Ⅰ型神经纤维瘤病周围神经病变的声像图表现

目的 探讨Ⅰ型神经纤维瘤病(NF1)周围神经病变的声像图特征及分型.方法 分析36例NF1周围神经病变的超声表现,并分析其病理意义.结果 NF1周围神经病变(36例)为表现多样的神经纤维瘤,其高频声像图可分为四型:多发结节型、丛状型、弥漫型及混合型.多发结节型5例,肿瘤多发,呈椭圆形及分叶状,边界清晰,表现以低回声为主的混合性回声;丛状型12例,表现为肢体周围神经主干较长范围增粗,失去正常神经束结构,呈蔓丛状、串珠样低回声;弥漫型14例,肿瘤皮下浸润生长,皮下软组织明显增厚,结构紊乱,弥漫分布丛状及结节样低回声.混合型5例,周围神经病变为弥漫型及多发结节型神经纤维瘤并存,声像图兼具两型表现.结论 NF1周围神经病变具有特征性声像图表现,与临床病理表现基本相符,有助于临床明确诊断并提供治疗依据.

Abstract：

Objective To investigate the ultrasonographic features corresponding to classification of peripheral neuropathies in neurofibromatosis type Ⅰ.Methods Thirty-six patients with peripheral neuropathies and diagnosis of neurofibromatosis type I were enrolled in this study.Ultrasound appearance of peripheral neuropathies was analyzed and divided into four groups according to their pathological classifications.There were 19 males and 17 females with age between 7-47 years.High frequency or board frequency ultrasound probes were used for scanning.Results The ultrasound findings were varied based on different pathological classifications.Multiple nodular class(n=5) was presented as multiple nodes.Ultrasound demonstrated that tumors were multiple,oval or lobulated,and had well defined margin,and the echogenic of lesions was mainly hypoechoic.Plexiform class (n=12) was presented as cluster nodes along the peripheral nerve trunk.Ultrasound showed trunk nerves in extremities became thicker in a long range and lost the normal structure,and catenular or racemosum in shape.The echogenicity was hypoechoic.Diffuse class (n=14) was diffuse subcutaneous disease.Tumors range was infiltrative.The subcutaneous soft tissue was thicken and echogenicity was increased.The structure of nerve bundle was disturbed and associated with multiple hypoechoic nodes.Mixture class (n=5) was defined as mixture of multiple nodular class and plexiform class.In this group,diffuse disorders were co-existed with multiple nodes disorder.Conclusions The ultrasonographic appearances of peripheral nerve disorders in neurofibromatosis type I was closely corresponded to their pathological characters.And the ultrasonic examination would be helpful to diagnosis and provide important information for therapy.