Rheumatoid arthritis (RA) is a chronic autoimmune disorder that primarily affects joints, causing pain, swelling, stiffness, and eventual joint damage. It occurs when the immune system mistakenly attacks healthy tissues, particularly the synovium (the lining of joints), leading to inflammation and joint destruction over time. Many complications are associated with RA, such as joint pain, osteoporosis, cardiovascular disorders, lung disorders, eye problems, vasculitis, and anemia. One of the rare complications associated with rheumatoid arthritis is Vaughan–Jackson Syndrome.
Vaughan–Jackson Syndrome is a rare condition characterised by the gradual rupture or degeneration of the extensor tendons in the fingers, typically starting with the little finger and progressing to others. This process is caused by chronic inflammation in the joints and tendons. Treatment for this syndrome often involves tendon repair or grafting to restore finger function.
The development of the extensor tendon reconstruction treatment plan was likely prompted by the need to address the specific challenges posed by Vaughan-Jackson syndrome. The combination of tendon rupture, joint involvement, and ulnar head prominence requires a comprehensive surgical approach.
Dr J Terrence Jose Jerome.
In a case study by J. Terrence Jose Jerome and Dharani Karunanithi, the authors highlighted and presented surgical techniques used for Vaughan–Jackson Syndrome. Their focus was on dorsal tenosynovectomy, distal ulnar resection, and extensor tendon reconstruction. This study aims to educate medical professionals on the surgical techniques used for this syndrome, as it is rare and poorly understood.
Vaughan–Jackson Syndrome typically presents with a sudden, painless loss of extension in the ulnar fingers. The most commonly ruptured tendons are those of the little finger, followed by the ring finger, with the ruptures progressing in descending order. Radiological signs of Vaughan–Jackson Syndrome include the "scalloping sign." Key indicators of this syndrome are extensor tendon ruptures, tenosynovitis, and involvement of the DRUJ, along with radiological evidence of Vaughan–Jackson lesions.
Contraindications to surgery include the risk of perivascular inflammation, boggy synovium, and intrinsic muscle contractions. Skin ulceration or thinning due to vasculitis also requires careful consideration before proceeding with surgery. The surgical anatomy of Vaughan–Jackson Syndrome focuses on the dorsum of the wrist, where the extensor tendons traverse through six compartments on the distal radius. Each tendon is enveloped by synovium, which becomes inflamed in rheumatoid arthritis, leading to tenosynovitis. Over time, synovitis causes bone erosion, ligament attenuation, and tendon weakening. Weakened tendons are prone to rupture, severely impacting wrist and hand function.
The significance of the case report lies in its contribution to the limited literature on Vaughan-Jackson syndrome, offering valuable insights for clinicians managing this complex condition.
In Vaughan–Jackson Syndrome, the DRUJ is particularly vulnerable, as it often shows signs of instability and erosion. The rupture of the ulnar extensor tendons, such as the extensor digiti minimi and extensor carpi ulnaris (ECU), is a common feature. The triangular fibrocartilage complex (TFCC) may also be damaged, further destabilizing the DRUJ and leading to conditions like caput ulnae syndrome, where dorsal displacement of the ulna and volar subluxation of the ECU tendon occur.
Due to the lack of well-established treatment guidelines for Vaughan–Jackson Syndrome, it is essential to study such cases and surgical processes for greater understanding.
The surgical management of Vaughan–Jackson Syndrome involves several key steps. The procedure begins with the patient in a supine position, and surgery is typically performed under supraclavicular brachial block anesthesia. A curvilinear incision is made along the dorsal aspect of the wrist, extending over the DRUJ. Skin flaps are carefully elevated to avoid damaging the sensory branches of the radial and ulnar nerves.
The extensor retinaculum is divided into two flaps: a proximal flap to accommodate distal ulna resection and a distal flap to protect the tendon reconstruction. The ruptured tendons are identified and repaired, often requiring tendon transfers or grafting. The distal ulna is resected to a level just proximal to the sigmoid notch of the radius, with care taken to avoid excessive resection, which could lead to further instability.
The ECU tendon is often affected in Vaughan–Jackson Syndrome, with volar subluxation being a common problem. To address this, the ECU tendon is split longitudinally, and a portion of the tendon is used for tenodesis to stabilize the DRUJ. The retinacular flaps are used to protect the reconstructed tendons and prevent bowstringing.
In cases where tendon damage is extensive, tendon transfers are performed. The extensor indicis proprius (EIP) tendon is often used as a donor for transferring to the ruptured extensor digiti minimi or extensor digitorum communis tendons. The tension in the tendon transfers is carefully adjusted to ensure proper hand function, with the wrist positioned in extension during the procedure to optimize tendon length and function.
After surgery, the patient’s wrist is immobilized in a splint for approximately four weeks. Once the splint is removed, passive and active-assisted therapy is initiated to restore finger and wrist function. Night splints are prescribed for an additional two weeks to prevent postoperative stiffness. By the sixth week, patients are usually able to regain full range of motion and no longer require splinting.
The outcomes of the surgical technique are assessed using a scoring system modified from the Radial Nerve Palsy Tendon Transfer Outcome Score. Additionally, the DASH score (Disabilities of the Arm, Shoulder, and Hand) is used to evaluate overall functional recovery.
The paper provides a detailed case illustration of a 62-year-old woman with long-standing rheumatoid arthritis who presented with sudden loss of extension in her ring and little fingers. Radiographs revealed distal radioulnar erosion and DRUJ arthritis, along with partial carpal fusion. The patient underwent surgical exploration, during which the ruptured tendons were identified and repaired using tendon transfers. The distal ulna was resected, and the ECU tendon was stabilized using tenodesis. Postoperatively, the patient regained full range of motion in her wrist and fingers and reported a DASH score of 4.5 at the 12-month follow-up, indicating excellent functional recovery.
Alternative treatment options for Vaughan-Jackson syndrome are limited. While non-surgical approaches like splinting and medication may provide temporary relief, they do not address the underlying structural issues,
While the surgical technique described in the paper is effective for managing Vaughan–Jackson Syndrome, potential complications include tendon ruptures, tendon adhesions, insufficient distal ulna resection, and recurrent tenosynovitis. The authors emphasise the importance of careful tension adjustment during tendon transfers to prevent postoperative stiffness and loss of flexion.
Vaughan–Jackson Syndrome is a rare complication of rheumatoid arthritis, causing extensor tendon rupture in the fingers due to chronic inflammation. A case study by J. Terrence Jose Jerome and Dharani Karunanithi explored surgical techniques, including dorsal tenosynovectomy, distal ulnar resection, and tendon reconstruction using tendon transfers like the extensor indicis proprius (EIP). The study’s patient regained full hand function post-surgery, with a DASH score of 4.5 after 12 months. Early diagnosis and intervention are critical for preventing further damage, though potential complications like tendon adhesions remain. Further research is needed to refine treatment strategies.
In conclusion, surgical intervention for Vaughan–Jackson Syndrome, involving tendon reconstruction, distal ulnar resection, and ECU tenodesis, is a viable option for restoring hand and wrist function in patients with this rare condition. The paper highlights the importance of early diagnosis and surgical management to prevent further tendon damage and loss of function. Further studies and long-term follow-up are needed to validate these findings and refine treatment strategies for this complex condition.
Reference :
1. Jerome, J. T. J., & Karunanithi, D. (2024). Extensor Tendon Reconstruction in Vaughan–Jackson Syndrome: Surgical Technique and a Case Illustration. Apollo Medicine. https://doi.org/10.1177/09760016241259845
(Input from various sources)
(Rehash/Yash Kamble/MSM)
