Congenital syphilis

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Congenital syphilis
Hutchinson teeth congenital syphilis PHIL 2385.rsh.jpg
Notched incisors known as Hutchinson's teeth which are characteristic of congenital syphilis
Specialty Infectious diseases   OOjs UI icon edit-ltr-progressive.svg
Symptoms Rash, fever, large liver and spleen, skeletal abnormalities [1]
Usual onsetUnborn baby, newborn baby or later [1]
DurationLifelong
TypesEarly & late [2]
Causes Treponema pallidum [2]
Diagnostic method Signs, symptoms, blood tests, CSF tests
PreventionAdequate screening and treatment in pregnant mother [2]
Treatment Antibiotic [3]
Medication Penicillin by injection; Procaine benzylpenicillin, benzylpenicillin, benzathine penicillin G [3]
Edvard Munch's Inheritance (1897-99) depicts a baby with congenital syphilis and its mother. Edvard Munch - Inheritance - Google Art Project.jpg
Edvard Munch's Inheritance (1897–99) depicts a baby with congenital syphilis and its mother.

Congenital syphilis is syphilis that occurs when a mother with untreated syphilis passes the infection to her baby during pregnancy or at birth. [4] It may present in the fetus, infant, or later. [1] [5] Clinical features vary and differ between early onset, that is presentation before 2-years of age, and late onset, presentation after age 2-years. [4] Infection in the unborn baby may present as poor growth, non-immune hydrops leading to premature birth or loss of the baby, or no signs. [2] [4] Affected newborns mostly initially have no clinical signs. [4] They may be small and irritable. [4] Characteristic features include a rash, fever, large liver and spleen, a runny and congested nose, and inflammation around bone or cartilage. [2] [4] There may be jaundice, large glands, pneumonia, meningitis, warty bumps on genitals, deafness or blindness. [4] [6] Untreated babies that survive the early phase may develop skeletal deformities including deformity of the nose, lower legs, forehead, collar bone, jaw, and cheek bone. [4] There may be a perforated or high arched palate, and recurrent joint disease. [2] [4] Other late signs include linear perioral tears, intellectual disability, hydrocephalus, and juvenile general paresis. [4] Seizures and cranial nerve palsies may first occur in both early and late phases. [4] Eighth nerve palsy, interstitial keratitis and small notched teeth may appear individually or together; known as Hutchinson's triad. [4]

Contents

It is caused by the bacterium Treponema pallidum subspecies pallidum when it infects the baby after crossing the placenta or from contact with a syphilitic sore at birth. [4] [5] It is not transmitted during breastfeeding unless there is an open sore on the mother's breast. [4] The unborn baby can become infected at any time during the pregnancy. [4] Most cases occur due to inadequate antenatal screening and treatment during pregnancy. [7] The baby is highly infectious if the rash and snuffles are present. [4] The disease may be suspected from tests on the mother; blood tests and ultrasound. [8] Tests on the baby may include blood tests, CSF analysis and medical imaging. [9] Findings may reveal anemia and low platelets. [4] Other findings may include low sugars, proteinuria and hypopituitarism. [4] The placenta may appear large and pale. [4] Other investigations include testing for HIV. [9]

Prevention is by safe sex to prevent syphilis in the mother, and early screening and treatment of syphilis in pregnancy. [6] One intramuscular injection of benzathine penicillin G administered to a pregnant woman early in the illness can prevent congenital syphilis in her baby. [10] Treatment of suspected congenital syphilis is with penicillin by injection; benzylpenicillin into vein, or procaine benzylpenicillin into muscle. [3] [9] During times of penicillin unavailability, ceftriaxone may be an alternative. [9] Where there is penicillin allergy, antimicrobial desensitisation is an option. [9] [11]

Syphilis affects around one million pregnancies a year. [12] In 2016, there were around 473 cases of congenital syphilis per 100,000 live births and 204,000 deaths from the disease worldwide. [13] Of the 660,000 congenital syphilis cases reported in 2016, 143,000 resulted in deaths of unborn babies, 61,000 deaths of newborn babies, 41,000 low birth weights or preterm births, and 109,000 young children diagnosed with congenital syphilis. [14] Around 75% were from the WHO's African and Eastern Mediterranean regions. [2] Serological tests for syphilis were introduced in 1906, and it was later shown that transmission occurred from the mother. [15]

Signs and symptoms

Untreated early syphilis infections results in a high risk of poor pregnancy outcomes, including saddle nose, lower extremity abnormalities, miscarriages, premature births, stillbirths, or death in newborns. Some infants with congenital syphilis have symptoms at birth, but many develop symptoms later. Symptoms may include rash, fever, large liver and spleen, and skeletal abnormalities. [16] Newborns will typically not develop a primary syphilitic chancre but may present with signs of secondary syphilis (i.e. generalized body rash). Often these babies will develop syphilitic rhinitis ("snuffles"), the mucus from which is laden with the T. pallidum bacterium, and therefore highly infectious. If a baby with congenital syphilis is not treated early, damage to the bones, teeth, eyes, ears, and brain can occur. [16]

Early

The face of a newborn infant displaying snuffles indicative of congenital syphilis The face of a newborn infant with Congenital Syphilis.tif
The face of a newborn infant displaying snuffles indicative of congenital syphilis
"Hereditary" syphilis: radiating fissures of the lips (1916). Diseases of children (1916) (14597432110).jpg
"Hereditary" syphilis: radiating fissures of the lips (1916).

This is a subset of cases of congenital syphilis. Newborns may be asymptomatic and are only identified on routine prenatal screening. If not identified and treated, these newborns develop poor feeding and runny nose. [16] By definition, early congenital syphilis occurs in children between 0 and 2 years old. [17]

Late

Gerard de Lairesse, Dutch Baroque painter who had congenital syphilis. Portrait of Gerard de Lairesse MET rl1975.1.140.R.jpg
Gérard de Lairesse, Dutch Baroque painter who had congenital syphilis.
Severe facial disfigurement caused by congenital syphilis, 1880 Syphilis face 1.jpg
Severe facial disfigurement caused by congenital syphilis, 1880

Late congenital syphilis is a subset of cases of congenital syphilis. By definition, it occurs in children at or greater than 2 years of age who acquired the infection trans-placentally. [17]

Symptoms include: [18]

A frequently-found group of symptoms is Hutchinson's triad, which consists of Hutchinson's teeth (notched incisors), keratitis and deafness and occurs in 63% of cases. [18]

Treatment (with penicillin) before the development of late symptoms is essential. [19]

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

Diagnosis

Serological testing is carried out on the mother and the infant. If the neonatal IgG antibody titres are significantly higher than the mother's, then congenital syphilis can be confirmed. Specific IgM in the infant is another method of confirmation. CSF pleocytosis, raised CSF protein level and positive CSF serology suggest neurosyphilis. [23]

Treatment

Centers for Disease Control and Prevention infographic about congenital syphilis. Syphilis infographic about pregnancy from CDC.jpg
Centers for Disease Control and Prevention infographic about congenital syphilis.

If a pregnant mother is identified as being infected with syphilis, treatment can effectively prevent congenital syphilis from developing in the fetus, especially if she is treated before the sixteenth week of pregnancy. [2] 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. [24] An affected child can be treated using antibiotics much like an adult; however, any developmental symptoms are likely to be permanent. [25]

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 the outcome for the infant. Features of a better outcome include less chance of stillbirth and of developing congenital syphilis. [26]

The Centers for Disease Control and Prevention recommends treating symptomatic or babies born to an 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. [27] 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. [25]

A Cochrane review found that antibiotics may be effective for serological cure but in general the evidence around the effectiveness of antibiotics for congenital syphilis is uncertain due to the poor methodological quality of the small number of trials that have been conducted. [28]

Epidemiology

Congenital Syphilis, Primary and Secondary Rates, United States, before 1999 Congenital Syphilis, Primary and Secondary Rates, by Year - United States, 1992-1998.jpg
Congenital Syphilis, Primary and Secondary Rates, United States, before 1999

Syphilis affects around one million pregnancies a year. [12] In 2016, there were around 473 cases of congenital syphilis per 100,000 live births and 204,000 deaths from the disease worldwide. [13] Of the 660,000 congenital syphilis cases reported in 2016, 143,000 resulted in deaths of unborn babies, 61,000 deaths of newborn babies, 41,000 low birth weights or preterm births, and 109,000 young children diagnosed with congenital syphilis. [14] Around 75% were from the WHO's African and Eastern Mediterranean regions. [2]

Cases in the United States have been increasingly rising in the 2010s. The Centers for Disease Control and Prevention (CDC) reported 918 cases for 2017, what is more than twice the figures of four years earlier. [29] Reports in 2023 show a rise of more than 900 percent in Mississippi over five years. [30]

History

Nineteenth century physicians held the belief that congenital syphilis was contracted from contaminated semen at time of conception. [15] Serological tests for syphilis were introduced in 1906, and it was later shown that transmission occurred from the mother. [15]

Related Research Articles

<span class="mw-page-title-main">Syphilis</span> Sexually transmitted infection

Syphilis is a sexually transmitted infection caused by the bacterium Treponema pallidum subspecies pallidum. The signs and symptoms of syphilis vary depending in which of the four stages it presents. The primary stage classically presents with a single chancre though there may be multiple sores. In secondary syphilis, a diffuse rash occurs, which frequently involves the palms of the hands and soles of the feet. There may also be sores in the mouth or vagina. In latent syphilis, which can last for years, there are few or no symptoms. In tertiary syphilis, there are gummas, neurological problems, or heart symptoms. Syphilis has been known as "the great imitator" as it may cause symptoms similar to many other diseases.

<span class="mw-page-title-main">Rubella</span> Human viral disease

Rubella, also known as German measles or three-day measles, is an infection caused by the rubella virus. This disease is often mild, with half of people not realizing that they are infected. A rash may start around two weeks after exposure and last for three days. It usually starts on the face and spreads to the rest of the body. The rash is sometimes itchy and is not as bright as that of measles. Swollen lymph nodes are common and may last a few weeks. A fever, sore throat, and fatigue may also occur. Joint pain is common in adults. Complications may include bleeding problems, testicular swelling, encephalitis, and inflammation of nerves. Infection during early pregnancy may result in a miscarriage or a child born with congenital rubella syndrome (CRS). Symptoms of CRS manifest as problems with the eyes such as cataracts, deafness, as well as affecting the heart and brain. Problems are rare after the 20th week of pregnancy.

<span class="mw-page-title-main">Congenital rubella syndrome</span> Medical condition

Congenital rubella syndrome (CRS) occurs when an unborn baby is infected with the rubella virus via maternal-fetal transmission and develops birth defects. The most common congenital defects affect the ophthalmologic, cardiac, auditory, and neurologic systems.

<span class="mw-page-title-main">Hemolytic disease of the newborn</span> Fetal and neonatal alloimmune blood condition

Hemolytic disease of the newborn, also known as hemolytic disease of the fetus and newborn, HDN, HDFN, or erythroblastosis fetalis, is an alloimmune condition that develops in a fetus at or around birth, when the IgG molecules produced by the mother pass through the placenta. Among these antibodies are some which attack antigens on the red blood cells in the fetal circulation, breaking down and destroying the cells. The fetus can develop reticulocytosis and anemia. The intensity of this fetal disease ranges from mild to very severe, and fetal death from heart failure can occur. When the disease is moderate or severe, many erythroblasts are present in the fetal blood, earning these forms of the disease the name erythroblastosis fetalis.

<span class="mw-page-title-main">Complications of pregnancy</span> Medical condition

Complications of pregnancy are health problems that are related to, or arise during pregnancy. Complications that occur primarily during childbirth are termed obstetric labor complications, and problems that occur primarily after childbirth are termed puerperal disorders. While some complications improve or are fully resolved after pregnancy, some may lead to lasting effects, morbidity, or in the most severe cases, maternal or fetal mortality.

<span class="mw-page-title-main">Vertically transmitted infection</span> Infection caused by pathogens that use mother-to-children transmission

A vertically transmitted infection is an infection caused by pathogenic bacteria or viruses that use mother-to-child transmission, that is, transmission directly from the mother to an embryo, fetus, or baby during pregnancy or childbirth. It can occur when the mother has a pre-existing disease or becomes infected during pregnancy. Nutritional deficiencies may exacerbate the risks of perinatal infections. Vertical transmission is important for the mathematical modelling of infectious diseases, especially for diseases of animals with large litter sizes, as it causes a wave of new infectious individuals.

<span class="mw-page-title-main">Group B streptococcal infection</span> Medical condition

Group B streptococcal infection, also known as Group B streptococcal disease or just Group B strepinfection,

<span class="mw-page-title-main">Erythema toxicum neonatorum</span> Medical condition

Erythema toxicum neonatorum is a common, non-threatening rash in newborns. It appears in 4-70% of newborns within the first week of life, and it typically improves within 1–2 weeks. It only occurs during the newborn period, but may appear slightly later in premature babies. The rash has a variable appearance. It typically includes blotchy red spots, often with overlying firm, yellow-white bumps or pus-filled boils. There may be only a few or many lesions. The lesions can appear almost anywhere on the body, and individual lesions may appear and disappear within hours. There are no other symptoms associated with erythema toxicum neonatorum, and the rash does not have any long-term effects on the skin. Erythema toxicum neonatorum is not harmful and does not require any treatment.

<span class="mw-page-title-main">Neurosyphilis</span> Infection of the central nervous system in a patient with syphilis

Neurosyphilis is the infection of the central nervous system in a patient with syphilis. In the era of modern antibiotics, the majority of neurosyphilis cases have been reported in HIV-infected patients. Meningitis is the most common neurological presentation in early syphilis. Tertiary syphilis symptoms are exclusively neurosyphilis, though neurosyphilis may occur at any stage of infection.

<span class="mw-page-title-main">Neonatal conjunctivitis</span> Medical condition

Neonatal conjunctivitis is a form of conjunctivitis which affects newborn babies following birth. It is typically due to neonatal bacterial infection, although it can also be non-infectious. Infectious neonatal conjunctivitis is typically contracted during vaginal delivery from exposure to bacteria from the birth canal, most commonly Neisseria gonorrhoeae or Chlamydia trachomatis.

<span class="mw-page-title-main">Syphilitic aortitis</span> Inflammation of the aorta

Syphilitic aortitis is inflammation of the aorta associated with the tertiary stage of syphilis infection. SA begins as inflammation of the outermost layer of the blood vessel, including the blood vessels that supply the aorta itself with blood, the vasa vasorum. As SA worsens, the vasa vasorum undergo hyperplastic thickening of their walls thereby restricting blood flow and causing ischemia of the outer two-thirds of the aortic wall. Starved for oxygen and nutrients, elastic fibers become patchy and smooth muscle cells die. If the disease progresses, syphilitic aortitis leads to an aortic aneurysm. Overall, tertiary syphilis is a rare cause of aortic aneurysms. Syphilitic aortitis has become rare in the developed world with the advent of penicillin treatments after World War II.

<span class="mw-page-title-main">Zika fever</span> Infectious disease caused by the Zika virus

Zika fever, also known as Zika virus disease or simply Zika, is an infectious disease caused by the Zika virus. Most cases have no symptoms, but when present they are usually mild and can resemble dengue fever. Symptoms may include fever, red eyes, joint pain, headache, and a maculopapular rash. Symptoms generally last less than seven days. It has not caused any reported deaths during the initial infection. Mother-to-child transmission during pregnancy can cause microcephaly and other brain malformations in some babies. Infections in adults have been linked to Guillain–Barré syndrome (GBS).

<span class="mw-page-title-main">Blueberry muffin baby</span> Medical condition

Blueberry muffin baby, also known as extramedullary hematopoiesis, describes a newborn baby with multiple purpura, associated with several non-cancerous and cancerous conditions in which extra blood is produced in the skin. The bumps range from one to seven mm, do not blanch and have a tendency to occur on the head, neck and trunk. They often fade by three to six weeks after birth, leaving brownish marks. When due to a cancer, the bumps tend to be fewer, firmer and larger.

Neonatal herpes simplex, or simply neonatal herpes, is a herpes infection in a newborn baby caused by the herpes simplex virus (HSV), mostly as a result of vertical transmission of the HSV from an affected mother to her baby. Types include skin, eye, and mouth herpes (SEM), disseminated herpes (DIS), and central nervous system herpes (CNS). Depending on the type, symptoms vary from a fever to small blisters, irritability, low body temperature, lethargy, breathing difficulty, and a large abdomen due to ascites or large liver. There may be red streaming eyes or no symptoms.

<span class="mw-page-title-main">Congenital cytomegalovirus infection</span> Medical condition

Congenital cytomegalovirus (cCMV) is cytomegalovirus (CMV) infection in a newborn baby. Most have no symptoms. Some affected babies are small. Other signs and symptoms include a rash, jaundice, hepatomegaly, retinitis, and seizures. It may lead to loss of hearing or vision, developmental disability, or a small head.

<span class="mw-page-title-main">Epidemiology of syphilis</span> Eradication efforts and prevalence of syphilis

Syphilis is a bacterial infection transmitted by sexual contact and is believed to have infected 12 million people in 1999 with greater than 90% of cases in the developing world. It affects between 700,000 and 1.6 million pregnancies a year, resulting in spontaneous abortions, stillbirths, and congenital syphilis. In Sub-Saharan Africa syphilis contributes to approximately 20% of perinatal deaths.

<span class="mw-page-title-main">History of syphilis</span>

The first recorded outbreak of syphilis in Europe occurred in 1494/1495 in Naples, Italy, during a French invasion. Because it was spread by returning French troops, the disease was known as "French disease", and it was not until 1530 that the term "syphilis" was first applied by the Italian physician and poet Girolamo Fracastoro. The causative organism, Treponema pallidum, was first identified by Fritz Schaudinn and Erich Hoffmann in 1905 at the Charité Clinic in Berlin. The first effective treatment, Salvarsan, was developed in 1910 by Sahachiro Hata in the laboratory of Paul Ehrlich. It was followed by the introduction of penicillin in 1943.

<span class="mw-page-title-main">Meningeal syphilis</span> Medical condition

Meningeal syphilis is a chronic form of syphilis infection that affects the central nervous system. Treponema pallidum, a spirochate bacterium, is the main cause of syphilis, which spreads drastically throughout the body and can infect all its systems if not treated appropriately. Treponema pallidum is the main cause of the onset of meningeal syphilis and other treponemal diseases, and it consists of a cytoplasmic and outer membrane that can cause a diverse array of diseases in the central nervous system and brain.

HIV in pregnancy is the presence of an HIV/AIDS infection in a woman while she is pregnant. There is a risk of HIV transmission from mother to child in three primary situations: pregnancy, childbirth, and while breastfeeding. This topic is important because the risk of viral transmission can be significantly reduced with appropriate medical intervention, and without treatment HIV/AIDS can cause significant illness and death in both the mother and child. This is exemplified by data from The Centers for Disease Control (CDC): In the United States and Puerto Rico between the years of 2014–2017, where prenatal care is generally accessible, there were 10,257 infants in the United States and Puerto Rico who were exposed to a maternal HIV infection in utero who did not become infected and 244 exposed infants who did become infected.

<span class="mw-page-title-main">Neonatal infection</span> Human disease

Neonatal infections are infections of the neonate (newborn) acquired during prenatal development or within the first four weeks of life. Neonatal infections may be contracted by mother to child transmission, in the birth canal during childbirth, or after birth. Neonatal infections may present soon after delivery, or take several weeks to show symptoms. Some neonatal infections such as HIV, hepatitis B, and malaria do not become apparent until much later. Signs and symptoms of infection may include respiratory distress, temperature instability, irritability, poor feeding, failure to thrive, persistent crying and skin rashes.

References

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  13. 1 2 Global progress report on HIV, viral hepatitis and sexually transmitted infections, 2021 (PDF). Geneva: World Health Organization. 2021. ISBN   978-92-4-003098-5. Archived (PDF) from the original on 2023-03-26. Retrieved 2023-05-08.
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  24. ↑ Congenital syphilis.
  25. 1 2 Arnold, S; Ford-Jones, L (November–December 2000). "Congenital syphilis: A guide to diagnosis and management". Paediatrics & Child Health. 5 (8): 463–469. doi:10.1093/pch/5.8.463. PMC   2819963 . PMID   20177559.
  26. ↑ Singh, Ameeta E.; Romanowski, Barbara (1 April 1999). "Syphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features". Clinical Microbiology Reviews. 12 (2): 187–209. doi:10.1128/CMR.12.2.187. PMC   88914 . PMID   10194456.
  27. ↑ "Sexually Transmitted Diseases: Treatment Guidelines, 2010 By: the CDC" . Retrieved July 21, 2019.
  28. ↑ Walker, GJ; Walker, D; Molano Franco, D; Grillo-Ardila, CF (15 February 2019). "Antibiotic treatment for newborns with congenital syphilis". The Cochrane Database of Systematic Reviews. 2019 (2): CD012071. doi:10.1002/14651858.CD012071.pub2. PMC   6378924 . PMID   30776081.
  29. ↑ "A Devastating Surge in Congenital Syphilis: How Can We Stop It?". Medscape. Retrieved 2023-02-15.
  30. ↑ Yang, Maya (2023-02-12). "Mississippi sees 900% rise in number of infants born with congenital syphilis". The Guardian. ISSN   0261-3077 . Retrieved 2023-02-15.

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