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Congenital Infantile Syphilis



This form of congenital syphilis is manifested either from birth, usually in the first two months, or at the age of two to four months. Diverse lesions of the skin, mucous membranes, bones, internal organs, nervous system and sensory organs are encountered in this stage. The development of such children is retarded both in growth and weight, they are weak, restless and hypotrophic. Their skin is wrinkled, dry and sallow. Hydrocephalus, periostitis of the skull bones and dilation of the cranial veins are frequently observed.

A peculiar specific lesion is found on the skin of the face, palms, soles and buttocks, which is known as Hochsinger's diffuse papular infiltration. The favoured sites of the process on the face are the skin around the mouth, the chin, the forehead and the superciliary arches. Several spots or diffuse erythema appear first on the involved areas. The skin here becomes indurated and thickened and acquires a dark-red colour. The involved areas are gradually covered with laminar scales. An extensive infiltrate often forms; it is pierced with deep fissures around the mouth because the infant cries and sucks. Radial scars remain for life in place of the fissures (Robinson-Fourniet scars). The retrospective diagnosis of congenital syphilis may be made from them many years later. Loss of hair, eyebrows and eyelashes occurs in infants if the diffuse infiltration formed of coalesced papules involves the skin of the eyelids, superciliary arches and scalp. Very many treponemas are found in the lesions of the diffuse infiltration.

Syphilitic pemphigus is another early and peculiar sign of congenital infantile syphilis. Vesicles the size of a pea or lentil, with a seropurulent content, and surrounded by a ring of infiltration form.

Tensed vesicles arranged symmetrically on an infiltrate base (usually on the soles and palms) are characteristic of syphilitic pemphigus. Somewhat less frequently the vesicles are localized on the flexor surfaces of the upper and lower limbs, and still less frequently on the face and trunk. They do not tend to coalesce and there are no accompanying subjective disorders. Many treponemas are easily detected in the contents of the vesicles. Syphilitic pemphigus, like Hochsinger's infiltration, is a peculiar manifestation of early congenital syphilis and never occurs in children with acquired syphilis.

Syphilitic papules are fairly often encountered in congenital infantile syphilis and resemble the lenticular papules of the secondary period of acquired syphilis, but in distinction, they tend to coalesce. A typical roseola is a very rare occurrence in infants.

The nasal mucosa is involved most frequently ( syphilitic rhinitis ). This affection occurs in intrauterine life and is therefore found at the child's birth. Less frequently it develops in the first month of life. Three stages of syphilitic rhinitis are distinguished. In the first ( erythematous ) stage there are mild swelling and induration of the mucous membranes, which has no substantial effect on the infant's condition. The second ( secretory ) stage is marked by a considerable swelling and copious purulent secretion. This is the most commonly encountered stage. Nasal breathing becomes difficult, noisy and wheezing, or the infant cannot breathe through the nose at all, as a result of which it is hard for him to suck at the breast and he becomes emaciated. In the third ( ulcerative ) stage, which is rarer, destructive processes form and cause destruction of the cartilaginous and bony tissue of the nasal septum, which may lead to the formation of a saddleback, goat-like or opera-glass nose. T. pallidum is often found in great numbers in the secretions of the nasal mucosa. Involvement of the laryngeal mucosa is much rarer, but if this happens (diffuse, inflammatory infiltration), the voice becomes hoarse and aphonia develops.

Bones are involved very often in congenital infantile syphilis (according to some authors in more than 80 per cent of cases). Infants may have syphilitic osteochondritis, periostitis and osteoperiostitis. Syphilitic osteochondritis of the long tubular bones (usually of the upper limbs) is most typical of congenital infantile syphilis. In the initial stages (grade I or II osteochondritis) the process is identified by means of radiographs. Irregular expansion of the zone of preliminary calcification (up to 2.0-2.5 mm; normally this is a regular clear strip up to 0.5 mm wide) is seen in the metaphysis on the borderline with the cartilage in such cases. This is grade I osteochondritis. In grade II osteochondritis this zone expands to 2-4 mm and spur-like outgrowths and indentation appear on the side facing the epiphysis. The epiphyses of infants of the first months of life have a cartilaginous structure and are therefore not demonstrated on the radiographs. Radiographs of the skeleton should be made no later than the first three months of life because these changes may disappear with time. In the absence of other signs of syphilis, the diagnosis of congenital syphilis cannot be made on the basis of grade I osteochondritis alone, because these changes may be encountered in the bones in other pathological conditions (hypervitaminosis D, hypotrophy, hypovitaminosis, etc.). In a far advanced process (grade III osteochondritis) the zone of preliminary calcification disappears as if washed away. A dark band of destruction, 2-5 mm wide, is found in the metaphysis. Replacement of bone by granulation tissue may lead to intrametaphyseal fractures and the development of Parrot's pseudo-paralysis: in this condition the infant presses the diseased upper limb to the trunk, flexes the diseased lower limb at the knee and hip joints, avoids active movement and cries when passive movements are made (nerve conduction is preserved and sensitivity and movements of the fingers and toes are therefore not impaired).

Syphilitic periostitis and osteoperiostitis in infants are mostly localized in the long tubular bones (which leads to the formation of mildly pronounced organic, tender swellings on the bone surface) and less frequently in the flat bones of the skull (which causes changes in its shape: frontal bossing of the skull, Olympian forehead). Syphilitic phalangitis or dactylitis may sometimes occur, which is extremely characteristic of congenital syphilis. The proximal phalanges are mainly involved; the pronounced character of the process diminishes in the direction of the distal phalanges. A diffuse or gummatous inflammatory process causes cylindrical or fusiform thickening of the bones; the fingers and toes swell and become bottle-shaped. Many phalanges are usually affected; in some cases the process is unilateral.

The eyes are the most frequently involved sensory organs. In congenital syphilis of this age chorioretinitis (involvement of the retina and vascular coat of the eye) is predominantly noted. A characteristic punctate 'salt-and-pepper' yellowish pigmentation forms on the periphery of the fundus of the eye, which, however, does not affect the infant's vision, as a rule.

A diffuse infiltrative process with subsequent sclerosis is found in many internal organs (liver, spleen, kidneys, lungs, testes).

Polyscleradenitis is found quite often: the inguinal, cubital, cervical and other lymph nodes become enlarged and firm.

Involvement of the nervous system in congenital infantile syphilis may be manifested by meningitis, meningoencephalitis and dropsy of the brain (hydrocephalus). Syphilitic meningitis may be acute or chronic. Restlessness and, in some cases, vomiting and short-term convulsions are encountered in these infants; they also cry without cause. Pareses, paralyses and pupillary disorders may be attendant to the symptoms listed above in meningoencephalitis. Hydrocephalus is marked by tenseness of the fontanelle, divarication of the sutures of the skull, enlargement of the skull, vomiting, and bulging of the eyes. To confirm the specific nature of involvement of the nervous system, the results of examination of the cerebrospinal fluid (lumbar puncture) are taken into account. The amount of protein and the cell count (lymphocytes) in the fluid are usually increased. The positive results of serological reactions and IFT and TPI in the fluid are also given due consideration.


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