Congenital Syphilis

What is Congenital Syphilis?

Congenital Syphilis (CS) is a form of syphilis that is present in utero as well as at birth. It occurs when a mother affected by secondary syphilis gives birth to a child. Untreated syphilis leads to a greater risk of having a poorer outcome of pregnancy. It can even cause mulberry molars in fetus. Syphilis can result in miscarriages, stillbirths, premature births, or even death of the newborn babies. The symptoms of the condition may become apparent at birth, although most babies develop the symptoms later.

Babies who are untreated can suffer from:

  • Deformities
  • Developmental delays
  • Seizures
  • Rashes
  • Fever
  • Anemia
  • Hepatosplenomegaly
  • Jaundice

The sores on the infected babies are generally infectious.

Congenital Syphilis Classification

CS can be subcategorized into two types:

Early congenital syphilis

In the early form of CS, the newborns are often asymptomatic and they can only be identified by routine prenatal screening. These newborns tend to develop rhinorrhea and poor feeding if they are not detected and treated in time. Early CS generally takes place in children who are aged between 0-2 years. Symptomatic newborns are born premature with pneumonia, skeletal abnormalities, hepatosplenomegaly, as well as a bullous skin disorder referred to as pemphigus syphiliticus. The various signs associated with early CS are listed below:

General symptoms

The general symptoms include:

  • Fever
  • Failure to thrive
  • Generalized lymphadenopathy
  • Intrauterine growth retardation

Skin problems

The symptoms associated with skin include:

  • Condyloma lata
  • Cutaneous maculopapular or the vesiculobullous rashes

Bone problems

CS may affect the bones in the following ways:

  • Periostitis
  • Osteochondritis
  • Pathological features
  • Painful pseudoparalysis

Kidney problems

The symptoms related to renal functions include:

  • Glomerulonephritis
  • Nephrotic syndrome

Mucosal problems

CS affects the mucosa in the following ways:

  • Mucous patches
  • Purulent or bloody rhinitis

Ocular problems

The following eye problems occur due to CS:

Lung problems

The lung symptoms include:

  • Pneumonia

CNS problems

The CNS is affected in the following ways:

  • Leptomeningitis
  • Cerebrovascular accidents

Symptoms in Laboratory

The following symptoms are observed in the laboratory:

  • Leukopenia
  • Leukocytosis
  • CSF pleocytosis
  • Thrombocytopenia
  • CSF increased protein
  • Increased liver enzymes
  • Coombs-negative hemolytic anemia

Late congenital syphilis

Late CS occurs in children who are 2 years of age or older. The infection is acquired trans-placentally. The common signs of late-CS include:

  • Saber shins
  • Swollen knees
  • Short maxillae
  • Hydrocephalus
  • Flaring scapulas
  • Hard palate defect
  • Mental retardation
  • Protruding mandible
  • Bilateral knee effusions
  • Sternoclavicular thickening
  • Deafness from disease of the auditory nerves
  • Frontal bossing (a prominence of one’s brow ridge)
  • Saddle nose (collapsing of the bony portion of the nose)
  • Inflammations of the cornea referred to as interstitial keratitis
  • Hutchinson’s teeth, involving blunting of the upper incisor teeth

Hutchinson’s triad, consisting of the symptoms of Hutchinson’s teeth, deafness and keratitis occurs in almost 63% of all cases.

Congenital Syphilis Causes

CS occurs due to infections caused by a bacterium known as Treponema pallidum. It is passed on from mother to child during the fetal development stage or at birth. Nearly 50% of all children who are infected with syphilis during the fetal stage die shortly before or after the birth.

Congenital Syphilis Symptoms

A number of symptoms are closely associated with CS. These include:

  • Fever
  • Anemia
  • Jaundice
  • Deafness
  • Irritability
  • Petechiae
  • Skin rashes
  • Sabre shins
  • Joint swelling
  • Enlarged liver
  • Frontal bossing
  • Enlarged spleen
  • Pseudoparalysis
  • Abnormal x-rays
  • Lymph node enlargement
  • Poorly developed maxillae
  • Failure to gain weight and thrive
  • Scarring of skin around the mouth, anus and genitals
  • Mulberry molars (i.e., permanent first molars having several poorly developed cusps)
  • Higoumenakis sign, or enlargement of sternal end of the clavicle in case of late CS
  • Hutchinson’s teeth (i.e., centrally notched teeth, widely spaced peg-shaped upper central incisors)
  • Rhagades, i.e., linear scars at angles of one’s mouth and nose that results from the bacterial infections of the skin lesions
  • Interstitial keratitis (IK), which involves inflammations of the cornea that can result in corneal scarring and potential blindness
  • Snuffles or syphilitic rhinitis; although it appears similar to rhinitis of common cold, it is even more severe, lasts for a longer duration, frequently incorporates bloody rhinorrhea, and also frequently associated with laryngitis

Congenital Syphilis Diagnosis

A doctor conducts a thorough physical examination while determining whether or not a child is having CS. Physical evaluation might reveal signs of swelling of the liver and spleen as well as inflammations of the bones. If the condition is suspected at birth, a doctor will examine the placenta for the symptoms of syphilis. Routine blood tests for syphilis are done at the time of pregnancy. The following blood tests may be carried out on a mother:

  • Rapid plasma reagin (RPR)
  • Venereal disease research laboratory test (VDRL)
  • Fluorescent treponemal antibody absorbed test (FTA-ABS)

A child or an infant may have some or all of the following tests:

  • Bone x-rays
  • Eye examinations
  • Lumbar puncture tests
  • Dark-field examination for detecting the syphilis bacteria under a powerful microscope

Congenital Syphilis Treatment

If a mother is diagnosed with syphilis, proper treatment can help to prevent the development of CS on the unborn child. This is especially so if a mother is treated before 16th week of pregnancy. The risks of contracting syphilis are greatest when a mother is still in the earlier phases of infections. However, the disorder can be transmitted at any stage during pregnancy, even at the time of the delivery. A woman in secondary phase of syphilis reduces her child’s risk of having CS by around 98% if she is treated before last month of her pregnancy. Antibiotics like penicillin can be used to treat an afflicted child. However, any developmental problems that may arise will most likely be permanent.

Kassowitz’s law states that better outcome for an infant is directly related to longer duration between infection of mother and pregnancy. This reduces the chances of stillbirth as well as of developing this disorder.

Congenital Syphilis Complications

The following complications may develop in patients suffering from CS:

  • Acidosis
  • Deafness
  • Blindness
  • Pancreatitis
  • Liver failure
  • Hypoglycemia
  • Severe anemia
  • Failure to thrive
  • Respiratory failure
  • Thrombocytopenia
  • Leukemoid reaction
  • Nephrotic syndrome
  • Meningoencephalitis
  • Nonimmune hydrops
  • Neurological problems
  • Pulmonary hemorrhage
  • Necrotizing enterocolitis
  • Deformity of facial features
  • Disseminated intravascular coagulation

Congenital Syphilis Prognosis

Many infants who get infected by this disorder early in pregnancy are stillborn. Appropriate treatment of the expecting mother reduces the risk of developing this disorder in the infant. Babies who get infected while passing through birth canal generally have a better prognosis.

Congenital Syphilis Prevention

Safer sexual practices can help to prevent the development of this disease. An individual should seek immediate medical assistance if she suspects herself of having syphilis or any other sexually transmitted diseases. Prenatal diagnosis and care can reduce the risks of a child getting infected by this disorder. Infants born to infected mothers who are properly treated with Penicillin during pregnancy have minimal risk for developing CS.

Congenital Syphilis Pictures

The images underneath show the physical appearance of newborns who are infected with this disease.

Picture of Congenital Syphilis

Picture 1 – Congenital Syphilis

Image of Congenital Syphilis

Picture 2 – Congenital Syphilis Image

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