Novel Surgical Treatment of an Intraretinal Juxtapapillary Hemangioblastoma Using Intraocular Diathermy Forceps: A Case Report.

The surgical treatment of intraretinal juxtapapillary retinal hemangioblastomas (JRHs) was previously contraindicated because of the significant risk of collateral damage to the macula and optic nerve. This case report discusses the effectiveness and safety of a novel surgical technique using intraocular bipolar diathermy forceps to coagulate feeder and draining blood vessels of an intraretinal JRH. The patient suffered from bilateral retinal hemangioblastomas with loss of visual function in one eye and the development of an intraretinal JRH in the other eye. Despite intensive treatment with intravitreal bevacizumab and subconjunctival triamcinolone acetonide, growth of the intraretinal JRH continued, macular exudation worsened, and visual acuity decreased. Surgical treatment was undertaken in which, first, the feeder and draining vessels of the JRH were identified by comparing the retinal imaging of the JRH with the imaging before the emergence of the JRH 4 years earlier. Then, retinal incisions were made above the blood vessels and parallel to the nerve fibers during a pars plana vitrectomy. Lastly, these vessels were lifted above the retinal surface and coagulated using intraocular diathermy forceps. Postoperatively, macular edema reduced, and visual acuity increased and remained stable for about 6 months. Using intraocular diathermy forceps, this case report demonstrates effective and safe intraretinal JRH blood vessel coagulation above the retinal surface. This novel surgical approach was able to delay the deterioration of visual acuity due to tumor growth and exudation in this patient. This suggests that coagulation with intraocular diathermy forceps can be considered an additional surgical treatment option for JRHs, especially those with an intraretinal growth pattern.

Detailed optical coherence tomography angiographic short-term response of type 3 neovascularization to combined treatment with photodynamic therapy and intravitreal bevacizumab.

PURPOSE: To explore the short-term vascular and structural changes of type 3 neovascularization using optical coherence tomography angiography (OCT-A) when treated with a combination of photodynamic therapy (PDT) and intravitreal bevacizumab (IVB), and to evaluate the course of different sequences of the combined therapies. METHODS: Thirty eyes of 29 treatment-naïve patients with a type 3 neovascularization were included in this prospective observational cohort study. They were all treated with PDT and IVB 2 weeks apart, starting either with PDT (PDT-first group) or IVB (IVB-first group). Optical coherence tomography angiography (OCT-A) imaging was performed at week 0, 2, 4 and 18, and best corrected visual acuity (BCVA) at week 0 and 18. Vascular, structural and functional features were graded and analysed over time. RESULTS: In all patients, at all follow-up visits, vascular and structural features were significantly more often decreased or resolved than unchanged or increased. Best corrected visual acuity (BCVA) significantly improved at 18 weeks. Vascular, structural and functional outcomes were all slightly better in the PDT-first group compared to the IVB-first group, although not statistically significant. CONCLUSION: Combined treatment of PDT and IVB is effective in short-term for type 3 neovascularization based on vascular and structural features. Initial treatment with PDT tended to be more effective than with IVB.

The Influence of Prolongation of Interruptions of Preoperative Posturing and Other Clinical Factors on the Progress of Macula-On Retinal Detachment.

PURPOSE: To evaluate whether prolongation of interruptions of preoperative posturing by sitting upright influences retinal detachment (RD) progression. The secondary objective was to find clinical factors to identify patients with a high risk for RD progression. DESIGN: Prospective cohort study. PARTICIPANTS: One hundred ninety-eight patients divided among 3 cohorts of patients with macula-on RD were included. Inclusion criteria were volume OCT scans of sufficient quality and smallest distance from the fovea to the detachment border of at least 1.25 mm. In the second and third cohort, 50 patients with only superior temporal RD were included. METHODS: Patients were admitted to the ward in anticipation of surgery. Preoperative bed rest and positioning were prescribed. The position of the RD border was determined based on OCT imaging performed at baseline, before and after the usual interruptions for meals or toilet visits. The duration of interruptions was prolonged with sitting upright for 20 minutes in cohort 2 and for 40 minutes in cohort 3. Various secondary outcome measures were defined, such as the baseline area of subretinal fluid (SRF) as measured on ultrasound images in the third cohort. MAIN OUTCOME MEASURES: The RD border displacement was determined. The worst RD progression from baseline was given by the shortest distance to the fovea in any of the OCT scans during follow-up. The worst relative RD progression from baseline was defined as the worst RD progression from baseline as a percentage of the baseline distance between RD border and fovea. RESULTS: The median duration of interruptions was 22, 41, and 58 minutes in cohorts 1, 2, and 3, respectively. The median RD border displacement during interruptions in patients with superior temporal RD was not significantly different among the cohorts (P = 0.28). The correlation coefficient between the SRF area at baseline and worst relative RD progression from baseline was 0.37 (95% confidence interval, 0.04-0.66; P = 0.009). CONCLUSIONS: We did not find a significant increase in RD progression after prolongation of interruptions by sitting upright. Patients with a larger area of SRF on ultrasound showed more RD progression from baseline.

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

PURPOSE: Only an endophytic growth pattern in juxtapapillary retinal hemangioblastoma (JRH) is an indication for surgical treatment, but classification of growth types is difficult using conventional imaging techniques. This case report describes the use of optical coherence tomography angiography (OCT-A) features for classification and treatment follow-up in a case with JRH. OBSERVATIONS: The JRH of this patient was easily detected with two different OCT-A methods in both en-face and cross-sectional B-scan images, and was classified as a sessile growth type. This growth type excluded the treatment option of vitreoretinal surgery with excision of the lesion or ligation of the feeder vessels. The patient was treated multiple times with intravitreal bevacizumab. Treatment follow-up with OCT-A initially revealed a stable extent of the JRH, with some slight flow deviations in en-face visualization, followed by a period of progressive growth of the lesion. CONCLUSIONS: OCT-A revealed the depth localization of the JRH and seems to be a valuable tool for JRH classification. Detailed classification may be useful when surgery is considered as a treatment strategy. Furthermore, treatment follow-up is possible with OCT-A, although imaging artifacts should be taken into account.

Treatment Effects in Retinal Angiomatous Proliferation Imaged with OCT Angiography.

PURPOSE: This prospective case series is aimed at exploring optical coherence tomographic angiography (OCT-A) as a treatment monitoring tool in patients treated for retinal angiomatous proliferation (RAP). METHODS: Twelve treatment-naïve RAP patients were included, with a median age of 79 years (range 65-90). Patients were imaged with an experimental 1,040-nm swept-source phase-resolved OCT-A instrument before and after treatment. Treatment consisted of either intravitreal bevacizumab or triamcinolone injections with or without photodynamic therapy (PDT). Abnormal blood flow after treatment was graded as increased, unchanged, decreased, or resolved. RESULTS: OCT-A images before and after treatment could be obtained in 9 patients. The median follow-up period was 10 weeks (range 5-19). After various treatments, the RAP lesion resolved in 7 patients, in 1 patient the OCT-A depicted decreased flow in the lesion, and 1 patient showed unchanged abnormal blood flow. Monotherapy with intravitreal bevacizumab injections resolved RAP in 1 out of 2 patients. Combined therapy of bevacizumab with PDT resolved RAP in 6 out of 7 patients. CONCLUSIONS: OCT-A visualized resolution of abnormal blood flow in 7 out of 9 RAP patients after various short-term treatment sequences. OCT-A may become an important noninvasive monitoring tool for optimizing treatment strategies in RAP patients.

Preoperative Posturing of Patients with Macula-On Retinal Detachment Reduces Progression Toward the Fovea.

PURPOSE: Traditionally, preoperative posturing consisting of bed rest and positioning is prescribed to patients with macula-on retinal detachment (RD) to prevent RD progression and detachment of the fovea. Execution of such advice can be cumbersome and expensive. This study aimed to investigate if preoperative posturing affects the progression of RD. DESIGN: Prospective cohort study. PARTICIPANTS: Ninety-eight patients with macula-on RD were included. Inclusion criteria were volume optical coherence tomography (OCT) scans could be obtained with sufficient quality; and the smallest distance from the fovea to the detachment border was 1.25 mm or more. METHODS: Patients were admitted to the ward for bed rest in anticipation of surgery and were positioned on the side where the RD was mainly located. At baseline and before and after each interruption for meals or toilet visits, a 37°×45° OCT volume scan was performed using a wide-angle Spectralis OCT (Heidelberg Engineering, Heidelberg, Germany). The distance between the nearest point of the RD border and fovea was measured using a custom-built measuring tool. MAIN OUTCOME MEASURES: The RD border displacement and the average RD border displacement velocity moving toward (negative) or away (positive) from the fovea were determined for intervals of posturing and interruptions. RESULTS: The median duration of intervals of posturing was 3.0 hours (interquartile range [IQR], 1.8-14.0 hours; n = 202) and of interruptions 0.37 hours (IQR, 0.26-0.50 hours; n = 197). The median RD border displacement was 2 µm (IQR, -65 to +251 µm) during posturing and -61 µm (IQR, -140 to 0 µm) during interruptions, a statistically significant difference (P < 0.001, Mann-Whitney U test). The median RD border displacement velocity was +1 µm/hour (IQR, -21 to +49 µm/hour) during posturing and -149 µm/hour (IQR, -406 to +1 µm/hour) during interruptions, a statistically significant difference (P < 0.001). CONCLUSIONS: By making use of usual interruptions of preoperative posturing we were able to show, in a prospective and ethically acceptable manner, that RD stabilizes during posturing and progresses during interruptions in patients with macula-on RD. Preoperative posturing is effective in reducing progression of RD.

Joint Segmentation of Retinal Layers and Focal Lesions in 3-D OCT Data of Topologically Disrupted Retinas.

Accurate quantification of retinal structures in 3-D optical coherence tomography data of eyes with pathologies provides clinically relevant information. We present an approach to jointly segment retinal layers and lesions in eyes with topology-disrupting retinal diseases by a loosely coupled level set framework. In the new approach, lesions are modeled as an additional space-variant layer delineated by auxiliary interfaces. Furthermore, the segmentation of interfaces is steered by local differences in the signal between adjacent retinal layers, thereby allowing the approach to handle local intensity variations. The accuracy of the proposed method of both layer and lesion segmentation has been evaluated on eyes affected by central serous retinopathy and age-related macular degeneration. In addition, layer segmentation of the proposed approach was evaluated on eyes without topology-disrupting retinal diseases. Good agreement between the segmentation performed manually by a medical doctor and results obtained from the automatic segmentation was found for all data types. The mean unsigned error for all interfaces varied between 2.3 and 11.9 µm (0.6-3.1 pixels). Furthermore, lesion segmentation showed a Dice coefficient of 0.68 for drusen and 0.89 for fluid pockets. Overall, the method provides a flexible and accurate solution to jointly segment lesions and retinal layers.

INTRAVITREAL VERSUS SUBRETINAL ADMINISTRATION OF RECOMBINANT TISSUE PLASMINOGEN ACTIVATOR COMBINED WITH GAS FOR ACUTE SUBMACULAR HEMORRHAGES DUE TO AGE-RELATED MACULAR DEGENERATION: An Exploratory Prospective Study.

PURPOSE: Current management of submacular hemorrhage (SMH) favors vitrectomy and gas with subretinal administration of recombinant tissue plasminogen activator (rtPA) over mere intravitreal rtPA injections and gas. In this study, we aimed to compare the effectiveness of both treatment modalities to displace submacular blood. METHODS: Twenty-four patients with SMH secondary to age-related macular degeneration were included. The SMH had to exist ≤14 days at time of surgery and SMH thickness had to be between 250 µm and 1,250 µm. Patients were randomized to either intravitreal injections of rtPA, perfluoropropane (C3F8) gas, and bevacizumab (n = 12) or vitrectomy with subretinal rtPA administration, intravitreal C3F8 gas, and bevacizumab (n = 12). The SMH volume change was measured on spectral domain optical coherence tomography postoperatively within a 2.5-mm cylinder centered at the fovea. RESULTS: Median relative volume reduction of subretinal blood at 6 weeks postoperatively was 97% (95% confidence interval: 91-99%) in the intravitreal rtPA group and 100% (95-100%) in the subretinal rtPA group and did not differ significantly between groups (P = 0.56). CONCLUSION: Both treatment modalities effectively displaced SMH in this exploratory clinical trial. To more definitely study the noninferiority of intravitreal rtPA with gas to subretinal rtPA, vitrectomy with gas, a larger clinical trial would be necessary.

Phase-Resolved Doppler Optical Coherence Tomographic Features in Retinal Angiomatous Proliferation.

PURPOSE: To study patients diagnosed with retinal angiomatous proliferation (RAP) based on conventional imaging techniques with phase-resolved Doppler optical coherence tomography (OCT) to detect and localize blood flow in RAP lesions; and to compare these findings to conventional imaging, which are mostly invasive and give limited information concerning intra- and transretinal blood flow. DESIGN: Single-center, consecutive observational case series. METHODS: Twelve treatment-naïve patients diagnosed with RAP based on fundus examination, fluorescein angiography, and indocyanine green angiography were included. Median age was 79 years (range 65-90). Patients were imaged with an experimental 1040 nm swept-source phase-resolved Doppler OCT instrument. Abnormal flow was defined as intraretinal neovascularization or retinal choroidal anastomosis. RESULTS: In 11 patients adequate phase-resolved Doppler OCT images were obtained showing abnormal blood flow in the RAP lesion. In 4 patients a retinal choroidal anastomosis was found, 3 patients showed intraretinal neovascularization connected with a pigment epithelial detachment, 2 patients showed only intraretinal neovascularization, and in 2 patients flow was limited to the subretinal or sub-retinal pigment epithelial space. CONCLUSIONS: Phase-resolved Doppler OCT is able to detect and localize abnormal blood flow within RAP lesions. Blood flow was mostly confined to the intraretinal structures with or without a connecting pigment epithelial detachment; in one-third of patients a retinal choroidal anastomosis was detected. The potential of angiography with phase-resolved Doppler OCT to accurately distinguish between normal and pathologic blood flow in addition to structural OCT data without invasive procedures will help to further elucidate both retinal and choroidal vascular pathologies like RAP.