Carmona S, Loureiro M J, Santos J, Oliveira A, Camacho R, Santos A I
Servicio de Medicina Nuclear, Hospital Garcia de Orta, Almada, Portugal.
Rev Esp Med Nucl Imagen Mol. 2013 Mar;32(2):98-101. doi: 10.1016/j.remn.2012.06.009. Epub 2012 Sep 12.
Pulmonary capillary hemangiomatosis is a rare disease, characterized by small capillaries proliferation, leading to pulmonary hypertension. The authors report a case of pulmonary capillary hemangiomatosis, and discuss its diagnostic difficulties. Special attention is presented to ventilation/perfusion scintigraphy, given both its importance to the evaluation of pulmonary hypertension, and its referred limited usefulness in pulmonary capillary hemangiomatosis. The few published cases of ventilation/perfusion scintigraphy on this entity have showed different patterns, which are discussed. This case presents a pattern with augmented perfusion on lung bases and normal ventilation, which has been described by other authors as typical for pulmonary capillary hemangiomatosis. The authors consider important to retain this pattern, when evaluating pulmonary hypertensive patients, given not only its possible ability to help on pulmonary capillary hemangiomatosis diagnosis, but also mainly its risk of misinterpretation as a decreased perfusion on upper lung lobes, leading to erroneous diagnostic hypotheses.
肺毛细血管血管瘤病是一种罕见疾病，其特征为小毛细血管增生，导致肺动脉高压。作者报告了一例肺毛细血管血管瘤病病例，并讨论了其诊断难点。鉴于通气/灌注闪烁扫描对评估肺动脉高压的重要性及其在肺毛细血管血管瘤病中有限的实用性，故对其予以特别关注。已发表的关于该疾病的通气/灌注闪烁扫描的少数病例呈现出不同的模式，对此进行了讨论。该病例呈现出肺底部灌注增强而通气正常的模式，其他作者已将其描述为肺毛细血管血管瘤病的典型表现。作者认为，在评估肺动脉高压患者时，保留这一模式很重要，这不仅是因为其可能有助于诊断肺毛细血管血管瘤病，还主要是因为存在将其误解为上肺叶灌注减少的风险，从而导致错误的诊断假设。
