TreatmentTreatment ParadigmParadigm ofof FacialFacial Pain:Pain: AA multimulti--disciplinarydisciplinary approachapproach

AndrewAndrew Kokkino,Kokkino, MDMD MedicalMedical DirectorDirector OregonOregon NeurosciencesNeurosciences InstituteInstitute GoalsGoals

¾¾ PresentPresent thethe commoncommon causescauses ofof facialfacial painpain ¾¾ DifferentiateDifferentiate thethe majormajor facialfacial painpain syndromessyndromes ¾¾ PresentPresent resultsresults ofof treatmenttreatment forfor facialfacial painpain syndromessyndromes CausesCauses ofof facialfacial painpain

¾ Def: pain in the facial region including orofacial and craniofacial pain. May be associated with local inflammatory, neoplastic, or neuralgia syndromes. Conditions featuring recurrent or persistent facial pain as the primary manifestation disease are referred to as FACIAL PAIN SYNDROMES. ¾ 138 possible causes of facial pain CausesCauses ofof facialfacial painpain

¾ Dental ¾ Neck conditions z Ache, abscess, oral ¾ TMJ disease, ¾ Sinus disease ¾ Ear (otalgia): infection ¾ Cancer ¾ Eye z Chordoma, meningioma, z Infection, inflammation, neuromas, virus, glaucoma, FB, esthesioneuroblastomas, shingles mets, dermoids ¾ Cerebral vascular dz ¾ Referred pain z z Aneurysms of basilar, z Nerve compression, angina posterior comm, SCA, cavernous sinus ¾ Psychogenic thrombosis z Depression, atypical features NeurologicNeurologic causescauses ofof facialfacial painpain

¾¾ TrigeminalTrigeminal neuralgianeuralgia ¾¾ SphenopalatineSphenopalatine neuralgianeuralgia ¾¾ PostPost--herpeticherpetic neuralgianeuralgia ¾¾ MigrainousMigrainous neuralgianeuralgia ¾¾ GlossopharyngealGlossopharyngeal neuralgianeuralgia ¾¾ AtypicalAtypical facialfacial painpain MostMost commoncommon causescauses ofof facialfacial painpain

• Temporomandibular joint and muscle disorder (TMJD) • Causes recurrent or chronic pain and dysfunction in the jaw joint and its associated muscles and supporting tissues • Second most commonly occurring musculoskeletal condition resulting in pain and disability (after chronic low back pain) • Affects approximately 5 to 12% of the population, with an • annual cost estimated at $4 billion • About half to two-thirds of those with TMJ disorders will seek treatment. Among these, approximately 15% will develop chronic TMJD PrevalencePrevalence ofof TMJTMJ

1818--2525 20.8%20.8% ¾ Cross-sectional study (mail questionnaire, response rate 71%) 2626--3535 29.8%29.8% ¾ (n=8,888) ¾ McFarlane TV2 ¾ 2002 3636--4545 30.0%30.0% ¾ Patients in a general medical practice, England ¾ One month period 46+46+ 27.6%27.6% prevalence of oro-facial pain PrevalencePrevalence ofof TMJTMJ symptomssymptoms

Males/Females ¾ PainPain fromfrom TMJTMJ 6.7%6.7% // 12.4%12.4% ¾ JointJoint soundssounds 12.0%12.0% // 16.5%16.5% ¾ DifficultyDifficulty openingopening jawjaw 8.2%8.2% // 11.2%11.2% ¾ BruxismBruxism 15.5%15.5% // 20.2%20.2% ¾ SensitiveSensitive teethteeth 30.0%30.0% // 38.9%38.9% ¾ BurningBurning mouthmouth 4.1%4.1% // 5.3%5.3% ¾ ChewingChewing difficultydifficulty 27.2%27.2% // 24.8%24.8% MajorMajor ClassificationClassification ofof TrigeminalTrigeminal PainPain -- BurchielBurchiel

Idiopathic TN 1 Sharp, shooting, shock-like, episodic lasting seconds with pain-free intervals

Idiopathic TN 2 Aching, throbbing, or burning more than 50% of the time. Constant background pain. Symptomatic TN AssocAssociationiation with multiple sclerossclerosisis (MS). Demyelination of the nerve or descending tracts. Episodic or constant pain

Postherpetic TN Facial herpes zoster. Normally V1. Marked by development of allodynia superimposed on burning dysesthisias. Trophic changes possible.

TNP: Neuropathic Pain from unintentional injury, cancer, stroke. Unremitting throbbing or burning pain in affected area.

TrigeminaTrigeminall Intentional injury to TN causing burning, crawling, itching, or tearing. deafferentation pain Atypical facial pain Facial pain in the context of a somatoform pain disorder. Often bilateral, outside of TN distribution with multiple pain complaints Anesthesia dolorosa Excruciating pain in an insensate facial region TrigeminalTrigeminal NeuralgiaNeuralgia

¾ EpidemiologyEpidemiology

z 4.7 /1,000,000 men

z 7.2 /1,000,000 women

z Peak incidence fifth to seventh decade

z Familial cases are rare TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ ClinicalClinical characteristicscharacteristics

z ““ElectricElectric ShockShock””

z ContinuousContinuous interictalinterictal painpain (worse(worse prognosis)prognosis)

z ParoxysmalParoxysmal disorderdisorder TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ TriggerTrigger zoneszones

z AsAs smallsmall asas 11--22 mmmm

z PainPain startsstarts inin triggertrigger zonezone andand spreadsspreads

z PainPain intensityintensity independentindependent ofof triggertrigger zonezone sizesize TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ TriggersTriggers--trivialtrivial stimulistimuli

z ColdCold airair

z TalkingTalking

z ChewingChewing

z ToothTooth brushingbrushing

z FacialFacial movementmovement TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ DiagnosisDiagnosis andand testingtesting

z ClinicalClinical presentationpresentation • Normal exam except for trigger zones • 15% have sensory loss that patient does not recognize TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ DiagnosisDiagnosis andand testingtesting

z InternationalInternational HeadacheHeadache SocietySociety criteriacriteria • Paroxysmal attacks of facial pain lasting seconds to less than 2 min • 4 of the following 5 characteristics

z Distribution along one or more divdivisionsisions of 5th nerve

z Sudden, intense, sharp, stabbing pain quality

z Severe pain

z Evidence of trigger zones

z No symptoms between attacks TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ DiagnosisDiagnosis andand testingtesting

z SecondarySecondary neuralgianeuralgia • MS • Neoplasm (epidermoid, acoustic neuroma, meningioma, trigeminal neuroma)

z MRI,MRI, MRAMRA TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ PathogenesisPathogenesis

z UnknownUnknown

z CentrallyCentrally mediatedmediated mechanismmechanism

z ChronicChronic focalfocal demyelinationdemyelination • Increased afferent firing • Impaired inhibitory mechanisms in trigeminal brainstem complex

TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment ¾ MedicationsMedications ¾ PercutaneousPercutaneous • Percutaneous Radiofrequency Rhizotomy • Glycerol injection • Balloon compression ¾ SurgicalSurgical • MVD • Rhizotomy • Peripheral denervation ¾ RadiosurgeryRadiosurgery TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment--MedicalMedical • Dilantin-1940

• 300-500 mg d • Tegretol-1962 • 400-800 mg/ d. Begin 200 mg/ day • Autoinduction of metabolism • Taper after pain free 4-6 wks • Side effects • 94% pain relief within 48 hrs • Baclofen-1980

• 40-80 mg d

• GABA analog

• Combination drug

• Supression of spinal trigeminal neurons TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment--MedicalMedical • Clonazapam-1976

• 1.5-8 mg/d

• Drowsiness, fatigue, dizziness • Valproic acid

• Antiepileptic

• 500-1500 mg d • Lamotrigine

• 150-400 mg d

• Na channel modulator

• Combination drug (dilantin, tegretol)

• Stevens-Johnson syndrome TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment--MedicalMedical

• Neurontin

• 900-2400 mg d

• Anecdotally effective

• Few side effects • Oxcarbazepine

• 600-1200 mg d

• Derivative of carbamazepine TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment--MedicalMedical • Primary: Carbamazepine, oxcarbazepine • Secondary: Lamotrigine • Tertiary: Gabapentin , phenytoin TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment-- MedicalMedical ¾¾ GeneralGeneral guidelinesguidelines

z DoDo notnot overtreatovertreat

z SmallestSmallest possiblepossible painpain relievingrelieving dosedose

z ToleranceTolerance toto medicationmedication withwith timetime

z AimAim forfor monotherapymonotherapy TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment Invasiveness ¾ MedicationsMedications Most ¾ SurgicalSurgical • MVD • Rhizotomy • Peripheral denervation ¾ PercutaneousPercutaneous • Percutaneous Radiofrequency Rhizotomy • Glycerol injection • Balloon compression Least ¾ RadiosurgeryRadiosurgery TrigeminalTrigeminal NeuralgiaNeuralgia InvasiveInvasive treatmenttreatment ¾ FailedFailed medicalmedical treatmenttreatment ¾ 50%50% ofof patientspatients requirerequire anan invasiveinvasive procedureprocedure ¾ TaylorTaylor youryour choicechoice ofof invasiveinvasive treatmenttreatment toto thethe patientpatient ¾ StartStart withwith thethe painpain distribution,distribution, ageage andand previousprevious proceduresprocedures ¾ TolerateTolerate patientpatient preferencespreferences withinwithin reasonreason ¾ MaintainMaintain fullfull complimentcompliment ofof proceduresprocedures PercutaneousPercutaneous ProceduresProcedures

¾¾ PercutaneousPercutaneous RadiofrequencyRadiofrequency RhizotomyRhizotomy (PRR)(PRR)

z PartialPartial destructiondestruction ofof gasseriongasserion ganglionganglion withwith heatheat

z GreaterGreater thanthan 90%90% reliefrelief withwith initialinitial procedureprocedure PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z CannulationCannulation ofof foramenforamen ovaleovale PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z CannulationCannulation ofof thethe foramenforamen ovaleovale PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z CannulationCannulation ofof thethe foramenforamen ovaleovale

z AimAim forfor medialmedial sideside PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z CannulationCannulation ofof thethe foramenforamen ovaleovale

z AimAim forfor medialmedial sideside PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z CannulationCannulation ofof thethe foramenforamen ovaleovale

z AimAim forfor medialmedial sideside PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique ¾¾ AvoidAvoid

z OralOral cavitycavity

z CarotidCarotid canalcanal

z ForamenForamen lacerumlacerum

z CavernousCavernous carotidcarotid PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z CannulationCannulation PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z StimulationStimulation • Pain reproduction • Curved needle PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z LesioningLesioning • Hypoalgesia rather than analgesia PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR techniquetechnique

z LesioningLesioning

PROPOSED PARADIGM USED TO CONVERT A STIMULUS INTENSITY TO AN INITIAL LESION Stimulation Probe Temperature Duration of Lesion Intensity (mV) (0C) (sec.) <0.3 60 60 0.3-0.4 65 60 0.4-0.8 70 60 0.8-1.0 75 60 >1.0 Abort and reposition electrode PercutaneousPercutaneous ProceduresProcedures

¾¾ PRRPRR

z SideSide effectseffects • Sensory loss • Moderate dysesthysia 5-25% • Severe dysesthysia 2-10% • Corneal sensory loss 20% • Keratitis <1% • Anesthesia dolorosa 1-5% • Weak mastication 53% PercutaneousPercutaneous ProceduresProcedures

¾ PRRPRR

z Outcome • Taha, Tew and Buncher. “A prospective 15-year follow up of 154 consecutive patients with trigeminal neuralgia treated by percutaneous stereotactic radiofrequency thermal rhizotomy.” J of Neurosurgery, 1995 • 14 yr recurrence rate - 25% • Rate of recurrence inversely proportional to density of lesion

z Anticipated ½ life of procedure about 3-5 years PercutaneousPercutaneous ProceduresProcedures

¾ GlycerolGlycerol rhizotomyrhizotomy

z Mild denervating agent

z 90% initially effective

z 28% recurrence within one year, 50% in 2 years

z Sensory loss in 26-71%

z Many initial failures

z Anticipated ½ life 2-3 years PercutaneousPercutaneous ProceduresProcedures

¾ BalloonBalloon CompressionCompression

z 80-90% initially effective

z 28% recurrence within 6 months

z Technical problems • General anesthetic • Large trochar • Non-selective with unknown degree of sensory loss • Bradycardia and hypotension

z Anticipated ½ life 2-3 years TrigeminalTrigeminal NeuralgiaNeuralgia TreatmentTreatment

¾ SurgicalSurgical • MVD • Rhizotomy • Peripheral denervation TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVD:MVD: MicrovascularMicrovascular DecompressionDecompression

z TreatmentTreatment ofof choicechoice forfor selectselect populationpopulation

z FailedFailed medicalmedical treatmenttreatment

z ClassicalClassical tictic symptomssymptoms respondrespond bestbest

z GeneralGeneral anesthesiaanesthesia andand ICUICU staystay

z MorbidityMorbidity ofof surgerysurgery TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z PositionPosition • Lateral oblique • Lumbar puncture TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z IncisionIncision • 2 fingers inside mastoid notch • Extend above superior nuchal line TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z IncisionIncision • Posterior emissary vein • Identify asterion TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z CraniectomyCraniectomy • Just below asterion • Extend superiorly and laterally to the transverse and sigmoid sinuses • 2.5 cm in diameter • Wax mastoid air cells TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z DuralDural openingopening

z RetractRetract cerebellumcerebellum mediallymedially andand inferiorlyinferiorly TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z DuralDural openingopening

z RetractRetract cerebellumcerebellum mediallymedially andand inferiorlyinferiorly TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z OpenOpen arachnoidarachnoid

z IdentifyIdentify petrotentorialpetrotentorial junctionjunction

z TransectTransect petrosalpetrosal veinvein TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD techniquetechnique

z QuickQuick looklook atat 7th7th andand 8th8th nervesnerves

z 5th5th NN entryentry zonezone

z DissectDissect arteriesarteries

z CoagulateCoagulate veinsveins atat entryentry

z IvalonIvalon spongesponge TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD

z ComplicationsComplications • Facial numbness 2% • Cranial nerve deficit 3% • Peri-operative morbidity 10% • Cerebral hemorrhage oror infarctioninfarction 1%1% • Peri-operative mortality .06% TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ MVDMVD longlong termterm resultsresults

z BarkerBarker etet al.al. NEJM,NEJM, 19961996 • 20 yr follow up on 1185 patients

z 80% complete pain relief after procedure

z 7.6% partial relief

z At 10 yrs 70% still had excellent results, 4 % partial relief

z Of patients with incomplete relief • 34% resumed medication • 22% ablative procedure and medication

z Recurrence rates 1-6%, most within 2 yrs TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ SurgicalSurgical treatmenttreatment

z MVDMVD

z RhizotomyRhizotomy • Last resort TrigeminalTrigeminal NeuralgiaNeuralgia

¾¾ SurgicalSurgical treatmenttreatment

z MVDMVD

z RhizotomyRhizotomy

z PeripheralPeripheral denervationdenervation • Interrupt afferents • Medically unfit patient • Immediate relief GammaGamma KnifeKnife RadiosurgeryRadiosurgery forfor TrigeminalTrigeminal NeuralgiaNeuralgia GammaGamma KnifeKnife RadiosurgeryRadiosurgery forfor TrigeminalTrigeminal NeuralgiaNeuralgia GammaGamma KnifeKnife RadiosurgeryRadiosurgery forfor TrigeminalTrigeminal NeuralgiaNeuralgia TrigeminalTrigeminal NeuralgiaNeuralgia

¾ RadiosurgeryRadiosurgery Kondziolka et al, 2001

z Technique: 80 Gy to 100% isodose line to nerve root entry zone

z Profile: n=220. Symptoms present 96 months on average. 61% previous surgery. 36% sensory disturbance. 70 year old median age TrigeminalTrigeminal NeuralgiaNeuralgia

¾ RadiosurgeryRadiosurgery

z Results

z Pain relief 82% (>50% relief)

z 64.9% complete pain relief at 6 months

z 70.3% at one yr

z 55.8% at 5 yrs

z 13% recurrent pain b/w 2-58 months

z Time to response: 2 months median

z Increased parasthesias 8-10%

z Dysesthesias 3.5% Dysesthesias 3.5% Kondziolka et al, 2002 z No motor deficits TrigeminalTrigeminal NeuralgiaNeuralgia

¾ Radiosurgery

z Advantages • Short procedure • Minimally invasive • Low complication rate • Less expensive than MVD

z Disadvantages • Unpredictable latency to relief GlossopharyngealGlossopharyngeal NeuralgiaNeuralgia

¾¾ Etiology:Etiology: vascularvascular compressioncompression ofof 99th andand 1010th cranialcranial nervesnerves ¾¾ Symptoms:Symptoms: deepdeep throatthroat painpain ¾¾ TreatmentTreatment

z MedicalMedical

z Operative:Operative: decompressiondecompression oror transectiontransection GlossopharyngealGlossopharyngeal NeuralgiaNeuralgia

¾¾ ResultsResults

z MicrovascularMicrovascular decompressiondecompression inin thethe managementmanagement ofof glossopharyngealglossopharyngeal neuralgia:neuralgia: analysisanalysis ofof 217217 cases.cases.

z Patel et al: Neurosurgery 50:705-11, 2002. ¾¾ 90%90% immediateimmediate reliefrelief ¾¾ SwallowingSwallowing difficultydifficulty ¾¾ BestBest resultsresults inin patientspatients withwith typicaltypical symptoms,symptoms, throatthroat painpain onlyonly ParadigmParadigm ofof treatmenttreatment

¾¾ Gatekeepers:Gatekeepers: familyfamily medicine,medicine, ER,ER, psychologists,psychologists, psychiatrists,psychiatrists, dentists,dentists, dentaldental specialistsspecialists ¾¾ ReferralReferral services:services: ENT,ENT, oraloral surgeons,surgeons, neurologists,neurologists, neurosurgeonsneurosurgeons ¾¾ CrossCross--talktalk andand communicationcommunication isis keykey ConclusionsConclusions

¾¾ FacialFacial painpain hashas manymany causescauses ¾¾ UnderstandingUnderstanding ofof medicalmedical treatmentstreatments andand surgicalsurgical approachesapproaches areare inin evolutionevolution ¾¾ MorbidityMorbidity ofof thesethese treatments,treatments, especiallyespecially gammagamma knifeknife radiosurgeryradiosurgery,, isis beingbeing reconsideredreconsidered asas itit developsdevelops intointo aa safesafe andand effectiveeffective techniquetechnique forfor thethe treatmenttreatment ofof trigeminaltrigeminal neuralgianeuralgia