Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and management
Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and management
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DOI：
10.3171/jns-07/09/0504
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发表时间：
2007-09-01
影响因子：
4.1
通讯作者：
Chen, Yu-Ray
中科院分区：
文献类型：
--
作者：
Diah, Enrina;Morris, David E.;Chen, Yu-Ray
物体。纤维异常增殖症(FD)是一种常见的骨肿瘤，可影响颅面部骨骼。囊变可能发生在现有的FD病变内，表现为急性临床恶化。由于现有关于这一实体的报道有限，本研究的目的是描述相对较大的一系列患者的囊变的表现和处理。对9例颌面部FD囊变性患者的人口学因素、临床表现、肿瘤类型、CT表现、手术治疗、术中所见及组织病理学结果进行了回顾。出现肿瘤时的平均年龄为14.9岁。7例患者在囊变发生急性变化之前已被诊断为FD；这种变化发生在最初诊断后的1-7年间。最常见的症状是突然肿块(78%的患者)和疼痛(67%)。未发现囊性变的易感因素。四名患者出现视力障碍；两名患者尽管进行了视神经减压，但仍出现失明。最常见的病变部位是蝶骨和额骨。囊性病变的表现从单纯性病变到动脉瘤样骨囊肿不等。囊性变可能在首次诊断数年后在FD病变内自发发生。CT扫描的评估在诊断和监测疾病进展以及治疗计划方面仍然有效。对于有症状或功能障碍的患者，应进行手术治疗。通过神经外科医生和整形外科医生之间的密切合作，促进了对这一实体的最佳管理。
Object. Fibrous dysplasia (FD) is a common osseous tumor that may affect the craniofacial skeleton. Cyst degeneration may occur within an existing FD lesion, manifesting as acute clinical deterioration. Because existing reports of this entity are limited, the object of this study was to describe the presentation and management of cyst degeneration in a relatively large series of patients.Methods. For nine patients who were treated for cyst degeneration of cramofacial FD the following data were reviewed: demographic factors, clinical presentation, tumor type, computed tomography (CT) findings, surgical management, intraoperative findings, and histopathological findings.Results. The mean age at tumor presentation was 14.9 years. Seven patients had received a diagnosis of FD before the acute changes of cyst degeneration occurred; this change occurred between I and 7 years after the initial diagnosis. The most common presenting symptoms were sudden mass enlargement (78% of patients) and pain (67%). No predisposing factor for cystic change was identified. Four patients presented with visual disturbance; blindness developed in two despite optic nerve decompression. Cyst degeneration occurred most commonly in the sphenoid and frontal bones. The cysts' appearance ranged from a simple lesion to aneurysmal bone cyst.Conclusions. Cyst degeneration may occur spontaneously within the FD lesion years after the initial diagnosis. Evaluation with CT scanning remains effective in the diagnosis and monitoring of disease progression, and in treatment planning. Surgical intervention is indicated for patients with symptoms or functional impairment. Optimal management of this entity has been facilitated through careful collaboration between neurosurgeons and plastic surgeons.