Mora A, Cortés C, Mateo E M, Feliu M, Cabarrocas E
Servicio de Anestesiología y Reanimación, Hospital General de la Vall d'Hebron, Barcelona.
Rev Esp Anestesiol Reanim. 1991 Mar-Apr;38(2):109-11.
Pulmonary lymphangiomyomatosis (PLM) is an idiopathic disease of females in fertile age. It results in respiratory failure characterized by obliteration of the small airways, emphysema, formation of bullae, hemoptysis, pneumothorax, pulmonary fibrosis, severe hypoxemia and reduced carbon monoxide diffusion capacity. The major lymphatic ducts are also involved, resulting in chylous pleural effusion and ascites. Pulmonary abnormalities improve objectively and subjectively after surgical therapy, which consists in bilateral oophorectomy. Its evolution results in death in no more than 10 years. We report the anesthetic approach to 2 patients with PLM. Patient 1 was a female who had already been diagnosed of PLM and had received medical and surgical therapy, requiring reoperation for the resection of cystic intestinal masses and abdominal eventration. Patient 2 had been scheduled for bilateral oophorectomy after a diagnosis of PLM. We discuss the clinical condition of both patients, the course of the disease, the previous treatments and the anesthetic technique in each case.
肺淋巴管平滑肌瘤病（PLM）是一种育龄期女性的特发性疾病。它会导致呼吸衰竭，其特征为小气道闭塞、肺气肿、肺大疱形成、咯血、气胸、肺纤维化、严重低氧血症以及一氧化碳弥散能力降低。主要淋巴管也会受累，导致乳糜性胸腔积液和腹水。手术治疗（包括双侧卵巢切除术）后，肺部异常在客观和主观上均有改善。其病情发展会在不超过10年内导致死亡。我们报告了2例PLM患者的麻醉方法。患者1为女性，已被诊断为PLM并接受了药物和手术治疗，因切除囊性肠肿块和腹壁疝需要再次手术。患者2在被诊断为PLM后计划进行双侧卵巢切除术。我们讨论了两名患者的临床状况、病程、既往治疗以及每种情况下的麻醉技术。
