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CME
Developmental eld reassignment in unilateral cleft lip:
Reconstruction of the premaxilla
Michael H. Carstens
I
Division of Plastic Surgery, Saint Louis University, USA
Address for correspondence: Michael H Carstens, Division of Plastic Surgery, Saint Louis University, USA. E-mail: michaelcarstens@mac.com
n t he 21
st
cent ur y t he gr eat est st i mul us f o r p r o gr ess i d ent i f i ab l e devel opment al fi el ds. Fi el d s ar e d i scr et e uni t s
i n cl ef t sur ger y w i l l co me f r o m mo r e a mo r e accur at e co mp o sed o f ect o d er m, meso d er m, end o d er m and neur al
mo d el o f f aci al d evel o p ment and ho w cl ef t s o r i gi nat e. cr est . At each l evel o f t he embr yo al l ger m l ayer s speci f i c t o
Vi ct o r Veau
[ 1]
accur at el y p r ed i ct ed t hi s: Al l cl ef t sur ger y t hat l evel can b e r ef er enced b ack t o d evel o p ment al zo nes
i s m e r e l y ap p l i e d e m b r y o l o g y. T h e r e v o l u t i o n i n o f t he neur axi s k no w n as a neuromeres. These ar e named
d evel o p ment al bi o l o gy has no t yet been i nco r p o r at ed i nt o usi ng t he no mencl at ur e o f neur o emb r yo l o gy. The mast er
sur gi cal p r act i ce. The d r aw i ngs and t er mi no l o gy used i n p l an o f t he ent i r e emb r yo i s d et er mi ned b y 6 prosomeres
t ext b o o k s t o d ay ar e b ased o n t he w o r k o f W i l hel m Hi s i n (p r o sencep hal o n-f o r ebr ai n), 2 mesomeres (mesencep hal o n-
t he 1870 s.
[ 2]
Cl ef t r ep ai r s ar e t her ef o r e d esi gned based o n mi d br ai n), 12 r hombomeres (r ho mbo encep hal o n-hi nd br ai n
t he anat o my as i t ap p ear s i n t he new b o r n. M easur ement s and 31 myel omeres (sp i nal co r d ).
[ 5-7]
ar e t ak en and t he t i ssues ar e geo met r i cal l y mani p ul at ed .
But t he anat o my o f a cl ef t as seen at t he t i me o f bi r t h i s f ar RUBENSTEIN
d i f f er ent f r o m i t s o r i gi nal co nf i gur at i o n i n t he emb r yo ni c
f ace. Fr o m i t s o nset at gast r ul at i o n, t he cl ef t i ng event Ap p l i cat i o ns o f neur o mer i c anat o my p r o vi d e a p o t ent i al
unl eashes p at ho l o gi c p r o cesses t hat p r ed i ct ab l y al t er t he em b r y o n i c m ap o f al l cr an i o f aci al st r u ct u r es w i t h
o r i gi nal emb r yo ni c anat o my o ver t i me t o p r o d uce w hat i mpo r t ant i mpl i cat i o ns f o r di agno si s and sur ger y. Excl usi ve
w e r eco gni ze at t er m as a cl ef t l i p . Lef t unco r r ect ed , t hese o f t h e cr an i al b ase (b asi sp h en o i d an d p o st er i o r ) an d
p r o cesses w i l l r emai n o p er at i ve t hr o ugho ut f aci al gr o w t h. p ar i et al bo ne, t he cr ani o f aci al skel et o n i s mad e excl usi vel y
Th i s exp l ai n s w h y geo met r i c cl ef t r ep ai r s r el ap se o ver f r o m neur al cr est . Thus t he cel l p o p ul at i o ns p r o d uci ng t he
t i me, r eq ui r i ng r evi si o n. et hmo i d , p r esp heno i d , p r emaxi l l a and vo mer al l o r i gi nat e
i n ant er o -p o st er i o r o r d er f r o m t he neur al f o l d s i n genet i c
Devel o p m en t al f i el d r ep ai r (DFR) i s b ased u p o n t h e
n eu r o m er i c m o d el o f cr an i o f aci al em b r yo l o gy.
[ 3 -4 ]
Th e
go al s o f DFR sur ger y ar e: (1) r eso l ut i o n o f al l p at ho l o gi c
p r o cesses o f cl ef t i ng (d ef i ci ency, d i sp l acement , d i vi si o n
and d i st o r t i o n); (2) d i ssect i o n al o ng embr yo ni c sep ar at i o n
p l anes (sub p er i o st eal r el ease); (3) p r eser vat i o n o f b l o o d
su p p l y t o t h e al veo l ar m u co p er i o st eu m ; (4 ) p r i m ar y
uni f i cat i o n o f t he d ent al ar ch; and (5) r eassi gnment o f al l
d evel o p ment al f i el d s t o t hei r co r r ect r el at i o nshi p s. Bef o r e
d escr i b i ng t he sur gi cal t echni q ue, cer t ai n b asi c co ncep t s
o f f i el d t heo r y sho ul d b e und er st o o d .
Cr ani o f aci al anat o my r esul t s f r o m t he assembl y o f sp eci f i c,
r egi st er w i t h t he 1
st
r ho mb o mer e (ab b r evi at ed r 1). The
i n f er i o r t u r b i n at e, p al at i n e b o n e, m ax i l l a, al i sp h en o i d
(gr eat er w i ng) and zygo ma ar i se f r o m t he neur al cr est
o f t h e 2
n d
r h o m b o m er e (r 2). Th e sq u am o u s t em p o r al ,
mand i b l e, mal l eus and i ncus ar e r 3 neur al cr est b o nes.
No n-neur al cr est cr ani o f aci al b o nes co me f r o m p ar axi al
meso d er m (PAM ) l yi ng i mmed i at el y ad j acent t o t he neur al
t u b e. PAM i s d i vi d ed i n t o i n d i vi d u al u n i t s sh ap ed l i k e
p o p co r n bal l s cal l ed somi t omeres, each o ne i n r egi st er w i t h
i t s co r r espo ndi ng neur o mer e. The f i r st seven so mi t o mer es
(Sm) co nt ai n t he myo b l ast s f o r al l muscl es o f t he o r b i t
and t he f i r st t hr ee p har yngeal ar ches. Fo r examp l e, t he
Indian J Plast Surg January-June 2007 Vol 40 Issue 1 75
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Carstens
m an d i b l e co m es f r o m r 3 n eu r al cr est an d al l m u scl es
o r i gi nat i ng f r o m i t ar i se f r o m Sm3. Begi nni ng w i t h Sm8 al l
somi t omer es under go a f ur t her t r ansf or mat i on i nt o somit es,
each havi ng a d er mat o me, myo t o me and scl er o t o me. The
f i r st f o ur so mi t es (d er i ved f r o m Sm8-Sm11) p r o d uce t he
cr ani al b ase p o st er i o r t o t he sp heno i d , t he muscl es o f t he
t o ngue and p ar t o f t he st er no cl ei d o mast o i d and t r ap ezi us.
These ar e cal l ed occi pi t al somi t es.
Di st ur b ances at a p ar t i cul ar neur o mer i c l evel can af f ect
p r esent s t he r eco nst r uct i ve ap p l i cat i o n o f t hese p r i nci p l es
t o t he sur ger y o f l ab i o maxi l l ar y cl ef t s.
The pathologic anatomy of cleft formation
Th e p at h o l o g i c an at o m y o f u n i l at er al an d b i l at er al
l abi o maxi l l ar y cl ef t s st ems f r o m a t i ssue def i ci ency st at e
l o cal i zed t o t he l o w er l at er al pi r i f o r m f o ssa. The t i ssue at
f aul t i s t he mesenchyme of t he i psi l at er al pr emaxi l l a. Neur al
cr est st em cel l s r esponsi bl e f or synt hesi s of t he pr esphenoi d
and et hmo i d ar i se f r o m t he mesencephal i c neur al f o l ds and
i n d i vi d u al o r mu l t i p l e f i el d s t o b e d ef i ci en t o r ab sen t . ar e genet i cal l y i dent i f i ed w i t h t he 1
st
r hombomer e (r 1). Not e
Th u s, i sol at ed cl ef t pal at e (u n asso ci at ed w i t h cl ef t l i p ) t hat t he basi spheno i d i s no t neur al cr est i n o r i gi n; i t co mes
r ep r esent s a d ef i ci ency st at e o f t he vo mer. Thi s o ccur s as f r om par axi al mesoder m f r om somi t omer e 1 (Sm1). Sm1 l i es
a sp ect r um. As t he vo mer i s p r o gr essi vel y smal l er, i t l i f t s j ust out si de t he neur al t ube at l evel r 1 and i s i n r egi st er w i t h
aw ay f r o m t he p l ane o f t he p al at al shel ves and t he cl ef t i t . I mmedi at el y caudal t o t hi s po pul at i o n ar e neur al cr est
ext en d s f o r w ar d t o w ar d t h e i n ci si ve f o r am en . I n m i l d cel l s i mmedi at el y abo ve t he r o st r al r ho mbo encephal o n i n
cases o f cl ef t p al at e asso ci at ed w i t h Pi er re Robi n sequence, r egi st er w i t h t he 2
nd
r ho mbo mer e (r 2). The mo st r o st r al
a r ed uct i o n i n t he ho r i zo nt al p l at e o f t he r 2 p al at i ne b o ne zo ne o f r 2 (her ei n r ef er r ed t o as r 2) i s t he l i kel y so ur ce
i s seen . So f t p al at e m u scl es ar e co n seq u en t l y n o r m al mat er i al f o r t he vo mer and PM x. The mo r e caudal zo ne o f
b ut d i vi d ed . As t he p at ho l o gy w o r sens, r ed uct i o n i n t he r 2 pr oduces i nf er i or t ur bi nat e (IT), pal at i ne bone (P), maxi l l a
ho r i zo nt al p l at e o f t he r 2 maxi l l a cr eat es t he w el l -k no w n (M x), al i spheno i d (AS) and zygo ma (Z).
ho r se-sho e p al at e cl ef t . Submucous cl ef t pal at e, o n t he
o t her hand , i nvo l ves p at ho l o gy i n t he 3
r d
p har yngeal ar ch. T h e se c e l l p o p u l at i o n s m i g r at e f o r w ar d i n t o t h e
So mi t o mer es 6 and 7 co nt ai n t he myo b l ast s o f l evat o r, d evel o p i n g f ace i n a st r i ct t em p o r o -sp at i al o r d er. Th e
uvul us, p al at o p har yngeus and sup er i o r co nst r i ct o r. These sp heno i d i s l ai d d o w n f i r st , f o l l o w ed b y t he et hmo i d . I n
can be gl o bal l y af f ect ed . Fr equent l y, p er si st ent VPI f o l l o w s l i k e manner, f o r mat i o n o f PM x i s t he p r er eq ui si t e f o r t he
a seemi ngl y si mp l e p al at o p l ast y, r equi r i ng f ur t her sur ger y. ap p ear ance o f V. Fo r mat i o n o f t he PM x and V r eq ui r es t he
Fai l ur e t o st r at i f y cl ef t pal at e by embr yol ogi c mechani sm p r e-exi st ence o f t he p er p end i cul ar p l at e o f t he et hmo i d
expl ai ns much of t he confusi on cur rent l y ext ent i n t he speech (PPE). Th e f u n ct i o n o f t h e PPE i s t o p r o vi d e a cel l u l ar
and surgi cal l i t er at ure. scaf f o l d b y w h i ch r 2 n eu r al cr est cel l s can r each t h e
mi d l i ne.
[ 10]
[ Fi gur es 1-2] I n ho l o p r o sencep hal y (HPE) t he
Fi n al l y. Tr eacher -Col l i ns syndr ome p r o vi d es an ex am p l e PPE can b e ab sent . PM x and V canno t d evel o p co r r ect l y;
i n w hi ch al l r 2 d evel o p ment al f i el d s o f t he mi d f ace ar e a w i d e b i l at er al cl ef t r esul t s.
[ 11-13]
af f ect ed : t h e m ax i l l ar y, p al at i n e an d z ygo m at i c b o n es
ar e al l smal l . The sep t um, vo mer and p r emaxi l l a (b ei ng The p i r i f o r m f o ssa o f humans and so me hi gh p r i mat es
r 1 st r uct ur es) ar e unaf f ect ed . Fo r t hi s r easo n, t he cent r al i s assemb l ed as t he f usi o n o f t he f r o nt al p r o cess o f t he
mi d f ace p r o j ect s no r mal l y w hi l e t he d i mensi o ns o f t he p r emaxi l l a (PM xF) and t he f r o nt al p r o cess o f t he maxi l l a
p al at e, maxi l l a and zygo ma ar e co nst r i ct ed .
Devel o p ment al f i el d s f o r m i n a sp eci f i c sp at i o -t emp o r al
se q u e n c e . Eac h o n e b u i l d s u p o n i t s p r e d e c e sso r s.
M ak i ng a f ace i s much ak i n t o assemb l i ng a ho use w i t h
magi cal p i eces o f Lego , each o ne o f w hi ch w i l l gr o w
o ver t i me. I magi ne a Lego ho use mad e f r o m 20 p i eces (4
o n t he f l o o r and 5 st o r i es hi gh). Al l p i eces ar e gr o w i ng
i nd ep end ent l y. I f a co r ner st o ne p i ece i s r emo ved , t he 19
r emai ni ng p i eces und er go a d ef o r mat i o n and t he ho use
t i l t s i nt o t he d ef i ci ency si t e. The mi ssi ng Lego pi ece i n cl ef t
l i p i s t he premaxi l l a. The p hysi o l o gi c b asi s o f DFR i s t he
r eco nst r uct i o n o f t he p r emaxi l l ar y f i el d .
[ 8-10]
Thi s chap t er
(M xF). I n al l o t her ver t eb r at e sk ul l s PM xF and M xF ar e
r ead i l y vi si b l e as t w o d i st i n ct en t i t i es.
[ 1 2 ]
Evo l u t i o n
f o r esho r t ened t he human sno ut . The t w o f i el d s b ecame
sup er i mp o sed , PMxF becomi ng t el escoped i nt er nal t o MxF.
Th i s l am i n at i o n i s r esp o n si b l e f o r t h e st r en gt h o f t h e
p i r i f o r m r i m. Pl at i ng o f t he p i r i f o r m b ut t r ess i n f r act ur e
r ep ai r s t ak es ad vant age o f t hi s b i co r t i cal anat o my.
PIRIFORM
The devel opment al f i el d i n w hi ch t he PMx r esi des consi st s
o f an ep i t hel i um and a mesenchyme. Fo r mat i o n o f t he
PMx r esul t s f r om i nt er act i ons bet w een t hese t i ssues. The
Indian J Plast Surg January-June 2007 Vol 40 Issue 1 76
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Developmental eld reassignment in unilateral cleft lip
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.2007 Carstens
2007 Walters
2007 Carstens
2007 Walters
Figure 1: Developmental eld map of the face. All neuromeres are color coded. Neural crest cells forming ethmoid, presphenoid, vomer
and premaxilla share common embryologic origin from 1
st
rhombomere (violet). Maxilla, zygoma, alisphenoid come from 2
nd
rhombomere
(blue). Nostril sill is union of r1 and r2. All craniofacial sutures contain neural crest cells. Craniofacial elds are neuroembryologic and follow
suture boundaries
pr emaxi l l a has sever al anat omi c subcomponent s, t hese ar e epi t hel i um cor respondi ng t o PmxA and t hen spi l l s over i nt o
assembl ed i n a st r i ct sequence.
[14-15]
In dent al t er mi nol ogy zone PMxB. The t i me sequence of dent al er upt i on (cent r al
t he cent r al i nci so r i s cal l ed A and t he l at er al i nci so r i s i nci sor A > l at er al i nci sor B) i s a mani f est at i on of t he r el at i ve
cal l ed B. A er upt s bef or e B. Ther ef or e t he cent r al i nci sor bi ol ogi c mat ur i t y of t he mesenchymal f i el d w i t hi n w hi ch
f i el d (PMxA) i s bi ol ogi cal l y ol der t han t he l at er al i nci sor each t oot h devel ops. The f r ont al pr ocess f i el d (PMxF) i s a
f i el d (PM x B). Neu r al cr est m esen ch ym e f l o w s f o r w ar d ver t i cal of f shoot of PMxB; t hi s subf i el d i s t he bi ol ogi cal l y
al ong t he pr evi ousl y-est abl i shed PPE. It f i r st encount er s t he new est t i ssue.
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Pat ho l o gy af f ect i ng t he PM x o ccur s as a sp ect r um b ased
o n t h i s o r i g i n al d evel o p m en t al p at t er n . A d ef i ci en cy
st at e o f t he PM x w i l l f i r st o ccur i n t he mo st d i st al asp ect
o f t he f r o nt al p r o cess (i e. at i t s mo st cr ani al ext ent ). As
t h e m esen ch ym al d ef i ci t w o r sen s, f r o n t al p r o cess w i l l
b e r ed uced i n a cr ani al -caud al gr ad i ent . Sco o p i ng o ut
o f t h e p i r i f o r m r i m r esu l t s; t h e n asal l i n i n g i s p u l l ed
d o w n as w el l . Th i s cau ses d ep r essi o n o f t h e al ar b ase
and a d o w nw ar d -l at er al d i sp l acement o f t he l at er al cr us.
Bi o l o gi c si gn al s f r o m PM xF r egu l at e ep i t h el i al st ab i l i t y
meso d er m f r o m t he 2
nd
so mi t o mer e f o r ms t he ant er i o r
hal f o f t he sq uamo us t emp o r al b o ne and t he cr ani al hal f
o f t he p ar o t i d gl and . Al l d er i vat i ves f r o m l evel r 2 can b e
map p ed o ut w i t hi n t he senso r y d i st r i b ut i o n o f V2, t he
nucl eus o f w hi ch r esi d es w i t hi n r 2.
[ 19-24]
Th e d evel o p m en t al an at o m y o f t h e n o se, p r o l ab i u m
and p r emaxi l l a has b een p r evi o usl y d escr i b ed i n t er ms
o f neur o mer i c t heo r y b y t hi s aut ho r.
[ 3-4,8,9]
I n co nt r ast t o
t he r est o f t he b o d y, al l der mi s and submucosa of t he head
and t her ef o r e af f ect l i p f o r mat i o n (vi d e i nf r a). When t he or i gi nat es from neur al crest cel l s, no t f r o m a d er mat o me
si gnal st r engt h i s mi ni mal l y d i st ur b ed t he l i p i s no r mal asso ci at ed w i t h a so mi t e.
[ 25]
Pr e-d er mal neur al cr est ar i ses
d esp i t e t he p i r i f o r m d i st o r t i o n. Ther ef o r e t he for me fr ust e f r o m t h r ee d i st i n ct z o n es o f t h e em b r yo . Th e d er m i s
mani f est at i o n o f p r emaxi l l ar y d ef i ci ency i s a cl ef t l i p no se o f t he f o r ehead , no se and vest i b ul ar l i ni ng co me f r o m
w i t h a p er f ect l y no r mal l i p .
[ 16]
t h e cau d al p r o sen cep h al o n (ab o ve p r o so m er es p 4-p 1).
Thi s p r o sencep hal i c neur al cr est (PNC) mi gr at es f o r w ar d
On ce t h e f r o n t al p r o cess i s el i m i n at ed , t h e d ef i ci en cy l i k e a gi gant i c gl aci er t o o ccup y t he neur al f o l d s ab o ve
st at e sh o w s u p i n t h e l at er al i n ci so r f i el d . Pr o gr essi ve p r o so mer es p 6 and p 5. The al ar hal f o f p 6 and p 5 cr eat es
d egr ees o f PM x d ef i ci en cy i n t h e l at er al i n ci so r f i el d t he t el encep hal o n (cer eb r um). The b asal hal ves o f p 6 and
cause i ncr ement al l o ss o f al veo l ar b o ne. No r mal al veo l ar p 5 p l us al l r emai ni ng p r o so mer es (p 4-p 1) synt hesi ze t he
d evel o p ment f o l l o w s a gr ad i ent . I t b egi ns at t he i nci si ve d i encep hal o n (ep i t hal amus, t hal amus and hyp o t hal amus).
f o r amen and p r o gr esses f o r w ar d . M i l d d ef i ci ency causes Th e n eu r al f o l d s ab o ve p 6 an d p 5 ar e st er i l e. Th ey
no t chi ng o n t he l ab i al sur f ace. As t he d ef i ci ency w o r sens co nt ai n t he p i t ui t ar y, o l f act o r y and o p t i c p l aco d es, b ut
t he no t ch d eep ens backward t o w ar d t he i nci si ve f o r amen. no neur al cr est . When PNC f l o w s f o r w ar d i nt o t hi s zo ne
A cr i t i cal l ack o f al veo l ar b o ne mass r esul t s i n o ut r i ght t he p l aco d es ar e act i vat ed and d er mi s i s f o r med . Nasal
f ai l ur e o f l at er al i nci so r d evel o p ment . vest i b ul ar l i ni ng f r o m t he cr i b r i f o r m p l at e f o r w ar d t o t he
i nt er nal nasal val ve co mes f r o m p 6 PNC. Al l r emai ni ng
BMP-4 si gnal s f r o m t hi s f i el d ar e d i r ect l y i mp l i cat ed i n t he f r o nt o nasal d er mi s f r o m t he i nt er nal nasal val ve f o r w ar d
mechani sm o f f usi o n b et w een t he l at er al l i p el ement and t o t he hai r l i ne co mes f r o m p 5 PNC.
t he p r o l ab i um.
[ 17-18]
BM P-4 emanat i ng f r o m PmxL f o r ms
a cr ani o -caud al chemi cal gr ad i ent . The st r engt h o f t hi s PNC sk i n shar es senso r y i nner vat i o n w i t h t he d ur a o f t he
gr ad i ent d ep end s up o n t he t o t al amo unt o f avai l abl e BM P- und er l yi ng f r o nt al l o b e. V1, t he senso r y ner ve f o r t hi s
4; t hi s i n t ur n i s p r o p o r t i o nal t o t he o ver al l mesenchymal co mmo n zo ne has i t s nucl eus w i t h t he 1
st
r ho mb o mer e
mass o f PM xB. Red uct i o n i n mass o f t he l at er al i nci so r (r 1). The neur o anat o my i s anal o go us f o r t he r est o f t he
f i el d r esul t s i n a d i mi nut i o n o f t he t o t al BM P-4 si gnal . Li p f ace. Rho mb o mer es r 2 and r 3 co nt ai n neur o ns sup p l yi ng
f usi o n f o l l o w s t hi s same gr ad i ent . M i l d w eak ness o f t he al l sk i n and d ur a i nner vat ed b y V2 and V3. Thi s i s t he
BM P-4 gr ad i ent w i l l r esul t i n no t chi ng o f t he ver mi l i o n. As embr yo l o gi c basi s f o r t he t r eat ment o f mi gr ai ne headaches
t he si t uat i o n w o r sens t he ext ent o f t he l i p cl ef t w o r sens
i n a cr an i al d i r ect i o n . Th e cl i n i cal sp ect r u m o f t h e so -
cal l ed mi ni mal cl ef t l i p d ef o r mi t y f ai t hf ul l y r ep r o d uces
t hi s b i o l o gi c seq uence.
[ 16]
I n summat i o n, var i at i o ns i n cl ef t s i nvo l vi ng t he p r i mar y
p al at e an d t h e l i p can b e u n d er st o o d as i n t er act i o n s
b et w een d eep p l ane f i el d s o f t he p r emaxi l l a, t he maxi l l a
and sup er f i ci al p l ane f i el d o f t he l at er al l i p el ement . The
mesenchyme o f t he l i p has a d i f f er ent embr yo l o gi c o r i gi n.
I t i s gen et i cal l y i d en t i f i ed w i t h t h e 2
n d
r h o m b o m er e.
Neu r al cr est cel l s f r o m r 2 p r o vi d e t h e d er m i s an d t h e
su b cu t an eo u s t i ssu e o f t h e al ar b ase, w h i l e p ar ax i al
w i t h Bo t ox
(bup i vi cai ne
hyd r o chl o r i d e). Ap p r oxi mat el y 3-5 cc p er si d e i s suf f i ci ent
(maxi mum d o se f o r 0.25%b up i vi cai ne b ei ng 1 cc/k g). At
t he end o f t he case, t he cent r al b l o ck i s r ei nf o r ced b y
b l o ck i ng t he i nf r ao r b i t al ner ves w i t h b up i vi cai ne, 1-2 cc
per si de. The chi l d r et ur ns t o r eco ver y pai n-f r ee. The i ni t i al
b l o ck ensur es t hat sub st ance P (a cr i t i cal med i at o r i n t he
p ai n cascad e r el eased i n r esp o nse t o sur gi cal t r auma) w i l l
no t b e p r o d uced . I n t he ab sence o f sub st ance P t he ent i r e
p o st o p p ai n r esp o nse i s al t er ed .
The sur gi cal f i el d s o f t he uni l at er al cl ef t ar e d ef i ned as
f o l l o w s. FI GURE (I n n eu r o m er i c t er m s: A = p 6 (r ed ) +
p 5 (t ur q uo i se), B = r 2 (p al e go l d ), C i s r 2 (yel l o w ) and D
i s r 2 + r 4 (yel l o w + o r ange). The p r o l ab i um i s d i vi d ed
i nt o t w o zo nes. Zo ne A i s t he t r ue p hi l t r um; i t measur es
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Figure 8: NPP-LCC ap has dual blood supply. Behind the surgical tape are:
(1) lateral branches from the ipsilateral anterior ethmoid artery of the columella;
and (2) vessels running along the dorsal surface of the lower lateral cartilage
that anastomose the nasopalatine artery with the lateral nasal artery. In front
of the tape, the nasopalatine (medial sphenopalatine) artery emerges from the
septopremaxillary junction. This tissue, otherwise called the B ap, reconstructs
the nasal oor (roof of the alveolar cleft) and the lateral nasal wall. This
corresponds to the missing premaxillary eld
Figure 9: Dissection of alveolar cleft. Elevation and division of mucoperiosteal
aps provides closure of nasal oor and oral aspect of primary palate. R2
premaxillary aps (violet) are sutured to r2 maxillary aps (yellow), providing
4 walls of the box. The cleft-side sliding sulcus ap closes off the front of the
Figure 10a: Anterior closure of unilateral alveolar cleft using sliding sulcus aps.
On the cleft side two incisions are required. (1) A medial-to-lateral L-shaped
releasing incision (red line) is placed in the gingival sulcus. It runs from the cleft
lateral to the buttress. There, it ascends vertically to the zygomatico-maxillary
junction. The subperiosteal ap is elevated widely up to the infraorbital foramen.
Because the periosteum is an intact sheet, the ap is stiff, being tethered to the
orbital rim. (2) A counter-incision (green line) is begun at the level of the nasal
oor and brought transversely from the piriform to the buttress. This releases the
lower (alveolar) mucoperiosteal ap. It freely translates two tooth units medially.
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This closes the anterior wall of the alveolar cleft
Figure 10b: Bilateral sliding sulcus aps are illustrated. Although I started out
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UCL Double Layer Periosteoplasty: Step 3
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box. Backwall closure occurs when vomerine mucoperiosteum is elevated and
sutured to the palatal shelf MxP. Septum is reduced and secured to midline. Cleft
site box lled with osteoinductive cytokine rhBMP-2 or autogenous bone graft.
This reconstructs the missing premaxillary Lego
piece
t he w i d t h o f t he co l umel l a. I t co nt ai ns p ai r ed ant er i o r
et hmo i d ar t er i es (t he t er mi nal b r anches o f t he i nt er nal
car o t i d syst em ) an d p ai r ed t er m i n al b r an ch es o f V1 .
Thus zo ne A co nt ai ns t w o neur o vascul ar d evel o p ment al
f i el d s. Zo ne B o f t he p r o l ab i um i s al l t i ssue l at er al t o t he
phi l t r um: t he no n-phi l t r al pr o bi um (NPP). The naso pal at i ne
(med i al sp heno p al at i ne) ar t er y, t he t er mi nal b r anch f r o m
t he i nt er nal maxi l l ar y syst em, sup p l i es t he NPP. Bi l at er al
cl ef t s have t w o NPP zo nes. The cl ef t -si d e al ar b ase i s zo ne
C. The l at er al l i p el ement b el o w t he al ar gr o o ve i s zo ne
with this technique, I no longer do a gingival release on the non-cleft side.
A one-sided release is sufcient. On the non-cleft side, wide subperiosteal
mobilization is combined with a full-thickness vertical releasing incision up
the buttress. This creates a huge, mobile bipedicle ap. The entire soft tissue
complex becomes centralized without tension. Nota bene: Surgeons working
under extreme condition (very wide clefts in remote areas with no access to
orthodontic management) bilateral sliding sulcus aps are very effective. They
will close any size defect
D. I t co nt ai ns a sp hi nct er l ayer, t he d eep o r b i cul ar i s o r i s
(DOO) and t he d i l at o r l ayer, t he sup er f i ci al o r b i cul ar i s o r i s
(SOO) muscl es. DOO d evel o p s i n co nj unct i o n w i t h t he o r al
muco sa w hi l e SOO d evel o p s w i t h t he sk i n. A l ayer o f f at
co nveni ent l y sep ar at es t hese t w o l ayer s.
[ 47-48]
Th e t r u e d est i n at i o n o f NPP so f t t i ssu e i s i n t h e n asal
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Developmental eld reassignment in unilateral cleft lip
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Figure 11 (Case1): Bilateral cleft lip with right unilateral cleft of primary palate and bilateral clefts of secondary palate. Soft tissue relationships one year after DFR
demonstrating stability of nasal tip projection. Comparison of CT scans at 3 months and 9 months show bony union of primary palate. Periapical view of alveolar cleft
site shows reconstitution of the periodontal ligament, a neural crest structure. This is not seen with conventional iliac crest graft. Serial 3-D skulls taken 9 months
apart support the Delaire dissection concept: wide subperiosteal undermining repositions the osteogenic soft tissue elds into a correct, centralized relationship.
Subsequent osteosynthesis of membranous bone now occurs in a correct centric distribution. Developmental eld reassignment promotes normal membranous bone
formation after the surgical procedure
f l o o r and t he l at er al nasal w al l . NPP r ep r esent s t he so f t t he l at er al cr us co r r esp o nd s t o t he f r o nt al p r o cess f i el d ,
t i ssu e en vel o p e co r r esp o n d i n g t o t h e l at er al z o n e o f PM xF. Lat er al w al l so f t t i ssue co r r esp o nd s t o t he f r o nt al
t h e p r em ax i l l a, PM x B + PM x F. Nasal f l o o r so f t t i ssu e p r o cess f i el d , PM xF. Nasal f l o o r so f t t i ssue co r r esp o nd s
co r r esp o nd s t o t he l at er al i nci so r f i el d , PM xB. So f t t i ssue t o t he l at er al i nci so r f i el d , PM xB. The r at i o nal e o f DFR
o f t he l at er al nasal w al l bet w een t he i nf er i o r t ur bi nat e and sur ger y i s t o r eassi gn t he mi spl aced f i el ds o f t he pr emaxi l l a
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i n t o co r r ect p o si t i o n . Wh en t h e NPP f l ap i s co m b i n ed
w i t h t he r ef l ect ed muco p er i o st eum f r o m t he mar gi ns o f
t he b o ny cl ef t a sur gi cal p o ck et i s cr eat ed . Thi s si t e i s
sub seq uent l y f i l l ed w i t h b o ne gr af t o r a b o ne-p r o d uci ng
cyt o k i ne (r hBM P-2) such t hat mi ssi ng p r emaxi l l ar y f i el d s
t i ssu e. Th e cym b a gr af t , i n co m b i n at i o n w i t h t h e NPP
f l ap , p r o vi d es mo r e t h an en o u gh t i ssu e t o r eco n st r u ct
t he mi ssi ng p r emaxi l l a.
M ar k i n gs ar e car r i ed o u t u si n g a m o d i f i cat i o n o f t h e
Figure 12 (Case 2): Post op CT scans of piriform fossa in this challenging cleft demonstrate leveling out of the nasal oor to support the alar base. Consolidation of
the alveolar cleft during primary DFR surgery maintains dimensions of the dental arch without collapse even after palatoplasty. Note symmetry of columellar-alara
relaltionships seen in lateral views
ar e r e-synt hesi zed .
The NPP f l ap f unct i o ns j ust l i k e a b oxt o p t o co ver t he
al veo l ar cl ef t . I t i s al mo st al w ays ad equat e. I am co nvi nced
t hat t he secr et i d ent i t y o f NPP i s t he o r i gi nal PM xB.
Because o f t he cl ef t t hi s t i ssue i s shi p w r eck ed o n t he
p r emaxi l l a. Unf o r t unat el y, NPP i s f r eq uent l y i nsuf f i ci ent
t o r ep l ace PM xF. Ever y t i me t he l at er al cr us i s r el eased
an d ad van ced a so f t t i ssu e d ef ect ap p ear s. Th e d ef ect
r ep r esen t s m i ssi n g PM x F. I n m y cl i n i cal ex p er i en ce,
t hi s i s al mo st al w ays t he si ze o f t he aur i cul ar cymb a. A
co mp o si t e gr af t o f ant er i o r aur i cul ar sk i n and car t i l age
f r o m t he cymb a i s t he mo st r el i ab l e r ep l acement f o r t hi s
M i l l ar d syst em. (Pai r ed ant er i o r et hmo i d neur o vascul ar
b und l es d ef i ne t he t r ue p hi l t r um; t hi s eq ual s t he w i d t h o f
t he co l umel l a.). Fi r st t o b e mar k ed i s p o i nt 2, t he j unct i o n
o f t he no r mal p hi l t r al co l umn w i t h t he w hi t e r o l l . The
w i d t h o f t he co l umel l a at i t s b ase i s t hen measur ed . Thi s
d i st ance (usual l y 6-10 mm) i s sub seq uent l y mar k ed al o ng
t he w hi t e r o l l med i al t o t he p hi l t r al co l umn as p o i nt 3.
Di st ance 2-3 i s t he w i d t h o f t he p hi l t r um; t hi s co nt ai ns
t he t w o ant er i o r et hmo i d ar t er i es (ap p r oxi mat el y 4-6 mm
ap ar t ). The mi d p o i nt o f t he p hi l t r um (p o i nt 1 i n t he M i l l ar d
syst em o r nad i r o f cup i d s b o w ) i s i r r el evant . Po i nt 13,
d ef i ned by t he bul ge o f t he f o o t p l at e o f t he med i al cr us o n
t he no n-cl ef t si d e, mar k s t he sho ul d er o f t he co l umel l a.
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Figure 13 (Case 3): MD Secondary deformities resulting from displacement of tissue from the midline corrected with subperiosteal centralization. Note changes in
the non-cleft alar base after DFR. Residual sag in left alar contour will need cartilage batten graft in the future. The straight-line scar in DFR repair simulates philtral
column.
The co r r esp o nd i ng p o i nt 12 o n t he cl ef t si d e w i l l be f o und t he i nf er i o r t ur bi nat e. Po i nt 9 i s t he t i p o f f l ap D w hi l e po i nt
d i sp l aced caud al l y and i nt er nal l y w i t h r esp ect t o p o i nt 13. 11 i s t he t i p o f t he f ut ur e no st r i l si l l C, so -cal l ed because i t
To t al p hi l t r al hei ght 2-13 sho ul d eq ual 3-12. Po i nt s 4 and i s i n co nt i nui t y w i t h t he al ar base C. The di mensi o ns o f C
10 mar k t he mi d p o i nt o f t he al ar b ases o n no n-cl ef t and can be r o ughl y mapped o ut by measur i ng t he no st r i l si l l o n
cl ef t si d es, r esp ect i vel y. t he no n-cl ef t si de. Thi s i s t he di st ance f r o m t he mi dpo i nt o f
t he no n-cl ef t al ar base (po i nt 4) t o t he i psi l at er al f o o t pl at e
On t he l at er al si de, po i nt s 9 and 11 mar k t he t er mi nus o f po i nt 13. Po i nt 8 mar k s t he nat ur al t r ansi t i o n o f t he w hi t e
sk i n w i t hi n t he nasal cavi t y. Thi s i s l o cat ed j ust ant er i o r t o r o l l . Di st ance 8-9 sho ul d mat ch 3-12.
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Dissection sequence
St ep 1. Lat er al di ssect i on: rescui ng t he nost r i l si l l
The l at er al l i p i s t ensed w i t h a si ngl e ho o k and t he sk i n-
muco sa mar gi n i s i nci sed p r o ceed i ng up w ar d f r o m 8 t o 9,
l o cat ed j ust b el o w and ant er i o r t o t he i nf er i o r t ur b i nat e.
Fr o m h er e t h e i n ci si o n sw i n gs ar o u n d l at er al l y t o 10,
t he mi d p o i nt o f t he al ar b ase, (b ut no t b eyo nd i t at t hi s
p o i nt ). I n t hi s manner, t he l at er al l i p f l ap D i s sep ar at ed
f r o m t h e al ar b ase C. Th i s st ep sep ar at es o r b i cu l ar i s
f r o m t he nasal i s. Fr o m p o i nt 9, a seco nd , more i nt er nal ,
i nci si o n d ef i nes t he t r i angul ar f l ap C . The b ase w i d t h o f
C can b e d ed uced b y measur i ng t he d i mensi o ns o n t he
no n-cl ef t si d e.
Fr o m t he l i p a l at er al ver mi l i o n f l ap (L) i s p ar ed o f f and
d i ssect ed d o w n t o t he al veo l ar cl ef t mar gi n. Pr o p er p ar i ng
o f L i ncl ud es a smal l st r i p o f l i p sk i n 2-3 mm w i d e b ecause
i t i s r o l l ed -i n , w i t h an ab n o r m al r el at i o n sh i p t o t h e
d i sect i o n o f a ver y l o ng f l ap . The B f l ap has t w o p ar t s: (1) a
sk i n/muco sa f l ap o f t he no n-p hi l t r al p hi l t r um (NPP) and (2)
a cho nd r o cut aneo us f l ap co nt ai ni ng t he l at er al co l umel l ar
sk i n and med i al cr us (LCC). B = NPP + LCC
B h as a r i c h b l o o d su p p l y. T h e NPP c o m p o n e n t i s
i r r i gat ed b y t he naso p al at i ne ar t er y. The LCC co mp o nent
get s sup p l y f r o m 2-3 l at er al b r anches o f t he i p si l at er al
ant er i o r et hmo i d . I n ad d i t i o n, vessel s i n co nt i nui t y w i t h
t he l at er al nasal syst em f r o m t he f aci al ar t er y r un al o ng
t he sur f ace o f t he al ar car t i l age i t sel f. Once el evat ed , B
i s sur p r i si ngl y l o ng, r eachi ng al l t he w ay acr o ss t he cl ef t
t o t he l at er al nasal r i m. [ Fi gur e B] i s i nset i nt o t he gap
cr eat ed b y ad vancement o f t he p 5 l at er al vest i b ul ar l i ni ng
(and t he l at er al cr us). Thi s achi eves t w o i mp o r t ant sur gi cal
go al s: (1) cep hal i c ad vancement o f t he med i al cr us w i t h
i ncr eased t i p p r o j ect i o n; and (2) so f t t i ssue augment at i o n
o f t h e l at er al n asal w al l . I n set o f B p er m i t s r el ease o f
t he t et her ed l at er al vest i b ul ar l i ni ng. Rep o si t i o ni ng t he
l at er al cr us no w o ccur s i n w hat w o ul d o t her w i se b e a
Y-V p at t er n wi t hout t he Y-V cl osure. Nat ur al al ar car t i l age
anat o my i s acco mp l i shed w i t ho ut p i nchi ng t o get her an
al r ead y-d ef i ci ent l i ni ng.
The f ut ur e cl ef t -si d ed p hi l t r al co l umn i s d et er mi ned b y
u n d er st an d i n g t h e em b r yo l o gy o f t h e p r o l ab i u m . Th e
t r ue p hi l t r um l i es b et w een p o i nt s 2 and 3 and co nsi st s
Figure 13a: Vissiarnarov ap harvested from scar in case 3 simulates non-philtral
philtrum and lls lateral piriform defect created by advancement of the lateral crus.
Note large pedicle from the external carotid-based nasopalatine artery. These
aps can be harvested in virtually all secondary cleft reconstructions
l ayer o f f at . The caud al mar gi n o f t he t w o muscl es d ef i nes
t he w hi t e r o l l and co nt ai ns t he l ab i al ar t er y.
[ 47]
St ep 2 . Medi al di ssect i on: t he NPP-l at er al col umel l ar
advancement fl ap
The med i al mar gi n o f t he cl ef t (zo ne B i n o ur d i agr am)
co nsi st s o f p r o l ab i al t i ssue l at er al t o t he t r ue p hi l t r um.
Thi s i s i n co nt i nui t y w i t h l at er al co l umel l ar sk i n and t he
med i al cr us o f t he al ar car t i l age. Co nveni ent l y, t he b l o o d
su p p l y o f t h ese t w o u n i t s i s a w at er sh ed p er m i t t i n g
und er l yi ng muscl e. I f t he sur geo n d o es no t d o t hi s, t he
sk i n w i l l p uck er i nw ar d at t he f i nal cl o sur e. The L f l ap i s
t he mo st o p t i o nal t i ssue o f t he ent i r e DFR o p er at i o n. I t i s
b est b r o ught med i al l y and i nt er p o sed b et w een t he nasal
vest i b ul ar l i ni ng and t he B f l ap . I t can al so b e used as a
f r ee gr af t t o t he l at er al nasal w al l . No t i ssue i s sacr i f i ced
i n DFR.
W i t h i t s ver mi l i o n st r i p p ed o f f, t he l at er al l i p mar gi n i s
n o w en t er ed an d t h e o r b i cu l ar i s i s sp l i t i n t o i t s d eep
(co n st r i ct o r ) an d su p er f i ci al (d i l at o r ) co m p o n en t s. Th e
deep or bi cul ar i s or i s (DOO) i s shap ed l i k e t he l et t er J and
sep ar at ed f r o m t he super fi ci al or bi cul ar i s or i s (SOO) b y a
o f t w o f i el d s (each sup p l i ed b y a sep ar at e b r anch o f t he
ant er i o r et hmo i d ar t er y). The p hi l t r al d er mi s co mes f r o m
p 5 p r o sencep hal i c neur al cr est and i s i nner vat ed b y V1.
The w i d t h o f t he p hi l t r um i s r o ughl y eq ual t o t hat o f t he
co l u m el l a, gen er al l y m easu r i n g 6-10 m m . Becau se t h e
p hi l t r um d evel o p s o n t o p o f t he r 2 p r emaxi l l a, i t r ecei ves
ad d i t i o nal b l o o d sup p l y f r o m t he t er mi nal b r anches o f
t he sp heno p al at i ne ar t er y (SPA) emer gi ng at t he l evel o f
t he sep t o p r emaxi l l ar y j unct i o n. Thus, t he phi l t r um has a
dual bl ood suppl y. Al l r emai ni ng p r o l ab i al sk i n and muco sa
(t he NPP) i s an r 2 d er i vat i ve, sup p l i ed b y t he naso p al at i ne
ar t er y and i nner vat ed b y V2. The p r o l ab i um o f a b i l at er al
cl ef t t hus co nsi st s o f f o ur d evel o p ment al uni t s b ased o n
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Figure 14 (Case 4): Developmental eld reassignment of soft tissue elds in bilateral lip/palate cleft repair demonstrates normalization of Cupids bow. Contrary to
current dogma the philtrum and columella are not short in the cleft condition; they are mismatched. By recognizing and reconstructing the missing premaxillary eld,
DFR corrects deciency-induced eld mismatch
emb r yo l o gy, b l o o d sup p l y and i nner vat i o n: t w o cent r al sept um. Just beneat h t he l at er al col umel l ar pi l l ar s l i e t he
p hi l t r al A f i el d s and t w o l at er al B f i el d s. medi al cr ur a of t he l ow er l at er al car t i l ages (LLC). Because
t hese pi l l ar s ser ve as t he bi ol ogi c t empl at e f or t he car t i l age
The embr yol ogy of t he col umel l a i s as f ol l ow s. The ant er i or t o f or m, di ssect i on of sk i n aw ay f r om t he cr ur a i s ext r emel y
cent r al l y-l ocat ed ski n comes f rom p5 and cont ai ns t he pai red di f f i cul t . The LLCs ar e t hus p5 neur al cr est der i vat i ves. The
ant er i o r et hmo i d ar t er i es. On ei t her si de o f t hat cent r al upper l at er al car t i l ages l i e above t he p6 vest i bul ar l i ni ng and
sw at ch, t he col umel l ar sk i n ext ends back w ar ds t ow ar d t he ar e t her ef or e of p6 neur al cr est or i gi n.
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Carstens
Co r r ect i o n o f t he cl ef t l i p no se r eq ui r es r el easi ng t he al ar
car t i l age f r o m t w o p o i nt s o f ent r ap ment .
[ 49]
The d ef i ci ency
st at e o f t he p r emaxi l l a causes a mechani cal d ef o r mat i o n
o f p er f ect l y no r mal p 5 sk i n o n b o t h si d es o f t he nasal
i nt r o i t us. I n t he l at er al nasal w al l , t he r 2 sk i n o ver l yi ng t he
p r emaxi l l ar y f r o nt al p r o cess (PM xF) i s r ed uced o r ab sent .
Co nseq uent l y, p 5 sk i n co nt ai ni ng t he l at er al cr us o f t he
al ar car t i l age i s d r agged d o w n i nt o t hi s si nk ho l e l o cat ed
j ust i n f r o nt o f t he i nf er i o r t ur b i nat e. The l at er al cr us i s
t her ef o r e f l at t ened . On t he med i al si d e, t he d ef i ci ency
The NPP-LCC f l ap no w r esemb l es a l o ng b o o t , shap ed l i k e
I t al y. The t o e o f t he b o o t ext end s al o ng t he p r o l ab i al
mar gi n. Beneat h t he heel o f t he b o o t l i es t he f o o t p l at e
o f t he al ar car t i l age. Thi s l and mar k co r r esp o nd s t o p o i nt
12. Gr asp i ng t he heel o f B p r o vi d es i nst ant access t o t he
m ed i al asp ect o f t h e m ed i al cr u s. Th i s i s a saf e p l an e
p er mi t t i ng d i ssect i o n o f t he al ar car t i l age r i ght i nt o t he
nasal t i p , w i t h t he f o l l o w i ng caveat . The B f l ap get s b l o o d
su p p l y f r o m t h e n aso p al at i n e ar t er y vi a 2 -3 b r an ch es
emer gi ng at t he j unct i o n o f t he p r emaxi l l a and vo mer.
o f t he r 2 p r emaxi l l ar y l at er al i nci so r f i el d (PmxB) cr eat es Gent l e sp r ead i ng al o ng t he med i al b o r d er o f t he car t i l age
a si n k h o l e i n t o w h i ch t h e p 5 l at er al co l u m el l ar sk i n w i l l r eveal t hese b r anches and p r eser ve t hem. Ad d i t i o nal
i s d i sp l aced . Thi s d i sp l aces t he med i al cr us o f t he al ar b l o o d sup p l y d escend s al o ng t he sk i n. Fo r mer l y I w o ul d
car t i l age d o w nw ar d and i nw ar d co mp ar ed w i t h t he no n- el evat e t hese NPP-LCC f l ap s co mp l et el y, never havi ng an
i ssue w i t h i schemi a. No w ho w ever, I t hi nk i t p r ud ent t o
p r eser ve t he naso p al at i ne b r anches w hen p o ssi b l e. Thi s
The LCC-NPP f l ap i s el evat ed as f o l l o w s. Under t ensi o n, t he t yp e o f bl unt d i ssect i o n i s mo r e t han suf f i ci ent t o ad vance
NPP sk i n i s el evat ed i n cont i nui t y w i t h t he l at er al col umel l ar t he nasal t i p .
w al l . The l at er al (i nt er nal ) i nci si o n i s made f i r st , separ at i ng
t h e sk i n f r o m t h e m u co sa al l t h e w ay t o t h e j u n ct i o n Th e sep t u m i s n o w d i ssect ed o u t an d f r eed f r o m t h e
bet w een t he pr emaxi l l a and vo mer. Al t ho ugh t hi s i nci si o n maxi l l ar y cr est unt i l i t si t s p assi vel y i n t he mi d l i ne. As
w i l l event ual l y be car r i ed up i n f r o nt o f t he sept um, i t i s gr o w t h p r o ceed s t he cent r al i zed sep t um w i l l no l o nger
advi sabl e t o st o p her e. Tensi o n can t hus be mai nt ai ned o n co nst i t ut e an ab no r mal f o r ce vect o r t et her i ng t he nasal
t he NPP w hi l e i t i s separ at ed f r o m t he phi l t r um pr o per. t i p .
Next , a seco nd i nci si o n, p ar al l el t o t he f i r st , ascend s f r o m St ep 3. Nasal di ssect i on: open-cl osed r hi nopl ast y
p o i n t 3 t o t h e b ase o f t h e co l u m el l a. I t t h en ext en d s At t hi s j unct ur e, a st and ar d i nf r acar t i l ageno us i nci si o n i s
i nt o t he no se al o ng t he l at er al si d ew al l o f t he co l umel l a. mad e. Thi s i nci si o n i s b r o ught al l t he w ay t o t he p i r i f o r m
Pr evi o u sl y I w o u l d co n t i n u e t h i s i n ci si o n al o n g t h e r i m, f o l l o w i ng t he nat ur al f o l d b et w een t he nasal sk i n
si d e o f t he co l umel l a (j ust ant er i o r t o t he ed ge o f t he and t he vest i b ul ar sk i n. The caud al ext ent o f t hi s i nci si o n
med i al cr us) and t hence b r i ng i t d i r ect l y i nt o t he no se t er mi nat es at t he i nt er nal b o r d er o f t he t r i angul ar no st r i l
as an i n f r acar t i l ag en o u s i n ci si o n . Th i s d esi g n m ad e si l l f l ap B . The exp o sur e gai ned vi a t he l at er al co l umel l a-
m e co n cer n ed ab o u t p o ssi b l e scar co n t r act u r e. At t h e i nf r acar t i l agi no us i nci si o n al l o w s a co mp l et e d i ssect i o n o f
suggest i o n o f Dr. Jo hn Rei ni sch, I b egan t o b r eak up t he t he d o r sal nasal sk i n envel o p e as d escr i bed by M cCo mb.
[ 50]
i nci si o n by el evat i ng a medi al l y-based r ect angul ar sk i n f l ap Th e su ccess o f t h e M cCo m b d i ssect i o n i s r eal l y an
o ccup yi ng t he caud al hal f o f t he co l umel l a. Just b eneat h em b r yo n i c f i el d sep ar at i o n . Th e d eep l ayer co m p r i ses
t he Rei ni sch f l ap l i es t he f o o t p l at e o f t he l at er al nasal t he p 6 vest i b ul ar ep i t hel i um and p 6 neur al cr est up p er
cl ef t si d e.
car t i l age. At i t s cep hal i c mar gi n, t he i nci si o n i s r esumed
al o ng l at er al co l umel l a. At t he l evel o f t he i nt er med i at e
cr u s t h e i n ci si o n t r an si t i o n s b en eat h t h e so f t t r i an gl e
i n t o a st an d ar d i n f r acar t i l agen o u s i n ci si o n an d i s t h en
co nt i nued al l t he w ay t o t he p i r i f o r m mar gi n.
The ant er i o r i nci si o n o f t he NPP-LCC f l ap i s no t suf f i ci ent t o
ad vance i t . A seco nd p ar al l el i nci si o n i s r equi r ed . FI GURE I t
st ar t s i n t he memb r ano us sep t um ab o ut hal f w ay up . I t i s
t hen car r i ed d o w nw ar d t o t he vo mer and t hen r e-d i r ect ed
f o r w ar d t o meet t he l at er al mar gi n o f t he B f l ap . No t e
t hat t he memb r ano us sep t al i nci si o n p r o vi d es i mmed i at e
exp o sur e o f t he car t i l agi no us sep t um.
l at er al car t i l ages, t he b l o o d sup p l y t o w hi ch co mes f r o m
b el o w vi a t he I CA. The sup er f i ci al l ayer i s p 5 nasal sk i n
and t he l o w er l at er al nasal car t i l ages, t he b l o o d sup p l y t o
w hi ch i s al so o f I CA d er i vat i o n. I nt er p o sed b et w een t hese
t w o l ayer s, l i k e a sand w i ch, i s t he SM AS l ayer o f f aci al
muscl es d er i ved f r o m t he 2
nd
p har yngeal ar ch (r 4-r 5). The
myo bl ast s co me f r o m so mi t o mer e 4. The nasal muscl es ar e
co mp ar t ment al i zed b y r 4 neur al cr est f asci a (t he SM AS).
The b l o o d sup p l y t o t hi s i nt er med i at e 2
nd
ar ch l ayer i s
f r o m t he f aci al ar t er y (ECA). Thi s mesenchyme p r o vi d es
an ad d i t i o nal so ur ce o f bl o o d sup p l y t o t he al ar car t i l ages.
Car ef u l d i ssect i o n o f t h e l at er al co l u m el l ar w al l s f r o m
t he co l umel l a d i scl o ses vessel s r unni ng al o ng t he med i al
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Developmental eld reassignment in unilateral cleft lip
sur f ace o f t he med i al cr ur a. These anast o mo se t he l at er al
nasal vessel s w i t h t he naso p al at i ne vessel s.
Al o n g t h e l at er al n asal r i m t h e d i ssect i o n i s car r i ed
r i gh t d o w n t o t h e p i r i f o r m m ar gi n . Th e p r o p er p l an e
f o r sep ar at i o n i s achi eved b y huggi ng t he sur f ace o f t he
car t i l age. The o ver l yi ng r 4 SM AS l ayer and t he p 5 sk i n
l ayer ar e l ef t i n anat o mi c co nt i nui t y. Ver y l i t t l e b l eed i ng i s
o ccasi o ned b y t hi s ap p r o ach. As o ne r eaches t he p i r i f o r m
r i m t h e p er i o st eu m i s i n ci sed an d st r i p p ed ver t i cal l y.
t hese so l ut i o ns mad e use o f an ant er i o r l y-b ased i nf er i o r
t ur bi nat e f l ap as descr i bed by No o r dho f f.
[53-54]
Al t ho ugh t hi s
t i ssue w o r ked ad equat el y, I had sever al r eser vat i o ns abo ut
i t : (1) t he d i ssect i o n i s sub t l e and d i f f i cul t t o t each; (2)
t he t i ssue t yp e i s d i st i nct and no t nat i ve t o t he nasal r i m;
and (3) heal i ng o f t he d o no r si t e can b e acco mp ani ed b y
cr ust i ng and bl eed i ng; and (4) i t d i d no t make embr yo l o gi c
sense. Pr o p er d i ssect i o n and i nset o f f l ap NPP co mb i ned
w i t h use o f a co mp o si t e f o r FTSG mad e t he t ur b i nat e f l ap
unnecessar y. The k ey t o get t i ng t he mo st o ut o f NPP i s t o
Sub p er i o st eal el evat i o n o f t he so f t t i ssues l at er al t o t he i ncl ud e t he p r o l ab i al ver mi l i o n w i t h t he sk i n.
p i r i f o r m r i m p r eser ves t he f aci al ar t er y ar cad e. The nasal
sk i n envel o p e i s t hen l i b er at ed cep hal i cal l y al l t he w ay t o St ep 4. Int r aor al di ssect i on: sl i di ng sul cus S fl ap
t he nasal b o nes. The r at i onal e and desi gn of t he sl i di ng sul cus mucoper i ost eal
f l ap st em d i r ect l y f r o m p i o neer i ng w o r k at t he Uni ver si t y
Desp i t e t h ese m an eu ver s, t h e vest i b u l ar l i n i n g i s st i l l o f Pi t t sb ur gh b y So t er eano s.
[ 43]
Thi s t echni q ue i nvo l ves
t i ght ! The med i a cr us has b een co mp l et el y r el eased and a g i n g i val r el ease o n t h e cl ef t si d e car r i ed o u t f r o m
ad van ced i n t o t h e n asal t i p b u t t h e l at er al cr u s b ei n g t h e al veo l ar cl ef t t o t h e b u t t r ess.
[ 42]
Th i s p er mi t s w i d e
r emai ns sp l ayed o ut and t et her ed . Rel easi ng t he l at er al subp er i o st eal d i ssect i o n o ver t he ent i r e f ace o f t he maxi l l a
cr us f r o m t he vest i b ul ar l i ni ng has b een ad vo cat ed i n t he b el o w t he i nf r ao r b i t al f o r amen as d escr i b ed b y Del ai r e.
[ 55-
p ast . Thi s i s t echni cal l y d i f f i cul t b ecause i t vi o l at es t he
56]
A 45 d egr ee b ack cut up t he b ut t r ess i s p er f o r med . The
embr yo l o gy (r ecal l t hat t he al ar car t i l age ar i ses as a neur al at t ached gi ngi val i s r el eased . The f l ap i s co ver ed o n i t s
cr est r esp o nse t o a p at t er n emb ed d ed i n t he vest i b ul ar und er sur f ace by a sheet o f p er i o st eum, r end er i ng i t r at her
ep i t h i l i u m ). Th e si z e an d sh ap e o f t h e cl ef t -si d e al ar st i f f. The Sot ereanos maneuver i s a mo b i l i zat i o n o f t he S
car t i l age has b een d emo nst r at ed t o b e no r mal co mp ar ed f l ap usi ng a co unt er -i nci si o n i n t he p er i o st eal sheet i t sel f
w i t h t he no n-cl ef t si d e.
[ 51]
Co nseq uent l y, t he p 5 vest i b ul ar p ar al l el t o t he gum l i ne. The co unt er -i nci si o n, l o cat ed hal f -
l i ni ng p r o gr am must b e no r mal as w el l . St ud i es at UNC w ay f r o m t he gi ngi val mar gi n t o t he i nf r ao r b i t al f o r amen,
d emo nst r at e t hat t he o ver al l sur f ace ar ea o f t he r ep ai r ed i s mad e b y j ust sco r i ng t hr o ugh p er i o st eum. I t ext end s
cl ef t no st r i l i s r ed uced b y 30%.
[ 52]
The si t e and d i mensi o ns f r o m t he p i r i f o r m mar gi n st r ai ght l at er al t o t he b ut t r ess.
o f t he d ef i ci t co r r esp o nd t o t he so f t t i ssues o f PM xF. At t hi s p o i nt i t j o i ns up w i t h t he p r evi o us b ack cut . These
t w o i nci si o ns mak e a r i ght angl e: t he p er i o st eal co unt er -
The so f t t i ssues o f t he al veo l ar cl ef t PM xB can b e f o und i nci si o n i s t r ansver se acr o ss t he maxi l l a and t he b ut t r ess
o n t he p r o l ab i um. The LCC-NPP f l ap ef f ect i vel y p r o vi d es i nci si o n i s ver t i cal . The co mbi nat i o n o f t hese t w o i nci si o ns
a vascu l ar i z ed r o o f f o r t h e b o n y cl ef t . Lat er al n asal r el eases t he l o w er muco p er i o st eal f l ap S f r o m t he up p er
w al l r el ease unco ver s a d ef ect t hat f r eq uent l y exceed muco p er i o st eum at t ached t o t he o r b i t .
t he r each o f LCC-NPP. The r el ease b egi ns at t he i nf er i o r
t ur b i nat e and co nt i nues al o ng t he j unct i o n o f vest i b ul ar When S i s r el eased , i t ad vances mesi al l y ab o ut t w o t o o t h
and nasal sk i n. At t he ap ex o f t he l at er al cr us i t b eco mes
V-shap ed . Due t o t he p r i o r M cCo mb d i ssect i o n t he l at er al
cr u s i s ad van ced cep h al i cal l y i n t o sym m et r y w i t h t h e
no r mal si d e. The i nt er nal nasal val ves w i l l ap p ear eq ual .
Lef t b eh i n d i s a r aw sp o t r o u gh l y t h e si z e o f t h e ear
cymba. A co mp o si t e gr af t usi ng ant er i o r sk i n and car t i l age
f r o m t he cymb a p r o vi d es a st ur d y r eco nst r uct i o n f o r t he
ai r w ay. The LCC-NPP f l ap , i nset w i t ho ut t ensi o n acr o ss t he
al veo l us, co mes t o r est at t he f o o t o f t he cymb a gr af t .
Exit the turbinate ap
As t h e DFR evo l ved , I em p l o yed var i o u s so l u t i o n s t o
p at ch t he d ef ect cr eat ed b y t he vest i b ul ar r el ease. One o f
uni t s. The mar gi n o f S t hat w as f r eed f r o m t he l at er al
b o r d er o f t he cl ef t no w ext end s acr o ss t he al veo l ar cl ef t
w i t ho ut t ensi o n and i s sut ur ed t o t he muco p er i o st eum o f
t he p r emaxi l l a. I n t hi s w ay, l i k e a sl i d i ng d o o r, t he S f l ap
seal s up t he ant er i o r asp ect o f t he al veo l ar cl ef t .
On t he no ncl ef t si d e, a si mi l ar sub p er i o st eal d i ssect i o n
i s d o ne w i t ho ut r eco ur se t o a gi ngi val r el ease. A l ar ge
b i p ed i cl e f l ap i s cr eat ed , p er mi t t i ng cent r al i zat i o n o f t he
pr evi o usl y l at er al i zed so f t t i ssue envel o pe. No t e t hat w hen
cl ef t l i p o ccur s w i t ho ut an al veo l ar cl ef t , b i l at er al S f l ap s,
el evat ed wi t hout gi ngi val rel ease, p er mi t p r o p er t ensi o n-
f r ee cent r al i zat i o n o f mi d f ace so f t t i ssues.
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Carstens
The S f l ap t hen co mes i n t w o var i et i es, each o f w hi ch has a
speci f i c benef i t f o r t he pat i ent . The si mpl e S f l ap i s el evat ed
i n t he subp er i o st eal p l ane acr o ss t he ent i r e f ace o f t he
maxi l l a bet w een t w o po i nt s o f ver t i cal r el ease: t he al veo l ar
cl ef t and l at er al w al l o f t he but t r ess. I t r emai ns at t ached
t o t he t eet h. A gi ngi val r el easi ng i nci si o n i s no t r equi r ed.
By vi r t ue o f i t s abi l i t y t o cent r al i ze t he so f t t i ssues o f t he
ent i r e mi d f ace, t he si mp l e S f l ap f ul f i l l s t w o i mp o r t ant
f unct i o ns: (1) t he cr eat i o n o f an est het i c so f t t i ssue dr ape ;
and (2) t he bi o l o gi cal l y act i ve o st eo genei c po t ent i al o f t he
muco per i o st eal f l ap el evat ed di r ect l y o f f t he l at er al i nci so r.
The sp ace w i l l f i l l i n ni cel y. I f o ne d esi r es, t he f l ap d o no r
si t e can b e gr af t ed w i t h Al l o d er m
.
Li mi t ed r eso ur ces and d i f f i cul t l o gi st i cs i n eco no mi cal l y
devel o pi ng co unt r i es f o r ce sur geo ns t r eat i ng cl ef t pat i ent s
t o ad o p t d i f f er en t an d cr eat i ve st r at egi es, p ar t i cu l ar l y
w hen t he al veo l ar cl ef t i s w i d e. These chi l d r en o f t en have
no access t o p r e-sur gi cal o r t ho p ed i cs. They may never
r ecei ve o r t ho d o nt i cs. They d o p o ssess a r ead y so ur ce o f
mu co p er i o st eal st em cel l s i s t r an sf er r ed f o r w ar d , t h u s b o ne gr af t f r o m t he r i b . I n such cases, achi evement o f a
enabl i ng bo ne depo si t i o n t o t ake pl ace i n cent r i cal l y f o r co nso l i d at ed ar ch and el i mi nat i o n o f o r o nasal f i st ul a ar e
bet t er f aci al skel et al pr oj ect i on. The dr aw back of t he si mpl e r easo nab l e go al s f o r p r i mar y cl ef t sur ger y. The t w o k ey
S f l ap i s t hat i t canno t pr o duce a muco per i o st eal f l ap t o f act o r s f o r success i n t hese p at i ent s ar e: (1) abi l i t y t o make
co ver o ver t he ant er i o r aspect o f t he al veo l ar cl ef t . I n such a si mp l e acr yl i c sp l i nt and secur e i t --2 mm scr ew s w o r k
cases, a r ect angul ar muco per i o st eal f l ap can be har vest ed per f ect l y w el l ; and (2) met i cul o us di ssect i o n o f t he al veo l ar
i mmedi at el y l at er al t o t he al veo l ar cl ef t and br o ught o ver cl ef t w i t h at t ent i o n t o i t s vascul ar anat o my.
f o r cl o sur e. Thi s desi gn w o r k s f o r nar r o w cl ef t s.
I f a dent al spl i nt can be f abr i cat ed, l i p adhesi o n can nar r o w
Th e co m p o u n d S f l ap (as d escr i b ed b y So t er ean o s) t he cl ef t d o w n t o d i mensi o ns p er mi t t i ng si mul t aneo us
i mp l i es a f ul l gi ngi val r el easi ng i nci si o n al l t he w ay f r o m DFR r ep ai r an d al veo l ar gr af t i n g w i t h a si m p l e S f l ap .
t h e al veo l ar cl ef t b ack t o t h e t u b er o si t y. Th i s i n vo l ves When t hi s i s no t p o ssi b l e, a co mp o und S f l ap w i l l cl o se
ad d i t i o n al o p er at i n g t i m e. Gi n g i val r el ease f r o m an t he gap . I n so me ver y w i d e p r i mar y uni l at er al cl ef t s I have
al v e o l u s co n t ai n i n g e r u p t e d p r i m ar y o r se co n d ar y used bi l at er al co mp o und S f l ap s t o successf ul l y cl o se gap s
d en t i t i o n i s t ech n i cal l y si m p l e. Th e b o n e i s so l i d an d o f 14-16 mm. Of co ur se, t hi s maneuver d i sco nnect s t he
t he sur gi cal p l ane easy t o f o l l o w. I nf ant s ar e a d i f f er ent f r enul um f r o m t he mi d l i ne. Po st o p er at i ve r emo d el i ng o f
mat t er. Pr i o r t o t he 4
t h
mo nt h o f l i f e maxi l l ar y and al veo l ar t he al veo l us r eest ab l i shes symmet r y. I n such cases, ar ch
b o ne i s t o o so f t t o w o r k w i t h. DFR sho ul d b e p er f o r med st ab i l i t y j ust i f i es t he ext ensi ve i nt r ao r al d i ssect i o n.
b et w een ages 4-6 mo nt hs. Di ssect i o n i s f aci l i t at ed usi ng
l o up e magni f i cat i o n, a # 15C o r Beaver bl ad e, an amal gam Seco n d ar y cl ef t r eco n st r u ct i o n f o l l o w s t h e sam e r u l es
p ack er and M o l t (# 9) o r Lo ui svi l l e p er i o st eal el evat o r s. b ut w i t h t he p r o vi so t hat gi ngi val r el ease i nci si o ns ar e
A co m p o u n d S f l ap can b e m o b i l i z ed t w o f u l l t o o t h f ast an d easy t o acco m p l i sh . Th e f l o o r o f t h e al veo l ar
w i d t hs. The mai n ad vant age o f t he co mp o und S f l ap i s r eco nst r uct i o n has al r eady been pr o vi ded by a pr e-exi st ent
t h e d r am at i c i n cr ease i n m u co p er i o st eal f l ap l en gt h i t p al at al r ep ai r. When o r t ho d o nt i c cap ab i l i t y exi st s, such
p r o vi d es. Thi s i s gener al l y t he w i d t h o f t w o d ent al uni t s. cl ef t s sho ul d f i r st b e exp and ed t o t he o r i gi nal w i d t h p r i o r
The ant er i o r asp ect o f t he al veo l ar cl ef t i s t hus co ver ed t o gr af t i ng. But w hen t he p at i ent has no access t o o r t ho ,
w i t h st em cel l -co nt ai ni ng muco per i o st eum. Di sadvant ages S f l ap al veo l ar r eco n st r u ct i o n can b e co m b i n ed w i t h
o f S f l ap r el at e t o t he t emp o r ar y anat o mi c d i sr up t i o n o f
p er i o co r o nal at t achment s.
The S f l ap has d i st i nct i nd i cat i o ns f o r use i n p r i mar y and
seco n d ar y cl ef t r ep ai r. Th ese ar e o f vi t al i m p o r t an ce,
p ar t i cul ar l y w hen o ne must o p er at e und er co nd i t i o ns t hat
ar e l ess t han i d eal . I n a p r i mar y cl ef t , w henever p o ssi b l e,
p r e-su r gi cal o r t h o d o n t i c co n t r o l o f t h e ar ch sh o u l d b e
acco m p l i sh ed . Th i s m ay al so i n vo l ve a l i p ad h esi o n .
Th e en t i r e ef f o r t i s d i r ect ed t o w ar d n o r m al i z i n g ar ch
d i m en si o n s. I n t h ese cases, co m p o u n d S f l ap s ar e n o t
r eq ui r ed . Cl o si ng t he ant er i o r w al l o f t he al veo l ar cl ef t
can b e acco mp l i shed w i t h a r ect angul ar sup er i o r l y-b ased
DFR t o p r o d uce a p er manent , aest het i cal l y p l easi ng and
p hysi o l o gi c r esul t i n o ne st age.
St ep 5. Di ssect i on of t he pr i mar y pal at e
Begi nni ng w i t h t he l at er al nasal w al l , t he muco p er i o st eum
i s el evat ed o f f t he maxi l l ar y al veo l ar b o ne. The p al at al
m ar gi n i s u n d er m i n ed as w el l . Th i s r esu l t s i n a l ar ge
muco p er i o st eal envel o p e. Thi s must b e sep ar at ed i nt o
nasal and o r al co mp o nent s. Thi s i s d o ne at t he l evel o f t he
sho ul d er o f t he al veo l us. The i nci si o n can b e ext end ed
b ack w ar d a f ew mm al o ng t he ed ge o f t he p al at al shel f.
On t he med i al si d e, t he sep t al muco p er i cho nd r i um and
t he vo mer i ne muco p er i o st eum ar e el evat ed i n co nt i nui t y.
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Developmental eld reassignment in unilateral cleft lip
Th e su r g eo n t h en p r o ceed s f o r w ar d t o el evat e t h e
m u co p er i o st eu m o f f t h e p r em axi l l a. Sep ar at i o n o f t h e
envel o p e o ccur s at t he sho ul d er o f t he p r emaxi l l a. A
vo mer muco p er i o st eal f l ap i s el evat ed and sut ur ed t o t he
nasal muco sa o f t he cl ef t maxi l l a.
A si x-si d ed b ox i s cr eat ed . The med i al and l at er al w al l s
ar e r aw b o n e. Th e f l o o r i s o r al m u co p er i o st eu m . Th e
ant er i o r w al l i s t he sl i d i ng sul cus muco p er i o st eum. The
r o o f i s t he nasal muco p er i o st eum + t he NPP f l ap . The
t o t h e GPA. Th i s p er m i t s d o w n w ar d d i ssect i o n o f t h e
muco p er i o st eum f r o m t he sho ul d er o f t he p r emaxi l l a o r
up w ar d d i ssect i o n f r o m t he o r al mar gi n. The p r emaxi l l a
r em ai n s al i ve, b ased o n i t s o t h er so u r ces. I n b i l at er al
p al at e cl ef t s, n o su ch p al at al an ast o m o si s ex i st s. Th e
muco p er i o st eum can o nl y b e r ef l ect ed up w ar d . I f t hi s i s
d o ne o n bo t h si d es and i f t he p r o l abi um i s si mul t aneo usl y
el evat ed , p r emaxi l l ar y necr o si s can ensue.
Thi s p r i nci p l e i s exact l y t he same as i n p r o l ab i al d eat h
p o st er i o r w al l i s t he vo mer f l ap . These f l ap s ar e l o ad ed r ep o r t ed b y M i l l ar d , a co mb i nat i o n o f b i l at er al r o t at i o n
w i t h und i f f er ent i at ed mesenchymal st em cel l s (M SC). The b ack cu t s (d est r o yi n g t h e an t er i o r et h m o i d su p p l y t o
cambi um l ayer o f t he p er i o st eum i s esp eci al l y r i ch i n st em t h e p h i l t r u m ) an d el evat i o n aw ay f r o m t h e u n d er l yi n g
cel l s. Al l n eu r al cr est M SCs co n t ai n m em b r an e-b o u n d p r emaxi l l a.
[ 55]
I n b i l at er al cl ef t l i p and p al at e, t he anat o my
r ecep t o r s f o r BM P. Thi s p o ck et i s no w r ead y t o b e f i l l ed o f b o t h p r o l ab i u m an d p r em ax i l l a co n si st s o f p ai r ed
w i t h r i b gr af t (i l i ac cr est gr af t i n o l d er chi l d r en) o r w i t h angi o so mes. Di sr up t i o n can o ccur i n o ne o r t he o t her
an r hBM P-2/ACS i mp l ant . w i t h i mp uni t y, b ut no t b o t h.
Al veo l ar r eco n st r u ct i o n , b o t h p r i m ar y an d seco n d ar y, Fo r t unat el y, t he r o b ust b l o o d sup p l y o f t he maxi l l a co mes
d em an d s p r eci se k n o w l ed g e r eg ar d i n g t h e vascu l ar t o o ur r escue. I nj ect i o n st ud i es d emo nst r at e t he maxi l l a
an at o m y o f m u co p er i o st eu m b o r d er i n g t h e al veo l ar t o b e su p p l i ed b y t h e p h ar yn geal b r an ch o f t h e f aci al
cl ef t . Thi s i s a negl ect ed ar ea o f cl ef t sur ger y. The b l o o d ar t er y an d f aci al b r an ch o f t h e ascen d i n g p h ar yn geal
su p p l y t o t h e p r em ax i l l a i s t h e m o st co m p l ex (an d ar t er y.
[ 56]
I t i s i nd ep end ent o f i t s muco p er i o st eal co ver.
p o t ent i al l y t r eacher o us). I t i s d er i ved f r o m t hr ee so ur ces. M et i cul o us d i ssect i o n o f t he l at er al al veo l ar cl ef t cr eat es
(1) Pr emaxi l l a i s an r 2 d er i vat i ve. Li k e al l mesenchymal a co n t i n u o u s m u co p er i o st eal sh eet u n i t i n g t h e l at er al
st r uct ur es caud al t o r 1, p r emaxi l l a i s sup p l i ed f r o m t he nasal w al l w i t h t hat o f t he har d p al at e. Thi s co nt ai ns a
ex t er n al car o t i d . Th e vascu l ar ax i s o f t h e vo m er an d m i r r o r -i m age ex t er n al car o t i d an ast o m o si s, t h i s t i m e
p r emaxi l l a i s t h e med i al sp h en o p al at i n e (n aso p al at i n e) b et w een t he med i al sp heno p al at i ne and gr eat er p al at i ne
ar t er y. PM x muco per i o st eum suppl i ed by t he SPA envel o ps ar t er i es. Thi s can b e st r i p p ed d o w nw ar d w i t h i mp uni t y. I t
t he bo ne f r o m abo ve-do w nw ar d, l i ke a cl o ak t hr o w n o ver a i s a l o ng f l ap , ext end i ng f r o m j ust i n f r o nt o f t he i nf er i o r
chai r. (2) Fr o m bel o w, PM x muco p er i o st eum i s co nt i nuo us t ur bi nat e al l t he w ay t o t he har d pal at e. The l at er al al veo l ar
w i t h t h at o f t h e r 2 m ax i l l ar y p al at al sh el f (M x P), t h e muco p er i o st eal f l ap i s o f suf f i ci ent si ze t o cl o se t he ent i r e
ar t er i al axi s o f w hi ch i s t he ext er nal car o t i d -b ased gr eat er f l o o r o f t he al veo l ar cl ef t .
p al at i ne ar t er y. (3) a seco nd anast o mo si s exi st s w i t hi n t he
muco p er i o st eum co ver i ng t he ant er i o r sup er i o r asp ect o f W h at t h en sh o u l d b e d o n e f o r ch i l d r en BCL(P)? M u st
t he p r emaxi l l a. Thi s zo ne i s i n co nt act w i t h t he so f t t i ssues o n e sacr i f i ce ar ch r eco n st r u ct i o n t o f aci al aest h et i cs o r
o f t he p r o l ab i um, a p 5 d er i vat i ve sup p l i ed b y t he i nt er nal vi ce ver sa? Th e an sw er (n o ! ) l i es i n a st ag ed ap p r o ach
car o t i d -b ased ant er i o r et hmo i d ar t er i es.
Pr em ax i l l ar y su r vi val d ep en d s u p o n m ai n t en an ce o f
suf f i ci ent co nt act bet w een t he bo ne and i t s envel o p i ng
muco per i o st eum. Al veo l ar cl ef t r eco nst r uct i o n canno t be
accompl i shed w i t hout st r i ppi ng aw ay t he vascul ar cover age
o f PM x, t o a gr eat er o r l esser degr ee. Thus, pr emaxi l l ar y
vascu l ar an at o m y d i ct at es t h e d i r ect i o n i n w h i ch t h e
muco per i o st eum must be el evat ed. Sur gi cal st r at egy f o r
t hi s maneuver i s a di r ect co nsequence o f t he cl ef t t ype.
I n uni l at er al p al at e cl ef t s, t he med i al al veo l ar cl ef t mar gi n
i s a co n t i n u o u s zo n e o f ext er n al car o t i d f r o m t h e SPA
w i t h car ef u l d i ssect i o n . Pr i m ar y l i p r eco n st r u ct i o n m u st
n o t vi o l at e t h e t i ssu e p l an e sep ar at i n g t h e p r o l ab i u m
an d m u co p er i o st eu m . Even i n a b i l at er al d i ssect i o n ,
5 0 % o f t h e p r em ax i l l a w i l l r em ai n p er f u sed b y b o t h SPA
an d AEA vessel s. Lat er al n asal w al l r eco n st r u ct i o n w i t h
t h e cym b a co m p o si t e g r af t i s ap p r o p r i at e, Li b er at i o n
o f t h e m ed i al cr u r a m u st b e d o n e w i t h o u t en t er i n g t h e
t er r i t o r y o f t h e m ed i al sp h en o p al at i n e ar t er y. I f t h i s
ap p ear s d i f f i cu l t , n asal el evat i o n an d l i p r evi si o n m ay
h ave t o b e st ag ed l at er. Al veo l ar cl ef t cl o su r e sh o u l d
b e d o n e b y el evat i n g t h e p r em ax i l l ar y m u co p er i o st eal
f l ap u p w ar d an d t h e m ax i l l ar y m u co p er i o st eal f l ap
d o w n w ar d .
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Carstens
W i d e b i l at er al cl ef t s r eq ui r e al t er nat i ve st r at egi es. Tw o
p r i n ci p l es t o avo i d t r o u b l e i n t h ese p at i en t s ar e: (1 )
m et i cu l o u s p r i m ar y cl o su r e o f t h e n asal f l o o r ; an d (2)
d el ayed gr af t i n g o f t h e al veo l ar cl ef t . A w i d e al veo l ar
r o o f d ef ect r equi r es bi g nasal f l o o r f l ap s. These ar e best
har vest ed at t he p r i mar y sur ger y, b ef o r e o t her t i ssues get
i n t he w ay. The k ey p o i nt her e i s t o no t cut t he l at er al
m u co p er i o st eal f l ap u n t i l t h e m ed i al f l ap i s h ar vest ed
f r o m t he p r emaxi l l a. Once t he d i mensi o ns o f med i al f l ap
ar e k n o w n , a su p er i o r l y-b ased l at er al f l ap o f su f f i ci en t
The t i p i s p o si t i o ned anat o mi cal l y usi ng t he Cr o ni n nasal
r et r act o r (Pad get t I n st r u m en t s). Th e m ed i al cr u r a ar e
b at t ened t o get her w i t h 5-0 PDS. Sut ur e susp ensi o n and
mo d i f i cat i o n o f t he al ar car t i l age can b e r ead i l y execut ed
by means o f t he o pen-cl o sed appr o ach as per t he sur geo ns
p r ef er en ce. Th e au t h o r s ap p r o ach i s p r ed i cat ed o n
t h e est ab l i sh m en t o f n o r m al f i el d r el at i o n sh i p s. W i d e
d i ssect i o n o f t he nasal so f t t i ssues co mb i ned w i t h r el ease
f r o m t hei r p i r i f o r m p r i so n al l o w s al l f i el d s t o be p assi vel y
hel d i n p o si t i o n by a nasal st ent i nser t ed at t he co ncl usi o n
si ze i s el evat ed t o co mp l et e t he ant er i o r nasal cl o sur e. o f t he p r o ced ur e. The aut ho r f i nd s t he Ko k en-t yp e si l i co n
At t hi s p o i nt w e ar e st i l l l ef t w i t h a w i d e al veo l ar f l o o r st ent s (Po r ex Co r p , New man, Geo r gi a) easy t o use. The
d ef ect and no t eno ugh t i ssue t o cl o se i t . What d o es o ne st ent i s p l aced at t he end o f t he sur ger y. Cl o sur e o f t he
d o ? One w ai t s i t o ut , usi ng a p al at al acr yl i c sp l i nt (secur ed no st r i l i nci si o n st ar t s at t he i nt er med i at e cr us w o r k i ng
w i t h p i ns o r 2 mm scr ew s) t o mai nt ai n t he t r ansver se ar ch med i al l y d o w n t o t he Rei ni sch f l ap . One t hen p r o ceed s
d i mensi o ns and p r event co l l ap se. l at er al l y t o t h e m ar gi n o f t h e l at er al n asal w al l . Th i s
i nvo l ves p l aci ng 2-3 sut ur es o ver 5 mm.
6 mo nt hs l at er, at pal at o pl ast y, t he l o ng muco per i o st eal
f l aps el evat ed f r o m t he har d pal at e pr o vi ded i deal co ver age The r emai nd er o f t he l at er al nasal i nci si o n w i l l b e f i l l ed b y
f o r t he al veo l ar cl ef t f l o o r. Bi l at er al co mpo und S f l aps ar e i nset o f t he B f l ap . Reco nst r uct i o n o f t he f r o nt al p r o cess
r ai sed. These w i l l adequat el y seal o f f t he ant er i o r f ace o f o f t he p r emaxi l l a ad d s t he mi ssi ng t i ssue t o t he l at er al
t he al veo l ar cl ef t . The gr af t (usi ng r hBMP-2 o r r i b) i s pl aced. nasal w al l (see b el o w ). Thi s f r eq uent l y i nvo l ves p l acement
On ce agai n , car e m u st b e t ak en t o p r even t t r an sver se o f a co mp o si t e f o r f ul l t hi ck ness gr af t i nt o t he d ef ect . The
co l l ap se u n t i l t h e b o n e gr af t h as h ar d en ed . I n t r ao r al med i al cr ur al co mp l ex i s t hen el evat ed w i t h r esp ect t o t he
spl i nt i ng i s t hus co nt i nued f o r ano t her 2-3 mo nt hs. sep t um w i t h 4-0 vi cr yl . Thi s al so cl o ses t he memb r ano us
sep t um co unt er -i nci si o n.
Seco n d ar y gr af t i n g i n b i l at er al cases f o l l o w s t h e sam e
p r i nci p l es. The vascul ar anat o my may have b een al t er ed St ep 2. Sof t t i ssue reconst r uct i on of t he premaxi l l a
(p r i m ar y su r g er y m ay h av e el ev at ed t h e p r o l ab i u m The r o o f o f t he mi ssi ng p r emaxi l l ar y f i el d i s r eco nst r uct ed
aw ay f r o m t he p r emaxi l l a). I n such cases so me d egr ee b ased o n met i cul o us cl o sur e o f t he nasal f l o o r. Thi s i s
o f r e v asc u l ar i z at i o n t o t h e p r e m ax i l l a o c c u r s o v e r car r i ed o u t u si n g a m o u t h gag, st ar t i n g an t er i o r l y at
t i m e . No n e t h e l e ss, d i sse c t i o n o f t h e p r e m ax i l l ar y t h e i n ci si ve f o r am en . Becau se t h e sp ace i s t i gh t u si n g
m u co p er i o st eu m m u st b e sp ar i n g. Al l t h at i s r eq u i r ed 5-0 Vi cr yl
b o ne
gr af t (Memphi s, Tennessee Sof amor -Danek ). The i mpl ant i s
pr epar ed by soak i ng a Hel i st at
t r i cal ci um phosphat e i s usef ul f or bul k i ng up t he cep hal i c ed ge t hr o ugh t he b ase o f t he co l umel l a and
t he i mpl ant at i on si t e. It act s passi vel y, a space-occupyi ng t hen b ack d o w n t o t he SOO as a mat t r ess sut ur e.
agent . One coul d use f r eeze-dr i ed bone as w el l (t he i deal
bul ki ng agent does not yet exi st .) The Mast ergr af t i s w r apped St ep 5 Fi nal adj ust ment s: fi nessi ng t he nost r i l fl oor
w i t h t he ACS col l agen sponge l i ke a f aj i t a or sushi r ol l and Af t er cl o su r e o f t h e l i p , t h e al ar b ase C i s, at t i m e,
pl aced i nt o t he def ect . I l i ke t o pl ace a ver t i cal component co mp r essed med i al l y by t he mo vement o f t he nei ghbo r i ng
i nt o t he al veol ar w al l s and a hor i zont al component bel ow D f l ap (l at er al l i p el em en t ). I f so , t h e al ar b ase m u st
t he nasal f l oor. The pocket cr eat ed by DFR di ssect i on can be b e t r an sl o cat ed l at er al l y. Th i s can b e acco m p l i sh ed b y
f i l l ed w i t h i l i ac cr est gr af t as w el l . exci si o n o f a cr escent o f sk i n f r o m t he l at er al l i p el ement .
C i s t hen el evat ed and secur ed t o t he l at er al vi a a b ur i ed
St ep 4. Cl osure of t he l i p: Del ai re concept s modi fi ed 5-0 PDS sut ur e. At t i mes i t i s necessar y t o el evat e t he
M any year s ago pi o neer i ng w o r k by Del ai r e demo nst r at ed al a co mp l et el y i n o r d er o b t ai n suf f i ci ent l y l at er al i zat i o n.
t hat w i de subper i ost eal di ssect i on yi el d si gni f i cant aest het i c p The t i p o f t he no st r i l si l l f l ap C i s no w sut ur ed j ust
benef i t s o f t i ssue dr api ng and, at t he same t i me, di d no t p o st er i o r t o t he co l umel l ar sho ul d er. Co nt i nui t y b et w een
i mpai r maxi l l ar y gr o w t h.
[57-58]
I ndeed, w hen co mpar ed w i t h t h e co l u m el l ar sh o u l d er an d t h e n o st r i l si l l i s n o w
supr aper i o st eal r el ease, t hi s appr o ach i s devel o pment al l y r eest ab l i sh ed . Per i al ar su t u r i n g w i t h i n ver t ed 5-0 PDS
co r r ect b ecau se i t sep ar at es t h e o st eo gen i c f u n ct i o n al sut ur es r est o r es t he al ar cr ease ver y ni cel y.
mat r i x (t he per i o st eum and o ver l yi ng so f t t i ssues) f r o m t he
pr o duct (t he pr emaxi l l a and maxi l l a). A f i nal caveat co ncer ns t he l i ni ng o f t he l at er al nasal def ect .
Exp er i ence w i t h t hi s t echni q ue sho w s t hat p r event i o n o f
The sut ur e seq uence i s as f o l l o w s:
Del ai re 1: Cent r al i zat i o n o f t he bi l at er al l y d i sp l aced so f t
t i ssues i s accompl i shed w i t h 4-0 nyl on sut ur es pl aced f r om
bel ow t he f l ap t o t he par anasal i s (l evat or ) i nser t i on i nt o t he
super f i ci al or bi cul ar i s or i s (SOO). Thi s i nser t i on does not
i nvol ve t he al ar base. It i s gener al l y l ocat ed one f i nger breadt h
l at er al t o t he al ar base. The non-cl ef t si de i s sut ur ed f i r st
t o bel ow t he ant er i or nasal spi ne. Thi s sut ur e ser ves as a
r ef er ence poi nt f or a si mi l ar sut ur e f r om t he cl ef t si de.
Suspensi on of t he DOO: Thi s sut ur e set s t he d ep t h o f t he
sul cus o n t he cl ef t si d e. At t he mo st cep hal i c mar gi n t he
DOO i s susp end ed f r o m t he sep t um w i t h a 4-0 PDS.
p o st o p er at i ve co nt r act i o n i s p ar amo unt . Thi s r eq ui r es
p l acem en t o f a Po r ex
si l i co n co n f o r m er si z ed t o t h e
p at i ent . The st ent sho ul d b e sut ur ed i n p l ace w i t h 4-0
nyl o n f o r 8 w eek s. I t i s i mp o r t ant t hat t he B f l ap no t b e
sut ur ed t o t he si d ew al l o f C w i t h any t ensi o n. I f t ensi o n
exi st s I have f o und i t p r ud ent t o use a smal l p i nch gr af t
o f r et r o aur i cul ar sk i n.
DISCUSSION
Whenever a sur gi cal p r o ced ur e f o r a co ngeni t al co nd i t i o n
i n a gr o w i ng chi l d l ead s t o a p r ed i ct abl e p at t er n o f r el ap se
o ver t i me t w o , i nescap abl e co ncl usi o ns must be d r aw n: (1)
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Carstens
t he b i o l o gi c r at i o nal e o f t he p r o ced ur e i s i nco r r ect ; and
(2) t he anat o mi c p at t er n o f r el ap se p o i nt s an accusat o r y
f i nger at t he p at ho l o gy. DFR sur ger y r ep r esent s a new
f o r m o f t h i n k i n g ab o u t cl ef t s b y i d en t i f i cat i o n an d
r ear r an g em en t o f sp eci f i c d evel o p m en t al f i el d s. Th e
p r i mar y p at ho l o gy o f cl ef t l i p i s hyp o p l asi a o r ab sence o f
t he d i st al p r emaxi l l a. DFR i s d esi gned t o r eco nst r uct t he
mi ssi ng p r emaxi l l a usi ng o st eo i nd uct i ve t echno l o gi es f o r
st em cel l co ncent r at i o n and d i f f er ent i at i o n such as r hBM P-
2 o r b y co nvent i o nal o st eo co nd uct i ve t echni q ue (b o ne
Th e m em b r an o u s b o n es o f t h e m axi l l a an d p r em axi l l a
ar e j u st a p r o d u ct o f t h e so f t t i ssu e f u n ct i o n al m at r i x
t hat sur r o unds t hem. As t he so f t t i ssue gr o w s, new bo ne
i s d ep o si t ed an d o l d b o n e r eso r b ed acco r d i n g t o t h e
mechani cal f o r ces p l aced o n t he bo ne. The p ur p o se o f
subper i ost eal cent r al i zat i on i n DFR i s t o change t he bi ol ogi c
r el at i o nshi p o f t he bo ne pr o duct t o t he f unct i o nal mat r i x.
Over t i me, t he f o r mer w i l l adapt t o t he l at t er i n i t s new,
cent r al i zed p o si t i o n. Ap p r eci at i o n o f t he sub p er i o st eal
pl ane as t he co r r ect appr o ach t o sur gi cal l y separ at e t he
gr af t i ng). DFR i nci si o ns ar e mad e o n t he b asi s o f vascul ar f unct i o nal mat r i x f r o m i t s pr o duct (bo ne) ensur es t ensi o n-
su p p l y an d em b r yo l o gy, n o t geo m et r i c m an i p u l at i o n . f r ee r el ease an d accu r acy o f m u scl e r ep ai r (i n d i vi d u al
Fo r t hi s r easo n t he d esi gn o f DFR p r esent s a ser i es o f muscl e uni t s can be i dent i f i ed by t uggi ng o n t hem f r o m
sp eci f i c so l ut i o ns t hat sp eak t o p r o b l ems i n cl ef t sur ger y bel o w ). Thi r t y year s o f w o r k by Del ai r e and o t her s co nf i r m
hi t her t o i nad eq uat el y ad d r essed : d i st o r t i o n o f t he nasal t he cl i ni cal accur acy and saf et y o f t hi s appr o ach. Reader s
envel o p e and sep t um, d i sp l acement o f so f t t i ssues aw ay ar e al so advi sed t o st udy t he pi o neer i ng w o r k o n pr i mar y
f r o m t he mi d l i ne, t he ent r ap p ed p o si t i o n o f t he med i al bo ne gr af t i ng do ne by Ro senst ei n.
[59-60]
Thi s 30 year f o l l o w -
cr us and t he hi d d en no st r i l si l l . DFR t r eat s mechani sms, up o f dent al devel o pment i n pat i ent s t r eat ed w i t h car ef ul
no t ap p ear ance. p r esur gi cal o r t ho d o nt i cs and r i b gr af t i ng d emo nst r at es
cl ear l y t he saf et y and advant ages o f gai ni ng co nt r o l o ver
The ext r ao r al d esi gn o f DFR i s i nvar i abl e; i t i s t he same f o r
t he al veo l ar cl ef t at pr i mar y sur ger y.
b o t h uni l at er al and b i l at er al cl ef t s. Devel o p ment al f i el d s
ar e r eco gn i zed , sep ar at ed an d r ear r an ged i n t o co r r ect
The co ncep t s and t echni q ues o f DFR ar e ap p l i cab l e t o al l
anat o mi c r el at i o nshi p s. The i nt r ao r al d esi gn var i es w i t h
f o r ms o f cl ef t s. Because i t separ at es o ut o sseo us pat ho l o gy
cl ef t t yp e. I f no al veo l ar cl ef t i s p r esent , sub p er i o st eal
f r o m so f t t i ssue p at ho l o gy, DFR i s a t r ue cut as yo u go
r el ease an d z ygo m at i co m ax i l l ar y b u t t r ess b ack cu t ar e
t echni q ue. I n seco nd ar y cl ef t sur ger y, DFR i s cap ab l e o f
su f f i ci en t t o ach i eve cen t r al i z at i o n o f t h e so f t t i ssu e
r escu i n g p r evi o u sl y vi o l at ed f i el d s an d r eu n i t i n g t h em
envel o p e. Gi ngi val r el ease i s no t necessar y. I f a p r i mar y
i nt o t hei r co r r ect f unct i o nal r el at i o nshi p s. Sl i d i ng sul cus
p al at e cl ef t exi st s, a co mp l et e sl i d i ng sul cus d i ssect i o n
f l ap s f unct i o n as el ast i c f l ap s as d ef i ned b y Go l d st ei n
[ 61-62]
w i t h gi ngi val r el ease w i l l al l o w f o r mesi al t r ansl o cat i o n
and can r ead i l y b e b r o ught i nt o t he mi d l i ne. Ab b e f l ap s
and co ver age o f t he al veo l ar cl ef t .
ar e vi r t ual l y unnecessar y, even i n sal vage cases i nvo l vi ng
t o t al l o ss o f t he p hi l t r um.
D FR i n cl u d e s a st r ai g h t - l i n e r e p ai r, r e su l t i n g i n an
anat o mi cal l y co r r ect p hi l t r al co l umn. The d i st o r t ed and
DFR i s a p r act i cal ap p l i cat i o n o f d evel o p ment al anat o my. I t
seem i n gl y f o r esh o r t en ed p h i l t r u m i n cl ef t s i s f am i l i ar
p r o vi d es a means t o anal yze membr ano us bo ne f o r mat i o n
t o al l p l ast i c su r geo n s. Fo r m an y year s, t h e r o t at i o n -
and p er i o st eal p hysi o l o gy. I t emb o d i es co ncep t s o f f aci al
ad vancement t echni q ue has b een used t o r eo r i ent t he
gr o w t h cent r al t o o r t ho do nt i cs. I t makes use o f neur o mer i c
p hi l t r um. So w hy i s p hi l t r al r o t at i o n no t r eq ui r ed i n DFR?
The ap p ear ance o f t he p hi l t r um i n cl ef t s st ems f r o m t he
o ver al l mal p o si t i o n o f t he so f t t i ssue i n a f al sel y l at er al i zed
st at e. Th u s, t h e p h i l t r u m an d co l u m el l a ap p ear t o b e
sho r t , b ut ar e act ual l y d i sp l aced i nt o t he nasal t i p . DFR
ach i eves d e-r o t at i o n o f t h e p h i l t r u m an d co l u m el l a b y
t he f o l l o w i ng: (1) f i el d sep ar at i o n; and (2) sub p er i o st eal
mo bi l i zat i o n. The ent i r e so f t t i ssue co mp l ex i n a uni l at er al
cl ef t i s l at er al l y d i sp l aced ar o u n d t h e co r n er o f t h e
p r emaxi l l a. When mo b i l i zed co r r ect l y o f f t he b o ne i t i s
b r o ught f o r w ar d ar o und t he cur ve o f t he b o ne and d r o p s
r i ght i nt o p l ace.
t heo r y t o map o ut and mani p ul at e d evel o p ment al f i el d s.
Pr o p er i mp l emen t at i o n an d st u d y o f cl ef t r ep ai r u si n g
DFR w i l l p r o vi d e a f o r um f o r d i al o g amo ng cr ani o f aci al
sur geo ns o r t ho d o nt i st s and d evel o p ment al b i o l o gi st s.
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development in normal and cleft lip and palate human fetuses 38. Chao M, Donovan T, Sotelo C, Carstens MH. In situ osteogenesis
using three-dimensional computer reconstruction. Cleft Lip Palate of hemimandible in a 9-year-old boy: Osteoinduction via stem cell
J 1991;28:49-54. concentration. J Craniofac Surg 2006;17:405-12.
Carstens MH. The spectrum of minimal clefting: Process-oriented 39. Ruberte J, Carretero A, Navarro M, Marcucio RS, Noden D.
cleft management in the presence of an intact alveolus. J Morphogenesis of blood vessels in the head muscles of avian
Craniofac Surg 2000;11:270-94. embryo: spatial, temporal, and VEGF expression analyses. Dev
Gong SG, Guo C. BMP4 gene is expressed at the putative site Dynam 2003;227:470-83.
of fusion in the midfacial region. Differentiation 2003;71:228-36. 40. Carstens M, Chin M, Ng T, Tom WK. Reconstruction of #7 facial
Ashique AM, Fu K, Richman JM. Endogenous bone morphogenetic cleft with distraction assisted in situ osteogenesis (DISO): Role of
proteins regulate outgrowth and epithelial survival during avian lip recombinant human bone morphogenetic protein-2 with Helistat
fusion. Development 2002;129:4647-60. activated collagen sponge. J Craniofac Surg 2005;16:1023-32.
Lumsden A, Krumauf R. Patterning the vertebrate neuraxis. 41. Chin M, Ng T, Tom WK, Carstens MH. Repair of alveolar clefts
Science 1996;274:1109-15. with recombinant human bone morphogenetic protein. J Craniofac
p. 75-88.
20. Noden DM. Patterning of avian craniofacial muscles. Dev Biol Surg 2005;16:778-89.
1986;116:347-56. 42. Chin M, Ng T, Tom WK, Carstens MH. 5
th
International Conference
21. Noden DM. Interactions and fates of avian craniofacial on Distraction Osteogenesis in Craniofacial Surgery. Paris,
mesenchyme. Development 1988;103:121-40. France; June, 2006.
22. Noden DM. Origins and patterns of craniofacial mesenchymal 43. Carstens MH. The sliding sulcus procedure: Simultaneous repair
tissues. J Craniofac Genet Dev Biol 1991;11:192-213. of unilateral clefts of the lip and primary palate-a new technique.
23. Noden DM, Trainor PA. Relations and interactions between cranial J Craniofac Surg 1999;10:415-29.
mesoderm and neural crest populations. J Anat 2005;207:575- 44. Demas PN, Sotereanos GC. Closure of alveolar clefts with
601. Corticocancellous block grafts and marrow: A retrospective study.
24. Helms JA, Cordero D, Tapadia MD. New insights into craniofacial J Oral Maxillofac Surg 1988;46:682-7.
morphogenesis. Development 2005;132:851-61. 45. Delaire J. The potential role of facial muscles in monitoring
25. Carlson BR. Human embryology and developmental biology, 3
rd
maxillary growth and morphogenesis. In: Carlson DS, McNamara
ed. Mosby: St. Louis; 2004. JA Jr, editors. Muscle Adaptation and Craniofacial Growth.
26. Vissarionov VA. Analysis of the results of reconstructive plastic (Center for Human Growth and Development, Craniofacial Growth
surgery of unilateral clefts of the upper lip based on data of the Monograph No. 8). University of Michigan: Ann Arbor, MI; 1978.
surgical section 1970-1977. In: Pathogenesis, treatment and p. 157-80.
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46. Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and
palate. I. Normal development and cleft palate. Br J Oral Maxillofac
Surg 1992;30:287-95.
47. Moss ML, Vilmann H, Das Gupta G, Skalak R. Craniofacial
growth in space-time. In: Carlson DS, McNamara Jr JA, editors.
Craniofacial biology. Center for Human Growth and Development,
University of Michigan: Ann Arbor, MI; 1981.
48. Park GC. The importance of accurate orbicularis repair of the
orbicularis oris muscle in unilateral cleft lip. Plast Reconstr Surg
1995;96:780-8.
49. Carstens MH. Correction of the bilateral cleft using the sliding
sulcus technique. J Craniofac Surg 2000;11:137-67.
50. Carstens MH. Correction of the unilateral cleft lip nasal deformity
reference as to the anatomy of the premaxilla. Previous papers
describing the non-existence of the human premaxilla debunked.
Butler, AB, Hodos W. Comparative Vertebrate Neuroanatomy. New
York: Wiley-Liss, 1997. Fundamental conservation of neuroembryologic
developmental units is described. Functional model relating motor and
sensory neural structures serving between axial and pharyngeal arch
musculature in alternating pairs of somitomeres.
Carlson BR. Human Embryology and Developmental Biology 3
rd
ed.
Philadelphia: Mosby, 2004. Very clear account of how an organism
forms with a good dose of modern biology. Exceptional illustrations.
using the sliding sulcus procedure. J Craniofac Surg 1999;10:346-
Francis-West PH, Robson I, Evans DJ. Craniofacial development:
the tissue and molecular interactions that control development of the
McComb H. Treatment of the unilateral cleft lip nose. Plast
head. Adv Anat Embryol Cell Biol 2003; 169:1-138. New concepts of
Reconstr Surg 1975;55:596-601.
Stenstrom SJ. The alar cartilage and the nasal deformity in
developmental biology applied to the head are succinctly reviewed.
unilateral cleft lip. Plast Reconstr Surg 1966;38:223-31. Futayama D. Evoution. Sunderland, Massachusetts: Sinauer, 2005. Up-
Drake AF, Davis JU, Warren DW. Nasal airway size in cleft and
to-date discussion of the mechanisms underlying evolution, including
noncleft children. Laryngoscope 1993;103:915-7.
Noordhoff MS, Chen YR, Chen KT. The surgical technique for the
Hox genes and the dawn of modern evolutionary developmental
complete unilateral cleft lip-nasal deformity. Op Tech Plast Surg
biology.
1995;2:164-74.
Gilbert S. Developmental Biology, 8th ed. Sunderland, Massachusetts,
Noordhoff MS. The surgical technique for the unilateral cleft
lip-nasal deformity. Noordhoff Craniofacial Foundation: Taipei,
2006. Written for advanced undergrads, this outstanding text is
Taiwan; 1997.
sumptuously-illustrated; the accompanying DVD and website, www.
Millard DR. Cleft Craft II. Bilateral and rare deformities. Little, devbio.com, have a wealth of detail including real-time movies of key
Brown: Boston; 1977. p. 259-65.
developmental processes. This is the medical professionals go-to
Siebert JW, Angrigiani C, McCarthy JG, Longaker MT. Blood
supply of the Lefort I maxillary segment: an anatomic study. Plast
source to understand molecular embryology.
Reconstr Surg 1997;100:843-51. Hall BK. The Neural Crest in Development and Evolution. New York:
Delaire J, Precious D, Gordeef A. The advantage of wide
Springer, 1997. Very readable discussion of the 4
th
germ layer and the
subperiosteal exposure in primary surgical correction of labial
source of nearly all craniofacial bones.
maxillary clefts. Scand J Plast Reconstr Surg Hand Surg
1989;22:147-51.
Hanken J, Hall BK. The Skull. Chicago: University of Chicago Press,
Precious DA, Delaire J. Surgical considerations in patients with cleft 1993. This series includes 3 volumes (Development, Patterns of
deformities. In: Bell WH, editor. Modern practice in orthognathic
Structural and Systematic Diversity, Functional and Evolutionary
and reconstructive surgery. WB Saunders: Philadelphia; 1992. p.
Mechanisms) summarizing the entire literature.
60. Rosenstein SW, Grassechi M, Dado DV. A long-term retrospective Huang R, Zhi Q, Patel K, Wilting J, Christ b. Contribution of single
assessment of facial growth, secondary surgery need and
somites to the skeleton and muscles of the occipital and cervical regions
maxillary lateral incisor status in a surgical-orthodontic protocol
in avian embryos. Anat Embryol 2000; 202:375-383. Mapping study of
for complete clefts. Plast Reconstr Surg 2003;111:1-13.
Rosenstein SW. Early bone grafting of alveolar cleft deformities.
neural crest cells from a single somite leading was later expanded.
J Oral Maxillofac Surg 2003;61:1078-81.
Hu D, Marcucio RS, Helms JA. A zone of frontonasal ectoderm
Goldstein MH. A tissue expanding vermilion myocutaneous ap
regulated patterning and growth in the face. Development 2003;
for lip repair. Plast Reconstr Surg 1984;73:768-70.
64.
51.
52.
53.
54.
55.
56.
57.
58.
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390-425.
61.
62.
63. Goldstein MH. The elastic ap for lip repair. Plast Reconstr Surg
1990;85:446-52.
ANNOTATED BIBLIOGRAPHY
Basic science/embryology
These r ef er ences p r o vi d e a b asi s f o r und er st and i ng t he
neur o mer i c syst em and d evel o p ment al f i el d s.
Barteczko K, Jacob M. A re-evaluation of the premaxillary bone in
humans. Anat Embryol (Berlin) 2004; 207:417-437. The denitive
130:1749-1758. Neural crest derivatives such as bone and cartilage
involve exchange of information between epithelia and mesenchyme.
The model reported here may explain frontal bone development as
programming by p5 skin/dermis and underlying p5 brain covered by
r1 dura. The presence of two distinct templates may map the frontal
bone into two distinct laminae, the interface between is a potential
space, the frontal sinus.
Jacobson AG. Somitomeres: mesodermal segments in the head and
trunk. In: Hanken J, Hall, BK (eds). The Vertebrate Skull. Vol I. Pp. 42-76.
Chicago: Univ Chicago Press, 1993. Clear discussion of somitomeres.
Both Carlson and Gilbert make use of this work in discussing how
embryonic tissues are segmentally organized. Fate maps of craniofacial
mesenchymal derivatives are based on this concept.
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Developmental eld reassignment in unilateral cleft lip
Kjaer I, Keeling JW, Fischer-Hansen B. The Prenatal Human Cranium
Normal and Pathologic Development. Copenhagen, Denmark:
Munksgaard, 1999. Slim volume packed with important ideas. Careful
analysis of craniofacial bone ossication is the conceptual basis for the
Lego model of sequential developmental eld assembly. Not afraid
to speculateahead of its time.
LeDouarin NM, Kalcheim C. The Neural Crest, 2
nd
ed. Cambridge, UK:
Cambridge University Press, 1999. Gerard Couly and Nicole LeDourain
revolutionized neural crest embryology with the quail-chick chimera
model. This laboratory has mapped out the origins and fate of neural
Puelles L, Rubenstein JLR. Forebrain gene expression domains and
the evolving prosomeric model. Trends Neurosci 2003;26:469-476.
Revised version of prosomeric theory.
Rubenstein, JLR, Regionalization of the prosencephalic neural plate.
Annu Rev Neurosci 1998; 21:445-477. Gene product mapping used
to dene prosomeres.
Tapadia MD, Cordero DR, Helms JA. Its all in your head: new insights
into craniofacial development and deformation. J Anat 2005;207:461 -
477. Excellent synthesis of new ideas, including the premandibular
arch. Illustrations are rst rate.
crest cells throughout the embryo. Cleft Lip and Palate Surgery
Liem, KF, Bemis WE, Walker WF, Grande L. Functional anatomy of the These references cover the bases of developmental eld theory
Vertebrates: An Evolutionary Perspective, 3
rd
ed. Belmont, California: Carstens MH. The sliding sulcus procedure: simultaneous repair
Thompson, 2001. No theory of human craniofacial development can of unilateral clefts of the lip and primary palate-a new technique. J
exist without reference to other vertebrates. The 4
th
edition includes Craniofac Surg 1999; 10:415-434. Principles of periosteal anatomy are
contributions of Hox genes and neuromeres. applied to surgical correction of soft tissues on both sides of the cleft.
Lumsden A, Krumauf R. Patterning the vertebrate neuraxis. Science Bardachs contributions are discussed in a new context. The resulting
1996; 274:1109-15. Key reference correlating neural content of centralization of the facial functional matrix leads to more anatomic
rhombomeres. deposition of membranous bone over time. Process theory (a model
Morriss-Kay GM, Wilkie AOM. Growth and the normal skull vault of cleft formation as a series of pathologic mechanisms/processes
and its alteraion in craniosynostosis: insights from Human genetics acting over time) was presented. Surgical correction would therefore
and experimental studies. J Anat 2005; 207:637-653. Morriss-Kay become a logical correction of each one of these processes such that
demonstrated in previous papers the origin of the parietal bone from the patient would grow into the repair.
PAM in mammals versus neural crest. Carstens MH. Correction of the unilateral cleft lip nasal deformity
Muller F, ORahilly. The timing and sequence of appearance of using the sliding sulcus procedure. J Craniofac Surg 1999; 10:346-
neuromeres and their derivatives in staged human embryos. Acta 364. Contribution of the decient piriform platform to alar cartilage
Anat 1997;158:83-99. The master plan of cranio-caudal development malposition is combined with concepts of mechanical displacement of
is described. This is the basis of homeotic-gene specic levels. the premaxilla and maxilla. Soft tissue deciency of the lateral nasal
ORahilly R, Muller F. Developmental Stages in Human Embryos. wall not yet linked to the premaxillary eld.
Washington, D.C.: Carnegie Institution Publication 637, 1987. The Carstens MH. Correction of the bilateral cleft using the sliding sulcus
Carnegie staging system for human embryos is described here. This technique. J Craniofac Surg 2000; 11:136-167. Commonality of
reference is used by embryologists world-wide. pathology between unilateral and bilateral clefts is emphasized
ORahilly R, Muller F. The Embryonic Human Brain: An Atlas but incision design reflects lack of understanding of soft tissue
of Developmental Stages, 2
nd
ed. New York: Wiley-Liss, 1999. developmental eld anatomy. Embryonic origin of facial musculature
Visualization of neuroaantomy in motion visually comprehensible for and the anatomy of the philtrum are discussed.
Carstens MH. The spectrum of minimal clefting: process-oriented cleft
Osumi-Yamashita N, Ninomiya Y, Doi H, Eto K. The contribution of management in the presence of an intact alveolus. J Craniofac Surg
any surgeon.
both forebrain and midbrain crest cells to the mesenchyme in the
frontonasal mass of mouse embryos. Dev Biol 1994;164:409-419.
Timing of when PNC, MNC and RNC populations complete migration
comes from this lab.
Osumi-Yamashita N, Ninomiya Y, Eto K. Mammalian craniofacial
embryology in vitro. In J Dev Biol 1997;41:187-194.
Puelles L. Brain segmentation and forebrain development in amniotes.
Brain Res Bull 2001; 55:695-710. Luis Puelles and John Rubenstein
discovered and mapped out the segmental organization of the forebrain
(prosencephalon). Neural crest in association with a given prosomere
creates external mesenchymal structures (forehead, nose, philtrum, eye),
the development of which coincides with the corresponding zones in the
brain. Anomalies are often shared. The brain predicts the face.
2000;11:270-94. For the rst time I was able to grasp the sequential
assembly of soft tissues around the cleft. The genetic signalling
relationships between the underlying premaxillary field and the
overlying soft tissues was as yet unknown. By this time I was aware
of neuromeric theory but had not yet envisioned the layering off facial
soft tissue elds over bone elds.
Carstens MH. Sequential cleft management with the sliding sulcus
technique and alveolar extension palatoplasty. J Craniofac Surg 2000;
11:437-469. As described by Delaire, cleft palate shares with cleft lip
common pathophysiologic mechanisms that, over time, produce the
nal anatomic state. Both buccal and lingual aspects of the alveolus
have a similar pattern of blood supply. Preservation of the entire
alveolar mucoperiosteum as a osteosynthetic developmental eld
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Carstens
is a key point of the surgery. The additional length gained by the
AEP procedure prevents residual anterior stula. The importance of
Schweckendiecks work is discussed.
Carstens MH. Functional matrix cleft repair. J Craniofac Surg
Carstens MH. Function Matrix Cleft Repair: Principles and Techniques.
Clin Plast Surg 2004; 31:159-189. Detailed discussion of how to do
it. As my understanding of developmental elds improved the FMR
became the DFR (Developmental Field Repair). All the principles of
FMR are preserved in the current technique. In DFR (as discussed
here), dissection of the philtrum and the lateral nasal wall are
with references.
Schendel SA, Tessier P, Tulasne J-F. Facial clefting disorders and
craniofacial dysostoses: skeletal considerations. In: Turvey TA, Vig
KWL, Fonseca RJ. Facial Clefts and Craniosynostosis: Principles and
Management. 1996. Philadelphia: WB Saunders, pp. 95-128. Good
skull photos of the synostoses.
Tessier P. Fentes orbito-faciales verticales et obliques (colobomas)
completes et frustes. Ann Chir Plast 1969;19:301-311. Original report
based on his empiric observations helped to organize facial clefts into
recognizeable patterns.
combined with the correct placement of the A1 ap and the deliberate Tessier P. Anatomical classications of facial, cranio-facial and laterofacial
reconstruction of the premaxilla with rhBMP-2. clefts. J Maxillofac Surg 1976;4:69-92. Seminal contribution in English
Carstens MH. Development of the facial midline. J Craniofac Surg establishing a geographic schematization of facial clefts. Developmental
2002;13:129-87. This paper discusses two topics, the neuroembryology eld model categorizes clefts into a neuromeric distribution explains
of the facial placodes and the neuromeric mapping of neural crest Tessiers observations on a neuroembryologic basis.
derivatives in the face. A three-dimensional model of facial development Vissiarionov VA. Analysis of the results of reconstructive plastic surgery
based on Carnegie stages is presented. of unilateral clefts of the upper lip based on data of the surgical section
Carstens MH. Neural tube programming and craniofacial cleft 1970-1977. In: Pathogenesis, treatment and prophylaxis of cosmetic
formation. I. The neuromeric organization of the head and neck. Eur deformities and deciencies. Moscow: Institute of Cosmetology; 1982,
J Paed Neurol 2004; 8:181-210. Save for some confusion about the pp. 118-21
number and relative contributions of occipital somites this paper Recombinant Human Bone Morphogenetic Protein-2
constitutes my rst-pass introduction into the neuromeric system. Relevant preclinical studies and clinical applications in the maxilla
Delaire J. The potential role of facial muscles in monitoring maxillary and mandible.
growth and morphogenesis. In Carlson DS, McNamara Jr JA (eds). Boyne PJ. Animal studies of application of rhBMP-2 in maxillofacial
Muscle Adaptation and Craniofacial Growth. Ann Arbor, MI: University reconstruction. Bone 1996; 19(suppl):83S-92S. Early work proving
of Michigan, 1978, pp.157-180 (Center for Human Growth and BMP resynthesizes membranous bone.
Development, Craniofacial Growth Monograph No. 8). Boyne PJ. A feasibility study evaluating rhBMP-2/absorbable collagen
Delaire J, Precious D, Gordeef a. The advantage of wide subperiosteal sponge for maxillary sinus oor augmentation. Int J Periodontic
exposure in primary surgical correction of labial maxillary clefts. Restorative Dent 1997;17:11-25. Fiirst clinical application in craniofacial
Scand J Plast Recons Surg 1989; 22:147-151. Pioneering work skeleton was to support maxillary implants.
on subperiosteal dissection directly following basic science of Boyne PJ. Application of bone morphogenetic proteins in the treatment
membranous bone formation represented a functional approach to of clinical oral and maxillofacial osseous defects. J bone Joint Surg
cleft repair. Delaire was the rst to emphasize physiological principles 2001; 83A(suppl)S146-S150. Demonstrates potential breadth of
of soft tissue-bone interaction over time. Reading Delaire opened the applications for Infuse.
door for me to visualize cleft formation as a 4-D process acting over Boyne PJ, Nath R, Nakamura A. Human recombinant BMP-2 in
time. DFR surgery is a direct descendent of this work. osseous reconstruction of simulated cleft palate defects. Br J Oral
Markus AF, Delaire J, Smith WP. Facial balance in cleft lip and palate Maxillofac Surg 1998; 36:84-90. Immediate grafting with BMP
I. Normal development and cleft palate. Br J Oral Surg 1992;30:287-
95. Force vector concept and its effects on bone structure presented
and illustrated with clarity.
Moss ML, Vilmann H, Das Gupta G, Skalak R. Craniofacial growth
in space-time. In Carlson DS, McNamara Jr JA (eds). Craniofacial
biology. Ann Arbor, MI: Center for Human Growth and Development,
University of Michigan, 1981. Balanced facial growth requireds that all
soft tissue developmental elds become physiologically centralized to
synthesize facial bone in the correct position over time.
Precious DA, Delaire J. Surgical considerations in patients with
cleft deformities. In: Bell WH (ed.). Modern practice in orthognathic
and reconstructive surgery. Philadelphia, WB Saunders; 1992; p.
390-425. Good discussion of Delaires technique is presented along
maintains the dental arch in primates. This was a key idea for eventual
incorporation into DFR.
Boyne PJ, Nakamura A, Shabahang S. Evaluation of long-term effect
of function on rhBMP-2 regenerated hemimandibulectomy defects. Br
J Oral Maxillofac Surg 1999; 37:344-352.
Carstens MH, Chin M, Li J. In situ osteogenesis (ISO): Regeneration
of 10-cm defect in porcine model using recombinant human bone
morphogenetic protein-2 (rhBMP-2) and Helistat absorbable collagen
sponge J Craniofac Surg 2005; 16: 1033-42.
Carstens M, Chin M, Ng T, Tom WK. Reconstruction of #7 facial cleft with
distraction assisted in situ osteogenesis (DISO): Role of recombinant
human bone morphogenetic protein-2 with Helistat activated collagen
sponge. J Craniofac Surg 2005 16(6): 1023-1032.
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Developmental eld reassignment in unilateral cleft lip
Chao M, Donovan T, Sotelo C, Carstens MH. In situ osteogenesis
of hemimandible in a 9-year-old boy: osteoinduction via stem cell
concentration. J Craniofac Surg 2006; 17:405-412. 12 cm segmental
mandibular defect reconstructed with BMP-directed ISO. Free bula
transfer was avoided. Response of the regenerate to surrounding soft
tissue forces was to remodel into a replica of the normal side.
Chi n M, Carstens MH. Di stracti on osteogenesi s wi th bone
morphogenetic protein enhancement: facial cleft repair in humans. Proc
4
th
International Congress on Cranial and Facial Distraction Processes.
Paris, France, 197-200, 2003. DISO was invented with the original #2,
Ruberte J, Carretero A, Navarro M, Marcucio RS, Noden DM.
Morphogenesis of blood vessels in the head muscles of avian
embryos: spatial, temporal and VEGF expression analysis. Dev
Dynam 2003;227:470-483. Important concept suggesting anatomic
basis of peripheral arterial system via Schwann cell induction from
peripheral nerves. BMP-directed neural crest release of VEGF to
promote rapid vascularization of surgical site was a concept derived
from this work.
Seeherman H, Wozney J, Li R. Bone morphogenetic protein delivery
systems. Spine 2002; 27(165):516-523. Carriers required to retain
#7 cleft repair which we published 2 years later. BMP-2 in the target environment are reviewed.
Chin M, Ng T, Tom WK, Carstens MH. Repair of alveolar clefts with Urist, MR, Strates BS. Bone morphogenetic protein. J Dent Res 1971;
recombinant human bone morphogenetic protein. J Craniofac Surg 50:1392-1406. The original report. PJ Boyne studied under Urist.
2005; 16(5):778-789. Original study done using DFR concepts in Valentin-Opran, Wozney J, Csiima C, Lilly L, Reidel GE. Clinical
secondary cleft cases. evaluation of recombinant human bone morphogenetic protein-2.
Ebara S, Nakayama K. Mechanisms for the action of bone Clin Orthop Related Res 2002;395:110-120. Relevant clinical data
morphogenetic proteins and regulation of their activity. Spine 2002 succinctly summarized. Very useful reference.
27(16S):S10-S15. Induction of osteoclasts from monocytes sets up Wozney J. Overview of bone morphogenetic proteins. Spine
negative feedback loop. 2002;27(165):52-58. Physical characteristics of BMP discussed.
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.
Carstens
EPILOGUE FOR COLLEAGUES IN INDIA AND
SOUTHEAST ASIA
I sh o u l d l i k e t o d ep ar t at t h i s p o i n t f r o m t r ad i t i o n al
acad emi c w r i t i ng t o ad d r ess t he r ead er i n a ver y p er so nal
w ay. M any o f yo u must t r eat chi l d r en and ad ul t s w i t h cl ef t s
u n d er t h e m o st d em an d i n g o f ci r cu m st an ces. Po ver t y,
p o o r co m m u n i cat i o n , d i st an ce f r o m m ed i cal cen t er s
and d i f f i cul t i es i n t r ansp o r t at i o n negat i vel y af f ect ever y
asp ect o f yo ur t r eat ment p l an. The same i s t r ue f o r yo ur
DFR w as b o r n i n p o st -w ar Ni car agua, und er t he har shest
o f co n d i t i o n s. I w as assi g n ed t h er e t o w o r k f o r t h e
Pan Amer i can Heal t h Or gani zat i o n (WHO) as a sur gi cal
co n su l t an t i n 1990-1992. At t h at t i m e, t h e b ack l o g o f
p l ast i c sur ger y cases w as est i mat ed at 10,000 p at i ent s.
Pr o b l ems w er e no t o nl y l o gi st i cal b ut al so t he cr eat i o n
o f manp o w er. Wo r k i ng w i t h Pr o f esso r Ar t ur o Go mez at
t he Uni ver si d ad Naci o nal d e Ni car agua i n Leo n and w i t h
a smal l gr o up o f l ay p eo p l e, t he f i r st med i cal f o und at i o n
i n t he hi st o r y o f Ni car agua w as f o r med . No el I caza, an
p at i ent s, no mat t er ho w mo t i vat ed t hey mi ght b e. Ti me an gr i cu l t u r al en gi n eer, gave i t t h e n ame Ni cap l ast . We
t ak en t o b r i n g a ch i l d i n f o r t r eat men t f r o m a r emo t e t r avel ed ar o und t he co unt r y, o per at i ng and w o r k i ng w i t hi n
l o cat i o n m ay p r esen t an i n t o l er ab l e st r ess o n f am i l i es var i o us ci t i es t o cr eat e sup p o r t f o r t hi s ap p ar ent l y cr azy
l i vi ng i n t hese ci r cumst ances. Fo r many, i f no t mo st , o f t he i d ea. Ni car agu a h ad d escen d ed i n t o ch ao s. Th e gr o ss
w o r l d s chi l d r en w i t h cl ef t s, t hese f act o r s mak e car ef ul , n at i o n al p r o d u ct w as j u st ab o ve t h at o f Hai t i , m ak i n g
w el l -r egul at ed mul t i d i sci p l i nar y car e a d i st ant mi r age so t hi s f o r mer l y act i ve exp o r t i ng nat i o n t he seco nd -p o o r est
easy t o t al k ab o ut , so d i f f i cul t t o achi eve. o f al l t he Amer i cas. Gi ven t hi s si t uat i o n, t he cr eat i o n o f
r eso ur ces t o r esusci t at e r eco nst r uct i ve sur ger y seemed
Fo r t hese p at i ent s t he sur gi cal t eam must set p r i o r i t i es an i mp o ssi b l e t ask .
t o acco mp l i sh t he maxi mum p hysi o l o gi c co r r ect i o n w i t h
t he mi ni mum o f sur ger i es. The go al s at t he f i r st st age At t h i s p o i n t , Car l o s an d Vi vi an Pel l as st ep p ed i n . Th ese
ar e: (1) a f unct i o nal nasal ai r w ay; (2) a uni t ed d ent al ar ch r em ar k ab l e p eo p l e, f r o m a h i gh l y ed u cat ed an d w eal t h y
w i t ho ut co l l ap se; (3) sub p er i o st eal d i ssect i o n d esi gned f am i l y, su r vi ved a co m m er ci al ai r l i n e cr ash i n 1 9 8 9 . 1 7 0
t o p r o m o t e cen t r i c f aci al g r o w t h ; an d (5 ) so f t t i ssu e p er i sh ed at t h e scen e. Th e Pel l as w er e evacu at ed t o
co r r ect i o n usi ng d evel o p ment al f i el d co ncep t s t o p l ace M i am i w h er e t h ey u n d er w en t n u m er o u s su r g er i es f o r
scar s i nt o emb r yo ni c sep ar at i o n p l anes. The go al s o f t he b u r n s an d f r act u r es i n cl u d i n g f r ee t i ssu e t r an sf er. Up o n
f o l l o w -u p st age ar e: (1) m et i cu l o u s t h r ee-l ayer cl o su r e r ecu p er at i n g t h ey r et u r n ed t o Ni car ag u a d et er m i n ed
o f t he so f t p al at e; and (2) use o f AEP (al veo l ar ext ensi o n t o h el p ch i l d r en w i t h b u r n s an d d ef o r m i t i es. Th ei r
p al at o p l ast y) f l ap s t o p r event ant er i o r f i st ul a. Sho ul d t he su p p o r t f o r Ni cap l ast r esu l t ed i n t h e est ab l i sh m en t
p at i ent b e unab l e t o r et ur n f o r f ur t her w o r k , t hi s sho ul d o f n e w r e si d e n cy t r ai n i n g at Un an - Le o n , i m p r o v e d
p r o vi d e at t he ver y l east an i nt act o r al cavi t y i n so me i n f r ast u ct u r e an d a co m p l et e m o d er n i z at i o n o f t h e
d egr ee o f al i gnment , a w o r k i ng ai r w ay, r easo nabl e sp eech p ed i at r i c b u r n u n i t i n t h e cap i t al ci t y, M an agu a. Par t n er
and an ap p ear ance t hat i s so ci al l y accep t ab l e. r el at i o n sh i p s w er e co n st r u ct ed w i t h I n t er p l ast , t h e
Un i ver si t y o f W i sco n si n , Ph ysi ci an s f o r Peace an d t h e
Devel o p m en t al Fi el d Reassi g n m en t cl ef t su r g er y w as AO Fo u n d at i o n i n Basel , Sw i t z er l an d . Each o n e o f t h ese
d esi gned w i t h t hese ci r cumst ances i n mi nd . I t i s a hi gh en t i t i es co n t r i b u t ed t o w ar d ex p an d ed r eco n st r u ct i ve
i n t el l ect /l o w t ech n o l o gy m et h o d . Th e o n l y st r u ct u r es su r g i cal car e an d ad van ced ed u cat i o n . I n t i m e, a n ew
mi ssi ng ar e a l at er al nasal w al l so f t t i ssue d ef ect (eq ual
t o t h e cym b a) an d an al veo l ar b o n e d ef ect (eq u al t o
t h e h o u si n g o f t h e l at er al i n ci so r ). Reco n st r u ct i o n o f
t h ese vi t al el em en t s i s w i t h i n t h e h an d s o f al l cl ef t
sur geo ns. Ther e ar e o nl y t w o r eq ui r ement s f o r DFR t o b e
p r o p er l y p er f o r med : (1) i n-d ep t h anat o mi c k no w l ed ge o f
emb r yo l o gi c f i el d anat o my; and (2) t he t echni cal ab i l i t y
t o mak e and secur e an acr yl i c p al at al sp l i nt . Ho w o ne
achi eves al veo l ar r eco nst r uct i o n i s i r r el evant . One may use
cancel l o us gr af t (r i b i n yo ung p at i ent s) + p l at el et enr i ched
p l asma. One may use r hBM P-2. What r eal l y mat t er s i s t o
f o l l o w b i o l o gi c p r i nci p l es.
f o u n d at i o n , u n d er t h e l ead er sh i p o f Vi vi an Pel l as, w as
est ab l i sh ed . Th e Aso ci aci o n Pr o Ni n o s Qu em ad o s d e
Ni car ag u a (APROQUEN) n o w h as d i r ect r esp o n si b i l i t y
f o r b u r n car e i n t h at co u n t r y.
The r esi d ency w as p ar t i cul ar l y i nno vat i ve. The l o ngest i n
Lat i n Amer i ca, i t d emand s f ul l gener al sur ger y + 3 year s o f
p l ast i c sur ger y, p r ep ar i ng o ne gr ad uat e ever y t hr ee year s.
Par t i ci p at i o n b y t he r esi d ent i n al l act i vi t i es w i t h vi si t i ng
t eams cr eat ed a b r o ad exp o sur e. M i ni -f el l o w shi p s w er e
cr eat ed. Each gr aduat e under go es f i nal o r al exami nat i o n i n
Engl i sh w i t h t he par t i ci pat i o n o f nat i o nal and i nt er nat i o nal
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Developmental eld reassignment in unilateral cleft lip
f acul t y. Al l t hr ee gr ad uat es r emai n i n t he p ub l i c sect o r.
Dr. Gust avo Her d o ci a w o r k at UNAN-Leo n w hi l e Dr. M ar i o
Per ez and Dr. Leo nel Br i ceno st af f t he APROQUEN b ur n
uni t . Over 4000 sur ger i es and 100,000 o ut p at i ent s have
b enef i t ed f r o m t hese ef f o r t s w i t ho ut a si ngl e d o l l ar f r o m
ab r o ad .
Bu t h u man r eso u r ces ar e n o t en o u gh . Wh i l e Ni cap l ast
co n t i n u es i t s val u ab l e w o r k i n Leo n , APROQUEN h as
su r ged f o r w ar d as a m o d er n -d ay m o d el o f a m ed i cal
12 year s ago I p r o p o sed t hi s p l an t o Car l o s and Vi vi an
Pel l as o ver r um o n a muggy M anagua ni ght o ver sever al
r o u n d s o f Fl o r d e Can a Ru m . I n 2 0 0 5 t h e H o sp i t al
M et r o p o l i t ano Vi vi an Pel l as o p ened i t s d o o r s. HM VP exi st s
b ecause p eo p l e car ed d eep l y, r ef used t o gi ve up ho p e and
w o r k ed unceasi ngl y t o mak e i t hap p en. And t he st unni ng
f act o f t he ent i r e Ni car agua exp er i ence i s t hat i t hap p ened
f r o m w i t hi n. I nf o r mat i o n r egar d i ng t hese f o und at i o ns i s
avai l ab l e t o al l sur geo ns o n t he I nt er net .
f o und at i o n w i t h a bo l d st r at egy: t he cr eat i o n o f a f i nanci al I w r i t e t hi s t o you, t he r eader, t o pr opose t hat i mpr ovement s
engi ne t o f i nance t he cl i ni cal w o r k and co st o f r unni ng i n cl ef t car e can al w ays b e m ad e, even u n d er d i f f i cu l t
a 1
st
-cl ass b ur n cent er i n an ext r emel y p o o r co unt r y. I n ci r cumst ances. Our p at i ent s sp eak f o r t hemsel ves. The
1972 a t er r i b l e ear t hq uak e d est r o yed much o f t he cap i t al p r i mar y t ask i s t o make i t p o ssi bl e f o r t he nat i o nal med i cal
ci t y, i ncl ud i ng t he mai n ho sp i t al f o r t he co unt r y. Ext ensi ve t eams t o achi eve t he l o gi st i cal sup p o r t r equi r ed t o p r o vi d e
o ut si d e ai d w as l o st t o co r r up t i o n; t he ho sp i t al w as never t h e car e. An d , i n t h i s r egar d , i n n o vat i ve ef f o r t s i n t h e
r ebui l t . 4 t emp o r ar y st r uct ur es bui l t 35 year s ago co nt i nue p r i vat e sect o r, b ack ed b y l ay p eo p l e w i t h t he d esi r e t o
t o p r o vi d e car e t o t he p o p ul at i o n. A hi gh q ual i t y cent er hel p , t o b yp ass r o ad b l o ck s and p r o vi d e b et t er ser vi ce.
o f excel l ence w as no nexi st ent .
As I l o o k b ack w ar d an d co n t em p l at e t h e evo l u t i o n o f
Bu r n s ar e a so ci o e co n o m i c d i se ase . Ni car ag u a h as d evel o p ment al f i el d t heo r y and t he DFR r ep ai r, I r eal i ze
1000 new b ur n cases p er mo nt h, 10% o f w hi ch r eq ui r e t hat i t f o l l o w ed a number o f st eps, so me f o r w ar d and so me
ad mi ssi o n. Gi ven t he many need s i n t he heal t h sect o r, b ack w ar d . Th e d r i vi n g f o r ce b eh i n d i n n o vat i o n co m es
p ubl i c r eso ur ces ar e gr o ssl y i nad equat e f o r t hese p at i ent s. f r o m r est l ess cur i o si t y and t he cap aci t y t o d emand t hat
Kno w i ng t hi s, APROQUEN p ar t ner ed w i t h o t her i nvest o r s anat o my must mak e sense. Nat ur e i s t r yi ng t o t el l us her
t o b ui l d t he mo st mo d er n (al b ei t smal l ) med i cal f aci l i t y i n secr et s i f w e can o nl y l ear n t o l i st en. As w e und er st and
t he co unt r y, w i t h st at e-o f -t he-ar t = r ad i o l o gy, sur ger y and ho w t he f ace i s co nst r uct ed , w e w i l l l ear n t he seq uence
i nt ensi ve car e. The f aci l i t y w o ul d ser ve t he i nt er nat i o nal t hat l ead s t o cl ef t f o r mat i o n. Thi s i nevi t ab l y w i l l d i r ect us
co mmuni t y and p r i vat e p at i ent s. I t w o ul d be t he ver y best t o w ar d b et t er t echni q ues and b et t er r esul t s. As a cl ef t -
i n Cent r al Amer i ca. Tr i p s t o M i ami w o ul d b e avo i d ed . But af f ect ed p er so n I k no w t he r eal i t y b ehi nd t he sur ger i es
t he k ey co ncep t i s t hat t hat APROQUEN w o ul d mai nt ai n w e p er f o r m f o r o ur p at i ent s. We must p ush f o r w ar d t o d o
5 1 % o w n er sh i p an d a p er cen t ag e o f p r o f i t s w o u l d bet t er. Ho pef ul l y, DFR sur ger y w i l l l ead yo u t o quest i o n o l d
t hus d i r ect ed t o t he b ur n ser vi ce and r ei nvest ed f o r t he assump t i o ns, st r i ve f o r p er f ect i o n and (mo st i mp o r t ant l y)
f o und at i o n. f i nd yo ur o w n i nno vat i o ns.
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