The third patient describes a twelve-year-old female who was referred internally from her metabolic consultant concerning an acute apical abscess. Medically, she had mucopolysaccharidosis IIIA (Sanfilippo Syndrome). Medical comorbidities included gastroesophageal reflux, megarectum, chronic constipation, reduced visual acuity, and behavioral and learning difficulties. Medication included lansoprazole, macrogol, and aripiprazole. She was under the care of a metabolic dietician who prescribed frequent fruit smoothies and fruit yoghurt consumption to aid bowel movements.\nComprehensive oral examination was constrained by difficulties in cooperation. Fortuitously, compliance permitted lateral oblique radiography (Figure ) and a diagnosis of acute apical abscess of the left mandibular first permanent molar (LL6) and second primary molar (LLE) and a retained primary first molar (LLD) was confirmed. Due to the extent of the infection, extraction was deemed the treatment of choice. An orthodontic opinion was obtained regarding the horizontal inclination of the left mandibular second premolar (LL5) and the benefits of extracting the maxillary left first permanent molar (UL6) as a compensating extraction. Insufficient cooperation mandated general anesthesia for a detailed examination, fissure sealants of the right maxillary and mandibular first permanent molars (UR6, LR6) and extraction of left maxillary and mandibular first permanent molars (UL6, LL6), left mandibular primary first and second molars (LLE, LLD), and fluoride varnish (22 600 ppm) application.
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.