Stanulla H, Ursinus W
Thoraxchir Vask Chir. 1976 Feb;24(1):15-23. doi: 10.1055/s-0028-1095859.
The clinical aspects, differential diagnosis and aetiology of the rare pulmonary glomangiosis are described by means of four cases observed in our clinic. It is a hamartogenous stenosing disease of the lungvessels, appearing predominately in children who suffer from congenital heart disease. It leads to pulmonary hypertension. The pulmonary glomangiosis is of practical importance: By palliative operation as the banding of the pulmonary artery the demand hypertension in the pulmonary circulation is suppressed artificially, so that in heavy cases a sufficient oxygenation is not provided, and the children die in the postoperative period. From the surgical aspect such a disease of the pulmonary vessels has to be supposed when the blood pressure distally of the experimentally banded pulmonary artery drops insufficiently. The authors recommend to exclude the disease of the vessels by excicion of lungtissue and immediate histologic investigation before the final banding is performed.
通过我们诊所观察到的4例病例，描述了罕见的肺血管球瘤病的临床特征、鉴别诊断及病因。它是一种肺血管错构瘤性狭窄疾病，主要发生于患有先天性心脏病的儿童，可导致肺动脉高压。肺血管球瘤病具有实际重要性：通过姑息性手术如肺动脉环扎术，人为地抑制肺循环中的需求性高血压，以至于在严重病例中无法提供足够的氧合，患儿会在术后死亡。从外科角度来看，当实验性环扎肺动脉远端血压下降不充分时，必须考虑存在这种肺血管疾病。作者建议在进行最终环扎之前，通过切除肺组织并立即进行组织学检查来排除血管疾病。
