ArticleFrontiers in surgery2026
Holocord syringomyelia associated with von Hippel-Lindau disease: a case series.
Article in Frontiers in surgery, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Abstract
Introduction: Holocord syringomyelia is a rare and severe form of syringomyelia, often associated with disruptions in cerebrospinal fluid (CSF) dynamics. In patients with von Hippel-Lindau (VHL) disease, multifocal spinal hemangioblastomas may contribute to the development of extensive syringomyelia. However, the pathogenesis and optimal management of VHL-associated holocord syringomyelia remain poorly defined. Materials and methods: A retrospective review was conducted on 136 patients diagnosed with VHL disease at our institution. Four patients with VHL-associated holocord syringomyelia were identified. Their clinical presentations, imaging characteristics, treatment strategies, and outcomes were systematically analyzed. Results: All four patients exhibited holocord syringomyelia accompanied by multiple intramedullary hemangioblastomas. Clinical manifestations varied markedly, ranging from completely asymptomatic to progressive neurological deficits. Surgical resection of selected symptomatic lesions resulted in clinical improvement in some patients, but radiological resolution of the syrinx was inconsistent and often incomplete. Conclusion: Holocord syringomyelia in VHL disease likely results from the cumulative effects of multiple segmental lesions rather than a single causative tumor. Management should prioritize symptom control and individualized decision-making, rather than aggressive treatment of the syrinx itself.
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