Ameloblastoma in a Three-Year-Old Child with Hurler Syndrome (Mucopolysaccharidosis Type I)

Mucopolysaccharidoses (MPS) are a family of genetic diseases associated with deficiency alpha-L iduronidase, which causes lack catabolism glycosaminoglycans (GAGs). Therefore, the accumulation GAGs determines wide spectrum symptoms, typically found in few syndromes like Hurler syndrome (HS). Among other specific manifestations, craniofacial abnormalities crucial for characterization this syndrome. Ameloblastoma is rare, benign, slow-growing, odontogenic tumor usually located mandible. Clear risk factors development ameloblastoma remain unknown, but black patients have fivefold increased risk. Clinically, it characterized by painless, variable-sized jaw swelling. Although classified as benign tumor, often has severe clinical outcome. The most common type solid/multicystic/conventional one. A computed tomography scan (CT) and without contrast gold standard evaluating kind neoplasia. Conservative or radical surgery mainstay treatment. In case report, we described an unusual assessment conventional interesting posterior left mandible 35-month-old child affected HS. This represented suggestive challenge both from diagnostic therapeutic point view. patient was disease-free at 2 years’ follow-up.