The fifth case describes a twelve-year-old female referred to the dental department internally from the metabolic department. Medically, she had Mucopolysaccharidosis type IVA (Morquio Syndrome (MPS IVA)), severe obstructive sleep apnea requiring nocturnal CPAP, bilateral conductive and sensorineural hearing loss, bilateral corneal clouding, left ventricular hypertrophy, congenital bilateral talipes equinovarus (surgically corrected), genu valgum (surgically corrected), atlanto-axial instability (awaiting surgical stabilisation), and short stature. Her MPS IVA was managed with Vimizim, an enzyme replacement therapy (ERT).\nThe patient was referred to the orthodontic department regarding canine ectopy and supernumerary teeth. A retrognathic mandible and proclined maxillary incisors contributed to an increased overjet of 7 mm. Intraorally microdontia, maxillary anterior spacing, and hypoplastic enamel were evident (Figure ). The soft tissues were healthy, and no caries were present. The maxillary left permanent canine (UL3) was palatally impacted and four supernumerary teeth were present radiographically (Figure ), distal to each third permanent molars (distomolars). The orthodontic treatment plan consisted of a sectional fixed appliance to idealize the space for the impacted canine tooth and a functional appliance to reduce the overjet. Orthodontic tooth movements progressed unremarkably.
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.