Decreased aortic growth and middle aortic syndrome in patients with neuroblastoma after radiation therapy

Background: Long-term CT follow-up studies are required in pediatric patients who have received intraoperative radiation therapy (IORT) and external beam radiation therapy (EBRT) to assess vascular toxicities and to determine the exact complication rate. Objective: To analyze with CT the effects of radiation therapy (RT) on the growth of the aorta in neuroblastoma patients.Materials and methods Abdominal CT scans of 31 patients with intraabdominal neuroblastoma (stage II–IV), treated with RT (20 IORT±EBRT, 11 EBRT alone), were analyzed retrospectively. The diameter of the abdominal aorta was measured before and after RT. These data were compared to normal and predicted normal aortic diameters of children, according to the model of Fitzgerald, Donaldson and Poznanski (aortic diameter in centimeters = 0.844 + 0.0599 × age in years), and to the diameters of a control group of children who had not undergone RT. Statistical analyses for the primary aims were performed using the chi-squared test, t-test, Mann-Whitney test, nonparametric Wilcoxon matched-pairs test and analysis of variance for repeated measures. Clinical files and imaging studies were evaluated for signs of late vascular complications of neuroblastoma patients who had received RT. Results: The mean diameter before and after RT and the growth of the aorta were significantly lower than expected in patients with neuroblastoma (P<0.05 for each) and when compared to the growth in a control group with normal and nonirradiated aortas. Among the patients who had received RT, there was no difference due to the type of RT. Seven patients from the IORT±EBRT group developed vascular complications, which included hypertension (five), middle aortic syndrome (two), death due to mesenteric ischemia (one) and critical aortic stenosis, which required aortic bypass surgery (two).Conclusion Patients with neuroblastoma who had received RT showed impaired growth of the abdominal aorta. Significant long-term vascular complications occurred in seven patients who received IORT±EBRT. Thus, CT evaluation of patients with neuroblastoma who receive RT should include not only reports of changes in tumor extension, but also documentation of perfusion, and the size and growth of the aorta and its branches over time

Presenting Baseline Coagulation of Infra Renal Ruptured Abdominal Aortic Aneurysm: A Systematic Review and Pooled Analysis

Background The incidence of coagulopathy in patients presenting with rAAA is not clear. The lack of high-quality evidence has led to various speculations, reliance on anecdotal experience, and suggestions about their appropriate haemostatic resuscitation. The aim of this systematic review is to establish the baseline coagulation status of infra renal ruptured abdominal aortic aneurysms (rAAA) against defined standards and definitions. Methods An electronic search of literature in Medline, CINHAL, Scopus Embase, and Cochrane library was performed in accordance with the PRISMA guidelines. Quality assessment of articles was performed using the Oxford critical appraisal skills programme (CASP) and their recommendation for practice was examined through National Institute for Health and Care Excellence (NICE). Information on platelet count, international normalisation ratio (INR), activated partial prothrombin time (aPTT), prothrombin time (PT) fibrinogen and D-dimer was extracted, and pooled analysis was performed in accordance with the definition of coagulopathy and its subtypes. Pooled prevalence of coagulopathies and 95% CI were estimated with a variance weighted random effects model. Results Seven studies, comprising 461 patients were included in this systematic review. Overall weighted prevalence of coagulopathy was 12.3% (95% CI 10.7-13.9), 11.7% for INR (95% CI 1-31.6), 10.1% for platelet count (95% CI 1-26.8), and 11.1% for aPTT (95% CI 0.78-31). Fibrinogen serum concentration level was normal in 97%, and 46.2% (n = 55) of patients had elevated D-dimer. Only 6% of the entire population demonstrated significant coagulopathy. DIC was noted in 2.4% of the population. Conclusion This first systematic review of literature on baseline coagulation of rAAAs suggests that the majority of these patients do not present with coagulopathy and only a minor proportion of patients present with significant coagulopathy