椎管内肠源性囊肿课件

1、CASE DISCUSSIONCASE DISCUSSION 2014-12-25 1椎管内肠源性囊肿 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 2椎管内肠源性囊肿 CASE 1 Patient: female, 34 years old With waist pain and the r

2、ight lower extremity radicular pain 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 (-) 3椎管内肠源性囊肿 CT 4椎管内肠源性囊肿 T1WI

3、T2WI STIR 5椎管内肠源性囊肿 CE-T1WI 6椎管内肠源性囊肿 CASE 2 Patient: male, 32years old Neck 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

4、 trauma Examination: passive posture, neck movement was restricted. double 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:

5、 the anal sphincter contraction weakness, anal reflex (-) Laboratory tests (-) 7椎管内肠源性囊肿 CT 8椎管内肠源性囊肿 T1WI T2WI CE-T1 9椎管内肠源性囊肿 T2WI CE-T1WI 10椎管内肠源性囊肿 PLEASE GIVE YOUR OPINION 11椎管内肠源性囊肿 手术记录 case1 12椎管内肠源性囊肿 手术记录 case2 13椎管内肠源性囊肿 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

6、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） 14椎管内肠源性囊肿 Neurenteric cysts MRI of thoracolumbar junction with intradural/ extramedullary neurenteric cyst at T11. (a) Midsagittal MRI demonstrates isointense lesion (arrow) on T1-weighted and (b) hyperintense

7、 signal (arrow) on T2-weighted 15椎管内肠源性囊肿 Cause Its pathogenesis is not clear. It general considered as a congenital disease related with endoderm development disorders. During embryonic development period, part of the endodermal tissues was abnormal posterior displacement and embedded in the neural

8、 tube. Heterotopic rests of epithelium of gastrointestinal tissue lead to eventual formation of compressive cystic lesions of the pediatric and adult spine. 16椎管内肠源性囊肿 Pathological mechanism Only containing endodermal components, similar with the digestive tract epithelium. Form the cyst wall epithe

9、lial cells can be single layer flat , cubic, columnar epithelium, pseudostratified columnar epithelium to stratified squamous epithelium, also can have mucous gland, serous gland, muscle and fat. Sometimes contain cilia and mucus secreting goblet cells. 17椎管内肠源性囊肿 Epidemiology account for 0.7-1.3% o

10、f 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 the epidural or intramedullary. neck or upper thoracic were most involved, lumbar and sacral rare. Often

11、accompanied with congenital spine deformities (like spina bifida) 18椎管内肠源性囊肿 Imaging findings MRI showed a round or oval cyst lesion, with smooth edges, the long axis direction the same as the spinal cord. but also can be irregular in shape. Cyst resulting in significant compression of the spinal co

12、rd, 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 protein constituents or intracystic hemorrhage, T1WI showed high signal or low T2WI signal Spinal cord e

13、mbedded syndrome No enhancement 19椎管内肠源性囊肿 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 clinical manifestations, remission and exacerbation of alternating; (3) the MRI display

14、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 and CT display other congenital malformations 20椎管内肠源性囊肿 Treatment Surgical treatme

15、nt, 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. 21椎管内肠源性囊肿 Differential diagnosis (1)蛛网膜囊肿: arachnoid cyst: (2)皮样和表皮样囊肿: dermoid and

16、 epidermoid cyst (3)囊性神经鞘瘤:cystic schwannoma (4)血管母细胞瘤:hemangioblastoma: (5)脂肪瘤:lipoma 22椎管内肠源性囊肿 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。 23椎管内肠源性囊肿 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, 24椎管内肠源性囊肿 皮样囊肿 F/35y: dermoid cyst 25椎管内肠源性囊肿 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