von Hippel-Lindau Disease Follow-up
- Author: Arun C Gulani, MD; Chief Editor: Hampton Roy Sr, MD more...
Further Outpatient Care
- Long-term follow-up care is important to detect any additional lesions.
Deterrence/Prevention
- Lifelong follow-up care is indicated when the family history is positive, since systemic manifestation can occur years after retinal hemangiomas are noted.
Complications
Visual complications of retinal angiomas include the following:
- Macular exudation
- Vitreous hemorrhage
- Nerve damage
- Iatrogenic complications from argon laser photocoagulation, cryotherapy, or irradiation
Prognosis
- Early diagnosis is essential, since angiomas have a poor prognosis unless they are treated.
- Of those patients with retinal angiomas, 25% have associated cerebellar hemangioblastomas.[10]
Maher ER, Bentley E, Yates JR, et al. Mapping of von Hippel-Lindau disease to chromosome 3p confirmed by genetic linkage analysis. J Neurol Sci. Dec 1990;100(1-2):27-30. [Medline].
Huson SM, Harper PS, Hourihan MD, et al. Cerebellar haemangioblastoma and von Hippel-Lindau disease. Brain. Dec 1986;109 (Pt 6):1297-310. [Medline].
Gass JD, Braunstein R. Sessile and exophytic capillary angiomas of the juxtapapillary retina and optic nerve head. Arch Ophthalmol. Oct 1980;98(10):1790-7. [Medline].
Champion KJ, Guinea M, Dammai V, et al. Endothelial function of von Hippel-Lindau tumor suppressor gene: control of fibroblast growth factor receptor signaling. Cancer Res. Jun 15 2008;68(12):4649-57. [Medline].
Gulani AC. Ocular color Doppler. 1995;1-5.
Gulani AC. Ocular color Doppler usage aids in diagnosis and evaluation of pathologies. Ocular Surgery News. 1998;16:54-5.
Gulani AC, Morparia H, Bhatti SS, et al. Colour Doppler sonography: a new investigative modality for intraocular space-occupying lesions. Eye. 1994;8 (Pt 3):307-10. [Medline].
Moore AJ. Ophthalmologic screening of von Hippel Lindau disease. Eye. 1992;5:90-2.
Wong WT, Chew EY. Ocular von Hippel-Lindau disease: clinical update and emerging treatments. Curr Opin Ophthalmol. May 2008;19(3):213-7. [Medline].
Pavesi G, Feletti A, Berlucchi S, et al. Neurosurgical treatment of von Hippel-Lindau-associated hemangioblastomas: benefits, risks and outcome. J Neurosurg Sci. Jun 2008;52(2):29-36. [Medline].

