淋巴管平滑肌瘤病的胸腹部CT表现

目的 探讨淋巴管平滑肌瘤病(LAM)的胸腹部CT影像特点.方法 回顾性分析8例经病理证实为LAM患者的胸腹部CT表现.结果 8 例胸部CT均有异常表现: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;3 例并肺大泡形成;1 例出现气胸;3 例出现胸腔积液.8 例均行腹部CT检查,4例有异常表现:肾血管平滑肌脂肪瘤4 例,其中1 例并腹膜后多发淋巴管肌瘤、腹膜后及盆腹腔积液,1 例并肝血管平滑肌脂肪瘤.结论 LAM 胸腹部CT表现具有一定特征性,即两肺散在或弥漫分布的大小在0.2～2.0 cm的囊腔影,伴或不伴腹部错构瘤、淋巴管肌瘤等.     

肺淋巴管肌瘤病的CT表现(附5例报告)

目的 探讨肺淋巴管肌瘤病CT表现以期提高对该病的认识水平。资料与方法 对5例经病理证实的肺淋巴管肌瘤病CT资料进行回顾性分析。所有病例均行胸部常规CT及HRCT扫描；其中1例行腹部CT扫描。结果 常规胸部CT见两肺密度减低，偶见囊状影，相应水平HRCT清晰显示全肺囊状影，无分布差异。随病程进展，囊状影数目增多，部分融合成肺大泡或气胸。1例伴右肾错构瘤及后腹膜淋巴结肿大，随访3年腹部病灶无明显改变。结论 常规CT可显示肺淋巴管肌瘤病部分肺部改变，而HRCT全面显示肺部异常，已成为本病的基本影像检查方法；肺外表现系重要的诊断佐证。     

肺淋巴管肌瘤病的HRCT诊断（附17例报告）

目的：探讨肺淋巴管肌瘤病的高分辨率C T （H RC T ）表现,以提高对该病的认识。方法回顾性分析17例经病理证实的肺淋巴管肌瘤病胸部HRCT表现及临床资料,所有病例肺活检前均行胸部 HRCT扫描及腹部B超检查,腹部B超发现异常病例者行腹部C T检查6例。结果17例患者均为育龄期女性,胸部H RC T均表现为两肺弥漫、大小不等圆形薄壁囊状影（17/17,100％）,2例部分融合成肺大泡（2/17,11．8％）,4例合并气胸（4/17,23．5％）,2例伴乳糜胸（2/17,11．8％）。腹部C T检查发现2例伴双侧肾错构瘤（2/17,11．8％）,1例伴右侧肾错构瘤（1/17,5．9％）,1例伴子宫错构瘤（1/17,5．9％）,2例伴肝脏错构瘤（2/17,11．8％）,3例伴腹膜后囊性淋巴管瘤（3/17,17．6％）。结论育龄期女性,胸部HRCT表现为两肺弥漫、均匀分布的圆形薄壁囊状影,应考虑肺淋巴管肌瘤病可能。应常规行腹部CT检查,腹部CT检查发现肝肾错构瘤,腹膜后囊性淋巴管瘤可协助诊断。     

肺淋巴管肌瘤病的CT表现（附4例分析）

目的: 探讨肺淋巴管肌瘤病CT表现,提高其诊断水平.材料和方法: 回顾性分析4例经病理证实的肺淋巴管肌瘤病CT表现.结果: 4例均行胸部常规CT及HRCT扫描,其中1例行腹部CT扫描. 常规胸部CT均可见两肺密度减低,偶见囊状影,相应水平HRCT清晰显示全肺囊状影,无分布差异.随病程进展,囊状影数目增多,部分融合成肺大泡或气胸.1例伴右肾错构瘤及后腹膜淋巴结肿大,随访3年腹部病灶无明显改变.结论: 肺淋巴管肌瘤病肺部改变,CT常规扫描表现为两肺密度减低、体积增大,HRCT能清晰显示呈特征性的两肺广泛分布的囊状影,其出现率100 %,合并肺外表现不少见.     

肺淋巴管平滑肌增生症的腹部CT表现

目的探讨肺淋巴管平滑肌增生症腹部病变的CT表现。方法回顾性分析7例经病理证实的肺淋巴管平滑肌增生症，7例均作腹部CT扫描，对其腹部CT表现进行研究分析。结果7例患者中6例有腹部阳性表现：肾血管平滑肌脂肪瘤2例，其中1例合并胸导管增大、胸腔积液；腹膜后淋巴结肿大2例，合并腹水、胸腔积液1例；肝脏血管平滑肌脂肪瘤1例；腹膜后淋巴管肌瘤合并子宫肌瘤1例。结论肺淋巴管平滑肌增生症可并发腹部多脏器病变，CT扫描对发现和诊断其腹部病变具有重要意义。     

淋巴管肌瘤病的影像学诊断(附2例报告)

目的　提高对淋巴管肌瘤病的影像学认识。方法　对 2例经病理证实的淋巴管肌瘤病患者的影像学表现及相关文献进行回顾研究。结果　 2例均为女性 ,胸片示双肺弥漫网状结节影。HRCT表现为两肺均匀分布的薄壁囊状影 ,边缘清晰 ,大小不等 ,均伴有后腹膜淋巴结肿大 ,大者约 2 .5cm ,部分融合。 1例伴有右肾血管平滑肌瘤及腹膜后平滑肌瘤 ,呈均匀中等强化。结论　淋巴管肌瘤病是一种罕见的位于淋巴器官的平滑肌细胞病态增生的疾病。两肺广泛均匀分布的薄壁囊状影为其胸部HRCT特征。可同时伴有肾脏及腹膜后血管平滑肌瘤 ,腹膜后淋巴结肿大等     

肺淋巴管肌瘤病的HRCT诊断

目的：探讨肺淋巴管肌瘤病的HRCT表现,提高对该病诊断水平。方法：回顾性分析9例经病理证实的肺淋巴管肌瘤病患者的HRCT资料。结果：所有病例均见双肺均匀弥漫分布的直径1～55mm薄壁囊状影,囊壁厚度从无法测量至2mm,囊腔间可见正常肺组织。2例伴小叶间隔增厚;1例伴磨玻璃影;2例伴气胸,其中1例为液气胸。结论：HRCT对肺淋巴管肌瘤病的诊断和鉴别诊断具有重要价值。     

肺淋巴管肌瘤病的CT诊断(附3例报告及文献复习)

目的探讨肺淋巴管肌瘤病的MSCT表现特征,提高诊断正确率。方法回顾性分析3例经活检病理证实的肺淋巴管肌瘤病的临床和CT表现,并结合相关文献加以分析讨论。结果 CT表现为两肺散在分布的大小不等的圆形、类圆形薄壁含气透亮影,其中两肺中下野分布的病灶明显较上肺野多,病灶大小从1.0mm~3.0cm不等,囊壁厚度2mm,并可见血管影走行在囊壁内。其中1例合并有肝脏血管平滑肌脂肪瘤。结论肺淋巴管肌瘤病具有特征性的CT表现,是诊断该疾病重要的影像学检查方法。     

淋巴管肌瘤病的影像表现

目的：探讨淋巴管肌瘤病(LAM)的影像学诊断价值。方法 回顾性分析15例经临床和病理证实的LAM胸部X线平片、HRCT,腹部CT和直接淋巴管造影(DLG)及DLG后胸腹部CT扫描等影像资料。结果 15例中,X线胸片上未见异常1例,双肺纹理增多3例,弥漫性小蜂窝状影或网格状影11例,气胸2例,胸腔积液14例。胸部常规CT和HRCT扫描显示15例均具有典型LAM表现,均可见两肺散在囊状影或广泛密布的囊状影。按Avila等肺部疾病程度分级标准：Ⅰ级3例;Ⅱ级5例;Ⅲ级7例。腹部CT显示14例在腹膜后、盆腔可见囊性淋巴管瘤9例,淋巴管肌瘤13例,二者共同存在7例,并发肝脏脂肪瘤和血管平滑肌脂肪瘤、肾脏小错构瘤及子宫肌瘤各1例。DLG检查,除1例淋巴管梗阻部位在腰3水平外,其余14例均可见胸导管不同程度的狭窄、梗阻及颈干和（或）锁骨下干和（或）支气管纵隔干淋巴管反流。DLG术后CT,除3例未显示胸导管出口梗阻外,其余12例显示胸导管出口梗阻情况与DLG基本一致。结论 HRCT对肺淋巴管肌瘤(PLAM)的诊断具有特征性价值,CT可发现腹部LAM,DLG和DLG后MSCT对因LAM引起的胸导管或淋巴管干梗阻部位的显示具有价值,可为手术治疗提供一定的帮助。     

肺淋巴管肌瘤病的胸部X线片和CT表现特点

目的:探讨肺淋巴管肌瘤病的胸部X线片、CT和HRCT的表现,以提高早期诊断水平。材料和方法:回顾性分析2例经病理证实的淋巴管肌瘤病的胸部X线、常规CT及HRCT表现。结果:2例胸片显示广泛的网格状、蜂窝状阴影。常规胸部CT见双肺野磨玻璃样改变,肺实质内多发囊状低密度灶。HRCT特征性表现为两肺弥漫性均匀分布的薄壁小囊状阴影。结论:常规CT可显示淋巴管肌瘤病部分肺部改变,而HRCT具有较特征性的表现,熟悉其特点有助于该病的早期诊断。     

肺淋巴管肌瘤病的CT诊断

目的分析肺淋巴管肌瘤病(PLAM)的CT表现,探讨其诊断及鉴别诊断价值。资料与方法回顾性分析2例病理确诊的PLAM患者资料,CT采用常规扫描,1mm薄层重组。结果PLAMCT表现为双肺多发边缘清楚的薄壁囊状影,直径约几毫米至2cm,囊壁隐约可见约1mm厚,囊间可见正常肺组织,病变呈弥漫分布,未见胸腔积液。结论PLAM的CT表现具有一定特征性,对其诊断及鉴别诊断有重要价值。     

Atypical presentation of lymphangioleiomyomatosis as jugular vein thrombosis

Lymphangioleiomyomatosis is a rare disorder of unknown origin that almost exclusively affects women of childbearing age. It is characterised by proliferation of abnormal smooth muscle cells (lymphangioleiomyomatosis cells) in the pulmonary interstitium and along the thoracic and abdominal lymphatics. Lymphangioleiomyomatosis may be associated with tuberous sclerosis complex. The most common manifestations of lymphangioleiomyomatosis are pulmonary symptoms, including progressive dyspnoea, recurrent pneumothoraces and chylous effusions. Extrapulmonary lymphangioleiomyomatosis as the initial presentation of the disease is highly unusual. We describe a patient with extrapulmonary lymphangioleiomyomatosis presenting as jugular vein thrombosis related to lower neck lymphangioleiomyoma. CT study showed bilateral lung cysts with left-sided hydropneumothorax and retroperitoneal lymphadenopathy. A left lower neck cystic lesion was seen with thrombosis of the adjacent left subclavian and internal jugular veins.Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease that affects women exclusively, typically during their reproductive years. A small percentage of patients have LAM in association with tuberous sclerosis complex (TSC). LAM is characterised by the abnormal proliferation of smooth muscle cells (LAM cells) in the lungs and in the thoracic and retroperitoneal lymphatics. Affected patients are at risk of developing renal hamartomas or angiomyolipomas. Patients with LAM characteristically present with chronic dyspnoea and cough and less commonly with spontaneous pneumothorax.At radiography, LAM manifests with normal-to-large lung volumes and interstitial reticular opacities that may be subtle. Unilateral pneumothorax and unilateral or bilateral pleural effusions are frequent radiographic findings. CT and high-resolution CT demonstrate bilateral diffuse thin-walled cysts surrounded by normal lung parenchyma. CT may also demonstrate associated pleural effusion or pneumothorax, thoracic or abdominal lymphadenopathy and other abdominal abnormalities, including angiomyolipomas, lymphangioleiomyomas and ascites.Manifestation of extrapulmonary LAM as an initial presenting symptom is rare. The most common forms of extrapulmonary LAM include renal angiomyolipoma, enlarged abdominal lymph nodes and lymphangioleiomyoma. Less commonly, ascites and hepatic angiomyolipoma may be present. If the diagnosis of extrapulmonary LAM precedes that of pulmonary LAM, the patient usually develops chest symptoms within one to two years.     

Lymphangioleiomyomatosis: abdominopelvic CT and US findings

PURPOSE: To describe the abdominal computed tomographic (CT) and ultrasonographic (US) findings in patients with thoracic lymphangioleiomyomatosis (LAM) and to relate the prevalence of the findings to the severity of pulmonary disease. MATERIALS AND METHODS: Eighty patients with LAM underwent chest and abdominopelvic CT and abdominopelvic US. The images were reviewed prospectively by one radiologist, and the abdominal findings were recorded and correlated with the severity of pulmonary disease at thin-section CT. RESULTS: Sixty-one (76%) of 80 patients had positive abdominal findings. The most common abdominal findings included renal angiomyolipoma (AML) in 43 patients (54%), enlarged abdominal lymph nodes in 31 (39%), and lymphangiomyoma in 13 (16%). Less common findings included ascites in eight (10%), dilatation of the thoracic duct in seven (9%), and hepatic AML in three (4%). A significant correlation (P =.02) was observed between enlarged abdominal lymph nodes and increased severity of lung disease. CONCLUSION: There are characteristic abdominal findings in patients with LAM that, in conjunction with the classic thin-section CT finding of pulmonary cysts, are useful in establishing this diagnosis.     

Pulmonary lymphangioleiomyomatosis: CT findings

Lymphangioleiomyomatosis, a rare disease occurring in women of childbearing age, is characterized by proliferation of smooth muscle in pulmonary lymphatic channels and mediastinal and abdominal lymph nodes. Chest radiographs typically reveal interstitial disease with normal lung volume. Pneumothorax and pleural effusions may be present. CT scans in eight patients with biopsy-proved pulmonary lymphangioleiomyomatosis were reviewed. The prominent feature of the disease was multiple thin-walled cysts throughout the lungs, best visualized on scans made with 1.5-mm collimation. Mediastinal and/or retrocrural lymphadenopathy, often not appreciated on the chest radiograph, was present in four of eight patients. CT can suggest a diagnosis of lymphangioleiomyomatosis when diagnosis by clinical presentation and chest radiographs is uncertain.     

肺淋巴管肌瘤病的临床及多层螺旋CT特点分析

目的:探讨肺淋巴管肌瘤病(PLAM)的多层螺旋CT(MSCT)表现,结合临床特点进行分析,以期提高对该病的诊断水平。方法:回顾性分析13例经病理证实的肺淋巴管肌瘤病患者的临床及影像资料。结果:13例均以进行性加重的呼吸困难为主要临床表现,4例伴有反复自发性气胸,3例伴乳糜胸,2例伴咯血,1例合并腹部淋巴管瘤。肺功能检查表现以阻塞性通气功能障碍为主合并弥散功能降低;MSCT表现为双肺弥漫分布大小不等的类圆形囊腔影,薄层重组和HRCT图像可清晰显示囊腔薄壁及常规CT不能显示的小的囊腔,囊壁外可见贴壁的血管影。结论:肺淋巴管肌瘤病的MSCT表现具有一定的特征性,结合临床可对多数病例做出正确的诊断。     

肺郎格汉斯细胞组织细胞增生症的CT表现

目的 分析肺郎格汉斯细胞组织细胞增生症(PLCH)的CT表现.方法 11例患者均行CT平扫,其中8例加HRCT扫描.11例均经病理确诊为PLCH.结果 11例PLCH的主要CT表现为囊腔和结节.2例仅表现为囊腔,1例仅表现为结节且多数结节伴空洞;余8例为囊腔与结节影合并存在,其中4例以囊腔为主要表现,无一例以结节影为主,2例出现间质性改变.病变的分布:1例仅有囊腔且位于左上肺;10例上、中、下肺均可见,其中2例以下肺及肋膈角区明显;3例以上叶和下叶背段明显,中叶及舌叶病变相对较轻;8例肋膈角区相对较轻.结论 PLCH的CT表现有一定的规律和特征,熟悉这些表现,有助于对该病的早期诊断.     

肺郎格汉斯细胞组织细胞增生症的CT表现及文献复习

目的 探讨肺郎格汉斯细胞组织细胞增生症（PLCH）的CT表现,提高对该病的认识。资料与方法 回顾性分析2例病理证实的PLCH患者的临床和CT资料。2例患者行多次胸部CT检查,其中1例为戒烟3年后复查,另1例因反复气胸复查。结果 1例早期PLCH的CT表现为双肺以中上肺野为主广泛分布的小片状、结节状、小环状病灶,戒烟后病灶吸收：1例晚期PLCH的CT表现为双肺以中肺野为主广泛分布的大小不等的圆形、类圆形低密度透光区,部分呈囊状和蜂窝状改变．并合并反复气胸。结论 PLCH的CT表现有一定特征性,结合临床可提高对此病的诊断。