第177课 病例探析（026）—股骨嗜酸性肉芽肿

上期试题：肝性脑病的正确概念应是B

A．肝功能衰竭并发精神病

B．肝功能衰竭所致的神经精神综合征

C．肝功能衰竭并发昏迷

D．肝功能衰竭并发脑水肿

E．肝脏疾病并发脑部疾病

患者，8岁，1个月前无明显诱因出现右髋疼痛，以夜间疼痛为著，无发热，局部皮肤无红肿，无跛行。

右股骨近端可见一椭圆形骨质破坏区，边界清晰，未见胸膜骨膜反应。

右股骨近端髓腔内见椭圆形低密度骨质破坏区，边界清晰，邻近骨皮质局部破坏中断，可见层状骨膜反应。

冠状位T1WI

左：冠状位T2WI抑脂像

右：冠状位T1WI抑脂增强像

右股骨近端可见一椭圆形长T1信号,T2WI抑脂像上呈略高信号影，边缘可见低信号环，周围骨组织内可见大片状水肿信号，周围软组织轻度肿胀。

增强扫描：骨质破坏区及周围水肿区呈明显强化，二者之间可见均匀光滑的低信号环。

       骨嗜酸性肉芽肿是朗格汉斯组织细胞增生症的一种，病因为明，现多认为是一种自身免疫缺陷性肿瘤样病变，具有自限自愈、病变多发等特点。好发于青少年，男多于女，20岁以下占56%，以单发多见，亦可多发，单发性病变好发于扁骨、不规则骨，以颅骨最常见，多发性病变以椎体多见。发生于长骨者多位于骨干或干骺端，较少累及骨骺。全身症状较少，局部有疼痛、肿胀、肿块，可合并病理性骨折。

       Bone eosinophilic granuloma is langerhans cells hyperplasia, a kind of disease because of Ming, is now considered to be more a kind of autoimmune defective tumor-like lesions, since the limit of self-healing, lesions in multiple characteristics. Occurs in teenagers, male more than female, under the age of 20, 56%, than that with single or multiple, single lesions in flat bones, irregular bones, with the skull, the most common multiple lesions in vertebral bodies. It occurs in the backbone or the epiphyseal area and is less involved in the epiphysis. Systemic symptoms are less, local pain, swelling, mass, can combine pathological fracture.

影像学表现主要有以下四种There are four main types of imaging：

       1、骨质破坏，表现为受累骨不规则形溶骨性骨质破坏，破坏区内可见残留小骨片或死骨影。对发生于脊柱的病变，CT可避免组织重叠，显示骨质破坏、死骨较平片有优势。MRI可显示骨质破坏区形态结构、信号的异常改变，病变于T1WI像上呈低信号，T2WI像上呈高信号。

      1、 Bone destruction is manifested in the irregular bone fracture of the affected bone, which can be seen in the area of destruction. In the case of spinal lesions, CT can avoid the overlap of tissue, showing that bone destruction and dead bone are superior. MRI can show the morphological structure and abnormal changes of the bone damage area. The lesion is low in T1WI and the T2WI is high signal.

      2、骨膜反应发生于四肢长管状骨者，骨膜反应范围常大于骨质破坏。CT重组可显示平行骨干的层状或葱皮样骨膜反应。MRI像上骨膜反应表现为T1WI呈低信号，T2WI呈高信号。

      2、The periosteum reaction occurs in the extremities of the extremities, and the periosteum reaction is usually greater than that of the bone. The CT recombination can show the lamellar or scallion skin periosteum reaction of the parallel backbone. MRI is shown as a low signal of T1WI and T2WI is high signal.

     3、软组织肿胀，骨质破坏周围出现不同程度软组织肿胀，MRI像上呈低信号，T2WI呈高信号。发生于脊柱者，MRI可观察椎管狭窄、脊髓受压情况。

      3、 The soft tissue was swelling, the bone damage was caused by different degree of soft tissue swelling, and the MRI image was low signal, and T2WI was high signal. In the spinal column, MRI can observe the stenosis of the spinal canal and the compression of the spinal cord.

      4、本病可多发骨骼同时受累。脊柱病变可出现椎体变扁，但患椎椎间隙大多正常，发生于颅骨者可出现骨质缺损及‘纽扣样’死骨；此外，本症有自限倾向，可断续出现发病、修复、在发病的现象，具有‘此起彼伏’的特点。

      4、The disease can be found in many bones at the same time. Spinal lesions can be flattened by vertebral body, but most of the vertebral intervertebral gaps are normal. Bone defects and 'button' dead bone can occur in the skull. In addition, the disease has a self-limiting tendency, which can intermittently appear the pathogenesis, repair, in the occurrence of the phenomenon, has the characteristics of 'this rise and fall'.