Carlson Matthew L, Beatty Charles W, Neff Brian A, Link Michael J, Driscoll Colin L W
Department of Otorhinolaryngology-Head and Neck Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA.
Arch Otolaryngol Head Neck Surg. 2010 Jun;136(6):566-75. doi: 10.1001/archoto.2010.68.
To describe presenting symptoms, evaluation findings, and surgical management of cranial base hyperostosis in patients with Camurati-Engelmann disease (CED).
Retrospective study and literature review.
The Mayo Clinic, Rochester, Minnesota.
A total of 306 patients diagnosed as having CED, including 12 primarily evaluated at our institution between 1968 and 2008, and 294 identified in the international literature.
Presenting symptoms, methods of diagnosis, treatment strategies, and patient outcomes.
One hundred seventy-three of 306 patients (56.5%) had radiographically proven skull base hyperostosis, whereas less than one-fourth were symptomatic. The most common manifestations of cranial base involvement were hearing loss (19.0%), headache (10.4%), exophthalmos (8.2%), and frontal bossing (7.2%); less common were vision changes, vertigo, facial weakness, symptomatic brainstem compression, facial numbness, and hyposmia. Although corticosteroids and bisphosphates may treat torso and extremity involvement, they demonstrate no benefit for symptomatic skull base disease. In select symptomatic patients, aggressive decompression surgery may provide the only means of treatment. Decompression surgery is more challenging with thick sclerotic bone, loss or obscuration of bony landmarks, and decreased supratentorial space. Patients must be counseled on the increased risks associated with surgery and the potential for redeposition of bone and recurrence of symptoms.
Physicians should include CED in the differential diagnosis for patients with radiographic evidence of skull base thickening and synchronous cranial neuropathies or symptoms of elevated intracranial pressure. In mild forms of the disease, the clinical course of patients should be followed with serial examination, audiometric testing, and radiography. In select patients with progressive cranial base symptoms, aggressive wide decompression of involved neurovascular structures may provide benefit.
描述卡穆拉蒂-恩格尔曼病（CED）患者颅底骨质增生的临床表现、评估结果及手术治疗方法。
回顾性研究及文献综述。
明尼苏达州罗切斯特市梅奥诊所。
总共306例被诊断为CED的患者，其中12例于1968年至2008年间在我们机构接受初步评估，294例来自国际文献。
临床表现、诊断方法、治疗策略及患者预后。
306例患者中有173例（56.5%）经影像学证实存在颅底骨质增生，而有症状者不到四分之一。颅底受累最常见的表现为听力丧失（19.0%）、头痛（10.4%）、眼球突出（8.2%）和额部隆起（7.2%）；较不常见的有视力改变、眩晕、面部无力、有症状的脑干受压、面部麻木和嗅觉减退。虽然皮质类固醇和双膦酸盐可治疗躯干和四肢受累，但对有症状的颅底疾病并无益处。在部分有症状的患者中，积极的减压手术可能是唯一的治疗方法。对于骨质硬化增厚、骨性标志缺失或模糊以及幕上空间减小的情况，减压手术更具挑战性。必须向患者告知手术相关的风险增加以及骨再沉积和症状复发的可能性。
对于有颅底增厚影像学证据及同时存在颅神经病变或颅内压升高症状的患者，医生应将CED纳入鉴别诊断。对于疾病的轻度形式，应通过系列检查、听力测试和影像学检查来跟踪患者的临床病程。对于部分有进行性颅底症状的患者，积极广泛地减压受累的神经血管结构可能有益。
