Treatment review of sight threatening circumscrib

　　 作者：James FT Li Yim1， Teresa Sandinha1， Jan M Kerr1， Diana Ritchie2， Ewan G Kemp1

【摘要】 To describe our clinical experience in treating circumscribed choroidal haemangioma (CCH) in a tertiary referral centre over a fifteen year period prior to photodynamic therapy.METHODS: The departmental database and photographic records of a tertiary referral center were used to identify patients who were treated for CCH between 1992 and 2007. Their case records were reviewed.RESULTS: Visual acuity improved (&>2 Snellen lines) in eleven patients (69%) remained stable in one patient (6%) and deteriorated in four patients (25%). Six of the seven treated with brachytherapy and three of the four treated with transpupillary thermotherapy achieved better visual acuity after treatment. 86% of patients treated within six months of onset of symptoms and 50% of patients treated after six months of onset of symptoms noted an improvement in visual acuity. Only one patient in our series had a final VA of 6/60 or worse. Mean followup was thirtyfive months.　CONCLUSION: Visual outcome is better when treatment is performed within 6 months of symptoms. The majority of patients achieved an improvement in visual acuity without any adverse effect following treatment.

【关键词】 circumscribed choroidal haemangioma; plaque brachytherapy; transpupillary thermotherapy

　INTRODUCTION

　　Choroidal haemangioma are benign vascular tumours that can be classified as circumscribed or diffused， the latter occurring in association with the SturgeWeber syndrome. Clinically， a circumscribed choroidal haemangioma (CCH) appears as an amelanotic orangered elevated mass on funduscopy. Ultrasound findings are high internal reflectivity on Ascan， dome shaped elevation and acoustic solidity on Bscan. Fluorescein angiography typically shows hyperfluorescence in the early prearterial phase and staining in the late phase. CCH are rare and can remain asymptomatic. However， progressive enlargement may occur[13] with ensuing visual impairment caused by exudative retinal detachment， retinal oedema， RPE changes or choroidal fibrosis. Many treatments have been used for symptomatic CCH such as cryotherapy， xenon arc or argon photocoagulation(PC)[4]， transpupillary thermotherapy(TTT)[58]， brachytherapy (PL)[912]， proton beam therapy(PBT)[1315]， external beam radiotherapy (EBT)[12，16]， stereotactic radiotherapy[17] and more recently photodynamic therapy (PDT)[1823]. The treatment modalities previously used in our department for CCH were: transpupillary thermotherapy and radiotherapy in the form of ruthenium 106 scleral plaque brachytherapy， proton beam therapy and external beam therapy.

　　Transpupillary thermotherapy uses a diode laser emitting at 810nm with a broad beam and raises the temperature in the treated tissue to 65℃ at the apex and 40℃ internally. This is the critical temperature that induces ischemic necrosis. Complications include cystoid macular oedema， preretinal fibrosis and focal iris atrophy[5]， and possibly branch retinal vascular occlusion[24]. Low dose radiation， less than or equal to 20Gy appears successful in controlling both circumscribed and diffuse choroidal haemangiomas associated with progressive retinal detachment. Lens sparing treatment avoids the risk of cataract. External beam therapy and brachytherapy have not been directly compared but brachytherapy appears to be effective for selected well circumscribed lesions. With brachytherapy the radiation dose is prescribed to the apex of the tumour. This results in corresponding higher surface dose due to exponential attenuation of radiation dose at depth. Treatment depth of 5mm results in a maximum surface dose in the order of 50Gy and very low risk of any late radiation toxicity except for juxtapapillary tumours where there is a risk of radiation dose to the optic disc and proximal optic nerve. For these cases external beam radiation is preferable. Complications of radiation treatment that have been reported are: radiation retinopathy[13] and cataract[25].

　　Photodynamic therapy (PDT) is currently the treatment for posteriorly located CCH. However， the long term results are not yet known and anteriorly located lesions cannot be targeted with PDT. The aim of this paper is to describe our clinical Table 1 CCH outcome following TTT or PL treatment

　　AuthornTreatmentVA improvement

　　(≥2 Snellen lines)(%)Mean followup(months)Current study4TTT7535GarciaArumi et al[6]8TTT5012Kamal et al[7]6TTT/ICG67212Fuchs et al[8]10TTT40313Gündüz et al[5]10TTT50844Current study7Ruthenium 106(Ru 106)7135Madreperla et al[11]8*Ru 1067525Aizman et al[9]5Palladium 1036019Zografos et al[10]31Cobalt 608124

　　*Two patients treated with Iodine 125 plaque

　　Table 2 Published series of visual outcomes for treatment of CCH

　　AuthornTreatmentImproved VA (%)Worse VA (n)VA &< 6/60 (n)Mean followup

　　(months)Zografos et al[10]31Cobalt PL8119624Hannouche et al[13]13PBT6203126Madreperla et al[11]23PC ， PL(8) ， EBT619NA66Schilling et al[16]36EBT3922NA54Lee et al[14]3PBT330224Zografos et al[15]31PBT71NA312Shields et al[26]96PC，TTT(3)，PL(15)，EBT(2)8630623*Aizman et al[9]5Palladium PL600119Current study16TTT， PL， PBT， EBT694135

　　*Patients followed up for at least 3 months， mean followup not available

　　experience in treating sight threatening circumscribed choroidal haemangioma with other modalities from 1992 to 2007.

　　MATERIALS AND METHODS

　　SubjectsTwentyone patients were identified with a diagnosis of CCH. Two of them were lost to follow up; three did not require any treatment and remain under review. Sixteen patients received treatment (eleven males and five females; age range 1475 years， mean 45 years old). Seven had brachytherapy， four had transpupillary thermotherapy， one proton beam therapy， two external beam therapy， one TTT followed by PL， 1 EBT followed by TTT. The most common indication for treatment was decreased visual acuity (twelve patients). Other indications were: progressive field defect in two patients and increase in size of CCH in one patient. The pretreatment visual acuity ranged from 1/120 to 6/5. After a mean followup of 35 months， bestcorrected posttreatment visual acuity ranged from 1/60 to 6/5.

　　Methods We performed a retrospective review of patients case records diagnosed with CCH between 1992 and 2007. Patients were identified through the departmental database and photographic records. Case records were reviewed for data collection. Location of CCH， indication for treatment， duration of symptoms prior to diagnosis， visual acuity (VA) pretreatment and best posttreatment， time interval from diagnosis to treatment and treatment modalities were recorded.

　　This cohort of patients was referred to our tertiary centre with a suspicious choroidal lesion or a CCH requiring further treatment not available locally. Diagnosis of CCH is made clinically following fundus examination， B scan ultrasonography and fluorescein angiography. Treatment is recommended when there is visual impairment or threat due to tumor location and associated subretinal fluid if present.

　　Choice of treatment was based on accessibilityTTT for posterior pole lesion， PL for anteriorly located lesions not accessible with TTT up to 5mm thick and PBT or EBT for lesions thicker than 5mm. TTT and PL were administered in our department while EBT at the Beatson Oncology Centre. PBT is performed at the Douglas Cyclotron Unit， Clatterbridge Centre for Oncology， Cheshire.

　　Ethical approval was sought from the West of Scotland Research Ethics Committee and was deemed not required as this was a retrospective study. The tenets of the Declaration of Helsinki were observed.

　　RESULTS

　　Visual acuity improved (defined as a gain of two or more lines on Snellen chart) in eleven patients (69%) following treatment and one patient (6%) retained the same level of visual acuity. Four patients (25%) experienced a deterior ation in their vision (loss of one or more lines) after treatment. Of the seven patients treated with brachytherapy alone， only one experienced a decrease in visual acuity while the others all noted an improvement in visual acuity. Among those four treated with TTT alone， only one patient noted a reduction in visual acuity. Only one patient in our series had a final VA of 6/60 or worse. This was an untreated patient who was referred with and retained a visual acuity of 1/36 and then lost to followup.

　　Six out of seven patients (86%) treated within 6 months of onset of symptoms compared to three out of six patients (50%) treated after 6 months of onset of symptoms noted an improvement in visual acuity following treatment.

　　Duration of symptoms prior to diagnosis ranged from two to eighteen months. Time to treatment from diagnosis ranged from same day to 24 months whereas time to treatment from onset of symptoms ranged from 2 to 28 months. No complications were reported following any of the forms of treatment used in this series after a mean followup of 35 months.

　　DISCUSSION

　　From our experience， it appeared that more patients had a better outcome when treated within six months of onset of symptoms compared to those treated six months after onset of symptoms (86% versus 50%). Similarly， in the series of Shields et al[26]， 42% of patients treated within six months and 72% of those treated after six months from the onset of symptoms had a poor visual outcome.

　　In terms of treatment modalities， an improvement of 2 or more lines was noted in 75% of those treated with TTT alone and 86% with brachytherapy alone. Overall， an improvement in visual acuity was noted in 69% of our patients with only one patient having a visual acuity of less than 6/60 after a mean followup of 35 months. Although our retrospective study involved a small population referred to a tertiary centre which precludes strong conclusions， this review of our outcomes compares favourably with those reported in the literature (Table 1， 2) (TTT/ICG Transpupillary thermotherapy with indocyanine green enhancement)

　　We currently recommend treatment within 6 months of onset of symptoms with PL for anteriorly located lesions not accessible with PDT. The visual outcome is influenced by the location of the haemangioma and the duration of symptoms prior to treatment. The majority of patients may expect to retain or achieve some improvement in their vision following treatment. No side effects were observed after a mean followup of 35 months. This paper adds to the small number of patients who benefited from Ruthenium plaque brachytherapy for CCH reported in the current literature.

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