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

<p>Abstract</p> <p>Introduction</p> <p>Kienböck'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.</p> <p>Case presentation</p> <p>A 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.</p> <p>Conclusion</p> <p>Kienböck'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.</p

J Fr Ophtalmol

Contrôler l’inflammation à long terme au cours des uvéites intermédiaires, postérieures ou totales, non infectieuses en limitant la iatrogénie est un véritable défi. Il n’existe pas de bilan standardisé qui assure une certitude diagnostique, mais quelques tests simples permettent de ne pas omettre les principales étiologies. L’ophtalmologiste qualifie le type d’atteinte puis le médecin interniste complète les investigations en fonction des constatations de l’ophtalmologiste. Photographies du fond d’œil, tomographies en cohérence optique, angiographies à la fluorescéine et au vert d’indocyanine doivent être envisagées au cours du diagnostic et du suivi. Les complications oculaires de l’inflammation sont nombreuses. Elles nécessitent une surveillance étroite et une prise en charge spécifique, médicale et parfois chirurgicale. Le nombre croissant de molécules disponibles permet d’optimiser la prise en charge de ces pathologies aux étiologies et présentations variées. Actuellement, la corticothérapie systémique reste le traitement de référence et les autres thérapeutiques sont envisagées en cas de mauvaise tolérance, corticorésistance ou corticodépendance. Le choix d’un traitement systémique, régional périoculaire ou intravitréen dépend de plusieurs facteurs : chronicité ou récurrence de l’uvéite, ancienneté, bilatéralité, association à une maladie inflammatoire systémique, présence de contre-indications à certains traitements et contraintes socio-économiques. Il est très important de trouver le meilleur compromis permettant un contrôle strict de l’inflammation oculaire à l’aide d’un traitement systémique et/ou local adapté tout en évitant les principales complications.Controlling long-term inflammation during non-infectious intermediate, posterior or panuveitis while limiting side effects remains challenging. There is no standardized pre-therapeutic evaluation providing diagnostic certainty, but some simple tests allow us to identifiy the main etiologies. The ophthalmologist identifies the type of uveitis, and the internist completes the investigations according to the ophthalmologist's findings. Fundus photographs, optical coherence tomography, and fluorescein and indocyanine green angiography should be considered during diagnosis and follow-up. Ocular complications of uveitis are numerous. They require close monitoring and specific medical and sometimes surgical management. The growing number of available drugs makes it possible to optimize the management of these conditions with varied etiologies and presentations. Currently, systemic corticosteroids remain the mainstay of therapy, and other alternatives are considered in the case of poor tolerance, steroid resistance or dependence. The choice of a systemic, periocular or intravitreal treatment depends on several factors: chronicity or recurrence of uveitis, duration, bilaterality, association with a systemic inflammatory disease, the presence of contraindications to certain treatments, and also socioeconomic constraints. It is of the utmost importance to find the best compromise allowing tight control of ocular inflammation by means of adapted systemic and/or local treatment while avoiding the main complications