A 48-year-old woman presented to the emergency department complaining of a five- to six-week history of persistent nasal drip, with fever and intermittent headaches for one month. She described a throbbing occipital headache radiating to the periorbital area bilaterally. She had been treated with several different antibiotics in the preceding four months for a presumptive diagnosis of sinusitis. Symptoms improved while on the medication, but then would quickly return once the antibiotic course was finished.
At presentation, the patient had a temperature of 102.0°F, and her skin was flushed and hot to the touch. She appeared uncomfortable, lying on the stretcher holding her head. Nasal congestion with surrounding erythema and clear rhinorrhea were noted. Her tympanic membranes were normal bilaterally, and her pharynx appeared normal with no tonsillar hypertrophy or exudate. Her neck was supple with no tender lymphadenopathy. Her heart maintained normal rhythm and rate, and her lungs were clear to auscultation bilaterally. She was neurologically intact. The rest of her physical exam was unremarkable.
Laboratory studies demonstrated a leukocytosis of 23.6 × 103/mcL with 89% segmented neutrophils and 6% lymphocytes. Besides an elevated alkaline phosphatase at 158, the rest of her laboratory evaluation and a CT scan of her brain were unremarkable. A lumbar puncture was attempted but unsuccessful. Ceftriaxone 2 g was given, and the patient was admitted for an MRI and fluoroscopic-guided lumbar puncture. An MRI of the brain subsequently demonstrated a left superior ethmoid cell defect causing CSF leakage through the ethmoid sinuses. (Photo.) CSF analysis was suggestive of bacterial meningitis, but no organisms were found. She was transferred to a tertiary care facility where she was successfully treated with endoscopic endonasal repair without recurrence of symptoms.
Cerebrospinal fluid (CSF) fistulas can occur from traumatic and atraumatic causes. Although the occurrence of a CSF fistula is rare, traumatic causes are the most common etiology, occurring in 70 to 80 percent of cases. CSF fistulas can also occur from eroding tumors, congenital defects, ENT infections, or increased intracranial pressures such as those with pseudotumor cerebri. In any of these cases, there is a destruction or breakdown of the bony architecture resulting in a defect of the cribriform plate. Immediate action is necessary to prevent ascending meningitis, which occurs in a third of patients with CSF leaks.
Whatever the cause of a CSF leak, patients most commonly present with unilateral clear rhinorrhea, which can easily be confused for allergic rhinitis. The fluid may be tested for beta-2 transferrin to confirm the CSF leak. The second most common presentation is active meningitis or a history of meningitis. Defects are most commonly in the sphenoid sinus and the ethmoid roof.
Management depends on etiology. If due to traumatic events, patients who present with small fistulas may be treated conservatively with head elevation, bed rest, and antibiotics. For those who do not respond to conservative management or who have large or nontraumatic fistulas, immediate surgical repair is needed. IV acetazolamide is given for those with pseudotumor cerebri to attempt to decrease intracranial pressure. A VP shunt is placed for those who present with recurring leaks or who do not respond appropriately to diuretics.
The etiology of this patient's CSF leak still remains uncertain. There was no recent trauma to suggest that a physical defect occurred. Cases of CSF leaks have been known to occur 20 years after a traumatic head injury, however, and this patient admitted to such an event 22 years previously. According to the patient, past CT scans and MRIs of the brain were negative, although small fistulas may be missed by radiological studies.
Our patient was also obese, and admitted to occasional headaches, which may also suggest pseudotumor cerebri as a cause. Our patient also had a history of sinus infections for more than four months, which also could be the source of the defect. Unfortunately, the mystery remains on whether the leak was present prior to the sinusitis causing similar symptoms. One thing, however, is certain. The multiple courses of antibiotic regimens that she received saved her life.
The authors are from the emergency medicine residency at Good Samaritan Hospital Medical Center in West Islip, NY.
Otolaryngol Head Neck Surg
Am J Rhinol
J Clin Neurosci
2002 Aug; 31 Suppl 1:S28.