| 强军,宋海乔,高万勤,赵可,李云东.淋巴管肌瘤病胸腹部临床与CT表现[J].中国医学影像技术,2009,25(10):1810~1812 |
| 淋巴管肌瘤病胸腹部临床与CT表现 |
| Clinical and CT manifestations of the chest and abdomen lymphangioleiomyomatosis |
| 投稿时间：2008-12-29 修订日期：2009-02-27 |
| DOI： |
| 中文关键词: 淋巴管肌瘤病 体层摄影术,X线计算机 |
| 英文关键词:Lymphangioleiomyomatosis Tomography, X-ray computed |
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| 中文摘要: |
| 目的 分析淋巴管肌瘤病(LAM)胸部及腹部的临床与CT表现。方法 对13例经病理证实的LAM胸腹部的临床与CT表现进行回顾性分析。结果 12例以肺内表现首发,均有进行性呼吸困难;其他表现包括反复发作的气胸、胸闷、咯血、咳嗽、乳糜胸等;病程中均未出现肺外表现,仅1例无意中发现腹部巨大肿物,但无肺内表现。胸部CT表现为两肺散在或弥漫分布囊腔影,大小2~20 mm,囊壁薄而清晰,并且大多数囊腔间肺实质正常;4例合并肺大泡形成;2例出现胸腔积液。10例行腹部CT检查,7例有异常表现:肾血管平滑肌脂肪瘤3例,其中1例并腹膜后多发淋巴管肌瘤、腹膜后及腹腔积液,1例并肝多发血管平滑肌脂肪瘤、脾脏增大;余4例为:腹膜后淋巴结肿大2例,腹腔积液1例,腹膜后肿块并脾脏增大1例。 结论 LAM胸腹部的临床表现无特异性,但CT表现有一定特征性,熟悉这些特点有助于早期诊断。 |
| 英文摘要: |
| Objective To analyze the clinical and CT manifestations of the chest and abdomen lymphangioleiomyomatosis (LAM). Methods The clinical and CT manifestations of 13 patients with LAM proved histopathologically were reviewed retrospectively. Results Twelve patients onset with intrapulmonary manifestations all had progressive dyspnea, other symptoms included pneumothorax of recurrent attacks, chest distress, hemoptysis, cough, chylothorax and so on. During the course of disease, 12 patients had no extrapulmonary symptoms, abdominal great goiter was found unintentionally in the rest one without any intrapulmonary symptoms. Pectoral CT manifestations included sporadic or asystematic cysts in pulmones with size of 2-20 mm, and most had thin and clear capsule wall. The lung parenchyma among cysts was mostly normal. Four patients had pneumatocele, 2 had pleural effusion. Abdominal CT was performed in 10 patients and 7 had abnormal findings: renal angiolipoleiomyoma (ALL) in 3 including one had retroperitoneal multiple lymphangiomyomas and effussion and seroperitoneum, another 2 had multiple liver ALL and spleen accretion. The rest 4 patients included retroperitoneal lymphadenectasis in 2, seroperitoneum in one, as well as retroperitoneal lump and spleen accretion in one patient. Conclusion Pectoral and abdominal symptoms in LAM are not specific, but the CT manifestations somehow specific, which are helpful to the identification and early diagnosis of LAM. |
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