Vaughan-Jackson Syndrome Workup

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

Imaging Studies

Radiography

Plain radiographs are imperative to assess the status of the distal radioulnar joint (DRUJ) and the wrist. The degree of joint destruction, subluxation, or dislocation, as well as the presence of bony prominences that may be directly responsible for tendon attrition, can be determined by obtaining good-quality plain radiographs in three projections (posteroanterior [PA], lateral, and oblique). (See the images below.)

Posteroanterior radiograph of the wrist following Posteroanterior radiograph of the wrist following wrist arthrodesis and resection of the distal ulna displays the scallop sign, which is the term used to describe the scooped-out appearance of the sigmoid notch of the radius that results from synovial proliferation and bone erosion.
Radiograph of a rheumatoid hand with metacarpophal Radiograph of a rheumatoid hand with metacarpophalangeal joint dislocations. These joints are incapable of active or passive extension.

Radiographic and clinical evaluation of the radiocarpal joint is necessary because the condition and function of this articulation may influence the choice of reconstructive options for the DRUJ. [31] If a mobile wrist is to be maintained, simple excision of the distal ulna may not be advisable, and alternatives (eg, the Sauve-Kapandji procedure) may help prevent or delay subsequent ulnar translation of the carpus. [32, 33]

Because the purpose of reestablishing extensor tendon function is to restore active metacarpophalangeal (MCP) joint extension, the MCP joints should be evaluated radiographically. Most authors would suggest that the reconstruction or replacement of badly damaged MCP joints should precede tendon restoration, though this point can be debated. Regardless of whether one favors primary MCP arthroplasty or combined tendon reconstruction and arthroplasty, adequate planning is impossible unless the condition of the MCP joints is known.

MRI, ultrasonography, and CT

Although magnetic resonance imaging (MRI) depicts effusion, synovitis, and even tendon involvement quite accurately, it has not proved to be predictive in the risk assessment of tendon rupture. [34]

Both ultrasonography and three-dimensional (3D) computed tomography (CT) have been utilized to demonstrate tendon ruptures on the dorsum of the hand and wrist and to evaluate the risk for potential tendon rupture. These techniques have not yet progressed to the point where they can provide a reliably accurate identification of tendons at risk for rupture. [35, 36, 37, 38, 39, 40]

Proper diagnosis can almost always be made on the basis of careful physical examination and plain radiography. Advanced imaging seldom influences the decision for surgery or the procedure itself; therefore, routine use of these studies is not currently advocated.

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Preoperative Evaluation

Thorough preoperative evaluation must precede any surgical procedure. This is particularly important in the case of the patient with rheumatoid disease who may be significantly debilitated. Rheumatoid arthritis (RA) is something of a misnomer; it is a systemic disease that may produce cardiac, pulmonary, and other organ system dysfunction.

Preoperative laboratory, cardiac, and respiratory evaluation is most appropriately directed by the primary care provider; the rheumatologist may function in this capacity. Other subspecialty evaluation may also be necessary because of specific organ system involvement. [41]

Medications used to treat rheumatoid disease may produce significant hematologic, hepatic, or renal effects that must be evaluated preoperatively. Certain medications (eg, corticosteroids, penicillamine, and methotrexate) may alter wound-healing potential and possibly increase the risk of infection.

Tumor necrosis factor (TNF) antagonists have improved the lives of many patients who have RA, but they are associated with an increased risk of opportunistic infection. Data regarding possible increased risk of postoperative infection in patients treated with these agents are scant and sometimes conflicting. [42]

It may be advantageous to adjust the dosing regimen of these agents in the perioperative period, though evidence-based recommendations cannot be made at this time. The desire to avoid surgical complications must be balanced against the fact that a flare of the rheumatoid process in the perioperative period from alterations in medication can also contribute significantly to morbidity. Rheumatologic consultation is recommended.

Rheumatoid involvement of the cervical spine leading to instability is a common occurrence that must be anticipated preoperatively. Even when regional anesthesia is planned in cases of upper-extremity reconstruction, the possibility always exists that general anesthesia and tracheal intubation will be necessary. Preoperative evaluation of cervical spine stability will allow appropriate precautions to be taken and potentially devastating complications to be avoided. [41]

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