Vaughan-Jackson Syndrome Clinical Presentation

Updated: Mar 25, 2019
  • Author: John A McAuliffe, MD; Chief Editor: Harris Gellman, MD  more...
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Presentation

History

Patients who have Vaughan-Jackson syndrome usually present with sudden inability to actively extend the metacarpophalangeal (MCP) joint of the small finger. This is seldom associated with significant discomfort, though symptoms of pain, instability, or catching of the distal radioulnar joint (DRUJ) may be present.

Often, a history of swelling is reported on the dorsum of the hand and wrist as a result of extensor tenosynovitis and underlying joint synovitis that may have been present for months to years. If medical attention is not sought in a timely fashion, patients may note that active MCP extension of the ring finger, long finger, and, finally, index finger is lost in succession. [9] A study that included 29 patients with multiple extensor tendon ruptures due to rheumatoid arthritis (RA) found that the mean duration between the first tendon rupture and involvement of the subsequent digit was 2.9 months. [28]

It is important to elicit a history of wrist and MCP joint pain or dysfunction that preceded the inability to extend the digits. Anatomic abnormalities of these articulations may influence the choice of available surgical reconstruction.

Warning signs of impending tendon rupture include the following:

  • MCP joint extensor weakness
  • Extension lag
  • Active tenosynovitis on the dorsum of the hand and wrist
  • Pain on the dorsum of the wrist associated with digital extension
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Physical Examination

The MCP joints are among the most commonly affected articulations in patients with RA; subluxation of the extensor tendons and subluxation of the joints are particularly common deformities. In patients with RA who have relatively normal MCP joints, loss of active extension is clinically obvious. Extensor tendon subluxation is usually visible, and patients with these deformities are often able to maintain MCP extension once the joint is passively positioned, a finding that further distinguishes such patients from those with tendon rupture. (See the image below.)

Typical appearance of the hand following rupture o Typical appearance of the hand following rupture of the extensor tendons of the ring and small finger.

The presence of a fixed deformity that cannot be passively corrected and radiographic findings confirming MCP joint subluxation or dislocation suggest that treatment of the joints is required in conjunction with treatment of the tendon rupture. Patients with significant fixed MCP deformity may require exploration of the tendons on the dorsum of the hand and wrist at the time of joint reconstruction to determine if the tendons are intact. [9, 29]

The DRUJ usually must be reconstructed at the time tendon continuity is restored. Range of motion (ROM), pain, synovitis, and instability of this joint must be documented. Wrist motion and stability must be evaluated because these findings may impact the choices made for surgical reconstruction of the DRUJ. Volar and ulnar subluxation of the carpus with respect to the radius and ulna often accompanies pathology involving the extensor tendons and the DRUJ. [23]

Posterior interosseous nerve (PIN) palsy caused by synovial proliferation at the elbow may also result in the loss of active MCP extension. [30] Nerve palsy does not usually produce the same pattern of loss of digital extension. When the PIN is involved, the ring and long fingers tend to lose extension first, and the tenodesis effect of wrist flexion resulting in MCP extension is still present. This finding can sometimes be difficult to demonstrate in the rheumatoid limb, in which passive ROM of the wrist or MCP joints may be limited by underlying disease.

Even after tendon rupture, associated synovitis may fix the distal tendon stump to adjacent soft tissues, resulting in an apparent tenodesis effect and further confounding efforts to distinguish these processes.

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