ABSTRACT
The Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. Diagnosis is usually made in childhood following the finding of liver neoplasms combined with slightly increased liver enzymes. There is a female predominance. Further findings are malformations of the heart, skeletal system and kidneys. Short-term follow-up of the liver lesions is recommended. Progressive disease can be treated by partial liver resection or transplantation. Diagnosis of the Abernethy malformation is based on various imaging modalities. We report the MRI and MRA findings of an Abernethy malformation in a 35-year-old woman with multiple liver cell adenomas and review the 32 previously published cases.
Subject(s)
Adenoma/diagnosis , Arteriovenous Fistula/complications , Arteriovenous Fistula/diagnosis , Liver Neoplasms/diagnosis , Magnetic Resonance Imaging , Portal Vein/abnormalities , Vena Cava, Inferior/abnormalities , Adult , Female , Humans , Liver Neoplasms/etiology , Portal Vein/pathology , Vena Cava, Inferior/pathologyABSTRACT
Several radiologic findings point toward the lentiform nucleus as a possible site of lesion in primary dystonia. Histologic examinations, however, have shown inconsistent results. 1H-magnetic resonance spectroscopy (MRS) has proved helpful to assess neuronal degeneration in a variety of basal ganglia disorders. MRS data of dystonia patients are, however, lacking so far. 1H-MRS centered on the lentiform nuclei was performed in 14 patients with primary focal hand dystonia and in 12 healthy control subjects using a 1.5-T MR imager. No statistically significant differences of N-acetylaspartate/creatine and lactate/creatine ratios were found between patients and control subjects. Based on these data, the authors found no evidence that primary focal dystonia was associated with a conspicuous loss of lentiform nucleus neurons or a marked disturbance of the aerobic metabolism, although minor abnormalities cannot be excluded because of the possibly limited sensitivity of the method.
