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眶颞部神经纤维瘤病:分类与治疗

Orbitotemporal neurofibromatosis: classification and treatment.

作者信息

Erb Melanie H, Uzcategui Nicolas, See Robert F, Burnstine Michael A

机构信息

Department of Ophthalmology, College of Medicine, University of California at Irvine, Irvine, CA, USA.

出版信息

Orbit. 2007 Dec;26(4):223-8. doi: 10.1080/01676830600987227.

Abstract

PURPOSE

To review the clinical findings in orbitotemporal neurofibromatosis and discuss treatment options. Clinical features, histopathologic characteristics, and treatment options are reviewed.

METHODS

A Medline literature search from 1966 to 2004 was performed, using the key words: orbitotemporal neurofibromatosis, orbitopalpebral neurofibromatosis, orbitofacial neurofibromatosis, cranio-orbital neurofibromatosis, and cranio-orbital-temporal neurofibromatosis, and the pertinent literature was reviewed. Additionally, our experience with two patients is reported. The surgical procedures are discussed.

CONCLUSION

The management of orbitotemporal neurofibromatosis is challenging. The planned surgical approach and extent of resection depend on the severity of the orbital soft tissue and bony involvement and on the visual potential. Ultimately, orbital exenteration may be needed for rehabilitation and cosmesis.

摘要

目的

回顾眶颞部神经纤维瘤病的临床发现并探讨治疗方案。对临床特征、组织病理学特点及治疗方案进行综述。

方法

利用关键词“眶颞部神经纤维瘤病”“眶睑部神经纤维瘤病”“眶面部神经纤维瘤病”“颅眶部神经纤维瘤病”及“颅眶颞部神经纤维瘤病”对1966年至2004年的医学文献进行检索,并对相关文献进行综述。此外,报告了我们对两名患者的治疗经验。对手术操作进行讨论。

结论

眶颞部神经纤维瘤病的治疗具有挑战性。手术方案及切除范围取决于眼眶软组织和骨受累的严重程度以及视力情况。最终,可能需要行眼眶内容剜除术以实现康复和美容目的。

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