Preiser's disease

Nine patients with the diagnosis of unilateral Preiser's disease were seen between 1970 and 1987. The mean age of four male and five female patients was 37 years (range, 20 to 70 years). The diagnosis was based on radiographic evidence of sclerosis, fragmentation, erosion, and collapse of the proximal pole of the scaphoid. Onset was usually insidious but two had a preexisting radial hypoplasia, a third had a modest scaphoid malunion, and a fourth had a recent fall. Treatment consisted of scaphoid excision and silicone rubber prosthetic replacement in three, debridement of necrotic bone fragments in one, and different periods of cast support and observation in the remaining five. No relationship to ulnar variance was seen. Mean follow-up was 6.9 years. Of the scaphoid implants, two had subluxated, but only one was painful. In the more conservatively treated group, pain and function were only modestly limiting in four, who returned to their original occupations as did the one treated by curettage of necrotic bone in the proximal pole. One patient (case 4) was severely incapacitated because of associated severe congenital anomalies of the lower extremities. Polyaxial tomography permitted a better assessment of the degree of involvement and carpal alignment. Preiser's disease is a rare affliction of the carpal scaphoid which may involve the entire bone in avascular changes but primarily leads to fragmentation and collapse of the proximal pole. A conservative approach to treatment is favored based on this experience.