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152
Fig. 7.10 A child with severe bowlegs, termed genu
varus. (From Tachdjian MO. Pediatric Orthopedics, 6th
ed. Philadelphia, PA: Herring; 2022. Reprinted with
permission)
Table 7.2 Etiologies of rickets
1. Vitamin D dietary deciency
2. Malabsorption states
3. Renal rickets
(a) Tubular defects (congenital)
(b) Glomerular disease (acquired)
4. Miscellaneous
(a) Neurobromatosis
(b) Phenytoin-associated
Hematologic Disease
Sickle Cell Disease
D. B. Kraft et al.
seen in sickle cell disease can occur anywhere in
the bone but are more typical in the metaphysis.
These children are also predisposed to osteomyelitis due to the already sludged vessels in the
metaphysis, predisposing the bone to bacterial
trapping. Even though Staphylococcus is the most
common organism retrieved, this patient population is also susceptible to infection with
Salmonella. This organism gains access to the circulatory system through small infarcts in the
intestinal wall and then enters the bone hematogenously. The treatment for the infarcts is appropriate hematologic care—hydration, analgesics, etc.
Antibiotic selection for osteomyelitis should take
into consideration the incidence of salmonella.
Leukemia
This is the most common malignancy of childhood, and the skeleton is not spared its ravages.
The bones by X-ray will show nondescript lytic
changes most characteristically seen in the
metaphyseal region and referred to as “metaphyseal banding.” The areas of osteopenia parallel
and adjacent to the physis; although suggestive of
leukemia, they are not pathognomonic of it.
Usually, the diagnosis has been made well before
skeletal complications develop; however, occasionally a child will present for the evaluation of “growing pains” only to have a workup reveal this disease.
Ordinarily “growing pains” occur in children
2–7years of age, affect primarily the legs, are symmetric (although not simultaneous), occur in early
evening or just after going to bed, and are NOT
associated with any systemic complaints. Any variation from the usual pattern should suggest a basic
workup to include X-rays and a complete blood
count with further diagnosis made with bone marrow biopsy. Patients with leukemia usually present
before 4 years of age with recurrent infections,
bleeding, fatigue, and lymphadenopathy.
The red cell deformation that occurs in sickle cell
patients due to the abnormal hemoglobin is
responsible for the skeletal changes. The abnormally shaped cells cause stasis and sludging in
small arterioles and capillaries, resulting in disrupted ow and bony necrosis. The bony infarcts
Congenital andNeurodevelopmental
This is the largest and most nondescript “wastebasket” of pathologic states, many of which have
severe impact on the pediatric skeleton. Included
7 Pediatric Orthopedics
here are congenital birth defects of no known etiology, such as proximal femoral focal deciency,
as well as genetic diseases transmitted in classic
Mendelian fashion (e.g., hemophilia) or due to
chromosomal defects (e.g., Down’s syndrome).
In addition, the neuromuscular diseases frequently have an immense impact on the skeleton,
as aberrant and eccentric muscular forces are created. Unfortunately, it is difcult to nd many
common themes that make an appreciation of the
skeletal impact easier to understand.
Osteogenesis Imperfecta
This disease is transmitted in a classic autosomal
dominant pattern with only rare exception. The
basic defect is one of abnormal collagen synthesis due to impotent osteoblasts. For this reason, it
has been grouped with other “sick” cell syndromes. Certainly, the osteoblasts are normal in
number but incapable of normal synthetic activity. The collagenous product of their incompetence is poorly formed and poorly cross-linked,
making it weak.
The subsequent bone that is made is similarly
architecturally thin and mechanically weak
(Fig. 7.11). The severity of the disease is as
expected—a function of the dose of abnormal
genetic material. Some of the severe homozygotes are stillborn due to intracranial bleeds
occurring in the perinatal period. As with most
genetic diseases, penetrance varies such that
some children have multiple fractures and severe
shortening and others less involved have only the
occasional fracture.
Typically, the bones are osteopenic with
thinned cortices and decreased diameter.
Multiple fractures with resulting deformities are
expected. These fractures respond to appropriate treatment, and healing is only slightly prolonged. Occasionally, it is necessary to correct
long-bone deformities operatively by performing multiple osteotomies in a single bone and
lining the resultant fragments up on an intramedullary rod that is capable of lengthening
with subsequent growth (Fassier-Duval growing
rod, Fig.7.12).
153
Fig. 7.11 The skeleton in severe osteogenesis imperfecta. (From Tachdjian MO. Pediatric Orthopedics, 6th
ed. Philadelphia, PA: Herring; 2022. Reprinted with
permission)
Scoliosis can also complicate this disease, and
its management can be very challenging, especially if surgical management is required to correct the deformity. It is very difcult to use spinal
instrumentation in the face of this osteopenic,
softened bone.
Almost all patients with osteogenesis imperfecta are seen by geneticists and primary care
clinics who guide the administration of bisphosphonates. Bisphosphonates inhibit osteoclasts
which increase the cortical diameter and cancellous bone density to effectively reduce fracture
incidence, pain, and improve ambulation in this
population.
Down Syndrome
First described in England by Langdon Down in
the 1800s, this syndrome has been shown to result
from a trisomy of the number 21 chromosome. It
154
abcde
D. B. Kraft et al.
Fig. 7.12 A 6-year-old girl with osteogenesis imperfecta
treated with a Fassier-Duval (FD) rod. (a) Patient had
acute bending of the male component after a fall. The FD
rod was placed 4years earlier. Radiograph also shows loss
of anchoring of the distal threaded portion of the male
component from the distal femoral epiphysis and proximal migration. (b) Radiograph after revision of the FD
rod with a larger diameter rod and a longer distal threaded
portion on the male component. (c) At age 8, distal migra-
is the most common chromosomal abnormality
and it occurs in approximately 1in 500 live births.
Because of its frequency, it is the prototype for the
other chromosomal abnormalities and the orthopedic manifestations tend to be somewhat common to all.
The many musculoskeletal problems experienced by children with Down syndrome are
largely related to the hypotonia, joint hypermobility, and ligamentous laxity that typify the
group. The ligamentous laxity results from an
inordinate number of elastic bers relative to the
number of collagen bers in ligament and joint
capsule. The joint changes typical of this disease
and other chromosomal diseases can be traced
directly to this ligamentous laxity. Specic manifestations include the following:
• C1–C2 instability: Due to laxity of the trans-
verse ligament of the odontoid process, anterior
translation of C1 on C2 occurs, frequently at
tion of the threaded head of the female component distal
to the greater trochanter was noted. (d) Revision of the
female component and repositioning of the threaded head
to the tip of the greater trochanter and bone grafting is
shown. (e) Follow-up radiograph at age 12 shows telescoping of the rod. (From Tachdjian MO. Pediatric
Orthopedics, 6th ed. Philadelphia, PA: Herring; 2022.
Reprinted with permission)
alarming degrees. Routine lateral cervical spine
radiographs in exion and extension should be
regularly obtained in these children to evaluate
them for this problem. This is particularly
important in the pre-participation evaluation
for competition in sporting activities.
• Hip subluxation and dislocation can occur
insidiously over time, again resulting from the
capsular laxity about the joint.
• Patellar subluxation is the cause of the typical
gait seen in the older child with Down syndrome. These children often walk with a stifflegged gait in an effort to preclude patellar
subluxation.
• Hypermobile atfeet and bunions are common, and management is primarily directed at
controlling the deformity, if possible, and
minimizing the pain, which is rarely a signicant problem. Despite xed deformities, it is
frequently surprising how well these children
compensate.
7 Pediatric Orthopedics
155
• Scoliosis is common and managed similarly
to those with idiopathic scoliosis.
• SCFEs are also common and more likely to be
unstable and high grade at presentation with
higher rates of osteonecrosis.
Skeletal Dysplasias
There are several hundred recognized skeletal
dysplasias, each with its own unique clinical
characteristics and specic skeletal abnormalities
(Fig.7.13). It is impossible to recall all of the features, which dene a given dysplastic condition,
especially in light of the fact that each is quite
rare. At best, generalizations can be employed to
assist in the diagnosis of a specic patient and
thereby guide the appropriate workup and referral to an individual skilled in denitive diagnosis.
The anticipated orthopedic problems, treatment,
and prognosis will hinge on the diagnosis.
When presented with an individual displaying
dysplastic ndings, especially short stature, chromosomal evaluation and standard X-rays are
good starting points once appropriate history
(especially family history) and a careful physical
examination have been carried out. The X-rays
should include a lateral of the cervical and thoracolumbar spine, an anteroposterior view of the
pelvis, and anteroposterior views of the wrists
and the knees. These views will allow one to
evaluate epiphyseal, physeal, metaphyseal, and
diaphyseal growth and their aberrations.
Most of the dysplasias tend to affect a specic
region of the bone; by assessing each region,
clues regarding the specic type of dysplasia can
narrow the differential. For example, spondyloepiphyseal dysplasia affects primarily epiphyseal growth as the name implies. One should
expect to see deformities of the epiphyseal nuclei
and disordered apophyseal growth. Conversely,
achondroplasia is a defect in physeal growth and
will, therefore, produce signicant shortening;
in fact, it is the most common cause of pathologic short stature.
Most of the skeletal dysplasias are genetically
transmitted, and a careful family history will
dene the pattern. Many, however, are spontaneous mutations or without a dened etiology. It is
important to keep in mind that by denition a
skeletal dysplasia is a GENERALIZED affectation of the skeleton with all bones showing some
changes. Obviously, the end of the bone growing
more rapidly will demonstrate the defect to a
greater degree; thus, the knee and wrist lms are
more likely to show changes than the hip or
elbow lms.
Achondroplasia
As an example of how a dysplasia affects the
skeleton, one should consider the most common,
achondroplasia. Transmitted as an autosomal
dominant mutation in FGFR3in most cases, it is
usually apparent at birth. The infant will be rhizomelically shortened; that is to say, the proximal
segment of the limbs is relatively shorter than the
middle or distal segments. In addition, the child
is disproportionately built since the limbs are
preferentially involved and, therefore, very short
relative to the spine and trunk. These children
follow the growth curve but several standard
deviations below normal, achieving a mature
height between 3 and 4ft. As with all of the true
dysplasias, intelligence is not impaired and life
expectancy is near normal.
Clinical Features
The child’s head shows attening of the nasal
bridge and prominent frontal bones (Fig. 7.14).
Both ndings are due to the disparity between the
normal intramembranous calvarial growth and
the retarded enchondral growth of the basilar portions of the skull. The extremities are short, with
each of the bones being short in length, but relatively normal in girth since periosteal bone formation remains relatively unaffected. The spine
and pelvis also show some decrease in height but
of greater signicance is the decrease in the interpedicular distance which effectively creates spinal stenosis. This, coupled with a hyperlordotic
156
l
Hyper
Hyperplasias Hypoplasias
Hyperchondroplasia
Enchondromatosis
D. B. Kraft et al.
Spondyloepiphysea
dysplasia
Multiple epiphyseal
dysplasia
Achondroplasia
Metaphyseal
dysostosis
Hypophosphatasia
Familial exostosis
Progressive
diaphyseal dysplasia
phosphatasemia
Osteopetrosis
Craniometaphyseal
dysplasia
Osteogenesis
imperfecta
Osteoporosis
Fig. 7.13 Dynamic classication of bone dysplasias. (From Tachdjian MO.Pediatric Orthopedics, 6th ed. Philadelphia,
PA: Herring; 2022. Reprinted with permission)
lumbar spine, leads to the development of symptoms at an early age. A major problem of the
older adolescent is obesity, which complicates
many of the other abnormalities. As adults, problems with multiple tendonitises and bursitises are
commonplace.
7 Pediatric Orthopedics
Fig. 7.14 A 6-year-old child with achondroplasia. Note
that his ngers reach to the level of his hips. (From
Tachdjian MO.Pediatric Orthopedics, 6th ed. Philadelphia,
PA: Herring; 2022. Reprinted with permission)
Neuromuscular Disease
Unlike the skeletal dysplasias which are intrinsic
abnormalities of the skeleton, neuromuscular
disorders are extrinsic but drastically alter the
normal skeleton due to the muscle imbalances
they create.
Common themes can be seen that emphasize
the fact that the problem is disparity in the agonist–antagonist relationship. Major joints tend
to dislocate, with the hip being a prime example.
The exor pattern tends to become dominant,
causing the femoral head to dislocate posteriorly. Scoliosis should be expected as asymmetry
of spinal muscle action alters normal balance. If
the neurologic defect is asymmetric, as in polio,
then the growth plates in one leg will experience
a different muscle pull than those of the other
and a leg-length discrepancy can be anticipated.
157
Cerebral Palsy (CP)
CP is a static neurologic disease of children due
to an insult to the immature brain during the perinatal period. The defect is, therefore, central,
damaging the normal inhibitory inuences on the
peripheral gamma efferent system. Without central dampening, the peripheral reex arc functions autonomously, and the result is increased
tone or spasticity.
Cerebral palsy can be classied physiologi-
cally or geographically.
Physiologic Classication
• Spastic: Hypertonia, hyperexia, and contractures are seen. This is the most common form
of the syndrome.
• Athetoid: This is far less common today than
it was in years past. Rh incompatibility and
erythroblastosis fetalis were a common etiology of this form.
• Rigid.
• Ballismic.
• Mixed.
Geographic Classication
• Hemiplegia: The most common form, affecting one side of the body (upper and lower
extremity), frequently associated with seizures.
• Diplegia: Both lower extremities predominate
the pattern, the person is usually still ambulatory.
• Quadriplegia: The most severe cases involve
children, with total body involvement, many
of whom exhibit cognitive decits and few of
whom will ever walk.
Cerebral palsy is really a syndrome rather
than a disease, and no two children are the
same. This makes comparison of procedures
and other treatments extremely difcult. The
muscles all tend to be spastic; however, the
muscle imbalance is created between spastic
and more spastic muscles. Contractures, joint
dislocations, limb deformities, and scoliosis
should all be anticipated.
158
D. B. Kraft et al.
Polio
With the introduction of the Salk vaccine in 1954,
this disease has become rare in the United States;
however, it is certainly not eradicated and may be
seen particularly in areas with high immigration
rates. The polio virus has unique predilection for
the anterior horn cells of the cord and the bulbar
portion of the brain. In most cases, the involvement is spotty, and the degree of paralysis is variable. The victim is left with a mix of normal
muscle, weak muscle, and absent muscle, creating
a broad spectrum of muscle imbalance in an
asymmetric distribution. It is important to remember that the sensory bers are NOT affected,
which gives these children a clear and distinct
benet over the children with spina bida.
Spina Bida
Despite the improvement in antenatal testing,
many children with myelodysplasia are born in
the United States each year (Fig. 7.15). Due to
open cord defects at a certain level, these children
have congenital paraplegia, lacking motor and
sensory modalities below the level of the defect.
The higher their level of defect, the poorer their
function, and hence, the prognosis. For example,
a child with a T12 level (the spinal roots that are
the last to function are T12) has no motor power
and no sensation below the waist. These children
will be wheelchair-conned and have bowel and
bladder compromise. Conversely, children with
an S1 level (the last functioning spinal level is
S1) will have only minimal motor involvement
and will usually walk without braces. Their major
problems are the bowel and bladder malfunction.
The absence of sensation below the level of the
lesion creates many additional problems for these
children. Not unlike a diabetic patient with severe
neuropathy, children with spina bida are prone to
foot ulceration, infection, and the development of
neuropathic joints. One recently identied problem
in this group is latex allergy. Perhaps due to repeated
catheterization with latex rubber catheters, these
patients can become severely sensitized to all latex
contact, to the point of anaphylaxis. Specic protocols are now used at the time of surgical procedures
to avoid contact with any latex products, including
gloves, catheters, and IV tubing.
Lastly, it is important to realize that these chil-
dren, as well as many of those with cerebral
palsy, are multiply handicapped. They can have
learning difculties, perceptual problems, hearing and visual impairments, and emotional
issues—all of which require a coordinated effort
by multiple specialists to provide optimal care.
Fig. 7.15 Clinical appearance of untreated myelomeningocele sac. Note the large protrusion of the meninges,
without protective skin. Breakdown of the sac usually
occurs, followed by further neurologic injury, meningitis,
and potentially encephalitis. (From Tachdjian
MO. Pediatric Orthopedics, 6th ed. Philadelphia, PA:
Herring; 2022. Reprinted with permission)
Regional Orthopedic Problems
The Pediatric Hip
Most of the showcase pediatric orthopedic maladies affect the hip. Several unique anatomic features predispose this joint to long-term problems
following septic, vascular, developmental, and
traumatic insults.
In the newborn, the upper end of the femur is
entirely cartilaginous, representing the secondary
ossication centers of both the greater trochanter
and the femoral head (capital femoral epiphysis)
as a composite chondroepiphysis. The two bony
ossication centers will develop within this one
cartilage mass and grow differentially to their
7 Pediatric Orthopedics
159
ultimate adult size and shape. Implicit in this fact
is that the growth of one is dependent on the
growth of the other. Normally, the bony centrum
of the capital femoral epiphysis should be radiographically visible by 3–6months of age.
The growth of this epiphysis is dependent primarily on the blood supply of the upper end of
the femur. Up until 1year of age, there is communication between the metaphyseal and epiphyseal circulations that protects the capital femoral
epiphysis from isolation in the event of an insult
to the epiphyseal side.
Unfortunately, as the physis thickens and
matures by 18 months of age, it becomes an
impenetrable barrier between the two circulations, leaving the epiphysis of the head totally
dependent on the epiphyseal vessels for its viability. Less than 10% of the femoral head is supplied
by the branch of the obturator artery through the
ligamentum teres. The epiphyseal vessels are
supplied by the medial and lateral circumex
branches of the femoral artery (Fig.7.16). This
vascular isolation of the upper end of the femur is
LCA
MCA
largely responsible for the disastrous complications of developmental dislocation of the hip
(DDH), Perthes’ disease, and slipped capital
femoral epiphysis (SCFE).
The acetabulum develops from two cartilage
segments. The rst is the triradiate cartilage, a
bilaminar physis that forms at the junction of the
ilium, ischium, and pubis. Integrity of this growth
plate is essential for acetabular height to be normal. The depth of the acetabulum is a function of
the cartilaginous labrum that circumferentially
surrounds the developing acetabulum. The acetabulum and proximal femur are forming simultaneously throughout development, and
aberrations of one will affect the normal development of the other.
Developmental Dysplasia oftheHip
(DDH)
The previous nomenclature “congenital dislocation” was changed to “developmental dislocation” in recognition of the fact that most of these
hips are located at birth and go on to dislocate in
the postnatal period. The incidence of this condition is about 1 per 1000 live births and is more
common in females. Although it is fair to say that
the etiology is unknown, it is important to recognize that there are both genetic and environmental
factors; hence, it is considered a multifactorial
trait. DDH is a true dysplasia (i.e., aberrant
growth), and NOT simply a femoral head that is
not located in the acetabulum. It is important to
stress this fact to the parents to assist them in
understanding the pathology. DDH encompasses
a spectrum of pathology ranging from acetabular
dysplasia to a subluxatable hip to dislocatable hip
to dislocated hip (Fig.7.17).
Early diagnosis is the key to optimal treatment
and the best prognosis. First, consider the risk
factors:
Fig. 7.16 Blood supply to the femoral head from the
medial circumex artery (MCA) and lateral circumex
artery (LCA), branches of the profunda femoris artery at the
level of the tendinous portion of the iliopsoas muscle. (From
Tachdjian MO.Pediatric Orthopedics, 6th ed. Philadelphia,
PA: Herring; 2022. Reprinted with permission)
• First-born
• Female
• Intrauterine breech positioning
• Positive family history
• Oligohydramnios
• Macrosomia
160
ab
Capsule
Labrum inverted
Transverse acetabular
ligament pulled upward
Ligamentum teres
elongated
Labrum from posterior and
superior border of acetabulum
interposed between femoral
epiphysis and acetabulum,
preventing reduction of femoral
head
D. B. Kraft et al.
Capsular
adhesions
Ligamentum teres
Fibrofatty pulvinar
in acetabulum
Fig. 7.17 Pathology of the dislocated hip that is irreducible as a result of intraarticular obstacles. (a) The hip is
dislocated. (b) The hip cannot be reduced on exion,
abduction, or lateral rotation. Obstacles to reduction are
inverted limbus, ligamentum teres, and brofatty pulvinar
With these in mind, a careful physical examination of the hips is the logical next step. In the
newborn, one should attempt to demonstrate laxity and instability. The Barlow test is performed
with the infant supine and the hips exed
(Fig. 7.18). As the hips are brought from the
abducted to adducted position, a positive test is
noted as the femoral head subluxates posteriorly
over the posterior rim of the acetabulum. This
would indicate instability. The Barlow is a provocative test: the hip is located, and the maneuver
dislocates it. Conversely, the Ortolani test is a
reduction maneuver; the hip is dislocated, and the
test reduces it (Fig.7.19). This is accomplished
by abducting the adducted hip and noting a palpable (but rarely audible) “clunk” as the femoral
head reduces over the posterior acetabular rim.
As the child gets older (by 3 months), the
dislocated hip tends to become xed in that
position, and the classic signs of instability disappear in favor of those indicating a xed dislo-
in the acetabulum. The transverse acetabular ligament is
pulled upward with the ligamentum teres. (From Tachdjian
MO. Pediatric Orthopedics, 6th ed. Philadelphia, PA:
Herring; 2022. Reprinted with permission)
cation deformity. Limited abduction is the most
important nding to note. Examining the child
on a rm surface, subtle differences in the
degrees of hip abduction may herald a dislocated hip on the restricted side. Similarly, viewing knee height with the child supine and the
hips and knees exed may reveal a positive
Galeazzi sign-one knee higher than the other—
again indicating a dislocation on the low side
(Fig.7.20).
Imaging studies are important in both diagnosis and treatment. Before 3months of age, much
of the proximal femur is cartilaginous and therefore not visible on X-ray, ultrasound is used up
until this time. Ultrasound has been helpful in the
diagnosis of DDH, as well as in dening relatively subtle degrees of acetabular dysplasia
(Fig. 7.21). The value of ultrasound after the
child is 3 months old decreases, and standard
X-rays assume a more central role. After
3months, many classic measurements are made
7 Pediatric Orthopedics
161
Fig. 7.18 The Barlow
test for developmental
dislocation of the hip in
a neonate. (a) With the
infant supine, the
examiner holds both of
the child’s knees, gently
adducts one hip, and
pushes posteriorly. (b)
When the examination is
positive, the examiner
will feel the femoral
head make a small jump
(arrow) out of the
acetabulum (Barlow
sign). When the pressure
is released, the head is
felt to slip back into
place. (From Tachdjian
MO.Pediatric
Orthopedics, 6th ed.
Philadelphia, PA:
Herring; 2022.
Reprinted with
permission)
a
b
Fig. 7.19 The Ortolani
test for developmental
dislocation of the hip in
a neonate. (a) The
examiner holds the
infant’s knees and gently
abducts the hip while
lifting up on the greater
trochanter with two
ngers. (b) When the
test is positive, the
dislocated femoral head
will fall back into the
acetabulum (arrow) with
a palpable (but not
audible) “clunk” as the
hip is abducted (Ortolani
sign). When the hip is
adducted, the examiner
will feel the head
redislocate posteriorly.
(From Tachdjian
MO.Pediatric
Orthopedics, 6th ed.
Philadelphia, PA:
Herring; 2022.
Reprinted with
permission)
a
b