Long-term risks of persistent ventral spinal CSF leaks in SIH: superficial siderosis and bibrachial amyotrophy

WI Schievink, M Maya, F Moser, M Nuño - Neurology, 2021 - AAN Enterprises
WI Schievink, M Maya, F Moser, M Nuño
Neurology, 2021AAN Enterprises
Background and Objectives Superficial siderosis, bibrachial amyotrophy, and spinal cord
herniation are unusual but serious long-term sequelae of persistent spontaneous spinal
CSF leaks in patients with spontaneous intracranial hypotension (SIH), particularly ventral
spinal CSF leaks. However, the risk of developing such sequelae has not been established
in this population. We undertook this study to determine the risk of these serious
complications of persistent ventral spinal CSF leaks. Methods This cohort study was …
Background and Objectives
Superficial siderosis, bibrachial amyotrophy, and spinal cord herniation are unusual but serious long-term sequelae of persistent spontaneous spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH), particularly ventral spinal CSF leaks. However, the risk of developing such sequelae has not been established in this population. We undertook this study to determine the risk of these serious complications of persistent ventral spinal CSF leaks.
Methods
This cohort study was conducted using data from a prospectively maintained database of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. The patient population consisted of a consecutive group of patients with SIH and persistent ventral spinal CSF leaks who were first seen within 1 year of onset of SIH symptoms and who had at least 1 year of follow-up.
Results
Among 51 patients with SIH and a persistent ventral spinal CSF leak, superficial siderosis developed in 6 patients and bibrachial amyotrophy in 2 patients during 280 patient-years of follow-up. The probability of these complications increased from 0% at 48 months to 4.5% (95% confidence interval [CI] 1.0%–28.0%) at 56 months, 10.5% (95% CI 3.0%–36.4%) at 96 months, 32.7% (95% CI 15.0%–62.8%) at 144 months, and 57.9% (95% CI 30.2%–87.6%) at 192 months. None of the patients developed spinal cord herniation.
Discussion
Among patients with SIH and a persistent ventral spinal CSF leak, the risk of developing serious long-term sequelae is considerable. This study shows that early treatment of a ventral spinal CSF leak offers a unique opportunity to prevent neurologic disability from superficial siderosis and bibrachial amyotrophy.
American Academy of Neurology
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