Orofacial manifestations in a child with Fanconi's syndrome

Orofacial and dental findings in a child with Fanconi's syndrome and accompanying glycogen storage disease are presented. Remarkable retardation of skeletal growth, resulting in dwarfism, was noted. Cephalometric analyses revealed that the size of the patient's craniofacial bones at 10 years of age was as small as that of the average 4-year-old child. Development of the permanent dentition was retarded for 3 to 4 years. Histologic examination of an exfoliated primary canine showed hypomineralization of the dentin structure.