CASE REPORT Journal of Oral Medicine, Oral Surgery, Oral Pathology and Oral Radiology;2015;1(1):36-38 36 AN ASSOCIATED POLYDACTYLY WITH RIGA – FEDE DISEASE: A RARE CASE REPORT Suman Basavraju 1 , Kanika Gupta Verma 2 , Metashi Singla 3 , Pradhuman Verma 4, * 1 Reader, Department of Periodontology; JSS Dental College, Mysore, Karnataka 2 Reader, 3 P.G Student, Department of Pedodontics & Preventive dentistry; Surendera Dental College, Sriganganagar, Rajasthan 4 Reader, Department of Oral Medicine & Radiology, Surendera Dental College, Sriganganagar, Rajasthan *Corresponding Author: Email: pradhuman_verma@rediffmail.com ABSTRACT: Riga-Fede disease occurs during early infancy and is characterized by a reactive traumatic lesion on ventral surface of the tongue. It results due to raking motion of tongue over the recently erupted natal, neonatal or primary mandibular incisors. The lesion causes pain during feeding practice, leading to nutritional deficiencies and retarded growth. The management includes conservative approach or extraction that promotes healing. This case report highlights the association of Riga-Fede disease with polydactyly in a two and half months old male child, along with the therapeutic approach to the condition. Keywords: Riga Fede disease, Traumatic ulcer, Neonatal teeth, Polydactyly. INTRODUCTION Riga-Fede disease occurs during early infancy and is characterized by a reactive traumatic lesion on ventral surface of the tongue 1 . It is an uncommon self-limiting persistent ulceration of the oral mucosa. The lesion was first described clinically by Riga in 1881 and in 1890 Fede added its histological description, thus named as Riga-Fede disease 2 . The ulceration usually involves the ventral surface of tongue due to raking motion over the incisal edges of recently erupted natal, neonatal or primary mandibular incisors 3 . However the buccal mucosa, lingual frenum, floor of mouth, labial mucosa and even dorsal surface of tongue can get involved 4,5 . Lesion appears as ulcer but can progress to an enlarged, fibrous mass with appearance of an ulcerative granuloma 3 . Usually, the lesion is seen within 6-8 months of age, being associated with natal or neonatal teeth, but it may also occur in infants upto 2 years of age due to repetitive tongue-thrusting habits and in children with familial dysautonomia 6-8 . The incidence of Riga-Fede disease in the presence of natal/neonatal teeth has been reported to be 6% to 10% 8 . The disease is associated with infection, dehydration, feeding problems leading to nutritional deficiencies. Thus, immediate management of disease is required, that includes composite addition or contouring of sharp incisal edges, extracting the associated tooth and modifying feeding behaviour with certain feeding appliances 9 . An early accurate diagnosis is mandatory to differentiate Riga-Fede disease from serious neurologic and hereditary disorders that also present similar oral ulcerations 10 . The present case report describes a rare occurrence of Riga Fede disease with polydactyly, and highlights its symptomatology and therapeutic approach. CASE REPORT A two and half months old boy was referred to the Department of Pedodontics and Preventive Dentistry by his paediatrician, with difficulty during feeding due to the presence of mandibular anterior teeth since birth. The infant's mother was concerned about the ulceration present on ventral surface of tongue, which she noticed around three weeks back. She observed that a feeding problem has developed and child cries often during feed. There was no history of any systemic problems, fever, dehydration and nutritional deficiencies. Prenatal, post natal and family history revealed no relevant findings. No developmental disorders or congenital syndromes were observed in his immediate family. General physical examination revealed an extra finger on both the hands of child (Figure 1). Intraoral examination revealed two natal teeth were present in the mandibular anterior region. The teeth were firmly attached and had sharp incisal edges that caused ulceration on the ventral surface of tongue. The lesion was round in shape having 2cm diameter, it was erythematous and raised, with an indurated border covered by yellowish white slough (Figure 2). There was no associated bleeding with the lesion. A biopsy of the lesion was not attempted due to the infant’s young age. However, a diagnosis of Riga- Fede disease was made because of the distinctive clinical signs of disease and associated natal teeth. Although literature reported various cases of Riga Fede disease, but an association of polydactyly with Riga-Fede disease makes this case rare in occurrence.