Bilateral lipomatous hamartoma of the tongue: A case report in a child with oral-facial-digital syndrome type VI.

Autor: Queiroz A; Department of Stomatology, School of Dentistry, University of São Paulo, São Paulo, Brazil., Pina PSS; Department of Stomatology, School of Dentistry, University of São Paulo, São Paulo, Brazil., Novaes MSP; Clinician of Special Laboratory of Laser in Dentistry (LELO), University of São Paulo, São Paulo, Brazil., Dutra B; Department of Stomatology, School of Dentistry, University of São Paulo, São Paulo, Brazil., de Sousa SCOM; Clinician of Special Laboratory of Laser in Dentistry (LELO), University of São Paulo, São Paulo, Brazil., Azevedo LH; Clinician of Special Laboratory of Laser in Dentistry (LELO), University of São Paulo, São Paulo, Brazil.
Jazyk: angličtina
Zdroj: Special care in dentistry : official publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry [Spec Care Dentist] 2024 Jul-Aug; Vol. 44 (4), pp. 1036-1040. Date of Electronic Publication: 2024 Jan 07.
DOI: 10.1111/scd.12958
Abstrakt: A hamartoma is a benign proliferation of typical mature cells specific to a particular anatomical site. In the oral cavity, they may occur as isolated cases or be associated with genetic syndromes. Oral-facial-digital syndrome type VI is a rare genetic disorder with an estimated incidence of one in 50,000-250,000 newborns. Here, we report a case of a 2-year-old boy diagnosed with oral-facial-digital syndrome type VI who was referred for evaluation of a bilateral and normochromic to slightly pinkish nodule on the lateral surface of the tongue. Clinically, the child presented hypotonia, low visual acuity, absence of oculocephalic reflex, delay in neuropsychomotor development, and polydactyly in the feet. Excisional biopsies of both sides of the tongue were performed using a 1.5 W high-power diode laser (wavelength of 980 nm), and histopathological analysis revealed abundant mature adipocytes predominantly arranged in lobules that mainly surrounded the minor salivary gland parenchyma. The surgical sites healed with no complications and the patient remains under follow-up for 10 months. Due to the limited literature on this syndrome and the frequent presence of tongue hamartomas in children, dentists need to be familiar with them.
(© 2024 Special Care Dentistry Association and Wiley Periodicals LLC.)
Databáze: MEDLINE
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