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EXTENSOR TENDON RUPTURE (VAUGHAN-JACKSON SYNDROME)

Introduction

Vaughan-Jackson syndrome, as the eponym suggests, was first described by OJ Vaughan-Jackson in elderly laborers with degenerative arthritis of the distal radioulnar joint (DRUJ). Ten years later, he described the process of attritional rupture of the digital extensor tendons in the rheumatoid hand, with which his name has become associated. Rheumatoid arthritis (RA) is the most common underlying etiology of tendon rupture in the hand and wrist. As the tendons generally rupture in an ulnar-to-radial direction, the condition is also frequently referred to as Caput Ulnae syndrome.

Related Anatomy

  • Extensor tendons of the fingers
  • DRUJ
  • MP joints of fingers

Pathophysiology

  • In RA, tendons may subluxate or dislocate dorsally due to the loss of normal supporting structures, which in turn leads to gradual attrition of tendons on the ulnar head.
  • Tendon wear may be exacerbated by osteophytes and sharp prominences resulting from primary or secondary degenerative changes of the bone.
  • Rheumatoid tenosynovitis affects tendons and interferes with normal blood supply to the tendons.

Incidence and Related Conditions

  • Vast majority of tendon ruptures occur in patients with RA, and the incidence of extensor tendon involvement is 10–15 times that of the flexor tendon involvement.
  • In patients with RA, tendon ruptures have been seen in the disease for ≤2 years to as long as 25 years; in most series, RA has been present for 10–15 years.

Differential Diagnosis

  • Extensor tendon subluxation at the metacarpophalangeal (MP) joint
  • MP joint subluxation or dislocation
  • Posterior interosseous nerve (PIN) palsy

Vaughan-Jackson (Caput Ulnae) Syndrome is rupture of the extensor tendons usually starting with the extensors to the little finger and ring finger. Osteoarthritis, or in the past commonly Rheumatoid Arthritis, causes excessive dorsal tenosynovitis and DRUJ degenerative changes which can attritionally rupture the extensor tendons. The EDM and EDC V and IV which are immediately dorsal to the deformed and dorsally subluxating distal ulna are most at risk. There are three suggested causes for the extensor tendon rupture: 1. The damaged sharp edges of the dorsally displaced head of the distal ulna cut the tendons by repeated rubbing of the tendons over the sharp edges which also ruptures the dorsal capsule of the DRUJ; 2. The dorsal tenosynovitiis directly attacks and weakens the extensor tendons until they ruputure under the ordinary load of finger extension; 3. In the case of Rheumatoid Arthritis, rheumatoid nodules can develop in the substance of the extensor tendon and destroy the tendon's structural integrity.

ICD-10 Codes

  • CAPUT ULNA SYNDROME (VAUGHAN-JACKSON SYNDROME)

    Diagnostic Guide Name

    CAPUT ULNA SYNDROME (VAUGHAN-JACKSON SYNDROME)

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    CAPUT ULNA SYNDROME (VAUGHAN-JACKSON SYNDROME) M24.832M24.831 

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
  • Extensor Tendon Ruptures (Vaughan-Jackson Syndrome)
    Extensor Tendon Ruptures (Vaughan-Jackson Syndrome)
  • No Tendodesis Effect demonstrated in 4th & 5th fingers with wrist in palmar flexion
    No Tendodesis Effect demonstrated in 4th & 5th fingers with wrist in palmar flexion
  • Caput Ulnae Syndrome (Vaughan-Jackson Syndrome); Red arrow - Dorsal dislocated distal ulna; Orange Arrow - Distal ends of ruptured extensor tendons and rheumatoid dorsal tenosynovitis.
    Caput Ulnae Syndrome (Vaughan-Jackson Syndrome); Red arrow - Dorsal dislocated distal ulna; Orange Arrow - Distal ends of ruptured extensor tendons and rheumatoid dorsal tenosynovitis.
Symptoms
Cannot extend ring & little fingers at the MP Joints
Pain, instability, or catching of the distal radioulnar joint (DRUJ) may be present
Typical History

The patient is a 43-year-old female who was diagnosed with rheumatoid arthritis 12 years ago.  Despite careful follow-up with her rheumatologist, her rheumatoid arthritis has been difficult to control.  Numerous medications (DMARD’s) were tried but she could not tolerate them.  For three months there has been persistent swelling on the dorsum of the right wrist and hand.  Two months ago she noted trouble straightening her fifth finger.  Ten days ago she felt a mild popping sensation and now cannot extend her ring and little fingers.  Twisting her forearm causes a click and pain at the ulnar side of the wrist.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
  • DRUJ OA (arrow) & Thumb CMC OA
    DRUJ OA (arrow) & Thumb CMC OA
  • DRUJ post Distal Ulna Resection
    DRUJ post Distal Ulna Resection
Treatment Options
Conservative
  • Conservative: continued medical management of RA is critical.
  • Preoperative laboratory, cardiac, and respiratory evaluation also important.
  • Evaluation of medications and drug options should be done by rheumatologists.
Operative
  • Prophylaxis: effective and provides function that is superior to that provided by any method of tendon repair/reconstruction. Patients with risk factors or persistent dorsal tenosynovitis that is unresponsive to medical management over a 6-month period should be considered candidates for such surgery.
  • Tendon continuity restoration: includes thorough dorsal tenosynovectomy with retinaculum transposition and resection or reconstruction of the DRUJ sufficient to remove the bony prominences that produced the tendon rupture.
  • Direct repair: rarely possible because of the attritional nature of this process and because the zone of tendon injury is usually quite long.
  • Bridge grafts: generally acceptable results; however, it has not gained widespread acceptance because tendon graft harvest may require additional operative time and surgical exposure.
  • Tendon transfer: most common method and often the best surgical alternative. Challenges include the possibility that the joints may become stiff or unstable, tendon beds may be less than ideal, motor tendons may have disease involvement or may be unavailable, and tenodesis may be limited by wrist stiffness. Method of transfer is determined primarily by the number of digits involved. 
Treatment Photos and Diagrams
  • Retinacular Flaps Made & Intact Extensors Exposed
    Retinacular Flaps Made & Intact Extensors Exposed
  • Retinacular Flaps and DRUJ Capsule
    Retinacular Flaps and DRUJ Capsule
  • Intact tendons with excess tenosynovium
    Intact tendons with excess tenosynovium
  • EDM Synovial Sheath with no Tendon
    EDM Synovial Sheath with no Tendon
  • Attritional defect in DRUJ Capsule which allowed arthritic ulnar head to rupture the extensor tendons( EDM, EDC V , EDC IV and partially rupture EDC III)
    Attritional defect in DRUJ Capsule which allowed arthritic ulnar head to rupture the extensor tendons( EDM, EDC V , EDC IV and partially rupture EDC III)
  • Extensor tendon ends proximally and distally with ulnar head exposed
    Extensor tendon ends proximally and distally with ulnar head exposed
  • Vaughan-Jackson Syndrome
    Vaughan-Jackson Syndrome
  • Arthritic Ulnar Head exposed with osteophytes that acted as sharp teeth which ruptured the extensor tendons
    Arthritic Ulnar Head exposed with osteophytes that acted as sharp teeth which ruptured the extensor tendons
  • Distal ulna has been removed by an osteotomy at the ulnar neck (Darrach procedure)
    Distal ulna has been removed by an osteotomy at the ulnar neck (Darrach procedure)
  • Resected arthritic ulnar head
    Resected arthritic ulnar head
  • Soft tissue pathology specimen
    Soft tissue pathology specimen
  • Vaughan-Jackson Syndrome Treatment by Adjacent Tendon Transfer and EIP Transfer
    Vaughan-Jackson Syndrome Treatment by Adjacent Tendon Transfer and EIP Transfer
  • Vaughan-Jackson Syndrome Treatment by Adjacent Tendon Transfer
    Vaughan-Jackson Syndrome Treatment by Adjacent Tendon Transfer
  • Flexor tenodesis now intact
    Flexor tenodesis now intact
CPT Codes for Treatment Options

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Common Procedure Name
Tendon transfer
CPT Description
Tendon transplant or transfer flexor/extensor forearm and/or wrist, single each tendon
CPT Code Number
25310
Common Procedure Name
Extensor tendon repair (with graft)
CPT Description
Repair, tendon or muscle, extensor, secondary with tendon graft, forearm
CPT Code Number
25274
Common Procedure Name
Excision distal ulna or Darrach, wafer procedure or excision ulnar styloid fragment
CPT Description
Excision distal ulna (Darrach procedure)
CPT Code Number
25240
Common Procedure Name
Extensor tenosynovectomy (dorsal)
CPT Description
Synovectomy, extensor tendon sheath, wrist, single compartment
CPT Code Number
25118
CPT Code References

The American Medical Association (AMA) and Hand Surgery Resource, LLC have entered into a royalty free agreement which allows Hand Surgery Resource to provide our users with 75 commonly used hand surgery related CPT Codes for educational promises. For procedures associated with this Diagnostic Guide the CPT Codes are provided above. Reference materials for these codes is provided below. If the CPT Codes for the for the procedures associated with this Diagnostic Guide are not listed, then Hand Surgery Resource recommends using the references below to identify the proper CPT Codes.

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Complications
  • Wound healing problems and infection are encountered in <5% of cases.
  • Extension lag at the MP joint is a frequently mentioned occurrence, although its incidence and magnitude are difficult to quantify.
  • Rerupture occurs in <5% of cases and is usually related to inadequate reconstruction of the distal ulna, which allows recurrence of dorsal displacement and attritional tendon wear.
  • Recurrent tenosynovitis occurs in <7% of cases after 3 to 8 years of follow-up after tenosynovectomy.
Outcomes
  • Hands with single-digit tendon ruptures show better outcomes than do those with multiple-digit involvement. In particular, extension lag appears to increase in direct proportion to the number of digits involved. A functional 70° arc of MP joint motion is commonly restored.
Key Educational Points
  • The extensor digitorium quinti (EDQ) and extensor digitorum communis (EDC) tendons to the ring and little fingers are the most susceptible to rupture.
References

New Articles

  1. Gong HS, et al. Extensor tendon rupture in rheumatoid arthritis: a survey of patients between 2005 and 2010 at five Korean hospitals. Hand Surg2012;17(1):43-7. PMID: 22351532
  2. Divani K, Subramanian P, Goldie B. Avoiding extensor tendon rupture after the use of palmar locking plates for distal radial fractures. J Hand Surg Eur 2015;40(2):215-6. PMID: 24194612

Reviews

  1. Abe A, et al. Extensor tendon rupture and three-dimensional computed tomography imaging of the rheumatoid wrist. Skeletal Radiol 2010;39(4):325-31. PMID: 19662401

Classics

  1. Clayton ML, et al. Extensor tendon rupture over the metacarpal heads. Hand 1983;15(2):149-50. PMID: 6884843
  2. Harvey FJ, Harvey PM. Three rare causes of extensor tendon rupture. J Hand Surg Am 1989;14(6):957-62. PMID: 2584656
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