Congenital Syphilis of Early Childhood

This period of congenital syphilis occurs in children between the ages of 1 and 4 years, and is distinguished by the scarcity of manifestations. Moist and eroded papules or condylomata latum usually occur, or papular lesions and flat condylomata form simultaneously in the anal region and on the genitals. The papular eruption (if present) is characterized by large lesions localized on the genitals, limbs, buttocks, less frequently on the face. The papules in the inguinal, axillary and other natural skin folds often undergo erosion. Hard, gummatous nodules may form on the skin of some patients.

Affections of the mucous membranes of the mouth (eroded papules), larynx, vocal cords (making the voice hoarse), and nose are sometimes found.

In addition to the skin and mucosal lesions described above, affections of the bone system, mainly periostitis of the long tubular bones (usually the tibia, less frequently the forearm bones), and involvement of the ocular coats are encountered in this period. Involvement of the liver and spleen are less frequent; there is no gross enlargement or firmness of these organs.

Syphilitic meningitis, meningoencephalitis, mental retardation and other affections of the nervous system have been described.

In many children of 1 to 4 years of age congenital syphilis follows a latent course with no clinical symptoms and is diagnosed only on the basis of positive serological blood reactions, which, however, may be negative in 15 to 20 per cent of children with the disease.

Late Congenital Syphilis

Late congenital syphilis is diagnosed in children at the age of 4 to 15 years and later.

In its course, late congenital syphilis resembles acquired tertiary syphilis because patients develop gummata or gummatous infiltrations of the skin, mucous membranes and bones (gummatous periostitis and osteoperiostitis), joints, internal organs and nervous system (gummatous meningitis). Tubercular syphilids may appear on the skin.

Besides the listed symptoms of late congenital syphilis, which are identical with tertiary syphilids of acquired syphilis, there are groups of symptoms encountered only in late congenital syphilis. They are divided into three groups. The first group consists of authentic, or unconditional, signs pathognomonic for late congenital syphilis. The second group is composed of accessory signs, which suggest congenital syphilis when there are other signs confirming the syphilitic infection (the results of serological blood tests, medical history and results of examination of the mother). The third group is formed of dystrophies (stigmata), which are encountered in many chronic infectious diseases and are not specific for congenital syphilis. The combination of several dystrophies in the presence of other evidence of the infection (e.g. positive Wassermann's test in the blood of the child and mother), however, enables the physician to make the correct diagnosis of the disease.

Hutchinson 's triad (interstitial keratitis, syphilitic labyrinthitis and Hutchinson's teeth) is an unconditional, or authentic, sign of late congenital syphilis.

Interstitial keratitis is characterized by redness and opacification of the cornea, photophobia, lacrimation, and stable impairment of visual acuity. In most cases first one eye and some time later the other are affected. The process lasts for a long time and resolution is extremely slow.

Syphilitic labyrinthitis, or labyrinthine deafness, occurring suddenly in a state of apparent well-being is characterized by diminished hearing, usually bilateral, and tinnitus. The process is associated with infiltrative inflammation of the labyrinth and degeneration of the acoustic nerve. If labyrinthitis occurs in a child whose speech is still undeveloped, there may be difficulties in pronouncing sounds or the child may become a deaf-mute.

Hutchinson 's teeth are marked by dystrophy of the two permanent upper median incisors which are shaped like a chisel and have a notched cutting edge. Before the eruption of the permanent teeth, Hutchinson's teeth are identified on a radiograph. The complete Hutchinson's triad is a very rare occurrence.

The accessory signs of later congenital syphilis are: sabre shins, syphilitic chorioretinitis and gonitis, Robinson-Fourniet's radial scars, frontal bossing of the skull, deformity of the nose (saddleback, opera-glass, and ‘goat’ nose), certain dystrophies of the teeth (kidney-shaped or 'tobacco-pouch' tooth), and some forms of neurosyphilis.

Sabre shins are marked by night pains and anterior bending of the tibia as a consequence of osteoperiostitis suffered in infancy. Sabre forearms are encountered far less frequently.

Chorioretinitis is characterized by small 'salt-and-pepper' pigmented areas on the fundus of the eye. The process involves the vascular coat of the eye and the optic disk. Since chorioretinitis may also be of tuberculous etiology, it is considered not an authentic but an accessory sign of late congenital syphilis.

Syphilitic gonitis, or Clutton's synovitis, or joint (Clutton was the first to describe this condition) follows a chronic course with impaired mobility and restriction of movements usually at the knees and, less frequently, at the elbows or ankles. There is neither increase in local temperature nor tenderness.

Serological tests, which are positive in almost all cases with gonitis, and the results of ex juvantibus therapy help in making the diagnosis.

Robinson-Fourniet's scars are arranged radially around the corners of the mouth, on the chin, and around the lips, and form as the result of Hochsinger's diffuse papular infiltration suffered in infancy. In some cases these scars may be rounded or pitted (like a thimble). Similar small scars may remain after a child had candidiasis, pyoderma or burn, which sometimes leads to diagnostic errors.

Frontal bossing of the skull in congenital syphilis forms in the first months of life, whereas in rickets this deformity becomes apparent by the second or third year of life. It results from the deposition of a diffusely ossifying infiltrate in the frontal and parietal bones, leading to a bulging of the frontal eminences and the formation of a groove between them. Localized syphilitic hydrocephalus is a contributing factor. Some children with congenital syphilis have a steeple skull.

Syphilitic rhinitis suffered in early infancy may sometimes cause deficient development of the bony or cartilaginous part of the nose resulting in deformities of the nose, which are very typical of late congenital syphilis. They include saddleback (protruding nostrils), opera-glass and 'goat' nose . In acquired syphilis a gummatous process may also cause deformities of the nose.

Among the variety of dental dystrophies, the following are the accessory signs of later congenital syphilis. A kidney-shaped (tobacco-pouch) tooth (the four masticating cusps of the first molar are underdeveloped and occur as thin projections; the tooth itself is small, the diameter of the masticating surface is less than the diameter of the neck, which makes the tooth domed) and a 'pike' tooth (hypoplasia of the masticatory surface of the canine tooth, from which a thin conical outgrowth resembling the tooth of a fish protrudes).

Affections of the nervous system in late congenital syphilis are diverse and are always attended with a positive T. pallidum immobilization test, though Wassermann's reaction is negative in some cases. Dementia (as a consequence of syphilis of the cerebral vessels), Jacksonian epilepsy, hemiparesis, hemiplegia, speech disorders, persistent headache (as a manifestation of syphilitic hydrocephalus), and tabes dorsalis are among the forms of neurosyphilis. In children with tabes dorsalis various pupillary disorders (anisocoria, the Argyll Rober-tson pupil, etc.) are mostly revealed.

Among the numerous and diverse dystrophies encountered in children whose parents have various infectious (e.g. tuberculosis) and non-infectious (e.g. alcoholism) diseases, we shall dwell here only on those which are most common in late congenital syphilis, though they are not specific of syphilis alone. These dystrophies (stigmata) may also form in the early periods (sometimes in infants); they are caused by metabolic disorders, endocrine dysfunction, disorders of cardiovascular and nervous activity. The following stigmata of late congenital syphilis are most significant: (1) Avsitidiisky's sign consisting in thickening of the sternal end of the clavicle as the result of diffuse hyperostosis; (2) a high 'lancet-like' , or 'gothic' hard palate ; (3) infantile little finger (Dubois-Gissard's sign); the little finger is short (Dubois" sign) and the little finger is somewhat deformed and turned inward (Gissard's sign); (4) axiphoidia, i.e. absence of the sternal xiphoid process; (5) Carabelli's cusp, the presence of a fifth auxiliary cusp on the masticatory surface of the first upper molar; (6) diastema, gaps between the upper incisors; (7) hypertrichosis in boys and girls and growth of hair on the forehead almost to the eyebrows.

These dystrophies are of diagnostic importance if two or more of them are encountered in combination with other signs (at least with one true authentic sign of Hutchinson's triad, or with more than one accessory sign) of syphilis or with the findings of serological examination and medical history verifying the presence of the infection in the children and their parents.

Serological blood tests which are positive in 70 to 80 per cent of patients with active manifestations, and particularly the TPI, which is positive in almost 100 per cent of patients, help in the diagnosis of late congenital syphilis.

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