脾脏血管性病变与淋巴瘤CT鉴别诊断

目的 运用CT区分脾脏血管性病变与淋巴瘤。方法 回顾性分析20例经手术、穿刺病理学检查证实的脾脏病变的发病年龄、性别、脾脏指数、病变大小、数目、有无液化、钙化、强化幅度、强化方式等特征,并进行统计学分析。结果 20例脾脏病变中,11例血管性病变(6例海绵状血管瘤,3例窦岸细胞血管瘤,2例硬化性血管瘤样结节性转化),9例淋巴瘤;两组间发病年龄、病变大小、数目、有无液化、钙化等差异无统计学意义;两组间脾脏指数、动脉期强化幅度差异具有统计学意义(P＜0.05)。5例海绵状血管瘤呈不均匀性强化,1例呈渐进性填充式强化,2例窦岸细胞血管瘤呈“雀斑征”,1例硬化性血管瘤样结节性转化呈“辐轮征”;9例淋巴瘤实质部分均呈均匀、轻中度强化。结论 脾脏血管性病变与淋巴瘤CT表现不同,CT有助于明确诊断。     

Report of a child with aortic aneurysm,orofacial clefting,hemangioma, upper sternal defect,and Marfanoid features: Possible PHACE syndrome

We report a female patient who had a scalp hemangioma, a cleft uvula, an upper sternal defect, pectus excavatum, arachnodactyly, pes planus, and joint hypermobility. She had rupture of an aortic aneurysm after minor trauma at 11 years of age. At 17 years of age, elective repair of a dilated, ectatic aorta was complicated by cerebral ischemia. Other vascular abnormalities in the proband included an aneurysm of the left subclavian artery, atresia of the right carotid artery, and calcified cerebral aneurysms. We believe that the proband's physical anomalies are best described by the PHACE (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) phenotypic spectrum. This spectrum of physical anomalies also includes sternal clefting and hemagiomas as part of the sternal malformation/vascular dysplasia (SM/VD) association, as found in our patient, and the acronym PHACES has also been used. We consider that the PHACE phenotypic spectrum is likely to be broader than previously recognized and includes orofacial clefting and aortic dilatation and rupture. Our patient also had skeletal anomalies that lead to consideration of Marfan syndrome as a diagnosis. It should be recognized that there is clinical overlap between PHACE syndrome and Marfan syndrome when aortic dilatation is present. We would also like to emphasize the minor nature of the cutaneous findings in our patient despite her severe vascular complications. This is in contrast to previous reports of large or multiple hemangiomas in PHACE syndrome. Published 2002 Wiley‐Liss, Inc.     

增殖细胞核抗原在血管瘤和血管畸形中的表达及其临床意义

目的探讨不同类型血管瘤、血管畸形和同一类型不同时期的内皮细胞增殖情况及肥大细胞计数的变化。方法采用免疫组织化学方法及细胞特染技术,对120例婴幼儿和28例成人血管瘤标本进行了增殖细胞核抗原(PCNA)检测和肥大细胞计数比较。结果增生期毛细血管瘤、混合型血管瘤PCNA表达为阳性,其他均为阴性。经统计学处理,各类增生期血管瘤PCNA表达阳性率及肥大细胞计数均明显高于消退期婴幼儿血管瘤及各类血管畸形,有显著差异(P<0.01)。结论PCNA的表达对血管瘤和血管畸形的鉴别诊断及治疗选择具有指导意义;血管瘤的内皮细胞增生与肥大细胞增多有密切关系。     

非典型肝海绵状血管瘤的CT诊断

目的探讨非典型肝海绵状血管瘤（hepatic cavernous hemangiomas, HCH）的CT表现及相关病理学基础.方法回顾性分析18例共21个非典型HCH的CT表现,包括病灶大小、平扫密度、动态增强模式及邻近区域改变.结果平扫有13个病灶呈非均匀密度表现.出现4种非典型动态增强模式：Ⅰ型（5例）表现为动脉期病灶快速均匀强化,门静脉期及延迟期持续强化;Ⅱ型（13例）动脉期周边结节状强化,门静脉及延迟期向心性强化,但不能完全填充整个病灶;Ⅲ型（2例）动脉期病灶呈中央强化,门静脉期及延迟期增强向周边区呈离心性强化;Ⅳ型（1例）动脉期无明显强化,门静脉期及延迟期呈轻度周边强化或不规则强化.HCH邻近区域的改变：有7个病灶可见动脉-门静脉分流（arterial-portal verous shunt, APVS）,4个病灶可见肝包膜回缩（hepatic capsular retraction, HCR）.以上非典型CT表现的出现在不同大小的病灶中有差别.结论非典型肝海绵状血管瘤CT表现的出现与HCH本身的病变结构特征密切相关,认识到这些非典型表现对HCH的诊断和鉴别诊断有重要意义.     

Transcatheter Arterial Sclerosing Embolization for the Treatment of Giant Propranolol-Resistant Infantile Hemangiomas in the Parotid Region

PurposeTo report the effectiveness and safety of transcatheter arterial sclerosing embolization (TASE) for the treatment of parotid infantile hemangiomas that did not respond appreciably to propranolol.Materials and MethodsA total of 21 infants (12 male and 9 female) with large propranolol-resistant infantile hemangiomas in the parotid region were enrolled in this study. During TASE, the feeding arteries of the lesions were embolized using pingyangmycin-lipiodol emulsion and polyvinyl alcohol particles (300–500 μm) to reduce the blood flow rate. All children were followed up as outpatients at 2 weeks and monthly thereafter. The curative effect was evaluated at the 1- and 3-month follow-up visits.ResultsNine lesions were located on the right side of the parotid gland, whereas 12 were located on the left side. The feeding arteries in all patients originated from branches of the external carotid artery. TASE was technically successful in all patients. The mean (± SD) maximal diameter of the hemangiomas significantly decreased from 6.50 cm ± 2.28 before treatment to 3.56 cm ± 1.84 at 1 month after TASE (P <. 05). Three months after TASE, the mean maximal diameter further significantly decreased to 1.94 cm ± 1.58 (P <. 05). During the follow-up period, 16 cases were rated as excellent and 5 as good; no recurrence or serious complications were noted. Minor side effects, such as slight pain, mild fever, and tissue swelling, were observed.ConclusionsTASE significantly decreased the size of the parotid hemangiomas with minor side effects during a short follow-up period.     

Multifocal infantile hemangiomas associated with concomitant giant hepatic rapidly involuting congenital hemangioma

We present a 9-day-old girl with multifocal cutaneous and hepatic infantile hemangiomas as well as a hepatic rapidly involuting congenital hemangioma. These two distinct vascular tumors have rarely been reported to co-occur. We additionally review the sonographic features that distinguish a hepatic congenital hemangioma from the hepatic infantile hemangioma.     

普萘洛尔治疗婴幼儿血管瘤的研究进展

婴幼儿血管瘤是婴幼儿时期最常见的先天性良性血管内皮细胞增生性肿瘤。普萘洛尔为非选择性β受体阻断药,临床常用于治疗心律失常、心绞痛、高血压等,普萘洛尔对血管瘤治疗作用的意外发现,使国内外掀起了对这个经典老药研究的热潮。普萘洛尔对婴幼儿血管瘤的疗效在临床观察中得到了肯定,其安全性及安全剂量尚需要大规模样本对照研究做进一步评估。