![]() ![]() In a retrospective study of 11 patients with β-2 transferrin-proven spontaneous CSF leaks, 72% of patients met the criteria for the diagnosis of IIH ( 5). Such overlap in the demographics is also shared by the primary empty sella syndrome, an endocrinologic entity in which chronically increased ICP may play a role ( 25, 26). Review of published cases suggests that a large majority of patients with spontaneous CSF leak are women with a mean BMI higher than 30 kg/m 2 ( Table 1). Similar to IIH patients, spontaneous CSF leak patients are often young or middle-age obese women ( 4, 24). Over the past two decades, several articles on this topic have been published mostly in the ENT and neurosurgical literature ( 2- 24), and have highlighted the similarities between the demographics of patients with IIH and those with spontaneous CSF leaks supporting a relationship between IIH and so-called spontaneous CSF leak ( Table 1). Idiopathic intracranial hypertension is increasingly recognized as a cause of primary spontaneous CSF leaks. Surgical repair + oral diuretics (in some cases) Rhinorrhea, headaches, rhinorrhea, meningitisĤ3 patients: >30, only one was 25 (at least 4% of the time)Įndoscopic repair + lumbar drain with continuous CSF pressure monitoringĮthmoid sinus ( 13), lateral sphenoid sinus ( 7), frontal sinus ( 1) Hearing loss, meningitis, otorrhea, rhinorrhea Roof of the ethmoid ( 6), sphenoid ( 5), cribiform plate ( 4) Surgical repair (+ CSF shunting in 3 patients) Lateral sphenoid recess ( 8), central sphenoid ( 4), ethmoid roof ( 4), cribiform ( 2), supraorbital/posterior frontal recess ( 2), frontal sinus ( 2) Rhinorrhea, visual disturbances, headachesĬraniotomy + LP shunt transverse sinus stenting Orthostatic headaches-after repair: elevated ICP related-symptoms Surgical repair/Lumbar punctures/Diuretics LP shunt in one of the patients, unknown for the other patient Surgical (craniotomy + sealing of the cribiform plate with muscle) One month later, he remained asymptomatic, with improvement of his papilledema. The CSF rhinorrhea markedly decreased after the procedure, and endoscopic repair of the leak was subsequently performed. He was placed on prophylactic antibiotics and underwent a ventriculoperitoneal shunt procedure aimed at decreasing his ICP. Repeat brain imaging showed a right cribriform plate encephalocele responsible for the CSF leak, in addition to previously known empty sella and bilateral transverse sinus stenoses ( Fig. His headaches improved, but around the same time he noticed CSF rhinorrhea. Repeat lumbar puncture showed elevated CSF OP at 33.5 cmH 2O. Repeat neuro-ophthalmic examination showed persistent, stable mild papilledema OU ( Fig. He consulted again for worsening of headaches, which were occurring daily and were associated with tinnitus, and transient visual obscurations. He had moderate bilateral papilledema with enlarged blind spots, but no or very mild headaches, and was most often doing well without treatment. More rarely, patients with a known diagnosis of IIH may develop a spontaneous CSF leak, presumably directly secondary to the chronically raised ICP with skull base erosion and meningoceles ( 2).Ī 43 year-old mildly overweight white man was followed for 3 years for mildly-symptomatic IIH treated only with episodic acetazolamide. The diagnosis of IIH is typically made a few weeks or months after surgical repair of the CSF leak, because of increased ICP resulting in classic IIH symptoms and signs ( 9). Some of these patients are asymptomatic or only have symptoms attributable to the CSF leak (such as rhinorrhea, CSF hypotension-related headaches, or bacterial meningitis) while the leak is active. Over the past 20 years, a few authors have suggested that so-called “primary spontaneous CSF leaks” might represent a form of IIH ( 2- 8). Idiopathic intracranial hypertension (IIH) is increasingly recognized as a cause of spontaneous CSF leak in the ENT and neurosurgical literature ( 2- 8). Spontaneous CSF leaks are sometimes referred to as high-pressure leaks when increased ICP contributes to the development of the CSF leak ( 2, 3). Non-traumatic CSF leak may be spontaneous in the absence of obvious cause, such as skull base abnormalities or bone erosion related to tumors or hydrocephalus ( 1, 2, 3). CSF leaks have been traditionally classified as traumatic or non-traumatic ( 1, 2).
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