Facial Pain
Summary
This chapter describes the main advances that have taken place in diagnosing and differentiating categories of facial pain. It focuses on the types of pain that have often previously been wrongly attributed to rhinosinusitis and gives advice on how these can be distinguished from one another and treated.
It is important to exclude non-sinus-related causes of facial pain before considering sinus surgery. If facial pain and pressure are the primary symptoms, they are unlikely to be due to sinus disease in the absence of any other nasal symptoms or signs.
Patients with facial pain who have no objective evidence of sinus disease as seen on nasal endoscopy are very unlikely to have rhinosinusitis or be helped by sinus surgery.
Introduction
Otorhinolaryngologists are in a good position to help the majority of patients with facial pain, as they have the expertise and equipment to diagnose whose facial pain is due to paranasal sinus disease and whose is not. This is particularly important because so many patients and their physicians mistakenly attribute their pain as being due to rhinosinusitis when this is not the case. Even in the group of patients who are referred to an ear, nose, and throat (ENT) specialist with a presumptive diagnosis of rhinosinusitis as the cause of their facial pain, only one in eight are found to have pain attributable to sinus disease.1
Tips and Tricks
Patients with a normal computed tomography (CT) scan are unlikely to have pain due to rhinosinusitis. (Note: Approximately one-third of asymptomatic patients have incidental mucosal changes on CT, so radiographic changes on their own are not indicative of symptomatic rhinosinusitis.)
Having excluded rhinosinusitis, the diagnosis of non-sinus-related facial pain is based on some key symptoms that can often point to the cause ( Fig. 10.1 ). Once a provisional diagnosis has been made, medical treatment should be tried to confirm it. Many ENT specialists feel out of their comfort zone in treating non-sinus-related facial pain, as they may have little experience in prescribing medication for this condition, but other specialists often face the same problem. ENT specialists are in a good position to help these patients, and this chapter will outline the way most categories of facial pain can be treated.
Sinogenic Facial Pain
Before describing the characteristics of facial pain secondary to rhinosinusitis, it is important to note that > 80% of patients with a history of chronic infective rhinosinusitis who are found to have purulent secretions visible on nasal endoscopy have no facial pain,2 and the majority of patients with nasal polyposis do not have pain.3 Both of these groups of patients can have pain when they have an acute infective exacerbation when the ostia become blocked, but they do not have chronic pain.
Children with chronic rhinosinusitis (CRS) very rarely complain of facial pain, even in the presence of florid purulent secretions. Also of note is the fact that a significant proportion of patients have persistent facial pain after endoscopic sinus surgery (ESS).4,5
Note
Not only does CRS not usually cause facial pain, but facial pain is not necessarily indicative of rhinosinusitis.
Tips and Tricks
Symptoms of lethargy, catarrh, and postnasal drip are unreliable in making a diagnosis of rhinosinusitis.
Bacterial Rhinosinusitis
Acute sinusitis usually follows an acute upper respiratory tract infection. If there is pain, it is usually unilateral, severe, associated with pyrexia in ~50% of cases, and patients have nasal obstruction. In maxillary sinusitis, unilateral facial and dental pain is a good predictor of true infection; this has been validated in studies using maxillary sinus aspiration.6 This differs from CRS, where there is a poor correlation between the site of facial pain and evidence of sinus pathology.7 CRS is frequently painless, or the pain occurs only during an acute exacerbation or if there is obstruction of the sinus ostia. An increase in the severity of pain on bending forward traditionally has been thought to be diagnostic of CRS, but this symptom is nonspecific and can occur with many other types of facial pain.
Key points in the history of sinus-related pain are an exacerbation of pain during an upper respiratory tract infection, an association with rhinologic symptoms, worsening of pain while flying or skiing, and a response to antibiotic medical treatment.
Examination
In acute frontal sinusitis, the patient is often pyrexial and has tenderness on the medial side of the orbital floor under the supraorbital ridge, where the frontal sinus is thinnest.
Endoscopic examination shows marked hyperemia of the mucosa, and purulent secretions are often visible. Acute sphenoiditis is uncommon and said to cause pain at the vertex of the head, but pain can be referred to the temporal region or the whole head. Facial swelling other than that caused by periorbital cellulitis, cavernous sinus thrombosis, or subgaleal infection usually results from dental sepsis.8
Caution
A normal nasal cavity showing no evidence of middle meatal mucopus or inflammatory changes makes a diagnosis of sinogenic pain most unlikely, particularly if the patient is currently in pain or has had pain within the past few days.
If the patient or physician is in doubt because the patient is asymptomatic on the day he or she is seen and the nasal endoscopy is normal, it is often useful to repeat the nasal endoscopy when the patient does have pain to clarify the diagnosis. It is extremely rare for a patient to have endoscopic evidence of rhinosinusitis when he or she returns with pain. Even the presence of inflammatory changes or infection does not indicate with any certainty that the pain is sinogenic, as it can occasionally be incidental.1 If it is incidental, this will become apparent, as the patient′s pain will persist after the rhinosinusitis has resolved.
Tips and Tricks
Ask the patient to point to the site of the pain, not only because it locates the pain, but also because the gesture often relays information about its nature, and the facial expression indicates its emotional significance to the patient.
Investigations
Plain sinus X-rays may be used to confirm a diagnosis of acute bacterial rhinosinusitis if an antral washout is being considered; however, plain films are very insensitive and nonspecific in diagnosing CRS.9
Caution
The interpretation of changes in the sinuses with CT scans must be treated with caution. Approximately 30% of asymptomatic patients will demonstrate mucosal thickening in one or more sinuses on CT. The presence of this finding is certainly not an indication that pain is sinogenic in origin.10,11
Be careful in making the diagnosis of recurrent acute rhinosinusitis (ARS), as this is unusual; patients who have two or more bacterial sinus infections within 12 months should be investigated for an immunodeficiency.12 An article on the management of sinogenic pain advises signi ficant caution when considering surgery in those patients [with facial pain] because of high long-term failure rates and the eventual identification of other causes of the pain in many cases.13 An analysis of 1713 patients following ESS showed a mean improvement rate of 91%, taking a range of symptoms into account,14 but this series did not specifically relate to facial pain. Many studies have looked at quality of life or encompass a range of nasal symptoms and pathologies, but they do not provide a sufficient breakdown of the different symptoms to analyze the effect of surgery on facial pain.
The studies that have looked at symptoms of facial pain and pressure in rhinosinusitis show that between 56 and 77% of patients who have facial pain are better after sinus surgery.15,16
Note
Patients who have ARS or CRS with facial pain are often helped by medical or surgical treatment.
Facial pain can be coincidental to rhinitis or rhinosinusitis (e.g., 15% of the population has a headache at least once a week).
Other Diseases of the Nose or Sinuses Causing Facial Pain
Although tumors rarely present with facial pain, constant, progressive pain, particularly if associated with other suspicious symptoms or neurologic signs, should alert the clinician. A thorough examination and appropriate imaging are mandatory to exclude the possibility of a tumor. One must remember that some lesions such as neuromas can have a long natural history and that pain may have been present for several years.
Stretching of the arterial tree that supplies the proximal portions of the cranial nerves and the dura within 1 cm of any venous sinus induces a headache and can cause facial pain. The supratentorial vessels and dura refer pain to the ophthalmic division of the trigeminal nerve (cranial nerve [CN] V1). Infratentorial structures refer pain to the distribution of the glossopharyngeal (CN 9) and vagus (CN10) nerves, along with the upper three cervical nerve dermatomes. Space-occupying lesions such as meningiomas, angiomas, and intracerebral metastases can induce facial pain by irritation of the trigeminal nerve along its intracerebral course. Syringobulbia, syphilis, and multiple sclerosis are rarer causes of central lesions that may result in facial pain. Raised intracranial pressure produces a bursting headache that is worse on coughing or straining and is associated with effortless vomiting. The fundi can show papilledema in ~30%. Lesions in the posterior cranial fossa produce occipital and upper neck pain, and supratentorial lesions with raised intracranial pressure produce pain at the vertex or over the frontal and temporal regions. Cerebrovascular accidents can cause such pain, but these symptoms may present only when the other, more distressing signs and symptoms of a stroke are resolving.
Carcinoma of the maxilla is uncommon. Patients, unfortunately, often present late when the disease has spread beyond the confines of the sinuses ( Fig. 10.2 ). Unilateral bloody purulent nasal discharge and obstruction are the most frequent presentations. Less common symptoms are infraorbital paresthesia, loose teeth or ill-fitting dentures, proptosis, deformity of the cheek, epiphora, nasal obstruction, and epistaxis. Pain is usually a late feature.
Nasopharyngeal carcinoma is also rare, but it presents most commonly in adults from the Far East and Mediterranean area. It often presents with cervical lymphadenopathy and middle ear effusions; however, its spread can involve CN5 and CN6 and cause facial pain.
Trotter triad c omprises unilateral middle ear effusion; elevation and immobility of the ipsilateral soft palate; and pain in the ear, jaw, or tongue. If a nasopharyngeal tumor extends intracranially, it may cause Godtfredsen syndrome: ophthalmoplegia, pain in the distribution of the trigeminal nerve, and tongue paralysis.
Pterygopalatine fossa syndrome is caused by malignant infiltration in this area producing maxillary dental pain, infraorbital and palatal anesthesia, pterygoid muscle paralysis, and blindness.
Foix syndrome includes ophthalmoplegia and trigeminal pain but without any tongue signs. It can be caused by an aneurysm abutting, a tumor invading, or a thrombosis of the cavernous sinus.
Tolosa-Hunt syndrome (recurrent painful ophthalmoplegia) presents with gnawing unilateral orbital pain with relapsing and remitting paralysis of CN3, CN4, and CN6. Occasionally, there is paresthesia of the forehead. It is caused by a lesion in the region of the cavernous sinus or the superior orbital fissure. It should be differentiated from ophthalmoplegic migraine, painful diabetic oculomotor palsy, and malignancy. Although this condition often responds to steroids, this is not diagnostic.
Raeder paratrigeminal syndrome presents as an intense pain in the area of CN V1. There are an associated myosis, ptosis, and facial hypoesthesia in the same area. The corneal reflex is reduced, but there is no reduced sweating, as in Horner syndrome. A lesion near the base of the middle cranial fossa at the medial border of the gasserian ganglion is responsible. It may be due to a carotid aneurysm, metastasis, or local invasion by a neoplasm.
Main Categories of Nonsinogenic Facial Pain
General Comments
Only about one in eight patients attending a rhinology clinic have pain that is attributable to rhinosinusitis.16 The remainder of patients who have nonsinogenic pain have either migraine or its variations, tension-type headache/midfacial segment pain, trigeminal nerve, neuropathic pain, or other specific neurologic conditions. Clinical examination and diagnostic tests rarely help to make a diagnosis (exceptions include magnetic resonance imaging [MRI] in multiple sclerosis or brainstem tumors and positron emission tomography [PET] scans that can show abnormalities in the hypothalamus in cluster headache). In facial pain, a diagnosis is primarily made on the basis of the history and response to treatment. The following broad characteristics are used to categorize the main types of facial pain.
Vascular Pain
Migraine, trigeminal-autonomic cephalgias, cluster headache, paroxysmal hemicrania, and SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) are grouped together as disease that may have a vascular origin. Vascular pain of various types can be associated with autonomic rhinologic symptoms, such as nasal congestion and rhinorrhea; this has led to confusion in arriving at a correct diagnosis. Other causes of facial pain are atypical forms of migraine,17,18 cluster headache, and paroxysmal hemicrania.
Migraine
Migraine has been defined by the International Headache Society (IHS)19 as an episodic headache lasting 4 to 72 hours with certain distinguishing features. These include throbbing head pain in attacks, often with a prodromal state and usually preceded by an aura that frequently contains visual phenomena. The pain is typically unilateral but may be bilateral. Nausea, vomiting, photophobia, and phonophobia often accompany the pain ( Fig. 10.3 ). The pain can affect the face as well as the head; a minority of patients can have pain confined to the periorbital area that rarely affects the cheek and nose alone. The operational diagnostic criteria for migraine are shown in Table 10.1.
Tips and Tricks
A history of more than two episodes of “sinusitis” a year should be treated with caution. Suspect that these are vascular in origin, usually variations of migraine. If they appear to be genuine bacterial episodes, then patients’ immunity status should be investigated.
Forms of Migraine
The condition has two main forms. One type, migraine without aura (previously called common migraine), affects ~75% of migraine sufferers. There is no premonition. The second type, migraine with aura (previously called classic migraine), affects 25% of migraine sufferers. The attacks are preceded by neurologic symptoms, such as visual disturbances and numbness. It is three times more common in women, and there is often a family history. Stress release, diet, the premenstrual state, and barometric pressure can induce attacks. This system of classification is conservative, and it is recognized that many patients fall outside these criteria yet have migraine.20 Thus, the diagnostic criteria are highly specific but less sensitive. Other conditions have some migrainous features, such as cluster headache and paroxysmal hemicrania. These, however, have cohesive groups of symptoms that allow them to be categorized separately. Many patients with facial pain do not neatly fit any diagnostic criteria. Some have migrainous features, such as nausea, an aura, and facial flushing. The author has noticed that a significant portion of patients with facial pain had migraine that involved the face, and a minority had migraine confined to the face.
Proposed theories of the cause of migraine have swung between being due to a primarily vascular or neural mechanism. Griggs and Nutt suggested migraine may be part of the spectrum of diseases known as channelopathies—disorders involving voltage-gated channels.20 The genetic component of migraine may be explained by the identification of migraine genes in familial hemiplegic migraine that affect the Ca2+ channel.21
In recent years, neuroimaging of the primary headache syndromes, such as migraine and cluster headaches, has begun to provide a glimpse of the neuroanatomical and physiologic basis of these conditions. Functional imaging with PET and magnetic resonance angiography (MRA) has documented activation in the midbrain, pons,22 and hypothalamic gray matter in cluster headache.23 Work by Edvinnson and Goadsby suggests activation of the trigeminal innervation of the cranial circulation due to vasoactive peptides, such as calcium gene-related peptide.24
A primary dysfunction of the midbrain endogenous antinociceptive system (periaqueductal gray and dorsal raphe nucleus and the neural control of cerebral blood flow) seems to be responsible.25 Neuroimaging has reconciled previous theories that migraine was solely vascular and now suggests that it is a neurovascular headache and an epiphenomenon of trigeminal activation.
It appears that, although vascular input predominates in migraine and myofascial nociception prevails in tension-type pain, there is a great deal of overlap. The pioneering work of Olesen suggests a neurovascular mechanism;26 this is supported by the finding that ~50% of patients with tension-type headache also have migraine. Olesen proposed a vascular-supraspinalmyogenic model that integrates the effects of pericranial myofascial afferents, activation of peripheral nociceptors from cephalic arteries, and convergence on the caudal nucleus of the trigeminal nerve, along with qualitative changes in the central nervous system (CNS).
Management of Migraine
The management of migraine begins with providing information to the patient, including suggestions on avoiding aggravating factors. Regular sleep, exercise, and a diet that avoids aggravating factors will help many patients, although there are no objective data to support this assertion. An assessment must be made on the severity based on the frequency, intensity of pain, and degree of disability. Pharmacological treatment consists of the management of acute attacks and preventive measures.
Current literature suggests preventive treatment should be considered if symptoms occur more than three times monthly, with a duration of symptoms more than 48 hours, and if there is a prolonged aura or failure to reduce acute symptoms.27 The options available for medication are shown in Table 10.2.
Acute antimigraine therapy is most likely to be beneficial if started early in an attack. Treatment can be divided into nonspecific and specific. The former consists of analgesia, such as paracetamol, codeine, and aspirin, or nonsteroidal antiinflammatory drugs (NSAIDs). An antiemetic may be added if there is associated nausea or vomiting. If headaches are severe, specific antimigrainous medications can be used. These include the triptans, or 5-HT1B/D receptor agonists. Ergotamine has to be carefully prescribed, as its overuse can cause severe headaches. Triptans are shown to be effective after the headache begins as long as they are given early. They constrict blood vessels and block neurogenic inflammation and neuropeptide release by a neuronal mechanism of action. Triptans should be prescribed with caution to patients with ischemic heart disease, a history of myocardial infarction, uncontrolled hypertension, or cerebrovascular disease.
Pizotifen is a 5-hydroxytriptamine antagonist that is very effective in the prophylaxis of migraine, but its side effects include weight gain and drowsiness. Propranolol, a β-receptor antagonist, also has some subclass of 5-HT2 effect. Patients with asthma should not be given β-blockers. Sodium valproate has been shown to be effective in the treatment of migraine. Women of childbearing age should be warned about the risk of teratogenicity.
Our experience in patients with facial pain who have migrainous features is that prophylactic antimigrainous medication may need to be continued for up to 6 weeks before its beneficial effects occur. It is often helpful for patients to keep a diary of their symptoms.
Tips and Tricks
If it is not possible to make a diagnosis at the first consultation, it is often helpful to ask the patient to keep a diary of his or her symptoms and have a trial of medical nasal treatment followed by review.
Cluster Headache
Cluster headache is defined as a primary neurovascular headache that is both severe and uncommon. It is characterized by recurrent, strictly unilateral attacks of headache that typically wake the patient and are retro-orbital or centered at the medial aspect of the orbit, of great intensity, and last up to usually 1 but can be up to 3 hours. The pain is also accompanied by ipsilateral signs of autonomic dysfunction, such as the ipsilateral para-sympathetic signs of rhinorrhea, lacrimation, impaired sweating, and sympathetic signs of miosis and ptosis28 ( Fig. 10.4 ). The most salient feature is its periodicity, which could be circadian or in terms of active or inactive bouts lasting 8 to 10 weeks annually, separated by clinical remission when the patient is completely pain free for at least 2 weeks between attacks. About 15 to 20% of patients suffer from chronic cluster headaches and have no significant remissions. Treatment includes sumatriptan injections and oxygen; prophylactic treatment includes verapamil and pizotifen (pizotyline).
Paroxysmal Hemicrania
Paroxysmal hemicrania has been described as an excruciating unilateral pain that is usually ocular and frontotemporal with short-lasting (2–45 min), frequent attacks (usually > 5 daily). By definition, at least one of the following autonomic symptoms should be present: nasal congestion (42%), rhinorrhea (36%), lacrimation (62%), conjunctival injection (36%) or, rarely, ptosis, eyelid edema, heart rate changes (bradycardia, tachycardia, and extrasystoles), increased local sweating, salivation, and facial flushing.29 Attacks may occur bilaterally even though they are usually more pronounced in the symptomatic side. These last from 5 to 45 minutes on each occasion, and they recur between 7 and 22 times daily. Remission rates vary from 3 months to 3 years. Rarely do these headaches develop into the chronic form. The ratio is said to be 1:4 for chronic to episodic paroxysmal hemicrania.30 Overall, the average age of onset is 30 to 40 years, but the spectrum ranges from 6 to 81 years. The episodic form tends to have an earlier age of onset.
Treatment of Paroxysmal Hemicrania
The condition′s complete or rapid response to indomethacin is said to differentiate paroxysmal hemicrania from cluster headache. However, recently the inclusion of this absolute response to make it a criterion has been questioned.31 The majority of patients with paroxysmal hemicrania respond to indomethacin within 24 hours. If not, a trial that entails increasing the dose to 75 mg daily after 3 days, followed by 150 mg daily after another 3 days, has been recommended.32 Another study by Antonaci et al recommended the Indotest, with an intramuscular injection of 50 mg indomethacin; the response is monitored to differentiate paroxysmal hemicrania from hemicrania continua, and other headache disorders with which it can be confused.33 They also noted that the Indotest is a useful tool in the clinical assessment of unilateral headaches by establishing the interval between indomethacin administration and the clinical response.
A need for a persistently high dose may imply a sinister underlying pathology.34 In cases where indomethacin fails to work, other drugs that have been suggested include calcium-channel blockers,35 naproxen, carbamazepine,36 and sumatriptan.37 The main features of paroxysmal hemicrania and cluster headaches are listed in Table 10.3.
Hemicrania Continua
Chronic paroxysmal hemicrania and hemicrania continua are two strictly unilateral headache disorders characterized by an absolute response to indomethacin. Hemicrania continua, first described by Sjaastad and Spierings, is a unilateral headache that is moderately severe without side shift, continuous but with fluctuations, with complete resolution of pain with indomethacin, and exacerbations that may be associated with autonomic features such as conjunctival injection, lacrimation, and photophobia to the affected side.38
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
