Case as discussed in today’s morning report by Dr. Lim. Thanks to Dr. Gardner for some thought-provoking questions leading to an excellent discussion.
22F no sig pmhx presented to the ED with 2d of a global, gradual-onset, throbbing headache. No similar or frequent headaches prior. Has also had URI symptoms the past few days including intermittent fever, cough, sore throat; no nausea, occasional post-tussive vomiting. No photophobia. No neck pain/stiffness. No sick contacts. Takes OCP’s.
- Her exam is normal, including bedside ultrasound optic nerve sheath diameter measurement.
- Labs including CBC, BMP and coags were unremarkable.
- Head CT was unremarkable.
- LP done for concern for meningitis showed opening pressure of 46 with normal CSF studies.
- Neurology eval: normal neuro exam; pt was admitted as MR couldn’t be facilitated from the ED. MR showed cavernous sinus thrombosis.
Cavernous sinus thrombosis is a type of cerebral venous sinus thrombosis (CVST). Most cases of CVST are diagnosed in women (75%). About 12 of every 100,000 births are complicated by CVST. Other common predisposing factors include prothrombotic states of any sort (e.g. our pt ended up having a Factor V Leiden mutation, and was also taking OCP), recent trauma (see Hillary Clinton), head/neck infections, neurosurgical or jugular vein procedures, IVDA, inflammatory conditions such as SLE.
Symptoms are commonly nonspecific and can range from headache to focal neuro symptoms/findings and enecpalopathy. Diagnosis is as it happened in our case. Stroke will be on the differential, though most of CVST cases will have subacute onset so the workup can proceed as with a subacute stroke, but the imaging should include MRV imaging, not just MR/MRA.
Always remember your basic ED ddx for bad headache causes: SAH, mass, infection, thrombosis, IIH, CO poisoning, glaucoma, and temporal arteritis.
Questions that came up:
- How sensitive is papilledema for raised ICP? There is no easy answer in the literature that I could find. Generally, it’s highly sensitive when done by experienced clinicians but it depends on the condition causing ICP and the age of the patient.
- Is ED optic nerve sheath measurement a good altenative for Ophto-performed dilated exam? For adults, there are several studies showing sensitivities ranging 88-100% (depends on what measure was used to determine raised ICP) with one study done on children showing 83% sensitivity.
- How prevalent is papilledema in Idiopathic intracranial hypertension/pseudotumor cerebri – if you have normal fundoscopic exam, does that rule out IIH? The vast majority of patients with IIH will have papilledema. There are, however, several reports of IIH in both adults and children without papilledema; LP opening pressure will diagnose those.
- So the patient has chronic headaches and papilledema and a normal head CT; can we call it IIH and stop there? No. IIH is a RULE OUT diagnosis, so you have to rule out all other causes including subtle mass, thrombosis, and infection. LP and MR/MRV (CT+ if can’t do MR)are necessary, as is basic labs to r/o anemia, blood pressure measurement to r/o malignant HTN.
Some images from http://eyewiki.aao.org and aao.org:
- Beri S, et al. Idiopathic intracranial hypertension without papilledema. Pediatr Neurol. 2010; 42:56-8.
- Wang,SJ, et al. Idiopathic intracranial hypertension without papilledema: a case-control study in a headache center. Neurology. 1998;51:245-9.
- Friedman DI. Papilledema and pseudotumor cerebri. Ophthalmol Clin North Am. 2001;14:129-47
- Filippidis A, et al. Cerebral venous sinus thrombosis: review of the demographics, pathophysiology, current diagnosis, and treatment. Neurosurg Focus. 2009;27:E3.