The second case describes an eight-year-old male with mucopolysaccharidosis type II (Hunter Syndrome). His metabolic management was weekly intravenous idursulfase. Additionally, he had autistic spectrum disorder and behavioral difficulties. He was referred to the dental department by his metabolic consultant for odontogenic pain. Upon questioning, he had a highly cariogenic diet and was regularly drinking honey mixed with water. On examination, mandibular second primary molars and first permanent molars were grossly carious. The maxillary first permanent molars and second primary molars also had caries and maxillary primary canines were mobile (Figures , ). He received dental treatment under general anesthesia, where an attempt was made to restore the mandibular right first permanent molar (LR6). The caries, however, was into the pulp and therefore, a decision was made to extract. Fissure sealants were placed on the right permanent premolars (UR5, UR4, LR5, LR4) and the maxillary left first premolar (UL4). The other premolars were partially erupted. All first permanent molars were extracted due to gross caries (UR6, UL6, LR6, LL6) in addition to maxillary primary second molars and canines (URE, URC, ULC, ULE). Regarding prevention, 22 600 ppm fluoride varnish was applied in addition to dietary counselling and oral hygiene instruction.
Case four describes a seven-year-old male with mucopolysaccharidosis type IIIB (Sanfilippo Syndrome). Medical comorbidities included mitral and aortic valve dysplasia, intellectual disability, behavioral challenges, abnormal gait, and impaired hearing. On clinical examination, he had coarse facial features and hirsutism.\nHe was referred to the dental department for gross caries (Figure ) and received comprehensive dental treatment under general anesthesia. This included composite restoration and fissure sealant provision of the maxillary left second primary molar (ULE) (Figure ) and multiple extractions; mandibular primary molars and canines (LRE, LRD, LRC, LLC, LLD, LLE), mandibular left primary central incisor (LLA), maxillary right primary lateral incisor (URB), and left central incisor (ULA). He also received 22,600ppm fluoride application.