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CASE REPORT
Year : 2014  |  Volume : 32  |  Issue : 4  |  Page : 333-337
 

Early detection of congenital syphilis


1 Professor and Head, Departments of Pedodontics and Preventive Dentistry, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur, Karnataka, India
2 Senior Lecturer, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur, Karnataka, India
3 Professor, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur, Karnataka, India
4 Reader, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur, Karnataka, India

Date of Web Publication17-Sep-2014

Correspondence Address:
Nagalakshmi Chowdhary
Department of Pedodontics and Preventive Dentistry, Sri Siddhartha Dental College and Hospital, BH Road, Agalakote, Tumkur - 572 107, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.140969

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   Abstract 

Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson's teeth.


Keywords: Diagnosis, Hutchinson′s teeth, late congenital syphilis


How to cite this article:
Chowdhary N, Rani BK, Mukunda K S, Kiran N K. Early detection of congenital syphilis . J Indian Soc Pedod Prev Dent 2014;32:333-7

How to cite this URL:
Chowdhary N, Rani BK, Mukunda K S, Kiran N K. Early detection of congenital syphilis . J Indian Soc Pedod Prev Dent [serial online] 2014 [cited 2019 Dec 5];32:333-7. Available from: http://www.jisppd.com/text.asp?2014/32/4/333/140969



   Introduction Top


Congenital syphilis is an infectious disease transmitted by an infected mother to her fetus. It is the oldest recognized infection, and continues to account for extensive global perinatal morbidity and mortality. [1],[2],[3],[4]

Syphilis among pregnant women and the consequent congenital syphilis is now re-emerging in many developing countries. Congenital syphilis is mainly a consequence of the lack of antenatal care (ANC) and control of sexually transmitted infections. [5] The bedrock of the prevention of congenital syphilis is the performance of syphilis serological screening during pregnancy, making appropriate treatment possible, and preventing vertical transmission. [5] Globally, just over 2 million pregnant women test positive for syphilis each year, comprising 1.5% of all pregnancies worldwide. This results in 692,100-1.53 million adverse pregnancy outcomes each year caused by syphilis. Approximately, 650,000 of these pregnancy complications result in perinatal death. [6],[7],[8],[9],[10],[11]

Most of the clinical signs of congenital syphilis develop later. Manifestations of this disease include mainly the triad of Hutchinson, characterized by Hutchinson's teeth, interstitial keratitis, and eighth nerve deafness. [12] Late congenital syphilis (recognized 2 or more years after birth) is a very rare clinical entity. [13] We are reporting here a case of late congenital syphilis who presented at the age of 12 years with only Hutchinson's teeth and mulberry molars.


   Case Report Top


A 12-year-old boy reported with irregular anterior teeth. On examination, notched upper central incisors [Figure 1] and mulberry molars [Figure 2] were noticed. Furthermore, retained deciduous maxillary lateral incisors, mandibular central, and lateral incisors were noticed. On orthopantomograph examination, congenital missing of maxillary and mandibular permanent tooth buds were noticed [Figure 3]. Considering the late congenital syphilis further investigations were carried out under the guidance of a pediatrician. The routine blood investigations revealed normal Hb%, total leukocyte count, differential leukocyte count and platelet count. Venereal Disease Research Laboratory (VDRL) test (screening test) was reactive at 1:64 dilutions and Treponema pallidum hemagglutination (specific test) was positive. Skeletal survey revealed no radiological evidence of periosteal lesions or perichondritis. Ultrasound examination of abdomen and pelvis showed no abnormality. Leg anteroposterior X-ray was normal. There was no lymphadenopathy and no apparent bony abnormality. There was no evidence of neurological or cardiovascular involvement.
Figure 1: Hutchinson's teeth

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Figure 2: Mulberry molars

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Figure 3: Congenital missing of permanent teeth

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His younger brother of age 9 years was examined, and same finding were noticed. VDRL testing was reactive at 1:128 dilutions and T. pallidum hemagglutinations was positive. Consent from a parent was taken, and systemic phase of treatment was carried out by pediatrician. Both children were treated with 3 weekly doses of benzathine penicillin 2.4 million units intramuscularly. Parents were advised for necessary investigations and treatment. On the follow-up after 4 weeks, VDRL testing was negative or nonreactive.

After completing systemic phase of treatment, dental procedures are carried out in both children. As the mulberry molars are the developmental defects with increased susceptibility to caries, so as a preventive restoration stainless steel crowns are placed. Elder brother received stainless steel crown i.r.t 85 [Figure 4] followed by esthetic restoration i.r.t 11, 21 [Figure 5]. Younger brother received stainless steel crowns i.r.t 55, 65, 75, 84, 85 and band and loop space maintainer i.r.t 74 [Figure 6] and [Figure 7]. Permanent first molars were not completely erupted in both the children to place stainless steel crowns.
Figure 4: Stainless steel crown - 85

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Figure 5: Esthetic restoration of notched upper central incisors - 11, 21

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Figure 6: Stainless steel crown - 55, 65

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Figure 7: Stainless steel crown - 75, 84 and 85, band and loop space maintainer - 74

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   Discussion Top


This paper presents typical cases of asymptomatic late congenital syphilis with only Hutchison's teeth. It is very important for a dentist to have a thorough knowledge of dental findings in systemic diseases, which helps in early diagnosis and further referral to concerned specialists. The present cases were diagnosed based only on the dental finding such as Hutchison's teeth and mulberry molars during school dental check-up. By this early diagnosis and systemic phase of treatment helped us in preventing a child from further complications, which could have been fatal. In 21 st century when it is thought to be eradicated, it is re-emerging due to pitfalls in ANC or screening.

The origins of syphilis have been discussed for many centuries. Two main theories have been proposed - the New World or Columbian theory and the Old World or pre-Columbian theory. The former holds that the syphilis was endemic in the part of the world now known as Haiti and was then acquired and carried to Europe by Columbus in the 1400s. The pre-Columbian theory purports that the syphilis originated in central Africa and was introduced to Europe prior to the voyage by Columbus. A third theory, the Unitarian theory, could be made to fit the pre-Columbian theory. This theory proposed that syphilis and the nonvenereal treponematoses were all manifestations of the same infection, with the observed clinical differences being due mainly to environmental factors, especially temperature. [14],[15] However, recent bacteriological work has demonstrated genetic differences between these organisms. [16] Regardless of the origins, however, it remains clear that by 1495, a widespread syphilis epidemic had spread throughout Europe. From there the disease spread to India in 1498. [15]

In 1905, the association of T. pallidum with syphilis was described by Schaudinn and Hoffman, who demonstrated spirochetes in Giemsa-stained smears of fluid from secondary syphilitic lesions. August von Wassermann devised a serum reaction test for syphilis in 1906, and serologic tests for syphilis were born. [14] Treatments for syphilis included mercury, organic arsenical compounds, and bismuth until the advent of penicillin. [17] In 1943, Mahoney et al. successfully treated the first four cases of syphilis with penicillin, and more than half a century later penicillin remains the drug of choice. [18] In our present cases, benzithene penicillin of 2.4 million unit was administered by pediatrician for 3 weeks.

Congenital syphilis occurs when T. pallidum crosses the placenta from an infected mother to the fetus during pregnancy or by contact with an infectious lesion during birth. Manifestations of congenital syphilis are divided into early and late signs based on the first 2 years of life. [1]

Late congenital syphilis is actually very rare and occurs in approximately 40% of untreated children. Syphilitic vasculitis around the time of birth can lead to dental abnormalities that occur in teeth that undergo calcification during the 1 st year of life. [19],[20] Sir Jonathan Hutchinson (1828-1913) from England described a triad in late congenital syphilis consisting of Hutchinson's teeth, interstitial keratitis and eighth nerve deafness. [4] Hutchinson's teeth are peg-shaped, notched central incisors, while mulberry molars are multicuspid molars. The deciduous teeth have an increased risk of dental caries. [21],[22]

The dental defect represents a single disruption to tooth crown formation a few weeks after birth or during the later development of the teeth; [23],[24] however, they only become apparent with the eruption of the permanent incisors and first molars around 6 years of age. [24],[25],[26] That is why this particular form of dental defect was not found on deciduous teeth. This process of dental defect is explained by inflammatory reaction induced by T. pallidum. The invasion of bacteria to proximity of dental germ in development contact to inhibition of ameloblasts, the cells responsible for the formation of tooth enamel. Permanent upper central incisor teeth often provide evidence for the diagnosis of late congenital syphilis. [24]

Interstitial keratitis is the typical ocular manifestation, usually diagnosed between 5 and 20 years of age. It can lead to secondary glaucoma or corneal clouding. [20] Eighth nerve deafness occurs in 3% of cases and is secondary to luetic involvement of the temporal bone. Eighth nerve involvement can be unilateral or bilateral, and it may be responsive to corticosteroids. Although it is usually diagnosed between 30 and 40 years of age, it often occurs in the first decades. [27]

A recent data from World Health Organization states that only 68% of women in developing countries receive ANC and of these about half do not attend ANC clinics until after the first trimester. [28] Current recommendations for the control of congenital syphilis include the performance of VDRL in the first prenatal appointment, with a repeat test at the beginning of the 3 rd semester and at birth. [29],[30] Adequate treatment for pregnant women is the best alternative, since this will prevent fetal infection, or promote cure before delivery. If prenatal treatment is not performed, the child must receive treatment as soon as possible during the neonatal period. [30]


   Conclusion Top


This paper high lightens the awareness of the importance of dental findings in systemic disease of late congenital syphilis, which is a very rare clinical entity and its importance to make early diagnosis, and its proper management can prevent further complications. Although an effective treatment is available since the introduction of penicillin in the mid-20 th century, it still remains as an important public health problem. These case reports of late congenital syphilis are a tragic reflection of pitfalls in the screening programs. More emphasis must be placed on primary prevention and appropriate screening programs so that maternal and neonatal syphilis can be identified and treated early, avoiding significant future morbidity.

 
   References Top

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6.Chakraborty R, Luck S. Syphilis is on the increase: The implications for child health. Arch Dis Child 2008;93: 105-9.  Back to cited text no. 6
    
7.Schmid GP, Stoner BP, Hawkes S, Broutet N. The need and plan for global elimination of congenital syphilis. Sex Transm Dis 2007;34:S5-10.  Back to cited text no. 7
    
8.Schmid G, Rowley J, Samuelson J, Tun Y, Guraiib M, Mathers C, et al. Global incidence and prevalence of four curable sexually transmitted infections (STIs): New estimates from WHO. In: Proceedings of the Global HIV/AIDS Surveillance Meeting (ISSTDR '09). London, UK; 2009.  Back to cited text no. 8
    
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11.Schulz KF, Cates W Jr, O'Mara PR. Pregnancy loss, infant death, and suffering: Legacy of syphilis and gonorrhoea in Africa. Genitourin Med 1987;63:320-5.  Back to cited text no. 11
    
12.World Health Organization. World Health Organization Global Burden of Disease Report, Geneva, Switzerland: WHO; 2002.  Back to cited text no. 12
    
13.Pessoa L, Galvão V. Clinical aspects of congenital syphilis with Hutchinson's triad. BMJ Case Rep. 2011 Dec 21; 1-3, 2011. pii: bcr1120115130. doi: 10.1136/bcr.11.2011.5130.  Back to cited text no. 13
    
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15.Singh AE, Romanowski B. Syphilis: Review with emphasis on clinical, epidemiologic, and some biologic features. Clin Microbiol Rev 1999;12:187-209.  Back to cited text no. 15
    
16.Oriel JD. The Scars of Venus. London, England: Springer-Verlag; 1994.  Back to cited text no. 16
    
17.Centurion-Lara A, Castro C, Castillo R, Shaffer JM, Van Voorhis WC, Lukehart SA. The flanking region sequences of the 15-kDa lipoprotein gene differentiate pathogenic treponemes. J Infect Dis 1998;177:1036-40.  Back to cited text no. 17
    
18.Mahoney JF, Arnold RC, Harris A. Penicillin Treatment of Early Syphilis-A Preliminary Report. Am J Public Health Nations Health 1943;33:1387-91.  Back to cited text no. 18
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20.De Santis M, De Luca C, Mappa I, Spagnuolo T, Licameli A, Straface G, et al. Syphilis Infection during pregnancy: Fetal risks and clinical management. Infect Dis Obstet Gynecol 2012;2012:430585.  Back to cited text no. 20
    
21.Ingall D, Sánchez PJ. Syphilis. In: Remington JS, Klein JO, editors. Infectious Diseases of the Fetus and Newborn Infant. 5 th ed. Philadelphia: W.B. Saunders; 2001. p. 643-81.  Back to cited text no. 21
    
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23.Curtis AC, Philpott OS. Prenatal syphilis. Med Clin North Am 1964;48:707-19.  Back to cited text no. 23
    
24.Hillson S, Grigson C, Bond S. Dental defects of congenital syphilis. Am J Phys Anthropol 1998;107:25-40.  Back to cited text no. 24
    
25.Bernfeld WK. Hutchinson's teeth and early treatment of congenital syphilis. Br J Vener Dis 1971;47:54-6.  Back to cited text no. 25
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26.Bauer WH. Tooth buds and jaws in patients with congenital syphilis: Correlation between distribution of Treponema pallidum and tissue reaction. Am J Pathol 1944;20:297-319.  Back to cited text no. 26
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27.Rothenberg R, Becker G, Wiet R. Syphilitic hearing loss. South Med J 1979;72:118-20.  Back to cited text no. 27
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29.Centers for Disease Control (CDC). Guidelines for the prevention and control of congenital syphilis. MMWR Morb Mortal Wkly Rep 1988;37 Suppl 1:1-13.  Back to cited text no. 29
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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