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Vaughan-Jackson Syndrome Clinical Presentation

  • Author: John A McAuliffe, MD; Chief Editor: Harris Gellman, MD  more...
 
Updated: Feb 01, 2016
 

History

Patients usually present with the 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.[27]

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, further distinguishing them from the patient 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, 28]

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. Evaluate wrist motion and stability, 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.[22]

Posterior interosseous nerve (PIN) palsy caused by synovial proliferation at the elbow may also result in the loss of active MCP extension.[29] 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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Contributor Information and Disclosures
Author

John A McAuliffe, MD Consulting Surgeon, Department of Orthopedics, Section of Hand Surgery, Broward Health Medical Center

John A McAuliffe, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Society for Surgery of the Hand

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

N Ake Nystrom, MD, PhD Associate Professor of Orthopedic Surgery and Plastic Surgery, University of Nebraska Medical Center

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine, Clinical Professor, Surgery, Nova Southeastern School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Arkansas Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

Joseph E Sheppard, MD Professor of Clinical Orthopedic Surgery, Chief of Hand and Upper Extremity Service, Department of Orthopedic Surgery, University of Arizona Health Sciences Center, University Physicians Healthcare

Joseph E Sheppard, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Orthopaedics Overseas, American Society for Surgery of the Hand

Disclosure: Nothing to disclose.

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Typical appearance of the hand following rupture of the extensor tendons of the ring and small finger.
Intraoperative image of a ruptured extensor tendon with the hand to the left. Note that the tendon ends cannot be reapproximated despite maximum tension.
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 metacarpophalangeal joint dislocations. These joints are incapable of active or passive extension.
 
 
 
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