Assistance Publique - Hôpitaux de Paris, Hôpital Necker - Enfants Malades, Service de chirurgie maxillo-faciale et plastique, Université Sorbonne Paris Cité, Université Paris Descartes, Paris, France.
Assistance Publique - Hôpitaux de Paris, Hôpital Necker - Enfants Malades, Service de chirurgie maxillo-faciale et plastique, Université Sorbonne Paris Cité, Université Paris Descartes, Paris, France.
J Craniomaxillofac Surg. 2019 Jun;47(6):909-914. doi: 10.1016/j.jcms.2019.03.032. Epub 2019 Apr 8.
Parry Romberg syndrome (PRS) is a condition characterized by progressive hemifacial atrophy, predominantly affecting the soft tissues. Associated bone retraction is a common clinical feature of PRS but has never been assessed. Here we used 3D imaging and Bayesian statistics in order to demonstrate and quantify bone atrophy in PRS.
Ten non-operated patients with PRS (4/10 males) and 12 age-matched controls (7/12 males) were included into the study. The average age at CT-scan was 9.67 ± 4.13 years for PRS patients and 12.5 ± 4.37 years for controls. Soft and hard tissue atrophy levels were quantified using computed tomography scans, based on the distances between surfaces of the affected side and the non-affected contralateral side, both for the skin and the bone. We used a hierarchical Bayesian model with clinical priors in order to assess the relationship between hard and soft tissue atrophies.
PRS patients had significant hard tissue atrophy, and atrophy extents were similar for soft and hard tissues. There was a trend for a correlation between the extent of hard tissue retraction and the extent of soft tissue retraction, and we could not demonstrate that the relationship between hard and soft tissue retractions was different in PRS and controls.
Our results indicated that bone atrophy was most probably a primary process rather than a phenomenon secondary to soft tissue retraction. We have provided the first assessment of bone atrophy in PRS patients using Bayesian statistics.
帕里罗伯格综合征（PRS）是一种以半侧面部进行性萎缩为特征的疾病，主要影响软组织。相关的骨退缩是 PRS 的一个常见临床特征，但从未被评估过。在这里，我们使用 3D 成像和贝叶斯统计来证明和量化 PRS 中的骨萎缩。
本研究纳入了 10 名未经手术治疗的 PRS 患者（4/10 名男性）和 12 名年龄匹配的对照者（7/12 名男性）。PRS 患者 CT 扫描的平均年龄为 9.67 ± 4.13 岁，对照组为 12.5 ± 4.37 岁。使用计算机断层扫描（CT）扫描，根据受影响侧和未受影响的对侧表面之间的距离，定量评估软组织和硬组织的萎缩程度，包括皮肤和骨骼。我们使用具有临床先验的分层贝叶斯模型来评估硬组织和软组织萎缩之间的关系。
PRS 患者存在明显的硬组织萎缩，软组织和硬组织的萎缩程度相似。硬组织回缩程度与软组织回缩程度之间存在相关性的趋势，但我们无法证明 PRS 患者和对照组之间硬组织和软组织回缩之间的关系不同。
我们的结果表明，骨萎缩很可能是一个原发性过程，而不是软组织回缩的继发现象。我们使用贝叶斯统计首次评估了 PRS 患者的骨萎缩情况。
