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.
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.