Preiser reported cases of rarifying osteitis of the scaphoid that were distinguished from a scaphoid fracture and suggested post-traumatic avascular necrosis [1, 2, 4]; after that, the condition was called Preiser disease [2]. Although Preiser’s original cases were questioned as they show a fracture and no sign of avascular necrosis [2], Preiser disease is still usually defined as post-traumatic avascular necrosis of the scaphoid without fracture. In this report, we also refer to post-traumatic avascular necrosis of the scaphoid as a Preiser disease.
Diagnosis and staging of Preiser disease are made using radiographic images. According to the classification by Herbert and Lanzetta, in the early stage, the radiological examination is normal (stage 1), and next, the ischaemic proximal pole appears relatively dense (stage 2), followed by collapse and cystic change (stage 3), and finally, progressive carpal collapse occurs (stage 4) [7]. However, in early cases, plain radiographs are not sensitive to enough to detect avascular necrosis. MRI with or without gadolinium administration is generally assumed to be the most sensitive modality to assess the vascularity of the scaphoid [8]. Classification based on MRI findings by Kalainov divided Preiser disease into two groups: diffuse ischaemia and necrosis of the scaphoid (type 1) and vascular changes present in only a section of bone (type 2) [9]. The findings of plain radiography and MRI in this report correspond to stage 3 of Herbert and Lanzetta and type 1 of Kalainov. There have been a few case reports of Preiser disease in children; however, none of them occurred in the scaphoid with immature ossification, like in this case. Therefore, radiographic images of this patient could not be compared with those of other cases. However, osteosclerosis and segmentation of the scaphoid in plain radiography and CT [1], and low intensity on both T1-WI and T2-WI on MRI [8, 10] were consistent with some radiographic feature of avascular necrosis of the scaphoid; therefore we diagnosed the patient with Preiser disease.
Various case reports and case series have been reported; however, therapeutic strategies for Preiser disease have not yet been standardised. Although a few reports have recommended conservative treatment for the early stage of Preiser disease [7, 9], recent reports have reported that immobilisation could not be indicated for adults Preiser disease [3, 11]. Surgical debridement [12], denervation [13], localised cortisone injection [9], closing radial wedge osteotomy [14], and vascularised or non-vascularised bone grafts [9, 15] have also been reported. Among them, vascularised bone grafting seems reliable for patients with stage 2 or 3 diseases [15,16,17]. For more aggressive cases, proximal row carpectomy or partial fusion, for example, four-corner fusion, was performed [3, 13].
The treatment for Preiser disease in children is unclear. We found four cases of Preiser disease in children [18,19,20,21]. One patient treated with a vascularised bone graft showed improvement in both clinically and radiologically [21]. Two patients treated with immobilisation showed a good clinical course [18, 20], and the last patient had no mention of the treatment [19]. The patients, in this case, were classified as stage 3 of Herbert and Lanzetta - recommended for vascularised bone grafting, and type 1 of Kalainov, which trends towards scaphoid collapse in adults. We chose conservative treatment because the patient was a child whose scaphoid was immature and pre-ossified. Lenoir reported that conservative treatment was only indicated in paediatric patients with spontaneous revascularisation [3]. Bergman also proposed that conservative treatment should be reserved for young patients, those with pauci-symptoms, and those at an early stage [22]. Since this is the first case report of Preiser disease before complete ossification of the scaphoid, we cannot say anything definitive about the treatment strategy. However, our experience suggests that conservative treatment may provide a cure for Preiser disease in children with immature ossification of the scaphoid without carpal collapse. Establishing a therapeutic strategy for Preiser disease in children requires the observation of even more cases.