Successful Surgical and Endovascular Multidisciplinary Therapy for Mid-aortic Syndrome with Complicated Atherosclerotic Comorbidities in an Older Patient

Mid-aortic syndrome (MAS) is a rare vascular disorder that causes refractory hypertension. A 76-year-old woman was hospitalized for acute heart failure (HF) with drug-resistant hypertension; other comorbidities included epigastric artery rupture, old myocardial infarction, an intraventricular thrombus, and a cerebral artery aneurysm. Angiography revealed severe narrowing of the descending aorta, which led to the diagnosis of MAS. Although intensive medical treatment improved her HF, optimal blood pressure (BP) could not be achieved. Percutaneous coronary intervention and surgical bypass for diseased aorta was then performed in two stages, resulting in the achievement of optimal BP and alleviation of HF.     

Comparison of Symptomatic Vasospasm after Surgical Clipping and Endovascular Coiling

Vasospasm, initial neurological damage, rebleeding, and periprocedural complications are associated prognostic factors for clinical outcomes after aneurysmal subarachnoid hemorrhage (SAH). In this study, factors related to delayed ischemic neurological deficit (DIND) are evaluated using data from our institute for the last 18 years. Data from 2001 to 2018 of patients with aneurysmal SAH who underwent surgical clipping (SC) or endovascular coiling (EC) within 7 days of onset were retrospectively analyzed. Cases of mortality within 5 days after treatment were excluded. Multivariate analysis was used to identify the risk factors for DIND. In total, 840 cases of SAH were assessed; among these cases, 384 (45.7%) and 456 (54.3%) were treated with SC and EC, respectively. The frequency of DIND in the EC group was significantly less than that in the SC group (11.8% vs. 17.7%; p = 0.016). In the results of multivariate analysis, internal carotid artery (ICA) aneurysm and hemorrhagic complications were the risk factors for DIND. Cilostazol administration and EC were significant factors for vasospasm prevention after aneurysmal SAH (odds ratio of ICA aneurysm: 1.59, hemorrhagic complications: 1.76, SC: 1.51, and cilostazol administration: 0.51, respectively). Cilostazol administration was also a significant factor in patients who were treated with EC. ICA aneurysm, treatment strategy, hemorrhagic complications, and cilostazol administration were associated with DIND. Oral administration of cilostazol and avoiding hemorrhagic complications were effective in DIND prevention. If both treatments are available for ruptured aneurysms, clinicians should choose EC on the basis of its ability to prevent DIND.     

Does Systemic Chemotherapy Influence Skeletal Growth of Young Osteosarcoma Patients as a Treatment-Related Late Adverse Effect?

The aim of this study was to investigate the influence of systemic chemotherapy on the skeletal growth of young osteosarcoma patients as a treatment-related late adverse effect. We reviewed the height data of 20 osteosarcoma patients (13 males and 7 females) aged ≤18 years. The average (±SD) age at diagnosis was 14.5 (±3.3) years. The average follow-up interval was 89.6 months. After wide resection of the affected bones, reconstruction with tumor prostheses and auto-bone grafting was carried out in 11 and 9 cases, respectively. Pearson’s correlation coefficient was calculated to evaluate the association between actual and predicted (using Paley’s multiplier method) heights. Z-scores were used to compare the initial and final heights with the Japanese national growth curve. Actual and predicted heights were correlated according to Pearson’s correlation coefficient (R = 0.503). Z-analysis showed that statistical significance (p = 0.04) was noted for the height data Z-scores of patients between ≤10 years and >10 years at the final follow-up. Systemic chemotherapy did not reduce skeletal growth in young osteosarcoma patients as a late adverse effect based on two different evaluation methods. However, patients aged ≤10 years at diagnosis may develop a short stature after systemic chemotherapy.     

Repeated Necrotizing Lymphadenitis with MEFV Gene Mutations

We herein report a 36-year-old man with repeated necrotizing lymphadenitis due to MEFV gene mutations. The patient''s chief complaints were a fever and painful cervical lymphadenopathy. We diagnosed him with necrotizing lymphadenitis based on the pathological findings of the lymph nodes and the exclusion of other differential diseases. The same episode recurred four times. We speculated the involvement of autoinflammatory backgrounds and detected MEFV gene mutations of E148Q (homo), P369S, and R408Q. Considering the elevation of interleukin-18, these mutations probably played roles in the repeated necrotizing lymphadenitis.     

Pancreas-Sparing Duodenectomy for a Recurrent Retroperitoneal Liposarcoma: Report of a Case

Pancreas-sparing duodenectomy (PSD), which allows preservation of the pancreas in its entirety, is a promising procedure for low-grade malignancies of the duodenum, the periampullary region, and the neighboring retroperitoneum. We report a case of recurrent retroperitoneal liposarcoma involving the second and third parts of the duodenum, which was extirpated using PSD, after a right hemicolectomy for tumor invasion of part of the colonic hepatic flexura. The Roux-en-Y jejunal limb was sutured to the duodenal bulb in an end-to-end fashion, and the biliary and pancreatic duct systems were reconstructed with end-to-side anastomoses, placing the jejunal limb distal to the bulbo-jejunostomy after a septoplasty to repair the adjacent pancreatic and biliary ducts. Because retroperitoneal liposarcoma has a low incidence of lymph node metastasis, pancreaticoduodenectomy may be inappropriate, especially if minimally extensive surgery can ensure organ preservation. Pancreas-sparing duodenectomy could be the most appropriate procedure for nonepithelial malignant tumors located around the duodenum distal to the pylorus, which have no involvement with the pancreatic parenchyma or periduodenal lymph nodes.     

Disease-associated CIAS1 mutations induce monocyte death, revealing low-level mosaicism in mutation-negative cryopyrin-associated periodic syndrome patients

Cryopyrin-associated periodic syndrome (CAPS) is a spectrum of systemic autoinflammatory disorders in which the majority of patients have mutations in the cold-induced autoinflammatory syndrome (CIAS)1 gene. Despite having indistinguishable clinical features, some patients lack CIAS1 mutations by conventional nucleotide sequencing. We recently reported a CAPS patient with mosaicism of mutant CIAS1, and raised the possibility that CIAS1 mutations were overlooked in "mutation-negative" patients, due to a low frequency of mosaicism. To determine whether there were latent mutant cells in "mutation-negative" patients, we sought to identify mutation-associated biologic phenotypes of patients' monocytes. We found that lipopolysaccharide selectively induced necrosis-like cell death in monocytes bearing CIAS1 mutations. Monocyte death correlated with CIAS1 up-regulation, was dependent on cathepsin B, and was independent of caspase-1. Cell death was intrinsic to CIAS1-mutated monocytes, was not mediated by the inflammatory milieu, and was independent of disease severity or anti-IL-1 therapy. By collecting dying monocytes after lipopolysaccharide treatment, we succeeded in enriching CIAS1-mutant monocytes and identifying low-level CIAS1-mosaicism in 3 of 4 "mutation-negative" CAPS patients. Our findings reveal a novel effect of CIAS1 mutations in promoting necrosis-like cell death, and demonstrate that CIAS1 mosaicism plays an important role in mutation-negative CAPS patients.     

Selected ventriculoplasty for idiopathic dilated cardiomyopathy with advanced congestive heart failure: midterm results and risk analysis

Background: To treat advanced heart failure due to idiopathic dilated cardiomyopathy, surgical ventricular restoration with mitral reconstruction was conducted and evaluated. Methods: In 95 patients (81 men, mean age: 54 years), New York Heart Association class III/IV was 44/51, and 33 patients (36%) were inotropic dependent preoperatively. Mitral regurgitation (≥2+) was noted in all patients. All patients underwent left ventriculoplasty (septal anterior ventricular exclusion in 38, partial left ventriculectomy in 57) and mitral reconstruction (repair 53, replacement 42). Fifty-two patients (55%) had concomitant tricuspid repair. Intra-aortic balloon pumping and left ventricular assist device was used in 24 patients and two patients, respectively. Results: Hospital mortality was 11.6% (11 of 95), with 6.6% (5 of 76) in elective and 31.6% (6 of 19) in emergency operations. The ejection fraction and cardiac index increased from 22.3 ± 6.3% to 27.2 ± 8.0% and from 2.3 ± 0.5 ml/m²/min to 2.8 ± 0.5 ml/m²/min, respectively (p < 0.001). The endodiastolic volume index, endosystolic volume index and diastolic dimension decreased from 232.9 ± 56.1 ml/m² to 160.0 ± 49.8 ml/m², from 178.9 ± 46.7 ml/m² to 113.8 ± 44.7 ml/m² and from 82.0 ± 9.0 mm to 68.9 ± 11.6 mm, respectively (p < 0.001). Late death occurred in 27 patients with 22 cardiac deaths. The mean NYHA class was 1.7 among the survivors. One-, 3- and 5-year survival rates were 72.8%, 61.4% and 50.5%, respectively. In the 62 patients who were non-inotropic dependent preoperatively, 1-, 3-, and 5-year survival rates (81.8%, 73.7% and 62.9%) were significantly better than the inotropic-dependent group (55.3%, 37.3% and 28.0%). Patients with mitral annuloplasty showed a significantly higher 5-year survival rate than patients with mitral valve replacement (59.6% vs 43.6%) in univariate analysis. By application of the exclusion site selection method, the two different ventriculoplasty procedures did not show significant difference in survival rates. Multivariate analysis showed that preoperative inotropes and old age were significant predictors for postoperative mortality. Conclusion: The selected ventriculoplasty in combination with mitral annuloplasty is a useful option for patients with an extremely dilated left ventricle in idiopathic dilated cardiomyopathy. Surgery should be considered before inotropic dependency occurs when prior medical treatment has failed.