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In a few children there is a later stage, predominantly causing fusiform radiolucent areas in the cortex and medulla of long bones, with a persistent organized periosteal reaction.

The stages of the bone involvement vary considerably: in some children syphilitic periostitis may be the only abnormality, but in others metaphysitis, periositis, and osteitis are not sequential but occur at the same time. The late effects may persist into the second decade, with cortical and medullary new bone and periosteal reaction, seen most commonly on the tibia. Osteolytic lesions may resemble the gumma of tertiary syphilis and chronic joint inflammation can persist, usually without bone destruction. Rarely, saber tibia can develop in children as a postcongenital syphilitic change. It has been suggested, and it is perhaps an easy way to explain these late changes, that they are really the result of secondary and tertiary syphilis, following an early primary infection through the placenta. The cardiovascular and central nervous systems are not affected.

Congenital syphilis can affect the stomach, although it is extremely rare. In one case (Mendel et al. 1956) a 10-year-old girl was found to have gastric ulceration which over a period of months progressed to simulate linitis plastica, with the stomach greatly decreased in size. It had become almost tubular, with rigid walls and obliterated mucosa. Her serological tests were positive (as were those of her mother and younger brother) and there was immediate clinical response to antibiotics. During the following months, there was improvement in the radiological appearance of the stomach but it never fully returned to normal. It was not until 2 years after her initial complaint that the skeleton was examined: there were symmetrical cortical erosions at the proximal ends of the diaphyses of the humeri, resembling healing gummas.

Differential Diagnosis

Congenital syphilis can be mistaken for many other conditions but if the age of the infants is considered, there will be fewer errors. The differential diagnosis includes scurvy, sickle cell disease, leukemia, rubella, rickets, cytomegalic inclusion disease, infantile hyperparathyroidism, and metastatic neuroblastoma, as well as infantile cortical hyperostosis. The later syphilitic skeletal lesions (sometimes called juvenile syphilis) must be distinguished from pyogenic arthritis, juvenile rheumatoid arthritis, and sickle cell disease. In small children repeated trauma (as in the battered baby syndrome) may cause periosteal and epiphyseal abnormalities, but the ribs are seldom affected in congenital syphilis, nor are there likely to be any fractures.

Most of the nonsyphilitic causes of similar skeletal changes do not result in symmetrical bone lesions (Fig. 35.15) and occur in different age groups. The serology will, of course, provide a positive answer in almost every case. Nevertheless, being aware of the possibility of congenital syphilis may be the most important way to make the correct diagnosis, especially when it is clinically unsuspected.

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Copyright: Palmer and Reeder