Preiser Disease

• Age: typically 30-50 years
• Sex: both sexes affected; several surgical series show a female predominance
• Laterality: usually unilateral
• Rarity: only a few hundred cases in the world literature

A pooled literature review of 53 publications captured only around 170 patients in a century.

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

Unlike acute fracture AVN, the necrosis here is primary, not the result of a displaced fragment losing its blood supply.

A radiology study described a three-layered scaphoid in Preiser disease: a proximal zone of osteonecrosis, a middle zone of repair, and a distal zone of viable marrow. Pathological fractures occurred only within the necrotic zone. This maps neatly onto the retrograde, distal-to-proximal blood supply.

Increased density does not mean "more bone". Living bone around the dead segment continues to be remodelled and partly resorbed, while the dead bone is not - so the necrotic area looks relatively dense on radiographs (the same principle as in Kienbock's and femoral head AVN).

The distal pole is normally well perfused, so idiopathic necrosis there is exceptional. When it does occur it implies an unusual insult acting on a marginally perfused (often slender) scaphoid rather than a simple watershed failure.

The distal pole is not the structural keystone of carpal alignment, so distal-pole necrosis is less likely to drive the DISI and collapse cascade. As in the illustrated case, an early, viable distal pole may revascularise over one to two years with immobilization and load reduction alone.

A dense distal pole must still be separated from an old tubercle fracture, an accessory ossicle, or degenerative sclerosis of the scaphotrapeziotrapezoid (STT) joint - all of which can mimic focal distal-pole osteonecrosis on plain films.

Because the term Preiser was coined for proximal or whole-scaphoid disease, a distal-pole case sits at the edge of the definition. Describe the necrosis by its actual location rather than forcing every dense scaphoid into the proximal-pole box.

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 shape in half of the wrists that could be evaluated (four of eight) - supporting a constitutional predisposition rather than a purely acquired insult, since the shape is present in the healthy wrist too.

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, and as an anatomical template for reconstruction planning.

• Pain: insidious radial-sided / dorsal wrist pain, worse with activity
• No significant trauma: or pain disproportionate to a trivial injury
• Weakness: reduced grip strength
• Stiffness: progressive loss of wrist motion
• Risk factors: steroid use, chemotherapy, connective-tissue disease, vibration exposure

A drill operator or manual worker with months of unexplained radial wrist pain is a classic vignette.

• Tenderness: over the anatomical snuffbox / scaphoid
• Swelling: dorsoradial fullness
• ROM: reduced flexion-extension, painful at extremes
• Grip: weak and painful
• Watson (scaphoid shift) test: may be positive if carpal instability has developed

Findings are non-specific; imaging makes the diagnosis.

Preiser's original 1910 description was controversial and some early "cases" were probably unrecognised fractures. Modern authors restrict the term to genuinely idiopathic AVN with no fracture, but the boundary with occult fracture and nonunion remains debated.

A systematic review found surgery gave greater pain relief than conservative treatment in Stages II-III, but the evidence is entirely Level IV case series. Some early, viable scaphoids revascularise with immobilization alone, so not every patient needs an operation.

A large single-centre series found pedicled vascularized bone grafts gave functional outcomes similar to salvage procedures but preserved the scaphoid in about 70% of cases - so the trade-off (preserve a partly dead bone vs remove it) is genuinely unsettled.

Even when grafts improve MRI signal, revascularisation of the proximal pole is frequently incomplete, and arthritis can still progress. This tempers enthusiasm for grafting in wholly necrotic (Kalainov type 1) scaphoids.

Closing radial wedge osteotomy can relieve pain and preserve motion, but in published series radiographic stage still progressed in many patients - suggesting it is symptom-modifying rather than disease-modifying.

As in other end-stage carpal collapse, both salvage options give reliable pain relief with broadly comparable function; choice is driven by cartilage status, demand and surgeon preference rather than high-quality comparative data.