RESUMO
Objective To analyze the imaging characteristics of Von Hippel-Lindau Syndrome (VHL)typeⅠ.Methods 12 patients diag-nosed as VHL type Ⅰ were collected and all the patients had underwent 3.0T MRI and 64-slice spiral CT plain and enhanced scan. Imaging data was analyzed by two senior radiologists to summarize its imaging featueres.Results Multiple hemangioblastomas (HGBLs)in cerebellum,spinal cord or brainstem were seen in all the patients.Flow void effect with varied degree was seen in the lesions.Dilated feeding and drainage blood vessels around lesions was seen in 6 cases.Multiple kidney and pancreas cysts were seen in all the patients.There were 4 cases with syringomyelia,7 cases with ocular retinal angioma,5 cases with renal cell carcinoma,2 cases with ground-glass-like lesions and micronodulars in lung.Conclusion VHL type Ⅰ has complex imaging performance.Ima-ging examination is important for early detection of VHL disease.