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Riga Fede disease in twins
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  1. Mebin George Mathew
  1. Pediatric and Preventive Dentistry, Saveetha Dental College, Saveetha Institute of Medical and Technical Sciences, Chennai, Tamil Nadu, India
  1. Correspondence to Dr Mebin George Mathew; mebingmathew{at}gmail.com

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Description

The presence of a tooth at birth is a rare occurrence and rarely reported in literature. Such teeth are known as natal teeth and occur in a prevalence of 1:3500.1 The presence of a natal tooth in the mandibular incisor region can lead to an extremely rare disorder known as Riga Fede disease (RFD), which is characterised by the ulceration of the ventral surface of the tongue.2 Though cases of natal teeth and RFD have been reported, the presenceof RFD in twins has not yet been reported in the literature.

A set of twins, both boys, aged 20 days reported to the department of Paediatric and preventive dentistry with chief complaint of teeth present in lower anterior region since birth and ulcer on the ventral surface of the tongue. They were full term infants and had normal weight for age at the time of presentation in the dental office. The mother complained of difficulty in breast feeding the children from the time of birth. On examination, natal tooth was seen in mandibular anterior region in both twins which were mobile. A circular ulcer, 1 × 1 cm, was seen in one twin (figure 1A), while the other twin had a circular ulcer measuring 2 × 2 cm (figure 1B). Family history was unremarkable to any developmental disorder. History and diagnosis confirmed the diagnosis of RFD. To prevent aspiration of the mobile natal teeth and allow the ulcer to heal, it was decided to extract both teeth. After obtaining consent from the parents, both teeth were extracted. After 2 weeks, both children returned for follow-up. The ulcer and extraction socket had completely healed and the mother was able to feed both children.

Figure 1

(A) Natal tooth and ulcer in twin 1. (B) Natal tooth and lingual ulcer in twin 2.

RFD is a benign condition which is caused by the repetitive forward and backward movement of tongue over mandibular incisors resulting in trauma.3 Though RFD is seen in healthy children, it has been frequently associated with neurological conditions such as microcephaly, cerebral palsy, Down syndrome, Lesch-Nyhan syndrome, Riley day syndrome and Tourette syndrome.4 Treatment for RFD is either grinding the sharp incisal edges or extraction of the tooth. Extraction of the natal tooth is usually undertaken to prevent aspiration. Failure to diagnose and treat these lesions properly may result in inadequate food intake, dehydration, growth retardation and permanent lingual deformity.5

Patient’s perspective

We had difficulty in feeding our children and felt very bad as they could not be fed well. The treatment rendered by dentist was very effective and now both our boys are well nourished.

Learning points

  • Riga Fede disease is a benign condition which requires treatment.

  • Extraction of the tooth or smoothening of incisal edges can be done.

  • Feeding of the infant is affected if treatment is not complete.

Ethics statements

Patient consent for publication

References

Footnotes

  • Contributors MGM has individually done the planning, conduct, reporting, conception and design, acquisition of interpretation of data for this case.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.