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Facial pain affects an estimated 1.9% of the world’s population, with twice as many women affected compared to men.
Because of its complex nature, a team-based approach is preferred in the diagnosis and treatment of facial pain.
A subset of migraine headache patients suffers from compression of peripheral nerves of the head and neck.
These patients can be diagnosed through an algorithmic approach involving thorough headache history, hand-held Doppler, local anesthetic blocks, BTX-A, and imaging studies.
Muscle compression was first identified as a cause of peripheral nerve compression migraine headaches but subsequently arteries, bone, and fascia have also been shown to be potential culprits.
Botox can be used to identify and treat trigger sites caused by muscular compression, but local anesthetic injection is more accurate in identifying compression from other causes.
Surgical treatment of symptomatic nerve compression can be via either an open or endoscopic approach, with decompression or avulsion of the nerve depending on the etiology.
Three randomized controlled trials found a 35%–57% elimination rate in migraine headache symptoms following migraine surgery.
The prevailing theory for poor response to peripheral nerve decompression migraine surgery is both missed diagnosis of a secondary compression site as well as incomplete release of all anatomic points of compression of a primary trigger site.
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Facial pain encompasses a broad range of pain confined to the head and neck region, including the oral cavity. Though accurate data are lacking on the prevalence, it has been estimated that facial pain affects 1.9% of the world’s population, with twice as many women affected compared to men. There is no single etiology as various anatomic structures and disease processes can result in facial pain. As such, it is not uncommon for multiple medical and surgical specialties (neurologists, dentists, otolaryngologists, plastic surgeons, neurosurgeons, pain management, physical medicine specialists, psychiatrists, etc.) to be involved in the treatment of facial pain. Paralleling the multidisciplinary nature of caring for patients with facial pain, there is an equal, if not greater, number of classification systems of facial pain. These include the most widely used International Classification of Headache Disorders (ICHD) as well as the classification systems from the American Academy of Orofacial Pain, Orofacial Pain Special Interest group of the International Association for the Study of Pain, International Headache Society, etc. Because of its complex nature, a team-based approach is preferred in the diagnosis and treatment of facial pain.
The third edition ICHD system (ICHD-3) classifies headaches based on symptoms rather than etiology. The most common type of recurrent headache is classified as episodic migraine with or without aura. Episodic migraines, in particular those associated with aura, are thought to originate from the central nervous system at the neuronal level of the brain. Criteria for episodic and chronic migraine headache are well defined based on headache quality and number of headache days per month ( Table 16.1 ). Chronic migraine is estimated to affect 0.9%–1.3% of the overall population. Chronic tension-type headache is distinct from chronic migraine in that these headaches are bilateral and not associated with phonophobia or photophobia. Though chronic tension-type headaches are more common than chronic migraine, affecting an estimated 2.2% of the total population, less is known about the pathophysiology of this entity.
Episodic migraine with or without aura | Five or more headaches |
Headaches last at least 4–72 hours (without treatment or unsuccessful treatment) | |
|
|
Nausea/vomiting or photophobia/phonophobia during headache attack | |
Chronic migraine with or without aura | ≥15 headache days per month for >3 months |
8 of the headache attacks per month must meet criteria for migraine headache (above) |
The economic effects of migraine headaches include direct and indirect costs. Direct costs include the medical costs to treat the condition while indirect costs include productivity loss and lost wages. As of 2018, total costs for migraine patients has been estimated at $2649 per year for each patient with episodic migraine, and $8243 per year for each patient with chronic migraines; 60% of these costs are estimated to be direct medical costs. A recent review of inpatient hospitalization data from 1997 to 2012 found that costs per hospitalization have been rising from $5939 per admission in 1997 to $21,576 in 2012, accounting for $1.2 billion in inpatient hospital expenditures in 2012. Headaches are among the top three most commonly reported pain conditions resulting in lost productivity at work, resulting in an average of 3.5 hours of lost productivity per week and an additional cost of $2600 to the employer in short-term disability and workers’ compensation claims.
Trigeminal neuralgia is characterized by intermittent, recurrent pain in the distribution of the trigeminal nerve that is described as “sharp and electric” in nature. Trigeminal neuralgia usually involves the second and third branches of the trigeminal nerve and rarely involves the ophthalmic division. The condition usually affects an older population, with typical onset in the sixth decade of life. Females are more commonly affected than males. The ICHD-3 criteria were recently updated to differentiate trigeminal neuralgia from painful trigeminal neuropathy. In contrast to trigeminal neuralgia, painful trigeminal neuropathy is characterized by a more continuous “burning or squeezing” pain. The distinctions for trigeminal neuralgia reflect the multiple proposed etiologies. These include classic trigeminal neuralgia due to neurovascular compression, secondary trigeminal neuralgia due to underlying medical condition (e.g., multiple sclerosis) or anatomic compression other than vascular (e.g., tumor), and idiopathic trigeminal neuralgia, in which the cause is unknown. Painful trigeminal neuropathy is typically attributed to herpes zoster, post-herpetic neuralgia, trauma, or neoplasm.
Trigeminal neuralgia is first diagnosed clinically based on symptoms. This should be followed by high resolution 3D MRI to evaluate for a neurovascular compressive lesion or posterior fossa lesion. Painful trigeminal neuropathy is likewise a clinical diagnosis and also warrants further workup based on the history of the patient.
First-line treatment of trigeminal neuralgia is with the sodium channel blockers carbamazepine or oxcarbazepine. This can be supplemented by additional agents, including lamotrigine, baclofen, pregabalin, or gabapentin. For patients with objective radiographic evidence of vascular compression, microvascular decompression should be considered. This procedure involves craniotomy with posterior fossa exploration and decompression of the affected trigeminal nerve branches. Microvascular decompression has a reported 73% success rate at 5 years for patients with vascular compression of the trigeminal nerve. Despite the high success rate, the risks involved in craniotomy must not be underestimated. Additional treatment options include stereotactic radiosurgery, balloon compression, and glycerol blockade.
Occipital neuralgia is defined by the ICHD-3 as a paroxysmal, shooting pain centered over the posterior scalp overlying the occipital nerve region. The pain typically begins at the base of the scalp and radiates anteriorly, and can be unilateral or bilateral. It is not uncommon for this condition to be associated with retro-orbital pain. The origin is traced to greater and/or lesser occipital nerves, or C2/C3 nerve roots. Occipital neuralgia has been associated with chronic migraine, tension headache, post-traumatic headache, and cervical spine abnormalities.
Diagnosis of occipital neuralgia is largely clinically based on the ICHD-3 criteria, though these headaches have been shown to respond to local anesthetic blocks. Local blocks are sensitive diagnostic tools for occipital neuralgia, but are not specific as other clinical entities such as migraine, tension headache, and cluster headache respond to local anesthetic blocks. Imaging adjuncts, such as MRI or ultrasound, can be used to help diagnose occipital neuralgia. MRI remains the gold standard to detect cervical spine and soft-tissue abnormalities that may be the causative factor for occipital neuralgia. It should be noted that a diagnosis of osteoarthritis of the cervical spine is common and, alone, does not suffice for the diagnosis of occipital neuralgia. Ultrasound can also be used to detect an occipital nerve entrapment – an enlargement in the nerve prior to an anatomic point of compression suggests external compression of an occipital nerve.
Management of occipital neuralgia is focused on the underlying pathophysiology. For postural-related occipital neuralgia, massage, cranial–cervical exercises, and physical therapy can be useful. Pharmacologic management of occipital neuralgia includes nonsteroidal anti-inflammatories, tricyclic antidepressants, anticonvulsants, gabapentin, and muscle relaxants. Botulinum toxin-A (BTX-A) has been used for occipital neuralgia, though the FDA-approved on-label indication for the use of BTX-A in headaches is specifically for the treatment of chronic migraine, which was approved in October 2010. Invasive interventions for the treatment of occipital neuralgia include pulsed radiofrequency ablation and subcutaneous occipital nerve stimulation. Pulsed radiofrequency ablation inhibits pain signaling by disrupting electrical conduction of nociceptive nerve fibers. Radiofrequency ablation is reported to provide 50% pain relief for 5–6 months. Subcutaneous occipital nerve stimulation involves insertion of electrodes in the C1/2 region with studies reporting a 62%–100% improvement in headache symptoms. Surgical management of occipital neuralgia will be covered in subsequent sections.
Migraine surgery, a form of peripheral nerve decompression surgery, began serendipitously after several patients reported unexpected elimination of their migraine headaches after forehead rejuvenation surgery. This observation was investigated with a retrospective study of forehead rejuvenation patients in which 79.5% of migraineurs reported significant improvement or complete elimination of their migraine symptoms. The contemporaneous introduction of BTX-A for the treatment of chronic migraine provided a physiologic mechanism by which migraine surgery affected headache symptoms – namely, chemical paralysis vs. surgical resection of muscle causing compression of peripheral nerves. This theory of anatomic muscle compression of peripheral nerves was investigated through multiple anatomic and translational studies. For instance, in the frontal region, the anatomic relationship of the corrugator supercilii, depressor supercilii, and procerus vis-à-vis the supraorbital and supratrochlear nerves was investigated. In the temporal region, the anatomic relationship between the zygomaticotemporal branch of the trigeminal nerve and surrounding muscular structures was measured and recorded. In the occipital region, anatomical studies found that the greater occipital nerve (GON) passed through the semispinalis capitis muscle to innervate the scalp. These findings provided an anatomic basis both to chemodenervate these muscles as a diagnostic test (or even provide non-surgical treatment in some patients) as well as to surgically decompress peripheral head and neck nerves by selective partial muscle resection.
This muscle compression theory was first tested by injecting migraine patients with BTX-A in the corrugator supercilii muscles; patients who had improvement in migraine symptoms subsequently underwent migraine surgery with resection of the corrugator supercilii muscles alone or with ablation of the zygomaticotemporal branch of the trigeminal nerve. Of these surgical patients, 45% of patients had significant improvement in headache symptoms while 50% reported complete elimination. Guyuron subsequently reported the first GON decompression procedure that involves partial resection of the semispinalis capitis muscle followed by transposition of an inferiorly based three-sided local adipose flap – essentially a subcutaneous nerve transposition. The results of these novel surgical procedures prompted the investigation of additional trigger sites including the frontal, temporal, occipital, and rhinogenic sites. Guyuron et al . performed a intervention–sham surgery comparison study investigating these trigger sites (except rhinogenic trigger sites) and reported statistically significant improvement in headache symptoms in the patients who underwent peripheral nerve decompression. The study found an 83.7% reduction in migraine headache symptoms in the intervention surgery group compared to 57.7% reduction in migraine headache symptoms in the sham surgery group (P<0.05). Additionally, 49.0% of patients in the intervention surgery group reported complete elimination of migraine headache symptoms as compared to 3.8% in the sham surgery group (P<0.001).
As anatomic understanding of peripheral nerve trigger sites grew, so did the realization that muscle compression was not the only culprit in nerve compression. Janis et al . published their findings that arteries play a compressive role in peripheral nerve compression migraine headaches. The first of these studies reported that the occipital artery is a compressive force in GON-mediated migraine headaches. Later studies demonstrated that arteries were implicated in nummular headaches since ablation of the offending vessel eliminated headaches in the affected region. Further studies also investigated the role that bony foramina and fascial bands can play in the peripheral nerve compression migraine headache trigger sites. Fallucco et al . classified the four most common bony foramen morphologies of the supraorbital nerve and foramen. More recent studies have found evidence linking the extracranial nerves to the trigeminal nucleus of the brainstem via bony canals, cranial sutures, and foramina of the skull. This provides an explanation for how extracranial peripheral nerve compression can trigger an intracranial migraine-like phenomenon. Each anatomic trigger point will be discussed in further detail in the following sections.
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