Twenty-six patients undergoing long-term hemodialysis who had hand and wrist abnormalities were examined for the presence of concurrent spinal disease. Six patients (23%) were found to have spinal disease on radiographs. In four, the findings resembled those of a destructive spondyloarthropathy, and in two, a less destructive erosive arthritis. A review of the spinal radiographs of 31 patients with normal hands and wrists revealed no such abnormalities. Postmortem examinations of two patients with spondyloarthropathy demonstrated extensive deposition of beta-2 microglobulin, an amyloid-like substance. The authors conclude that the disease is similar in both areas and represents a skeletal response to chronic renal failure and long-term hemodialysis. The disease may occur more frequently with an increased duration of hemodialysis, in one area of the skeleton if another area is already affected, and is probably not infectious if multiple areas are involved.

Lückenschädel, scalloping of the posterior surface of the petrous pyramids, falx hypoplasia, falx fenestration, and tentorial hypoplasia with wide incisura and tiny posterior fossa are readily identified by computed tomography (CT) in patients with Chiari II malformation. Enlargement of the foramen magnum may be appreciated on axial section CT in some cases. None of these findings is pathognomonic and each may be observed in some patients with other conditions. In the aggregate, however, these findings strongly suggest the presence of Chiari II malformation.

We found that a PSV of greater than 200 cm/s was the best criterion for the diagnosis of IMA stenosis. The sensitivity, specificity, PPV, NPV, and accuracy for the PSV were 90%, 97%, 90%, 97%, and 95%, respectively.

We describe a patient with idiopathic arterial calcification of infancy and the following unusual features of the disease: (1) prolonged survival until age 11 years, (2) discordance between the extent of vascular calcification and clinical manifestations of arterial luminal occlusion, (3) a large area of myocardial calcification, (4) symptoms of cerebral-vascular insufficiency, and (5) spontaneous resolution of hypertension.