Volume 94  ·   Issue 19  ·  2014

This issue of Chinese Medical Journal published 6 articles on the diagnosis and treatment of spinal cord tumors, including 3 intramedullary tumors and 3 extramedullary tumors. This reflects that in the field of spinal cord surgery, the diseases diagnosed and treated by neurosurgeons in China are still spinal cord tumors. Comments are given below.

To explore the clinical features and microsurgical treatment of spinal intramedullary cavernomas (SICs).

A total of 21 patients with pathologically confirmed SIC undergoing microsurgical resection of cavernomas at Beijing Tiantan Hospital and Taian Central Hospital from June 2005 to December 2012 were reviewed retrospectively.

The postoperative neurological status improved (n=13) and remained unchanged (n=6) and aggravated (n=2).

The clinical features of SICs are related with their different pathological processes. Magnetic resonance imaging (MRI) is specific for the diagnosis of SICs. Appropriate strategies and refined microsurgical techniques are essential for improving the clinical efficacies of SICs.

To explore the prognostic factors of intramedullary high grade astrocytomas.

Retrospective analyses were conducted for 21 surgical patients with high grade astrocytoma in spinal cord during 2008 to 2012 at our hospital. Their preoperative and postoperative profiles were recorded and evaluated by modified McCormick classification scheme.

Their median age was 32.5 years. There were anaplastic astrocytoma (n=14) and glioblastoma (n=7). The prognoses of high grade astrocytomas were correlated with pathology grade and MIB-1 index. No statistic significance existed in age, gender, McCormick score, extent of resection, radiotherapy or chemotherapy.

Intramedullary high grade astrocytoma has a low incidence, but its outcome is poor. Once definitely diagnosed, operation is recommended as early as possible. Frozen pathology should be performed to determine the extent of resection. After operation, chemotherapy and radiotherapy are also suggested.

To explore the microsurgical skills of multi-segment cervical ependymomas and evaluate preoperative and postoperative neurological functions.

A total of 61 patients with multi-segment cervical ependymoma from January 2008 to July 2013 at our hospital were analyzed to summarize their clinical features, imaging findings, diagnosis, tumor resection and changes of neurological functions.

Total (n=58) and subtotal (n=3) resections were performed. And postoperative nerve functions unchanged (n=43) and aggravated (n=18). There was no mortality.

Proper planning and refined microsurgical techniques are essential in the treatment of multi-segment cervical ependymomas.

To explore the clinical classification and surgical management of cervicothoracic intraspinal lipomas.

A total of 22 patients with cervicothoracic intraspinal lipomas were analyzed retrospectively with regards to clinical manifestations, radiographic features, intraoperative findings, surgical techniques and follow-ups.

Total (n=4), subtotal (n=7) and partial (n=11) resection was performed. Long-term neurological outcomes were evaluated by modified McCormick classification scheme. Their symptoms improved (n=15), unchanged (n=3) and deteriorated (n=4). And cervicothoracic intraspinal lipomas could be classified into extradural, transitional, chaotic and secondary intramedullary groups.

Different groups of cervicothoracic intraspinal lipomas vary in the degree of resection and surgical efficacy. Total resection may be performed on most extradural lipomas. The surgical objective of transitional lipomas is decompression. Chaotic and secondary intramedullary lipomas should target effective resection to avoid neurological function injury. Intraoperative use of laser facilitates tumor resection. Intraoperative electrophysiological monitoring protects spinal cord.

To explore the clinical classification and selection of surgical approaches for cervical spinal dumbbell tumors.

The clinical data of 87 patients with cervical spinal dumbbell tumors undergoing surgical operations from January 2005 to December 2012 at our hospital were analyzed retrospectively. According to the size of inner and outer parts of tumors and the presence or absence of spinal bone damage, the cervical spinal dumbbell tumors were divided into 4 types of intraspinal predominant (Ⅰ, n=48), extraspinal predominant (Ⅱ, n=1), intrapinal and extraspinal without damage of spinal bone (Ⅲ, n=15) and intrapinal and extraspinal type with damage of spinal bone (Ⅳ, n=7). Different surgical approaches were selected on the basis of tumor classification: posterior median-hemilamina approaches for type Ⅰ tumors, lateral-muscle gap approaches for type Ⅱ tumors, ateral-muscle gap-hemilamina or lateral-muscle gap-posterior median-hemilamina approaches for type Ⅲ tumors, posterior far lateral-muscle gap-hemilamina or posterior median-muscle gap-hemilamina approaches plus posterior occipital cervical or cervical spinal bone graft fusion and internal fixation for type Ⅳ tumors.

Among them, 83 cases underwent total resection and another 4 subtotal resection in one-stage operation. The postoperative follow-up period had a range of 9 months to 6 years (mean, 3.2 years). There was no recurrence of tumors for total resection and 1 case of tumor recurrence for subtotal resection. During the follow-up period, the clinical manifestations of 85 patients improved while another 2 deteriorated. And there was no occurrence of spinal deformity.

Clinical classification of cervical spinal dumbbell tumor plays an important guiding role in the selection of surgical approaches. Adopting appropriate surgical approaches based on tumor type can not only improve the rate of total resection of tumor but also reduce the incidence of postoperative spinal deformity.

To observe the effects of intra-operative combined dosing of dezoxine and dexmedetomidine on sedation, analgesia and the incidence of untoward events during and after surgery in teenagers undergoing horizontal concomitant strabismus surgery.

For this prospective and randomized trial, approval was obtained from the Ethical Committee and informed consent from their parents. A total of 60 patients aged 10-16 years undergoing strabismus surgery at Second Affiliated Hospital from September to December 2013 were collected. The treatment group received an intravenous dose of dezoxine 0.1 mg·kg^-1 at 15 minutes before surgery. And another intravenous injection of dexmedetomidine was administered at 0.4 μg·kg ^-1·h^-1 until the end of the first ocular muscle correcting. The control group received the same volume of normal saline. Observational parameters including visual analogue scale (VAS), Ramsay scores, self-rating anxiety scale (SAS), heart rate (HR), blood pressure and the incidence of untoward events such as nausea and vomiting or arrhythmia were recorded.

The VAS pain scores in the treatment group at T2-T5 decreased significantly versus the control group (P<0.05). The Ramsay scores in the treatment group at T2-T3 increased and significantly differences existed with the control group (P<0.05). SAS scores showed significant differences between pre-operative and post-operative periods in the treatment group (P<0.05). The treatment group has shorter operative duration with 5 cases of dragging pain in the treatment group versus 25 in the control group (P<0.05). During dragging reflex, HR was lower in the control group at T2 and T3. There were significant differences with the control group (P<0.05).

A combination of dezoxine and dexmedetomidine provide multiple benefits for patients undergoing strabismus surgery. Compared with simple local anesthesia, it effectively attenuates pain and anxiety and provides mild sedation and better cooperation during strabismus surgery.

To explore the characteristics of cognitive impairment in depressive elders in Shanghai.

A total of 1 068 participants randomly selected from 4 communities in Shanghai underwent neuropsychiatric and psychiatrists clinical assessment. Among them, 102 depressive ones with a score of geriatric depression scale (GDS) >10 and 102 non-depressive ones with a score of GDS≤10 were selected as depression and non-depression groups respectively. The SPSS statistic software V17.0 was used.

Significant differences existed between the depressive and non-depressive patients in the total score of Montreal Cognitive Assessment (MoCA) (t=2.353), trail making B task (t=2.236), attention (t=2.621), sustained attention (t=2.381), calculation (t=2.612) and fixed orientation (t=2.259) (P<0.05). The negative correlation had significant inter-group differences in the total score of MoCA (r=-0.142), attention (r=-0.161), sustained attention (r=-0.160), calculation (r=-0.150), fluency (r= -0.156), delayed recall (r=-0.175) and orientation to place (r=-0.172) (P<0.05). Cognitive impairment in depression group had lower scores than non-depression group in the total score MoCA (14.9±7.3 vs 17.5±6.7), attention (1.1±0.8 vs 1.4±0.8), calculation (1.9±1.2 vs 2.3±1.1) and fixed orientation (1.7±0.7 vs 1.8±0.5) (P<0.05).

In depressive elders, cognitive impairment occurs in multiple cognitive domains of attention, executive function and orientation to place. There is a negative correlation between GDS score and MoCA. The higher GDS score, the worse cognitive function.

To describe the MRI features of Iliotibial band friction syndrome (ITBFS), in order to improve the understanding and diagnosis of ITBFS.

The MR findings of 16 patients (18 knees)of clinically diagnosis ITBFS were reviewed retrospectively.

The MRI features of ITBFS: (1)Ill- defined abnormal signal intensty extended to the lateral femoral epicondyle.(2) Poorly defined abnormal signal intensity presented deep to the ITB adjacent to the lateral femoral epicondyle. (3)Fluid collections medial to the ITB adjacent to thelateral femoral epicondyle.(4)The part of ITB over the lateral femoral epicondyle was thicker.(5)Joint effusion.(6)Other abnormal signs.

MRI is a relatively good method for detection of ITBFS.

To comparative study of CT and MRI appearances in renal cell carcinoma associated with XP11.2 translocation/TFE gene fusion (XP11.2 RCC) and papillary renal cell carcinoma (PRCC).

12 patients with XP11.2 RCC and 18 patients with PRCC were retrospectively studied, and the data was analyzed by AVONA and chi-square text.

12 patients with XP11.2 RCC and 18 patients with PRCC, cystic components (2 vs 11, P<0.05), calcification(0 vs 6,P<0.05), hemorrhage (9 vs 5,P<0.05), homogeneous enhancement(10 vs 7,P<0.05) and had lymph node (3 vs 0) or hepatic metastasis (1vs 0)(P<0.05). On unenhanced CT, the density of XP11.2 RCC was greater than PRCC, normal renal cortex or medulla (P<0.05). Their degree of enhancement were less than normal renal cortex on all enhanced phases (P<0.05). The enhancement degree of XP11.2 RCC was higher than PRCC (on all phases) and renal medulla (on cortical and medullary phase) (P<0.05), but less than normal renal medulla on the delayed phase (P<0.05). The enhancement degree of PRCC was lower than renal medulla on all phases (P<0.05). The XP11.2 RCC was isointense on T₁-weighted imaging, hypointense on T₂-weighted imaging. The PRCC was isointense or hypointense on T₁-weighted imaging, isointense on T₂-weighted imaging.

The CT and MRI could show imagings features of XP11.2 RCC and PRCC, and these features were helpful in predicting a specific subtype of renal cell carcinoma.

To explore the effect of single-dose dexmedetomidine on recovery period after sevoflurane anesthesia with spontaneous respiration in pediatric patients undergoing cleft lip and palate repair.

A total of 60 American Society of Anesthesiologists (ASA) Ⅰ-Ⅱ pediatric patients undergoing cleft lip and palate repair from October to December 2013 were randomly divided into groups D and C (n=30 each). Dexmedetomidine 0.5 μg/kg (group D) or an equal volume of normal saline (group C) was ad ministered intravenously over a period of 10 min at 30 min before the end of surgery. Anesthesia was induced and maintained with sevoflurane under spontaneous ventilation. Heart rate, mean arterial pressure (MAP), hemoglobin oxygen saturation (SpO ₂), respiratory rate, tidal volume (V_T) and pressure of end-tidal carbon dioxide (P_ETCO₂) were recorded at the time before induction (T0), 30 min before the end of surgery (T1), 20 min before the end of surgery (T2), 15 min before the end of surgery (T3), 10 min before the end of surgery (T4), the end of surgery (T5), extubation (T6), 5 min after transferal into post-anesthesia care unit (PACU) (T7), 1 h after surgery (T8), extubation time, length of PACU stay, fentanyl consumption and adverse events were all recorded. The incidence and severity of coughing and emergence agitation were assessed.

Compared to T0, MAP and heart rate at T1 to T5 all decreased in two groups (P<0.05). MAP and heart rate at T6 both increased in two groups and group C was higher than group D (P<0.05). No inter-group differences existed in SpO_2,respiratory rate, V_T or P_ETCO₂. The incidence of coughing and emergence agitation (30% and 13.3%), fentanyl consumption of group D (0.8±2.1 μg) were all significantly lower than that of group C [(66.7% vs 56.7%) and (4.9±6.50) μg, P<0.05]. Length of PACU stay in group D was shorter than that in group C [(15±6) vs (23±19) min,P<0.05]. No inter-group difference existed in extubation time or adverse events.

A single intravenous dose of dexmedetomidine is effective in reducing emergence agitation and coughing, shortening length of PACU stay and improving the quality of recovery period after sevoflurane anesthesia in pediatric patients undergoing cleft lip and palate repair.

To evaluate the clinical outcomes of unibody bifurcated stent-graft in endovascular repair (EVAR).

Retrospective analyses were conducted for the clinical data and postoperative follow-up results of 125 patients(102 cases from Shanghai Changhai Hospital and another 23 from Shanghai Changzheng Hospital)undergoing EVAR with unibody bifurcated stent-graft from January 2008 to January 2013.

The technical success rate was 100%. Perioperative complications including typeⅠendoleak (n=3, 2.4%) and type Ⅱ endoleak (n=4, 3.2%). The incidence of typeⅠendoleak in challenging neck cases was higher than non-challenging ones. And the difference was statistically significant (P=0.001). Except for 1 dead case, the remainder was followed up for a mean of 26.4±1.5 (1-60) months. Neither aneurysm rupture nor stent-graft migration occurred. Late type Ⅰ endoleak occurred (n=2, 1.6%). There were left lower extremity arterial thrombosis (n=1, 0.8%) and surgical reintervention (n=1, 0.8%). Among 3 dead cases, 2 died from acute myocardial infarction and another 1 contrast-induced nephropathy.

Unibody bifurcated stent-graft is both safe and efficacious in the treatment of abdominal aortic aneurysm without the risk of long-term migration. Moreover, it has excellent outcomes for hostile neck or narrow abdominal aortic bifurcation.

To explore the effects of differencial expression of WISP1 upon the radiosenstivity and migration in glioma cell lines M059K and M059J, as well as WISP1 knockdown cell line M059K-WISP1-.

The expression of WISP1 in different cell lines was detected by Western blot assay. The lentivirus mediated-RNA interference technology was employed to knockdown the endogenous expression of gene WISP1 in human glioma cells(M059K). A stable cell line was established by infecting M059K cells with the lentivirus particles, the transfection efficiency was examined by fluorescence microscopy. The radiosensitivity and migration ability of cells were assessed by Colony-forming and Scratch Wound Assays respectively.

WISP1 was highly expressed in M059K cells compared with M059J cells. M059K cells were less radiosensitive than M059J cells, and had a better ability of migration. M059K cells were transfected into by the lentiviral vector pGLV3/H1/GFP+ puro vector -WISP1. And M059K-WISP1- cell line with stable WISP1 downregulation was established successfully. Western blot demonstrated significantly downregulated WISP1 expression in M059K-WISP1- cells, which showed obviously improved radiosensitivity in Colony-forming assay and suppressed migration ability in Scratch Wound Assay. Colony-forming assay based on the muti-target/single-hit model indicated that radiosensitivity of M059K-WISP1- was significantly decreased.

Compared with WISP1 low expression cell line M059J, WISP1 high expression cell line M059K was less radiosensitive and had a better ability of migration. We have successfully established a glioma cell line M059K-WISP1- stably downregulating WISP1, which shows a better radiosensitivity and a lowered migration activity in vitro. This may provide a novel therapeutic target for the treatment of human glioma.

To evaluate the effects of 4-aminopyridine-3-methyl hydroxide (4-AP-3-MeOH) in rat's acute spinal cord injury.

A total of 12 adult male SD rats (250-300 g) were randomly divided into treatment (n=6) and control (n=6) groups. After compressing segment T11 of spinal cord for 30 min, the injured segment received 1 ml 4-AP-3-MeOH (100 μmol/ml) by topically application in treatment group while the control group received 1 ml saline. Somatosensory evoked potential (SSEP) was detected in both groups at pre-injury, 30 min post-injury and post-dosing. Then Luxol fast blue (LFB) staining of target spinal segment was performed.

In treatment group, the values of SSEP at pre-injury, 30 min post-injury and post-dosing were 1.26±0.35, 0.03±0.05 and 0.45±0.19 μv respectively. Comparing SSEP of 30 min post-injury with post-dosing, the difference was statistically significant ( P<0.01). While in control group, the values of SSEP at pre-injury, 30 min post-injury and post-dosing were 1.05±0.39, 0.01±0.02 and 0.02±0.02 μv respectively. Comparing SSEP of 30 min post-injury with post-dosing, there was no statistical difference (P>0.05). After 30 min injury, there were swelling and bleeding of spinal cord. LFB staining showed that both gray and white matter had swelling and bleeding and central canal was destroyed with varying degrees of demyelination.

After 30 min of acute spinal cord injury, there are bleeding of gray and white matter with varying degrees of demyelination. Topical usage of K⁺ blocker 4-AP-3-MeOH can effectively improve the conduction of SSEP after acute spinal cord injury in rats.

To evaluate the biocompatibility of SLS-produced titanium alloy scaffold in vitro and investigate the therapeutic effects in repairing segmental bone defects.

Porous titanium alloy scaffolds were produced by SLS and their surfaces were either left untreated or acid etched. In vitro, mouse pre-osteoblasts (MC3T3-E1 cells) were cultured on these 2 group scaffolds, and then cell proliferation and differentiation were examined after cell seeding. In vivo, bone defects were artificially made in 15 New Zealand rabbits and the porous titanium specimens were implanted into the radius of rabbits for 3 months. The regulating checks of X-ray were determined. The osteointegration of the implants was investigated by Micro-CT and histological examination at 12 weeks after surgery.

A gradual increase in cell-specific ALP synthesis by cells cultured in both groups was observed with longer culture time (14 d). ALP activity did not differ significantly between two groups(0.834±0.092 vs 0.815±0.081, P>0.05). Both Micro-CT and the histological analysis indicated that the titanium alloy scaffolds had excellent ability to facilitate the osteointegration in vivo. The results were significantly different between the empty control and the 2 different surface modifications of SLS-implants (25.4%±4.2% vs 23.6%±8.4% vs 12.3%±4.7%,P<0.05). Between the groups with implants, the number of bone points was not significantly different, irrespective of the surface treatment (25.4%±4.2% vs 23.6%±8.4%,P>0.05).

Selective Laser Sintering-Produced porous titanium alloy scaffold possessed admirable biocompatibility in vitro. It also could be contributed to the healing of long tulular bone defect. The porous Ti6Al4V implant not only reduced the stress-shielding but also exerted appropriate osteoconductive properties.

To evaluate the efficacies of transgenetic bone mesenchymal stem cells (BMSCs) carring basic fibroblast growth factor (bFGF) gene on repairing severe ligament injury.

The genes of bFGF and enhanced green fluorescent protein (eGFP) were transfected into BMSCs through adenovirus vector. According to different cell transplantations, the operated rabbits were divided into 3 groups of transgenetic BMSCs, BMSCs and no cells. And group 4 was normal ligament group. The distribution of transgenetic BMSCs in ligament injury area, the expression of bFGF and ligament specific extracellular matrix, i. e. type Ⅰ collagen, type Ⅲ collagen, fibronectin, alpha-smooth muscle actin and vimentin, histology and biomechanics of ligament healing were recorded at different timepoints.

The transgenetic BMSCs could be observed in injury area within one month. The expression of bFGF in the transgenetic BMSCs group was significantly higher than that in the control groups at Days 3-14 (P<0.001) and peaked at Day 7. In the transgenetic BMSCs group, the gene expression of ligament specific extracellular matrix was significantly higher than that in the control groups within one month (P<0.001). And immunohistochemistry showed strong positive expression. After 3 months, in group 1, hematoxylin and eosin staining showed the arrangement and density of collagen fibers of healing area were better than that in the control groups. And biomechanical testing showed that tensile load were stronger than that in the control groups (P<0.05).

Transgenetic BMSCs carrying bFGF gene can obviously promote ligament injury healing and increase ligament healing strength.

To explore the effects of exercise training on motor functional recovery and pyramidal tract regeneration in hypertensive rats with focal cerebral infarction.

Middle cerebral artery occlusion (MCAO) model was generated by electric coagulation on stroke-prone renovascular hypertensive Sprague-Dawley rats. The rats were randomly allocated to three groups of sham (n=15), exercise training (n=10) and control (n=12). The exercise training group had running wheel exercise. The neurological function was evaluated with the modified neurological severity scores(mNSS)and forelimb grip strength test at Days 3, 7, 14, 21, 28, 35 and 42 post-ischemia. The pathological changes of neurons around infarct cortex were measured by Nissl staining. At Day 42 post-ischemia, anterograde tract tracers of biotinylated dextran amine (BDA) and cascade blue-labeled dextran amine (CB) were injected into cortex. And axonal extension of ipsi- and contra-lesional pyramidal tract was observed at Day 14 post-injection.

The mNSS score declined in the exercise training group(4.0±1.1, 2.7±0.7, 2.6±0.5)versus those in the control group(6.0±1.3, 5.6±1.0, 5.6±1.1)at Days 28, 21 and 35 post-ischemia (all P<0.01). The grip strength of paralytic forelimb increased in the exercise training group((379±41, 344±15, 430±48, 471±47, 454±17)g)versus those in the control group((276±8, 170±5, 236±12, 283±14, 317±15)g)at Days 14, 21, 28, 35 and 42 post-infarction (allP<0.05). Compared with the control group(0.571±0.060), exercise training (0.734±0.035) reduced neuron loss (P<0.05). Moreover, the percentage of the number of BDA-positive, midline-crossing fibers over the number of labeled fibers in cerebral peduncle ipsilateral to injection site increased in the exercise training group (0.096 8±0.022 6) versus those in the control group (0.014 2±0.003 9) at the level of cervical enlargement (P<0.016 7). The percentage of CB immunofluorescence in striatum ipsilateral to lesion-sided motor cortex over those in ischemic cortex was lower in the control group(0.521±0.020)and the exercise training group(0.499±0.034)than that in the sham group(0.824±0.017)(allP<0.01). However, no significant difference existed between control and exercise training groups (P>0.05).

Exercise training promotes the recovery of motor function and contralesional pyramidal tract regeneration after cerebral infarction.

To explore the effects of autotransplantation of NT-3 gene modified olfactory ensheathing cell (OEC) and neural stem cell (NSC) complex adhering to collagen protein-heparin sulfate scaffold on motor function of rats with cerebral hemorrhage.

The cerebral hemorrhage model was established with caudate nucleus bleeding in Wistar rats. The animals were randomly divided into 4 groups: A (transplantation of NT-3 modified OEC-NSC complex adhering to scaffold), B (transplantation of NT-3 modified OEC-NSC complex), C (transplantation of scaffold) and D (blank control group). The motor function of hind limbs was assessed at Day 1, 3, 7, 14 and 30 post-transplantation respectively. The survival, distribution and differentiation of transplanted cells were tested by immunohistochemistry and fluorescent staining.

The neurological functional score of group A(2.12±0.12, 1.50±0.11, 0.52±0.08)or B(2.10±0.16, 1.79±0.09, 0.91±0.10)was obviously inferior to group C/D at Days 7, 14 and 30. No significant difference existed between groups C and D(P>0.05). The scores of A were markedly lower than those of B at Days 14 and 30(P<0.05,P<0.01). The numbers of surviving NSCs and cells migrating to focal area after transplantation was much more in group A than those in other groups.

Autotransplantation of NT-3 gene modified OEC-NSC complex adhering to collagen protein-heparin sulfate scaffold may markedly ameliorate the motor function of cerebral hemorrhagic in rats. And the transplanted NSCs have better capacities of survival, migration and differentiation.

With the development of medical science, the treatment of craniocerebral trauma in the acute phase has made great progress, and the mortality rate in the acute phase has decreased significantly. The subsequent problem is that the number of patients in the "vegetative state" is gradually increasing, and the disability rate of craniocerebral trauma has not decreased due to the advancement of treatment methods. How to improve the various neurological functions of persistent vegetative state (PVS) is still a worldwide treatment problem. Studies have shown that high cervical spinal cord electrical stimulation can promote wakefulness in patients with long-term coma. A case of coma after severe traumatic brain injury was recovered after high cervical spinal cord epidural stimulation (cSCS). cSCS have been used as a treatment to manage chronic pain and reduce the onset of hemiplegic spasticity.

With the development of functional imaging technology and the emergence of positron emission tomography (PET), it is of great significance for the differentiation of benign and malignant lymph node lesions, but it is limited in clinical application due to its shortcomings such as high price, large radiation and long time consumption. Magnetic resonance diffusion weighted imaging (DWI), as a completely non-invasive and radiation-free functional imaging technology, has achieved good clinical results in the application of tumor and lymph node lesions in recent years. The value of DWI and PET/CT in differentiating benign and malignant lymph nodes is reviewed.

The child was a 14-year-old girl. He came to the clinic because he found that his vision gradually declined for 2 years. The patient's parents are not intimate married, and there is no ethnic minority origin in the family. Physical examination: wide and flat nose bridge, maxillary dysplasia, tooth dysplasia, missing 2 teeth, and periumbilical neoplasia. Ocular examination: visual acuity: right eye visual acuity 0.15, correction 0.5, left eye visual acuity 0.25, correction 0.5, intraocular pressure 20 mmHg (1 mmHg =0.133 kPa) in right eye, 21 mmHg in left eye, no conjunctiva congestion in both eyes, transparent cornea, kp (–), shallow peripheral anterior chamber, peripheral anterior iris adhesion. The texture of the iris is loose, the iris matrix becomes thin and atrophic, holes form, the pupil is ectopic downward, the shape is irregular, and the pigment collar is everted (Figure 1，Figure 2), a small amount of pigmentation in the anterior capsule of the crystal, no obvious opacity in the vitreous body, fundus examination: the optic disc boundary is clear, orange-red, C/D is about 0.2, and the fovea reflectivity is clear. No obvious abnormalities were found in binocular visual field examination; Corneal endothelioscopy: corneal endothelial cell count right eye 2 141/mm²2 044/mm in left eye²There was no obvious abnormality in endothelial morphology. The ultrasound biomicroscopy of both eyes showed that the anterior chamber of both eyes was shallow, and the depth of the central anterior chamber was 1.90 mm in the right eye and 1.53 mm in the left eye. The iris root in all directions adhered to the corneal endothelial surface forward, the chamber angle was closed, the pupil shape was irregular and deviated, the lens position was acceptable, the suspensory ligament was visible, and the anterior vitreous body was mildly opaque (Figure 3，Figure 4)。 After asking for the medical history, her mother had undergone binocular anti-glaucoma surgery in an external hospital 2 years ago. Based on the clinical manifestations and eye examination, the diagnosis was "Axenfeld-Rieger syndrome". Eyewear was given to correct vision, and regular follow-up visits were instructed to observe the changes of intraocular pressure.

A 61-year-old male, Han nationality, developed lower limb weakness with pigmentation and slow walking 5 years ago. Before 5 days before admission, he had sudden pain and weakness in his right lower limb, and was hospitalized in the Department of Vascular Surgery of Qiqihar No.1 Hospital with "lower limb arteriosclerosis obliterans". He denied the history of hypertension and coronary heart disease, cerebral thrombosis and cerebral hemorrhage, trauma, surgery and allergy. There was no history of exposure to the epidemic area, no history of smoking and alcoholism. A family genetic history is denied. Physical examination: General vital indications are stable, pigmentation is visible in the right lower limb, skin of the lower limb is hypothermic, femoral artery pulsation is palpable, and dorsal pedis artery pulsation disappears. The contralateral foot artery is palpable. There was no deformity in both lower limbs, muscle strength and muscle tone were normal, pain and temperature perception in both lower limbs decreased slightly, and no obvious positive signs were seen in the remaining nervous system. CT angiography of lower extremities showed no obvious occlusion of lower extremity arteries. Lumbar MRI was performed: intraspinal cyst, spinal cord compression (Figure 1)。 The patient was referred to neurosurgery for further treatment. Thoracic 12 lumbar 1 laminectomy of spinal conus enterogenic cyst was performed under general anesthesia. Postoperative pathological diagnosis: T12~ L1 spinal cord cavity occupying space, monolayer and stratified cubic epithelial cysts, consistent with spinal enterogenic cysts (Figure 2)。