Abstract

Hunter's syndrome is a mucopolysaccharidosis that compromises cell function and generates tissue accumulations of glycosaminoglycans, which can lead to death, especially in severe forms. However, patients generally reach adulthood, in a way that emphasizes the need for medical and dental care in this period in the face of changes, which commonly include: progressive cognitive limitation, breathing difficulties due to pulmonary and airway disorders, and cardiac and skeletal disorders. It is noticed mainly difficulty in mouth opening, dental positioning, and bone configuration alteration in dental practice. Enzyme replacement therapy effectively reduces urinary glycosaminoglycans and the volume of the liver and spleen. In contrast, cardiac and joint results are variable and are generally unsatisfactory about the heart valves, trachea and bronchi, hearing, and eyes. The follow-up is multidisciplinary, and in dental practice, the concern is with breathing difficulties and limited mouth opening, which can make it difficult to treat oral needs that are not necessarily related to the syndrome..