Canil K, Fitzgerald P, Lau G
Division of Pediatric Surgery, Children's Hospital at Chedoke-McMaster, Hamilton, Ontario.
J Pediatr Surg. 1994 Sep;29(9):1186-8. doi: 10.1016/0022-3468(94)90796-x.
Chylothorax in the absence of tumor or trauma is uncommon. Lymphangiomatosis of the bone, although extremely rare, has been associated with chylothorax. The authors describe the case of a 12-year-old boy who presented with a symptomatic left chylothorax associated with lymphangiomatosis of the ribs, scapula, and clavicle. Despite tube thoracostomies and the initiation of total parenteral nutrition, massive losses of chyle persisted, resulting in hypoproteinemia and severe lymphopenia. Control of the chylothorax was achieved by a parietal pleurectomy and application of fibrin glue (Tisseel). In the literature there are 16 cases of chylothorax associated with lymphangiomatosis of the bone. Their presentation, treatment, and outcome are reviewed. Conservative treatments such as dietary manipulations or thoracenteses were rarely successful. Thoracotomy with parietal pleurectomy on the side of the effusion is usually effective in controlling the chylothorax. Lymphangiomatosis should be considered a diagnostic possibility for any child who presents with a chylothorax.
无肿瘤或创伤情况下的乳糜胸并不常见。骨淋巴管瘤病虽然极为罕见，但与乳糜胸有关。作者描述了一名12岁男孩的病例，该男孩出现有症状的左侧乳糜胸，伴有肋骨、肩胛骨和锁骨的淋巴管瘤病。尽管进行了胸腔闭式引流术并开始了全胃肠外营养，但乳糜大量流失仍持续存在，导致低蛋白血症和严重淋巴细胞减少。通过壁层胸膜切除术和应用纤维蛋白胶（Tisseel）实现了乳糜胸的控制。文献中有16例骨淋巴管瘤病相关的乳糜胸病例。对其临床表现、治疗及转归进行了综述。饮食调整或胸腔穿刺等保守治疗很少成功。在积液侧进行开胸壁层胸膜切除术通常能有效控制乳糜胸。对于任何出现乳糜胸的儿童，都应考虑淋巴管瘤病的诊断可能性。
