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Maladia Dupuytren PDF
Maladia Dupuytren PDF
I. Generaliti
I. 1. Istoric i Epidemiologie
I. 2. Etiologie
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diabet, dezvolt boala Dupuytren (19,20). Se pare c microangiopatia diabetic
conduce la ischemie i implicit la ngroarea fasciei palmare.
Marii fumtori, alcoolismul, epilepsia, sunt condiii asociate frecvent cu
boala Dupuytren (11,12,13,). Burge i colab., au raportat un risc crescut la
alcoolici, fumtori, pacieni cu hipercolesterolemie i cu infecie HIV (14.15).
Exist meniuni citate de Liss (16) care asociaz boala cu expunere la vibraii i
munc manual. De asemenea exist raportri de cazuri de boal care au aprut
dup lezarea chirurgical a minii (17,18).
Transmiterea autozomal dominant a bolii cu penetran incomplet este
agreat de muli autori. (21). Aproximativ o treime din pacienii cu Dupuytren ai
descendenilor din Europa de Nord au un istoric familiar pozitiv de boal.
2
Fig. 1 complexul fascial palmar: aponevroza radial (RA), aponevroza ulnar (UA), aponevroza
central, aponevroza palmodigital i fascia degetelor. NL: ligament natatory, PA: fascia palmar,
TLPA: ligament transvers al aponevrozei palmare. (dup Rayan GM. Palmar fascial complex
anatomy and pathology in Dupuytrens disease. Hand Clin. 1999;15:75.)
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Structurile fasciale din regiunea palmodigital sunt complexe.
n boala Dupuytren sunt implicate urmtoarele componente fasciale superficiale:
- band pretendinoas
- band spiral
- fascia digital lateral
- ligament Grayson
- ligament Natatory
Dup Luck (26), componentele longitudinale normale ale aponevrozei
palmare superficiale poart titulatura de benzi, iar esutul afectat de corzi.
Coarda spiral are patru origini: banda pretendinoas, banda spiral, stratul
digital lateral i ligamentul Grayson (28). Aceast coard apare mai ales la
degetul mic, dar poate afecta i inelarul. La nivelul regiunii palmare este
localizat superficial de pachetul neurovascular, distal de articulaia
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metacarpofalangian i trece apoi profund fa de pachetul neurovascular. La
nivelul degetului merge lateral de pachetul neurovascular i implic stratul digital
lateral.
Cele mai frecvente corzi digitale sunt cele centrale, spirale i laterale. Ele
sunt responsabile de contracturi de flexie ale articulaiilor interfalangiene
proximale. Coarda abductorului mic al degetului cunoscut sub numele de coarda
digital izolat i are originea n tendonul abductorului mic al degetului, dar
poate aprea i n vecintatea fasciei muchiului sau baza falangei proximale.
I. 4. Fiziopatologie
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acestor afeciuni sunt similare bolii Dupuytren. Se sugereaz c pacienii cu aceste
fibromatoze adiionale prezint mai multe recurene i extensii dup tratament
chirurgical.
II.1. Diagnostic
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constau mai rar n noduli Garrod, sau n leziuni fibrozante peste articulaiile
interfalangiene proximale. Aceste leziuni constau n pierderea anurilor,
ngroare sau hiperkeratoz. Apar la mai puin de jumtate din pacieni, n spacial
la nivelul inelarului (44,45).
Nodulul Dupuytren este o mas ferm de esut moale fixat att la piele ct
i la fascia profund. Originea lui este din componentele superficiale ale fasciei
palmare sau digitale. De obicei este bine definit i se vede la suprafa. Noduluii
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sunt localizai la palm sau la degete. Nodulii sunt de obicei nedureroi. Pot cauza
durere dac se asociaz cu tenosinovit stenozant
Fig.7. Stadiile clinice n boala Dupuytren (Ann R Coll Surg Engl 2006)
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afectat. Corzile sunt responsabile de contracturile de flexie ale articulatiilor.
Denivelrile arat ataarea dermului palmar la fascie. Limitarea funcional
rezult din scderea ariei de micare a minii.
Stadiul rezidual (stadiul avansat). n acest stadiu, nodulii dispar, rmne
contractura de flexie a articulaiei i corzi fibroase groase i dure. La nivelul
degetelor, compresiunea nervilor poate cauza tulburri de sensibilitate..
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II.2. Evoluia clinic
- apariia unui nodul sau corzi intr-o zon care anterior a fost indemn;
Durata medie de evoluie a bolii Dupuytren este de aproximativ 4-5 ani, dup care
leziunile se stabilizeaz. Degetele rmn fixe n flexie, limitnd astfel
funcionalitateam mnii
Diagnosticul diferenial are n vedere alte afeciuni ale minii ce produc noduli
sau contractur:
- contractur articular intrinsec;
- ganglioni palmari;
- incluziuni chistice sau epidermoide;
- tenosinovit stenozant, sau nodulard;
- hiperkeratoz ocupaional;
- calus;
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- corpi strini;
- tumoare cu celule gigante de esuturi moi;
- sarcom epiteloid;
- modificri secundare n artrita reumatoid.
III Tratament
Obiective:
- mbuntirea funcionalitii
- reducerea deformrii
- prevenirea recurenelor
11
Indicaii
Fasciotomia
12
O variant mai recent este fasciotomia percutan, adoptat de un grup de
reumatologi francezi i repopularizat de Foucher i ali chirurgi din Euopa. Acest
procedeu minim invaziv se poate efectua n cabinet sub anestezie loco-regional.
Implic multiple locuri de punctur cu acul i secionarea corzii cu ajutorul
suprafeei n poziie oblic a acului. (64).
Dermofasciectomia
Dermofasciectomia este utilizat n caz de recuren, mai ales la degete.
Fascia afectat i pielea de deasupra se excizeaz complet i pentru nchiderea
plgii se folosete gref de piele n toat grosimea (65,66).
13
Gref de piele
Lambouri
14
Pacient la 2 sptmni dup intervenie n palm i degetulV. Se observ extensia total a
degetului (dup Richard A. Bernstein).
III. 4. Complicaii
Complicaiile precoce:
- Hematomul
- Pierderea lamboului de piele
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- Infecia
- Lezarea nervului sau arterei digitale. Msurile preventive constau n
izolarea i disecia atent a pachetului vasculonervos cu folosirea unei lupe
magnificatoare.
Complicaii tardive:
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