Althaus C, Sundmacher R
Universitäts-Augenklinik, Heinrich-Heine-Universität Düsseldorf.
Klin Monbl Augenheilkd. 1996 Apr;208(4):239-42. doi: 10.1055/s-2008-1035203.
In Sturge-Weber syndrome choroidal hemangiomas occur in up to 55%. With a few exceptions it is a diffuse hemangioma and it always affects the eye ipsilateral to the naevus flammeus. To the best of our knowledge, a contralateral circumscribed choroidal hemangioma has not yet been described.
We present a 34-year-old male caucasian with a left-sided naevus flammeus and a right-sided circumscribed choroidal hemangioma. Ipsilateral to the naevus, the left eye showed typical vascular anomalies of the conjunctiva and episclera as well as in the anterior chamber angle with anterior synechia formation and circumscribed iris atrophy without secondary glaucoma. Retinal and choroidal vasculature was normal. In the contralateral eye the anterior segment was normal, but there was a juxtafoveolar circumscribed choroidal hemangioma of about 7.5 x 4.5 mm along the superior temporal vascular arcade. The tumor was detected when vision dropped to 0.4 due to an exsudative retinal detachment over the tumor involving the fovea.
Complete regression of the exsudative detachment occurred under oral Acetazolamide (1000 mg/d), but after dose reduction to 500 mg/d the exsudation recurred. Thereafter, the surface of the tumor was treated with argongreen grid lasercoagulation in 3 sessions under Acetazolamide cover. Following scar formation the retina has stayed completely attached even after discontinuation of oral Acetazolamide therapy with a follow-up of now 7 months.
Visual prognosis after exsudative macula detachment is depended on rapid retinal reattachment. Therefore, early and effective treatment is mandatory. Systemic application of Acetazolamid (1000 mg/d orally) led to a rapid reattachment of the retina with an excellent visual result. However, dose-reduction was followed by an immediate recurrency. We therefore suggest to perform an Argongreen grid lasercoagulation of the tumor surface while the patient is still under systemic Aceazolamid. This strategy has proven successful in this case.
在斯特奇-韦伯综合征中，脉络膜血管瘤的发生率高达55%。除少数例外情况外，它是一种弥漫性血管瘤，且总是累及与焰色痣同侧的眼睛。据我们所知，尚未有对侧局限性脉络膜血管瘤的相关描述。
我们报告一名34岁的白种男性，左侧有焰色痣，右侧有局限性脉络膜血管瘤。与痣同侧的左眼显示结膜、巩膜以及前房角典型的血管异常，伴有前粘连形成和局限性虹膜萎缩，无继发性青光眼。视网膜和脉络膜血管系统正常。对侧眼眼前节正常，但沿颞上血管弓有一个约7.5×4.5毫米的近黄斑区局限性脉络膜血管瘤。当视力因肿瘤上方累及黄斑的渗出性视网膜脱离降至0.4时发现该肿瘤。
口服乙酰唑胺（1000毫克/天）后渗出性视网膜脱离完全消退，但剂量减至500毫克/天后渗出复发。此后，在乙酰唑胺的掩护下分3次对肿瘤表面进行氩绿格栅激光光凝治疗。形成瘢痕后，即使在停用口服乙酰唑胺治疗且随访7个月后，视网膜仍完全附着。
渗出性黄斑脱离后的视力预后取决于视网膜的快速复位。因此，早期和有效的治疗是必需的。全身应用乙酰唑胺（口服1000毫克/天）可使视网膜快速复位并获得极佳的视力结果。然而，剂量减少后立即复发。因此，我们建议在患者仍接受全身乙酰唑胺治疗时对肿瘤表面进行氩绿格栅激光光凝治疗。该策略在本病例中已被证明是成功的。
