先天性眼眶纤维化14例临床分析  

Clinical analysis of 14 cases of congenital orbital fibrosis

作　　者：肖利华[1] 王毅[1] 鲁小中[1] 杨新吉[1] 苏帆[1] 朱慧[1] 赵海萍[1] 

机构地区：[1]武警总医院眼眶病研究所,北京100039

出　　处：《中华眼科杂志》2012年第8期679-682,共4页Chinese Journal of Ophthalmology

摘　　要：目的分析先天性眼眶纤维化的临床表现和影像学特征。方法回顾性系列病例研究。分析2005年1月至2009年12月在武警总医院眼眶病研究所就诊的先天性眼眶纤维化患者临床资料14例。其中男性6例，女性8例；年龄3个月至5岁8例，6～8岁4例，17和18岁各1例。结果所有患者均为出生后家长即发现患眼异常，主诉包括眼球突出（4例）或内陷（2例）、斜视（5例）、睑裂闭合不全（7例）、结膜充血或流泪（4例）和眼球运动障碍（12例）。眼部检查：视力（能配合检查者7例）0．1～0．3共4例，余3例各为0．05、光感及指数，均不能矫正。眼球突出5例（1—2mm）、眼球内陷3例、斜视或眼球移位13例、睑裂闭合不全或睑退缩5例、眼球运动障碍几近固定者6例（其他8例均有不同程度运动障碍）、代偿头位3例、结膜充血5例、轻度上睑下垂3例。所有病例均无家族史。影像学检查13例行眼眶cT扫描，均发现球后不规则高密度软组织病变，同时合并邻近眼外肌增厚，球后结构紊乱。9例行MRI扫描，T1WI均为中信号，T2WI均为中低信号，其中5例增强扫描显示病变轻、中度增强。手术及病理所见：2例手术探查，术中均行眼球牵拉试验，阻力明显。术中可见球后被质硬纤维组织包裹，眼外肌增厚变硬。病变部分切除后，病理检查显示病变包含泪腺，眼外肌内可见纤维组织增生和变性，其中可见少许淋巴细胞。结论先天性眼眶纤维化是一种先天性眼眶病变，以眼球内陷，眼球运动障碍，弱视等为临床特征，影像学显示为球后不规则软组织影，治疗效果不佳。Objective The purpose of this study was to show the clinical and imaging characteristics in patients with congenital orbital fibrosis. Methods A retrospective review of a series of 14 patients with congenital orbital fibrosis hospitalized in Institute of Orbital Disease during 2005 to 2009 have been characterized. Patients aged from 3 months to 18 years old, the median age was 7 years old, 6 cases （43%） were male. Results Ocular changes were found at birth in all patients, symptoms at onset of the disease included dysfunction of eye movement （12 cases）, lagophthalmos （7 cases）, diplopia （5 cases） , exophthalmos （4 cases） , conjunctival congestion （4 cases） and endophthalmos （2 cases）. The results of medical examinations including visual acuity, exophthalmos or endophthalmos, diplopia with or without compensative head station, displacement of the eye, lagophthalmos, blephroptosis and ocular dyskinesia all have been record. In the 14 cases, 13 patients have undertaken the CT imaging examination, soft tissue lesions have been found in the orbit with high density and irregular shape, accompanied with thickened ocular muscles and anatomical changes. Of the 9 cases which underwent MRI examination, all of the lesions showed medium signals in T^WI, with medium to low signals in TEWI. Local excision has been performed in 2 patients, we found that the orbital lesions packaged with hard fibrous tissues around, and the ocular muscles were thick and stark. Pathological examination showed fibrosis and degeneration in the lesions, lacrimal gland and ocular muscles. Some lymphocyte infiltration also has been observed. Conclusions Congenital orbital fibrosis is an orbital disease occurs at birth and is characterized with series of signs such as endophthalmos or exophthalmos, ocular dyskinesia and dip｜opia. Imaging examinations often find abnormal mass with irregular shape and soft tissue density in orbit. Till now, no efficient therapy has been established.

关 键 词：眶疾病 纤维化 眼球内陷 眼球运动障碍 诊断显像 

分 类 号：R777.5[医药卫生—眼科]