眼眶神经纤维瘤病的CT表现

Neurofibromatosis Type 1 Orbitalis CT Manifestations

目的 :探讨眼眶CT扫描诊断神经纤维瘤病I型 (NF1)的应用。材料和方法 :回顾性分析 17例具有完整临床资料的NF1眼眶CT征象 ,全部病例均经手术病理和 /或临床证实。结果 :NF1眼眶CT的主要表现为 :上睑肥厚 ,眶周及颞、颧部丛状神经纤维瘤 ;蝶骨发育不良 ,蝶骨大小翼或额骨眶板缺失 ;颅眶骨畸形 ,眶腔不对称性扩大 ,眶上、下裂及视神经孔扩大 ;眼球突出 ,体积增大 ;眶内软组织肿块 ;泪腺肿大 ,眼外肌肥厚 ,视神经增粗。结论 :眼眶CT扫描对NF1病变侵犯范围、能否手术和手术方式选择具有重要的应用价值。

Purpose: To evaluate the value of orbital CT scanning in neurofibromatosis type 1. Materials and Methods: Seventeen cases with neurofibromatosis were performed by CT scans in Axial and Coronal slice and confirmed by operation and pathology were studied retrospectively. Results: The main CT manifestations of neurofibromatosis in orbit were as follow : (1)superior eyelid thickening,plexiform neurofibromas surrounding orbital,temporal and zygomatic regions. (2)sphenoid bone dysplasia,bony defect in the wing of the sphenoid bone or orbital plate. (3)cranial and orbital bone malformation,orbital cavity asymmetrically enlarging,superior orbital fissure and inferior orbital fissure and optic canal enlarging. (4)eyeball protruding,volume of eyeball increasing. (5)Soft tissue masses in the orbit. (6)Lacrimal gland swelling,muscles of eye thickening,optic nerve thickening. Conclusion: Orbital CT scanning is important modality for the diagnosis and treatment of neurofibromatosis type 1.