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.
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.