Familial acute aortic dissection associated with a novel ACTA2 germline variant.

Aortic dissection is a life-threatening cardiovascular disease. Hereditary disorders are responsible for a small percentage of cases. Nonetheless, it is important to identify genetic causes, as they are often autosomal dominantly inherited and are of life-saving importance if we can identify persons at risk. Mutations of the ACTA2 gene are the most common cause of non-syndromic familial aortic disease. Exploration of the genetic background in suspected familial cases and determination of the exact etiology are mandatory for management and establishing appropriate follow-up strategies due to the risk of fatal recurrences. Herein, we present a 21-year-old male with a familial acute aortic dissection associated with novel ACTA2 germline variant and discuss the management and surveillance considerations.

Laparoscopic cholecystectomy during the COVID-19 pandemic in a tertiary care hospital in Germany: higher rates of acute and gangrenous cholecystitis in elderly patients.

BACKGROUND: The COVID-19 pandemic caused a global health crisis in 2020. This pandemic also had a negative impact on standard procedures in general surgery. Surgeons were challenged to find the best treatment plans for patients with acute cholecystitis. The aim of this study is to investigate the impact of the COVID-19 pandemic on the outcomes of laparoscopic cholecystectomies performed in a tertiary care hospital in Germany. PATIENTS AND METHODS: We examined perioperative outcomes of patients who underwent laparoscopic cholecystectomy during the pandemic from March 22, 2020 (first national lockdown in Germany) to December 31, 2020. We then compared these to perioperative outcomes from the same time frame of the previous year. RESULTS: A total of 182 patients who underwent laparoscopic cholecystectomy during the above-mentioned periods were enrolled. The pandemic group consisted of 100 and the control group of 82 patients. Subgroup analysis of elderly patients (> 65 years old) revealed significantly higher rates of acute [5 (17.9%) vs. 20 (58.8%); p = 0.001] and gangrenous cholecystitis [0 (0.0%) vs. 7 (20.6%); p = 0.013] in the "pandemic subgroup". Furthermore, significantly more early cholecystectomies were performed in this subgroup [5 (17.9%) vs. 20 (58.8%); p = 0.001]. There were no significant differences between the groups both in the overall and subgroup analysis regarding the operation time, intraoperative blood loss, length of hospitalization, morbidity and mortality. CONCLUSION: Elderly patients showed particularly higher rates of acute and gangrenous cholecystitis during the pandemic. Laparoscopic cholecystectomy can be performed safely in the COVID-19 era without negative impact on perioperative results. Therefore, we would assume that laparoscopic cholecystectomy can be recommended for any patient with acute cholecystitis, including the elderly.

Predictors and severity of intestinal ischaemia following on-pump cardiac surgery: a retrospective, propensity-matched analysis.

OBJECTIVES: Risk factors associated with intestinal ischaemia after heart surgery have been previously explored; however, a paucity of data exists with regard to extent of intestinal ischaemia in patients requiring surgical intervention. The purpose of this study is to assess predictors of abdominal exploration and extent of ischaemia following cardiac surgery. METHODS: A retrospective single-centre study was performed at a university hospital. The patient sample included consecutive patients between 2009 and 2020 who first received cardiac and then abdominal exploration during the same hospital stay. Control group patients were identified by 1:1 propensity matching. Logistic regression was performed to identify risk factors for laparotomy. Patients of the laparotomy group were further analysed for intraoperative findings from required abdominal operations. RESULTS: A total of 6832 patients were identified, of whom 70 (1%) underwent abdominal exploration. The median time to exploratory laparotomy was 6 days with no difference between intraoperatively confirmed ischaemia versus those who underwent negative exploration. Thirty-day mortality was 51%. Prior diagnosis of COPD or administration of 2 or more vaso-inotropes during the postoperative phase was independent risk factors for exploratory laparotomy. Vaso-inotrope use was a strong independent predictor of extent of intestinal ischaemia as well as for 30-day mortality. Degree of intestinal ischaemia was also an independent predictor of 30-day mortality. CONCLUSIONS: Intestinal ischaemia is a feared complication after cardiac surgery with high mortality, often necessitating multiple abdominal procedures. Administration of 2 or more vaso-inotropes in the postoperative phase of cardiac procedure is a strong predictor for the degree of ischaemia and 30-day mortality.

Defining early recurrence in patients with resected primary colorectal carcinoma and its respective risk factors.

PURPOSE: There is no evidence-based definition of early recurrence following resection of colorectal cancer. The purpose of this study is to define a point that discriminates between early and late recurrence in patients who have undergone colorectal cancer resection with curative intent and to analyze associated risk factors. METHODS: A retrospective single-center cohort study was performed at a university hospital recognized as a comprehensive cancer center, specializing in colorectal cancer surgery. Patient data were retrieved from a prospectively maintained institutional database. Included patients underwent resection for primary, non-metastatic colorectal carcinomas with curative intent between 1995 and 2010. Aims of the study were (1) to define the optimal cut-off point of recurrence-free survival based on overall survival using a minimum p value approach and (2) to identify patterns of initial recurrence and putative risk factors for early recurrence using regression models. RESULTS: Recurrence was diagnosed in 412 of 1893 patients. Statistical analysis suggested that a recurrence-free survival of 16 months could be used to distinguish between early and late recurrence based on overall survival (p < 0.001). Independent risk factors for early recurrence included advanced pT categories (pT3,4/ypT3,4) and positive lymph node status (pN+/ypN+). Early recurrence was independent of site of recurrence and was associated with worse prognosis. CONCLUSIONS: Recurrence of colorectal carcinoma within 16 months after primary treatment should be labeled as "early." Tumor categories pT3,4/ypT3,4 and positive lymph node status pN+/ypN+ are predictive of early recurrence.

Primary aortoduodenal fistula - overlooked because of guidelines?

Primary aortoduodenal fistula is an uncommon yet mostly lethal finding. We present a case of a 63 year-old male who exhibited significant upper gastrointestinal bleeding and hemorrhagic shock. Repeated endoscopies did not detect any source of bleeding. Emergency laparotomy disclosed an aortoduodenal fistula. Despite intense medical efforts for several months the patient did not fully recover and treatment was limited to palliative care. In light of the substantial mortality associated with this condition, computed tomography imaging should be performed in case of doubt to prevent delayed diagnosis.

Silenced ZNF154 Is Associated with Longer Survival in Resectable Pancreatic Cancer.

Pancreatic cancer has become the third leading cause of cancer-related death in the Western world despite advances in therapy of other cancerous lesions. Late diagnosis due to a lack of symptoms during early disease allows metastatic spread of the tumor. Most patients are considered incurable because of metastasized disease. On a cellular level, pancreatic cancer proves to be rather resistant to chemotherapy. Hence, early detection and new therapeutic targets might improve outcomes. The detection of DNA promoter hypermethylation has been described as a method to identify putative genes of interest in cancer entities. These genes might serve as either biomarkers or might lead to a better understanding of the molecular mechanisms involved. We checked tumor specimens from 80 patients who had undergone pancreatic resection for promoter hypermethylation of the zinc finger protein ZNF154. Then, we further characterized the effects of ZNF154 on cell viability and gene expression by in vitro experiments. We found a significant association between ZNF154 hypermethylation and better survival in patients with resectable pancreatic cancer. Moreover, we suspect that the cell growth suppressor SLFN5 might be linked to a silenced ZNF154 in pancreatic cancer.

Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor.

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth and common local recurrence, with rare distant metastases. PATIENTS AND METHODS: We present a retrospective study of nineteen patients who were diagnosed with DFSP and operated at our institution in > 10-year period. We examined the clinicopathological parameters with special emphasis on the margin status regarding the clinical outcome and the follow-up. RESULTS: A total of eight cases underwent re-excision at our institution following primary excision or incisional biopsy performed at a different institution. Seven cases received excision after incisional biopsy at our institution. Four patients developed recurrent disease following primary excision with histological R0 margins at other institutions and received re-excision at our institution. All excisions at our institution resulted in R0 margins with no recurrence recorded at last follow-up (6 to 175; mean 84 months). The mean margin for those who received resection at our institution was 1.67 cm. Negative margins upon primary excision were achieved using a mean margin width of 2.04 cm. Most common tumor localization was the trunk (10 cases). CONCLUSION: Awareness of this rare entity is important for a prompt diagnosis and a proper management of the disease. The greatest clinical challenge in the management of DFSP is achieving local control. Complete excision of the tumor with surgical margin widths of at least 2 cm is recommended.