The ABCs of Oral DIAGNOSIS in the Pediatric Patient

12/22/17

1

TheABCsofOralDIAGNOSIS

inthePediatricPatient

Juan F. Yepes DDS, MD, MPH, MS, DrPHAssociate Professor –Indiana University-

Clinical Associate Professor –University at Buffalo-Riley Hospital for Children, Indianapolis, Indiana

Diplomate ABOM, ABDPH, ABPD

[email protected]

The ABCs of Oral Diagnosis in the Pediatric Patient

DisclaimersI do not have any affiliation with any commercial company. My only interest is exclusively academic and for the benefit of PATIENTS, residents, RDH, dental assistants, managers, dentists, dental therapist, and physicians.

All clinical pictures were authored by myself unless otherwise listed on the slide.

Patients and/or parent consent was received for all photos and radiographs.

12/22/17

2

Learning Objectives

1. To review the most common oral lesions in children

2. To learn how to develop a correct differential diagnosis.

3. To understand the rationale behind the current treatments.

Bestmethodtolearnoraldiagnosisinthepediatricpatient?

OralDiagnosis

12/22/17

3

Ulcersintheoralcavityinchildren

arealltheSAME?

Yepes’s approach

AGE !!!Pre-K childKindergarten child?Teenager baby?


arealltheSAME?

Yepes’s approach

MEDICAL HISTORY !!!Chronic GI issuesLesions in other places (skin – genitals)Periodicity (every 90 days?)Other systemic symptomsJoint pain

Not assuming the role of the PCP


arealltheSAME ?

Yepes’s approach

Ulcer history !!!First timeOftenAlmost dailyFor a long time (more than 2 weeks)

12/22/17

4


arealltheSAME?

Yepes’s approach

Clinical appearance !!!Round?Irregular?Location àGum line?, NK mucosa?Keratinized mucosaCluster?


arealltheSAME ?

NO!

RASHerpes virusHerpanginaHand foot mouth diseaseCrohn’s diseaseCyclic neutropeniaSLETrauma

Erythema multiformeMononucleosisBechet's diseaseMAGIC syndromePFAFA syndrome

Behcet’sdisease

• BDisanidiopathiccondition,chronic,relapsing,multi-systemic,characterizedbyrecurrentoralandgenitalulcers,oculardiseaseandskinlesions.

• TheprevalenceishigherincountriesaroundtheMediterraneansea

• TheprevalenceintheUSvariesbetween0.2– 5.2per100,ooo

• BDismorecommoninfemales(inNorthAmerica)

• Thediagnosisisbasedonclinicalcriteria

12/22/17

5

Behcet’sdisease

• ThereareNOTpathognomoniclaboratorytestforBD

• Diagnosisrequirestheobservationofrecurrentoralulcerations(threeepisodeswithinany12monthperiod)plusANYtwoofthefollowing:recurrentgenitalulcers,eyelesions,skinlesionsorapositivepathergytest.

• ManagementofBDischallengingà useofanti-TNF-α,colchicinesteroids,immunomodulatorsandimmunosuppressants.

Francisco,9year-old

9yearsold,boy,Caucasian

Thisseemstohaveallstartedwithavirusthatmostofhisfamilygot.

“Hestartedwithsmallwhitebumpsonhistongue(earlyApril)verypainful.ParentstookhimtothepediatricianwhoprescribedChlorhexidine.Then,parentstookhimtothepediatricdentist.Hispediatricdentistsaidthathistonguewasinflamedandprescribedasteroidrinse(Dexamethasone).Withinthenext2days(May12)thelesion(s)weregotworse.Hisdentistthensendhimtoanoralsurgeon.Hecouldn’tgivetheparentsanyideawhatitwasandsaidsometimesthingslikethisjusthappenandifyougetridoftheopeningthenitwouldheal.ParentscalledhispediatricianagainwhoreferhimtotheENT.TheENTphysicianreferredagaintopediatricdentistry“

12/22/17

6

9yearsold,boy,Caucasian

Neverbefore.Firsttimeofsomethinglikethis

PastMedicalHistoryPMH:UnremarkableMedications:NonePastSurgicalhistory:UnremarkableAllergies:NKDASocialhistory:Excellentfamilysupport

Reviewofsystems:Neurologic:NosymptomsGI:OccasionalGI“disturbances”(notwellexplainedbythemom)Immune:NosymptomsCardiovascular:Nosymptoms

PE: Withinnormallimits,exceptforbelowidealpercentileweightandheight

InflammatoryBowelDisease:Crohn’sDisease

• IBSisachronicrelapsingdisorderofunknownetiology(probablyimmunerelated)thatencompassestwodifferentconditions:Crohndisease(CD)andulcerativecolitis(UC)

• InCDtheinflammationoccuranywhereintheGItract(includingthemouth)

• CDcausesabdominalpain,diarrhea,weightlossandinsomecasesanorexia

• TheannualincidenceofpediatricCDintheUSisbetween0.2-8.5casesper1000,000

• Approximately10%ofpatientswithCDhaveoralmucosaulcers,andtheoralmanifestationsoccasionallyprecedeGIsymptoms

• OralulcersinCDoftenhaveinduratedbordersandarehistologicallydifferentfromRAS

Ulcersintheoralcavity?arealltheSAME (causedbyavirus!)

12/22/17

7

RecurrentAphthousStomatitis

RecurrentAphthousStomatitis• RASisthemostcommonulcerativediseaseoftheoralmucosa.

•AGE– AGE- AGE

• Healthyindividuals.

• Involvementoftheheavilykeratinizedmucosaofthepalateandgingivaisuncommon.

• Oftencomplexdifferentialdiagnosis:neutropenia,Crohn’s,SLE,etc..

• Severalfactorshavebeenproposedasapossibleetiology.

• Extensiveresearchhasfocusedonimmunologicalfactors,butadefinitiveetiologyofRAShasnotbeenconclusiveestablished.

RAS

Major

Minor

Herpetiform

Lessthan1cmHealwithoutscars

Largerthan1cmPersistsforweeksandmonthsHealwithscar

Differentialdiagnosis


12/22/17

8


Epidemiology

• Approximately20%ofthegeneralpopulationisaffectedbyRAS.

• TheepidemiologyofRASisinfluencedbythepopulationstudied,diagnosticcriteriaandenvironmentalfactors.

• Inchildren,prevalenceofRASmaybeashighas39%andisinfluencedbythepresenceofRASinoneorbothparents.

• TheonsetofRASseemstopeakbetweentheagesof10and19yearsbeforebecominglessfrequentinadvancedage,gender,orrace.

Sollecito T. Oral soft tissue lesions. Dental Clinics of North America 2005; 49: 1


Etiology

Localfactors: TraumaNegativeassociationwithsmokingChangesofsalivapH

Microbialfactors HelicobacterpyloriàNostrongassociationS.sanguisàAntigenstimulant

UnderlyingMedical - Behčet’ssyndromeCondition - MAGICsyndrome:mouthandgenitalulcerswith

inflammationofthecartilage- Crohn’sdisease- Cyclicneutropenia- PFAPAsyndrome:periodicfever,RAS,pharyngitis,andcervicaladenitis



Etiology

HereditaryandGenetic TheroleofheredityistheFactors BESTdefinedunderlyingcause

ofRAS

ChildrenwithRAS+parentshavea90%chanceofdevelopingRAS

HLA-A2,HLA-B5,HLAB12


12/22/17

9

Recurrent Aphthous Stomatitis

Etiology

AllergicFactors HypersensitivitytofoodMicrobialheatshockproteinsSodiumSulfateà toothpaste**

Immunologicfactors - AbnormalproportionofCD4andCD8- Elevatedlevelsofinterleukin-2- ElevatedlevelsofIFNalpha- Local– dysregulatedcell-mediatedimmuneresponseà accumulationofTcells(CD8).

Nutritionalfactors Smallnumberassociationwithlowlevelsofiron,folate,zinc,VitaminsB

Recurrent Aphthous Stomatitis

Clinical Manifestations

• RASpatientsusuallyexperienceashortprodromalburningsensationthatlastfrom2to48hoursbeforeanulcerappears.

*NOGINGIVITIS

•Ulcersareround,welldefinedwitherythematousmarginsandshallowulceratedcentercoveredbyayellowpseudomembrane.

• Usuallydevelopinnon-keratinizedmucosa.

• Theylastapproximately7to10days.

• Histologicalcharacteristicsarenospecific.


Treatmentà TOPICAL

• Thetreatmentdependsonthefrequency,size,andnumberofulcers.

• Patientswithoccasionalepisodesofminoraphthousulcersexperiencereliefwithtopicaltherapy

Zilactin®,Orabase®,CankerMelts®,Amlexanox®

• Patientswithmorefrequentormoreseverediseaseà Topicalsteroids(Fluocinonide0.05%)

• Topicalantibiotics:Tetracyclinemouthrinseshavebeenreportedtodecreaseboththehealingtimeandpainofthelesionsinseveraltrials.Morerecentlyà PenicillinGtroches

12/22/17

10


Treatmentà SYSTEMIC

• Shortcourseofsystemicsteroids(prednisone).

• Pentoxifylline(PTX)amethylxanthinerelatedtocaffeine,hasbeenusedformanyyearstotreatintermittentlegcrampsinpatientswithperipheralvasculardisease.PTXimprovescirculationincreasingtheflexibilityofRBC.PTXhasalsoshowntodecreaseinflammationbyitself.

• SeveralreportsoftheuseofPTX,400mgthreetimesaday.

• Othermedications:colchicine,thalidomideandDapsone.

Worthington H, et al. Interventions for preventing mucositis for patients receiving treatment. Cochrane Database Systematic Reviews 2007; 14

Thornhill MH, Baccaglini L, et al. A RCT of pentoxifylline for the treatment ofRAS. Arch Dermatol 2007; 143: 463-470

UlcersintheoralcavityarealltheSAME ?

MostCommonulcerscausedbyvirusintheoralcavityofchildren

12/22/17

11

MostCommonViralInfectionsofTheOralCavity

RNAà CoxsackievirusgroupA

DNAà HerpesSimplexVirusHumanPapillomaVirus


RNAàCoxsackievirusgroupA


RNA à Coxsackievirus group A

www.bilbo.bio.purdue.edu

üHerpangina

üAcutelymphonodularpharyngitis

üHand-foot-and-mouthdisease

12/22/17

12

Most Common Viral Infections of The Oral Cavity


Pinto A. Pediatric soft tissue lesions. Dental Clinic of NA 2005; 49: 241-258

Herpangina Oral ulcerations limited to the soft palate, uvula tonsils, and fauces.

Incidence of the disease peaks during the initial months of summerand fall.

Sudden fever, sore throat, headache, dysphagia, and malaise followed in 24 to 48 hours by erythema and vesicular eruption.



Pinto A. Pediatric soft tissue lesions. Dental Clinic of NA 2005; 49: 241-258

HFMD Frequently seen in epidemics outbreaks in day care or school agechildren.

Mild headache and malaise followed by skin and oral lesions.

Presence of limb lesions.


DNA àHerpes virusHuman Papilloma Virus

12/22/17

13

Oral Herpetic Infections

• Herpes virus cause a primary infection whenthe patient initially contacts the virus and then remain latent within the nuclei of specificcells for the life of the individual.

• HSV 1, and VZV remain latent in sensorynerve ganglia.

Photo courtesy of Dr. Craig Miller


Primary herpes virus infections

• The incidence of primary infections with HSV-1 increases after 6 months.

• The incidence reaches a peak between 2 and 3 years of age.

• A significant percentage of primary herpes infections are subclinicalor cause pharyngitis difficult to distinguish from URI.

• Significant prodromal with generalized marginal gingivitis.

• Primary HSV in healthy children is usually self limiting disease.

• Treatment: palliative Antiviral ?


• After reactivation, HV can cause localizedsymptomatic or asymptomatic recurrentinfections.

•They are transmitted from host to hostby direct contact with saliva or genitalsecretions.

12/22/17

14


Recurrent herpes simplex infections

• Following resolution of a primary HSV infection, the virus migrateto the trigeminal nerve ganglion à latent state

• Reactivation of virus may followexposure to cold, sunlight, stresstrauma, or immunosuppression

• “cold sore” or “fever blister”



• Several studies have been published comparing topical antiviral medications for treating RHV

•Topical penciclovir (Denavir®) reduces the duration and pain of RHV by 1 or 2 days

•Topical acyclovir has been reported to decrease duration of RHL lesionsby 12 hours and found to be more effective than N-docosanol (abreva®)

• Other topical products

• Systemic treatment



• Differential diagnosis:

RAS (NO prodromal symptoms and NO gingivitis)

Coxsackie viral infections (hand-foot and mouth – herpangina)

Erythema multiforme

• Laboratory testing: It may be necessary to diagnose atypical presentations.

Gold standard à tissue culture

Cytology smears àTznack smear

Immunology test à (DFA)

12/22/17

15

Edward,10year-old

ErythemaMultiforme

Erythema Multiforme

12/22/17

16

• Erythemamultiforme(EM)isatypicallymild,selflimiting,andrecurringmucocutaneousreactioncharacterizedbytargetlesionsoftheskinandmucousmembranes.

• Greatvariabilitybetweenepisodes

• Typicalageisbetween7and21years.Morefemalesthanmales.

• EMischaracterizedbysymmetricallydistributedskinlesions.

ErythemaMultiforme

Leaute et al. Diagnosis, classification, an management of EM and SJS. Arch Dis Child 2000; 83: 347

Etiology

• Herpessimplexvirus(HSV)istheinfectiousagentin60%to70%ofthecases.

• HSVantigensareexpressedintheendothelialcellsofthebloodvesselsandkeratinocytesofEMlesionsà targetfortheimmuneattack.

• EM:drugsprecipitatesomecasesofEM(sulfonamides:trimethoprim-sulfamethoxazole,NSAIDs,PNC,etc.)

ErythemaMultiforme


ClinicalPresentation

• Thelesionsareinafixedpositionwithasymmetricdistribution.

• Acentralblisterorareaofnecrosismaybepresent.

• Prodromalsymptomsarerare,andfewsystemicsymptomsarepresentduringtheEMepisode.

•Oralmucosallesionsoccurinmorethan70%ofcasesofEMà althoughlesswellrecognized,EMdoespresentasoralmucosalulcerationswithfeworNOskinlesions.

• Preferredsitesofinvolvementincludethelips,alveolarmucosa,andpalate.

•Orallesionsarepainfulandmaycompromisedspeechandeatingà healwithoutscarring

ErythemaMultiforme


12/22/17

17

ClinicalPresentation

•MildsymptomsassociatedwithEMaretypicallytreatedsymptomatically.

• Topicalcorticosteroidsuspensionsprovidesymptomaticreliefofpainfuloralulcers

• Systemicantiviralagents(valacyclovir500mgbidà abortiveorValacyclovir500mgbidx1yearsuppressive)

• Systemicsteroids:48to72hours

ErythemaMultiforme


WhatwelearnedfromPatriciaFrancisco,andEdward?

1. Not all the ulcers in the oral cavity in children are the SAME !!!

2. “tips” for the differential diagnosis (not always work àuse glasses!)

3. Different treatments

Josephine….from4y.o.until12y.o.

12/22/17

18

4years4monthsold

PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimits.Nocarieslesionswerenoted.Follow-up:6months.Excellentoralhygiene

5years2monthsold

PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimits.Nocarieslesionswerenoted.FU:6months.Excellentoralhygiene

6years2monthsold

PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitis.Nocarieslesionswerenoted.Excellentoralhygiene

LONG HISTORY…................

7yearold

PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitis.Nocarieslesionswerenoted.Excellentoralhygiene

7year8monthsold

PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitisandsome“blisters”inthelipsandgums.Nocarieslesionswerenoted.Moderateplaque.Treatment:reinforceOH

8year6monthsold

PastMedicalHistory:UnremarkableNomedications NoallergiesOralexamwithinnormallimitsexceptformarginalgingivitis.Carieslesionswerenotedontooth#A(MO)andtooth#B(DO).Excellentoralhygiene

12yearold

PastMedicalHistory:Unremarkable Nomedications NoallergiesOralexamrevealedinflammationanderythemaonfacialgingiva(mandibleandmaxilla)withNOplaqueaccumulation.Diagnosis:PubertygingivitisTreatment:Patientreferredtoperiodonticsforassessmentandtreatment

12yearold

EmergencyAppointmentChiefComplain:“tissuesloughingfromthebackofthemouth”PE:Notedgeneralizedgingivitis.Gingivaappearstobesloughing(butgoodOH).Largeulcerationsnotedlingualto#20and#21.Theclinicalexamrevealednoprecipitatingfactorsforgingivitis

12/22/17

19

12yearold5months

PMH:Unremarkable Nomedications NoallergiesErythemaassociatedwith“peelingoff”wasnotedoverthegingivaaroundeanteriormaxillaryandmandibularteeth.

Impression: Desquamativegingivitis

- Erosivelichenplanus(ELP)- Mucousmembranepemphigoid(MMP)- Pemphigusvulgaris(PV)

Plan:StopallOHproductsandexcisional(“punch”)biopsy

Desquamative Gingivitis ???

Des

quam

ativ

e G

ingi

vitis

(p

edia

tric

pat

ient

s)

• Clinicaltermtodescribered,painful,“peelingoff”gingiva.

• Atleastthreedifferentmucocutaneousconditionspresentasdesquamativegingivitisinchildren.

• Desquamativegingivitiscanbemistakenforplaqueinducegingivitisandthiscanleadtodelayeddiagnosisandinappropriatetreatment.

• Thereislossofstipplingandthegingivamaydesquamateeasilywithminimaltrauma

Robinson N. Desquamative gingivitis: A sing of mucocutaneous disorders.Australian Dental Journal 2003; 48: 4

Des

quam

ativ

e G

ingi

vitis

(p

edia

tric

pat

ient

s)

Robinson N. Desquamative gingivitis: A sing of mucocutaneous disorders.Australian Dental Journal 2003; 48: 4

• Gingivalerosivelichenplanus(ELP)

•MucousMembranePemphigoid(MMP)

•PemphigusVulgaris(PV)

• Crohn’sdisease,LinearIgA,plasmacellgingivitis

12/22/17

20

Pemphigusandpemphigoidaretwoofagroupofbullousdiseasesaffectingtheoralmucosaandskin.

Theyarebothautoantibody-mediateddisease,althoughthetargetantigensarequitedifferentintypeandlocation.

Mucousmembranepemphigoid(MMP)comprisesaheterogeneousgroupofdisorderscharacterizedbysub-epithelialseparationandthedepositionofIgandcomplementalongthebasementmembrane(BMZ).

PemphigusischaracterizedbyacantholysiswithintheepitheliumowingtothebindingofIgGauto-antibodiestodesmogleins.(1)

Pemphigusaffectsbetween0.1and0.5patientsper100,000dependingonthepopulationstudied.(2)

(1) NguyenDQ.Cicatricialpemphigoid:diagnosisandtreatment.IntOphtalmol Clin1996;6:41-60(2) AhmedAR.Pemphigus:currentconcepts.AnnIntMedicine1980;92:396-405

Pemphigus Vulgaris I

Diet Garlic

Drugs sulfhydryl radicalpenillaminecaptoprilnonthiolrifampicindiclofenac

Viruses HVS

Other factors Smokingpesticides

Pemphigus Vulgaris V

Etio

logy

Pemphigus àAuto-antibodies àUnknown

Suppression Removal

Swanson DL. Pemphigus vulgaris and plasma exchange. J Am Acad Dermatology 1983; 81: 258-60

AzathioprineCyclophosphamideMethotrexateGoldCyclosporinePrednisone

Oportunistic infectionsBone marrow suppressionRenal failureSkin problemsBladder problems

Pemphigus Vulgaris VI

12/22/17

21

WhatwelearnedfromJosephine?

The importance of medical and dental history!!!!

Theresa,10y.o.

10yearold,girl,Caucasian

A10-year-oldgirlpresentedtothepediatricdentistforaregular6monthsrecall.Theextra-oralexamwaswithinnormallimits.Theintraoralexamwaswithinnormallimits,exceptforawelllocalizederythemaatthegingivaoftooth#8.Theperiodontalexamdidnotshowincreaseinthegingivalpocketoftooth#8andnobleedingwasobserved.Fairoralhygiene.

12/22/17

22

LocalizedJuvenileSpongioticGingivalHyperplasia

Thislesionisconsideredauniqueanddistinctiveformofinflammatorygingivalhyperplasiaseeninyoungpatients(averageage11.8years),predominantlyfemaleandgenerallyfoundinthemaxillaryanteriorregion

ThistypeoflesionwasfirstdescribedbyDarlingetal.asjuvenilespongioticgingivitis.

AftertheinvestigationofalargersamplesizebyChangetal.themoreaccuratetermLJSGHhadbeensuggested.

Itappearsasabrightredraisedovergrowthwithapapillaryorfinelygranularsurface,however,itdoesnotseemtobeaplaquerelatedlesion.


Thelesionpresentsasasmall(averagesizewas6mm),localizedandeasilybleedingovergrowthonthegingivaofachild.

Itisusuallygiventheclinicaldiagnosisofpyogenicgranulomaandfrequentlyseeninconjunctionwithorthodonticbrackets,whichmaybepurelycoincidentwiththepatientpopulation.

Thelesionisnotpainful,butbledeasily.


Thehistologicpresentationisanexophyticlesionwithasubtlepapillaryarchitecturecomposedofinterconnectingbandsofepithelialhyperplasia.

Thehistologyisuniqueandcharacterizedbyprominentintercellularedema(spongiosis)andneutrophilicexocytosis.

Thepresenceofhighlyvascularconnectivetissuecoresisseencontainingmostlyacute,butwithsomechronicinflammatorycells

LJSGHdisplaysasagingivalovergrowthratherthanapureinflammatoryprocesswithminimaltonotissueswelling.

12/22/17

23


Theetiologyisunknownandthelesiondoesnotrespondtoperiodontaltreatmentshowingalackofassociationwithplaqueorcalculus.

Darlingetal.comparedjuvenilespongioticgingivitis/LJSGHwithpubertygingivitisandfoundseveraldistinguishingfeaturesincludingalackofimmunoreactivityforestrogenandprogesteronereceptorsinLJSGH

TreatmentforLJSGHisconservativesurgicalexcisionandthecarbondioxidelaserisidealfortreatingthislesion.Theyoungageofthepatientmakeslaserablationapreferredandveryefficientprocedure,welltoleratedbythispopulationofpatients.Recurrenceisrareandwhenitdoesoccur,maybeduetoincompleteremovalofthelesion.

WhatwelearnedfromTheresa?

Alex..3 year-old

12/22/17

24

3 yearold,girl,Caucasian

Patientisa3yearoldhealthygirlwhopresentedtothepediatricdentalofficewith“whitestuff”inhermouthforthelastweek.Nocomplainofpain.

PMH:Noncontributory Allergies:NKDA

Medications:None

Reviewofsystems:Essentiallynegativeexceptforarecentepisodeofotitismedia

Basedontheclinicalpicturesandhistory,whichofthefollowingdiagnosisatthispointyouwillplacehigherinthedifferential?

1. Candidiasis

2. Candidiasis

3. Candidiasis

4. Other(IdiopathiccandidiasisL)

Case 4

RBC: 4.5 mm³

Platelet: 250,000

Hg: 14 gr/dlWBC: 6,300

Lymphocytes: 10%

Patient laboratory results

12/22/17

25

Clinicalfeatureswhichmayindicateimmunodeficiency

• Threeormoreepisodesofotitismediain6months• Persistentpurulenteardischarge• Twoormoreserioussinusinfectionwithinoneyear• Twoormoreepisodesofpneumoniawithinoneyear• Failuretothrive• Recurrentdeepskininfections• Persistentcandidiasis• Familyhistory

Primary Immunodeficiency

Both

B-cell

T-cell

Primary Immunodeficiency Disorders

•Bcell(antibody)deficiencies

•Tcelldeficiencies

•CombinationBandTcelldeficiencies

•Defectivephagocytes

•Complementdeficiencies

•Unknown(idiopathic)

Primary Immunodeficiency

Both

BcellTcell

12/22/17

26

Immunity to infection

The areas of the immune system to consider are:

• Humoral immunity (B cells and Immunoglobulins production

• Cell-mediated immunity (T cells), neutrophils

• Complement cascade

T cell disorders

B cell defects

Phagocyte disorders

Complement disorders

Selective IgADeficiency

stem cell

Myeloid progenitor cell

Neutrophil Monocyte

Lymphoid progenitorcell

B-cell

Ig-A

12/22/17

27

Selective IgA deficiency

• SelectiveIgAdeficiencyisoneofthemostcommontypesofprimaryimmunodeficiency

• Manypatientsgoundiagnosedbecausetheyareneversickenoughtobeseenbyadoctor

• PatientwithselectiveIgAdeficiencydoproducealltheotherIg

• Thecauseisunknown

• ChildrenwithselectiveIgAdeficiencyareatriskofinfection.abouthalfhaverepeatedinfectionsoftheears,sinusandairway

• ChildrenwithIgAdeficiencyareatincreasedriskforanaphylacticreactions

WhatwelearnedfromAlex?

Sophia

Hillary

Bobby

Kawne

12/22/17

28

Review of Patient I• Age:13y.o,girl.

• ChiefComplaint:“Thereissomethinginmygums”

• HPI:Asymptomaticlesionbetween10and11.Unknownduration.

pedunculated.

• PastMedicalHistory(PMH):

- Hypothyroidism

- Anemia

- Visionproblems

• PastDentalHistory(PDH):Lastvisittothedentist:18monthsago

June 9 2011

Themicroscopicsectionsrevealapapillarynoduleofmucosathatissurfacebyparakeratoticstratifiedsquamousepitheliumwhichformsseveralshortprojectionsofmucosawithconnectivetissuecores.

Theunderlinelaminapropiaconsistsoffibrousconnectivetissuewithscatteredsmallvascularchannelsandneuralbundles.

Diagnosis:Papilloma

Review of Patient III• Age:4y.o,girl.

• ChiefComplaint:“Thereissomethinginthebackofmymouth”

• HPI:Asymptomaticlesionatthejunctionbetweenhardandsoftpalateattherightside.Unknownduration.Wellpedunculated.


Unremarkable


12/22/17

29

Review of Patient IV• Age:17y.o,boy.

• ChiefComplaint:“Thereissomethinginthefrontofmymouth”

• HPI:Asymptomaticlesionbetweenmaxillarycentralincisors.Unknownduration.Wellpedunculated.


Asthmawellcontrolled.UsingsteroidsPRN


July 17 2011

HPV

üSquamousPapilloma

üVerrucaVulgaris(commonwart)

üCondylomaAcuminatum

üMultifocalEpithelialHyperplasia(HeckDisease)

Oral and Maxillofacial Pathology, Neville, Dam et al , 2009

Squamouspapilloma

-Benignproliferationofstratifiedsquamousepithelium-ThelesionisinducedbyHPV-Exactmodeoftransmissionisunknown-Equalfrequencyinboysandgirls-Morecommonplaces:tongue,lipsandsoftpalate.-Thepapillomaisusuallysolitary-Histopathology:proliferationofkeratinizedstratifiedsquamousepitheliumin“fingerlikeprojections”withfibro-vascularconnectivetissuecores.

Oral and Maxillofacial Pathology, Neville, Dam et al , 2009

12/22/17

30

CondylomaAcuminatum

CondylomaacuminatumisaSTDappearingmostfrequentlyasasoft,pinkcauliflowerlikegrowth.

Theconditionishighlycontagious

Bothgendersareaffectedequally

Thepeakincidenceisbetween17to20

Thehistologyshowsà papillarylesions

MultifocalEpithelialHyperplasia(Heck’sDisease)

-Virusinduced,localizedproliferationoforalsquamousepitheliumthatwasfirstdescribedinNativeAmericans-AssociatedwithHPV13and32-Insomepopulations,atmanyas39%ofchildrenareaffected.-Theconditionusuallyaffectsmultiplemembersofthesamefamily-Multiple,flattened,soft,nontenderpapulas,usuallyclusterandwiththesamecoloroftheoralmucosa.

O ral and M axillo facial P ath o lo gy, N eville, D am et al , 2009

Humanpapillomavirusinfectionoftheoralmucosa

12/22/17

31

Humanpapillomavirusinfectionsoftheoralmucosa

Classifica

tion • Papillomavirusesaresmall,doublestrandedDNAviruses.

• HumancanbeinfectedonlybyHPV’s,notbypapillomavirusesfoundinanimals.

• TheHPVgenomecontainseightopenreadingframes(ORFs)whicharepotentiallycodingsitesofsixearlyproteins(E)andtwolateproteins(L).TheL1ORFisusedtoidentifythedifferenttypesofHPVbecauseitisthemostconservedoftheeightORFswithinthegenome.(1)

1. Rautava J, Syrjanen S. Human papillomaviruses infections in the oral mucosa. JADA 2011; 142(8):905-914


Classifica

tion • Investigatorshavedescribedmorethan120differentHPVtypesonthebasis

oftheisolationandsequencingofcompletegenomes.(1)

•MostHPVthatinfectoralmucosasitebelongtothealphapapillomaviruses,whichconsistof15species.(2)

• Todate,investigatorshaveidentifies30HPVgenotypes:15high-risktypes,3typesthatprobablyarehighriskand12low-risktypes.

1.RautavaJ,SyrjanenS.Humanpapillomavirusesinfectionsintheoralmucosa.JADA2011;142(8):905-914


Classifica

tion

RautavaJ,SyrjanenS.Humanpapillomavirusesinfectionsintheoralmucosa.JADA2011;142(8):905-914

HPV-16

LCRà Regulationandvirusgeneexpressionandvirusreplication

HPV-16

Majorcapsideprotein

Minorcapsideprotein

Membranesignalingprotein

E1

E2

E3E4

E5

E6E7

12/22/17

32

MariaFernanda14year-old

(thisismyonlychangetogetinvitedagain)

IdiopathicBoneSclerosisvs.

Cemento-osseousdysplasia

PrinciplesofRadiographicInterpretation

12/22/17

33


“Thereisaradiopacity,nottoobig,nottoosmall,looksactuallyfunny…..locatedattheapexoftooth#28”.

Condensingosteitis


DifferentialInterpretation

RadiographicDescription

Somedefinitions

12/22/17

34

SummaryofInterpretationSteps

1. Radiopaque/Radiolucent/mix2. Welldefined/illdefined/mix3. Corticated/non-corticated/mix4. Location,sizeandshape5. Whathappeninthe“neighborhood”6. Differentialinterpretation

let’spracticewithsomefilms

SummaryofInterpretationSteps

1. Radiopaque/Radiolucent/mix(mymom..)2. Welldefined/illdefined/mix(mymom)3. Corticated/non-corticated/mix(mymom)4. Location,sizeandshape5. Whathappeninthe“neighborhood”6. Differentialinterpretation

12/22/17

35

Mostcommonjawlesionsin

children

4 Mostcommonjawlesionsin

children

BoneDiso

rders 1. Idiopathic Bone Sclerosis

2. Fibrous Dysplasia

3. Simple Bone Cyst

4. Cemento-osseous dysplasia

12/22/17

36

IdiopathicBoneSclerosis

(notcondensingosteitis!)


IBSisafocalsolitaryscleroticlesionthatarisesinthelate1storearly2nddecadeoflife.

Itscauseisunknown.

Itisasymptomatic,isnotassociatedwithinflammation,andmayremainstaticordemonstrateslowgrowththatusuallystopswhenthepatientreachesskeletalmaturity.


In90%ofpatientsitoccursinthemandible,usuallynearthefirstmolarorsecondmolarorpremolar.

Atimaging,IBSisradiopaque,welldefined,welllocalized,non-corticated,locatedattheapexofvitalteeth.Norootresorptionandnoteethdisplacement.

Somepatientsmayhavemultiplelesions.

12/22/17

37

SimpleBoneCyst

-Traumaticbonecyst,hemorrhagicbonecyst,solitarybonecyst

-Simplebonecyst:Itisacavitywithinbonethatislinedwithconnectivetissue.Itmaybeemptyoritmaycontainfluid

-Itisnotatruecyst

-Etiologyunknown,howeverà Localizedaberrationinnormalboneremodelingormetabolism

-Firsttwodecadesoflife(mean17years)

-Females2:1

-Expansionispossiblebutunusual,discoverybychance

SimpleBoneCyst

àMandible

àPosteriormandible

àAssociatedwithcemento-osseousdysplasiaandfibrousdysplasia

àMargin:Welldefinedtoill-definedborder

àInternalstructure:Radiolucent,itmay appearmultilocular,althoughthelesiondoesnotcontaintruesepta

àNoeffectonthesurroundingteeth

àLaminaduraintactandvitalitypositive

12/22/17

38

FibrousDysplasia

LocalizedchangeinnormalbonemetabolismàReplacementofallcomponentsofnormalbonebyfibroustissuecontainingvaryingamountsofabnormalappearingbone

Solitaryormultiple(monostotic70%ofallcases)

McCune-Albrightsyndrome

Asymptomatic,rareassociatedwithpain

Thelesionmaybecomeactiveà Pregnant,etc..

Theskullisinvolvedin10to25%ofcases

Patientsbetween10and30yearsold

12/22/17

39

FocalCemento-OsseousDysplasia

Focalcemento-osseousdysplasia-LocalizedchangeinnormalbonemetabolismàReplacementofthecomponentsofnormalbonewithfibroustissueandcementum-likematerial,abnormalboneoramixtureofthetwo

-Neartheapexofatooth

-Middleage,females9:1,Afro-American

-Incidentalfinding

-Theinvolvedteetharevital

-Sometimesquitelarger

Source: Oral Radiology, Principles and Interpretation. White et al ,2000

Focalcemento-osseousdysplasia

-Epicenterusuallyattheapexofatooth

-Welldefined,Irregularlyshaped

-Internalstructure:Variesà Radiolucentarea

Mixedstage:RadiopaqueandRadiolucentareas

Maturestage:Radiopaque

-Laminaduraoftheteethinvolvedwiththelesionislost

-Rare:resorptionoftheroots

12/22/17

40

let’scomebacktoMariaFernanda…..

Accordingtothepatient'smother“shehadherwisdomteethout2monthsagoandshehadaknotinthearearightaftertheextractionbuteventuallywentaway”.Then2monthslaterwithswellingandpain overtheleftmandible.

Momconsultedwiththeoralsurgeon.Noconcernsovertheareaofextractionoftooth#17

Painpartiallycontrolledwithibuprofenandantibiotics(?)

Accordingtothepatient,“mywholejawhurts,thosethreeteethareaching(18-19-20)”

Initiallyreferredtotheendodontistforrootcanaloftooth#20

Mydifferentialdiagnosisà Neuropathicpain ?

Differentialdiagnosisà Neuropathicpain?

Neuropathicpainischronicpainconditioncausedbyanalterationintheperipheralorcentralnervoussystem(trigeminalneuralgia,atypicalodontalgia,burningmouthsyndrome,traumaticneuropathies,post-herpeticneuralgia,andcomplexregionalpainsyndrome

OftenneuropathicpainismisdiagnosedwhichcanleadtounnecessaryTREATMENT

Vascularcompression,radiation,inflammation,trauma,infection,etc.canleadtoneuropathicpain

12/22/17

41

Differentialdiagnosisà Neuropathicpain?

Pain:short– electrical– sharp– shooting– paresthesia– swelling

Extractionofthirdmandibularmolars,dentalinjections,implanttreatments,andendodontictreatmentsarethemostcommonproceduresindentistryassociatedwithneuropathicpain

Treatment:Amitriptyline,pregabalin,gabapentin

Suggestedtoseeaneurologist

WhatwelearnedfromMariaFernanda?

1. Radiographic interpretation (probably the MOST important goal)

2. Common bone lesions in children (just remember one: IBS)

3. Neuropathic pain in children (you can delete that from your brain immediately)

4. Use of CBCT in pediatric dentistry (who cares?)

Special thank you to Dr. Jennifer Garvey, DABPD, pediatric dentist in Greenville, SC

Mary,4year-old

12/22/17

42

4.5year-old,girl,Hispanic

6 months ago, parents noticed intraoral swelling of thegingiva (not a lot of pain)...Dad said it was mostly aroundthe back teeth. He took them to their general dentist.

PMH: Unremarkable

Allergies: NKDA

OrofacialGranulomatosisI

Orofacialgranulomatosishasbecomeawell-acceptedandunifyingtermencompassingavarietyofclinicalpresentations(biopsy=non-specificgranulomas)

Orofacialgranulomatosis~Aphthousstomatitis(idiopathicbutappearstobeanabnormalimmunereaction)

SystemicdiseasethatmimicOG:Crohn’sdisease,sarcoidosis,tuberculosis

Severaltriggersareinvolved

Themajorityofpatientsareadults.However,whenisinchildrenà strongassociationwithasymptomaticinflammatorygastrointestinalprocess (differentfromCrohn’sdisease)anassociatedwithdietarytriggers

Clinicalpresentationishighlyvariable.Mostcommonsite:LIPS

Whenthesignsarecombinedwithfacialparalysisandfissuretongue:___________________________

Intraoral:Tongueandgingiva(swellinganderythema)

Melkersson-Rosenthalsyndrome

Orofacial Granulomatosis II

ThediagnosisofOGà histopathology:presenceofgranulomasassociatedwithnegativestainsfororganismsandnoforeignmaterial

Treatment:

• Firstgoalà identifythepossiblecause(noteasyatall)

• Inchildrenà StrongconsiderationtodietaryallergensoranassociationwithunderlyingGIdisease

• Topicaluseofsteroids(similartoRAS),TNF-α antagonist(infliximab),intra-lesionalinjectionsofsteroids

Prognosisà highlyvariable

12/22/17

43

WhatwelearnedfromMary?

1. The importance of a detailed medical history (Most important goal)

2. Orofacial Granulomatosis (well…....not to common)

SoftTissueLesionsinChildren

andAdolescents(besideswealreadyreviewed)

IdiopathicGingivalFibromatosis

Idiopathic gingival fibromatosis (IGF) is an enlargement localized orgeneralized of the gingival tissue characterized by an expansion andaccumulation of the connective tissue, mainly collagen type I.

Gingival fibromatosis is commonly induced as a side effect ofsystemic drugs (such as phenytoin, nifedipine, and cyclosporine).

12/22/17

44

IdiopathicGingivalFibromatosis

However, in some cases the gingival overgrowth is idiopathic. Theenlargement is more prominent during the eruption of the primary andpermanent teeth. Poor oral hygiene has been also associated with thecondition.

The diagnosis is established through history, clinical examination andhistopathology.

Surgical treatment including gingivectomy and gingivoplasty are usually thetreatment.

DermoidCyst

Dermoid cyst are malformations that are common in the oralcavity.

Dermoid cyst are development lesions found inside organs ortissues as a result of the inclusion of tissue from diverse sources(ectoderm, mesoderm or endoderm)

Dermoid cyst of the oral cavity are often relatively softunfluctuating masses, frequently adhered to the child’s hyoidbone.

DermoidCyst

The differential diagnosis includes lipoma, ranula, thyroglossal duct cyst,cystic hygroma, andmalignant tumors.

Boys aremore affected than girls by a ratio of 3:1.

Almost one in five of the dermoid cyst that occur in the head and neck areaare located in the floor of the mouth and often they cause tongueelevation.

Usually they are diagnosed at young ages. The treatment is completesurgical excision and they have a good prognosis.

12/22/17

45

Mucocele• Commonlesionoftheoralmucosathatresultsfromruptureofsalivaryglandduct

andspillageofmucinintothesurroundingsofttissues

• Themostcommonreason:TRAUMA

• Itisnotatruecyst(noepitheliallining)

• Typicallytheyaredomeshapedswellingthatcanrangefrom1to2mm

• Mostcommonlesioninchildren

• Oftentranslucent

• Fluctuantatpalpation.Fromafewdaystofewyears:Historyofrecurrentswelling

Mucocele

• ThelowerlipisbyfartheMOSTcommonsite

• Somemucocelesareshort-livedlesionsthatruptureandhealbythemselves

• Somemucocelesarechronicinnatureandsurgicalexcisionisnecessary

• Excellentprognosis

PyogenicGranuloma

Itisacommontumorlikegrowthoftheoralcavitythattraditionallyhasbeenconsideredtobenon-neoplastic.

Unrelatedwithinfectionandgranulomas!

Itisanexuberanttissueresponsetolocalirritationortrauma.

Itisasmooth,lobulatedmass,usuallypedunculated.

Microscopicevaluationshowsahighlyvascularproliferation.

Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology 3 edition

12/22/17

46

Pyogenic Granuloma

Etiology:

-connective tissue reaction to injury or other stimulus

-Hormonal changes/puberty

-Composed of hyperplastic granulation tissue

Treatment:

-surgical excision

-Frequently recurs

Neville, Damm, Allen, Bouquot. Oral and Maxillofacial Pathology 3 edition

Pyogenic Granuloma

EruptionCyst

Theeruptioncyst(orinsometextbooks,eruptionhematoma)developsfromseparationofthedentalfolliclefromaroundthecrownofatoothwhoiserupting.

Theeruptioncystisasoft,swellinginthegingivaoverlyingthecrownofaneruptingprimaryofpermanenttooth.Themajorityofcasesoferuptioncystsareseeninchildrenundertheageof10.

Thelesionismostcommonlyassociatedwiththecentralpermanentincisorsorcentralprimarymolars.

Treatmentisusuallynotrequiredbecausetheeruptioncystrupturesspontaneously.

Leiomyomas

Leiomyomas are benign tumors that originate from smoothmuscle.

The most common place that leiomyomas are found is the uterinemyometrium. However, leiomyomas are also found in the gastrointestinal tract,skin and lower extremities.

Leiomyomas are rare in the oral cavity, the most common place is the lipsfollowed by tongue, cheeks, palate and gingiva.

12/22/17

47

LeiomyomasUsually the lesion is asymptomatic, slow-growing. In children is rare.Histopathology has a key role in establishing the diagnosis.

The differential diagnosis when is located in the oral cavity is Mucocele andfibromas. Leiomyomas in the oral cavity are characterized by a solitary, usuallyovoid andmobile mass covered by normal appearing epithelium.

The consistency is usually firm with a well-defined margins. The treatment ofleiomyomas in the oral cavity is the complete resection of the lesion.

GiantCellFibromaThegiantcellfibromaisafibroustumorthatisprobablyunrelatedwithchronictrauma(differencewiththetraumaticfibroma).

Typicallythegiantcellfibromaisasymptomaticandrepresents2%to5%ofalloralfibrousproliferationssubmittedforbiopsy.

Acommondifferentialdiagnosisispapilloma.Comparedwiththeirritationfibroma,thelesionoccursatayoungerage.

Thereisaslightlyfemalepredilection.Themandibulargingivaisaffectedtwiceasoftenasthemaxillarygingiva.Thepalateisalsoacommonplace.Fromthehistopathologyperspective,thehallmarkisthepresenceoffibroblastwithinthesuperficialconnectivetissue.Thetreatmentisconservativesurgicalexcision.

PeripheralGiantCellGranuloma

-Foundonlyingingiva

-Usuallydistaltoincisors

-Maycauseboneresorption

-Appearasredorbluebroad-basedmasses

-Morefrequentinfemales

12/22/17

48

Peripheral Giant Cell Granuloma

Etiology:

-Hyperplastic connective tissue response to gingival tissue injury

-Histologically see multinucleated giant cells

-Similar in appearance to pyogenic granuloma

Treatment:

-Surgical excision

-Recurrence is uncommon

ThankyousoMUCH!!!!!!!!!

JuanF.YepesDDS,MD,MPH,[email protected]

The ABCs of Oral DIAGNOSIS in the Pediatric Patient

Documents

Transcript of The ABCs of Oral DIAGNOSIS in the Pediatric Patient