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[一例合并多发肝动门脉分流的肺动静脉瘘]

[A case of pulmonary arteriovenous fistula associated with multiple hepatic arterioportal shunts].

作者信息

Shukuya Takehito, Atsuta Ryo, Morio Yoshiteru, Sato Koichi, Suzuki Tsutomu, Seyama Kuniaki, Takahashi Kazuhisa

机构信息

Department of Respiratory Medicine, Juntendo University, School of Medicine.

出版信息

Nihon Kokyuki Gakkai Zasshi. 2007 Sep;45(9):715-9.

Abstract

We report a case of pulmonary arteriovenous fistula with multiple hepatic arterioportal shunts. A 23-year-old man was admitted for evaluation of a nodular shadow in the right lower lung field. Chest CT showed a homogeneous and well-defined nodular shadow in the right S7. 3-dimensional CT revealed an arteriovenous fistula composed of a feeding artery and a draining vein in the right S7. Abdominal enhanced CT revealed a diffusely heterogeneous pattern in the liver parenchyma and the portal vein staining diffusely at the late arterial phase. We diagnosed pulmonary arteriovenous fistula associated with multiple hepatic arterioportal shunts. Pulmonary arteriovenous fistula is often associated with hereditary hemorrhagic telangiectasia (HHT). This case did not meet the criteria for HHT at this time. However, we could not exclude the possibility of HHT because clinical manifestations of HHT become apparent after 40 years old among 10% of patients. The natural history and treatment of HHT are not clear. We concluded that we should be careful to detect possible manifestations as HHT when we diagnose and follow up patients with pulmonary arteriovenous fistula.

摘要

我们报告一例伴有多发肝动门静脉分流的肺动静脉瘘病例。一名23岁男性因右下肺野结节影入院评估。胸部CT显示右S7有一个均匀且边界清晰的结节影。三维CT显示右S7有一个由供血动脉和引流静脉组成的动静脉瘘。腹部增强CT显示肝实质呈弥漫性不均匀强化,门静脉在动脉晚期弥漫性染色。我们诊断为伴有多发肝动门静脉分流的肺动静脉瘘。肺动静脉瘘常与遗传性出血性毛细血管扩张症(HHT)相关。该病例目前不符合HHT的诊断标准。然而,我们不能排除HHT的可能性,因为10%的患者中HHT的临床表现会在40岁以后才显现出来。HHT的自然病史和治疗方法尚不清楚。我们得出结论,在诊断和随访肺动静脉瘘患者时,应谨慎检测可能出现的HHT表现。

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