13 Disorders of Epiphyses and

Epiphyseal Growth
 NUTRITION OF THE EPIPHYSIS
AND ITS EPIPHYSEAL PLATE

• Most pressure epiphyses are covered essentially by articular

cartilage and receive blood vessels only through their "bare bone
areas." Others, such as the femoral head, being completely intra-
articular and completely covered by articular cartilage.

• The epiphyseal blood vessels are also responsible for the nutrition
of the growing cells of the epiphyseal plate therefore, ischemia of
the epiphysis is associated with ischemia of the epiphyseal plate
and a subsequent disturbance of longitudinal growth of the bone.
 AVASCULAR NECROSIS
OF BONE

• It is a common phenomenon, inasmuch as after every fracture, a

minute area of each fracture surface undergoes avascular necrosis.
Furthermore, free bone grafts, which initially are both avascular and
necrotic, eventually become replaced by living bone.
 IDIOPATHIC AVASCULAR
NECROSIS OF EPIPHYSES
(THE OSTEOCHONDROSES)

Incidence and Etiology

• The osteochondroses, in general, are most common during the
middle years of growth, from the ages of 3 to 10.
• They affect boys more frequently than girls, and the lowerlimbs are
more often involved than the upper limbs.
• Osteochondrosis of a given epiphysis is bilateral in approximately
15% of involved children.
• The common denominator in the osteochondroses is avascular
necrosis of the epiphyseal center, there is less agreement about the
mechanism of the initial loss of blood supply.
• Certain factors, such as genetically determined vascular
configuration, may have predisposing influence.
• In the idiopathic type, however, less severe trauma may produce a
complication, such as a pathological fracture, in already necrotic
bone.
Pathogenesis and Pathology
1. Early Phase of Necrosis (the Phase of A vascularity)
After obliteration of the blood vessel to the epiphysis from
whatever cause, the osteocytes and the bone marrow cells within
the epiphysis die.

2. Phase of Revascularization with Bone Deposition and Resorption

This phase represents the vascular reaction of the surrounding
tissues to dead bone.
3. Phase of Bone Healing
• Eventually, bone resorption ceases and bone deposition continues
so that the fibrous and granulation tissue are slowly replaced by
new bone.

4. Phase of Residual Deformity

• Once bony healing of the epiphysis is complete, its contour remains
relatively unchanged.
Clinical Features and Diagnosis
• In the phase of revascularization, particularly if a pathological
fracture develops in the subchondral bone, the child experiences
pain.
• A synovial effusion develops, which accounts for local tenderness
and painfullimitation of motion in the joint, especially abduction
(Fig. 13.9).
• If not treated, the symptoms and signs tend to be intermittent, but
gradually the muscles controlling the joint exhibit some degree of
disuse atrophy (Fig. 13.9).
Principles of Treatment
• Osteochondrosis is a self-limiting disease with or without
treatment.
• The principles of treatment are concerned with the prevention of
abnormal forces on the epiphysis during its vulnerable phases of
revascularization and healing.
 Osteochondrosis of the Femoral Head
{Legg-Perthes’ Disease)

Incidence and Etiology

• Legg-Perthes' disease occurs most frequently between the ages of 3
and 11 years and is five times more common in boys (particularly
physically active boys) than girls.

• The original occlusion of the precarious blood supply to the femoral

head is caused by the excessive fluid pressure of a synovial effusion
in the hip joint,either inflammatory or traumatic.
• Recently, Gueck and associates have found antithrombotic factor
deficiencies and hypofibrinolysis in some children with Legg-
Perthes‘ disease and suggest that the resultant thrombophilia may
well be of etiological significance.
Pathogenesis and Pathology
• It is well to emphasize the importance of the pathological
subchondral fracture and of secondary subluxation of the hip as
harmful factors in the pathogenesis of deformity in Legg-Perthes'
disease.
Clinical Features and Diagnosis
• Early phase of necrosis accounts for the fact that the child is seldom
brought to a physician until the phase of revascularization, or even
later. The pain in this disease may be felt in the region of the hip,
but may also be referred to the knee.
• The specific limitation of hip joint motion involves abduction and
internal rotation. The disuse atrophy is most noticeable in the upper
part of the thigh (Fig. 13.9).
• The child walks with a limp of the antalgic, or protective, type
( protecting the hip against pain by rapidly taking weight off the foot
on the involved side with each step) and exhibits a Trendelenburg
sign.
Complications
• Subchondral fracture in the epiphysis
• Subluxation of the joint
• Flattening of the epiphysis (coxa plana) with resultant incongruity
• Late degenerative joint disease (osteoarthritis)
Treatment
• The methods of treatment of Legg-Perthes‘ disease in the past (all
of which had been based on the avoidance of weightbearing) for
several years through various types of so-called weight-relieving
braces, to a sling an
• An effective, albeit cumbersome form of entertainment is
weightbearing in abduction plaster casts (Petrie) (Fig. 13.10) or in
some type of removable abduction brace (Fig 13.11 ).
• Surgical procedures, such as varus femoral osteotomy and
innominate (Salter) osteotomy are designed to prevent or overcome
subluxation of the involved hip.

• They have been used successfully children older than the age of 5
years with more than half the femoral head involve, who
consequently have a bad prognosis. The operation is performed
before any deformity has developed.
 OSTEOCHONDROSIS OF THE CAPITELLUM
(PANNER'S DISEASE)

• Afflicting children between the ages of 3 and 11 years.

• Panner's disease is manifest by pain and slight swelling in the elbow,
as well as by restriction of joint motion.
• The radiographic appearance is typical of osteochondrosis in other
pressure epiphyses.
• Treatment consists of providing the child with a sling during periods
of discomfort
 OSTEOCHONDROSIS OF A METATARSAL HEAD
(FREIBERG'S DISEASE)

• Freiberg’s disease is more common in girls.

• Most of those afflicted have a congenitally long second metatarsal,
or a short first metatarsal, both of which cause excessive pressures
on the head of the second metatarsal.
• The patient complains of pain in the fore foot on standing and
walking.
• Examination reveals local thickening and tenderness as well as
painful restriction of motion in the metatarsal phalangeal joint.
• The radiographic appearance is typical of all osteochondroses
(Fig. 13.19).
• Non-operative treatment by means of low heeled shoes and a stiff
rocker-bottom sole may relieve the symptoms from the
complication of degenerative joint disease.
• Frequently, excision arthroplasty ( accomplished by removal of the
distorted metatarsal head or of the base of the phalanx) is
necessary for permanent relief.
 OSTEOCHONDROSIS OF SECONDARY CENTERS OF
OSSIFICATION IN THE SPINE
(SCHEUERMANN'S DISEASE)
Incidence and Etiology
• This fairly common but poorly understood disorder, which affects
both boys and girls, usually begins at puberty and progresses during
adolescence until vertebral growth has ceased in the late teens.
• In at least some of the patients, the disorder is inherited with an
autosomal dominant pattern.
• It commonly involves the epiphyseal plates (physes) of three or four
adjoining vertebral bodies in the midthoracic region.
Pathogenesis and Pathology
• A consistent finding in the involved vertebral bodies is the presence
of herniation of the intervertebral disc through the anterior portion
of the epiphyseal plate into the body of the vertebra (Schmorl's
node).
• As a result, there is less disc material between the vertebral bodies,
and the intervertebral disc space narrows.
Clinical Features and Diagnosis
• The child is usually noticed by the parents, or by a school physician,
to have "poor posture or "round shoulders" at about the time of
puberty.
• Examination reveals an exaggerated kyphosis in the thoracic region
and a compensatory exaggerated lordosis in the lumbar region (Fig.
13.20).
• There may be local tenderness over the Spinous processes of the
involved thoracic vertebrae.
• The intervertebral diss spaces are uniformly narrow but the
involved vertebral bodies are wedge-shape (Fig. 13.21 ).
Treatment
• An effective method of treatment to help prevent such progression
of deformity during growth is the Milwaukee brace which is
normally used for scoliosis (lateral curvature), but which can be
modified for the treatment of kyphosis.
• Such a brace is used for approximately 1 year.
 SPECIFIC OSTEOCHONDROSES OF PRIMARY CENTERS
OF OSSIFICATION

Osteochondrosis of the Tarsal Navicular (Kohler's Disease)

• During the early phase of necrosis, Kohler's disease is symptomless,
but in the phase of revascularization, the child usually complains of
mild pain in the mid-foot and tends to walk withan antalgic limp.
• Examination reveals local tenderness and swelling due to a synovial
effusion in the region of the navicular.
• The radiographic findings are comparable to those already
described for all osteochondroses earlier chapter (Fig. 13.22).
Treatment
• A walking cast may be required for a few weeks to relieve an
episode of acute pain, but this is unusual.
• Williams and Cowell have reported that the short-term use of such
a cast reduces the duration of symptoms from more than 1 year to
3 months.
Osteochondrosis of the Lunate (Kienbock's Disease)
• The child may complain of mild back pain but is otherwise healthy.
• Examination reveals a slight kyphosis and, occasionally, muscle
spasme.
• Radiographic examination reveals a striking change in the vertebral
body, the ossified part of which becomes wafer thin and sclerotic
(Fig. 13.24 ).
• Because this disorder is self limiting, treatment is aimed at relieving
symptoms.
• A temporary spinal brace usually is sufficient.
 TANGENTIAL AVASCULAR NECROSIS OF A PRESSURE
EPIPHYSIS (OSTEOCHONDRITIS DISSECANS}

Incidence and Etiology

• Osteochondritis dissecans usually occurs in older children and
young adults; boys are afflicted more frequently than girls.
• The etiology of the initial avascular necrosis in osteochondritis
dissecans is not known.
• The observations that this clisease is sometimes familial and, in
some patients, associated with osteochondrosis elsewhere, suggest
that there may be a preexisting abnormality in the epiphysis that
plays a predisposing role.
Clinical Features and Diagnosis
• There are usually neither symptoms nor clinical signs during the
quiet, early phase of necrosis.
• During the revascularization phase, the patient may experience
intermittent local pain with relatively little disturbance of joint
function
• Examination reveals a moderate synovial effusion in the joint, slight
disuse atrophy of surrounding muscles, but little restriction of joint
motion.
• Radiographically, the lesion is characterized by a small isolated
segment of subchondral bone separated from its bed by a
radiolucent line that represents soft tissue (Figs. 13.25 and 13.26).
Treatment
• This involves restriction of activity and may even necessitan
temporary relief of weightbearing from the joint to encourage
healing.
• If the segmen is large and involves a weightbearing surface the
joint, particularly in the knee, arthroscopic surgery involving
insertion of peg shaped bone grafts accelerate healing of the
fragmen bed and prevent its separation.
 NONTRAUMATIC OSTEONECROSIS OF THE
FEMORAL HEAD IN ADULTS

• This idiopathic type of femoral head necrosis in adults also known

as Chandler's disease.
• More often seen in middle-aged persons who have a history of
some generalized disorder such as alcoholism, or who have
received systemic adrenocorticosteroids (as reported by Cruess) for
an unrelated condition.
• The patient complains of severe pain, either in the hip or referred to
the knee, and notices a slowly progressiv stiffening of the joint.
• The earliest diagnosis is possible with MRI.
• The sclerotic segment may be demarcated from the rest of the head
by irregular areas of rarefaction and sclerosis; it may have collapsed,
or become impacted, with resultant incongruity of the joint (Fig.
13.27).
• Treatment frequently involves surgical operations, such as a varus
osteotomy of the femur or a Sugioka-type osteotomy that rotates
the femoral head and neck "upside down, so that the uninvolved
part of the femoral head comes to bear weight.

• If, however, the entire femoral head is involved, the patient will
require a prosthetic hip joint replacement, either unipolar or
bipolar, depending on the state of the acetabulum.
 NONTRAUMATIC OSTEONECROSIS OF
THE KNEE IN ADULTS

• This disorder, a form of avascular necrosis, is also known as

spontaneous osteonecrosis of the knee or SONK

• The average age at onset is older than 60 years, and it occurs more
commonly in women than men.

• Although acute, severe pain in the knee may precede radiographic

changes by 6 months.
• The diagnosis can be made earlier by scintigraphy (a bone scan)
because of increased uptake of the radionuclide in the medial
femoral condyle, which indicates an attempt at revascularization of
the necrotic bone.

• It can be managed by either an osteocartilaginous allograft, as

advocated by Gross, or a high tibial osteotomy if the area of
necrosis is not extensive; otherwise, a prosthetic knee joint
replacement is indicated.
 PARTIAL AVULSION OF THE TIBIAL TUBERCLE
(OSGOOD-SCHLATTER‘S DISEASE)

• The child complains of local pain aggravated by kneeling on

the tibial tubercle, by direct blows, and by running.
• Clinically, a prominent subcutaneous swelling, some of
which is due to reaction in the soft tissues, is apparent in
the region of the tibial tubercle (Fig. 13.28).
• Radiographically, the proximal part of the tibial tubercle
exhibits irregular areas of bone deposition and bone
resorption when compared to the tubercle on the normal
side (Fig.13.29).
• Osgood-Schlatter's disease is usually self limiting, in which
case the tibial tubercle becomes completely ossified over a
period of about 2 years.
 POSTTRAUMATIC AVASCULAR NECROSIS OF
SUBCHONDRAL BONE

• In children, posttraumatic avascular necrosis of a pressure epiphysis

is most likely to occur after fracture separation of the upper femoral
epiphysis or after traumatic dislocation of the hip.
• In adults, fractures of the neck of the femur and traumatic
dislocation of the hip are the most common causes of posttraumatic
a vascular necrosis.
 DISORDERS OF EPIPHYSEAL GROWTH
SLIPPED UPPER FEMORAL EPIPHYSIS
(ADOLESCENT COXA VARA)
Incidence and Etiology
• Slipping of the upper femoral epiphysis is most likely to develop in
older children and adolescents, from me age of 9 years to the end
of growth, and is more common in boys than girls.
• The etiology of slipped upper femoral epiphysis is not entirely
understood.
• The experimental investigation of Harris suggests than an imbalance
between growth hormone and sex hormones.
Pathogenesis and Pathology
• The femoral epiphysis usually slips slowly and progressively and
leads to a progressive coxa vara deformity with secondary
remodeling of the femoral neck; the posterior periosteal
attachment remains intact.
• An acute injury superimposed on this pathological process may
cause a sudden further slip- an acute-on-chronic slip.
Clinical Features and Diagnosis
• The most common initial symptom is mild discomfort arising in the
hip but referred to the knee.
• In the early stages there is usually a slight limp, most noticeable
when the patient is tired.
• As the slip progresses, a Trendelenburg-type gait develops (the
patient's trunk leans toward the affected side as weight is borne on
the affected lower limb.
• Radiographic examination of the upper end of the femur in two
projections. A minimal slip is always more obvious in the lateral
projection than in the anteroposterior projection (Fig. 13.34).
Treatment
• The aim of treatment in the early stages is to prevent further slip of
the epiphysis.
• If the femoral head has slipped chronically and minimally (less than
1 cm in the lateral projection), it should be surgically stabilized in
situ by means of a centrally placed cannulated threaded screw with
the guidance of an image intensifier, after which weightbearing may
be resumed.
 TIBIA VARA (BLOUNT'S DISEASE}

• This disorder is more common in girls than boys.

• In the early stages of tibia vara, there are no symptoms.
• Examination reveals a characteristic varus deformity of the knee, a
deformity that is particularly striking when it is unilateral.
• Radiographically, there defective ossification of the medial portion
the upper tibial epiphysis, a beaked appcarance of the underlying
metaphysis, and obvious retardation of longitudinal growth in the
medial side of the tibia (Fig. 13.39).
• Treatment in the early stages of tibia vara in young children is
aimed at preventing progression of the varus deformity.
• This can sometimes be accomplished by means of night splint of the
type used for physiological bow legs.
• In older children, the varus deformity progresses despite splinting.
It can be corrected only by osteotomy of tibia, which may have to
be repeated on one or more occasions during the remaining growth
period.
 MADELUNG'S DEFORMITY

• An epiphyseal growth clisturbance may devliop on the meclial

(ulnar) side of the distal radial epiphysis as the result of a localized
form of epiphyseal dysplasia.
• It is characterized by prominence of the distal end of the ulna on the
dorsum of the wrist and forward displacement of the hand in
relation to the forearm (Fig. 13.40).
• Treatment is aimed at correction of the rather ugly
deformity as well as improvement of wrist function.

• This is best accomplished by excision of the distal portion of

the ulna and osteotomy of the deformed distal end of the
radius.
 SCOLIOSIS

• The broad term scoliosis refers to a lateral curvature of the spine;

thus, scoliosis is a deformity rather than a specific disease or
disorder.
• At the outset a few terms should be defined :
1 nonstructural scoliosis is a reversible lateral curvature of the spine
without rotation.
2 structural scoliosis is an irreversible lateral curvature of the spine
with rotation of the vertebral bodies in the abnormal area (major
curve).
Idiopathic Scoliosis
Incidence and Etiology
• Idiopathic scoliosis is a relatively common musculoskeletal
deformity in approximately 0.5% of the population.
• The infantile type, which appears between birth and 3 years of age,
is more common in boys and, for reasons unknown.
• The juvenile type, which appears between the ages of 4 and 9 years,
and the more common adolescent type, which first becomes
apparent between the ages of 10 years and the end of growth.
Pathogenesis and Pathology
• The most important aspect of the pathogenesis of the deformity of
scoliosis is its progression with skeletal growth that is particularly
rapid during adolescence.
• As the lateral curvature and the coexistent rotation of the spine
increase, secondary changes develop in the vertebrae and ribs due
to progressive growth disturbance.
Clinical Features and Diagnosis
• Idiopathic scoliosis begins slowly, insidiously, and painlessly. Thus, in
the early stage of its development, the patient is not aware of the
curvature, and because it is well concealed by clothing at this stage,
the parents are not aware of it either.
• Later, the parents may observe that one shoulder is higher than the
other, one shoulder blade is more prominent than the other, or one
hip protrudes more than the other.
• Radiographic examination, which should include an anteroposterior
and lateral projection of the full length of the spine in the standing
position, reveals a curvature that is always more marked than
would be expected from the external physical appearance.
• Assessment of the scoliosis by MRl is indicated in the presence of
any neurological deficit, neck stiffness, or headache.
Treatment
• Nonoperative Methods.
Exercises designed to prevent the progression of idiopathic
scoliosis have been proven ineffectual, as have body casts.
• Operative Treatment.
Idiopathic scoliosis with a curve of more than 40° that is, is best
treated by the combined operation of mechanical correction of the
curvature by internal spinal instrumentation and spinal fusion.
 SPONDYLOLYSIS

Incidence and Etiology

• Once thought to be a congenital defect, sporndylolysis is now
known to develop during postnatal life.
• Moreover, the incidence spondylolysis has been discovered to
increased with age not only during the growing year but also during
adult life.
• The most common type is secondary to the aforementioned
bilateral defect in the pars interarticularis of the neural arch
(spondylolysis).
Clinical Features and Treatment
• In the majority of individuals with spondylolysis, the defect
produces neither symptoms nor signs.
• After an injury or chronic strain, however, the fibrous tissue in the
defect may be stretched. The resultant pain may persist for many
months and necessitate the use of a lumbosacral-type brace.
 SPONDYLOLISTHESIS

Incidence and Etiology

• Some degree of spondylolisthesis of a lower lumbar vertebra is
detectable in approximately 2% of adults.
• The most common type is secondary to the aforementioned
bilateral defect in the pars interarticularis of the neural arch
(spondylolysis).
Clinical Features and Diagnosis
• Spondylolytic spondylolisthesis usually becomes manifest during
childhood by the gradual onset of low back pain that is aggravated
by standing, walking, and running and relieved by lying down .
• The associated clinical deformity, which is characterized by a "step"
in the lumbosacral region at the level of the spondylolisthesis and
an increased lumbar lordosis above (Fig. 13.55 ).
Treatment
• Spondylolisthesis may cause no symptoms, in which case the
patient should be examined clinically and radiographically at regular
intervals to detect any progression.
• Progressive forward slip is an indication for stabilization of the
unstable segment by means of a local spinal fusion.
• The latter is the most effective type of fusion for spondylolisthesis.