Late congenital syphilitic oculopathy is a disease of the eye, a manifestation of late congenital syphilis. It can appear as:

- Interstitial keratitis – this commonly appears between ages 6 and 12. Symptoms include lacrimation and photophobia. Pathological vascularization of the cornea cause it to turn pink or salmon colored. 90% of cases affect both eyes.

- Episcleritis or scleritis – nodules appear in or overlying the sclera (white of eye)

- Iritis or iris papules – vascular infiltration of the iris causes rosy color change and yellow/red nodules.

- Chorioretinitis, papillitis, retinal vasculitis – retinal changes can resemble retinitis pigmentosa.

- Exudative retinal detachment

Congenital syphilis is categorized by the age of the child. Early congenital syphilis occurs in children under 2 years old, and late congenital syphilis in children at or greater than 2 years old. Manifestations of late congenital syphilis are similar to those of secondary syphilis and tertiary syphilis in adults.

A positive VDRL of Treponema pallidum immobilization test confirms diagnosis of luetic(syphilitic) interstitial keratitis

If a pregnant mother is identified as being infected with syphilis, treatment can effectively prevent congenital syphilis from developing in the fetus, especially if he or she is treated before the sixteenth week of pregnancy. The fetus is at greatest risk of contracting syphilis when the mother is in the early stages of infection, but the disease can be passed at any point during pregnancy, even during delivery (if the child had not already contracted it). A woman in the secondary stage of syphilis decreases her fetus's risk of developing congenital syphilis by 98% if she receives treatment before the last month of pregnancy. An afflicted child can be treated using antibiotics much like an adult; however, any developmental symptoms are likely to be permanent.

Kassowitz’s law is an empirical observation used in context of congenital syphilis stating that the greater the duration between the infection of the mother and conception, the better is the outcome for the infant. Features of a better outcome include less chance of stillbirth and of developing congenital syphilis.

The Centers for Disease Control and Prevention recommends treating symptomatic or babies born to infected mother with unknown treatment status with procaine penicillin G, 50,000 U/kg dose IM a day in a single dose for 10 days. Treatment for these babies can vary on a case by case basis. Treatment cannot reverse any deformities, brain, or permanent tissue damage that has already occurred.

This is a partial list of human eye diseases and disorders.

The World Health Organization publishes a classification of known diseases and injuries, the International Statistical Classification of Diseases and Related Health Problems, or ICD-10. This list uses that classification.

The underlying cause must be treated as soon as possible to stop the disease process. Corticosteroid drop can be used to minimize the scarring on the cornea along with antibiotic cover. However, residual scarring cannot be avoided which can result in long term visual impairment and corneal transplantation is not suitable due to high rejection rate from the corneal vascularization.

There are several methods to diagnose meningeal syphilis. One of the most common ways include visualizing the organisms by immunofluorescence and dark field microscopy. Dark field microscopy initially had the finding that the spirochete has a corkscrew appearance and that it is spirillar and gram (-) bacteria. Another method would also be through the screening test and serology. Serology includes two types of antibody test: Nontreponemal antibody test and Treponemal antibody test (specific test). The Nontreponemal antibody test screens with VDRL (Venereal Disease Research Lab) and RPR (Rapid Plasma Reagin). The Treponemal antibody test (specific test) confirms with FTA-ABS (Fluorescent treponemal antibody-absorption). Brain imaging and MRI scans may be used when diagnosing patients; however, they do not prove to be as effective as specific tests. Specific tests for treponemal antibody are typically more expensive because the earliest anitbodies bind to spirochetes. These tests are usually more specific and remain positive in patients with other treponemal diseases.

Dark ground microscopy of serous fluid from a chancre may be used to make an immediate diagnosis. Hospitals do not always have equipment or experienced staff members, and testing must be done within 10 minutes of acquiring the sample. Sensitivity has been reported to be nearly 80%; therefore the test can only be used to confirm a diagnosis, but not to rule one out. Two other tests can be carried out on a sample from the chancre: direct fluorescent antibody testing and nucleic acid amplification tests. Direct fluorescent testing uses antibodies tagged with fluorescein, which attach to specific syphilis proteins, while nucleic acid amplification uses techniques, such as the polymerase chain reaction, to detect the presence of specific syphilis genes. These tests are not as time-sensitive, as they do not require living bacteria to make the diagnosis.

Prophylaxis consists of periodic administration of Vitamin A supplements. WHO recommended schedule, which is universally recommended is as follows:

- Infants 6–12 months old and any older children weighing less than 8 kg - 100,000 IU orally every 3–6 months

- Children over 1 year and under 6 years of age - 200,000 IU orally every 6 months

- Infants less than 6 months old, who are not being breastfed - 50,000 IU orally should be given before they attain the age of 6 months

Death from congenital syphilis is usually due to bleeding into the lungs.

Blood tests are divided into nontreponemal and treponemal tests.

Nontreponemal tests are used initially, and include venereal disease research laboratory (VDRL) and rapid plasma reagin (RPR) tests. False positives on the nontreponemal tests can occur with some viral infections, such as varicella (chickenpox) and measles. False positives can also occur with lymphoma, tuberculosis, malaria, endocarditis, connective tissue disease, and pregnancy.

Because of the possibility of false positives with nontreponemal tests, confirmation is required with a treponemal test, such as treponemal pallidum particle agglutination (TPHA) or fluorescent treponemal antibody absorption test (FTA-Abs). Treponemal antibody tests usually become positive two to five weeks after the initial infection. Neurosyphilis is diagnosed by finding high numbers of leukocytes (predominately lymphocytes) and high protein levels in the cerebrospinal fluid in the setting of a known syphilis infection.

Treatment can occur in two ways: treating symptoms and treating the deficiency. Treatment of symptoms usually includes the use of artificial tears in the form of eye drops, increasing the humidity of the environment with humidifiers, and wearing wraparound glasses when outdoors. Treatment of the deficiency can be accomplished with a Vitamin A or multivitamin supplement or by eating foods rich in Vitamin A. Treatment with supplements and/or diet can be successful until the disease progresses as far as corneal ulceration, at which point only an extreme surgery can offer a chance of returning sight.

The most popular treatment forms for any type of syphilis uses penicillin, which has been an effective treatment used since the 1940s.

Other forms also include Benzathine penicillin, which is usually used for primary and secondary syphilis (it has no resistance to penicillin however). Benzathine penicillin is used for long acting form, and if conditions worsen, penicillin G is used for late syphilis.

Diagnosis commonly occurs later in childhood and often occurs incidentally in asymptomatic patients or as a cause of visual impairment. The first symptoms are commonly found during routine vision screenings.

A number of examinations can be used to determine the extent of the syndrome and its severity. Fluorescein angiography is quite useful in diagnosing the disease, and the use of ultrasonography and optical coherence tomography (OCT) are helpful in confirming the disease. Neuro-ophthalmic examinations reveal pupillary defects (see Marcus Gunn Pupil). Funduscopic examinations, examinations of the fundus of the eye, allow detection of arteriovenous malformations. Neurological examinations can determine hemiparesis and paresthesias. Malformations in arteriovenous connections and irregular functions in the veins may be distinguished by fluorescein angiographies. Cerebral angiography examinations may expose AVMs in the cerebrum. MRIs are also used in imaging the brain and can allow visualization of the optic nerve and any possible atrophy. MRI, CT, and cerebral angiography are all useful for investigating the extent and location of any vascular lesions that are affecting the brain. This is helpful in determining the extent of the syndrome.

Cytomegalic inclusion body disease (CIBD) is a series of signs and symptoms caused by cytomegalovirus infection, toxoplasmosis or other rare infections such as herpes or rubella viruses. It can produce massive calcification of the central nervous system, and often the kidneys.

Cytomegalic inclusion body disease is the most common cause of congenital abnormalities in the United States. It can also cause pneumonia and other diseases in immunocompromised patients, such as those with HIV/AIDS or recipients of organ transplants.

The following are not classified as diseases of the eye and adnexa (H00-H59) by the World Health Organization:

- (B36.1) Keratomycosis — fungal infection of the cornea

- (E50.6-E50.7) Xerophthalmia — dry eyes, caused by vitamin A deficiency

- (Q13.1) Aniridia — a rare congenital eye condition leading to underdevelopment or even absence of the iris of the eye

Various systems are affected.

- CNS abnormalities – microcephaly, mental retardation, spasticity, epilepsy, periventricular calcification

- Eye – choroidoretinitis and optic atrophy

- Ear – sensorineural deafness

- Liver – hepatosplenomegaly and jaundice due to hepatitis

- Lung – pneumonitis (interstitial pneumonitis)

- Heart – myocarditis

- Thrombocytopenic purpura, haemolytic anaemia

- Late sequelae in individuals asymptomatic at birth – hearing defects and reduced intelligence

The formation of gummata is rare in developed countries, but common in areas that lack adequate medical treatment.

Syphilitic gummas are found in most but not all cases of tertiary syphilis, and can occur either singly or in groups. Gummatous lesions are usually associated with long-term syphilitic infection; however, such lesions can also be a symptom of benign late syphilis.

Although most recognized for its correlation with the onset of glaucoma, the malformation is not limited to the eye, as Axenfeld syndrome when associated with the PITX2 genetic mutation usually presents congenital malformations of the face, teeth, and skeletal system.

The most characteristic feature affecting the eye is a distinct corneal posterior arcuate ring, known as an "embryotoxon". The iris is commonly adherent to the Schwalbe's line (posterior surface of the cornea).

Diagnosis

One of the three known genetic mutations which cause Rieger Syndrome can be identified through genetic samples analysis. About 40% of Axenfeld-Rieger sufferers have displayed mutations in genes PITX2, FOXC1, and PAX6. The difference between Type 1, 2, and 3 Axenfeld Syndrome is the genetic cause, all three types display the same symptoms and abnormalities.

The OMIM classification is as follows:

Detection of any of these mutations can give patients a clear diagnosis and prenatal procedures such as preimplantation genetic diagnosis, Chorionic villus sampling and Amniocentesis can be offered to patients and prospective parents.

According to a Cochrane review of 2012, controversies remain regarding type of surgery, non-surgical intervention and age of intervention.

The aims of treatment are as follows:

The elimination of any amblyopia

A cosmetically acceptable ocular alignment

long term stability of eye position

binocular cooperation.

The treatment for Bonnet–Dechaume–Blanc syndrome is controversial due to a lack of consensus on the different therapeutic procedures for treating arteriovenous malformations. The first successful treatment was performed by Morgan et al. They combined intracranial resection, ligation of ophthalmic artery, and selective arterial ligature of the external carotid artery, but the patient did not have retinal vascular malformations.

If lesions are present, they are watched closely for changes in size. Prognosis is best when lesions are less than 3 cm in length. Most complications occur when the lesions are greater than 6 cm in size. Surgical intervention for intracranial lesions has been done successfully. Nonsurgical treatments include embolization, radiation therapy, and continued observation. Arterial vascular malformations may be treated with the cyberknife treatment. Possible treatment for cerebral arterial vascular malformations include stereotactic radiosurgery, endovascular embolization, and microsurgical resection.

When pursuing treatment, it is important to consider the size of the malformations, their locations, and the neurological involvement. Because it is a congenital disorder, there are not preventative steps to take aside from regular follow ups with a doctor to keep an eye on the symptoms so that future complications are avoided.

The diagnosis is based on the patient's sexual history and on physical examination revealing a painless, "beefy-red ulcer" with a characteristic rolled edge of granulation tissue. In contrast to syphilitic ulcers, inguinal lymphadenopathy is generally mild or absent. Tissue biopsy and Wright-Giemsa stain are used to aid in the diagnosis. The presence of Donovan bodies in the tissue sample confirms donovanosis. Donovan bodies are rod-shaped, oval organisms that can be seen in the cytoplasm of mononuclear phagocytes or histiocytes in tissue samples from patients with granuloma inguinale.

They appear deep purple when stained with Wright's stain. These intracellular inclusions are the encapsulated Gram-negative rods of the causative organisms. They were discovered by Charles Donovan.

The first known name for this condition was "serpiginous ulcer", which dates to 1882. The proper clinical designation for donovanosis is now "granuloma inguinale". A granuloma is a nodular type of inflammatory reaction, and inguinale refers to the inguinal region, which is commonly involved in this infection. The disease is commonly known as donovanosis, after the Donovan bodies which are a diagnostic sign.

The causative organism, "Klebsiella granulomatis", was called "Calymmatobacterium granulomatis", and some sources still use this classification, from the Greek "kalymma" (a hood or veil), referring to the lesions that contain the bacteria. Prior to this, it was called "Donovania granulomatis", named after the Donovan bodies.

The specific name "granulomatis" refers to the granulomatous lesions. The organism was recently reclassified under the genus "Klebsiella", a drastic taxonomic change since it involved changing the organism's phylum. However, polymerase chain reaction techniques using a colorimetric detection system showed a 99% similarity with other species in the "Klebsiella" genus.

Despite many distinguishing features, the clinical spectrums of following diseases may overlap with chancroid:

- Primary syphilis

- Genital herpes

Practical clinical approach for this STI as Genital Ulcer Disease is to rule out top differential diagnosis of Syphilis and Herpes and consider empirical treatment for Chancroid as testing is not commonly done for the latter.

This remains undetermined at the present time. A recent study by Major et al. reports that:

"Prematurity, family history or secondary ocular history, perinatal or gestational complications, systemic disorders, use of supplemental oxygen as a neonate, use of systemic medications, and male sex were found to be significant risk factors for infantile esotropia."

Further recent evidence indicates that a cause for "infantile strabismus" may lie with the input that is provided to the visual cortex. In particular, neonates who suffer injuries that, directly or indirectly, perturb binocular inputs into the primary visual cortex (V1) have a far higher risk of developing strabismus than other infants.

A paper published by Eltern für Impfaufklärung, a German Anti-Vaccination activist group, cites a study by The Robert Koch Institute (RKI), claiming significant correlation between children who received Vaccinations and the onset of cause of Spine, Face & Eye Asymmetry.

From bubo pus or ulcer secretions, "H. ducreyi" can be identified. PCR-based identification of organisms is available. Simple, rapid, sensitive and inexpensive antigen detection methods for "H. ducreyi" identification are also popular. Serologic detection of "H. ducreyi" is and uses outer membrane protein and lipooligosaccharide.