Yamakami Y, Mieno T, Tashiro T, Moriuchi A, Nasu M
Second Department of Internal Medicine, Oita Medical University, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1994 Mar;32(3):261-5.
A 34-year-old woman was admitted to our hospital because of exertional dyspnea and ascites. Chest roentgenogram showed hyperinflation of the lung and diffuse reticular shadows. Abdominal CT showed retroperitoneal tumors. Ascites was considered to be chylous. These findings strongly suggested the diagnosis of pulmonary lymphangiomyomatosis. Under lumbar anesthesia, laparotomy was performed, and the histological finding of the retroperitoneal tumor revealed angiomyoma. Bilateral oophorectomy was performed. Medroxyprogesterone at 15 mg/day was administered postoperatively. In advanced cases of this disease, open lung biopsy or even transbronchial lung biopsy is often avoided because of severe cardiopulmonary dysfunction. However, such cases associated with retroperitoneal tumor, laparotomy is quite useful not only in making the diagnosis but also in the treatment of this disease.
一名34岁女性因劳力性呼吸困难和腹水入住我院。胸部X线片显示肺过度充气和弥漫性网状阴影。腹部CT显示腹膜后肿瘤。腹水被认为是乳糜性的。这些发现强烈提示肺淋巴管平滑肌瘤病的诊断。在腰麻下行剖腹手术，腹膜后肿瘤的组织学检查显示为血管肌瘤。行双侧卵巢切除术。术后给予甲羟孕酮15mg/天。在这种疾病的晚期病例中，由于严重的心肺功能障碍，通常避免进行开胸肺活检甚至经支气管肺活检。然而，对于伴有腹膜后肿瘤的此类病例，剖腹手术不仅对诊断有用，而且对该病的治疗也很有用。
