Bilateral Preiser Disease in a Patient with Sickle Cell Anemia

Abstract

Preiser disease is a rare condition of avascular necrosis of the scaphoid occurring in the absence scaphoid fracture or trauma. While the etiology of Preiser disease remains unknown, it has been associated with steroid use, chemotherapy, and infrequently with systemic diseases. No reports have associated Preiser disease with hemoglobinopathy. Due to the rarity of Preiser disease, management remains controversial and evidence is limited. Here, we describe the case of a 32-year-old right-hand dominant male with sickle cell anemia and a 4-year history of bilateral wrist pain. Radiographs and gadolinium-enhanced magnetic resonance imaging revealed bilateral Preiser disease. He was successfully managed with a 1,2 intercompartmental supraretinicaular artery vascularized bone graft to the right scaphoid.

Introduction

Preiser disease is atraumatic and avascular necrosis (AVN) of the scaphoid. Only a small number of cases have been described since it was first reported by Preiser in 1910. 1 2 There remains no consensus regarding the etiology or treatment of the condition.

Preiser disease has been described in association with several conditions including steroid use, chemotherapy, and vasculitis. 1 3 Hemoglobinopathy is a recognized cause of AVN, and while postulated as a cause of Preiser disease, there is only one reported case with a suspected etiology of myelodysplastic disorder. 2 Bilateral Preiser disease is exceedingly rare with only three reported cases in the literature. 2 4 5 Here, we describe the unique clinical case of bilateral AVN of the scaphoid in a patient with sickle cell anemia and a history of steroid and cyclosporine treatment following a bone marrow transplant.

Case Report

A 32-year-old right-hand dominant male pianist presented to the emergency department with a 4-year history of bilateral wrist pain. His pain was localized to the radial aspect of each wrist, dull in nature, and gradually worsened until presentation. There was no history of trauma to either wrist. The pain in his right wrist worsened acutely causing him to present for care. Radiographs were performed, a diagnosis of right scaphoid fracture was suspected, and he was referred to a hand specialist clinic.

His past medical history was significant for sickle cell anemia. He received treatment with a bone marrow transplant 5 years prior. Subsequently, he developed graft versus host disease (GVHD) and was treated with prednisone and cyclosporine. Complications from his sickle cell anemia included AVN of both knees. At presentation, his bone marrow transplant was stable, and he no longer required treatment for GVHD. He was a nonsmoker.

On physical examination, he demonstrated swelling and tenderness of the radial wrist in the location of the scaphoid tubercle and anatomical snuffbox. He also described pain of the same quality and location, although less severe of the left wrist. Grip strength was assessed as 7 kg on the right and 25 kg on the left. He demonstrated 45 degrees of flexion and 25 degrees of extension on the right wrist, and 65 degrees of flexion and 35 degrees of extension on the left wrist.

Updated standard radiographic views (anterior–posterior, lateral, and oblique) of both wrists were reviewed. Examination of the right wrist revealed no fracture, dorsal intercalated ligament instability (DISI) deformity, or rotatory subluxation of the scaphoid ( Fig. 1A, B ). The left wrist radiographs revealed mild scaphoid collapse with no DISI deformity ( Fig. 2A, B ). A gadolinium-enhanced magnetic resonance imaging (MRI) was ordered and revealed bilateral edema of the entire scaphoid bones ( Figs. 3 and 4 ). The patient was diagnosed with bilateral AVN of the scaphoid, stage I ( Table 1 ). 6 He was provided with a trial of nonoperative management with splinting and oral-analgesics. After 2 months of the treatment, he returned to clinic with no relief of his pain. A corticosteroid injection (methylprednisolone 20 mg) was administered into the right wrist at the radiocarpal joint, and a thorough discussion was had with the patient regarding operative management with a 1,2 intercompartmental supraretinacular artery (1,2-ICSRA) vascularized bone graft (VBG). 7 8 The corticosteroid injection provided transient relief for approximately 8 months. Consent was obtained and a VBG using the 1,2-ICSRA was performed to the right scaphoid. Briefly, a 3 × 5 mm cortical window was made on the dorsoradial aspect of the scaphoid, a small defect was created with curettage and then filled with a press-fit vascularized graft from the radial styloid region between the first and second dorsal tendon compartments (1,2-ICSRA). The surgical procedure has been described previously in greater detail by Boyer et al (1998). 7 Postoperatively, the patient was kept in a short-arm cast for a total of 6 weeks. This was followed by a splint that was weaned over approximately 10 weeks. The patient then underwent weekly therapy for 2 months with treatment focus on strength and motion.

Fig. 1.

Posteroanterior ( A ) and lateral ( B ) view X-rays of the right wrist.

Fig. 2.

Posteroanterior ( A ) and oblique ( B ) view X-rays of the left wrist showing changes at the scaphoid.

Fig. 3.

Sagittal magnetic resonance imaging of right wrist showing signal change throughout the entire scaphoid bone.

Fig. 4.

Sagittal magnetic resonance imaging of left wrist showing signal change throughout the entire scaphoid bone.

Table 1. Herbert and Lanzetta classification of Preiser disease 6 .

Stage I	Normal radiograph; signal change on magnetic resonance imaging or bone scan
Stage II	Increased density of the scaphoid proximal pole
Stage III	Fragmentation of the proximal pole without pathological fracture
Stage IV	Carpal collapse

At the 3-month follow-up, he noted resolution of right wrist pain. His grip strength had increased to 26 kg on the right postoperative side. The left grip strength had decreased to 12 kg; surgery was planned for the left wrist, but he has deferred surgery to pursue his piano career. He has been reassessed regularly, on average twice per year, for the past 7 years following surgery on his right wrist. At his most recent follow-up, grip strength has improved from 7 kg preoperatively to 20 kg on the right while he has had some deterioration of grip strength, from 25 to 16 kg, on his left. He has maintained good functional range of motion from 60 degrees flexion bilaterally to 30 degrees extension on the right and to 45 degrees on the left. He has had some mild recurrence of his right wrist discomfort and persistent discomfort of the left wrist but has fully returned to his career as a pianist and occasionally performs construction work. Recent X-rays, 7 years after surgery show some progression of the AVN bilaterally ( Fig. 5 ). Despite this, he stated that his right wrist was better than the left in terms of function and discomfort. There has not been a degenerative collapse or arthritic process in the wrist.

Fig. 5.

Posteroanterior follow-up X-rays of the right ( A ) and left ( B ) wrists showing slight bilateral progression of the avascular changes and early arthritic changes on the left.

Discussion

AVN of the scaphoid was first described by Preiser in 1910. 9 Although the patients in Preiser’s original report developed necrosis in association with trauma, Preiser disease continues to be recognized as a diagnosis of AVN of the scaphoid in the absence of trauma. Numerous other factors have been suggested to precipitate Preiser disease including steroid use, trauma, smoking, alcohol, vasculitis, chemotherapy, scleroderma, and diabetes ( Table 2 ). 2 While sickle cell anemia is recognized to rarely cause AVN of bone, there are no reports of scaphoid AVN. 10 The cumulation of steroid use, chemotherapy, and hemoglobinopathy may have all likely played a role in the development of scaphoid AVN in this patient.

Table 2. Factors that may contribute to the development of atraumatic avascular necrosis of the scaphoid.

Extrinsic	Instrinsic
a Multiple factors may likely have contributed to bilateral Preiser disease in the patient presented.
Steroid use a	Vasculitis
Chemotherapy a	Hemoglobinopathy a
Tobacco use	Myelodysplastic syndromes
Alcohol use	Hypercholesterolemia
	Endocarditis
	Diabetes
	Thumb hypoplasia
	Scapholunate advanced collapse

Most studies classify Preiser disease based on the Herbert and Lanzetta four-stage classification, although all patients in their series had isolated AVN of the proximal pole ( Table 1 ). 6 An alternative classification was suggested by Kalainov et al based on MRI findings and their observation that patients with diffuse disease (Type 1) have poorer outcomes than patients with isolated proximal pole involvement (Type 2). 11

The rarity of Preiser disease makes it difficult to adequately study its natural history and treatment. Evidence is limited to case studies and series. Late stage disease, characterized by fragmentation of the proximal pole or carpal collapse, is treated with wrist salvage procedures: proximal row carpectomy, 12 scaphoid excision and 4-corner arthrodesis, 11 and total wrist fusion. 6 11 Several treatment strategies have been proposed for early stage disease, which would be more consistent with our patients right wrist, including splinting, 4 6 11 cortisone injections, 11 and vascularized and non-VBG when the cartilaginous surface remains intact. 2 13 14 In eight patients with Stage I and II disease treated with VBGs, Moran et al 13 demonstrated resolution of pain in seven patients with maintenance of grip strength and range of motion. Postoperative MRI demonstrated evidence of scaphoid revascularization in all patients; however, complete revascularization of the proximal pole in no patient.

In a review of 126 cases in the literature, Lenoir et al 2 concluded that conservative management was ineffective in the early stages of the disease. They advocated that VBGs may halt the progression of stage II disease without eliminating the possibility for salvage procedures if the disease progressed. The same view was expressed by Sokolow et al 3 in their report detailing the successful use of cancellous and VBGs in disease as extensive as stage III.

In a separate review of cases, Lauder and Trumble concluded that successful nonoperative management of Preiser disease had not been reported. 15 They recommend revascularization procedures for earlier stage disease and salvage procedures for late stage disease.

Our study patient presented with earlier phase disease, with maintenance of the cartilaginous surface of the scaphoid and structural integrity of the carpus. We offered a careful trial of conservative management, as the possibility of revascularization and resolution of the disease could not be excluded. However, close monitoring was critical to study response and ensure that there was no progression to carpal collapse, which would exclude the possibility of reconstructive surgery. Reconstructive surgery was a viable option for our patient when his disease displayed no response to conservative management and may be a first-choice procedure to reduce pain and attempt to heal the bone. VBGs do not preclude future salvage procedures in the event that the bone grafting is not successful, or the disease process continues. However, it may delay the need for salvage procedures and help patients maintain maximal mobility. In this case, the patient demonstrated improved wrist range of motion on the right following surgery and while he had persistent dull wrist pain, this has reduced in intensity. As he is pursuing his music career, he has refused surgical intervention for the left wrist and has not yet developed the severe pain that prompted his initial presentation to hospital for the right wrist. This case highlights AVN of the scaphoid, etiology, nonoperative, and surgical options for these complex patients.