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.
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.