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.
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.