Review Question - QID 220553

This patient presents with chronic atraumatic wrist pain and images demonstrating avascular necrosis (AVN) of the scaphoid, also known as Preiser's disease (answer choice 2).

Dr. Georg Preiser first described idiopathic AVN of the scaphoid (Preiser's disease) in 1910. It is a rare condition leading to chronic and progressive radial-sided wrist pain. The average age of onset is approximately 45 years, with an incidence of <1 per 100,000 annually. Diagnosis is made with wrist radiographs demonstrating sclerosis and fragmentation of the proximal pole of the scaphoid in the absence of a fracture. Classification of this condition was first described on standard radiographs by Herbert and Lanzetta in 1994; Stage 1: normal x-ray with a positive bone scan, Stage 2: increased density of the proximal pole with generalized osteoporosis, Stage 3: fragmentation of the proximal pole with or without a fracture, and Stage 4: carpal collapse with or without surrounding osteoarthritis. Since then, Kalainov et al. and Schmitt et al. have developed classification systems based on contrast-enhanced MRI. Treatment consists of NSAIDs and immobilization for minimally symptomatic patients and progresses to surgical treatment options such as microfracture drilling, revascularization procedures, allograft replacement, proximal row carpectomy, or 4-corner fusion.

Bellringer et al. characterized Preiser's disease as idiopathic AVN of the scaphoid, first described in 1910 by Dr. Georg Preiser. It is rare and not fully understood, with many proposed etiologies of this condition. Radiographs and MRI are warranted in this condition to make a proper diagnosis and to assess the surrounding joints. Treatment is based on individual patient factors and the stage of disease.

Bergman et al. performed an updated literature review on Preiser's disease. They note that the scaphoid is the second most common carpal bone to demonstrate AVN, behind the lunate (Kienböck's disease). Their treatment algorithm consists of separating the age of the patient and the stage of the disease first. Younger patients presenting with Herbert stage 1 or 2 disease and minimal symptoms typically receive conservative treatment first. Patients who don't meet these criteria and present with Herbert stage 1 disease receive arthroscopic resection. Those with stage 2 disease receive a vascularized bone graft. Finally, those with stage 3 disease receive a proximal row carpectomy or arthrodesis. They conclude that Preiser's disease is exceedingly rare and, therefore, has created difficulty in performing high-quality studies to standardize treatment.

Figure A is an AP radiograph of the wrist demonstrating sclerosis and proximal collapse of the scaphoid. Figure B is a coronal STIR MRI of the wrist demonstrating marrow edema and cyst formation in the scaphoid with partial collapse.

Incorrect answer choices:
Answer 1: Kienböck's disease is AVN of the lunate and is not present in this patient based upon the imaging studies. AVN of the scaphoid is instead seen in this patient.
Answer 3: Acute scapholunate ligament injury is absent in this patient. There was no acute injury, and the MRI does not demonstrate injury to the scapholunate ligament or widening of this joint.
Answer 4: Basilar thumb arthritis or 1st CMC joint arthritis is not present in this patient based on the imaging studies. This is a common complaint, but not the primary contributor to this patient's pain.
Answer 5: AVN of the trapezium is exceedingly rare and not present in this patient based upon the imaging studies provided.