Casereports AnnalsandEssencesof Dentistry

Vol. VI Issue 1 J an Mar 2014 19

doi:10.5368/aedj.2014.6.1.3.3
RIGA FEDE DISEASE : A CASE REPORT

1
Priya Singhal

1
Post graduate student
2
Sumit Bhateja

2
senior Lecturer
3
Manoj Vengal

3
Professor and Head


1-3
Department of Oral Medicine and Radiology, Vyas Dental College & Hospital, J odhpur(RAJ ASTHAN) ,INDIA


ABSTRACT:. The natal and neonatal teeth have been reported to cause ulceration on the ventral surface of the tongue in
neonates and infants, which may affect the childs feeding habits. This appearance was described by Riga and Fede and
hence been termed as Riga-Fede disease. We present a case report of a 3month old male infant presenting with a neonatal
tooth in the lower jaw and ulcerated ventral surface of the tongue causing difficulty in feeding.

KEYWORDS: Ri ga-Fede , Ul cerati on, Natal teeth, Neonatal teeth

INTRODUCTION

The presence of teeth in the newborns is uncommon,
varying from 1:6000- 1:800.
1
Natal teeth are those which
are present at birth and Neonatal teeth are those which
erupt during the first 30 days of life. They can cause
certain amount of morbidity to the newborns and infants
ranging from pain during suckling, swallowing to nutritional
deficiencies and occasional aspiration of the sparsely
attached natal tooth or teeth.

Case Report

A 3 month old male infant was brought to the Dept of
Oral Medicine and Radiology with a chief complaint of a
tooth present in the mandibular anterior region(Figure 1)
which erupted 10 days after the birth causing difficulty in
feeding the child. Also the mother reported that child had
discomfort during suckling. On intraoral examination a
tooth was present in the mandibular anterior region of jaw
which was milk white in color, was immobile and hard on
palpation In relation to the tooth an irregular shaped
ulcer(Figure 2) was present on the ventral surface of the
tongue measuring approx 3x4cm in its greatest diameter.
Anamnesis was non contributory. Treatment plan
formulated was extraction of the tooth(figure 3,4).Upon
follow-up examination after 1 week the ulceration was
found to be completely healed .

Di scussi on

The lesion was first described by Antonio Riga in 1881
and Francesco Fede done subsequent histological studies
in 1890.
2
Calderelli (1857) first described the condition in a
cachectic infant which was later referred to by several
Italian authors as afta cachectica.Riga-Fede is a rare
condition of benign ulceration caused by repetitive trauma
to the lingual tissues by the tooth in children younger than
two years of age.
3
Most frequently it involves the ventral
surface of the tongue or the lingual fraenum because the
tongue is raked over the teeth.

The terms natal and neonatal tooth proposed by
Massler and Savara (1950) were limited only to the time of
eruption and not to the anatomical, morphological and
structural characteristics.
4
Morphologically, natal and
neonatal teeth may be conical or may be of normal size
and shape and opaque yellow-brownish in color.
5

Spouge and Feasby (1966) classified these teeth on
the basis of clinical characteristics, into: Maturewhen
they are fully developed in shape and comparable in
morphology to the primary teeth; immaturewhen their
structure and development are incomplete.
6

Hebling(1997)classified natal teeth into 4 clinical
categories: 1. shell-shaped crown poorly fixed to the
alveolus by gingival tissue and absence of a root; 2. solid
crown poorly fixed to the alveolus by gingival tissue and
little or no root; 3. eruption of the incisal margin of the
crown through gin- gival tissue; 4. edema of gingival tissue
with an unerupted but palpable tooth
7


Etiology is unknown,but some factors may be related to
this which includes superficial position of the tooth germ,
infection or malnutrition, febrile states, hormonal
stimulation, hereditary transmission of a dominant
autosomal gene, osteoblastic activity inside the tooth germ
and hypovitaminosis. The most acceptable theory is based
on the result of the superficial localization of the dental
follicles probably related to a hereditary factor.
1

Syndromes associated with natal and neonatal teeth
include Ellis-van Creveld (chondroectodermal dysplasia),
Pachyonychiacongenita (J adassohn -Lewandowsky)
Hallerman - Streiff (occulomandibulodyscephaly with
hypotrichosis), Rubinstein - Taybi, Steatocystoma
multiplex, Pierre-Robin, Cyclopia, Pallister - Hall, Short rib
- polydactyly type II, Wiedeman - Rautenstrauch (neonatal
progeria), Cleft lip and palate, Pfeiffer, Ectodermal
dysplasia, Craniofacial dysostosis, Multiple steacystoma,
Sotos, Adrenogenital, pidermolysis bullosa simplex
including Van der Woude and Walker-Warburg
Syndromes.
8
Casereports AnnalsandEssencesof Dentistry

Vol. VI Issue 1 J an Mar 2014 20










Fi g.1. Neonatal tooth i n mandi bular anterior regi on



Fi g.2. Ul cerati on present on ventral surface of
tongue i n rel ati on to neonatal tooth


Fi g-3 and Fi g.4. Extracti on of tooth and extracted neonatal tooth respectivel y



Differential diagnosis includes granular cell tumour,
myofibroma,sarcoma,extra-nodal lymphoma,congenital
syphilis,tuberculosis, agranulocytosis,traumatic ulcer.
9
According to Zhu & King (1995) RFD by itself is not an
indication for extraction.
10
Conservative approach can be
used to deal with small ulcers.Grinding of sharp edges of
the teeth and composite coverage are conservative
treatment options. Composite resin coverage of the incisal
edges , grinding of sharp incisal edges and topical
application of Dologel grinding of the sharp incisal edges
and topical application of steroids have been the various
modalities of treatment carried out in order to resolve the
lesions. If the ulcerated area has been large even a
rounded incisal edge would be the preferred choice.
11
Extraction may be needed to alleviate feeding difficulties
or complications like Riga-Fede disease. Extraction may
also be indicated if childs age is ten days or above and
child has appropriate amounts of Vitamin K in the blood.

Otherwise prophylactic administration of vitamin K (0.5 -
1.0 mg, I,m) is advocated before and after extraction,
since vitamin K is essential for the production of
prothrombin in the liver as there could be risk of
haemorrhage.

CONCLUSION

Failure to diagnose or treat such a lesion can result in
poor nutritional status of the child, resulting in growth
retardation either it be physical or mental.therefore it is of
utmost importance for an oral physician to take a proper
history so as to recognise the condition early and manage
effectively.




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Vol. VI Issue 1 J an Mar 2014 21
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Corresponding Author


Dr. Pri ya Singhal
Department of Oral Medicine Diagnosis &
Radiology
Vyas Dental College & Hospital
J odhpur(RAJ ASTHAN) ,INDIA
Email. Id : prsinghal95@gmail.com