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Table 5. Practice Guidelines for Surveillance of von Hippel-Lindau Syndrome (VHL)

Examination/Test  Condition Screened For Starting Age/Frequencya 
OphthalmoscopyRetinal hemangioblastomaFrom infancy; every 6 to 12 mo
Fluorescein angioscopyRetinal hemangioblastomaIf needed (not routinely performed)
Plasma or 24-hour urinary catecholamines and metanephrinesPheochromocytomaFrom age 2 y; yearly and as clinically indicated when blood pressure is elevated
Enhanced MRI of brain/spinebCNS and peripheral hemangioblastomaFrom age 11 y; every 1 to 2 y and if symptoms appear
CT of abdomen with and without contrast (substitute MRI every other year)Renal, pancreatic, and adrenal neoplasms and cystsFrom age 18 y, earlier if indicated; yearly; alternate CT and MRI (reduces radiation)
Ultrasound of abdomenRenal, pancreatic, and adrenal neoplasms and cystsYearly from age 8 to 18 y, earlier if indicated; MRI as clinically indicated
MRI and CT of IACs, audiology, neurologyEndolymphatic sac tumorAny age for hearing loss, tinnitus, or vertigo

CNS = central nervous system; CT = computerized tomography; IACs = internal auditory canals; MRI = magnetic resonance imaging.
aFrequencies of exams or tests may be increased at organ sites of VHL lesions being monitored.
bBrain MRIs may be used to examine areas of the IACs for signs of endolymphatic sac tumors (ELSTs). If signs or symptoms of ELSTs are present, examine IACs by CT and MRI.
Adapted from Pithukpakorn and Glenn [6]; Choyke et al. [4]; and Lonser et al.[5]

References

  1. Choyke PL, Glenn GM, Walther MM, et al.: von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology 194 (3): 629-42, 1995.  [PUBMED Abstract]

  2. Lonser RR, Glenn GM, Walther M, et al.: von Hippel-Lindau disease. Lancet 361 (9374): 2059-67, 2003.  [PUBMED Abstract]

  3. Pithukpakorn M, Glenn G: von Hippel-Lindau syndrome. Community Oncology 1 (4): 232-43, 2004.