Congenital syphilis

Congenital syphilis
Classification and external resources
Notched incisors known as Hutchinson's teeth which are characteristic of congenital syphilis
ICD-10	A50
ICD-9	090
DiseasesDB	12744
eMedicine	ped/2193
MeSH	D013590

Congenital syphilis is 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 molars in the fetus. Syphilis can cause miscarriages, premature births, stillbirths, 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, anemia, and jaundice. 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.^[1]

Classification

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

Symptomatic newborns, if not stillborn, are born premature, with hepatosplenomegaly, skeletal abnormalities, pneumonia and a bullous skin disease known as pemphigus syphiliticus.^[3]

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^[4]

• blunted upper incisor teeth known as Hutchinson's teeth
• inflammation of the cornea known as interstitial keratitis
• deafness from auditory nerve disease
• frontal bossing (prominence of the brow ridge)
• saddle nose (collapse of the bony part of nose)
• hard palate 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.^[4]

Treatment (with penicillin) before the development of late symptoms is essential.^[5]

Signs and symptoms

• abnormal x-rays.
• Hutchinson's triad, a set of symptoms consisting of deafness, Hutchinson's teeth (centrally notched, widely-spaced peg-shaped upper central incisors), and interstitial keratitis (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.
• lymph node enlargement.
• jaundice.
• pseudoparalysis.
• snuffles, the name given to rhinitis in this situation. When chronic, this can lead to saddle nose deformity.
• rhagades, linear scars at the angles of the mouth and nose result from bacterial infection of skin lesions.
• Higoumenakis sign, enlargement of the sternal end of clavicle in late congenital syphilis.

Death from congenital syphilis is usually through pulmonary hemorrhage.

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.^[6] 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 pregnancy, the better is the outcome for the infant. Definers of a better outcome include less chance of stillbirth and of developing congenital syphilis.^[7]

References

1. ^ http://www.niaid.nih.gov/factsheets/stdsyph.htm accessed 10/17/2006.
2. ^ http://www.pitt.edu/~SUPER1/lecture/lec13881/008.htm
3. ^ eMedicine - Syphilis : Article by Muhammad Waseem.
4. ^ ^{a b} http://www.pitt.edu/~SUPER1/lecture/lec13881/016.htm
5. ^ Sexually Transmitted Diseases Treatment Guidelines - 2002
6. ^ Congenital syphilis.
7. ^ Singh, Ameeta E.; Barbara Romanowski (1 April 1999). "Syphilis: Review with Emphasis on Clinical, Epidemiologic, and Some Biologic Features". Clinical Microbiology Reviews 12 (2): 187–209. PMC 88914. PMID 10194456. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=88914. Retrieved 2008-06-26. 

External links

• Images of Congenital Syphilis Manifestations
• 00655 at CHORUS