Gao Yane, Xie Xiaofeng, Li Jiao, Mu Qingshuai, Wang Xingrong, Wang Shuya
Department of Ophthalmology, Affiliated Eye Hospital of Shandong University of Traditional Chinese Medicine, Jinan, China.
Department of Ophthalmology, Shandong Provincial Key Laboratory of Integrated Traditional Chinese and Western Medicine for Prevention and Therapy of Ocular Diseases Shandong Academy of Eye Disease Prevention and Therapy, Jinan, China.
Front Med (Lausanne). 2024 Dec 20;11:1493834. doi: 10.3389/fmed.2024.1493834. eCollection 2024.
Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressure and vascular resistance, leading to systemic venous hypertension and potential right heart failure. These elevated pressures can extend to ocular veins, resulting in complications such as central retinal vein occlusion (CRVO). This case report highlights a rare instance of CRVO combined with cilioretinal artery occlusion (CilRAO), an uncommon ocular manifestation associated with PAH.
A 13-year-old girl with a history of surgically repaired ventricular septal defect presented with sudden vision loss and a central visual field defect. Investigation confirmed CRVO and an unusual concurrent CilRAO. Although laboratory tests were inconclusive, the echocardiographic examination suggested severe pulmonary arterial hypertension (PAH). The patient received treatment with Bosentan and traditional Chinese medicine, which improved her vision to 20/20, though a paracentral scotoma remained.
This case underscores the critical link between systemic cardiovascular abnormalities and ocular health in PAH, where elevated venous pressure can result in severe and distinctive ocular manifestations. The co-occurrence of CRVO and CilRAO in this patient highlights the susceptibility of cilioretinal arteries to hemodynamic changes, as these arteries lack autoregulatory capacity. Additionally, this case demonstrates the potential for positive outcomes in ocular lesions with targeted PAH therapy. Importantly, it emphasizes the need for vigilance when managing complex cases where conclusions cannot be drawn solely from ocular findings. A multidisciplinary approach and comprehensive diagnostics are essential for identifying underlying causes, ensuring active treatment, and preventing permanent vision loss and serious systemic complications.
肺动脉高压（PAH）的特征是肺动脉压力和血管阻力升高，导致体循环静脉高压和潜在的右心衰竭。这些升高的压力可延伸至眼静脉，导致诸如视网膜中央静脉阻塞（CRVO）等并发症。本病例报告突出了一例罕见的CRVO合并睫状视网膜动脉阻塞（CilRAO）的情况，CilRAO是一种与PAH相关的不常见眼部表现。
一名13岁女孩，有室间隔缺损手术修复史，出现突发视力丧失和中心视野缺损。检查确诊为CRVO以及罕见的并发CilRAO。尽管实验室检查结果不明确，但超声心动图检查提示严重肺动脉高压（PAH）。患者接受了波生坦和中药治疗，视力提高到20/20，但仍残留旁中心暗点。
本病例强调了PAH中全身心血管异常与眼部健康之间的关键联系，其中静脉压升高可导致严重且独特的眼部表现。该患者CRVO和CilRAO同时出现，突出了睫状视网膜动脉对血流动力学变化的易感性，因为这些动脉缺乏自动调节能力。此外，本病例证明了针对PAH的治疗对眼部病变可能产生积极效果。重要的是，它强调在处理无法仅从眼部检查结果得出结论的复杂病例时需要保持警惕。多学科方法和全面诊断对于确定潜在病因、确保积极治疗以及预防永久性视力丧失和严重全身并发症至关重要。
