Syphilis is a communicable disease caused by Treponema pallidum, which belongs to the Spirochaetaceae family.

 Congenital syphilis is caused by transplacental transmission of spirochetes; the transmission rate approaches 100%. Perinatal death may result from congenital infection in more than 40% of affected, untreated pregnancies. Among survivors, manifestations traditionally have been divided into early and late stages. Manifestations are defined as early if they appear in the first 2 years of life and late if they develop after age 2 years.

Because inflammatory changes do not occur in the fetus until after the first trimester of pregnancy, organogenesis is unaffected. Nevertheless, all organ systems may be involved. With early-onset disease, manifestations result from transplacental spirochetemia and are analogous to the secondary stage of acquired syphilis. Congenital syphilis does not have a primary stage. Late-onset disease is seen in patients older than 2 years and is not considered contagious.

Transmission of T pallidum is rare before the fourth month of gestation due to the presence of Langhans layer of chorion.This membrane disappeares after the fifth month of gestation after which the transmission is possible.

Early manifestations of congenital infection are varied and involve multiple organ systems. The most striking lesions affect the mucocutaneous tissues and bones. Mucous patches, rhinitis, and condylomatous lesions are highly characteristic features of mucous membrane involvement in congenital syphilis.

Nasal fluid is highly infectious. Snuffles are followed quickly by a diffuse maculopapular desquamative rash that involves extensive sloughing of the epithelium, particularly on the palms and soles and about the mouth and anus. In contrast to acquired syphilis, a vesicular rash and bullae may develop. These lesions are highly infectious.

Hepatomegaly is reported in almost 100% of cases, and biochemical evidence of liver dysfunction usually is observed.

The manifestations of Congenital syphilis can be grouped as

1.    Metaphysitis

2.    Periosteitis

3.    Osteitis

 

Metaphysitis: From fetal circulation the spirochetes gain access to the metaphyseal vasculature, where they proliferate. Subsequently, there is formation of granulation tissue that encroaches upon the zone of provisional calcification and remodelling. Hence, there is replacement of the proliferating cartilage by the syphilitic granulation tissue which gives the appearance of lucent metaphyseal bands on radiographs of long bones.

The zone of provisional calcification is weak and can lead to slipping off of the epiphysis.

Wimberger’s Sign of congenital syphilis: Bilaterally symmetrical lucent lesions seen along the medial aspect of tibial metaphysis .

Periosteitis: The infiltration of periosteum by spirochetes produces solid and laminated periosteal reaction that is diffuse and bilaterally symmetrical and affects nearly all the long bones.

Till this phase , all the changes and be reversed with effective chemotherapy.

Osteitis: This phase occurs in those infants who have not received and chemotherapy on in whom the drug regimen has been unsuccessful.

There is invasion of the diaphysis producing lytic lesion with a well defined sclerotic zone around.

 

There is exuberant periosteitis and cortical overgrowth, as seen in these radiographs. The cortical overgrowth and periosteal reaction together crate and undulating appearance if the cortex, producing the so called Sober shin deformity in tibia.

Other features:

Cluttons Joints.

Hutchison’s teeth.