Imaging diagnosis of the lymphangioleiomyomatosis / 中华放射学杂志

ABSTRACT

Objective Toinvestigatethevalueofimagingdiagnosisofthe 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 the14patients alsohadhepaticlipomaandangiomyolipomas.Onepatienthadrenal 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.