Approach to the Patient with Facial Pain

Acute glaucoma is associated with orbital and adjacent facial pain but the diagnosis is usually obvious because of corneal clouding and other eye changes. Subacute glaucoma can be more subtle on physical examination and can lead to a late or missed diagnosis.

Facial pain or headache should not be readily attributed to refractive errors or eye strain.

Chronic facial pain accompanied by hyperpathia or allodynia can be a manifestation of a central pain syndrome. The most common of these involves the contralateral thalamus and is usually postischemic. Generally, complete hemisensory loss will be present but on occasion, the loss can be restricted to the trigeminal distribution alone. This form of central pain may readily respond to tricyclic antidepressants such as amitriptyline or to antiepileptic medications such as lamotrigine or gabapentin.

Anesthesia dolorosa is a central pain syndrome that may follow any of the procedures performed to control TN. It is thought to be a consequence of excessive deafferentation and is characterized by the simultaneous presence of a constant, very unpleasant dysesthesia and marked cutaneous facial sensory loss. The syndrome may not surface for weeks or months after an ablative procedure. Neurosurgeons have observed that lessening the degree of deafferentation can lessen the likelihood of anesthesia dolorosa following an ablative trigeminal procedure. This syndrome can be difficult to treat either medically or surgically. Experimental approaches utilizing either invasive or noninvasive brain stimulation technologies could prove quite effective in the near future. A very few patients with significant trigeminal deafferentation may develop self-inflicted and difficult-to-treat ulceration, typically involving the ala nasi and designated “trigeminal trophic syndrome.”

Burning mouth syndrome is a chronic and persistent syndrome of bilateral dysesthesias likened to the sensations following exposure to a too hot liquid involving the oral mucosa. Xerostomia and dysguesia may be present. Anxiety, irritability, and other mood changes are frequently present. It is most commonly encountered in perimenopausal or postmenopausal women and is sometimes confused with other facial pain syndromes.

PEARLS FOR FACIAL PAIN

• Only TN responds unequivocally to carbamazepine or oxcarbazepine. TACs should not.

• Pain in the ophthalmic division alone should raise considerable doubts regarding the diagnosis of TN.

• TN sometimes evinces tearing or conjunctival injection but this is not common; when these manifestations are prominent, consider SUNCT as a possibility.

• Paroxysmal hemicranial headache and chronic hemicranial headache very characteristically respond to indomethacin.

• Patients with CH appear restless and agitated. This is in very sharp contrast to migraineurs who very characteristically show a great reluctance to move.

• Migraineurs not infrequently awaken with morning headache or possibly facial pain; if a patient awakens with pain regularly from sleep or experiences pain at another time on a circadian basis, strongly consider CH.

• Patients with SUNCT or SUNA will not respond to medications with the possible exception of lamotrigine.

• Alcohol can be an instantaneous trigger in patients with CH during cluster periods. Alcohol is a more subtle, slower trigger in migraine.

• Migraine is extremely common, CH is not uncommon, TN is not uncommon, paroxysmal and chronic hemicranial headaches are uncommon, and SUNCT and SUNA are very uncommon.

• Frequently recurrent facial pain without fever or purulent discharge is more likely to be migraine, less likely to be sinusitis.

CONCLUDING OBSERVATIONS

It should always be remembered that patients with a chronic complaint, even if that complaint has been thoroughly investigated can develop new symptoms and become refractory to previously effective therapy. Changes in complaint or condition in an otherwise familiar patient could reflect a serious underlying pathology and may merit repeat neuroimaging. Patients with chronic facial pain may harbor an undisclosed malignancy or inflammatory process or a chronic infectious process. The presence of neurologic deficits on examination, be it a cranial nerve palsy, a sensory deficit in the trigeminal distribution, Horner syndrome, or the presence of subjective complaints of dysesthesias, diplopia, hearing loss, or disequilibrium should alert the practitioner to these possibilities. Similarly, the presence of cervical lymphadenopathy, chronic nasal obstruction, proptosis, lid edema, serous otitis, or an objective bruit is a potentially grave sign.

Patients with deficits should be appropriately imaged and referred. If patients with TN do not immediately respond to medication, they should be seen by a neurologist or neurosurgeon. If patients with TN fail medical therapy, consideration of early invasive treatment is generally appropriate. Because of its rarity, the diagnosis of GN should be confirmed by a neurologist or neurosurgeon. Patients with unusual headache syndromes should have their diagnosis confirmed by a neurologist and, if management difficulties are encountered, managed by a neurologist with headache expertise. Patients with HZ ophthalmicus should be seen by an ophthalmologist even if the globe appears uninvolved. Patients with refractory PHN can be expected to do poorly following ablative therapy.

Patients with chronic facial pain would truly benefit from a medical home provided by either their primary care physician or an appropriate specialist, perhaps a neurologist, who can propose new avenues of investigation or therapy and help limit inappropriate interventions.

Key Points

• TN is probably the most common facial pain disorder, is recognized clinically by unilateral bouts of lancinating pain primarily in the second and third divisions of the trigeminal nerve.

• TN is too frequently missed by practitioners but is generally quite treatable.

• GN is relatively rare but pain quality is similar to that in TN but involves the throat, posterior tongue, or ear.

• PHN involving the eye and upper face commonly follows HZ ophthalmicus, which is the second most common site of HZ neuropathy, comprising 10% to 20% of all cases.

• The primary headache disorders presenting as facial pain include CH and the other TACs; however, migraine without aura may present with prominent facial involvement and is not infrequently mistaken for recurrent sinusitis.

• PIFP is a diagnosis of exclusion and should be considered in cases where other causes such as TN, primary headache, or TMJ are poor clinical fits.

• Common causes of facial pain—purulent sinusitis, acute glaucoma, dental infections, sialadenitis—should not be overlooked nor should they be clinically opaque.

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