Yamaki Haruna, Ejima Masaru, Takeguchi Takaya, Kagohashi Chihiro, Hanawa Shunya, Kubota Natsushi, Hanawa Kotaro, Higashi Seishi, Hanada Satoko, Taki Reiko
Department of Respiratory Medicine, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo, 180-8610, Japan.
Department of Radiology, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino-shi, Tokyo, 180-8610, Japan.
Respir Med Case Rep. 2025 Mar 19;55:102195. doi: 10.1016/j.rmcr.2025.102195. eCollection 2025.
Gorham-Stout disease (GSD) is a rare vascular disease of lymphatic origin characterized by progressive osteolysis that commonly causes chylothorax owing to the leakage of lymphatic fluid from dissolved bones. We report a case of refractory chylothorax that was diagnosed as elderly onset GSD and treated successfully using multidisciplinary approaches. A 78-year-old male presented with persistent cough and shortness of breath caused by massive left pleural effusion. Thoracentesis and pleural biopsy revealed an initial diagnosis of idiopathic chylothorax. The patient underwent continuous thoracic drainage and several percutaneous thoracic duct embolization with subcutaneous octreotide acetate injection and a low-fat diet. Thoracoscopic surgery was performed to control the secondary empyema through the drain tube and repair the pleural lesions with lymphatic leakage. A sufficient decrease in pleural fluid volume allowed temporary removal of the thoracic drainage tube. However, thoracic drainage was resumed for refractory chylothorax when the patient gradually developed neck pain after three months. Computed tomography, magnetic resonance imaging, and bone scintigraphy revealed growing osteolytic lesions in the cervical and thoracic spine regions, and biopsy demonstrated numerous dilated thin-walled capillary structures with lymphocyte infiltrates. After diagnosing GSD and introducing sirolimus, the patient's symptoms improved, and thoracic drainage was discontinued. Multidisciplinary treatments successfully halted disease progression for over a year. The case report emphasizes the systematic examination to identify rare etiology in refractory chylothorax treatment.
戈勒姆-斯托特病（GSD）是一种罕见的起源于淋巴管的血管疾病，其特征为进行性骨质溶解，由于溶解的骨骼中淋巴液渗漏，常导致乳糜胸。我们报告一例难治性乳糜胸病例，该病例被诊断为老年起病的戈勒姆-斯托特病，并采用多学科方法成功治疗。一名78岁男性因大量左侧胸腔积液出现持续咳嗽和气短。胸腔穿刺和胸膜活检初步诊断为特发性乳糜胸。患者接受了持续胸腔引流、多次经皮胸导管栓塞术，同时皮下注射醋酸奥曲肽并采用低脂饮食。通过引流管进行胸腔镜手术以控制继发性脓胸，并修复有淋巴渗漏的胸膜病变。胸腔积液量充分减少后，暂时拔除了胸腔引流管。然而，三个月后患者逐渐出现颈部疼痛，因难治性乳糜胸再次进行胸腔引流。计算机断层扫描、磁共振成像和骨闪烁显像显示颈椎和胸椎区域出现不断增大的骨质溶解病变，活检显示有大量扩张的薄壁毛细血管结构伴淋巴细胞浸润。诊断为戈勒姆-斯托特病并使用西罗莫司后，患者症状改善，胸腔引流停止。多学科治疗成功阻止疾病进展超过一年。该病例报告强调在难治性乳糜胸治疗中进行系统检查以确定罕见病因。
