Fidalgo I, Ortega F, Alustiza J, Pastor E, Cabrera A
An Esp Pediatr. 1980 Dec;13(12):1085-90.
Four cases of congenital pulmonary lymphangiectasis were observed during a five year period. This represents an incidence of 1.11 per 10,000 cases among alive newborns and of 53 per 10,000 cases among pediatric necropsias performed during the same period. One case was observed in the clinical context of a generalized hemangiolymphangiomatosis, another was associated to tetralogy of Fallot and the remaining two cases were associated to obstruction of pulmonary venous return. Although one of the pathogenic theories generally accepted in the formation of pulmonary lymphangiectasis points to the presence of either hypertension or obstruction of pulmonary venous drainage, it is possible that such situation is only circumstantial. Among 40 personal cases of obstruction of pulmonary venous return proved anatomically, only in the two cases presented were pulmonary lymphangiectasis demonstrated. The presence of dysplastic elements in pulmonary tissue in cases of lymphangiectasis suggests that a more possible mechanism is a primary defect in the development of pulmonary lymphatics.
在五年期间观察到4例先天性肺淋巴管扩张症。这意味着在存活新生儿中发病率为每10000例中有1.11例，在同期进行的儿科尸检中每10000例中有53例。1例在全身性血管淋巴管瘤病的临床背景下观察到，另1例与法洛四联症相关，其余2例与肺静脉回流受阻相关。尽管在肺淋巴管扩张症形成中普遍接受的一种致病理论指出存在高血压或肺静脉引流受阻，但这种情况可能只是偶然的。在经解剖证实的40例个人肺静脉回流受阻病例中，只有所呈现的2例显示有肺淋巴管扩张症。淋巴管扩张症病例中肺组织存在发育异常成分提示，更可能的机制是肺淋巴管发育的原发性缺陷。
