Hutchinson teeth are abnormal permanent upper central incisors that are peg-shaped and notched, usually with obvious thinning and discoloration of enamel in the area of the notching; they are widely spaced and shorter than the lateral incisors; the width of the biting surface is less than that of the gingival margin 1). Hutchinson teeth are hypoplastic notched permanent teeth, most commonly of the upper central incisors and they are smaller and more widely spaced than normal and are notched on their biting surfaces. Hutchinson teeth is a sign of congenital syphilis.
If your dentist concludes that your child does have Hutchinson teeth, the next step is a course of treatment. First and foremost, seek medical help for treating your child’s syphilis. Consultation with a pediatric infectious disease specialist will be very helpful in the workup, diagnosis, and management of patients with congenital syphilis. Next up is dealing with the abnormally formed teeth. The National Institutes of Health lists the following options 2):
- Dental restorations
Congenital syphilis is caused by transmission of the spirochete Treponema pallidum from the mother to the fetus, resulting in a multitude of clinical presentations ranging from asymptomatic, premature birth, and a wide array of clinical signs and symptoms to stillbirth 3). Rates for congenital syphilis are on the rise, with 2017 having the most cases of congenital syphilis since 1997 4). Congenital syphilis is a nationally notifiable disease with each case being reported to the Centers for Disease Prevention and Control (CDC) by all 50 states.
Most cases of congenital syphilis are seen in women without proper prenatal care or in those who receive improper treatment 5). Syphilis screening is part of routine standard of care in all pregnant women in the United States, and it has shown to decrease rates of congenital syphilis 6). Treatment with penicillin in pregnant women with syphilis is 98% effective at preventing congenital syphilis 7). While congenital syphilis can cause severe illness and fetal demise, most neonates born with congenital syphilis are asymptomatic at birth 8). However, in untreated infants clinical manifestations usually appear by three months of age and commonly include:
- Hepatomegaly: This is the most common finding and may occur with splenomegaly. Biopsy of the liver followed by darkfield microscopy may reveal the spirochete. Liver function tests may be abnormal.
- Jaundice: Jaundice may or may not be present depending on the extent of liver injury.
- Rhinitis: One of the first clinical presentations, usually in the first week of life. Copious, persistent white discharge is noted, which contains spirochetes that can be visualized under darkfield microscopy.
- Generalized Lymphadenopathy: Generalized, non-tender lymphadenopathy is also a common finding.
- Rash: Rash usually appears one to two weeks after rhinitis. Small red or pink colored maculopapular lesions may be commonly seen on the back, buttocks, posterior thigh and soles of the feet. The rash progresses to desquamation and crusting.
If there is a failure to diagnose congenital syphilis early, persistent inflammation may lead to scarring and gumma formation 9). Clinical findings in late congenital syphilis includes 10):
- Skin and mucous membrane gummas and perioral fissures/scarring
- Facial changes including frontal bossing, saddle nose, prominent maxilla
- Anterior bowing of the shin called saber shins.
- Hutchinson teeth which are hypoplastic notched permanent teeth, most commonly of the upper central incisors
- Intellectual disability and cranial nerve palsies
- Sensorineural hearing loss and changes in vision
- Eye involvement may lead to interstitial keratitis, secondary glaucoma, and corneal scarring
Hutchinson triad (Hutchinson teeth, interstitial keratitis, and sensorineural hearing loss) is relatively specific for congenital syphilis 11).
Congenital syphilis diagnosis
Maternal screening for syphilis in early pregnancy is of prime importance in preventing congenital syphilis, and it is considered the standard of care in the United States. Maternal treatment of syphilis with penicillin is 98% effective at preventing congenital syphilis 12).
The following laboratory and radiographic abnormalities can aid in the diagnosis of congenital syphilis:
- Histologic examination of the placenta and cord for the typical pathological changes and presence of spirochetes
- Darkfield microscopic examination of nasal discharge if present
- Long bone radiographs that may show findings of pathologic fractures, metaphyseal serration, localized demineralization, and osseous destruction 13).
- Chest x-ray which may show diffuse opacification of both lung fields
- Non-treponemal tests: Venereal Disease Research Laboratory (VDRL) or Rapid Plasma Reagin (RPR)
- Specific treponemal tests: Fluorescent treponemal antibody absorption (FTA-ABS) and/or micro hemagglutination test for antibodies to T.Pallidum (MHA-TP) testing
- CSF abnormalities including reactive CSF VDRL, CSF pleocytosis, elevated CSF protein
- CSF PCR for detection of treponemal DNA
Initial workup for an infant born to a woman with reactive non-treponemal and treponemal testing should follow the recommendations of the CDC 14) or American Academy of Pediatrics as listed in Red Book 15).
Congenital syphilis treatment
Treatment for congenital syphilis when the disease is confirmed or likely to be present is:
- Infants up to 4 weeks of age: Aqueous crystalline penicillin G, 50,000 units/kg per dose intravenously every 12 hours in the first seven days of life. After 7 days of life, 50,000 units/kg per dose intravenously (IV) every 8 hours for 10 to 14 days. Alternatively, procaine penicillin G, 50,000 units/kg/day intramuscularly for 10 to 14 days 16).
- Infants older than 4 weeks and older children: Aqueous penicillin G, 50,000 units/kg per dose every 6 hours intravenously for 10 to 14 days 17).
The management of an asymptomatic infant potentially exposed to syphilis but unlikely to have the disease is controversial. These are infants of mothers who received adequate treatment more than 4 weeks before delivery. However, most experts and the CDC recommends benzathine penicillin G 50,000 units/kg per dose intramuscularly (IM) in a single dose in these asymptomatic children 18).
Congenital syphilis prognosis
Excellent prognosis if diagnosed and treated appropriately in a timely fashion. Syphilis is easily treated with penicillin. However, there is an increased risk for worse outcomes and possible death in:
- Premature infants
- Those who have a delay or do not receive proper treatment
- Patients who display an extensive spread of the disease with multiple organ failure
- Infants with a severe Jarisch-Herxheimer reaction upon treatment
Congenital syphilis complications
Delayed diagnosis and treatment can lead to late, persistent clinical features of intellectual disability, skin gummas, scarring, hearing deficits and skeletal abnormalities. Initiation of treatment in some infants can lead to a Jarisch–Herxheimer reaction leading to fevers, chills, hypotension, and possibly fetal death as a result of an inflammatory response to the dying spirochetes.
References [ + ]
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|2.||↵||Tooth – abnormal shape. https://medlineplus.gov/ency/article/003064.htm|
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|10.||↵||Dorfman DH, Glaser JH. Congenital syphilis presenting in infants after the newborn period. N. Engl. J. Med. 1990 Nov 08;323(19):1299-302.|
|11.||↵||Fiumara NJ, Lessell S. Manifestations of late congenital syphilis. An analysis of 271 patients. Arch Dermatol. 1970 Jul;102(1):78-83.|
|13.||↵||Rasool MN, Govender S. The skeletal manifestations of congenital syphilis. A review of 197 cases. J Bone Joint Surg Br. 1989 Nov;71(5):752-5.|
|14.||↵||Workowski KA, Bolan GA., Centers for Disease Control and Prevention. Sexually transmitted diseases treatment guidelines, 2015. MMWR Recomm Rep. 2015 Jun 05;64(RR-03):1-137.|
|15.||↵||Red Book: 2015 Report of the Committee on Infectious Diseases, 30th ed, Kimberlin DW (Ed), American Academy of Pediatrics, Elk Grove Village, IL 2015. p.755.|
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