Idiopathic avascular necrosis of the scaphoid (Preiser’s disease): a case report and review of the literature

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.