The FIHS will post each month a new issue related to pathologies of the upper extremity. After a month, the text will be available in the « files » section.



The issue for the month will be : The Preiser's disease

Dr Constantin SOKOLOW/F.I.H.S

What is the Preiser's disease?

This disease is defined as an aseptic necrosis of one of the carpal bones : the carpal (or wrist) is made of a very precise assembly of eight small bones, in two rows, under the two bones of the forearm :

The first row : the scaphoïd, the lunate, the triangular making a united chain linked by strong ligaments (the pisiform is in front of the triangular), authorises torsion movements.

The second row : the trapezium, the trapezoid, the capitate and the hamate, is forming a rigid bloc between the first row and the base of the metacarpals.

These various bones – as any tissue in the organism – are living elements and therefore need a nutritive blood supply brought by very small arteries (arterioles) that have been well studied thanks to anatomic dissections and radiological injections: in the case of suppression of the blood supply to one of the bones, the bone suffers and the bone tissues “die”: that is the necrosis. At a physiological state, the tissues – and the bones – are constantly facing destruction due to the aging of the cells, balanced by a tissue creation thanks to the supply of young cells (these cells being brought by the blood); in the case of a rarefaction of the blood supply, a disequilibrium happens and the destruction is more important than the reconstruction. Thus appears the necrosis.

At the carpal level, the necrosis process more often affects two bones :

The lunate : from far the most frequent ; its necrosis has been described by KIENBOCK in 1910,

The scaphoïd: very rare, was described by PREISER in 1910,

The scaphoïd, one of the biggest carpal bones, is located on the exterior side of the wrist, it is extremely mobile in all the wrist movements.
This pathology is extremely rare, and thus quite unknown. The reason why it happens remains mysterious. This necrosis happens without any trauma or any triggering factor and generally affects young adults : the very small number of published cases makes it really hard to propose any precise statistics concerning this pathology.

What are the clinical signs ?

The patient suffers from a vague wrist pain located on the outside (on the thumb side). The clinical examination is quite poor : there sometimes is a scaphoïd sensibility and a constant loss of strength. The lack of results during the examination usually leads to “diagnosis wanderlust”.

What are the complementary exams ?

Standard radiological shots must be taken, the face and the profile (often, at the first stages of the disease, one will have to take picture of the sane side to compare both images).

They will have to be interpreted according to the context: the most common error is to mistake it for a pseudo-arthrosis (non-union fracture), which will lead to a therapeutic error heavy of consequences.
Complementary examinations will have to be done as soon as the diagnosis is made :

A MRI will give precise details on the state of the lesions,

An arthro-scanner will precise the state of the bone structure and especially the state of the cartilages of the scaphoïd and the radius: these two elements are important when deciding upon the surgery option.

Some radiological modifications seems to be quite similar with the Kienböck’s disease :

Stage I: normal radiological image ; that’s the beginning. The MRI and the scintigraphy are abnormal ;

Stage II : alteration of the contrasts in the scaphoïd, predominant on the proximal pole, near the radius : the shape of the scaphoïd is preserved.

Stage III : the shape of the scaphoïd is altered, and it sometimes is fragmented ; the upper portion is collapsed and the image witnesses a fracture.

Stage IV : important collapse of the scaphoïd, with images of a wrist’s arthrosis between the scaphoïd and the radius.

What are the possible treatments?

The surgery option has been very simple for a long time : the scaphoïd was taken out and two options were offered :

a resection of the first row of the carpal bones,

replacing the scaphoïd by a prosthetics.

Each intervention had advantages and inconveniences and it seems that only the first type of intervention had had good results in the long term.

Currently, it seems that, in certain cases, we can avoid excising the scaphoïd, mainly when its shape is still more or less conserved and when the cartilage is still fine ; at this stage we recommend a bonegraft :

either conventional,

Or with microsurgery, by the supply of vascular bonegraft.
These interventions are more technically difficult but have given good results.

What is the prognosis ?

This pathology is exceptional and has benefited from recent findings in vascular anatomy and microsurgery that have led to a different approach of the surgery treatment. However it must remain in the realm of highly specialised hand surgeons.