Preiser Disease

Preiser disease is idiopathic avascular necrosis (osteonecrosis) of the scaphoid occurring without an acute fracture. It was first described by Georg Preiser in 1910. The scaphoid is the second most common carpal bone to undergo osteonecrosis after the lunate (Kienbock's disease).

Aetiology is multifactorial and, by definition, often unknown:

Risk Factors and Associations

1. Slender ("type 2") scaphoid morphology

   • A narrow scaphoid has a sparser intra-osseous vascular network
   • Over-represented in operative Preiser series compared with the wider "type 1" scaphoid

2. Repetitive load and vibration

   • Manual labour, drilling, impact sports
   • Cumulative microtrauma to a marginally perfused bone

3. Drugs and systemic disease

   • Corticosteroids, cytotoxic chemotherapy, alcohol
   • Systemic lupus erythematosus, vasculitis, sickle cell disease, gout, renal disease

4. Idiopathic

   • In many patients no cause is ever identified - this is the "core" Preiser group

Pathophysiology cascade:

1. Compromised perfusion of a marginally vascularised (often slender) scaphoid
2. Ischaemia of the proximal pole (the watershed zone)
3. Osteocyte death; dead trabecular bone is not resorbed, producing relative sclerosis on X-ray
4. Subchondral microfracture and fragmentation
5. Loss of scaphoid height, carpal malalignment (DISI) and instability
6. Peri-scaphoid radiocarpal and midcarpal arthritis

Key Imaging Points

General Complications

Progression of disease (untreated or after symptom-only surgery):

• Fragmentation, scaphoid collapse, DISI and peri-scaphoid arthritis
• Some early viable scaphoids instead revascularise and stabilise

Complex regional pain syndrome (CRPS):

• Risk after any wrist surgery; minimise with early motion and good analgesia

Stiffness:

• Common after prolonged immobilization or fusion; early hand therapy mitigates it

Prognostic factors:

Bottom line: outcomes are driven by when the disease is caught. Caught early and viable, the scaphoid may be preserved or even revascularise; caught late with collapse and arthritis, salvage gives dependable pain relief but never restores a normal wrist.

Global epidemiology. Preiser disease is rare worldwide - only a few hundred cases exist in the literature, and most hand surgeons see only one or two in a career. It clusters in adults aged 30-50, with several surgical series reporting a female predominance, and is associated with corticosteroids, chemotherapy, alcohol, connective-tissue disease and a slender scaphoid morphology. There is no population incidence figure and no implant registry, because the operations performed (grafts, osteotomies, carpectomies and fusions) are not arthroplasty.

No single global guideline - practice rests on case-series evidence. No society publishes a formal Preiser treatment guideline. Practice converges on a staging-driven algorithm derived from Level IV-V evidence.

Registry and high- vs limited-resource practice. Where MRI is freely available, early (Stage I) disease is detected and viable scaphoids can be grafted or simply observed before collapse. Where MRI is scarce, diagnosis is later and total wrist fusion becomes the dependable salvage because it needs no specialist implant and reliably abolishes pain. Vascularized grafting and microsurgical reconstruction are concentrated in specialist hand units.

Consent essentials (universal). For joint-preserving surgery, counsel that proximal-pole revascularisation is often incomplete and arthritis may still progress. For salvage surgery, counsel explicitly on the motion-versus-pain-relief trade-off (PRC and four-corner fusion preserve some motion; total fusion removes motion but most reliably relieves pain), and on progression risk if early disease is managed non-operatively.