Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension: Type, Time course, and Treatment of underlying Spinal Cerebrospinal Fluid Leak.
Jennifer Harris1, Wouter Schievink3, Marcel Maya2, Javier Galvan2, Rachelle Taché3
1Neurology, 2Radiology, Cedars Sinai Medical Center, 3Cedars Sinai Neurosurgical Inst
Objective:

Spontaneous spinal CSF leaks cause intracranial hypotension (SIH) and also may cause infratentorial superficial siderosis (iSS), but the rate of development among different CSF leak types and outcome of treatment are not known. We determined the time interval from SIH onset to iSS and the outcome of treatment.

Background:
iSS is an uncommon, but increasingly diagnosed condition. Cranial and particularly spinal dural pathology is responsible for the majority of cases of iSS. Spontaneous spinal CSF leaks cause SIH and also may cause iSS. However, the prevalence and rate of development of iSS among different CSF leak types and best treatment are unknown.
Design/Methods:

1589 patients with SIH underwent neuroimaging and iSS was detected in 57 (23 men and 34 women, mean age 41.3) (3.6%). We examined the type of underlying CSF leak by various imaging modalities. Percutaneous and surgical procedures were used to treat the CSF leak.

Results:

iSS was detected in 46 (10.3%) of 447 patients with ventral CSF leaks, in 2 (3.9%) of 51 patients with dural ectasia, in 5 (2.6%) of 194 patients with CSF-venous fistulas, in 4 (0.9%) of 457 patients with simple meningeal diverticula, and in none of the 101 patients with lateral CSF leaks or the 339 patients with leaks of indeterminate origin (p<0.001). The median latency period from SIH onset to iSS was 126 months. Ventral CSF leaks could not be eliminated with percutaneous procedures in any patient and surgical repair was associated with low risk (<5%) and resulted in resolution of the CSF leak in all patients in whom the exact site of the CSF leak could be determined. Other types of CSF leak were treated with percutaneous or surgical procedures.

Conclusions:

iSS can develop in most types of spinal CSF leak, including CSF-venous fistulas, but mainly in chronic ventral CSF leaks, which require surgical repair.

10.1212/WNL.0000000000201753