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Background and Objectives: Jejunal artery (JA) and ileal artery (IA) aneurysms constitute less than 3% of all visceral artery aneurysms (VAAs), carrying a risk of rupture as high as 30%, and a mortality of 20%. Though many etiologies have been reported in the literature, no mention exists on a causal association between these aneurysms and inflammatory bowel diseases (IBD). We present the first case of a JA aneurysm related to Crohn’s Disease (CD) together with a review of the literature. Materials and Methods: A 74-year-old male presenting with CD intestinal relapse and an incidental finding at the computed tomography enterography (CTE) of a 53 × 47 × 25mm apparently intact JA pseudoaneurysm, arising from the first and second jejunal branches, underwent coil embolization followed by small bowel resection, with an uneventful outcome. We also included the review of literature on JA and IA aneurysms, analyzing all reports published in PubMed and Scopus from 1943 to July 2022. Results: 60 manuscripts with 103 cases of JA and IA aneurysms in 100 patients were identified. Among cases with available data, 34 (33.0%) presented acutely with rupture, 45 (43.7%) were described as non-ruptured. 83 (80.6%), and 14 (13.6%) were JA and IA aneurysms, respectively, having a median size of 15 (range:3.5–52) mm. Atherosclerosis (16.5%), infections (10.7%), and vasculitides/connective tissue disorders (9.7%) represented the main causes mentioned. Mean age was 53.6 (±19.2) years, male patients being 59.4%. One third of patients (32.4%) were asymptomatic. Overall, treatment was indicated in 63% of patients, with surgery and endovascular procedures performed in 61.9% and 38.1% cases, respectively. The technical success rate of endovascular treatment (EVT) was 95.8%. The mortality rate was 11.8%, being higher (21.2%) in the rupture group. Conclusions: The prompt treatment accomplished in our case granted a successful outcome. JA and IA aneurysms should be included among local complications of IBD. Considering their high potential for rupture, regardless of size, a low threshold for endovascular or surgical treatment should be applied.

Patient: Male, 30-year-old Final Diagnosis: End stage renal disease secondary to hypertensive nephropathy Symptoms: Uremic symptoms Medication:— Clinical Procedure: — Specialty: Transplantology Objective: Rare disease Background: En bloc pediatric kidneys (EBPK) are one potential solution to increase the number of organs available in the donor community, thus promoting transplantation of these allografts into adult recipients. However, EBPK transplantation has been traditionally considered suboptimal due to concerns for perioperative complications, mainly vascular thrombosis. We report an en bloc kidney transplantation using vascular grafts from another deceased donor to extend the EBPK aorta and vena cava and create a tension-free anastomosis with recipient external iliac vessels. Case Report: A pair of 2-month-old female en bloc kidneys weighting 6 kg were transplanted to a 30-year-old adult male. Prolonged cold ischemic time (CIT) was related to high refusal rate and long travel from Nevada to Miami. Prior to transplantation, the EBPK were connected to the LifePort Renal Preservation Machine® and deemed transplantable only after showing a significant improvement in perfusion parameters. Back-table reconstruction was conducted through an end-to-end anastomosis between an adult deceased donor common iliac artery and vein grafts to the inferior vena cava and aortic distal ends, respectively. The patient displayed immediate graft function (IGF) without any postoperative complications, showing a creatinine of 1.5 mg/dl at 4-month follow-up. Conclusions: Use of renal preservation machine (RPM) and refined back-table reconstruction of these allografts are important tools to improve the significant discard rate and improve outcomes of EBPK.

Urologic complications can still occur following kidney transplantation, sometimes requiring multiple radiological and/or surgical procedures to fully correct the problem. Previously proposed extravesical ureteral reimplantation techniques still carry non-negligible risks of the patient developing urologic complications. About 10 years ago, a new set of modifications to the Lich-Gregoir technique was developed at our center, with the goal of further minimizing the occurrence of urologic complications, and without the need for initial ureteral stent placement. It was believed that an improvement in the surgical technique to minimize the risk of developing urologic complications was possible without the need for stent placement at the time of transplant. In this report, we describe the advantages of this technique (i.e., mobilized bladder, longer spatulation of the ureter, inclusion of bladder mucosa with detrusor muscle layer in the ureteral anastomosis, and use of a right angle clamp in the ureteral orifice to ensure that it does not become stenosed). We also retrospectively report our experience in using this technique among 500 consecutive (prospectively followed) kidney transplant recipients transplanted at our center since 2014. During the first 12mo post-transplant, only 1.4%(7/500) of patients developed a urologic complication; additionally, only 1.0%(5/500) required surgical repair of their original ureteroneocystostomy. Five patients(1.0%) developed a urinary leak, with 3/5 having distal ureteral necrosis, and 1/5 subsequently developing a ureteral stricture. Two other patients developed ureteral stenosis, one due to stricture and one due to ureteral stones. These overall results are excellent when compared with other reports in the literature, especially those in which routine stenting was performed. In summary, we believe that the advantages in using this modified extravesical ureteroneocystostomy technique clearly help in lowering the early post-transplant risk of developing urologic complications. Importantly, these results were achieved without the need for ureteral stent placement at the time of transplant.

BACKGROUND General anesthesia is associated with high morbidity/mortality in comorbid older adults. Thoracic continuous spinal anesthesia/analgesia (TCSA) may be an alternative for major abdominal surgery. We report a one-year experience of the use of TCSA in an Italian geriatric center. METHODS Retrospective review of case notes of high-risk older patients (ASA class ≥III) who underwent TCSA for major abdominal surgery between May 2017-May 2018. TCSA was performed with a 21-gauge Tuohy-shaped spinal needle and a 24-gauge catheter (level of insertion between T6-7 and T10-11). Hyperbaric bupivacaine or levobupivacaine (two boluses of 2.5 mg) plus fentanyl (10-25 µg) were injected before incision, followed by additional doses if needed. Intrathecal levobupivacaine plus fentanyl were infused for 72 hours after surgery. We described the anesthesiologic management and evaluated the incidence of postoperative complications. RESULTS Ninety patients (age 84.4±6.9 years, 53.3% male) were enrolled. High comorbidity, according to the Geriatric Index of Comorbidity, was found in 64.4% of the patients. Mean Metabolic Equivalents were 2.58±0.99. Switching to general anesthesia was required in one case. Intraoperative noradrenaline (0.17±0.12 mcg/kg/min) was required in 70.6%. Additional intravenous paracetamol or weak opioids were required in 23% and 2.2% of patients, respectively. No direct complications of TCSA were reported. Cardiac, respiratory and surgical complications occurred in 15.6%, 13.3% and 13.3% of patients, respectively. Delirium occurred in 14.4%. Hospital mortality was 5.6%. CONCLUSIONS TCSA may be a valid alternative to general anesthesia in high-risk older patients undergoing major abdominal surgery. Further studies are needed to confirm its safety and benefits.

BACKGROUND The use of allografts with multiple renal arteries has increased in the era of laparoscopic donor nephrectomy. Although several studies recommend reconstructing lower pole arteries (LPAs) to reduce risk of urologic complications, it is common opinion to ligate upper pole arteries (UPAs) with a diameter less than 2 mm because of increased risk of thrombosis related to their reconstruction. This retrospective study evaluates the feasibility and safety of reconstructing thin UPAs during living-donor kidney transplantation, with the goal of maintaining the integrity of the graft and assuring its maximal function. MATERIAL AND METHODS Data from 922 living-donor kidney transplants performed between 2009 and 2019 were reviewed. Six cases with UPAs were identified (0.65%). The study endpoints were incidence of allograft vascular and urologic complications, slow graft function, delayed graft function, graft failure, and graft and patient survival. RESULTS The UPAs had a mean diameter of 1.8±0.28 mm. Methods of reconstruction included: interposition graft (n=2), end-to-side anastomosis inside the renal hilum to a branch of the main renal artery (n=3), and side-to-side anastomosis with the main renal artery (n=1). Additional reconstruction of LPAs (n=2) and main renal arteries (n=2) was performed. During a median (range) follow-up of 14.5 (9-49) months no complications were observed. CONCLUSIONS Ex vivo reconstruction of UPAs with a diameter less than 2 mm is worth attempting, particularly in the setting of living-donor kidney transplantation.

Background Prevalence and incidence of hepatic haemangioma are estimated from autopsy series only. Although benign and generally asymptomatic, hepatic haemangioma can cause serious complications. Aims The aim of the study was to assess the prevalence of hepatic haemangioma and to attempt to quantify the risk of major complications such as spontaneous rupture. Methods We retrospectively analyzed the radiology database of a Regional University Hospital over a 7-year period: the radiological records of 83,181 patients who had an abdominal computed tomography or magnetic resonance scan were reviewed. Diagnoses made at imaging were reviewed and related to clinical course. Results Hepatic haemangioma was diagnosed in 2071 patients (2.5% prevalence). In 226 patients (10.9%), haemangioma had diameter of 4 cm or more (giant haemangioma). The risk of bleeding was assessed on patients without concomitant malignancies. Spontaneous bleeding occurred in 5/1067 patients (0.47%). All 5 patients had giant haemangioma: 4 had exophytic lesions and presented with haemoperitoneum; 1 with centrally located tumour experienced intrahepatic bleeding. Conclusion Giant haemangiomas have a low but relevant risk of rupture (3.2% in this series), particularly when peripherally located and exophytic. Surgery might be considered in these cases.

BACKGROUND Common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease and is a heterogeneous group of antibody deficiency syndromes characterized by hypogammaglobulinemia, recurrent bacterial infections, and frequent autoimmune manifestations. Liver transplantation (LT) is rarely performed in patients with CVID and associated end-stage liver disease. CASE REPORT We report the successful results of 2 patients who underwent LT with pre-existing diagnosis of CVID. Case 1: A 21-year-old man affected by CVID and HCV-related cirrhosis underwent LT in December 2010 with a 67-year-old deceased donor liver graft. At the time of LT, MELD score was 30. The early post-LT course was characterized by a biliary stricture treated with Roux-en-Y repackaging of the anastomosis. Neither main infections nor acute rejection were detected during the postoperative period. After 43 months follow-up, the patient is alive and well with a histological recurrence of hepatitis C grade 1 and stage 2 according to Metavir staging. Case 2. On March 2013, a 53-year-old woman developed HBV-related fulminant liver failure and underwent urgent LT utilizing a 21-year-old deceased donor liver graft. The postoperative course was characterized by relaparotomy for hemoperitoneum. CMV infection was diagnosed 5 months after LT and resolved after valganciclovir therapy. After 6 months, mild acute rejection was diagnosed and treated with steroids. The patient is currently alive and well. The immunosuppressive regimen was based on Advagraf and early steroids discontinuation in both patients. CONCLUSIONS LT should not be precluded to patients with CVID and end-stage liver diseases. Immunosuppression has a key role in this category of patients to balance the higher risk of rejection and serious infections.

BACKGROUND The purpose of this study was to investigate immunological risk factors associated with BS (Biliary Strictures) after LT (Liver Transplantation). MATERIAL AND METHODS The study included 192 patients out of 273 adult liver transplantations performed from August 2005 to December 2012, with a radiological or surgically proven biliary stricture. About 35 potential risk factors for biliary strictures were studied. Results A biliary stricture was diagnosed in 22.9% of transplants; the anastomotic type and the non-anastomotic type complicated the transplantation in 18.2% and 7.8% of cases, respectively. Six patients (3.1%) presented both of forms. Univariate analysis using logistic regression showed that preoperative serum bilirubin level >2 mg/dl (P=0.040), donor age >46 years old (P=0.007), positive crossmatch (P=0.007), product of donor age and preoperative Model for End Stage Liver Disease (DMELD) >710 (P=0.011), occurrence of acute or chronic rejection (P=0.004; P=0.003), and biliary leak (P=0.002) were all significantly associated with the development of biliary stricture. At the multivariate analysis, Primary Biliary Cirrhosis (PBC) (P=0.019), donor age >46 years (P=0.008), crossmatch positivity (P=0.001), and acute or chronic rejection (P=0.005; P=0.043) appeared to be the only variables independently associated with the development of a biliary stricture. Conclusions Immunological risk factors (PBC, crossmatch positivity, acute and chronic rejection) emerged as being the most important variables associated to the development of biliary strictures after LT.

AIM:To report a single-centre experience with the novel Associating Liver Partition and Portal vein ligation for Staged hepatectomy (ALPPS) technique and systematically review the related literature. METHODS:Since January 2013, patients with extended primary or secondary liver tumors whose future liver remnant (FLR) was considered too small to allow hepatic resection were prospectively assessed for the ALPPS procedure. A systematic literature search was performed using PubMed, Scopus and the Cochrane Library Central. RESULTS:Until July 2014 ALPPS was completed in 9 patients whose mean age was 60 ± 8 years. Indications for surgical resection were metastases from colorectal cancer in 3 cases, perihilar cholangiocarcinoma in 3 cases, intrahepatic cholangiocarcinoma in 2 cases and hepatocellular carcinoma without chronic liver disease in 1 case. The calculated FLR volume was 289 ± 122 mL (21.1 ± 5.5%) before ALPPS-1 and 528 ± 121 mL (32.2 ± 5.7%) before ALLPS-2 (p < 0.001). The increase in FLR between the two procedures was 96 ± 47% (range: 24-160%, p < 0.001). Additional interventions were performed in 4 cases: 3 patients underwent Roux-en-Y hepaticojejunostomy, and one case underwent wedge resection of a residual tumor in the FLR. The average time between the first and second step of the procedure was 10.8 ± 2.9 days. The average hospital stay was 24.1 ± 13.3 days. There was 1 postoperative death due to hepatic failure in the oldest patient of this series who had a perihilar cholangiocarcinoma and concomitant liver fibrosis; 11 complications occurred in 6 patients, 4 of whom had grade III or above disease. After a mean follow-up of 17.1 ± 8.5 months, the overall survival was 89% at 3-6 and 12 months. The recurrence-free survival was 100%, 87.5% and 75% at 3-6-12 months respectively. The literature search yielded 148 articles, of which 22 articles published between 2012 and 2015 were included in this systematic review. CONCLUSION:The ALPPS technique effectively increased the resectability of otherwise inoperable liver tumors. The postoperative morbidity in our series was high in accordance with the data from the systematic review. Age, liver fibrosis and presence of biliary stenting were predisposing factors for postoperative morbidity and mortality.

Wilson's disease (WD) is an autosomal recessive disorder characterized by copper overload. In this disease, inadequate hepatic excretion leads to copper accumulation in the liver, brain, kidney, and cornea. Severe neurological symptoms can develop in patients with WD, often in the absence of relevant liver damage: it is unclear whether liver transplantation (LT) could reverse neurological symptoms, and at present LT is not recommended in this setting. We report a case of regression of neurological symptoms in a patient affected by WD with prevalent neurological involvement. A 19-year-old man with disabling neuropsychiatric symptoms from WD that included frontal ataxia, akinesia, dystonia, tremors, and behavioral disorders in the presence of preserved liver function (Model for End-Stage Liver Disease score=7; Child-Turcotte-Pugh score=A5) underwent LT in November 2009. At the time of LT, encephalic magnetic resonance imaging (MRI) indicated diffuse neurodegenerative alterations involving subtentorial and supratentorial structures; bilateral Kayser-Fleischer ring was present. Four years after LT, laboratory tests show normalized copper metabolism and excellent liver function test results. Encephalic MRI shows a substantial improvement of already-known signal alterations at nuclei thalamus and putamen, mesencephalon, and pons. Kayser-Fleischer ring disappeared from the right eye, but a little remnant is still visible in the left eye. At neurological examination, all of the previous symptoms and signs are no longer present and behavioral disorders are no longer present; psychosocial functions are completely restored. The present case provides some evidence that LT may be a valid therapeutic option for WD patients with marked neurological impairment, particularly in those no longer responsive to chelation therapy.

Patients with syndrome X have been found to have an abnormal coronary blood flow reserve. The physical performance during exercise, however, has been incompletely investigated. Cardiopulmonary exercise testing (CPX) is a reliable noninvasive method to provide indexes of lung, heart, circulation, and muscle functions. In 15 patients (10 women) with syndrome X and in age and sex-matched normal individuals, CPX was performed twice a day (8 am and 4 pm) on two separate occasions 2 months apart. Time and oxygen consumption at peak exercise, at ventilatory anaerobic and electrocardiographic thresholds, as well as norepinephrine plasma concentrations at each work load and at peak exercise in both tests were obtained. In syndrome X in both evaluations, the 4 pm performance was characterized by an earlier onset of both ventilatory anaerobic and electrocardiographic thresholds despite lower values of VO 2 and double-product, and by a greater peak ST segment depression despite similar total exercise time, VO 2 , and double-product. No difference between tests was found in the norepinephrine response to exercise. Normal subjects showed reproducible CPX and hormonal responses in the two tests. Thus these data may suggest a circadian variation of coronary vascular response to exercise in patients with syndrome X, leading to a lower ischemic treshold early in the afternoon. The parallel earlier onset of the ventilatory anaerobic threshold may reflect a concomitant abnormal muscular blood flow response (that is, vasoconstriction of working muscle arteries), suggesting a link between coronary and peripheral circulations.

Percutaneous coronary intervention (PCI) is routinely performed in patients with non-ST elevation acute coronary syndromes after pretreatment with clopidogrel and periprocedural administration of unfractionated heparin on a weight-adjusted basis. Although activated clotting time (ACT) monitoring is encouraged to verify the adequacy of anticoagulation during the procedures, this is not a common practice in many laboratories. The authors describe 4 cases of patients with bifurcation lesions involving the left anterior descending coronary artery, who developed periprocedural thrombosis with acute transmural ischemia. All patients had inadequate ACT measurements, despite conventional heparin dosage and ongoing clopidogrel treatment. In order to achieve complete anticoagulation, patients were switched to bivalirudin, which determined a prompt effect on measured ACT. This therapeutic regimen, coupled with further intervention, allowed resolution of the thrombotic complication without bleeding. This report suggests the feasibility of a strategy of bivalirudin use in patients who have some degree of heparin 'resistance' in the setting of complicated PCI.

The authors report the case of an adult male patient with a congenital ventricular septal defect who underwent an invasive assessment of his heart disease. Here, the septal defect was identified in the perimembranous area and a pulmonary-to-systemic flow ratio of 1.4:1 was calculated. At coronary angiography, a single coronary artery was found. This particular anomaly consisted of a proximal right coronary artery originating from a septal branch of the left anterior descending coronary artery and a distal right coronary artery arising from the distal left circumflex artery. Such an association constitutes an exceedingly rare congenital condition.

The regeneration of tissue to treat mucogingival recession defects is well established but these defects pose additional challenges for the clinician in the creation and maintenance of a space under the guiding membrane. We used titanium-reinforced membranes of expanded polytetrafluoroethylene (ePTFE) to facilitate the creation and maintenance of such spaces in 12 patients with mucogingival recession defects. The membranes were judged according to their ease of handling and placement and the regenerative results; the patients were evaluated for gingival recession and the quantity of attached gingiva before and for as long as 15 months after treatment. At 15 months, the average gingival recession was 1.4 mm (pretreatment = 5.3 mm), and the attached gingiva measured 3.3 mm (pretreatment = 1.4 mm). Titanium-reinforced ePTFE membranes result in a simpler, faster, and more predictable surgical procedure. Comparison of titanium-reinforced ePTFE membranes to other available procedures awaits assessment in clinical trials.