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8/15/2019 Cephalgia and Dental 2 http://slidepdf.com/reader/full/cephalgia-and-dental-2 1/18 Review Article Orofacial Pain: A Guide for the Headache Physician Martina K. Shephard, BDent(Hons), MBBS(Hons), FRACDS; E. Anne MacGregor, MD, FFSRH; Joanna M. Zakrzewska, MD, FDSRCS, FFPMRCA Orofacial pain represents a significant burden in terms of morbidity and health service utilization.It includes very common disorders such as toothache and temporomandibular disorders, as well as rare orofacial pain syndromes. Many orofacial pain conditions have overlapping presentations,and diagnostic uncertainty is frequently encountered in clinical practice.This review provides a clinically orientated overview of common and uncommon orofacial pain presentations and diagnoses, with an emphasis on conditions that may be unfamiliar to the headache physician. A holistic approach to orofacial pain management is important, and the social, cultural, psychological and cognitive context of each patient needs to be considered in the process of diagnostic formulation, as well as in the development of a pain management plan according to the biopsychosocial model. Recognition of psychological comorbidities will assist in diagnosis and management planning. Key words: cranial neuralgia, dental pain, headache, multidisciplinary management, orofacial pain, psychological comorbidity Abbreviations: AAOP American Academy of Orofacial Pain, GCA giant cell arteritis, ICHD International Classification of Headache Disorders, MMP masticatory myofacial pain, MRI magnetic resonance imaging, NVOP neurovas- cular orofacial pain, RCT randomized controlled trial, RDCTMD Research Diagnostic Criteria for Temporo- mandibular Disorders, TMD temporomandibular disorder, TN trigeminal neuralgia (Headache 2014;54:22-39) Orofacial pain may be defined as pain localized to the region above the neck, in front of the ears and below the orbitomeatal line, as well as pain within the oral cavity. 1 It includes pain of dental origin and temporomandibular disorders (TMDs), and thus is widely prevalent in the community. Up to a quarter of the population reports orofacial pain (excluding dental pain), and up to 11% of this is chronic pain. 2 Patients with orofacial pain present to a variety of clinicians, including headache physicians, dentists, maxillofacial surgeons, otolaryngologists, neurologists, chronic pain clinics, psychiatrists, and allied health professionals such as physiotherapists and psychologists. 3,4 Orofacial pain is associated with significant morbidity and high levels of health care utilization. 5 This review presents a clinically orientated over- view of orofacial pain presentations and diagnoses. The scope of orofacial pain includes common dis- orders such as dental pain and TMDs, as well as a number of rare pain syndromes. Pain in the orofacial region is derived from many unique tissues such as teeth, meninges, and cornea. This results in several unique physiological mechanisms that have been well From the Oral Medicine Unit, Eastman Dental Hospital, UCLH NHS FoundationTrust,London, UK (M.K.Shephard); Centre for Neuroscience & Trauma, Blizard Institute of Cell and Molecular Science, Barts and the London School of Medi- cine and Dentistry, London, UK (E.A. MacGregor); Eastman Dental Hospital, UCLH NHS Foundation Trust, London, UK (J.M. Zakrzewska). Address all correspondence to M.K. Shephard, Oral Medicine Unit, Eastman Dental Hospital, UCLH NHS Foundation Trust, London WC1X 8LD, UK. Accepted for publication September 30, 2013. Conflict of Interest : No authors report a relevant conflict of interest. ISSN 0017-8748 doi: 10.1111/head.12272 Published by Wiley Periodicals, Inc. Headache © 2013 American Headache Society 22

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Review Article

Orofacial Pain: A Guide for the Headache Physician

Martina K. Shephard, BDent(Hons), MBBS(Hons), FRACDS; E. Anne MacGregor, MD, FFSRH;

Joanna M. Zakrzewska, MD, FDSRCS, FFPMRCA

Orofacial pain represents a significant burden in terms of morbidity and health service utilization. It includes very common

disorders such as toothache and temporomandibular disorders, as well as rare orofacial pain syndromes. Many orofacial pain

conditions have overlapping presentations, and diagnostic uncertainty is frequently encountered in clinical practice.This review

provides a clinically orientated overview of common and uncommon orofacial pain presentations and diagnoses, with an

emphasis on conditions that may be unfamiliar to the headache physician. A holistic approach to orofacial pain management

is important, and the social, cultural, psychological and cognitive context of each patient needs to be considered in the processof diagnostic formulation, as well as in the development of a pain management plan according to the biopsychosocial model.

Recognition of psychological comorbidities will assist in diagnosis and management planning.

Key words: cranial neuralgia, dental pain, headache, multidisciplinary management, orofacial pain, psychological comorbidity

Abbreviations: AAOP American Academy of Orofacial Pain, GCA giant cell arteritis, ICHD International Classification of 

Headache Disorders, MMP masticatory myofacial pain, MRI magnetic resonance imaging, NVOP neurovas-

cular orofacial pain, RCT randomized controlled trial, RDCTMD Research Diagnostic Criteria for Temporo-

mandibular Disorders, TMD temporomandibular disorder, TN trigeminal neuralgia

(Headache 2014;54:22-39)

Orofacial pain may be defined as pain localized to

the region above the neck, in front of the ears and

below the orbitomeatal line, as well as pain within the

oral cavity.1 It includes pain of dental origin and

temporomandibular disorders (TMDs), and thus is

widely prevalent in the community. Up to a quarter of 

the population reports orofacial pain (excluding dental

pain), and up to 11% of this is chronic pain. 2 Patients

with orofacial pain present to a variety of clinicians,

including headache physicians, dentists, maxillofacial

surgeons, otolaryngologists, neurologists, chronic pain

clinics, psychiatrists, and allied health professionals

such as physiotherapists and psychologists.3,4 Orofacial

pain is associated with significant morbidity and high

levels of health care utilization.5

This review presents a clinically orientated over-

view of orofacial pain presentations and diagnoses.

The scope of orofacial pain includes common dis-orders such as dental pain and TMDs, as well as a

number of rare pain syndromes. Pain in the orofacial

region is derived from many unique tissues such as

teeth, meninges, and cornea. This results in several

unique physiological mechanisms that have been well

From the Oral Medicine Unit, Eastman Dental Hospital,

UCLH NHS Foundation Trust, London, UK (M.K. Shephard);

Centre for Neuroscience & Trauma, Blizard Institute of Cell

and Molecular Science, Barts and the London School of Medi-

cine and Dentistry, London, UK (E.A. MacGregor); Eastman

Dental Hospital, UCLH NHS Foundation Trust, London, UK

(J.M. Zakrzewska).

Address all correspondence to M.K. Shephard, Oral Medicine

Unit, Eastman Dental Hospital, UCLH NHS Foundation

Trust, London WC1X 8LD, UK.

Accepted for publication September 30, 2013.

Conflict of Interest : No authors report a relevant conflict of 

interest.

ISSN 0017-8748doi: 10.1111/head.12272

Published by Wiley Periodicals, Inc.Headache© 2013 American Headache Society

22

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reviewed.6 Because of these unique mechanisms and

the requirement for specialist knowledge of the

complex anatomy and physiology of the orofacial

region, diagnosis may be difficult. Many patients haveconsulted multiple clinicians for their condition yet

remain undiagnosed or with an incorrect diagnosis.7,8

Our aim is to provide the headache physician with a

guide to orofacial pain presentations and diagnoses

informed by our clinical experience in the fields of 

medicine as well as dentistry, and to review the litera-

ture relevant to these conditions. We provide an over-

view of the common presentations of orofacial pain

including dental causes of pain, non-dental causes of 

intraoral pain, and extraoral facial pain syndromes, as

the signs and symptoms of many of these conditions

can overlap significantly, causing diagnostic difficulty.

We also present a discussion of history, diagnosis,

and management considerations relating to the

biopsychosocial model of diagnostic formulation and

management.This approachis particularly relevant and

important in the field of orofacial pain given the signifi-

cant level of psychological distress and social dysfunc-

tion that is associated with these disorders.9,10 As with

other types of chronic pain, there is often a mismatch

between the patient’s expectation of a cure for their

pain,and thereality that formany types of chronic pain,a cure is seldompossible. Medicine alone does nothave

the tools to manage a condition that has a neurophysi-

ological cause but is also experienced emotionally,

socially,financially,and spiritually.11 Recognition of psy-

chological comorbidities is essential for appropriate

diagnosis and successful pain management.

METHODOLOGY

This is a narrative, clinically orientated review of 

orofacial pain conditions encountered in a specialist

orofacial pain clinic, with references to relevant lit-

erature. Quotations from patients are included to

illustrate relevant points as these also form part of the

evidence base.12 The types of orofacial pain have been

divided into sections as shown in Figure 1. Informa-

tion regarding dental causes of orofacial pain is

included as this area is often unfamiliar to medical

practitioners. Evidence-based management options,

Fig 1.—Classification of orofacial pain.

Headache   23

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as far as possible, for the specific diagnoses are sum-

marized and presented in a tabulated form (Table). In

the second half of the paper, we discuss holistic man-

agement approaches to orofacial pain.

TYPES OF OROFACIAL PAIN

Dental Pain.—There are few causes for dental

pain; however, because of significant neural conver-

gence in the jaws and face, it may be referred, poorly

localized, or misdiagnosed. The 4 major causes of 

dental pain are pulpitis, cracked tooth syndrome,

dental abscess, and dentine sensitivity.13 These are

often acute conditions, but because they are common,

they may coexist with other chronic pains.14

Both the dental pulp and periodontal ligament

contain nociceptors. Nociceptive output in these

areas is triggered by changes in pressure and theeffect of inflammatory mediators.

Pulpitis.—Pulpitis is the term used to describe pain

because of inflammation of the dental pulp, and it is

usually due to dental caries. Inflammation of the pulp

leads to accumulation of extracellular fluid, inflam-

matory mediator release, and vasodilatation, which

causes an elevation of pressure within the pulp

chamber, which is a non-compliant space. The pres-

sure increases further as venous stasis and eventually

pulp necrosis occur, with release of inflammatory

mediators and necrotic cell contents. Elevated pres-sure and inflammatory chemicals activate nociceptors

in the pulp chamber causing pain.

Reversible pulpitis is defined as a transient pain

in response to specific stimuli (hot, cold, sweet), which

occurs when the pulp is inflamed. These symptoms

resolve when the cause of the inflammation is treated.

The pain of reversible pulpitis may be described as

fleeting, shooting, stabbing, or sensitive.

Irreversible pulpitis is characterized by spontane-

ous pain, which may be worsened by or persist fol-

lowing the removal of a stimulus such as heat or cold.

It is an indicator of incipient pulpal necrosis.The pain

of irreversible pulpitis is often described as persistent,

throbbing, dull, or aching. It may be worsened by

physical activity and head movement.

Pulpal pain is often poorly localized as the

inflammation is restricted to the pulp chamber and is

thus not affecting proprioceptive nerve fibers, which

are located in the periodontal ligament. It is common

for patients to be unable to localize the exact source

of the pain. Pulpal pain may respond to simple or

opioid-based analgesics, but the pain of irreversible

pulpitis will not resolve until pulpal necrosis has

occurred or the pulpal tissue has been mechanically

removed (by endodontic treatment).

If pulpal inflammation and infection reaches the

base of the pulp chamber, an area known as the apex

or root tip, it may extrude through the apical foramen

into the periodontal space (Fig. 2). This will cause

pain due to stimulation of nociceptors in the peri-

odontal ligament space, and the pain will be well

localized due to involvement of periodontal ligament

proprioceptive fibers. Extrusion of inflammatory fluid

and necrotic cell products into the periodontal space

causes pain because of pressure effects, and the toothwill become exquisitely tender to touch or biting. This

leads to the pain becoming very well localized, and

the source of pain may be readily identified by gentle

tapping on the tooth. When inflammation and infec-

tion has progressed through the apical foramen, it is

described as a periapical abscess.

Dental infection may progress into the bone,

under the oral mucosa or into soft tissue spaces, and

form an abscess or spreading infection, with resultant

ongoing pain.

Cracked Tooth Syndrome.—Cracked tooth syn-drome occurs when a crack has occurred in the dental

hard tissues and reaches the pulp chamber. The crack

is usually not visible to the naked eye. Pain because of 

cracked tooth syndrome is classically intermittent,

provoked on biting or releasing biting on a hard

object, and is notoriously difficult to diagnose. It may

be described as sharp or sensitive, and is usually

related to mastication. The tooth may also become

sensitive to hot and cold stimuli. It is thought that the

pain is due to fluid shifts within the dentine tubules,

which are generated due to pressure differences as

the crack opens and closes during mastication. It can

be extremely difficult to diagnose.15

Dentine Sensitivity.—Pain because of dentine sen-

sitivity is classically stimulated by exposure to cold,

heat, sweet foods/drinks, and mechanical trauma such

as toothbrushing. The sensation is due to the move-

ment of fluid in dentinal tubules in response to

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osmotic or temperature-related effects. Dentinal

tubules contain the processes of cells residing in thedental pulp (odontoblasts), and fluid movement

appears to trigger nociceptive output by mechanisms

that are as yet unclear. Gingival recession can lead to

exposure of the endings of dentine tubules, as can loss

of enamel on the crown of the tooth. Dentinal sensi-

tivity is described as very rapid, fleeting, shooting

pain, or sensitivity, and is always in response to an

identifiable stimulus.

NON-DENTAL INTRAORAL PAIN

Intraoral pain may also arise from non-dental

structures.16 Oral mucosal malignancies such as squa-

mous cell carcinoma or salivary gland carcinoma may

be painful because of ulceration or perineural invasion.

Inflammatory oral mucosal diseases such as oral

lichen planus, recurrent aphthous stomatitis, vesi-

culobullous diseases, and oral mucosal infections such

as candidiasis or herpes viruses (herpes simplex, vari-

cella zoster) may all cause significant oral pain.

Patients with hematinic deficiencies, diabetes, hema-tological malignancies, HIV/AIDS, and Behçet’s

disease may have significant oral mucosal pain and/or

ulceration. Examination will usually reveal the asso-

ciated oral mucosal abnormalities.17

Pain maybe experienced in theoral cavity,face,and

neck because of salivary gland pathology. Blockage of a

major salivary gland duct may be due to infection,

mechanical obstruction by tumors, docholithiasis, or

ductal strictures. Obstruction of the duct will lead to

pain as the gland fills with saliva, which cannot be

released. Pain due to chronic ductal obstruction typi-

cally worsens preprandiallyor during meal times. Infec-

tion of the salivary glands will result in gland swelling,

pain, and erythema/warmth of the overlying skin.

Post-Traumatic Trigeminal Neuropathic Pain/ 

Atypical Odontalgia.—This definition encompasses

intraoral pain that is localized to a non-diseased den-

toalveolar structure, such as a tooth or an area of 

Fig 2.—Dental anatomy.

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alveolar ridge from which a tooth has previously been

extracted.18 The pain is often described as “burning,”

“shooting,” or “shock-like,” and there may be signifi-

cant hyperalgesia and allodynia of the affected

region, often with an associated area of hypoesthesia

or dysesthesia. The pain is usually continuous, with

some patients experiencing evoked severe episodes.

The area is usually clearly defined with little radia-

tion.19,20 Patients have described it as “nails being hit

the whole time”or “kicked in the face and left bruised

and burning.”

Controversy remains about nomenclature and

criteria for these conditions, and in this article, we

differentiate them by the presence or absence of a

precipitating event. It has been proposed that formal

neurophysiological testing would help distinguish

those with neuropathic pain compared with inflam-matory causes.20-22 Patients with trigeminal neuro-

pathic pain have an identifiable traumatic episode

preceding the onset of the pain. The precipitating

event may include physical trauma such as facial

fractures, iatrogenic trauma such as restorative,

endodontic, or oral surgical procedures (apicectomy,

extraction, implant placement), prolonged severe

infection of dentoalveolar structures, or dental proce-

dures carried out with ineffective anesthesia.23 Tri-

geminal neuropathic pain is persistent and severe,

and associated with a high level of psychological dis-tress and a risk of further iatrogenic harm because of 

patients seeking ongoing dental or surgical interven-

tions for relief of pain.

Atypical odontalgia or persistent dentoalveolar

pain refers to a similar clinical presentation without a

clear precipitating event.24,25 “Persistent dentoalveo-

lar pain” is an ontological definition describing the

symptoms and signs without attributing a causation

or mechanism. Such definitions are developed using

analysis of patient interviews.26,27 These conditions are

usually managed along the same pathways as for

other neuropathic pain.28 Until there are internation-

ally agreed diagnostic criteria based on case–control

studies and more well-conducted trials have been

carried out, treatment of these conditions can vary

substantially between clinicians, leaving patients con-

fused and continually consulting in hope that a “cure”

will be found.

Burning Mouth Syndrome.—Burning mouth syn-

drome describes a collection of symptoms affecting

the oral cavity, including a “burning” or painful sen-

sation, often with an associated alteration in taste

sensation and an altered perception of the quality and

quantity of saliva. The symptoms are most commonly

localized to the tongue.29,30 On clinical examination,

the oral mucosa appears entirely normal. The area of 

abnormal sensation does not typically follow ana-

tomic boundaries, is usually bilateral, and is continu-

ously present. Patients may describe their symptoms

as “discomfort” rather than pain. One patient

described their symptoms as a “Prickly feeling like an

injection wearing off,” and when choosing photo-

graphic images as representative of their symptom,

many choose images of fire.31 Other causes of oral

burning sensations such as hematinic deficiencies,dia-betes, other systemic diseases, and oral infections

should be ruled out. The condition is most common in

perimenopausal or postmenopausal females, and is

strongly associated with psychological comorbidities

such as anxiety and depression.32 Patients often

report that their symptoms are worsened during

periods of psychological stress. The etiology of the

condition is unclear,although recent studies have sug-

gested the presence of a small-fiber sensory neuro-

pathy, thus suggesting it is a form of neuropathic

pain,33,34 but others propose a steroid dysregulationmechanism.35 The condition can be difficult to

manage, and a variety of RCTs have been reported,

which include drug therapies and cognitive behavior

therapy.36-38 Research on this condition is difficult to

conduct in part due to its rarity and a lack of animal

models; however, studies are being undertaken that

indicate evidence of central changes on functional

magnetic resonance imaging (MRI), thus supporting

the hypothesis that there are definite neurophysi-

ological elements to this condition, rather than it

being a psychosomatic condition as has been previ-

ously suggested.

FACIAL PAIN WITH/WITHOUT

INTRAORAL PAIN

TMDs.—TMDs are the most common causes of 

orofacial pain, affecting 10-15% of the population.39,40

Presenting features include pain localized to the

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pre- and post-auricular areas, the angle and ramus of 

the mandible, and the temporal region. There may be

associated clicking,sticking, or locking of the temporo-

mandibular joints. The pain may be intermittent or

continuous, and is usually described as dull, aching, or

throbbing, or in the words of patients: “weight on the

side of the face getting heavier and heavier,”“pressure

feeling,” “elastic band that is too tight,” or “needles

digging in.” Some patients experience pain that is sharp

or shooting in character, intermixed with dull continu-

ous pain. The pain commonly radiates into the tempo-

ral or occipital regions into the neck and across the

malar region of the face; it can be unilateral or bilat-

eral, and of varying severity. There may be an associ-

ated bruxing or clenching habit. The pain is typically

aggravated by opening the mouth wide, yawning, or

chewing. There may be limitation of mouth opening.41

TMD has historically been classified using the

Research Diagnostic Criteria into myofascial pain,disc

displacement, and other disorders,42,43 as the Interna-

tional Classification of Headache Disorders (ICHD)-II

of TMD was not useful in clinical settings.2 Newer clas-

sification criteria refer to myalgia, myofascial pain with

referral, and myalgia with disc involvement.41

A large prospective cohort study is currently

underway in the USA investigating the prognostic

factors related to the development of TMD.44-46 Par-

ticipants with and without TMD participate in abattery of psychometric, biometric, and genetic tests.

Baseline data on the psychological characteristics of 

the TMD cases demonstrate that this population

shows higher levels of distress, catastrophizing, and

increased somatic awareness compared with non-

TMD controls. A number of other studies have

reported similar findings.47-49

TMD has been linked with other psychological

and chronic pain conditions, including fibromyalgia,

back pain, headaches, chronic widespread pain, and

hypermobility.50-53 Degenerative temporomandibular

 joint disease is rare but may occur in rheumatoid

arthritis. Interest has been raised recently in the pos-

sibility of TMD-related headache, which may involve

aspects of peripheral and central sensitization.54

Management of TMD is primarily conservative,

as in the majority of cases, the disorder is self-limiting.

Careful explanations are crucial as it has been shown

that patients experience a considerable amount of 

uncertainty both in terms of diagnosis and then man-

agement, as dentists also often find it difficult to

manage.55-57 Approximately 10% of patients develop

chronic pain, and this has been linked to fibromyalgia,

depression, and chronic widespread pain.58 Therapies

used for TMD include simple analgesia, tricyclic anti-

depressants, occlusal splints or bite guards, diet modi-

fications, physiotherapy, cognitive behavioral therapy,

and surgery.59-61 Evidence for the majority of these

therapeutic options is poor, and there remains con-

siderable confusion about the best form of manage-

ment.7 Surgery is only indicated for TMD with

significant functional limitation or in cases with asso-

ciated degenerative joint disease or disc dysfunc-

tion.62 Education, psychological support and self-

management strategies are recommended as part of amultidisciplinary approach to the management of 

TMD, and these should be done early to reduce

costs.63-65 There remains considerable variation in the

way TMD is diagnosed and managed partly due to

conflicting evidence. It is anticipated that the large

US-based Orofacial Pain: Prospective Evaluation and

Risk Assessment (OPPERA) study will provide more

robust evidence, as it is a prospective study that has

enrolled asymptomatic participants.44-46

Giant Cell Arteritis.—Giant cell arteritis (GCA) is

an important differential diagnosis in any patient overthe age of 50 years presenting with temporal or pre-

auricular pain. This condition is potentially vision-

threatening and needs to be identified and treated

as a matter of urgency. The pain of GCA is often

described as “throbbing” and continuous, and may be

associated with jaw claudication, visual symptoms,

and systemic illness,including musculoskeletal pain in

the upper limbs (polymyalgia rheumatica). Clinical

examination may demonstrate a reduced pulse in a

tortuous temporal artery. Blood tests for erythrocyte

sedimentation rate and C-reactive protein (CRP)

should be performed urgently as these will assist in

confirmation of the diagnosis, followed by temporal

artery biopsy.66 If the clinical presentation is strongly

suggestive of GCA, treatment with high-dose cortico-

steroids should be commenced prior to the receipt of 

test results, and urgent referral to ophthalmology

should be made to avoid loss of vision.67

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Post-Herpetic Neuralgia.—Post-herpetic neuralgia

is a neuropathic pain arising after reactivation of 

herpes virus species (most commonly varicella

zoster), which may involve branches of the trigeminal

nerve. The first division is most commonly affected;

however, involvement of the second or third divisions

may result in facial or intraoral neuropathic pain.

The pain is unilateral and restricted anatomically to

1 or more branches of the trigeminal nerve, and

is described as “burning” and continuous.18 History

often reveals an episode of herpes virus reactivation

including the presence of “blisters,” “ulcers,” or

vesicles on the skin or intraorally, associated with

extreme pain in the same region, which typically pre-

cedes the appearance of the vesicles. Pain may persist

for up to 6 weeks following an episode of herpes

virus reactivation, and allodynia is often present onexamination. Ongoing pain and neurological abnor-

malities 3-6 months following the acute episode is

classified as post-herpetic neuralgia and is common

in elderly patients, resulting in considerable impact

on quality of life.68 There is often a complaint of 

severe itching. Management is as for other types of 

neuropathic pain.69 However, it is important to treat

the acute episode with high-dose antiviral medica-

tions and even tricyclic antidepressants in elderly

patients as they are at higher risk of developing post-

herpetic neuralgia. Antiviral medication should becommenced within 72 hours of the onset of rash/

vesicles but may be started up to 7 days following

onset, particularly in immunocompromised or older

individuals.70

Trigeminal Neuralgia.—Trigeminal neuralgia (TN)

is a condition characterized by episodic, usually

unilateral, severe attacks of facial pain, which are

often described as “shooting,” “electric shock-like,”

or “stabbing.” Metaphors used by patients include

“plugged into the mains and switched on and off” and

“rockets and explosions.”The pain attacks are of very

short duration (seconds) with a refractory period, and

periods of complete pain remission may occur, which

can last for months or even years.71 With time, the

remission periods tend to shorten. Some patients

describe a continuous dull ache or burning after an

acute attack, eg, “red hot iron being pushed and

turned inside the cheek,” and if this sensation persists,

it has variously been labeled as atypical TN, type 2

TN,72 or TN with concomitant pain.73 The pain is

restricted to the anatomical boundaries of divisions of 

the trigeminal nerve and most commonly affects the

second and third divisions. The pain is triggered by

a variety of light touch stimuli, including talking,

eating or tooth-brushing, face-washing, or cold winds.

Patients will usually be able to identify a discrete

“trigger zone” within which sensory stimuli will

produce a pain attack. It is a severe and disabling

pain, and has a significant impact upon quality of life.

One of our patients described her TN experience as

follows:“My whole life was falling apart. My husband

was losing weight, everything was falling apart in our

house, my job and there was nothing I could do about

the pain.”

In some patients, there is an identifiable cause,such as an intracranial lesion or a vascular compres-

sion of the trigeminal nerve.74 TN is reported in up to

3.8% of patients with multiple sclerosis.75 For this

reason, imaging (MRI) forms an essential part of 

the work-up for TN. Management will depend on

whether there is an identifiable cause, but is primarily

medical, and aims to achieve symptom relief. Medical

management involves the use of anticonvulsants such

as carbamazepine or oxcarbazepine.74,76 Second line

agents include lamotrigine and baclofen. Because of 

the increasing number of anticonvulsants now avail-able, many patients are referred unnecessarily late for

surgical interventions that can offer the best quality-

of-life outcomes.77 Surgical procedures such as micro-

vascular decompression may be performed if there is

imaging evidence of a lesion affecting the trigeminal

nerve root, and the disease is causing a significant

impact on quality of life.78,79 Other less invasive sur-

gical procedures such as radiofrequency thermoco-

agulation, glycerol rhizotomy, balloon compression,

or gamma knife surgery tend to provide shorter

periods of pain relief and have a higher risk of sensory

loss.They are used in patients who are medically unfit

for major surgery such as microvascular decompres-

sion. It remains difficult for patients and clinicians to

make decisions about treatment due to a lack of high-

quality evidence. Some data suggest that many

patients prefer a surgical option rather than ongoing

medical management.80

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Glossopharyngeal Neuralgia.—Glossopharyngeal

neuralgia has a similar presentation to TN, although

the location of the pain is different. Patients may

experience paroxysmal attacks of pain felt deeply in

the throat, ear, or posterior aspect of the tongue. The

triggers for pain attacks include chewing, talking,

drinking, and swallowing. The condition is usually

managed medically with anticonvulsant drugs.

Refractory cases may require surgery in the form of 

microvascular decompression.81

Anesthesia Dolorosa.—Anesthesia dolorosa is a

condition arising from damage to the trigeminal

nerve, usually during surgery for TN. The condition

develops 3-6 months following the traumatic incident.

It is characterized by “painful numbness.” Patients

will report continuous facial pain in an area of numb-

ness, often described as “burning,” “pressure,” or“stinging.” This is a typical patient description: “The

right side of my face, from my chin to above my right

eye, is numb and I frequently experience a ‘crawling’

sensation on the right side of my face and scalp.Also,

my face has quite a bit of pressure and feels as though

it is being pulled or tugged, as if in a visor.”

The pain is persistent, severe, and associated

with a high level of psychological distress and

comorbidity. It is often resistant to treatment. The

area involved may include all 3 divisions of the tri-

geminal nerve. Examination findings may includeobjective sensory deficits, allodynia, and hyperalgesia

or hypoalgesia.82 Management options are as for

neuropathic pain, with psychological support playing

a significant role. In the new ICHD classification,

this entity has been named “painful post-traumatic

trigeminal neuropathy.”18

Persistent Idiopathic Facial Pain/Atypical Facial

Pain.—This term is used to describe a facial pain pre-

sentation that does not fit the clinical pattern for any

other diagnosis and is relatively rare.18,83 It is often

continuous,“nagging” and “dull” in nature, and is not

restricted by neurological anatomical boundaries.84,85

An example of a patient’s description of the pain is:

“Concrete poured into my head and then moving

around.”

There is a high level of associated psychological

comorbidity and a high prevalence of chronic pain

elsewhere in the body.5,32 It is often associated with

conditions such as irritable bowel syndrome and

chronic widespread pain. The etiology of the condi-

tion is unclear, although recent research has sug-

gested the possibility of a pathophysiology similar to

trigeminal neuropathic pain.86,87 There is often a

history of mental health problems that may predate

the pain. Management is often difficult and includes

medical and psychological input, using a multidisci-

plinary team approach.88-90 Because of the very broad

definition that has been proposed in the new ICHD

classification, this diagnosis will continue to be

applied to a very heterogeneous group of patients and

thus limit further research into the condition.18

HEADACHE-RELATED FACIAL PAIN

Migraine and Neurovascular Orofacial Pain.—Migraine may manifest as facial pain either because

of referral or as a phenomenon referred to as atypical

or lower half migraine.91 Some authors have sug-

gested the presence of a separate entity that they

have named neurovascular orofacial pain (NVOP).92

This is a rare presentation and may mimic a number

of other orofacial pain diagnoses. The pain is usually

experienced in the distribution of the second or third

divisions of the trigeminal nerve and is episodic.

Attacks generally last for longer than 60 minutes. It is

often described as “throbbing” and may have accom-panying autonomic signs or systemic symptoms such

as nausea. Patients may also complain of dental sen-

sitivity, which can introduce diagnostic difficulties as

patients pursue treatment for a perceived dental

source of pain. NVOP has features in common with

migraine as well as trigeminal autonomic ceph-

alalgias, and it is suggested that NVOP may represent

“relocated” migraine.93 It is important to differentiate

NVOP from dental pulpal pathology, with which it is

often confused due to the presence of dental sensitiv-

ity during attacks. A case series of 7 lower facial

migraines showed that all cases responded to triptans,

and 3 responded to migraine prophylactic measures.94

Case–control studies from a range of different clinical

settings are necessary in order to provide more evi-

dence for the presence of this entity, as its manage-

ment can be substantially different to other orofacial

pain diagnoses.

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Trigeminal Autonomic Cephalalgias.—This group

includes cluster headache, paroxysmal hemicrania,

short-lasting unilateral neuralgiform headache

attacks with cranial autonomic features, and short-

lasting unilateral neuralgiform headache attacks with

conjunctival injection and tearing. These headaches

are characterized by unilateral head or facial pain

with cranial autonomic features that occur ipsilater-

ally and at the same time as the pain. 18 Patients with

these disorders may present to facial pain clinics, as

the facial pain component may be more significant

than the headache. Accurate history-taking is essen-

tial in formulating this diagnosis, as patients may be

unaware of the autonomic symptoms unless specifi-

cally asked. Comprehensive discussion of these disor-

ders may be found in the literature. However, more

careful phenotyping and larger case series are neces-sary to determine which of these diagnoses are

unique entities and which may represent a continuum

in the natural history of these disorders.95,96

TMD-Related Headache.—Recent studies have

described an association between TMD and head-

ache. Many patients with TMD also report headache,

and in some cases, there is a clear relationship

between temporomandibular joint-related triggers

and headache onset.97 TMD is also common among

migraine and tension-type headache sufferers.98

A HOLISTIC APPROACH TO FACIAL PAIN

The History.—Accurate and comprehensive

history-taking is essential in order to gather sufficient

information in order to formulate a diagnosis and

treatment plan.99 The medical consultation has been

described as “a transaction that involves translation,”

and further that “the physician’s concern is to trans-

late the subjective experience of illness into the rec-

ognizable discourse of medicine.”100 It has also been

suggested that we should not be “taking” a history but

“receiving it.”100 Inaccurate or inappropriate “transla-

tion” can lead to inaccuracy of diagnosis and impair

the therapeutic relationship.

Our unit advocates the use of a structured or

semistructured history in order to ensure consistency

in history-taking and documentation, and to assist in

diagnostic accuracy. An open-ended style of history-

taking, rather than an interrogative approach, often

yields important information and ensures that

patients feel they have been listened to and their

health beliefs understood.101,102 Building a therapeutic

relationship is essential in the assessment and man-

agement of chronic pain. Ensuring sufficient duration

for the initial consultation, allowing the patient time

to speak and express their ideas regarding the pain,

and eliciting and understanding patient expectations

are all essential for successful pain management.103 A

recent study of 12 patients interviewed preconsulta-

tion and post-consultation in a pain clinic, without the

knowledge of the clinicians involved, provided some

of these comments:

I guess what the appointment has done is

drawn a line under it and made me think, well,

that’s fine but nothing can be done about it so I

 just need to get on with things.Even though I haven’t come away with a

cure, I feel in a better position to cope with my

symptoms.

I felt xxx listened to me more than the other

healthcare professionals I have seen and took

into account the effects the pain was having on

my life in general, rather than just treating me as

a diagnosis.104

In addition to the standard pain history, psychiat-

ric comorbidities must be identified and addressed

early in the therapeutic relationship, as they may havebeen present before the onset of the pain.15 It is also

essential to elicit detailed information regarding

social history, major life events, psychosocial stressors,

and the impact of pain on the patient’s ability to

participate in the activities of daily living. Many

patients with facial pain who present to secondary

care or pain clinics have attended consultations with a

large number of primary and secondary care provid-

ers,and may have had multiple investigations or inter-

ventions for their pain.7,102,105 This is illustrated by the

following patient quotation: “A lot of people would

think [that consulting a] dentist, max facs [maxillo-

facial surgery], neurologist was already over the top

but I wanted to be certain that I’d tried everything.”

These patients often have a significant level of 

psychological distress, and this can impact negatively

on the therapeutic relationship and management

strategies. Understanding the patient’s expectations

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and illness beliefs, and assessing negative prognostic

factors such as catastrophizing or low self-efficacy

levels is essential in order to formulate an appro-

priate treatment plan using the biopsychosocial

model that will then require a multidisciplinary team

approach.106

Psychological screening tools such as the

National Institutes of Clinical Excellence depression

screening questions, the Hospital Anxiety and

Depression Scale, Patient Health Questionnaire, and

the Beck Depression Inventory are useful for quan-

tifying the degree of psychological comorbidity.107 The

inclusion of an objective measure of pain impact on

quality of life is essential in every facial pain consul-

tation; the Graded Chronic Pain Scale, Brief Pain

Inventory (including the extended version),108 the

Pain Catastrophizing Scale, and the EuroQoL scaleare useful tools. However, these measures need to be

carefully interpreted in the context of the patient’s

comorbidities. As 1 patient commented: “And if 

you’re very depressed and it’s hard to verbalize how

you feel about things, or whether you can’t just mark

on a scale between nought and ten what your pain is

like, you know, what’s your pain, is it nought or is it

ten?”31

Diagnostic Considerations.—There is also the pro-

pensity for clinicians to “label” patients with a diag-

nosis, with the expectation that this will enable thepatient to accept the condition and progress with

treatment. This approach may be helpful for some

patients – 1 patient stated: “I was quite relieved to

have a diagnosis . . . although I had hoped I would

come away with a solution for a cure, I am happy now

that I know the cause and that it is not serious.” 104

However, our experience is that this is often not

the case. Some facial pain presentations are diagnos-

tically challenging, and the evolution of symptoms

over time may either clarify, or rule out, the diagnosis

initially given. Extant classification systems may also

hinder diagnosis or result in inaccurate labeling. It has

been found that the number of patients whose symp-

toms could not be classified as a specific diagnosis was

larger in ICHD-II than in ICHD-I, with particular

difficulty experienced in patients with persistent idio-

pathic facial pain.109 In a study examining the useful-

ness of the ICHD-II classification criteria,only 56% of 

patients were successfully diagnosed with orofacial

pain using ICHD-II.2 ApplyingAmericanAcademy of 

Orofacial Pain (AAOP)/Research Diagnostic Criteria

for Temporomandibular Disorders (RDCTMD) crite-

ria, a further 37% were diagnosed with masticatory

myofascial pain (MMP), and further published criteria

enabled the remainingpatients to be allocatedto other

predefined diagnoses. The authors concluded that

while MMP is clearly defined by AAOP and the

RDCTMD, expansion of ICHD-II was needed so as to

integrate more orofacial pain syndromes.

It may be better to give no diagnosis rather than

the wrong diagnosis, as revising a diagnosis that has

previously been presented to the patient as definitive

can be damaging to the therapeutic relationship and

the patient’s confidence in the clinician. The use of a

grading system such as “definite,”“probable,” or “pos-sible” has been suggested for use when diagnosing

neuropathic pain.110 This classification could be

extended to other orofacial pain diagnoses as a means

of managing the uncertainty in providing diagnoses

for conditions that have varied clinical presentations.

Ontological approaches to the diagnosis and classifi-

cation of facial pain syndromes aim to reduce the

problems associated with “labeling” and focus on the

use of purely descriptive terms with no inferences

made regarding mechanism or etiology.27

“Labeling” or compartmentalizing patients intodiagnostic categories also ignores the multifaceted

nature of chronic pain syndromes, particularly

orofacial pain. The patient is not the diagnosis –

rather the pain condition has occurred in a patient

who exists within a milieu of social, cultural, psycho-

logical, and cognitive influences. Patients’ beliefs

about their condition will also affect their disability

and outcome,111 as the quote in Figure 3 illustrates.

Recognizing the significance of these contributory

factors to the overall presentation is essential for

effective therapeutic dialogue as well as good man-

agement of pain.

This concept has been further explored in a

recent series of qualitative studies examining

patients’ experience and perception of orofacial

pain.26,102,105 As with any other chronic pain psycho-

logical factors will increase pain disability.112

Orofacial pain syndromes often co-exist with signifi-

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cant psychological morbidity, probably more so than

other types of chronic pain,10,113 and are risk factors

for chronicity.114

Addressing these factors is essentialfor appropriate management of the orofacial pain, as

treatment outcome has been shown to be related to

psychological comorbidity.49 Affective as well as inter-

pretative and cognitive factors play an important role

in the patient’s perception of pain. One small quali-

tative study found that their patients perceived their

orofacial pain to “have no limits and to repressively

permeate all aspects of their existence: social, practi-

cal, and emotional.”105 This illustrates the significant

impact that orofacial pain can have on quality of life,

and provides a focal point for assessment of painmanagement outcomes. Patients need to know that

although the sensation of pain may not be completely

alleviated by treatment, the impact of pain upon their

daily life can certainly be modulated.

Management.—Chronic pain management should

be holistic in nature and approach, and involves

addressing all the factors that modulate the pain expe-

rience.7 Addressing unrealistic patient expectations is

important for setting achievable treatment goals.

There remains a common perception that pain should

always be curable, as demonstrated in this quote from

a patient:“Many don’tunderstandthe pain I feel.They

think I should be over this pain by now. Others feel I

should seek other doctors. They feel there should be

something to relieve this terrible pain and ask me why

I’m not trying to find it, if it is so bad.”

Pain as defined by International Association for

the Study of Pain is both a “sensory and emotional

experience,” and it should be managed as such. A

recent study has shown that chronic musculoskeletal

pain can be experienced as a “constant adversarial

struggle,” and the researchers suggest that patient and

clinician expectations of a diagnosis and cure need

to be challenged.115 Beliefs, coping strategies, and

catastrophizing predict functioning in patients with

chronic pain, and this should be considered when

individualizing pain management programs.116 This

extends to patients’ beliefs about medication as these

will influence adherence.117

Successful pain management is also related to the

patient’sself-efficacy beliefs andability to learn anduse

positive coping strategies.118 Recognition of the contri-

bution of social, psychological, and lifestyle factors to

the pain experience, as expressed in the patient quote

earlier, is essential for taking the next steps in chronicpain management and achieving a reduction in the

impact of pain on quality of life. The provision of 

support for these next steps is a fundamental part of 

multidisciplinary pain management. Pain management

programs delivered in group settings normalizes the

pain experience, and the concept of an improved pain

experience because of observation of others with a

similar complaint is also expressed by the patient

quoted earlier. Newer techniques such as an e-learning

cognitive behavioral therapy model can be helpful.119

Supported but self-directed pain management focusedaround lifestyle changeandreducing theimpact of pain

on quality of life is a more effective management

approach in chronic pain than the traditional, didactic

biomedical model, but these strategies need to be tai-

lored appropriately.120

CONCLUSION

Orofacial pain in its fullest definition affects up to

a quarter of the population, and the associated mor-

bidity, social impact, and health costs can be high if 

these conditions are not accurately diagnosed and

managed in a timely fashion. Recognition of the

significant contribution and high prevalence of psy-

chological distress and comorbidity is essential for

successful management.Multidisciplinary approaches

and a biopsychosocial model of pain management are

an essential adjunct to established evidence-based

medical and surgical management of these conditions.

Fig 3.—Extract from a patient letter about treatment of her

orofacial pain.

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Table.—Summary of Management

Management of dental painPulpitis Removal of irritant, eg, dental caries and restorative or endodontic treatment, or extractionCracked tooth Reducing movement across crack, eg, full-coverage restoration of tooth, may require endodontic

treatment or extractionDentine sensitivity Desensitizing agent application, dental restoration to cover exposed dentine

Abscess Remove source of infection

Management of non-dental intraoral painOral malignancy Surgical resection  ±  chemoradiotherapy

Opioid analgesiaInflammatory oral

mucosal diseaseTopical anti-inflammatory agentsSystemic and topical steroidsSystemic immunosuppressants (rarely)

Salivary duct blockage Sialography, irrigation, and ductal dilatationEndoscopic removal of stoneLithotripsy of stoneAntibiotics for infectionSurgical removal of gland

Trigeminal neuropathicpain

Topical anestheticsTricyclic antidepressants

AnticonvulsantsPsychological approaches

Atypical odontalgia As aboveBurning mouth

syndromeClonazepamTricyclic antidepressantsPsychological approaches

Management of facial pain with/without intraoral painTemporomandibular

 joint disorderConservative management and simple analgesiaSurgical intervention (in specific scenarios)Psychological approaches

Giant cell arteritis Early initiation of high-dose systemic steroidsPost-herpetic neuralgia Systemic antiviral medication

Tricyclic antidepressantsAnticonvulsants

Psychological approachesTrigeminal neuralgia Carbamazepine/oxcarbazepineLamotrigine, pregabalinMicrovascular decompression and other ablative procedures

Glossopharyngealneuralgia

As for trigeminal neuralgiaSurgical management options include microvascular decompression

Anesthesia dolorosa Tricyclic antidepressantsAnticonvulsantsPregabalin/gabapentinor  combinations of above drugsPsychological approaches

Persistent idiopathicfacial pain

Tricyclic antidepressantsSNRIsCognitive behavioral therapy

Management of headache-related facial painMigraine/neurovascular

orofacial painAcute:Simple analgesia/NSAIDs  ±  anti-emeticsTriptansPreventative:Beta-blockersTricyclic antidepressantsTopiramate/sodium valproateGabapentin

Headache   33

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 Acknowledgment: JZ undertook this work at UCL/ 

UCLHT, who received a proportion of funding from

the Department of Health’s NIHR Biomedical Research

Centre funding scheme.

STATEMENT OF AUTHORSHIP

Category 1(a) Conception and Design

Martina K. Shephard, Joanna M. Zakrzewska

(b) Acquisition of Data

Martina K. Shephard, Joanna M. Zakrzewska

(c) Analysis and Interpretation of Data

Martina K. Shephard, E. Anne MacGregor,

Joanna M. Zakrzewska

Category 2

(a) Drafting the Manuscript

Martina K. Shephard(b) Revising It for Intellectual Content

Martina K. Shephard, E. Anne MacGregor,

Joanna M. Zakrzewska

Category 3

(a) Final Approval of the Completed Manuscript

Martina K. Shephard, E. Anne MacGregor,

Joanna M. Zakrzewska

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