Case Study Demonstrates Viability of Surgical Reconstruction for Vaughan-Jackson Syndrome

The case showcases that surgical intervention, including techniques like distal ulna resection, ECU tendon stabilization, and tendon transfer, can effectively address the functional limitations caused by Vaughan-Jackson syndrome. The case study is scheduled for publication in the Oct-Dec 2024 issue of Apollo Medicine and is available online ahead of its print release. 

Vaughan-Jackson syndrome is described as a gradual rupture of the extensor tendons resulting from tendon attrition at the wrist. Rheumatoid arthritis and osteoarthritis are commonly linked to it. Vaughan-Jackson syndrome causes significant functional impairment and negatively impacts the quality of life and functional impairment.

“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,” the lead author Dr J Terrence Jose Jerome, Department of Orthopedics, Hand, and Reconstructive Microsurgery, Olympia Hospital & Research Centre, Trichy, Tamil Nadu, told Medical Dialogues.

Furthermore, he added that “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.”

Due to the lack of established treatment guidelines for this rare condition, it is necessary to explore and refine surgical techniques. Therefore, Dr Jose Jerome and colleagues aimed to showcase a successful surgical technique in patients presenting with Vaughan-Jackson Syndrome.

In patients, Vaughn-Jackson lesion was indicated by the radiological scalloping sign and was confirmed through surgical observations, including tenosynovitis of the extensor tendons, tendon thinning, synovitis of the distal radioulnar joint (DRUJ), and ruptures of the ulnar extensor tendons.

For the surgery informed consent was taken from all the patients, and surgical interventions including distal ulna resection, dorsal tenosynovectomy, and reconstruction of extensor tendon rupture were performed successfully in patients.

After surgery, the patient was immobilized with a wrist splint for 4 weeks. Once the splint is removed, a therapy program that includes both passive and active-assisted exercises for the fingers and wrist extensors begins. Night splints were used for another 2 weeks. By the sixth week post-surgery, the patient is allowed full range of motion and no longer needs splinting.

For the assessment of outcomes simplified scoring system, adapted from the authors' Radial Nerve Palsy Tendon Transfer Outcome Score was employed. The DASH score was also utilized to evaluate the overall functional result.

In the case report, the author concluded that surgical management should be considered a viable option for patients with Vaughan-Jackson syndrome. By addressing tendon rupture, joint instability, and ulnar prominence, surgery can improve hand function and quality of life. This has implications for clinical practice, encouraging clinicians to consider surgical intervention when appropriate.

“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,” said the lead author Dr Jose Jerome.

Reference: Jerome JTJ, Karunanithi D. Extensor Tendon Reconstruction in Vaughan–Jackson Syndrome: Surgical Technique and a Case Illustration. Apollo Medicine. 2024;0(0). doi:10.1177/09760016241259845