Reduction of extramedullary erythropoiesis and amelioration of anemia in a ß-thalassemia patient treated with thalidomide.

ß-thalassemia patient treated with thalidomide: dimensional reduction of EMH foci (MRI evaluation) and reduction of hematological responce at follow-up.

Giant Internal Carotid Artery Aneurysm of the Cavernous Segment.

A 70-year-old woman with a history of hypertension developed acute onset of severe tightness headache accompanied by vomiting. Patient reported having right diplopia and occasional and temporary right peripheral facial paralysis for two weeks. Neurological examination revealed only a right squint. Cerebral multi-detector computed tomographic angiography (MD-CTA) and Magnetic Resonance Imaging (MRI) revealed a giant right internal carotid aneurysm (2.7 cm) at the cavenous segment (Figure 1A, 1B). No abnormalities were noted in subarachnoid spaces and white - grey matter. A selective digital subtraction angiography (DSA) of the right internal carotid artery confirmed the giant aneurysm at the cavenous segment (Figure 2A, 2B). Subsequently endovascular flow-diverting stent was deployed at the cavernous segment of the right internal carotid artery to treat over time the giant aneurysm. Her clinical course has no neurological symptoms and she was discharged after two days with established over time clinical and imaging follow-up. Giant intracranial aneurysms and vascular anomalies of the internal carotid are rare, and are defined "giant" those greater than 2.5 cm in diameter (1-4). Conservative treatment of giant intracranial aneurysms have a mortality rates of 65-100% in 2-5 years (4). Clinical presentation of internal carotid aneurysms at the petrous segment can manifest from no-symptom to headache, cranial nerve disturbs and epistaxis (1- 4). MD-CTA and MRI are the two gold standard methods for diagnosis in patients with suspected internal carotid artery and intracranial artery aneurysm (1,5). Internal carotid aneurysms at the cavenous segment are located in a surgically difficult accessible area, therefore endovascular percutaneous techniques are considered the first lines treatments (6).

Novel Benchmark Values for Open Major Anatomic Liver Resection in Non-cirrhotic Patients: A Multicentric Study of 44 International Expert Centers.

OBJECTIVE: This study aims at establishing benchmark values for best achievable outcomes following open major anatomic hepatectomy for liver tumors of all dignities. BACKGROUND: Outcomes after open major hepatectomies vary widely lacking reference values for comparisons among centers, indications, types of resections, and minimally invasive procedures. METHODS: A standard benchmark methodology was used covering consecutive patients, who underwent open major anatomic hepatectomy from 44 high-volume liver centers from 5 continents over a 5-year period (2016-2020). Benchmark cases were low-risk non-cirrhotic patients without significant comorbidities treated in high-volume centers (≥30 major liver resections/year). Benchmark values were set at the 75th percentile of median values of all centers. Minimum follow-up period was 1 year in each patient. RESULTS: Of 8044 patients, 2908 (36%) qualified as benchmark (low-risk) cases. Benchmark cutoffs for all indications include R0 resection ≥78%; liver failure (grade B/C) ≤10%; bile leak (grade B/C) ≤18%; complications ≥grade 3 and CCI ® ≤46% and ≤9 at 3 months, respectively. Benchmark values differed significantly between malignant and benign conditions so that reference values must be adjusted accordingly. Extended right hepatectomy (H1, 4-8 or H4-8) disclosed a higher cutoff for liver failure, while extended left (H1-5,8 or H2-5,8) were associated with higher cutoffs for bile leaks, but had superior oncologic outcomes, when compared to formal left hepatectomy (H1-4 or H2-4). The minimal follow-up for a conclusive outcome evaluation following open anatomic major resection must be 3 months. CONCLUSION: These new benchmark cutoffs for open major hepatectomy provide a powerful tool to convincingly evaluate other approaches including parenchymal-sparing procedures, laparoscopic/robotic approaches, and alternative treatments, such as ablation therapy, irradiation, or novel chemotherapy regimens.

Imaging of ring-shaped lateral ventricular nodules (RSLVNs).

Ring-shaped lateral ventricular nodules (RSLVN) are small and round nodules attached on the ependyma of lateral ventricles with unknown nature. They are considered "leave me alone lesions" and differential diagnosis includes subependymal grey matter heterotopia, subependymomas, subependymal hamartomas, and subependymal giant cell astrocytomas. In this short article, we report imaging findings of RSLNVs discovered in five patients, underlining the pivotal role of neuroimaging in the diagnostic path.

How to deal with right hepatic artery coming from the superior mesenteric artery during minimally invasive pancreaticoduodenectomy: A systematic review.

BACKGROUND: Anatomical variations in the liver arterial supply are quite common and can affect the surgical strategy when performing a minimally invasive pancreaticoduodenectomy (MIPD). Their presence must be preemptively detected to avoid postoperative liver and biliary complications. DATA SOURCES: Following the PRISMA guidelines and the Cochrane protocol we conducted a systematic review on the management of an accessory or replaced right hepatic artery (RHA) arising from the superior mesenteric artery when performing an MIPD. RESULTS: Five studies involving 118 patients were included. The most common reported management of the aberrant RHA was conservative (97.0%); however, patients undergoing aberrant RHA division without reconstruction did not develop liver or biliary complications. No differences in postoperative morbidity or long-term oncological related overall survival were reported in all the included studies when comparing MIPD in patients with standard anatomy to those with aberrant RHA. CONCLUSIONS: MIPD in patients with aberrant RHA is feasible without increase in morbidity and mortality. As preoperative strategy is crucial, we suggested planning an MIPD with an anomalous RHA focusing on preoperative vascular aberrancy assessment and different strategies to reduce the risk of liver ischemia.

Bariatric surgery reduces the risk of pancreatic cancer in individuals with obesity before the age of 50 years: A nationwide administrative data study in France.

BACKGROUND: Obesity is a well-established risk factor for pancreatic cancer. Bariatric surgery has demonstrated superior results in terms of weight loss and obesity-related comorbidities compared to medical and behavioral treatments. The aim of this study is to evaluate the effect of bariatric surgery on pancreatic cancer incidence in individuals with obesity. METHOD: Individuals with a diagnosis of obesity were retrieved from the French national hospital discharge database. We conducted a cohort study comparing the risk to develop pancreatic cancer in individuals with obesity with and without history of bariatric surgery; the inverse probability of treatment weighting (IPTW) method was performed to assess the uncertainty around the results. Moreover, a subgroup analysis according to age at the time of bariatric surgery was performed to study its impact on the risk of pancreatic cancer. Finally, possible differences depending on the type of bariatric procedure (sleeve gastrectomy vs Roux-en-Y gastric bypass) were also explored. RESULTS: 160,129 (Bariatric Surgery group) and 1,263,804 (control group) patients with 5.2 ± 1.9 and 6.0 ± 1.9 years of follow-up respectively were included. A significant reduced risk to develop pancreatic cancer during follow-up was identified for the bariatric surgery group in the overall population (HR: 0.567). However, this reduced risk was only observed in the 18-50 years group. These results were furtherly confirmed after IPTW analysis. No difference was found between different bariatric procedures. CONCLUSION: Bariatric surgery has a protective effect against pancreatic cancer in the 18-50 years population. High-quality prospective studies are needed to confirm these results.

Challenges in Bariatric Surgery: Outcomes in Patients Having Three or More Bariatric Procedures.

INTRODUCTION: Over the last two decades, a progressive increase in failure rate of bariatric surgery (BS) has occurred in conjunction with an exponential increase in BS worldwide. Bariatric surgeons are confronted with challenging situations in patients with a complex bariatric history. In this study, we aim to evaluate the feasibility and outcomes of revisional BS in patients with at least two or more previous bariatric procedures. METHODS: Data were retrospectively retrieved from a prospectively held database of bariatric procedures performed at our tertiary referral bariatric center and included procedures done from February 2013 up to April 2019 by a single center. RESULTS: Thirty patients underwent a third bariatric procedure. The median age was 40 (18-57) and 54 (27-69) years at the time of the first and the last procedures, respectively. Laparoscopic adjustable gastric banding was the first procedure in 26 patients. The complication rate was 33%; no patient required additional surgery because of postoperative complications. A total weight loss of 29.6% and an excess loss of 53.4% were obtained at a mean follow-up of 61 months after the last redo bariatric procedure. CONCLUSION: This study indicates that redo BS either conversional or revisional is feasible and effective in patients with a complex bariatric history including two or more previous procedures. Careful patients' selection is mandatory and extensive information should be given on the increased risk of postoperative complications.

Long-Term Neuroradiological and Clinical Evaluation of NBIA Patients Treated with a Deferiprone Based Iron-Chelation Therapy.

Neurodegeneration with brain iron accumulation (NBIA) comprises various rare clinical entities with brain iron overload as a common feature. Magnetic resonance imaging (MRI) allows diagnosis of this condition, and genetic molecular testing can confirm the diagnosis to better understand the intracellular damage mechanism involved. NBIA groups disorders include: pantothenate kinase-associated neurodegeneration (PKAN), mutations in the gene encoding pantothenate kinase 2 (PANK2); neuroferritinopathy, mutations in the calcium-independent phospholipase A2 gene (PLA2G6); aceruloplasminemia; and other subtypes with no specific clinical or MRI specific patterns identified. There is no causal therapy, and only symptom treatments are available for this condition. Promising strategies include the use of deferiprone (DFP), an orally administered bidentate iron chelator with the ability to pass through the blood-brain barrier. This is a prospective study analysis with a mean follow-up time of 5.5 ± 2.3 years (min-max: 2.4-9.6 years) to define DFP (15 mg/kg bid)'s efficacy and safety in the continuous treatment of 10 NBIA patients through clinical and neuroradiological evaluation. Our results show the progressive decrease in the cerebral accumulation of iron evaluated by MRI and a substantial stability of the overall clinical neurological picture without a significant correlation between clinical and radiological findings. Complete ferrochelation throughout the day appears to be of fundamental importance considering that oxidative damage is generated, above, all by non-transferrin-bound iron (NTBI); thus, we hypothesize that a (TID) administration regimen of DFP might better apply its chelating properties over 24 h with the aim to also obtain clinical improvement beyond the neuroradiological improvement.

Imaging of superior ophthalmic vein: A pictorial overview.

The Superior Ophthalmic Vein (SOV) is the largest vein of the orbit and represents an important orbital venous drainage pathway. SOV is well identifiable on CT and MRI, and its alterations may be a clue for differential diagnosis. In this pictorial work we illustrate the most frequent conditions in which SOV appearance may be influenced by various pathologies, providing a pictorial guide for imaging interpretation.

Imaging of cerebral amyloid angiopathy-related inflammation (CAA-ri).

CAA-ri is a reversible and rare encephalopathy that may manifest in patients with CAA. In this short article, we describe CT and MRI findings of CAA-ri in a 67-year-old man presented to emergency with sudden aphasia and acute cognitive dysfunction, underlining the pivotal role of neuroimaging in the diagnostic path.

Clinical and Economic Impact of Bariatric Surgery Post Liver Transplantation: a Nationwide, Population-Based Retrospective Study.

PURPOSE: Prevalence of obesity in liver transplant recipients is increasing with the overall epidemic augmentation of severe obesity, the effects of immunosuppressive drugs, and lifestyle changes which are responsible for de novo obesity development or aggravation of pre-existing obesity. The aim of this study is to analyze the differences in overall mortality, re-hospitalization rate, and hospitalization-related costs between patients undergoing bariatric surgery after liver transplantation and patients undergoing bariatric surgery alone. MATERIALS AND METHODS: Twenty patients with history of liver transplantation who underwent bariatric surgery were analyzed from the French National Hospital Discharge Database. Overall mortality, re-hospitalization rate, length of stay for bariatric procedure, and the costs of bariatric surgery hospitalization and eventual re-hospitalizations were compared to a group of 360,846 patients who underwent bariatric surgery alone from 2010 to 2019. Furthermore, a 1:1 propensity score matching analysis was conducted. RESULTS: Patients with a history of liver transplantation showed an increased risk of overall mortality (HR: 7.66, p = 0.0047) and increased costs of hospitalization for bariatric surgery (8250 ± 4822€ vs 5583 ± 3398€, p = 0.0005). No differences in length of stay, re-hospitalization rate, and costs were found after multivariate analysis. After propensity score matching analysis, a significant increased cost of hospitalization (8250 ± 4822€ vs 6086 ± 1813€, p = 0.0195) still resulted for the liver transplantation group. CONCLUSION: Bariatric surgery represents the best treatment for obesity and its related associated medical problems. Our study highlights an increased risk of overall all-cause mortality and increased costs of hospitalization in this population compared to patients undergoing bariatric surgery alone.

Bioabsorbable Glycolide Copolymer is Effective in Reducing Staple Line Bleeding in Sleeve Gastrectomy.

BACKGROUND: Postoperative bleeding from the staple line after sleeve gastrectomy occurs in 2-8% of patients and it is associated with increased length and cost of hospitalization and may demand reoperation to gain hemostasis. Reinforced staplers are used by bariatric surgeons to reduce the incidence of postoperative leak but can have a role in avoiding bleeding. The aim of this study is to analyze the effects of reinforcement on the whole gastric staple line during sleeve gastrectomy on postoperative bleeding. METHODS: Four hundred forty-eight consecutive patients undergoing sleeve gastrectomy from November 2012 to April 2019 were divided into two groups. In the Top-only group only the top staple shot was realized with reinforced material (GORE® SEAMGUARD®) while in the Bottom-to-Top group, patients received full staple line reinforcement. Statistical analysis focused on postoperative leak and bleeding, and length of stay; a propensity score matching analysis was performed to reduce between-group characteristics imbalance. RESULTS: One hundred forty-five (Top-only) and 303 (Bottom-to-Top) patients were included. Four (2.8%) patients in the Top-only group and none in the Bottom-to-Top group experienced severe bleeding (p = 0.004); the difference was still significant after propensity score matching. Length of stay was significantly shorter for the Bottom-to-Top group before and after propensity score matching (4 vs. 5 days, p < 0.001). CONCLUSIONS: Staple line buttressing reduces the incidence of severe postoperative bleeding when performed on the whole staple line, and it is associated with a shorter hospitalization.

Incisional hernia repair after kidney transplantation in a tertiary high-volume center: outcomes from a 10-year retrospective cohort study.

BACKGROUND AND AIM: Incisional hernia (IH) after Kidney Transplantation (KT) is a challenging complication due to both technical reasons and patients' complexity. Data regarding outcomes of hernia repair in KT recipients are uncertain, since the biggest part of previous papers focused on risk factors for incisional hernia occurrence and not on its outcomes. Aim of the study was to focus on risk factors for incisional hernia recurrence after surgical repair in KT recipients. METHODS: Data regarding all consecutive patients undergoing kidney transplantations from January 2011 until September 2020 in Montpellier University Hospital were retrospectively collected from a single institutional database. RESULTS: After a median follow-up of 48 months (IQR25-75 31-59), data from 1546 consecutive KT were collected. 83 patients underwent 99 incisional hernia surgeries after KT, with 14 patients that had one recurrence (14.4%) and 2 patients that experienced two recurrences (2.4%). Total recurrence rate was 16.8%. At univariate analysis, the only factor associated with an incisional hernia recurrence was having undergone to at least one previous abdominal surgery other than KT (p value 0.002). Overall morbidity was 15% (n = 15), with most of complications classified as mild (59%). No mortality related to incisional hernia repair occurred. CONCLUSION: IHs after KT represent an important condition. Its surgical management is challenging due to its anatomical complexity and patient's status. This is the largest sample size in the literature of patients treated for IH after KT and it shows that a previous surgery other than the KT is a risk factor for hernia recurrence after surgical repair, without regarding surgical technique or other comorbidity and therapeutical factors.

Penetrance of the V203I variant of the PRNP gene: report of a patient with stroke-like onset of Creutzfeld-Jacob Disease and review of published cases.

Creutzfeldt-Jakob disease (CJD) is usually sporadic, but 10-15% of cases are caused by autosomal-dominant pathogenic variants in the prion protein gene (PRNP). A few PRNP variants show low penetrance. We report the case of a 64-year-old man, admitted to the ward with acute onset of aphasia; death occurred 6 weeks later. Brain MRI, EEG pattern and brain pathology were consistent with CJD diagnosis. Genetic analysis revealed a heterozygous V203I variant. We summarized the key clinical findings in patients carrying the V203I variant who were described to date. We also discuss the hypothesis as to whether V203I is a risk factor for CJD rather than a Mendelian disease-associated variant, as well as the possible implications of such hypothesis in the clinical scenario.

Head CT in centenarians: emergency and non-emergency findings.

INTRODUCTION: Our retrospective study evaluates head CTs performed in our Hospital in the last 11 years (2009-2019) in centenarian patients. OBJECTIVES: To estimate the correlation between reasons for examination and CT findings in emergency and to evaluate if there was the expected involution of the brain by analyzing some non-emergency neuroradiological parameters (NENP). MATERIAL AND METHODS: 62 Head CTs performed on patients aged 100 and over were reviewed. They were evaluated emergency CT findings and NENP (leukoencephalopathy, enlargement of the ventricular volume, presence of vascular calcifications). For comparison, NENP were also assessed in two relatively younger populations of 62 patients aged between 65-70 (called "65-70") and 85-90 ("85-90"). RESULTS: In cases of suspected traumatic brain lesions, 11.9% (n = 5/42) of centenarians showed a cerebral bleeding; as concerns suspected stroke we found a higher concordance between clinical suspicion and CT features, 46.6% (n = 7/15). As regards NENP, no significant differences were found with respect to the "85-90" population in terms of severity of leukoencephalopathy, enlargement of the ventricular volume and presence of vascular calcifications. CONCLUSIONS: In emergency, CT plays a pivotal role in defining an immediate diagnosis and from a medico-legal point of view, resulting together with clinical observation the main strategy in the management of centenarians. By the analysis of NENP we may support that centenarians' brains on CT are similar to those of "85-90".