Von Hippel Lindau Syndrome

contributed by: Dr. Mohd Ilyas
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Findings
Spinal cord shows diffusely increased signal intensity in cervical; dorsal part, extending upto the cervico-medullary junction and the pons, on T2/STIR images. Multiple T1/T2, hypointense lesions are seen within the spinal cord; left part of inferior cerebellar vermis.

On post-contrast images, there is presence of multiple hyper-enhancing well-defined lesions within the dorsal spinal cord, largest at D7 level, measuring 14x8mm and hyperenhancing inferior cerebellar vermian lesion, measuring 15x13mm.

Screening of brain revealed in situ VP shunt on right side and hyper-enhancing lesion within the posterior chamber of right eye (Retinal blastoma). No hydrocephalus seen in the current scan.

Diffuse IV disc bulge with right paracentral protrusion at L4-L5 level causing narrowing of rigth lateral recess and neural foramina with compression of right traversing & exiting nerve roots at this level.

Diffuse IV disc bulge with left paracentral protrusion at L5-S1 level causing moderate narrowing of left lateral recess and neural foramina with compression of left traversing and exiting nerve roots at this level.
Diagnosis
Von-Hippel-Lindau Syndrome with Myelomalacia with Degenerative Lumbar Spondylosis.
Dr. Mohd Ilyas MD is a Consultant Radiologist from with experience of 3-5 years post-graduated from Government Medical College Jammu. Specialized in Reporting of X-RAYS , CT-SCAN , MRI , MAMMOGRAM cases with expertise in Neuro-imaging , Spine-imaging , Joints , Trauma , Stroke-imaging
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