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首页> 外文期刊>Journal of plastic, reconstructive & aesthetic surgery: JPRAS >A classification system to guide orbitozygomatic reconstruction in Treacher-Collins syndrome
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A classification system to guide orbitozygomatic reconstruction in Treacher-Collins syndrome

机译：指导Treacher-Collins综合征眼眶y重建的分类系统

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Dear Sir, We propose a classification system of the orbitozygomatic skeletal deformities seen in Treacher-Collins syndrome (TCS), a complex autosomal dominant disorder of cranio-facial morphogenesis. The classification is designed both to characterize the degree of deformity and as a guide to the reconstructive planning. TCS results in varying degrees of retromicrognathia, maxillary hypoplasia and orbitozygomatic dysplasia. The severity of the soft tissue deformities generally parallel the underlying skeletal dysmorphology. The spectrum of mandibular deformity seen in Hemi-facial Microsomia (HFM) was originally successfully classified by Pruzansky and since its introduction, the system or one of its variants has underpinned the management of the mandible and TMJ in this condition. The challenges in developing a coherent treatment philosophy for orbito-zygomatic dysplasia in TCS are very similar to those seen in treating the mandible in HFM. Both conditions present with varying degrees of skeletal hypoplasia which creates increasing challenges in providing additional bone stock for reconstruction. Both conditions present varying degrees of abnormality in both the shape and position of the affected skeletal elements and in both conditions there are functional problems related to the severity of the deformity. We have therefore applied the same concepts to devise a classification system to guide the craniofacial surgeon during orbitozygomatic reconstruction in TCS.
机译：尊敬的主席先生，我们提出了在Treacher-Collins综合征（TCS）中发现的眶oz骨骼畸形的分类系统，这是一种复杂的常染色体显性颅面形态异常。该分类既可以表征变形程度，又可以指导重建规划。 TCS导致不同程度的反白细胞增多症，上颌发育不全和眶or肌发育异常。软组织畸形的严重程度通常与潜在的骨骼变形相似。下颌面畸形（HFM）中观察到的下颌畸形频谱最初是由Pruzansky成功分类的，并且自引入以来，该系统或其变体之一为在这种情况下下颌骨和TMJ的管理奠定了基础。为TCS中的眶zy不典型增生制定连贯的治疗方案所面临的挑战与在HFM中治疗下颌骨所遇到的挑战非常相似。两种情况都存在不同程度的骨骼发育不全，这在提供额外的骨储备以进行重建方面提出了越来越多的挑战。两种情况在受影响的骨骼元件的形状和位置上都表现出不同程度的异常，并且在两种情况下都存在与畸形的严重程度有关的功能性问题。因此，我们采用了相同的概念来设计分类系统，以指导颅面外科医师在TCS的眶oz关节重建术中使用。

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