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目的探讨气管血管瘤的临床表现、诊断、鉴别诊断、病理学特征、治疗及预后,提高对该病的诊疗水平。方法回顾性分析四川大学华西医院收治的1例气管血管瘤患者的临床资料并进行相关文献复习。以“气道血管瘤”或“支气管血管瘤”为检索词检索中国期刊网全文数据库、万方数据库及维普数据库,以“Trachea”OR“Bronchus”AND“Hemangioma”为检索词检索Pub Med、Ovid Medline及Embase数据库,检索时间为1976年1月1日至2016年10月31日。结果患者为32岁男性,主诉为“背痛、咯血10~+d”。其胸部增强CT提示食管壁全程增厚,食管管腔变窄,纤维支气管镜显示气管及双侧支气管广泛血管瘤样改变,胃镜检查提示食管静脉瘤。患者气管内病变广泛,但经内科保守治疗后,咯血症状明显缓解,随访至今病情未复发。经上述数据库检索,有34篇文献36例患者明确诊断为气管血管瘤并且具有相关临床资料。其临床症状主要为反复咯血,影像表现不具有特征性,组织病理学表现为扩张、薄壁、管腔大小不一的血管或增生的毛细血管,免疫组织化学染色显示血管内皮细胞特异性CD34及Ⅷ因子阳性。结论血管瘤是一种常发生于婴儿及儿童头颈部的良性肿瘤,其在成人气管、支气管内极其罕见。病因尚不清楚。临床及影像表现不具有特征性,与淋巴管瘤易混淆。确诊需依赖组织病理学检查,纤维支气管镜及CT胸部血管三维重建成像在整个诊治过程中起重要作用。气管血管瘤的预后较好。
Objective To investigate the clinical manifestations, diagnosis, differential diagnosis, pathological features, treatment and prognosis of tracheal hemangiomas and to improve the diagnosis and treatment of the disease. Methods The clinical data of 1 patient with tracheal hemangioma admitted to West China Hospital of Sichuan University were retrospectively reviewed and relevant literatures were reviewed. Retrieval of Chinese Journal Net Full-text Database, Wanfang Database and VIP Database with “Trachea ” OR “Bronchus ” AND “Hemangioma ”To search Pub Med, Ovid Medline and Embase databases for search terms from January 1, 1976 to October 31, 2016. Results The patient was a 32-year-old male and complained of “back pain, hemoptysis 10 ~ + d ”. The thoracic enhanced CT tip thickened esophageal wall, esophageal lumen narrowing, bronchoscopy and trachea and bilateral bronchial extensive hemangiomatous changes, endoscopy showed esophageal varices. Patients with extensive tracheal lesions, but conservative treatment by internal medicine, hemoptysis symptoms were significantly relieved, the disease has not relapsed since the follow-up. After the above database search, 34 articles of 36 patients with definite diagnosis of tracheal hemangioma and has relevant clinical data. The main clinical symptoms were recurrent hemoptysis, imaging features were not characteristic, histopathological manifestations of expansion, thin-walled, lumen size of blood vessels or hyperplastic capillaries, immunohistochemical staining showed that vascular endothelial cell-specific CD34 and Ⅷ factor positive. Conclusion Hemangiomas are benign tumors that commonly occur in the head and neck of infants and children and are extremely rare in adult trachea and bronchi. The cause is not clear. Clinical and imaging performance is not characteristic, easily confused with lymphangioma. Diagnosis depends on histopathological examination, fibrobronchoscopy and CT thoracic vascular three-dimensional reconstruction of the entire process of diagnosis and treatment play an important role. The prognosis of tracheal hemangiomas is better.