Treatment of the Budd-Chiari syndrome with percutaneous transluminal angioplasty. Case report and review of the literature.

A patient with the Budd-Chiari syndrome due to membranous obstruction of the right hepatic vein and long segmental obstruction of the inferior vena cava who was successfully treated with percutaneous transluminal angioplasty is described. Review of the literature revealed 14 prior cases of balloon dilatation of the hepatic venous system: 10 of the hepatic portion of the inferior vena cava, three of the right hepatic vein, and one of the left hepatic vein. Follow-up ranged from six months to 37 months. No serious complications were reported. All attempts at dilatation were successful, but reocclusion occurred in six patients, three of whom had occlusion of the hepatic vein. All but one patient with reocclusion, however, underwent repeated angioplasty, which was successful in all cases attempted. Two patients who had repeated angioplasty required no further therapy, but two patients required a total of three angioplasties and two patients required four angioplasties. Successful angioplasty was accompanied by resolution of clinical symptoms in all patients described. It is concluded that percutaneous transluminal angioplasty is a safe and effective mode of therapy in the management of the Budd-Chiari syndrome due to membranous obstruction of the hepatic portion of the inferior vena cava or the hepatic veins.

Intracranial extension and bone destruction in orbital pseudotumor.

Three cases of surgically proved pseudotumor of the orbital apex with intracranial extension occurred. All demonstrated bony destruction. Varying degrees of ophthalmoplegia and visual loss were present in all three. Previous computed tomographic descriptions of the patterns of orbital pseudotumor have not included bone destruction. Further, intracranial extension has been reported in only one patient. These three cases are reported to emphasize the fact that while these manifestations may be rare, it is appropriate to include orbital pseudotumor in the differential diagnosis of orbital apex lesions that are associated with both bone destruction and/or intracranial extension.

Spinal cord sarcoidosis. Case report.

A case of spinal cord sarcoidosis is presented. Diagnosis was established from a biopsy of the hilar lymph node. The patient was successfully treated with corticosteroid drugs. The literature is reviewed and the deleterious effect of surgery is stressed.

The open ring. A new imaging sign in demyelinating disease.

Because demyelinating disease of the brain occasionally presents with large ring-enhancing lesions on computed tomography (CT) scans and magnetic resonance images (MRIs), the authors sought to determine whether the ring pattern differed from that found in other common brain lesions with ring enhancement. Published MRI and CT scans of patients with adrenoleukodystrophy (23), and multiple sclerosis or similar demyelinating disorders (21), as well as a variety of tumors (44) and infections (44) matched to the demyelinating lesions by year of publication, in which ring enhancement was evident, were photographed. Photographs without diagnostic identification were presented randomly to two independent observers. The observers rated the contrast enhancement pattern as (1) open ring, with enhancement in the border of the lesion abutting the white matter; (2) closed ring; or (3) uncertain. For all diagnostically certain cases (n = 112), inter-rater agreement was excellent (kappa = 0.75). As an average of the two reviewers, scans for 11 of 132 cases were read as uncertain; 89% of adrenoleukodystrophy cases, 41% of the multiple sclerosis cases, 3% of tumors, and 9% of infections were classified as having the open-ring pattern. Overall, 66% of demyelinating lesions had an open-ring pattern compared with 7% of the non-demyelinating lesions (chi2 = 41.2, p < 0.0001). An open-ring pattern of enhancement is more likely to be associated with demyelinating lesions than with nondemyelinating lesions.

Flexural planar xanthomas: report of four cases.

Four patients with primary hyperlipoproteinemia, Frederickson's type IIb, were observed to have symmetrical planar xanthomas affecting flexures: finger webs, antecubital and popliteal fossae, axillae, and intergluteal cleft. Other types of xanthomas were also present and serum lipid electrophoresis showed type IIb pattern. We draw attention to the peculiar pattern of symmetrical planar xanthomatosis hitherto described only with type III disease and some forms of normolipemic xanthomatoses.

Alagille syndrome. A case report.

A 5-year-old physically and mentally retarded female child born of non-consanguineous parents, who had had disseminated skin lesions for 4 1/2 years, is presented. She had persistent neonatal jaundice associated with clay-coloured stools and generalized pruritus which receded by the age of 2 years. Examination revealed characteristic facies, moderate hepatosplenomegaly, cardiac murmur and widespread smooth yellow papules and nodules on ears, trunk, bony prominences and palms. Ophthalmic examination revealed corneal opacities. Liver function tests and lipidogram were abnormal. A diagnosis of Watson-Alagille Syndrome was made on the basis of characteristic facies, xanthomatosis and cholestatic jaundice.

Neonatal middle cerebral artery infarction: association with elevated maternal anticardiolipin antibodies.

A full-term neonate was born to a 41-year-old woman via elective primary cesarean section for frank breech presentation after a 41-week pregnancy. Starting at 6 hr of age the infant presented with multiple episodes of apnea and cyanosis, in association with moderate hypotonia, subsequently requiring assisted ventilatory support for 2 days. Computerized axial tomography of the brain revealed infarction in the distribution of the left middle cerebral artery. Magnetic resonance imaging of the brain showed a left middle cerebral artery territory infarct and also a small right posterior-temporal infarct. Magnetic resonance angiography of the head and neck was normal, however, inferring that the vascular infarction was peripheral in location. Maternal anticardiolipin antibodies were elevated. This is only the fifth reported case of cerebral infarction in a newborn in association with elevated maternal anticardiolipin antibodies.