Idiopathic Avascular Necrosis of the Scaphoid: Preiser's Disease

Treatment options for patients with Preiser's disease are challenging and varied. This article thus provides a systematic review of existing studies on the outcomes of the treatments for patients suffering from Preiser's disease in order to investigate the most appropriate management of Preiser's in each stage of the disease.

We followed PRISMA guidelines while performing the study, and reviewed 107 papers in all languages from 1981 till November 2020 and included 42 studies that met the eligibility criterion. Studies investigating the outcome of one or more treatment options for Preiser's disease were eligible. Besides, quantitative analysis on 130 individuals (135 wrists) of the included studies was performed.

The results show that in stages II and III, pain score reduces more in surgical approach than in conservative treatment. In stage II of the Preiser's, Vascularized Bone Grafting (VBG) was more effective in pain alleviation and improvement in wrist range of motion (ROM) and Mayo Modified Wrist Score (MMWS) than immobilization. Proximal Row Carpectomy (PRC) in stage III causes more pain relief and ROM improvement than VBG and conservative treatments like prescribing NSAIDs. Inconsistent evidence was found in case studies of stage IV; however, they generally favour PRC and SILASTIC implants. Surgical studies of stage I are not yet enough for making a conclusive assessment.

Surgical treatment outcomes seem more satisfactory in all Preiser's disease stages. Specifically, VBG for patients of stage II provided they have no scaphoid degenerative changes, and PRC seems more satisfactory for stage III.

IV; systematic review of case reports and case series on Preiser's disease.

There is no established treatment standard for patients with idiopathic avascular necrosis of the scaphoid, also known as Preiser Disease. We evaluated outcomes of operative interventions performed for patients diagnosed with Preiser Disease and assessed scaphoid morphology in the contralateral wrists.

We performed a retrospective review of all patients undergoing surgery for Preiser disease between 1987 and 2019 at our institution. A total of 39 wrists in 38 patients were identified. The mean age was 37 years at the time of surgery, and the median follow-up time was 5.3 years. The patients were classified according to the Herbert and Kalainov classifications. Pre- and postoperative pain and functional outcomes were evaluated, and Mayo Wrist Scores were calculated. Reoperations for complications were recorded. Scaphoid shapes were assessed for wide/type 1 and slender/type 2 scaphoids in the contralateral unaffected wrist in patients with unilateral disease.

Overall, pain and Mayo Wrist Scores improved, while flexion/extension decreased slightly and grip strength remained stable. In a comparison of the 2 main surgery groups, 17 wrists with a pedicled vascular bone graft and 12 wrists with salvage surgery (4-corner fusion/proximal row carpectomy) showed similar functional outcomes. Similar outcome scores were found regardless of preoperative Herbert or Kalainov classifications. Radiographic morphologic evaluation of the contralateral side determined that 4 of 8 patients had a slender scaphoid shape, which has been shown to have a more limited vascular network when compared to full scaphoids.

A treatment algorithm of Preiser disease is lacking and the optimal surgical treatment remains controversial. Pedicled vascular bone grafts had similar functional outcomes as salvage procedures, but preserving the scaphoid was possible in 70% of the pedicled vascular bone graft cases. A slender scaphoid is potentially more common in patients with Preiser disease who undergo surgery.

Therapeutic IV.

Preiser’s disease or aseptic necrosis of the scaphoid is a rare condition whose treatment, whether surgical or non-surgical, is not yet well defined. Its etiology remains unknown, and the treatment options depend on the disease’s progression. Two classifications summarize the progress of this condition; the first by Hebert has four stages based on radiographic findings and the other by Kalainov defines two types according to extent of necrosis on MRI. A review of literature and our experience has led us to propose a decision-making algorithm for its therapeutic management, from conservative treatment to surgical treatment. The surgical procedure is chosen based on the disease stage and the extent of necrosis. According to Zaidemberg, in the early stages of the disease, treatment with a vascularized graft is the preferred solution. For more advanced stages, several techniques are available ranging from scaphoidectomy with potential prosthetic replacement or proximal row carpectomy to more radical solutions such as carpal arthrodesis. However, given the rarity of this disease, the indications for surgery remain tricky.

La maladie de Preiser ou ostéonécrose aseptique du scaphoïde est une affection rare dont les indications de traitement, chirurgical ou non, demeurent sujet à débat. Son étiologie reste inconnue et les solutions thérapeutiques dépendent de l’avancée de la maladie. Deux classifications déterminent l’état d’avancement de l’affection, en 4 stades radiographiques comme l’a défini Herbert et en deux types selon Kalainov, ce dernier caractérisant l’étendue de la nécrose démontrée par l’IRM. Une revue de la littérature et notre expérience nous amènent à proposer un algorithme décisionnel de la conduite thérapeutique qui s’étend du traitement conservateur à la cure chirurgicale. Le type d’intervention chirurgicale choisie varie en fonction du stade évolutif et de l’étendue de la nécrose. Aux stades précoces de la maladie, le traitement par apport d’un greffon vascularisé selon Zaidemberg représente de nos jours le meilleur choix. Pour les stades plus avancés, plusieurs techniques sont éligibles : scaphoïdectomie avec remplacement prothétique ou résection de la rangée proximale des os du carpe, ou des techniques plus radicales d’arthrodèse de différents types. Cependant, compte tenu de la rareté de cette maladie, les indications opératoires demeurent délicates.

Avascular necrosis of the scaphoid, often referred to as Preiser’s disease, is little known despite the scaphoid being the second most frequently involved location in avascular pathology of the carpal bones after the lunate (Kienböck’s disease). Nonetheless, very few cases have been reported in the medical literature over the last century. Its pathophysiology is not completely elucidated although the unique vascularization of the scaphoid and several risk factors seem to be recurrently incriminated. Two new classifications based on modern imaging have appeared in the last decade and are now considered essential supplements to Herbert and Lanzetta’s original radiographic classification. Because of the lack of formal treatment guidelines, a wide range of different treatments have been explored over time, contributing to the confusion around this pathology. Adding to this confusion, are the numerous terms used to designate the condition and lack of knowledge concerning its pathophysiology, risk factors, clinical and radiographic presentation. The aim of this review is therefore to explore and clarify Preiser’s disease in terms of history, terminology, pathophysiology, clinical considerations and imaging and to propose a simple updated treatment algorithm based on the results provided by a thorough review of literature (53 publications, 170 patients) since Georg Preiser’s original and controversial description in 1910.

La nécrose avasculaire du scaphoïde, souvent appelée "maladie de Preiser", est une maladie peu connue bien que le scaphoïde soit le siège le plus fréquemment impliqué dans l’ostéonécrose des os du carpe après le lunatum (maladie de Kienböck). Très peu de cas ont été rapportés dans la littérature médicale. Sa physiopathologie n'est pas complètement élucidée bien que la vascularisation spécifique du scaphoïde et plusieurs facteurs de risque semblent être incriminés de façon récurrente. Deux nouvelles classifications basées sur l'imagerie moderne sont apparues au cours de la dernière décennie et sont désormais considérées comme des compléments essentiels à la classification radiographique originale d'Herbert et Lanzetta. En raison de l'absence de recommandation sur la prise en charge thérapeutique, un large éventail de traitements différents a été exploré et proposé au fil du temps, ce qui a contribué à la confusion qui entoure cette pathologie. Les nombreux termes utilisés pour désigner cette pathologie, le manque de connaissances concernant sa physiopathologie, les facteurs de risque, la présentation clinique et radiographique contribuent à cette confusion. L'objectif de cette revue était donc d'explorer et de clarifier l’entité connue sous le terme de maladie de Preiser (histoire, terminologie, physiopathologie, considérations cliniques, imagerie) et de proposer un algorithme de traitement simple et actualisé basé sur les résultats fournis par une revue approfondie de la littérature (53 publications, 170 patients) depuis la description originale et controversée de Georg Preiser en 1910.

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