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Surgical and intensive care patients are at a heightened risk for arterial embolization due to pre-existing conditions such as age, hypercoagulability, cardiac abnormalities and atherosclerotic disease. Most arterial emboli are clots that originate in the heart and travel to distant vascular beds where they cause arterial occlusion, ischemia, and potentially infarction. Other emboli form on the surface of eroded arterial plaque or within its lipid core. Thromboemboli are large clots that dislodge from the surface of athesclerotic lesions and occlude distal arteries causing immediate ischemia. Atheroemboli, which originate from fracturing the lipid core tend to cause a process of organ dysfunction and systemic inflammation, termed cholesterol embolization syndrome. The presentation of arterial emboli depends on the arterial bed that is affected. The most common manifestations are strokes and acute lower limb ischemia. Less frequently, emboli target the upper extremities, mesenteric or renal arteries. Treatment involves rapid diagnosis, which may be aided by precise imaging studies and restoration of blood flow. The type of emboli, duration of presentation, and organ system affected determines the treatment course. Long-term therapy includes supportive medical care, identification of the source of embolism and prevention of additional emboli. Patients who experienced arterial embolism as a result of clots formed in the heart should be anticoagulated. Arterial emboli from atherosclerotic disease of the aorta or other large arteries should prompt treatment to reduce the risk for atherosclerotic progression, such as anti-platelet therapy and the use of statin drugs. The use of anticoagulation and surgical intervention to reduce the risk of arterial embolization from atherosclerotic lesions is still being studied.

During a study of hereditary motor and sensory neuropathy‐Lom in Bulgaria, a previously unrecognized neurological disorder was encountered, mainly in Wallachian Gypsies, who represent a relatively recent genetic isolate. The disorder has been termed the congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome to emphasize its salient features. Fifty individuals from 19 extended pedigrees were identified and examined clinically and electrophysiologically. At least 1 patient from each family was admitted to the hospital in Sofia for full investigation. Pedigree analysis indicates autosomal recessive inheritance. The disorder is recognized in infancy by the presence of congenital cataracts and microcorneas. A predominantly motor neuropathy beginning in the lower limbs and later affecting the upper limbs develops during childhood and leads to severe disability by the third decade. Associated neurological features are a moderate nonprogressive cognitive deficit in most affected individuals together with pyramidal signs and mild chorea in some. Accompanying nonneurological features include short stature, characteristic facial dysmorphism, and hypogonadotrophic hypogonadism. Nerve conduction studies suggest a hypomyelinating/demyelinating neuropathy, confirmed by nerve biopsy. The CCFDN syndrome is thus a pleomorphic autosomal recessive disorder displaying a combination of neurological and nonneurological features. Ann Neurol 1999;45:742–750

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HE literature on anesthetic concerns and perioperative care of patients with mediastinal masses has focused almost exclusively on anterior mediastinal masses. Posterior mediastinal masses traditionally have been suggested to carry a low risk of anesthetic implications. 1 We present the case of a patient with a posterior mediastinal mass who experienced hemodynamic and respiratory decompensation upon induction of general anesthesia and required urgent transition to cardiopulmonary bypass (CPB).Our case illustrates the importance of real-time imaging provided by transesophageal echocardiography (TEE) in explaining the etiology of intractable hemodynamic instability and a new finding of significant pericardial and left pleural effusion not seen on preoperative imaging. TEE made clear the urgent need to initiate CPB because it revealed a near total compression of the left atrium, which was obstructing delivery of volume to the left ventricle. In addition, this case demonstrates the inadequacy of "stand-by" CPB and the need for every institution to establish an interdisciplinary team to develop, before surgery, a formal plan for the perioperative care of patients with mediastinal masses. CASE REPORTA 45-yr-old, previously healthy woman was transferred to our facility for further management of her mediastinal mass. Six weeks before this admission, she had presented to her primary care provider with shortness of breath, cough, and intermittent low-grade fever. At that time, she received a clinical diagnosis of pneumonia and was prescribed a course of antibiotics and corticosteroids. Upon follow-up, her presenting symptoms had not improved, and she was experiencing dysphagia and right-side chest pain. A chest radiogram showed a large mediastinal mass.Subsequent computed tomography scanning ( fig. 1A and 1B) showed a 17-cm ϫ 15-cm ϫ 13-cm posterior mediastinal mass extending from the lateral right hemidiaphragm to the left upper chest wall. The scan showed that the mass displaced the trachea, mainstem bronchi, and right lung and widely splayed the tracheal carina. External compression of the airway was minimal, and at no point was the lumen caliber of the trachea or mainstem bronchi diminished significantly. A significant right pleural effusion was present, and a small amount of fluid was noted posterior to the left lung. The heart was shifted to the left, and encroachment of the left atrium by the mass could be seen. No pericardial effusion was present. Anatomic distortion and stretching of the pulmonary arteries and veins, the superior vena cava, and the proximal aorta were noted, although no significant compression of these vessels was present. There was almost complete compression of the esophagus in the thorax. No encasement or tissue invasion of thoracic structures by the mass was apparent.Upon admission to our institution, the patient was hemodynamically stable and maintaining adequate oxygen saturation on 2 l/min O 2 by nasal cannula. In an upright, 90-degree-angle sitting position, where she reported she was mos...