Shiraga Kazuhiro, Terui Keita, Ishihara Kazuaki, Shibuya Kiyoshi, Saito Eriko, Ito Kenichiro, Hiramoto Ryugo
Department of Pediatrics, Matsudo City Hospital Children's Medical Centre, Matsudo, Chiba, Japan.
Ann Thorac Cardiovasc Surg. 2014;20 Suppl:654-8. doi: 10.5761/atcs.cr.12-02244. Epub 2013 Oct 3.
A 21-month-old Japanese boy was admitted with cough and hypoxemia. Chest X-ray showed massive right pleural effusion, which consisted of chyle. Computed tomography showed poor contrast area at superior and anterior mediastinum. Magnetic resonance imaging showed granular T2-low area at the same area. Lymphangioscintigraphy revealed a hot spot at superior mediastinum. These findings lead us to diagnose as mediastinal lymphangioma accompanied with chylothorax. Noninvasive treatments including total parenteral nutrition, administration of octreotide and sclerotherapy were tried, but all of them proved to be ineffective. Transfusions of blood products were frequently needed during these therapies. On the 48th hospital day, the mediastinal tumor and the thymus were excised through a median sternotomy. A leakage point of lymph into the intrathoracic space was not found, in spite of preoperative administration of milk with dye. Since the pleural effusion had continued to be drained, pleuroperitoneal shunt was placed on the 90th hospital day. The shunting amount continued to decline soon after the shunting, and had been under 10 ml/day since the 142nd day. The shunt was removed on the 148th day. There has been no reaccumulation of the pleural effusion and no recurrence of the mediastinal tumor for 1 year of observation.
一名21个月大的日本男孩因咳嗽和低氧血症入院。胸部X线显示右侧大量胸腔积液，积液为乳糜液。计算机断层扫描显示上纵隔和前纵隔区域造影剂充盈不佳。磁共振成像显示同一区域有颗粒状T2低信号区。淋巴管闪烁造影显示上纵隔有一个热点。这些发现使我们诊断为纵隔淋巴管瘤伴乳糜胸。尝试了包括全胃肠外营养、奥曲肽给药和硬化治疗在内的非侵入性治疗，但均被证明无效。在这些治疗过程中经常需要输注血液制品。在住院第48天，通过正中胸骨切开术切除纵隔肿瘤和胸腺。尽管术前给予了含染料的牛奶，但未发现淋巴液漏入胸腔的漏点。由于胸腔积液持续引流，在住院第90天放置了胸膜腹膜分流管。分流后分流液量很快持续下降，自第142天起每天低于10毫升。在第148天移除了分流管。观察1年期间，胸腔积液未再积聚，纵隔肿瘤未复发。
