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Preiser's disease or idiopathic avascular necrosis of the scaphoid is a rare condition where ischemia and necrosis of the scaphoid bone occurs without previous fracture. It is thought to be caused by repetitive micro trauma or side effects of drugs (e.g., steroids or chemotherapy) in conjunction with existing defective vascular supply to the proximal pole of the scaphoid. Wrist radiography or CT coupled with MRI is the imaging modality of choice in the diagnosis of this rare entity.Here, we report a case of Preiser's disease of the left wrist in a 17-year-old female patient who presented with left wrist pain of 2 years duration in the absence of trauma history or causative drug use. The diagnosis was made by wrist X-ray and MRI. She was managed by Physiotherapy and wrist immobilization using wrist and forearm support as well as NSAIDS (Meloxicam).

Background Idiopathic avascular necrosis of the lunate is known as Kienböck's disease and that of the scaphoid is known as Preiser's disease. Because the prevalence of coexisting Kienböck's and Preiser's diseases is very low, standardized stages of disease and treatments are not established. Case Presentation We report coexisting avascular necrosis of the scaphoid and lunate in a 68‐year‐old woman with no history of steroids or other risk factors. We treated her with proximal row carpectomy with capsular interposition technique. A distal‐based dorsal capsular flap was prepared and repaired the palmar capsule. At the last follow‐up, she had no pain and had gained improved range of wrist motion. There was no arthritic change at the newly formed radiocapitate joint. Conclusions In the case of collapsed lunate and scaphoid with avascular necrosis, the proximal row carpectomy procedure has an advantage. Proximal row carpectomy with dorsal capsular interposition can be performed when the lunate or scaphoid cannot be saved. Arthritic changes of the capitate head and distal radius lunate facet can be covered with the dorsal capsule.

Background: Idiopathic avascular necrosis of the lunate is known as Kienböck's disease and that of the scaphoid is known as Preiser's disease. Because the prevalence of coexisting Kienböck's and Preiser's diseases is very low, standardized stages of disease and treatments are not established. Case Presentation: We report coexisting avascular necrosis of the scaphoid and lunate in a 68‐year‐old woman with no history of steroids or other risk factors. We treated her with proximal row carpectomy with capsular interposition technique. A distal‐based dorsal capsular flap was prepared and repaired the palmar capsule. At the last follow‐up, she had no pain and had gained improved range of wrist motion. There was no arthritic change at the newly formed radiocapitate joint. Conclusions: In the case of collapsed lunate and scaphoid with avascular necrosis, the proximal row carpectomy procedure has an advantage. Proximal row carpectomy with dorsal capsular interposition can be performed when the lunate or scaphoid cannot be saved. Arthritic changes of the capitate head and distal radius lunate facet can be covered with the dorsal capsule. [ABSTRACT FROM AUTHOR]

An irreparable scaphoid proximal pole is challenging to treat because of the fragment size and limited blood supply. Salvage surgery, such as partial wrist fusion or proximal row carpectomy, may be performed but is not ideal for young patients. There are few reports of proximal scaphoid reconstruction using rib osteochondral grafts. Four patients were treated with rib osteochondral graft for reconstruction of the scaphoid proximal pole. The patients had a mean postoperative follow-up of 24 months. The mean age at the time of surgery was 30 years. Outcome measurements included total active range of wrist motion arc, grip strength, and wrist function score. We also evaluated the progression of osteoarthritis and changes in carpal height. No complications occurred at the donor site. The range of motion improved from 82° to 95° before and after surgery. Grip strength improved from 22 kg to 33 kg before and after surgery. There was a remarkable improvement in the modified wrist function scores of Green and O'Brien from 40 points to 70 points before and after surgery. No progression of arthrosis was seen on the radiographs of all the patients. There was no significant difference in the carpal height ratio before and after surgery. Proximal scaphoid fractures may require reconstruction of the articular surfaces of the radius, capitate, and lunate. Reconstruction with a rib osteochondral graft is flexible, easy to fabricate, and can reconstruct the three articular surfaces. [ABSTRACT FROM AUTHOR]

Case: We present the case of a patient who developed scaphoid avascular necrosis in the setting of scaphoid hypoplasia, 37 years after an index finger repositioning for thumb agenesis, due to Holt-Oram syndrome. Conclusion: To the best of our knowledge, this is a unique case of scaphoid avascular necrosis in a patient with Holt-Oram syndrome with scaphoid hypoplasia who underwent index finger repositioning 37 years earlier. Her wrist pain was successfully treated with scaphoid excision and intercarpal fusion. [ABSTRACT FROM AUTHOR]

Idiopathic avascular necrosis of the scaphoid bone, Preiser's disease, was originally described as a deteriorative pathology whereby the osseous structure necroses due to loss of blood supply. It may present with multifactorial etiology, which is still largely not well understood. We describe a case of Preiser's disease in a 70-year-old female, with worsening pain and loss of range of motion in her right wrist over a two-year period. Past medical history was significant for Sjogren's disease, fibromyalgia, and dystonia. Pain began several months following traumatic right dorsal wrist injury. Diagnosis of traumatic scaphoid fracture was originally suspected. Conservative treatment was unsuccessful. Radiographs did not demonstrate evidence of primary fracture. CT scan and MRI demonstrated osteonecrosis of the proximal pole of the scaphoid, but no evidence of fracture, either residual or healing, was found. Proximal row carpectomy was performed for avascular necrosis of the scaphoid. Histology confirmed diagnosis and verified absence of fracture. Postoperatively, the patient's pain and range of motion improved. This report combines histological findings of Preiser's disease with radiographic images which may ameliorate understanding of the clinical pathophysiology. We describe an unusual manifestation of Preiser's disease whereby a single traumatic event, in the absence of fracture, led to idiopathic scaphoid avascular necrosis, which may have been associated with Sjogren's syndrome and fibromyalgia. These conditions may have negatively impacted microvasculature and decreased bone mineral density, inversely correlated with the production of fatty marrow, facilitating the onset of osteonecrosis in the scaphoid., Competing Interests: No potential conflict of interest was reported by the author(s)., (© 2024 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.)

Preiser's disease or idiopathic avascular necrosis of the scaphoid is a rare condition where ischemia and necrosis of the scaphoid bone occurs without previous fracture. It is thought to be caused by repetitive micro trauma or side effects of drugs (e.g., steroids or chemotherapy) in conjunction with existing defective vascular supply to the proximal pole of the scaphoid. Wrist radiography or CT coupled with MRI is the imaging modality of choice in the diagnosis of this rare entity. Here, we report a case of Preiser's disease of the left wrist in a 17-year-old female patient who presented with left wrist pain of 2 years duration in the absence of trauma history or causative drug use. The diagnosis was made by wrist X-ray and MRI. She was managed by Physiotherapy and wrist immobilization using wrist and forearm support as well as NSAIDS (Meloxicam)., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Idiopathic avascular necrosis (AVN) of the carpal bones of the wrist is rare. The lunate and the scaphoid are the two most commonly affected, the lunate being the more common of the two. AVN of a fractured proximal pole of the scaphoid is a well-known problem following trauma; however idiopathic AVN of the scaphoid not related to acute trauma is a rare event with few cases described in literature. There is no consensus on the optimal surgical management of these conditions, although treatment is largely based on the stage at presentation and the expertise and repertoire of the surgeon. In this paper, we present a review of the symptoms, classifications, risk factors and management of these rare conditions. [ABSTRACT FROM AUTHOR]

Background: 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., Methods: 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., Results: 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., Conclusion: 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., Level of Evidence: IV; systematic review of case reports and case series on Preiser's disease., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Hypohidrotic ectodermal dysplasia (HED), also called Christ-Siemens-Touraine (CST) syndrome, is a rare genetic syndrome that affects structures of ectodermal origin, such as the nails, teeth, hair, sweat glands, and skin. Deficiency or absence of these anatomical structures can result in hypotrichosis, hypodontia, hypohidrosis, or anhidrosis. Most cases show X-linked inheritance associated with mutations in the ectodysplasin ( EDA) gene, although autosomal dominant or recessive inheritance patterns have also been observed. Avascular necrosis of the scaphoid bone is common after a fracture and can also be associated with systemic disease or chronic steroid administration, while idiopathic avascular necrosis of the scaphoid is very rare. This report presents a patient with HED who developed idiopathic avascular necrosis of the scaphoid and discusses the potential association between the two. Level of Evidence: V, diagnostic study. [ABSTRACT FROM AUTHOR]

In 1910, Georg Preiser (1876–1913) described five cases of rarifying osteitis. Based on his imaging studies, he diagnosed post-traumatic avascular necrosis (AVN) of the scaphoid without any sign of primary fracture. This was followed by an article in 1911 in which Preiser related his findings to Kienböck’s disease and Köhler’s disease of the tarsal navicular. Upon searching the literature, we found descriptions and discussions of Preiser’s imaging; however, the original images have never been published. We reproduce Preiser’s original imaging in this current review. All of these appear to show a fracture and no signs of AVN, suggesting that Georg Preiser misinterpreted his findings. There is no apparent uniformity in the literature regarding the definition, description, or aetiology of Preiser’s disease, and it is for this reason that we find the use of eponyms to be confusing. [ABSTRACT FROM PUBLISHER]

The term Preiser's disease typically is used to describe idiopathic avascular necrosis of the scaphoid, but there have been a number of putative etiologies considered. It is rare and the natural history is not fully understood. Management of the condition should be based on patient factors as well as the stage of disease with regard to the scaphoid and the surrounding wrist. This chapter appraises the available evidence and aims to provide the reader with a framework to manage this rare condition., Competing Interests: Disclosure The authors declare no conflicts of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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., (Copyright © 2022. Published by Elsevier Masson SAS.)

Background: Atraumatic, idiopathic, avascular necrosis of carpal scaphoid – Preiser’s disease is a very rare disease. Since the condition is so uncommon there is no clear therapeutic recommendation.Methods: A 15 year’s follow-up of a patient with complete avascular necrosis of the scaphoid is presented. He was treated with subtotal excision of scaphoid and four corner arthrodesis. The patient was invited for a control three and fifteen years after surgery. Range of movement, grip power, pain and satisfaction with the procedure was measured with the Mayo Wrist Score (MWS). Functional ability of his arm was measured with Dissability of Arm and Shoulder (DASH).Results: Independent examiner measured his functional ability with MWS where he got 65 points out of 100. The patient fulfilled DASH questionary where he gained 6.6 points (0 is the best, 100 is the worst). Results didn’t change between the control three and fifteen years after surgery.Conclusions: Similary to some other authors we concluded that four corner arthrodesis is a reliable procedure for advanced stages of Preiser’s disease.

Abstract: Idiopathic avascular necrosis of the scaphoid, also called Preiser''s disease, has been diagnosed in only 150 cases. Its clinical and radiological description is well established, but there is no consensus regarding its treatment. We report the case of a 53-year-old woman, with an advanced disease; treated arthroscopically by partial resection of the scaphoid, and insertion of an adapted pyrocarbon implant. Preliminary results at 6months were satisfying in terms of pain. However, there was little improvement in range of motion, with a final range of 150°. [Copyright &y& Elsevier]

Elderly patients suffering from avascular necrosis of a carpal bone in both wrists are extremely rare. We report a case of an elderly kendo (Japanese fencing) competitor who sustained Preiser's disease in the left hand following the occurrence of Kienböck's disease in the right hand. The current case demonstrates the importance of raising awareness of these diseases as potential sports-related problems in the elderly. [ABSTRACT FROM AUTHOR]

Avascular necrosis involving more than one carpal bone is rare, and the appropriate treatment for this condition has not yet been established. In this report, two patients with concomitant Preiser's and Kienböck's disease who also had severe wrist pain without obvious traumas were treated by closing radial wedge osteotomy (CRWO). Clinical evaluation showed that CRWO was effective against both conditions and indicated that it is more suitable for early stage than stage II diseases of the Hebert and Lichtman classifications. [ABSTRACT FROM AUTHOR]

Reports of simultaneous carpal avascular necrosis in more than 1 bone are rare. This is a report of simultaneous Kienböck’s disease and Preiser’s disease in a 50-year-old woman with a remote history of steroid use but without a history of trauma or systemic illness. [Copyright &y& Elsevier]

Purpose: To examine the outcome of vascularized bone grafts (VBGs) used for the treatment of Preiser’s disease. Methods: Over a 10-year period (1993–2003) 8 pedicled VBGs were performed as the primary treatment for Preiser’s disease. All patients had preoperative magnetic resonance imaging (MRI) scans that confirmed the diagnosis of avascular necrosis of the scaphoid. Bone revascularization was evaluated by examining preoperative and postoperative MRIs. All patients had a reverse-flow pedicled VBG from the distal radius. The average patient age was 40 years and the mean follow-up period was 36 months. Postoperative evaluation included range of motion, grip strength, pain evaluation, and Mayo wrist scores. Results: All MRIs showed evidence of revascularization, with improvement in T2 and/or T1 signal; however, a consistent finding on MRI was incomplete revascularization of the entire proximal pole. Wrist motion averaged 55% of the unaffected side after surgery. Grip strength remained stable. Seven patients had long-term improvement in pain. The average Mayo wrist score was 68, with 1 patient rated as excellent, 1 as good, 5 as fair, and 1 as poor. There was 1 reconstructive failure that resulted in a proximal row carpectomy less than 1 year after the surgery. Conclusions: In this series VBGs led to reduced pain levels and preserved the radiocarpal wrist motion in most patients. Inability to revascularize the proximal pole of the scaphoid and ongoing wrist arthritis appear to persist despite revascularization attempts. VBGs for Preiser’s disease should be limited to patients with early stage disease (Herbert stages I or II) without evidence of radiocarpal arthritis and no evidence of carpal instability. Type of study/level of evidence: Therapeutic, Level IV. [Copyright &y& Elsevier]

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., (Copyright © 2021 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

Simultaneous avascular necrosis of multiple carpal bones is rare. Concomitant avascular necrosis of scaphoid and lunate has been reported only once. We report one more case of this rare condition which can be a cause of wrist pain. Steroid intake is a known risk factor for avascular necrosis but in our case the patient had been taking herbal medicines for joint pain the composition of which was not known. Probably the presence of steroid in these medicines was the cause of avascular necrosis in this case. [ABSTRACT FROM AUTHOR]

A case of Preiser’s disease treated with closing radial wedge osteotomy is reviewed and the therapeutic effect is reported. A good result was obtained in the short term and we believe that this surgical method could become one of the choices for surgical treatment to alleviate pain in Preiser’s disease. [Copyright &y& Elsevier]

There is no evidence-based treatment protocol for Preiser's disease. This case report studies seven cases treated by pronator quadratus pedicled bone graft. Three years after the operation, all patients had good or excellent results. This technique is advocated for stage 2 Preiser's disease. [ABSTRACT FROM AUTHOR]

In 1910, Georg Preiser (1876-1913) described five cases of rarifying osteitis. Based on his imaging studies, he diagnosed post-traumatic avascular necrosis (AVN) of the scaphoid without any sign of primary fracture. This was followed by an article in 1911 in which Preiser related his findings to Kienböck's disease and Köhler's disease of the tarsal navicular. Upon searching the literature, we found descriptions and discussions of Preiser's imaging; however, the original images have never been published. We reproduce Preiser's original imaging in this current review. All of these appear to show a fracture and no signs of AVN, suggesting that Georg Preiser misinterpreted his findings. There is no apparent uniformity in the literature regarding the definition, description, or aetiology of Preiser's disease, and it is for this reason that we find the use of eponyms to be confusing., (© The Author(s) 2013.)