Table 2.
Suggested surveillance modalities and frequency for neurologic manifestations of von Hippel-Lindau disease (Adapted from Choyke et al.54)
| Test | Lesions Evaluated | Start age (frequency) |
|---|---|---|
| Opthalmoscopy | Retinal hemangioblastoma | Infancy (yearly) |
| Magnetic resonance imaging of craniospinal axis* | Craniospinal hemangioblastoma | 11 years of age (yearly) |
| Computed tomography and magnetic resonance imaging of internal auditory canals* | Endolymphatic sac tumor | Onset of symptoms (hearing loss, tinnitus, vertigo, or unexplained balance difficulties) |
| Audiologic function tests | Endolymphatic sac tumor | When clinically indicated |
| Ultrasound of abdomen | Renal carcinoma/cyst, pancreatic neuroendocrine tumor/cyst | 8 years of age (yearly; magnetic resonance imaging as clinically indicated) |
| Computed tomography of abdomen* | Renal carcinoma/cyst, pancreatic neuroendocrine tumors/cysts | 18 years of age or earlier if clinically indicated (yearly) |
| Plasma or 24-hour urinary catecholamines and metanephrines | Pheochromocytoma | 2 years of age (yearly and when blood pressure is elevated) |
*
Imaging typically performed with and without intravenous contrast