椎管内肠源性囊肿

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1、CASE DISCUSSION,2014-12-25,Vocabulary,hypoalgesia hapldi:s 痛觉减退 Hypoesthesia hapsi:z 感觉迟钝 Myodynamia madanmj 肌力 anal sphincter sfkt 肛门括约肌 congenital dysplasia displeizi 先天性发育不良 spina bifida span bfd 脊柱裂,CASE 1,Patient: female, 34 years old With waist pain and the right lower extremity radicular pain

2、 for 6 months. There was no fever, no headache, no surgery, no history of trauma Examination: hypoesthesia at the front of the thigh, Bilateral Lower limbs myodynamia IV, muscle tension(-),Bilateral Babinski sign(-) Laboratory tests (-),CT,T1WI T2WI STIR,CE-T1WI,CASE 2,Patient: male, 32years old Nec

3、k and shoulder and bilateral upper limbs pain with limbs numbness and weakness for more than 6 years, The symptoms were aggravating with limited mobility for 10 days. There was no fever, no headache, no surgery, no history of trauma Examination: passive posture, neck movement was restricted. double

4、upper limbs apparent hypoalgesia, bilateral upper limbs myodynamia II; the left lower limb myodynamia I, the right lower limb myodynamia II. muscle tension(-). Bilateral Hoffmann sign (+), Babinski syndrome (+), Rectal touch: the anal sphincter contraction weakness, anal reflex (-) Laboratory tests

5、(-),CT,T1WI T2WI CE-T1,T2WI CE-T1WI,PLEASE GIVE YOUR OPINION,手术记录 case1,手术记录 case2,Intraspinal neurenteric cysts,神经肠囊肿上皮细胞的显微照片。 (a)囊肿壁:显示单层柱状和立方上皮细胞贴附由型胶原组成的基底层(H和E,200) (b)柱状上皮、立方上皮和分泌粘液的杯状细胞,高倍镜(H和E,1000) (C)假复层纤毛柱状上皮的肠源性囊肿壁高倍镜(H和E,1000),Neurenteric cysts,MRI of thoracolumbar junction with intrad

6、ural/ extramedullary neurenteric cyst at T11. (a) Midsagittal MRI demonstrates isointense lesion (arrow) on T1-weighted and (b) hyperintense signal (arrow) on T2-weighted,Cause,Its pathogenesis is not clear. It general considered as a congenital disease related with endoderm development disorders. D

7、uring embryonic development period, part of the endodermal tissues was abnormal posterior displacement and embedded in the neural tube. Heterotopic rests of epithelium of gastrointestinal tissue lead to eventual formation of compressive cystic lesions of the pediatric and adult spine.,Pathological m

8、echanism,Only containing endodermal components, similar with the digestive tract epithelium. Form the cyst wall epithelial cells can be single layer flat , cubic, columnar epithelium, pseudostratified columnar epithelium to stratified squamous epithelium, also can have mucous gland, serous gland, mu

9、scle and fat. Sometimes contain cilia and mucus secreting goblet cells.,Epidemiology,account for 0.7-1.3% of spinal axis tumors. Mostly occurs in children and adolescents 11days-40years Male predilection: proportion of men to women as (2-3):1 most occured in the subdural extramedullary 90%, a few in

10、 the epidural or intramedullary. neck or upper thoracic were most involved, lumbar and sacral rare. Often accompanied with congenital spine deformities (like spina bifida),Imaging findings,MRI showed a round or oval cyst lesion, with smooth edges, the long axis direction the same as the spinal cord.

11、 but also can be irregular in shape. Cyst resulting in significant compression of the spinal cord, cyst wall is thin and uniform. T1WI shows mainly low /equal /slightly higher than CSF T2WI shows high/iso signal to CSF, usually homogenious Signal strength depends on the cyst contents, more proteinco

12、nstituents or intracystic hemorrhage, T1WI showed high signalor low T2WI signal “Spinal cord embedded syndrome“ No enhancement,Diagnosis,The following characteristics should be highly suspected:(1) in male adolescents;(2) partial nerve root pain or spinal cord compression symptoms as the main clinic

13、al manifestations, remission and exacerbation of alternating;(3) the MRI display cystic lesions in the cervical or thoracic intraspinal subdural extramedullary, cyst signal similar with CSF, with thin cyst wall, no enhancement effect; with“Spinal cord embedded syndrome“(4) X-ray plain radiography an

14、d CT display other congenital malformations,Treatment,Surgical treatment, particularly total resection with rare recurrence partial resections recurrence rate as high as 37%,Jesse J. Savage etal. Neurenteric cysts of the spine. J Craniovertebr Junction Spine. 2010 Jan-Jun; 1(1): 5863.,Differential d

15、iagnosis,(1)蛛网膜囊肿: arachnoid cyst: (2)皮样和表皮样囊肿: dermoid and epidermoid cyst (3)囊性神经鞘瘤:cystic schwannoma (4)血管母细胞瘤:hemangioblastoma: (5)脂肪瘤:lipoma,蛛网膜囊肿,蛛网膜囊肿:青年人多见,一般位于脊髓背侧髓外硬膜下或硬膜外,信号强度在各序列上均与脑脊液一致,无强化。一般不合并其他先天性畸形。,表皮样囊肿,M/35years: epidermoid cyst 多位于腰骶部髓外硬膜下,信号多不均匀,大多数无强化,皮样囊肿,F/35y: dermoid cyst,囊性神经鞘瘤,囊性神经鞘瘤:一般沿神经根走行分布,典型的肿瘤呈哑铃状,通过扩大的椎间孔向硬膜外生长。病变信号不均,增强扫描囊壁及实性部分明显强化。,血管母细胞瘤,血管母细胞瘤:囊肿型血管母细胞瘤表现为髓内“囊肿壁结节”,壁结节明显强化,继发性脊髓空洞症及异常流空血管信号常见,脂肪瘤,脂肪瘤:可发生于椎管内任何节段,T1WI和T2WI病灶均呈高信号,应用脂肪抑制序列后,病灶内的高信号明显被抑制。而蛋白含量高的肠源性囊肿信号强度不受脂肪抑制序列影响。,谢谢,

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