Vaughan-Jackson Syndrome 

  • Author: John A McAuliffe, MD; Chief Editor: Harris Gellman, MD   more...
 
Updated: Sep 17, 2010
 

Background

Vaughan-Jackson syndrome is the disruption of the digital extensor tendons, beginning on the ulnar side with the extensor digiti minimi (EDM) and extensor digitorum communis (EDC) tendon of the small finger. If the underlying pathology is not treated, sequential rupture of the ring, long, and index finger EDC tendons occurs; ultimately, rupture of the extensor indicis proprius (EIP) may follow.[1]

Rheumatoid arthritis is the most common underlying etiology of tendon rupture in the hand and wrist and is the usual clinical setting in which the term Vaughan-Jackson syndrome is used. O. J. Vaughan-Jackson's first report of extensor tendon rupture described 2 elderly laborers with degenerative arthritis of the distal radioulnar joint (DRUJ).[2] He found that the tendons were frayed and disrupted directly over a bony prominence projecting from the head of the ulna in both cases. In a subsequent report that appeared a decade later,[3] he described the process of attritional rupture of the digital extensor tendons in the rheumatoid hand, with which his name has become associated.

Other, less common, patterns seen in rheumatoid arthritis include rupture of the extensor pollicis longus (EPL) in the vicinity of Lister's tubercle within the third dorsal compartment[4] and rupture of the flexor pollicis longus and index finger's flexor digitorum profundus tendons within the carpal tunnel (ie, Mannerfelt syndrome).[5]

An image depicting Vaughan-Jackson syndrome can be seen below.

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

Tendon ruptures in Vaughan-Jackson syndrome are primarily caused by gradual attrition of the ulnar head, which, in rheumatoid arthritis, may subluxate or dislocate dorsally due to loss of the normal supporting structures. The process of tendon wear is further accentuated when osteophytes and sharp prominences result from primary or secondary degenerative changes of the bone. Attrition of the unstable distal ulnar stump following partial ulnar excision (Darrach procedure) has also been described as a cause of digital extensor tendon rupture.[6, 7]

Direct invasion of the tendons and interference with normal tendon blood supply from rheumatoid tenosynovitis have been implicated as contributing causative factors. The cascade of wrist and DRUJ deformities produced by rheumatoid arthritis often results in palmar and ulnar subluxation of the extensor carpi ulnaris (ECU) tendon, taking it out of harm's way. The ECU and stout radial wrist extensors are seldom ruptured.[4, 8, 9]

A rupture of the extensor pollicis longus (EPL) tendon within the third dorsal compartment is occasionally seen as a complication following minimally displaced fracture of the distal radius in patients without rheumatoid arthritis. Other mechanical factors unrelated to rheumatoid disease that have been described as causes of extensor tendon rupture include abnormalities of the ulnar head from either traumatic subluxation or Madelung deformity; deformity of the radius or ulna following fracture; and orthopedic hardware on the dorsal surface of the distal radius. Bony prominences and local inflammatory changes resulting from both Kienbock disease and calcium pyrophosphate dihydrate crystal deposition disease (pseudogout) also have resulted in tendon rupture.[10, 11, 12, 13, 14, 15, 16]

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Epidemiology

Frequency

United States

The vast majority of tendon ruptures occur in patients with rheumatoid arthritis, and the incidence of extensor tendon involvement is 10-15 times that of involvement of the flexor tendons. Although some studies have reported tendon rupture to be more common in the dominant hand, this has not been a universal finding.[4]

Mortality/Morbidity

Patients with rheumatoid arthritis are remarkably adept at coping with hand deformity and dysfunction. In patients with relatively normal metacarpophalangeal (MCP) joint function, loss of the ability to extend the digits and open the hand to grasp large objects imparts obvious functional limitations. Other individuals with severe MCP joint deformity or subluxation may not even be aware that extensor tendon rupture has occurred.

Age

Tendon rupture caused by osteoarthritis, although relatively rare, is more common in older persons, whereas no age restriction exists for tendon rupture caused by rheumatoid arthritis. The pattern of rheumatoid joint involvement can vary dramatically among individuals, sometimes even from one extremity to the other in the same patient. Tendon ruptures have been seen in patients who have had rheumatoid arthritis for as little as 2 years to as long as 25 years, although in most series, rheumatoid disease has been present for an average of 10-15 years.[17]

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Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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, American Society for Surgery of the Hand, and Orthopaedics Overseas

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

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

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

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

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, and Arkansas Medical Society

Disclosure: Nothing to disclose.

References

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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.
Anteroposterior 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 extension.
 
 
 
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