Objective:

To analyze the clinical presentations, imaging characteristics, treatment strategies, and outcomes of patients with VHL-associated holocord syringomyelia.

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Key Findings:

• Four patients (2.9%) with VHL disease were identified to have holocord syringomyelia.
• All patients exhibited multiple intramedullary hemangioblastomas.
• Clinical manifestations ranged from 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.

Interpretation:

Holocord syringomyelia in VHL disease likely arises from multiple segmental lesions rather than a single tumor, necessitating individualized management focused on symptom control.

Limitations:

• The study is limited by its retrospective nature and small sample size.

Conclusion:

Management of holocord syringomyelia in VHL disease should prioritize symptom control and individualized decision-making.