TheimpactofOIonjawfunctionandoralhealth-relatedqualityoflife

HeadofcenterHansGjørupconsultantorthodontist,DDS,PhD

CenterforOralHealthinRareDiseasesDept.ofMaxillofacialSurgery,AarhusUniversityHospital,Denmark

CenterforOralHealthinRareDiseasesCollaborationbetween

Dept.ofMaxillofacialSurgery,AarhusUniversityHospitalDepartmentofDentistryandOralHealth,AarhusUniversity

Centralization of specialized oral health care service for people with rare inherited diseases

DentalsymptomsinadultswithOI

•  Dentinogenesisimperfecta(DI)– Discolorationofteeth–  Pulpalobliteration– Deviationsindentalmorphology

•  DIin25%ofpatientswithOI–  Type3-4: 94%withDI–  Type1: 2%withDI

•  Enamelandcrownfractures

•  Thuesenetal2018

CraniofacialmorphologyandocclusioninpatientswithOI

•  Relativemandibularprognatismandmandibularoverjet–  Stenviketal1985– WaltimoSirenetal2005

•  MostseverecraniofacialabnormalitiesinOItype3and4

•  NearnormaljawmorphologyinOItype1–  Jensen&Lund1997

•  Mandibularoverjet&posteriorcrosbiteprevalentiOItype3and4–  Bendixenetal.2018

Nessecitiesfortheabilitytochew,eat,speak,andsmile:Structuresoftheorofacialcomplex

Jaw&teeth

TandlægeforeningenDK ThiemeAtlasofAnatomy

MusclesTMjoint&discus

ThepresenceofdentalabnormalitieslikeDIandskeletalabnormalitieslikeOI,

aswellasthepresenceofmalocclusionmighthaveimpacton

jawfunctionandoralhealth-relatedqualityoflife

Badjawfunction=TemporomandibulardisorderTMD

•  TMDareaheterogeneousgroupofpathologiesaffectingthetemporomandibularjoint(TMJ),thejawmuscles,orboth.

•  McNeillC.Managementoftemporomandibulardisorders:conceptsandcontroversies.JProsthetDent1997;77:510-22.

•  Theyarecharacterizedbyaclassicallydescribedtriadofclinicalsigns:– muscleand/orTMJpain;– TMJsounds;– andrestriction,deviation,ordeflectionofthemouthopeningpath

•  LaskinDM.Etiologyofthepain-dysfunctionsyndrome.JAmDentAssoc1969;79:147-53.

Aimsofstudyonjawfunction

•  toassesstheoccurrenceoftemporomandibulardisorders(TMD)–  inadultswithosteogenesisimperfectaand

•  tocomparetheoccurrenceofTMD– accordingtoseverityofOI

•  ResearchDiagnosticCriteriaforassessentoftemporomandibulardisordersRDC/TMD

–  DworkinSF,LerescheL.Researchdiagnosticcriteriafortemporomandibulardisorders:review,criteria,examinationsandspecifications,critique.JCraniomandibDisordFacialOralPain1992;6:301-55

ThiemeAtlasofAnatomy

Studypopulation:75adultswithgeneticallyverifiedOI

OImild(type1) Females MalesNumbers 31 25Age(yr)mean 49(14.5) 49(17.4)Age(yr)range 23-72 22-80

OIsevere(type3/4) Females MalesNumbers 9 10Age(yr)mean 49(12.3) 44(10.3)Age(yr)range 37-72 28-63

Diagnosesonmyofacialpainin56withOII–19withOIIII/IV–100general–100TMD

ThiemeAtlasofAnatomy

5 1 1045

51 18 9055

0%

20%

40%

60%

80%

100%

OII OIIII/IV

General TMD

RDC/TMDI-myofacialpain

Myofacialpain(1) Nopain

ns

ChronicPainGrade(CPG0-4)in56withOII-19withOIIII/IV

•  CPG0:noTMDpaininthepriorsixmonths

•  CPG1:lowdisabilityandlowpainintensity

•  CPG2:lowdisabilityandhighpainintensity

•  CPG3:highdisabilityandmoderatelylimiting

•  CPG4:highdisabilityandseverelylimiting

210

1

4418

0%

20%

40%

60%

80%

100%

OII OIIII/IV

ChronicPainGrade

CPGII CPGI CPG0

ns

ConclusionsonOIandjawfunctionBendixenetal2018

•  IrrespectiveofOIseverity,veryfewpatientshadTMDdiagnosesonmyofacialpain,discdisplacement,orjointdisorder

•  ThebodilypaincomplaintsfrequentlyoccurringinOIwasnotreflectedintheorofacialarea

Nessecitiesfortheabilitytochew,eat,speak,andsmile:Structuresoftheorofacialcomplex

Jaw&teeth

TandlægeforeningenDK ThiemeAtlasofAnatomy

MusclesTMjoint&disc

ThepresenceofdentalabnormalitieslikeDIandskeletalabnormalitieslikeOI,

aswellasthepresenceofmalocclusionmighthaveimpacton

jawfunctionandoralhealth-relatedqualityoflife

Aimofstudyonoralhealth-relatedqualityoflifeinpatientswithrareskeletaldisorders

•  Toassesstheimpactuponoralhealth-relatedqualityoflifeintworarediseases,characterizedbythepresenceofbothskeletalanddentalsymptoms(XLHandOI)

•  Tocomparetheimpactonoralhealthrelatedqualityoflifeaccordingtothedisease,XLHorOI

Twocross-sectionalstudiesonandOIrespectiveXLHinDenmark

X-linkedHypophosphatemia•  PHEXmutation,X-linked•  Renaldepletionofphosphateand

hypophosphatemia•  Hypomineralizationandsofteningof

bones

•  Shortstatureandbowedlegs•  Enlargementofgrowthplates•  Costachondralthickening

–  “rachiticrosary”

•  Pulpitisandpulpnecrosis–  inteethwithoutdecay

•  Dentalabscesses

•  RecruitmentbyMDPhDSigneSBeck-Nielsen

OsteogenesisImperfecta•  Interdiciplinaryassessment

–  Clinical,radiological,histological,genetical,QoL,dexa,otomicroscopical,dentaletc

RecruitmentbyMDPhDJannieDHald

StudypopulationsXLHandOI

XLH Females MalesNumbers 24 11Age(yr)mean(SD) 41(14.6) 42.5(19.6)Age(yr)range 21-75 19-73

OI Females MalesNumbers 40 35Age(yr)mean(SD) 49(13.9) 48(15.7)Age(yr)range 23-73 22-80

Method:quantifyingtheimpactoforalhealthonqualityoflife

Tool:OralHealthImpactProfileOHIP-DSladeandSpencer1994.DevelopmentandevaluationoftheOralHealthImpact

Profile.CommunityDentHealth11:3-11

Standardizedquestionnaire•  49questions•  Frequencyofsymptomsorexperiences

•  Score0–40=never4=daily/always

7domaines

•  Functionallimitations•  Physicalpain•  Psychologicaldiscomfort•  Physicaldisability•  Psychologicaldisability•  Socialdisability•  Handicap

OHIPprofilesbymeans(t-test)inOIsevere,OImild,XLHandReference

P<0.05

Comparison(Wilcoxonrank-sumtest)OIpatients(N=75)andXLHpatients(N=35)

Comparison(Wilcoxonrank-sumtest)MildOI(N=56)andsevereOI(N=19)

Conclusionsonoralhealthrelatedqualityoflife•  InbothXLHandOIgroups,thedomainescoresinalldomaines-exceptone(socialdisability)-deviatefromreferencegroup

•  TheimpactofXLHexceedstheimpactofOIinnearlyalldomaines–  Functionallimitations–  Pain–  Psychologicaldiscomfort–  Psychologicaldisability– Handicap

•  AccordingtoOHIP,theimpactofsevereOIhardlyexceedstheimpactofmildOI

ThankyouforyourattentionThankyouforsupportfrom

•  participatingpatients•  TheDanishSocietyforOsteogenesis

imperfecta•  CareforBrittleBones(C4BB)

Thankyouforcollaborationwith•  coworkersat

–  OdenseUniversityHospital–  AarhusUniversityHospital

•  Deptofmedicinandendocrinology•  CenterforRareDiseases

–  DepartmentofDentistryandOralHealth,AarhusUniversity

•  thestaffat–  Dept.MaxillofacialSurgery,Aarhus

UniversityHospital–  DepartmentofDentistryandOralHealth,

AarhusUniversity

HansGjørup:hangjo@rm.dk