The International Classification of Headache Disorders 2nd Edition



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11. HEADACHE OR FACIAL PAIN ATTRIBUTED TO DISORDER OF CRANIUM, NECK, EYES, EARS, NOSE, SINUSES, TEETH, MOUTH OR OTHER FACIAL OR CRANIAL STRUCTURES


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 rhinosinusitis

11.6 Headache attributed to disorder of teeth, jaws or related structures

11.7 Headache or facial pain attributed to temporomandibular joint (TMJ) disorder

11.8 Headache attributed to other disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structures

Coded elsewhere:

Headaches that are due to head or neck trauma are classified under 5. Headache attributed to head and/or neck trauma. Neuralgiform headaches are classified under 13. Cranial neuralgias and central causes of facial pain.

General comment

Primary or secondary headache or both?

When a new headache occurs for the first time in close temporal relation to a craniocervical disorder, it is coded as a secondary headache attributed to that disorder. This is also true if the headache has the characteristics of migraine, tension-type headache or cluster headache. When a pre-existing primary headache is made worse in close temporal relation to a craniocervical disorder, there are two possibilities, and judgment is required. The patient can either be given only the diagnosis of the pre-existing primary headache or be given both this diagnosis and the diagnosis of headache attributed to the craniocervical disorder. Factors that support adding the latter diagnosis are: a very close temporal relation to the craniocervical disorder, a marked worsening of the pre-existing headache, very good evidence that the craniocervical disorder can aggravate the primary headache and, finally, improvement or resolution of the headache after relief from the craniocervical disorder.
Definite, probable or chronic?

A diagnosis of Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures usually becomes definite only when the headache resolves or greatly improves after effective treatment or spontaneous remission of the craniocervical disorder. If this disorder cannot be treated effectively or does not remit spontaneously, or when there has been insufficient time for this to happen, a diagnosis of Headache probably attributed to the [specified] craniocervical disorder is usually applied.

If the craniocervical disorder is effectively treated or remits spontaneously but headache does not resolve or markedly improve after 1 month, the persisting headache has other mechanisms. Nevertheless, A11.9 Chronic post-craniocervical disorder headache is described in the appendix. Headaches meeting these criteria exist but have been poorly studied and the appendix entry is intended to stimulate further research into such headaches and their mechanisms.


Introduction


Disorders of the cervical spine and of other structures of the neck and head have not infrequently been regarded as the commonest causes of headache, since many headaches originate from the cervical, nuchal or occipital regions or are localised there. Moreover, degenerative changes in the cervical spine can be found in virtually all people over 40 years of age. The localisation of pain and the x-ray detection of degenerative changes have been plausible reasons for regarding the cervical spine as the most frequent cause of headaches. However, large-scale controlled studies have shown that such changes are just as widespread among individuals who do not suffer from headaches. Spondylosis or osteochondrosis cannot therefore be seen as the explanation of headaches. A similar situation applies to other widespread disorders: chronic sinusitis, temporomandibular joint disorders and refractive errors of the eyes.

Without specific criteria it would be possible for virtually any type of headache to be classified as Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cranial structures, and this problem existed in the past. It is not sufficient merely to list manifestations of headaches in order to define them, since these manifestations are not unique. The purpose of the criteria in this chapter is not to describe headaches in all their possible subforms, but rather to establish specific causal relationships between headaches and facial pain and the disorders of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth and other facial or cranial structures where these exist. For this reason it has been necessary to identify strict specific operational criteria for cervicogenic headache and other causes of headache described in this chapter. It is not possible here to take account of diagnostic tests that are unconfirmed or for which quality criteria have not been investigated. Instead the aim of the revised criteria is to motivate as a future task the development of reliable and valid operational tests to establish specific causal relationships between headaches and craniocervical disorders that are currently available only to a very limited extent.

Headache disorders attributed to causes included here for the first time are 11.2.3 Headache attributed to craniocervical dystonia and 11.3.4 Headache attributed to ocular inflammatory disorders.

11.1 Headache attributed to disorder of cranial bone

Diagnostic criteria:

A. Pain in one or more regions of the head or face fulfilling criteria C and D

B. Clinical, laboratory and/or imaging evidence of a lesion within the cranial bone known to be, or generally accepted as, a valid cause of headache1

C. Pain develops in close temporal relation to and is maximal over the bone lesion

D. Pain resolves within 3 months after successful treatment of the bone lesion



Note:

1. Most disorders of the skull (eg, congenital abnormalities, fractures, tumours, metastases) are usually not accompanied by headache. Exceptions of importance are osteomyelitis, multiple myeloma and Paget’s disease. Headache may also be caused by lesions of the mastoid, and by petrositis.

11.2 Headache attributed to disorder of neck

Comment:

Headache attributed to disorder of neck but not fulfilling the criteria for any of 11.2.1 Cervicogenic headache, 11.2.2 Retropharyngeal tendonitis or 11.2.3 Craniocervical dystonia is not sufficiently validated.

11.2.1 Cervicogenic headache

Previously used term:

Cervical headache
Coded elsewhere:

Headache causally associated with cervical myofascial tender spots is coded as 2.1.1 Infrequent episodic tension-type headache associated with pericranial tenderness, 2.2.1 Frequent episodic tension-type headache associated with pericranial tenderness or 2.3.1 Chronic tension-type headache associated with pericranial tenderness.
Diagnostic criteria:

A. Pain, referred from a source in the neck and perceived in one or more regions of the head and/or face, fulfilling criteria C and D

  1. Clinical, laboratory and/or imaging evidence of a disorder or lesion within the cervical spine or soft tissues of the neck known to be, or generally accepted as, a valid cause of headache1

  2. Evidence that the pain can be attributed to the neck disorder or lesion based on at least one of the following:

1. demonstration of clinical signs that implicate a source of pain in the neck2

2. abolition of headache following diagnostic blockade of a cervical structure or its nerve supply using placebo- or other adequate controls3

D. Pain resolves within 3 months after successful treatment of the causative disorder or lesion

Notes:

1. Tumours, fractures, infections and rheumatoid arthritis of the upper cervical spine have not been validated formally as causes of headache, but are nevertheless accepted as valid causes when demonstrated to be so in individual cases. Cervical spondylosis and osteochondritis are NOT accepted as valid causes fulfilling criterion B. When myofascial tender spots are the cause, the headache should be coded under 2. Tension-type headache.

2. Clinical signs acceptable for criterion C1 must have demonstrated reliability and validity. The future task is the identification of such reliable and valid operational tests. Clinical features such as neck pain, focal neck tenderness, history of neck trauma, mechanical exacerbation of pain, unilaterality, coexisting shoulder pain, reduced range of motion in the neck, nuchal onset, nausea, vomiting, photophobia etc are not unique to cervicogenic headache. These may be features of cervicogenic headache, but they do not define the relationship between the disorder and the source of the headache.

3. Abolition of headache means complete relief of headache, indicated by a score of zero on a visual analogue scale (VAS). Nevertheless, acceptable as fulfilling criterion C2 is 90% reduction in pain to a level of <5 on a 100-point VAS.

11.2.2 Headache attributed to retropharyngeal tendonitis

Diagnostic criteria:

A. Unilateral or bilateral non-pulsating pain in the back of the neck, radiating to the back of the head or to the whole head and fulfilling criteria C and D

B. Swollen prevertebral soft tissues, in adults measuring >7 mm at the level between C1 and C4 (special x-ray technique may be required)

C. Pain is aggravated severely by bending the head backwards

D. Pain is alleviated within 2 weeks of treatment with non-steroidal anti-inflammatory drugs in their recommended doses



Comments:

Body temperature and erythrocyte sedimentation rate (ESR) are usually elevated. Although retroflexion of the neck most consistently aggravates pain, this also usually happens with rotation and swallowing. The transverse processes of the upper three vertebrae are usually tender to palpation.

In several cases amorphous calcific material has been aspirated from the swollen prevertebral tissues. Thin calcification in prevertebral tissues is best seen on CT.

Upper carotid dissection should be ruled out.

11.2.3 Headache attributed to craniocervical dystonia

Diagnostic criteria:

A. Sensation of cramp, tension or pain in the neck, radiating to the back of the head or to the whole head and fulfilling criteria C and D

B. Abnormal movements or defective posture of neck or head due to muscular hyperactivity

C. Evidence that pain is attributed to muscular hyperactivity based on at least one of the following:

1. demonstration of clinical signs that implicate a source of pain in the hyperactive muscle (eg, pain is precipitated or exacerbated by muscle contraction, movements, sustained posture or external pressure)

2. simultaneous onset of pain and muscular hyperactivity

D. Pain resolves within 3 months after successful treatment of the causative disorder



Comment:

Focal dystonias of the head and neck accompanied by pain are pharyngeal dystonia, spasmodic torticollis, mandibular dystonia, lingual dystonia and a combination of the cranial and cervical dystonias (segmental craniocervical dystonia). Pain is caused by local contractions and secondary changes.

11.3 Headache attributed to disorder of eyes

11.3.1 Headache attributed to acute glaucoma

Diagnostic criteria:

A. Pain in the eye and behind or above it, fulfilling criteria C and D

B. Raised intraocular pressure, with at least one of the following:

1. conjunctival injection

2. clouding of cornea

3. visual disturbances

C. Pain develops simultaneously with glaucoma

D. Pain resolves within 72 hours of effective treatment of glaucoma

11.3.2 Headache attributed to refractive errors

Diagnostic criteria:

A. Recurrent mild headache, frontal and in the eyes themselves, fulfilling criteria C and D

B. Uncorrected or miscorrected refractive error (eg, hyperopia, astigmatism, presbyopia, wearing of incorrect glasses)

C. Headache and eye pain first develop in close temporal relation to the refractive error, are absent on awakening and aggravated by prolonged visual tasks at the distance or angle where vision is impaired

D. Headache and eye pain resolve within 7 days, and do not recur, after full correction of the refractive error



11.3.3 Headache attributed to heterophoria or heterotropia (latent or manifest squint)

Diagnostic criteria:

A. Recurrent non-pulsatile mild-to-moderate frontal headache fulfilling criteria C and D

B. Heterophoria or heterotropia has been demonstrated, with at least one of the following:

1. intermittent blurred vision or diplopia

2. difficulty in adjusting focus from near to distant objects or vice versa

C. At least one of the following:

1. headache develops or worsens during a visual task, especially one that is tiring

2. headache is relieved or improved on closing one eye

D. Headache resolves within 7 days, and does not recur, after appropriate correction of vision



11.3.4 Headache attributed to ocular inflammatory disorder

Diagnostic criteria:

A. Pain in the eye and behind or around it, fulfilling criteria C and D

B. Ocular inflammation diagnosed by appropriate investigations

C. Headache develops during inflammation

D. Headache resolves within 7 days after relief of the inflammatory disorder



Comment:

Ocular inflammation takes many forms, and may be categorised variously by anatomical site (ie, iritis, cyclitis, choroiditis), by course (acute, subacute, chronic), by presumed cause (infectious agents that are endogenous or exogenous, lens-related, traumatic), or by type of inflammation (granulomatous, non-granulomatous).

11.4 Headache attributed to disorder of ears

Coded elsewhere:

Headache attributed to acoustic neuroma is coded as 7.4.2 Headache attributed directly to neoplasm. Headache attributed to a lesion, not of the ear, giving rise to referred otalgia is coded according to the site and/or nature of the lesion.
Diagnostic criteria:

A. Headache accompanied by otalgia and fulfilling criteria C and D

B. Structural lesion of the ear diagnosed by appropriate investigations

C. Headache and otalgia develop in close temporal relation to the structural lesion

D. Headache and otalgia resolve simultaneously with remission or successful treatment of the structural lesion



Comment:

There is no evidence that any pathology of the ear can cause headache without concomitant otalgia. Structural lesions of the pinna, external auditory canal, tympanic membrane or middle ear may give rise to primary otalgia associated with headache.

However, only about 50% of all cases of earache are due to structural lesions of the external or middle ear. Disorders outside this region may lead to referred otalgia as a result of radiation of pain into the ear region. Sensory fibres of the fifth, seventh, ninth and tenth cranial nerves project into the auricle, external auditory canal, tympanic membrane and middle ear. For this reason referred pain from remote structural lesions in any of the anatomical regions to which these nerves project can be felt as referred otalgia. Since these are not disorders of the ear they are coded elsewhere according to the site and/or nature of the lesion(s).


11.5 Headache attributed to rhinosinusitis

Coded elsewhere:

“Sinus headaches”
Diagnostic criteria:

A. Frontal headache accompanied by pain in one or more regions of the face, ears or teeth and fulfilling criteria C and D

B. Clinical, nasal endoscopic, CT and/or MRI imaging and/or laboratory evidence of acute or acute-on-chronic rhinosinusitis1;2

C. Headache and facial pain develop simultaneously with onset or acute exacerbation of rhinosinusitis

D. Headache and/or facial pain resolve within 7 days after remission or successful treatment of acute or acute-on-chronic rhinosinusitis



Notes:

1. Clinical evidence may include purulence in the nasal cavity, nasal obstruction, hyposmia/anosmia and/or fever.

2. Chronic sinusitis is not validated as a cause of headache or facial pain unless relapsing into an acute stage.


Comments:

Other conditions that are often considered to induce headache are not sufficiently validated as causes of headache. These include deviation of nasal septum, hypertrophy of turbinates, atrophy of sinus membranes and mucosal contact. The last, however, is defined in the appendix under A11.5.1 Mucosal contact point headache.

Migraine and tension-type headache are often confused with 11.5 Headache attributed to rhinosinusitis because of similarity in location of the headache. A group of patients can be identified who have all of the features of 1.1 Migraine without aura and, additionally, concomitant clinical features such as facial pain, nasal congestion and headache triggered by weather changes. None of these patients have purulent nasal discharge or other features diagnostic of acute rhinosinusitis. Therefore it is necessary to differentiate 11.5 Headache attributed to rhinosinusitis from so-called “sinus headaches”, a commonly-made but non-specific diagnosis. Most such cases fulfil the criteria for 1.1 Migraine without aura, with headache either accompanied by prominent autonomic symptoms in the nose or triggered by nasal changes.


11.6 Headache attributed to disorder of teeth, jaws or related structures

Diagnostic criteria:

A. Headache accompanied by pain in the teeth and/or jaw(s) and fulfilling criteria C and D

B. Evidence of disorder of teeth, jaws or related structures

C. Headache and pain in teeth and/or jaw(s) develop in close temporal relation to the disorder

D. Headache and pain in teeth and/or jaw(s) resolve within 3 months after successful treatment of the disorder



Comment:

Disorders of the teeth usually cause toothache and/or facial pain, and those causing headache are rare. Pain from the teeth may be referred, however, and cause diffuse headache. The most common cause of headache is periodontitis or pericoronitis as the result of infection or traumatic irritation around a partially-erupted lower wisdom tooth.

11.7 Headache or facial pain attributed to temporomandibular joint (TMJ) disorder

Diagnostic criteria:

A. Recurrent pain in one or more regions of the head and/or face fulfilling criteria C and D

B. X-ray, MRI and/or bone scintigraphy demonstrate TMJ disorder

C. Evidence that pain can be attributed to the TMJ disorder, based on at least one of the following:

1. pain is precipitated by jaw movements and/or chewing of hard or tough food

2. reduced range of or irregular jaw opening

3. noise from one or both TMJs during jaw movements

4. tenderness of the joint capsule(s) of one or both TMJs

D. Headache resolves within 3 months, and does not recur, after successful treatment of the TMJ disorder



Comment:

Pain from the temporomandibular joint or related tissues is common. It is due to the so-called temporomandibular joint disorders (eg, disk displacements, osteoarthritis, joint hypermobility) or rheumatoid arthritis, and may be associated with myofascial pain and headache.

11.8 Headache attributed to other disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structures

Diagnostic criteria:

A. Headache, with or without pain in one or more regions of the face, fulfilling criteria C and D

B. Evidence of disorder, other than those described above, of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure

C. Headache develops in close temporal relation to, or other evidence exists of a causal relationship with, the disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure

D. Headache resolves within 3 months after successful treatment of the disorder



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11.1 Headache attributed to disorder of cranial bone

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11.2 Headache attributed to disorder of neck

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11.2.2 Retropharyngeal tendonitis

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11.2.3. Craniocervical dystonia

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11.3 Headache attributed to disorder of eyes

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11.4 Headache attributed to disorder of ears; 11.5 Headache attributed to rhinosinusitis

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11.6 Headache attributed to disorder of teeth, jaws or related structures

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11.7 Temporomandibular joint disorder

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