Resection with limb salvage in an Asian male adolescent with Ewing’s sarcoma: A case report

BACKGROUNDEwing’s sarcoma is a highly malignant primary bone tumor that commonly affects children. For young patients, multidisciplinary treatment and limb salvage are recommended, and surgical plans considering the growth potential and bone activity after tumor resection are essential.CASE SUMMARYAn 11-year-old Asian boy had a 1-mo history of a right-sided limping gait. Imaging revealed a proximal tumor with bone destruction and physeal involvement over the right femoral neck. He was diagnosed with stage IV (T1N0M1aG3) Ewing’s sarcoma with bilateral lung metastases. Neoadjuvant chemotherapy decreased the tumor size and confined it to the metaphyseal region. The patient underwent four stages of surgery: wide tumor excision plus reconstruction with vascular fibular bone graft plus internal fixation; repeat open reduction and internal fixation; femoral lengthening with orthosis after physeal maturity; and orthosis removal and bone elongation (approximately 6 cm). Following surgery, he could walk without discomfort and had almost equal-sized bilateral femoral heads, indicating physis preservation. The surgery was successful, and normal femoral head growth was achieved after complete remission. The patient was able to resume normal activities with equal length of the bilateral lower limbs.CONCLUSIONTumor treatment and reconstruction following resection are important in skeletally immature patients with Ewing’s sarcoma to improve quality of life.     

Combination of extracorporeal membrane oxygenation and continuous renal replacement therapy in critically ill patients: a systematic review

Introduction

Extracorporeal membrane oxygenation (ECMO) is used in critically ill patients presenting acute cardiac and/or pulmonary dysfunctions, who are at high risk of developing acute kidney injury and fluid overload. Continuous renal replacement therapy (CRRT) is commonly used in intensive care units (ICU) to provide renal replacement and fluid management. We conducted a review to assess the feasibility, efficacy and safety of the combination of ECMO and CRRT and to illustrate the indications and methodology of providing renal replacement therapy during the ECMO procedure.

Method

We searched for all published reports of a randomized controlled trial (RCT), quasi-RCT, or other comparative study design, conducted in patients undergoing ECMO plus CRRT. Two reviewers independently selected potential studies and extracted data. We used the modified Jadad scale and the Newcastle-Ottawa for quality assessment of RCTs and non-RCTs, respectively. Statistical analyses were performed using RevMan 5.2.

Results

We identified 19 studies meeting the eligibility criteria (seven cohort, six case control, one historically controlled trial and five studies of technical aspects). There are three major methods for performing CRRT during ECMO: ‘independent CRRT access’, ‘introduction of a hemofiltration filter into the ECMO circuit (in-line hemofilter)’ and ‘introduction of a CRRT device into the ECMO circuit’. We conducted a review with limited data synthesis rather than a formal meta-analysis because there could be greater heterogeneity in a systematic review of non-randomized studies than that of randomized trials. For ECMO survivors receiving CRRT, overall fluid balance was less than that in non-CRRT survivors. There was a higher mortality and a longer ECMO duration when CRRT was added, which may reflect a relatively higher severity of illness in patients who received ECMO plus CRRT.

Conclusions

The combination of ECMO and CRRT in a variety of methods appears to be a safe and effective technique that improves fluid balance and electrolyte disturbances. Prospective studies would be beneficial in determining the potential of this technique to improve the outcome in critically ill patients.

Electronic supplementary material

The online version of this article (doi:10.1186/s13054-014-0675-x) contains supplementary material, which is available to authorized users.     

Surgery for hepatocellular carcinoma with tumor thrombosis in inferior vena cava: A case report

BACKGROUNDHepatocellular carcinoma (HCC) accompanied by a tumor thrombus is very common. However, the treatment strategy is controversial and varies by the location of the thrombus.CASE SUMMARYWe report herein a case of HCC with a tumor thrombus in the suprahepatic inferior vena cava (IVC), which was successfully treated by hepatectomy combined with thrombectomy following sorafenib chemotherapy. A 47-year-old woman with chronic hepatitis was diagnosed with HCC. Computed tomography and magnetic resonance imaging showed that the tumor lesion was located in the right half of the liver, and a tumor thrombus was detected in the suprahepatic IVC near the right atrium. After multi-departmental discussion and patient informed consent, right major hepatectomy and total removal of the tumor thrombus were successfully performed under cardiopulmonary bypass. There were no serious complications after surgery. Following sorafenib treatment, no recurrence has been detected so far (11 mo later).CONCLUSIONSurgical treatment followed by adjuvant sorafenib therapy might be an acceptable choice for HCC patients with tumor thrombosis in the IVC.     

Primary extraskeletal Ewing’s sarcoma of the lumbar nerve root: A case report

BACKGROUNDEwing’s sarcoma (ES) is a highly aggressive bone malignancy. Extraskeletal ES (EES) originating in the spinal canal is extremely rare. Herein, we report on a rare case of EES with a primary lumbar spinal nerve root including the complete diagnosis and treatment.CASE SUMMARYA young female patient presented with a complaint of right lower limb pain for 1 mo. Magnetic resonance imaging (MRI) revealed an 11 mm × 14 mm × 31 mm mass in the lumbar epidural region extending at the fifth lumbar spine (L5) level toward the right L5 neural foramen. Our initial diagnosis was an epidural schwannoma. The patient underwent total laminectomy, tumor resection and pedicle screw internal fixation and the L5 root tumor was found to have been completely removed intraoperatively. Histopathological examination of the lesion showed a typical ES with a large number of small, round cells. Immunohistochemistry analysis indicated positive CD99 and S100. After surgery, the patient received chemotherapy and radiotherapy with a 1 year of follow-up and no recurrent tumors or new lesions were found upon spine MRI and positron emission tomography/computed tomography reexamination.CONCLUSIONClinically, ES outside the bone should be considered when nerve root tumors are encountered inside and outside the spinal canal and the diagnosis should be determined by pathological biopsy. After surgical resection, chemotherapy and radiotherapy should be performed. After treatment, active follow-up and regular review should be completed.     

Superior vena cava drainage improves upper body oxygenation during veno-arterial extracorporeal membrane oxygenation in sheep

IntroductionDifferential hypoxia is a pivotal problem in patients with femoral veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) support. Despite recognition of differential hypoxia and attempts to deliver more oxygenated blood to the upper body, the mechanism of differential hypoxia as well as prevention strategies have not been well investigated.MethodsWe used a sheep model of acute respiratory failure that was supported with femoral VA ECMO from the inferior vena cava to the femoral artery (IVC-FA), ECMO from the superior vena cava to the FA (SVC-FA), ECMO from the IVC to the carotid artery (IVC-CA) and ECMO with an additional return cannula to the internal jugular vein based on the femoral VA ECMO (FA-IJV). Angiography and blood gas analyses were performed.ResultsWith IVC-FA, blood oxygen saturation (SO₂) of the IVC (83.6 ± 0.8%) was higher than that of the SVC (40.3 ± 1.0%). Oxygen-rich blood was drained back to the ECMO circuit and poorly oxygenated blood in the SVC entered the right atrium (RA). SVC-FA achieved oxygen-rich blood return from the IVC to the RA without shifting the arterial cannulation. Subsequently, SO₂ of the SVC and the pulmonary artery increased (70.4 ± 1.0% and 73.4 ± 1.1%, respectively). Compared with IVC-FA, a lesser difference in venous oxygen return and attenuated differential hypoxia were observed with IVC-CA and FA-IJV.ConclusionsDifferential venous oxygen return is a key factor in the etiology of differential hypoxia in VA ECMO. With knowledge of this mechanism, we can apply better cannula configurations in clinical practice.

Electronic supplementary material

The online version of this article (doi:10.1186/s13054-015-0791-2) contains supplementary material, which is available to authorized users.     

Endometrial stromal sarcoma extending to the pulmonary artery: A rare case report

BACKGROUNDEndometrial stromal sarcoma (ESS) is a rare malignant mesenchymal tumor. Early in the disease, the findings on magnetic resonance imaging are similar to those of leiomyoma. When the lesion involves both vascular and cardiac tissue, it might be misdiagnosed as intravenous leiomyomatosis, which is not common in the clinic. CASE SUMMARYWe present the case of a 34-year-old female patient with tumor embolus, which extended from the right iliac vein and ovarian vein to the inferior vena cava (IVC), and then to the right atrium and right ventricle, and finally protruded into the pulmonary artery. The patient had undergone a hystero-myomectomy 7 years previously. Based on the findings of the imaging examinations, the diagnosis of intravenous leiomyomatosis was considered preoperatively. The patient then underwent complete resection of the endovascular and intracardiac tumor embolus. The postoperative pathology results confirmed metastatic ESS with endovascular and intracardiac involvement. The patient was discharged from hospital in good condition, and there was no sign of recurrence 5 mo after the operation.CONCLUSIONExtending from the iliac vein and ovarian vein to the IVC, this metastatic ESS invaded both vascular and cardiac tissues. For patients with ESS involving vascular and cardiac tissues, pathological examinations are essential for the differential diagnosis, such as intravenous leiomyomatosis. In addition, due to the high recurrence rate of ESS, long-term and close follow-up evaluation is necessary.     

Venous line filtration: a novel technique for cases involving inferior vena caval and right atrial tumor and associated thrombus: a two-case series

Two patients are presented with right atrial tumors, who were considered to be at risk for tumor thrombus migration down the venous line into the cardiopulmonary bypass (CPB) circuit during surgical excision, which may lead to compromised or interrupted venous drainage. An arterial line filter was placed in the venous line to capture any material that might become dislodged and embolize into the circuit. Vacuum-assisted venous drainage, at approximately -50 mmHg, was used to overcome any resistance caused by the filter in the venous line. In one case, tumor thrombus obstructed the filter, requiring the use of the bypass line around the filter, so venous return could be maintained. In both cases, thrombus was found in the filter. The first case was a 44-year-old female (81 kg, 137 cm, body surface area (BSA) 1.66 m2) who presented with a metastatic pheochromocytoma with associated thrombus arising from the left adrenal gland and extending into the inferior vena cava (IVC) and right atrium. The second case was a 37-year-old male (95 kg, 178 cm, BSA 2.17 m2), who presented with a very large angiosarcoma tumor involving almost the entire right atrium. We have shown that venous line filtration with vacuum-assisted venous drainage can be performed safely, and should be considered in cases where there is tumor thrombus in the vena cava or right atrium, which may threaten venous return.     

Transthoracic echo: A sensitive tool for detecting cardiac extension of renal cell carcinoma?

Renal cell carcinoma is a common urological malignancy with the unique ability to invade the inferior vena cava (IVC) and to extend into the right atrium of the heart. Of those with Renal cell carcinoma only 4%-25% are found to have IVC invasion and of those only 2%-10% extend into the right atrium. If treated surgically, extension of tumor thrombus is not a determinant of survival; therefore it is imperative to determine the presence and extent of tumor thrombus in order to determine surgical approach and tumor resection. To date this has been primarily accomplished by magnetic resonance imaging and computed tomography. We present a case of 61 years old African American woman in which transthoracic echocardiography provided a more accurate determination/characterization of the presence and degree of tumor thrombus and extension.     

Coexisting calcified inferior vena cava thrombus and adrenal hemorrhage in the neonate: Report of three cases

Coexisting calcified inferior vena cava (IVC) thrombus and adrenal hemorrhage are uncommon in children. We present three such cases diagnosed by ultrasound in three boys who were born after 41-, 38-, and 38-week gestations, respectively. In all cases the pregnancy and delivery were unremarkable. The birthweight was 3,630 g, 3,210 g, and 3,390 g, respectively. Left adrenal hemorrhage occurred in all cases. Associated left renal vein thrombus occurred in two cases. The mean followup was 8.5 years. In two cases ultrasound showed persistence of a calcified thrombus and residual stenosis of the IVC. In the third case the IVC thrombus had almost disappeared. © 1994 John Wiley & Sons, Inc.     

Core biopsies can be used to distinguish differences in expression profiling by cDNA microarrays

The primary focus of this work was to determine the feasibility of obtaining representative expression array profiles from clinical core biopsies. For this purpose we performed six 16-gauge needle core biopsies and an excision biopsy on each of two different human xenografts, one from an Ewing’s sarcoma cell line and the second from neuroblastoma cell line grown in Beige-Scid mice. Three of the six core biopsies were processed separately and the remaining three were pooled and processed together. As the initial RNA material isolated from the core biopsies was not sufficient for microarray analysis, an amplification procedure using a modified Eberwine protocol was performed, and the amplified products applied onto a 6000-feature human cDNA microarray. Comparisons of the array results from core biopsies (amplified RNA) and surgical specimens (non-amplified RNA) showed maintenance of the expression profile as assessed by hierarchical clustering. Gene expression profiles obtained from microarray analysis clearly differentiated the Ewing’s sarcoma from the neuroblastoma with both core and excisional biopsies as starting material. Pooling the core biopsies did not improve the concordance with excisional biopsies. In conclusion, our results suggest that core biopsies can be used as a suitable and reliable material for the determination of tumor genetic profiles.     

Role of the emergency department in implementing an extracorporeal membrane oxygenation cardiopulmonary resuscitation

The Royal Brisbane and Women's Hospital has introduced an extracorporeal membrane oxygenation (ECMO) cardiopulmonary resuscitation (E-CPR) service with collaboration between ED and ICU teams for refractory cardiac arrest patients. E-CPR is potentially beneficial to patients who do not gain return of spontaneous circulation after conventional advanced cardiac life support treatments, provided specific demographic and biochemical inclusion criteria are met. A joint ICU and ED decision is reached to commence ECMO flow. We discuss our rationale to use the ED and the emergency physician role in leading the multidisciplinary team, with ICU leading the cannulation team. The development of ED processes and the increased availability of this intervention can significantly impact the survivability of refractory cardiac arrest with good neurological outcomes.     

Extracorporeal Membrane Oxygenation with rituximab‐combined chemotherapy in AIDS‐associated primary cardiac lymphoma: A case report

Although effective combination of antiretroviral medications is being developed, the incidence of non‐Hodgkin lymphoma (NHL) with human immunodeficiency/acquired immunodeficiency syndrome (HIV/AIDS) still remains significantly higher than that in individuals without infection. Primary cardiac lymphoma (PCL) is an NHL that involves the heart and/or the pericardium. PCL is very rare and often causes serious complications, which can be a diagnostic challenge. To our knowledge, no study has reported the measurement of rituximab concentration under venoarterial extracorporeal membrane oxygenation (VA‐ECMO). Herein, we report the case of a 54‐year‐old male patient with AIDS‐associated primary cardiac NHL who developed right ventricular outflow tract obstruction. The patient experienced fatigue and dyspnea on exertion. Contrast‐enhanced computed tomography showed a bulky tumor mass in his right atrium and ventricle, and an echocardiogram revealed severe hypokinesis of his heart and poor cardiac output. A biopsy was performed, and immunohistochemistry revealed diffuse large B‐cell lymphoma. Therefore, he was treated with rituximab‐combined chemotherapy under VA‐ECMO. Blood levels of rituximab were measured during chemotherapy with VA‐ECMO. Thereafter, he was temporarily discharged from the hospital. This clinical case suggests that VA‐ECMO and rituximab‐combined chemotherapy are useful in rescuing patients with severe cardiopulmonary failure due to AIDS‐associated PCL.     

The impact of CRRT modality in patients with AKI receiving ECMO: A nationwide registry study in Taiwan

PurposePatients receiving extracorporeal membrane oxygenation (ECMO) commonly develop acute kidney injury (AKI) and frequently require continuous renal replacement therapy (CRRT). The impact of different CRRT modalities on survival in patients receiving ECMO remains unclear.Materials and methodsUsing claims data from Taiwan's National Health Insurance Research Database, a total of 1077 patients who received ECMO and either continuous venovenous hemofiltration (CVVH) or continuous venovenous hemodialysis (CVVHD) for AKI were identified. Inverse probability of treatment weighting was applied using propensity scores to balance the baseline covariates of the two groups. The primary outcome was in-hospital morality.ResultsWe identified 1077 patients (mean age 57.9; 71.8% men). Postcardiotomy shock (49.2%) was the most frequently reported indication for ECMO. The CVVH group had a lower risk of in-hospital mortality (68.4% vs. 76.9%; odds ratio 0.65; 95% confidence interval [CI] 0.50–0.85) compared with the CVVHD group. The CVVH group also had a shorter mean ICU stay compared with the CVVHD group (mean difference −4.59 days, 95% CI −9.15 to −0.03 days).ConclusionOur results suggest that compared with CVVHD, CVVH may be associated with a lower risk of in-hospital mortality in patients with AKI who receive ECMO.     

Impact of connecting methods of continuous renal replacement therapy device on patients underwent extracorporeal membrane oxygenation: A retrospectively observational study

ObjectiveThe objective of this study was to compare the safety and efficiency of different extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT) connection methods.BackgroundThe number of patients receiving ECMO is increasing, and the fields of application are getting wider. However, patients receiving ECMO are prone to acute kidney injury and fluid overload requiring CRRT. There are few comparative studies of two different systems of connecting CRRT device and ECMO from safety and efficacy perspective.MethodsThis retrospective observational study included patients receiving ECMO in the extracorporeal life support centre of the First Affiliated Hospital of Nanjing Medical University from June, 2015, to December, 2020. Patients were divided into the parallel system group and integrated system group according to the connecting method between ECMO circuit and CRRT line. The outcomes were discharge survival rate, CRRT therapeutic dose completion rate, CRRT catheterisation time, CRRT initiating time, local bleeding at the CRRT catheter site, mean filter life, ECMO circuit thrombosis, ECMO air leakage, or blood leakage due to CRRT.ResultsThirty patients in the parallel system group and 70 patients in the integrated system group were finally included. The discharge survival rate and CRRT therapeutic dose completion rate were not significantly different between the two groups. The parallel system group had significant longer CRRT initiating time (49.0 ± 12.1 min vs. 14.6 ± 2.1 min, P < 0.001) and shorter filter life (11.5 ± 3.2 h vs. 47.3 ± 14.0 h, P < 0.001) than the integrated system group. The occurrence rate of local bleeding was 93.3% in the parallel system group, and there is no bleeding case in the integrated system group. There was no case of ECMO circuit thrombosis from CRRT as well as ECMO air or blood leakage caused by CRRT in either group. ECMO therapy can be adapted by adjusting the position of the CRRT outlet in the integrated system.ConclusionsConnecting CRRT and ECMO as an integrated system might accelerate CRRT initiation, avoid local bleeding, and prolong filter life compared to the parallel system. The chance of developing CRRT-related ECMO circuit leak and thrombosis is manageable.     

Successful management of infected right iliac pseudoaneurysm caused by penetration of migrated inferior vena cava filter: A case report

BACKGROUNDIndwelling inferior vena cava (IVC) filters might cause various complications, including filter penetration, filter fracture, filter migration, and thrombosis of the IVC. Penetration and migration complications are common, while a caudal migrated double-basket filter with associated infected iliac pseudoaneurysm has seldom been reported.CASE SUMMARYWe report a 64-year-old female admitted for sudden onset of severe right abdominal pain after IVC filter placement for 3 mo. The patient had a history of failed endovascular IVC filter retrieval. Computed tomography showed that the retrieval hook of the filter penetrated the right common iliac artery and vein, leading to right iliac artery pseudoaneurysm accompanied by right ureteral obstruction with ipsilateral hydronephrosis, and bilateral iliac veins were occluded. Emergency open repair was performed to remove the IVC filter, the right iliac pseudoaneurysm, and the compromised segments of the iliac veins and IVC with right common iliac artery reconstruction. Staphylococcus aureus was isolated from the tissue culture. The patient was discharged on postoperative day 12 with anticoagulation therapy and antibiotic therapy after discharge. Six-month follow-up computed tomography revealed that the right common iliac artery was patent, and only mild hydronephrosis was detected.CONCLUSIONAn indwelling IVC filter, even ‘embedded’ within organized thrombus, could still cause life-threatening complications. Open procedures remain the last resort for IVC filters with severe complications.     

右心声学造影在静脉-静脉体外膜肺氧合插管中的应用初探

目的 观察右心声学造影在静脉-静脉体外膜肺氧合（V-V ECMO）插管中的应用价值。方法 收集18例接受经右股静脉及右颈内静脉插管V-V ECMO治疗的重症肺炎致急性呼吸衰竭患者，根据插管时是否行右心声学造影分为造影组（n=8）和对照组（n=10），测量并记录其中心静脉压（CVP）及心输出量（CO）。运转ECMO后，对造影组患者行心血管常规超声检查及右心声学造影，观察并调整下腔静脉内套管尖端位置；对照组患者仅行心血管常规超声，观察下腔静脉瓣处血流方向及下腔静脉内套管尖端位置。测量并记录2组患者下腔静脉内套管尖端与膈肌的距离（DCTD）及左股动脉氧分压，比较组间DCTD、左股动脉氧分压、CVP及CO差异。结果 造影组DCTD[5.14（4.68，5.81）mm]和左股动脉氧分压[90.05（85.06，95.33）mmHg]均高于对照组[3.31（2.96，3.69）mm、78.61（71.82，81.04）mmHg，Z=-1.93、-2.20，P均<0.05]；组间CVP和CO差异无统计学意义（Z=-1.79，-1.80，P均>0.05）。结论 右心声学造影有助于V-V ECMO插管时调整下腔静脉内套管尖端位置，减少再循环，提高临床治疗效果。     

Tumor extending through inferior vena cava into the right atrium. A late recurrence of renal cell carcinoma

Renal cell carcinoma is a tumor with a distinct feature; it can invade through renal vein to the inferior vena cava (IVC) and growths intravascularly extending sometimes into the right cardiac chambers. Previous reports of renal cancer with extension to the right atrium at the time of diagnosis have been published. We present the case of a patient with a right atrial mass arising from the IVC found by echocardiography, which was subsequently proved to be a recurrence of renal cell carcinoma 5 years after radical nephrectomy.     

Algoritmo de movilización temprana para el paciente crítico. Recomendaciones de expertos

IntroductionIntensive care unit (ICU)-acquired weakness is developed by 40%-46% of patients admitted to ICU. Different studies have shown that Early Mobilisation (EM) is safe, feasible, cost-effective and improves patient outcomes in the short and long term.ObjectiveTo design an EM algorithm for the critical patient in general and to list recommendations for EM in specific subpopulations of the critical patient most at risk for mobilisation: neurocritical, traumatic, undergoing continuous renal replacement therapy (CRRT) and with ventricular assist devices (VAD) or extracorporeal membrane oxygenation (ECMO).MethodologyReview undertaken in the Medline, CINAHL, Cochrane and PEDro databases of studies published in the last 10 years, providing EM protocols/interventions.Results30 articles were included. Of these, 21 were on guiding EM in critical patients in general, 7 in neurocritical and/or traumatic patients, 1 on patients undergoing CRRT and 1 on patients with ECMO and/or VAD. Two figures were designed: one for decision-making, taking the ABCDEF bundle into account and the other with the safety criteria and mobility objective for each.ConclusionsThe EM algorithms provided can promote early mobilisation (between the 1^st and 5^th day from admission to ICU), along with aspects to consider before mobilisation and safety criteria for discontinuing it.     

连续性肾脏替代治疗嵌入体外膜肺氧合管路连接方式的研究进展

体外膜肺氧合联合连续性肾脏替代治疗能有效改善和预防液体过负荷、清除炎症介质,提高危重患者的抢救成功率。该文介绍了连续性肾脏替代治疗嵌入体外膜肺氧合管路的不同连接方式的优缺点、护理操作及其风险,提出结合患者病情选择最佳连接模式,减少相关并发症的发生,为连续性肾脏替代治疗嵌入体外膜肺氧合患者的护理提供参考。