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late congenital syphilis

ICD-10 Codes

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Description

Late congenital syphilis, also known as late-onset congenital syphilis, typically manifests after two years of life. This condition can cause a wide range of symptoms and signs, including:

  • Gummatous ulcers that tend to involve the nose, septum, and hard palate [13]
  • Periosteal lesions that result in saber shins and bossing of the frontal and parietal bones [13]
  • Neurosyphilis is usually asymptomatic, but juvenile paresis and tabes may develop [13]
  • Optic atrophy, sometimes leading to blindness, may occur [13]

Late congenital syphilis can also cause late-latent syphilis, which is characterized by the presence of infection more than 12 months ago. This stage can affect many parts of the body, including the brain, eyes, nerves, heart, blood vessels, liver, bones, and joints.

It's worth noting that late congenital syphilis is not contagious, as it is a result of the transmission of the spirochete Treponema pallidum from the mother to the fetus during pregnancy. The features of late-onset CS are the consequence of protracted inflammation of organs and tissues resulting in permanent scars or destruction of affected organs [15].

References:

[13] Late congenital syphilis typically manifests after 2 years of life and causes gummatous ulcers that tend to involve the nose, septum, and hard palate and periosteal lesions that result in saber shins and bossing of the frontal and parietal bones. Neurosyphilis is usually asymptomatic, but juvenile paresis and tabes may develop. Optic atrophy, sometimes leading to blindness, may occur.

[15] Late Congenital Syphilis. Late onset disease is seen in children older than 2 years of age and is not contagious. The features of late onset CS are the consequence of protracted inflammation of organs and tissues resulting in permanent scars or destruction of affected organs [21,22]

Additional Characteristics

  • Gummatous ulcers that tend to involve the nose, septum, and hard palate
  • Periosteal lesions that result in saber shins and bossing of the frontal and parietal bones
  • Neurosyphilis (juvenile paresis and tabes)
  • Optic atrophy (sometimes leading to blindness)

Signs and Symptoms

Late Congenital Syphilis Signs and Symptoms

Late congenital syphilis typically manifests after 2 years of life, and its symptoms can be quite severe. Some of the common signs and symptoms of late congenital syphilis include:

  • Gummatous ulcers: These are a type of skin lesion that tend to involve the nose, septum, and hard palate [11].
  • Saber shins: This is a condition where the shin bone becomes deformed, giving it a "saber" shape [8][11].
  • Bossing of the frontal and parietal bones: This refers to an abnormal prominence or swelling of these bones in the skull [11].
  • Neurosyphilis: This can manifest as juvenile paresis (a type of paralysis) or tabes (a condition that affects the nerves) [11].
  • Optic atrophy: This is a condition where the optic nerve becomes damaged, which can lead to blindness [11].

It's worth noting that these symptoms can be quite varied and may not always appear in every individual with late congenital syphilis. However, if left untreated, this disease can have serious consequences for an affected child.

References: [8] - Symptoms of Syphilis in Newborns [11] - Late Congenital Syphilis Typically Manifests After 2 Years of Life

Additional Symptoms

  • Optic atrophy
  • Neurosyphilis
  • Gummatous ulcers
  • Saber shins
  • Bossing of the frontal and parietal bones

Diagnostic Tests

Diagnostic Tests for Late Congenital Syphilis

Late congenital syphilis, also known as late-onset congenital syphilis, is a condition that occurs when the spirochete Treponema pallidum is transmitted from a pregnant individual to the fetus. The diagnosis of late congenital syphilis can be challenging due to maternal antibodies in newborns.

Clinical History and Physical Signs

The diagnosis of late congenital syphilis is often based on clinical history, distinctive physical signs, and positive serologic tests [2]. The Hutchinson triad of interstitial keratitis, Hutchinson incisors, and 8th cranial nerve deafness is diagnostic for late congenital syphilis [3].

Serologic Tests

Serologic testing plays a crucial role in the diagnosis of late congenital syphilis. The nontreponemal serologic tests for syphilis, such as the Venereal Disease Research Laboratory (VDRL) or the Rapid Plasma Reagin (RPR) tests, can detect antibody to cardiolipin [6]. However, these tests may not be specific and can produce false-positive results.

Diagnostic Tests

The diagnostic tests for late congenital syphilis include:

  • Blood tests: These can show if you have a syphilis infection. The results help providers determine the fetus's risk for complications [5].
  • Lumbar puncture (spinal tap): This test involves removing a sample of cerebrospinal fluid to detect any signs of syphilis in the central nervous system.
  • Skeletal survey: This test involves taking X-rays of the bones to check for any abnormalities.
  • Hearing evaluation: This test is used to assess hearing and identify any potential problems.
  • Eye examination: This test is used to examine the eyes for any signs of syphilis.

Other Assessments

In addition to these tests, other assessments may be performed to confirm the diagnosis of late congenital syphilis. These include:

  • Dark-field examination: This test involves examining a sample of fluid or tissue under a microscope to detect the presence of Treponema pallidum.
  • Bone x-ray: This test is used to examine the bones for any abnormalities.

References

[1] Mar 28, 2024 - Two types of serological tests for syphilis are available: treponemal-specific tests (TTs) and nontreponemal tests (NTTs). Both must be used to confirm the diagnosis of late congenital syphilis.

[2] Exams and Tests · Bone x-ray · Dark-field examination to detect syphilis bacteria under a microscope · Eye examination · Lumbar puncture (spinal tap) to remove ...

[3] The signs and symptoms of early and late congenital syphilis are

Additional Diagnostic Tests

  • Blood tests
  • Eye examination
  • Lumbar puncture (spinal tap)
  • Skeletal survey
  • Hearing evaluation
  • Dark-field examination
  • Bone x-ray

Treatment

Treatment for Late Congenital Syphilis

Late congenital syphilis typically presents in children after the age of 2, and its symptoms can vary widely [14]. In terms of treatment, benzathine penicillin G is recommended as the first-line therapy for late latent syphilis, including cases caused by congenital syphilis [2].

For those with congenital syphilis, treatment should be undertaken as described. Specifically, a total dose of 7.2 million units of benzathine penicillin G administered intramuscularly (IM) in three doses over one week is recommended [2]. This regimen has been shown to be effective in treating late latent syphilis and congenital syphilis.

It's worth noting that the treatment for proven or highly probable congenital syphilis is aqueous crystalline penicillin G for 10 days, as per recommendation [8].

Key Points:

  • Benzathine penicillin G is recommended for late latent syphilis and congenital syphilis.
  • A total dose of 7.2 million units administered over one week is the standard treatment regimen.
  • Aqueous crystalline penicillin G is recommended for proven or highly probable congenital syphilis.

References:

[2] Late Latent Syphilis: Benzathine penicillin G 7.2 million units total, administered as 3 doses of 2.4 million units IM each at 1-week intervals ... [8] Treatment for proven or highly probable congenital syphilis is aqueous crystalline penicillin G for 10 days (AII).

Recommended Medications

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Differential Diagnosis

Late congenital syphilis can be challenging to diagnose due to its non-specific symptoms and the presence of maternal antibodies in the infant's system. However, a comprehensive differential diagnosis is essential to rule out other conditions that may present similarly.

Key Conditions to Consider:

  • Other congenital infections: Toxoplasmosis, rubella, cytomegalovirus (CMV), and herpes simplex virus can all cause similar symptoms in infants.
  • Genetic disorders: Certain genetic conditions, such as osteogenesis imperfecta or Ehlers-Danlos syndrome, may present with bone abnormalities or other systemic features.
  • Metabolic disorders: Conditions like hypophosphatasia or rickets can cause skeletal deformities and other systemic symptoms.
  • Neurological disorders: Conditions like cerebral palsy or neurofibromatosis type 1 (NF1) can present with neurological symptoms, including developmental delays or seizures.

Clinical Features to Consider:

  • Hutchinson's triad: This classic triad of interstitial keratitis, Hutchinson's incisors, and eighth nerve deafness is highly suggestive of late congenital syphilis.
  • Bone abnormalities: Abnormal bone growth, fractures, or other skeletal deformities can be indicative of late congenital syphilis.
  • Eye abnormalities: Inflammation of the cornea (interstitial keratitis) or other eye problems can be a sign of late congenital syphilis.
  • Hearing loss: Eighth nerve deafness is a common feature of late congenital syphilis.

Diagnostic Tests:

  • Serological tests: Non-treponemal and treponemal tests, such as the VDRL or RPR, can be used to diagnose late congenital syphilis. However, these tests may be positive due to maternal antibodies, making interpretation challenging.
  • Imaging studies: Long-bone radiographs and other imaging studies can help identify bone abnormalities and other systemic features.

Conclusion:

A comprehensive differential diagnosis is essential when considering a diagnosis of late congenital syphilis. Clinicians must carefully evaluate the patient's clinical presentation, laboratory results, and imaging studies to rule out other conditions that may present similarly. A thorough understanding of the key conditions to consider, clinical features to look for, and diagnostic tests to perform will aid in accurate diagnosis and treatment.

References:

  • [10] Congenital syphilis occurs when the spirochete Treponema pallidum is transmitted from a pregnant individual to the fetus.
  • [14] Signs and symptoms of late congenital syphilis (over age 2) include: Abnormal bone growth. Eye abnormalities, like inflammation of the cornea. Hearing loss. Teeth that are notched or have gaps between them (Hutchinson's teeth).
  • [15] Diagnosis of congenital syphilis can be difficult because maternal nontreponemal and treponemal immunoglobulin G (IgG) antibodies can be transferred through the placenta to the fetus, complicating the interpretation of reactive ...

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