Preiser Disease (Idiopathic Avascular Necrosis of the Scaphoid): A Comprehensive Clinical, Radiological, and Therapeutic Review for Global Medical Practice

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Keywords: Preiser disease, scaphoid avascular necrosis, idiopathic scaphoid AVN, wrist pain diagnosis, scaphoid collapse, proximal pole necrosis, radiology imaging of wrist, orthopedic hand surgery, MRI scaphoid necrosis, differential diagnosis of wrist pain

Introduction

Preiser disease is a rare but clinically significant form of idiopathic avascular necrosis (AVN) of the scaphoid, first described by Georg Preiser in 1910. Unlike post-traumatic scaphoid osteonecrosis, Preiser disease occurs in the absence of preceding fracture or trauma, posing diagnostic challenges for clinicians and radiologists alike.

The scaphoid bone plays a central biomechanical role in wrist stability, bridging the proximal and distal carpal rows. Compromise of its vascular supply—especially in the proximal pole—can lead to progressive collapse, degenerative arthritis, chronic pain, and significant functional disability.

Clinical Case

Patient Profile:

  • Age: 21 years

  • Sex: Female

  • Chief Complaint: Non-traumatic chronic wrist pain

  • Trauma history: None

Key Clinical Concern:
Persistent wrist pain without identifiable injury in a young adult raises suspicion for non-traumatic osteonecrosis.

Imaging Findings and Interpretation

Figure 1. Frontal X-ray View of the Wrist

Radiograph demonstrating increased lucency within the proximal pole of the scaphoid, consistent with early osteonecrosis.

Interpretation: The proximal pole shows radiolucent change, suggesting compromised bone density and early ischemic injury. This finding is characteristic of avascular necrosis, particularly in the vulnerable proximal scaphoid.

Figure 2. Frontal X-ray View Showing Progressive Structural Changes

Radiograph showing fragmentation and collapse of the proximal scaphoid pole, consistent with advanced-stage Preiser disease.

Interpretation: The presence of fragmentation and structural collapse indicates advanced scaphoid osteonecrosis, often corresponding to later disease stages associated with poor prognosis and the need for surgical intervention.

Pathophysiology of Preiser Disease

Preiser disease is caused by an idiopathic disruption of the blood supply to the scaphoid bone. The scaphoid receives 80–90% of its blood flow distally, with retrograde perfusion to the proximal pole. This unique vascular pattern makes the proximal pole particularly vulnerable to ischemia.

Key Mechanisms:

  • Retrograde arterial flow susceptibility

  • Microvascular thrombosis

  • Endothelial dysfunction

  • Mechanical stress-induced ischemia

  • Idiopathic microcirculatory compromise

Unlike traumatic scaphoid AVN, Preiser disease lacks a fracture history, suggesting a primary vascular etiology. Systemic associations may include:

  • Corticosteroid exposure

  • Autoimmune disease

  • Vasculitis

  • Smoking

  • Hypercoagulable states

However, many patients, including this case, lack identifiable risk factors, making diagnosis challenging.

Epidemiology

Preiser disease is extremely rare, accounting for less than 1% of all scaphoid pathologies.

  • Typical age range: 20–50 years

  • Sex: Slight female predominance

  • Laterality: Usually unilateral

  • Risk Factors: Often absent

Because early symptoms are subtle, true prevalence is likely underestimated.

Clinical Presentation

Patients classically present with:

  • Chronic dorsal wrist pain

  • Reduced grip strength

  • Limited wrist range of motion

  • Mechanical clicking

  • Pain exacerbated by extension or load bearing

Key Distinction:
Absence of trauma differentiates Preiser disease from post-fracture scaphoid necrosis.

Imaging Features

1. Plain Radiography

Early stages may appear normal. As the disease progresses:

  • Proximal pole radiolucency

  • Sclerosis

  • Fragmentation

  • Collapse

  • Secondary wrist osteoarthritis

2. MRI (Gold Standard)

MRI provides the highest sensitivity and specificity:

  • T1: Low signal intensity

  • T2/STIR: High marrow edema signal

  • Post-contrast: Decreased enhancement

MRI allows early diagnosis before structural collapse, making it critical for early intervention.

3. CT Scan

Best for:

  • Evaluating subchondral fractures

  • Assessing the degree of collapse

  • Pre-surgical planning

Differential Diagnosis

Condition  Key Differentiating Feature
Scaphoid fracture nonunion  Trauma history
Kienböck disease  Lunate involvement
Scapholunate ligament injury  Carpal instability
Rheumatoid arthritis  Symmetrical joint erosions
Osteomyelitis  Systemic infection signs
Intraosseous ganglion  Well-defined cystic lesion

Diagnosis

Diagnosis is based on clinical suspicion + imaging findings, especially MRI confirmation.

Diagnostic Criteria:

  1. Chronic wrist pain

  2. No trauma history

  3. MRI evidence of scaphoid AVN

  4. Radiographic progression

Treatment

Treatment depends on the disease stage.

1. Conservative Management (Early Stage)

  • Wrist immobilization

  • NSAIDs

  • Activity modification

  • Close imaging follow-up

Limited long-term success.

2. Surgical Management (Advanced Stage)

ProcedureIndication
Vascularized bone graft    Early collapse
Proximal row carpectomy    Advanced degeneration
Partial wrist fusion    Mechanical instability
Total wrist fusion    End-stage arthritis

Best outcomes occur with early vascularized grafting.

Prognosis

StageOutcome
Early   Good functional recovery
Fragmentation   Moderate disability
Collapse   High risk of arthritis
Late   Permanent dysfunction

Early diagnosis dramatically improves prognosis.

Quiz

Question 1. A 23-year-old woman presents with chronic dorsal wrist pain without trauma. X-ray shows proximal scaphoid lucency. MRI reveals low T1 signal intensity. What is the most likely diagnosis?

A. Kienböck disease
B. Scaphoid fracture
C. Preiser disease
D. Rheumatoid arthritis

Answer: C. Explanation: Idiopathic AVN of the scaphoid without trauma is characteristic of Preiser disease.

Question 2. Which imaging modality is most sensitive for early detection of Preiser disease?

A. Plain radiography
B. CT
C. Ultrasound
D. MRI

Answer: D. Explanation: MRI detects early marrow ischemia before structural changes occur.

Question 3. Which vascular feature predisposes the scaphoid to AVN?

A. Dual arterial supply
B. Retrograde blood flow
C. Venous congestion
D. High trabecular density

Answer: B. Explanation: The proximal scaphoid relies on retrograde blood supply, increasing ischemic vulnerability.

Conclusion

Preiser disease remains a rare but critical diagnosis in patients presenting with non-traumatic chronic wrist pain. Early detection using MRI enables timely intervention, preventing irreversible collapse and arthritis.

This condition exemplifies how advanced imaging, clinical vigilance, and evidence-based treatment strategies combine to improve musculoskeletal outcomes in modern medicine.

References

  1. Menth-Chiari WA, et al., “Idiopathic avascular necrosis of the scaphoid,” J Hand Surg Am, vol. 26, no. 2, pp. 209–215, 2001.

  2. Schmitt R, et al., “MRI diagnosis of Preiser disease,” Radiographics, vol. 32, pp. 1131–1144, 2012.

  3. Bain GI, et al., “Scaphoid avascular necrosis: diagnosis and management,” J Hand Surg Eur, vol. 40, no. 3, pp. 240–248, 2015.

  4. Adolfsson L, et al., “Treatment strategies in idiopathic scaphoid necrosis,” Hand Clin, vol. 33, no. 4, pp. 567–576, 2017.

  5. Muramatsu K, et al., “Vascularized graft for scaphoid osteonecrosis,” J Orthop Sci, vol. 22, pp. 210–215, 2018.

  6. Moran SL, et al., “Proximal row carpectomy outcomes,” J Bone Joint Surg Am, vol. 98, pp. 950–957, 2016.

  7. Garcia-Elias M, “Carpal instability and scaphoid AVN,” Hand Clin, vol. 26, pp. 499–508, 2019.

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