Von Hippel-Lindau Syndrome Clinical Trial
Official title:
Pilot Study of Intravitreal Injection of Ranibizumab (rhuFAB V2) for Advanced Ocular Disease of Von Hippel-Lindau (VHL) Disease
Verified date | August 15, 2007 |
Source | National Institutes of Health Clinical Center (CC) |
Contact | n/a |
Is FDA regulated | No |
Health authority | |
Study type | Interventional |
This study will examine whether he drug ranibizumab can slow or stop the growth of angiomas
(blood vessel tumors) in patients with Von Hippel-Lindau syndrome (VHL). Angiomas commonly
develop in the back of the eye on the retina and the optic nerve in patients with VHL.
Although these tumors are not cancerous, they may cause significant vision loss. Current
treatments, including laser therapy, cryotherapy, and vitrectomy, may not be successful or
possible for all patients. Ranibizumab decreases production of VEGF, a growth factor that is
important for the formation of new blood vessels and that is elevated in patients with VHL.
Preliminary findings from other studies suggest that ranibizumab can reduce retinal
thickening caused by vessel and tumor growth and improve vision.
Patients 18 years of age and older with retinal angiomas due to VHL in one or both eyes and
central vision loss of 20/40 or worse may be eligible for this study. Participants undergo
the following tests and procedures:
- Medical history, physical examination, electrocardiogram (EKG) and blood tests.
- Eye examination, including eye pressure measurement and dilation of the pupils to
examine the retina.
- Fluorescein angiography to evaluate the eye's blood vessels. For this test, a yellow dye
is injected into an arm vein and travels to the blood vessels in the eyes. Pictures of
the retina are taken using a camera that flashes a blue light into the eye. The pictures
show if any dye has leaked from the vessels into the retina, indicating possible blood
vessel abnormality.
- Optical coherence tomography to measure retinal thickness. The eyes are examined through
a machine that produces cross-sectional pictures of the retina. These measures are
repeated during the study to determine changes, if any, in retinal thickening.
- Stereoscopic color fundus photography to examine the back of the eye. The pupils are
dilated with eye drops to examine and photograph the back of the eye.
- Electroretinogram (ERG) to measure electrical responses generated from within the
retina. For this test, the patient sits in a dark room for 30 minutes with his or her
eyes patched. Then, a small silver disk electrode is taped to the forehead, the eye
patches are removed, the surface of the eye is numbed with eye drops, and contact lenses
are placed on the eyes. The patient looks inside an open white globe that emits a series
of light flashes for about 20 minutes. The contact lenses sense small electrical signals
generated by the retina when the light flashes.
- Ranibizumab injections to treat ocular angiomas. Ranibizumab is injected through a
needle into the eye's vitreous (gel-like substance that fills the inside of the eye).
Seven injections are given over a 28-week period. Before each injection, the surface of
the eye is numbed with anesthetic eye drops. This is followed by injection of another
anesthetic into the lower portion of the eye in the clear tissue surrounding the white
of the eye. After a few minutes, the ranibizumab is injected into the vitreous. Patients
receive ranibizumab injections at the first visit (during enrollment) and again at 4, 8,
12, 16, 20 and 24 weeks after the first injection. At the 28-week visit, the doctor will
determine if further treatment is needed. Patients can continue to have injections every
4 weeks until 1 year of follow-up (54 weeks).
At each injection visit, participants repeat most of the tests described above to evaluate
the response to treatment and return a week later for another eye examination.
Status | Completed |
Enrollment | 5 |
Est. completion date | August 15, 2007 |
Est. primary completion date | |
Accepts healthy volunteers | No |
Gender | All |
Age group | 18 Years and older |
Eligibility |
- INCLUSION CRITERIA: 1. Participant must understand and sign the informed consent. 2. Participant must be at least 18 years of age. 3. Participant must have retinal angiomas secondary to VHL in one or both eyes. 4. Participant must have either optic nerve tumors or peripheral tumors that have caused central vision loss of 20/40 or worse. 5. Participant must have clear ocular media and adequate papillary dilation to permit good quality stereoscopic fundus photography. 6. All women of childbearing potential must have a negative urine pregnancy test at baseline, and be willing to undergo urine pregnancy testing immediately prior to each injection, and monthly for at least 2 months following the last dose of ranibizumab. 7. Women of child-bearing potential who are sexually active and men who are sexually active are required to use two forms of birth control during the course of the study. EXCLUSION CRITERIA: 1. History (within past 5 years) or evidence of severe cardiac disease (apparent in electrocardiogram abnormalities, clinical history of unstable angina, acute coronary syndrome, myocardial infarction, revascularization procedure within 6 months prior to baseline, atrial or ventricular tachyarrythmias requiring ongoing treatment). 2. History of stroke within 12 months of study entry. 3. History within the past five years of a chronic ocular or periocular infection (including any history of ocular herpes zoster). 4. Current acute ocular or periocular infection. 5. Any major surgical procedure within one month of study entry. 6. Known serious allergies to fluorescein dye. 7. Previous participation in a clinical trial (for either eye) involving anti-angiogenic drugs (pegaptanib, ranibizumab, anecortave acetate, Protein Kinase C inhibitors, etc). 8. Previous intravitreal drug delivery (e.g., intravitreal corticosteroid injection or device implantation) in the study eye. 9. History of vitrectomy surgery in the study eye. 10. History of glaucoma filtering surgery in the study eye. 11. History of corneal transplant in the study eye. |
Country | Name | City | State |
---|---|---|---|
United States | National Institutes of Health Clinical Center, 9000 Rockville Pike | Bethesda | Maryland |
Lead Sponsor | Collaborator |
---|---|
National Eye Institute (NEI) |
United States,
Manski TJ, Heffner DK, Glenn GM, Patronas NJ, Pikus AT, Katz D, Lebovics R, Sledjeski K, Choyke PL, Zbar B, Linehan WM, Oldfield EH. Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA. 1997 May 14;277(18):1461-6. — View Citation
Status | Clinical Trial | Phase | |
---|---|---|---|
Completed |
NCT01967537 -
Evaluation of 68Gallium-DOTATATE PET/CT for Detecting Neuroendocrine Tumors
|
Phase 2 | |
Terminated |
NCT01266070 -
TKI 258 in Von Hippel-Lindau Syndrome (VHL)
|
Phase 2 | |
Terminated |
NCT00673816 -
Sunitinib Malate to Treat Advanced Eye Disease in Patients With Von Hippel-Lindau Syndrome
|
Phase 1/Phase 2 | |
Terminated |
NCT00330564 -
Evaluation of Sunitinib Malate in Patients With Von Hippel-Lindau Syndrome (VHL) Who Have VHL Lesions to Follow
|
Phase 2 | |
Terminated |
NCT03001349 -
68Ga-DOTA-TOC PET/CT in Imaging Participants With Neuroendocrine Tumors
|
Early Phase 1 | |
Recruiting |
NCT01496625 -
National Eye Institute Biorepository for Retinal Diseases
|
||
Completed |
NCT02859441 -
A Phase I/II Trial for Intravitreous Treatment of Severe Ocular Von Hippel-Lindau Disease Using a Combination of the PDGF Antagonist E10030 and the VEGF Antagonist Ranibizumab
|
Phase 1/Phase 2 | |
Completed |
NCT01436227 -
Pazopanib Hydrochloride in Treating Patients With Von Hippel-Lindau Syndrome
|
Phase 2 | |
Completed |
NCT00075348 -
Genetic Study to Identify Gene Mutations in Participants Previously Enrolled in Clinical Trial NCI-99-C-0053 Who Have Von Hippel-Lindau Syndrome or Are at Risk for Von Hippel-Lindau Syndrome
|
N/A |