Department of Neurosurgery, The Second Hospital of Jilin University, Changchun, China.
Department of Neurosurgery, Chian-Japan Union Hospital of Jilin University, Changchun, China.
Medicine (Baltimore). 2024 Apr 19;103(16):e37869. doi: 10.1097/MD.0000000000037869.
Eosinophilic angiocentric fibrosis (EAF) is considered to be a kind of benign IgG4-related disease, and it is more often found in the nasal cavity. We present a pretty rare case of orbital EAF that is unlike any other reported case for this case is an IgG4 negative orbital EAF and successfully treated by the fronto orbitozygomatic approach surgery.
This is a 68-year-old man from a rural area of Inner Mongolia Autonomous Region, went to our hospital for a 2-month history of vision loss with a local hospital orbital computer tomography which showed that there was a lesion in his left orbit. The inspection of the patient revealed that the patient left eye was protruding outward and the left eyelid unable to complete open or close. And his left eyeball movement had difficulty in all directions. Postoperative pathology diagnosed that this was a case of IgG4-negative EAF case.
Orbital EAF.
Surgical radical resection and postoperative glucocorticoid therapy.
After surgery, the left eye vision of this patient increased to 0.6 tested in the standard logarithmic visual acuity chart. And his left eyeball movement dysfunction and eyeball outward protruding get a partially relief.
EAF occurring in the orbit is a very rare disease and immunohistochemical results of EAF can be IgG4 negative.
嗜酸性血管中心性纤维化为一种良性 IgG4 相关疾病,多发生于鼻腔。我们报道了一例罕见的眼眶嗜酸性血管中心性纤维化为 IgG4 阴性眼眶嗜酸性血管中心性纤维化,该患者通过额眶颧入路手术成功治疗。
患者为 68 岁男性,来自内蒙古自治区农村,因 2 个月视力丧失就诊于我院,当地医院眼眶计算机断层扫描显示其左侧眼眶有病变。患者检查发现左眼突出,左眼睑无法完全睁开或闭合,左眼各向眼球运动均困难。术后病理诊断为 IgG4 阴性嗜酸性血管中心性纤维化。
眼眶嗜酸性血管中心性纤维化。
手术彻底切除,术后糖皮质激素治疗。
术后患者左眼视力提高至标准对数视力表的 0.6,左眼眼球运动障碍和眼球突出部分缓解。
发生于眼眶的嗜酸性血管中心性纤维化为一种非常罕见的疾病,嗜酸性血管中心性纤维化的免疫组化结果可为 IgG4 阴性。