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Table of Contents   
SHORT COMMUNICATION  
Year : 2011  |  Volume : 22  |  Issue : 3  |  Page : 498
Natal teeth in an infant with congenital hypothyroidism


1 Department of Pediatrics, Sri Lakshmi Narayana Institute of Medical Sciences, Puducherry, India
2 Department of Pediatrics, JIPMER, Puducherry, India

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Date of Submission12-Oct-2010
Date of Decision18-Jan-2011
Date of Acceptance22-Feb-2011
Date of Web Publication3-Nov-2011
 

   Abstract 

Teeth erupting at birth are referred to as natal teeth. It is a common and benign finding in the neonatal period. However, they may be associated with genetic syndromes like Ellis Van Creveld syndrome and Hallermann-Streiff syndrome. We report here a case of natal teeth in an infant with congenital hypothyroidism.

Keywords: Congenital hypothyroidism, infant, natal teeth

How to cite this article:
Venkatesh C, Adhisivam B. Natal teeth in an infant with congenital hypothyroidism. Indian J Dent Res 2011;22:498

How to cite this URL:
Venkatesh C, Adhisivam B. Natal teeth in an infant with congenital hypothyroidism. Indian J Dent Res [serial online] 2011 [cited 2019 Oct 14];22:498. Available from: http://www.ijdr.in/text.asp?2011/22/3/498/87088
Teeth that are present since birth are referred to as natal teeth. The incidence of natal teeth is about 1 in 3000 live births. Lower central incisors are the most commonly noted natal teeth; however, other teeth may also erupt at birth. [1] They can cause feeding problems, ulceration of the ventral part of tongue and frenulum, loosening and risk of aspiration. Ninety percent of these teeth are primary and 10% are supernumerary. [2] Natal teeth are associated with genetic conditions like Hallermann-Strieff syndrome, Ellis Van Creveld syndrome, pachyonychia congenita, Down's syndrome, cleft lip, cleft palate, and cyclopia. We report here an interesting case of natal teeth in an infant with congenital hypothyroidism.


   Case Report Top


A 3 month old female infant who was first born to non-consanguineously wed parents by spontaneous vaginal delivery at a rural government hospital was brought with complaints of noticing difficulty in passing stools since birth. The mother did not remember the birth weight of her infant. The infant was on exclusive breastfeeds since birth.

The baby had a hoarse cry since birth and umbilical swelling since day 6 of life. There was no history of prolonged neonatal jaundice or swelling in the neck or stridor or respiratory distress. There was no history of feeding difficulty or lethargy or temperature instability. The baby had passed meconium after 48 h of life. There was no history suggestive of hypothyroidism or hyperthyroidism in the mother. The mother was not exposed to any drug or environmental toxin known to cause thyroid problems in the fetus. The baby was noted to have two teeth in the lower jaw from birth [Figure 1].
Figure 1: Infant with natal teeth

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On examination, the baby was normothermic with coarse facies, large tongue, and dry skin. The hair was sparse and brownish in color. The anterior fontanel was 2×2 cm. The posterior fontanel was closed. There was no sutural separation. There were two mandibular central incisors which were firmly attached and yellowish in color with conical edges. (There were no ulcerations in the base of the tongue nor did the mother have any soreness of nipple.) There was mild pallor and no icterus, clubbing, lymphadenopathy, or dependent edema. There was a 3×3 cm umbilical swelling which was spontaneously reducible. Anthropometric measurements revealed a length of 47 cm, weight of 3.2 kg, head circumference of 36 cm, and chest circumference of 32 cm.The upper segment/lower segment ratio was 1:1.47. Based on the above features, congenital hypothyroidism was suspected and investigated accordingly.

Roentgenogram of both knee joints revealed absent upper tibial epiphysis. An abdominal radiograph showed nonspecifi c bowel loop dilatations. Supernumerary teeth were excluded by an oral radiograph. Hemogram revealed a hemoglobin level of 8 g/dl and a differential count of 68% neutrophils, 8% eosinophils, 22% lymphocytes, and 2% stab forms. Peripheral smear revealed normocytic hypochromic RBCs with mild anisocytosis, few target cells, few polychromatic cells, neutrophilia with toxic change, mild eosinophilia and few reactive lymphocytes, and adequate platelets. The thyroid profile revealed thyroid stimulating hormone (TSH) level which was >100 microIU/ml. Neurosonogram was normal. The thyroid profile of the mother was normal. The infant was started on levothyroxine at 10 mcg/kg/day.

The natal teeth were left alone after discussing with the dentist, as theywere not loose and not interfering with feeding. On follow-up 2 months later, the infant had attained head control, was rolling over, was cooing, was passing stools daily without difficulty and the umbilical swelling was becoming less prominent. The natal teeth were not causing any problem in feeding.


   Discussion Top


Congenital hypothyroidism associated with skeletal developmental delay including delayed dentition is a well-known fact. The interesting aspect of this case is that this infant had premature eruption of teeth (natal teeth) although the upper tibial epiphysis had not yet appeared. The cause of natal teeth in hypothyroidism could possibly be explained if the mother had gestational Grave's disease, the diagnosis of which may be difficult during pregnancy. In Grave's disease, elevated maternal anti-TSH receptor antibody levels could lead to fetal hyperthyroidism and that could explain the premature dental eruption. And a hyperthyroid fetal environment would eventually cause central hypothyroidism in the infant. [3] But in our case, the TSH levels were more than 100 micro IU/ml, disproving this hypothesis. Another possible mechanism would be exposure of mother to environmental toxins like polychlorinated biphenyls (PCB). Fetal exposure to PCB leads to the development of hypothyroidism (elevated TSH) and tooth eruption at birth [4],[5] in addition to gingival hypertrophy, irregular calcification of bones, and Addison's disease. In such cases, the natal teeth are believed to erupt since the teeth find less resistance and can easily penetrate the mandible. [5] However, in our case, there was no history of exposure to PCB in the mother. The association of natal teeth and congenital hypothyroidism has been reported as a part of Hallermann-Strieff syndrome, [6] which is characterized by microcephaly, small cerebellum, underdeveloped mandible, natal teeth, beak-shaped nose, and cataracts. Natal teeth and umbilical hernia have been described as a part of Teebi hypertelorism syndrome, [7] characterized by prominent forehead, hypertelorism, broad nasal tip, long philtrum, natal teeth, low set ears, preauricular fistulas, clinodactyly of the fifth finger and umbilical hernia. Neonatal tooth has been reported in a child with Pierre Robin syndrome with congenital hypothyroidism and congenital heart defects. [8] In our case, there were no features to suggest either Hallermann-Strieff syndrome, Teebi hypertelorism syndrome, or Pierre Robin syndrome. To the best of our knowledge this is the first case report of isolated natal teeth and congenital hypothyroidism in the literature so far.


   Conclusion Top


This case report highlights the importance of screening for hypothyroidism in the neonatal period to prevent adverse developmental outcomes. Peripheral health centers and district hospitals should be provided with test kits so that they can screen for hypothyroidism more diligently. Regarding natal teeth, further observations and studies are needed to fully understand the pathogenesis of natal teeth in the context of congenital hypothyroidism.

 
   References Top

1.Bodenhoff J, Gorlin RJ. Natal and neonatal teeth: Folklore and fact. Pediatrics 1963;32:1087-93.  Back to cited text no. 1
[PUBMED]    
2.Adekoya-Sofowora CA. Natal and neonatal teeth: A review. Niger Postgrad Med J 2008;15:38-41.  Back to cited text no. 2
[PUBMED]    
3.Chan GW, Mandel SJ. Therapy insight: management of Graves' disease during pregnancy. Nat Clin Pract Endocrinol Metab 2007;3:470-8.   Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Lopez-Espinosa MJ, Vizcaino E, Murcia M, Fuentes V, Garcia AM, Rebagliato M, et al. Prenatal exposure to organo chlorine compounds and neonatal thyroid stimulating hormone levels. J Expo Sci Environ Epidemiol 2010;20:579-88.   Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Miller RW. Congenital PCB poisoning: A reevaluation. Environ Health Perspect 1985;60:211-4.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Hou JW. Hallermann-Strieff syndrome associated with small cerebellum, endocrinopathy and increased chromosomal breakage. Acta Pediatr 2003;92:869-71.  Back to cited text no. 6
    
7.Koenig R. Teebi hypertelorism syndrome. Clin Dysmorphol 2003;12;187-9.  Back to cited text no. 7
    
8.Toromanovic A, Tahirovic H. Congenital hypothyroidism associated with neonatal tooth, Pierre-Robin syndrome and congenital heart defects. J Pediatr Endocrinol Metab 2009;22:881-2.  Back to cited text no. 8
[PUBMED]    

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Correspondence Address:
C Venkatesh
Department of Pediatrics, Sri Lakshmi Narayana Institute of Medical Sciences, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.87088

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