一名女性因全身性淋巴管异常导致乳糜胸和缩窄性心包炎：病例报告
Chylothorax and constrictive pericarditis in a woman due to generalized lymphatic anomaly: a case report.
作者信息
Du Hongchun, Xiong Mai, Liao Huai, Luo Yifeng, Shi Huijuan, Xie Canmao
机构信息
Department of Respiratory Medicine, The First Affiliated Hospital, Sun Yat-sen University, No 58, Zhongshan 2nd Road, Guangzhou, 510080, China.
Department of Cardiac Surgery, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.
出版信息
J Cardiothorac Surg. 2018 Jun 5;13(1):59. doi: 10.1186/s13019-018-0752-3.
BACKGROUND
Generalized lymphatic anomaly (GLA) is characterized by diffuse or multicentric proliferation of dilated lymphatic vessels resembling common lymphatic malformations. Compared with soft tissue or bone involvement, thoracic involvement may be associated with a worse prognosis.
CASE PRESENTATION
We reported a case of GLA with chylothorax and constrictive pericarditis in a 29-year-old woman. This patient exhibited remarkable features, including a continuously hemorrhagic chylothorax, constrictive pericarditis, and involvement of bone and neck lymph nodes. After attempting to manage her condition with conservative treatment, the patient underwent pericardial stripping surgery. Exploration revealed abundant hyperplasia of tubular tissue in the aortopulmonary window in both pleural cavities.
CONCLUSIONS
This case highlights the importance of maintaining the clinical suspicion of GLA during the follow-up of chylothorax patients. Aggressive pericardial surgery, which is important for both diagnosis and treatment, should be performed in patients with GLA with constrictive pericarditis.
背景
广泛性淋巴管异常（GLA）的特征是扩张的淋巴管呈弥漫性或多中心性增殖，类似于常见的淋巴管畸形。与软组织或骨骼受累相比，胸部受累可能预后更差。
病例报告
我们报告了一例29岁女性的GLA合并乳糜胸和缩窄性心包炎。该患者表现出显著特征，包括持续性出血性乳糜胸、缩窄性心包炎以及骨骼和颈部淋巴结受累。在尝试采用保守治疗管理其病情后，患者接受了心包剥脱手术。探查发现双侧胸腔主动脉肺动脉窗处有大量管状组织增生。
结论
该病例强调了在乳糜胸患者随访过程中保持对GLA临床怀疑的重要性。对于合并缩窄性心包炎的GLA患者，积极的心包手术对于诊断和治疗都很重要，应予以实施。
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