Akcakus Mustafa, Koklu Esad, Bilgin Mehmet, Kurtoglu Selim, Altunay Levent, Canpolat Mehmet, Budak Nurten
Division of Neonatology, Department of Paediatrics, School of Medicine, Erciyes University, Kayseri, Turkey.
Neonatology. 2007;91(4):256-9. doi: 10.1159/000098172. Epub 2006 Dec 22.
Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops, and the prognosis is reported to be very poor. Treatments for lymphangiectasia have included corticosteroids for patients with primary inflammatory conditions, dietary modifications, surgical resection for isolated lesions, octreotide, antiplasmin therapy and fibrin glue pleurodesis. However, there is no experience with pleurodesis by autologous blood therapy in the literature. We present a newborn with primary pulmonary lymphangiectasis who developed progressively profuse chylous pleural effusions after enteral full feeding from the 8th day of life and improved with pleurodesis by autologous blood therapy.
先天性肺淋巴管扩张症是一种罕见疾病，可能在产前表现为胸腔积液和水肿，据报道其预后很差。淋巴管扩张症的治疗方法包括针对原发性炎症性疾病患者使用皮质类固醇、饮食调整、对孤立性病变进行手术切除、使用奥曲肽、抗纤溶治疗以及纤维蛋白胶胸膜固定术。然而，文献中尚无自体血疗法进行胸膜固定术的经验。我们报告了一名患有原发性肺淋巴管扩张症的新生儿，该患儿自出生第8天经肠道完全喂养后逐渐出现大量乳糜性胸腔积液，并通过自体血疗法进行胸膜固定术而病情改善。
