Wang Xianggui, Yan Jianhua
From the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, the People's Republic of China.
J Craniofac Surg. 2014 Jul;25(4):e356-8. doi: 10.1097/SCS.0000000000000801.
The coexistence of multiple cavernous hemangiomas and venous malformation is an extremely rare clinical condition. In this report, we describe a patient showing initial multiple cavernous hemangiomas followed years later by a recurrence of multiple cavernous hemangiomas along with the appearance of venous angioma coexisting in the same orbit.
A 52-year-old woman was referred with a gradually progressive proptosis and upward displacement of her left eye that were present for 10 years. The clinical features, computed tomography and ultrasonography examination, surgery, and histopathologic findings from this patient with multiple cavernous hemangiomas coexisting with venous angioma in the same orbit are presented.
Color Doppler ultrasonography and computed tomography scan displayed more than 10 well-defined homogenous masses in the left orbit measuring 0.5 × 0.5 to 1.0 × 0.8 cm with no blood flow. Anterior orbitotomy of the left eye was performed. Fifteen accessible, distinct, red-purple, round masses were excised with tumors measuring 0.5 × 0.5 × 0.6 to 1.0 × 1.0 × 1.0 cm. Histopathologic analysis confirmed the diagnosis of multiple cavernous hemangiomas. Eight years later, she again developed left proptosis. Color Doppler ultrasonography and computed tomography disclosed multiple smoothly outlined homogeneous masses in the left orbit ranging in size from 5 × 4 to 28 × 16 mm, along with some scattered, high-density, vein-stone shadows within the mass. An anterior orbitotomy was again performed. At surgery, 4 violaceous, well-defined, and cone-shaped masses were removed with minimal bleeding. Histopathologic examination confirmed the diagnosis of multiple cavernous hemangiomas with coexisting venous angioma. There was no recurrence of orbital hemangioma at 2 years after the second surgery.
Orbital cavernous hemangioma and venous angioma may share a yet-to-be-described common link.
多发性海绵状血管瘤与静脉畸形并存是一种极其罕见的临床病症。在本报告中,我们描述了一名患者,最初表现为多发性海绵状血管瘤,数年后多发性海绵状血管瘤复发,同时在同一眼眶出现静脉血管瘤。
一名52岁女性因左眼渐进性眼球突出和上移位10年前来就诊。本文介绍了该同一眼眶内多发性海绵状血管瘤与静脉血管瘤并存患者的临床特征、计算机断层扫描和超声检查、手术及组织病理学检查结果。
彩色多普勒超声和计算机断层扫描显示左眼眶内有10多个边界清晰的均匀肿块,大小为0.5×0.5至1.0×0.8厘米,无血流信号。对左眼进行了前路眼眶切开术。切除了15个可触及的、 distinct、红紫色、圆形肿块,肿瘤大小为0.5×0.5×0.6至1.0×1.0×1.0厘米。组织病理学分析确诊为多发性海绵状血管瘤。8年后,她再次出现左眼球突出。彩色多普勒超声和计算机断层扫描显示左眼眶内有多个轮廓光滑的均匀肿块,大小从5×4至28×16毫米不等,肿块内还有一些散在的高密度静脉石阴影。再次进行了前路眼眶切开术。手术中切除了4个紫罗兰色、边界清晰的锥形肿块,出血极少。组织病理学检查确诊为多发性海绵状血管瘤合并静脉血管瘤。第二次手术后2年眼眶血管瘤无复发。
眼眶海绵状血管瘤和静脉血管瘤可能存在尚未明确的共同联系。