Congenital syphilis persists in the United States largely because a significant number of women do not receive serologic testing until late in pregnancy, if at all, which in turn is related to absent or late prenatal care. Systemic findings in patients with early congenital syphilis (age 2 years or younger) include hepatosplenomegaly, characteristic changes of the long bones on radiographic examination, abdominal distention, desquamative skin rash, low birth weight, pneumonia, and severe anemia. Late manifestations (age 3 or older) result from scarring during early systemic disease and include Hutchinson teeth, Mulberry molars, abnormal facies, cranial nerve VIII deafness, bony changes such as saber shins and perforations of the hard palate, cutaneous lesions such as rhagades, and neurosyphilis. Cardiovascular complications are unusual in late congenital syphilis.
Ocular inflammatory signs of syphilis may present at birth or decades later and include uveitis, interstitial keratitis, optic neuritis, glaucoma, and congenital cataract. A multifocal chorioretinitis and, less commonly, retinal vasculitis are the most frequent uveitic manifestations of early congenital infection. They may result in a bilateral “salt-and-pepper” fundus, which affects the peripheral retina, posterior pole, or a single quadrant. These changes are not progressive, and the patient may have normal vision. A less commonly described funduscopic variation is that of a bilateral secondary degeneration of the RPE, which may mimic retinitis pigmentosa with narrowing of the retinal and choroidal vessels, optic disc pallor with sharp margins, and morphologically variable deposits of pigment.
Nonulcerative stromal interstitial keratitis, often accompanied by anterior uveitis, is the most common inflammatory sign of untreated late congenital syphilis, occurring in up to 50% of cases, most commonly in girls (Fig 7-43). Keratouveitis is thought to be an allergic response to T pallidum in the cornea. Symptoms include intense pain and photophobia. The cornea may be diffusely opaque, with reduced vision, even to the level of light perception only. Blood vessels invade the cornea, and when they meet in the center of the visual axis after several months, the inflammation subsides and the cornea partially clears. Late stages show deep ghost (nonperfused) stromal vessels and opacities. Although the iritis accompanying interstitial keratitis may be difficult to observe because of corneal haze, secondary guttata and hyaline strands projecting into the angle provide evidence of anterior segment inflammation. Glaucoma may also occur. The constellation of interstitial keratitis, cranial nerve VIII deafness, and Hutchinson teeth is called the Hutchinson triad.