Preiser Disease

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

Why it is examined. The condition is rare but a classic exam topic, because it forces reasoning from first principles: scaphoid vascular anatomy, the difference between AVN and nonunion, and a staged treatment algorithm where no guideline exists.

Who gets it. Adults, typically aged 30-50. Both sexes are affected, with a female predominance in several surgical series, and the disease is usually unilateral. Only a few hundred cases exist in the world literature: a pooled review of 53 publications captured only around 170 patients in a century.

A diagnosis of exclusion. Insidious pain without significant trauma is the giveaway in the history. Before calling it Preiser disease, exclude an occult fracture or an established nonunion, which carries proximal-pole AVN of its own.

Natural history. The disease evolves over months to years:

• Early (Stages I-II): a viable scaphoid may revascularise or remain stable
• Mid (Stage III): fragmentation and loss of proximal-pole volume
• Advanced (Stage IV): collapse and peri-scaphoid arthritis, a SNAC-like pattern

The blood supply. Roughly 70-80% of the scaphoid's blood, including the entire proximal pole, comes from the dorsal scaphoid branch of the radial artery. It enters distally, near the dorsal ridge and waist, and runs retrograde toward the proximal pole. The distal pole and tubercle are supplied by volar branches.

Why the proximal pole dies first. No vessel enters the proximal pole directly, so it is the watershed, last to perfuse and first to die. The same anatomy explains the delayed union of proximal pole fractures, and why they fail to unite.

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Aetiology. Multifactorial and, by definition, often unknown. The recognised associations are:

• Slender ("type 2") scaphoid morphology, with a sparser intra-osseous vascular network (discussed below, with the strength of the evidence)
• Repetitive load and vibration: manual labour, drilling and impact sports, causing cumulative microtrauma to a marginally perfused bone
• Drugs: corticosteroids, cytotoxic chemotherapy and alcohol, the last two implicated in case reports
• Systemic disease: systemic lupus erythematosus, vasculitis, sickle cell disease, gout and renal disease
• Idiopathic: in many patients no cause is ever identified, and this is the "core" Preiser group

The cascade. From perfusion to arthritis, in order:

1. Compromised perfusion of a marginally vascularised (often slender) scaphoid
2. Ischaemia of the proximal pole, the watershed zone
3. Osteocyte death
4. Subchondral microfracture and fragmentation
5. Loss of scaphoid height, carpal malalignment (DISI) and instability
6. Peri-scaphoid radiocarpal and midcarpal arthritis

Why dead bone looks dense. Increased density does not mean more bone. Living bone around the dead segment continues to be remodelled and partly resorbed while the dead trabecular bone is not, so the necrotic area looks relatively sclerotic on radiographs. The same principle applies in Kienbock's disease and femoral head AVN.

The reported case. A 10-year-old with an incompletely ossified scaphoid illustrates both the diagnostic difficulty and the capacity for recovery. Initial radiographs showed osteosclerosis, CT showed segmentation and collapse of the ossific nucleus, and both T1- and T2-weighted MRI showed low signal throughout the scaphoid. The imaging did not show the oedema or fracture morphology expected after an acute injury.

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Recovery. Because the ossific nucleus remained biologically active, three weeks in a thumb-spica cast followed by two months in an orthosis was followed by progressive remodelling. The distal pole remodelled first and the proximal pole followed by one year; the whole scaphoid had remodelled by two years, and growth was normal at five years.

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The slender scaphoid. The constitutional factor most often invoked for idiopathic scaphoid necrosis is shape. Scaphoid morphology is broadly divided into a wide (type 1) and a slender (type 2) form, and the slender scaphoid carries a sparser intra-osseous vascular network. That leaves its already-marginal proximal pole even more vulnerable when perfusion is challenged by load, drugs or systemic disease.

The evidence for it. In the Mayo series of 39 operated wrists, Amundsen and colleagues assessed the unaffected contralateral wrist in patients with unilateral disease and found a slender scaphoid in half of the wrists that could be evaluated (four of eight). The shape being present in the healthy wrist too is what supports a constitutional predisposition rather than a purely acquired insult.

How loosely to hold it. The association rests on a handful of contralateral wrists in one retrospective series with no control group, and the authors themselves write only that a slender scaphoid is potentially more common. It is a plausible mechanism with a reasonable anatomical basis, not an established risk factor.

Put the other wrist to work. Because Preiser disease is usually unilateral, the contralateral scaphoid is a free control. Image it:

• to establish the patient's baseline morphology
• to exclude asymptomatic bilateral disease
• as an anatomical template for reconstruction planning

Herbert-Lanzetta stages the disease on plain radiographs; Kalainov and Schmitt read the extent and pattern of necrosis on cross-sectional imaging.

History. Insidious radial-sided or dorsal wrist pain, worse with activity, with no significant trauma or pain disproportionate to a trivial injury. Grip strength falls and wrist motion is progressively lost. Ask about steroid use, chemotherapy, connective-tissue disease and vibration exposure: a drill operator or manual worker with months of unexplained radial wrist pain is the classic vignette.

Examination. Tenderness over the anatomical snuffbox and scaphoid, dorsoradial fullness, reduced flexion-extension that is painful at the extremes, and a weak, painful grip. The Watson (scaphoid shift) test may be positive if carpal instability has developed. The findings are non-specific; imaging makes the diagnosis.

The differential. Radial-sided wrist pain in an adult has several mimics, and MRI is the single best discriminator. Preiser disease shows diffuse low T1 marrow signal of the scaphoid without a fracture line; each alternative has its own pattern.

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Plain radiographs first. PA, lateral and scaphoid (ulnar-deviation) views give the Herbert-Lanzetta stage: sclerosis in Stage II, fragmentation and cysts in Stage III, collapse and peri-scaphoid arthritis in Stage IV, and any DISI on the lateral. Films are normal in Stage I, so a normal radiograph never excludes the diagnosis.

MRI is the key test. Use T1 and T2 sequences and consider contrast. Diffuse low T1 signal confirms AVN, and the absence of a fracture line distinguishes Preiser disease from nonunion. MRI also detects Stage I disease, assesses lunate viability and separates complete from partial necrosis (Kalainov), which guides graft suitability.

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CT for bony detail. CT defines trabecular detail, fragmentation, cyst location, collapse and any subtle fracture line, so it excludes an occult fracture or nonunion and plans surgery. Sagittal-oblique reformats along the scaphoid axis are the most useful.

Arthroscopy in selected cases. It assesses the cartilage directly, allows articular damage to be graded and can be combined with debridement. It helps decide between joint-preserving surgery and salvage when imaging is equivocal.

Staged care without a guideline. There is no level I evidence and no society guideline, and management is built on small case series. Immobilise early disease, graft viable non-arthritic scaphoids, and salvage once the carpus collapses or arthritis appears.

Stage III and the evidence. In the systematic review of case series, proximal row carpectomy gave the best pain relief and motion in Stage III, while the algorithm still grafts a Stage III wrist without arthritis. Assess the cartilage and carpal alignment carefully before choosing.

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Progression. Untreated, or after surgery that only treats symptoms, the disease runs on to fragmentation, scaphoid collapse, DISI and peri-scaphoid arthritis. Some early viable scaphoids instead revascularise and stabilise.

CRPS. Complex regional pain syndrome is a risk after any wrist surgery; minimise it with early motion and good analgesia.

Stiffness. It is common after prolonged immobilisation or fusion, and early hand therapy mitigates it.

Timing decides the outcome. 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.

Related pages: Kienbock's Disease is the same problem one bone over and the far commoner carpal osteonecrosis - worth reading alongside, because the two coexisted in two of the seven wrists in the radial-osteotomy series cited above, and radial-shortening logic crosses between them; Scaphoid Fractures and Scaphoid Nonunion for the injuries this is mistaken for and must be distinguished from - the discriminator is where the fracture line sits relative to the necrotic zone; Carpal Instability (DISI/VISI) for the collapse pattern that marks late presentation and closes the door on joint preservation; Midcarpal Arthritis for the degenerative endpoint that decides between proximal row carpectomy and four-corner fusion; and Avascular Necrosis of the Hip for the systemic risk factors - corticosteroids, chemotherapy, connective-tissue disease - that are shared across every site of osteonecrosis.