線状骨症-頭蓋骨硬化症-大脳症の歯科学的所見

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  • Osteopathia Striata with Cranial Sclerosis-megalencephaly: Dental Diagnosis and Observations

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Osteopathia striata with cranial sclerosis-megalencephaly is a bone dysplasia characterized by hypertelorism, macrocephaly, frontal bossing, a broad nasal bridge, palate anomalies, hearing defects, and mental retardasion. The radiographic findings include cranial sclerosis, linear striations in the long bones and iliac wings, small poorly aerated sinuses, scoliosis, and increased bone density. The sensory defects are disabling, but the condition generally is not life threatening. An eight-year ten-month old Japanese girl was brought to the out-patient ward of the Pedodontics and Orthodontics clinic, Tokyo Dental College Suidoubashi Hospital with the chief complaint of Orthodontic treatment and oral treatment.<BR>In conclusion it was found that the maxillary-mandibular bone failed to thrive. The stricture of the maxillary dentition and many delayed eruption teeth were seen.<BR>The possible aetiology, possible inferences for the developing dentition, and the need for careful assessment prior to ankylosis of the temporomandibular joints and maxillary-mandibular bone, orthodontic therapy are highlighted.

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