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Persistent lingual ulceration (Riga-Fede disease) in an infant with Down syndrome and natal teeth: a case report.

Senanayake MP, Karunaratne I - J Med Case Rep (2014)

Bottom Line: Riga-Fede disease is a rare pediatric condition in which chronic lingual ulceration results from repetitive trauma.The lesion had progressed over the past six months.Awareness of the benign nature of this rare condition by pediatricians and dental practitioners is important as it will allay anxiety and avoid unnecessary biopsy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatrics, Faculty of Medicine, University of Colombo, Kynsey Road, Colombo 008, Sri Lanka. Manouri.senanayake@gmail.com.

ABSTRACT

Introduction: Riga-Fede disease is a rare pediatric condition in which chronic lingual ulceration results from repetitive trauma. Neonatal teeth or underlying neuro-developmental disorders which include Down syndrome are described as causative factors, but to the best of our knowledge, this is the first case report of both Down syndrome and natal teeth coexisting. The need for early extraction in the presence of two risk factors is highlighted in this case report.

Case presentation: An 18-month-old Sinhalese male presented with an ulcerating lingual mass on the ventral surface of the tongue. The lesion had progressed over the past six months. He also had clinically diagnosed Down syndrome.The ulcer was non-tender, indurated, and had elevated margins. It was not bleeding and two natal teeth in lower central dentition were seen in apposition with the lesion. There was no regional lymphadenopathy but the ulcer was causing concerns as it mimicked a malignant lesion. A clinical diagnosis of Riga-Fede disease caused by raking movements of the tongue against anterior natal teeth by a child who was developmentally delayed and prone to suck on his tongue was made. The mother was reassured and the natal teeth were extracted.

Conclusions: Early extraction of natal teeth is recommended only if there is a risk of aspiration or interference with breast feeding. Although Down syndrome is among the neuro-developmental conditions that lead to this lesion, its occurrence is usually at an older age. The presence of natal teeth together with Down syndrome caused the lesion to occur in infancy. Awareness of the benign nature of this rare condition by pediatricians and dental practitioners is important as it will allay anxiety and avoid unnecessary biopsy. This case also highlights the impact of two risk factors and needs consideration as an added indication for the early extraction of natal teeth.

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Riga-Fede disease in a child with Down syndrome.
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Figure 2: Riga-Fede disease in a child with Down syndrome.

Mentions: An 18-month-old Sinhalese male with clinically diagnosed Down syndrome was presented to our institution due to concerns regarding a persistent ulcerating lingual mass on the ventral surface of his tongue. The ulcer had first been noticed around age of 11 months. He was otherwise in good health apart from occasional upper respiratory tract infections. There had been no difficulty in feeding and his tongue movements were normal. He was delayed in speech, as well as other milestones such as motor skills and hand use. He was not receiving any regular developmental therapy. He had been tested and found negative for hypothyroidism at birth and again at six months. There had been no cardiac or any other congenital abnormalities detected. The echocardiography had been normal.The ulcer was non-tender, indurated, not bleeding, and was in apposition with two natal teeth in lower central dentition (FiguresĀ 1 and 2). There was no regional lymphadenopathy. A clinical diagnosis of Riga-Fede disease due to repetitive trauma resulting from raking movements of the tongue against anterior natal teeth was made. The mother was reassured and the natal teeth were extracted.


Persistent lingual ulceration (Riga-Fede disease) in an infant with Down syndrome and natal teeth: a case report.

Senanayake MP, Karunaratne I - J Med Case Rep (2014)

Riga-Fede disease in a child with Down syndrome.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4150422&req=5

Figure 2: Riga-Fede disease in a child with Down syndrome.
Mentions: An 18-month-old Sinhalese male with clinically diagnosed Down syndrome was presented to our institution due to concerns regarding a persistent ulcerating lingual mass on the ventral surface of his tongue. The ulcer had first been noticed around age of 11 months. He was otherwise in good health apart from occasional upper respiratory tract infections. There had been no difficulty in feeding and his tongue movements were normal. He was delayed in speech, as well as other milestones such as motor skills and hand use. He was not receiving any regular developmental therapy. He had been tested and found negative for hypothyroidism at birth and again at six months. There had been no cardiac or any other congenital abnormalities detected. The echocardiography had been normal.The ulcer was non-tender, indurated, not bleeding, and was in apposition with two natal teeth in lower central dentition (FiguresĀ 1 and 2). There was no regional lymphadenopathy. A clinical diagnosis of Riga-Fede disease due to repetitive trauma resulting from raking movements of the tongue against anterior natal teeth was made. The mother was reassured and the natal teeth were extracted.

Bottom Line: Riga-Fede disease is a rare pediatric condition in which chronic lingual ulceration results from repetitive trauma.The lesion had progressed over the past six months.Awareness of the benign nature of this rare condition by pediatricians and dental practitioners is important as it will allay anxiety and avoid unnecessary biopsy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatrics, Faculty of Medicine, University of Colombo, Kynsey Road, Colombo 008, Sri Lanka. Manouri.senanayake@gmail.com.

ABSTRACT

Introduction: Riga-Fede disease is a rare pediatric condition in which chronic lingual ulceration results from repetitive trauma. Neonatal teeth or underlying neuro-developmental disorders which include Down syndrome are described as causative factors, but to the best of our knowledge, this is the first case report of both Down syndrome and natal teeth coexisting. The need for early extraction in the presence of two risk factors is highlighted in this case report.

Case presentation: An 18-month-old Sinhalese male presented with an ulcerating lingual mass on the ventral surface of the tongue. The lesion had progressed over the past six months. He also had clinically diagnosed Down syndrome.The ulcer was non-tender, indurated, and had elevated margins. It was not bleeding and two natal teeth in lower central dentition were seen in apposition with the lesion. There was no regional lymphadenopathy but the ulcer was causing concerns as it mimicked a malignant lesion. A clinical diagnosis of Riga-Fede disease caused by raking movements of the tongue against anterior natal teeth by a child who was developmentally delayed and prone to suck on his tongue was made. The mother was reassured and the natal teeth were extracted.

Conclusions: Early extraction of natal teeth is recommended only if there is a risk of aspiration or interference with breast feeding. Although Down syndrome is among the neuro-developmental conditions that lead to this lesion, its occurrence is usually at an older age. The presence of natal teeth together with Down syndrome caused the lesion to occur in infancy. Awareness of the benign nature of this rare condition by pediatricians and dental practitioners is important as it will allay anxiety and avoid unnecessary biopsy. This case also highlights the impact of two risk factors and needs consideration as an added indication for the early extraction of natal teeth.

Show MeSH
Related in: MedlinePlus