Vaughan-Jackson syndrome

Vaughan-Jackson syndrome describes a rare syndrome of sequential atraumatic extensor tendon ruptures in the hand, usually in the setting of rheumatoid arthritis of the distal radioulnar joint.

Clinical presentation

The presentation is with reduced extension of the fingers . Typically, the fifth digit is the first to lose extension, and then sequentially the fourth, the third, and then finally the second digit . While there may be pain in the wrist over the fourth dorsal compartment, there is often no new pain in the fingers or hands . The ruptured and retracted ends of the tendon may be palpable .

Pathology

The syndrome is classically seen in patients with rheumatoid arthritis of the distal radioulnar joint . However, Vaughan-Jackson syndrome has also been reported to be secondary to other arthridites (e.g. osteoarthritis) and pathologies (e.g. Kienböck disease) .

In affected patients with rheumatoid arthritis, there is progressive subluxation and dorsal displacement of the ulnar head . This impinges on and causes progressive mechanical stress to the extensor digitorum, and to a lesser extent the extensor digiti minimi, tendons . Eventually, due repeated this mechanical stress at this site of impingement, likely in combination with tenosynovitis from rheumatoid arthritis as well, there is sequential disruption of the tendons beginning from the ulnar side .

Radiographic features

Plain radiograph

Features of distal radioulnar arthritis is common, with exact features depending on the exact underlying arthropathy . In patients with rheumatoid arthritis, the scallop sign may be predictive of a high risk of extensor tendon rupture .

MRI

MRI is the most sensitive modality for radiographic evaluation of both tendonopathy and underlying arthropathy .

Treatment and prognosis

Ideally management is preventative, through regular physical examinations screening for extensor tendon weakness or restriction, and referring patients for tenosynovectomy +/- ulnar head surgery if deemed to be at high risk . In patients presenting after rupture, surgical repair may be an option, but return to full range of motion in the fingers may not be possible .

History and etymology

The condition was first described in a case series of two patients with osteoarthritis by OJ Vaughan-Jackson in 1948 .