Massimo L, Di Iorio G, Martone A, Sottile R, Retolatto A, Loiodice F, Campa A, Angelone S
I Divisione di Pediatria, Ospedale Santobono, USL 40 Napoli, Italia.
Pediatr Med Chir. 1991 Jan-Feb;13(1):99-103.
G.L.D. is a rare syndrome characterized by chylothorax, chylous ascites and lymphedema associated to minor symptoms following from lymphangiectasis or to lymphangiomatosis. This syndrome is caused by congenital dysplasia of lymphatic vessels and has, generally, an extremely severe prognosis. In the present papers the authors describe one case of G.L.D. in a six months old infant with chylous ascites, chylothorax, lymphedema, hemangioma and a history of fetal ascites. The preservative (dietetic and evacuative) therapy was not resolutive, therefore an exploratory laparotomy was necessary, but no surgical solution was possible because of the absence of chylous vessels in the anatomical region of the principal lymphatic route. However, laparatomy led to a progressive recovery of ascites and chylothorax, probably due to thrombosis of dysplasic lymph vessels and to neoformation of collateral circulation.
戈尔登综合征（G.L.D.）是一种罕见的综合征，其特征为乳糜胸、乳糜性腹水和淋巴水肿，并伴有淋巴管扩张或淋巴管瘤病引发的轻微症状。该综合征由淋巴管先天性发育异常引起，通常预后极差。在本文中，作者描述了一例6个月大婴儿的戈尔登综合征病例，该婴儿患有乳糜性腹水、乳糜胸、淋巴水肿、血管瘤且有胎儿腹水病史。保守（饮食和排泄）治疗未能解决问题，因此有必要进行剖腹探查，但由于主要淋巴途径的解剖区域没有乳糜管，无法进行手术治疗。然而，剖腹手术导致腹水和乳糜胸逐渐恢复，这可能是由于发育异常的淋巴管血栓形成以及侧支循环的重新形成。
