Sharma Subramanya S, Saravanan C, Sathyabama V, Satish C
Department of Oral & Maxillofacial Surgery, Aadhiparasakthi Dental College & Hospital, Melmaruvathur, Tamilnadu, India.
BMJ Case Rep. 2013 Jan 10;2013:bcr2012007487. doi: 10.1136/bcr-2012-007487.
Osteopetrosis is a rare congenital (autosomal type) disorder of the skeletal system. Several variants have been described in the literature with grossly variant prognosis and clinical behaviour. Several reports of intractable osteomyelitis of the jaw bones secondary to osteopetrosis, particularly the mandible, have been published widely. However, there is no published report of the complete mandible sequestrating de novo, in the literature. An overview of this spectrum of sclerotic bone disease, its presentation in the oro-facial region, the diagnostic challenge it poses and the management dilemma it offers to the maxillofacial surgeon is discussed and a protocol for managing this disease effectively is presented. A clinical illustration of the complexities of management of osteopetrosis-induced osteomyelitis of jaw bones is demonstrated with a very rare case in which the entire mandible had sequestrated.
骨质石化症是一种罕见的先天性（常染色体类型）骨骼系统疾病。文献中描述了几种变体，其预后和临床行为差异很大。继发于骨质石化症，尤其是下颌骨的颌骨难治性骨髓炎的几份报告已广泛发表。然而，文献中没有关于完整下颌骨从头开始发生骨分离的发表报告。本文讨论了这种硬化性骨病的概况、其在口腔面部区域的表现、它带来的诊断挑战以及它给颌面外科医生带来的管理困境，并提出了有效管理这种疾病的方案。通过一个非常罕见的整个下颌骨发生骨分离的病例，展示了骨质石化症引起的颌骨骨髓炎管理复杂性的临床实例。
