Table 1. 2017 screening guidelines from the VHL Alliance.

VHL, von Hippel-Lindau; [50]

Age  (years)	Screening Recommendations
1 to 4	Begin yearly eye examinations with the indirect ophthalmoscope to rule out retinal lesions. Begin annual neurological screening for nystagmus, strabismus, white pupil, vision and hearing impairment, and blood pressure abnormalities.
5 to 15	Continue the routine screening described in the previous age group. Begin lifelong biochemical screening for fractionated metanephrines, including normetanephrine and plasma free metanephrines at age 5. Begin audiology assessments every 2 to 3 years or yearly in the presence of hearing impairment, tinnitus, or vertigo.
16 and older	Continue the routine screening described in the previous age groups. Begin yearly quality contrast and noncontrast ultrasounds to rule out renal, pancreatic and adrenal tumors. Begin MRI scans (no less than a 1.5T MRI performed with and without contrast) every 2 to 3 years of the brain, cervical, thoracic, and lumbar spine with visualization of the posterior fossa and inner ear/petrous temporal bone to rule out endolymphatic sac tumors and neuraxis hemangioblastomas.