2012年 · 第125卷第02期

Abstract：Background The prognosis for patients with gastric cancer and synchronous liver metastases is very poor.However,a standard therapeutic strategy has not been well established.The clinical benefit and prognostic factors after hepatic surgical treatment for liver metastases from gastric cancer remain controversial.Methods Records of 105 patients who underwent gastrectomy regardless of hepatic surgical treatment for gastric cancer with synchronous liver-only metastases in our center between 1995 and 2010 were retrospectively reviewed.Results The overall survival rate for the 105 patients was 42.1％,17.2％,and 10.6％ at 1,2,and 3 years,respectively,with a median survival time of 11 months.Multivariate survival analysis revealed that the extent of lymphadenectomy (D) (P ＜0.001),lymph node metastases (P ＜0.001),extent of liver metastases (H) (P=0.008),and lymphovascular invasion (P=-0.002) were significant independent prognostic factors for survival.Among patients who underwent D2 lymphadenectomy,those who underwent hepatic surgical treatment had a significantly improved survival compared with those who underwent gastrectomy alone (median survival,24 vs.12 months; P ＜0.001).However,hepatic surgical treatment was not a prognostic factor for patients who underwent D1 lymphadenectomy (median survival,8 vs.8 months;P=0.495).For the 35 patients who underwent gastrectomy plus hepatic surgical treatment,D2 lymphadenectomy (P ＜0.001),lymph node metastases (P=-0.015),and extent of liver metastases (H1 vs.H2 and H3) (P=-0.017) were independent significant prognostic factors for survival.Conclusions D2 lymphadenectomy plus hepatic surgical treatment may provide hope for long-term survival of judiciously selected patients with hepatic metastases from gastric cancer.Patients with a low degree of lymph node metastases and H1 liver metastases would make the most appropriate candidates.However,if D2 dissection cannot be achieved,hepatic surgical treatment is not recommended。

Abstract：Background The continual and rapid development of techniques which are used for diagnosis and treatment makes management of colorectal cancer more difficult depending on single discipline.Colorectal cancer multidisciplinary team (MDT) working model is recommended by UK and other countries,but there is little information on the impact of MDT working on management of colorectal cancer in China.The aim of this study was to assess the effect on management of colorectal cancer after the inception of an MDT.Methods A total of 595 consecutive colorectal cancer patients were referred to the Department of Gastroenterological Surgery,the pre-MDT cohort include 297 patients,recruited from January 1999 to November 2002,and the MDT cohort had 298 patients enrolled from December 2002 to September 2006.Information recorded included:TNM stage from histological reports,degree of differentiation,the number of examined lymph nodes and CT TNM staging performed or not,and its accuracy,including local and distant recurrence.Results The number of examined lymph nodes and the accuracy of TNM staging by CT in the MDT group were significantly more than those in pre-MDT group.CT TNM staging was more accurate in the MDT group compared to the pre-MDT group (P=-0.044).The rate of tumor recurrence in the MDT group was lower than pre-MDT group (log-rank test,P ＜0.001).Multivariate analysis revealed that age (P=0.001),management after inception of the MDT (P=0.002),degree of differentiation (P=0.003),number of examined lymph nodes (P=0.002),and TNM stage (P=0.000) were important factors that independently influence overall survival.Conclusions The inception of MDT working improved the diagnostic accuracy and overall survival of colorectal cancer patients.MDT working promoted communication and cooperation between disciplines and ensured high-quality diagnosis,evidence-based decision making,and optimal treatment planning。

Abstract：Background Polyunsaturated omega-3 fatty acids may beneficially influence healing processes and patient outcomes.The aim of this research was to study the clinical efficacy of fish oil enriched total parenteral nutrition in elderly patients after colorectal cancer surgery.Methods Fifty-seven elderly patients with colorectal cancer were enrolled in this prospective,randomized,double-blind,controlled clinical trial.All patients received isocaloric and isonitrogenous total parenteral nutrition by continuous infusion (20-24 hours per day) for seven days after surgery.The control group (n=28) received 1.2 g/kg soybean oil per day,whereas the treatment group (n=29) received 0.2 g/kg fish oil and 1.0 g/kg soybean oil per day.Blood samples were taken pre-operatively,and at days one and eight after the operation.The plasma levels of CD4,CD8,CD4/CD8,interleukin 6 (IL-6) and tumor necrosis factor a (TNF-a) were measured.Clinical outcomes were then analysed.Results Patient characteristics were comparable between the two groups.At day eight post-surgery,IL-6,TNF-α and CD8 titres were lower in the treatment group when compared to the control group; these results reached statistical significance.In the treatment group,there were fewer infectious complications and incidences of systemic inflammatory response syndrome (SIRS),and shorter lengths of hospital stay were observed.The total cost of medical care was comparable for the two groups.No serious adverse events occurred in either group.Conclusions Fish oil 0.2 g/kg per day administrated to elderly patients after colorectal surgery was safe and may shorten the length of hospital stay and improve clinical outcomes。

Abstract：Background It is a challenge for the surgeons to accurately diagnose the pancreatic masses preoperatively,which decides the choice of surgical managements and subsequently results in different survivor outcomes,operative complications,and mortality rates.The purposes of this study were to evaluate the diagnostic role that intra-operative puncture biopsy may play in pancreatic masses and to explore the relevant factors influencing the diagnosis.Methods A retrospective study was performed on 94 in-patients admitted to Peking University First Hospital for pancreatic masses during the period from June 1994 to December 2007.They all underwent intra-operative puncture biopsy during exploratory laparotomy.The sensitivity and specificity of intra-operative puncture biopsy were calculated and the relevant factors to the diagnosis of biopsy were selected for the statistical analysis.Results The overall sensitivity,specificity,positive predictive value,and negative predictive value of intra-operative puncture biopsy were 76.0％,94.7％,98.3％ and 50.0％,respectively.The analysis of bivariate correlations showed that the size of the pancreatic masses (P=-0.000),the number of puncture biopsies (P=0.000),and the presence of pancreatic fibrosis (P=-0.012) had statistic significance for the diagnosis.But the multivariate analysis identified the size of the pancreatic masses (P=0.004) and the number of puncture biopsies (P=0.000) as independent predictive factors for intra-operative puncture biopsy.In addition,as the number of puncture biopsies increased,the sensitivity and specificity of diagnosis was improved (P=0.000).The sensitivity and specificity of intra-operative puncture biopsy were found to be lower for the pancreatic masses less than 25 mm compared with the masses larger than 25 mm (P=0.000).It was noted,however,that even if the masses were less than 25 mm,the sensitivity and specificity could be improved significantly as the number of puncture biopsies reached 3 to 6 (P=0.007).Conclusions Intra-operative puncture biopsy is simple and accurate for qualitatively differentiating various types of pancreatic masses.Three to 4 biopsies could significantly improve the diagnostic effect for pancreatic masses,even if the masses are less than 25 mm in size。

Abstract：Background The recent onset or deterioration of lower extremity ischemia is highly associated with intravascular thrombus.Treatment of these thrombotic occlusions is challenging.Pulse-spray catheter directed thrombolysis (PS-CDT) refers to the technique of intermittent forcefully injecting the thrombolytic agent into the thrombus to fragment it and increase the surface area available for enzymatic action.This study was designed to evaluate the efficacy and safety of PS-CDT in patients with recent onset or deterioration of lower extremity ischemia.Methods From August 2008 to March 2009,44 patients with acute or chronic lower extremity ischemia were recruited in this prospective study,which included 37 men and 7 women ranging from 15 to 83 years old (mean age (51.1±17.4) years).PS-CDT through a multi-side-hole thrombolytic catheter by using urokinase was conducted in all patients.The progression of thrombolysis was assessed and graded by angiography.Adjunctive therapies were used to correct underlying lesions.The follow-up period was 12 months.Results In the 44 patients,the average total dose of urokinase for each patient was (2120000±1100000) IU (median 2000000 IU),with a median duration of lysis of 48 hours.The rate of initial technical success was 97.7％.The rate of clinically successful lysis was 81.8％.Early (＜30 days) and late (from 30 days to 12 months) amputation rates were both 4.5％ (2/44).The overall amputation rate was 9.1％ (4/44).No mortality was recorded during thrombolysis and follow-up period (12 months).No major bleeding or allergic reaction was seen during thrombolytic therapy.11.4％ had symptoms of distal embolization.The primary patency rate for the arteries that were clinically successfully thrombolyzed as compared with those that failed to lysis was 83.3％ vs.57.1％,respectively,at 1 year.Conclusions PS-CDT,combined with adjunctive therapies,is associated with good safety and efficacy in recent-onset or deterioration of lower extremity ischemia.Successful thrombolysis may be accompanied by better outcomes。

Abstract：Background This retrospective study was undertaken to analyze the outcome of hepatic resection in fifty-two patients with unresectable hepatocellular carcinoma (HCC) between January 2004 and December 2008.Methods Among these fifty-two patients,the mean diameter of the tumor was 7.9 cm (4.4-15.5 cm,median 8.5 cm) prior to the first transcatheter arterial chemoembolization (TACE).After 1-6 times of TACE (median 2),the median tumor diameter was reduced to 4.2 cm (0-8.4 cm) prior to resection.The duration between the last TACE treatment and sequential resection varied from one to six months (median 2.7 months).Serum α-fetoprotein (AFP) levels were abnormal in thirty-eight out of the fifty-two patients.In AFP producing HCCs,AFP levels returned to normal (≤400 μg/L) in twenty-five out of thirty-eight patients.Hepatic segmentectomy,multiple hepatic segmentectomy or partial hepatic resection were performed in forty-five patients,two underwent extended left hemihepatectomy,and one underwent right posterior branch portal vein thrombectomy.One patient received a right hemihepatectomy and three had left hemihepatectomies.Results Complete tumor radiological response (CR) occurred in five patients (9.6％).There were three cases of perioperative mortality in the fifty-two patients (5.8％).One patient underwent salvaged orthotopic liver transplantation,and twenty-one patients observed tumor recurrence within two years.The 1-,3- and 5-year survival rates of the fifty-two patients were 77.0％ (n=40),55.0％ (n=29),and 52.0％ (n=28),respectively.The median survival time after surgery was 49 months (95％ confidence interval 7.5-52.7 months).Conclusions TACE treatment provides a better chance for HCC resection in patients initially diagnosed with unresectable HCC.Furthermore,liver resection should be performed once the tumor is downstaged to be compatible for successful resection

Abstract：Background In the past several decades we have seen multiple advances in the reconstruction for girls born with vaginal agenesis.This study aimed to evaluate the technical feasibility,anatomical and functional outcomes of one-stage laparoscopic and gasless laparoscopic vaginoplasty with sigmoid colon for the patients of vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome).Methods We did a retrospective review of a total of 150 women with Mayer-Rokitansky-Kuster-Hauser syndrome treated at Beijing Anzhen Hospital,Capital Medical University from March 2006 to August 2010.The patients were divided into the CO2 pneumoperitoneum laparoscopic group and the abdominal wall lift of gasless laparoscopic group.Sigmoid colon vaginoplasty approaches were performed in all of the patients.The surgical techniques,perioperative results,complications,anatomical and functional outcomes of vaginoplasty were recorded.Results All procedures were performed successfully.Significant differences in the operative time and intraoperative blood loss existed in the laparoscopic vaginoplasty group compared with the gasless laparoscopic vaginoplasty group.The patients who underwent sigmoid colon vaginoplasty had good cosmetic results without the problem of excessive mucus production.The postoperative complications were minimal.During a mean follow-up of 15.6 months,no stenosis or shrinkage was encountered.The subjective sexual satisfaction rate with the surgical outcomes in all patients was 83.3％.Conclusions Laparoscopic or gasless laparoscopic vaginoplasty with sigmoid colon are effective and feasible approaches for women with congenital vaginal agenesis.The procedures have satisfactory anatomical and functional results。

Abstract：Background Endometriosis is a controversial and enigmatic disease.Deep infiltrating endometriosis (DIE) is responsible for painful symptoms and is the least understood type of endometriosis.Little work has been devoted to define the location of DIE lesions and its relationships with pain.The aim of the study was to investigate the relationship between the anatomical distribution of DIE lesions and pain symptoms.Methods Clinical data from 354 patients between May 2003 and December 2007 with laparoscopically diagnosed endometriosis were collected including 177 DIE patients and 177 non-DIE patients.The pain symptoms,including dysmenorrhea (DM),chronic pelvic pain (CPP,defined as intermittent or permanent pelvic pain,not related to the menstruation and longer than 6 months),deep dyspareunia (pelvic pain at intercourse) and dyschezia (pelvic pain with defecation),were recorded for every patient before operation.Endometriotic lesions were recorded by their anatomical distributions,the depth of infiltration and lesion colors.And the relationship between the anatomical distribution of DIE lesions and pain symptoms was analyzed.Pearson's chi-square test or Fisher's exact test,one-way analysis of variance (ANOVA) and linear regression and binary Logistic regression were used for statistical analysis.Results The duration ((13.79±3.94) years) of pain suffering in DIE patients was much longer than that of non-DIE patients (P ＜0.01).In DIE patients,60.7％ of the uterosacral ligament (USL) nodules were bilateral (P ＜0.01); 44.6％ of the cul-de-sacs were completely blocked.Rectum invasion was observed in 19.9％ of DIE patients (P=0.03); pelvic adhesion was also more common.Up to 98.41％ of the deep infiltrative lesions were located in the posterior pelvic compartment.DIE lesions were also found in bladder (1.58％),USL (67.08％),cul-de-sac (12.02％),recto-vaginal septum (12.66％),rectum and rectosigmoid junction (2.85％) and ureter (3.80％).The odds ratio of USL-DIE for CPP,deep dyspareunia,dyschezia were 2.52,1.29 and 2.24 respectively.And the depth of infiltration correlated with the severity of dysmenorrhea.Conclusions DIE lesions were associated with severe pain symptoms.The main distribution of DIE lesions was in the posterior pelvic compartment,and was more widespread and severe in DIE patients.Moreover,resection of these DIE lesions are very important to treat the pain symptoms。

Abstract：Background Frequent premature ventricular complexes from the right ventricular outflow tract (RVOT-PVCs) are associated with left ventricular dysfunction.This study adopted two-dimensional speckle tracking imaging to evaluate global and regional left ventricular myocardial function in patients with frequent RVOT-PVCs.Methods This study included 30 patients with frequent RVOT-PVCs and 30 healthy subjects.Aortic systolic velocity-time integral (AoVTI) and myocardium strain in circumferential (CS),radial (RS) and longitudinal (LS) directions were evaluated by conventional echocardiography and speckle tracking imaging.All values of patients with RVOT-PVCs were recorded during sinus (PVC-S) and PVC beats (PVC-V).Results Significant differences were demonstrated in global CS,RS and LS between the control subjects and the PVC-V (CS:(17.46±2.48)％ vs.(11.52±3.28)％,RS:(48.26±10.20)％ vs.(20.92±9.78)％,LS:(19.89±2.62)％ vs.(11.79±3.66)％,P ＜0.01),and in segmental RS and LS of nearly all the left ventricular segments.Statistical differences in segmental CS between the PVC-V and the control subjects were only observed in anterior,anteroseptal and septal segments (only seen in anteroseptal and septal segments at apex).Furthermore,V/S AoVTI (AoVTI during the PVC beat divided by AoVTI during the sinus beat,then multiplied by 100％) correlated with coupling interval (r=0.67,P ＜0.001) and global strain (CS:r=0.48,P=0.007; RS:r=0.65,P ＜0.001; LS:r=0.65,P ＜0.001).Conclusions Frequent RVOT-PVCs can induce global and regional left ventricular systolic dysfunction.The reduction of hemodynamic parameters relates to the coupling interval and the global systolic function。

Abstract：Background The radial approach has been increasingly used as an alternative to femoral access.And more procedures using repeated transradial coronary intervention (r-TRI) are performed.However,few data about r-TRI has been obtained.Therefore,we investigated the safety and feasibility of r-TRI using the same route.Methods A total of 423 consecutive eligible patients undergoing r-TRI were enrolled in the r-TRI group,and 846 patients with initial TRI (i-TRI) were assigned to the i-TRI group in a 2:1 matching ratio compared to r-TRI group.The primary endpoint included the success rate of the procedure and the incidence of vascular related complications.Results The baseline clinical characteristics in the two groups were comparable.The success rate of procedures in the r-TRI and i-TRI was similar (96.0％ vs.97.5％,P=0.130).In subgroup analysis (coronary angiography only or angiography with pecutaneous coronary intervention),similar results were also observed.The puncture numbers and incidence of radial artery spasm in the r-TRI group were significantly higher than in the i-TRI group (P=-0.024 and P ＜0.001,respectively).The other procedural outcomes in the two groups were identical.With respect to the incidence of overall vascular related complication and independent events,there were no significant differences in spite of a higher incidence of radial artery occlusion (RAO)in the r-TRI group (RAO:1.2％ vs.0.7％,P=-0.521).The patients in the i-TRI group had more comfortable feeling than patients in the r-TRI group (P=-0.001).Conclusions R-TRI produces a comparable procedure success rate and incidence of vascular complication when compared to i-TRI.It should be considered as an acceptable and safe procedure。

Abstract：Background Pulmonary complications are a major cause of mortality after operation for cancer of the gastric cardia or esophagus.Although the risk involved in gastric cardiectomy or esophagectomy associated with a concurrent major pulmonary operation is expected to be much higher,it has seldom been evaluated on the basis of clinical experience.The aim of this study was to investigate the possibility and feasibility of the gastric cardiectomy or esophagectomy associated with a major pulmonary operation.Methods From August 2003 to January 2011,14 patients underwent concurrent gastric cardiectomy or esophagectomy and a major pulmonary operation in our hospital.This included eight for pulmonary invasion of esophageal carcinoma,and six for synchronous lung tumor.All patients underwent systematic lymph node dissection for cardiac or esophageal cancer.To prevent postoperative complications,the operative approach and dissection procedures for cardiac or esophageal carcinoma were modified according to the associated pulmonary operation and the extent of cancer invasion.All thoracotomies for cardiectomy or esophagectomy were performed on the same side as the major pulmonary operation.Results All patients underwent a curative operation.There were no deaths or postoperative complications in the six synchronous lung tumor patients.In the eight pulmonary invasion patients,one patient died of respiratory failure 11 days after operation,and postoperative complications developed in four of them,but none was fatal.Six patients were still alive.Conclusions Curative gastric cardiectomy or esophagectomy associated with concurrent major pulmonary operation is not contraindicated in patients in good condition.In selected patients,when the operative procedures for cardiectomy or esophagectomy are appropriately modified to minimize the effect of the associated pulmonary operation,the treatment is associated with a low operative morbidity and mortality with an acceptable long-term survival。

Abstract：Background Previous studies have different viewpoints about the clinical impact of methicillin resistance on mortality of hospital-acquired bloodstream infection (BSI) patients with Staphylococcus aureus (S.aureus).The objective of this study was to investigate the mortality of hospital-acquired BSI with S.aureus in a military hospital and analyze the risk factors for the hospital mortality.Methods A retrospective cohort study was performed in patients admitted to the biggest military tertiary teaching hospital in China between January 2006 and May 2011.All included patients had clinically significant nosocomial BSI with S.aureus.Multivariate Logistic regression analysis was used to identify the risk factors for hospital mortality of patients with S.aureus BSI.Results One hundred and eighteen patients of more than one year old were identified as clinically and microbiologically confirmed nosocomial bacteraemia due to S.aureus,and 75 out of 118 patients were infected with methicillin-resistant S.aureus (MRSA).The overall mortality of nosocomial S.aureus BSI was 28.0％.Methicillin resistance in S.aureus bacteremia was associated with significant increase in the length of hospitalization and high proportion of inappropriate empirical antibiotic treatment.After Logistic regression analysis,the severity of clinical manifestations (APACHE Ⅱ score) (odds ratio (OR) 1.22,95％ confidence interval (CI) 1.12-1.34) and inadequacy of empirical antimicrobial therapy (OR 0.25,95％ CI 0.09-0.69) remained as risk factors for hospital mortality.Conclusions Nosocomial S.aureus BSI was associated with high in-hospital mortality.Methicillin resistance in S.aureus has no significant impact on the outcome of patients with staphylococcal bacteremia.Proper empirical antimicrobial therapy is very important to the prognosis。

Abstract：Background Proper rotational alignment during total knee arthroplasty (TKA) is important for adequate postoperative patellofemoral and tibiofemoral kinematics,as well as for achieving balanced flexion space at 90°.The effects of computer navigation-assisted total knee replacement and conventional total knee arthroplasty on rotational alignment,mechanical axis,component position and clinical outcomes were compared.Methods Two methods were used in 82 patients and the rotation of the femoral and tibial components in the transverse plane,the combined rotation of the two components,the mismatch between them,and the mechanical axis of the lower limb were analyzed.All of these parameters were measured from postoperative radiographs and computed tomography images.Functional outcomes were compared at 6 weeks and 6 months postoperatively.Results Significant differences were found between the two techniques (P ＜0.05) in the following parameters:average rotation of the femoral component ((1.51±3.55)° vs.(-0.63±3.04)°); combined rotation of the femoral and tibial components (2.85±4.07)° vs.(0.28±3.43)°); and mismatch between the femoral and tibial components ((1.44±4.55)° vs.(-0.43±2.86)°).Differences in the rotation of the tibial component were not statistically significant.The prevalence of outliers (malalignment ＞±3° intemal/external rotation) of the femoral component (31.7％ vs.12.5％) and the tibial component (36.6％ vs.15％) were significantly reduced when the navigation system was used (P＜0.05).In addition,while patients in the navigation group had significantly better mechanical axis and functional outcomes at 6 weeks after surgery (P ＜0.05),there was no significant difference between the two groups (P ＞0.05) with respect to functional outcomes at 6 months.Conclusion The navigation system exhibited higher accuracy than the conventional technique in the transverse and coronal plane,and provided better early functional outcomes。

Abstract：Background Invasive fungal infections such as candidiasis and mold infections cause significant morbidity and mortality in seriously ill patients.Micafungin is an echinocandin antifungal agent with potent activity against most species of Candida and Aspergillus.We did this meta-analysis to clarify whether micafungin offers superior efficacy and safety compared with other antifungal agent for treating infections associated with invasive candidiasis.Methods We did a meta-analysis of randomized controlled trials to examine whether micafungin has superior efficacy and safety compared with other antifungal agents recommended by the treatment guidelines for fungal infection.Seven trials involving 2913 patients were included in this analysis.Odds ratios (OR) and 95％ confidence intervals (CI) were calculated.Results Micafungin was associated with significantly better treatment success compared with the comparator antifungal agents (modified intention to treat,2851 patients; random-effects model,OR 1.20,95％ CI 1.00-1.45,P=0.0487).In addition,micafungin was more effective than the comparators for antifungal prophylaxis of neutropenic patients undergoing hematopoietic stem cell transplantation (OR 1.47,95％ CI 1.08-2.00,P=0.01).Although there was no significant difference between the compared regimens in terms of the incidence of adverse drug effects (OR 0.94,95％ CI 0.77-1.11),fewer patients treated with micafungin withdrew from the studies because of adverse events (OR 0.64,95％ CI 0.44-0.94).Conclusions Micafungin has a good safety and tolerability profile,with an efficacy at least comparable to the other antifungal agents.Micafungin offers advantages over other agents for antifungal prophylaxis.Micafungin offers an appropriate alternative for antifungal prophylaxis rather than the treatment of invasive candida infections。

Abstract：Despite important advances in the diagnosis and treatment of acute pulmonary embolism (APE),assessment of risk and appropriate management of patients remains a difficult task in clinical practice.In addition to hemodynamic instability and critically clinical condition,acute right ventricular dysfunction (RVD) is a major determinant of in-hospital outcomes.The purpose of this review is to discuss the results of these recent developments.Some outcome evaluation,clinical assessment,and therapeutic implications are also included。

Abstract：Objective To assess the experimental and clinical data regarding the effects of electromagnetic fields (EMFs) on fracture non-union.Data sources The English language literature regarding EMFs on fracture non-union were searched using MEDLINE,Web of Science and Embase,for the period January 2006 to June 2011.The search terms were electromagnetic fields and non-union/bone marrow stem cells (BMSCs)/bone.Study selection Articles were included in the review if they were related to the use of EMFs on BMSCs or bone tissue.Papers without full manuscripts available were excluded.Results The basic and clinical research in this field,while somewhat limited,supports the insightful application of EMFs to ameliorate disability due to fracture non-union.Conclusions Further basic and clinical research to validate the use of EMFs in facilitating function and bone reparative processes in fracture non-union is required。

Abstract：Gangliocytic paraganglioma of the duodenum is an extremely rare disease.Few cases have been reported in the literature from 1957 to 2010.We reported a 67-year-old man with gangliocytic paraganglioma of the duodenum。

Abstract：The Freeman-Sheldon syndrome (FSS) is a rare congenital syndrome,characterized with myopathy and dysplasia.The musculoskeletal and soft-tissue manifestations often require orthopedic and plastic surgery.We reported a case of 8-year-old girl with FSS operated on for scoliosis。

Abstract：Pleomorphic xanthoastrocytoma (PXA) is a rare benign tumor that is usually located in the superficial cerebral hemisphere.Most reports of PXAs have included only a single case or small series.Therefore,the data with respect to the natural history of this tumor are fragmentary.We report a case of a PXA in the unusual location of the right lateral ventricle with extensive subarachnoid dissemination.To our knowledge,this is a rare case of PXA in the lateral ventricle.In addition,extensive subarachnoid space dissemination of this distinctly benign type of glioma is exceedingly rare.In our case,there was meningeal dissemination and metastases to the bilateral trigeminal nerves and oculomotor nerves.The neuroradiographic features,tumor location,and dissemination were reviewed。