Marfan syndrome is an autosomal dominant connective tissue disorder that affects multiple systems, including the skeletal, ligamentous, oculofacial, pulmonary, abdominal, neurological, and cardiovascular systems. Cardiovascular complications, which involve the aorta and aortic valve, contribute most significantly to patient morbidity and mortality. A literature review was conducted on pathophysiology of the disease and recommendations for early diagnosis and treatment. Diagnosis largely relies on clinical features and a thorough history. Echocardiogram is used for monitoring aortic abnormalities and disease progression. Aortic valve-sparing surgery is indicated in any valvular abnormality and in patients with a murmur. Aortic root replacement is indicated prophylactically in women who want to give birth with diameters greater than 40 mm, anyone with a diameter greater than 50 mm, and progressive dilatation of greater than 5 mm per year. Medical management involves antihypertensive therapy. It is imperative for all health care providers to understand the clinical features, progression, and management of Marfan syndrome to appropriately care for their patients. Ensuring regular follow-up and adherence to medical and surgical prophylaxis is essential to patient well-being.

Background:The purpose of this study was to evaluate the frequency of and outcome in patients with isolated posterior fossa uptake on cerebral perfusion scan. Methods: A retrospective review was undertaken of electronic medical records over 6 years from 713 consecutive patients undergoing technetium-99m ethyl cysteine dimer cerebral perfusion scans to identify isolated posterior fossa activity. Results: Of the 13 patients with isolated posterior fossa activity, 10 deteriorated to death and three had life support withdrawn. They survived an average of 0.24 day ± 0.27 standard deviation (SD) days after scanning and an average of 1.03 ± 0.33 SD days on life support. Conclusion: Unilateral or bilateral isolated posterior fossa technetium-99m ethyl cysteine dimer activity indicates imminent or impending brain death and a poor prognosis.