Occurrence of neuroblastoma and asymmetric crying facies." Case report and review of the literature

responsible for thromboembolic phenomena in SLE. Histologic studies of patients with lupus with extremity, intestinal, or hepatic venous thrombosis have revealed an underlying endophlebitis, presumably mediated by immune complex deposition. 9-~ Specific antiendothelial cell antibodies have recently been discovered in patients with lupus2 ~ We believe that immune-mediated venulitis may be responsible for the cerebral venous sinus thrombosis in our patient. The treatment of this patient posed many questions. We elected not to use anticoagulation because of fear of a hemorrhagic intracerebral infarction. Ligation of the thrombosed internal jugular vein was another option considered to prevent intracardiac extension and subsequent pulmonary embolization. However, because pulmonary embolization is a rare complication of internal jugular vein thrombosis, the surgical literature recommends a conservative approach. ~2 Additionally, although ligation may prevent embolization, it compounds the problem of increased intracranial pressure. We decided that ligation of the internal jugular vein in our patient would be done only if pulmonary embolism developed. We elected conservative management with steroids and serial lumbar punctures. When these measures failed and concern for visual loss heightened, lumboperitoneal shunting was performed to provide permanent relief from increased intracranial pressure. In the past, it has been speculated that certain cases of pseudotumor cerebri may have been caused by unrecognized cerebral dural venous sinus obstruction. 6 This case demonstrates that internal jugular vein thrombosis may be a previously unsuspected accompaniment of these events.