Congenital syphilis
Encyclopedia
Congenital syphilis is syphilis
Syphilis
Syphilis is a sexually transmitted infection caused by the spirochete bacterium Treponema pallidum subspecies pallidum. The primary route of transmission is through sexual contact; however, it may also be transmitted from mother to fetus during pregnancy or at birth, resulting in congenital syphilis...

 present in utero and at birth, and occurs when a child is born to a mother with secondary syphilis. Untreated syphilis results in a high risk of a bad outcome of pregnancy, including mulberry molar
Mulberry molar
Mulberry molars are a dental condition usually associated with congenital syphilis, characterized by multiple rounded rudimentary enamel cusps on the permanent first molars....

s in the fetus. Syphilis can cause miscarriage
Miscarriage
Miscarriage or spontaneous abortion is the spontaneous end of a pregnancy at a stage where the embryo or fetus is incapable of surviving independently, generally defined in humans at prior to 20 weeks of gestation...

s, premature birth
Premature birth
In humans preterm birth refers to the birth of a baby of less than 37 weeks gestational age. The cause for preterm birth is in many situations elusive and unknown; many factors appear to be associated with the development of preterm birth, making the reduction of preterm birth a challenging...

s, stillbirth
Stillbirth
A stillbirth occurs when a fetus has died in the uterus. The Australian definition specifies that fetal death is termed a stillbirth after 20 weeks gestation or the fetus weighs more than . Once the fetus has died the mother still has contractions and remains undelivered. The term is often used in...

s, or death of newborn babies. Some infants with congenital syphilis have symptoms at birth, but most develop symptoms later. Untreated babies can have deformities, delays in development, or seizures along with many other problems such as rash, fever, hepatosplenomegaly
Hepatosplenomegaly
Hepatosplenomegaly is the simultaneous enlargement of both the liver and the spleen . Hepatosplenomegaly can occur as the result of acute viral hepatitis or infectious mononucleosis, or it can be the sign of a serious and life threatening lysosomal storage disease...

, anemia
Anemia
Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...

, and jaundice
Jaundice
Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...

. Sores on infected babies are infectious. Rarely, the symptoms of syphilis go unseen in infants so that they develop the symptoms of late-stage syphilis, including damage to their bones, teeth, eyes, ears, and brain.

Early

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 rhinorrhea
Rhinorrhea
Rhinorrhea or rhinorrhoea is a condition where the nasal cavity is filled with a significant amount of mucous fluid. The condition, commonly known as "runny nose", occurs relatively frequently and is not usually considered dangerous. Rhinorrhea is a common symptom of allergies or certain diseases,...

. By definition, early congenital syphilis occurs in children between 0 and 2 years old. After, they can develop late congenital syphilis.

Symptomatic newborns, if not stillborn
Stillbirth
A stillbirth occurs when a fetus has died in the uterus. The Australian definition specifies that fetal death is termed a stillbirth after 20 weeks gestation or the fetus weighs more than . Once the fetus has died the mother still has contractions and remains undelivered. The term is often used in...

, are born premature, with hepatosplenomegaly
Hepatosplenomegaly
Hepatosplenomegaly is the simultaneous enlargement of both the liver and the spleen . Hepatosplenomegaly can occur as the result of acute viral hepatitis or infectious mononucleosis, or it can be the sign of a serious and life threatening lysosomal storage disease...

, skeletal abnormalities, pneumonia
Pneumonia
Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...

 and a bullous skin disease known as pemphigus syphiliticus.

Late

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.

Symptoms include
  • blunted upper incisor teeth known as Hutchinson's teeth
    Hutchinson's teeth
    Hutchinson's teeth are a sign of congenital syphilis. Babies with this have teeth that are smaller and more widely spaced than normal and which have notches on their biting surfaces...

  • inflammation of the cornea known as interstitial keratitis
    Keratitis
    Keratitis is a condition in which the eye's cornea, the front part of the eye, becomes inflamed. The condition is often marked by moderate to intense pain and usually involves impaired eyesight.-Types:...

  • deafness from auditory nerve disease
  • frontal bossing (prominence of the brow ridge)
  • saddle nose
    Saddle nose
    Saddle nose is a condition associated with congenital syphilis, relapsing Polychondritis, Wegener's granulomatosis, cocaine abuse, and Leprosy, among other conditions. It is characterized by a loss of height of the nose, because of the collapse of the bridge....

     (collapse of the bony part of nose)
  • hard palate
    Palate
    The palate is the roof of the mouth in humans and other mammals. It separates the oral cavity from the nasal cavity. A similar structure is found in crocodilians, but, in most other tetrapods, the oral and nasal cavities are not truly separate. The palate is divided into two parts, the anterior...

     defect
  • swollen knees
  • saber shins
  • short maxillae
  • protruding mandible


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.

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

Signs and symptoms

  • abnormal x-rays.
  • Hutchinson's triad
    Hutchinson's triad
    Hutchinson's triad is named after Sir Jonathan Hutchinson . It is a common pattern of presentation for congenital syphilis, and consists of three phenomena: interstitial keratitis, Hutchinson incisors, and eighth nerve deafness.- References :...

    , a set of symptoms consisting of deafness, Hutchinson's teeth
    Hutchinson's teeth
    Hutchinson's teeth are a sign of congenital syphilis. Babies with this have teeth that are smaller and more widely spaced than normal and which have notches on their biting surfaces...

     (centrally notched, widely-spaced peg-shaped upper central incisors), and interstitial keratitis
    Keratitis
    Keratitis is a condition in which the eye's cornea, the front part of the eye, becomes inflamed. The condition is often marked by moderate to intense pain and usually involves impaired eyesight.-Types:...

     (IK), an inflammation of the cornea which can lead to corneal scarring and potentially blindness.
  • mulberry molars (sixth year molars with multiple poorly developed cusps).
  • frontal bossing.
  • poorly developed maxillae.
  • enlarged liver.
  • enlarged spleen.
  • petechiae.
  • other skin rash.
  • sabre shins.
  • anemia
    Anemia
    Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...

    .
  • lymph node enlargement.
  • jaundice
    Jaundice
    Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...

    .
  • pseudoparalysis.
  • snuffles
    Snuffles
    Snuffles can refer to:* Not a common term for a runny nose - see Rhinitis.* A cartoon character - see Snuffles .* A nickname for Sirius Black in the Harry Potter books.* A type of teddy bear made by Gund - see Gund Snuffles...

    , the name given to rhinitis
    Rhinitis
    Rhinitis , commonly known as a stuffy nose, is the medical term describing irritation and inflammation of some internal areas of the nose. The primary symptom of rhinitis is nasal dripping. It is caused by chronic or acute inflammation of the mucous membrane of the nose due to viruses, bacteria or...

     in this situation. When chronic, this can lead to saddle nose
    Saddle nose
    Saddle nose is a condition associated with congenital syphilis, relapsing Polychondritis, Wegener's granulomatosis, cocaine abuse, and Leprosy, among other conditions. It is characterized by a loss of height of the nose, because of the collapse of the bridge....

     deformity.
  • rhagades
    Rhagades
    Rhagades are linear scars at the angles of the mouth and nose. They can be a result from bacterial infection of skin lesions. They are associated with congenital syphilis....

    , linear scars at the angles of the mouth and nose result from bacterial infection of skin lesions.
  • Higoumenakis sign
    Higoumenakis sign
    Higouménakis' sign is a unilateral enlargement of the sternoclavicular portion of the clavicle, seen in congenital syphilis. It's an end result of neonatal periostitis....

    , enlargement of the sternal end of clavicle in late congenital syphilis.


Death from congenital syphilis is usually through pulmonary hemorrhage
Pulmonary hemorrhage
Pulmonary hemorrhage is an acute bleeding from the lung, especially in the upper respiratory tract and the endotracheal tube. When evident clinically, the condition is usually massive, associated with bleeding in other sites as well as more than one third of the lungs...

.

Treatment

If a pregnant mother is identified as being infected with syphilis, treatment can effectively prevent congenital syphilis from developing in the unborn child, especially if she is treated before the sixteenth week of pregnancy. The child 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 (should the child have not contracted it already). However, a woman in the secondary stage of syphilis decreases her child'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
Empirical
The word empirical denotes information gained by means of observation or experimentation. Empirical data are data produced by an experiment or observation....

 observation
Observation
Observation is either an activity of a living being, such as a human, consisting of receiving knowledge of the outside world through the senses, or the recording of data using scientific instruments. The term may also refer to any data collected during this activity...

 used in context of congenital syphilis stating that the greater the duration between the infection
Infection
An infection is the colonization of a host organism by parasite species. Infecting parasites seek to use the host's resources to reproduce, often resulting in disease...

 of the mother and pregnancy
Pregnancy
Pregnancy refers to the fertilization and development of one or more offspring, known as a fetus or embryo, in a woman's uterus. In a pregnancy, there can be multiple gestations, as in the case of twins or triplets...

, the better is the outcome for the infant
Infant
A newborn or baby is the very young offspring of a human or other mammal. A newborn is an infant who is within hours, days, or up to a few weeks from birth. In medical contexts, newborn or neonate refers to an infant in the first 28 days after birth...

. Definers of a better outcome include less chance of stillbirth and of developing congenital syphilis.

External links

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