Von Hippel Lindau Disease:
Genetic, Clinical and Imaging Features

Peter L. Choyke, M.D., Gladys M. Glenn, M.D., Ph.D., McClellan M. Walther, M.D., Nicholas J. Patronas, M.D., W. Marston Linehan, M.D., Berton Zbar, M.D.

Radiology (March) 146:629-642,1995

Appendix 2

Test NIH CAMBRIDGE, U.K. HAWAII
Urinary Catechol From age 2 every 1-2 years Every year At least once or if BP is elevated
Ophthalmoscopy From infancy yearly From age 5 yearly From age 6 yearly
Fluorescein Angioscopy Not routine Routine from age 10 yearly Not routine
Enhanced MRI of Brain and Spine From age 11 every 2 years;**
After age 60 every 3-5 years
 From mid teens every 3 yrs to age 50 then every 5 years Begin after age 20 at least once per decade
Abdominal CT/US From age 11;
US yearly
From age 20;
CT yearly*** or every other year
(US as needed to evaluate CT findings)		 From in mid teens; US every year, CT every 3 years Begin age 15-20 then US every year

*Annually at onset of puberty and during pregnancy
**Examine middle ear for symptoms of hearing loss, tinnitus or vertigo
***After age 60 and still no evidence of VHL, CT can be performed every two years and MRI of CNS

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