Kay SPJ: Cleft Hand, in Green DP (Ed) : Green's Operative Hand Surgery. Philadelphia, Pa, Churchill Livingston, 1999, PP 402-414

1.
The inheritance of the deformity shown in Figure 1 is most commonly:
1- Autosomal-recessive.
2- Autosomal-dominant.
3- X-linked dominant.
4- Mitochondrial.
5- Sporadic.
PREFERRED RESPONSE: 2
DISCUSSION: Cleft hand and cleft foot malformations are commonly inherited as
autosomal-dominant traits and are associated with a number of syndromes. An autosomal-
recessive and an x-linked inheritance pattern have also been described, but these are much
less common and are usually atypical. In the common autosomal-dominant condition, nearly
one third of the known carriers of the gene show no hand or foot abnormalities. This is
known as reduced penetrance. The disorder may be variably expressed; affected family
members often exhibit a range from mild abnormalities in one limb only to severe anomalies
in four limbs. Variable expressivity and reduced penetrance can cause difficulty in
counseling families regarding future offspring in an affected family. Many patients have a
cleft hand that may be caused by the split-hand, split-foot gene (SHFM1) localized on
chromosome 7q21.
 Kay SPJ: Cleft hand, in Green DP (ed): Green's Operative Hand Surgery. Philadelphia, Pa, Churchill Livingston, 1999,
pp 402-414.
2. Examination of a 12-year-old girl with bilateral anterior knee pain reveals

excessive femoral anteversion and excessive external tibial torsion. The patient
has no patellofemoral instability. Nonsurgical management consisting of muscle
strengthening and nonsteroidal medication has failed to relieve the patient's
pain. Treatment should now consist of:
1- Corrective osteotomies with internal rotation of the distal part of the tibia and/or
external rotation of the femur.
2- External rotation of the distal part of the tibia.
3- Internal rotation of the distal part of the femur.
4- Arthroscopic retinacular release.
5- A patellar realignment procedure.
DISCUSSION: Children with symptomatic severe torsional malalignment of the lower
extremity and patellofemoral pathology show excessive femoral anteversion and external
tibial torsion on physical examination and analysis of gait. The functional effect of this
torsional malalignment is centered about the knee joint. If nonsurgical management fails to
alleviate patellofemoral pain, definitive surgical treatment should consist of corrective
osteotomies, including internal rotation of the distal part of the tibia or external rotation of the
femur, or both. Patients with surgical correction by osteotomy show an improved gait pattern
and appearance of the extremity and a marked decrease in knee pain. External rotation of the
distal part of the tibia or internal rotation of the distal part of the femur worsens the torsional
malalignment. No additional soft-tissue realignment procedures, including retinacular release
or patellar realignment, are required.
 Delgado ED, Schoenecker PL, Rich MM, Capelli AM: Treatment of severe torsional malalignment syndrome. J Pediatr
Orthop 1996;16:484-488.
3. Which of the following patients with cerebral palsy is considered the ideal
candidate for a selective dorsal rhizotomy?
1- An ambulatory 6-year-old patient with spastic diplegia
2- An ambulatory 10-year-old patient with spastic right hemiplegia
3- An ambulatory 16-year-old patient with spastic diplegia
4- A nonambulatory 8-year-old patient with spastic quadriplegia
5- A nonambulatory 18-year-old patient with rigid quadriplegia
DISCUSSION: The enthusiasm with which dorsal rhizotomy was received led to the
broadening of selection criteria with poorer results. The ideal candidate is an ambulatory 4-
to 8-year-old child with spastic diplegia who does not use assistive devices or have joint
contractures. The child must be old enough to actively participate in the rigorous
postoperative physical therapy program. The use of the procedure in an ambulatory 16-year-
old patient is less desirable because joint contractures will most likely have developed to a
varying degree. The hemiplegic child is best treated by orthopaedic interventions.
 Oppenheim WL: Selective posterior rhizotomy for spastic cerebral palsy: A review. Clin Orthop 1990;253:20-29.
 Renshaw TS, Green NE, Griffin PP, Root L: Cerebral palsy: Orthopaedic management. J Bone Joint Surg Am
1995;77:1590-1606.
 Richards BS (ed): Orthopaedic Knowledge Update: Pediatrics. Rosemont, Ill, American Academy of Orthopaedic
Surgeons, 1996, pp 19-27.
4. A 3-year-old boy sustains a complete paralysis following a high thoracic spinal

cord injury consistent with a SCIWORA-type injury (spinal cord injury without
radiographic abnormality). Subsequent progressive spinal deformity will
develop in what percent of patients with this injury?
1- 10%
2- 25%
3- 50%
4- 75%
5- Greater than 75%
DISCUSSION: Spinal cord injury in skeletally immature patients almost always leads to the
development of paralytic spinal deformity. The age at injury is the most important factor
affecting the development of scoliosis. Spinal cord injury that occurs more than 1 year prior
to skeletal maturity is almost always followed by the development of scoliosis. In one study,
scoliosis developed in 100% of children who were younger than age 10 years at the time of
spinal cord injury. Scoliosis can occur after injury at any level. Spasticity is often a
contributing factor. Up to two thirds of patients who have paralytic scoliosis prior to skeletal
maturity will eventually require surgery for curve control.
 Mayfield JK, Erkkila JC, Winter RB: Spine deformity subsequent to acquired childhood spinal cord injury. J Bone Joint
Surg Am 1981;63:1401-1411.
 Lancourt JE, Dickson JH, Carter RE: Paralytic spinal deformity following traumatic spinal cord injury in children and
adolescents. J Bone Joint Surg Am 1981;63:47-53.
 Dearolf WW III, Betz RR, Vogel LC, Levin J, Clancy M, Steel HH: Scoliosis in pediatric spinal cord injured patients. J
Pediatr Orthop 1990;10:214-218.
5. A 12-year-old girl has progressive development of cavus feet. Examination

reveals slightly diminished vibratory sensation on the bottom of the foot.
Reflexes are 1+ at the knees and ankles. Motor examination shows that all
muscles are 5/5 in the foot, except the peroneal and anterior tibial muscles are
rated as 4+/5. Which of the following studies is considered most diagnostic?
1- Nerve conduction velocity studies

2- Biopsy of the quadriceps femoris muscle
3- Biopsy of the sural nerve
4- DNA testing
5- Chromosomal analysis
DISCUSSION: The patient most likely has a form of Charcot-Marie-Tooth disease, or

hereditary motor sensory neuropathy (HMSN). The most common varieties can now be
diagnosed by DNA testing. Mutations have been detected in the peripheral myelin protein-22
(PMP-22) gene in HMSN type IA and in the connexin gene in the x-linked HMSN. Specific
DNA diagnosis is useful in genetic counseling. Routine chromosomal testing most likely
would not detect these mutations. Nerve conduction velocity study results are normal in
some types of HMSN, and delayed nerve conduction, when found, indicates a peripheral
neuropathy but does not specify the type or inheritance pattern. Biopsy of the sural nerve or
of the quadriceps can be informative in some patients, but is not as specific as DNA testing.
These procedures are most often reserved for patients with negative DNA test results.
 Chance PF: Molecular genetics of hereditary neuropathies. J Child Neurol 1999;14:43-52.
 Bell C, Haites N: Genetic aspects of Charcot-Marie-Tooth disease. Arch Dis Child 1998;78:296-300.
6. A 2-year-old girl was born with the toe deformity shown in Figure 2. She has
difficulty wearing shoes despite having adequate room in the toe box.
Management at this time should consist of:
1- Stretching exercises, followed by taping

of the toes in a derotational maneuver.
2- Lengthening of the extensor digitorum
longus tendon of the second toe and
release of the metatarsophalangeal joint
dorsal capsule.
3- Resection arthroplasty of the proximal
interphalangeal joint of the third toe, with
release of the volar plate.
4- Tenotomy of the flexor digitorum longus and brevis of the third toe.
5- A girdlestone-taylor transfer of the flexor digitorum longus to the extensor
digitorum longus of the third toe.
DISCUSSION: The patient has a congenital curly toe deformity of the third toe, and
tenotomy of the toe flexors is highly effective for this problem. Stretching and taping are
ineffective for this deformity. The position of the second toe is secondary; therefore,
procedures on that toe are unnecessary and ineffective. The flexor to extensor transfer is a
more complicated procedure that produces negligible results, or may even worsen the
deformity. Resection arthroplasty is contraindicated because it causes abnormal growth of
the toes.
 Hamer AJ, Stanley D, Smith TW: Surgery for curly toe deformity: A double-blind, randomized, prospective trial. J
Bone Joint Surg Br 1993;75:662-663.
 Ross ER, Menelaus MB: Open flexor tenotomy for hammer toes and curly toes in childhood. J Bone Joint Surg Br
1984;66:770-771.
 JA: The child's foot, in Morrissy RT, Weinstein SL (eds): Lovell & Winter's Pediatric Orthopaedics, ed 4.
Philadelphia, Pa, Lippincott-Raven, 1996, pp 1077-1135.
7. Figure 3 shows the radiograph of an asymptomatic 10-year-old boy.

Management should consist of:
1- Physical therapy.
2- Restriction from contact sports.
3- Periodic observation, but no activity
restriction.
4- Immobilization with a
thoracolumbosacral orthosis (TLSO).
5- Direct surgical repair.
DISCUSSION: Asymptomatic spondylolysis in a child or adolescent should be observed for

the possible development of spondylolisthesis, but no other active intervention is needed.
The initial treatment of choice for symptomatic spondylolysis includes rest and activity
modifications, nonsteroidal anti-inflammatory drugs, physical therapy, bracing, and casting.
Immobilization with a TLSO or pantaloon spica cast may permit healing of an acute pars
fracture. Rarely, surgical treatment may be necessary. Surgical options include
posterolateral L5-S1 fusion or direct repair of the pars defect.
 Pizzutillo PD, Hummer CD III: Nonoperative treatment for painful adolescent spondylolysis or spondylolisthesis. J
Surgeons, 1996, pp 129-137.
8. A 12-year-old girl has had lower back pain for the past 6 months that interferes
with her ability to participate in sports. She denies any history of radicular
symptoms, sensory changes, or bowel or bladder dysfunction. Examination
reveals a shuffling gait, restriction of forward bending, and tight hamstrings.
Radiographs show a grade III spondylolisthesis of L5 on S1, with a slip angle of
20 degrees. Management should consist of
1- Brace treatment.
2- Laminectomy, nerve root decompression, and in situ fusion of l4 to the sacrum.
3- In situ fusion of l4 to the sacrum.
4- Excision of the l5 lamina
5- Physical therapy.
DISCUSSION: Indications for surgical treatment of spondylolisthesis include pain and/or

progression of deformity. Specifically, surgery is necessary when there is persistent pain or a
neurologic deficit that fails to respond to nonsurgical therapy, there is significant slip
progression, or the slip is greater than 50%. For patients with mild spondylolisthesis, in situ
posterolateral L5-S1 fusion is adequate. In patients with more severe slips (greater than
50%), extension of the fusion to L4 offers better mechanical advantage. Postoperative
immobilization may be achieved with instrumentation, casting, or both. In patients with a
slip angle of greater than 45 degrees, reduction of the lumbosacral kyphosis with
instrumentation or casting is desirable to prevent slip progression. Laminectomy alone is
contraindicated in a child. Nerve root decompression is indicated if radiculopathy is present
clinically.
 Seitsalo S, Osterman K, Hyvarinen H, Tallroth K, Schlenzka D, Poussa M: Progression of spondylolisthesis in children
and adolescents: A long-term follow-up of 272 patients. Spine 1991;16:417-421.
 Newton PO, Johnston CE II: Analysis and treatment of poor outcomes following in situ arthrodesis in adolescent
spondylolisthesis. J Pediatr Orthop 1997;17:754-761.
9. Figures 4a through 4c show the clinical photographs and radiographs of a 12-

month-old boy who has progressive difficulty wearing shoes because of the
length of the second toe, as well as width of the forefoot. Management should
consist of
1- Form-fitted shoes.
2- Amputation of the second toe at the
metatarsophalangeal joint.
3- Amputation of the first ray and
amputation of the second toe.
4- Amputation of the second ray.
5- An MRI scan of the foot, a CT scan
of the chest, and a biopsy of the foot
with the possibility of ankle
disarticulation amputation.
DISCUSSION: The patient has macrodactyly involving the second ray, with significant
enlargement of the width and height of the foot. The radiographs show widening of the
interval between the first and second metatarsal and between the second and third metatarsal.
With this degree of involvement, amputation of the second ray with excision of the
overgrowth of affected soft tissue provides the most consistent desired reduction in foot size.
A threaded Steinmann pin should be inserted across the remaining metatarsals until healing
has occurred. Patients with macrodactyly should be examined to exclude neurofibromatosis
type 1 and Klippel-Trenaunay-Weber syndrome.
 Sullivan JA: The child's foot, in Morrissy RT, Weinstein SL (eds): Lovell & Winter's Pediatric Orthopaedics, ed 4.
10. Figures 5a and 5b show the radiographs of an 11-year-old boy who felt a pop and
immediate pain in his right knee as he was driving off his right leg to jam a
basketball. Examination reveals that the knee is flexed, and the patient is unable
to actively extend it or bear weight on that side. There is also a large effusion.
Management should include
1- Ice and elevation, followed by graduated range-of-motion exercises.

2- A long leg cast.
3- Excision of the fragment.
4- Open reduction and internal fixation.
5- Observation until maturity, followed by anterior cruciate ligament repair.
DISCUSSION: Fractures through the cartilage on the inferior pole of the patella, the so-
called sleeve fracture, are often difficult to diagnose because of the paucity of ossified bone
visible on the radiographs. If the fracture is missed and the fragments are widely displaced,
the patella may heal in an elongated configuration that may result in compromise of the
extensor mechanism function. The treatment of choice is open reduction and internal fixation
using a tension band wire technique to achieve close approximation of the fragments and
restore full active knee extension.
 Heckman JD, Alkire CC: Distal patellar pole fractures: A proposed common mechanism of injury. Am J Sports Med
1984;12:424-428.
 Tolo VT: Fractures and dislocations around the knee, in Green NE, Swiontkowski MF (eds): Skeletal Trauma in
Children. Philadelphia, Pa, WB Saunders, 1994, vol 3, pp 380-382.
11. A 2-year-old child has been referred for management of congenital kyphosis.
Neurologic examination is normal, and radiographs show a type I congenital
kyphosis. Which of the following anomalies is seen in the MRI scan shown in
Figure 6?
1- Chiari II malformation
2- Syrinx
3- Diastematomyelia
4- Meningocele
5- Tethered cord
DISCUSSION: There is a high incidence of intraspinal anomalies in patients with congenital

scoliosis and kyphosis. Bradford and associates reported an incidence rate of 38% in 42
patients. The MRI scan shows that the filum terminale is thickened and adherent distally in
the spinal canal. Although the conus is at L1, which may be normal, neurologic dysfunction
may occur with further growth. There are no signals of high intensity within the cord that
would suggest a syrinx. A Chiari II malformation would be found in the upper cervical
region, not shown in this MRI scan. Meningocele and diastematomyelia are not present.
 Bradford DS, Heithoff KB, Cohen M: Intraspinal abnormalities and congenital spine deformities: A radiographic and
MRI study. J Pediatr Orthop 1991;11:36-41.
 Mimaston MJ: Occult intraspinal anomalies and congenital scoliosis. J Bone Joint Surg Am 1984;66:588-601.
12. A 15-year-old boy with a type I hereditary sensory motor neuropathy (Charcot-
Marie-Tooth disease) reports recurrent ankle sprains and significant pain in the
hindfoot and midfoot despite orthotic management. Examination reveals that he
walks with a drop foot and has dynamic clawing of the toes. Clinical
photographs of the left foot are shown in Figure 7. Management should consist
of:
1- Revision of the current orthotics.
2- Metatarsal osteotomies to correct cavus.
3- A sliding calcaneal osteotomy to correct hindfoot varus.
4- Triple arthrodesis, with anterior transfer of the posterior
tibialis.
5- Extensor transfer to the metatarsal necks, soft-tissue
releases, and anterior transfer of the posterior tibialis
tendon.
DISCUSSION: The clinical photographs show a patient with a type I hereditary sensory
motor neuropathy, who has cavus feet with a flexible hindfoot. The Coleman block test
shows that the hindfoot corrects into valgus. To prevent progressive cavus, patients with this
condition may benefit from soft-tissue releases at a younger age while the foot is flexible.
Once there is fixed deformity, combined soft-tissue and bone procedures usually are
necessary. Metatarsal osteotomies will correct the cavus, but will do nothing for the drop
foot. Transfer of the extensor hallucis longus to the neck of the first metatarsal and modified
transfer of the extensor digitorum longus to the dorsum of the foot will prevent further claw
toes and improve foot dorsiflexion. Anterior transfer of the posterior tibialis tendon will also
aid in dorsiflexion. Calcaneal osteotomy should be reserved for fixed hindfoot varus that
does not correct with block testing, and triple arthrodesis should be avoided as long as
possible because the long-term outcome is poor.
 Beaty JH (ed): Orthopaedic Knowledge Update 6. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1999,
pp 235-245.
 Coleman SS: Complex Foot Deformities in Children. Philadelphia, Pa, Lea & Febiger, 1983, pp 147-165.
 Thometz JG, Gould JS: Cavus deformity, in Drennan JC (ed): The Child's Foot and Ankle. New York, NY, Raven
Press, 1992, pp 343-353.
13. Figures 8a through 8d show the radiographs and CT scans of a 14-year-old girl
who has a painful, rigid planovalgus foot. Management consisting of arch
supports and anti-inflammatory drugs failed to provide relief. A below-knee
walking cast resulted in pain resolution, but she now reports that the pain has
recurred. Management should now consist of:
1- Custom orthotics.
2- A below-knee non-weight-bearing cast.
3- Exploration and resection of the calcaneocuboid joint.
4- Exploration and resection of the talocalcaneal joint.
5- Triple arthrodesis.
DISCUSSION: Tarsal coalitions commonly present in the preadolescent age group as a rigid,
planovalgus foot. Small coalitions of the calcaneonavicular joint or the middle facet of the
talocalcaneal joint can be excised with interposition of fat or muscle tissue. Isolated
calcaneocuboid joint coalitions are very rare. This patient has an associated large
talocalcaneal coalition; therefore, resection is contraindicated. Surgery is warranted after
failure of nonsurgical management, and because of the involvement of two joints, the only
viable option for the severely symptomatic foot is triple arthrodesis.
 Vincent KA: Tarsal coalition and painful flatfoot. J Am Acad Orthop Surg 1998;6:274-281.
 Olney BW: Tarsal coalition, in Drennan JC (ed): The Child's Foot and Ankle. New York, NY, Raven
Press, 1992, pp 169-181.
14. Figures 9a and 9b show the spinal radiographs of a 3-year-old child with short
limb dwarfism. The lateral radiograph is obtained with maximal lumbar
extension. Management should consist of
1- Close clinical follow-up.

2- A thoracolumbosacral orthosis.
3- Anterior spinal fusion.
4- Posterior spinal fusion.
5- Fibroblast growth factor replacement.
DISCUSSION: The patient has kyphosis in association with achondroplasia. The AP

radiograph shows decreased interpedicular distance at the lower lumbar vertebrae, a feature
considered to be a distinctive sign of achondroplasia. Most patients with achondroplasia have
kyphosis, and this usually resolves spontaneously. When the fixed component is greater than
30 degrees, however, brace treatment is recommended. Spinal fusion is seldom required.
 Pauli RM, Breed A, Horton VK, Glinski LP, Reiser CA: Prevention of fixed angular kyphosis in
achondroplasia. J Pediatr Orthop 1997;17:726-733.
15. A 10-year-old boy with severe hemophilia A (factor VIII) sustained an injury to
his right forearm 2 hours ago when a classmate fell on his arm during a scuffle.
Examination reveals moderate swelling in the forearm, decreased sensation in
the distribution of the median and ulnar nerves, and pain on passive extension of
the fingers. What is the most appropriate sequence of treatment?
1- Measurement of volar compartment pressures and, if elevated, a bolus transfusion
of 4 unit/kg of factor VIII concentrate, followed by continuous transfusion of
factor VIII
2- Splinting of the extremity with the elbow flexed and the wrist in extension,
elevation of the extremity, observation, and if no improvement, continuous
transfusion of factor VIII (4 unit/kg)
3- A bolus transfusion of 4 unit/kg of factor VIII concentrate, followed by
continuous transfusion of factor VIII, measurement of forearm compartment
pressures, splinting of the extremity with the elbow and wrist flexed, and surgical
release of the volar forearm compartments if pressures are elevated
4- A bolus transfusion of 4 unit/kg of factor VIII concentrate, followed by
continuous transfusion of factor VIII and an emergency fasciotomy
5- A bolus transfusion of 4 unit/kg of factor VIII concentrate, laboratory studies
consisting of serum electrolytes and creatine phosphokinase levels, IV injections
of bicarbonate to alkalize the urine, and renal dialysis
DISCUSSION: The patient has severe hemophilia with a volar forearm hemorrhage and an
emerging compartment syndrome. Therefore, it is critical to normalize the clotting
deficiency as the first step in treatment. In a patient who has a factor VIII level of less than
1% and no inhibitors to factor VIII, transfusion with 4 unit/kg will typically raise the factor
VIII level to 100%. Continuous transfusion can then be used to maintain this level.
Compartment pressures can be safely measured after infusion of factor VIII. Because the
hemorrhage is of limited duration and any surgery is considered serious in a patient with
hemophilia, the compartment pressure should be measured before making a decision
regarding a fasciotomy. However, it is important to note that the use of factor VIII
concentrates allows both emergency and elective surgery provided that adequate hematology
backup is available. Splinting the elbow and wrist in flexion reduces the pressure in the volar
compartments, protects the forearm from further trauma, and makes the patient more
comfortable.
 Greene WB: Diseases related to the hematopoietic system, in Morrissy RT, Weinstein SL (eds): Lovell & Winter's
Pediatric Orthopaedics, ed 4. Philadelphia, Pa, Lippincott-Raven, 1996, pp 345-391.
 Greene WB, McMillan CW: Nonsurgical management of hemophilic arthropathy, in Barr JS (ed): Instructional Course
Lectures 38. Park Ridge, Ill, American Academy of Orthopaedic Surgeons, 1989, pp 367-381.
 Naranja RJ Jr, Chan PS, High K, Esterhai JL Jr, Heppenstall RB: Treatment considerations in patients with
compartment syndrome and an inherited bleeding disorder. Orthopaedics 1997;20;706-711.
16. Figures 10a and 10b show the radiographs of an athletic 9-year-old boy who has
activity-related anterior knee pain with intact active knee extension.
Examination reveals tenderness to palpation over the inferior pole of the patella.
There is no effusion or ligamentous instability. Initial management should
consist of:
1- long leg cast immobilization for 6 weeks.

2- open reduction and internal fixation.
3- activity restrictions and nonsteroidal anti-inflammatory drugs.
4- cessation of sports for 6 to 18 months.
5- diagnostic arthroscopy.
DISCUSSION: The radiographs show fragmentation of the inferior pole of the patella. This
finding, along with the clinical presentation, is most consistent with Sindig-Larsen-Johansson
disease. This is an overuse syndrome commonly seen in boys ages 9 to 11 years. The
differential diagnosis includes bipartite patella and patellar sleeve fracture. Like most
overuse syndromes, Sindig-Larsen-Johansson disease responds to activity modification and
nonsteroidal anti-inflammatory drugs. While symptoms usually resolve with short periods of
activity restriction, radiographic findings may persist.
 Stanitski CL: Anterior knee pain syndromes in the adolescent. J Bone Joint Surg Am 1993;75:1407-1416.
 Stanitski CL: Anterior knee pain syndromes in the adolescent, in Schafer M (ed): Instructional Course Lectures 43.
Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1994, pp 211-220.
17. A 2-week-old infant has been referred for evaluation of nonmovement of the left
hip. History reveals that the patient was delivered 6 weeks premature by
cesarean section. Examination reveals no fever, and there is mild swelling of the
thigh. Passive movement of the hip appears to elicit tenderness and very limited
hip motion. A radiograph of the pelvis shows mild subluxation of the left hip.
The next step in evaluation should consist of:
1- Aspiration of the left hip.
2- Application of a pavlik harness.
3- A gallium scan.
4- An MRI scan of the spine.
5- Modified bryant traction.
DISCUSSION: The diagnosis of bone and joint sepsis in a newborn is difficult because of
the relative lack of obvious signs and symptoms. Fever is usually absent. A study of 34
newborns with osteomyelitis identified prematurity and delivery by cesarean section as
predisposing factors. In that study, the most common clinical findings were pseudoparalysis,
local swelling, and pain on passive movement. Because early diagnosis is so important, any
infant who exhibits these findings should be suspected as having bone or joint sepsis. Once
the area of involvement is identified, aspiration is mandatory. In newborns who have an
infection about the hip, radiographs may reveal subluxation. In this patient, septic arthritis
must be ruled out by aspiration of the hip. Developmental dysplasia of the hip is not painful
and is not accompanied by localized swelling. If no purulent material is obtained at the time
of hip aspiration, an arthrogram should be obtained to rule out epiphysiolysis of the proximal
femur. Because the area of involvement has been identified by clinical examination, a
gallium scan or MRI scan of the spine is not indicated.
 Knudsen CJ, Hoffman EB: Neonatal osteomyelitis. J Bone Joint Surg Br 1990;72:846-851.
 Morrissy RT: Bone and joint sepsis, in Morrissy RT, Weinstein SL (eds): Lovell and Winter's Pediatric Orthopaedics,
ed 4. Philadelphia, Pa, Lippincott-Raven, 1996, pp 579-624.
18. Figure 11 shows the lateral radiograph of a 16-year-old boy who has been unable
to participate in sports activities because of pain in the anterior aspect of the
knee. He states that the pain is aching in nature and is located in the region of
the tibial tuberosity. He denies having joint effusion or symptoms of instability.
Management should consist of
1- Phonophoresis treatment and a program of

quadriceps stretching.
2- Application of a hinge, post, and shell design
functional knee brace.
3- Application of a hinge, post, and strap design
functional knee brace.
4- An MRI scan of the knee, a CT scan of the chest,
a technetium Tc 99m bone scan, liver function
tests, and a biopsy of the proximal tibia.
5- Excision of the ossicle and prominence of the
tibial tuberosity.
DISCUSSION: The prognosis for most patients with Osgood-Schlatter disease is good.
When the secondary ossification center unites with the main body of the tibial tubercle, the
patellar tendon has a more rigid anchor, and heterotopic ossification and its associated
reaction often become quiescent. However, even after closure of the growth plates, some
patients have persistent symptoms. Excision of the ossicle and prominence of the tibial
tuberosity decompresses the patellar tendon and allows most patients to resume sports
activities. Nonsurgical modalities are ineffective. Better results have been reported after
excision than after drilling of the tubercle. Excision of the ossicle is not indicated prior to
skeletal maturity because symptoms will resolve in most patients when the secondary
ossification center unites.
 Flowers MJ, Bhadreshwar DR: Tibial tuberosity excision for symptomatic Osgood-Schlatter disease. J Pediatr Orthop
1995;15:292-297.
 Greene WB: Osteochondrosis and tibia vara, in Canale ST, Beaty JH (eds): Operative Pediatric Orthopaedics, ed 2.
Philadelphia, Pa, Mosby, 1995, pp 804-854.
19. A 10-lb, 2-oz infant who was born via a difficult breech delivery 12 hours ago is
now being evaluated for hip pain. Although the infant is resting comfortably,
examination reveals that the patient is not moving the right lower extremity and
manipulation of the right hip causes the infant to cry. The Galeazzi sign is
positive. An AP radiograph of the pelvis shows proximal and superior migration
of the right proximal femoral metaphysis. What is the most likely diagnosis?
1- Teratologic hip dislocation
2- Septic right hip
3- Congenital coxa vara
4- Transphyseal fracture of the proximal femur
5- Postpartum ligamentous laxity
DISCUSSION: Transphyseal fractures of the proximal femur at birth are more likely to occur
in large newborns after a difficult delivery. At rest, the patients are comfortable and show a
pseudoparalysis; however, passive motion of the lower extremity results in discomfort.
Teratologic hip dislocations will have a positive Galeazzi sign, but are not painful.
Development of a septic hip would be unlikely within 12 hours postpartum. Congenital coxa
vara is typically painless. Postpartum ligamentous laxity might account for a positive
Ortolani sign, but is painless.
 Weinstein JN, Kuo KN, Millar EA: Congenital coxa vara: A retrospective review. J Pediatr Orthop 1984;4:70-77.
 Beaty JH: Orthopaedic Knowledge Update 6. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1999, pp
409-425.
20. A 10-year-old girl has been referred for evaluation of a prominence at the lower
cervical spine. The patient is asymptomatic, and the examination reveals no
evidence of neurologic abnormality. A radiograph and CT scans are shown in
Figures 12a through 12c. What is the most likely diagnosis?
1- Tuberculosis
2- Congenital kyphosis
3- Blastomycosis
4- Aneurysmal bone cyst
5- Osteoblastoma
DISCUSSION: Tuberculosis is uncommon in the cervical spine but has a relatively greater
incidence in young children. In a review of 40 patients with lower cervical spine
involvement (C2 to C7), 24 were younger than age 10 years at presentation. In children, the
disease is characterized by more extensive involvement with the formation of large abscesses.
In older patients with lower cervical tuberculosis, the disease is more localized but is more
likely to cause paraplegia. Four-drug antituberculosis therapy should be used. For patients
with pain or neurologic dysfunction, anterior excision of diseased bone and grafting are
indicated. Whether vertebral body excision and grafting should be done in an asymptomatic
10-year-old child is debatable. The CT scan shows a large "cold" abscess that is partially
calcified.
 Hsu LC, Leong JC: Tuberculosis of the lower cervical spine (C2 to C7): A report on 40 cases. J Bone Joint Surg Br
1984;66:1-5.
 Loder RT: The cervical spine, in Morrissy RT, Weinstein SL (eds): Lovell & Winter's Pediatric Orthopaedics, ed 4.
21. Which of the following types of iliac osteotomy provides the greatest potential for
increased coverage?
1- Ganz periacetabular
2- Pemberton innominate
3- Salter innominate
4- Sutherland double innominate
5- Steele triple innominate
DISCUSSION: The degree of acetabular dysplasia and the age of the child are important
considerations when choosing what type of osteotomy to perform. The ability to obtain
concentric reduction is a prerequisite of all osteotomies that redirect the acetabulum.
Procedures that cut all three pelvic bones allow more displacement and, therefore, correction
of acetabular dysplasia. The closer the osteotomy is to the acetabulum, the greater the
coverage of the femoral head. Compared with the other acetabular osteotomies, the Ganz
periacetabular osteotomy provides the greatest potential for correcting acetabular deficiency
because there are no bone or ligamentous restraints to limit correction, but it has the
disadvantage of being a technically demanding procedure. The amount of coverage provided
by the Salter osteotomy is limited.
 Millis MB, Poss R, Murphy SB: Osteotomies of the hip in the prevention and treatment of osteoarthritis, in Eilert RE
(ed): Instructional Course Lectures XLI. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1992, pp 145-
154.
 Weinstein SL: Developmental hip dysplasia and dislocation, in Morrissy RT, Weinstein SL (eds): Lovell & Winter's
22. The mother of a 26-month-old boy reports that he has been unwilling to bear
weight on his left lower extremity since he awoke this morning. She denies any
history of trauma. He has a temperature of 99.4 degrees F (37.4 degrees C), and
examination reveals that abduction of the left hip is limited to 30 degrees.
Laboratory studies show a WBC of 11,000/mm3 and an erythrocyte
sedimentation rate of 22 mm/h. A radiograph of the pelvis is shown in Figure 13.
1- Aspiration of the hip.

2- A bone scan.
3- An MRI scan.
4- Bed rest, frequent temperature
checks, and re-evaluation in 24 - 48
hrs.
5- Hospital admission and placement
in traction.
DISCUSSION: The most likely diagnosis is transient synovitis. Initial management should
consist of bed rest and serial observation to rule out atypical septic arthritis of the hip. In an
unreliable family situation, hospitalization for bed rest and observation may be indicated.
Other disorders such as proximal femoral osteomyelitis, leukemia, juvenile rheumatoid
arthritis, pelvic osteomyelitis, diskitis, and arthralgia secondary to other inflammatory
disorders should be considered. However, these disorders are unlikely because of the paucity
of abnormal clinical signs exhibited by the patient. On the other hand, transient synovitis of
the hip in children is a diagnosis of exclusion; other possibilities should be explored if the
patient's symptoms do not follow a typical course and resolve in 4 to 21 days.
 Frymoyer JW (ed): Orthopaedic Knowledge Update 4. Rosemont, Ill, American Academy of Orthopaedic Surgeons,
1993, pp 505-513.
23. A 10-year-old girl with a monoarticular pattern of juvenile rheumatoid arthritis

(JRA) has had a 3-cm limb-length discrepancy since age 8 years when
inflammation in the right knee came under good medical control. Because her
right leg is longer, the patient states that she would like her legs to be close to
equal in length in the future. A growth-remaining chart is shown in Figure 14.
1- Immediate proximal right tibial physeal arrest.
2- Immediate distal right femoral physeal arrest.
3- A shoe lift and follow-up in 2 years.
4- Observation with the expectation that the discrepancy may correct itself and not
require surgery.
5- Limb lengthening of the shorter left limb.
DISCUSSION: In a subgroup of patients with monoarticular JRA and a limb-length

discrepancy that developed before the age of 9 years, Simon and associates showed that a
subsequent growth deceleration on the affected side may correct a large part of the difference
in length. This possibility would make surgery unnecessary and should prompt further
observation.
 Simon S, Whiffen J, Shapiro F: Leg-length discrepancies in monoarticular and pauciarticular juvenile rheumatoid
arthritis. J Bone Joint Surg Am 1981;63:209-215.
 Ansell BM, Bywaters EGL: Growth in Still's disease. Ann Rheum Dis 1956;15:295-319.
24. A 14-year-old girl with polyarticular juvenile rheumatoid arthritis (JRA) has
severe neck pain and reports the onset of urinary incontinence. A lateral
radiograph and lateral tomogram of the cervical spine are shown in Figures 15a
and 15b. An MRI scan of the upper cervical spine is shown in Figure 15c.
1- A rigid cervical orthosis.

2- A soft cervical collar.
3- Posterior C1-2 fusion with halo immobilization.
4- Administration of methotrexate.
5- Activity restrictions.
DISCUSSION: The plain radiograph and tomogram show an abnormality of the upper
cervical spine, with erosion of the dens. The MRI scan shows evidence of cord impingement.
The cervical spine is frequently involved in polyarticular JRA. Stiffness and autofusion are
commonly seen, but C1-2 instability can also occur secondary to synovitis and bony erosion.
Basilar invagination is rare in JRA. There is no consensus regarding fusion in the
asymptomatic patient. In patients with symptoms and neurologic signs, C1-2 posterior fusion
is indicated.
 Fried JA, Athreya B, Gregg JR, Das M, Doughty R: The cervical spine in juvenile rheumatoid arthritis. Clin Orthop
1983;179:102-106.
 Hensinger RN, DeVito PD, Ragsdale CG: Changes in the cervical spine in juvenile rheumatoid arthritis. J Bone Joint
Surg Am 1986;68:189-198.
25. Figures 16a and 16b show the radiographs of an otherwise healthy 3 1/2-year-old
boy who has an isolated deformity of the left leg. Definitive primary treatment
of this condition should consist of:
1- Distraction osteogenesis.
2- An intramedullary nail and onlay bone
graft.
3- A short leg cast.
4- A vascularized free fibular graft.
5- Plate fixation and an autogenous bone
graft.
DISCUSSION: Treatment of congenital pseudarthrosis of the tibia is problematic. To

achieve union, a resection of the pseudarthrosis, stabilization, and bone grafting must be
performed. Simple cast immobilization does not yield union. There are various options for
the resection, immobilization, and grafting. On the first surgical attempt, retrograde
intramedullary nailing offers the best chance for success by transfixing the ankle and subtalar
joints with abundant autogenous bone grafting. Distraction osteogenesis and vascularized
free fibular graft are reserved as salvage procedures.
 Gilbert A, Brockman R: Congenital pseudarthrosis of the tibia: Long-term follow-up of 29 cases treated by
microvascular bone transfer. Clin Orthop 1995;314:37-44.
 Boero S, Catagni M, Donzelli O, Facchini R, Frediani PV: Congenital pseudarthrosis of the tibia associated with
neurofibromatosis - 1: Treatment with Ilizarov's device. J Pediatr Orthop 1997;17:675-684.
 Anderson DJ, Schoenecker PL, Sheridan JJ, Rich MM: Use of an intramedullary rod for the treatment of
congenital pseudarthrosis of the tibia. J Bone Joint Surg Am 1992;74:161-168.
26. Figure 17 shows the radiograph of a 2-year-old girl who sustained a fracture of
the femur in a fall while walking with her parents. History reveals that this is
her third long bone fracture, having sustained a humerus fracture 1 year ago
and a fracture of the opposite femur 9 months ago. There is no family history of
any similar problem. Examination reveals distinctly blue sclerae, normal
appearing teeth, and no skin lesions. What is the most likely cause of this
patient's disorder?
1- A quantitative defect of type I collagen synthesis

2- A deficiency of vitamin D
3- Parental abuse
4- Abnormal osteoclast function
5- Excess excretion of keratin sulfate in the urine
DISCUSSION: Osteogenesis imperfecta (OI) is a genetically determined disorder of type I

collagen synthesis that is characterized by bone fragility. This patient has had three fractures
of the long bones by age 2 years, with the last one occurring after relatively minor trauma.
The patient's history and clinical features are consistent with a diagnosis of Sillence type IA
OI. Type I OI is the mildest and most common form. Inheritance is autosomal-dominant;
however, as in this patient, new mutations are frequent. Type I is subclassified into the A
type (absence of dentinogenesis imperfecta) and B type (presence of dentinogenesis
imperfecta). The sclerae are blue, and the first fractures usually occur in the preschool years
after walking has begun. Cells from individuals with type I OI largely demonstrate a
quantitative defect of type I collagen; they synthesize and secrete about half the normal
amount of type I procollagen. Qualitative mutations that lead to an abnormal type I
procollagen molecule result in more severe types of the disorder. There are no indications
that this child has been abused. Radiographs of the femur show no evidence of rickets,
pyknodysostosis, or osteopetrosis. Morquio syndrome, characterized by excess excretion of
keratin sulfate in the urine, is not associated with bone fragility.
 Kocher MS, Shapiro F: Osteogenesis imperfecta. J Am Acad Orthop Surg 1998;6:225-236
 Sillence DO, Senn A, Danks DM: Osteogenesis imperfecta: An expanding panorama of variants. Clin Orthop
1981;159:11-25.
 Cole WG: Etiology and pathogenesis of heritable connective tissue diseases. J Pediatr Orthop 1993;13:392-403.
27. An 8-year-old boy with severe hemophilia A (factor VIII) and no inhibitor is
averaging eight transfusions per month for bleeding into the right ankle.
Examination shows synovial hypertrophy; range of motion consists of 0 degrees
of dorsiflexion and 20 degrees of plantar flexion. The patient's knees, elbows,
and left ankle have no restriction of motion. Standing radiographs of the right
ankle are shown in Figure 18. Management should consist of
1- Prophylactic transfusions three

times per week.
2- Application of ankle-foot
orthoses.
3- Ankle synovectomy.
4- Ankle arthrodesis performed
with physeal protection.
5- Pantalar arthrodesis.
DISCUSSION: The patient has bilateral hypertrophic synovitis that is causing repeated
hemarthroses and progressive arthropathy. Ankle synovectomy in patients with hemophilia is
effective in significantly reducing the rate of joint bleeding and in slowing the progression of
the arthropathy; therefore, bilateral synovectomies is the treatment of choice. Range of
motion can be effectively maintained after ankle synovectomy. Bracing and prophylactic
transfusions would be ineffective at this time. Ankle arthrodesis should be reserved for
patients with severe pain. Compared with patients who have juvenile rheumatoid arthritis,
patients with hemophilia generally do not have involvement of the subtalar joint and rarely
require a pantalar arthrodesis.
 Greene WB: Synovectomy of the ankle for hemophilic arthropathy. J Bone Joint Surg Am 1994;76:812-819.
 Greene WB: Chronic inflammatory arthridities and diseases related to the hematopoietic system, in Drennan JC (ed):
The Child's Foot and Ankle, New York, NY, Raven Press, 1992, pp 461-482.
28. Figures 19a through 19c show radiographs of the cervical spine of an
asymptomatic patient with Down syndrome who wants to participate in a Special
Olympics running event. The neurologic examination is normal. Management
should consist of:
1- an MRI scan.
2- fusion from the occiput to C2.
3- fusion of C1-C2.
4- application of a cervical collar and no participation in any sports.
5- periodic follow-up examinations and no contact sports.
DISCUSSION: An atlanto-dens interval (ADI) of up to 4 mm in children is considered

normal. Children with Down syndrome have increased ligamentous laxity, with atlantoaxial
instability occurring in as many as 15% to 20% of patients. These patients are at risk for
catastrophic injury following minor trauma and should be routinely screened for instability,
generally beginning when the patient starts to walk. Patients with an ADI of greater than 5
mm should avoid contact sports and high-risk activities such as gymnastics, diving, the high
jump, and the butterfly stroke. The American Academy of Pediatrics Committee of Sports
Medicine and Fitness guidelines recommend that lateral views of the cervical spine in neutral,
flexion, and extension should be obtained in all children with Down syndrome who wish to
participate in sports. Patients with normal radiographs and examinations do not need repeat
radiographs, although some authors suggest that instability increases with age, and therefore
recommend repeat radiographs every 5 years. Cervical spine fusion in patients with Down
syndrome has a high rate of complications and should be performed only on patients with
symptoms and evidence of myelopathy.
 American Academy of Pediatrics Committee of Sports Medicine and Fitness: Atlantoaxial instability in Down
syndrome. Pediatrics 1995;96:151-154.
 Copley LA, Dormans JP: Cervical spine disorders in infants and children. J Am Acad Orthop Surg 1998;6:204-214.
 Tredwell SJ, Newman DE, Lockitch G: Instability of the upper cervical spine in Down syndrome. J Pediatr Orthop
1990;10:602-606.
29. Compared with amputation, limb salvage for osteosarcoma of the distal end of
the femur will result in
1- An improved chance for long-term survival.
2- An improved quality of life.
3- A lessened need for chemotherapy.
4- A better functional outcome.
5- A reduced need for reoperation.
DISCUSSION: Major advances in diagnostic imaging, neoadjuvant chemotherapy, and

surgical techniques have allowed limb salvage to be performed as an alternative to
amputation in children with osteosarcoma. The outcome of treatment of nonmetastatic, high-
grade osteosarcoma of the distal femur was studied in 227 patients from 26 institutions. The
authors found no difference in the long-term survival or quality of life between patients
treated with limb salvage and those treated with amputation. Patients treated with limb
salvage had a higher rate of reoperation, but a better functional outcome.
 Rougraff BT, Simon MA, Kneisl JS, Greenberg DB, Mankin HJ: Limb salvage compared with amputation for
osteosarcoma of the distal end of the femur: A long-term oncological, functional, and quality-of-life study. J Bone Joint
Surg Am 1994;76:649-656.
 Simon MA: Limb salvage for osteosarcoma. J Bone Joint Surg Am 1988;70:307-310.
30. Examination of a 7-year-old boy reveals 20 degrees of valgus following a lawn

mower injury to the lateral femoral epiphysis. Treatment consists of total distal
femoral epiphyseodesis and varus osteotomy. Following surgery, he has a limb-
length discrepancy of 3 cm and 5 degrees of genu valgum. Assuming that he
undergoes no further treatment, the patient's predicted limb-length discrepancy
at maturity would be how many centimeters?
1- Less than 7
2- 7 to 10
3- 11 to 13
4- 14 to 17
5- Greater than 17
DISCUSSION: The distal femoral epiphysis grows approximately 1 cm per year and in boys,
growth ceases at approximately age 16 years. Therefore, the patient's limb-length
discrepancy at maturity would be 12 cm (9 cm plus the 3-cm discrepancy he has from the
previous surgery).
 Little DG, Nigo L, Aiona MD: Deficiencies of current methods for the timing of epiphyseodesis. J Pediatr Orthop
1996;16:173-179.
 Westh RN, Menelaus MB: A simple calculation for the timing of epiphyseal arrest: A further report. J Bone Joint Surg
Br 1981;63:117-119.
31. When the iliac apophysis starts ossifying in the normal adolescent, growth of the
sitting height or trunk height is characterized by
1- No appreciable change during the remainder of growth for either boys or girls.
2- An increase of 3 to 5 cm in girls and an increase of 5 to 8 cm in boys.
DISCUSSION: Studies by Anderson and associates have resulted in a growth-remaining

chart for sitting height that shows an increase of 3 to 5 cm in girls and an increase of 5 to 8
cm in boys. Future growth of the spine may impact brace longevity and fit.
 Anderson M, Hwang SC, Green WT: Growth of the normal trunk in boys and girls during the second decade of life:
Related to age, maturity, and ossification of the iliac epiphyses. J Bone Joint Surg Am 1965;47:1554-1564.
 Zaoussis AL, James JIP: The iliac apophysis and the evolution of curves in scoliosis. J Bone Joint Surg Br
1958;40:442-453.
32. A 10-year-old girl was thrown over the handlebars of her bicycle and landed
directly on her left shoulder. She was treated with a figure-of-8 strap and
analgesics. Follow-up examination 2 weeks later reveals that the lateral end of
the clavicle is superiorly dislocated relative to the acromion. A radiograph of the
shoulder shows calcification lateral to the coracoid process at the level of the
acromion, and the clavicle is superiorly displaced. Management should consist
of
1- supportive immobilization until the patient is pain-free.
2- coracoclavicular ligament repair.
3- open reduction and internal fixation of the acromioclavicular dislocation.
4- resection of the distal clavicle and Weaver and Dunn reconstruction.
5- open biopsy of the calcific mass.
DISCUSSION: In adults, a direct blow on the acromion usually results in an

acromioclavicular dislocation. In children, however, the usual injury from this mechanism is
a physeal fracture of the lateral clavicle. The clavicular shaft fragment, analogous to the
metaphyseal portion of a physeal fracture, herniates through the periosteum, leaving the distal
periosteal sleeve in contact with the lateral (distal) physeal fragment. The treatment of choice
is immobilization until the patient is pain-free.
 Falstie-Jensen S, Mikkelsen P: Pseudodislocation of the acromioclavicular joint. J Bone Joint Surg Br 1982;64:368-
369.
 Havranek P: Injuries of the distal clavicular physis in children. J Pediatr Orthop 1989;9:213-215.
33. Figures 20a and 20b show the radiographs of an obese 15-year-old boy who has
severe left groin pain and is unable to bear weight following a minor injury.
Treatment should consist of :
1- Fixation with one or two screws.
2- Cast immobilization.
3- Manipulative reduction with single screw fixation.
4- In situ fixation with multiple screws.
5- Open epiphyseodesis.
DISCUSSION: The radiographs and history are consistent with an acute unstable slipped
capital femoral epiphysis. Aronson and Loder documented an increased rate of osteonecrosis
associated with manipulative reduction. They recommended bed rest with skin traction to
allow the synovitis to resolve, followed by in situ pinning. They noted, however, that many
of these slips reduced with anesthesia and positioning on a fracture table. Biomechanic
studies have shown a slight increased resistance to shear stress when two screws are used, but
it is unknown if this is significant in the clinical setting. Open epiphyseodesis does not
provide postoperative stability; therefore, adjunctive fixation or immobilization is required.
Numerous studies have noted the inadvisability of using multiple screws. Casting has a high
rate of complications, including chondrolysis and progression of the slip.
 Aronson DD, Loder RT: Treatment of the unstable (acute) slipped capital femoral epiphysis. Clin Orthop 1996;322:99-
110.
 Karol LA, Doane RM, Cornicelli SF, Zak PA, Haut RC, Manoli A II: Single versus double screw fixation for treatment
of slipped capital femoral epiphysis: A biomechanical analysis. J Pediatr Orthop 1992;12:741-745.
 Stanitski CL: Acute slipped capital femoral epiphysis: Treatment alternatives. J Am Acad Orthop Surg 1994;2:96-106.
34. What is the recommended treatment of a skeletally immature 12-year-old boy

who has an anterior cruciate ligament-deficient knee?
1- Reduced activity, rehabilitation exercises, and functional bracing until the patient
is near skeletal maturity
2- Bone-patellar tendon-bone autograft reconstruction
3- Allograft reconstruction
4- Hamstring tendon intra-articular repair using a centrally placed tibial tunnel and
an over-the-top femoral attachment
5- Extra-articular repair
DISCUSSION: Traditional surgeries for anterior cruciate ligament-deficient knees carry the
potential risk of premature physeal closure in young athletes. Therefore, most surgeons are
reluctant to recommend intra-articular reconstruction using bone tunnels with bone-patellar
tendon-bone autografts or hamstring tendons. The current recommendation for young
athletes is activity modification, rehabilitation, and functional bracing until the patient is near
skeletal maturity. At that time, for the very symptomatic patient, the treatment of choice is
intra-articular repair of the anterior cruciate ligament. If a skeletally immature patient
continues to have instability despite rehabilitation and bracing, a modification of the femoral
tunnel to the over-the-top position will not place the lateral femoral physis at risk for
premature closure and deformity. A centrally placed tibial tunnel will minimize the risk of
angular deformity and minimize limb-length discrepancy if physeal arrest occurs.
 Barry P: Anterior cruciate ligament injuries, in Andrews JR, Timmerman LA (eds): Diagnostic and Operative
Arthroscopy. Philadelphia, Pa, WB Saunders, 1997, p 358.
 McCarroll JR, Shelbourne KD, Porter DA, Rettig AC, Murray S: Patellar tendon graft reconstruction for midsubstance
anterior cruciate ligament rupture in junior high school athletes: An algorithm for management. Am J Sports Med
1994;22:478-484.
 Nottage WM, Matsuura PA: Management of complete traumatic anterior cruciate ligament tears in the skeletally
immature patient: Current concepts and review of the literature. Arthroscopy 1994;10:569-573.
 Stanitski CL: Anterior cruciate ligament injury in the skeletally immature patient: Diagnosis and treatment. J Am Acad
Orthop Surg 1995;3:146-158.
35. Figures 21a and 21b show the radiographs of a 12-year-old patient with an L4-
level myelomeningocele who has scoliosis that has been slowly progressing for
the past several years. There has been no loss of motor function. An MRI scan
shows no syringomyelia or increased hydrocephalus. Management should
consist of:
1- Follow-up with repeat radiographs in 6 months.

2- Brace treatment.
3- Posterior spinal fusion with instrumentation.
4- Anterior and posterior spinal fusion with instrumentation.
5- Anterior spinal fusion with instrumentation.
DISCUSSION: Scoliosis is a common occurrence in children with myelomeningocele, with
the incidence increasing as the neurologic level moves cephalad. The rate of pseudarthrosis
for isolated anterior or posterior fusions has been reported as high as 75%. The combination
of anterior and posterior fusions with some type of instrumentation has been shown to
decrease the rate of pseudarthrosis to 20%. Brace treatment in smaller curves can be used as
a temporizing measure to delay surgery, but as with idiopathic scoliosis, the brace is
ineffective for larger curves. Observation is not indicated with a curve of this magnitude.
 Ward WT, Wenger DR, Roach JW: Surgical correction of myelomeningocele scoliosis: A critical appraisal of various
spinal instrumentation systems. J Pediatr Orthop 1989;9:262-268.
 Muller EB, Nordwall A: Brace treatment of scoliosis in children with myelomeningocele. Spine 1994;19:151-155.
36. A 3-year-old child is referred for evaluation of bowed legs. History reveals no
dietary deficiencies; however, family history is significant for several members
with bowed legs. Examination reveals genu varum, and the child is in the 5th
percentile for height and weight. Laboratory studies show normal renal
function, a normal calcium level, a decreased phosphate level, and an elevated
alkaline phosphatase level. A plain radiograph of the lower extremities is shown
in Figure 22. What is the most likely diagnosis?
1- Blount's disease
2- Chondrometaphyseal dysplasia
3- Renal osteodystrophy
4- Vitamin D-deficient rickets
5- Vitamin D-resistant rickets
DISCUSSION: The differential diagnosis of genu varum includes physiologic genu varum,
Blount's disease, skeletal dysplasia, and metabolic bone disease. Children with Blount's
disease are generally in the 95th percentile for height and weight, and usually multiple family
members are not affected. The radiographs show widening of the physis and metaphyseal
flaring. In Blount's disease, the characteristic radiographic changes involve only the tibia,
and at this age, most commonly show beaking of the medial metaphysis. Skeletal dysplasias,
such as chondrometaphyseal dysplasia, are associated with short stature, and the radiographic
changes are similar to those seen here. However, laboratory studies in these children will be
within normal limits. Children with chronic renal disease will often be of short stature, and
the radiographic findings are again similar to those shown here. However, BUN and
creatinine levels are elevated and phosphate levels are elevated rather than decreased in
children with renal disease. The absence of dietary deficiencies and positive family history
rules out vitamin D-deficient rickets. There are four types of vitamin D-resistant rickets:
failure of production of 1,25-dihydroxy vitamin D, phosphate diabetes (hypophosphatemic
rickets), end organ insensitivity to vitamin D, and renal tubular acidosis. All types of vitamin
D-resistant rickets are resistant to treatment with physiologic doses of vitamin D. The
patient's clinical picture, family history, laboratory studies, and radiographs are most
consistent with hypophosphatemic rickets. This entity is inherited as a sex-linked dominant
trait.
 Evans GA, Arulanantham K, Gage JR: Primary hypophosphatemic rickets: Effect of oral phosphate and vitamin D on
growth and surgical treatment. J Bone Joint Surg Am 1980;62:1130-1138.
 Loeffler RD Jr, Sherman FC: The effect of treatment on growth and deformity in hypophosphatemic vitamin D-resistant
rickets. Clin Orthop 1982;162:4-10.
 Loder RT, Johnston CE II: Infantile tibia vara. J Pediatr Orthop 1987;7:639-646.
 Bassett GS, Scott CI: The osteochondrodysplasias, in Morrissy RT (ed): Pediatric Orthopaedics, ed 3. Philadelphia, Pa,
JB Lippincott, 1990, vol 1, pp 91-142.
37. A 14-year-old boy sustained a femoral neck fracture in a fall from a tree and
underwent open reduction and internal fixation 6 months ago. Follow-up
examination now reveals an antalgic Trendelenburg gait and painful range of
motion. A radiograph is shown in Figure 23, and a CT scan shows a nonunion.
Treatment should consist of
1- Revision in situ pinning and

application of a cast.
2- Hip fusion.
3- Curettage of the nonunion,
intertrochanteric valgus osteotomy,
and revision internal fixation.
4- Non-weight-bearing on the affected
side and electrical stimulation of the
fracture site.
5- Vascularized fibula grafting and
application of a cast.
DISCUSSION: The coxa vara deformity and fracture nonunion should be treated
simultaneously; therefore, the treatment of choice is curettage of the nonunion,
intertrochanteric valgus osteotomy, and revision internal fixation. In addition, valgus
osteotomy will convert the shear forces across the nonunion to compression, aiding in healing
of the nonunion. None of the other procedures addresses both issues, and hip fusion is
inappropriate under these conditions.
 Lam SF: Fractures of the neck of the femur in children. J Bone Joint Surg Am 1971;53:1165-1179.
 Canale ST, Beaty JH: Pelvic and hip fractures, in Rockwood CA Jr, Wilkins KE, Beaty JH (eds): Fractures in Children.
38. A 22-month-old child has scrapes and bruises on his head and a severe deformity
of the forearm after being thrown from a car as an unrestrained passenger in a
motor vehicle accident. Examination reveals a Glasgow Coma Scale score of 12.
Prior to treatment of the forearm, management should include
1- A mannitol infusion of 0.25 to 1 g/kg.
2- High-dose iv methylprednisolone, consisting of a 30 mg/kg bolus, followed by 5.4
mg/kg/h for 23 hours.
3- An immediate CT scan.
4- An electroencephalogram.
5- Radiographs of the skull.
DISCUSSION: As CT scanning has become available, the use of radiographs of the skull has
decreased in importance for evaluation of head trauma. The indications for CT scanning for
suspected head trauma include any degree of obtundation, focal neurologic deficit, history of
a high-velocity injury, amnesia for the injury, progressive headache, persistent vomiting,
children younger than age 2 years, serious facial injury, posttraumatic seizure, skull
penetration, or a Glasgow Coma Scale score of 13 or less. Evidence of improved outcome
with use of steroids in head trauma is lacking. Steroids are useful for increased intracranial
pressure caused by brain tumors or abscesses. High-dose IV methylprednisolone is indicated
for spinal cord trauma and improves the ultimate degree of recovery of function. When
herniation is suspected in a patient with asymmetric neurologic findings or the patient's
condition is deteriorating rapidly, a mannitol infusion may be used.
 Hall DE: Head injuries, in Hoekelman RA (ed): Primary Pediatric Care. St Louis, Mo, Mosby, 1997, pp 1709-1712.
Nelson WE, Behrman RE, Kliegman RM (eds): Nelson Essentials of Pediatrics. Philadelphia, Pa, WB Saunders,
1998, p 712.
pp 123-130.
39. Examination of a 5-year-old boy with amyoplasia shows a flexion contracture of

70 degrees of the right knee. The active arc of motion is from 70 degrees to 90
degrees, and the opposite knee has a flexion contracture of 10 degrees. Both hips
are dislocated with flexion contractures of 10 degrees, passive hip motion is from
10 degrees to 90 degrees of flexion, and the feet are plantigrade and easily
braceable. Despite a daily stretching program, the parents and physical
therapists note that it is increasingly difficult for him to walk because of the
flexion contracture of the right knee. Management of the knee flexion
contracture should now include
1- intense physical therapy.
2- an intramuscular injection of botulinum toxin A.
3- radical posterior soft-tissue release.
4- supracondylar femoral extension osteotomy.
5- gradual correction with a circular ring external fixator.
DISCUSSION: Most children with amyoplasia are ambulatory and when a decrease in
function occurs because of a severe contracture, it must be addressed. A radical posterior
soft-tissue release, including the posterior knee capsule and often the collateral ligaments and
the posterior cruciate ligament, is needed to obtain extension. After the age of 1 year,
aggressive physical therapy will do little to correct a contracture. Botulinum toxin A is
indicated for spasticity and is contraindicated with severe contractures. Supracondylar
femoral extension osteotomy works well, but will remodel at an average rate of 1 degree per
month, which is not considered ideal in a young patient. Gradual correction with a circular
ring external fixator is an option, but a soft-tissue release will also most likely be needed for a
contracture of this severity.
 Sarwark JF, MacEwen GD, Scott CI Jr: Amyoplasia (a common form of arthrogryposis). J Bone Joint Surg Am
1990;72:465-469.
 DelBello DA, Watts HG: Distal femoral extension osteotomy for knee flexion contracture in patients with
arthrogryposis. J Pediatr Orthop 1996;16:122-126.
 Sells JM, Jaffe KM, Hall JG: Amyoplasia, the most common type of arthrogryposis: The potential for good outcome.
Pediatrics 1996;97:225-231.
40. A 13-year-old girl who is 2 years postmenarche has been referred for
management of scoliosis. She denies any history of back pain. Radiographs
show a right thoracic curve of 35 degrees. She has a Risser sign of 4 and a bone
age of 15.5 years. Management should consist of
1- A low-profile spinal orthosis.
2- Observation and follow-up radiographs in 6 months.
3- Anterior spinal fusion with instrumentation.
4- Posterior spinal fusion with instrumentation.
5- In situ posterior spinal fusion.
DISCUSSION: Because the patient is skeletally mature with a curve of less than 40 degrees,
there is no benefit to bracing and surgery is not indicated. Management should consist of
observation and follow-up radiographs in 6 months.
 Lonstein JE, Carlson JM: The prediction of curve progression in untreated idiopathic scoliosis during growth. J Bone
Joint Surg Am 1984;66:1061-1071.
1993, pp 447-459.
41. In children with isolated zone II lacerations of the flexor tendon, poor digital
motion is best correlated with
1- a patient age of less than 5 years.
2- a patient age of greater than 15 years.
3- early passive motion.
4- immobilization for 3 weeks.
5- immobilization for 6 weeks.
DISCUSSION: In a recent study on restoration of motion following zone I and zone II flexor
tendon repairs in children, age was found to have no effect on the results of zone II tendon
repairs. Early passive motion offered no better results than immobilization for 3 weeks.
Immobilization for more than 4 weeks correlated with poorer results.
 O'Connell SJ, Moore MM, Strickland JW, Frazier GT, Dell PC: Results of zone I and zone II flexor tendon repairs in
children. J Hand Surg Am 1994;19:48-52.
42. In a longitudinal study of children with spastic diplegia, analysis of long-term

function will most likely reveal
1- a deterioration of gait stability and an increase in double support time.
2- a deterioration of gait stability and a decrease in double support time.
3- improved excursion about the knee, ankle, and pelvis.
4- improvement in the popliteal angle.
5- increases in single support time.
DISCUSSION: In a longitudinal study of 18 patients with spastic diplegia over a period of

32 months, three-dimensional gait analysis revealed a deterioration of gait stability with
increases in double support time and decreases in single support time. Kinematic data also
identified a loss of excursion about the knee, ankle, and pelvis. Interestingly, the static
examination of the children showed a decrease in the popliteal angle over time. The authors
concluded that ambulatory ability tends to worsen over time in children with spastic diplegia.
 Johnson DC, Damiano DL, Abel MF: The evolution of gait in childhood and adolescent cerebral palsy. J Pediatr
Orthop 1997;17:392-396.
43. Examination of a 7-year-old girl with myelomeningocele reveals calcaneal

deformities of both feet. She ambulates on both extremities wearing ankle-foot
orthoses and has no upper extremity aids. She has grade 5/5 motor strength to
the tibialis anterior muscles and absent motor strength to the triceps surae.
There is no varus or valgus deformity of the hindfoot, and the skin over the heels
is intact; however, mild callosities are present. Management should consist of
1- bilateral calcaneal osteotomies.
2- modification of the ankle-foot orthoses.
3- extra-articular subtalar arthrodesis.
4- transfer of the anterior tibial tendon to the calcaneus.
5- Achilles tendon tenodesis to the fibula.
DISCUSSION: A calcaneal deformity of the foot may occur in children who have low
lumbar myelomeningocele. Strong dorsiflexors overcome a weak or absent gastrocnemius-
soleus complex, leading to downward growth of the calcaneal apophysis. The deformity is
usually progressive and does not respond to nonsurgical management. Most authorities
recommend transfer of the tibialis anterior muscle through the interosseous membrane to the
posterior aspect of the calcaneus. This procedure has been reported to be effective in limiting
progression of the deformity. An extra-articular subtalar arthodesis, a treatment option for
valgus deformity of the hindfoot, is not indicated. Similarly, Achilles tendon tenodesis to the
fibula, an option for managing valgus of the ankle, is not indicated. Calcaneal osteotomy
may be used in older children with severe calcaneal deformity.
 Stott NS, Zionts LE, Gronley JK, Perry J: Tibialis anterior transfer for calcaneal deformity: A postoperative gait
analysis. J Pediatr Orthop 1996;16:792-798.
 Georgiadis GM, Aronson DD: Posterior transfer of the anterior tibial tendon in children who have a myelomeningocele.
J Bone Joint Surg Am 1990;72:392-398.
44. Figure 24 shows the radiograph

of a 4-year-old girl with spina
bifida. Examination reveals an
L3 motor level, excellent sitting
and standing balance, and
satisfactory range of motion at
the hips. Management should
consist of:
1- Observation.
2- Closed reduction and cast immobilization.
3- Bilateral medial open reduction of the hips.
4- Anterior open reduction with femoral shortening.
5- Valgus osteotomy.
DISCUSSION: Children with spina bifida and bilateral symmetrical dislocation of the hips
usually do not require treatment. A level pelvis and good range of motion of the hips are
more important for ambulation than reduction of bilateral hip dislocations. Because the
patient has good sitting and standing balance and good range of motion, maintenance of that
range of motion and symmetry is more important than reduction. Surgery is not
recommended.
 Heeg M, Broughton NS, Menelaus MB: Bilateral dislocation of the hip in spina bifida: A long-term follow-up study. J
45. Posterior spinal fusion for scoliosis should be performed on a patient with
Duchenne muscular dystrophy when
1- The patient is still ambulatory.
2- Lordotic posture is present.
3- The forced vital capacity (FVC) is less than 30% of the predicted value.
4- Curve magnitude measures 25 degrees or greater.
5- Orthotic management fails.
DISCUSSION: Progressive scoliosis develops in most patients with Duchenne muscular

dystrophy. The onset of spinal deformity typically follows the cessation of walking, and
curves can be expected to progress about 10 degrees per year. Posterior spinal fusion with
instrumentation should be performed as soon as a curve of 25 degrees or greater is
documented and before deterioration of pulmonary function (a FVC of less than 30%)
precludes surgery. Patients with kyphotic posture tend to progress more rapidly than those
with lordotic posture. Brace treatment is contraindicated because it is not definitive and it
may mask curve progression while pulmonary function is concomitantly worsening.
pp 635-651.
 Mubarak SJ, Morin WD, Leach J: Spinal fusion in Duchenne muscular dystrophy: Fixation and fusion to the
sacropelvis? J Pediatr Orthop 1993;13:752-757.
46. Figures 25a and 25b show the radiograph and MRI scan of a 7 1/2-year-old boy
who has been limping for 1 year. His pain has worsened over the past 2 weeks,
and his parents note swelling over the dorsum of the foot for the past 4 days.
Examination reveals no fever, and laboratory studies show a WBC of
6,700/mm3, an erythrocyte sedimentation rate of 26 mm/h, and a normal C-
reactive protein level. What is the most likely diagnosis?
1- Acute hematogenous osteomyelitis

2- Kohler's disease
3- Tarsal coalition
4- Tuberculosis
5- Lisfranc fracture-dislocation
DISCUSSION: The diagnosis of tuberculous osteomyelitis in children is often delayed. In

one series of 23 children, the average interval between the onset of symptoms and definite
diagnosis was 4.3 months. In these patients, the presenting signs and symptoms were found
to be mild, with the most common signs being localized swelling (69.6%) and a painful
disability of the involved limbs (65.2%). A mild elevation of the erythrocyte sedimentation
rate may be present, but the C-reactive protein level is usually normal. In patients who have
osteoarticular tuberculosis, an MRI scan generally shows large intra-articular effusions,
periarticular osteoporosis, and gross thickening of the synovial membrane. Differential
diagnosis between tuberculosis and pyogenic arthritis is difficult, and an accurate diagnosis
usually requires biopsy of synovial tissue. Aspiration of synovial fluid often results in
insufficient information to make a diagnosis. Treatment generally consists of surgical
debridement and combined antituberculous chemotherapy with isoniazid, ethambutol, and
rifampin.
 Wang MN, Chen WM, Lee KS, Chin LS, Lo WH: Tuberculous osteomyelitis in young children. J Pediatr Orthop
1999;19:151-155.
 Watts HG, Lifeso RM: Tuberculosis of the bones and joints. J Bone Joint Surg Am 1996;78:288-298.
47. A 10-year-old boy who plays baseball reports acute pain after throwing a softball
from the outfield to second base. A radiograph is shown in Figure 26.
1- observation during healing of the fracture,

followed by bone marrow or corticosteroid
injection.
2- Biopsy and appropriate chemotherapy.
3- Curettage and bone grafting.
4- High-dose systemic corticosteroids.
5- Pulsed electromagnetic fields.
DISCUSSION: The patient has a fracture through a unicameral bone cyst, as evidenced by
the "falling leaf" sign on the radiograph. Following healing of the fracture, treatment should
consist of corticosteroid injection or bone marrow injection. Some cysts heal with the
fracture and do not require injections. Biopsy is unnecessary because the radiograph shows
that the cyst is benign. Curettage and bone grafting are seldom necessary because these cysts
regularly heal with injections. Corticosteroids are useful when injected into the cyst, but are
not used systemically. Pulsed electromagnetic fields have not been used therapeutically in
this condition.
 Yandow SM, Lundeen GA, Scott SM, Coffin C: Autogenic bone marrow injections as a treatment for simple bone cyst.
J Pediatr Orthop 1998;18:616-620.
48. The mother of an otherwise healthy 1-month-old

infant reports that he is not moving his left leg after
falling from his high chair 2 days ago. He has a
temperature of 99.5 degrees F (37.5 degrees C).
Examination reveals that the left thigh is moderately
tender to palpation. Because the infant is
apprehensive, range of motion is difficult to quantify,
but appears to be normal at the hips and ankles.
Range of motion of the left knee is approximately 25
degrees to 90 degrees. A radiograph of the leg is
shown in Figure 27. Management should consist of
1- application of a long leg splint and admission to the hospital for further evaluation
and consultation.
2- application of a long leg cast, with follow-up radiographs in 3 days.
3- immediate application of a spica cast under general anesthesia.
4- a CBC, an erythrocyte sedimentation rate, blood cultures, aspiration of the distal
femur for culture, and hospital admission with administration of IV antibiotics if
required.
5- a CBC, an erythrocyte sedimentation rate, a technetium bone scan, and
consultation for bone marrow aspiration.
DISCUSSION: The patient has a bucket-handle fracture of the distal femur with bilateral
corner fractures of the distal femur and a transverse fracture of the proximal tibia. These
fractures are virtually pathognomonic of child abuse. The infant should be admitted to the
hospital, and child protection services should be notified for investigation of possible abuse.
A skeletal survey should be obtained, along with laboratory studies that include a CBC, a
platelet count, a prothrombin time, a partial thromboplastin time, and a bleeding time.
 Akbarnia BA: The role of the orthopaedic surgeon in child abuse, in Morrissy RT, Weinstein SL (eds): Lovell &
Winter's Pediatric Orthopaedics, ed 4. Philadelphia, Pa, Lippincott-Raven, 1996, pp 1315-1334.
 Black GB: Child abuse fractures, in Letts RM (ed): Management of Pediatric Fractures. New York, NY, Churchill
Livingstone, 1994, pp 931-944.
49. A 12-year-old girl with juvenile rheumatoid arthritis (JRA) has had chronic pain
and synovitis about the knee that is now well-controlled medically. Examination
reveals 20 degrees of valgus at the knee. Knee range of motion shows 10 degrees
to 90 degrees of flexion. Treatment should consist of
1- Arthroscopic synovectomy.
2- Open synovectomy.
3- Staple hemiepiphyseodesis.
4- Knee arthrodesis.
5- Varus osteotomy.
DISCUSSION: Children with JRA frequently have valgus in association with

hypervascularity because of chronic inflammation. This is normally caused by overgrowth of
the medial femoral epiphysis. Staple hemiepiphyseodesis, if done early, can reverse the
deformity. Osteotomy is usually unnecessary at this age, and there is a risk of stiffness of the
knee following the procedure. Synovectomy may be helpful but will not prevent or correct a
deformity.
 Rydholm U, Brattstrom H, Bylander B, Lidgren L: Stapling of the knee in juvenile chronic arthritis. J Pediatr Orthop
1987;7:63-68.
50. Figure 28 shows the radiograph of a 6-year-old girl who has a right thoracic
scoliosis that measures 60 degrees. Examination shows multiple cafe-au-lait
spots, and family history reveals that the child's mother has the same disorder.
The gene responsible for this disorder codes for
1- dystrophin.
2- frataxin.
3- neurofibromin.
4- peripheral myelin protein.
5- sulfate transport protein.
DISCUSSION: The patient has the dystrophic type of scoliosis seen in patients with
neurofibromatosis type I (NF-1). The NF-1 gene is located on chromosome 17 and codes for
neurofibromin, believed to be a tumor-suppresser gene. Abnormalities in the dystrophin gene
are seen in Duchenne muscular dystrophy and Becker muscular dystrophy. A mutation in the
frataxin gene is responsible for Friedreich ataxia. The most common type of hereditary
motor and sensory neuropathy (Charcot-Marie-Tooth), HMSN type IA is caused by a
complete duplication of the peripheral myelin protein gene. A defect in the cellular sulfate
transport protein results in undersulfation of proteoglycans seen in diastrophic dysplasia.
 JH: Orthopaedic Knowledge Update 6. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1999, pp 225-
234.
51. Figures 29a and 29b show the radiographs of a 13-year-old competitive gymnast
who has had elbow pain for the past 2 weeks. The pain is worse with tumbling
activities. Examination reveals a mild effusion and slight limitation of extension
and forearm rotation with no locking. Initial management should consist of:
1- elbow arthroscopy.
2- arthrotomy and internal fixation of the lesion.
3- cessation of gymnastic activities.
4- use of an elbow brace and continued gymnastic activities.
5- open drilling of the lesion.
DISCUSSION: The radiographs show a lesion in the capitellum that is consistent with
osteochondritis dissecans. There is no evidence of a loose body at this time. Initial
management should consist of cessation of gymnastic activities. Nonsteroidal anti-
inflammatory drugs and ice may help to alleviate acute symptoms; most symptoms usually
resolve in 6 to 12 weeks. The patient may then begin range-of-motion and strengthening
exercises, with a slow return to activities once full range of motion and good strength have
been achieved. However, the prognosis for a return to high-level competitive gymnastics is
guarded. Surgery is indicated for intra-articular loose bodies, a locked elbow, or failure of
nonsurgical management. Surgery may be done either open or arthroscopically. Loose
bodies should be removed, and cartilage flaps should be debrided. The results of bone
grafting and internal fixation generally have been poor. Drilling the base of the defect may
stimulate replacement with fibrocartilage, but the benefits of this procedure are not well
documented.
 Maffulli N, Chan D, Aldridge MJ: Derangement of the articular surfaces of the elbow in young gymnasts. J Pediatr
Orthop 1992;12:344-350.
 Bauer M, Jonsson K, Josefsson PO, Linden B: Osteochondritis dissecans of the elbow: A long-term follow-up study.
Clin Orthop 1992;284:156-160.
 Tivnon MC, Anzel SH, Waugh TR: Surgical management of osteochondritis dissecans of the capitellum. Am J Sports
Med 1976;4:121-128.
52. A 12-year-old boy who has had a 1-month history of right thigh pain and a limp
reports worsening of the pain after a fall, and he can no longer walk or bear
weight on the involved extremity. Radiographs of the pelvis reveal a slipped
capital femoral epiphysis with moderate to severe displacement. While
positioning the patient on the fracture table for screw fixation, partial reduction
of the slip is achieved. No further reduction maneuvers are attempted, and the
epiphysis is stabilized with a single cannulated screw. What complication is most
likely to develop following this procedure?
1- Infection
2- Chondrolysis
3- Nonunion
4- Osteonecrosis
5- Epiphyseal arrest
DISCUSSION: Traditional classification of slipped capital femoral epiphyses is based on the

following temporal criteria: acute (symptoms that persist for less than 3 weeks); chronic
(symptoms that persist for more than 3 weeks); or acute on chronic (acute exacerbation of
long-standing symptoms). A newer classification differentiates between a stable slip where
weight bearing is possible and an unstable slip if it is not. Reduction of an unstable slip often
occurs unintentionally with induction of anaesthesia and positioning of the patient for
surgery. The rate of satisfactory results is lower primarily because of a much higher
incidence of osteonecrosis following internal fixation of an unstable slip.
 Loder RT, Richards BS, Shapiro PS, Reznick LR, Aronson DD: Acute slipped capital femoral epiphysis: The
importance of physeal stability. J Bone Joint Surg Am 1993;75:1134-1140.
Surgeons, 1996, pp 151-159.
53. Figure 30 shows the AP radiograph of a 9-

month-old girl who has been referred for
evaluation of unequal leg lengths.
Examination reveals symmetrical abduction
of the hips. When the hips are flexed 90
degrees, the right knee height is greater than
the left knee. The girth of the right thigh
and calf is larger than the contralateral side.
There are no cutaneous lesions, and
examination of the spine is normal. The
infant is moving all extremities equally and
spontaneously. Management should consist
of:
1- assessment of the bone age in the left wrist

and hand.
2- an MRI scan of the spine.
3- an ultrasound of the hips.
4- a renal ultrasound.
5- a Pavlik harness.
DISCUSSION: Hemihypertrophy or hemihypotrophy is usually idiopathic, and either the leg

or the entire side of the body may be involved. In the infant or young child, it is often
difficult to determine which side is abnormal if the condition is mild. Because of the
association of Wilms' tumor with hemihypertrophy, these patients should undergo a yearly
renal ultrasound until at least age 5 years. Other conditions that may exhibit
hemihypertrophy include Klippel-Trenaunay-Weber syndrome, Proteus syndrome, and
neurofibromatosis. In this patient, the mild hemihypertrophy is idiopathic. Because of the
normal spinal examination and absence of neurologic findings, an MRI scan is unnecessary.
The absence of clinical and radiographic evidence of hip dysplasia makes both an ultrasound
of the hips and application of a Pavlik harness unnecessary.
 Richards BS (ed): Orthopaedic Knowledge Update: Pediatrics. Rosemont Ill, American Academy of Orthopaedic
Surgeons, 1996, pp 185-193.
 Sponseller PD: Localized disorders of bone and soft tissue, in Morrissy RT, Weinstein SL (eds): Lovell and Winter's
 Beals RK: Hemihypertrophy and hemihypotrophy. Clin Orthop 1982;166:199-203.
54. What is the mechanism of action of an intramuscular injection of botulinum type

A toxin in reducing spasticitiy?
1- Blocks the release of presynaptic acetylcholine release at the neuromuscular
junction and end plate
2- Acts as a gamma-aminobutyric acid (GABA) agonist
3- Inhibits afferent fibers in the dorsal root of the spinal nerve
4- Competitively blocks the acetylcholine receptor
5- Destroys the neuromuscular junction
DISCUSSION: The use of intramuscular botulinum type A toxin has been shown to be a
useful adjuvant in the management of dynamic deformity in patients with cerebral palsy.
Botulinum type A toxin is a neurotoxin produced by Clostridium botulinum that works by
interfering with presynaptic acetylcholine release at cholinergic nerve terminals. At the
cellular level, the mechanism involves endocytosis of the intact botulinum toxin molecule by
cells in the end plate, followed by disulfide cleavage and translocation of the light chain into
the cytosol where it disrupts the normal binding of the synaptosomal vesicles to the axon
terminal membrane. Neither the nerve terminal nor the neuromuscular junction is damaged.
The muscle paralysis is reversible and dose-dependent. Baclofen is a neuropharmacologic
agent that functions as a GABA agonist. Dorsal rhizotomy is a neurosurgical procedure that
reduces spasticity by dividing afferent (excitatory) fibers in the posterior rootlet of the spinal
nerves.
 Koman LA, Mooney JF III, Smith B, Goodman A, Mulvaney T: Management of cerebral palsy with botulinum-A toxin:
Preliminary investigation. J Pediatr Orthop 1993;13:489-495.
 Brin MF: Botulinum toxin: Chemistry, pharmacology, toxicity, and immunology. Muscle Nerve Suppl 1997;6:S146-
168.
55. A 5-year-old boy has had right hip pain and a limp for the past 3 months.
Examination of the right hip reveals irritability and restricted abduction and
internal rotation. AP and lateral radiographs of the hips are shown in Figures
31a and 31b. Initial management should consist of
1- A Salter innominate osteotomy.

2- A shelf acetabuloplasty.
3- A varus femoral osteotomy.
4- Symptomatic treatment, including traction, activity modification, and nonsteroidal
anti-inflammatory drugs.
5- Abduction bracing.
DISCUSSION: A favorable prognosis can be expected in up to 70% of children with Legg-

Calve-Perthes disease who are younger than age 6 years. Containment treatment has not
been shown to alter the outcome in this age group. The goals of treatment in this patient are
to reduce pain (synovitis), restore motion, and improve function. Symptomatic treatment
modalities include bed rest, traction, crutches, activity modification, and nonsteroidal anti-
inflammatory drugs.
 Herring JA: The treatment of Legg-Calve-Perthes disease: A critical review of the literature. J Bone Joint Surg Am
1994;76:448-458.
Surgeons, 1996, pp 161-166.
56. Hamstring lengthening and posterior transfer of the rectus femoris will be most
successful in a patient with cerebral palsy who has which of the following gait
abnormalities?
1- Excessive knee flexion through stance, stiff knee during swing, and rectus femoris
activity in swing
2- Excessive knee flexion through stance and continuous quadricep activity in stance
and swing
3- Excessive knee flexion through stance, short stride length, and continuous
hamstring activity in swing
4- Knee hyperextension in stance, knee extension during swing, and rectus femoris
activity in swing
5- Knee hyperextension in stance, continuous rectus femoris activity in stance and
swing, and drop foot
DISCUSSION: Children with cerebral palsy typically ambulate with a crouched gait
characterized by excessive flexion of the hips and knees during stance. Many patients exhibit
co-contracture of the quadriceps and hamstrings, causing a stiff-knee gait. Normally, the
rectus femoris fires at the initiation of swing and in terminal swing through initial contact.
Prolonged activity of the rectus femoris throughout the swing phase interferes with normal
knee flexion. This contributes to a stiff knee during swing phase and prevents clearance of
the foot. Lengthening of the hamstrings alone will not improve foot clearance. Hamstring
lengthening is contraindicated when there is hyperextension during stance. Transfer of the
rectus femoris to one of the knee flexors has been shown to improve knee flexion during
swing by an average of 15 degrees. This allows improved foot clearance.
 Gage JR, Perry J, Hicks RR, Koop S, Werntz JR: Rectus femoris transfer to improve knee function of children with
cerebral palsy. Dev Med Child Neurol 1987;29:159-166.
 Sutherland DH, Santi M, Abel MF: Treatment of stiff-knee gait in cerebral palsy: A comparison by gait analysis of
distal rectus femoris transfer versus proximal rectus release. J Pediatr Orthop 1990;10:433-441.
57. Figures 32a and 32b show the radiographs of a

13-year-old boy who sustained a fracture while
playing football 1 week ago. Management at the
time of injury included application of a cast and
the use of crutches. A follow-up office visit
reveals a normal neurologic examination, and
the patient reports no discomfort with the cast
and crutches. Management should now include:
1- cast wedging in the outpatient clinic.

2- closed reduction under anesthesia and
application of a new long leg cast.
3- reduction and placement of an intramedullary rod.
4- anatomic open reduction and compression plating with interfragmentary screws.
5- pins and plaster to maintain the reduction.
DISCUSSION: Stable fractures and minimally displaced fractures in children can and should
be treated by closed methods. Because loss of reduction is common, alignment of tibia
fractures must be monitored closely for the first 3 weeks after cast application. This is most
easily handled in a cooperative patient by cast wedging. Some children require application of
a second cast under general anesthesia 2 to 3 weeks after injury, particularly if the subsidence
of swelling has caused the cast to loosen. Surgical indications include the presence of soft-
tissue injuries, unstable fracture patterns, fractures associated with compartment syndrome,
and the child with multiple injuries. Surgical options in children include percutaneous pins,
external fixation, plates and screws, and intramedullary nails.
 Heinrich SD: Fractures of the shaft of the tibia and fibula, in Rockwood CA, Wilkins KE, Beaty JH (eds): Fractures in
Children, ed 4. Philadelphia, Pa, Lippincott-Raven, 1996, pp 1340-1346.
pp 583-595.
58. A 14-year-old patient with an L3 myelomeningocele underwent anterior and

posterior spinal fusion for a curve of 50 degrees. Follow-up examination 1 week
after the procedure now reveals persistent drainage from the posterior wound.
Results of laboratory cultures show Streptococcus viridans, Staphylococcus
aureus, and Enterococcus. In addition to IV antibiotics, surgical irrigation, and
debridement, management should include
1- Removal of all hardware.
2- Temporary placement of antibiotic beads.
3- Wound closure over drains.
4- Bedside dressing changes.
5- A RAST test for latex allergy.
DISCUSSION: The rate of wound infections has dramatically decreased with the routine use
of prophylactic antibiotics. Factors known to increase the risk of infection include
instrumentation, prolonged surgical time, excessive blood loss, poor perioperative nutritional
status, a history of surgery, and a history of infection. The use of allograft does not result in
an increased rate of infection. Adequate treatment requires early diagnosis and intervention.
Temperature elevation and persistent wound drainage are highly suspicious for infection. An
erythrocyte sedimentation rate and a WBC are not useful in diagnosis unless serial
examinations show rising levels. Patients should be taken to the operating room where the
entire wound can be reopened, irrigated, and debrided. Bone graft can be washed and
replaced. Hardware should not be removed. The wound should be closed over suction
drains. IV antibiotics should be given for a period of at least 10 days, followed by 6 weeks
orally. Leaving the wound open to granulate with dressing changes results in prolonged
hospitalization, inadequate treatment of the infection, and a poor cosmetic result.
 Lonstein JE: Complications of treatment, in Bradford DS, Lonstein JE, Moe JH, et al (eds): Moe's Textbook of
Scoliosis and Other Spinal Deformities, ed 2. Philadelphia, Pa, WB Saunders, 1987, p 476.
pp 713-721.
59. What is the primary mechanism of injury for the fracture shown in Figures 33a
and 33b?
1- Hyperdorsiflexion
2- External rotation of the foot
3- Internal rotation of the foot
4- Adduction of the foot and ankle
5- Excessive eversion of the foot and ankle
DISCUSSION: The radiographs show a triplane fracture of the ankle. In adolescence,

closure of the distal tibial physis starts peripherally at the anteromedial aspect of the medial
malleolus and extends posteriorly and laterally. The anterolateral quadrant of the physis is
the last to close, making this region the most susceptible to separation. When the foot is
twisted into external rotation, the anterolateral portion of the epiphysis is avulsed by the pull
of the anterior tibiofibular ligament. When this fragment alone is avulsed, the result is a
juvenile Tillaux fracture. When the fracture extends to involve the remainder of the physis
and posterior metaphysis, as in this patient, the result is a triplane fracture.
Surgeons, 1996, pp 267-272.
 Dias LS, Giegerich CR: Fractures of the distal tibial epiphysis in adolescence. J Bone Joint Surg Am 1983;65:438-444.
 Kling TF Jr: Operative treatment of ankle fractures in children. Orthop Clin North Am 1990;21:381-392.
60. Figure 34 shows the standing AP radiograph of

a 2-year-old girl who has a left bowleg
deformity. Her mother states that she first
noticed the problem when the child began
walking at age 10 months, and the deformity has
worsened over the past 6 months. Examination
reveals a definite lateral thrust of the knee
during the stance phase of gait. Management
should consist of:
1- observation.
2- a proximal tibial and fibular osteotomy.
3- daytime ambulatory bracing.
4- elevation of the medial tibial plateau.
5- an MRI scan of the knee.
DISCUSSION: Infantile tibia vara is a developmental condition characterized by a varus

angulation of the proximal end of the tibia that is caused by a growth disturbance of the
proximal medial physis. In a study of 42 affected extremities in 24 children younger than age
3 years, it was found that daytime ambulatory brace treatment favorably altered the natural
history of tibia vara. Another study of 27 patients with stage II Langenskiöld disease found a
success rate of 70% (improved alignment without the need for osteotomy) using brace
treatment. These authors also noted that children with unilateral disease were more likely to
obtain correction of the deformity compared with those with bilateral disease. In this patient,
observation is not warranted because untreated tibia vara has a significant risk for progressive
worsening. Osteotomy is best reserved for those patients who, despite bracing, do not show
satisfactory clinical and radiographic improvement by age 4 years. Elevation of the medial
tibial plateau is a treatment option for older patients who have more advanced disease. An
MRI scan would not provide any useful clinical information at this time.
 Zionts LE, Shean CJ: Brace treatment of early infantile tibia vara. J Pediatr Orthop 1998;18:102-109.
 Richards BS, Katz DE, Sims JB: Effectiveness of brace treatment in early infantile Blount's disease. J Pediatr Orthop
1998;18:374-380.
 Raney EM, Topoleski TA, Yaghoubian R, Guidera KJ, Marshall JG: Orthotic treatment of infantile tibia vara. J Pediatr
Orthop 1998;18:670-674.
61. Figures 35a and 35b show the radiographs of a 7-year-old patient who has
progressive deformity of the right thigh accompanied by a dull persistent pain
radiating to the knee. Examination reveals an obvious bulge in the right thigh,
with flexion of the hip beyond 50 degrees only if the hip is allowed to externally
rotate. Management should consist of
1- multiple osteotomies and femoral rodding.

2- pharmocologic doses of vitamin D and phosphate.
3- biopsy, followed by appropriate chemotherapy.
4- pamidronate therapy.
5- radiation therapy and a bone marrow transplant.
DISCUSSION: The patient has radiographic signs of osteogenesis imperfecta, including

osteopenia, mild acetabular protrusio, cortical thinning, and bowing associated with anterior
stress fracturing. The treatment of choice is correction of the bow with osteotomies, followed
by intramedullary fixation to prevent further deformity. Biphosphonates, such as
pamidronate, may be useful in increasing bone density and preventing fractures. Large
multicenter studies on biphosphonate efficacy are currently in progress.
 Zionts LE, Ebramzadeh E, Stott NS: Complications in the use of the Bailey-Dubow extensible nail. Clin Orthop
1998;348:186-195.
 Luhmann SJ, Sheridan JJ, Capelli AM, Schoenecker PL: Management of lower-extremity deformities in osteogenesis
imperfecta with extensible intramedullary rod technique: A 20-year experience. J Pediatr Orthop 1998;18:88-94.
 Glorieux FH, Bishop NJ, Plotkin H, Chabot G, Lanoue G, Travers R: Cyclic administration of pamidronate in children
with severe osteogenesis imperfecta. N Engl J Med 1988;339:947-952.
62. Figures 36a and 36b show the MRI scans of a 15-year-old girl who has had pain
and recurrent hemarthrosis in the knee for the past year. Plain radiographs are
normal. What is the most likely diagnosis?
1- Hemangioma of the knee
2- Hemophilia
3- Discoid lateral meniscus
4- Torn medial meniscus
5- Pauciarticular-type juvenile rheumatoid arthritis (JRA)
DISCUSSION: In paediatric patients who have pain and recurrent hemarthrosis in the knee,
haemangioma is often seen as an internal derangement of the knee, and long delays in
diagnosis are common. An MRI scan is non-invasive and will best aid in diagnosis. In this
patient, the MRI scan shows a hemangioma with no evidence of meniscal injury or discoid
meniscus. Hemophilia is unlikely because the patient is female. The presence of
hemarthrosis makes JRA an unlikely diagnosis.
 Price NJ, Cundy PJ: Synovial hemangioma of the knee. J Pediatr Orthop 1997;17:74-77.
63. A 2-year-old child has marked hypotonia and depressed reflexes. History
reveals that the child was normal at birth and developed normally for the first
year. The child also began to ambulate, but lost this ability during the next 6
months. Laboratory studies show a creatine phosphokinase level that is within
the normal range. DNA testing confirms a deletion in the survival motor neuron
(SMN) gene. What is the most likely diagnosis?
1- Rett syndrome
2- Spinal muscular atrophy, type 2
3- Congenital muscular dystrophy
4- Duchenne muscular dystrophy
5- Congenital myotonic dystrophy
DISCUSSION: The patient has spinal muscular atrophy, type 2. This type is intermediate in
severity between the Werdnig-Hoffmann type (type 1) and the Kugelberg-Welander type
(type 3). It normally manifests itself between the ages of 3 and 15 months. Survival until
adolescence is common. All three types of spinal muscular atrophy have been linked to the
SMN gene at the 5q12.2-13.3 locus. DNA testing is available and is preferred to muscle
biopsy because it is less invasive and more definitive.
 Biros I, Forrest S: Spinal muscular atrophy: Untangling the knot? J Med Genet 1999;36:1-8.
 Zerres K, Wirth B, Rudnik-Schoneborn S: Spinal muscular atrophy: Clinical and genetic correlations.
Neuromuscul Disord 1997;7:202-207.
64. A 13-year-old boy sustains a valgus stress injury to the knee while playing
football, and he is unable to bear weight after the injury. Examination reveals
tenderness medially superior to the joint line. The knee is held in flexion, and he
has a large effusion and localized medial swelling. Plain radiographs show no
obvious fracture. What is the next diagnostic step?
1- Arthroscopy
2- MRI scan
3- Stress radiographs
4- Tomogram
5- Arthrogram
DISCUSSION: In the skeletally mature individual, this mechanism of injury will often result
in a sprain of the medial collateral ligament. In skeletally immature patients, the same
mechanism can cause a fracture of the distal femoral physis. If the fracture is nondisplaced,
the plain radiographs may show only soft-tissue swelling or effusion. While the MRI scan
may show edema in the soft tissues on the medial side of the knee and even an abnormality of
the physis, stress radiographs provide a quicker and less expensive means of making the
diagnosis. Arthroscopy and arthrography would not be helpful in making the diagnosis.
Arthroscopy may result in further displacement of the injury.
 Smith L: Concealed injury to the knee. J Bone Joint Surg Am 1962;44:1659-1660.
 Beaty JH, Kumar A: Fractures about the knee in children. J Bone Joint Surg Am 1994;76:1870-1880.
65. Figure 37 shows the clinical photograph of a 1-day-old infant who weighed 10.25
lb at birth. Examination reveals an absent right Moro reflex and limited active
motion of the right shoulder, elbow, and wrist, but flexion of the fingers. Passive
range of motion of the shoulder and elbow is normal. What is the most likely
diagnosis?
1- Pseudoparalysis secondary to fracture of the
proximal humerus
2- Cervical myelomeningocele
3- Erb palsy
4- Arthrogryposis
5- Cerebral palsy and spastic hemiplegia
DISCUSSION: The patient's right upper extremity is held in the "head waiter's" posture
with the shoulder internally rotated, the elbow extended, and the wrist in flexion. The Erb
type of obstetrical brachial plexus palsy involves the C5 and C6 nerve root, and
occasionally, as in this child, the C7 nerve root. Obstetrical palsy is a traction injury, and
is associated with a high birth weight, shoulder dystocia, cephalopelvic disproportion, or
the use of forceps. Erb palsy is four times more common than injury to the entire plexus
or injury to the C8 and T1 nerve roots. It results from the shoulder being depressed while
the head and neck are laterally rotated, extended, and tilted in the opposite direction.
Most patients recover wrist extension and elbow flexion. Patients with residual weakness
of shoulder external rotation and abduction will benefit from release of the pectoralis
major, latissimus dorsi, and teres major, with transfer of the latissimus dorsi and the teres
major to the posterosuperior aspect of the rotator cuff. Recent studies using arthrograms
and CT scans have shown a higher incidence of posterior glenoid deficiency and posterior
subluxation than that observed with plain radiographs. The posterior subluxation or
dislocation can be effectively reduced by tendon release and transfer procedures.
 Hoffer MM, Phipps GJ: Closed reduction and tendon transfer for treatment of dislocation of the glenohumeral joint
secondary to brachial plexus birth palsy. J Bone Joint Surg Am 1998;80:997-1001.
 Pearl ML, Edgerton BW: Glenoid deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am
1998;80:659-667.
 Waters PM, Smith GR, Jaramillo D: Glenohumeral deformity secondary to brachial plexus birth palsy. J Bone
Joint Surg Am 1998;80:668-677.
66. Figure 38 shows the radiograph of a 5-year-old child who sustained a type III
supracondylar fracture. Examination reveals the absence of a radial pulse, but
an otherwise well-perfused hand. Following closed reduction and percutaneous
pinning, the radial pulse remains absent; however, the hand is pink and well
perfused. Management should now include
1- close observation with frequent neurovascular checks.
2- emergency angiography.
3- emergency exploration of the brachial artery.
4- removal of pin fixation and exploration of the brachial
artery.
5- thrombectomy.
DISCUSSION: In a study of over 400 patients with displaced supracondylar fractures, 3.2%
of the fractures were associated with the absence of the radial pulse with an otherwise well-
perfused hand. Based on this study, a period of close observation with frequent
neurovascular checks should be completed before attempting invasive correction of the
problem. Because of the satisfactory results with expectant management, angiography,
exploration, removal of fixation and exploration, and thrombectomy are contraindicated.
 Sabharwal S, Tredwell SJ, Beauchamp RD, Mackenzie WG, Jakubec DM, Cairns R: Management of pulseless pink
hand in pediatric supracondylar fractures of humerus. J Pediatr Orthop 1997;17:303-310.
67. Figures 39a and 39b show the radiographs of an otherwise healthy 10-year-old
boy who has had thigh pain and a limp for the past 9 months. Examination
reveals that the left lower extremity is 1 cm shorter, with reduced flexion,
abduction, and internal rotation on the left side. The patient is at the 50th
percentile for height and the 90th percentile for weight. Serum studies will most
likely show:
1- An elevated thyroid-stimulating hormone level.

2- An elevated estrogen level.
3- Elevated blood urea nitrogen and creatinine levels.
4- Growth hormone deficiency.
5- Normal laboratory values.
DISCUSSION: The patient has a slipped capital femoral epiphysis (SCFE) at a younger than
average age (average age 13.5 years for boys and 12.0 years for girls); therefore, an etiology
that is not idiopathic must be considered. Hypothyroidism can result in a SCFE, but these
children typically fall into the category of less than the 10th percentile for height. SCFE may
develop in children with a growth hormone deficiency who have undergone hormonal
replacement. Osteodystrophy caused by chronic renal failure may result in a SCFE, but the
bone quality is markedly osteopenic on radiographs and the children are chronically ill with
both low height and weight percentiles. An elevated estrogen level results in physeal closure
and is protective to physeal slippage. Therefore, this child will most likely have normal
laboratory values.
 Loder RT, Hensinger RN: Slipped capital femoral epiphysis associated with renal failure osteodystrophy. J Pediatr
Orthop 1997;17:205-211.
 Loder RT, Wittenberg B, DeSilva G: Slipped capital femoral epiphysis associated with endocrine disorders. J Pediatr
Orthop 1995;15:349-356.
68. A 7-year-old patient has had a painless limp for several months. Examination
reveals pain and spasm with internal rotation, and abduction is limited to 10
degrees on the involved side. Management consists of 1 week of bed rest and
traction, followed by an arthrogram. A maximum abduction/internal rotation
view is shown in Figure 40a, and abduction and adduction views are shown in
Figures 40b and 40c. The studies are most consistent with
1- Catterall II involvement.
2- Tubercular synovitis.
3- Herring type A involvement.
4- Hinge abduction.
5- Osteochondritis dissecans.
DISCUSSION: The radiographs show classic hinge abduction. The diagnostic feature is the
failure of the lateral epiphysis to slide under the acetabular edge with abduction, and the
abduction view shows medial dye pooling because of distraction of the hip joint. Persistent
hinge abduction has been shown to prevent femoral head remodelling by the acetabulum.
Radiographic changes are characteristic of severe involvement with Legg-Calve-Perthes
disease. The Catterall classification cannot be well applied without a lateral radiograph, but
this degree of involvement would likely be considered a grade III or IV. Because the lateral
pillar is involved, this condition would be classified as type C using the Herring lateral pillar
classification scheme.
 Reinker KA: Early diagnosis and treatment of hinge abduction in Legg-Perthes disease. J Pediatr Orthop 1996;16:3-9.
69. A 14-year-old football player has had thigh pain and weakness following a full-
contact scrimmage 24 hours ago. He recalls that he felt a sharp pain in his back after
colliding with a much heavier player. Examination reveals that the spine is minimally
tender to palpation in the upper lumbar region. Motor testing reveals quadriceps
weakness bilaterally, and a reverse straight leg raising test is positive. Plain
radiographs of the thoracolumbar spine are normal. A myelogram, a CT scan with
contrast, and an MRI scan are shown in Figures 41a through 41c. What is the most
likely diagnosis?
1- Disk herniation
2- Congenital spinal stenosis
3- Intraspinal tumor
4- Vertebral end plate fracture
5- Facet subluxation
DISCUSSION: Fracture of the vertebral end plate is a relatively uncommon injury that is
most often seen in adolescent boys. The injury is characterized by traumatic displacement of
the vertebral ring-apophysis into the spinal canal and associated disk herniation. Over one
third of these injuries are seen in children with lumbar Scheuermann disease. The injury
most frequently involves the midlumbar vertebra, and symptoms are often indistinguishable
from those associated with a herniated disk. The injury is usually not visible on plain
radiographs. The diagnosis is typically made after obtaining MRI or contrast CT scans.
Treatment consists of laminotomy and excision of the osteochondral fragments.
pp 635-651.
 Epstein NE, Epstein JA, Mauri T: Treatment of fractures of the vertebral limbus and spinal stenosis in five adolescents
and five adults. Neurosurgery 1989;24:595-604.
70. Figure 42 shows the radiograph of a 12-year-old

boy who has a limp and pain in the left hip with
athletic activity. Examination reveals decreased
abduction and internal rotation of the left hip,
with pain at the extremes of motion and a 1-cm
limb-length discrepancy. Management should
consist of
1- total hip arthroplasty.
2- innominate osteotomy.
3- varus osteotomy of the proximal femur.
4- valgus osteotomy of the proximal femur.
5- a shoe lift.
DISCUSSION: The radiograph shows changes that are most consistent with Legg-Calve
Perthes disease. Valgus extension osteotomy is the salvage procedure of choice in patients
with late symptomatic Perthes disease with severe joint incongruity. Prerequisites for valgus
extension osteotomy include an adequate range of hip adduction and proof of improved
congruity in the new position. Total hip arthroplasty is not a good alternative in the young
patient. Varus osteotomy would further shorten the extremity and place a flattened portion of
the femoral head in the acetabulum. A prerequisite of the innominate osteotomy is a
congruent reduction.
 Skaggs DL, Tolo VT: Legg-Calve-Perthes disease. J Am Acad Orthop Surg 1996;4:9-16.
 Quain S, Catterall A: Hinge abduction of the hip: Diagnosis and treatment. J Bone Joint Surg Br 1986;68:61-64.
71. The mother of a 5-year-old child reports that he has had a fever of 103 degrees F
(39.4 degrees C), leg swelling, and has been unwilling to bear weight on his right
lower leg for the past 7 days. Examination reveals point tenderness at the distal
femur. Aspiration at the metaphysis yields 10 mL of purulent fluid, and a Gram
stain reveals gram-positive cocci. In addition to hospital admission, management
should include
1- IV antibiotics.
2- IV antibiotics and immobilization of the lower extremity.
3- IV antibiotics, surgical incision and drainage of the abscess, and immobilization
of the lower extremity.
4- IV antibiotics and repeated aspirations of the metaphyseal region.
5- Oral antibiotics with careful monitoring of blood levels.
DISCUSSION: The patient has a subperiosteal abscess. Because aspiration revealed 10 mL

of purulent fluid, the treatment of choice is surgical incision and drainage of the abscess,
followed by immobilization to reduce the risk of pathologic fracture. With an adequate
response to IV antibiotics and a susceptible bacterium, the patient may then be switched to
oral antibiotics.
 Kasser JR (ed): Orthopaedic Knowledge Update 5. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1996,
pp 149-161.
72. Figure 43 shows the lateral radiograph of a 12-year-old boy with mild
osteogenesis imperfecta who injured his left elbow after pushing his brother.
Treatment should consist of
1- closed reduction and cast
immobilization.
2- closed reduction and percutaneous
pinning.
3- open reduction and fixation using an
absorbable suture.
4- open reduction and fixation using
two Kirschner wires and a figure-of-
8 tension band of absorbable suture.
5- open reduction and fixation using an
intramedullary screw.
DISCUSSION: The patient has a displaced fracture of the apophysis of the olecranon for
which most authorities recommend surgical treatment. In older children, stability of the
reduction may be achieved by the use of two parallel medullary Kirschner wires and a figure-
of-8 tension band loop of either stainless steel wire or absorbable suture. The use of an
absorbable suture does not require removal of the implant. Absorbable suture alone is best
used in very young patients who have this type of injury. An intramedullary screw would
pose an unnecessary risk of future growth disturbance. A displaced, isolated fracture of
the apophysis of the olecranon is an unusual injury in a child. It has been suggested by
several authors that children who have osteogenesis imperfecta may be especially prone to
this injury. One study reported seven of these fractures occurring in five children who had
the mild form of osteogenesis imperfecta (Sillence type IA). The authors of this study
suggest that the diagnosis of osteogenesis imperfecta be considered in any child who has a
displaced fracture of the apophysis of the olecranon, especially when the injury is associated
with relatively minor trauma.
 Stott NS, Zionts LE: Displaced fractures of the apophysis of the olecranon in children who have osteogenesis
imperfecta. J Bone Joint Surg Am 1993;75:1026-1033.
 Gaddy BC, Strecker WB, Schoenecker PL: Surgical treatment of displaced olecranon fractures in children. J Pediatr
Orthop 1997;17:321-324.
 Dormans JP, Rang M: Fractures of the olecranon and radial neck in children. Orthop Clin North Am 1990;21:257-268.
73. Figure 44 shows the radiograph of an 11-

year-old girl who has hip pain. Further
diagnostic workup should include
1- Renal ultrasound.
2- Echocardiogram.
3- Electromyogram.
4- MRI scan of the spine.
5- An iliac crest biopsy.
DISCUSSION: The patient has severe acetabular protrusio, a condition that is frequently
associated with Marfan syndrome. An echocardiogram is necessary to rule out the most
serious consequence of this syndrome, aortic root widening, which can lead to aortic valve
dysfunction or fatal aortic rupture. An electromyogram may be indicated for Charcot-Marie-
Tooth disease, which is associated with acetabular dysplasia, but not protrusio. The renal
ultrasound, the MRI scan, and the biopsy would be of no value in this patient. Protrusio can
also be seen in patients with osteogenesis imperfecta and juvenile rheumatoid arthritis.
 Steel HH: Protrusio acetabuli: Its occurrence in the completely expressed Marfan syndrome and its musculoskeletal
component and a procedure to arrest the course of protrusion in the growing pelvis. J Pediatr Orthop 1996;16:704-718.
 Wenger DR, Ditkoff TJ, Herring JA, Mauldin DM: Protrusio acetabuli in Marfan's syndrome. Clin Orthop
1980;147:134-138.
74. Figure 45 shows the radiograph of a 2-year-old patient who has progressive
lumbar scoliosis as the result of hemivertebra. Examination reveals no
associated cutaneous lesions, and an MRI scan shows no associated intraspinal
anomalies. Treatment should consist of:
1- hemivertebra excision.
2- anterior and posterior spinal fusion with
instrumentation from T4 to L4.
3- convex anterior hemiepiphyseodesis.
4- convex posterior hemiarthrodesis.
5- an orthosis.
DISCUSSION: In a retrospective review of 10 patients treated with hemivertebra excision for

hemivertebra in the levels of T12 to L3, the procedure was found to be safe and effective.
The procedure provided an average curve correction of 67 degrees and was greatest in
patients who were younger than age 4 years at the time of surgery. Long anterior and
posterior fusion with instrumentation is not the treatment of choice at this age. Either anterior
hemiepiphyseodesis or posterior hemiarthrodesis in this isolated hemivertebra setting would
be inadequate. Brace treatment is ineffective in management of the primary curvature.
 Callahan BC, Georgopoulos G, Eilert RE: Hemivertebral excision for congenital scoliosis. J Pediatr Orthop
1997;17:96-99.
75. A 10-year-old girl with a history of obstetrical brachial plexus palsy has been
referred for evaluation. Examination reveals a severe adduction internal rotation
contracture of the shoulder and a mild flexion contracture of the elbow. Hand
function is normal. Radiographs show mild glenohumeral joint incongruity. To
achieve the best functional outcome, management should consist of:
1- Physical therapy to stretch the tight structures.
2- A humeral rotational osteotomy.
3- Anterior shoulder release and posterior muscle transfers.
4- Anterior shoulder release.
5- Shoulder fusion.
DISCUSSION: The patient has an upper plexus palsy (Erb palsy) with severe shoulder
contracture. While physical therapy for stretching is the treatment of choice to prevent
contracture in the newborn, it is unlikely to be of benefit in the older child with an established
contracture. Contracture release alone or in combination with muscle transfers can improve
the cosmetic appearance, and in the case of a mild deformity, may also improve function.
These procedures are less likely to help when there is deformity of the shoulder joint or when
arthritic changes are present. The procedure of choice for an older child with joint deformity
is rotational osteotomy of the proximal humerus because it can improve cosmesis and
function, even in the face of joint deformity.
 Jahnke AH Jr, Bovill DF, McCarroll HR Jr, James P, Ashley RK: Persistent brachial plexus birth palsies. J Pediatr
Orthop 1991;11:533-537.
 Strecker WB, McAllister JW, Manske PR, Schoenecker PL, Dailey LA: Sever-L'Episcopo transfers in obstetrical palsy:
A retrospective review of 20 cases. J Pediatr Orthop 1990;10:442-444.
 Goddard NJ, Fixsen JA: Rotation osteotomy of the humerus for birth injuries of the brachial plexus. J Bone Joint Surg
Br 1984;66:257-259.
76. A 4-year-old boy sustained a nondisplaced, but complete, fracture of the left
proximal tibial metaphysis 1 year ago. The fracture healed uneventfully in an
anatomic position. Examination of the injured extremity now reveals 18 degrees
of valgus compared with 3 degrees of valgus on the opposite side. Management
should now include:
1- Observation.
2- A knee-ankle-foot orthosis.
3- A medial proximal tibial epiphyseodesis.
4- A proximal tibial osteotomy.
5- Lateral proximal tibial stapling.
DISCUSSION: The development of a valgus deformity after this type of fracture is a well-
known occurrence, and the patient's parents should be informed about this risk. In a patient
who is age 4 years, the natural history is one of gradual correction by the development of a
physiologic varus deformity at the distal tibial physis; therefore, no active intervention is
needed at this time. Bracing has no effect on the deformity, and the child is too young for
any procedure on the growth plate. Proximal tibial osteotomy is reserved until the patient
nears skeletal maturity because of the risk of recurrence of the deformity. Lateral stapling
can be done near skeletal maturity if the deformity persists, but this is unlikely to be
necessary.
 Zionts LE, MacEwen GD: Spontaneous improvement of posttraumatic tibia valga. J Bone Joint Surg Am
1986;68:680-687.
 Beaty JH, Kumar A: Fractures about the knee in children. J Bone Joint Surg Am 1994;76:1870-1880.
77. A 16-year-old boy with spastic quadriplegic

cerebral palsy has been referred for evaluation
and management of scoliosis. His parents
report increasing problems with sitting
balance, positioning, and hygiene because of
the deformity. The radiograph shown in
Figure 46 reveals a lordoscoliosis of 105
degrees with marked pelvic obliquity.
Attempts at correcting the pelvic obliquity on
supine bending radiographs show significant
rigidity. Management should consist of:
3- Anterior and posterior spinal fusion.
4- Electrical stimulation.
5- Wheelchair modifications.
DISCUSSION: Spinal stabilization is the treatment of choice in patients with severe scoliosis
who have progressive positioning, sitting balance, and/or hygiene problems despite maximal
nonsurgical management. Pelvic rigidity and marked frontal plane deformity necessitate
anterior and posterior procedures so as to maximize correction and fusion.
 Weinstein SL (ed): The Pediatric Spine: Principles and Practice. New York, NY, Raven Press, 1994, pp 977-997.
1993, pp 447-459.
78. A 10-year-old boy reports a gradual onset of weakness; however, he is fully
ambulatory. History reveals that he has a 17-year-old brother who has just
stopped walking because of a similar condition. Laboratory studies show a
creatine kinase level of 5,480 IU/L (normal 25 to 232 IU/L), and examination
shows a slightly positive Gower sign. What is the most likely diagnosis?
1- Spinal muscular atrophy, type II
2- Myotonic dystrophy
3- Duchenne muscular dystrophy
4- Becker muscular dystrophy
5- Hereditary motor sensory neuropathy, type II (HMSN)
DISCUSSION: The patient has Becker muscular dystrophy. Patients with this condition have
a slower rate of progression of disease compared with patients who have Duchenne muscular
dystrophy, and walking may continue into the late teens. The creatine kinase level is not as
high as in Duchenne muscular dystrophy, which can range from 20,000 to 30,000 IU/L.
Becker muscular dystrophy is allelic to Duchenne muscular dystrophy, resulting in a
mutation in the dystrophin gene. Myotonic dystrophy is characterized by a progressive
inability to relax the muscles after contracture. The Gower sign is not helpful in this disease.
Patients with Charcot-Marie-Tooth disease, one type of which is also known as HMSN type
II, do not have elevated creatine kinase levels and usually present with a foot deformity.
Spinal muscular atrophy, type II, usually presents with severe weakness in the second year of
life.
 Matsuo M: From molecular diagnosis to gene therapy. Brain Dev 1996;18:167-172.
 Darras BT: Molecular genetics of Duchenne and Becker muscular dystrophy. J Pediatr 1990;117:1-15.
 Gutmann DH, Fischbeck KH: Molecular biology of Duchenne and Becker's muscular dystrophy: Clinical applications.
Ann Neurol 1989;26:189-194.
79. Figure 47 shows the radiograph of a 2-day-old girl who has been referred for
swelling and limited use of the right upper extremity. The second of twins, the
infant was breech and delivered with forceps at age 38 weeks, weighing 5.37 lb.
Difficulty in moving the arm was noted shortly after birth. Examination shows
no active motion of the shoulder, elbow, or wrist. Active finger flexion and
extension are present. The elbow is mildly swollen, and passive motion shows
lack of full extension of 20 degrees, lack of full flexion of 15 degrees, and no
restriction of pronation or supination. What is the most likely diagnosis?
1- Obstetrical brachial plexus palsy
2- Congenital dislocation of the elbow
3- Congenital dislocation of the radial head
4- Arthrogryposis
5- Transphyseal fracture of the distal humerus
DISCUSSION: Fractures involving the entire distal humeral physis may be a complication
of a difficult delivery. Basing the diagnosis on radiographs can be difficult at this age
because the secondary ossification center of the lateral condyle has not developed. The key
to the diagnosis is the constant relationship of the radius and ulna, with medial and posterior
displacement of the forearm relative to the humerus. An ultrasound can be obtained to
confirm the diagnosis in newborns. Because the fracture is through cartilage, examination
may reveal only mild swelling, and crepitation may be muffled or not apparent. The lack of
apparent active motion of the shoulder, elbow, and wrist is secondary to pseudoparalysis.
Child abuse is a common mechanism of this injury in a child who is age 1 month to age 3
years.
 Beaty JH, Wilkins KE: Fractures involving the entire distal humeral physis, in Rockwood CA, Wilkins KE, Beaty JH
(eds): Fractures in Children, ed 4. Philadelphia, Pa, Lippincott-Raven, 1996, vol 3, pp 790-801.
 Dias JJ, Lamont AC, Jones JM: Ultrasonic diagnosis of neonatal separation of the distal humeral epiphysis. J Bone
Joint Surg Br 1988;70:825-828.
 Gruel CR, Sullivan JA: Transcondylar fractures of the distal humerus, in Letts RM (ed): Management of Pediatric
Fractures. New York, NY, Churchill Livingstone, 1994, pp 199-209.
80. Examination of a 12-year-old girl with a painful flatfoot deformity reveals

tenderness in the region of the sinus tarsi and no appreciable subtalar motion.
Radiographs are shown in Figures 48a through 48c. Two attempts to relieve her
symptoms by cast immobilization fail to relieve the pain. Management should
now consist of:
1- triple arthrodesis.
2- manipulation of the foot under general anesthesia.
3- continued nonsurgical management until the synchondrosis ossifies.
4- Resection of the coalition and interposition with the extensor digitorum brevis.
5- Medial closing wedge osteotomy of the calcaneus.
DISCUSSION: Surgical treatment is indicated for a symptomatic tarsal coalition that has
failed to respond to nonsurgical management. In this patient, the radiographs reveal a
calcaneonavicular coalition and no degenerative changes. The patient is symptomatic, and
two attempts at use of a short leg walking cast have failed to provide relief. For
calcaneonavicular coalitions, good results have been reported following resection and
interposition of the extensor digitorum brevis. A retrospective study of this procedure
achieved good to excellent results in 58 of 75 feet (77%). Degenerative arthritis or persistent
pain following resection of a coalition is a reasonable indication for a triple arthodesis. A
medial closing wedge osteotomy of the calcaneus may be indicated for a rigid flatfoot with
severe valgus deformity. There are no studies documenting the long-term effectiveness of a
manipulation under general anesthesia for this condition.
 Gonzalez P, Kumar SJ: Calcaneonavicular coalition treated by resection and interpostion of the extensor digitorum
brevis muscle. J Bone Joint Surg Am 1990;72:71-77.
Surgeons, 1996, pp 211-218.
81. When counseling a patient with hypophosphatemic rickets, which of the

following scenarios will always result in a child with the same disorder?
1- Female patient who has a female child
2- Female patient who has a male child
3- Male patient who has a female child
4- Male patient who has a male child
5- Disorder not inherited
DISCUSSION: Hypophosphatemic rickets is an inherited disorder that is transmitted by a

unique sex-linked dominant gene. Therefore, if a male patient has a female offspring, his
affected X chromosome will be transmitted and all of his female children will have
hypophosphatemic rickets. All male offspring of a male patient will be unaffected. All
offspring of a female patient have a 50% chance of having the disorder. Understanding the
inheritance of hypophosphatemic rickets facilitates early diagnosis and early treatment.
Medical treatment with phosphorus and some types of vitamin D (most authors recommend
calcitriol) improves, but does not fully correct, the mineralization defect in
hypophosphatemic rickets. However, if medical treatment is begun before the child begins
walking, the growth plate is then adequately protected and a bowleg deformity will most
likely be prevented.
 Evans GA, Arulanantham K, Gage JR: Primary hypophosphatemic rickets: Effect of oral phosphate and vitamin D on
growth and surgical treatment. J Bone Joint Surg Am 1980;62:1130-1138.
 Greene WB, Kahler SG: Hypophosphatemic rickets: Still misdiagnosed and inadequately treated. South Med J
1985;78:1179-1184.
82. A 15-year-old boy with Duchenne muscular dystrophy has a progressive scoliosis
that now measures 55 degrees. He is in foster care and is no longer ambulatory.
Because posterior spinal fusion with instrumentation is the recommended
treatment, the patient participates in a thorough discussion of the risks and
benefits of the procedure. However, he refuses the surgery. The physician
should now
1- Obtain a court order to perform the surgery.
2- Follow the patient clinically.
3- Place the patient in a brace.
4- Perform the surgery with permission from the legal guardians.
5- Perform the surgery with the consent of two surgeons.
DISCUSSION: Traditionally, patients have been viewed as ignorant about medical matters
and ill-equipped to determine what is in their best interest. This has been especially true for
minors. However, recent informed consent policies are now based on the patient's right to
self-determination. While most spinal surgeons would agree that spinal fusion improves
pulmonary function, sitting balance, and comfort, they would also agree that this comes at
considerable risk in a patient with compromised pulmonary function and ultimately, a
terminal condition. With increasing frequency, young people older than age 14 years are
gaining greater autonomy in decision making about their health care matters. This includes
do not resuscitate orders when young patients are terminally ill, as well as in less serious
situations. Surgery could be performed with the permission of the legal guardians; however,
in this situation it is preferable to follow the patient clinically until he consents to surgery
along with the legal guardians. Bracing is contraindicated.
 Reich WT (ed): Encyclopedia of Bioethics. New York, NY, Simon and Schuster, 1995, pp 1256-1265.
 Confidential health services for adolescents. Council on Scientific Affairs, American Medical Association. JAMA
1993;269:1420-1424.
 Holder AR: Minors' rights to consent to medical care. JAMA 1987;257:3400-3402.
83. Figure 49 shows the radiograph of a 3-year-old child with progressive bowlegs.
Laboratory studies show a calcium level of 9.5 mg/dL (normal 9.0 to 11.0
mg/dL), a phosphorus level of 4.2 mg/dL (normal 3 to 5.7 mg/dL), and an
alkaline phosphatase level of 305 IU/L (normal 104 to 345 IU/L). What is the
most likely diagnosis?
1- Blount's disease
2- Hypophosphatemic rickets
3- Nutritional rickets
4- Schmid metaphyseal dysostosis
5- Jansen metaphyseal dysostosis
DISCUSSION: The patient has bowlegs associated with very wide physes, particularly noted
at the hips. The widening of the growth plates is a classic sign of rickets; however, the
normal levels of calcium, phosphorus, and alkaline phosphatase rule out both nutritional and
hypophosphatemic rickets. Patients with nutritional rickets or hypophosphatemic rickets
have hypophosphatemia and increased alkaline phosphatase levels. Jansen metaphyseal
dysostosis has very severe radiographic findings that are not found in this patient; however,
these radiographic findings are classic for Schmid metaphyseal dysostosis. This disorder is
caused by a mutation in the gene for type X collagen, which is found only in the growth
plates of growing children.
 Lachman RS, Rimoin DL, Spranger J: Metaphyseal chondrodysplasia - Schmid type: Clinical and radiographic
delineation with a review of the literature. Pediatr Radiol 1988;18:93-102.
 Warman ML, Abbot M, Apte SS, et al: A type X collagen mutation causes Schmid metaphyseal chondrodysplasia. Nat
Genet 1993;5:79-82.
84. Figures 50a and 50b show the standing clinical photographs of a 12-year-old boy
who has had increasing pain in the left foot for the past 9 months. He reports
that the pain is activity related, aching in nature, and localized to the medial
aspect of the midfoot and hindfoot. History reveals that he sustained a puncture
wound located superior and posterior to the medial malleolus from a plate glass
window 18 months ago. Examination reveals no restriction of ankle or subtalar
motion, normal neurovascular status, no masses, and a well-healed 1.5-cm
laceration posterior to the superior aspect of the medial malleolus. Inversion
strength of the foot is decreased to grade 3/5. Radiographs of the foot show no
bony abnormalities. Treatment should consist of
1- application of a UCBL orthoses.
2- application of an ankle-foot orthosis.
3- transfer of the flexor digitorum longus to the posterior tibialis tendon.
4- a lengthening osteotomy of the calcaneus.
5- talocalcaneal arthrodesis.
DISCUSSION: The photographs show a planovalgus posture of the foot. The foot deformity
and decreased inversion strength are secondary to laceration of the posterior tibial tendon 18
months ago. If the injury had been recognized acutely, optimal treatment would have
consisted of repair of the tendon; however, contracture now precludes that possibility.
Therefore, transfer of the flexor digitorum longus or flexor hallucis longus is the preferred
treatment. In adults with posterior tibial dysfunction, the entire tendon is typically
degenerated and the transfer must be anchored through a drill hole in the navicular. In this
patient, the distal end of the posterior tibial tendon is a satisfactory insertion site.
Lengthening osteotomy of the calcaneus could be combined with the tendon transfer if the
patient had a fixed deformity of the foot. UCBL orthoses and an ankle-foot orthosis are not
considered good long-term solutions for a 12-year-old patient.
 Mosca VS: Flexible flatfoot and skewfoot, in Drennan JC (ed): The Child's Foot and Ankle. New York, NY, Raven
Press, 1992, pp 355-376.
 Myerson MS: Adult acquired flatfoot deformity: Treatment of dysfunction of the posterior tibial tendon, in Springfield
DS (ed): Instructional Course Lectures 46. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1997, pp 393-
405.
85. Which of the following clinical scenarios represents an appropriate indication

for convex hemiepiphysiodesis/hemiarthrodesis in the treatment of a child with a
congenital spinal deformity?
1- A 3-year-old child with a hemivertebra opposite a contralateral bar and thoracic
scoliosis that measures 53 degrees
2- A 4-year-old child with a fully segmented L1 hemivertebra and scoliosis that
measures 80 degrees
3- A 4-year-old child with a fully segmented T10 hemivertebra and scoliosis that
measures 50 degrees
4- A 4-year-old child with a posterolateral hemivertebra at the thoracolumbar
junction and a kyphoscoliotic deformity that measures 45 degrees
5- A 10-year-old child with a hemivertebra and scoliosis that measures 50 degrees
DISCUSSION: Convex hemiarthrodesis and hemiepiphysiodesis are procedures designed to

gradually reduce curve magnitude in congenital scoliosis because of hemivertebrae. They are
used to surgically create an anterior and posterior bar to arrest growth on the convexity of the
existing deformity. Success of the technique is predicated on continued growth on the
concave side of the deformity. Prerequisites for this procedure include curves of limited
length (less than or equal to five vertebrae), curves of reasonable magnitude (less than 70
degrees), absence of kyphosis, concave growth potential, and appropriate age (younger than
age 5 years).
 Winter RB, Lonstein JE, Denis F, Sta-Ana de la Rosa H: Convex growth arrest for progressive congenital scoliosis due
to hemivertebrae. J Pediatr Orthop 1988;8:633-638.
86. Figures 51a through 51c show the radiographs of a 7-year-old soccer player who
reports a gradual onset of midfoot pain that began shortly after the start of
soccer season. He states that the pain is worse with activity and is partially
alleviated by rest. Examination reveals soft-tissue swelling, and tenderness and
warmth in the region of the talonavicular and navicular cunieform joints.
1- Biopsy.
2- Curettage and bone grafting.
3- Open reduction and fixation.
4- Immobilization with a long leg cast and
no weight bearing.
5- With a short leg walking cast or fracture boot.
DISCUSSION: Osteochondrosis of the tarsal navicular (Kohler disease) is an infrequent
cause of midfoot pain in children, and the etiology is unknown. The typical radiographic
findings include flattening and irregular ossification of the tarsal navicular. The medial
cunieform and talus maintain their normal articular contours. The acute process is best
treated with rest and immobilization. A short leg walking cast results in relief of pain and a
quicker return to activity compared with orthotics, although long-term success is similar with
either method of treatment. Children may return to activities when the symptoms subside.
The radiographic appearance of the talus begins to normalize by about 8 to 10 months
following the onset of symptoms.
 Lutter LD: Sports-related injuries, in Drennan JC (ed): The Child's Foot and Ankle. New York, NY, Raven Press,
1992.
87. A 10-year-old child with cerebral palsy undergoes bilateral hamstring

lengthening for severe knee flexion contractures, and knee immobilizers are
applied postoperatively. Examination at the initial postoperative check 2 hours
after surgery reveals that she can dorsiflex her toes on the right foot, but not on
the left foot. The physician should now
1- Repeat the examination in a few hours.
2- Remove the left knee immobilizer and flex the knee.
3- Apply long leg casts that include the feet.
4- Obtain an electromyogram and nerve conduction velocity studies.
5- Perform peroneal nerve exploration.
DISCUSSION: Children with cerebral palsy are often difficult to examine. However, this
patient clearly has a peroneal nerve deficit, most likely from the acute stretch after the
hamstring lengthening. The nerve has the best chance of recovery if it is relaxed by flexing
the knee. Once the nerve has recovered, gradual knee extension can be accomplished.
 Aspden RM, Porter RW: Nerve traction during correction of knee flexion deformity: A case report and calculation. J
Bone Joint Surg Br 1994;76:471-473.
 Heydarian K, Akbarnia BA, Jabalameli M, Tabador K: Posterior capsulotomy for the treatment of severe flexion
contractures of the knee. J Pediatr Orthop 1984;4:700-704.
88. Figures 52a and 52b show the plain radiographs of a 12-year-old girl who has
right distal leg pain. She reports that symptoms are present with weight-bearing
activities and improve with rest. Examination reveals diffuse tenderness over the
distal tibial metaphysis and mild swelling. A photomicrograph of the biopsy
specimen is shown in Figure 52c. What is the most likely diagnosis?
1- Giant cell tumor
2- Aneurysmal bone cyst
3- Unicameral bone cyst
4- Eosinophilic granuloma
5- Ewing's sarcoma
DISCUSSION: This lytic lesion is in the epiphyseal-metaphyseal region of the distal tibia.
The most common lesion in this area is a giant cell tumor. Although these lesions are most
commonly seen in adults, they can also occur in the skeletally immature patient. The
photomicrograph shows a lesion with multiple giant cells, the nuclei of which are similar to
those in the background stroma; this finding is characteristic of giant cell tumors. Giant cells
can be seen in many benign lesions, including aneurysmal bone cysts, Brown tumors, and
eosinophilic granuloma. These lesions usually have fewer giant cells with less nuclei. The
location of this lesion in the epiphyseal-metaphyseal area is not seen in aneurysmal bone
cysts, unicameral bone cysts, Ewing's sarcoma, or eosinophilic granuloma.
 Picci P, Manfrini M, Zucchi Z, et al: Giant cell tumor of bone in skeletally immature patients. J Bone Joint Surg Am
1983;65:486-490.
89. A 15-year-old boy with epilepsy who is treated with phenytoin sustains a
vertebral compression fracture during a breakthrough seizure. Radiographs of
the spine reveal generalized osteopenia. What is the most likely cause of the
osteopenia?
1- Induced osteoporosis
2- Acquired osteomalacia
3- Calcitonin effect
4- Hyperparathyroidism.
5- Disuse osteoporosis
DISCUSSION: As a side effect of treatment, phenytoin induces osteomalacia, or rickets, in

growing children, through interference with metabolism of vitamin D. Oral supplementation
of vitamin D can minimize this effect in patients who are undergoing prolonged treatment
with phenytoin.
 Chung S, Ahn C: Effects of anti-epileptic drug therapy on bone mineral density in ambulatory epileptic children. Brain
Dev 1994;16:382-385.
90. Figure 53 shows the pedigree of a family with an unusual type of muscular
dystrophy. This pedigree is most consistent with what type of inheritance
pattern?
1- Autosomal-dominant
2- Autosomal-recessive
3- X-linked dominant
4- X-linked recessive
5- Mitochondrial inheritance
DISCUSSION: The pedigree documents involvement of male offspring only, and it also
shows transmission through an uninvolved female carrier. This inheritance pattern is most
consistent with a x-linked recessive inheritance. It would be inconsistent with a dominant
inheritance pattern unless there was incomplete penetrance. Autosomal-recessive inheritance
would be possible only if the family member labeled II.F was also a carrier of the same gene;
however, this is unlikely. Mitochondrial inheritance is possible, but as with autosomal
patterns, mitochondrial inheritance normally affects both male and female offspring. It is
transmitted only through the maternal line.
 Gelehrter TD, Collins FS: Principles of Medical Genetics. Baltimore, Md, Williams & Wilkins, 1990, pp 27-45.
91. Which of the following is considered the most accurate test to determine the
amount of limb-length discrepancy in a patient with a knee flexion contracture
of 35 degrees?
1- Standing block test
2- Standing AP radiograph of the pelvis with blocks under the foot of the short leg
3- CT scanogram
4- Scanogram
5- Measurement from the anterior superior iliac spine to the medial malleolus
DISCUSSION: Flexion contractures and angular deformities of a limb cause inaccurate limb-
length measurement results with most clinical methods. A CT scanogram is more accurate
than standard scanograms for determining limb length in patients with knee flexion
contractures of 30 degrees or more. The cost and time necessary to complete the
examinations are comparable, but the CT scanogram delivers only 20% of the radiation
needed for standard scanograms.
 Aaron A, Weinstein D, Thickman D, Eilert R: Comparison of orthoroentgenography and computed tomography in the
measurement of limb-length discrepancy. J Bone Joint Surg Am 1992;74:897-902.
 Tachdjian MO: Pediatric Orthopedics. Philadelphia, Pa, WB Saunders, 1990, pp 2867-2870.
92. Figures 54a and 54b show the radiograph and MRI scan of a 7-year-old boy who
has a painful right thoracic scoliosis that measures 35 degrees. Neurologic
examination is normal. Management should consist of
1- Repeat radiographs in 6 months.

2- A technetium TC 99m bone scan.
4- Anterior and posterior spinal fusion.
5- A neurosurgical consultation.
DISCUSSION: Because hydrosyringomyelia, with or without an Arnold-Chiari

malformation, is now being recognized as the etiology of many infantile and juvenile
idiopathic scolioses, management should consist of a neurosurgical consultation.
Observation with follow-up radiographs is not an option in curves of this magnitude. A
technitium Tc 99m bone scan is unnecessary because the etiology of the curve has been
identified. Although spinal fusion may be needed in the future, it should not be undertaken
before the neurosurgical problem has been addressed.
 Zadeh HG, Sakka SA, Powell MP, Mehta MH: Absent superficial abdominal reflexes in children with scoliosis: An
early indicator of syringomyelia. J Bone Joint Surg Br 1995;77:762-767.
 Schwend RM, Hennrikus W, Hall JE, Emans JB: Childhood scoliosis: Clinical indications for magnetic resonance
imaging. J Bone Joint Surg Am 1995;77:46-53.
 Farley FA, Song KM, Birch JG, Browne R: Syringomyelia and scoliosis in children. J Pediatr Orthop 1995;15:187-
192.
93. A 10-month-old girl has the spinal deformity shown in Figures 55a and 55b with
no apparent neurologic finding. The next step in evaluation should be to obtain
1- a genitourinary ultrasound.
2- an MRI scan of the spine.
3- an AP radiograph of the pelvis.
4- an electromyogram and nerve conduction velocity studies.
5- an echocardiogram.
DISCUSSION: Approximately 60% of patients with a congenital spine abnormality have

associated malformations outside the spinal column. Genitourinary abnormalities are
probably the most common, occurring in up to 37% of patients. These are usually anatomic
anomalies, such as renal agenesis, duplication, fusion, and ectopia. A genitourinary
ultrasound is the least invasive screening tool. Other associated anomalies include cervical
vertebral abnormalities, VATER syndrome, and intraspinal abnormalities such as
diastematomyelia. An MRI scan is not recommended as part of the screening examination;
however, if the patient had neurologic signs or symptoms, an MRI scan would be indicated.
 Beals RK, Robbins JR, Rolfe B: Anomalies associated with vertebral malformations. Spine 1993;18:1329-1332.
 Bradford DS, Heithoff KB, Cohen M: Intraspinal abnormalities and congenital spine deformities: A radiographic and
MRI study. J Pediatr Orthop 1991;11:36-41.
94. A 12-year-old girl has scoliosis at T5-T10 that measures 62 degrees. A clinical
photograph of the axilla is shown in Figure 56. Management should consist of
2- In situ posterior spinal fusion.
3- Posterior spinal fusion with
segmental instrumentation.
4- Anterior spinal fusion with
instrumentation.
5- Anterior and posterior spinal
fusion with posterior segmental
instrumentation.
DISCUSSION: Neurofibromatosis type 1 (NF-1) is an autosomal-dominant disorder affecting

about 1 in 4,000 people. NF-1 causes tumors to grow along various types of nerves and
affects the development of non-nervous tissues, such as bone and skin. The gene for NF-1 is
located on the long arm of chromosome 17 and codes the protein neurofibromin. Research
indicates that NF-1 acts as a tumor-suppressor gene and, as such, plays an important role in
the control of cell growth and differentiation. Axillary and inguinal freckling is considered a
good diagnostic marker for NF-1. The hyperpigmented spots that measure from 2 mm to 4
mm may be congenital, but these typically appear and increase later in life. Scoliosis is the
most common musculoskeletal disorder of NF-1. The curves are frequently dystrophic,
kyphotic, and have a high risk of pseudarthrosis following spinal fusion. Anterior and
posterior spinal fusion with rigid posterior segmental instrumentation is the treatment of
choice.
 Goldberg Y, Dibbern K, Klein J, Riccardi VM, Graham JM Jr: Neurofibromatosis type 1: An update and review for the
primary pediatrician. Clin Pediatr 1996;35:545-561.
 Kim HW, Weinstein SL: Spine update: The management of scoliosis in neurofibromatosis. Spine 1997;22:2770-2776.
95. A 12-year-old girl has bilateral developmentally dislocated hips. History reveals
no previous treatment, and she reports no discomfort. Good long-term clinical
results are most likely to occur with
1- Surgical reduction of both hips and stabilization with a pelvic procedure.
2- No development of false acetabula.
3- Performance of surface replacements.
4- Abductor strengthening exercises.
5- Chiari osteotomy.
DISCUSSION: The natural history of complete developmental dislocation of the hip is

dependent on two factors: bilaterality and the presence or absence of a false acetabulum.
Patients with bilateral dislocations may have low back pain because of hyperlordosis, but
they tend to have less disability than patients with unilateral dislocations who have secondary
problems related to limb-length inequality. Degenerative joint disease and clinical disability
are most likely to develop in patients with completely dislocated hips and well-developed
false acetabula. In a 12-year-old child who has bilateral developmental hip dislocations, it
would be difficult to obtain surgical treatment results that are better than the natural history of
the disorder. Abductor strengthening exercises are unlikely to influence the long-term
outcome in this disorder. Surface replacements are not indicated in young asymptomatic
patients.
 Weinstein SL: Natural history of congenital hip dislocation (CDH) and hip dysplasia. Clin Orthop 1987;225:62-76.
 Wedge JH, Wasylenko MJ: The natural history of congenital dislocation of the hip: A critical review. Clin Orthop
1978;137:154-162.
96. Thyroid hormone regulates skeletal growth at the physis by stimulation of

1- Proteoglycan and aggrecan synthesis in the zone of proliferation.
2- Fibroblast growth factor (FGF) activity.
3- Transforming growth factor type beta (TGF-beta=) and parathyroid hormone-
related protein (PTHrP) activity.
4- Chondrocyte proliferation.
5- Chondrocyte hypertrophy, type X collagen synthesis, and alkaline phosphatase
activity.
DISCUSSION: Children with hypothyroidism have delayed bone age, reduced thickness of
the physis, disorganization of the cartilage columns of the physis, and impaired
differentiation of proliferating chondrocytes into hypertrophic cells. As a result, these
children have severe growth retardation, and slipped capital femoral epiphysis may develop
because of mechanical weakening of the physis. Thyroid hormone regulates terminal
differentiation of the growth plate chondrocytes, with a resultant increase in type X collagen
and alkaline phosphatase. These substances are important factors in matrix mineralization.
Insulin-like growth factors and FGF-2 appear to act synergistically to stimulate mitotic
activity of the growth plate chondrocytes. TGF-beta= and PTHrP stimulate proteoglycan
synthesis and mitotic activity of the chondrocytes and inhibit type X collagen and alkaline
phosphatase activity.
 Ballock RT: Regulation of skeletal growth and maturation by thyroid hormone, in Buckwalter JA, Ehrlich MG, Sandell
LJ, Trippel SB (eds): Skeletal Growth and Development: Clinical Issues and Basic Science Advances. Rosemont, Ill,
American Academy of Orthopaedic Surgeons, 1998, pp 301-317.
 Rosier RN, O'Keefe RJ, Reynolds PR, Hicks DG, Puzas JE: Expression and function of TGF-beta= and PTHrP in the
growth plate, in Buckwalter JA, Ehrlich MG, Sandell LJ, Trippel SB (eds): Skeletal Growth and Development: Clinical
Issues and Basic Science Advances. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1998, pp 285-299.
 Trippel SB: IGF-I and FGF-2 in growth plate regulation, in Buckwalter JA, Ehrlich MG, Sandell LJ, Trippel SB (eds):
Skeletal Growth and Development: Clinical Issues and Basic Science Advances. Rosemont, Ill, American Academy of
Orthopaedic Surgeons, 1998, pp 263-283.
97. A newborn has an anterolateral bow of the tibia and a duplication of the great
toe. Which of the following conditions will develop as the infant grows?
1- Lisch nodules and axillary freckling
2- Fracture of the tibia with pseudarthrosis
3- Increased tibial bowing and varus deformity
4- Decreased tibial bowing and limb-length discrepancy
5- Progressive valgus of the ankle
DISCUSSION: Anterolateral bowing of the tibia is normally associated with congenital

pseudarthrosis of the tibia. This, in turn, is associated with neurofibromatosis. Posterior
bowing is more benign and usually corrects spontaneously. However, anterolateral bowing
also corrects spontaneously, and the limb-length discrepancy may be the only remaining
sequela when associated with duplication of the great toe. Lisch nodules and axillary
freckling are pathognomonic findings in neurofibromatosis but would not be expected in this
patient because this type of tibial deformity is not associated with neurofibromatosis.
 Weaver KM, Henry GW, Reinker KA: Unilateral duplication of the great toe with anterolateral tibial bowing. J Pediatr
Orthop 1996;16:73-77.
98. During stabilization of a slipped capital femoral epiphysis, the screw penetrates
into the joint. The screw is repositioned so that it is within the femoral head.
This transient penetration of the hip joint will most likely lead to
1- An increased risk of chondrolysis.
2- An increased risk of osteonecrosis.
3- An increased risk of stiffness.
4- An increased time to closure of the physis.
5- No deleterious effect.
DISCUSSION: Chondrolysis may be associated with unrecognized permanent penetration of
the joint space by a pin or screw. However, transient penetration by the guide wire or screw
is not associated with this problem. One study described 11 hips in which there was transient
intraoperative penetration of the joint space by a guide wire or screw. These patients were
followed for at least 2 years, with none showing any clinical or radiographic evidence of
chondrolysis. Another retrospective study of 55 slipped epiphyses described 11 hips with
transient intraoperative pin penetration, with none showing development of chondrolysis.
There are no studies to suggest that transient pin penetration leads to osteonecrosis, stiffness,
or premature physeal closure.
 Zionts LE, Simonian PT, Harvey JP Jr: Transient penetration of the hip joint during in situ cannulated-screw fixation of
slipped capital femoral epiphysis. J Bone Joint Surg Am 1991;73:1054-1060.
 Vrettos BC, Hoffman EB: Chondrolysis in slipped upper femoral epiphysis: Long-term study of the etiology and natural
history. J Bone Joint Surg Br 1993;75:956-961.
99. The mother of a 3-month-old infant state that she has difficulty positioning the
infant's legs during diaper changes. Examination reveals limited abduction of
both hips and a negative Ortolani sign. A radiograph reveals bilaterally
dislocated hips. Initial management consists of guided reduction in a Pavlik
harness, with weekly follow-up. Figures 57a and 57b show the radiograph and
CT scan obtained after 6 weeks in the harness. Management should now consist
of
1- Placement of the hips in wider abduction and continued use of the harness.
2- Increased flexion of the hips and continued use of the harness.
3- Removal of the harness and application of an ilfeld splint.
4- Removal of the harness and application of a von rosen splint.
5- Removal of the harness, followed by closed or open reduction.
DISCUSSION: In an infant younger than age 6 months with a complete dislocation of the hip
that is not initially reducible, the Pavlik harness may be used for a trial of guided reduction.
When the harness is used in these patients, the infant should be followed at weekly intervals
to see if reduction has been achieved. If the hip does not reduce after 3 to 4 weeks of harness
wear, the harness should be discontinued, and closed or open reduction should be considered
to avoid secondary deformation of the posterolateral acetabulum, also known as Pavlik
harness pathology. Changing to other abduction braces is not indicated.
 Jones GT, Schoenecker PL, Dias LS: Developmental hip dysplasia potentiated by
inappropriate use of the Pavlik harness. J Pediatr Orthop 1992;12:722-726.
 Atar D, Lehman WB, Grant AD: Pavlik harness pathology. Isr J Med Sci 1991;27:325-
330.
 Weinstein SL: Developmental hip dysplasia and dislocation, in Morrissy RT, Weinstein
SL (eds): Lovell and Winter's Pediatric Orthopaedics, ed 4. Philadelphia, Pa, Lippincott-
Raven, 1996, pp 903-950.
100. A 6-year-old boy with severe spastic quadriplegic cerebral palsy is

nonambulatory. Examination reveals 10 degrees of hip abduction on the left
and 30 degrees on the right with the hips and knees extended. The Thomas test
shows 20 degrees of flexion bilaterally, and Ely test results are 3+/4 bilaterally.
Radiographs show a center edge angle of 0 degrees on the left and -10 degrees
on the right. The neck shaft angles are 170 degrees bilaterally. Which of the
following procedures would offer the best results?
1- Proximal femoral resections
2- Bilateral adducter, iliopsoas, and hamstring lengthenings
3- Bilateral varus derotation shortening osteotomies
4- Injection of botulinum toxin into the adducters bilaterally
5- Posterior branch obturator neurectomies bilaterally
DISCUSSION: The patient has bilateral subluxated hips, with nearly vertical neck shaft
angles; therefore, the treatment of choice is varus derotation osteotomy. Shortening of the
bone on one or both sides may be necessary to allow adequate range of motion
postoperatively. In patients this age and with this degree of bony deformity, soft-tissue
releases are not likely to lead to hip stability. Botulinum toxin has been shown to be effective
in the treatment of ankle equinus, but its efficacy in other areas has not been demonstrated as
yet. The indications for obturator neurectomy are unclear at present. Proximal femoral
resection is a salvage procedure for long-standing hip dislocations that are symptomatic and
not reconstructable.
 Tylkowski CM, Rosenthal RK, Simon SR: Proximal femoral osteotomy in cerebral palsy. Clin Orthop 1980;151:183-
192.
 Brunner R, Baumann JU: Long-term effects of intertrochanteric varus-derotation osteotomy on femur and acetabulum
in spastic cerebral palsy: An 11- to 18-year follow-up study. J Pediatr Orthop 1997;17:585-591.

Kay SPJ: Cleft Hand, in Green DP (Ed) : Green's Operative Hand Surgery. Philadelphia, Pa, Churchill Livingston, 1999, PP 402-414

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1.

The inheritance of the deformity shown in Figure 1 is most commonly:

2. Examination of a 12-year-old girl with bilateral anterior knee pain reveals

4. A 3-year-old boy sustains a complete paralysis following a high thoracic spinal

5. A 12-year-old girl has progressive development of cavus feet. Examination

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DISCUSSION: The patient most likely has a form of Charcot-Marie-Tooth disease, or

1- Stretching exercises, followed by taping

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9. Figures 4a through 4c show the clinical photographs and radiographs of a 12-

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42. In a longitudinal study of children with spastic diplegia, analysis of long-term

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