von Hippel-Lindau Disease Treatment & Management

  • Author: Arun C Gulani, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Aug 2, 2011
 

Surgical Care

von Hippel-Lindau disease is usually a progressive disease. Therapy should begin as soon as the diagnosis is made. Surgical treatment may consist of argon laser photocoagulation, cryotherapy, fluid drainage, scleral buckling, penetrating diathermy, vitreous surgery, or endodiathermy.

Argon laser photocoagulation is effective in treating angiomatosis retinae. Treatment consists of a large spot size, with low-intensity, long-duration burns directed at the angioma. Repeated laser treatment is required, except for small tumors. Obliteration of the tumor is confirmed by clinical observation and fluorescein angiography. If the tumor turns yellowish, photocoagulation becomes difficult because of the poor penetration of laser light.

Cryotherapy, a repetitive freeze/thaw technique, may be used to treat anterior angiomas and larger posterior angiomas. To minimize the risk of hemorrhage, no more than 2 or 3 freeze/thaw cycles should be used per session. Multiple sessions generally are needed to arrest tumors. Resolution of the macula edema and improved visual acuity are common results of tumor eradication by cryotherapy.

Scleral buckle and fluid drainage methods may be needed to treat larger tumors, tumors associated with retinal detachment, angiomas resistant to cryotherapy, or tumors involving subretinal exudation. Large tumors may develop surface membranes and vitreous traction, leading to vitreous hemorrhage or rhegmatogenous retinal detachment. Such complications may require treatment by vitreous surgery, endodiathermy, or scleral buckling techniques.

Penetrating diathermy under a lamellar scleral bed is an effective treatment of larger angiomas.

Endodiathermy may be used, instead of vitreous surgical techniques or scleral buckling procedures, to treat vitreous hemorrhage or retinal detachment.

Radiation, applied in various forms, is another field among future directions in the care for this incessant pathology.

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Consultations

The ophthalmologist has a critical role to play in the management of von Hippel-Lindau disease.

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Contributor Information and Disclosures
Author

Arun C Gulani, MD  Director, Gulani Vision Institute

Arun C Gulani, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, and International Society of Refractive Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Bartiss, OD, MD  Medical Director, Ophthalmology, Family Eye Care of the Carolinas

Michael J Bartiss, OD, MD is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, and North Carolina Medical Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. 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].

  2. Huson SM, Harper PS, Hourihan MD, et al. Cerebellar haemangioblastoma and von Hippel-Lindau disease. Brain. Dec 1986;109 (Pt 6):1297-310. [Medline].

  3. 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].

  4. 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].

  5. Gulani AC. Ocular color Doppler. 1995;1-5.

  6. Gulani AC. Ocular color Doppler usage aids in diagnosis and evaluation of pathologies. Ocular Surgery News. 1998;16:54-5.

  7. 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].

  8. Moore AJ. Ophthalmologic screening of von Hippel Lindau disease. Eye. 1992;5:90-2.

  9. Wong WT, Chew EY. Ocular von Hippel-Lindau disease: clinical update and emerging treatments. Curr Opin Ophthalmol. May 2008;19(3):213-7. [Medline].

  10. 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].

 
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von Hippel-Lindau disease. Clinical picture of the retina, showing a pair of dilated and tortuous feeder vessels coursing on the surface of the retina from the optic nerve head toward the angioma (which is not seen in this image because it is in the extreme periphery).
von Hippel-Lindau disease. Color Doppler image of the dilated feeder vessels with flow patterns in opposite directions as indicated by red for the artery and blue for the vein. The abbreviation ON designates the optic nerve with its blood vessels.
von Hippel-Lindau disease. Spectral display of the aberrant feeder vessels.
 
 
 
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