Low-dose proton radiotherapy for pediatric choroidal hemangioma: A case series.

Management of pediatric choroidal hemangioma complicated by large exudative retinal detachment can be challenging, with few options available. Limited data have been published on outcomes following proton radiotherapy (PRT) for management of these patients. In this retrospective case series, nine patients were treated with a low-dose PRT regimen of 20 Gy(relative biological effectiveness [RBE]) in 10 fractions, and two were treated with 15 Gy(RBE) in four fractions. Visual acuity improved in seven patients (64%) and remained stable in the remaining four (36%). In patients with imaging follow-up (10 patients), subretinal fluid resolved in nine patients (90%) and tumor thickness decreased or remained stable in 10 (100%). Complications were observed in eight of 11 patients (73%). One patient developed grade 2 cataract; otherwise, no grade ≥2 complications were observed.

Consensus Statement for the Management and Treatment of Port-Wine Birthmarks in Sturge-Weber Syndrome.

Importance: Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome involving the skin, brain, and eyes. Consensus recommendations for management are lacking. Objective: To consolidate the current literature with expert opinion to make recommendations that will guide treatment and referral for patients with port-wine birthmarks (PWBs). Evidence Review: In this consensus statement, 12 nationally peer-recognized experts in dermatology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included risk stratification, optimum treatment strategies, and recommendations regarding light-based therapies. A systematic PubMed search was performed of English-language articles published between December 1, 2008, and December 1, 2018, as well as other pertinent studies identified by the expert panel. Clinical practice guidelines were recommended. Findings: Treatment of PWBs is indicated to minimize the psychosocial impact and diminish nodularity and potentially tissue hypertrophy. Better outcomes may be attained if treatments are started at an earlier age. In the US, pulsed dye laser is the standard for all PWBs regardless of the lesion size, location, or color. When performed by experienced physicians, laser treatment can be safe for patients of all ages. The choice of using general anesthesia in young patients is a complex decision that must be considered on a case-by-case basis. Conclusions and Relevance: These recommendations are intended to help guide clinical practice and decision-making for patients with SWS and those with isolated PWBs and may improve patient outcomes.

Management of diffuse choroidal hemangioma in Sturge-Weber syndrome with Ruthenium-106 plaque radiotherapy.

PURPOSE: To evaluate Ruthenium-106 plaque radiotherapy in the treatment of diffuse choroidal hemangioma (DCH) associated with serous retinal detachment. METHODS: A retrospective analysis was performed in five patients treated for DCH associated with Sturge-Weber syndrome (SWS). In all cases, Ruthenium-106 plaque therapy with a target apex dose of 30.98-47.36 Gy (mean:38.9 Gy) was performed. The outcomes of treatment were regression of DCH, assessed by B-scan ultrasonography; resolution of serous retinal detachment, measured by B-scan ultrasonography and optical coherence tomography (OCT); changes in best corrected visual acuity (BCVA) and the development of radiation-related complications. All investigations were repeated 3 months after treatment and then at six monthly intervals within 22-122 months (mean: 62 months) of follow-up. RESULTS: The initial BCVA of the affected eyes ranged from counting fingers at 1 m to 0.1 by the Snellen chart. Mean tumor basal diameter was 16.7 mm (range: 13.8 to 18.5 mm) and mean tumor thickness was 4.4 mm (range: 2.4 to 5.8 mm). Tumor regression was found in all cases with the prompt resolution of subretinal fluid. In three patients, BCVA improved and in two it remained stable. During the follow-up period, in one case secondary glaucoma was treated with transscleral cyclophotocoagulation, and in another case, recurrence of the hemangioma was treated with repeated Ruthenium-106 plaque irradiation and transpupillary therapy. CONCLUSION: Ruthenium-106 plaque radiotherapy is an effective and safe treatment option for DCH associated with SWS. Brachytherapy led to tumor regression and resolution of serous retinal detachments, and visual stabilization was achieved in most cases.

Standard fractionation low-dose proton radiotherapy for diffuse choroidal hemangiomas in pediatric Sturge-Weber syndrome.

Sturge-Weber syndrome is a nonhereditary congenital neurocutaneous syndrome characterized by leptomeningeal angiomatosis, facial nevus flammeus, and diffuse choroidal hemangioma, which when complicated by total retinal detachment, portend a poor prognosis. Management is often limited to salvage external beam irradiation. We present a modified proton therapy technique for young children with total bullous retinal detachments that uses standard fractionation low-dose proton radiotherapy to decrease the risk of radiation complications. Treatment techniques for young children who cannot cooperate with conventional radiation protocols are also described.

Sturge-Weber syndrome and dermatomal facial port-wine stains: incidence, association with glaucoma, and pulsed tunable dye laser treatment effectiveness.

BACKGROUND: Laser therapy is the optimal approach for treating port-wine stains, but whether it is effective for patients with facial dermatomal port-wine stains and Sturge-Weber syndrome is undetermined. This project aimed to verify the incidence of Sturge-Weber syndrome and glaucoma within the population, compare the response in syndromic and nonsyndromic patients with facial dermatomal port-wine stains to laser, and determine the treatment response in relation to dermatome and color. METHODS: The authors retrospectively reviewed 874 patients with facial port-wine stains (203 displayed a dermatomal pattern). Pretreatment and posttreatment photographs were assessed clinically and recorded on a statistically reliable percentage gradient. RESULTS: Sturge-Weber syndrome was diagnosed in 30 patients. Twelve patients had glaucoma, with the port-wine stains involving the ophthalmic (V1) division of the trigeminal nerve. The highest proportion of patients with glaucoma came from those with involvement of the maxillary (V2) and/or mandibular (V3) division of the trigeminal nerve. No patients had Sturge-Weber syndrome with V3 port-wine stains alone. After laser therapy, only 45 percent of syndromal patients and 55 percent of nonsyndromic patients had a satisfactory outcome (>50 percent) in color and size reduction of dermatomal port-wine stains. CONCLUSIONS: The incidence of Sturge-Weber syndrome was 3 percent in patients with a facial port-wine stain. There was an increased risk of Sturge-Weber syndrome with involvement of V1 port-wine stains and no risk with involvement of V3 port-wine stains alone. Laser treatment produced unsatisfactory outcomes in patients with facial dermatomal port-wine stains. V3 port-wine stains responded best and V2 worst to laser.

Proton or photon irradiation for hemangiomas of the choroid? A retrospective comparison.

PURPOSE: The aim of this study was to compare, on a retrospective basis, the results of therapy in patients with uveal hemangioma treated with photon or proton irradiation at a single center. METHODS: From 1993 to 2002 a total of 44 patients were treated. Until 1998 radiotherapy was given with 6 MV photons in standard fractionation of 2.0 Gy 5 times per week. In 1998 proton therapy became available and was used since then. A dose of 20 to 22.5 Cobalt Gray Equivalent (CGE) 68 MeV protons was given on 4 consecutive days. Progressive symptoms or deterioration of vision were the indications for therapy. RESULTS: Of the 44 patients treated, 36 had circumscribed choroidal hemangiomas and 8 had diffuse choroidal hemangiomas (DCH) and Sturge-Weber syndrome. Of the patients, 19 were treated with photons with a total dose in the range of 16 to 30 Gy. A total of 25 patients were irradiated with protons. All patients with DCH but 1 were treated with photons. Stabilization of visual acuity was achieved in 93.2% of all patients. Tumor thickness decreased in 95.4% and retinal detachment resolved in 92.9%. Late effects, although generally mild or moderate, were frequently detected. In all, 40.9% showed radiation-induced optic neuropathy, maximum Grade I. Retinopathy was found in 29.5% of cases, but only 1 patient experienced more than Grade II severity. Retinopathy and radiation-induced optic neuropathy were reversible in some of the patients and in some resolved completely. No differences could be detected between patients with circumscribed choroidal hemangiomas treated with protons and photons. Treatment was less effective in DCH patients (75%). CONCLUSIONS: Radiotherapy is effective in treating choroidal hemangiomas with respect to visual acuity and tumor thickness but a benefit of proton therapy could not be detected. Side effects are moderate but careful monitoring for side effects should be part of the follow-up procedures.

[Sturge-Weber syndrome: medical management of choroidal hemangiomas]. / Le syndrome de Sturge-Weber: prise en charge thérapeutique des hémangiomes choroïdiens.

PURPOSE: To investigate the outcome of irradiation of complicated choroidal hemangiomas in Sturge-Weber syndrome. PATIENTS AND METHODS: The charts of 6 patients (7 eyes) with Sturge-Weber syndrome and choroidal hemangiomas were reviewed. An exudative retinal detachment was the indication for treatment in all cases. The mean age of the 6 patients was 13 years (range, 4 to 20 years). The minimum follow-up time was 1 year. Patients were checked for initial and final best-corrected visual acuity, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. The patients were treated with radiotherapy. A total dose of 20 Grays was applied to 7 eyes: 2 with a circumscribed choroidal hemangioma underwent proton therapy and 5 with diffuse hemangioma were treated by external beam irradiation. RESULTS: Complete resolution of the subretinal fluid was achieved in all cases with the tumor height decreased. Visual acuity improved to 1 line or more in 5 eyes and remained stable in 2 eyes. Two cases that underwent proton therapy developed radiation retinopathy. CONCLUSION: External beam radiation is an effective and safe option in the management of choroidal hemangiomas complicated by retinal detachment. Based on our experience, proton therapy should be reserved for sporadic circumscribed choroidal hemangioma.

Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge-Weber syndrome.

PURPOSE: To report bilateral choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome. METHODS: Case report. RESULTS: A 6-year-old male with a left facial nevus flammeus and a history of a left cerebral angioma had been followed 2 years for increasing esotropia. Examination demonstrated bilateral diffuse choroidal hemangiomas with overlying exudative retinal detachments. After bilateral external beam radiotherapy, the retinal detachments resolved and vision improved. CONCLUSION: Patients with Sturge-Weber syndrome and unilateral facial nevus flammeus may harbor bilateral choroidal hemangiomas. Clinical manifestations of the Sturge-Weber syndrome are characteristically unilateral and ipsilateral to the facial nevus flammeus. Bilateral choroidal hemangiomas associated with bilateral facial nevus flammeus are rare. 1,2 We report bilateral diffuse choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome.

Long-term results after low dose ocular irradiation for choroidal haemangiomas.

AIM/BACKGROUND: The most common choice of treatment for choroidal haemangiomas (CH) in the past has been the employment of scatter photocoagulation of the surface. This management often requires repetitive treatment or additional invasive management due to massive exudative detachment of the retina. The aim of this retrospective study was to investigate the outcome of the alternative application of low dose external beam irradiation with high energetic photons on these tumours. METHODS: A total absorbed dose of 20 Gy was applied to a total of 51 symptomatic eyes: 36 with a circumscribed CH of the posterior pole and 15 with diffuse CH as part of the Sturge-Weber syndrome. The indication for treatment was an exudative retinal detachment including or threatening the fovea. The mean follow up times in each group were 4.5 and 5.3 years, respectively. Out of a group of 33 patients from whom reliable data could be derived, 17 had symptoms lasting longer than 6 months. RESULTS: In 23 cases (63.8%) with circumscribed CH complete resolution of the subretinal fluid was achieved; the remaining 13 cases (36.2%) showed residual serous detachment distant to the fovea. The visual acuity improved by two or more lines in 14 cases (38.9%), remained stable in 14 cases (38.9%), and decreased in eight cases (22.2%). The functional success was dependent on the lag duration between onset of first subjective symptoms and treatment. The morphological results with diffuse CH were similar to those of the group of circumscribed CH. The visual acuity (VA) at last examination was improved in seven cases (46.6%); in the remaining eight cases, VA was unchanged or had deteriorated. The poor functional outcome in the latter was mainly attributable to secondary glaucoma. CONCLUSION: External beam irradiation is a useful and a low invasive therapeutic option for CH. A successful functional outcome is dependent on the time delay between first onset of symptoms and the beginning of therapy, the formation of subretinal fibrosis, and also on secondary glaucoma in the case of Sturge-Weber syndrome.

[Successful treatment of choroid hemangioma with secondary changes caused by Sturge-Weber syndrome]. / Die erfolgreiche Behandlung eines Aderhauthämangioms mit Sekundärveränderungen bei Sturge-Weber-Syndrom.

A 12 year old patient was treated for Sturge-Weber syndrome with choroidal haemangioma and total exsudative retinal detachment. The diagnosis was supported by echography and thin-layer computed tomography. Because of the exsudative retinal detachment photocoagulation could not be performed. Treatment by percutaneous radiotherapy with Caesium 137 was performed. The retina was reattached by application of 30 Gray. A flat pigmented scar developed in the area of the haemangioma at the posterior pole of the eye. No side effects appeared. The follow-up time is 26 months.

Basal cell carcinoma developing in a nevus flammeus.

A 44-year-old man with Sturge-Weber syndrome developed a basal cell carcinoma at the site of a nevus flammeus on his right cheek, which had been treated with a course of grenz rays at six years of age without obvious improvement. He also exhibited erythematous papulovesicular lesions on the upper trunk, which histologically proved to be transient acantholytic dermatosis. The association of nevus flammeus and basal cell carcinoma is extremely rare; to the best of our knowledge, only four similar cases have been previously reported.

[Radiotherapy of the hemangioma of the choroid]. / Zur Strahlentherapie des Aderhauthämangioms.

For the first time, a report is given on the percutaneous radiotherapy of hemangiomas of the choroid which could not be treated by photocoagulation. Diagnosis and control examinations of these tumors were performed by ophthalmoscopy, fluorescence angiography, and ultrasonography. Ten eyes (nine patients, three out of them with Sturge-Weber's disease) were treated between 1967 and 1982. A first group (five eyes, five patients) treated until 1974 received a target volume dose of 1,65 to 6,5 Gy combined with subsequent photocoagulation. All these eyes could be preserved. In addition, the second group (five eyes, four patients) treated since 1975 with unique radiotherapy showed better results with respect to visual acuity. The average follow-up period was four years (seven months up to nine years). Late effects were not observed. Even in case of advanced disease with retinal ablation and secondary glaucoma, an inactivation of the hemangioma of the choroid and a simultaneous regression of the secondary alterations could be achieved. According to our experiences, enucleations can be prevented by an irradiation with a target volume dose of 20 to 30 Gy.