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 first case describes a six-year-old female with mucopolysaccharidosis type I (Hurler Syndrome) who was referred to the dental department by her metabolic consultant. She received a bone marrow transplant at age 2 years, 2 months. Medically, she also had cleft lip (repaired at 36 weeks), obstructive sleep apnea (hypopnea syndrome), adenotonsillectomy (completed at 23 months), dysplastic mitral valve and regurgitation, kyphoscoliosis, corneal clouding, mixed bilateral hearing loss, and behavioral difficulties.\nShe was initially referred to clinical genetics due to a family history of cleft lip and palate. Clinically, it was observed that she had coarse facial features, developmental delay with associated nystagmus and hepatomegaly, and was subsequently referred to the metabolic department. It was noted that she had absence of alpha-iduronidase in the white blood cells with increased urinary glycosaminoglycans. Genetically, she had a compound heterozygote mutation (c1045G < T, c1469T) in the alpha-IDUA gene.\nShe was referred to the pediatric dental department aged six years, seven months with odontogenic pain. Intraorally, she had a high arched palate, anterior open bite, crowding, and generalized gross caries and plaque induced gingivitis (Figures , , ). She received extractions of all primary maxillary teeth, mandibular primary molars, and mandibular left central incisor, which was lingually displaced, in addition to 22 600 ppm fluoride varnish. The treatment was completed under general anesthesia aged six years, nine months.