Abstract
The differential diagnosis of orofacial pain can be very extensive. Emphasis should be placed on correct diagnosis and treatment rather than on symptomatic management. Each disorder is described according to the International Classification of Headache Disorders, 3rd edition (ICHD-3) classification and diagnostic criteria. The pathophysiology of the disorder, the clinical picture, and the management (both medical and interventional, if applicable) are reviewed.
Keywords
glossopharyngeal neuralgia, orofacial pain, postherpetic neuralgia, TMJ dysfunction, trigeminal neuralgia
Introduction
The incidence and prevalence of orofacial pain in the general population is very high. Care and emphasis should be placed on correct diagnosis and treatment rather than on symptomatic management. Each disorder is described according to the International Classification of Headache Disorders, 3rd edition (ICHD-3) classification and diagnostic criteria. The pathophysiology of the disorder, the clinical picture, and the management (both medical and interventional if applicable) are reviewed.
The ICHD-3 criteria provide a systematic classification for headache and orofacial pain and are divided into three parts: the primary headaches, the secondary headaches, and cranial neuralgias central and primary facial pain ( Table 23.1 ).
| Part I: The Primary Headaches |
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| Part II: The Secondary Headaches |
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| Part III: Painful Cranial Neuropathies, Other Facial Pains and Other Headaches |
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We largely discuss sections 11 and 13 of the ICHD-3. However, before considering the diagnoses that are commonly attributed to orofacial pain, it is relevant to provide a brief comment on eliciting the key components of the history and physical examination in the evaluation of headache and orofacial pain. It is important to take a stepwise, systematic approach to the patient’s pain. This requires a fundamental knowledge of the ICHD-3 criteria. It should be noted that in one series of 97 consecutive patients presenting to a tertiary care neurologic facility, 29% were not classifiable using the ICHD criteria.
An appropriate physical examination includes a thorough neurologic assessment (including gait, pronator drift, Romberg sign, reflex testing; Hoffman and Babinski signs), heart and carotid auscultation, fundoscopic examination, cervical range of motion (ROM including atlanto-axial and atlanto-occiptial joint), a musculoskeletal evaluation with careful detail to myofascial tenderness and trigger points, maneuvers that provoke radicular signs (Spurling test), cervical facet examination, and Waddell signs of nonorganic pain (tenderness to palpation, stimulation, distraction, regional disturbance in function, and overreaction). Tables 23.2 and 23.3 list the ominous findings and characteristics indicating the need for neuroimaging evaluation, respectively.
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| Urgent |
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| Routine |
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Anatomy and Pathophysiology
The trigeminal system provides the relay system for pain and touch sensation to the face and motor function to the muscles of mastication. The trigeminal system is a bilateral structure that spans from the midbrain to the medulla and is composed of four nuclei: the mesencephalic nucleus, the main sensory nucleus, a spinal nucleus of cranial nerve (CN) V, and the motor nucleus. The caudal portion of the trigeminal system nucleus is referred to as the spinal nucleus of CN V and is composed of three regions, in cephalad to caudal order: the subnucleus oralis, the subnucleus interplaris, and the subnucleus caudalis. The subnucleus caudalis is very similar in structure and function to the dorsal horn and extends down to the second or third cervical level. The primary afferent synapses ipsilaterally in the nucleus caudalis and the second order neuron crosses to join the contralateral spinothalamic tract. The trigeminal pathway is termed the ventral trigeminothalamic tract and terminates in the ventral posteromedial (VPM) nucleus of the thalamus.
Activation of nuclei near the trigeminocervical complex may explain the associated aura and symptoms attributed to different headache disorders by either activation of wide dynamic neurons, ephaptic transmission, or by sheer proximity to the complex (solitary nucleus, nucleus ambiguous, or dorsal nucleus of vagus nerve).
Goadsby demonstrated the trigeminocervical convergence mechanism. He electrically stimulated the superior sagittal sinus of adult monkeys, and C-fos was expressed in the superficial laminae of the dorsal horn of C1 and C2 but not in C3.
Anthony examined patients with occipital headache, and he summarized that “projection of pain…overlap between trigeminal nucleus and upper cervical cord…form a column of cells forming the posterior horn…to C4.” Consequently, the trigeminocervical complex with the hypothesized reciprocal interactions described by Goadsby appear to introduce potential neuromodulatory/ablative sites for a variety of headache disorders in contrast to the accepted headache generator foci ( Table 23.4 ).
| ICHD-3 | Diagnosis |
|---|---|
| 11 | Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure |
| 11.1 | Headache attributed to disorder of cranial bone |
| 11.2 | Headache attributed to disorder of neck |
| 11.2.1 | Cervicogenic headache |
| 11.2.2 | Headache attributed to retropharyngeal tendonitis |
| 11.2.3 | Headache attributed to craniocervical dystonia |
| 11.3 | Headache attributed to disorder of eyes |
| 11.3.1 | Headache attributed to acute glaucoma |
| 11.3.2 | Headache attributed to refractive errors |
| 11.3.3 | Headache attributed to heterophoria or heterotropia (latent or manifest squint) |
| 11.3.4 | Headache attributed to ocular inflammatory disorder |
| 11.4 | Headache attributed to disorder of ears |
| 11.5 | Headache attributed to disorder of the nose or paranasal sinuses |
| 11.6 | Headache attributed to disorder of teeth or jaw |
| 11.7 | Headache or facial pain attributed to temporomandibular disorder (TMD) |
| 11.8 | Head or facial pain attributed to inflammation of the stylohyoid ligament |
| 11.9 | Headache or facial pain attributed to other disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure |
Headache Attributed to Disorder of Cranial Bone
The diagnostic criteria include pain in one or more regions in the head and face with clinical, laboratory, or imaging evidence of a lesion within the cranial bone known to be a valid evidence of generating headache. The source of the pain must be in close temporal association to and be maximal over the bone lesion and with resolution of the pain after successful treatment of the bone lesion. Most disorders of the skull are not painful with the exception of osteomyelitis, multiple myeloma, and Paget disease.
Headache Attributed to Disorder of Neck
These constellations of disorders involve pain referral from neck structures to the head/and or face. The diagnostic criteria hinges on what is excepted as “being generally accepted as a valid cause of headache.”
Cervicogenic Headache
Cervicogenic headache is pain attributed to a disorder or lesion within the cervical spine or soft tissues that is generally accepted to cause headache or facial pain (see Chapter 22 ).
Retropharyngeal Tendonitis
Retropharyngeal tendonitis (also called longus colli tendonitis) is described as either unilateral or bilateral nonpulsatile pain in the posterior neck radiating to the occiput or entire head accompanied by swollen prevertebral soft tissue measuring greater than 7 mm in adults anterior to the upper cervical vertebral bodies. The pain is exacerbated by neck extension and less commonly with neck rotation and swallowing. The transverse process of the upper three vertebral bodies is tender to palpation. The pain is alleviated within 2 weeks of treatment with antiinflammatory medications. Imaging studies are needed to exclude carotid dissection and in some cases CT aspiration of amorphous calcific material from the swollen periverterbal tissues.
Acute retropharyngeal tendinitis typically occurs in the third through sixth decade of life and presents as a triad of neck pain, odynophagia, and fever. Treatment is usually conservative and includes nonsteroidal antiinflammatory drugs (NSAIDs) or a short course of corticosteroids and it is self-limited in most cases.
Craniocervical Dystonia
Craniocervical dystonia (CCD) is characterized by crampy or “tension-type pain” in the posterior neck radiating to the occiput or entire head accompanied by defective posture of the head or neck due to muscular hyperactivity. The pain is exacerbated by muscle contraction, movement, external pressure, or sustained posture. The pain resolves within 3 months of successful treatment of the underlying muscle hyperactivity. These dystonias include pharyngeal dystonia, spasmodic torticollis, mandibular dystonia, or lingual dystonia.
The prevalence of CCD was estimated to be 1.1–6.1 per 100,000 people with an incidence of 1.1 per 100,000 per year. Studies on the pathophysiology of CCD suggest functional defects in dopamine signaling. Treatment involves physical therapy, muscle relaxants, and botulinum toxin injections.
Headache Attributed to Rhinosinusitis
This is a secondary cause of frontal headache and pain in one or more region of the face, ears, or teeth that is accompanied by clinical, radiographic, endoscopic, or laboratory evidence of acute rhinosinusitis ( Fig. 23.1 ). Clinical causes include purulence within the nasal cavity, nasal obstruction, new-onset hyposmia/anosmia, and/or fever. The headache/facial pain onset must be congruent with the acute rhinosinusitis and must resolve within 7 days after remission or successful treatment. Conditions that are not considered as causing this headache include deviated septum, nasal turbinate hypertrophy, and sinus membrane atrophy. Chronic sinusitis is not validated as a cause of headache or facial pain unless there is an underlying acute exacerbation.
Headache Attributed to Disorder of Teeth, Jaws, or Related Structures
Disorders of the teeth, jaws, or related structures typically cause toothache and facial pain, and less commonly headache. Pain from the teeth may be referred and cause diffuse headache, as in periodontitis or pericoronitis because of infection or traumatic irritation around the wisdom teeth. The pain is both temporally and structurally related to a disorder of the teeth and/or jaw and is relieved within 3 months of successful treatment of the underlying pathology.
Acute periodontal nociceptive pain is treated with rest (reduced mechanical stimulation), NSAIDs, topical local anesthetics, and analgesics. Chronic periodontal disease is an immune-mediated inflammatory process that results in destruction of the teeth and the surrounding anchoring bone.
Typically, intraoral lesions are self-limited and resolve within a few weeks. If symptoms persist, dental, or ENT referral is warranted. Some common painful mucosal conditions are listed in Table 23.5 .
| Category | Condition |
|---|---|
| Infections | Herpetic stomatitis |
| Varicella zoster | |
| Candidiasis | |
| Acute necrotizing gingivostomatitis | |
| Immune/autoimmune | Allergic reactions (toothpaste, mouthwashes, topical medications) |
| Erosive lichen planus | |
| Benign mucous membrane pemphigoid | |
| Aphthous stomatitis and aphthous lesions | |
| Erythema multiforme | |
| Graft-versus-host disease | |
| Traumatic and iatrogenic injuries | Factitial, accidental (burns: chemical, solar, thermal) |
| Self-destructive behaviors (rituals, obsessive behaviors) | |
| Iatrogenic (chemotherapy, radiation) | |
| Neoplasia | Squamous cell carcinoma |
| Mucoepidermoid carcinoma | |
| Adenocystic carcinoma | |
| Intracranial tumors | |
| Neurologic | Burning mouth syndrome and glossodynia |
| Neuralgias | |
| Postviral neuralgias | |
| Posttraumatic neuropathies | |
| Dyskinesias and dystonias | |
| Nutritional and metabolic | Vitamin deficiencies (B 12 , folate) Mineral deficiencies (iron) |
| Diabetic neuropathy | |
| Malabsorption syndromes | |
| Miscellaneous | Xerostomia, secondary to intrinsic or extrinsic conditions |
| Referred pain from esophageal or oropharyngeal malignancy | |
| Mucositis secondary to esophageal reflux | |
| Angioedema |
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