Riga-Fede Disease – A Review

INTRODUCTION

Riga – Fede is an interesting and quite characteristic disease. Ulcers of the lingual frenum and mucosa in neonates with natal and neonatal lower incisors are referred to as Riga-Fede disease¹.

For the past 200 years, many reports of infants born with teeth or teeth erupting immediately after birth have appeared in medical and dental literature. These teeth have been referred as 'natal teeth', 'congenital teeth', and 'dentitio praecox'². In modern literature, Massler and Savara defined natal teeth as teeth present in the oral cavity at birth and 'neonatal teeth' those which erupt during neonatal period ie from birth to thirtieth day of life. They suggested that ulceration on the ventral surface of the tongue caused by these teeth is a consequence of the fact that the tongue in infants lies between the alveolar ridges³.

In the general population, the prevalence of natal or neonatal teeth ranges from 1:1000 to 1:30,000 depending upon the type of study⁴. The incidence of natal teeth is usually quoted in the range of 1:2000 to 1:3500 live births⁵. Leung⁶ reported the incidence of natal teeth to be 1:3392 live births. Kates, Needleman and Holmes in their study reported incidence of 1:716 live births⁷. In both studies, all cases involved teeth in the mandibular incisor region. Riga fede disease is seen in 6-10 percent cases of natal teeth^8,9. Riga – Fede disease may also occur in older infants after the eruption of primary lower incisors with repetitive tongue thrusting habits¹⁰ and in children with familial dysautonomia (insensitivity to pain).¹¹

An important aspect is that this lesion may be the initial presentation for some serious underlying medical problems; therefore, it is imperative to review the pertinent medical and dental literature.

TERM

In 1857 Cardarelli described the condition, which was later referred to by several Italian authors as “afta cachettica”. Its chief characteristics were ulceration on the lingual frenum of infants associated with general wasting, leading to “exitus lethalis”. Antonio Riga, an Italian physician in 1881, was the first to really draw the medical world's attention to the malady, while the first histologic examination was that of F. Fede in 1890¹². It has subsequently been known as “Riga-Fede disease”.

Other authors have referred it as “Riga's disease”, “sublingual growth in infants”, “sublingual ulcer”, “sublingual granuloma”, “reparative lesion of the tongue”, “neonatal sublingual traumatic ulceration”, and “traumatic atrophic glossitis”^1,13,14

This lesion was of particular interest in Italy because it was frequently associated with malnourished infants and often resulted in death^12,15. In the United States, it was noted that the lesion was primarily seen in the healthy infants shortly after the eruption of primary lower incisors.^12,15

ETIOLOGY

Natal teeth present with hypoplastic enamel and underdeveloped roots, with resultant mobility hence, causing trauma to the oral soft tissues¹⁶. The most common cause for this lesion is from mechanical trauma produced by the tongue thrusting against erupted teeth during nursing.¹⁷ Constant trauma may create ulceration sufficient to interfere with proper suckling and feeding and put the neonate at risk for nutritional deficiencies.¹⁶

Some reports related an association with a sharp tooth, a dental restoration, or a dental appliance¹⁸. This possible etiology is supported by the fact that the most frequent location is the tongue. Since, the tongue is an exceptionally mobile and functional structure; it gets frequently traumatized during mastication and phonation¹⁷.

CLINICAL PRESENTATION

Typically the lesion begins as an ulcerated area on the ventral surface of the tongue with repeated trauma, it may progress to an enlarged, fibrous mass with appearance of an ulcerative granuloma. It may interfere with proper suckling and feeding and put the neonate at risk for nutritional deficiencies¹⁶. In an infant, pain associated with an ulcerated oral lesion often results in dehydration, feeding difficulties and failure to thrive¹⁰. In such cases, dental intervention may be required.

HISTOLOGIC FEATURES

It has been described as fibroblastic and histiocytic proliferations, which infiltrate between striated muscle bundles. Degenerating and regenerating muscle cells and varying number of eosinophils, neutrophils, and plasma cells are also seen¹⁴.

ASSOCIATED MEDICAL DISORDERS

Riga-Fede disease occurs almost exclusively in children with cerebral palsy. It persists in infants who have severe cerebral palsy and who cannot control spasticity of the tongue hence the repeated ulcerations¹⁹. Besides the possibility of cerebral palsy, Lesch-Nyhan syndrome, familial dysautonomia, or congenital indifference to pain may be the underlying problem and the tongue biting may be the initial presentation. ^20,21

Lesch–Nyhan syndrome (LNS) is an X-linked recessive disorder of purine metabolism, which manifests clinically as hyperuricemia, growth impairment, spasticity, mental retardation and self-mutilation²². Oro-dental abnormalities are quite frequent in familial dysautonomia. Self- injurious behavior is caused mainly by profound sensory loss¹⁷. Rakocz et al¹¹ and Eichenfleld et al²² reported case of familial dysautonomia with Riga-Fede disease.

Congenital indifference to pain is also usually manifested in children by a history of unrecognized trauma, indifference to painful stimuli, or self-mutilation²³. Awareness to orodentalself-mutilation as the primary teeth erupt in these patients is important as the patient is usually normal with respect to intelligence, development, psychological adjustments and other sensory perceptions²¹.

DIFFERENTIAL DIAGNOSIS

A lesion with similar clinical characteristics and identical histological features has been reported as “traumatic eosinophilic granuloma”.²⁴ However, the latter lesion has been reported to occur during late adulthood and not restricted in location to the tongue; it may occur in the cheek-buccal mucosa, the vestibule, gingiva, or palate. Since these lesions have identical histomorphology and clinical characteristics and frequently associated with a history of trauma, the term “traumatic ulcerative granuloma with stromal eosinophilia” (TUGSE) was proposed by Elzay.¹⁸

TREATMENT

The primary objective should be to eliminate the source of trauma so that healing should take place¹⁰. The treatment of the lesion has varied over the years. Historically, the most common modality of treatment has been excision of the lesion and/or extraction of the offending teeth.¹⁸

The child should be assessed daily for dehydration. If conservative treatment options do not lead to rapid resolution of this lesion, it may be necessary to extract the lower incisors. It is usually not necessary to remove the lesion itself, as it will normally resolve after the trauma is eliminated. However, if the lesion persists after the removal of teeth, an excisional biopsy should be performed.¹⁰

Feeding methods should be employed, so that trauma to the tongue may be reduced. Bottle with a larger hole in the nipple or a sippy cup that requires less vigorous sucking can be used. Depending on the age of infant, the parent can also attempt feeding by spoon to minimize trauma to the tongue.¹⁰

Bhaskar and Lilly²⁵ reported a case treated by radiation. Welbom²⁴ reported a case which received no treatment. All cases were reported to have healed without recurrence.

The disease can also be managed in some cases without extraction²⁶. Elimination of the sharp edges of teeth was found to be helpful. Light cured composite resin (dental restorative material) can also be used to provide a smooth rounded surface for the tongue to pass over. However, inadequate enamel surface area for resin bonding, difficulties of field control, achieving adequate retention for composite resin is questionable. If the restoration fails, the composite resin could be swallowed or inhaled. Parents should be made aware of this possible complication.¹⁷

Baghdadi et al¹⁷ reported a case of a Riga – Fede disease in a ten-month old infant male with lower central incisors. The ulcer resolved after the sharp incisal edges were smoothened and topical triameinolone was applied. 

Hegde et al¹⁶ reported a case of twenty eight day old female with an ulcerated area on the ventral surface of the tongue, with two neonatal teeth in the mandibular anterior region. Mother complained of child exhibiting pain during suckling. Extraction of the teeth was carried out under topical anesthesia which was tolerated well by the patient. The lesion fully resolved after ten days. summarizes the various treatment options.

CONCLUSION

Riga-Fede disease is most commonly associated with natal and neonatal teeth. The lesion is present on the ventral surface of the tongue as an ulcerated area that results in difficulty in suckling and feeding. The pediatrician's concern over the infant's failure to gain weight due to the ulceration's interference with suckling warrants rapid attention. A close collaboration between the medical and dental teams would help to provide optimum care to the neonate/infant with the rapid resolution of the symptoms.