Spontaneous Cerebrospinal Fluid Leak at the Clivus: Two Case Reports and Literature Review

Abstract

Spontaneous or non-traumatic cerebrospinal fluid leaks comprise 5%-10% of all cerebrospinal fluid (CSF) rhinorrhea [1,2]. Generally, CSF rhinorrhea can occur at the cribriform plate, sella, sphenoid sinus, or ethmoid air cells [3,4]. However, primary CSF rhinorrhea due to clival defect is extremely rare. The current study describes two cases of spontaneous CSF rhinorrhea through the clivus which were repaired with endoscopic endonasal transsphenoidal approach. Such cases are extremely rare and upon literature review, only 16 cases with clival defect are reported thus far. The peculiar aspect of this case report is related to its rarity.

Primary Spontaneous Inferior Clival Cerebrospinal Fluid Leak

“…Additionally, since the patient characteristics associated with spontaneous CSF leaks and IIH are so similar, several authors have hypothesized that spontaneous CSF leaks may be a variant of IIH [7,10,16,17]. The importance of increased intracranial pressure in spontaneous CSF rhinorrhea is also evidenced radiologically by frequently observed empty sella syndrome (80%) and arachnoid pits (63%) and a thinned and broadly attenuated skull base [18]. Clival CSF leaks have been seen in congenital bone malformations such as Marfan's syndrome [19].…”

“…It is likely that the patient's posterior-inferior clival defect could have been detected earlier if this were done, effectively preventing her from developing bacterial meningitis in 2019. It is important to note that while bacterial meningitis secondary to CSF leak may develop in as many as 15% of cases, this number dramatically increases to as high as 47% in cases where a clival defect is involved [18]. Hence, it may be prudent for clinicians to more aggressively workup patients for suspected CSF leaks given the potentially dire sequelae.…”