Differences in diffusion tensor imaging parameters of brain white matter tracts between patients with myotonic dystrophy type 1 and type 2 - a retrospective single-centre study.

INTRODUCTION: The main aim of our study was to compare diffusion tensor imaging (DTI) parameters in patients with myotonic dystrophy types 1 and 2 (DM1 and DM2). CLINICAL RATIONALE FOR THE STUDY: To ascertain whether DTI could be used to assess the integrity of white matter tracts in the brain and identify any abnormalities or disruptions in connectivity between different brain regions in patients with DM. By providing a more detailed understanding of the structural changes in the brain associated with DM, could DTI potentially be used to develop more effective treatments for the cognitive and neurological symptoms of the disorder? MATERIAL AND METHODS: We retrospectively compared MRI scans of 19 patients with DM1 to those of 23 healthy, matched controls, and of 16 patients with DM2 to those of 20 healthy, matched controls, and finally compared the DM1 and DM2 samples. Fraction anisotropy (FA), mean diffusivity (MD), radial diffusivity (RD), and axial diffusivity (AD) values were assessed using Tract Based Spatial Statistics (TBSS). RESULTS: In patients with DM1, a statistically significant decrease in the values of the FA parameter was revealed in 45/48 white matter tracts compared to patients with DM2. There was no statistically significant decrease in the values of the FA parameter in patients with DM2 compared to DM1. The values of MD and RD were significantly higher in 47 tracts in DM1 patients compared to DM2 patients. AD values were significantly higher in all 48 tracts in DM1 patients compared to DM2 patients. There were no tracts with increased MD, AD, or RD values in DM2 patients compared to DM1. CONCLUSIONS: Our results indicate diffuse disintegration of white matter pathways in DM patients, especially in the DM1 group. The damage to all types of fibres (association, commissural, and projection) may explain the diversity of clinical symptoms, which were more severe in the DM1 group of patients than in the DM2 group. CLINICAL IMPLICATIONS: DTI in patients with DM may help us to understand the neural mechanisms underlying brain involvement during the disease. In future, it may help to identify biomarkers for disease progression and treatment response.

Can Color Doppler Ultrasound Be Effectively Used as the Follow-Up Modality in Patients Undergoing Splenic Artery Aneurysm Embolization? A Correlational Study between Doppler Ultrasound, Magnetic Resonance Angiography and Digital Subtraction Angiography.

Splenic artery aneurysm (SAAs) rupture is associated with a high mortality rate. Regular surveillance with imaging before and after intervention is crucial to guide best evidence treatment. The following study aimed to determine the efficacy of color Doppler ultrasound imaging (DUS) compared to digital subtraction angiography (DSA) and magnetic resonance angiography (MRA) as a follow-up modality after selective coil embolization of true SAAs. We analyzed data from 20 patients, 15 females (48.1 ± 16.1 years) undergoing selective SAA coil embolization using detachable fibered embolization coils. Imaging using DUS, MRA, and DSA was performed 3 months after the initial embolization or the consequent re-embolization procedure. Primary clinical success, defined as Class I aneurysm occlusion, on 3-month follow-up was seen in 16 (80.0%) patients. DUS had a sensitivity of 94.4% and a specificity of 42.9% when compared to DSA and 92.3% and 30%, respectively, when compared to MRA in identifying Class I aneurysm occlusion. The positive predictive value (PPV) of DUS in identifying the need for re-embolization was 75.0%, while the NPV of DUS in these terms was 90.5%. DUS showed a high sensitivity in detecting aneurysm occlusion and clinical success, simultaneously exhibiting poor specificity. Still, with caution, this follow-up modality could be used for monitoring select low-risk patients after selective embolization of SAAs. DUS could provide a higher cost-to-benefit ratio, enabling more systematic post-procedural follow-up, as it is far more commonly used compared to MRA and non-invasive compared to DSA.

Role of DTI-MRI parameters in diagnosis of ALS: useful biomarkers for daily practice? Tertiary centre experience and literature review.

INTRODUCTION: Despite the rapid development of neuroimaging techniques, the diagnosis of amyotrophic lateral sclerosis (ALS) remains a significant challenge. Magnetic resonance imaging (MRI) is important for ruling out ALS mimickers, while Diffusion Tensor Imaging (DTI) is a useful tool for the identification of cortical tract damage. The aim of this study was to identify the optimal set of DTI parameters to support the diagnosis of ALS that could be applied to everyday MRI and be used as a disease biomarker in daily practice. MATERIAL AND METHODS: Forty-seven ALS patients and 55 age- and gender-matched healthy individuals underwent MRI using a 1.5-Tesla scanner including a DTI sequence with 30 spatial directions and a b-value 0/1,000 s/mm2. Two independent researchers measured the DTI parameters: fractional anisotropy (FA), TRACE and apparent diffusion coefficient (ADC) using freehand regions of interest (ROIs) placed along both corticospinal tracts (CSTs), starting at the level of the internal capsule and ending at the medulla. RESULTS: Statistical significance was only achieved for fractional anisotropy (FA) (ALS vs controls, p < 0.001). The highest sensitivity was found in the brainstem (cerebral peduncles, pons and pyramids) where it ranged from 72.3% to 80.9%, whereas the highest specificity was observed at the level of the internal capsule (94.6%). The combined highest sensitivity and specificity was obtained in the pons (72.3% and 72.7%, respectively). Classifier based positive predictive values for Youden index cut-off scores varied between 60.7% and 69.4%. CONCLUSIONS: Fractional anisotropy (FA) measured at the level of the brainstem was shown to be the single most relevant parameter in differentiating patients with ALS from healthy subjects. This has the potential to become an ALS-specific biomarker for patient identification in daily practice.

Selective occlusion of splenic artery aneurysms with the coil packing technique: the impact of packing density on aneurysm reperfusion correlated between contrast-enhanced MR angiography and digital subtraction angiography.

PURPOSE: The aim was to evaluate the relationship between coil packing densities after splenic artery aneurysm (SAA) treatment using detachable microcoils and rates of SAA reperfusion and to suggest a post-treatment surveillance protocol using contrast-enhanced MRA. MATERIALS AND METHODS: Evaluated were 16 patients (4 men; mean age 46.7), who underwent true SAA embolization using detachable microcoils (Concerto, Medtronic). SAAs were treated by selective coil packing (CP) or stent-assisted coil exclusion (SAC). Contrast-enhanced magnetic resonance angiography (CE-MRA) and digital subtraction angiography (DSA) were performed at 3 months post-procedure and correlated. RESULTS: Primary CP was used in 13 patients, while SAC was used in three patients. On follow-up, complete aneurysm occlusion was seen in seven patients (43.8%). Sac reperfusion occurred in nine patients (56.2%) and was demonstrated in all CE-MRA and six DSA studies. Mean aneurysm packing density was 20.10 ± 8.05% for the CP group and 32.90 ± 11.95% for the SAC group (p = 0.038). There was a significant difference in the incidence of aneurysm sac reperfusion on CE-MRA study between CP and SAC (9 vs. 0). No sac reperfusion was seen in aneurysms with packing densities ≥ 29%, irrespective of either embolization method. CONCLUSION: Favorable midterm results for coil packing of SAAs seem to depend on the coil packing density with a coil volume approximately a quarter of the aneurysm volume being most effective. Follow-up should involve the use of CE-MRA as this modality has been shown to be superior over DSA in detecting aneurysm reperfusion and coil compaction. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Promoting False-Lumen Thrombosis after Thoracic Endovascular Aneurysm Repair in Type B Aortic Dissection by Selectively Excluding False-Lumen Distal Entry Tears.

PURPOSE: To evaluate the efficacy and clinical outcomes of ancillary endovascular procedures in promoting false-lumen (FL) thrombosis (FLT) and preventing aortic expansion in patients after thoracic endografting for type B dissections. MATERIALS AND METHODS: This retrospective review included 15 patients (12 men and 3 women; mean age, 59.6 y). Mean aortic diameter at the time of ancillary treatment was 47.4 mm. Different techniques were used as single procedures or sequentially: covered stent occlusion of detached visceral artery entry tears, occlusion of single entry tears with vascular plugs, or aortic endograft occlusion of multiple FL entry tears. FL embolization with ethylene vinyl alcohol copolymer was performed when selective occlusion was considered insufficient to close distal entry tears. Apart from endovascular aneurysm repair, all procedures were performed percutaneously under local anesthesia. If FL diameter increase persisted after 6-month follow-up computed tomographic (CT) angiography, another intervention was planned; otherwise, yearly follow-up was performed. RESULTS: Mean clinical follow-up duration was 43.8 months (range, 8 d to 86.8 mo), with no in-hospital mortality. Estimated overall survival rates were 93.3%, 86.6%, and 77% at 12, 24, and 48 months, respectively. Three late deaths occurred, one of which was dissection-related at 40 months. Eight surviving patients (53%) had total FLT and 3 had partial FLT with stable aortic diameter on follow-up CT angiography. FL diameter increased in one patient, requiring further intervention. CONCLUSIONS: Selective exclusion of new distal entry tears remaining after thoracic endovascular aneurysm repair can stabilize abdominal aortic expansion and promote FLT.

Improving the results of transarterial embolization of type 2 endoleaks with the embolic polymer Onyx.

INTRODUCTION: Type 2 endoleaks (T2E) occur in 10 to 20% of patients after endovascular abdominal aortic aneurysm repair (EVAR) and remain a significant clinical issue. AIM: To evaluate the efficacy and clinical outcomes of transarterial treatment of persistent type II endoleaks after EVAR using the liquid embolic Onyx. MATERIAL AND METHODS: From February 2012 to August 2015 transarterial T2E embolization was attempted in 22 patients (21 men, median age: 73, range: 62-88 years). Indications for treatment included an increase in the diameter of the aneurysm sac above 5 mm and a persistent endoleak observed for more than 6 months. Mean time from EVAR to endoleak treatment was 43 months (range: 2-125 months). RESULTS: Primary technical success was achieved in 17 (77.3%) patients and secondary technical success in 81.8%, with 0% in-hospital mortality. The mean procedure time was 95 ±48 min, with an average fluoroscopy time of 54 ±25 min. The mean amount of Onyx used was 7.5 ±6.6 ml. Clinical success was seen in 17/21 patients with follow-up imaging (80.9%). Mean follow-up time was 17 months (range: 3-38 months). CONCLUSIONS: Onyx has been shown to effectively stabilize previous aneurysm growth as a result of the T2E in the majority of our patients. Transarterial embolization of T2E can be significantly improved as compared to previously reported results by using liquid embolic polymers such as Onyx.

Transarterial Chemoembolization Prior to Liver Transplantation in Patients with Hepatocellular Carcinoma.

BACKGROUND: Transarterial chemoembolization (TACE) induces ischemic tumor necrosis, which is intensified by regional chemotherapy. By reducing the active tumor tissue, it can be assumed that patients on the waiting list for liver transplantation may benefit from this locoregional treatment. The aim of this study was to assess the relevance of TACE in hepatocellular carcinoma (HCC) patients before liver transplantation. MATERIAL AND METHODS: A retrospective analysis was performed on data of 229 patients who were transplanted for HCC. A group of 75 patients were treated with TACE prior to liver transplantation. Tumor necrosis related to pretransplantation locoregional treatment was assessed in an explanted liver and classified into extensive (51-100%), moderate (26-50%) and limited (<25%) grades. Five-year recurrence-free survival was estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS: In total, 143 HCC lesions were treated with TACE. Extensive necrosis was found in 63 (44.0%) tumors. Moderate and limited necrosis were observed in 42 (29.4%) and 38 (26.6%) tumors, respectively. In 36 (58.1%) explanted livers, every tumor was classified as extensively necrotic. The 5-year recurrence-free survival was estimated as 81.6% in the group not treated with TACE prior to liver transplantation (TACE-) and as 73.1% in the TACE+ group (p=0.169). Among patients not fulfilling the Milan criteria, 5-year recurrence-free survival was 63.1% in TACE- and 65.1% in TACE+ (p=0.656). CONCLUSIONS: In conclusion, TACE prior to liver transplantation is effective in inducing tumor necrosis. However, evidence of benefits in long-term results after liver transplantation requires further confirmation.

[Heart failure as an independent prognostic factor for endovascular method of abdominal aortic aneurysm treatment]. / Niewydolnosc serca jako niezalezny czynnik prognostyczny w leczeniu tetniaka aorty brzusznej sposobem wewnatrznaczyniowym.

Endovascular aneurysm repair (EVAR) has been accepted as an alternative to traditional open surgery in selected patients. Now it is a widely accepted standard. In case of contraindications for open repair, after accomplishing including criteria for EVAR, the patient can be treated by this method. Despite the minimally invasiveness of this treatment, several complications may occur during or after EVAR. Complications arise from the limitations of the method and improper patient selection. We report a case of patient with heart failure and complications after EVAR.

[The skin incisions (blow holes) for treatment of massive subcutaneous emphysema]. / Blow holes ­ metoda leczenia masywnej odmy podskórnej .

BACKGROUND: Subcutaneous emphysema (SE) is rarely life-threatening, although it may create significant discomfort to patients. It may impede eye opening, movement of the limbs and sometimes causes stridor and respiratory distress. We describe two cases of SE, in which small incisions in the skin helped to relieve symptoms. CASE REPORTS: Case 1. A 64-year-old male was admitted to ITU, having been intubated after blunt chest trauma during a traffic accident. Initial presentation included respiratory failure, massive SE of the face, neck and chest, and fractured ribs with bilateral pneumothorax and bilateral lung contusion. Ventilation with BiPAP with 15 cm H2O PEEP was commenced and a right chest drain was inserted. This resulted in rapid improvement of gas exchange, but SE became progressively larger. On the second day, several 2 cm skin incisions were made bilaterally in the subclavicular regions; immediately a loud hiss of escaping air was heard and the patient's condition improved rapidly. He was extubated after seven days and made a full recovery. Case 2. A 42-yr-old male was admitted to ITU three days after a street fight because of rapidly progressing SE, extending to the head, neck, chest, abdomen and legs. He was suffering from pneumomediastinum, pneumopericardium, and broken ribs, hyoid bone and Th10 spinous process. An emergency tracheostomy was performed and blow holes were made in both subclavicular regions. This resulted in rapid improvement and he was discharged home after two weeks in hospital. DISCUSSION AND CONCLUSION: Several methods of treatment for severe SE have been described, including pleural drainage, subcutaneous insertion of pig-tail drains, iv cannulas or large bore drains. The method described, albeit not always successful, is simple and can be applied in every setting.

Time-resolved contrast-enhanced MR angiography: Value of hemodynamic information in the assessment of vascular diseases.

BACKGROUND: To assess the quality of images obtained from time-resolved MRA together with the accuracy of this technique in diagnosing vascular diseases and the usefulness of haemodynamic information provided by this method. MATERIAL/METHODS: The study group included 120 patients with different vascular pathologies excluding of intracranial vessels. All patients underwent time-resolved MRA on 1.5T unit. Results were correlated with other imaging techniques: DSA (n=36), CTA (n=28), Doppler ultrasound (n=71) and intraoperative findings (n=10). Independently, two radiologists evaluated the MRA studies assessing the quality of the images in a 3 point scale (3 - good, 1 - poor), as well as the presence or absence of haemodynamic information (3 - relevant dynamic information, 2 - irrelevant dynamic information, 1 - lack of dynamic information) for different vascular pathologies. RESULTS: Mean quality of MRA examinations was 2.94 (reader A and B) and was similar for different pathologies (kappa value =0.757). The mean grading (reader A and B) for the presence of dynamic information was above 2 for the following pathologies: celiac artery branch pseudoaneurysm (3), vascular malformation (3), subclavian steal syndrome (2.5), Leriche's syndrome (2.25), aortic dissection (2.06), renal artery stenosis (2.03); and below 2 for: pelvic arterial occlusive disease (1.75), abdominal aortic aneurysm (1.31), carotid artery stenosis (1.1), thoracic aortic aneurysm (1.0). Kappa value was 0.802. The sensitivity was 95%, specificity 96% and positive predictive value 98%. CONCLUSIONS: Time-resolved MRA provides good quality images and enables reliable diagnosis of vascular pathologies.

Emergency endovascular treatment for ruptured type B dissection in the abdominal aorta.

Despite successful endograft placement in the thoracic aorta, dissections remain problematic in the abdominal aorta. Herein, the authors describe two successful cases of endovascular treatment of ruptured abdominal aortic dissections. One patient, despite previously undergoing successful thoracic endograft placement, presented with a ruptured false channel and was treated by excluding major re-entries with a covered renal stent and stent-graft limb. A second patient, with a ruptured dissection superimposed on a preexisting abdominal aortic aneurysm, was treated with thoracic and abdominal stent-grafts. In both patients, progressive healing of the aorta occurred, with patients presenting no symptoms at an average follow-up of 20 months.

An attempt to assess the influence of primary disease on the results of therapeutic rehabilitation in an early post-operative period in orthotopic liver transplant recipients.

BACKGROUND: A retrospective analysis of the effects of early postoperative rehabilitation in patients undergoing orthotropic liver transplantation in correlation to the primary liver disease leading to transplantation. MATERIALS AND METHODS: In between 2000 and 2003 in the Department of General, Transplant and Liver Surgery of the Medical University of Warsaw Central Teaching Hospital, 213 orthotropic liver transplants (OLT) were performed. Full rehabilitation records were available for 136 patients. For statistical analysis, patients were divided into 5 different groups representing different causes of primary liver insufficiency. RESULTS: Achieved early rehabilitation results show that there was a strong correlation between the time at which physiotherapy was introduced and the ability to achieve a fully upright position in correlation to the primary cause of liver disease necessitating transplantation. The shown relationship is probably a direct effect of the time of disease progression before transplantation is required. The worst results were observed in group no. 4 where patients with acute and subacute liver insufficiencies were included, even though these patients had the lowest mean age. CONCLUSIONS: 1. The achieved results confirm the general compensation rule which states that the process of healing strongly depends on the time of disease development. 2. An analysis of the time, when rehabilitation was initiated and the ability of the patient to achieve a fully upright position in relation to the cause of liver insufficiency suggests that the reason for most delays is the inability to develop adequate compensational mechanisms as a result of rapid disease progression in cases of acute liver insufficiency.

Allotransplantation of cultured parathyroid progenitor cells without immunosuppression: clinical results.

BACKGROUND: Hypoparathyroidism is a well-known consequence of extensive thyroid and parathyroid surgery. Allotransplantation of cultured parathyroid cells can be considered as an alternative to vitamin D3 and calcium supplementation in treatment of hypoparathyroidism. We present the long-term allotransplant activity in 85 patients who had undergone cellular allotransplantation for surgical hypoparathyroidism. Also, a modified technique to prepare parathyroid explants is described for obtaining a new nonimmunogenic cell population. METHODS: From March 1990 to December 2004, 85 patients underwent 116 allotransplantations of cultured parathyroid cells. Mean recipient age was 46.2+/-11.1 years. Donors were selected from patients undergoing parathyroidectomy for secondary and tertiary hyperparathyroidism. RESULTS: After 6 weeks of cultivation and freezing, the parathyroid cells decreased their normal human leukocyte antigen (HLA) class I ABC expression and were free of HLA class II positive cells. The viability of cultured cells was 95.15+/-2.94%. Eighty-five patients underwent primary allotransplantation. Of these, 25 patients subsequently underwent a repeat procedure. In six cases, the parathyroid cells were obtained from the same donor and in 19 cases from a different donor. For all patients, the mean cellular allograft survival was 6.35+/-13.08 months. In 64 patients (55.1%), the allografts retained their endocrine function for more than 2 months. CONCLUSIONS: The present study has shown that in some patients parathyroid cell allotransplantation may be considered a method of treatment for permanent hypoparathyroidism after thyroid surgery. Graft function and/or survival did not depend on the baseline viability or secretory activity of cultured cells used for transplantation.

Secondary kidney transplantation in a patient 16 years after simultaneous pancreas and kidney transplantation--a case report.

Simultaneous pancreas and kidney transplantation (spktx) is currently the most effective method of treatment of type 1 diabetes complicated by renal insufficiency. The first successful spktx in Poland was performed in the Department of General, Vascular and Transplant Surgery of the Warsaw Medical University on the 4th of February 1988. Since then 70 spktx were performed in our Department. We present a 44-year-old patient who after 16 years of good function of both transplanted organs presented with elevated creatinine levels (>4 mg/dl) as a result of chronic rejection of the kidney allograft. On the 22nd of January 2005 the patient underwent secondary kidney transplantation. The immunosuppresive protocol consisted of MMF, CsA and steroids. Humanized anti-lL-2 monoclonal antibodies (daclizumab) were used as pre-procedure induction. Using a mid-line incision the new kidney graft was anastomosed to the recipient left external iliac vessels. The ureter was anastomosed with the bladder without anti reflux procedures and the allograft was placed in the retroperitoneum below the previously transplanted kidney. Graftectomy of the first kidney allograft was not performed. After surgery, normal creatinine parameters were restored to a level of 1, 1 mg/dl and an increase in urine output was noted from 1 to 4 liters per day. Oral intake of foods was resumed on the 4th postoperative day and no early complications were observed. 12 months observation period confirmed stabile function of both transplanted organs. Secondary kidney transplantation in patients after spktx is technically possible and may be considered an option in patients with diminishing function of the first kidney allograft.

The comparison of treatment results of type 1 diabetes mellitus complicated by end-stage diabetic nephropathy in patients undergoing simultaneous pancreas and pre-emptive kidney transplantation (SPPkTx) and patients enrolled into the dialysis program--a cohort study.

OBJECTIVE: A cohort study was conducted to compare treatment of patients with type 1 diabetes mellitus and end-stage diabetic nephropathy. PATIENTS AND METHODS: 47 type 1 diabetic patients required renal replacement therapy in years: 2001-2005 were enrolled. Simultaneous pancreas and preemptive kidney transplant (sppktx) was performed in 18 (group I). Group II consisted of 29 patients who entered dialysis program. Survival rate for patients from both groups was estimated. Transplanted organ function was evaluated for group II. Lipid profile and its correlation with thickness of carotid media was assessed. Impact of sppktx on diabetic retinopathy was investigated. Cost and life quality were compared between groups. RESULTS: Two-year cumulative recipient survival rate for group I and II was 100% and 96%, respectively. One-year cumulative survival rate for transplanted pancreas was 88% and for kidney grafts 94%. In group I cholesterol and triglyceride level before transplantation were: 207 +/- 38 mg/dl and 133 +/- 65 mg/dl and decreased after transplantation to 155 +/- 20 mg/dl and 78 +/- 25 mg/dl, respectively (p < 0.05). No difference of carotid media thickness was observed between groups. Stabilization of retinopathy was observed in 91.6% non-blind recipients. During the first year of the follow-up the costs of transplantation doubled those of dialysis therapy but in the second year the costs of dialysis exceeded the costs required for transplanted patients. CONCLUSION: Despite of major surgery and introduction of immunosuppression in group I, results did not differ significantly between groups during a two-year follow-up. After sppktx, stabilization of the carotid media was slower than the normalization of lipids. At the second year, transplantation is less expensive than dialysis.

A matched case-control study on the prognosis of native kidney neoplasia in renal transplant recipients.

A matched case-control study was performed to compare the prognosis of six renal transplant recipients with a control group of non-transplant patients, both groups presenting with native kidney tumors. Patients were matched for age, gender, neoplasm histology, and TNM classification. The follow-up ranged from 8 to 131 months (median 32 months) after nephrectomy or nephroureterectomy of the native kidney. Five out of six recipients retained good graft function. No evidence of recurrent local disease or distant metastasis of kidney neoplasms was observed in either group. In the transplant group, two patients developed basal cell carcinoma, and one, lung cancer and a disseminated lymphoma. Among control group patients, prostate and bladder cancer were noted. Prognosis of early-stage kidney malignancy in the transplant and non-transplant group was comparable despite immunosuppression; however, recovery from the primary kidney malignancy did not exclude the risk of developing a new type of neoplasm in either group.