Vaughan-Jackson-like syndrome as an unusual presentation of Kienböck's disease: a case report

Abstract

IntroductionKienböck's disease is a condition of osteonecrosis of the lunate bone in the hand, and most patients present with a painful and sometimes swollen wrist with a limited range of motion in the affected wrist. Vaughan-Jackson syndrome is characterized by the disruption of the digital extensor tendons, beginning on the ulnar side with the extensor digiti minimi and extensor digitorum communis tendon of the small finger. It is most commonly associated with rheumatoid arthritis. We describe a case of a patient with an unusual presentation of Kienböck's disease with symptoms similar to those of Vaughan-Jackson syndrome.Case presentationA 40-year-old man of Indian ethnic origin with no known history of trauma presented to our clinic with a ten-day history of an inability to extend his right little and ring fingers with associated pain in his right wrist. He was being treated with long-term steroids but had no other significant medical history. His examination revealed an inability to extend the metacarpal and phalangeal joints of the right ring and little fingers with localized tenderness over the lunate bone. Spontaneous disruption of the extensor tendons was diagnosed clinically and, after radiological investigation, was confirmed to be secondary to dorsal extrusion of the fragmented lunate bone. The patient underwent surgical repair of the tendons and had a full recovery afterward.ConclusionKienböck's disease, though rare, is an important cause of spontaneous extensor tendon rupture. The original description of Vaughan-Jackson syndrome was of rupture of the extensor tendons of the little and ring fingers caused by attrition at an arthritic inferior radioulnar joint. We describe a case of a patient with Kienböck's disease that first appeared to be a Vaughan-Jackson-like syndrome.

“Butterfly flap”: The retinaculum to the rescue of the extensors

“…Etiology is multiple, but mainly involves inflammatory or degenerative arthropathy of the distal radio-ulnar joint: rheumatoid arthritis [2], chondrocalcinosis [3], gout [4], Madelung's disease [5] or, more rarely, Kienböck's disease [6] or dorsal carpal osteophyte [7].…”

La plastie papillon : le rétinaculum au secours des extenseurs

“…Les étiologies de ces ruptures sont multiples, dominées par les arthropathies inflammatoires ou dégénératives de l'articulation radio-ulnaire distale (RUD) : la polyarthrite rhumatoïde [2], la chondrocalcinose [3], la goutte [4], la maladie de Madelung [5], plus rarement : maladie de Kienböck [6], ostéophyte du carpe dorsal [7].…”

Rotura espontánea del aparato extensor secundaria a artrosis de la articulación radiocubital distal

“…Mean age 73 years (66-81). Mean follow-up five years (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). In 7 cases multiple tendon involvement, mainly those responsible for the middle, ring and little fingers were seen.…”

“…La rotura de los tendones extensores de la mano a nivel de la articulación radio-cubital distal es un problema relativamente frecuente en pacientes con artritis reumatoide 1 . Sin embargo, es menos frecuente en alteraciones locales de la articulación, como las secundarias a artrosis 2 , secuelas de fractura, deformidad congénita 3 o enfermedad de Kienböck 4,5 .…”

“…Unión (sutura tipo pulvertaft) extensor común 3.º dedo con extensor común 2.º dedo Unión (sutura tipo pulvertaft) extensores 5.º dedo y extensor común 4.º y parcial 3.º a flexor superficial 4.º dedo transpuesto Rotura con afectación tendinosa del 3. er , el 4.• y el 5.• dedos correspondiente al caso número 6. mediante resección cubital distal presentaban inestabilidad del muñón distal. No existió ningún caso de reintervención por nueva rotura al final del periodo de estudio, con 5 años de seguimiento medio(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”