摘要
目的 探讨颅内原发性Rosai-Dorfman病(Rosai-Dorfman disease,RDD)伴上矢状窦后段血栓患者的临床、影像学和病理学特点。方法 选取解放军总医院第六医学中心收治的1例伴有上矢状窦后段血栓的RDD患者,回顾分析其临床、影像学及病理学资料,并复习相关文献。结果 本例患者女性,34岁,慢性病程,反复头痛2年,双眼视物模糊1年余。查体:左眼视力下降至0.8,双眼视盘边界不清,伴水肿和局部静脉扩张。头颅MRI示上矢状窦旁局部脑膜强化,头颅磁共振静脉成像示上矢状窦后段血栓形成至闭塞。脑脊液检查2次示颅内压增高。病理学检查:肉眼观,硬脑膜明显增厚变硬,上矢状窦后段完全闭塞,呈灰白鱼肉样改变;镜下观,脑膜组织内淋巴细胞伴纤维组织增生及大量浆细胞浸润,大量成熟浆细胞、组织细胞和淋巴细胞呈“明暗相间”排列,部分组织细胞内可见吞噬的淋巴细胞和浆细胞(“伸入现象”)。硬膜组织细胞免疫组化染色CD1a、Langerin阴性,CD68、S-100和BRAF阳性。行全身麻醉下病变硬膜手术切除,术后头痛及视物模糊缓解,预后良好。既往文献报道,RDD影像学特点对诊断有提示意义,而病理组织学的“明暗相间”排列和“伸入现象”是确诊的金标准。结论 颅内原发性RDD伴上矢状窦后段血栓罕见,主要表现为颅内压增高症候群,确诊需病变脑膜病理检查,病变脑膜手术切除为首选治疗方法。
Objective To investigate the clinical, imaging and pathological features of patients with intracranial primary Rosai-dorfman disease(RDD) with posterior segment thrombosis of the superior sagittal sinus. Methods One RDD patient with thrombosis in the posterior segment of the superior sagittal sinus admitted to the Department of Neurology, The Sixth Medical Center of PLA General Hospital on 23rd September 2019 was selected. The detailed clinical, imaging and pathological data were collected and analyzed, and the relevant literature were reviewed. Results It was a 34-year-old female, with chronic onset, recurrent headache for two years, blurred vision in both eyes for more than one year. Physical examination showed left eye visual acuity decreased to 0.8, binocular optic disc boundary was unclear, with edema and local venous dilation. Cranial MRI showed enhanced regional meningeal next to the superior sagittal sinus, and cranial magnetic resonance venography showed thrombosis to occlusion of the posterior segment of the superior sagittal sinus. Twice cerebrospinal fluid examinations showed increased intracranial pressure. As to pathological examination, under the naked eye, the dura mater was obviously thickene d and stiffened, and the posterior segment of the superior sagittal sinus was completely occluded, showing grayish fish-like changes. Under the microscope, lymphoid tissue and fibrous tissue were hyperplasia in meningeal tissue with a large amount of plasm a cell infiltration. A large number of mature plasma cells, histiocytes and lymphocytes were arranged like "light and dark alternation", and phagocytosed lymphocytes and plasma cells could be seen in some histiocytes( "emperipolesis phenomenon"). Immunohistochemical staining of dural tissue cells was negative for CD1a and Langerin, positive for CD68, S-100 and BRAFV600E. After surgical resection of diseased dura mater under general anesthesia, the symptoms of headache and blurred vision were relieved, and the prognosis was good. Referring to the published literature, the imaging features of RDD could support the diagnosis. The "light and dark alternation" and "emperipolesis phenomenon" in pathology were the gold standard for diagnosis of RDD. Conclusions Intracranial primary RDD with posterior segment thrombosis of the superior sagittal sinus is rare. The main manifestation is intracranial hypertension syndrome.Diagnosis requires pathological features of the diseased meninges, and surgical excision of the diseased meninges is the preferred treatment.
作者
刘升阳
姚玉鑫
赵丽涛
姚生
刘建国
Liu Shengyang;Yao Yuxin;Zhao Litao;Yao Sheng;Liu Jianguo(Department of Anesthesiology,The Sixth Medical Center of PLA General Hospital,Beijing 100048,China)
出处
《北京医学》
CAS
2022年第5期432-435,440,共5页
Beijing Medical Journal
关键词
ROSAI-DORFMAN病
上矢状窦后段血栓
影像学
病理
Rosai-Dorfman disease(RDD)
posterior segment of superior sagittal sinus thrombosis
imaging
pathology
作者简介
通信作者:赵丽涛,Email:ZLT252256@163.com。
