Parry-Romberg Syndrome and an Associated Complex Vascular Lesion Managed With Hybrid Strategies: A Case Report.

Neurosurgery practice Pub Date : 2024-01-11 eCollection Date: 2024-03-01 DOI:10.1227/neuprac.0000000000000080
Rory Hagstrom, Howard A Riina, Gavin W Britz, Vera Sharashidze, Charlotte Chung, Hannah Weiss, Robert F Spetzler, Peter Kim Nelson
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Abstract

Background and importance: Parry-Romberg syndrome (PRS) is a condition of unknown etiology, most commonly characterized by progressive facial hemi-atrophy due to breakdown of the underlying skin and soft tissues. PRS has been associated with neurologic disease such as intracranial aneurysms and vascular malformations. However, its exact role in the incidence of neurovascular disease remains unknown.

Clinical presentation: We present a case of a complex fusiform aneurysm involving the A1 segment of the left anterior cerebral artery in a 21-year-old man with PRS. The patient initially presented with a left giant fusiform/dissecting internal carotid artery aneurysm initially managed with overlapping flow diverters. His post-flow diverter treatment course was confounded by progressive growth of the aneurysm with worsening mass effect on the optic chiasm leading to complete visual loss in the left eye and a right hemifield cut. To manage the aneurysm recurrence, retreatment with surgical bypass-supported carotid occlusion was performed. Thereafter, visual symptoms improved, but complete normalization of vision was not achieved. The patient remained clinically stable for several years until follow-up imaging demonstrated a novel adjacent recurrence involving the ipsilateral A1 segment, previously noted to be angiographically normal. The patient underwent superselective angiography followed by endovascular occlusion of the new fusiform lesion.

Discussion: The case presented here illustrates the hybrid roles of endovascular and surgical treatment modalities in treating complex vascular lesions. The recurrence of the lesion following initial treatment strategies introduces questions about how the nature and development of the lesion affected the success of treatment at each stage. Considerations for the efficacy of each stage of treatment in this case include the patients underlying connective tissue disorder, the pediatric etiology of the lesion, and the timing of each treatment option given these factors.

Conclusion: In addition to understanding the biological behavior of flow diversion for complex intracranial aneurysms, the role of PRS in the incidence of neurovascular disease should also be considered when applicable. More research into the mechanisms of PRS in neurovascular disease is needed.

Parry-Romberg综合征和相关的复杂血管病变用混合策略管理:一个病例报告。
背景和重要性:Parry-Romberg综合征(PRS)是一种病因不明的疾病,最常见的特征是由于底层皮肤和软组织的破坏导致进行性面部半萎缩。PRS与神经系统疾病如颅内动脉瘤和血管畸形有关。然而,它在神经血管疾病发病率中的确切作用尚不清楚。临床表现:我们报告一例复杂梭状动脉瘤累及左侧大脑前动脉A1段的21岁男性PRS。患者最初表现为左侧巨大梭状/夹层颈内动脉动脉瘤,最初采用重叠分流器治疗。他的分流后治疗过程因动脉瘤的进行性生长和视交叉肿块效应的恶化而混乱,导致左眼完全视力丧失和右半视野割伤。为了治疗动脉瘤复发,手术旁路支持颈动脉闭塞进行了再治疗。此后,视力症状有所改善,但视力未完全恢复正常。患者在临床上保持稳定数年,直到随访影像学显示新的邻近复发累及同侧A1节段,之前血管造影显示正常。患者接受了超选择性血管造影,随后进行了新的梭状病变的血管内闭塞。讨论:这里的病例说明了血管内和手术治疗方式在治疗复杂血管病变中的混合作用。在最初的治疗策略后,病变的复发带来了关于病变的性质和发展如何影响每个阶段治疗成功的问题。在这种情况下,每个治疗阶段的疗效考虑因素包括患者潜在的结缔组织疾病,病变的儿科病因,以及在这些因素下每个治疗选择的时机。结论:在了解复杂颅内动脉瘤分流的生物学行为的同时,在适用时还应考虑PRS在神经血管疾病发生率中的作用。PRS在神经血管疾病中的作用机制有待进一步研究。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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