orthopedics cong. disord
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CONGENITAL (DEVELOPMENTAL) ABNORMALITIES OF THE SKELETON
Incidence:0,7% of neonates are born with multiplex developmental anomalies
Morpo!o"ic#! $peci#!i%&:In congenital malformations more tissues are aected, than it is apparent at rstsight:#p!#$%ic or d&$p!#$%ic %i$$'e$
blood vessels, nerves, muscles
Bone$ #nd oin%$ #re #!$o d&$p!#$%ic or #p!#$%ic
Ae%io!o"& * C#'$e$Gene%ic + inherited disorders
Dominant inheritance !0% of rst degree relatives are aected "ecessive # $!% & of ' siblings( are aected )#lin*ed inheritance father passes +abnormal x#gene to daughter carrier(
who passes is to child -ultifactorial inheritance
In%r#'%erine d#,#"e rst trimester.( /oxins Drugs thalidomide ontergan( radiation
%#!ido,ide + Con%er"#n (-./-) + e0cep% in %e 1SA2 3ere FDA #d
# $%ric% con%ro! o4er 5%er#%o!o"ic#!6 %ri#! o7 ne3 dr'"$2 '$ed inpre"n#n% 3o,en ('$ed #$ $ed#%i4e$2 %e$%ed 8r$% + pro9#9!& + ino!oc#'$% c#,p$
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C!#$$i8c#%ion 1eneralised bone d2splasia 3nomal2 due to intrauterine patholog2: constriction rings 42poplasia incomplete development( gigantismus
onstriction rings of the hand and legonse5uence: terminal end( parts ofextremit2 ma2 be missing
Duplication eg6 pol2dact2l2, delta#phalanx of thumb ac* of separation of parts of extremit2 eg6 s2ndact2l2, s2nostosis( Decient development of extremit2 or part of extremit2(
# longitudinal# transversal
De8cien% de4e!op,en% o7 e0%re,i%&or p#r% o7 e0%re,i%&:8 Tr#n$4er$#!de7ec%Ter,in#! %&peco,p!e%e !#c o7 di$%#! p#r% o7e0%re,i%&
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De8cien% de4e!op,en% o7 e0%re,i%&or p#r% o7 e0%re,i%&:9ame of transversal decienc2according to the level of damage:
omplete decienc2
artial decienc2 of femur or tibia* in pro0i,#!2 ,idd!e or di$%#!%ird
omplete or partial decienc2 oftarsus, metatarsus, toes
De8cien% de4e!op,en% o7 %e 3o!e or p#r% o7 #n e0%re,i%&
PHOCOMELIA * %r#n$4er$#! in%erc#!#%ed (ie $e",en% in 9e%3een %3o9one$) !#c o7 e0%re,i%&
ac* of humerus forearm, hand onl2 ac* of humerus forearm and hand onl2 ac* of forearm humerus and hand onl2
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De8cien% de4e!op,en% o7 %e 3o!eor p#r% o7 #n e0%re,i%&8 A: 'pper e0%re,i%& +!on"i%'din#!de4e!op,en%#! de8cienc& +pre#0i#! (on r#di#! $ide)
; B: !o3er e0%re,i%&pre#0i#! (on %i9i#! $ide)
8 A6 'pper e0%re,i% po$%#0i#!('!n#r $ide * ,i$$in" '!n#)8 B: !o3er e0%re,i%&
!on"i%'din#! de8cien% de4e!op,en%po$%#0i#! (89'!#r $ide * ,i$$in"89'!#)
De8cien% de4e!op,en% o7 %e 3o!eor p#r% o7 #n e0%re,i%&8 M'!%ip!e de4e!op,en%#! #no,#!& "ight upper armTr#n$4er$#! de8cienc& (7ore#r,,i$$in") right leg: po$%#0i#! de8cienc&(89'!# e,i,e!i# < 89'!# ,i$$in") left leg:pre#0i#! con"eni%#! #no,#!& :,i$$in" %i9i#
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de4e!op,en%#! de8cienc& o7 9o%'pper e0%re,i%ie$ : "ight:pre#0i#! * on %e 7ore#r, (r#di'$,i$$in")
eft:in%erc#!#r %r#n$4er$#! de8cienc&(Fore#r, #r%ic'!#%e$ %o $o'!der)
Pre#0i#! con"eni%#! de7or,i%&o7 9o% 'pper e0%re,i%ie$: eft:r#di'$ #p!#$&2po!!e0 &pop!#$&right:r#di'$ #ndpo!!e0 &pop!#$&
Lo3er e0%re,i%&Lon"i%'din#! de4e!op,en%#!de8cienc&:pro0i,#! 7oc#!7e,or#!de7ec%2C!#$$i8c#%ion:
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P#pp#$2 -.=>
pro0i,#! 7oc#!7e,or#! de7ec% (IV)
8 pro0i,#! 7oc#!7e,or#! de7ec%:+ Le7% : III+ Ri"%: VII
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8 1!n# &pop!#$&; Di$!oc#%ion o7 e#d o7 r#di'$
;Oper#%ion:1!n# re$ec%ion2p!#ce,en% o7 r#di'$ in%o %e ,idd!e%ird o7 '!n#
1!n# de8cien%c&2di$!oc#%ion o7 e#d o7r#di'$
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De!%# p#!#n08 %rip#!#n"e#!po!!e0%&pe >
$'pern',er#r& ,idd!e p#!#n0
De!%# p#!#n0%rip#!#n"e#!po!!e0 %&pe > $'pern',er#r& ,idd!e p#!#n0
Si,i!#r c#$e * De!%# p#!#n08 -/ &e#r o!d "ir!
Oper#%ion: re$ec%ion o7 de!%#p#!#n0; in%erp#!#n"e#! #r%rode$i$
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Ti9i# &pop!#$&8 -2@ &e#r o!d ci!d8 ri"% !e": $or%er 4#r'$
Ti9i# &pop!#$&8>rd o7 ri"% %i9i# i$ ,i$$in"
Ti9i# &pop!#$i#
8Oper#%ion: Fi9'!# pro Ti9i#pro0i,#!!& 89'!# in%o c#!c#ne'$ di$%#!!&
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Ti9i# &pop!#$&8 Preop 0*r#&
Ti9i# &pop!#$&8e!on"#%ion o7 pro0i,#! %i9i#
Ti9i# #p!#$& 9o% !e"$ : $e4ere%or$ion#! de7or,i%& e'ino4#r'$po$i%ion o7 9o% 7ee%
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Ti9i# #p!#$& * oper#%ion: 89'!# in%o7e,'r (no%c) pro0i,#!!&2 #nd in%oc#!c#ne'$ di$%#!!&
Ti9i# #p!#$i#8 Po$%op *r#&
Ti9i# #p!#$&; $#,e c#$e #% - &e#r$
O9ec%i4e$ in %e ,#n#"e,en% o7 con"eni%#! de8cienc& o7 e0%re,i%ie$:&6 Improvement of function$6 osmetic esthetic outloo*
/reatment options:&6 onservative: exercises , splints orthoses$6 =perations # corrective procedures
>6 rosthesis'6 "ehabilitation
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Ai, o7 %re#%,en%:/reatment of existing deformities, prevention of later deformities3spects of operative treatment:
/iming Is there a spontaneous tendenc2 to correction ?
3bilit2 to cooperate 9umber of operations expected @unctional adaptabilit2 s2chological disorders Apper or lower extremit2 is involved?
Ti,in":E#r!& oper#%ion ,'$% 9e done:
in rapid deterioration of deformit2 in circulator2 disturbance of /he extremit2 due to growth in defrormed
position If possible, nish operation until school age