Retinal Hemangioblastomas Research Articles

Pediatric patients with von Hippel-Lindau and hemangioblastomas treated successfully with belzutifan.

Hemangioblastoma is the most common tumor associated with von Hippel-Lindau (VHL), and are a leading cause of mortality. We present five pediatric patients with VHL-associated hemangioblastomas treated with belzutifan, a hypoxia-inducible factor 2a (HIF2a) inhibitor. Three patients were started on belzutifan due to vision loss from progressive retinal hemangioblastomas. Within oneyear of treatment, all three patients had improvement in hemangioblastoma size and visual acuity. For patients with intracranial lesions, belzutifan resulted in an improvement in neurologic symptoms and hemangioblastoma size. Four patients experienced grade 1-2 anemia and two patients required a dose reduction. Our report suggests that belzutifan can be an effective therapy for pediatric, adolescent, and young adult patients with VHL-associated hemangioblastomas.

Genotype-phenotype correlation of ocular von Hippel-Lindau disease in Koreans.

This scientific report aims to investigate the genotype-phenotype correlations of retinal hemangioblastoma (RH) in von Hippel-Lindau (VHL) disease. The study included 77 patients with genetically confirmed VHL disease who visited an ophthalmology clinic for the evaluation of RH. The presence, location, and size of RH were evaluated, Patients were categorized into three groups based on variants: HIF-1α binding site missense (HM), non-HIF-1α binding site missense (nHM), and truncating (TR) mutations. Fifty-six patients (72.7%) had RH in either eye, and 24 had bilateral RH. Sixteen patients (20.8%) had juxtapapillary RH in either eye. Nine patients had RH ≥ 2.0 disc diameters in size. VHL c.208G>A variant was the most frequent single mutation. Compared with patients having nHM mutations (15 patients) in VHL gene, patients with HM mutations (33 patients) or TR mutations (26 patients) presented a greater number of eyes affected (p = 0.007 and 0.004, respectively), a greater number of RH (p = 0.012 and 0.003, respectively), and more frequent presentation of large RH ≥ 2.0 disc diameters (p = 0.012, and 0.013, respectively). In conclusion, this study provides a deeper understanding of the genetic spectrum of VHL disease in Korean VHL disease and highlights the importance of the location of missense mutations regarding the risk of RH.

Phenotypic and Genotypic Features of a Chinese Cohort with Retinal Hemangioblastoma.

Purpose: To delineate the genotype and phenotype of RH in a Chinese cohort. Methods: A group of 51 Chinese probands with RH across 76 eyes was assembled and underwent complete retinal imaging examinations. Sanger sequencing and universal primer quantitative fluorescent multiplex-polymerase chain reaction (UPQFM-PCR) were employed for mutation detection in the coding region of the Von Hippel-Lindal (VHL) gene. For frequency calculation, our series was combined with three large cohorts of East Asian descent through a literature review. Results: The Von Hippel-Lindal (VHL) syndrome was excluded in fifteen patients (median age: 32.00 years) with unilateral solitary RH. Thirty-six patients of younger ages (median: 22.00 years, p = 0.008, Mann-Whitney test) conformed to the diagnostic criteria of the VHL syndrome, and thirty-four patients were genetically confirmed. There were four novel variants identified in the VHL gene. Codons 167, 161 and 86 exhibited a mutation occurrence of more than 5% after pooling with literature data, and the large genomic deletion demonstrated a frequency of 17.65%. The RHs were classified as "extrapapillary", "juxtapapillary" and "mixed" types in 53, 7 and 5 eyes, respectively. Almost all extrapapillary RH lesions were found in the peripheral retina. Hemangioblastomas in the central nervous system (CNS) were observed in 25 out of 31 kindreds (80.65%) with full systemic evaluation data. Conclusions: VHL-associated RH might exhibit earlier onset than non-VHL RH. Large genomic deletions were observed at a notably high frequency in the Chinese series with VHL-associated RH, which might be associated with East Asian ethnicity background. RH could potentially serve as an early indicator of CNS hemangioblastoma.

SUCCESSFUL TREATMENT OF VON HIPPEL-LINDAU DISEASE-ASSOCIATED RETINAL CAPILLARY HEMANGIOBLASTOMA WITH BELZUTIFAN IN A PEDIATRIC PATIENT.

The authors describe a case of a retinal capillary hemangioblastoma (RCH) in a pediatric patient with von Hippel-Lindau (VHL) syndrome that was successfully treated with systemic belzutifan. The clinical course was documented with serial fundus examinations and multimodal imaging, including Optos widefield fundus photography and optical coherence tomography. A literature review was conducted to look for similar cases and/or discussion. A left RCH was noted on a standard VHL surveillance retinal examination of a then 15-year-old male patient with VHL syndrome. Over the course of 17 months, this RCH was treated with focal laser therapy, photodynamic therapy, cryotherapy, bevacizumab injection, and endolaser ablation. Complications of these treatments included subretinal fluid and vitreomacular traction necessitating laser retinopexy, scleral buckle, and pars plana vitrectomy with membrane stripping. After a 6-month interval from the last local therapy (endolaser treatment), there was minimal regression of the lesion, and many concerning features persisted. At 22 months from presentation, the patient started belzutifan 120 mg PO daily with subsequent regression in size and less perfusion to the hemangioblastoma within 4 months. The patient is tolerating the systemic belzutifan with only the expected normocytic anemia and has not required transfusion therapy after 12 months of treatment. von Hippel-Lindau disease is a rare and serious condition associated with multiple types of benign and malignant tumors. Belzutifan is tolerated in the adolescent population and can provide a systemic treatment alternative for VHL-associated RCH.

Significant Improvement in Retinal Capillary Hemangioblastoma With Belzutifan.

Significant Improvement in Retinal Capillary Hemangioblastoma With Belzutifan.

Role of optical coherence tomography angiography in retinal tumors: A narrative review.

Intraocular tumors constitute a small subset of cases in ophthalmologic practice. Proper diagnosis of intraocular tumors is crucial because some pose threat to vision and life, while others may indicate underlying systemic disorders. Intraocular tumors comprise benign and malignant lesions affecting the retina, choroid, optic disc, iris, and ciliary body. Retinal tumors can be classified as vascular, neural, glial, and retinal pigment epithelial tumors. Optical coherence tomography angiography (OCTA) is a noninvasive imaging modality employed in diagnosis and management of retinal and choroidal vascular diseases, and has enhanced our knowledge in better understanding of the vascular physiology and pathology. Multiple case reports and small series evaluating the role of OCTA in retinal tumors are published in literature. OCTA helps in better understanding of the vascularity of intraocular tumors. In addition to this, OCTA has its role in clinical practice. It helps in identification of small retinal capillary hemangioblastoma (RCH), assessment of treatment response, and identification of tumor recurrence in RCH. It aids in identification of retinal astrocytic hamartoma missed on clinical examination and differentiating retinal astrocytic hamartoma and presumed solitary circumscribed retinal astrocytic proliferation. It helps in assessment of risk of tumor recurrence in retinoblastoma. It helps in differentiating tumors of retinal pigment epithelium (RPE) origin from pigmented tumors of the choroid. It also helps in detection of choroidal neovascular membrane in combined hamartoma of the retina and RPE.

Optical Coherence Tomography Angiography-Navigated Laser Photocoagulation of Retinal Hemangioblastomas in Patients With von Hippel-Lindau Disease.

To describe optical coherence tomography angiography (OCTA)-guided navigated laser photocoagulation (LP) using the Navilas Laser System for treating retinal hemangioblastomas (RHs) associated with von Hippel-Lindau disease (VHLD). Patients with VHLD were screened using ophthalmoscopy and widefield OCTA. Detected RHs were classified with regard to tumor morphology (endophytic, sessile, exophytic, recurrent) and size. Then, 6 × 6- or 3 × 3-mm2 en face OCTA scans of the RHs were uploaded to the Navilas system, generating a merged image combining the scan and Navilas fundus photography. LP was planned by placing laser spots in the OCTA scan and executed with the Navilas system. Treatment efficacy was assessed by conducting OCTA scans immediately after LP and at follow-up visits. Fifteen RHs were detected in 10 patients (median, one RH; range, one to four). Twelve RHs were treatment naive (exophytic [3], sessile [3], and endophytic [6]), and there were three recurrent RHs in pretreated areas. Total applied energy per tumor correlated with tumor size (P < 0.001). After a mean first follow-up of 3.6 ± 1.5months (range, 0.9-5.3), nine RHs exhibited complete regression (60%), five partial regression (33.3%), and one no regression (6.7%). No correlation between tumor morphology and treatment success was observed (P = 0.32). However, a correlation between treatment success and tumor size trended toward significance (P = 0.08), with a 100% success rate observed for small RHs. OCTA-guided LP via the Navilas Laser System is a promising technique, especially beneficial for targeting small RHs. Combining OCTA and ophthalmoscopy improves tumor detection, underscoring the utility of this approach. OCTA-guided LP enables highly precise and safe treatment of early-stage RHs, minimizing possible complications caused by LP or the tumor itself.

Oral Hypoxia-Inducible Factor 2α Inhibitor Belzutifan in Ocular von Hippel-Lindau Disease: Subgroup Analysis of the Single-Arm Phase 2 LITESPARK-004 Study

ObjectiveTo report the efficacy of oral HIF-2α inhibitor belzutifan in participants with von Hippel-Lindau disease-associated retinal hemangioblastomas in LITESPARK-004. DesignSubgroup analysis of the phase 2, single-arm, open-label LITESPARK-004 study. ParticipantsAdults with ≥1 von Hippel-Lindau disease-associated measurable renal cell carcinoma tumor not requiring immediate surgical intervention were eligible. Methods and InterventionParticipants received oral belzutifan 120 mg once daily until disease progression or unacceptable treatment-related toxicity. Main Outcome MeasuresEfficacy of belzutifan in retinal hemangioblastomas was a secondary end point, measured as response (improved, stable, or progressed) by independent reading center certified graders based on color fundus imaging performed every 12 weeks using the investigator’s preferred imaging standards. Additional assessments, where available, included optical coherence tomography and ultra-widefield fluorescein angiography. ResultsAmong 61 participants in LITESPARK-004, 12 had ≥1 evaluable active retinal hemangioblastoma in 16 eyes at baseline per independent reading center. As of April 1, 2022, the median follow-up for participants with ocular von Hippel-Lindau disease at baseline was 37.3 months. All 16 eyes were graded as improved, with a response rate of 100.0% (95% confidence intervals, 79.4–100.0). No new retinal hemangioblastomas or ocular disease progression were reported as of data cutoff date. Eight participants had additional multimodal eye assessments performed at the National Institutes of Health study site. Among this subgroup, 10 of 24 hemangioblastomas in 8 eyes of 6 participants measured ≥500 μm in greatest linear dimension at baseline and were further analyzed. All 10 hemangioblastomas had a mean area reduction of ≥15% by month 12 and ≥30% by month 24. ConclusionsBelzutifan showed promising activity against ocular von Hippel-Lindau disease, including capacity to control retinal hemangioblastomas, with effects sustained for >2 years while on treatment.

Belzutifan in Individuals with von Hippel-Lindau Retinal Hemangioblastomas: Institutional Experience and Review of the Literature

Introduction: The systemic HIF-2 alpha inhibitor, belzutifan, has been approved for use in patients with von Hippel-Lindau disease (VHL)-associated renal cell carcinoma, central nervous system (CNS) hemangioblastomas, and pancreatic neuroendocrine tumors. This drug has also shown promise in controlling VHL retinal hemangioblastomas (RHs), but little work has been published on the use of the drug in this setting. Methods: We conducted a retrospective review of patients with VHL-associated RHs followed by the retina service at our institution who were treated with systemic belzutifan. Patient age, gender, genotype, presence of systemic tumors, indication for the drug, initial dose, adjusted dose, side effects, and tumor response were recorded. We also conducted a literature search for all manuscripts describing the effect of belzutifan on VHL-associated ocular tumors. Results: We identified 12 eyes of 7 patients with VHL-associated ocular tumors who were treated with belzutifan at our institution. Of these, 5 eyes of 3 patients had progressing ocular tumors when belzutifan was started. Of the 7 total patients, 2 were treated for renal cell carcinoma, 2 for CNS hemangioblastomas, 2 for RHs, and one for pancreatic neuroendocrine tumors. Initial dose was 120 mg PO daily in 6 patients and 80 mg PO daily in 1 patient. The dose was reduced in all but 1 patient due to side effects. The ocular tumors were controlled in all patients with an average follow-up of 13 months (range 4–24 months). Literature review identified 7 manuscripts that described belzutifan-mediated control of ocular tumors in patients with VHL-associated RHs in 21 patients. Conclusion: The drug belzutifan shows great promise for controlling RHs and preventing vision loss in patients with VHL. Further work needs to address the optimal dose, role of the drug as a neoadjuvant therapy, and long-term efficacy and tolerability of the drug in a larger cohort of patients with ocular tumors.

Response of a von Hippel-Lindau-associated optic nerve hemangioblastoma to belzutifan.

Purpose: To demonstrate the response of a juxtapapillary retinal hemangioblastoma (JRH) to belzutifan in a patient with Von-Hippel-Lindau syndrome (VHL). Methods: This case report includes fundus photography and optical coherence tomography to compare a juxtapapillary retinal hemangioblastoma (JRH) before and 24 months after treatment with belzutifan. Results: An 18-year-old woman with VHL was diagnosed with a juxtapapillary retinal hemangioblastoma (JRH) at age 13. The retinal lesion progressively worsened over 5 years and required two sessions of laser therapy with minimal improvement but loss of visual field. She was treated with the oral HIF2a-inhibitor, belzutifan, with minimal side effects. At 24 months of therapy her JRH has decreased in height from 1258 microns to 874 microns and width from 4448 µm to 3786 µm and vision is 20/20. Conclusion: Belzutifan demonstrates impressive clinical activity in VHL-associated JRH.

Abstract CT221: Belzutifan, a hypoxia-inducible factor-2α (HIF-2α) inhibitor, for von Hippel-Lindau (VHL) disease-associated neoplasms: Long-term results of the phase 2 LITESPARK-004 study

Abstract Introduction: Belzutifan is a first-in-class HIF-2α inhibitor approved for patients (pts) with VHL disease-associated renal cell carcinoma (RCC), central nervous system (CNS) hemangioblastomas (HB), or pancreatic neuroendocrine tumors (pNET) not requiring immediate surgery based on initial results from the phase 2 LITESPARK-004 study (NCT03401788). We present results from pts with more than 4 years of follow-up from LITESPARK-004. Methods: Adults with germline VHL alteration, ≥1 measurable nonmetastatic RCC tumor, no RCC tumor &amp;gt;3 cm requiring immediate surgery, and no prior systemic anticancer therapy received oral belzutifan 120 mg once daily until disease progression, unacceptable toxicity, or withdrawal. The primary end point was objective response rate (ORR) in VHL-associated RCC per RECIST v1.1 by independent review. Secondary end points included ORR in CNS HB and pNET; duration of response (DOR), time to response (TTR), progression-free survival (PFS), and time to surgery (TTS) in RCC, CNS HB, and pNET; and safety. Results: As of April 3, 2023, 36 of 61 pts (59%) were continuing treatment. Median follow-up was 49.9 mo (range, 48.2-58.1). Efficacy data are shown in the table. In the 4 years preceding belzutifan treatment, 46 pts (75%) underwent a total of 86 VHL-related tumor reduction procedures (surgery or radiation therapy). Since starting belzutifan, 16 pts (26%) underwent 18 tumor reduction procedures (RCC, n = 13; CNS HB, n = 3; retinal HB, n = 2). Safety profile remained unchanged with long-term follow-up. Conclusions: With a median follow-up of more than 4 years, belzutifan continues to demonstrate clinically meaningful ORR and durable responses in VHL-associated RCC, CNS HBs, and pNET. Fewer tumor reduction procedures were observed after pts started belzutifan treatment. These results support belzutifan as a standard of care for this pt population. Citation Format: Ramaprasad Srinivasan, Othon Iliopoulos, Kathryn E. Beckermann, Vivek Narayan, Benjamin L. Maughan, Stephane Oudard, Tobias Else, Jodi K. Maranchie, Ane B. Iversen, Jerry Cornell, Rodolfo F. Perini, Yanfang Liu, W. Marston Linehan, Eric Jonasch. Belzutifan, a hypoxia-inducible factor-2α (HIF-2α) inhibitor, for von Hippel-Lindau (VHL) disease-associated neoplasms: Long-term results of the phase 2 LITESPARK-004 study [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 2 (Late-Breaking, Clinical Trial, and Invited Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(7_Suppl):Abstract nr CT221.

Indocyanine green-enhanced transpupillary thermotherapy for juxtapapillary retinal capillary hemangioblastoma.

To study the clinical presentation and treatment outcomes of indocyanine green-enhanced transpupillary thermotherapy (ICG-TTT) for treatment-naïve juxtapapillary retinal capillary hemangioblastoma (JRCH). A prospective interventional case series. The technique involved ICG dye infusion 45 seconds prior to application of TTT. The main study outcomes were local tumor control, resolution of subretinal fluid (SRF), and improvement in best-corrected visual acuity (BCVA). Eight eyes of seven patients (5 males and 2 females) were included. The mean age was 26 years (range: 5-56 years). Systemic evaluation revealed von-Hippel Lindau (VHL) disease in five patients. The most common location was the temporal aspect of the optic disc (5 eyes). The mean basal diameter was 2.9 mm (range: 1-8 mm), and tumor thickness was 1.4 mm (range: 1-4 mm). All eight eyes were treated with multiple sessions of ICG-TTT (mean: 3 sessions). Six eyes received adjuvant intravitreal injection of dexamethasone implant (4 eyes) and/or bevacizumab (4 eyes). Post treatment, six eyes (75%) had tumor regression with reduction of SRF. One eye had a partial response with persisting SRF, and one eye showed poor response to TTT for which external beam radiotherapy was performed. At the last follow-up (median: 11 months; range: 6-29 months), the BCVA remained stable in seven eyes and improved in one eye (hand motion to 20/40). Multiple ICG-TTT sessions can be considered as an alternative treatment option for JRCH with effective local tumor control and SRF resolution.

ZEISS PLEX Elite 9000 Widefield Optical Coherence Tomography Angiography as Screening Method for Early Detection of Retinal Hemangioblastomas in von Hippel-Lindau Disease.

To evaluate early detection of retinal hemangioblastomas (RHs) in von Hippel-Lindau disease (VHLD) with widefield optical coherence tomography angiography (wOCTA) compared to the standard of care in ophthalmologic VHLD screening in a routine clinical setting. We conducted prospective comparisons of three screening methods: wOCTA, standard ophthalmoscopy, and fluorescein angiography (FA), which was performed only in uncertain cases. The numbers of detected RHs were compared among the three screening methods. The underlying causes for the lack of detection were investigated. In 91 eyes (48 patients), 67 RHs were observed (mean, 0.74 ± 1.59 RH per eye). FA was performed in eight eyes. Ophthalmoscopy overlooked 25 of the 35 RHs detected by wOCTA (71.4%) due to the background color of the choroid (n = 5), small tumor size (n = 13), masking by a bright fundus reflex (n = 2), and masking by surrounding retinal scars (n = 5). However, wOCTA missed 29 RHs due to peripheral location (43.3%). The overall detection rates were up to 37% on the basis of ophthalmoscopy alone, up to 52% for wOCTA, and 89% for FA. Within the retinal area covered by wOCTA, the detection rates were up to 46.7% for ophthalmoscopy alone, up to 92.1% for wOCTA, and 73.3% for FA. The overall low detection rate of RHs using wOCTA is almost exclusively caused by its inability to visualize the entire peripheral retina. Therefore, in unclear cases, FA is necessary after ophthalmoscopy. Within the imageable retinal area, wOCTA shows a high detection rate of RHs and therefore may be suitable to improve screening for RHs in VHLD.

Ocular Manifestations of Von Hippel-Lindau Disease: a Rare Sight- Threatening Condition

Introduction : Von Hippel-Lindau (VHL) disease is a rare phacomatosis characterized by multi-organ neoplasia which usually manifests in young adulthood. Ocular manifestations of this disease are frequently presented initially before the involvement of other organ systems.&#x0D; Case Illustration : An 18-year-old girl came with a chief complaint of photopsia in the left eye and complete vision loss in the right eye for five years. The visual acuity of the right eye was no light perception and the left eye was 0.8. The anterior segment of the right eye showed iris neovascularization. The posterior segment of the left eye demonstrated multiple elevated red-yellowish lesions fed by a dilated and tortuous vessel. The head CT scan revealed multiple lesions at the pons and cerebellum. The patient was diagnosed with multiple retinal and brain hemangioblastomas due to VHL disease, neovascular glaucoma, and chronic retinal detachment of the right eye due to sequelae of VHL. The patient was treated with cryotherapy and laser photocoagulation of the left eye. Five-month post-cryotherapy follow-up showed the lesions were still active then she underwent the second cryotherapy. The lesions became whitish in appearance with smaller feeder vessels one month after the second cryotherapy and the visual acuity was preserved.&#x0D; Discussion : Retinal hemangioblastoma is the most common ocular manifestation of VHL disease. Prolonged ischemia causes sight-threatening complications including neovascular glaucoma and exudative retinal detachment. Prompt treatment of detected lesions is crucial for visual preservation.&#x0D; Conclusion : Early detection of VHL ocular manifestations plays a critical role in establishing the diagnosis and initiating treatment for a better prognosis.

Retinal phakomatosis and Von Hippel-Lindau peripheral capillary hemangioblastoma: proposal for staged surgery.

Retinal capillary hemangioblastoma (RCH) is present in about half of the carriers of the VHL (von Hippel-Lindau) gene mutation and can lead to the evolution of blindness. Herewith is a proposal for surgical intraretinal feeder vessel ligature to induce ischemia the phakoma and to resolve the exudative secondary retinal detachment, with satisfying results at the 6 months follow-up end point. The patient underwent a chandelier-assisted 23 G pars plana vitrectomy with valved trocars. A bimanual transretinal ligature of the hemangioblastoma's feeder vessel was performed, followed by localized endolaser to ablate the surrounding retina and capillaries, and a Polydimethylsiloxane 1000 (PDMS 1000) final tamponade. After 3 months, exchange PDMS/air/balanced salt solution (BSS), phacoemulsification, and in-the-bag intraocular lens (IOL) implantation were performed. At sixth month, fundus fluorescein angiography (FFA) showed a significant reduction of blood flow in the phakoma. Final best-corrected visual acuity (BCVA) was 6/6 (9 lines gain obtained compared to the baseline time). This surgical technique seems to be effective and promising for the treatment of VHL retinal capillary hemangioblastomas and their related retinal complications.

Belzutifan for treatment of giant retinal hemangioblastoma with extrascleral extension associated with von Hippel-Lindau syndrome

Abstract Purpose: To describe the clinical response and safety profile of the novel HIF-2ɑ inhibitor belzutifan in treating a giant retinal hemangioblastoma (RHB) with extrascleral extension associated with von Hippel-Lindau syndrome (VHL). Methods: A 71-year-old woman with VHL presented with a giant RHB with extrascleral extension in her only remaining eye. She had no light perception OD and intraocular pressure (IOP) was 48. She requested enucleation due to chronic pain, but, due to concern for significant bleeding given the size of the neoplasm, a trial of belzutifan was initiated. Results: Within three months of treatment initiation, the patient reported an 80% reduction in pain. MRI showed 30% reduction in longest tumor diameter. Dose adjustments were guided by serum hemoglobin levels, allowing the patient to remain on the medication for over a year with continued tumor regression on MRI and avoid enucleation. Conclusion: RHB with extrascleral extension is exceedingly rare and its treatment is complex, often requiring enucleation or external beam radiotherapy. This report demonstrates the use of belzutifan to safely and successfully reduce ocular tumor burden of complicated RHB with extrascleral extension, ultimately decreasing the need for enucleation.

Tumors of the Retina, Optic Disc and Retinal Pigment Epithelium

Vascular tumors of the retina include retinal capillary hemangioma (retinal hemangioblastoma), retinal cavernous hemangioma, retinal racemose hemangioma, and vasoproliferative tumor of the retina. Although they are benign and rare tumors, vascular tumors of the retina are important due to the potential for vision loss through ocular complications, as well as the possibility of morbidity and mortality from accompanying systemic diseases. Ophthalmologists have an important role in diagnosing and implementing necessary screening and treatments. This article will describe the vascular tumors of the retina and optic nerve head.

Current Treatment Approaches for Vascular Tumors of the Retina and Optic Disc

The vascular tumors of the retina and optic disc include retinal hemangioblastoma (capillary hemangioma), retinal cavernous hemangioma, retinal racemose hemangioma (Wyburn-Mason Syndrome) and retinal vasoproliferative tumor. Although retinal vascular tumors are not common, in some cases they can lead to diagnostic and therapeutic challenges. Sector laser photocoagulation, cryotherapy, transpupillary thermotherapy, photodynamic therapy, intravitreal anti-vascular endothelial growth factor (anti-VEGF) therapy, plaque radiotherapy, and endoresection procedures are commonly in the treatment of retinal hemangioblastoma. Cryotherapy, transpupillary thermotherapy, photodynamic therapy, intravitreal anti-VEGF therapy and dexamethasone implants, plaque radiotherapy, and endoresection methods can be applied in retinal vasoproliferative tumor. No treatment in usually necessary in retinal cavernous and racemous hemangiomas.

Surgical and visual outcomes after vitreoretinal surgery for complex retinal capillary hemangioblastoma.

We evaluated the anatomical and functional outcomes after vitreoretinal surgery for complex retinal capillary hemangioblastoma (RCH). This was a retrospective case series of 15 patients (16 eyes) with tractional or combined retinal detachment (RD) managed with pars plana vitrectomy and tumor endoresection (ER) with/without feeder vessel ligation. The mean age at the time of surgery was 30 years (range, 14-46 years). The most common tumor locations were inferotemporal (six eyes) and temporal (six eyes) quadrants. Indications for surgery included exudative RD with fibrovascular proliferation (eight eyes), combined RD (five eyes), vitreous hemorrhage (four eyes), and rhegmatogenous RD (two eyes). Tumor destruction was performed with laser and/or cryotherapy in nine eyes (57%) and ER in seven eyes (43%). Feeder vessel was ligated and cauterized in 10 (63%) and six eyes (37%), respectively. Anatomical success after initial surgery was 50% (eight eyes), which improved to 88% (14 eyes) after they underwent a repeat procedure for recurrent RD (eight eyes). At the last visit, visual acuity improved in seven eyes (44%), was stable in four eyes (25%), and worsened in five eyes (31%) with a mean follow-up of 29 months (6-79 months). Comparison between the ER group and the laser/cryotherapy group revealed no significant difference in final retinal reattachment rate (89% vs. 86%, P > 0.05), with better visual outcomes in laser/cryotherapy group (57% vs. 78%, P < 0.05). Pars plana vitrectomy with/without tumor endoresection can be a safe and effective treatment option for complex RCHs, achieving good tumor control and anatomical success with limited functional success.

Pre-Induction Scalp Block in a Patient of von HippelLindau Disease: Promising Use in High-Risk Neurosurgical Procedures

Multiple modalities have been tried for post-craniotomy pain relief, but controversy exists as to the optimal approach. Pre-induction scalp block is a promising technique in high-risk neurosurgical procedures, which reduces the haemodynamic response to skull pin insertion and craniotomy incision, and affords post-operative analgesia. von hippel-lindau (VHL) disease is a rare autosomal dominant disorder with multi-system involvement and various visceral manifestations, the most common of these-being cerebellar hemangioblastoma. Other anomalies include pancreatic-renal cysts, erythrocytosis, hypernephroma, pheochromocytoma and retinal capillary hemangioblastoma. Central nervous system (CNS) hemangioblastomas are often an early manifestation of the disease, and are typically seen in the second decade of life. The majority of hemangioblastomas occur in the cerebellum followed by the spinal cord and brain stem. They are benign vascular tumours but can cause neurological symptoms secondary to pressure or haemorrhage. We hereby present a case of previously diagnosed VHL with recurrent cerebellar hemangioblastoma scheduled for neurosurgical tumour excision and the beneficial use of a pre-induction scalp block to compliment anaesthesia for the patient.