Preiser's Disease (Scaphoid AVN)

                              Dr. KS Dhillon

Idiopathic avascular necrosis (AVN) of the scaphoid bone was first described by Georg Preiser in 1910 [1-3]. It is a rare condition. It can cause pain and sometimes swelling around the anatomical snuffbox. It can be associated with loss of strength and reduced range of motion in the wrist [4].

The blood supply of the scaphoid mainly comes from branches of the radial artery. Most of the supply enters through the distal portion of the scaphoid [5]. There are a few nutritional vessels that directly enter the scaphoid through the proximal pole. The proximal pole is a vascular terminal zone dependent largely on intraosseous blood flow [6]. Preiser disease results from a disruption of this blood supply [6,7]. Radiographs may show scaphoid sclerosis without visible fracture, and magnetic resonance imaging will show signal changes. Late radiographic changes include cystic changes, fragmentation, and collapse of the scaphoid [4,8,9].

Epidemiology

AVN of the scaphoid is rare with an incidence of less than 1 per 100,000 annually. The average age of onset is 45 years.

Clinical Presentation

The patients usually present dorsoradial wrist pain.

Imaging

Radiographs show sclerosis and fragmentation of the proximal pole without evidence of fracture. Radiographs are used to identify the involved portion of the scaphoid (proximal, whole bone) and the severity of Preiser disease. Severity has been staged according to the Herbert-Lanzetta Classification [4]. Stage I represents normal radiographs, but a positive bone scan; stage II an increased density of the proximal pole and generalized osteoporosis; stage III fragmentation of the proximal pole with or without a pathological fracture; and stage IV a pattern of carpal collapse and osteoarthritis.  

An MRI can further allow classification into complete vs partial involvement.

Kalainov et al. identified two categories of avascular necrosis according to the location of necrosis. Type 1 in which necrosis involved the entire scaphoid and type 2 in which the necrosis was only found in the proximal pole [10].

Diagnosis

The diagnosis is made by taking a history, doing a physical examination, and taking x rays.

Treatment

Nonoperative treatment with NSAIDs and immobilization of the wrist is effective in about 20% of cases.

When nonoperative management fails surgery is required. Operative techniques include:

• Drilling

• Revascularization

• Allograft replacements

• Proximal row carpectomy and scaphoid excision with four-corner fusion are considered salvage procedures

References

1. Preiser G. Eine typische posttraumatische und zur spontanfraktur fuhrende ostitis des naviculare carpi. Fortschr Geb Roentgenstr. 1910;15:189–97. 

2. Kallen AM, Strackee SD. On the history and definition of Preiser’s disease. J Hand Surg Eur Vol. 2014;39(7):770–6. 

3. Green N, Osmer JC. Small bone changes secondary to systemic lupus erythematosus. Radiology. 1968;90(1):118–20.

4. Herbert TJ, Lanzetta M. Idiopathic avascular necrosis of the scaphoid. J Hand Surg Br. 1994;19(2):174–82.

5. Gelberman RH, Menon J. The vascularity of the scaphoid bone. J Hand Surg Am. 1980;5(5):508–13. 

6. Schmitt R, Frohner S, van Schoonhoven J, Lanz U, Golles A. Idiopathic osteonecrosis of the scaphoid (Preiser’s disease)--MRI gives new insights into etiology and pathology. Eur J Radiol. 2011;77(2):228–34.

7. Vidal MA, Linscheid RL, Amadio PC, Dobyns JH. Preiser’s disease. Ann Chir Main Memb Super. 1991;10(3):227–35.

8. De Smet L, Aerts P, Walraevens M, Fabry G. Avascular necrosis of the carpal scaphoid: Preiser’s disease: report of 6 cases and review of the literature. Acta Orthop Belg. 1993;59(2):139–42. 

9. Kalainov DM, Cohen MS, Hendrix RW, Sweet S, Culp RW, Osterman AL. Preiser’s disease: identification of two patterns. J Hand Surg Am. 2003;28(5):767–78.

10. D.M. Kalainov, M.S. Cohen, R.W. Hendrix, S. Sweet, R.W. Culp, A.L. Osterman. Preiser's disease: identification of two patterns J Hand Surg, 28 (2003), pp. 767-778.