Table 1.

Follow-up evaluations recommended for patients with VHL disease.^*

Follow-up evaluation	Initial age	Frequency	Comments
Retinal assessment	< 1 year	Every 6-12 months	Annually, before age 30
Anamnesis and physical examination by a specialist	1 year	Yearly
Blood pressure and heart rate	2 years	Yearly
Determination of metanephrine levels	5 years	Yearly	Measurement in plasma preferred, but fractionated metanephrines may be measured in 24-h urine samples
MRI of the neuraxis (brain and spinal cord)	11 years	Every 2 years	Performed with and without contrast (no contrast during pregnancy); can be coordinated with MRI of the abdomen; thin slices in the posterior fossa and temporal bone; single MRI of the inner ear canal at 15 years of age
Audiometry	11 years	Every 2 years
MRI of the abdomen	15 years	Every 2 years	Performed with and without contrast (no contrast during pregnancy); assess kidneys, pancreas, and adrenal glands; can be coordinated with MRI of the neuraxis

^*Adapted from the VHL Family Alliance - VHL handbook⁽⁹⁾.