Retinal Capillary Hemangioma Research Articles

Treatment of retinal capillary hemangioma using 810 nm infrared laser.

Presentation of the efficacy of infrared laser for the treatment of retinal capillary hemangioma (RCH). The treatment and follow-up of nine eyes (fourteen tumors of different sizes and localizations) in seven patients (five children) with RCH. Infrared diode laser (810 nm) was used for modified transpupillary thermotherapy (TTT) in long exposition mode and power between 200 and 1200 mW with a beam diameter of 2 mm (indirect ophthalmoscope, +28 D or +40 D lens) or 0.5 mm-3 mm (slit-lamp) depending on the diameter and localisation of the hemangioma. We achieved complete destruction of the tumor with flat chorioretinal atrophic scar in all cases. Only one tumor regrowth was observed and re-treatment in this case was necessary. Treatment was combined with brachytherapy in a one case. There was one serious complication- total exudative retinal detachment, causing permanent deterioration in visual acuity despite pars plana vitrectomy (PPV). Other complications such as haze and vitreal hemorrhage were transient. The final best corrected visual acuity (BCVA) ranged from 20/20 to counting fingers at 2 feet. Infrared laser can be considered an acceptable therapeutic option for RCH especially for centrally localized lesions. We believe that the role of this therapy will increase in the future.

Hemangioblastoma in the Setting of Von Hippel Lindau Disease: A Case Report

A 21-year-old Chinese gentleman with no known medical illness, presented with a history of right painless blurring of vision with central scotoma of two weeks duration. He also had a history of multiple episodes of seizures prior to presentation. Visual acuity was 1/60 with unremarkable anterior segment findings and no relative afferent pupillary defect. Fundus examination of the right eye revealed dilated and tortuous retinal veins with multiple retinal capillary hemangiomas and sub retinal hard exudates at the macula with edema. A diagnosis of Von Hippel Lindau disease was made when a posterior fossa mass suggestive of hemangioblastoma with obstructive hydrocephalus was seen on computed tomography of the brain. Craniotomy with nodule excision was performed. The retinal capillary hemangiomas were treated with the combination of laser photocoagulation and intravitreal Ranibizumab injections. Visual acuity subsequently improved to 6/36.

PARS PLANA VITRECTOMY IN ADVANCED CASES OF VON HIPPEL-LINDAU EYE DISEASE.

To investigate spectrum of patients with Von Hippel-Lindau disease (VHL) that required pars plana vitrectomy and evaluate anatomical and functional outcomes of surgery. Twenty-three patients who underwent surgery for advanced VHL eye disease were assessed by genetic tests, diagnostic tests for systemic lesions, and clinical eye examination. The vitrectomized eyes were divided into two groups: with or without retinotomy (group R vs. NR). Functional and anatomical outcome was analyzed and compared between the groups. All patients had central nervous system hemangioblastomas and 57% had other systemic tumors. Point germline mutations, large partial deletions, and complete vhl gene deletions were found in 64%, 27%, and 9% of patients, accordingly. Destruction of hemangioblastomas by retinotomy, laser, or cryotherapy and anatomical attachment of the retina were achieved in all eyes. Preoperative mean distance best-corrected visual acuity was logarithm of the minimum angle of resolution 2.66 (20/9,140) in group R and 1.76 (20/1,150) in group NR (P < 0.05). At 6 months postoperatively, distance best-corrected visual acuity improved in 20 eyes (83%). After over 24 months postoperatively, distance best-corrected visual acuity remained better than preoperatively in 36% in the R group and in 70% in the NR group of eyes. During 24 months postoperatively in 17 eyes, new retinal capillary hemangiomas developed. The mean number of new retinal capillary hemangiomas per eye was higher in group R than in group NR (3.14 vs. 0.70; P < 0.01). In group R, number of new retinal capillary hemangioblastoma was higher in retinal segments where retinotomy was performed (n = 29) than in other areas (n = 13) (P < 0.01). Advanced VHL eye disease correlates with occurrence of central nervous system and systemic lesions. Spectrum of vhl gene mutation in the patients corresponds to that of the general VHL population. Pars plana vitrectomy in advanced VHL eye disease can improve or preserve visual function, but postoperative progression of ocular VHL disease can be accelerated in cases where retinotomy is performed.

An Update on the Ophthalmologic Features in the Phakomatoses.

Neurofibromatosis type 1, tuberous sclerosis complex, and Von Hippel-Lindau disease, historically classified as the phakomatoses, are hereditary multisystem disorders characterized by the presence of hamartoma, which carry the risk of malignant transformation. The alteration of tumor suppressor genes seems to be at the basis of their pathophysiogenetic mechanism. Lisch and choroidal nodules in neurofibromatosis type 1, retinal astrocytomas in tuberous sclerosis complex, and retinal capillary hemangioma in Von Hippel-Lindau disease are the principal ophthalmic hamartomatous manifestations. The advent of novel imaging techniques such as near infrared reflectance and optical coherence tomography has provided unprecedented insight on the choroidal and retinal features of these diseases. These methods have improved early diagnosis and the ongoing surveillance in these conditions. Among an array of treatment modalities, antivascular endothelial growth factor therapy has been used in the management of retinal hamartomas but results have been varied. This review is an update on the pathophysiogenetic mechanisms, ophthalmic manifestations, and novel treatment strategies in the phakomatoses with emphasis on the role of imaging techniques.

Optic Nerve Head Hemangioma - An Unusual Presentation of Von Hippel- Lindau Disease

Von Hippel–Lindau (VHL) disease is an autosomal dominant neoplastic disorder in which multiple benign or malignant tumours and cysts with specific histopathologic features develop in the tissues of the central nervous system and in visceral organs. Retinal capillary hemangioma is the most frequent and often the earliest manifestation of VHL disease. However, optic nerve head hemangioma is a rare manifestation. We report a case of optic nerve head haemangioma in an Asian male which on extensive evaluation proved to be a case of VHL disease associated with pheochromocytoma.

Long-Term Results After Proton Beam Therapy for Retinal Papillary Capillary Hemangioma

To evaluate the potential benefit and risk of proton beam therapy in the treatment of symptomatic retinal papillary capillary hemangioma. Retrospective interventional case series. This study included patients presenting with symptomatic exudative retinal papillary capillary hemangioma with or without association with von Hippel-Lindau disease. All patients were treated either as a first or a secondary treatment option by proton beam therapy between 2001 and 2009. The minimum follow-up was 30 months. Eight eyes of 8 patients (3 male and 5 female, with a mean age of 36 years [range 22-80 years]) were treated for symptomatic papillary retinal hemangioma. The median interval between onset of macular edema and proton beam therapy was 1.7 months (range 0.5-3.3 months). The median follow-up period was 84 months (range 32-106 months) between proton beam treatment and last follow-up. Exudation completely resolved in all but 1 patient after 4.2 months on average (range 2.8-7.2 months). Mean visual acuity prior to proton beam irradiation was 0.7 logMAR (0.2 DIN (DIN 58220 norm)) (range 2-0.3 logMAR) and declined to 0.8 logMAR (0.16 DIN; range 2-0.1 logMAR) at last follow-up examination (no statistical significance, P=.071). The anatomic outcome after proton beam therapy for retinal papillary hemangioma is convincing, whereas functional outcome may be compromised because of tumor location, long-persisting macular edema, extensive exudation, and poor initial visual acuity. In patients with extended retinal detachment surgical intervention was still necessary. Although proton beam therapy is proven to be a therapeutic option, treatment will remain challenging.

Renal tumor size is an independent prognostic factor for overall survival in von Hippel–Lindau disease

To evaluate the effect of renal cell carcinoma (RCC) on survival in von Hippel-Lindau (VHL) disease and to assess the relationship between tumor size and survival. In this retrospective cohort study, the medical records of 72 patients who presented with VHL disease between 1994 and 2012 were reviewed. Clinical VHL-related characteristics were analyzed, and the prognostic value of renal tumor size for overall survival was assessed by using Cox regression models. Of the 72 VHL patients, 42 (58.3 %) and 30 (41.7 %) were male and female, respectively. The mean age was 37.9 years, and the median follow-up period was 61.5 months. In terms of VHL-related manifestations, 40 (55.6 %) had RCC, 46 (63.8 %) had hemangioblastoma in the cerebellum, 10 (13.9 %) had hemangioblastoma in the spinal cord, 34 (47.2 %) had a pancreatic mass, 18 (25.0 %) had pheochromocytoma, and 14 (19.4 %) had retinal capillary hemangioma. RCC was a major cause of mortality: Of the 11 patients who died, nine (12.5 %) died due to RCC progression. The 5-year overall survival rate was 85.6 % for all patients, 96.9 % for patients without RCC, 83.6 % for patients with RCC < 3 cm, and 75.8 % for patients with RCC ≥ 3 cm. Multivariable analysis showed that RCC ≥ 3 cm was an independent predictor of overall survival (HR 9.87, 95 % CI 1.17-83.00, p = 0.035) along with age (HR 1.05, 95 % CI 1.01-1.10, p = 0.027). Renal tumor size was an independent prognostic factor for overall survival in VHL disease. This observation will be helpful for planning RCC treatment in VHL disease.

Massive exudative retinal detachment following photodynamic therapy for retinal hemangioma in von Hippel–Lindau Syndrome

Photodynamic therapy (PDT) is a common treatment on retinal capillary hemangioma. We applied PDT to a patient with von Hippel-Lindau(VHL) syndrome and she developed severe massive exudative retinal detachment the next day, which is a rare complication for PDT. After intraocular anti-VEGF agent and peribulbar dexamethasone several times to the patient, her subretinal fluid disappeared and hemangiomas atrophied. Treatment with Anti-VEGF agent and corticosteroid is effective for such complication.

A Case Report of Primary Vitrectomy in Multiple Bilateral Retinal Capillary Hemangiomas Accompanying Epiretinal Membrane

Purpose: To report the clinical result of pars plana vitrectomy (PPV) as the primary treatment in a patient with multiple bilateral retinal capillary hemangiomas (RCH) accompanying epiretinal membrane (ERM). Case summary: A 17-year-old female patient visited our clinic for decreased bilateral vision 4 days in duration. At the first visit, the vision in her right eye was 20/25 and, 20/100 in her left eye. Bilateral multiple retinal capillary hemangiomas were observed at the peripheral retina in both eyes on fundus examination, fluorescein angiography (FAG) and optical coherence tomography (OCT). ERM and associated macular edema (ME) were noted in her left eye. For her left eye, focal laser photocoagulation (PC) was performed 6 times. ME was decreased and her vision improved to 20/25. However, 3 months after the initial visit, her vision decreased to 20/400 accompanied with exacerbated ERM and ME. Therefore, PPV was performed in her left eye. ERM and associated ME were decreased and her vision improved to 20/25 after 18 months. PPV was performed in the right eye immediately after her vision worsened (best corrected visual acuity, BCVA 20/40) and ERM occurred. After 15 months later, vision in her right eye improved to 20/20 and ERM was also decreased. J Korean Ophthalmol Soc 2014;55(6):928-935

Photodynamic Therapy for Juxtapapillary Retinal Capillary Hemangioma

Various treatment modalities have been described for retinal capillary hemangioma. Our purpose is to present a case of juxtapapillary retinal capillary hemangioma treated with photodynamic therapy. A 69-year-old woman with no previous ocular history presented with blurred vision and photopsias in the right eye three months ago. At presentation, her best corrected visual acuity was 6/9 in the right eye and 6/6 in the left eye. The anterior segment was totally normal and IOP was normal in both eyes as well. Dilated fundoscopy revealed a yellowish, well-circumscribed, elevated area with blood vessels, on the inferior margin of the right optic disc, as optic disc edema. Fluorescein angiography and angiogram with indocyanine green confirmed the diagnosis of juxtapapillary retinal capillary hemangioma. The patient was treated with photodynamic therapy with verteporfin and three months later her visual acuity was 6/7.5 in the right eye, while the lesion was slightly smaller. These findings remained stable at the one-year follow-up. In conclusion, photodynamic therapy offers promising anatomical and functional results for juxtapapillary retinal capillary hemangioma, providing visual acuity improvement or even stabilization and restriction of enlargement of the lesion.

Persistent exudative retinal detachment after photodynamic therapy and intravitreal bevacizumab injection for multiple retinal capillary hemangiomas in a patient with von Hippel–Lindau disease

Photodynamic therapy (PDT) has been used in treating peripheral retinal capillary hemangioma (RCH) with satisfactory results. We report a rare case of von Hippel-Lindau (VHL) disease with three large peripheral RCHs, treated with PDT and intravitreal bevacizumab injection (IVB), who developed persistent bullous exudative retinal detachment (RD) despite significant tumor regression. The patient is a sporadic case of VHL disease, with a de novo nonsense mutation in codon 161 with C → T transition at nucleotide position 694 of the VHL gene. Multiple RCHs were noted in both eyes. Four small RCHs were found in the left eye and were treated with laser photocoagulation. Three large RCHs in the peripheral retina of the right eye were complicated with cystoid macular edema and subretinal fluid accumulation. The RCHs were treated with PDT combined with IVB, and bullous exudative RD developed on the second day after treatment. Three months after PDT, the tumors had regressed significantly, but exudative RD persisted, despite multiple IVB and intravitreal triamcinolone acetonide injection (IVTA). External drainage with sclera buckling, IVB, and IVTA were performed, and the retina attached after surgical intervention. The application of PDT in the treatment of RCHs and its possible complications are discussed.

Photodynamic therapy for juxtapapillary retinal capillary hemangioma: A case report

AbstractPurpose Various treatment modalities have been described for retinal capillary hemangioma therapy, including observation, laser photocoagulation, cryotherapy, radiotheraphy and vitreoretinal surgery. Our purpose is to present a case of juxtapapillary retinal capillary hemangioma treated with photodynamic therapy(PDT.Methods A 69‐year‐old woman with no previous ocular history presented with blurred vision and photopsias in the right eye since three months. The patient underwent a thorough ophthalmological examination, including visual acuity measurement by means of Snellen charts, slit‐lamp examination, intraocular pressure(IOP) measurement and dilated fundoscopy.Results At presentation, her best corrected visual acuity (BCVA) was 6/9 in the right eye and 6/6 in the left eye. The anterior segment was totally normal and IOP was normal in both eyes as well. Dilated fundoscopy revealed a yellowish, elevated area with blood vessels, on the inferior margin of the right optic disc, as optic disc edema. Fluorescein angiography and angiogram with indocyanin green confirmed the diagnosis of juxtapapillary retinal capillary hemangioma. The patient was treated with PDT with verteporfin and three months later her visual acuity was 6/7.5 in the right eye and the lesion was slightly smaller.Conclusion Retinal capillary hemangioma should be included in the differential diagnosis of optic disc edema. PDT offers promising results as far as anatomical and functional results, providing visual acuity improvement or even stabilization and restriction of enlargement of the lesion.

Transvitreal Endoresection of Refractory Retinal Capillary Hemangioblastoma After Feeder Vessel Ligation

Retinal capillary hemangioma (RCH) is a developmental vascular tumor occurring sporadically or associated with Von Hippel-Lindau syndrome. Treatment for large solitary RCH is challenging. Laser, cryotherapy, radiotherapy, photodynamic therapy, and en bloc resection for advanced cases have been described in the literature without consensus on standard management. A novel technique of 20-gauge transvitreal endoresection of solitary peripheral RCH after ligating the feeder vessels is described. Tumors were successfully resected after ligating the feeder vessels bimanually in a closed and more controlled ocular environment. Feeder vessel ligation is intended to compensate for the involution time of the caliber of feeder vessels, when postoperative hemorrhage is more likely to occur. Another advantage of ligating the blood vessels is to secure hemostasis without using heavy endocautery. Postoperatively, proliferative vitreoretinopathy remains a concern. Pending a larger randomized trial, performing endoresection in RCH that is large and unresponsive to conventional treatment is an acceptable option.

Photodynamic therapy for retinal capillary hemangioma

To describe the results of photodynamic therapy (PDT) for juxtapapillary and peripheral retinal capillary hemangioma (RCH). Interventional case series of four eyes (four patients) with juxtapapillary RCH and one eye (one patient) with peripheral RCH. Two eyes with juxtapapillary RCH had received two sessions of full-fluence, double-duration PDT; whereas other two eyes had received single session of half-fluence, single-duration PDT. The peripheral RCH was treated with a single session of full-fluence, single-duration PDT. Two patients had von Hippel-Lindau disease. Follow-up duration ranged from 4 months to 1 year. Pre-PDT visual acuity (VA) ranged from 20/200 to HM (juxtapapillary RCH) and 20/100 (peripheral RCH). Among the eyes with juxtapapillary RCH, tumor regression with partial resolution of macular edema was noted in two eyes (one eye each with half-fluence and full-fluence PDT), whereas two eyes had no change in tumor size with persistent macular edema. VA remained stable in three eyes and declined in one eye. In an eye with peripheral RCH, regression of tumor and macular edema with VA improvement was noted. Post-PDT complications included epiretinal membrane (one eye) and transient exudative retinal detachment (one eye). PDT can be effective in reducing macular edema associated with RCH but this does not always correspond with an improvement in VA especially for juxtapapillary tumors.

A case of vasoproliferative retinal tumor complicated by neovascular glaucoma.

To report a case of vasoproliferative retinal tumor complicated by neovascular glaucoma that showed remission after vitrectomy surgery to remove the tumor. A 78-year-old man was referred to the hospital with a complaint of visual loss in his right eye. Corrected visual acuity was 0.5 in the right eye and 1.2 in the left eye. The right eye showed mild vitreous hemorrhage and an elevated lesion in the lower fundus. Fluorescein angiography demonstrated leakage from the tumor, and a 4-mm lesion of high density was seen on computed tomography, and we diagnosed it as retinal capillary hemangioma. In this case, von Hippel-Lindau disease was negative. During the course of the disease, peripheral anterior synechia and neovascularization was seen in the iris and angle, and vitreous surgery was enforced to remove the tumor because of increased intraocular pressure. The tumor was removed as almost one mass. Histologically, the tumor was composed of glial cells and small vessels proliferation. We diagnosed vasoproliferative retinal tumor. Seven months after surgery, the intraocular pressure stabilized, and neovascularization of the iris and angle disappeared. Visual acuity was 0.03 due to epiretinal membrane and optic atrophy. There were no signs of recurrence. The vitreous surgery is one of the effective treatment methods against vasoproliferative retinal tumor complicated by neovascular glaucoma.

Massive exudative retinal detachment after photodynamic therapy for peripheral retinal capillary hemangioma.

To report a case requiring emergency surgical intervention to treat a progressive massive retinal detachment after photodynamic therapy for a peripheral retinal capillary hemangioma associated with von Hippel-Lindau disease. A 38-year-old woman with von Hippel-Lindau disease had a peripheral retinal capillary hemangioma in the left eye. At the initial visit, the best-corrected visual acuity was 20/250 in the left eye. Photodynamic therapy was applied to treat the hemangioma. After 1 day, a massive retinal detachment and vitreous hemorrhage occurred. Three days after photodynamic therapy, vitrectomy was performed to treat the retinal detachment, which progressed with visual deterioration to hand motions. A diagnosis of exudative retinal detachment was made because no retinal breaks were identified. The retina was reattached with external drainage and gas tamponade. One month postoperatively, the best-corrected visual acuity recovered to 20/100 in the left eye, and the vascular activity of the hemangioma stabilized. Photodynamic therapy monotherapy to treat retinal capillary hemangioma might result in a massive exudative retinal detachment requiring surgery.

Intravitreal anti-VEGF injection for the treatment of progressive juxtapapillary retinal capillary hemangioma: a case report and mini review of the literature

We report a case of a patient known to have a von Hippel–Lindau disease with documented progressive juxtapapillary retinal capillary hemangioma (JRCH) with well-preserved visual acuity (VA) and visual field (VF). The patient received a single injection of intravitreal ranibizumab (IVR). Six months after IVR injection, the JRCH showed reduced vascularization, fibrosis, and mild shrinkage, and VA and VF remained unchanged. IVR therapy might therefore be considered as an alternative treatment for progressive JRCH, especially in patients with well-preserved VA and VF.

The Role of Photodynamic Therapy in Non-malignant and Malignant Eye Disorders

Photodynamic therapy (PDT) has represented an important treatment modality in many ocular disorders for more than a decade. The introduction of verteporfin-PDT to the treatment of neovascular age-related macular degeneration and polypoidal choroidal vasculopathy has rescued millions of patients from vision loss and blindness around the world. The most well-known vascular tumors in the eye treated with PDT include retinal capillary hemangioma, choroidal hemangioma, retinal vasoproliferative tumor or Wyburn-Mason syndrome. The discovery of the role of vascular endothelial cell growth factor in age-related macular degeneration and the revolution of its treatment by anti-vascular endothelial cell growth factor agents reduced the need for PDT in age-related macular degeneration; however, the use of PDT in the treatment of PCV is dramatically increasing. Furthermore, clinical results obtained with PDT, in combination with angiogenesis inhibitors, vascular disrupting agents, or/and anti-inflammatory compounds as adjuvant therapies, may well keep PDT as one the main treatment options. This review summarizes the present role and future possible improvements of ocular PDT.

A case of von Hippel–Lindau disease with juxtapapillary retinal capillary hemangioma and nutcracker phenomenon

The aim of this study was to evaluate a patient with von Hippel-Lindau (VHL) syndrome and to discuss the clinical manifestations. A 25-year-old woman presented to our clinic with blurred vision for 2 months. Best corrected visual acuity in right eye was 20/50, and fundoscopic examination revealed juxtapapillary endophytic retinal capillary hemangioma. Examination findings on left eye were normal. On optical coherence tomography, exudation was prominent on macula. History revealed cerebellar operation because of cerebellar hemangioblastoma. On abdominal ultrasonography, liver hemangiomas and pancreatic cysts were seen, confirmed by abdominal computerized tomography (CT). Abdominal CT imaging also revealed nutcracker phenomenon. Transthoracic echocardiography showed atrial septal aneurysm without shunt. The patient refused ocular treatment. At 3-month control, ophthalmic findings were the same. To our knowledge, this is the first case of VHL disease reported to be associated with nutcracker phenomenon and atrial septal aneurysm. Systemic evaluation and regular follow-up should be recommended to subjects with VHL disease.

Von Hippel–Lindau disease: Family study

Case reportThe case of five members of a family who suffer from Von Hippel–Lindau disease (VHL) is presented. It is shown that retinal capillary hemangioma was the main ophthalmic symptom, with hemovitreous and tractional retinal detachment as the main complications. DiscussionVHL disease is rare, but very serious, even fatal, and thus it is important to obtain an early diagnosis by ophthalmoscopy, in order to change the visual prognosis and life expectancy, not only for the patient, but also for the family.