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RECURRENT RESPIRATORY

PAPILLOMATOSIS

Presentant : Ichsan Juliansyah Juanda, dr.

Supervisor : Agung Dinasti Permana, dr., M.Kes.,Sp. TT!K" #K$

%torhinolaryngologyead and &ec' Surgery Departement

(aculty %) Medicine *&PADDr. asan Sadi'in +eneral ospital

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LITERATURE READING "A0I&+!(A0I&+

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I&T0%D*1TI%&

Most common benignneoplasm of the larynx amongchildren

– 2nd

  most common cause ofpediatric hoarseness

Disease of viral etiology and

May involve entire

aerodigestive tract

Benign disease, Morbidity due

to airway involvement and risk

of malignant conversionByron ! Bailey, "ead # $eck %urgery&'tilaryngology, (th editon, )ippincot *illiams #

*ilkins, +hiladephia, 21-

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I&T0%D*1TI%&

Diagnosed at 2 and - years of age

delay from onset of symptoms

averaging about 1 year

.(/ of the children have been

diagnosed before their (th birthday

0hildhood uvenile onset recurrent

respiratory papillomatosis & '33+4

– more aggressive

5dulthood adult onset recurrent

respiratory papillomatosis& 5'33+4

Byron ! Bailey, "ead # $eck %urgery&'tilaryngology, (th editon, )ippincot *illiams #*ilkins, +hilade hia, 21-

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2PID2MI%"%+3 

AD*"T %&S2T

+eaks 2&- yrs

%light male predominance )ess aggressive

(/ pts need 7 ( proceduresover their lifetime as opposed

to 72(/ of children who cansay the same

1I"D%%D %&S2T

'ften at 2&- yrs old

Boys 8 9irls $o gender:ethnicdi;erence

More aggressive 1

child -!- per year

-

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2TI%"%+3 #P4$

D$5 containing virus & ., and 11

• 5lso cause genital warts

 =ype 11 appear to have a more

obstructive airway course early in the

disease and a greater need for

tracheotomy

'ther types identi?ed

 =ype 1> and 1@ most malignant

potential4 Byron ! Bailey, "ead # $eck %urgery&'tilaryngology, (th editon,)ippincot *illiams # *ilkins, +hiladephia, 21-

(

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2TI%"%+3   • P4 In)ection Associated 5ith

+reater 00P Disease Severity

• P4 or P4 /6 1o!In) re7uiredmore surgical intervention

suggesting more severemani)estation

>

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2TI%"%+3 P4 in)ection process initiates in 8asal layer

4iral D&A enters the cell

D&A then transcri8ed into 0&A

0&A translated into viral proteins

Byron ! Bailey, "ead # $eck %urgery&'tilaryngology, (th editon, )ippincot *illiams # *ilkins, +hiladephia,21-

.

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2TI%"%+3   ost immune response compromised 

Mal)unction o) cell mediatedresponse

Stealth li'e e9ect on immune

surveillance

"+A infection can be activelyepressed or latent 

"+A D$5 detected in the

normal mucosa o) 00Ppatients

0eactivation  can occur atany time

A%00P could be

– 5ctivation of latent virusacCuired since birth

– 5ctivation of infectioncontracted during adult life

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Pedunculated masses

5ith ;ngerli'e

pro

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MA10%S1%PI1

Sessile or pedunculated and irregular eophytic

clusters

Pin'ish to 5hite in coloration1

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2act mode o) transmission unclear

1hildhood onset lin'ed to mothers 5ith genital P4 in)ecti

Most li'ely to 8e ;rst 8orn, vaginally delivered to primigra

Adult!onset 00P possi8ly associated 5ith oral!genital cont

T0A&SMISSI%&

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T0A&SMISSI%&

5lthough there is close relationship between '33+ and maternal

condylomata, few patients exposed to genital warts at birth

manifest clinical symptoms

– &ot 5ell understood

Direct contact via the birth canal is the most likely method of

maternal&fetal transmission of "+A

– The ma-(–>(2

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Immunodeficiency Increases

Severity of RRP16

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"esion 1haracteristics

5ny site along aero&digestive track, most often occur at sites

where ciliated and sCuamous epithelium are uxtaposed

Most common 00P sites:

– )umen vestibuli

– $asopharyngeal surface of soft palate

– )aryngeal surface of epiglottis

– Fpper:lower margins of ventricle– Fndersurface of vocal folds

– 0arina

– Bronchial spurs

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"esion 1haracteristics

Airal D$5 detected at normal mucosa  recurrent after surgical

removal

Most common 00P condition:

– =racheostomiGed patient stoma and mid thoracic trachea

areas

where iatrogenic trauma to ciliated epithelium often induced

metaplasia4

– +rolonged == used alongside of the respiratory mucosa

– 93D risk factor for persistence of disease  need additional

research

1(

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Patient immunity timing, length, and volume of virus

exposure4

"ocal traumas  intubation, extra esophageal reIux4 must be

important in the development of 33+

+atients with 5'33+ had more li)etime seual partners and

a higher )re7uency o) oral se than matched controls

S21%&DA03 (A1T%0S1.

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1"I&I1A" (2AT*02S

allmar' triad:

– +rogressive hoarseness

– %tridor

– 3espiratory distress

Most often present with dysphonia

%tridor is usually 2nd symptom to manifest

Jnspiratory  biphasic

0hronic cough, recurrent pneumonia, failure to thrive,

dyspnea, dysphagia may be present

%ometimes undiagnosed until respiratory distress result

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00P >T2 +02AT

MAS?*20AD20@33+ often misdiagnosedas

Asthma

1roup

Tracheomalacia

Allergies

4ocal nodules

ronchitis

1<

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1"I&I1A" (2AT*02S

2tralaryngeal spread o) papillomas

  – 16&6/ children and 1>/ adults

– Most freCuent sites

 %ral cavity

 Trachea

 ronchi 

 Pulmonary

Dissemination

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PATI2&T ASS2SSM2&T

• 'nset of symptomsK• 3ate of progressionK

• 5ssociated infectionK

•

"ow is the cryK• +resence of respiratory distressK

• Luality of voice changeK  Etiology 

• "istory

• Air5ay trauma6 previous intu8ationB

Perinatal period

STD history

Parental condylomata6P4 

21

4ocal cord nodules S 8 l tti t

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4oice characteristics

)ow&pitched, coarse, Iuttering voice 8 subglotticlesion

"igh&pitched, cracking, aphonic, or breathy 8glottic lesion "oarseness 5)*5N% indicates someabnormality in structure:function

$eonates 05$ present with papillomatosis

C 4ocal cord nodulesC Tracheomalacia #stridor since8irth$C 4ocal cord paralysis

C Su8glottic cystsC Su8glottichemangiomaC Su8glottic stenosis

Alternative Diagnosis to thin'a8out:

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PATI2&T ASS2SM2&T

Physical 2am– 3espiratory rate:degree of distress

$asal ala Iaring

Fse of accessory neck # chest muscles

– 1yanosis6air hunger

0hild may be sitting with hyperextended neck

Jf child is very sick, examination should beperformed in setting where resuscitation: endoscopiceCuipment and possible tracheotomy is 35DJ)Navailable i!e! '3, 3, J0F4

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P3SI1A" 2AMI&ATI%&

– 5uscultation of airway with stethoscope

– 5irway endoscopy needed for de?nitive diagnosis

Olexible ?beroptic consider +5=J$=

cooperation4, smallest P 1!< mm, seCuentialvideo

xam under anesthesia esp! if patient wonQtcooperate4

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2AMI&ATI%&

$ormal 3espiratory cycle Shorter inspiratory, longer

epiratory 

%tridor of laryngeal origin   Inspiratory, progress to

8iphasic as air5ay narro5ing progress

+laced on various position  to elicit any chages on

stridor  &o changes 00P usually

'xygen saturation should be observed

+ulmonary testing  *here asthma is likely diagnosis

Blood gas analysis

2(

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MA"I+&A&T T0A&S(%0MATI%&

1&./ of patients with 33+

5dvanced disease, usually pulmonary extension

6rd or -th decade of life

)esions contain "+A type 11 and type >

+erien et al

5verage duration to malignant transformation 1

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T02ATM2&T M%DA"ITI2S

Surgical

–

Microlaryngoscopy with  cups forceps removal

– Microdebrider

– 0'2 laser

– +hono&Microsurgical

– E=+:$dN59 laser

– Olash scan lasers

Ad

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2@

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2<

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E ours post operative painF 4oice ?uality post operative

The choice to use microde8rider vs. 1% laser not onlydepends upon the a)orementioned )actors #cost,procedure time, pain, etc.$ 8ut also, the characteristicso) the lesions

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021*002&T 02SPI0AT%03PAPI""%MAT%SIS STA+I&+

5ssess)unctionalparameters,

Diagrammatically catalogssu8siteinvolvement 

5ssigns a ?nalnumeric score to the patientTs

current etento) disease 

StagingSystem

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ADJ*4A&T T20API2S

5pproximately 2/ need some formof aduvant therapy

0riteria • U - surgical procedure:year• Distal or metastatic disease• 3apid re&growth of papilloma with

airway compromised

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6-

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1ID%(%4I0

;ectiveness in intra&lesional route 3oute

Jnection of (mg:m) into papilloma bed

Microbedding total volume 2 m)

3epeating at -&> week intervals up to > months

vidence

0hadha 0omplete vs +artial response (./ vs 6(/4

McMurray $o statistical di;erence %ide ;ect 5nimal %tudies4

Malignant dysplasia of intra&lesional site

$ephrotoxicity and Mamary 5denocarcinoma

)indsay $o Malignant transformation in human

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6>

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6.

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6@

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6<

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-1

0ecom8inant )usion protein derived :

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sp2G

5ctivity has been demonstrated in genital 5art

treatment

0linical responses observed in P4 / ! negative

lesions

+ediatric patients   improving clinical course

Derkay, 2(4

2. patients #H (, E M$ aged 2&1@ years old

5fter baseline de8ul'ing surgery    sp2G

--g subseCuently monthly for H doses over /-

days

0ecom8inant )usion protein derived :

M. Bovis 1+ heat shoc' protein /#sp/$

2G protein o) P4 /

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P4 4accine

4A11I&2S A4AI"A"2 

+ardasil #Merc'$

Luadrivalent

1ervari #+laoSmithKline$ 

Bivalent 

Phase II trials have demonstrated ecellent sa)ety without

maor side&e;ects

Phase III trials have shown e9ective prevention o) genital

5art epression and progression to 0J$ JJ:JJJ

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ighlights

• 33+ is a )rustrating, captous  disease with the potential

)or mor8id  conseCuences due to its involvement o) the

air5ay and the ris' o) malignant trans)ormation!

• &o single modality o) therapy  shown e;ective, goals of

surgical therapy  a sa)e air5ay

• Many  ad

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TA&K 3%*

->

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"J%='3N %ir Morrell MackenGie 1@6.&1@

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sexuallytransmitteddisease in humans

1- million women,or about 1/ ofthe femalepopulation of child&bearing age are

D$5 positive buthave no visiblelesions while morethan @ millionwomen, or >/ ofthe at riskpopulation, are"+A antibodypositive but D$5negative

0linically apparent"+A infection has

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0esarean %ection KK

%eems to be an obvious risk reducerfor 33+ transmission, but

– "igher morbidity and mortality for

  the mother

– "igher cost compared to vaginal  delivery

– 5pprox! 1 in - children delivered

  vaginally to mothers with active

  condylomatous lesions will contract

  33+

– Oew cases have reported in utero

  development of the disease

-