Schlolaut Bettina, Schild Hans Heinz, Pfannschmidt Joachim, Kaiser Dirk
Department of Thoracic Surgery, Lung Clinic Heckeshorn at HELIOS Hospital Emil von Behring, Berlin, Germany.
Department of Radiology, University of Bonn, Bonn, Germany.
Thorac Cardiovasc Surg Rep. 2014 Dec;3(1):64-6. doi: 10.1055/s-0034-1394162. Epub 2014 Oct 31.
Chylothorax originating in a patient with Schimmelpenning syndrome is rare and poses a problem in diagnosis and treatment. A 22-year-old male was admitted with dyspnea indicative of a large pleural chylous effusion. Besides conservative dietary treatment measures, the chylous effusion was drained (2,000 mL/day). Computed tomography-lymphography after ligation of the thoracic duct and pleurectomy revealed a small collateral flow of chylous fluid toward the chest wall and entering the thorax. Eventually, local radiation therapy with 36 Gy effectively treated the chylothorax. Five months later, an epitheloid angiosarcoma developing from a preexisting cutaneous lesion was detected and treated by surgical resection.
起源于施密尔彭宁综合征患者的乳糜胸很罕见，在诊断和治疗方面存在问题。一名22岁男性因呼吸困难入院，提示存在大量胸腔乳糜性积液。除了保守的饮食治疗措施外，还对乳糜性积液进行了引流（每天2000毫升）。在胸导管结扎和胸膜切除术后进行的计算机断层扫描淋巴造影显示，有少量乳糜液向胸壁侧支流动并进入胸腔。最终，36 Gy的局部放射治疗有效地治愈了乳糜胸。五个月后，检测到由先前存在的皮肤病变发展而来的上皮样血管肉瘤，并通过手术切除进行了治疗。
