From the Guidelines
The incidence of spontaneous Cerebrospinal Fluid (CSF) leaks is approximately 5 per 100,000 individuals annually.
Incidence of Spontaneous CSF Leaks
- The estimated annual incidence of Spontaneous Intracranial Hypotension (SIH) is reported to be around 3.7 per 100,000 in some studies 1, but a more recent study estimates it to be around 5 per 100,000 1.
- It is essential to note that the true incidence of this condition may be higher due to underdiagnosis and misdiagnosis 1.
- The incidence of secondary intracranial hypotension symptoms related to dural puncture is estimated to be around 2% to 8% of cases 1.
Causes and Risk Factors
- The main causes of intracranial hypotension are CSF leakage through a dural defect, leaking meningeal diverticulum, and CSF-venous fistula 1.
- Clinical risk factors for the development of SIH include spinal osteophytes, weakened ectatic dura/meningeal cysts, and a history of bariatric surgery 1.
- The pathophysiologic mechanism of headache symptoms and various neurological deficits in patients with intracranial hypotension is not well understood but likely multifactorial and may be attributed to compensatory venodilitation, blood volume expansion, and dural sinus stretching as well as downward traction on the meninges, nerves, and brain 1.
From the Research
Incidence of Spontaneous Cerebrospinal Fluid (CSF) Leaks
- The incidence of spontaneous spinal CSF leaks has been estimated at 5 per 100,000 per year, with a peak around age 40 years 2.
- Women are affected more commonly than men, with a demographic overlap between idiopathic intracranial hypertension (IIH) patients and those with spontaneous CSF leaks 3.
- Spontaneous skull base cerebrospinal fluid leaks occurring without any apparent cause are rare, and those patients that present such leaks simultaneously, or successively, in multiple locations are even rarer 4.
Prevalence of IIH in Spontaneous Skull Base CSF Leak Patients
- The prevalence of definite IIH was 19.4% in patients with spontaneous skull base CSF leaks, with a striking demographic overlap between IIH patients and those with spontaneous CSF leaks 3.
- The true prevalence of IIH is likely higher than identified by using classic criteria, as an active CSF leak may serve as an auto-diversion for CSF, thereby "treating" the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation 3.
Treatment Options for Spontaneous CSF Leaks
- Treatment options for spontaneous CSF leaks include bed rest, epidural blood patching, percutaneous placement of fibrin sealant, and surgical CSF leak repair 2.
- Innovative strategies for the treatment of spinal CSF leaks have been developed, including open surgical techniques, minimally invasive tubular techniques, and endovascular embolization of CSF-venous fistulas 5.
- Full endoscopic repair of spontaneous ventral cerebrospinal fluid leaks in the spine is a viable option, allowing for removal of bony spicules/osteophytes and dural repair without the morbidity associated with traditional open approaches 6.