THE PATHOLOGY OF PERTHES DISEASE

Perthes disease can be defined as idiopathic avascular necrosis of the femoral head in
childhood. The initiating insult triggering the onset of Perthes disease remains unknown,
making a true description of the pathology difficult. However a great deal more is known about
Perthes disease pathogenesis, much of which has been gained by correlating the few true
histological specimens with radiographs, and also by examining animal models of Perthes
disease.

Infection, trauma, transient synovitis and coagulopathies were all at one time or another
linked with the initial onset of Perthes disease. While trauma may play a part in Perthes
disease it is unlikely to be the only factor as traumatic avascular necrosis often behaves
differently from Perthes disease. In truth, the initiating factor remains unknown.

However what is clear is that the femoral head blood supply is very dependent on the
ascending branches of the lateral femoral circumflex, in turn a branch of profunda femoris. A
condition very similar but not identical to Perthes can be created in piglets by tying off these
and other ascending vessels, along with the repetitive trauma of weight bearing. In Perthes
disease it is well known that the bone age is delayed, an average of 2 years in girls and 1
year in boys. This means the amount of cartilage present in the developing femoral head is
larger and the ossific nucleus smaller. In one theory these vessels have to traverse a larger
than normal cartilaginous enlage to get to get to the epiphysis and are this vulnerable. A
particular strain of rat called the SHR rat also has a delayed bone age and the onset of a
Perthes-like condition occurs in 50% of male rats that stand on their hind limbs to feed. When
they are prevented from weight bearing avascular necrosis does not occur.

The few human specimens available show an enlarged cartilaginous enlage,

fibrocartilagenous and fibrovascular proliferation and a disorganisation of the growth plate.
Depending where the biopsy is taken, areas of granulation tissue or endochondral ossification
can be seen. Some of the “new bone” that has managed to form can be seen to be
reinfarcted.

The stages of Perthes disease are well accepted – (i) initial, (ii) fragmentation, (iii)
reossification and (iv) healed. In the intial phase, the ossific nucleus is not growing due to the
avascular necrosis, bit the cartilaginous enlage continues to enlarge, receiving nutrition from
the synovial fluid. Next the femoral head starts to collapse. The pathology here (at least from
animal models) is that osteoclasts are removing the dead bone. Resorptive phase may be a
better term for this stage than fragmentation. There is an uncoupling of the normal
mechanism whereby bone resorption is followed by bone formation. This gives the
appearance on radiographs of a central sequestrum which continues to mineralise and is
dense, and surrounding lytic areas yet to ossify. Eventually the neo-cartilage in the femoral
head undergoes endochondral ossification with the invasion of blood vessels followed by
osteoblasts. Eventually all the dead bone is removed and replaced with new bone. This bone
is then remodelled into lamellar bone and the process is complete. However is important to
note that the lag between resorption and formation leave the largely cartilaginous head very
soft and subject to the deformation of the loads placed on it.

Finally the circulation re-establishes itself and ossification proceeds from lateral to medial and
posterior to anterior. Deformity depends on the final shape of the cartilaginous enlage once it
is ossified. It is unusual to see Perthes disease recur, although bilaterality runs at 10%.