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首页> 外文期刊>Journal of bone and mineral research: the official journal of the American Society for Bone and Mineral Research >Chiari I Malformation and Basilar Invagination in Fibrous Dysplasia: Prevalence, Mechanisms, and Clinical Implications
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Chiari I Malformation and Basilar Invagination in Fibrous Dysplasia: Prevalence, Mechanisms, and Clinical Implications

机译：Chiari I畸形和纤维发育不良的基础术语：患病率，机制和临床意义

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ABSTRACT Fibrous dysplasia (FD) is a mosaic disorder of benign fibro‐osseous lesions, which may be associated with extraskeletal features as part of McCune‐Albright syndrome (MAS). Cranial base abnormalities, including Chiari I malformation (CM1), in which the cerebellum extends below the foramen magnum, and secondary basilar invagination (BI), in which the odontoid prolapses into the posterior cranial fossa, are potentially serious complications of metabolic bone disorders. The purpose of this study was to determine the prevalence, natural history, and risk factors for CM1 and BI in patients with FD/MAS, and to determine mechanisms of cranial base deformities. Clinical and radiographic data from subjects in an FD/MAS natural history study were evaluated and compared to normal controls. In 158 patients with craniofacial FD, 10 (6.3%) cases of CM1 and 12 (7.6%) cases of BI were diagnosed. No cranial base abnormalities were identified in 10 control subjects. Craniomorphometric and volumetric analyses identified cranial constriction and cranial settling as the primary mechanisms of cranial base abnormalities, whereas intracranial hypertension was a contributing factor in a minority of subjects. Longitudinal analyses found progression of odontoid position with age, but no progression of tonsillar position. No endocrinopathies were associated with CM1. MAS endocrinopathies associated with BI included hyperthyroidism (odds ratio [OR] 12.0; 95% confidence interval [CI], 2.9 to 55.6; p? ?0.01), precocious puberty (OR 5.6; 95% CI, 1.2 to 26.0; p? ?0.05), and hypophosphatemia (OR 7.7; 95% CI, 1.9 to 27.0; p? ?0.01). Scoliosis was associated with both CM1 (OR 4.8; 95% CI, 1.1 to 22.8; p? ?0.05) and BI (OR?=?infinity; 95% CI, 4.7 to infinity; p? ?0.01). This study successfully characterized cranial base abnormalities in FD/MAS and the pathophysiological connection between them. These findings support routine screening for cranial base abnormalities in patients with craniofacial FD, as well as aggressive management of contributory risk factors. ? 2018 American Society for Bone and Mineral Research.
机译：摘要纤维发育不良（FD）是一种良性纤维 - 骨质病变的马赛克病症，其可能与麦克伦 - 醇综合征（MAS）的一部分有关。颅底异常，包括Chiari I畸形（CM1），其中小脑弥漫在粉刺粒以下延伸，并且次生的基础术（BI），其中所述Odontoid脱血窝流入后颅骨，是代谢骨紊乱的可能性严重并发症。本研究的目的是在FD / MAS患者中确定CM1和BI的患病率，自然历史和危险因素，并确定颅底畸形机制。评估来自FD / MAS自然历史研究中的受试者的临床和放射线图，并与正常对照进行比较。在158例患有颅面FD的患者中，诊断患病的10例（6.3％）CM1和12（7.6％）病例。在10个对照科目中没有鉴定颅底异常。颅骨形状和体积分析确定了颅内收缩和颅骨沉降作为颅基异常的主要机制，而颅内高血压是少数科目的贡献因素。纵向分析发现了随着年龄的增长，但没有扁桃体地位的进展。没有内分泌治疗与cm1有关。与Bi包含甲状腺功能亢进的MAS内分泌术（差距[或] 12.0; 95％置信区间[CI]，2.9至55.6;p≤x≤0.01），预焦青春期（或5.6; 95％CI，1.2至26.0; P. α.<0.05）和次磷血症（或7.7; 95％CI，1.9至27.0;p≤≤0.01）。脊柱症与Cm1（或4.8; 95％CI，1.1至22.8;p≤≤0.05）和Bi（或？=Δfigate; 95％Ci，4.7至无穷大的; P 1 0. <0.p≤0）。该研究成功地表征了FD / MAS中的颅底异常和它们之间的病理生理学联系。这些发现支持颅面FD患者的颅底异常的常规筛查，以及对贡献风险因素的激进管理。还2018年美国骨骼和矿物学研究。

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