Proposed changes to current definitions of ICHD-3 regarding headache and facial pain attributed to the disorder of the nose or paranasal sinuses

Abstract

Background

Clinicians encounter difficulties in differentiating between headache/facial pain of true sinogenic origin, and clinically similar pain related to primary headache disorders, such as migraine. The International Classification of Headache Disorders and International Classification of Orofacial Pain, together with clinical definitions of acute and chronic rhinosinusitis as refined by the European Position Paper on Rhinosinusitis and Nasal Polyps, have produced a unique opportunity to improve the current diagnostic criteria of headache/facial pain attributed to rhinosinusitis.

Methods

An international multidisciplinary panel reviewed clinical evidence regarding the overlap of primary headaches and rhinosinusitis in order to harmonize and clarify diagnostic frameworks.

Results

The proposal integrates validated rhinologic definitions into headache and facial pain classifications. Key suggestions include the removal or adjustment of non-specific criteria (e.g., headache exacerbated by pressure applied over the paranasal sinuses) which also frequently occur in primary headache disorders. To enhance specificity, evidence-based negative predictors — such as the absence of nausea, osmophobia or photophobia and phonophobia — are introduced. Only for chronic rhinosinusitis, it has been proposed to include endoscopic or radiological evidence of inflammation, as necessary to confirm the diagnosis.

Conclusion

Aligning ICHD-4 with contemporary rhinologic guidelines through the use of positive and negative predictors may help improve diagnostic accuracy, ensure appropriate therapy and increase the reliability of trial design.

Keywords

rhinosinusitis orofacial pain sinus headache sinogenic facial pain paranasal sinuses sinusitis

Introduction

The interface between neurology and otorhinolaryngology presents significant diagnostic challenges, especially with regards to pain.^1,2 Thanks to the work of the scientific community, the International Classification of Headache Disorders (ICHD) and International Classification of Orofacial Pain (ICOP) provide definitions which may help overcome these problems.^3,4 However, global organisations have updated their rhinosinusitis classifications since ICHD-3 and ICOP through the European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS 2020) and the International Consensus Statement on Allergy and Rhinology (ICAR-Rhinosinusitis 2021).^5,6 Additionally, the therapeutic landscape has shifted considerably in recent years: the introduction of targeted treatments, such as calcitonin gene-related peptide (receptor) antagonists for migraine and biologics for chronic rhinosinusitis (CRS), demands precise diagnostic differentiation to ensure appropriate study design and treatment allocation. Moreover, recent clinical evidence has emerged regarding the overlap between primary headache disorders and pain of true sinogenic origin.^7–11

A data-driven update to the current classifications of headache and facial pain attributed to rhinosinusitis is justified, as it promises to improve diagnostic specificity. This proposal is submitted by an international panel of authors spanning rhinology, neurology, and primary care. By integrating well-validated rhinologic definitions — such as those from EPOS 2020 and ICAR-RS 2021 — the authors seek to refine the ICHD/ICOP framework to reduce misdiagnosis and prioritize evidence-based care. This proposal is based on current ICHD-3 text with changes suggested only when consensus of all authors was reached. Proposed deletions are stroked out and additions presented in italics. (Table 1).

Table 1.

Summary of proposed changes to key aspects of ICHD-3.

	Current ICHD-3	Proposal for ICHD-4
Rhinosinusitis Definitions	General clinical/imaging evidence of infection or inflammation	Strict alignment with EPOS 2020 and ICAR-RS 2021 definitions
Positive Predictors	Temporal pattern (co-occurrence and co-remission of pain and nasal symptoms) may promote overlap with migraine	Temporal pattern must be supported by lack of negative predictors.
	Headache exacerbation by pressure.	Removal of these criteria, as they are not common in rhinosinusitis and present also in primary headache disorders.
	Ipsilaterality of pain and sinus involvement.
Negative Predictors	Not explicitly required for diagnosis apart from the generic criterion ‘D’.	Mandatory absence of vegetative symptoms to decrease most prevalent misdiagnosis of migraine.

11.5 Headache/facial pain attributed to disorder of the nose or paranasal sinuses

The term ‘sinus headache/facial pain’ ~~is outmoded because~~ has been applied both to primary headache disorders and to headache ~~supposedly~~^I attributed to various conditions involving nasal or sinus structures. This term should be used only for cases with clear causality confirmed by fulfilling definitions described in this chapter.

Description:

Headache/facial pain caused by a disorder of the nose and/or paranasal sinuses and associated with other symptoms and/or clinical signs of the disorder.

Rationale for the change:

It has been suggested that 80–90% of self-diagnosed chronic ‘sinus headache’ cases are caused by migraine.^2,12 However, sinogenic pain (i.e., true ‘sinus headache/facial pain’) may accompany sinonasal disorders. In this context the term ‘sinus headache’, that still remains in use both by patients and healthcare providers, could be retained if it strictly reflects evidence-based definitions.

11.5.1 Headache/facial pain attributed to acute^II rhinosinusitis (ARS)

Description:

Headache/facial pain caused by acute rhinosinusitis and associated with other symptoms and/or clinical signs of this disorder. ARS is divided into:

− viral - duration of symptoms <10 days

− post-viral - increase in symptoms >5 days or persistent symptoms >10 days with <12 weeks duration

− bacterial ARS - at least three symptoms/signs from: discoloured discharge, severe local pain, fever > 38°C; raised CRP/ESR; exacerbation of symptoms after initial improvement

Diagnostic criteria:

Any new headache/facial pain fulfilling criterion C

~~Clinical, nasal endoscopic and/or imaging evidence of acute rhinosinusitis.~~

Evidence of acute rhinosinusitis is demonstrated by sudden onset of two or more of the following symptoms lasting from ≥ 5 days to ≤ 12 weeks, one of which should be condition 1. or 2.:

nasal blockage or obstruction or congestion

discoloured nasal discharge (anterior or posterior nasal drip)

facial pain or pressure

reduction or loss of smell in adults OR cough in children.^III

(C)

Evidence of causation demonstrated by ~~at least two~~ both of the following:

~~1. headache has developed in temporal relation to the onset of rhinosinusitis~~

~~1. either or both~~ at least one of the following:

headache/facial pain has developed or worsened in temporal relation to the onset or worsening of acute rhinosinusitis

headache/facial pain has significantly improved or resolved in parallel with improvement in or resolution of the acute rhinosinusitis

headache/facial pain has developed after acute viral upper respiratory tract infection (i.e., common cold). ^IV

2. headache/facial pain is NOT accompanied by any of the following symptoms:

nausea and/or vomiting

photophobia and phonophobia

osmophobia^V

~~3. headache is exacerbated by pressure applied over the paranasal sinuses~~ ^VI

~~4. in the case of a unilateral rhinosinusitis, headache is localized and ipsilateral to it.~~ ^VII

(D) Not better accounted for by another ICHD-3/ICOP diagnosis.¹

Notes:

¹ Migraine and 2. Tension-type headache can be mistaken for 11.5.1 Headache attributed to acute rhinosinusitis because of similarity in location of the headache and, in the case of migraine, because of the commonly accompanying nasal autonomic symptoms. The presence or absence of ~~purulent~~ discoloured nasal discharge and/or other features diagnostic of acute rhinosinusitis help to differentiate these conditions.

Comments:

Pain due to pathology in the nasal mucosa or related structures is usually perceived as frontal or facial but may be referred more posteriorly. Simply finding pathological changes on imaging of acute rhinosinusitis, correlating with the patient's pain description, is not enough to secure the diagnosis of 11.5.1 Headache attributed to acute rhinosinusitis. Treatment response to local anaesthesia is compelling evidence, but may also not be pathognomonic.

An episode of 1. Migraine may be triggered or exacerbated by nasal or sinus pathology.

Discharge discolouration occurs in most but not all acute rhinosinusitis definitions. ⁵ However, it should be noted that ARS can manifest with nasal discharge that is not discoloured, especially in the early stages of viral infections. ¹³ In these instances, the diagnosis should be guided by temporal pattern: a single episode of ARS symptoms that last at least 5 days as opposed to recurring episodes of pain lasting up to 3 days in case of primary headache disorders.

Acute rhinosinusitis often develops from acute viral upper respiratory tract infection (i.e., common cold) ¹⁴ and current consensus recognizes the overlap of these two conditions. ^5,6 However, the overlap is not complete and some cases of common cold may not fulfil ARS diagnostic criteria (when nasal symptoms are not present). Alternatively, ARS may develop when viral infection is not active anymore in case of post-viral ARS. It may mean that in some cases symptoms should be classified as both: 11.5.1 Headache attributed to acute rhinosinusitis AND 9.2.2.1 Acute headache attributed to systemic viral infection while in other situations just one diagnosis is accurate.

Acute rhinosinusitis is relatively rarely bacterial. In these cases patients may report severe, sometimes unilateral facial pain ¹⁵ that is accompanied by fever above 38°C, double sickening (exacerbation of symptoms after initial improvement) and raised ESR/CRP.

Rationale for the change:

Definitions of acute rhinosinusitis are similar at their core independently whether it is viral, post-viral, recurrent or bacterial disease.^5,6

Implementing well validated rhinosinusitis definitions^5,6 into ICHD/ICOP would help in improving diagnostic accuracy of non-ENT experts. According to these definitions endoscopic or computed tomography (CT) confirmation is unnecessary and often impossible to acquire in ARS.

ICHD-3 11.5.1 definition allows attribution of headache to ARS if just the first two conditions from original ICHD-3 criteria C are met. However, in primary headaches with nasal cranial autonomic symptoms (CAS) the same conditions may be met creating possible confusion in diagnosis.¹⁶ This is why we propose to modify the criteria so that these symptoms are supported by further clinical features.

Field testing of ICHD-3 criteria for viral and post-viral ARS showed that photophobia, phonophobia, nausea or vomiting are rare in ARS.^17,18 Osmophobia is also rarely present in ARS^17,18 while common in migraine patients.¹⁶ The authors of this proposal understand that ICHD-3 only rarely uses negative criteria in its definitions (e.g., in chapters ‘2. Tension-type headache’ or ‘4.9 hypnic headache’). However, in case of ARS, some negative predictors have been described, and can improve diagnostic accuracy in the absence of stronger positive predictors.

Exacerbation of headache in ARS by pressure applied over the paranasal sinus is not supported by evidence. One study indicates that although facial tenderness may be present in ARS it is not a reliable clinical sign.¹⁹ Tenderness seems to be significant only in relatively rare bacterial ARS where isolated unilateral maxillary sinus hypersensitivity to touch improves diagnostic accuracy.^15,20 Another reason for removing this condition is evidence that people with migraine report exacerbation of headache by pressure probably as part of migraine-associated allodynia which may contribute to diagnosing migraine as rhinosinusitis.¹⁶

Evidence supports unilateral facial pain (not headache) as a symptom of a relatively rare bacterial ARS but not other, more common ARS subtypes.^15,20

In headache disorders accompanied by nasal cranial autonomic symptoms clear rhinorrhoea may falsely indicate ARS. This is why we suggest that only discoloured discharge should be left in the criteria. This should increase criteria specificity for a price of decreased sensitivity. To accommodate for confirmed ARS cases with clear rhinorrhoea ‘Comments’ section now includes relevant information.

11.5.2 Headache/facial pain attributed to chronic or recurring ^IX rhinosinusitis

Description:

Headache/facial pain caused by a chronic infectious or inflammatory disorder of the paranasal sinuses and associated with other symptoms and/or clinical signs of the disorder.

Diagnostic criteria:

Any headache/facial pain fulfilling criterion C

~~Clinical, nasal endoscopic and/or imaging evidence of current or past infection or other inflammatory process within the paranasal sinuses~~

Evidence of chronic rhinosinusitis is demonstrated by two or more of the following symptoms lasting ≥ 12 weeks, one of which should be condition 1. or 2.:

nasal blockage or obstruction or congestion

discoloured nasal discharge (anterior or posterior nasal drip)

facial pain or pressure

reduction or loss of smell in adults OR cough in children

Chronic rhinosinusitis is confirmed by at least one of the following conditions:

at least one endoscopic sign:

oedema/mucosal obstruction¹

nasal polyps

mucopurulent discharge

mucosal changes on CT within the ostiomeatal complex and/or sinuses².^X

Evidence of causation demonstrated by ~~at least two~~ both of the following:

~~1. headache has developed in temporal relation to the onset of chronic rhinosinusitis~~ ^XI

~~2. headache waxes and wanes in parallel with the degree of sinus congestion and other symptoms of the chronic rhinosinusitis~~ ^XI

either or both of the following:

headache/facial pain has developed or worsened in temporal relation to the onset or exacerbation of chronic rhinosinusitis.^XII

headache/facial pain has significantly improved or resolved in parallel with improvement in or resolution of the chronic rhinosinusitis or of its exacerbation.

none of the following:

pulsating headache

headache is exacerbated by physical activity

headache is accompanied by photophobia and phonophobia^XIII

~~headache is exacerbated by pressure applied over the paranasal sinuses~~ ^XIV

~~in the case of a unilateral rhinosinusitis, headache is localized and ipsilateral to it~~ ^XV

Not better accounted for by another ICHD-3 diagnosis.

Notes:

¹ Oedema/mucosal obstruction on endoscopy can be seen during migraine attack (possibly due to trigeminal autonomic reflex activation).²¹

² Minimal thickening, involving only 1 or 2 walls and not the ostial area is unlikely to represent rhinosinusitis.^5,6

Comment:

~~It has been questioned whether chronic sinus pathology can produce persistent headache. Recent studies seem to support such causation.~~ Headache and/or facial pain are considered negative predictors of chronic rhinosinusitis in otorhinolaryngological practice^9,22,23 with most patients reporting non-painful ‘pressure’. ‘Non-type 2’ chronic rhinosinusitis variant (i.e., chronic rhinosinusitis without nasal polyps) seem to be more often associated with pain than ‘type-2’ (i.e., chronic rhinosinusitis with nasal polyps).^24–28 ~~However~~ Due to high prevalence of non-painful chronic rhinosinusitis, pathological changes seen on imaging or endoscopy correlating with the patient's pain description are not on their own enough to secure the diagnosis of 11.5.1 Headache attributed to chronic rhinosinusitis. In this context, primary headache disorders (i.e., migraine) and idiopathic midfacial pain disorders should be excluded whenever headache/facial pain attributed to chronic rhinosinusitis is suspected.^29–31^XVI

There is poor correlation of patient-reported pain location and actual anatomical location of sinusitis. ^32–34 However, in case of localised chronic rhinosinusitis ipsilateral headache/orofacial pain have been described in some cases of sphenoid fungal ball ³⁵ and odontogenic sinusitis. ³⁶

Rationale for the change:

Recurrent rhinosinusitis should be classified under ARS. For chronic rhinosinusitis (CRS) the guidelines recommend using ‘exacerbation’.⁵

Similarly to criterion B for ARS (see above) validated rhinosinusitis definitions are proposed to improve diagnostic accuracy of non-ENT experts.

ICHD-3 11.5.2 definition allows attributing headache to CRS if criteria C1 and C2 are met. However, in chronic primary headaches with nasal CAS the same conditions are present. Effectively, a similar situation to ICHD-3 criteria for ARS could occur: a misdiagnosis based on temporal co-occurrence of headache with autonomic symptoms.¹⁶ This is why we propose to modify the criteria in a manner where these points must be supported by other conditions.

We postulate adding ‘worsening of symptoms’ and ‘association with exacerbations’ as evidence of causality (underlined).

Symptoms listed in point C2 have been validated as negative predictors of CRS.²² The use of negative predictors has been explained above regarding the ARS criteria proposal.

Facial tenderness (but not exacerbation of headache due to pressure applied over the paranasal sinuses) was not confirmed in CRS.¹⁹ However, it may occur in CRS exacerbations although as an unreliable clinical symptom.¹⁹ Moreover, there is evidence that people with migraine report exacerbation of headache in response to pressure and that it is associated with allodynia.¹⁶ Consequently, this point may unnecessarily lead to diagnosing migraine as CRS.

This point has been explained in ‘Comments’ section above.

The role of nasal endoscopy in diagnosis of rhinogenic headache/facial pain is to confirm purulent discharge, oedema or polyps in order to support rhinosinusitis diagnosis. Normal results of nasal endoscopy together with a normal sinus CT scan provides the best available evidence to exclude sinusitis as an active pathology.^5,6,37,38

A11.5.3 Headache attributed to disorder of the nasal mucosa, turbinates or septum

Diagnostic criteria:

Any headache fulfilling criterion C

Clinical, nasal endoscopic and/or imaging evidence of a hypertrophic, inflammatory and or tumorous process within the nasal cavity¹

Evidence of causation demonstrated by at least two of the following:

headache has developed in temporal relation to the onset of the intranasal lesion, or led to its discovery

headache has significantly improved or significantly worsened in parallel with improvement in (with or without treatment) or worsening of the nasal lesion

headache has significantly improved following local anaesthesia of the mucosa in the region of the lesion and does not improve with placebo anaesthesia

headache is ipsilateral to the site of the lesion

Not better accounted for by another ICHD-3 diagnosis.

Note:

~~Examples are concha bullosa and nasal septal spur.~~

Rationale for retaining in appendix:

There are plenty of low-grade studies on mucosal contact-point headache/pain. However, there is no good quality evidence supporting or rejecting its existence. That is why there are currently no grounds to include A11.5.3 Headache attributed to disorder of the nasal mucosa, turbinates or septum (i.e., mucosal contact-point headache or facial pain) in ICHD/ICOP. There is evidence that nasal or sinus tumours can cause pain. For that reason, unilateral nasal/facial pain warrants a nasal endoscopy or imaging. However, concha bullosa or nasal septal spurs as causes of headache/facial pain are not supported by convincing evidence.

Comment on Idiopathic Midfacial Pain (IMFP) in future ICHD/ICOP

While initially described by otorhinolaryngologists due to its mimicking chronic rhinosinusitis presentation, level 2 evidence suggests Idiopathic Midfacial Pain (IMFP) or ‘midfacial segment pain’ is a neurological disorder.^{9,29,30,39,40} Data from available studies indicates a specific clinical profile: bilateral, non-pulsating pain, localized to the nasal, infraorbital, and zygomatic regions and absence of vegetative symptoms.^31,40 Unlike persistent idiopathic facial pain, which is often unilateral and lower-face dominant, IMFP is predominantly bilateral and in the midfacial regions. However, studies with broader inclusion criteria suggest certain overlap of pain location or associated symptoms (i.e., facial pressure) with primary headaches and rhinosinusitis.⁴¹ These symptoms, when encountered in otorhinolaryngological practice, cannot be classified under the current versions of ICHD-3 or ICOP. Meanwhile, misidentification may lead to unnecessary sinonasal interventions that fail to provide lasting pain relief. It is clear that surgical treatments are often patient-driven, due to widespread beliefs amongst both patients and the medical community that midfacial pain is sinogenic in origin, unless proven otherwise. A formal IMFP inclusion into the classification could facilitate interdisciplinary research needed to further elucidate its phenotype, and promote an appropriate diagnosis and treatment.

Limitations

The development of this proposal was based on expert opinion rather than systematic review of literature. The unanimous consensus was reached during open discussion where each expert could propose modifications to current criteria and proposals from other panel participants. Furthermore, we acknowledge limitations regarding the restricted panel's composition despite efforts to include diverse clinical perspectives and support from some of the key scientific societies in this area. Finally, the scientific evidence is growing in the area of headache/facial pain attributed to sinonasal disorders: The value of established tools has been reassessed and new aids are in development.^42,43 This may affect future evidence in regard to this type of headache/facial pain.

Conclusions

ICHD-3 and ICOP criteria of rhinogenic pain are crucial in defining clinical and scientific boundaries of this headache/facial pain subtype. Diagnostic specificity may be improved by aligning them with current definitions of rhinosinusitis. This can be further refined by incorporating evidence-based positive and negative predictors, and removing those clinical symptoms which have proven of little diagnostic value. The above should help to improve diagnosis and treatment and, as a result, the quality of life of patients.

Article highlights

This is a proposal to align future ICHD/ICOP versions with current rhinosinusitis definitions.

The panel recommends adding negative criteria of headache attributed to rhinosinusitis (absence of nausea, photophobia, phonophobia and osmophobia).

Authors suggest removing unreliable criteria: exacerbation by pressure and unilateral symptoms.

The proposal underlines lack of strong evidence for headache/facial pain in anatomical variations of nasal turbinates or septum.

Footnotes

Acknowledgements

None

ORCID iDs

Marcin Straburzyński

Adrian M. Agius

Claire Hopkins

Amber U. Luong

Eugenio De Corso

Arne May

Ethical considerations

Not applicable

Consent to participate

Not applicable

Consent for publication

The authors agree to publish the ICHD-4 viewpoint with Cephalalgia.

Author contributions

Marcin Straburzyński: Conceptualization, Funding acquisition, Methodology, Project administration Writing – original draft, Writing – review & editing

Adrian M. Agius: Conceptualization, Writing – original draft, Writing – review & editing

Claire Hopkins: Writing – review & editing

Amber U. Luong: Writing – review & editing

Eugenio De Corso: Writing – review & editing

Arne May: Writing – review & editing, Supervision

Timothy L. Smith: Writing – review & editing

Casper P. Zwetsloot: Writing – review & editing

Richard R. Orlandi: Writing – review & editing

Wytske Fokkens: Conceptualization. Writing – review & editing, Supervision

Funding

The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: Funded by the Polish Minister of Science under ‘the Regional Initiative of Excellence Program’.

Declaration of conflicting interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Data availability statement

Open Practices: Not applicable

Supplemental Material: Not applicable

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Proposed changes to current definitions of ICHD-3 regarding headache and facial pain attributed to the disorder of the nose or paranasal sinuses

Abstract

Background

Methods

Results

Conclusion

Keywords

Introduction

11.5 Headache/facial pain attributed to disorder of the nose or paranasal sinuses

Description:

Rationale for the change:

11.5.1 Headache/facial pain attributed to acuteII rhinosinusitis (ARS)

Description:

Diagnostic criteria:

Notes:

Comments:

Rationale for the change:

11.5.2 Headache/facial pain attributed to chronic or recurring IX rhinosinusitis

Description:

Diagnostic criteria:

Notes:

Comment:

Rationale for the change:

A11.5.3 Headache attributed to disorder of the nasal mucosa, turbinates or septum

Diagnostic criteria:

Note:

Rationale for retaining in appendix:

Comment on Idiopathic Midfacial Pain (IMFP) in future ICHD/ICOP

Limitations

Conclusions

Article highlights

Footnotes

Acknowledgements

ORCID iDs

Ethical considerations

Consent to participate

Consent for publication

Author contributions

Funding

Declaration of conflicting interests

Data availability statement

References

11.5.1 Headache/facial pain attributed to acute^II rhinosinusitis (ARS)

11.5.2 Headache/facial pain attributed to chronic or recurring ^IX rhinosinusitis