Hyland Liam D, Elsayed Abdelrahman, Hawari Mohammad
Thoracic Surgery Department, Nottingham University Hospitals NHS Trust, UK.
Ann Thorac Surg Short Rep. 2024 Jul 4;2(4):665-668. doi: 10.1016/j.atssr.2024.06.020. eCollection 2024 Dec.
Gorham-Scout disease (GSD) is a rare skeletal disorder of unknown etiology characterized by progressive osteolysis and excessive lymphovascular proliferation. Chylothorax is a life-threatening complication. A teenager presented with a left pleural effusion on a background of chronic flank collection secondary to lymphovascular malformation. Cytology of the fluid confirmed a chylothorax and rib excision biopsy confirmed GSD. The patient underwent thoracotomy, decortication, and pleurectomy, which helped to stabilize the chylothorax; ongoing management involved radiotherapy and bisphosphonates. GSD is a rare disease that can develop a number of severe complications including chylothorax. Management via surgical and radiotherapeutic techniques can provide long-term remission.
戈勒姆-斯库特病（GSD）是一种病因不明的罕见骨骼疾病，其特征为进行性骨质溶解和过度的淋巴管增生。乳糜胸是一种危及生命的并发症。一名青少年在继发于淋巴管畸形的慢性胁腹积液背景下出现左侧胸腔积液。液体的细胞学检查确诊为乳糜胸，肋骨切除活检确诊为GSD。患者接受了开胸手术、纤维板剥脱术和胸膜切除术，这有助于稳定乳糜胸；后续治疗包括放疗和双膦酸盐。GSD是一种罕见疾病，可引发包括乳糜胸在内的多种严重并发症。通过手术和放射治疗技术进行管理可实现长期缓解。
