Diagnostica per Immagini nella malattia di Dupuytren

Body:

La malattia di Dupuytren (chiamata anche contrattura di Dupuytren) è una condizione fibroproliferativa idiopatica che colpisce i tessuti palmari superficiali delle mani e delle dita. Mentre il disturbo è benigno, la morbilità associata è elevata a causa delle limitazioni dell’attività quotidiana. La  fibrosi progressiva si sviluppa nel grasso sottocutaneo, formando dapprima noduli che determinano contrazione dei  tessuti e successivamente contrazione in flessione delle dita. La malattia può coinvolgere   qualsiasi dito, ma  il mignolo è più comune.

 ANATOMIA

Il normale palmo e la fascia digitale sono costituiti da una serie di bande sottocutanee volar la cui funzione principale è quella di stabilizzare la pelle durante la presa. L’aponeurosi palmare (Figura sopra) inizia sul bordo distale del tunnel carpale, prendendo origine dal legamento carpale trasversale e dall’aponeurosi distale del palmaris longus. Nella mano si distende in una forma triangolare appiattita, quindi si divide in quattro bande pretendinee (P) che si sovrappongono ai tendini ed alle guaine dei  flessori delle dita. I legamenti palmari trasversali prossimali (T) corrono tra le fasce pretendinee nella metà del palmo (Figura in alto a destra). Le fasce pretendinee terminano approssimativamente alle articolazioni MCP. Situati alle estremità distali delle fasce pretendinose, i legamenti natatori (frecce nere) sono la principale componente trasversale della fascia palmare, orientati perpendicolarmente all’apparato flessore e scorrendo tra le cifre adiacenti.

Nelle dita, la fascia digitale è composta da più strutture interconnesse (Figura 3), nessuna delle quali è ordinariamente visibile su immagini RM standard a meno che non siano ispessite patologicamente in corde. I fogli digitali laterali  si estendono lungo le superfici radiale e ulnare di ciascun dito dai legamenti natatori fino al livello dell’articolazione interfalangea distale. Le Le fasce o cordoni  spirali (frecce gialle) accoppiate iniziano alle estremità distali delle bande pretendinee superficiali, quindi si estendono distalmente viaggiando in profondità fino ai legamenti natatori, dorsali ai fasci neurovascolari. Le fasce o cordoni  spirali  terminano sugli aspetti prossimali dei fogli laterali.

ESAME OBIETTIVO

Grado 0: estensione completa del palmo e delle dita (il palmo della mano puo essere appoggiato perfettamente sul piano di un tavolo).

Grado1: non si può appoggiare perfettamente la mano sul tavolo, con perdita della totale estensione delle dita. La perdita arriva fino a 45 gradi.

Grado 2: la perdita di estensione arriva fino a 90 gradi, fino a 135 gradi (Grado 3), fino a 180 gradi (Grado 4).

Dove si può localizzare?

Valore dell’imaging nella malattia di Dupuytren

La maggior parte dei casi di Dupuytren presenta risultati clinici caratteristici e non richiede imaging per confermare la diagnosi. L’Imaging è altresì necessario per confermare la diagnosi ed ecludere patologie diverse, specie nelle forme cliniche di Dupuytren che non presentano deformita in flessione delle dita. Una serie prospettica di 10 pazienti ha mostrato che in pazienti con una diagnosi clinica nota di Dupuytren, l’imaging RM è stato in grado di rappresentare con precisione l’estensione anatomica della malattia (numero, dimensioni e profondità di noduli e corde).

Un cordone pretendineo termina all’incirca in corrispondenza del  legamento natatorio e può causare una contrattura in flessione sull’articolazione MCP, ma non dovrebbe influire sull’articolazione PIP. Questi cordoni non si dirigono vicino ai fasci neurovascolari.

L’ecografia mostra la stessa capacità interpretativa della Risonanza Magnetica nell’identificazioni degli ispessimenti nodulari e dei cordoni caratteristici della malattia di Dupuytren.

Yacoe et al.  hanno mostrato che l’intensità del segnale dei noduli era correlata istologicamente alla loro cellularità, con noduli che erano isointensi rispetto al muscolo (su entrambe le immagini pesate in T1 e T2) essendo relativamente cellulari (i pazienti in questo studio erano probabilmente nella seconda fase involutiva di malattia) e quelli con bassa intensità del segnale sono ipocellulari (stadio residuo). Questa osservazione ha una potenziale rilevanza per la gestione: il tasso di recidiva post-operatoria per i noduli cellulari (negli stadi proliferativi o involutivi) è molto più elevato rispetto a quelli resecati nella fase avanzata, quindi i chirurghi cercano di evitare di operare nella fase iniziale della malattia. Tuttavia, nessuno studio di imaging ha valutato specificamente i pazienti nella prima fase proliferativa in cui l’attività mitotica è elevata e il tasso di recidiva post-operatoria è più alto. L’intensità del segnale delle corde non sembra correlarsi con i risultati istologici, con la maggior parte delle corde ipocellulari e collagene patologicamente indipendentemente dalle loro intensità del segnale MR.

l’identificazione di corde in prossimità dei fasci neurovascolari avvisa il chirurgo di un aumento del rischio per tali strutture durante la dissezione. In questo l?ecografia, rispetto alla RM, ha maggiore accuratezza diagnostica quando corredata da esame color.Doppler.

Come detto la L’imaging oltre a confermare la diagnosi clinica di Dupuytren, permette di riconoscere eventuali altre cause della sintomatologia dolorosa e funzionale.

Articolo a cura del Dott. Sergio De Bac

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