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颅面骨纤维结构不良累及眶额部:单中心回顾性研究 38 例

Craniofacial Fibrous Dysplasia in Fronto-Orbital Region: A Single-Center Retrospective Study of 38 Cases.

机构信息

Department of Neurosurgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

Department of Neurosurgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

出版信息

World Neurosurg. 2024 Jan;181:e1130-e1137. doi: 10.1016/j.wneu.2023.11.054. Epub 2023 Nov 22.

Abstract

OBJECTIVE

This study presents the clinical characteristics, imaging manifestations, and surgical experience in 38 patients diagnosed with craniofacial fibrous dysplasia in fronto-orbital region (foFD).

METHODS

We retrospectively analyzed the clinical data from 38 patients who had surgery for foFD. The surgical procedure typically involved extensive tumor removal, followed by immediate reconstruction of the frontal bone and orbit using synthetic materials. Additionally, 9 patients underwent simultaneous microscopic decompression of the optic canal.

RESULTS

Common clinical manifestations included progressive fronto-orbital bone deformity (35), proptosis (28), orbital dystopia (21), and visual impairment (9). The disease primarily affecting the frontal bone (38), the sphenoid bone (28), and the ethmoid bone (24). The optic canal was involved in 9 patients with functional impairment. Computed tomography scans in all 38 cases revealed satisfactory repair material positioning and complete resolution of frontal deformities. Among the 9 patients who underwent optic canal decompression, 7 experienced partial recovery of visual acuity after surgery.

CONCLUSIONS

In the surgical treatment of foFD, it is crucial to achieve maximal bone resection and repair skull defects, while decompressing the optic canal can provide significant benefits for patients with decreased visual function preoperatively. The use of preformed artificial materials offers advantages in aesthetic restoration after lesion excision.

摘要

目的

本研究介绍了 38 例额眶纤维发育不良(foFD)患者的临床特征、影像学表现和手术经验。

方法

我们回顾性分析了 38 例因 foFD 接受手术治疗的患者的临床资料。手术过程通常包括广泛的肿瘤切除,然后立即使用合成材料重建额骨和眼眶。此外,9 例患者同时进行视神经管显微镜减压。

结果

常见的临床表现包括进行性额眶骨畸形(35 例)、眼球突出(28 例)、眼眶移位(21 例)和视力障碍(9 例)。疾病主要累及额骨(38 例)、蝶骨(28 例)和筛骨(24 例)。9 例伴有视神经功能障碍的患者行视神经管减压术。所有 38 例患者的 CT 扫描均显示修复材料定位良好,额骨畸形完全矫正。在 9 例行视神经管减压术的患者中,7 例术后视力部分恢复。

结论

在 foFD 的手术治疗中,实现最大程度的骨切除和修复颅骨缺损至关重要,而视神经管减压术可为术前视力下降的患者带来显著益处。在病变切除后,使用预制人工材料进行美容修复具有优势。

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