Capillary Hemangioblastoma of the Retina and von Hippel–Lindau Disease

Chew, Emily Y.; Schachat, Andrew P.

doi:10.1016/b978-1-4557-0737-9.00130-2

Cited by 8 publications

(4 citation statements)

References 124 publications

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“…The treatment of RCH is based on tumor size, location, and secondary complications [11,12]. The patient can be observed without treatment or treated with laser therapy, cryotherapy, photodynamic therapy with verteporfin [7,[13][14][15], or vitrectomy [10,[16][17][18][19][20].…”

Section: Introductionmentioning

confidence: 99%

Clinical Results of Various Treatments for Retinal Capillary Hemangioma

Won

Lee

Shin

et al. 2020

Korean J Ophthalmol

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We report the clinical outcomes of retinal capillary hemangioma (RCH) after the application of various treatments. Methods: We performed a retrospective chart analysis of eight eyes treated for RCH between August 2009 and January 2018. During the follow-up period, the status and progression of the RCHs were checked by fundus photography, fluorescein angiography, and optical coherence tomography, and additional treatments were applied when necessary. Results: Three of the five patients had bilateral RCH, and two had unilateral RCH. Six eyes received laser photocoagulation; two eyes received cryotherapy, and one eye received intravitreal Avastin injection. Three eyes each had intravitreal triamcinolone injection, subtenon triamcinolone injection, and intravitreal dexamethasone injection to control inflammation. Also, two patients took oral prednisolone, and one patient used prednisolone eye drops to control inflammation. Two eyes underwent vitrectomy and scleral buckling due to deterioration of the epiretinal membrane (ERM) and vitreal traction, respectively. As a result of those treatments, the tumors were stable in five of the eight eyes. However, one eye is now in a pre-phthisis state, and one patient who refused treatment showed progression of the tumor, ERM, and traction. Conclusions: Because RCHs vary in size, the degree of inflammation, and symptoms, this disorder should be actively treated on a case-by-case basis. Fluorescein angiography should be used periodically to determine recurrence of the tumor or inflammation, and the appropriate treatment should be repeated as necessary. Moreover, regular systemic screening tests for von Hippel-Lindau disease should be performed in RCH patients to ensure that they have no abnormalities other than in the eye.

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Section: Introductionmentioning

confidence: 99%

Clinical Results of Various Treatments for Retinal Capillary Hemangioma

Won

Lee

Shin

et al. 2020

Korean J Ophthalmol

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“…RHs are typically asymptomatic at early stages; nevertheless, in advanced stages, these tumors turn exudative, resulting in diffuse retinal edema with hard exudates which often form a ring or "circinate" pattern around the vascular lesion. Vision loss is usually caused by exudation affecting the macula, glial proliferation or posterior hyaloid contraction related to the tumor and, in severe cases, tractional and/or exudative retinal detachment [4][5][6]. Intraocular pressure elevation secondary to iris neovascularization and neovascular glaucoma has been described [4,5].…”

Section: Introductionmentioning

confidence: 99%

“…Vision loss is usually caused by exudation affecting the macula, glial proliferation or posterior hyaloid contraction related to the tumor and, in severe cases, tractional and/or exudative retinal detachment [4][5][6]. Intraocular pressure elevation secondary to iris neovascularization and neovascular glaucoma has been described [4,5]. Diagnosis of VHL disease is based on clinical criteria and confirmed by molecular testing [5,6].…”

Section: Introductionmentioning

confidence: 99%

Treatment of Capillary Hemangioblastoma in Von Hippel-Lindau Disease: Case Report and Online Survey of Treatment Preferences among Experts

Valdés-Lara

Alvarado

Elizondo-Camacho

et al. 2020

Case Rep Ophthalmol

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This case report aims to describe the diagnosis, treatment, and evolution of bilateral, asymmetrical retinal capillary hemangioblastomas treated with argon laser and intravitreal anti-vascular endothelial growth factor and also reports the results of an online survey of treatment preferences among retina and vitreous specialists. A previously healthy 23-year-old female presented to our Retina Department complaining of progressive visual loss in her right eye. Visual acuity at admission was 20/300 in her right eye and 20/20 in her left eye. Anterior segment findings were unremarkable and fundoscopy revealed the presence of retinal capillary hemangioblastomas in both eyes. In the right eye, the hemangioblastoma was associated with pronounced exudation and macular edema; in the left eye, the lesion was quiescent. After a complete anamnesis and genetic counseling, Von Hippel-Lindau disease was diagnosed. Treatment with laser photocoagulation was performed on both eyes. One dose of 0.5 mg intravitreal ranibizumab was applied to the right eye. Two months after treatment, the right eye Case 38 demonstrated improved visual acuity (20/100). Moreover, an important decrease in tumor dimensions and a reduction of vessel tortuosity was seen in both eyes. At 18 months of followup, the patient maintains a good visual acuity without recurrence of the treated tumors. Laser treatment should be considered as the primary treatment option for patients with capillary hemangioblastomas with and without exudation and can be combined with intravitreal antiangiogenics if exudation is significant. Inactive smaller lesions without exudation are likely to have an excellent response to laser treatment alone. Management should be individualized since no consensus between experts has been reached.

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“…Many tumors are recalcitrant to treatment including intravitreous injections of steroids or anti-vasogenic endothelial growth factor (VEGF), cryotherapy, radiation and laser, including photodynamic therapy. 1 , 2 , 3 , 4 , 5 It is not uncommon for these eyes to progress to blindness due to exudation, tractional retinal changes, or neovascular glaucoma; and it has been predicted that 25% of eyes will be rendered permanently blind (vision less than 20/400). 2 , 6 An alternative form of management to halt the progression of disease, maintain vision and provide ocular salvage is explored in this case report.…”

Section: Introductionmentioning

confidence: 99%

Treatment of juxtapapillary hemangioblastoma by intra-arterial (ophthalmic artery) chemotherapy with bevacizumab

Francis

Slakter

Abramson

et al. 2018

American Journal of Ophthalmology Case Reports

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PurposeTo report on two cases of treatment-refractory juxtapapillary hemagioblastomas that were managed with intra-arterial bevacizumab delivered via the ophthalmic artery.ObservationsCase 1 is a 35 year-old man with juxtapapillary hemangioblastoma who continued to have progressive tractional retinal detachment, optic nerve neovascaularization and cystoid macula edema (CME) despite heavy prior treatment with intravitreal anti-vascular endothelial growth factor (VEGF) and steroid intravitreal injections and laser. Case 2 is a 41 year-old woman with juxtapapillary hemangioblastoma who had progressive tractional retinal detachment, CME and visually-threatening intraocular pressure elevation despite treatment with anti-VEGF injection and laser. Both cases were treated with three infusions of intra-arterial bevacizumab delivered via the ophthalmic artery. Both tumors demonstrated measurable decrease in height, stability of their secondary retinal changes and minimal requirement for additional treatment at 30 mos and 26 mos follow-up, respectively for cases 1 and 2.Conclusions and importanceThese cases suggest that higher-dose, targeted delivery of anti-VEGF to hemangioblastomas via ophthalmic artery injection may be useful in stabilizing the disease and abating the typical progression of secondary retinal pathology, at least in the first two years after treatment.

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Capillary Hemangioblastoma of the Retina and von Hippel–Lindau Disease

Cited by 8 publications

References 124 publications

Clinical Results of Various Treatments for Retinal Capillary Hemangioma

Clinical Results of Various Treatments for Retinal Capillary Hemangioma

Treatment of Capillary Hemangioblastoma in Von Hippel-Lindau Disease: Case Report and Online Survey of Treatment Preferences among Experts

Treatment of juxtapapillary hemangioblastoma by intra-arterial (ophthalmic artery) chemotherapy with bevacizumab

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