COSESCA LEAKAGE

SUFE

Which of the following categories of slipped capital femoral epiphysis (SCFE) is associated with
the highest rate of bilaterality:
1) Idiopathic SCFE
3) Radiation therapy
2) Renal failure
5) Growth hormone deficiency
4) Hypothyroidism
Renal failure is associated with the highest risk (90%) of bilaterality. By contrast, idiopathic
SCFE has a 20% risk of bilaterality
initially and a further 10% to 20% risk until maturity.
Correct Answer: Renal failure

Which of the following factors should prompt a workup for an underlying disorder in a patient
with slipped capital femoral
epiphysis:
1) A bilateral slip
3) Weight under the 50th percentile for age
2) Weight over the 50th percentile for age
5) A slip of grade II or higher
4) A positive family history
A positive age-weight test is defined as a patient who is under the 50th percentile of weight for
age or older than 16 years of age
at presentation with slipped capital femoral epiphysis. These patients should have a workup for
underlying renal or endocrine
disease. A positive family history is found in 5% to 10% of patients with slipped capital femoral
epiphysis.
Correct Answer: Weight under the 50th percentile for age

Which of the following categories of slipped capital femoral epiphysis (SCFE) is associated with
the highest risk of a grade III
slip:
1) Idiopathic SCFE
3) Radiation therapy
2) Renal failure
5) Growth hormone deficiency
4) Hypothyroidism
A grade III slip refers to more than 50% displacement of the epiphysis. Renal failure is
associated with the highest risk (43%) of
progressing to a grade III slip. Fortunately, these slips are usually stable.
Correct Answer: Renal failure

A 13-year-old boy has a left slipped capital femoral epiphysis which has displaced 75%. He is
unable to bear weight on the limb.
The other hip has no clinical or radiographic abnormalities. Your preferred treatment is which of
the following:
1) Gentle reduction of the slip and fixation with a cannulated screw
3) Cuneiform osteotomy
2) Hip spica cast
5) Free vascularized fibular grafting
4) Subtrochanteric osteotomy
This is an unstable slip. It has a much higher chance of avascular necrosis than a stable slip.
Since the degree of the slip will
increase the shear forces across the healing physis and decrease the function of the hip, some
method of improving this is
justified. Gentle reduction of the epiphysis, without forceful internal rotation, may increase the
risk of avascular necrosis.
Avascular necrosis is a significant risk in many series of cuneiform (metaphyseal; Fish)
osteotomies. Subtrochanteric osteotomy is
not justified in patients with an acute slip until it is healed. It has a high rate of chondrolysis.
Free vascularized bone graft may be
an option if avascular necrosis develops, but is not indicated at this time. A hip spica cast is also
often followed by chondrolysis
and delayed epiphyseodesis.Correct Answer: Gentle reduction of the slip and fixation with a
cannulated screw

Of all slipped capital femoral epiphyses, which percentage is unstable:

1) 5%
3) 25%
2) 15%
5) 50%
4) 35%
Unstable slipped capital femoral epiphysis places the patient at a high risk of avascular necrosis
(up to 47%). Fortunately, it
comprises only about 5% of all slips.Correct Answer: 5%

Which of the following factors is not associated with increased risk for progressive slippage after
internal fixation of slipped
capital femoral epihysis:
1) Acute component
3) Cushing disease
2) Hyperthyroidism
5) Male gender
4) Persistent pain after fixation
All of the factors listed, except gender, have been implicated in cases with increased progression
of slipped capital femoral
epiphysis even after pinning. Patients with these factors need close follow-up with a prolonged
period of nonweightbearing or
redirectional osteotomies.Correct Answer: Male gender

A 13-year-old boy who underwent in situ fixation of slipped capital femoral epiphysis 1 year ago
calls your office to complain of
knee pain on the other side. He is able to bear his weight on the leg. You recommend:
1) No sports for 1 month and an office visit if the symptoms continue
3) Crutches and an office visit within 24 hours
2) An office visit within the next 2 weeks for evaluation
5) Arthroscopy of the knee
4) Magnetic resonance image of the knee and an office visit if the results are abnormal
This patient most likely has a contralateral slipped capital femoral epiphysis. It may even be in
the â preslipâcategory. Acute
progression to an unstable slip is possible at any time and may lead to avascular necrosis and
permanent loss of motion.
Therefore, urgent examination with physical examination and plain radiographs is necessary.
Correct Answer: Crutches and an office visit within 24 hours

Which of the following conditions has the highest rate of bilaterality:

1) Slipped capital femoral epiphysis
3) Developmental dysplasia of the hip
2) Legg-Calvé-Perthes disease
5) Fibular hemimelia
4) Discoid lateral meniscus
Slipped capital femoral epiphysis has a bilaterality rate of 25% to 40%. Legg-Calvé-Perthes
disease, developmental dysplasia of
the hip, discoid lateral meniscus, and fibular hemimelia have bilateral rates of less than
20%.Correct Answer: Slipped capital
femoral epiphysis

Patients who have had a unilateral slipped capital femoral epiphysis (SCFE) are at what odds of a
contralateral slip compared to
the general population:
1) 2 times
3) 200 times
2) 20 times
5) 20,000 times
4) 2,000 times
Patients who have presented with a unilateral SCFE are at approximately 2,000 times the risk of
a contralateral slip compared to
the general population. The risk of a contralateral slip in a juvenile is approximately 1/10,000,
which provides justification for
prophylactic pinning of a second side if a surgeon thinks it is warranted.Correct Answer: 2,000
times

A patient with slipped capital femoral epiphysis (SCFE) should have an endocrine workup if
presenting with which of the following
features:
1) Bilateral involvement
3) Age <10 or >15 years
2) Body mass index greater than the 95th percentile for age
5) Female gender
4) Negative family history
Endocrine workup is only indicated for age ,10 or .15 years, or stature less than the 10th
percentile. Bilaterality, obesity, and
negative family history are common findings in idiopathic SCFE. Although SCFE is more
common in males, it is not uncommon in
females.Correct Answer: Age <10 or >15 years

DDH
Risk factors for developmental dysplasia of the hip (DDH) include all of the following except:
1) Oligohydramnios
3) Congenital muscular torticollis
2) Firstborn child
5) Male gender
4) Identical twin with DDH
Developmental dysplasia of the hip is caused by both genetic and mechanical factors. Crowding
and laxity are common factors.
Females, pregnancies with oligohydramnios, firstborn children, infants with congenital muscular
torticollis, and those with an
affected identical twin are at increased risk. Also parental hip dysplasia, congenital dislocation of
the knee, and breech position
increase the risk of DDH.Correct Answer: Male gender

Upon review of a plain film series for developmental dysplasia of the hip (DDH),
contraindication to periacetabular osteotomy is
suggested by:
1) Cup medialization
3) Center edge angle of 5°
2) Excessive acetabular index
5) No cartilage space maintained on abduction view
4) Anterior coverage of less than 5° on false profile
None of the options necessarily preclude periacetabular osteotomy as a treatment option for
DDH provided that the patient wishes
to proceed; however, little or no cartilage space, or poor concentric reduction of hip joint would
suggest poor outcome with this
procedure.
Correct Answer: No cartilage space maintained on abduction view

The result of treatment of developmental dysplasia of the hip with Salter osteotomy is is worse
with which of the following:
1) A higher degree of the dislocation before treatment (Tonnis grade)
3) Higher preoperative acetabular index
2) Bilaterality
5) Performing open reduction at a separate surgery than the osteotomy
4) Increased age at surgery
Salter osteotomy is effective in treating developmental dysplasia of the hip in young children.
The result is worse with higher
degrees of dislocation as assessed by the Tonnis system. It is better if the open reduction (if
needed) is performed as a separate
step than the osteotomy. The other factors have not been shown to be predictive.Correct Answer:
A higher degree of the
dislocation before treatment (Tonnis grade)

A 4-year-old girl with developmental hip dysplasia is advised to have a Salter innominate
osteotomy. When the family asks
about the long-term survivorship of the reconstruction, the surgeon tells them that good 30-year
follow-up results are likely in at
least what percentage of patients:
1) 30%
3) 70%
2) 50%
5) 98%
4) 85%
Thirty-year survivorship analysis shows good to excellent results in at least 85% of
patients.Correct Answer: 85%

PERTHES DISEASE
Which rating best describes a childâ s hip affected with Perthesâdisease that has healed with
aspherical incongruity:
1) Catterall 3
3) Herring C
2) Catterall 4
5) Stulberg 5
4) Stulberg 4
The Catterall and Herring classifications are used during the evolution of Perthesâdisease to
guide treatment; they are used
during the fragmentation stage. It is impossible to know what the Catterall or Herring class was
after healing has occurred. The
Stulberg classification gives prognosis after healing. Stulberg 4 is aspherical but congruous.
Stulberg 5 is aspherical and
incongruous. Patients classified with Stulberg 5 hips typically have degenerative joint disease in
early adulthood.
Correct Answer: Stulberg 5

A 5-year-old girl is evaluated for Legg-Calvé-Perthes disease. She is in the fragmentation

phase, and rotation is 10° internal
and 15° external. Her epiphyseal extrusion index is 15%. Recommended treatment includes:
1) Femoral osteotomy
3) Traction
2) Iliac osteotomy
5) Observation
4) Shelf procedure
The patientâ s restricted range of motion is normal for a patient in the fragmentation phase of
Legg-Calvé-Perthe disease. The
patientâ s degree of epiphyseal extrusion is not extreme. Observation is the most appropriate
course of treatment.Correct Answer:
Observation

What is the risk of hip arthroplasty for 50-year-old patients with Legg-Calvé-Perthes disease:
1) 10%
3) 33%
2) 25%
5) 75%
4) 50%
Long-term study has shown that 50% of 50-year-old patients with Legg-Calvé-Perthes disease
will have degenerative changes
in their hips significant enough to make them candidates for hip arthroplasty.Correct Answer:
50%

A 5-year-old patient with Legg-Calvé-Perthes disease has lateral pillar B involvement. He has
15% epiphyseal extrusion.
Treatment should consist of:
1) Observation
3) Iliac osteotomy
2) Femoral osteotomy
5) External fixation
4) Combined femoral and iliac osteotomy
Patients who have Legg-Calvé-Perthes disease with onset before 6 years of age have a good
prognosis unless they have lateral
pillar involvement in the B/C border or C categories.Correct Answer: Observation

CLUB FOOT
Equinovarus positioning of the foot is normal during which stage of embryonic life:
1) No stage
3) Thirteenth to fifteenth week
2) Eighth to tenth week
5) Twentieth to twenty-third week
4) Seventeenth to nineteenth week
As the foot matures, it passes through a normal stage when it resembles a clubfoot in the eighth
to tenth week. After this, the foot
normally corrects itself.Correct Answer: Eighth to tenth week

The Ponseti method of clubfoot cast treatment starts with which of the following steps:
1) Pronation of the forefoot
3) Dorsiflexion of the ankle
2) Dorsiflexion of the first ray
5) Internal rotation of the foot
4) External rotation of the foot
Dorsiflexion of the first ray is the first step in the Ponseti method of cast treatment. Dorsiflexion
decreases the cavus component.
Pronation of the forefoot is the opposite of dorsiflexion and produces the opposite desired
motion.
Dorsiflexion of the ankle should be performed after the Achilles tenotomy.
External rotation of the foot is performed later.
Internal rotation of the clubfoot is never done.
Correct Answer: Dorsiflexion of the first ray

When correcting a clubfoot by the Ponseti method, the lateral mold on the foot must be placed
against which of the following
structures:
1) The head of the talus
3) The cuboid
2) The calcaneus
5) The tibia
4) The fifth metatarsal
Ponseti describes rotating the clubfoot against the head of the talus to allow the lateral side of the
foot (calcaneus and cuboid) to
rotate laterally.Correct Answer: The head of the talus

Which of the following is a principle of the Ponseti technique for correction of a clubfoot:
1) Avoid use of casts
3) Perform a comprehensive release
2) Avoid use of braces
5) Minimize surgery to bones and joints
4) Osteotomies produce correction
The Ponseti technique, which was developed and tested by Ignacio Ponseti, MD, involves slow,
gradual correction of a clubfoot
using casts, a tenotomy if necessary to release the Achilles tendon, and maintenance of
correction using braces (foot abduction
orthoses) for several years. The technique avoids dissection of the growing bones and joints of a
childâ s foot because of the
associated risks of stiffness and growth disturbance.Correct Answer: Minimize surgery to bones
and joints

Which of the following physical findings is uncommon in a patient with a unilateral untreated
clubfoot:
1) Thin calf
3) Plantar-medial prominence of the talus in the foot
2) Loss of prominence of the heel
5) Decreased length of the foot
4) Medial midfoot crease
In a patient with unilateral untreated clubfoot, certain findings are apparent when compared with
the unaffected foot. Common
findings include a thinner calf, shorter-appearing foot, loss of heel prominence, and a medial
midfoot crease. The talus is
prominent anterolaterally in the foot but not on the plantar-medial side.
Correct Answer: Plantar-medial prominence of the talus in the foot

Prior to treatment, this pathologic finding characterizes clubfoot:

1) The talar head and neck are deviated medially.
3) The navicular is positioned more laterally than normal.
2) The dome of the talus is flattened.
5) The tendoachilles inserts in a more lateral position than normal.
4) The foot has a rocker deformity.
The talar head and neck are deviated medially. This deformity cannot be corrected surgically but
may improve with growth.
The dome of the talus is not originally flattened, but it may become this way after repeated
manipulation.
The navicular is positioned more medially than normal and may touch the medial malleolus.
The foot has either a normal arch or a cavus deformity (the opposite of a rocker bottom). The
rocker bottom foot may
develop as a result of over-vigorous manipulation of a clubfoot against a tight heelcord.
The tendoachilles inserts are more medially positioned than normal on the calcaneus.
Correct Answer: The talar head and neck are deviated medially.

In the surgical correction of a clubfoot, the following clinical or radiographic finding indicates
that a child should have a plantar
release:
1) Anterior extrusion of the talus
3) Medial subluxation of the calcaneocuboid joint
2) Inferior subluxation of the calcaneocuboid joint
5) Lack of ability to dorsiflex the ankle
4) Plantarflexion of the first ray
Plantarflexion of the first ray, especially if the first ray is not colinear with the talus on the lateral
radiograph, should serve as an
indication for a plantar release.
Anterior extrusion of the talus is common because of the tightness of the Achilles tendon and the
posterior capsule.
Inferior subluxation of the calcaneocuboid joint is not a feature of clubfoot.
Medial subluxation of the calcaneocuboid joint, in combination with the clinical appearance of a
curved lateral border of the
foot, should serve as an indication for a lateral release, not a plantar release.
Inability to dorsiflex the ankle is an indication for a posterior release.
Correct Answer: Plantarflexion of the first ray

SCOLIOSIS
For a patient who has thoracic idiopathic scoliosis of the surgical range, the distance between the
thecal sac and the apical
thoracic pedicle on the concave side is:
1) Less than 1 mm
3) 3 mm
2) 2 mm
5) 5 mm
4) 4 mm
The distance between the apical thoracic pedicle and the thecal sac is less than 1 mm on the
concave side.Correct Answer: Less
than 1 mm

The width of the pedicles in a patient with idiopathic scoliosis in the surgical range is narrowest
in the:
1) Thoracic spine on the convex side
3) Sacral spine
2) Lumbar spine on the convex side
5) Lumbar spine on the concave side
4) Thoracic spine on the concave side
The width of the pedicles is less in the thoracic spine than in the lumbar or sacral spine, and less
on the concavity than on the
convexity. The mean width of the thoracic pedicles on the concave side at the apex in skeletally
mature patients is only 3
mm.Correct Answer: Thoracic spine on the concave side
Platelet calmodulin levels correlate with which of the following phenomena:
1) Progressive adolescent idiopathic scoliosis
3) Progressive slipped capital epiphysis
2) Progressive Scheuermann kyphosis
5) Progressive clubfoot
4) Bilateral Legg-Perthes disease
Platelets contain actin and myosin and, therefore, have some features similar to skeletal muscle.
Calmodulin is a calcium-binding
receptor protein that regulates intracellular calcium. Platelet calmodulin levels are increased in
progressive adolescent idiopathic
scoliosis.Correct Answer: Progressive adolescent idiopathic scoliosis

Patients with which one of the following curve types associated with idiopathic scoliosis are at
increased risk of shortness of
breath:
1) Thoracic curves larger than 80°
3) Thoracolumbar curves larger than 100°
2) Thoracolumbar curves larger than 80°
5) Lumbar curves larger than 100°
4) Lumbar curves larger than 80°
Idiopathic scoliosis is associated with measurable decreases in pulmonary function in thoracic curves
larger than 60° to 70°, but
a clinically significant increase in risk of shortness of breath has been shown only in thoracic curves
larger then 80°.
Correct Answer: Thoracic curves larger than 80°

Which of the following patients with infantile idiopathic scoliosis can be observed without a magnetic
resonance image (MRI):
1) A patient with a curve less than 20°
3) A patient with a right thoracic curve
2) A patient with a normal neurologic examination
5) A patient without any pain
4) A patient younger than 2 years old
Patients with idiopathic infantile scoliosis have a 22% incidence of abnormalities that can be viewed on
MRI. Syrinx and Chiari
malformation are the most common abnormalities, with a similar rate as that found in patients with
juvenile idiopathic scoliosis.
The left thoracic curve pattern is most commonly seen in infantile patients, but a right thoracic curve
is not protective. Age,
freedom from pain, or a normal neurologic examination are also not protective. Magnetic resonance
imaging is recommended for
all infantile curves larger than 20°.
Correct Answer: A patient with a curve less than 20°

In patients with idiopathic adolescent scoliosis, clinically noticeable shortness of breath from restrictive
lung disease begins to
occur at a mean curve of:
1) 45° thoracic
3) 55° thoracic
2) 45° lumbar
5) 95° thoracic
4) 75° thoracic
Clinically noticeable pulmonary compromise begins to occur at a mean curve of 75° thoracic,
although a decrease can be
measured by pulmonary function testing with curves as little as 50°. Cor pulmonale does not occur
until a curve is larger then
100°.
Correct Answer: 75° thoracic

Which of the following statements is true of patients with idiopathic scoliosis not treated surgically
(long-term follow-up data)
when compared to nonscoliotic controls:
1) Patients had an increased mortality rate.
3) Patients had an increased prevalence of back pain.
2) Patients had an increased rate of neuropathy.
5) Patients had an increased rate of spinal fractures.
4) Patientsâcurves did not worsen after adulthood.
Patients with idiopathic scoliosis have a mortality rate that is not statistically different from the general
population. They do not
have a significant risk of neuropathy or spine fracture. Their curves worsen in maturity if they are
greater than approximately 40Â
°. They also have an increased prevalence of back pain.
Correct Answer: Patients had an increased prevalence of back pain.

In patients with single thoracic idiopathic scoliosis treated with posterior pedicle screw constructs, the
distal extent of the fusion
may be stopped at which of the following levels with respect to the neutral vertebra and still routinely
maintain balance:
1) Three levels above
3) One level above
2) Two levels above
5) Two levels below
4) One level below
In single thoracic idiopathic scoliosis, the distal extent of the fusion may be stopped at one level above
the neutrally rotated
vertebra and still maintain trunk balance.
Correct Answer: One level above
Numerous braces are available for controlling spinal deformities in growing children. Which type of
brace is presented here
(Slide):
1) Boston brace
3) Milwaukee brace
2) Charleston brace
5) Providence brace
4) Wilmington brace
The Milwaukee brace is the first modern scoliosis brace to demonstrate efficacy and to achieve
widespread use. The Milwaukee
brace is a cervicothoracolumbar orthosis. All of the other braces listed are thoracolumbosacral
orthoses.
The Boston brace is prefabricated and is selected from measurements taken of the patient.
The Charleston brace is a night-only brace and bends the patient to reverse the curve.
The Wilmington brace is made of thermoplastic material for rapid, on-site fabrication.
The Providence brace is made using computer-assisted machining to help achieve maximal correction.
Correct Answer: Milwaukee brace
A patient has idiopathic scoliosis measuring 62° thoracic convex to the right and 40° lumbar convex
to the left (Slide). The
sagittal film shows a relatively decreased thoracic kyphosis and decreased lumbar lordosis. The patient
is 13 years old and is 2
months postmenarchal. Which of the following treatments is most appropriate:
1) Milwaukee brace treatment
3) Posterior fusion from T4 to L3
2) Posterior fusion from T4 to L2
5) Posterior fusion from T4 to L5
4) Posterior fusion from T4 to L4
This curve may be classified as a King type II or III. The curve is too large for bracing. Only the
thoracic curve must be fused.
Because the stable vertebra is L2, the fusion does not need to go below this level. Other acceptable
options include anterior
thoracic instrumentation and fusion from T4 to T12 or posterior instrumented fusion to L1.Correct
Answer: Posterior fusion from
T4 to L2
This radiograph (Slide) shows a 9-year-old boy with scoliosis. From which of the following conditions is
the boyâ s scoliosis likely
to have resulted:
1) Marfanâ s syndrome
3) Neurofibromatosis
2) Osteogenesis imperfecta
5) Juvenile idiopathic scoliosis
4) Fibrous dysplasia
The patientâ s scoliosis is the result of neurofibromatosis. A sharp focal curve over few vertebrae,
thinning of apical pedicles, and
spindling of the ribs are symptoms of neurofibromatosis that are indicated in the radiograph.Correct
Answer: Neurofibromatosis

Based on the clinical photograph (Slide 1) and radiographs (Slide 2) of this 11-year-old boy, which of
the following conditions is
demonstrated:
1) Idiopathic scoliosis
3) Tuberous sclerosis
2) Fibrous dysplasia
5) Diastematomyelia
4) Neurofibromatosis 1
This patient has many typical features of neurofibromatosis 1: subcutaneous neurofibromas, large â
coast of Californiaâcafe-aulait spot with several others, and a dystrophic curve at a young age.
Surgery is recommended.Correct Answer: Neurofibromatosis
1

Which of the following statements is true of scoliosis in patients with Charcot-Marie-Tooth disease:
1) Scoliosis responds well to bracing.
3) Scoliosis is rarely progressive.
2) Spinal cord monitoring is usually normal.
5) Thoracic kyphosis is less than in idiopathic scoliosis.
4) Thoracic curves more commonly have an apex convex to the left than the right.
Scoliosis is more common in patients with Charcot-Marie-Tooth disease than in the general population
(between 10% to 33%).
Only 15% of curves are halted by bracing. Scoliosis is commonly associated with thoracic kyphosis
and with a left thoracic curve
pattern. Intraoperative spinal cord monitoring is often difficult to elicit even at baseline due to the
underlying neurologic
disorder.Correct Answer: Thoracic curves more commonly have an apex convex to the left than the
right.

A clinical photograph (Slide 1) and radiographs (Slide 2) of a 13-year-old girl, who is neurologically
normal, are presented. She
does not report any pain. The most likely diagnosis is:
1) Fibrous dysplasia
3) Idiopathic scoliosis
2) Blue-rubber bleb nevus syndrome
5) Klippel-Trenaunay syndrome
4) Neurofibromatosis-1
This patient has neurofibromatosis-1. She has a dystrophic scoliosis that is sharply angulated and
involves only four vertebrae
(Cobb levels T12-L3). The pedicles are thinned, and the endplates are scalloped. She also has
subcutaneous
neurofibromas.Correct Answer: Neurofibromatosis-1
Risk factors for superior mesenteric artery syndrome after adolescent idiopathic scoliosis surgery
include all of the following
except:
1) Decreased correction of thoracic curve with preoperative bending
3) Lenke lumbar modifier B or C
2) Body mass index (BMI) below the 25th percentile
5) Use of iliac crest bone graft
4) Staged surgical correction
Superior mesenteric artery syndrome occurs more often in patients with decreased BMI, larger and
stiffer thoracic curves, lumbar
apical translation of Lenke B or C, and two staged procedures.Correct Answer: Use of iliac crest bone
graft

An infant presents with idiopathic scoliosis. Which of the following factors indicates observation as the
treatment of choice:
1) Age younger than 1 year
3) Rib-vertebra phase 2
2) Rib-vertebral angle difference >20°
5) Left thoracic curve
4) Curve of 47°
Rib-vertebral angle difference >20° rib-vertebra phase 2, and curves >45° indicate a likely
progression. The majority of infantile
curves are left thoracic, so this is not a factor. A patient younger than 1 year of age has a higher
chance of spontaneous
resolution than an older patient. Therefore, absent other risk factors, observation is the initial
recommend treatment.Correct
Answer: Age younger than 1 year

Which of the following factors predicts a lesser degree of impairment of pulmonary function in patients
with adolescent idiopathic
scoliosis:
1) Increased Cobb angle
3) Increased cephalad apex of the curve
2) Decreased number of vertebrae in the curve
5) Pectus excavatum in addition to the scoliosis
4) Decreased thoracic kyphosis
An increased Cobb angle, increased cephalad apex of the curve, decreased thoracic kyphosis, and
pectus excavatum in addition
to the scoliosis are factors that predict a greater degree of pulmonary impairment. A lower number of
vertebrae in the curve
signals less impairment.Correct Answer: Decreased number of vertebrae in the curve

All except which of the following neurologic/muscular disorders can present undiagnosed in a patient
with scoliosis:
1) Friedreichâ s ataxia
3) Syringomyelia
2) Charcot-Marie-Tooth disease
5) Spinal cord tumor
4) Duchenne muscular dystrophy
Scoliosis can be caused by any neurologic disorder that affects trunk balance. Scoliosis is seen in most
patients with Friedreichâ s
ataxia, a disorder in which patients present with an ataxic gait in preadolescence or adolescence. The
curve becomes significant at
about the same time as the ataxia. Scoliosis can also be seen in 10% of patients with Charcot-Marie-
Tooth disease. This condition
is characterized by cavus feet, intrinsic atrophy, and occasional hip dysplasia; no significant pain or
clumsiness is present.
Scoliosis is seen in many patients with syringomyelia. The syrinx and scoliosis both develop silently
with no noticeable weakness
until both the syrinx and the curve are significant. Spinal cord tumor may present with scoliosis in an
ambulatory patient.
Duchenne muscular dystrophy leads to scoliosis in most patients, but the patients become
nonambulatory several years before
the curve develops.Correct Answer: Duchenne muscular dystrophy

All except which of the following structural disorders often causes scoliosis and presents undiagnosed
in patients:
1) Marfan syndrome
3) Osteogenesis imperfecta
2) Ehlers-Danlos syndrome
5) Achondroplasia
4) Loeys-Dietz syndrome
Although achondroplasia causes kyphosis, it is not associated with scoliosis to a significant degree.
Marfan syndrome, EhlersDanlos syndrome, osteogenesis imperfecta, and Loeys-Dietz syndrome (a
defect in TGF-beta receptor protein) are frequently
associated with scoliosis.Correct Answer: Achondroplasia

In the workup of scoliosis, what is/are the best method(s) to assess skeletal maturity during a
patientâ s peak height velocity
(Risser grade 0):
1) Greulich and Pyle atlas (hand/wrist radiographs)
3) Tanner-Whitehouse-III RUS (radius, ulna, small bones of hand) method
2) Modified Sauvegrain method (olecranon radiographs)
5) B, C, and D
4) Triradiate cartilage closure
Precise information about skeletal maturity during peak growth rate is important in deciding treatment
options in patients with
scoliosis. The modified Sauvegrain method, Tanner-Whitehouse-III RUS method, and triradiate
cartilage closure are each accurate
and reliable markers of maturity that the orthopedic surgeon can use to quickly assess pediatric
patients.
Correct Answer: B, C, and D

Three years ago, a 20-year-old female patient underwent posterior spinal fusion for adolescent
idiopathic scoliosis. She asks
what would happen if the implants were removed. The surgeonâ s most likely response will be:
1) The spine will not change alignment, if the fusion is solid.
3) The scoliosis is likely to increase, even if the fusion is solid.
2) The sagittal alignment will likely change, even if the fusion is solid.
5) Increased back pain is likely.
4) Fracture of the fusion mass is likely.
After removal of implants, the sagittal alignment is likely to change, with kyphosis increasing by 10º
in nearly half of all patients
and by more than 20º in 10% of patients. By contrast, scoliosis increases significantly in only 5% of
patients. Most patients who
have implants removed experience an improvement in symptoms. Fracture of the fusion mass is rare.
Correct Answer: The sagittal alignment will likely change, even if the fusion is solid.

Which of the following syndromes has the highest incidence of scoliosis as a percentage of affected
patients:
1) Marfan syndrome
3) Down syndrome
2) Loeys-Dietz syndrome
5) Prader-Willi syndrome
4) Rett syndrome
Marfan syndrome, due to a mutation in fibrillin-1, causes spinal deformity in two-thirds of patients.
Rett syndrome, caused by a
mutation in MECP-2, results in neurological balance disorder and may cause scoliosis in up to half of
affected patients. Scoliosis is
seen in smaller numbers of patients with Prader-Willi, Down, and Loeys-Dietz syndromes.
Correct Answer: Marfan syndrome

A 2-year-old boy with a congenital heart anomaly has a 40° thoracolumbar curvature. Standing
posteroanterior and lateral
radiographs reveal vertebral anomalies indicative of congenital scoliosis. Which of the following
patterns of congenital scoliosis
has the worst prognosis for progression?
1) Block vertebrae
3) Fully segmented hemivertebra
2) Unilateral unsegmented bar
5) Nonsegmented hemivertebra
4) Unilateral unsegmented bar with a contralateral fully segmented hemivertebra
Congenital spinal deformity is caused by structural abnormalities in the vertebrae that can result in
asymmetric growth, such as
scoliosis or kyphosis. It has been classified in 2 types. Type I involves defects of formation and type II
involves defects of
segmentation. However, in many instances, deformities can be a mixture of both. Defects of formation
include segmented or
unsegmented hemivertebrae and wedge vertebrae. Defects of segmentation include block vertebrae,
unilateral bars, or unilateral
bars with hemivertebrae.
The potential for progression is dependent on the growth potential of the anomalies. The presence of
healthy-appearing disks
between the hemivertebra and its normal counterparts indicates good growth potential and risk for
progression. A unilateral bar
on the opposite side of a segmented hemivertebra acts as a tether on the concave side of the curve
and has the most likelihood
for progression.
Children with congenital scoliosis also have a significant incidence of associated anomalies, both
intraspinal and other organ
systems. About 30% have a spinal dysraphism such as diastematomyelia, meningocele or lipoma.
Other associated anomalies
include Klippel-Feil syndrome (25%), genitourinary tract abnormalities (30%), cardiac defects (12%),
and Sprengelâ s deformity
(10%).
Correct Answer: Unilateral unsegmented bar with a contralateral fully segmented hemivertebra

Six months ago, an 11-year-old premenarchal girl with adolescent idiopathic scoliosis had a right
thoracic curve from T5 to T12
measuring 20°. Her physical examination was normal. She returned to the office and a standing
posteroanterior radiograph
demonstrates a 28° right thoracic curve from T5 to T12; she is Risser stage 0. A lateral radiograph
shows a thoracic kyphosis
of 10°. At this time, you recommend:
1) Repeat radiograph in 6 months
3) Full-time use of a thoracolumbosacral orthosis
2) Thoracic flexibility exercises
5) Posterior spinal fusion with instrumentation
4) Electrical stimulation
In skeletally immature patients with adolescent idiopathic scoliosis and curves approaching 30° with
documented progression,
bracing may be effective at preventing further progression of the curve. Risk of progression in
adolescent idiopathic scoliosis is
related to curve magnitude and remaining growth potential. The risk of further progression in this
patient is 68%, and bracing is
indicated. Electrical stimulation and physical therapy have not been shown to affect the natural history
of scoliosis. Surgery may
be indicated in patients with more severe curves. In the sagittal plane, hypokyphosis is usually
present in adolescent idiopathic
scoliosis.Correct Answer: Full-time use of a thoracolumbosacral orthosis

A 15-year-old boy with adolescent idiopathic scoliosis has a right thoracic curve from T5 to T11
measuring 45° and a left
thoracolumbar curve from L1 to L4 measuring 32°. He is Risser stage 2 and has a hypokyphotic
thoracic spine. Bending films
demonstrate moderate flexibility in the lumbar curve. He was prescribed a thoracolumbosacral
orthosis since age 14, but his
scoliosis has progressed. His physical exam reveals a prominent right rib hump and mild right shoulder
elevation. His head is
centered above his pelvis. His neurological examination is normal. You recommend:
1) Continued full-time use of the orthosis until skeletal maturity
3) Repeat evaluation in 6 months
2) Discontinuation of the orthosis due to failure
5) Posterior spinal fusion of the thoracic curve only with instrumentation
4) Posterior spinal fusion T5-L4 with instrumentation
This patient has a right thoracic curve with a compensatory left lumbar curve pattern of adolescent
idiopathic scoliosis. There has
been documented progression into the surgical range despite bracing, and he still has some growth
remaining. Surgical
intervention is indicated. This curve pattern (King II, Lenke D) can be approached posteriorly with
thoracic fusion alone to the
neutral and stable vertebra and instrumentation to obtain and maintain correction. The unfused
lumbar curve will spontaneously
correct to balance the fused thoracic curve. Care must be taken to avoid fusion into the lower lumbar
spine and preserve motion
segments.Correct Answer: Posterior spinal fusion of the thoracic curve only with instrumentation

A 1-year-old male infant is referred by his pediatrician for evaluation of possible scoliosis. Otherwise,
he is healthy. His physical
exam reveals normal neurologic function, plagiocephaly and a flexible thoracic curve. Radiographs
reveal a left thoracic curve
with a Cobb angle of 36° and no vertebral anomalies. The apical ribs are in Phase I, and the rib-
vertebral angle difference is
18°. At this time, management should include:
1) Observation
3) Full-time use of a thoracolumbosacral orthosis
2) Serial body casting to obtain correction
5) Spinal instrumentation without fusion
4) Posterior spinal fusion
Infantile idiopathic scoliosis is rare in this country and not well understood. It is more common in
Europe, occurs more frequently
in boys, and left thoracic curves predominate. Plagiocephaly, or a flattening of the posterior skull on
the convex side of the spinal
curvature, is frequently found in these patients, suggesting a postural cause of both.
There are 2 types of infantile idiopathic scoliosis: resolving and progressive. Distinguishing between
the 2 types has obvious
consequences regarding prognosis and treatment. Prior to Mehtaâ s work, identification of the type of
infantile idiopathic scoliosis
was difficult, because it was not related to curve magnitude, age at onset, rate of progression, or
degree of rotation.
Mehta showed that the 2 groups were distinguishable by the relationship of the ribs to the apical
vertebral body on the
posteroanterior radiograph. Ribs that do not overlap the vertebral body are in Phase I, and ribs that
do overlap the vertebral body
on the convexity of the curve are in Phase II. The rib-vertebral angle is constructed by the intersection
of a line perpendicular to
the apical vertebral endplate with a line drawn along the long axis of the corresponding rib. The rib-
vertebral angle (RVA)
difference is the difference of the RVA of the concave and convex ribs of the apical vertebra. In
scoliosis, the convex ribs form a
more acute angle than the concave ribs, so this difference is >0. Mehta concluded that curves in which
the ribs are in Phase I and
the RVA difference is < 20° have a better prognosis (resolving type) and require just observation.
Treatment for progressive
curves includes serial body casts, orthoses, or surgery for severe curves. Various surgical approaches
include posterior spinal
fusion, instrumentation without fusion to allow spinal growth, or anterior convex hemiepiphysiodesis
with posterior
hemiarthrodesis.Correct Answer: Observation