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.
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Differential Diagnosis
Vaughan-Jackson (Caput Ulna) Syndrome is rupture of the extensor tendons usually starting with the extensors to the fifth 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 rupture 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 tendons structural integrity.
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.
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