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首页> 外文期刊>Cardiovascular pathology: the official journal of the Society for Cardiovascular Pathology >Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association.
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Blue rubber bleb nevus syndrome and pulmonary hypertension: an unusual association.

机译：蓝色橡胶泡痣综合征与肺动脉高压：一种不寻常的关联。

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INTRODUCTION: Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital systemic angiodysplasia with multiple vascular malformations in the skin, gastrointestinal tract and, less often, in other internal organs and the brain. CASE REPORT: A 36-year-old man with past history of BRBNS was admitted to our hospital for progressive dyspnea and fatigue. Primary pulmonary hypertension (PPH) was diagnosed. He then developed acute abdominal pain and dyspnea, dying in a few hours due to sudden cardiac arrest. Postmortem examination demonstrated angiomatous lesions located in the skin, small bowel, heart, lungs, liver and thyroid. The lesions were slightly raised, soft and compressible and microscopically consisted of dilated vascular channels lined by a flattened endothelium. The vascular wall was formed by several layers of smooth muscle cells, intermixed with abundant aggregates of elastic lamellae and thin collagen fibers. Luminal thrombi were a frequent finding. In the small bowel, we identified the presence of an abnormally large artery directly opening into a thin-walled venous channel. The most striking finding in the lungs was the presence of thrombi of varying age in the lumen of segmental and elastic arteries, as well as muscular arteries and arterioles. Severe medial hypertrophy of muscular arteries and muscolarization of arterioles were also present. Intimal proliferative lesions and plexiform lesions were never observed. CONCLUSION: The pulmonary findings are consistent with recurrent thromboembolic events from shunts in the visceral lesions. To our knowledge, this is the first report of BRBNS with visceral arterovenous (AV) fistulae complicated by thromboembolic pulmonary hypertension (PH).
机译：简介：蓝色橡胶泡痣综合征（BRBNS）是一种罕见的先天性系统性血管增生，在皮肤，胃肠道以及其他内部器官和大脑中较少见，具有多种血管畸形。病例报告：一名具有BRBNS病史的36岁男子因进行性呼吸困难和疲劳而入院。诊断为原发性肺动脉高压（PPH）。然后，他出现了急性腹痛和呼吸困难，由于突然的心脏骤停而死于几个小时。验尸检查显示血管瘤病变位于皮肤，小肠，心脏，肺，肝和甲状腺。病变略微升高，柔软且可压缩，并且在显微镜下由扩张的血管通道组成，内衬扁平的血管。血管壁由几层平滑肌细胞形成，并与丰富的弹性薄片和薄胶原纤维聚集在一起。发光血栓是常见的发现。在小肠中，我们发现存在一条直接通向薄壁静脉通道的异常大动脉。肺部最显着的发现是节段性和弹性动脉腔以及肌肉性动脉和小动脉内腔中存在不同年龄的血栓。肌肉动脉严重内侧肥大和小动脉粘膜化也存在。从未观察到内膜增生性病变和丛状病变。结论：肺部发现与内脏病变分流复发血栓栓塞事件一致。据我们所知，这是BRBNS合并内脏动静脉瘘和血栓栓塞性肺动脉高压（PH）的首次报道。

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