Related Experiment Video
Updated: Aug 6, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Holocord syringomyelia associated with von Hippel-Lindau disease: a case series
Shijie Li1, He Wang2, Tao Liu1
1Department of Neurosurgery, Peking University First Hospital, Beijing, China.
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.
