Sturge-Weber syndrome (SWS) is a congenital neurological disorder that is characterized by hamartomas involving the skin, brain, and eyes and marked by the presence of the facial port-wine stain, which consequently leads to various ocular complications. Among all ocular comorbidities, glaucoma is the most frequently witnessed in SWS patients with a prevalence of 30%–70%. If glaucoma is refractory to conventional medical management, surgical intervention can be considered. Common complications of glaucoma procedures in SWS are choroidal detachment and suprachoroidal hemorrhage. Moreover, we report a 6-year-old girl, known case of unilateral congenital glaucoma secondary to SWS. Despite being on maximal antiglaucoma drops and undergoing multiple surgical interventions, the patient had uncontrolled intraocular pressure of her right eye. A decision to proceed with Ahmed glaucoma valve implantation (AGVI) to the right eye was made. In the immediate postoperative period, the patient developed hemorrhagic choroidal detachment and exudative retinal detachment. A trial of oral propranolol (1.5–2 mg/kg/day) was then initiated for 4 months. After 30 days from oral propranolol course initiation, we started noticing a significant improvement of the hemorrhagic choroidal and exudative retinal detachment. Spontaneously, a marked reduction in subretinal fluid and suprachoroidal hemorrhage was also seen. Thus, the improvement was correlated with the propranolol therapy. Here, we report a significant improvement of the postoperative complications of AGVI in a patient with SWS, following 4 months of oral propranolol course (1.5–2 mg/kg/day). Further studies are needed to determine the dosage, duration, and optimal mechanism by which propranolol works in this situation.
Case Rep Ophthalmol 2021;12:859–869

中文翻译：

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普萘洛尔治疗 Sturge-Weber 综合征 Ahmed 青光眼瓣膜植入术后出血性脉络膜和渗出性视网膜脱离的有效性：病例报告和文献综述

Sturge-Weber 综合征 (SWS) 是一种先天性神经系统疾病，其特征是错构瘤累及皮肤、大脑和眼睛，并以面部葡萄酒色斑为特征，从而导致各种眼部并发症。在所有眼部合并症中，青光眼最常见于 SWS 患者，患病率为 30%–70%。如果青光眼对常规药物治疗无效，可以考虑手术干预。SWS 中青光眼手术的常见并发症是脉络膜脱离和脉络膜上腔出血。此外，我们报告了一名 6 岁女孩，已知的继发于 SWS 的单侧先天性青光眼病例。尽管服用了最大量的抗青光眼药水并接受了多次手术干预，但患者的右眼眼压仍不受控制。决定对右眼进行 Ahmed 青光眼瓣膜植入术 (AGVI)。术后即刻出现出血性脉络膜脱离和渗出性视网膜脱离。然后开始了为期 4 个月的口服普萘洛尔（1.5–2 mg/kg/天）试验。从口服普萘洛尔疗程开始 30 天后，我们开始注意到出血性脉络膜和渗出性视网膜脱离的显着改善。自发地，还观察到视网膜下液和脉络膜上血的显着减少。因此，改善与普萘洛尔治疗相关。在这里，我们报告了 4 个月口服普萘洛尔疗程（1.5-2 mg/kg/天）后，SWS 患者 AGVI 术后并发症的显着改善。需要进一步研究以确定剂量，
Case Rep Ophthalmol 2021；12:859–869