Legras A, Mordant P, Le Pimpec-Barthes F, Riquet M
Service de chirurgie thoracique, université Paris 5, hôpital européen Georges-Pompidou, Assistance publique-Hôpitaux de Paris, 20, rue Leblanc, 75015 Paris, France.
Rev Pneumol Clin. 2013 Oct;69(5):272-7. doi: 10.1016/j.pneumo.2013.04.002. Epub 2013 May 18.
Primary thoracic lymphatic diseases are both infrequent and probably under diagnosed. The two major forms are lymphangiomas and lymphangiectasias. Lymphangiomas are focal proliferation of well-differentiated lymphatic vessels. Childhood lymphangiomas may follow embryologic disorders. Adult lymphangiomas are more likely secondary to lymphatic obstruction. When associated with typical CT and MRI features, their surgical resection is not mandatory, whereas in case of diagnostic difficulties or related complications, surgical resection is the rule. Lymphangiectasias are congenital or acquired pathologic lymphatic dilatation from pleura and interlobular septa without any proliferation. These diseases can be limited to one pulmonary lobe, or can involve the whole lymphatic network. In case of communication between the lymphangiectasias and the thoracic duct, symptoms may include chyloptysis, chylothorax, and chylopericardium. Lymphangio MRI allows visualisation of the lymphangiectasis and thoracic duct. Surgical treatment may be required in case of resistance to medical treatment.
原发性胸段淋巴管疾病既不常见，也可能存在诊断不足的情况。两种主要类型是淋巴管瘤和淋巴管扩张症。淋巴管瘤是分化良好的淋巴管的局灶性增生。儿童淋巴管瘤可能与胚胎发育异常有关。成人淋巴管瘤更可能继发于淋巴管阻塞。当具有典型的CT和MRI特征时，不一定需要手术切除，而在诊断困难或出现相关并发症的情况下，则通常需要手术切除。淋巴管扩张症是胸膜和小叶间隔的先天性或后天性病理性淋巴管扩张，无任何增生。这些疾病可局限于一个肺叶，也可累及整个淋巴网络。当淋巴管扩张症与胸导管相通时，症状可能包括乳糜痰、乳糜胸和乳糜心包。淋巴管MRI可显示淋巴管扩张症和胸导管。如果药物治疗无效，可能需要手术治疗。
