Fibrous dysplasia (FD) is characterized by expansile fibro-osseous lesions that may occur in association with endocrinopathies as part of McCune–Albright syndrome (MAS). Craniofacial FD is a significant source of morbidity and most commonly involves the gnathic bones. There is a critical need to understand the natural history and risk factors for gnathic FD progression to develop preventative trials and identify candidates for intervention. The purpose of this study was to characterize gnathic FD lesion expansion and to identify risk factors associated with lesion growth. Patients with gnathic FD and serial CT imaging were evaluated. Volumetric analyses of CT scans were performed using MIM Encore software. Generalized mixed model analysis was used to account for intra-subject correlation, with FD lesion volume as the dependent variable. In addition to age, effects of MAS-associated endocrinopathies, sex, disease severity, and bisphosphonate treatment were evaluated. A total of 104 total lesions in 52 patients were characterized longitudinally. Median age at initial scan was 8.8 years (range 3.4–18.8), and median age at final scan was 16.8 years (range 6.9–33.4 years). The median number of scans per subject was 4 (range 2–14). FD lesion volume increased with age (2.50 cm³/yr, 95% confidence interval [CI] 1.95–3.04, p < 0.001). However, lesion expansion rate decreased over time (−0.05 cm³/yr, 95% CI −0.07 to 0.04, p < 0.001). Mandibular lesions tended to expand at a greater rate than maxillary lesions (p < 0.001). Growth hormone excess was associated with accelerated expansion rate (p = 0.002). Other MAS-associated endocrinopathies, pubertal status, sex, weight, lesion density, disease severity, and bisphosphonate treatment were not associated with lesion volume or expansion. Gnathic FD lesion expansion is most rapid in younger children and declines as patients approach adulthood. The availability of quantitative natural history data will guide clinicians in identifying patients who are candidates for medical and surgical interventions and clinical trials for preventative therapies. Published 2023. This article is a U.S. Government work and is in the public domain in the USA.

中文翻译：

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颌骨纤维发育不良的病变扩展：自然史、进展指标和对双磷酸盐的反应

纤维性发育不良 (FD) 的特点是扩张性纤维骨病变，可能与作为 McCune-Albright 综合征 (MAS) 一部分的内分泌疾病相关。颅面 FD 是发病的重要来源，最常见的是颌骨。迫切需要了解下颌 FD 进展的自然史和危险因素，以开展预防性试验并确定干预候选者。本研究的目的是表征下颌 FD 病变扩展并确定与病变生长相关的危险因素。对下颌 FD 和连续 CT 成像的患者进行了评估。使用 MIM Encore 软件对 CT 扫描进行体积分析。使用广义混合模型分析来解释受试者内相关性，以 FD 病变体积作为因变量。除了年龄之外，还评估了 MAS 相关内分泌疾病、性别、疾病严重程度和双膦酸盐治疗的影响。对 52 名患者的 104 个病变进行了纵向表征。初次扫描时的中位年龄为 8.8 岁（范围 3.4-18.8），最终扫描时的中位年龄为 16.8 岁（范围 6.9-33.4 岁）。每个受试者的扫描中位数为 4 次（范围 2-14）。FD 病变体积随着年龄的增长而增加（2.50 cm ³ /年，95% 置信区间 [CI] 1.95–3.04，p  < 0.001）。然而，病变扩张率随着时间的推移而降低（-0.05 cm ³ /年，95% CI -0.07至0.04，p  < 0.001）。下颌病变的扩张速度往往高于上颌病变 (p < 0.001)。生长激素过量与加速扩张率相关（p  = 0.002）。其他 MAS 相关内分泌疾病、青春期状态、性别、体重、病变密度、疾病严重程度和双膦酸盐治疗与病变体积或扩张无关。下颌 FD 病变扩张在年幼的儿童中最快，并随着患者接近成年而下降。定量自然历史数据的可用性将指导临床医生识别适合进行内科和外科干预以及预防性治疗临床试验的患者。2023 年出版。本文是美国政府的作品，在美国属于公共领域。