Early surgical treatment of retinal hemangioblastomas

Abstract

ABSTRACT.Purpose: To evaluate the clinical course after early surgical treatment with excision of retinal hemangioblastomas (RHs) before development of major complications. Methods: Interventional case series of four eyes (four patients) with a peripheral RH that had not yet been treated by laser or cryotherapy prior to surgery. All eyes underwent 23-gauge vitrectomy with lesion excision. One patient underwent ligation of the feeder vessel prior to lesion excision. Best-corrected visual acuity and clinical course were assessed during a follow-up period of at least 4 years. Results: Four patients (mean age 27.3 years; range 19-32) were included, of whom two had von Hippel-Lindau syndrome. Visual acuity improved in three patients (mean 4.8 lines; range 3-10) and remained stable at 0.0 logMAR in one patient. There were no intraoperative complications. Postoperative complications included transient mild vitreous haemorrhage (n = 2), and local epiretinal membrane formation at the excision location (n = 1). At 4 years postoperatively, there were no long-term complications. There was one case of a new lesion, which was effectively treated with laser. Conclusion: Vitrectomy with RH excision seems to be an effective approach for larger RHs and could be considered an early treatment option in selected cases. Postoperative complications were limited in scope of this case series. Important points to consider during vitrectomy are effective closure of feeder and draining vessels as well as complete removal of posterior hyaloid and epiretinal membranes in order to avoid postoperative vitreous haemorrhage and proliferative vitreoretinopathy.

Retinal haemangioblastomas in von Hippel–Lindau germline mutation carriers: progression, complications and treatment outcome

“…These common progression‐related complications in ocular VHL disease may therefore be prevented with the expectation of a lesser need of multiple interventions and a greater chance of treatment complications. Treatment through vitreoretinal surgical intervention in an early stage might therefore be beneficial for MM carriers as they seem to have a more complicated progression of ocular VHL disease (van Overdam et al ).…”

Pharmacological Restoration of Visual Function in a Zebrafish Model of von-Hippel Lindau Disease

“…These lesions are normally associated with retinal neovascularisation through formation of arterial feeder vessels [30]. Currently there are no effective drug therapies for VHL disease and treatment of retinal hemangioblastomas relies on repeated surgeries or laser photocoagulation [30]. Our data demonstrates that reduction of ectopic ocular neovascularisation with sunitinib can restore vision in vhl -/- .…”

“…Development of retinal hemangioblastomas is a significant cause of visual morbidity in VHL patients. These lesions are normally associated with retinal neovascularisation through formation of arterial feeder vessels [30]. Currently there are no effective drug therapies for VHL disease and treatment of retinal hemangioblastomas relies on repeated surgeries or laser photocoagulation [30].…”

Classification and treatment follow-up of a juxtapapillary retinal hemangioblastoma with optical coherence tomography angiography

“…Based on our good experience with early surgical treatment of retinal hemangioblastomas in the retinal periphery, 13 we considered feeder vessel ligation or excision of the JRH during the planned removal of oil. For this reason, imaging by several modalities was performed preoperatively, to determine the growth type of the lesion to be excised.…”