Table 10.4.

VHL surveillance guidelines*

Period	Surveillance test	Frequency	NIH	DCG	VHLFA
0–4 year	Ophthalmoscopy	Annual	+	+	+
0–4 years	Physical exam	Annual	+	+	+
2 years onwards	Plasma or urinary metanephrines	Annual	+	5 years onwards	8 years onwards or earlier if needed
5–15 years	Ophthalmoscopy	Annual	+	+	+
5–15 years	Physical exam	Annual	+	+	+
5–14 years	Hearing exam	Annual	When clinically indicated	+	Audiology assessment once. Annual assessments if any hearing loss, tinnitus or vertigo is found
8 years onwards	Ultrasound of abdomen	Annual	+	Once between 8 and 14 years of age	Annually from 8 years or earlier if clinically indicated
11 years onwards	MRI of craniospinal axis	Annual	+	MRI once between 8 and 14 years of age
15 years onwards	Ophthalmoscopy	Annual	+	+	+
15 years onwards	Neurological exam	Annual		+
15 years onwards	MRI of craniospinal axis including inner ears	Every 2 years	Annual	+	+
15 years onwards	Ultrasound or MRI of abdomen	Annual	Ultrasound annually, MRI as needed	+	Every 2 years
18 years onwards	CT abdomen	Annual	+
15 years onwards	Plasma metanephrines, normetanephrines and chromogranin A	Annual	Plasma or urinary catecholamines annually	+	+
15 years onwards	Hearing exam	Annual	When clinically indicated	+	Every 2 years
Pregnancy	MRI craniospinal axis without contrast	Once at fourth month of pregnancy			+

^*+, recommendation is substantially similar to the guideline by the organization.

NIH, US National Institutes of Health; DCG, Danish Coordination Group for VHL; VHLFA, VHL Family Alliance; MRI, magnetic resonance imaging.