2011/2012
José Miguel Al es Loba o
The Su gical app oach o Pec us Exca a um:
P esen and Fu u e insigh s
Ma ço, 2012
Mes ado In eg ado em Medicina
Á ea: Ci u gia Pediá ica
T abalho e e uado sob a O ien ação de:
P o . D . Tiago Hen iques-Coelho
T abalho o ganizado de aco do com as no mas da e is a:
Eu opean Jou nal o Pedia ic Su ge y
José Miguel Al es Loba o
The Su gical app oach o Pec us Exca a um:
P esen and Fu u e insigh s
Ma ço, 2012
1
Ag adecimen os
Ao P o . D . Tiago Hen iques-Coelho, meu o ien ado , pela disponibilidade que
demons ou pa a ou i um aluno de 5ºano com uma p opos a e ence a os
es o ços pa a que uma pequena ideia possa quiçá c esce e da u os mais
a de.
Aos P o esso es, que mesmo ó ãos do í ulo, o am-no in eg almen e nes e
meu longo pe cu so académico, apoiando-me e azendo-me c esce passo a
passo, em especial a P o . D . Augus a Sil ei a.
À minha Mãe e ao meu Pai pela o ça e c ença que semp e demons a am em
mim, com um beijo mui o sen ido à minha Mãe po me aze ques iona udo e
um especial ab aço ao meu Pai po semp e me e en ado mos a que quando
pomos um pé na Lua de emos e Ma e no ho izon e.
À Ma a po se quem é e pela o ça que me ansmi e dia iamen e, nunca
esmo ecendo e sendo peça undamen al nes e meu pe cu so na Faculdade de
Medicina.
Finalmen e à minha A ó, que ce amen e eluzi ia um o gulho que
empalidece ia o sol ao me e Médico o mado, ado a a que me pudesses e
ago a.

2
Resumo
Pec us Exca a um é uma pa ologia p e alen e em idade pediá ica, pode
a ec a a é 1 em cada 400 indi íduos e le a a dis unção ca díaca e pulmona .
Es a doença p esume-se se de o igem gené ica, le ando a um c escimen o
excessi o de es u u as ca ilaginosas es e nais. O gene esponsá el po es e
enó ipo ainda é desconhecido. Es a pa ologia ca ac e iza-se habi ualmen e po
diminuição na capacidade de exe cício assim como pe da de au o-es ima com
consequências a dias no no mal desen ol imen o social da c iança.
Uma abo dagem ci ú gica pode minimiza as complicações no desen ol imen o
psico-social e isiológico da c iança. As ci u gias mais u ilizadas são os
p ocedimen os de Ra i ch e de Nuss. O p ocedimen o de Ra i ch consis e
numa ci u gia com exposição de es u u as ósseas e co eção local de de ei os
cos ocond ais. O p ocedimen o de Nuss é minimamen e in asi o e eco e a
uma ba a p é- o mada que é colocada na ca idade o ácica com auxílio de
ib oscopia.
Nes e a igo os au o es suma izam o que é co en emen e emp egue pa a
co igi es a pa ologia de o ma ci ú gica e não-ci ú gica assim como
p ocedimen os de diagnós ico u ilizados pa a ca ego iza a mesma. Também
a aliamos as p incipais di e enças en e ambas as écnicas ci ú gicas
en a izando as an agens e des an agens en e ambas. De olhos pos os no
u u o, os au o es ambém es abelecem o que conside am se o p imei o
modelo expe imen al pa a Pec us Exca a um assim como e e em pela
p imei a ez uma possí el no a abo dagem ci ú gica pa a a co ecção des a
de o midade.
3
The Su gical App oach o Pec us Exca a um: P esen and
Fu u e insigh s
José Miguel A. Loba o
Facul y o Medicine o he Uni e si y o Opo o
Al. P o . He nâni Mon ei o 4200 – 319 Po o, Po ugal
[email p o ec ed]
Add ess o he co esponding au ho :
José Miguel A. Loba o
Facul y o Medicine o he Uni e si y o Opo o
Al. P o . He nâni Mon ei o 4200 – 319 Po o, Po ugal
[email p o ec ed]
4
Abs ac
Pec us Exca a um is a p e alen pa hology in he pedia ic age g oup, i can
a lic up o 1 in 400 indi iduals and lead o pulmona and ca diac dys unc ion.
This disease appea s o ha e a gene ic o igin, causing an excessi e g ow h o
ca ilaginous s e nal s uc u e. The gene esponsible o his pheno ype is no
ye known. This pa hology is commonly cha ac e ized by diminished exe cise
capaci y as well as a lowe sel -es eem wi h la e consequences in he child’s
no mal social de elopmen .
A su gical app oach can minimize he psycho-social de elopmen al and
physiological dis ess caused by his disease. The mos equen ly used su gical
app oaches a e he Ra i ch and Nuss p ocedu es. The Ra i ch p ocedu e
consis s o an open ches su ge y wi h exposi ion local co ec ion o he
cos ochond al de ec s. The Nuss p ocedu e is minimally in asi e and eso s o
a p e- o med ba ha is placed inside he ho ax using ib oscopy.
In his a icle, he au ho s summa ize wha is cu en ly employed o su gically
and non-su gically co ec his pa hology, as well as diagnos ic p ocedu es
u ilized o ca ego ize he disease. We also assess he main di e ences be ween
bo h su gical echniques emphasizing hei ad an ages and disad an ages
ega ding each o he . Looking owa ds he u u e, he au ho s also es ablish
wha hey conside o be he i s expe imen al model o pec us exca a um as
well as e e ing o he i s ime a pu a i e new app oach o he su gical
co ec ion o his illness.
Key wo ds: Pec us Exca a um; Expe imen al model; Ra i ch p ocedu e; Nuss
P ocedu e
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In oduc ion
Pec us Exca a um (PE) is a majo congeni al anomaly which a ec s mos ly
men o e women on a 5:1 a io and occu s in 1:400 childbi hs. I consis s o
changes in he de elopmen o he cos al a ches and he s e num which cause
s e nal dep ession, o en comp essing medias inic s uc u es and usually
in ol ing he lowe s e num and cos al ca ilages. I is p esen a bi h bu mos
equen ly mani es s i sel du ing ea ly childhood, he se e i y o he de o mi y is
usually exace ba ed when pube y ends. Ca diac and pulmona y comp essions
a e esponsible o physiologic and s uc u al changes. [1-3]
Psychosocial issues can a ise om his pa hology and i s exace ba ion occu s
in he adolescen .
Some su gical p ocedu es ha e been success ully applied o he co ec ion o
his pa hology. The cu en end is Nuss p ocedu e ha has an ob ious
es he ical ad an age compa ed o Ra i ch p ocedu e.
The pu pose o his s udy is o e iew he main ea men op ions o PE, ocus
on he ad an ages and disad an ages, as well as p opose an expe imen al
p o ocol o an al e na i e app oach o his pa hology.
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Conclusion
Cu en ly he e a e wo well documen ed and well es ablished su gical
app oaches o PE. Bo h he Ra i ch and he Nuss p ocedu e ha e hei
ad an ages and disad an ages. I he decision is on he pa ien ’s side, Nuss
p ocedu e is p e e ed p obably because o he inal aes he ic esul s wi hou an
an e io ches sca .
O he al e na i es such as Vacuum bell appea o be limi ed in applicabili y
whils he magne ic mini-mo e is jus now showing i s i s esul s unde going
clinical ials.
P oposal o an expe imen al p o ocol
Pec us Exca a um is an in e es ing a ea o esea ch. The e is no well-
es ablished animal model. The e is a mouse s ain o Ma an disease ha
ca ies PE and he wo k o Haje e al which documen a su gical animal model
o PE. [31]
- Expe imen al design
Young Wis a a s will be weaned a 21 days o age. A he 30 h day o li e hey
will be anes he ized and subjec ed o a midline longi udinal incision in he
s e num wi h di ision o pec o al muscles and subsequen exposi ion o he
cos al and s e nal ca ilages. The an e io po ion o he s e nal g ow h pla e will
be des oyed by elec ocoagula ion (Haje e al). The incision will be closed wi h
su u es h ough he pec o al muscles and skin.
The con ol g oup will consis o animals wi hou any su gical p ocedu e.
A 90 days o age he su i ing a s will be adiog aphically analyzed o
compa e he leng h and cu a u e o he s e num. I is expec ed ha he G oup
submi ed o su gical p ocedu e will ha e acqui ed some o m o Pec us
de o mi y.
The animals wi h induced PE will be submi ed o he placemen o o hodon ic
he molabile wi es ac oss 4 adjoining ibs and i s con ala e al coun e pa s. The

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ixa ion o hese wi es will eso o mic oimplan s ha will a ach he me al
a ches o he osseous s uc u es. One implan will be placed on he cos al angle
on he ib in i s an e io su ace and ano he one on he con ala e al ib in he
exac same place. The o he wo implan s which sus ain each a ch will be
ixa ed o he s e num. The a ch o each se o con ala e al ibs will hus be
ixa ed by 4 implan s. A Sham g oup will unde go he exac same su gical
p ocedu e bu he a ches will no be a ached o he bone and will emain
subcu aneously unde he pec o al muscles.
Radiog aphic e alua ion will be assessed a 120 and 150 days o age in bo h
g oups and he animals will be eu hanized and a ge o assessmen bo h by
imaging and his ologically a 180 days o age.
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15
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25. Ha ison, M.R., e al., Magne ic Mini-Mo e P ocedu e o pec us exca a um: I.
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2007. 42(1): p. 81-5; discussion 85-6.
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6(2)(ABR/JUN).
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concisely abou he majo esul s o he s udy.
Acknowledgemen s and g an in o ma ion: Financial suppo should be s a ed.
Re e ences should be limi ed o impo an ecen publica ions. Only hose
a icles ci ed in he ex may be included in he e e ence lis . The e e ences
a e indica ed in he ex , ables and illus a ions by A abic numbe s in squa e
b acke s. The lis o e e ences is placed a he end o he a icle and lis s he
e e ences in o de o hei appea ance in he ex . Fo a icles wi h mo e han 3
au ho s he e m “e al.” is placed a e he hi d au ho .
A icles in jou nals:
1 Me zelde ML, Kuble J, Pe e sen C e al. Lapa oscopic neph ou e e ec omy
in child en: a p ospec i e s udy on Ligasu e e sus Clip/Liga ion. Eu J Pedia
Su g 2006; 16: 241–244
2 Wedel T, Ta azzoli K, Sollne S e al. Mi ochond ial myopa hy (complex I
de iciency) associa ed wi h ch onic in es inal pseudo-obs uc ion. Eu J Pedia
Su g 2003; 13: 201–205