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。

Imaging diagnosis of the lymphangioleiomyomatosis

Objective To investigate the value of imaging diagnosis of the lymphangioleiomyomatosis( LAM ). MethodsFifteen patients with LAM confirmed by pathological assessment were analyzed retrospectively for radiologic findings. They had chest radiograph, chest highresolution CT (HRCT),abdominal CT, direct lymphangiography(DLG), chest CT and abdominal CT after DLG. Results Chest radiograph findings included normal (1), increasing of lung markings (3),disseminated honeycomb or reticular pattern ( 11 ), pneumothorax ( 2 ), and pleural effusion ( 14 ). Chest conventional CT and HRCT showed typical imaging manifestation of PLAM in all cases, including sporadic or disseminated cysts in bilateral lungs. According to the grading standard of pulmonary disease made by Avila et at, there were 3 cases in grade Ⅰ , 5 cases in grade Ⅱ and 7 cases in grade Ⅲ . Fourteen of 15 patients with LAM had positive abdominal CT findings in retroperitoneum and pelvic cavity. Common abdominal CT findings included cystic lymphangioma in 9 of 14 patients, lymphangiomyoma in 13 and both coexisting in 7.One of the 14 patients also had hepatic lipoma and angiomyolipomas. One patient had renal angiomyolipomas; and one patient had hysteromyoma. All 15 cases underwent DLG, 1 cases had lymphatic obstruction in the lumbar 3 level, the remaining 14 cases had varying degrees of thoracic duct stenosis, or obstruction. Neck trunk, subclavian trunk and bronchial trunk showed lymphatic reflux. On post-DLG CT,thoracic duet outlet obstruction was not demonstrated in 3 cases, the remaining 12 cases showed thoracic outlet obstruction, consistent with the DLG findings. Conclusion HRCT is a useful diagnostic method showing characteristic findings of PLAM. MSCT can help to detect abdominal LAM. DLG and MSCT after DLG have value in displaying obstruction site of thoracic duct or lymphatic trunks and provide guidance for operative treatment.