a similar one observed by a fatal result 
ensued from that disease. The dissipation of syphilitic eruptions 
has also occurred during pregnancy, J and as the result of vaccina- 
* Vide Cazenave and Schedel, “ Practical Synopsis of Cutan. Dis.,” etc., Phila., 
1829, p. 353; Rayer, “ Traite des mal. de la peau,” Paris, 1835 ; Lamarche, “ De 
l’erysipele salutaire,” These de Paris, 1856, and the excellent articles of Mauriac ; 
“ Etude clinique sur l’influence d’erysipelas dans la syphilis,” Paris, 18*73; pub- 
lished also in the “ Gaz. des hopitaux,” Nouv. ser., 18*73, pp. 305, 321, 346, 385, 
410, 443, 466, 506, 546, 569, 594, 601. See, also, Bidenkap’s case (cited by Baura- 
ler, von Ziemssen’s “Cyclopedia,” Am. ed., vol. iii, p. 98, 18*75). 
f Rayer, op. cit., p. 546 (Mauriac); see also Jourjon, “ Infl. des mal. aigues sur 
quelques manifestations cutan. de la syph.,” Th&se de Paris, 18*70. 
f Cazenave, “ Traite des syphilides,” p. 593 (Mauriac). 
* Gore, “Lancet,” Sept. 2, 1858. 
| Diday (quoted by Mauriac, l. c.). 
A “Traite des angines,” pp. 110-112. 
() “Sur l’angine sypliilitique” (cited by Mauriac). 
| Gilbert, “Bull, de l’acad. de med.,” 1851, tom. xvii, p. 156. 8 
A CONTRIBUTION TO THE STUDY OF CONGENITAL SYPHILIS. 
tion,* and there is a case on record where the latter apparently ex- 
erted a curative influence in caries of the pharyngeal vault, f 
The remarkable power of erysipelas over the cutaneous svphilides 
has suggested its artificial production as a therapeutic agent in these 
while their behavior under the operation of the vaccine 
virus led to the now almost forgotten practice of Lukonski.* It has, 
finally, even been proposed by an enthusiastic pupil of M. Hardy to 
inoculate the poison of small-pox in cases of syphilis which have 
resisted all other methods of treatment. | 
It is sufficiently evident, then, that a reciprocal antagonism exists 
between the poison of syphilis and that of a number of acute dis- 
eases. By what pathological law this is brought about is, in the 
present state of our knowledge of the mutual relations of disease, a 
matter of pure speculation. In the case whose history has been 
recited above it is interesting to contrast the curative effect of the 
exanthematous fever on the ulceration in the throat with the persist 
ence of the latter during the acute pulmonary affection. 
This remarkable influence of the febrile state upon syphilitic 
inflammation and ulceration of the nasal passages and throat is also, 
in a measure, true of simple inflammatory conditions of these cavities. 
It were foreign to the purpose of the present article to elaborate this 
latter and cognate subject, and I shall therefore simply offer for your 
consideration the fact that simple catarrhal inflammation of these 
regions occasionally disappears completely, and is permanently cured 
during the course of an acute febrile disease. Whether this occur 
as a phenomenon of so-called “ substitution,” or as the result of a 
profound impression made upon the nutrition of the parts by virtue 
of which abnormal secretion is arrested and the inflamed tract placed 
in a condition favorable to resolution, can only be determined by the 
accumulation of more exact scientific data concerning the reciprocal 
antagonism of pathological processes. 
Without, then, attempting any special explanation or generali- 
zation, I present the foregoing observations from my clinical ex- 
perience as a contribution to the study of an interesting but imper- 
fectly understood subject. 
* Vide “Revue med.,” 1861, tom. i, p. 157, Jeltzinzki. 
\ Jeltzinzki, l. c., “ Sur la cure radicale de la syphilis par la vaccination.” 
f Sabatier, “ Propositions sur l’erysipele considere principalement comme 
moyen curatif dans les mal. cutanees,” etc., These de Paris, 1831. 
# Jeltzinzki, l. c. 
|| Garrigue, “ De l’influence des mal. aig. sur les diatheses,” These de Paris 
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