Poggiani C, Auriemma A, Bellan C, Pasinetti G, Colombo A
Divisione di Patologia Neonatale, Ospedali Riuniti, Bergamo.
Minerva Pediatr. 1998 Mar;50(3):91-6.
Imaging investigations and other findings observed in a term infant with a multicentric hepatic hemangioendothelioma, admitted to the Intensive Care Unit at the age of 13 days because of non specified feeding difficulties and dyspnoea, are presented. Physical examination revealed cardiac bruit and congestive heart failure with marked hepatomegaly; in addition there were multiple small skin hemangiomas. Echocardiography was negative, abdominal sonography showed multiple round lesions of mixed echogenicity in the liver, large vascular channels, a right hepatic artery and hepatic veins enlarged, a caliber of the aorta below the level of the superior mesenteric artery reduced. The infant was additionally investigated by whole-body scintigraphy with 99mTc-labeled red blood cells to determine the possibility of coexistence of other visceral hemangiomas and by MR, in which the tumor manifested as multiple well-circumscribed space-occupying nodules of high signal intensity on T2-weighted images with evidence of fast flow. The baby underwent furosemide and steroid therapy: serial two-dimensional US scans showed change in echogenicity, responding to therapy. Doppler sonography has proven to be also very useful in the monitoring therapy determining changes in flow pattern and velocity at the level of hepatic, cerebral and renal vessels: before therapy we observed a reduction of the diastolic flow until the zero line through the internal carotid artery and renal artery with an increase of the Resistance Index. It means that this important component can be compromised in the presence of a hepatic hemangioendothelioma.
本文介绍了一名足月婴儿的影像学检查及其他检查结果。该婴儿患有多中心性肝血管内皮瘤,因不明原因的喂养困难和呼吸困难于13日龄时入住重症监护病房。体格检查发现心脏杂音和充血性心力衰竭,伴有明显肝肿大;此外还有多个小的皮肤血管瘤。超声心动图检查结果为阴性,腹部超声显示肝脏有多个混合回声的圆形病变、大的血管通道、右肝动脉和肝静脉增粗,肠系膜上动脉水平以下的主动脉管径变窄。该婴儿还接受了99mTc标记红细胞的全身闪烁扫描,以确定是否存在其他内脏血管瘤,以及磁共振成像检查,磁共振成像显示肿瘤在T2加权图像上表现为多个边界清晰的高信号强度占位结节,有血流加速迹象。婴儿接受了速尿和类固醇治疗:系列二维超声扫描显示回声性有变化,对治疗有反应。多普勒超声在监测治疗过程中也被证明非常有用,可确定肝、脑和肾血管水平的血流模式和速度变化:治疗前,我们观察到通过颈内动脉和肾动脉的舒张期血流减少至零线,阻力指数增加。这意味着在存在肝血管内皮瘤的情况下,这一重要组成部分可能会受到影响。