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.     

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。     

HRCT和CT淋巴管成像在淋巴管平滑肌瘤病中的诊断价值

目的 探讨HRCT和CT淋巴管成像在淋巴管平滑肌瘤病中的诊断价值。方法 回顾性搜集56例确诊为淋巴管平滑肌瘤病患者的临床和影像资料,所有患者均行胸部HRCT平扫,其中45例于直接淋巴管造影(DLG)后行胸腹盆部CT扫描(CTL),分析胸部病变的HRCT及胸腹盆部CTL影像表现,并采用分类变量资料中的构成比进行统计描述。结果 56例患者的胸部HRCT表现为双肺弥漫大小不等的薄壁含气囊肿56例,肺内磨玻璃密度影16例,肺内结节8例,肺不张32例,支气管血管束增粗12例,小叶间隔增厚25例,心包积液6例,纵隔淋巴结增大9例,胸腔积液45例,气胸12例。45例患者CTL均显示不同部位的碘油异常沉积：胸导管末端24例,右淋巴导管7例,肺内7例,肺门7例,纵隔20例,心包1例,胸腔7例,肋间17例,腋窝6例,横膈周围32例,脊柱旁27例,腹盆腔45例,双髂41例,双侧腹股沟36例,会阴部3例。此外,3例伴有肝脏血管平滑肌脂肪瘤,8例伴有肾脏血管平滑肌脂肪瘤,3例伴有小肠壁弥漫增厚,31例伴有腹盆腔积液。结论 HRCT和CTL能够显示淋巴管平滑肌瘤病的一些特征性表现,同时伴有不同部位的淋巴管异常,为...     

肺淋巴管平滑肌瘤病高分辨率CT影像学分析

目的分析肺淋巴管肌瘤病(PLAM)的临床特征及高分辨率CT(HRCT)表现,进一步提高对该病的认识。方法回顾性分析3例经病理证实并行HRCT检查的LAM患者资料,分析其胸部HRCT特点。结果 3例均以进行性呼吸困难为主要临床表现,1例伴有反复自发性气胸,1例伴乳糜胸及腹膜后淋巴管扩张。HRCT示双肺弥漫囊样影,分布均匀,大小形态较一致,部分囊腔有融合,病变直径为2.0～20.0mm的薄壁囊腔,壁厚1.5～2.0mm,病变早期囊腔之间可见正常肺组织。结论 PLAM主要临床特征是进行性呼吸困难,常伴有乳糜胸、气胸、腹膜后淋巴管扩张、淋巴管瘤等,HRCT对该病的早期诊断、鉴别诊断具有重要价值。     

腹膜后淋巴管肌瘤病1例

<正>淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)是一种罕见病,主要发生在肺部,肺外主要发生在腹膜后、盆腔淋巴结、子宫、纵隔、颈部等。目前国内报道主要集中在胸部LAM的CT表现,尚未查到国内关于该病在MR方面的报道。现报道1例腹膜后淋巴管肌瘤病,描述其MRI表现。患者女,36岁。1年前无明显诱因出现右上腹不适,     

系统性红斑狼疮合并淋巴管受累的CT淋巴管成像影像表现

目的 探讨系统性红斑狼疮合并淋巴管受累的CT淋巴管成像影像表现。方法 回顾性搜集15例确诊为系统性红斑狼疮合并淋巴管受累患者的临床和影像资料，所有患者均于直接淋巴管造影术(DLG)后行常规胸腹盆部CT扫描，同时重建CT淋巴管成像(CTL)图像，分析CTL及常规胸腹盆部影像表现，并采用分类变量资料中的构成比进行统计描述。结果 15例患者CTL均显示不同部位的碘油异常沉积：胸导管末端10例，右淋巴导管5例，肺内3例，肺门3例，纵隔11例，胸腔8例，肋间1例，腋窝2例，腹盆腔15例，左髂6例，右髂10例，腹股沟15例，臀部2例，肠壁外1例，肝门1例。15例患者常规胸腹盆部CT表现为肺内磨玻璃密度影10例，斑片状实变影2例，肺内结节6例，肺不张11例，支气管扩张1例，蛙卵征4例，小叶间隔增厚6例，心包积液4例，纵隔脂肪间隙浑浊5例，胸腔积液12例，胸壁皮下水肿4例，叶间裂积液6例，叶间裂增厚13例。此外，2例伴有小肠壁弥漫增厚，5例伴有肠系膜肿胀，11例伴有腹盆腔积液，8例伴有腹壁皮下水肿。结论 CTL不仅能够直接显示受累淋巴管的异常，而且能够显示常规CT胸腹盆部异常表现，为本病的诊断和治疗提...     

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     

弥漫性混合型纤维肌发育不良1例

本文报道弥漫性混合型纤维肌发育不良1例。患者因意识障碍13 h入院。CT血管成像检查示双侧颈内动脉至腹主动脉范围内全身大动脉、中动脉管腔弥漫性扩张, 伴多发局限性管腔狭窄及狭窄后瘤样扩张, 动脉瘤形成, 呈典型"串珠样"改变。头颅CT平扫示右侧丘脑片状低密度影, 边界模糊, 考虑为右侧丘脑急性梗死。综合影像学检查考虑为弥漫性混合型纤维肌发育不良。     

淋巴管肌瘤病与肝硬化相关乳糜腹淋巴循环异常的影像学比较研究

目的 比较淋巴管肌瘤病(LAM)和肝硬化相关乳糜腹淋巴循环异常的影像学特点.方法 回顾性分析19例LAM相关乳糜腹和22例肝硬化相关乳糜腹的直接淋巴管造影(DLG)和CT淋巴管成像(CTL)资料,比较两组的淋巴循环异常影像特征.结果 与肝硬化相关乳糜腹相比,LAM相关乳糜腹更易出现造影对侧髂腰部的对比剂异常反流(68....     

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。     

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.     

淋巴管肌瘤病的CT诊断(附二例报告及文献复习)

目的 描述淋巴管肌瘤病的CT表现。方法 回顾分析2例经病理证实的淋巴管肌瘤病的胸腹CT表现。结果 2例胸部高分辨率CT表现为两肺均匀或不均匀分布的薄壁囊状影，1例伴乳糜胸。腹部CT于2例检出腹膜后淋巴结肿大。1例伴有肾脏及腹膜后血管平滑肌脂肪瘤，另1例伴腹膜后淋巴管肌瘤。结论 淋巴管肌瘤病的胸腹部CT表现有一定特征性。熟悉其胸腹部CT表现有助于该病的诊断，但最终确诊须依靠组织病理学检出。     

Cystic lung disease associated with eosinophilic granuloma and tuberous sclerosis: air trapping at dynamic ultrafast high-resolution CT.

Thin-walled lung cysts can occur in association with advanced pulmonary eosinophilic granuloma and lymphangioleimyomatosis (LAM). In LAM, cysts occur as an isolated abnormality or in association with tuberous sclerosis. The cause of these cysts is unclear, but some investigators have postulated that they result from air trapping. To determine if lung cysts in these two diseases are associated with air trapping, the authors performed dynamic ultrafast high-resolution computed tomography (DUHRCT) during forced expiration in two patients (one with eosinophilic granuloma and one with tuberous sclerosis) with lung cysts and correlated the results with results of pulmonary function tests. DUHRCT demonstrated focal and diffuse air trapping; in some lung regions, a less than normal increase in lung attenuation during forced exhalation was evident. These studies do not allow a conclusion regarding the mechanism of cyst formation in eosinophilic granuloma and LAM-tuberous sclerosis, but they confirm the association between lung cysts and morphologic findings of air trapping.     

Pulmonary lymphangioleiomyomatosis. High-resolution CT findings in 11 patients and compared with the literature

We retrospectively evaluated the high-resolution computed tomographic (HRCT) findings in 11 women patients with pulmonary lymphangioleiomyomatosis (LAM). Numerous, thin-walled cysts distributed diffusely throughout the lungs and ranged in size from a few millimeters to several centimeters were seen on HRCT in all patients. Other findings at HRCT included interlobular septal thickening (six patients), ground-glass opacity (one patient) and architectural distortion (one patient). Our study found that HRCT imaging findings were similar to the literatures reported, except that majority of our cases also have interstitial changes. HRCT may help establish this diagnosis when diagnosis by clinical findings is uncertain and chest radiographs showed normal or nonspecific findings.     

Pulmonary lymphangioleiomyomatosis--review and personal results

Pulmonary lymphangioleiomyomatosis (LAM) is characterised by the proliferation of smooth muscle cells in the peribronchial, perivascular and perilymphatic tissue resulting in severe destructive changes of the lung parenchyma. We reviewed the literature and compared the findings in chest radiograph and CT with our own results obtained in a group of 12 patients suffering from histological proven LAM. The main findings of LAM in chest radiograph are a diffuse reticular opacity and an increased lung volume, often large cysts are visible. The CT scans reveal that cystic lesions are present in all patients, but the reported size of the cystic lesions is contradictory. We postulate two main types of LAM: the one resulting in a diffuse distribution of multiple small cysts, partially demonstrating a honey-combing pattern, the other showing severe destruction of the lung parenchyma due to large cysts. The presence of fibrotic changes and architectural distraction does not exclude LAM. So chest radiograph as well as computed tomography reveal neither uniform nor pathognomonic findings. Differential diagnosis of LAM seems to be more difficult than most authors believe.     

肺撕裂伤的CT诊断

目的：探讨肺撕裂伤的CT诊断。方法：对21例胸部外伤或全身多部位复合伤后1~3h行多层螺旋CT检查,并于治疗1-30天后复查。结果：肺撕裂伤CT主要表现为气囊肿10例,气液囊肿7例,肺血肿4例。21例肺撕裂伤患者均伴有不同程度肺挫裂伤;其中肋骨骨折16例,胸腔积液13例,气胸8例,胸背部皮下气肿7例,纵膈气肿6例,胸椎骨折6例。结论：肺撕裂伤的CT表现具特征性,CT对伤后定位、定性诊断,对临床诊断及治疗具有重要指导价值。     

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.     

Differentiation between Birt-Hogg-Dubé syndrome and lymphangioleiomyomatosis: quantitative analysis of pulmonary cysts on computed tomography of the chest in 66 females

Backgrounds

Since Birt–Hogg–Dubé syndrome (BHDS) and lymphangioleiomyomatosis (LAM) share some clinical manifestations (multiple pulmonary cysts with pneumothorax, renal tumors, and sometimes skin lesions), the differential diagnosis of the two diseases becomes problem especially in female patients. This study aims to quantify pulmonary cysts in computed tomography (CT) of females with BHDS and those with LAM and also to identify the independent parameters for differentiating the two diseases.

Methods

Fourteen patients with BHDS and 52 with LAM were studied. In CT scans, lung fields were defined as areas with fewer than −200 Hounsfield units (HU) and pulmonary cysts as areas consisting of 10 or more consecutive pixels with fewer than −960 HU. The extent, number, size and circularity of cysts were calculated by using hand-made software and compared between the two diseases. Moreover, the lung fields were divided into six zones and analyzed for the distribution of cysts. Finally, a stepwise discriminant analysis employing quantitative measurements of cysts and clinical features was performed.

Results

The two diseases were significantly different in all quantitative measurements of cysts. Stepwise discriminant analysis accepted the following four variables: the family history of pneumothorax within the second degree relatives, lower-medial zone predominance of cysts, diffusing capacity and mean size of cysts in this order.

Conclusion

The quantitative characteristics of pulmonary cysts are significantly different between BHDS and LAM. The independent parameters for differentiating the two diseases are the family history of pneumothorax, zonal predominance of cysts, diffusing capacity and size of cysts.     

Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features

PURPOSE: To retrospectively compare the frequencies of computed tomographic (CT) findings in patients with lymphangioleiomyomatosis (LAM) and patients with tuberous sclerosis complex (TSC) and LAM. MATERIALS AND METHODS: Institutional review board approval and informed consent were obtained for the HIPAA-compliant study. In 256 patients with LAM (mean age, 44 years) and 67 patients with TSC/LAM (mean age, 40 years), CT scans of the chest, abdomen, and pelvis were reviewed by a single radiologist. The fraction of lung involvement with cysts was estimated from high-spatial-resolution CT scans. Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. Confidence intervals and hypothesis tests of differences in frequencies, comparison of age quartiles, RIDIT analysis, analysis of variance, and correlation coefficients were used in the statistical analysis. RESULTS: Patients with LAM had more extensive lung involvement (RIDIT score, 0.36) and higher frequency of LALM (29% vs 9%, P<.001), thoracic duct dilatation (4% vs 0, P=.3), pleural effusion (12% vs 6%, P=.2), or ascites (10% vs 6%, P=.3). Patients with TSC/LAM had higher frequency of noncalcified pulmonary nodules (12% vs 1%, P<.01), hepatic (33% vs 2%, P<.001) and renal (93% vs 32%, P<.001) AMLs, nephrectomy (25% vs 7%, P<.001), or renal artery embolization (9% vs 2%, P<.05). CONCLUSION: The extent of lung disease is greater in LAM than TSC/LAM. Hepatic and renal AMLs and noncalcified lung nodules are more common in TSC/LAM, while lymphatic involvement-thoracic duct dilatation, chylous pleural effusion, ascites, and LALM-is more common in LAM.