ABSTRACT
In this series, we discuss the role of optical coherence tomography angiography (OCTA) in assessing response to treatment in intraocular vascular tumors. This is a series of two cases: Multiple retinal capillary hemangioblastoma (RCH) treated with laser photocoagulation and diffuse choroidal hemangioma (DCH) with radiotherapy. In large RCH and DCH, optical coherence tomography (OCT) showed significant reduction of subretinal and intraretinal fluid. But post-treatment mean tumor vascular density (MTVD) was slightly reduced. In one small RCH, vascular loop was seen suggesting minimal residual disease. So, OCTA helps in identifying treatment inadequacy and understanding alternate mechanism involved in treatment response in vascular tumors.
ABSTRACT
The aim of this study is to compare the optical coherence tomography angiography (OCTA) and fundus fluorescein angiography (FFA) features of retinal capillary hemangioblastoma (RCH). This is an observational case series of three patients with von Hippel朙indau (VHL) disease and one patient with juxtapapillary RCH. All patients underwent FFA with a mydriatic fundus camera and OCTA with swept-source angio OCT. The FFA and OCTA characteristics of tumors were compared. In our series, FFA could identify tumors as small as the width of a third-order retinal artery, which was missed on clinical examination. OCTA identified these tiny tumors, but only those closer to the posterior pole. Both FFA and OCTA could identify the intrinsic vasculature and feeder vessel in juxtapapillary RCH. On OCTA, the tumors were better defined than in FFA. The depth of the lesion can be identified on OCTA. Feeder and the draining vessels could be identified precisely in OCTA than FFA, particularly in small tumors. OCTA can identify tumors in VHL missed on clinical examination. It can identify the feeder vessel and intrinsic vasculature of sessile juxtapapillary RCH and aids in its diagnosis. Tumors are better defined in OCTA than FFA due to the absence of leakage. However, FFA can identify nearly all the early tumors, but OCTA fails to image the peripheral tumors due to its smaller field and prolonged acquisition time. Technological advances and the development of wide-field OCTA in the future can be helpful in identifying all the unsuspected tumors in VHL disease.
ABSTRACT
An 18-year-old boy presented to us with bilateral retinal hemangioblastoma and von Hippel-Lindau disease with history of cerebral capillary hemangioblastoma and embryonic cell carcinoma of left testes. The vision in the right eye was already lost with development of secondary closed angle glaucoma, optic atrophy with subsequent development of bullous keratopathy. The multiple retinal angiomatous lesions in the seeing left eye were treated with various modalities like triple freeze thaw cryopexy, focal lasers and transpupillary thermo therapy in multiple sittings over a period of almost 20 years since detection. One particular angiomatous lesion in the left eye was showing resistance to all the above mentioned modalities and was finally successfully treated with verteporfin and photodynamic therapy to achieve complete regression without any post-treatment complication and with a sustained 20/20 vision till a follow-up of 15 months.