Orthopedic Pediatrics Review | Dr Hutaif Pediatric Orth -...

Scientific References

: Carroll R: Risk Management Handbook for Health Care Organizations, ed 4. Hoboken, NJ, Jossey-Bass, 2003, p 1142.**
**Burrington-Brown J: Working smart: Does the privacy rule allow us to release patient information over the telephone without authorization? J AHIMA 2003;74:62.**
**2****. A 13-year-old boy injured his knee playing basketball and is now unable to bear weight. Examination reveals tenderness and swelling at the proximal anterior tibia, with a normal neurologic examination. AP and lateral radiographs are shown in Figures 1a and 1b. Management should consist of
1- MRI.
2- a long leg cast.
3- fasciotomy of the anterior compartment.
4- open reduction and internal fixation.
5- patellar advancement.
PREFERRED RESPONSE: 4**
**DISCUSSION: The patient has a displaced intra-articular tibial tuberosity fracture; therefore, the treatment of choice is open reduction and internal fixation. Periosteum is often interposed between the fracture fragments and prevents satisfactory closed reduction. Fortunately, most patients with this injury are close to skeletal maturity and therefore, growth arrest and recurvatum are unusual. Nondisplaced fractures can be treated with a cast, but displaced fractures are best treated with open reduction and internal fixation. Intra-articular fractures can disrupt the joint surface and are sometimes associated with a meniscal tear; therefore, arthroscopy may be needed at the time of open reduction and internal fixation.**
**REFERENCES: McKoy BE, Stanitski CL: Acute tibial tubercle avulsion fractures. Orthop Clin North Am 2003;34:397-403.**
**Zionts LE: Fractures around the knee in children. J Am Acad Orthop Surg 2002;10:345-355.**
**3****. A 12-year-old boy sustained a grade III open tibial fracture 1 week ago and underwent multiple debridements and fracture fixation. He now has a soft-tissue defect that measures 6 cm × 6 cm, with an area of exposed bone and muscle on the distal medial leg that is a few centimeters proximal to the ankle. Management of the soft-tissue defect should now consist of
1- a gastrocnemius flap.
2- a split-thickness skin graft.
3- vacuum-assisted closure (VAC), followed by possible skin graft.
4- wet-to-dry dressing, followed by possible skin graft.
5- below-knee amputation.
PREFERRED RESPONSE: 3**
**DISCUSSION: The soft-tissue defect is in a very difficult position - the distal tibia. The defect is too distal for a gastrocnemius flap, and the exposed bone precludes an immediate skin graft.
A free flap and skin graft would be required for closure. VAC is very effective in soft-tissue defects such as this one. Healthy granulation tissues form quickly. VAC can be the definitive treatment, or it can be used before skin grafting. Wet-to-dry dressings could promote granulation, but the process is hastened substantially by VAC. Amputation is not a consideration because there are no signs of infection or fracture healing problems at this time.**
**REFERENCES: Mooney JF III, Argenta LC, Marks MW, et al: Treatment of soft tissue defects in pediatric patients using the V.A.C. system. Clin Orthop 2000;376:26-31.**
**Caniano DA, Ruth B, Teich S: Wound management with vacuum-assisted closure: Experience in 51 pediatric patients. J Pediatr Surg 2005;40:128-132.**
**4****. A 6-year-old child sustained a closed nondisplaced proximal tibial metaphyseal fracture
1 year ago. She was treated with a long leg cast with a varus mold, and the fracture healed uneventfully. She now has a 15-degree valgus deformity. What is the next step
in management?
1- Proximal tibial/fibular osteotomy with acute correction and pin fixation
2- Proximal tibial/fibular osteotomy with gradual correction and external fixation
3- MRI of the proximal tibial physis
4- Medial proximal tibial hemiepiphysiodesis
5- Continued observation
PREFERRED RESPONSE: 5**
**DISCUSSION: The tibia has grown into valgus secondary to the proximal fracture. This occurs in about one half of these injuries, and maximal deformity occurs at 18 months postinjury. The deformity gradually improves over several years, with minimal residual deformity. Therefore, treatment at this age is unnecessary as there is a high rate of recurrence and complications regardless of technique. The valgus deformity is not a result of physeal injury or growth arrest. Medial proximal tibial hemiepiphysiodesis is an excellent method of correcting the residual deformity but is best reserved until close to the end of growth.**
**REFERENCES: Brougham DI, Nicol RO: Valgus deformity after proximal tibial fractures in children. J Bone Joint Surg Br 1987;69:482.**
**McCarthy JJ, Kim DH, Eilert RE: Posttraumatic genu valgum: Operative versus nonoperative treatment. J Pediatr Orthop 1998;18:518-521.**
**Robert M, Khouri N, Carlioz H, et al: Fractures of the proximal tibial metaphysis in children: Review of a series of 25 cases. J Pediatr Orthop 1987;7:444-449.**
**5****. To control most spontaneous bleeding into the knee in children with hemophilia, factor VIII must be replaced to what percentage of normal?
1- 0% to 10%
2- 20% to 30%
3- 40% to 50%
4- 60% to 70%
5- 80% to 90%
PREFERRED RESPONSE: 3**
**DISCUSSION: The knee is the most common location of spontaneous bleeding in children with hemophilia. Treatment generally requires replacement to 40% to 50% of normal. For surgery, the replacement should be to 100%. The plasma level generally rises 2% for every unit
(per kg body weight) of factor VIII administered. **
**REFERENCES: Rodriquez-Merchan EC: Management of the orthopaedic complications of hemophilia. J Bone Joint Surg Br 1998;80:191-196.**
**Koval KJ (ed): Orthopaedic Knowledge Update 7. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, p 235.**
**6****. A 6-year-old girl is referred for the elbow injury seen in Figure 2. What is the most appropriate treatment?
1- Immobilization in a long arm cast for 3 weeks
2- Immobilization in a long arm cast for 8 weeks
3- Open reduction and immobilization in a long-arm cast for 3 weeks
4- Open reduction and internal fixation with smooth pins
5- Open reduction and internal fixation with a screw
PREFERRED RESPONSE: 4**
**DISCUSSION: The patient has a displaced lateral condyle fracture; therefore, simple immobilization for 3 to 8 weeks is likely to result in malunion or nonunion. Closed reduction of such injuries is rarely successful. The fracture is unstable, so fixation is required after open reduction. Because the fixation must cross the physis, smooth pins are indicated for the skeletally immature elbow. Open reduction with fixation has been shown to reduce the risk of delayed union and malunion.**
**REFERENCES: Beaty JH, Kasser JR: The elbow: Physeal fractures, apophyseal injuries
of the distal humerus, avascular necrosis of the trochlea, and T-condylar fractures, in Beaty JH, Kasser JR (eds): Fractures in Children, ed 5. Philadelphia, PA, Lippincott Williams & Wilkins, 2001, pp 625-703.**
**Rutherford A: Fractures of the lateral humeral condyle in children. J Bone Joint Surg Am 1985;67:851-856.**
**Hasler CC, von Laer L: Prevention of growth disturbances after fractures of the lateral humeral condyle in children. J Pediatr Orthop B 2001;10:123-130.**
**7****. A patient who underwent closed reduction of the hips as an infant now reports
pain. An abduction internal rotation view shows an incongruous joint. Based on the findings shown in Figure 3, what is the most appropriate type of pelvic osteotomy for
the right hip?
1- Salter-type innominate
2- Pemberton-type
3- Triple innominate
4- Ganz or Bernese periacetabular
5- Chiari
PREFERRED RESPONSE: 5**
**DISCUSSION: Pelvic osteotomies that redirect hyaline cartilage over the femoral head offer the potential for long-term preservation of the hip; however, salvage procedures such as the Chiari osteotomy are indicated in patients without a concentrically reducible hip. Ito and associates reported that moderate dysplasia and moderate subluxation without complete obliteration of the joint space and a preoperative center-edge angle of at least minus 10 degrees are desirable selection criteria.**
**REFERENCES: Ohashi H, Hirohashi K, Yamano Y: Factors influencing the outcome of Chiari pelvic osteotomy: A long-term follow-up. J Bone Joint Surg Br 2000;82:517-525.**
**Ito H, Matsuno T, Minami A: Chiari pelvic osteotomy for advanced osteoarthritis in patients with hip dysplasia. J Bone Joint Surg Am 2004;86:1439-1445.**
**8****. An 18-year-old girl with quadriplegic cerebral palsy underwent posterior spinal fusion from T2 to the pelvis 3 weeks ago. She now has a low-grade fever and mild midline erythema in a 1-cm area from which there is slight clear yellowish drainage. What is the next most appropriate step in management?
1- Oral antibiotics empirically
2- Culture of the drainage and oral antibiotics
3- Aspiration of the wound and IV antibiotics empirically
4- Wound culture with incision and debridement, IV antibiotics, and retention of instrumentation
5- Wound culture with incision and debridement, IV antibiotics, and removal of instrumentation followed by bracing
PREFERRED RESPONSE: 4**
**DISCUSSION: The presence of drainage 3 weeks after surgery is a sign of wound infection. This infection most likely involves deep tissues until proven otherwise. Oral or IV antibiotics, in the absence of debridement, are not sufficient. Removal of the hardware would lead to rapid progression of the scoliosis in a spine that has been surgically destabilized by removal of the facet joints. The appropriate treatment is debridement with wound culture, IV antibiotics, and retention of hardware. The wound should be closed over drains.**
**REFERENCES: Theiss SM, Lonstein JE, Winter RB: Wound infections in reconstructive spine surgery. Orthop Clin North Am 1996;27:105-110.**
**Richards BS: Delayed infections following posterior spinal instrumentation for the treatment of idiopathic scoliosis. J Bone Joint Surg Am 1995;77:524-529.**
**9****. A 13-year-old girl is referred for a painful progressive valgus deformity of the right knee. Examination reveals an antalgic gait with an obvious valgus deformity. The right distal femur has a palpable, tender mass with erythema and warmth. Figures 4a and 4b show a clinical photograph and a radiograph. Management should consist of
1- needle biopsy of the distal femur.
2- open reduction and internal fixation with a locking plate.
3- closed reduction and fixation with a reamed antegrade locking
intramedullary nail.
4- referral to an orthopaedic oncologist for staging studies, biopsy, and
definitive management.
5- IV antibiotics for 6 weeks.
PREFERRED RESPONSE: 4**
**DISCUSSION: The radiograph shows a pathologic fracture through a destructive lesion of the distal femur metaphysis with osteolytic and osteoblastic features. The lateral cortex is destroyed, and there is periosteal new bone formation. These findings are consistent with malignancy, most likely an osteogenic sarcoma. Patients with suspected malignant tumors are best managed by surgeons with specific expertise in orthopaedic oncology. The biopsy of a malignant lesion should be deferred to the surgeon who is capable of definitive management of the patient.**
**REFERENCES: Enneking W: Principles of musculoskeletal oncologic surgery, in Evarts C (ed): Surgery of the Musculoskeletal System. New York, NY, Churchill Livingston, 1990.**
**Herring JA: General principles of tumor management, in Herring JA (ed): Tachdjian’s Pediatric Orthopaedics, from the Texas Scottish Rite Hospital for Children, ed 3. Philadelphia, PA, WB Saunders, 2002, pp 1897-1900.**
**10****. An 18-month-old boy with obstetric brachial plexus palsy is being evaluated for limited right shoulder motion. Physical therapy for the past 6 months has failed to result in improvement of the contracture. Which of the following studies is necessary prior to any shoulder reconstruction?
1- Electromyography
2- MRI of the shoulder joint
3- MRI of the brain
4- Radiograph of the elbow
5- Aspiration of the right shoulder
PREFERRED RESPONSE: 2**
**DISCUSSION: The child sustained a brachial plexus injury at birth, and internal rotation/adduction contractures frequently develop at the shoulder. Initial treatment should consist of physical therapy to increase the range of motion. If this fails, as in this patient, MRI is used to evaluate the glenohumeral joint. Commonly, there is joint deformity with increased retroversion of the glenoid and even posterior shoulder subluxation. If the deformity is mild, an anterior release, coupled with teres major and latissimus transfers, is very effective. If the deformity is severe and the shoulder is unreconstructable, then humeral derotation osteotomy is the procedure of choice. MRI of the brain, a radiograph of the elbow, and aspiration of the shoulder would not be helpful.**
**REFERENCES: Waters PM: Update on management of pediatric brachial plexus palsy.
J Pediatr Orthop B 2005;14:233-244. **
**Waters PM, Bae DS: Effect of tendon transfers and extra-articular soft-tissue balancing on glenohumeral development in brachial plexus birth palsy. J Bone Joint Surg Am
2005;87:320-325. **
**Moukoko D, Ezaki M, Wilkes D, et al: Posterior shoulder dislocation in infants with neonatal brachial plexus palsy. J Bone Joint Surg Am 2004;86:787-793.**
**11****. Where is the underlying defect in a rhizomelic dwarf with the findings shown in
Figure 5?
1- Type I collagen
2- Type II collagen
3- Collagen oligomeric protein (COMP)
4- Sulfate transport
5- Fibroblast growth factor receptor 3
PREFERRED RESPONSE: 5**
**DISCUSSION: The radiograph shows the typical findings of achondroplasia. The defect is in fibroblast growth factor receptor 3. The pedicles narrow distally in the lumbar spine. The pelvis is low and broad with narrow sciatic notches and ping-pong paddle-shaped iliac wings. This is often called a champagne glass pelvis. Type I collagen abnormalities are typically found in osteogenesis imperfecta, and type II collagen defects are found in spondyloepiphyseal dysplasia and Kneist syndrome. COMP is defective in multiple epiphyseal dysplasia. Sulfate transport defects are seen in diastrophic dysplasia.**
**REFERENCES: Johnson TR, Steinbach LS: Essentials of Musculoskeletal Imaging. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2004, pp 809-812.**
**Caffey J: Achondroplasia of the pelvis and lumbosacral spine: Some roentgenographic features. Am J Roentgenol 1958;80:449.**
**12****. A 2-year-old boy has complete absence of the sacrum and lower lumbar spine. What is the most likely long-term outcome if no spinal pelvic stabilization is performed?
1- Progressive paralysis
2- Neck extension contracture
3- Inability to sit without using the hands for support
4- Progressive hip dislocation
5- Sexual dysfunction
PREFERRED RESPONSE: 3**
**DISCUSSION: Without stabilization, progressive kyphosis will develop between the spine and pelvis. The kyphosis progresses to the point that the child must use his or her hands to support the trunk, and therefore is unable to use his or her hands for other activities. Neck extension contracture does not usually develop. Neurologic deficit, including sexual dysfunction, is generally present at birth and static.**
**REFERENCES: Tachdjian MO: The spine: Congenital absence of the sacrum and lumbosacral vertebrae (lumbosacral agenesis), in Wickland EH Jr (ed): Pediatric Orthopaedics, ed 2. Philadelphia, PA, WB Saunders, 1990, vol 3, p 2228.**
**Renshaw TS: Sacral agenesis: A classification and review of twenty-three cases. J Bone Joint Surg Am 1978;60:373-383.**
**13****. Which of the following findings is most prognostic for the ability of a young child with cerebral palsy to walk?
1- Ability to sit independently by age 2 years
2- Ability to creep by age 2 years
3- Ability to roll by age 2 years
4- Pattern of cerebral palsy (quadriplegia, diplegia, hemiplegia)
5- Type of motor dysfunction (spastic, ataxic, dyskinetic, hypotonic)
PREFERRED RESPONSE: 1**
**DISCUSSION: Several studies have shown that sitting ability by age 2 years is highly prognostic of walking. Molnar and Gordon reported that children not sitting independently by age 2 years had a poor prognosis for walking. Wu and associates reported that children sitting without support by age 2 years had an odds ratio of 26:1 of walking compared with those unable to sit. This was far higher than the odds ratios for cerebral palsy location, motor dysfunction, crawling, creeping, scooting, or rolling.**
**REFERENCES: Molnar GE, Gordon SU: Cerebral palsy: Predictive value of selected clinical signs for early prognostication of motor function. Arch Phys Med Rehabil 1976;57:153-158.**
**Wu YW, Day SM, Strauss DJ, et al: Prognosis for ambulation in cerebral palsy: A population-based study. Pediatrics 2004;114:1264-1271.**
**14****. A 2-year-old girl has had a 2-day history of fever and refuses to move her left shoulder following varicella. Laboratory studies show an erythrocyte sedimentation rate of
75 mm/h and a peripheral WBC count of 18,000/mm**P3P**. What is the most common organism in this scenario?
1- Kingella kingae
2- Group A beta-hemolytic streptococcus
3- Group B streptococcus
4- Staphylococcus epidermidis
5- Staphylococcus aureus
PREFERRED RESPONSE: 2**
**DISCUSSION: The most common bacterial etiologic agent following varicella is group A beta-hemolytic streptococcus. The other organisms are much less common. Staphylococcus aureus is the most common bone infection organism. Staphylococcus epidermidis is increasingly a bone infection organism. Group B streptococcus occurs more commonly in newborns. Kingella kingae is a common joint pathogen but is not as common following varicella.**
**REFERENCES: Schreck P, Schreck P, Bradley J, et al: Musculoskeletal complications of varicella. J Bone Joint Surg Am 1996;78:1713-1719.**
**Mills WJ, Mosca VS, Nizet V: Orthopaedic manifestations of invasive group A streptococcal infections complicating primary varicella. J Pediatr Orthop 1996;16:522-528.**
**15****. Which of the following is considered the best method to measure limb-length discrepancy in a patient with a knee flexion contracture?
1- Obtain a standard scanogram
2- Obtain a lateral CT scanogram
3- Measure the distance from the anterior superior iliac spine to the medial malleolus
4- Measure the distance from the umbilicus to the medial malleolus
5- Stand the patient on blocks to measure the difference in the heights of the
iliac wings
PREFERRED RESPONSE: 2**
**DISCUSSION: The most effective way to measure a limb-length discrepancy in a patient with a knee flexion contracture is a lateral CT scanogram. All the other methods listed provide inaccurate results with a knee flexion contracture because the measurements are made in the coronal plane.**
**REFERENCES: Aaron A, Weinstein D, Thickman D, et al: Comparison of orthoroentgenography and computed tomography in the measurement of limb-length discrepancy. J Bone Joint Surg Am 1992;74:897-902.**
**Tachdjian MO: Clinical Pediatric Orthopaedics: The Art of Diagnosis and Principles of Management. Stamford, CT, Appleton and Lange, 1997, pp 237-240.**
**16****. A 5-year-old boy sustained an elbow injury. Examination in the emergency department reveals that he is unable to flex the interphalangeal joint of his thumb and the distal interphalangeal joint of his index finger. The radial pulse is palpable at the wrist, and sensation is normal throughout the hand. Radiographs are shown in Figures 6a and 6b. In addition to reduction and pinning of the fracture, initial treatment should include
1- repair of the posterior interosseous nerve.
2- repair of the median nerve at the elbow.
3- neurolysis of the anterior interosseous nerve.
4- observation of the nerve palsy.
5- immediate electromyography and nerve conduction velocity studies.
PREFERRED RESPONSE: 4**
**DISCUSSION: The findings are consistent with a neurapraxia of the anterior interosseous branch of the median nerve. This is the most common nerve palsy seen with supracondylar humerus fractures, followed closely by radial nerve palsy. Nearly all cases of neurapraxia following supracondylar humerus fractures resolve spontaneously, and therefore, further diagnostic studies and surgery are not indicated.**
**REFERENCES: Cramer KE, Green NE, Devito DP: Incidence of anterior interosseous nerve palsy in supracondylar humerus fractures in children. J Pediatr Orthop 1993;13:502-505.**
**Sood MK, Burke FD: Anterior interosseous nerve palsy: A review of 16 cases. J Hand Surg Br 1997;22:64-68.**
**17****. An 11-year-old basketball player reports that he felt a painful pop in the left knee when he stumbled while running. He is unable to bear weight on the extremity and cannot actively extend the knee against gravity. Examination reveals a large knee effusion.
A lateral radiograph is shown in Figure 7. Management should consist of
1- physical therapy for quadriceps strengthening exercises.
2- a long leg cast with the knee fully extended.
3- excision of the fragment.
4- suture reattachment of the patellar tendon to the tibial tuberosity.
5- open reduction and tension band fixation.
PREFERRED RESPONSE: 5**
**DISCUSSION: The radiograph shows an avulsion fracture, or “sleeve fracture,” of the distal pole of the patella. The distal fragment is much larger than it appears on the radiograph because it largely consists of cartilage; therefore, excision of the fragment is contraindicated. The treatment of choice is open reduction and tension band fixation to correct patella alta and restore the extensor mechanism.**
**REFERENCES: Maguire JK, Canale ST: Fractures of the patella in children and adolescents.
J Pediatr Orthop 1993;13:567-571.**
**Grogan DP, Carey TP, Leffers D, et al: Avulsion fractures of the patella. J Pediatr Orthop 1990;10:721-730.**
**18****. Figures 8a and 8b show the clinical photograph and radiograph of a 4-month-old infant who has a left foot deformity. Examination reveals that the foot deformity is an isolated entity, and the infant has no known neuromuscular conditions or genetic syndromes. Which of the following studies will best confirm the diagnosis?
1- MRI of the foot
2- Static ultrasound examination of the foot in dorsiflexion
3- Lateral radiograph of the foot in maximum plantar flexion
4- Lateral radiograph of the foot in maximum dorsiflexion
5- CT of the foot
PREFERRED RESPONSE: 3**
**DISCUSSION: The clinical photograph shows a rocker-bottom deformity, and the lateral radiograph suggests a congenital vertical talus deformity. A lateral radiograph of the foot in maximum plantar flexion is needed to demonstrate the fixed position of the deformity with malalignment of the talar-metatarsal axis. A fixed dislocation of the navicular on the talus differentiates a congenital vertical talus from the oblique talus with talonavicular subluxation.**
**REFERENCES: Kumar SJ, Cowell HR, Ramsey PL: Vertical and oblique talus. Instr Course Lect 1982;31:235-251.**
**Kodros SA, Dias LS: Single-stage correction of congenital vertical talus. J Pediatr Orthop 1999;19:42-48.**
**Herring JA: Disorders of the foot, vertical talus, in Herring JA (ed): Tachdjian’s Pediatric Orthopaedics, from the Texas Scottish Rite Hospital for Children, ed 3. Philadelphia, PA,
WB Saunders, 2002, pp 959-967.**
**19****. An 8-year-old girl was treated for a Salter-Harris type I fracture of the right distal femur
2 years ago. Examination reveals symmetric knee flexion, extension, and frontal alignment compared to the contralateral knee. She has 1-cm of shortening of the right femur. History reveals that she has always been in the 50th percentile for height, and her skeletal age matches her chronologic age. Radiographs are shown in Figure 9. What is the expected consequence at maturity?
1- 6-cm limb-length discrepancy with the right femur longer
2- 6-cm limb-length discrepancy with the left femur longer
3- 12-degree varus deformity
4- 18-degree valgus deformity
5- 20-degree recurvatum deformity
PREFERRED RESPONSE: 2**
**DISCUSSION: The child has a near complete central physeal arrest of the distal femur and worsening limb-length discrepancy will develop. She is growing at the average rate for the population. The distal femoral physis grows at a rate of roughly 9 mm per year. Girls finish their growth at approximately age 14 years. Thus, at maturity the left leg will be 6.4 cm longer than the right. An angular deformity has not developed at this point and her arrest is central; therefore, angular deformity is unlikely to develop in any plane.**
**REFERENCES: Little DG, Nigo L, Aiona MD: Deficiencies of current methods for the timing of epiphysiodesis. J Pediatr Orthop 1996;16:173-179.**
**Moseley CF: Assessment and prediction in leg-length discrepancy. Instr Course Lect 1989;38:325-330.**
**20****. Examination of an obese 3-year-old girl reveals 30 degrees of unilateral genu varum.
A radiograph of the involved leg with the patella forward is shown in Figure 10. Management should consist of
1- continued observation until skeletal maturity.
2- fitting for a valgus-producing hinged knee-ankle-foot orthosis.
3- lateral proximal tibial hemiepiphysiodesis.
4- proximal tibiofibular osteotomy and acute correction.
5- proximal tibiofibular epiphysiodesis and osteotomy with lengthening.
PREFERRED RESPONSE: 4**
**DISCUSSION: The clinical scenario describes infantile tibia vara (Blount’s disease). The radiograph shows severe deformity with the characteristic Langenskiold stage 3 changes of the medial proximal tibial metaphysis that distinguish it from physiologic bowing. The preferred treatment is proximal tibiofibular osteotomy with acute correction into slight valgus to unload the damaged area of the physis. This method provides the best results in patients younger than age 4 years. Continued observation would result in progressive deformity. Bracing is most effective in younger children with less severe deformity. Lateral proximal tibial hemiepiphysiodesis relies on growth of the injured medial physis for correction and would result in severe tibial shortening in this young child. Complete epiphysiodesis also produces severe shortening and requires multiple lengthening procedures.**
**REFERENCES: Johnston CE II: Infantile tibia vara. Clin Orthop 1990;255:13-23.**
**Richards BS, Katz DE, Sims JB: Effectiveness of brace treatment in early infantile Blount’s disease. J Pediatr Orthop 1998;18:374-380.**
**21****. What is the most important consideration in the preoperative evaluation of a child with polyarticular or systemic juvenile rheumatoid arthritis (JRA)?
1- Cervical spine assessment
2- Temporomandibular joint (TMJ)/jaw assessment
3- Dental assessment
4- Stress dosing with corticosteroids
5- Opthalmology examination
PREFERRED RESPONSE: 1**
**DISCUSSION: The cervical spine may be involved in a child with polyarticular or systemic JRA; fusion or instability can occur. Radiographic assessment of the cervical spine should include lateral flexion-extension views. The potential exists for spinal cord injury during intubation or positioning in the presence of an unstable cervical spine. Limitations of the TMJ and micrognathia may affect ease of intubation and administration of anesthesia via a mask. If the TMJ and jaw are involved, some patients may have dental findings such as dental caries and even abscesses which can affect surgery. Some children, particularly those with systemic arthritis, may be taking corticosteroids long-term and may need stress dosing with complex surgeries. Although it is important to routinely check for uveitis and iritis in children with JRA, this usually is not needed preoperatively. Uveitis and iritis are less likely in a child with systemic JRA.**
**REFERENCES: Cassity JT, Petty RE (eds): Textbook of Pediatric Rheumatology, ed 5. Philadelphia, PA, WB Saunders, 2005.**
**Ilowite N: Current treatment of juvenile rheumatoid arthritis. Pediatrics 2002;109:109-115.**
**Ruddy S, Harris ED, Sledge CB (eds): Kelley’s Textbook of Rheumatology, ed 6. Philadelphia, PA, WB Saunders, 2001.**
**Hamalainen M: Surgical treatment of juvenile rheumatoid arthritis. Clin Exp Rheumatol 1994;12:S107-S112.**
**22****. A 15-year-old boy has a mass at the knee. Radiographs show an aggressive tumor involving the proximal tibia, and biopsy findings reveal a high-grade osteosarcoma. Staging studies show that the tumor impinges on the neurovascular bundle. The tumor enlarges during preoperative chemotherapy. Management should now consist of
1- prolonged chemotherapy.
2- radiation therapy.
3- amputation.
4- marginal excision, sparing the neurovascular bundle.
5- wide excision, including the neurovascular bundle.
PREFERRED RESPONSE: 3**
**DISCUSSION: Limb salvage procedures have become the usual treatment for even high-grade osteosarcomas. However, tumors associated with pathologic fracture, tumors encasing the neurovascular bundle, and tumors that enlarged during preoperative therapy and are adjacent to the neurovascular bundle require amputation.**
**REFERENCES: Springfield D: Bone and soft-tissue tumors, in Morrissy RT, Weinstein SL (eds): Lovell and Winter’s Pediatric Orthopaedics, ed 4. Philadelphia, PA, Lippincott-Raven, 1996, pp 423-468.**
**23****. Figure 11 shows the radiograph of a 2-year-old child with marked genu varum and tibial bowing. Based on these findings, what is the best initial course of action?
1- Obtain serum phosphorous, calcium, and alkaline phosphatase levels.
2- Obtain a scanogram to assess for limb-length discrepancy.
3- Perform bilateral valgus osteotomies to correct the deformities.
4- Measure the child for a varus prevention orthosis.
5- Educate the family about physiologic genu varum and conduct a follow-up examination in 6 months.
PREFERRED RESPONSE: 1**
**DISCUSSION: The radiograph shows multiple wide physes, consistent with a diagnosis of rickets. A low serum phosphorous level and an elevated alkaline phosphatase level are the hallmarks in diagnosing familial hypophosphatemic Vitamin D-resistant rickets. Serum calcium is usually normal or low normal. This disease is inherited as an X-linked dominant trait and usually presents at age 18 to 24 months. The disease results from a poorly defined problem with renal phosphate transport in which normal dietary intake of vitamin D is insufficient to achieve normal bone mineralization. Renal tubular dysfunction is associated with urinary phosphate wasting. Treatment involves oral phosphate supplementation, which can cause hypocalcemia and secondary hyperparathyroidism. To prevent associated problems, high doses of Vitamin D are administered. While obtaining a scanogram may be clinically indicated in an associated limb-length discrepancy, and subsequent corrective surgery may be indicated, either of these choices would not be the first course of action. An orthosis may slow the progression of genu varum in this disorder but is less important than establishing the correct diagnosis to begin pharmacologic treatment. This amount of varum and tibial bowing far exceeds the normal limits of physiologic genu varum. Skeletal dysplasias usually are not associated with abnormal laboratory values.**
**REFERENCES: Herring JA: Metabolic and endocrine bone diseases, in Tachdjian’s Pediatric Orthopaedics, ed 3. New York, NY, WB Saunders, 2002, pp 1685-1743.**
**Sillence DO: Disorders of bone density, volume, and mineralization, in Rimoin DL, Conner JM, Pyerite RE, et al (eds): Principles and Practice of Medical Genetics, ed 3. New York, NY, Churchill Livingstone, 1995, pp 1996-2002.**
**24****. Figure 12 shows the radiograph of a 15-year-old boy with cerebral palsy who has pain at the first metatarsophalangeal joints. He is a community ambulator. Management consisting of accommodative shoes has failed to provide relief. What is the treatment
of choice?
1- Custom-molded night orthotics
2- Double osteotomy of the first metatarsals
3- Crescentic osteotomy of the first metatarsals
4- Distal realignment (modified McBride)
5- First metatarsophalangeal joint arthrodeses
PREFERRED RESPONSE: 5**
**DISCUSSION: While other surgeries have provided some success, first metatarsophalangeal joint arthrodesis has the highest overall success rate compared to other surgeries in ambulatory and nonambulatory children with cerebral palsy. The recurrence rate is unacceptably high with the other procedures listed above. In contrast, neurologically normal children are amenable to osteotomies and soft-tissue procedures.**
**REFERENCES: Davids JR, Mason TA, Danko A, et al: Surgical management of hallux valgus deformity in children with cerebral palsy. J Pediatr Orthop 2001;21:89-94.**
**Jenter M, Lipton GE, Miller F: Operative treatment for hallux valgus in children with cerebral palsy. Foot Ankle Int 1998;19:830-835.**
**25****. What risk factor is most associated with progression of idiopathic scoliosis to a curve requiring surgery?
1- Curve magnitude of more than 20 degrees at menarche
2- Curve magnitude of more than 30 degrees at the peak height velocity
3- Curve magnitude of more than 30 degrees at skeletal age 12 years
4- Curve magnitude of more than 30 degrees at Risser grade 2
5- Curve flexibility of less than 50% at Risser grade 2
PREFERRED RESPONSE: 2**
**DISCUSSION: The magnitude of the curve at the time of the peak height velocity is the most prognostic sign in relationship to surgery. More than 70% of curves that measure more than
30 degrees at this time are likely to reach surgical range.**
**REFERENCES: Little DG, Song KM, Katz D, et al: Relationship of peak height velocity to other maturity indicators in idiopathic scoliosis in girls. J Bone Joint Surg Am 2000;82:685-693.**
**Sanders JO, Moreland M, Bassett G: Idiopathic scoliosis: Prevalence and natural history, in DeWald R (ed): Spinal Deformities: The Comprehensive Text. New York, NY, Thieme Medical Publishers, 2003.**
**26****. The parents of a 15-month-old child report that he is not yet walking. Further evaluation, rather than reassurance and observation, should be conducted if the child is not performing what other activity?
1- Talking
2- Sitting
3- Building a tower of blocks
4- Drinking from a cup
5- Scribbling on paper
PREFERRED RESPONSE: 2**
**DISCUSSION: A child not ambulating at age 15 months is still within normal limits. The
child should be able to sit by age 9 months. The remaining milestones listed are reached later
in development.**
**REFERENCES: Behrman RE, Kliegman RM, Jenson HB: The first year, in Fletcher J, Thorp D, Davis RE (eds): Nelson Textbook of Pediatrics, ed 17. Philadelphia, PA, WB Saunders, 2004,
p 33.**
**27****. Of the following clinical situations, which is most likely to lead to osteonecrosis associated with a slipped capital femoral epiphysis (SCFE)?
1- A girl younger than age 15 years
2- A boy younger than age 15 years
3- An unstable SCFE
4- A stable SCFE
5- A stable SCFE associated with morbid obesity
PREFERRED RESPONSE: 3**
**DISCUSSION: Osteonecrosis of the femoral head is the most devastating complication of SCFE. There is a 47% incidence of ischemic necrosis associated with an unstable SCFE. By definition, the patient with an unstable SCFE is unable to bear weight even with crutches. Osteonecrosis is most likely associated with the initial femoral head displacement rather than the result of either tamponade from hemarthrosis or from gentle repositioning prior to stabilization. Age, sex, and obesity are not risk factors for osteonecrosis.**
**REFERENCES: Loder RT, Richards BS, Shapiro PS, et al: Acute slipped capital femoral epiphysis: The importance of physeal stability. J Bone Joint Surg Am 1993;75:1134-1140.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 2, pp 711-745.**
**28****. An 8-year-old boy has had pain and swelling around the right knee for the past 4 weeks. He recalls bumping it about 4 weeks ago. He has no pain in other joints, and denies any fevers, chills, or other symptoms. A radiograph is shown in Figure 13. Laboratory studies show a WBC count of 9,700/mm** P3P**, an erythrocyte sedimentation rate of 18 mm/h, and a C-reactive protein level of 3.7 mg/L. What is the next most appropriate step
in management?
1- Chemotherapy and radiation therapy
2- Intravenous antibiotics, protected weight bearing, and a repeat C-reactive protein after improvement
3- Open biopsy and debridement of the site, followed by intravenous antibiotics
4- Technetium Tc 99m bone scan
5- Empiric oral antibiotics and repeat laboratory studies in 1 week
PREFERRED RESPONSE: 3**
**DISCUSSION: The history and laboratory findings are consistent with osteomyelitis of the patella. The radiograph reveals bone destruction in the patella; therefore, the next most appropriate step is open biopsy and debridement of the site. Aspiration of the knee joint may be needed to rule out septic arthritis prior to patellar debridement. With this amount of bone destruction, surgical debridement is helpful to obtain cultures and to remove necrotic material. Administering antibiotics without any prior culture increases the risk of negative cultures later and a potentially incorrect choice of antibiotic. A neoplasm should be included in the differential. It would be inappropriate to initiate chemotherapy and radiation therapy without a biopsy-confirmed diagnosis. A bone scan is likely to demonstrate uptake, but radiographs have already localized the abnormality to the patella.**
**REFERENCES: Morrisy RT: Bone and joint sepsis, in Morrissy RT, Weinstein SL (eds): Lovell and Winter’s Pediatric Orthopaedics, ed 5. Philadelphia, PA, Lippincott Williams & Wilkins, 2001, pp 466-470.**
**Dormans JP, Drummond DS: Pediatric hematogenous osteomyelitis: New trends in presentation, diagnosis, and treatment. J Am Acad Orthop Surg 1994;2:333-341.**
**29****. A 2-year-old child is being evaluated for limb-length and girth discrepancy. As a newborn, the patient was large for gestational age and had hypoglycemia. Current examination shows enlargement of the entire right side of the body, including the right lower extremity and foot. The skin shows no abnormal markings, and the neurologic examination is normal. The spine appears normal. Radiographs confirm a
2-cm discrepancy in the lengths of the lower extremities. Additional imaging studies should include
1- bone age of the left wrist.
2- MRI of the spine.
3- MRI of the brain.
4- renal and abdominal ultrasonography.
5- hip ultrasonography.
PREFERRED RESPONSE: 4**
**DISCUSSION: The patient may have Beckwith-Wiedemann syndrome (BWS), which consists of exophthalmos, macroglossia, gigantism, visceromegaly, abdominal wall defects, and neonatal hypoglycemia. Hemihypertrophy develops in approximately 15% of patients with BWS. Patients with hemihypertrophy that is the result of BWS have a 40% chance of developing malignancies such as Wilms’ tumor or hepatoblastoma; therefore, frequent ultrasound screening is recommended until about age 7 years. The absence of nevi and vascular markings helps to rule out other causes of hemihypertrophy, such as neurofibromatosis, Proteus syndrome, and Klippel-Trenaunay syndrome. Bone age estimations are not accurate at this young age but may become more useful later to help predict the timing of epiphysiodesis procedures.**
**REFERENCES: DeBaun MR, Tucker MA: Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry. J Pediatr 1998;132:398-400.**
**Ballock RT, Wiesner GL, Myers MT, et al: Hemihypertrophy concepts and controversies.
J Bone Joint Surg Am 1997;79:1731-1738.**
**Carpenter CT, Lester EL: Skeletal age determination in young children: Analysis of three regions of the hand/wrist film. J Pediatr Orthop 1993;13:76-79.**
**30****. A 12½-year-old boy reports intermittent knee pain and limping that interferes with his ability to participate in sports. He actively participates in football, basketball, and baseball. He denies any history of injury. Examination shows full range of motion without effusion. Radiographs reveal an osteochondritis dissecans (OCD) lesion on the lateral aspect of the medial femoral condyle. MRI scans are shown in Figures 14a
and 14b. Initial treatment should consist of
1- immobilization.
2- arthroscopic evaluation of fragment stability.
3- transarticular drilling of the lesion with 0.045 Kirschner wire.
4- arthroscopic excision of the fragment and microfracture of underlying
cancellous bone.
5- excision of the fragment and mosaicplasty.
PREFERRED RESPONSE: 1**
**DISCUSSION: This skeletally immature patient has a small OCD lesion that appears stable, and he has not undergone any treatment. Therefore, a trial of immobilization until pain resolves is the best initial choice. Thereafter, cessation of sport activities for 4 to 6 months may allow healing of the lesion. Surgical treatment of juvenile OCD lesions is reserved for unstable lesions, patients who have not shown radiographic evidence of healing and are still symptomatic after 6 months of nonsurgical management, or patients who are approaching skeletal maturity. Good results with stable in situ lesions that have failed to respond to nonsurgical management have been reported with both transarticular and retroarticular drilling. Results after excision alone are poor at 5-year follow-up, and it is unclear if microfracture will improve the long-term outcome. Mosaicplasty may be the next best option for patients who remain or become symptomatic after excision of the fragment and microfracture.**
**REFERENCES: Wall E, Von Stein D: Juvenile osteochondritis dissecans. Orthop Clin North Am 2003;34:341-353.**
**Kocher MS, Micheli LJ, Yaniv M, et al: Functional and radiographic outcome of juvenile osteochondritis dissecans of the knee treated with transarticular arthroscopic drilling.
Am J Sports Med 2001;29:562-566.**
**31****. A 14-year-old boy undergoes application of a circular frame with tibial and fibular osteotomy for gradual limb lengthening. He initiates lengthening 7 days after surgery. During the first week of lengthening, he reports that turning of the distraction devices is becoming increasingly difficult. On the 9th day of lengthening, he is seen in the emergency department after feeling a pop in his leg and noting the acute onset of severe pain. What complication has most likely occurred?
1- Joint subluxation and acute ligament rupture
2- Incomplete corticotomy at the time of surgery with spontaneous completion and acute distraction
3- Premature consolidation of the osteotomy with breakage of bone
transfixation wire
4- Fracture through the bone regenerate
5- Fracture of the tibia through a unicortical half-pin track
PREFERRED RESPONSE: 2**
**DISCUSSION: Incomplete corticotomy may result from osteotomy with limited soft-tissue stripping and exposure. When the patient begins distraction, tension develops at all wire/half-pin and bone interfaces, leading to increasing difficulty in distraction and limb pain. Sudden spontaneous completion of the osteotomy with continued tension applied by the fixator results in acute distraction of the osteotomy with severe pain. Premature consolidation is unlikely this early following the initial surgery.**
**REFERENCES: Birch JG, Samchukov ML: Use of the Ilizarov method to correct lower limb deformities in children and adolescents. J Am Acad Orthop Surg 2004;12:144-154.**
**Noonan KJ, Leyes M, Forriol F, et al: Distraction osteogenesis of the lower extremity with use of monolateral external fixation: A study of two hundred and sixty-one femora and tibiae. J Bone Joint Surg Am 1998;80:793-806.**
**32****. What is the most common primary malignant bone or cartilage tumor in children?
1- Ewing’s sarcoma
2- Giant cell tumor
3- Osteosarcoma
4- Chondrosarcoma
5- Osteochondroma
PREFERRED RESPONSE: 3**
**DISCUSSION: Osteosarcoma is the most common primary malignant bone tumor (5.6 per
1 million children younger than age 15 years), and Ewing’s sarcoma is second (2.1 per
1 million children). Giant cell tumor and chondrosarcoma are rare in children. Osteochondroma is more common than any of the above tumors in children, but it is not malignant.**
**REFERENCES: Himelstein BP, Dormans JP: Malignant bone tumors of childhood. Pediatr Clin North Am 1996;43:967-984.**
**Pierz KA, Womer RB, Dormans JP: Pediatric bone tumors: Osteosarcoma, Ewing’s sarcoma, and chondrosarcoma associated with multiple hereditary osteochondromatosis. J Pediatr Orthop 2001;21:412-418.**
**Arndt CA, Crist WM: Common musculoskeletal tumors of childhood and adolescence.
N Engl J Med 1999;341:342-352.**
**33****. What is the peak period of onset in children with pauciarticular juvenile
rheumatoid arthritis?
1- Before age 2 years
2- Between the ages of 2 and 4 years
3- Between the ages of 4 and 8 years
4- Between the ages of 8 and 12 years
5- During adolescence
PREFERRED RESPONSE: 2**
**DISCUSSION: Approximately one half of patients with juvenile rheumatoid arthritis (JRA) have the pauciarticular form, which by definition includes only patients with fewer than five joints involved. The peak period of onset is between the ages of 2 and 4 years, with half of the affected children coming to medical attention before age 4 years. The knee is most often affected, with the ankle-subtalar and elbow joints next in frequency. The average duration of the disease is
2 years and 9 months, with half the cases lasting less than 2 years. **
**REFERENCES: Arthritis, in Herring JA (ed): Tachdjian’s Pediatric Orthopaedics, ed 3.
St Louis, MO, WB Saunders, 2002, pp 1811-1839.**
**Griffin PP, Tachdjian MO, Green WT: Pauciarticular arthritis in children. JAMA
1963;184:23-28.**
**34****. A 10-year-old girl who is Risser stage 0 has back deformity associated with neurofibromatosis type 1 (NF1). She has no back pain. Examination shows multiple cafe-au-lait nevi with normal lower extremity neurologic function and reflexes. Standing radiographs of the spine show a short 50-degree right thoracic scoliosis with a kyphotic deformity of 55 degrees (apex T8). A 10-degree progression in scoliosis has occurred during the past 1 year. There is no cervical deformity. MRI shows mild dural ectasia, primarily in the upper lumbar region. Management should consist of
1- observation with repeat radiographs in 6 months.
2- a thoracolumbosacral orthosis (TLSO).
3- in situ posterior spinal fusion without instrumentation, followed by full-time TLSO bracing.
4- anterior spinal convex hemiepiphysiodesis.
5- combined anterior and posterior spinal arthrodesis with instrumentation.
PREFERRED RESPONSE: 5**
**DISCUSSION: Scoliotic deformities in patients with NF1 are often dysplastic with short, angular curves. Posterior arthrodesis is made more difficult by the presence of kyphosis and of weak posterior elements caused by dural ectasia. Combined anterior and posterior spinal arthrodesis is generally preferred for progressive dysplastic curves to maximize deformity correction and to decrease the risk of pseudarthrosis. Anterior fusion may also prevent crankshaft phenomenon in young children. Brace treatment is not effective for large, rigid, or dysplastic curves.**
**REFERENCES: Kim HW, Weinstein SL: Spine update: The management of scoliosis in neurofibromatosis. Spine 1997;22:2770-2776.**
**Funasaki H, Winter RB, Lonstein JB, et al: Pathophysiology of spinal deformities in neurofibromatosis: An analysis of seventy-one patients who had curves associated with dystrophic changes. J Bone Joint Surg Am 1994;76:692-700.**
**35****. In obstetrical brachial plexus palsy, which of the following signs is associated with the poorest prognosis for recovery in a 2-month-old infant?
1- Persistent inability to bring the hand to the mouth with the elbow stabilized at
the side
2- Persistent inability to actively abduct the arm past 90 degrees
3- Persistent inability to externally rotate the shoulder past 20 degrees
4- Persistent unilateral ptosis, myosis, and anhydrosis
5- History of clavicle fracture at birth
PREFERRED RESPONSE: 4**
**DISCUSSION: Persistent Horner’s sign (ptosis, myosis, and anhydrosis) is a sign of proximal injury, usually avulsion of the roots from the cord which disrupts the sympathetic chain. Root rupture or avulsion proximal to the myelin sheath has less chance of healing. Two-month-old infants with persistent weakness in the other areas described may still have a good prognosis for recovery. Concurrent clavicle fracture has been shown to have no prognostic value.**
**REFERENCES: Clarke HM, Curtis CG: An approach to obstetrical brachial plexus injuries. Hand Clin 1995;11:563-581.**
**Narakas AO: Injuries to the brachial plexus, in Bora FW (ed): The Pediatric Upper Extremity: Diagnosis and Management. Philadelphia, PA, WB Saunders, 1986, p 247.**
**36****. A 6-year-old boy with acute hematogenous osteomyelitis of the distal femur is being treated with intravenous antibiotics. The most expeditious method to determine the early success or failure of treatment is by serial evaluations of which of the following studies?
1- CBC count with differential
2- MRI
3- CT
4- Radiographs
5- C-reactive protein (CRP)
PREFERRED RESPONSE: 5**
**DISCUSSION: Successful antibiotic treatment of osteomyelitis should lead to a rapid decline in the CRP. The CRP should decline after 48 to 72 hours of appropriate treatment. Imaging studies will take much longer to show resolution of bone infection.**
**REFERENCES: Unkila-Kallio L, Kallio MJ, Eskola J, et al: Serum C-reactive protein, erythrocyte sedimentation rate, and white blood cell count in acute hematogenous osteomyelitis of children. Pediatrics 1994;93:59-62.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 3, pp 1841-1860.**
**37****. A 6-year-old girl has a painless spinal deformity. Examination reveals 2+ and equal knee jerks and ankle jerks, negative clonus, and a negative Babinski. The straight leg raising test is negative. Abdominal reflexes are asymmetrical. PA and lateral radiographs are shown in Figures 15a and 15b. What is the next most appropriate step in management?
1- MRI of the spinal axis
2- Physical therapy
3- A brace for scoliosis
4- Observation, with reevaluation in 6 to 12 months
5- Posterior spinal fusion from T6 to T12
PREFERRED RESPONSE: 1**
**DISCUSSION: The patient has an abnormal neurologic exam as shown by the abnormal abdominal reflexes. Furthermore, she has a significant curve and is younger than age 10 years. These findings are not consistent with idiopathic scoliosis. MRI will best rule out syringomyelia or an intraspinal tumor. Bracing and surgery are not indicated for this small curvature prior to obtaining an MRI scan.**
**REFERENCES: Ginsburg GM, Bassett GS: Back pain in children and adolescents: Evaluation and differential diagnosis. J Am Acad Orthop Surg 1997;5:67-78.**
**Schwend RM, Hennrikus W, Hall JE, et al: Childhood scoliosis: Clinical indications for magnetic resonance imaging. J Bone Joint Surg Am 1995;77:46-53.**
**38****. Figure 16 shows the radiograph of a 7-year-old boy who sustained a pathologic fracture of the left humerus 1 day ago. Initial management should consist of
1- a sling and swathe.
2- needle biopsy of the lesion.
3- a corticosteroid injection of the lesion.
4- curettage and bone packing of the lesion.
5- insertion of an intramedullary rod.
PREFERRED RESPONSE: 1**
**DISCUSSION: The radiograph shows a pathologic fracture through a unicameral (simple) bone cyst (UBC). This is the most common location and presentation of a UBC. Less than 10% of UBCs heal spontaneously following a fracture. Urgent biopsy is not indicated because the lesion appears benign and the histology of fracture callus may be misinterpreted as osteosarcoma. After the fracture heals with the use of a sling and swathe, the UBC may be treated with a minimally invasive procedure such as injection of bone marrow and/or demineralized bone matrix. The chance for success is relatively low in an active cyst located adjacent to the physis. More invasive procedures, such as curettage, Rush rod fixation, or cannulated screw decompression, have been described but are rarely necessary for treatment of upper
extremity cysts. **
**REFERENCES: Rougraff BT, Kling TJ: Treatment of active unicameral bone cysts with percutaneous injection of demineralized bone matrix and autogenous bone marrow. J Bone Joint Surg Am 2002;84:921-929.**
**Robosch A, Saraph V, Linhart WE: Flexible intramedullary nailing for the treatment of unicameral bone cysts in long bones. J Bone Joint Surg Am 2000;82:1447-1453.**
**Wilkins RM: Unicameral bone cysts. J Am Acad Orthop Surg 2000;8:217-224.**
**39****. Figure 17 shows the AP radiograph of a 5-year old child who has mild short stature and a painless bilateral gluteus medius lurch. Initial work-up should include
1- a bone scan.
2- a skeletal survey.
3- MRI of the hips.
4- CT of the hips.
5- a CBC count and a C-reactive protein.
PREFERRED RESPONSE: 2**
**DISCUSSION: Bilateral flattening of the femoral heads suggests multiple epiphyseal dysplasia; therefore, a skeletal survey is indicated to look for involvement of other epiphyses. Unilateral flattening of the femoral head would suggest Legg-Perthes disease.**
**REFERENCES: Sponseller PD: Skeletal dysplasias, in Morrissy RT, Weinstein SL (eds): Lovell and Winter’s Pediatric Orthopaedics, ed 5. Philadelphia, PA, Lippincott Williams & Wilkins, 2001, pp 269-270.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 1, pp 689-691.**
**40****. A 7-year-old girl with spinal muscular atrophy (SMA) type II has popping of the left hip. Examination reveals painless subluxation of the joint in adduction with palpable reduction in abduction. Radiographs show coxa valga, subluxation of the left hip, and pelvic obliquity with elevation of the left hemipelvis. Treatment should consist of
1- observation.
2- bilateral adductor and iliopsoas releases, with nighttime abduction bracing.
3- proximal femoral varus osteotomy with internal fixation.
4- proximal femoral varus osteotomy with volume-reducing periacetabular osteotomy.
5- proximal femoral varus osteotomy with shelf acetabular augmentation.
PREFERRED RESPONSE: 1**
**DISCUSSION: Observation is the treatment of choice. Hip subluxation and dislocation are not uncommon in patients with SMA type II who are unlikely to be ambulatory. Scoliosis occurs in these patients 100% of the time and frequently creates pelvic obliquity. However, in long-term follow-up, patients with SMA type II and hip dislocations had little associated pain or functional limitations because of hip instability. In addition, recurrent hip subluxation after surgical treatment has been documented. Given the rarity of symptoms from hip instability in long-term follow-up, and the possibility of recurrent dislocation, surgical intervention for hip instability may expose SMA type II patients to undue surgical risk for minimal if any functional gain.**
**REFERENCES: Sporer SM, Smith BG: Hip dislocation in patients with spinal muscular atrophy. J Pediatr Orthop 2003;23:10-14.**
**Thompson CE, Larsen LJ: Recurrent hip dislocation in intermediate spinal atrophy.
J Pediatr Orthop 1990;10:638-641.**
**41****. A newborn with myelomeningocele has no movement below the waist and has bilateral hips that dislocate with provocative flexion and adduction. What is the best treatment option for the hip instability?
1- A Pavlik harness with the hips in 90 degrees of flexion and 60 degrees
of abduction
2- A spica cast with the hips in 100 degrees of flexion and 70 degrees of abduction
3- Observation with range-of-motion exercises to minimize contractures
4- Open reduction through an anterior hip approach
5- Open reduction through a medial hip approach
PREFERRED RESPONSE: 3**
**DISCUSSION: The status of the hips (located or dislocated) in children with thoracic-level myelomeningocele has no effect on the functional outcome of these patients. Management of unstable hips in this population should be limited to treatment of the contractures that may lead to poor limb positioning in either braces or a wheelchair. The use of the Pavlik harness and/or spica cast is contraindicated because they would promote flexion and abduction contractures. In the past, open reduction either through an anterior or medial approach had been performed with a high incidence of redislocation and other complications, with little functional gain for the child.**
**REFERENCES: Gabriel KG: Natural history of hip deformity in spina bifida, in Sarwark JR, Lubicky JP (eds): Caring for the Child With Spina Bifida. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2001, pp 89-103.**
**Schoenecker PL: Surgical management of hip problems in children with myelomeningocele, in Sarwark KR, Lubicky JP (eds): Caring for the Child With Spina Bifida. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2001, pp 117-131.**
**42****. A 14-year-old boy reports a 4-month history of increasing backache with difficulty walking long distances. His parents state that he walks with his knees slightly flexed and is unable to bend forward and get his hands to his knees. He denies numbness, tingling, and weakness in his legs and denies loss of bladder and bowel control. A lateral radiograph of the lumbosacral spine is shown in Figure 18. What is the best surgical management for this condition?
1- Vertebrectomy of L5
2- Posterior spinal fusion with or without instrumentation from L4 to S1
3- Posterior spinal fusion without instrumentation from L5 to S1
4- Anterior spinal fusion from L4 to L5
5- Direct repair of the spondylolysis defect
PREFERRED RESPONSE: 2**
**DISCUSSION: The patient has a grade 4 spondylolisthesis. Optimal surgical management is posterior spinal fusion from L4 to the sacrum. The use of instrumentation is controversial. Vertebrectomy is typically reserved for spondylo-optosis (grade 5) cases. Spinal fusion from
L5 to S1 usually is not successful for a slip that is greater than 50%. Isolated anterior spinal fusion has not been successful, and direct repair of the pars defect is only useful for spondylolysis without spondylolisthesis. **
**REFERENCES: Lenke LG, Bridwell KH: Evaluation and surgical treatment of high-grade isthmic dysplastic spondylolisthesis. Instr Course Lect 2003;52:525-532.**
**Ginsburg GM, Bassett GS: Back pain in children and adolescents: Evaluation and differential diagnosis. J Am Acad Orthop Surg 1997:5:67-78.**
**43****. Duchenne’s muscular dystrophy is a genetic disorder that is transmitted by which of the following modes of inheritance?
1- X-linked
2- Autosomal-dominant
3- Autosomal-recessive
4- Chromosomal duplication
5- Chromosomal deletion
PREFERRED RESPONSE: 1**
**DISCUSSION: Patients with Duchenne’s muscular dystrophy show progressive muscular weakness because of the absence of dystrophin and have the clinical picture of progressive muscle weakness. The condition is an X-linked genetic disease.**
**REFERENCES: Fitzgerald RH, Kaufer H, Malkani AL: Orthopaedics. St Louis, MO, Mosby Year Book, 2002, pp 1573-1583.**
**Smith SA, Swaiman HF: Muscular dystrophies, in Swaiman KF, Ashwall S (eds): Pediatric Neurology Principles and Practice, ed 3. St Louis, MO, Mosby, 1999, pp 1235-1237.**
**44****. A 4-month-old infant is referred for evaluation of congenital scoliosis. The child has no congenital heart anomalies, and a renal ultrasound shows that he has one kidney. Examination reveals mild scoliosis and a large hairy patch on the child’s back. Neurologic evaluation is normal for his age. A clinical photograph and radiograph are shown in Figures 19a and 19b. Initial management should consist of
1- referral to a plastic surgeon to remove the hairy patch.
2- MRI of the entire spine.
3- physical therapy and repeat evaluation and radiographs in 1 year.
4- anterior and posterior fusion of the anomalous regions of the spine to
prevent deformity.
5- voiding cystourethrography.
PREFERRED RESPONSE: 2**
**DISCUSSION: Congenital anomalies of the spine, including failure of formation and failure of segmentation, are associated with other anomalies in other organ systems that develop at the same time. These include anomalies in the genitourinary system, cardiac anomalies, Sprengel’s deformity, radial hypoplasia, and gastrointestinal anomalies including imperforate anus and trachealesophageal fistula. Spinal dysraphism is the most common associated abnormality. McMaster found an 18% incidence before the common use of MRI. Bradford and associates reported on 16 of 42 patients with congenital spinal anomalies and spinal dysraphism using MRI. Neural axis lesions may be associated with visible midline abnormalities such as a hairy patch or nevus. The child has already had a cardiac and renal work-up, and based on the findings of the hairy patch and congenital vertebral anomalies, MRI of the entire spine is prudent at this time. Spinal fusion is indicated for progressive congenital scoliosis or kyphosis. Physical therapy does not affect the natural history of congenital scoliosis.**
**REFERENCES: McMaster MJ: Occult intraspinal anomalies and congenital scoliosis. J Bone Joint Surg Am 1984;66:588-601.**
**Bradford DS, Heithoff KB, Cohen M: Intraspinal abnormalities and congenital spine deformities: A radiographic and MRI study. J Pediatr Orthop 1991;11:36-41.**
**McMaster MJ, David CV: Hemivertebrae as a cause of scoliosis: A study of 104 patients.
J Bone Joint Surg Br 1986;68:588-595. **
**45****. A 12-year-old boy reports limping and chronic knee pain that is now inhibiting his ability to participate in sports. Clinical examination and radiographs of the knee are normal. Additional evaluation should include
1- mechanical alignment radiographs.
2- stress radiographs of the knee.
3- comparison radiographs of both knees.
4- an erythrocyte sedimentation rate and a C-reactive protein.
5- examination of the hip.
PREFERRED RESPONSE: 5**
**DISCUSSION: While all of the answers may be appropriate, radiating pain from hip pathology must be excluded. At this age, a slipped capital femoral epiphysis is likely. Therefore, the hip must be examined.**
**REFERENCES: Kocher MS, Bishop JA, Weed B, et al: Delay in diagnosis of slipped capital femoral epiphysis. Pediatrics 2004;113:322-325.**
**Matava MJ, Patton CM, Luhmann S, et al: Knee pain as the initial symptom of slipped capital femoral epiphysis: An analysis of initial presentation and treatment. J Pediatr Orthop 1999;19:455-460.**
**46****. In children with moderate to severe osteogenesis imperfecta (OI), intravenous pamidronate therapy has been shown to increase the thickness of cortical bone. This occurs primarily as a consequence of
1- increased bone turnover in the cortical area.
2- inhibition of osteoclast-mediated bone resorption.
3- improved mineralization of cortical bone.
4- improved osteoblast organic matrix production.
5- improved organization of collagen matrix.
PREFERRED RESPONSE: 2**
**DISCUSSION: Histologic studies have shown that increased bone turnover is the rule in OI. Pamidronate (and all bisphosphonates) reduce osteoclast-mediated bone resorption. Osteoblastic new bone formation on the periosteal surface of long bones is minimally impaired. With inhibition of osteoclastic bone resorption on the endosteal surface, the cortex of the bone can begin to thicken as it does with normal growth in individuals unaffected by OI. Mineralization and collagen matrix organization are not directly affected by pamidronate.**
**REFERENCES: Zeitlin L, Fassier F, Glorieux FH: Modern approach to children with osteogenesis imperfecta. J Pediatr Orthop B 2003;12:77-87.**
**Falk MJ, Heeger S, Lynch KA, et al: Intravenous bisphosphonate therapy in children with osteogenesis imperfecta. Pediatrics 2003;111:573-578.**
**Glorieux FH, Bishop NJ, Plotkin H, et al: Cyclic administration of pamidronate in children with severe osteogenesis imperfecta. N Engl J Med 1998;339:947-952.**
**47****. Split posterior tibial tendon transfer is used in the treatment of children with cerebral palsy. Which of the following patients is considered the most appropriate candidate for this procedure?
1- A 6-year-old child with athetosis and a flexible equinovarus deformity of the foot
2- A 6-year-old child with spastic hemiplegia and a rigid equinovarus deformity of the foot
3- A 6-year-old child with spastic hemiplegia and a flexible equinovarus deformity of the foot
4- A 10-year-old child with spastic quadriplegia and rigid valgus deformities of
the feet
5- A 15-year-old child with spastic diplegia and rigid equinovalgus deformities of the feet
PREFERRED RESPONSE: 3**
**DISCUSSION: Split posterior tibial tendon transfers are best performed in patients with spastic cerebral palsy who are between the ages of 4 and 7 years and have flexible equinovarus deformities. Rigid deformities typically require bony reconstruction procedures. Tendon transfers in patients with athetosis are unpredictable.**
**REFERENCES: Green NE, Griffin PP, Shiavi R: Split posterior tibial-tendon transfer in spastic cerebral palsy. J Bone Joint Surg Am 1983;65:748-754.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 2, pp 1142-1152.**
**48****. Late surgical treatment of posttraumatic cubitus varus (gunstock deformity) is usually necessitated by the patient reporting problems related to
1- tardy ulnar nerve palsy.
2- posterior glenohumeral subluxation.
3- posterolateral rotatory subluxation of the elbow.
4- poor appearance.
5- snapping medial triceps.
PREFERRED RESPONSE: 4**
**DISCUSSION: Cubitus varus, elbow hyperextension, and internal rotation are all typical components of the gunstock deformity. This deformity results from malunion of a supracondylar fracture of the humerus. All of the problems listed above have been reported as sequelae of a gunstock deformity, although the malunion usually causes no functional limitations. Unacceptable appearance is the most common reason why patients or parents request
corrective osteotomy. **
**REFERENCES: O’Driscoll SW, Spinner RJ, McKee MD, et al: Tardy posterolateral rotatory instability of the elbow due to cubitus varus. J Bone Joint Surg Am 2001;83:1358-1369.**
**Gurkan I, Bayrakci K, Tasbas B, et al: Posterior instability of the shoulder after supracondylar fractures recovered with cubitus varus deformity. J Pediatr Orthop 2002;22:198-202.**
**Spinner RJ, O’Driscoll SW, Davids JR, et al: Cubitus varus associated with dislocation of both the medial portion of the triceps and the ulnar nerve. J Hand Surg 1999;24:718-726.**
**49****. An 11-year-old boy sustained an ankle injury while playing football. Figure 20 shows an AP radiograph obtained the day of injury. Treatment should consist of
1- closed manipulation and a long leg cast.
2- closed manipulation and a short leg walking cast.
3- a long leg cast and long-term follow-up to rule out growth arrest.
4- open reduction and internal fixation with a transphyseal lag screw and
a non-weight-bearing cast.
5- open reduction and internal fixation with fixation parallel to the physis and
a non-weight-bearing cast.
PREFERRED RESPONSE: 5**
**DISCUSSION: The child has an injury involving both the growth plate and the articular surface of the ankle. Because of the significant displacement, open reduction and internal fixation is indicated to realign the physis and joint surface. The best method of fixation to avoid growth arrest is one that does not cross the physis. This is usually achieved by a transverse epiphyseal screw parallel to the physis. If the metaphyseal fragment was large enough, a transverse metaphyseal screw could be used instead. The incidence of growth arrest following physeal ankle injuries is as high as 50%, and long-term follow-up is indicated.**
**REFERENCES: Cass JR, Peterson HA: Salter-Harris Type-IV injuries of the distal tibial epiphyseal growth plate, with emphasis on those involving the medial malleolus. J Bone Joint Surg Am 1983;65:1059-1070.**
**Barmada A, Gaynor T, Mubarak SJ: Premature physeal closure following distal tibia physeal fractures: A new radiographic predictor. J Pediatr Orthop 2003;23:733-739.**
**50****. A 3-year-old child has bilateral genu varum and short stature. Radiographs show physeal widening and generalized osteopenia. The femora and tibiae show anterolateral bowing. Laboratory studies show low normal serum calcium values, significantly decreased serum phosphate levels, and normal parathyroid hormone (PTH), alkaline phosphatase, and vitamin-D levels. These findings are consistent with
1- nutritional rickets.
2- renal osteodystrophy.
3- primary hyperparathyroidism.
4- hypophosphatasia.
5- vitamin D-resistant rickets.
PREFERRED RESPONSE: 5**
**DISCUSSION: Children with vitamin D-resistant rickets are short in stature and have genu varum, physeal widening, and generalized osteopenia. The abnormality in inherited vitamin
D-resistant rickets is the renal tubule’s inability to resorb phosphate leading to hypophosphatemia. Laboratory findings in the condition are normal or near normal serum calcium values, significantly decreased serum phosphate levels, elevated alkaline phosphatase levels, and normal PTH and vitamin-D levels. The most common form is inherited as an
X-linked dominant trait. Nutritional rickets has a normal or low serum phosphate levels, normal or low serum calcium values, and decreased levels of 25(OH) vitamin D and
1,25-dihydroxyvitamin D. Hypophosphatasia is a rare condition characterized by a deficiency of alkaline phosphatase in the serum and tissues, leading to generalized abnormal mineralization of bone. Primary hyperparathyroidism usually is caused by a parathyroid adenoma, and the child generally has abdominal problems and hypercalcemic crisis. Laboratory findings include elevated serum calcium values, alkaline phosphatase levels, and PTH levels, and decreased serum phosphate levels. Children with renal osteodystrophy tend to have genu valgum, and laboratory findings include elevated serum phosphate, alkaline phosphatase, and PTH levels, and low serum calcium values. Findings of renal disease include elevated BUN and creatinine.**
**REFERENCES: Herring JA: Metabolic and endocrine bone diseases, in Herring JA (ed): Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, pp 1686-1710.**
**Zaleske DJ: Metabolic and endocrine abnormalities, in Morrissy RT, Weinstein SL (eds): Lovell and Winter’s Pediatric Orthopaedics, ed 5. Philadelphia, PA, Lippincott Williams & Wilkins, 2001, pp 181-203.**
**51****. A 14-year-old girl has a painful hallux valgus deformity that has not responded to shoe modifications. Figure 21 shows a standing AP radiograph. What is the most appropriate surgical procedure?
1- Distal soft-tissue realignment
2- Distal first metatarsal osteotomy with distal soft-tissue realignment
3- Proximal first metatarsal osteotomy with distal soft-tissue realignment
4- Proximal and distal first metatarsal osteotomy
5- Osteotomy of the proximal phalanx
PREFERRED RESPONSE: 4**
**DISCUSSION: The radiograph reveals an increased first-second intermetatarsal angle
and a congruent metatarsophalangeal joint with an abnormal distal metatarsal articular
angle. Correction of both of these abnormalities requires a proximal and distal first
metatarsal osteotomy. **
**REFERENCES: Coughlin M: Juvenile bunions, in Coughlin MJ, Mann RA (eds): Surgery of the Foot and Ankle, ed 6. St Louis, MO, Mosby, 1993, pp 297-339.**
**Peterson HA, Newman SR: Adolescent bunion treated with double osteotomy and longitudinal pin fixation of the first ray. J Pediatr Orthop 1993;13:80-84.**
**52****. Figure 22 shows the radiograph of a 7-year-old boy who underwent retrograde elastic nailing of a femoral shaft fracture. What is the most common problem following
this procedure?
1- Unacceptable shortening
2- Osteonecrosis of the femoral head
3- Malunion
4- Persistent pain and irritation at the nail insertion site
5- Rotational malalignment
PREFERRED RESPONSE: 4**
**DISCUSSION: Several large clinical studies have shown that the most common problem after elastic nailing of a femoral shaft fracture is persistent pain and irritation at the nail insertion site. Unacceptable shortening and malunion are very rare in a 7-year-old patient. Rotational malalignment also is unusual. Osteonecrosis has been reported in solid antegrade nailing but not with elastic nailing of femoral shaft fractures in skeletally immature patients.**
**REFERENCES: Flynn JM, Luedtke LM, Ganley TJ, et al: Comparison of titanium elastic nails with traction and a spica cast to treat femoral fractures in children. J Bone Joint Surg Am 2004;86:770-777.**
**Flynn JM, Hresko T, Reynolds RA, et al: Titanium elastic nails for pediatric femur fractures: A multicenter study of early results with analysis of complications. J Pediatr Orthop 2001;21:4-8.**
**Ligier JN, Metaizeau JP, Prevot J, et al: Elastic stable intramedullary nailing of femoral shaft fractures in children. J Bone Joint Surg Br 1988;70:74-77.**
**53****. A newborn girl with an isolated unilateral dislocatable hip is placed in a Pavlik harness with the hips flexed 100 degrees and at resting abduction. Figure 23 shows an ultrasound obtained 2 weeks later. What is the next step in management?
1- Reposition the harness to hold the hips in 70 degrees of abduction
2- Closed reduction and arthrography under anesthesia
3- Open reduction and a spica cast
4- Continued harness treatment in the current position
5- Spica cast
PREFERRED RESPONSE: 4**
**DISCUSSION: The infant has a well-positioned hip in the Pavlik harness and treatment should be continued in the current position. The success rate is over 90% with the use of this device for a dislocatable hip. Ultrasound is a useful tool to confirm appropriate positioning of the cartilaginous femoral head during treatment. If the femoral head is not reduced after 2 to
3 weeks in the harness, this mode of treatment should be abandoned. Forceful extreme abduction can cause osteonecrosis of the femoral epiphysis and should be avoided. Closed reduction, arthrography, and spica casting are indicated if the hip cannot be maintained in a reduced position with the harness.**
**REFERENCES: Lehmann HP, Hinton R, Morello P, et al: Developmental dysplasia of the hip practice guideline: Technical report. Committee on Quality Improvement, and Subcommittee on Developmental Dysplasia of the Hip. Pediatrics 2000;105:E57.**
**Haynes RJ: Developmental dysplasia of the hip: Etiology, pathogenesis, and examination and physical findings in the newborn. Instr Course Lect 2001;50:535-540.**
**54****. During the early swing phase of the normal gait cycle, what lower extremity muscle is primarily contracting?
1- Tibialis posterior
2- Tibialis anterior
3- Vastus medialis
4- Adductor longus
5- Gastrocnemius
PREFERRED RESPONSE: 2**
**DISCUSSION: Electromyography during walking reveals the tibialis anterior muscle is active during early swing, allowing the foot to clear the ground. All of the other muscles are quiet, as the limb moves forward through space with minimal muscular effort. The other muscles are primarily active during weight acceptance or push-off.**
**REFERENCES: Gage JR: An overview of normal walking. Instr Course Lect 1990;39:291-303.**
**Wootten ME, Kadaba MP, Cochran GV: Dynamic electromyography II: Normal patterns during gait. J Orthop Res 1990;8:259-265.**
**55****. A 6-month-old child is seen in the emergency department with a spiral fracture of the tibia. The parents are vague about the etiology of the injury. There is no family
history of a bone disease. In addition to casting of the fracture, initial management should include
1- a skeletal survey to rule out other fractures.
2- a punch biopsy of the skin for collagen analysis to rule out
osteogenesis imperfecta.
3- DNA testing for osteogenesis imperfecta.
4- blood studies for calcium, phosphorus, and alkaline phosphate levels.
5- blood studies for parathyroid hormone levels.
PREFERRED RESPONSE: 1**
**DISCUSSION: Unwitnessed spiral fractures should raise the possibility of child abuse, especially prior to walking age. With nonaccidental trauma being considered in the differential diagnosis, a skeletal survey is indicated to determine if there are other fractures in various stages of healing.**
**REFERENCES: Kempe CH, Silverman FN, Steele BF, et al: The battered-child syndrome. JAMA 1962;181:17-24.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 3, pp 2079-2082.**
**56****. What is the primary indication for performing an arthroscopic synovectomy on a patient with hemophilia that is the result of factor VIII deficiency?
1- Joint pain with radiographic evidence of joint space narrowing
2- Joint stiffness that has not improved with physiotherapy and bracing
3- Recurrent joint bleeding despite optimal medical management
4- Prophylaxis for joint preservation in severe hemophilia (factor VIII level <1%)
5- Decreasing ambulatory endurance despite optimal medical management
PREFERRED RESPONSE: 3**
**DISCUSSION: Improved medical management has changed musculoskeletal outcomes for individuals with hemophilia. Patients with severe hemophilia receiving prophylactic administration of factor VIII may never develop a target joint that requires further orthopaedic intervention. Patients with moderate hemophilia and those patients with severe hemophilia not receiving prophylactic treatment will still develop joints that have recurrent hemarthroses. When recurrent hemarthrosis continues despite optimal medical management, synovectomy is indicated. While synovectomy is predictable in its ability to decrease joint bleeding, it does not necessarily improve joint range of motion or prevent the development of hemophilic arthropathy over time. It will not reverse articular damage to the joint once it has developed.**
**REFERENCES: Dunn AL, Busch MT, Wyly JB; et al: Arthroscopic synovectomy for hemophilic joint disease in a pediatric population. J Pediatr Orthop 2004;24:414-426.**
**Journeycake JM, Miller KL, Anderson AM, et al: Arthroscopic synovectomy in children and adolescents with hemophilia. J Pediatr Hematol Oncol 2003;25:726-731.**
**57****. The rate of complications after in situ pinning of a chronic slipped capital femoral epiphysis is highest with placement of the screw in what quadrant of the femoral head?
1- Anterior superior
2- Anterior inferior
3- Central
4- Posterior superior
5- Posterior inferior
PREFERRED RESPONSE: 1**
**DISCUSSION: The rate of complications increases as the pin moves farther from the ideal position, which is the center of the head. This is the strongest argument for the use of a single pin. The highest rate of complications, primarily osteonecrosis and pin penetration, is associated with pin placement in the anterior superior quadrant.**
**REFERENCES: Raney EM, Ogden JA: Slipped capital femoral epiphysis. Current Ortho 1995;9:111-116.**
**Stambough JL, Davidson RS, Ellis RD, et al: Slipped capital femoral epiphysis: An analysis of 80 patients as to pin placement and number. J Pediatr Orthop 1986;6:265-273**
**58****. What is the incidence and significance of anterior cruciate ligament laxity following tibial eminence fractures in skeletally immature individuals?
1- Common and frequently symptomatic
2- Common and infrequently symptomatic
3- Common but generally resolves spontaneously
4- Rare but when present, usually symptomatic
5- Rare and if present, infrequently symptomatic
PREFERRED RESPONSE: 2**
**DISCUSSION: Measurable anterior cruciate ligament laxity, while frequently seen after tibial eminence fractures, usually does not cause symptoms. It is found even in patients whose fractures have been anatomically reduced and fixed, leading to speculation that it is due to stretching of the ligament at the time of injury.**
**REFERENCES: Willis R, Blokker C, Stall TM, et al: Long-term follow-up of anterior
eminence fractures. J Pediatr Orthop 1993;13:361-364.**
**Smith JB: Knee instability after fracture of the intercondylar eminence of the tibia.
J Pediatr Orthop 1984;4:462-464.**
**59****. A full-term newborn has webbing at the knees, rigid clubfeet, a Buddha-like posture of the lower extremities, and no voluntary or involuntary muscle action at and below the knees. Radiographs of the spine and pelvis reveal an absence of the lumbar spine and sacrum. What maternal condition is associated with this diagnosis?
1- Alcoholism
2- Drug abuse
3- Down syndrome
4- Diabetes mellitus
5- Idiopathic scoliosis
PREFERRED RESPONSE: 4**
**DISCUSSION: The history, physical examination, and radiographic findings are consistent with type IV sacral agenesis or caudal regression syndrome. These children are born with no lumbar spine or sacrum. The T12 vertebra is often prominent posteriorly. Popliteal webbing and knee flexion contractures are common with this diagnosis. There is a higher incidence of this diagnosis when the mother has diabetes mellitus. Maternal drug abuse and alcoholism can produce phenotypically unique children but without the findings described here. Maternal idiopathic scoliosis is not associated with caudal regression syndrome.**
**REFERENCES: Chan BW, Chan KS, Koide T, et al: Maternal diabetes increases the risk of caudal regression caused by retinoic acid. Diabetes 2002;51:2811-2816.**
**Zaw W, Stone DG: Caudal regression syndrome in twin pregnancy with type II diabetes.
J Perinatol 2002;22:171-174. **
**60****. Figure 24 shows the sitting AP and lateral spinal radiographs of a nonambulatory
12½-year-old boy with Duchenne muscular dystrophy who is being evaluated for scoliosis. The lumbar curve from T12 to L5 measures 36 degrees, and the thoracic curve from T3 to T12 measures 24 degrees on the AP radiograph. He has 5 degrees of pelvic obliquity. His forced vital capacity is 45% of predicted for height and weight. What is the most appropriate treatment for the spinal deformity?
1- Posterior spinal fusion from T2 to L5 with segmental instrumentation
2- Anterior spinal fusion from L1 to L4, followed by posterior spinal fusion from
T2 to the sacrum with segmental instrumentation including iliac fixation
3- Custom-molded spinal orthosis worn 23 hours per day until skeletal maturity
4- A spinal orthosis until age 14 years, followed by posterior spinal fusion with segmental instrumentation
5- Adapted wheelchair seating with a custom-molded back support to correct scoliosis and kyphosis
PREFERRED RESPONSE: 1**
**DISCUSSION: Posterior spinal fusion is the treatment of choice for scoliosis in patients with Duchenne muscular dystrophy once they are no longer able to walk. This treatment improves quality of life and upright wheelchair positioning. Its effect on pulmonary function is less clear, as pulmonary function will continue to decline because of the underlying muscle disease. While bracing and wheelchair modifications may slow the progression of the curve, progression will continue. Surgical intervention at this stage does not have to include the pelvis, which, in general, is indicated in curves of greater than 40 degrees, and when pelvic obliquity is greater than 10 degrees. Fixation to the pelvis should also be considered in lumbar curves where the apex is lower than L1. Surgical treatment usually can be safely performed if the vital capacity is greater than 35%.**
**REFERENCES: Hahn GV, Mubarak SJ: Muscular dystrophy, in Weinstein SL (ed): The Pediatric Spine, ed 2. Philadelphia, PA, Lippincott Williams & Wilkins, 2001, pp 819-832.**
**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.**
**61****. A 3-year-old child has refused to walk for the past 2 days. Examination in the emergency department reveals a temperature of 102.2 degrees F (39 degrees C) and limited range of motion of the left hip. An AP pelvic radiograph is normal. Laboratory studies show a WBC count of 9,000/mm** P3P**, an erythrocyte sedimentation rate (ESR) of 65 mm/h, and a
C-reactive protein level of 10.5 mg/L (normal < 0.4). What is the next most appropriate step in management?
1- Technetium Tc 99m bone scan
2- Intravenous antibiotics
3- Oral antibiotics
4- CT of the hips
5- Aspiration of the left hip
PREFERRED RESPONSE: 5**
**DISCUSSION: Examination reveals an irritable hip, creating a differential diagnosis of transient synovitis versus pyogenic hip arthritis. Kocher and associates described four criteria to help predict the presence of infection: inability to bear weight, fever, ESR of more than 40 mm/h, and a peripheral WBC count of more than 12,000/mm** P3P**. This patient meets three of the four criteria, with a positive predictive value of 73% to 93% for joint infection. Therefore, aspiration of the hip is warranted, with a high likelihood that emergent hip arthrotomy will be indicated. Ideally, intravenous antibiotics should be administered after culture material has been obtained from needle aspiration of the hip. An urgent bone scan is better indicated as a screening test for sacroiliitis or diskitis. If the arthrocentesis proves negative, CT or MRI of the pelvis may be indicated to rule out a pelvic or psoas abscess.**
**REFERENCES: Del Beccaro MA, Champoux AN, Bockers T, et al: Septic arthritis versus transient synovitis of the hip: The value of screening laboratory tests. Ann Emerg Med 1992;21:1418-1422.**
**Kocher MS, Mandiga R, Zurakowski D, et al: Validation of a clinical prediction rule for the differentiation between septic arthritis and transient synovitis of the hip in children. J Bone Joint Surg Am 2004;86:1629-1635.**
**Kocher MS, Zurakowski D, Kasser JR: Differentiating between septic arthritis and transient synovitis of the hip in children: An evidence-based clinical prediction algorithm. J Bone Joint Surg Am 1999;81:1662-1670.**
**62****. A 2-year-old girl has had a swollen right knee for the past 7 weeks. There is no history of significant trauma, and she has not had a fever or been ill. Her parents report that she is stiff in the morning but otherwise does not report pain. A CBC count and erythrocyte sedimentation rate are normal. Treatment with naproxen at appropriate doses for the past 2 weeks has resulted in some improvement. Radiographs show only soft-tissue swelling. Examination reveals a healthy-appearing child with a warm and swollen right knee that is only slightly tender but lacks full extension by 20 degrees. What is the next most appropriate step in management?
1- MRI
2- Arthrocentesis for synovial fluid cell count and bacterial culture
3- Ophthalmology consultation
4- Angiotensin converting enzyme (ACE) level
5- Technetium radioisotope bone scan
PREFERRED RESPONSE: 3**
**DISCUSSION: Up to 30% of children with juvenile rheumatoid arthritis (increasingly known now as juvenile idiopathic arthritis or JIA) already have potentially damaging uveitis at the time of diagnosis. This patient has typical oligoarticular JRA (JIA) and therefore is at significant risk for uveitis. MRI, radioisotope scanning, or an ACE level most likely would not provide additional useful diagnostic information because intra-articular derangement, osteomyelitis, or sarcoidosis are all unlikely. Arthrocentesis and triamcinolone hexacetonide joint injection might be indicated if continued use of nonsteroidal medication does not result in improvement, but should be held off for at least an additional 4 to 6 weeks to see if continued use of naproxen results in control of the arthritis.**
**REFERENCES: Wolf MD, Lichter PR, Ragsdale CG: Prognostic factors in the uveitis of juvenile rheumatoid arthritis. Ophthalmology 1987;94:1242.**
**Cassidy JT, Petty RE: Textbook of Pediatric Rheumatology. Philadelphia, PA, WB Saunders, 2001, p 220.**
**Chalom ED, Goldsmith DP, Koehler MA, et al: Prevalence and outcome of uveitis in a regional cohort of patients with juvenile rheumatoid arthritis. J Rheumatol 1997;24:2031-2034.**
**63****. A 12-year-old girl has back pain after falling 20 feet and landing in the sitting position. She has no fractures or other injuries, and her neurologic examination is normal. A lateral radiograph, transverse CT scan, and reformatted sagittal CT scan are shown in Figures 25a through 25c. Which of the following methods is associated with the best long-term outcome?
1- Hyperextension casting of the thoracolumbar spine for 6 weeks
2- In situ posterior fusion with instrumentation
3- Posterior fusion with instrumentation, with sagittal plane correction
4- Posterior decompression, followed by posterior fusion with instrumentation, with sagittal plane correction
5- Anterior decompression and partial corpectomy, with anterior instrumentation
PREFERRED RESPONSE: 3**
**DISCUSSION: The patient has a displaced burst fracture. Fusion with instrumentation has shown better results than casting alone. Posterior fusion with instrumentation, with sagittal plane correction, yields the best results. Decompression occurs indirectly with correction of the kyphosis. Anterior decompression is unnecessary.**
**REFERENCES: Lalonde F, Letts M, Yang JP, et al: An analysis of burst fractures of the spine in adolescents. Am J Orthop 2001;30:115-120.**
**Clark P, Letts M: Trauma to the thoracic and lumbar spine in the adolescent. Can J Surg 2001;44:337-345.**
**Been HD, Bouma GJ: Comparison of two types of surgery for thoraco-lumbar burst fractures: Combined anterior and posterior stabilization vs posterior instrumentation only. Acta Neurochir (Wien) 1999;141:349-357.**
**64****. Figure 26 shows the radiograph of an otherwise healthy Caucasian 5-year-old boy who has a painless limp. What is the best treatment option?
1- Shelf procedure
2- Salter osteotomy
3- Chiari osteotomy
4- Varus derotation osteotomy
5- Physical therapy and range-of-motion exercises
PREFERRED RESPONSE: 5**
**DISCUSSION: The prognosis of Legg-Perthes disease in children younger than age 6 years is good. There is no indication that surgical treatment will improve the outcome. Range-of-motion exercises to prevent contracture may be helpful.**
**REFERENCES: Herring JA, Kim HT, Browne R: Legg-Calve-Perthes disease: Prospective multicenter study of the effect of treatment on outcome. J Bone Joint Surg Am 2004;86:2121-2134.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 1, pp 691-704.**
**65****. A 12-year-old girl who has a history of frequent tripping and falling also has bilateral symmetric hand weakness, high arched feet, absent patellar and Achilles tendon reflexes, and excessive wear on the lateral border of her shoes. She reports that she has multiple paternal family members with similar deformities. She most likely has a defect of
what protein?
1- Peripheral myelin protein-22
2- Dystrophin
3- Type I collagen
4- Alpha-L-iduronidase
5- Cartilage oligomeric matrix protein
PREFERRED RESPONSE: 1**
**DISCUSSION: The girl shows clinical features of hereditary motor sensory neuropathy type 1, Charcot-Marie-Tooth disease. The most common type of this autosomal-dominant disease is due to an underlying defect in the gene coding for peripheral myelin protein-22 on chromosome 17. Many other less common mutations have been identified in this family of neuropathies. Dystrophin is a protein that is abnormal in Duchenne’s muscular dystrophy, which affects males and is diagnosed earlier. Type I collagen is defective in osteogenesis imperfecta.
Alpha-L-iduronidase is defective in mucopolysaccharidosis type I, Hurler’s syndrome. Defective cartilage oligomeric matrix protein is associated with some forms of multiple epiphyseal dysplasia.**
**REFERENCES: Patel PI, Roa BB, Welcher AA, et al: The gene for the peripheral myelin protein PMP-22 is a candidate for Charcot-Marie-Tooth disease type 1A. Nat Genet 1992;1:159-165.**
**Harding AE: From the syndrome of Charcot, Marie and Tooth to disorders of peripheral myelin proteins. Brain 1995;118:809-818.**
**66****. A 12-year-old boy with an ankle fracture undergoes closed reduction under sedation in the emergency department. Figure 27 shows a lateral radiograph of the ankle after two attempts at closed reduction. Based on these findings, treatment should now consist of
1- at least two more attempts at closed reduction in the emergency department before the patient’s sedation wears off.
2- at least two attempts at closed reduction in the operating room under general anesthesia with muscle relaxation.
3- acceptance of the reduction because the alignment is satisfactory and growth problems are rare with Salter-Harris type I fractures.
4- open reduction, extraction of any interposed periosteum, and smooth wire fixation to prevent nonunion.
5- open reduction, extraction of any interposed periosteum, and smooth wire fixation to decrease the chance of premature physeal closure.
PREFERRED RESPONSE: 5**
**DISCUSSION: The widening of the physis associated with incomplete reduction of this fracture suggests that periosteum is interposed at the fracture site. Clinical and animal study findings suggest that the interposed periosteum may lead to premature physeal closure. Repeated forceful attempts at reduction may subject the physis to further injury and should be avoided. Growth problems are common in children with Salter-Harris type I fractures of the lower extremities. Nonunions are rare in children with Salter-Harris type I fractures.**
**REFERENCES: Barmada A, Gaynor T, Mubarak SJ: Premature physeal closure following distal tibial physeal fractures: A new radiographic predictor. J Pediatr Orthop 2003;23:733-739.**
**Gruber HE, Phieffer LS, Wattenbarger JM: Physeal fractures: Part II. Fate of interposed periosteum in a physeal fracture. J Pediatr Orthop 2002;22:710-716.**
**67****. A 9-year-old girl has pain over the fifth toe that is aggravated by shoe wear. Clinical photographs are shown in Figures 28a and 28b. Treatment of this deformity should consist of
1- extensor digitorum longus tenotomy.
2- extensor digitorum longus tenotomy with dorsal fifth metatarsophalangeal (MTP) joint capsulotomy.
3- dorsal V-Y plasty for skin contracture, combined with extensor digitorum longus tenotomy and dorsal fifth MTP capsulotomy.
4- a dorsal and plantar racquet-shaped incision around the fifth toe, combined with extensor digitorum longus tenotomy and circumferential fifth MTP joint release (Butler procedure).
5- plantar proximal phalangeal resection.
PREFERRED RESPONSE: 4**
**DISCUSSION: The major obstacle to overcome in the surgical treatment of this cock-up deformity is recurrence. Dorsal releases can be performed; however, chronic dislocation of the fifth MTP joint usually needs to be addressed with plantar release as well. Chronic dorsal soft-tissue contractures may be overcome with translation of the toe into a plantar-based incision, as described originally by Cockin and accredited to Butler. This is the treatment of choice. Resection of the proximal phalanx improves symptoms but induces a secondary deformity; this procedure is usually reserved for skeletally mature individuals.**
**REFERENCES: Black GB, Grogan DP, Bobechko WP: Butler arthroplasty for correction of adducted fifth toe: A retrospective study of 36 operations between 1968 and 1982. J Pediatr Orthop 1985;5:439-441.**
**Paton RW: V-Y plasty for correction of varus fifth toe. J Pediatr Orthop 1990;10:248-249.**
**Coughlin MJ, Mann RA: Lesser toe deformities, in Coughlin MJ, Mann RA (eds): Surgery of the Foot and Ankle, ed 5. St Louis, MO, Mosby, 1986, pp 132-157.**
**68****. What acetabular procedure for developmental dysplasia of the hip does not require a concentric reduction of the femoral head in the acetabulum?
1- Salter innominate osteotomy
2- Pemberton innominate osteotomy
3- Dega innominate osteotomy
4- Triple innominate osteotomy
5- Staheli shelf procedure
PREFERRED RESPONSE: 5**
**DISCUSSION: All of the reorientation innominate osteotomies require a concentric reduction of the hip. The Staheli shelf procedure may be performed even with the hip subluxated, but it is a salvage procedure that covers a portion of the femoral head with capsular fibrocartilage rather than hyaline cartilage.**
**REFERENCES: Staheli LT, Chew DE: Slotted acetabular augmentation in childhood adolescence. J Pediatr Orthop 1992;12:569-580.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 1, pp 618-650.**
**69****. Figure 29 shows the AP radiograph of a 14-year-old boy. The radiographic findings are most consistent with what pathologic process?
1- Septic arthritis
2- Hemophilia
3- Juvenile rheumatoid arthritis (JRA)
4- Adolescent Blount’s disease
5- Infantile Blount’s disease
PREFERRED RESPONSE: 5**
**DISCUSSION: The severe depression of the proximal medial tibial epiphysis is most consistent with the diagnosis of neglected infantile Blount’s disease. Blount’s disease in adolescents produces a deformity in the metaphyseal region. Septic arthritis and JRA affect both sides of the joint. Hemophilia produces a characteristic widening of the intercondylar notch.**
**REFERENCES: Thompson GH, Carter JR: Late-onset tibia vara (Blount’s Disease).
Clin Orthop 1990;255:24-35.**
**Raney EM, Topoleski TA, Yaghoubian R, et al: Orthotic treatment of infantile tibia vara.
J Pediatr Orthop 1998;18:670-674.**
**70****. A 5-year-old boy has had pain in the right foot for the past month. Examination reveals tenderness and mild swelling in the region of the tarsal navicular. Radiographs are shown in Figure 30. Management should consist of
1- biopsy of the tarsal navicular.
2- curettage and bone grafting of the tarsal navicular.
3- CBC count, C-reactive protein level, erythrocyte sedimentation rate, blood cultures, and IV antibiotics.
4- symptomatic treatment with restriction of weight bearing or application of short leg cast.
5- medial column lengthening of the foot through the tarsal navicular.
PREFERRED RESPONSE: 4**
**DISCUSSION: The child has the classic findings of Kohler’s disease or osteochondrosis of the tarsal navicular. The cause of this condition is not known, but osteonecrosis and mechanical compression have been proposed. Children generally report midfoot pain over the tarsal navicular and limping. Physical findings include tenderness, swelling, and occasionally redness in the region of the tarsal navicular. Radiographs show sclerosis and narrowing of the tarsal navicular. The natural history of the condition is spontaneous resolution and reconstitution of the navicular. Symptomatic treatment with restriction of weight bearing or casting is recommended.**
**REFERENCES: Karp M: Kohler’s disease of the tarsal scaphoid. J Bone Joint Surg
1937;19:84-96.**
**Borges JL, Guille JT, Bowen JR: Kohler’s bone disease of the tarsal navicular. J Pediatr Orthop 1995;15:596-598.**
**71****. A 9-year-old child sustained a fracture-dislocation of C-5 and C-6 with a complete spinal cord injury. What is the likelihood that scoliosis will develop during the remaining years of his growth?
1- 10%
2- 20%
3- 50%
4- 70%
5- 100%
PREFERRED RESPONSE: 5**
**DISCUSSION: The incidence of late spinal deformity after complete spinal cord injury in children depends on the level of the spinal cord injury and the age of the patient at the time of injury. If a cervical level injury occurs before age 10 years, paralytic scoliosis will develop in virtually 100% of patients.**
**REFERENCES: Brown JC, Swank SM, Matta J, et al: Late spinal deformity in quadriplegic children and adolescents. J Pediatr Orthop 1984;4:456-461.**
**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, et al: Scoliosis in pediatric spinal cord-injured patients.
J Pediatr Orthop 1990;10:214-218.**
**72****. Figures 31a and 31b show the radiograph and MRI scan of an otherwise normal
3-month-old infant who has a spinal deformity. MRI reveals no intraspinal anomalies. What is the next step in management?**
**
1- Posterior spinal fusion with instrumentation
2- Anterior-posterior hemiepiphysiodesis
3- Brace management
4- Cardiac and renal evaluation
5- Hemivertebrectomy and fusion
PREFERRED RESPONSE: 4**
**DISCUSSION: Congenital scoliosis in an infant warrants evaluation of the renal, cardiac, and neurologic systems because frequently there is concurrent pathology. Progression in this instance is possible but not certain; therefore, progression must be documented prior to any surgical intervention. Close observation with serial radiographs every 4 to 6 months is appropriate. All of the surgical options listed may be reasonable choices in the future, but cardiac evaluation is the most important issue at this time.**
**REFERENCES: Basu PS, Elsebaie H, Noordeen MH: Congenital spinal deformity:
A comprehensive assessment at presentation. Spine 2002;27:2255-2259.**
**Hedequist D, Emans J: Congenital scoliosis. J Am Acad Orthop Surg 2004;12:266-275.**
**73****. A 22-month-old girl has cerebral palsy. Which of the following findings is a good prognostic indicator of the child’s ability to walk in the future?
1- Asymmetric tonic neck reflex
2- Moro reflex
3- Extensor thrust
4- Positive parachute reaction
5- Absent foot placement
PREFERRED RESPONSE: 4**
**DISCUSSION: For the parachute test, the examiner holds the child prone and then lowers the child rapidly toward the floor. The parachute reaction is normal or positive if the child reaches toward the floor. The Moro or startle reflex should not be present beyond age 6 months. Asymmetric tonic neck reflex, extensor thrust, and absent foot placement are abnormal findings at any age.**
**REFERENCES: Bleck EE: Orthopaedic Management in Cerebral Palsy. Lavenham, Suffolk, The Lavenham Press, 1987, pp 121-139.**
**Tachdjian MO: The neuromuscular system: Cerebral palsy, in Wickland EH Jr (ed): Pediatric Orthopaedics, ed 2. Philadelphia, PA, WB Saunders, 1990, vol 2, p 1621.**
**74****. The husband of a 22-year-old woman has hypophosphatemic rickets. The woman has no orthopaedic abnormalities, but she is concerned about her chances of having a child with the same disease. What should they be told regarding this disorder?
1- Their sons will have a 50% chance of having this X-linked dominant disorder.
2- All of their daughters will be carriers or will have this disorder.
3- They should be advised to not have any children as the risk of having boys with the disorder and girls who will be carriers is too hard for any parent.
4- As long as the woman does not carry the trait, the children will not be affected because the husband has the disease and this is an X-linked dominant disorder.
5- Their sons or daughters may be born with this disorder, but males are more severely affected.
PREFERRED RESPONSE: 2**
**DISCUSSION: Hypophosphatemia is a rare genetic disease usually inherited as an X-linked dominant trait. The fact that the woman has no skeletal manifestations would indicate that the husband has the X-linked mutation. The disease is more severe in boys than it is in girls. The husband will not transmit the disease to his sons. However, all of their daughters will be affected either with the disease or as carriers. If the woman has the disease or the trait, there is a 50% chance that her sons will inherit the disease and a 50% chance that her daughters will be carriers or have a milder form of the disease. Parents should be advised to have genetic counseling so they can be informed when deciding whether to have children.**
**REFERENCES: Herring JA: Metabolic and endocrine bone diseases, in Tachdjian’s Pediatric Orthopaedics, ed 3. New York, NY, WB Saunders, 2002, pp 1685-1743.**
**Sillence DO: Disorders of bone density, volume, and mineralization, in Rimoin DL, Conner JM, Pyerite RE, et al (eds): Principles and Practice of Medical Genetics, ed 4. New York, NY, Churchill Livingstone, 2002.**
**Staheli LT: Practice of Pediatric Orthopedics. Philadelphia, PA, Lippincott Williams & Wilkins, 2001.**
**75****. A 9-year-old boy sustained a traumatic brain injury and right lower extremity trauma in an accident involving a motor vehicle and a pedestrian. Initial evaluation in the emergency department reveals an obtunded patient who is breathing spontaneously and withdraws appropriately to painful stimuli. After initial resuscitation and stabilization, a CT scan reveals a right parietal intracranial hemorrhage. Radiographs of the swollen right thigh are shown in Figures 32a and 32b. Management of the fractured femur should ultimately consist of
1- immediate hip spica casting.
2- closed reduction and percutaneous pin fixation supplemented by a hip spica cast.
3- placement in 90-90 traction after insertion of a distal femoral traction pin.
4- insertion of a reamed antegrade intramedullary nail starting at the piriformis fossa, stopping the nail short of the distal femoral growth plate.
5- closed reduction and stabilization using retrograde flexible intramedullary nails.
PREFERRED RESPONSE: 5**
**DISCUSSION: A child with a traumatic brain injury generally achieves significant neurologic recovery and has a more favorable prognosis than an adult. Early stabilization of fractures facilitates transportation of the child for diagnostic tests and decreases the incidence of shortening and malunion. Surgical treatment of the fracture is indicated when cerebral perfusion pressure has stabilized. Casting or traction is not the most appropriate treatment of a femoral fracture in a child of this age with a brain injury. Fracture reduction is difficult to maintain if the brain injury leads to spasticity, and transportation within the hospital for tests is more difficult. Insertion of a reamed antegrade intramedullary nail inserted at the piriformis fossa is associated with a small risk of osteonecrosis of the femoral head. The transverse femoral fracture in this patient is ideally suited for stabilization with flexible intramedullary nails. Ligier and associates treated 123 femoral shaft fractures in children with flexible intramedullary nails, including
35 patients with head injury. In one patient with hemiplegia and a urinary tract infection, a deep wound infection developed, necessitating nail removal. The remaining patients all healed without major complications. Heinrich and associates treated 78 diaphyseal femoral fractures with flexible intramedullary nails, including 14 with head injury. No major complications were reported and all fractures healed.**
**REFERENCES: Tolo VT: Management of the multiply injured child, in Rockwood CA, Wilkins KE, Beaty JH (eds): Fractures in Children, ed 4. Philadelphia, PA, Lippincott-Raven, 1996,
pp 83-95.**
**Ligier JN, Metaizeau JP, Prevot J, et al: Elastic stable intramedullary nailing of femoral shaft fractures in children. J Bone Joint Surg Br 1988;70:74-77.**
**Heinrich MS, Drvaric DM, Darr K, et al: The operative stabilization of pediatric diaphyseal femur fractures with flexible intramedullary nails: A prospective analysis. J Pediatric Orthop 1994;14:501-507.**
**Canale ST, Tolo VT: Fractures of the femur in children. Instr Course Lect 1995;44:255-273.**
**76****. Figure 33 shows the oblique radiograph of an 11-year-old boy who has a mild left flatfoot deformity. Examination reveals that subtalar motion is limited and painful. Despite casting for 6 weeks, the patient reports foot pain that limits participation in sport activities. A CT scan shows no subtalar joint abnormalities. Management should
now include
1- manipulation of the foot under general anesthesia.
2- peroneal lengthening.
3- coalition resection with interposition of fat or muscle.
4- distal calcaneal lengthening osteotomy.
5- triple arthrodesis.
PREFERRED RESPONSE: 3**
**DISCUSSION: The radiograph shows an incompletely ossified calcaneonavicular coalition. When symptomatic, a trial of cast immobilization is reasonable. If this fails to provide relief, the preferred treatment is resection of the coalition. Before attempting surgery, a CT scan should be obtained to rule out ipsilateral subtalar coalition. Recurrence of the coalition is usually prevented with interposition of autogenous fat graft or with local interposition of the extensor digitorum brevis muscle. Approximately 80% of patients treated in this manner have decreased pain and improved subtalar motion. When the flatfoot deformity is mild, calcaneal lengthening or medial translation osteotomy is unnecessary. Primary triple arthrodesis may be indicated if degenerative changes are present in the subtalar or midfoot joints. Peroneal lengthening
has been described for treatment of the peroneal spastic flatfoot without demonstrable
tarsal coalition.**
**REFERENCES: Gonzalez P, Kumar SJ: Calcaneonavicular coalition treated by resection and interposition of the extensor digitorum brevis muscle. J Bone Joint Surg Am 1990;72:71-77.**
**Vincent KA: Tarsal coalition and painful flatfoot. J Am Acad Orthop Surg 1998;6:274-281.**
**Luhmann SJ, Rich MM, Schoenecker PL: Painful idiopathic rigid flatfoot in children and adolescents. Foot Ankle Int 2000;21:59-66.**
**77****. A nonambulatory verbal 6-year-old child with spastic quadriplegic cerebral palsy has progressive bilateral hip subluxation of more than 50%. There is no pain with range of motion, but abduction is limited to 20 degrees maximum. An AP radiograph is seen in Figure 34. Management should consist of
1- percutaneous bilateral adductor tenotomy.
2- oral baclofen.
3- phenol injection into the obturator nerve.
4- open adductor tenotomy with neurectomy of the anterior branch of the
obturator nerve.
5- open adductor tenotomy with release of the iliopsoas and bilateral proximal femoral varus derotation osteotomy.
PREFERRED RESPONSE: 5**
**DISCUSSION: The natural history of the patient’s hips, if left untreated, is gradual progression to dislocation. To prevent future pain, prevention of dislocation is often helpful. The patient is too old for soft-tissue releases alone. Therefore, the treatment of choice is medial release of both hips to obtain 45 degrees or better of hip abduction in conjunction with psoas tenotomy and bilateral femoral varus osteotomies.**
**REFERENCES: Presedo A, Oh CW, Dabney KY, et al: Soft-tissue releases to treat spastic hip subluxation in children with cerebral palsy. J Bone Joint Surg Am 2005;87:832-841.**
**Miller F, Bagg MR: Age and migration percentage as risk factors for progression in spastic hip disease. Dev Med Child Neurol 1995;37:449-455.**
**78****. Figures 35a through 35c show the clinical photograph and radiographs of a 15-year-old boy who stubbed his toe 1 day ago while walking barefoot in the yard. Management should consist of
1- buddy taping of the great toe to the second toe for 3 weeks and use of a
hard-soled shoe.
2- buddy taping of the great toe to the second toe for 3 weeks and application of
a short leg cast.
3- buddy taping of the great toe to the second toe for 3 weeks, use of a hard-soled shoe, and a short course of antibiotics.
4- nail removal in the emergency department, buddy taping of the great toe to the second toe for 3 weeks, and use of a hard-soled shoe.
5- irrigation and open reduction, with or without fixation, and a short course
of antibiotics.
PREFERRED RESPONSE: 5**
**DISCUSSION: The patient has an open fracture of the physis of the distal phalanx with a portion of the nail bed interposed in the physis. Seymour initially described this injury in the distal phalanges of fingers. Optimal treatment consists of removing the interposed tissue, irrigating the fracture, and a short course of antibiotics. The nail should be preserved to provide stability.**
**REFERENCES: Kensinger DR, Guille JT, Horn BD, et al: The stubbed great toe: Importance of early recognition and treatment of open fractures of the distal phalanx. J Pediatr Orthop 2001;21:31-34.**
**Pinckney LE, Currarino G, Kennedy LA: The stubbed great toe: A cause of occult compound fracture and infection. Radiology 1981;138:375-377.**
**Seymour N: Juxta-epiphysial fracture of the terminal phalanx of the finger. J Bone Joint Surg Br 1966;48:347-349.**
**79****. Figure 36 shows the radiograph of a 14-year-old boy who has been treated in the past for Perthes’ disease with an abduction brace. He now has hip pain that limits his activity, and nonsteroidal anti-inflammatory drugs have failed to provide relief. What is the most appropriate treatment?
1- Proximal femoral varus osteotomy
2- Salter innominate osteotomy
3- Distal transfer of the greater trochanter
4- Shelf acetabuloplasty
5- Hip arthrodesis
PREFERRED RESPONSE: 4**
**DISCUSSION: Several authors have reported good success in relieving pain with shelf acetabuloplasty. This patient’s Perthes’ disease is in the healed phase; therefore, proximal femoral varus and Salter innominate osteotomies aimed at improving containment are not indicated. The medial one half of the patient’s femoral head is markedly deformed, and rotating it into a weight-bearing position with proximal femoral valgus osteotomy is unlikely to relieve pain. Hip arthrodesis can always be performed as a salvage procedure if the shelf acetabuloplasty fails.**
**REFERENCES: Daly K, Bruce C, Catterall A: Lateral shelf acetabuloplasty in Perthes’ disease: A review of the end of growth. J Bone Joint Surg Br 1999;81:380-384.**
**Yoo WJ, Choi IH, Chung CY, et al: Valgus femoral osteotomy for hinge abduction in Perthes disease: Decision-making and outcomes. J Bone Joint Surg Br 2004;86:726-730.**
**80****. A newborn girl is referred for evaluation of suspected hip instability. What information from her history would place her in the highest risk category?
1- History of maternal diabetes mellitus
2- Frank breech presentation
3- Female gender
4- Concomitant metatarsus adductus
5- Twin gestation
PREFERRED RESPONSE: 2**
**DISCUSSION: Breech positioning has been noted as the risk factor that most increases the relative risk of developmental dysplasia of the hip in multiple series and meta-analysis. All the other factors also increase the risk but to a lesser magnitude.**
**REFERENCES: Lehmann HP, Hinton R, Morello P, et al: Developmental dysplasia of the hip practice guideline: Technical report. Committee on Quality Improvement, and Subcommittee on Developmental Dysplasia of the Hip. Pediatrics 2000;105:E57.**
**Haynes RJ: Developmental dysplasia of the hip: Etiology, pathogenesis, and examination and physical findings in the newborn. Instr Course Lect 2001;50:535-540.**
**81****. A teenager is undergoing a correction of deformity and lengthening of the femur. Distractions are proceeding as expected; however, during his 6-week follow-up examination, the patient reports that the distraction motors have become harder to turn over for the past 2 to 3 days. Figures 37a and 37b show current radiographs. What is the most likely complication being encountered?
1- Incomplete corticotomy
2- Knee subluxation
3- Wire breakage
4- Poor regenerate bone formation
5- Premature consolidation
PREFERRED RESPONSE: 5**
**DISCUSSION: Premature consolidation is a complication that is unique to gradual bone lengthening after corticotomy. Causes include excessive latency period, inadequate distraction rate, exuberant bone formation, patient compliance problems, and mechanical failure of the distraction apparatus. The femur and fibula are most commonly involved. This patient did not have an incomplete corticotomy, as initial distraction occurred before the distraction device was noted to seize up. The radiographs show bowing of the Ilizarov wires and mature regenerate bone, both suggestive of premature consolidation. No wire breakage or joint subluxation is seen on the radiographs. Treatment for premature consolidation includes continuing distraction until the consolidation bridge ruptures, or additional surgery may include closed rotational osteoclasis or repeat corticotomy.**
**REFERENCES: Paley D: Problems, obstacles and complications of limb lengthening, in Maiocchi AB, Aronson J (eds): Operative Principles of Ilizarov. Baltimore, MD, Williams & Wilkins, 1991, p 360.**
**Herring JA: Limb length discrepancy, in Herring JA (ed): Tachdjian’s Pediatric Orthopaedics,
ed 3. Philadelphia, PA, WB Saunders, 2002, p 1088.**
**82****. A 4-year-old child was born with bilateral congenital radial clubhands. Which of the following associated conditions is a contraindication to centralization of the hands on
the ulna?
1- Congenital scoliosis
2- Hypoplastic thumb
3- Tracheoesophageal fistula
4- Imperforate anus
5- Lack of elbow flexion
PREFERRED RESPONSE: 5**
**DISCUSSION: Patients born with bilateral radial clubhands may have difficulty getting their hands to their mouth. The centralization procedure would take away that ability if there is a lack of elbow flexion.**
**REFERENCES: Green DP, Hotchkiss RN, Pederson WC: Green’s Operative Hand Surgery,
ed 4. New York, NY, Churchill Livingstone, 1999, pp 344-349.**
**Herring JA: Tachjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002,
vol 1, pp 382, 511.**
**83****. A 6-year-old Little League pitcher has had pain in the right elbow for the past 2 weeks. Examination reveals mild lateral elbow joint tenderness with full range of motion and no effusion or collateral laxity. A radiograph is shown in Figure 38. Initial management should consist of
1- cessation of throwing activities.
2- a long arm cast for 3 months.
3- a corticosteroid injection into the elbow joint.
4- excision of the fragment.
5- arthroscopic drilling of the lesion.
PREFERRED RESPONSE: 1**
**DISCUSSION: The radiograph shows osteochondritis dissecans (OCD) of the capitellum, one manifestation of “pitcher’s elbow.” The lesion is nondisplaced, and healing is possible if the inciting throwing activities are curtailed. Long arm cast treatment may be reasonable for the noncompliant patient but should not exceed 6 weeks duration. Surgical treatment is indicated for loose bodies or cartilage flaps. Elbow OCD lesions are now being seen in younger children as more participate in organized sports, especially baseball and gymnastics.**
**REFERENCES: Bauer M, Jonsson K, Josefsson PO, et al: Osteochondritis dissecans of the elbow: A long-term follow-up study. Clin Orthop 1992;284:156-160.**
**Takahara M, Ogino T, Sasaki I, et al: Long term outcome of osteochondritis dissecans of the humeral capitellum. Clin Orthop 1999;363:108-115.**
**Byrd JW, Jones KS: Arthroscopic surgery for isolated capitellar osteochondritis dissecans in adolescent baseball players: Minimum three-year follow-up. Am J Sports Med
2002;30:474-478.**
**84****. The parents of a 10-year-old boy with Down syndrome are seeking sports clearance for participation in the high jump at the Special Olympics. He is asymptomatic, and the neurologic examination is normal. The hips and patellae are clinically stable. Radiographs of the cervical spine in flexion and extension show a maximum
atlanto-dens interval (ADI) of 6 mm. Based on these findings, what recommendation should be made?
1- Clearance for all sports activities
2- Avoidance of contact sports, high jump, and diving
3- Application of a hard cervical collar during sports events
4- Application of a halo vest
5- Posterior atlantoaxial arthrodesis
PREFERRED RESPONSE: 2**
**DISCUSSION: In approximately 15% of children with Down syndrome, atlantoaxial instability develops because of ligament laxity, making them susceptible to spinal cord injury with relatively minor trauma. The American Academy of Pediatrics recommends lateral flexion-extension views of the cervical spine in any patient with Down syndrome who wishes to participate in sports. A normal ADI is up to 4 mm. Patients with Down syndrome with an ADI of more than 5 mm should not participate in contact sports or sports with a high risk for neck injury, such as diving, gymnastics, high jump, or butterfly stroke. Cervical fusion has a very high rate of complications in patients with Down syndrome and is recommended only for patients who have myelopathic signs or symptoms.**
**REFERENCES: Atlantoaxial instability in Down syndrome: Subject review. American Academy of Pediatrics Committee on Sports Medicine and Fitness. Pediatrics 1995;96:151-154.**
**Tredwell SJ, Newman DE, Lockitch G: Instability of the upper cervical spine in Down syndrome. J Pediatr Orthop 1990;10:602-606.**
**Segal LS, Drummond DS, Zanotti RM, et al: Complications of posterior arthrodesis of the cervical spine in patients who have Down syndrome. J Bone Joint Surg Am 1991;73:1547-1554.**
**85****. An 18-month-old infant with myelomeningocele and rigid clubfeet has grade 5 quadriceps and hamstring strength, but no muscles are functioning below the knee. What is the best treatment option for the rigid clubfeet?
1- Triple arthrodesis
2- Soft-tissue releases as necessary
3- Tendon transfers to balance the feet in a neutral plantigrade position
4- Physical therapy for range of motion and stretching
5- Botulinum injections followed by serial casting
PREFERRED RESPONSE: 2**
**DISCUSSION: This child has the potential to walk and therefore should have all the contracted structures in the feet released as necessary to place the feet in a plantigrade position for fitting of ankle-foot orthoses. Physical therapy, manipulation, and casting may provide some benefit in a newborn with flexible feet but are not effective in an older infant with rigid clubfeet. Botulinum injections and tendon transfers are of no use because there are no muscles functioning below the knee. Tendon releases are more effective than tendon transfers in children with myelomeningocele.**
**REFERENCES: Mazur JM: Management of foot and ankle deformities in the ambulatory child with myelomeningocele, in Sarwark JR, Lubicky JP (eds): Caring for the Child with Spina Bifida. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2001, pp 155-160.**
**Dias LS: Surgical management of acquired foot and ankle deformities, in Sarwark JR, Lubicky JP (eds): Caring for the Child with Spina Bifida. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2001, pp 161-170.**
**86****. Figures 39a and 39b show the current radiographs of an 8-year-old girl who has had pain in the left thigh for the past 3 months. She was recently diagnosed with hypothyroidism and started treatment 1 week ago. Examination reveals a mild abductor deficiency limp on the left side. She lacks 30 degrees internal rotation on the left hip compared with the right hip. Management should consist of
1- abductor muscle strengthening.
2- a left 1-½ hip spica cast.
3- closed reduction and pinning of the left hip.
4- symptomatic treatment with crutch walking and nonsteroidal
anti-inflammatory drugs.
5- in situ pinning of both hips.
PREFERRED RESPONSE: 5**
**DISCUSSION: The radiographs confirm a slipped capital femoral epiphysis of the left hip, as well as a widened growth plate on the contralateral hip. This is considered a stable slip because the patient is able to walk. Treatment options for stable slips include in situ pinning, bone graft epiphysiodesis, and in some centers severe slips are treated with primary osteotomy and epiphyseal fixation. Percutaneous in situ fixation is the most popular and widely used method of treatment. This juvenile patient has an endocrine condition and a widened growth plate on the right side; therefore, strong consideration should be given to pinning the contralateral hip
“pre-slip.” Muscle strengthening, hip spica casting, and closed reduction have no place in the primary treatment of a stable slipped capital femoral epiphysis.**
**REFERENCES: Loder RT, Richards BS, Shapiro PS, et al: Acute slipped capital femoral epiphysis: The importance of physeal stability. J Bone Joint Surg Am 1993;75:1134-1140.**
**Loder R, Wittenberg B, DeSilva G: Slipped capital femoral epiphysis associated with endocrine disorders. J Pediatr Orthop 1995;15:349-356.**
**Aronson DD, Carlson WE: Slipped capital femoral epiphysis: A prospective study of fixation with a single screw. J Bone Joint Surg Am 1992;74:810-819.**
**87****. A 7-year-old boy with spastic diplegia is a limited community ambulator. He has a moderately severe crouched gait. The parents request a treatment that will result in a permanent decrease in lower extremity muscle tone. This is best accomplished with
1- tone-reduction ankle-foot orthoses (AFOs).
2- intramuscular injections of botulinum-A toxin.
3- an intrathecal baclofen injection.
4- selective posterior rhizotomy.
5- fractional tendon lengthening of bilateral hamstring and gastrocnemius muscles.
PREFERRED RESPONSE: 4**
**DISCUSSION: Posterior rhizotomy provides a permanent reduction in tone of spastic muscles. Potential drawbacks of the procedure include excessive muscle weakness, hip dislocation, and spinal deformity. Intramuscular botulinum-A toxin results in permanent blockade of presynaptic release of acetylcholine across the neuromuscular junction. The clinical effect usually resolves after 3 to 6 months due to neural regeneration. Tone-reduction AFOs have not been shown to reduce tone. A baclofen pump could offer prolonged reduction in tone, but not a single intrathecal injection.**
**REFERENCES: Arens LJ, Peacock WJ, Peter J: Selective posterior rhizotomy: A long-term follow-up study. Childs Nerv Syst 1989;5:148-152.**
**Koman LA, Paterson Smith B, Balkrishnan R: Spasticity associated with cerebral palsy in children: Guidelines for the use of botulinum-A toxin. Paediatr Drugs 2003;5:11-23.**
**Johnson MB, Goldstein L, Thomas SS, et al: Spinal deformity after selective dorsal rhizotomy in ambulator patients with cerebral palsy. J Pediatr Orthop 2004;24:529-536.**
**88****. Figure 40 shows the radiographs of a 2-year-old boy who has a deformed leg. The patient is ambulatory and has no pain. What is the most appropriate management?
1- Observation
2- Patellar tendon bearing (PTB) orthosis
3- Osteotomy to correct the deformity and fixation with an intramedullary rod
4- Vascularized fibular graft from the contralateral leg
5- Amputation
PREFERRED RESPONSE: 2**
**DISCUSSION: The patient has a prefractured stage of congenital pseudarthrosis of the tibia and is at risk for fracture. The PTB orthosis may prevent or delay the fracture. Osteotomy is frequently complicated by nonunion. When established nonunion does not respond to intramedullary nailing and bone grafting, vascularized grafting may succeed. Amputation is a salvage procedure.**
**REFERENCES: Murray HH, Lovell WW: Congenital pseudarthrosis of the tibia: A long-term follow-up study. Clin Orthop 1982;166:14-20.**
**Crossett LS, Beaty JH, Betz RR, et al: Congenital pseudarthrosis of the tibia: Long-term
follow-up study. Clin Orthop 1989;245:16-18.**
**89****. Where is the most common site for tuberculosis (TB) spondylitis in children?
1- Anterior aspect of the lower thoracic region
2- Anterior aspect of the cervical spine
3- Posterior elements of the lower thoracic region
4- Posterior elements of the cervical spine
5- Transverse process of the lower lumbar spine
PREFERRED RESPONSE: 1**
**DISCUSSION: In children, the main route of infection in skeletal TB is through hematogenous spread from a primary source. The mycobacterium is deposited in the end arterials in the vertebral body adjacent to the anterior aspect of the vertebral end plate. Thus, the anterior portion of the vertebral body is most commonly involved. The lower thoracic region is the most common segment; next in decreasing order of frequency are the lumbar, upper thoracic, cervical, and sacral regions.**
**REFERENCES: Teo HE, Peh WC: Skeletal tuberculosis in children. Pediatric Radiol 2004;34:853-860.**
**Herring JA: Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, vol 1, pp 1831-1835.**
**90****. Examination of a 13-year-old boy with asymptomatic poor posture reveals increased thoracic kyphosis that is fairly rigid and accentuates during forward bending. The neurologic examination is normal. Spinal radiographs show 10 degrees of scoliosis at Risser stage 2, and there is no evidence of spondylolisthesis. A standing lateral view of the thoracic spine is shown in Figure 41. The kyphosis corrects to 50 degrees. Management should consist of
1- electrical stimulation.
2- a Charleston bending brace at night.
3- an extension-type spinal orthosis.
4- posterior spinal fusion with instrumentation.
5- anterior spinal release and posterior spinal instrumentation.
PREFERRED RESPONSE: 3**
**DISCUSSION: The radiograph shows excessive thoracic kyphosis (normal 20 degrees to
50 degrees) with multiple contiguous vertebral wedging and end plate irregularity, all consistent with the diagnosis of Scheuermann’s kyphosis. The patient is skeletally immature; therefore, there is the potential for progression of the kyphotic deformity. Extension bracing has shown efficacy in the treatment of Scheuermann’s kyphosis that measures 50 degrees to 74 degrees, and has actually reduced the curvature permanently in some patients. A thoracolumbosacral orthosis may be used if the apex of kyphosis is at T7 or lower. Indications for surgical treatment are controversial, but spinal fusion most likely should not be considered for a painless kyphosis measuring less than 75 degrees. **
**REFERENCES: Murray PM, Weinstein SL, Spratt KF: The natural history and long-term follow-up of Scheuermann kyphosis. J Bone Joint Surg Am 1993;75:236-248.**
**Wenger DR, Frick SL: Scheuermann kyphosis. Spine 1999;24:2630-2639.**
**Tribus CB: Scheuermann’s kyphosis in adolescents and adults: Diagnosis and management.
J Am Acad Orthop Surg 1998;6:36-43.**
**91****. What is the most important sign of impending modulation with rapid progression of a spinal deformity in neurofibromatosis?
1- Apical curve rotation
2- Anterior vertebral body erosions
3- Cervical spine involvement
4- Penciling of three or more ribs
5- Curve magnitude of more than 50 degrees
PREFERRED RESPONSE: 4**
**DISCUSSION: Rib penciling is the only singular factor; 87% of the curves progressed significantly in patients with three or more penciled ribs. Modulation in neurofibromatosis scoliosis implies the change from an idiopathic type to a dysplastic type of curve with rapid progression and the need for aggressive stabilization by fusion.**
**REFERENCES: Crawford AH, Schorry EK: Neurofibromatosis in children: The role of the orthopaedist. J Am Acad Orthop Surg 1999;7:217-230.**
**Durrani AA, Crawford AH, Chouhdry SN, et al: Modulation of spinal deformities in patients with neurofibromatosis type 1. Spine 2000;25:69-75.**
**92****. A 6-year-old child has a fixed flexion deformity of the interphalangeal (IP) joint of the right thumb. The thumb is morphologically normal, with a nontender palpable nodule at the base of the metacarpophalangeal joint. Clinical photographs are shown in Figures 42a and 42b. Based on these findings, what is the treatment of choice?
1- Complete release of the proximal annular pulley of the flexor sheath
2- Removal of the nodule in the flexor pollicis longus
3- Fractional lengthening of the flexor pollicis longus tendon at the musculotendinous junction
4- Steroid injection into the palpable nodule
5- No treatment because this condition normally spontaneously resolves
PREFERRED RESPONSE: 1**
**DISCUSSION: The child has a trigger thumb deformity. A trigger thumb is a developmental mechanical problem rather than a congenital deformity. The anomaly generally is not noted at birth. A fixed flexion deformity of the IP joint of the thumb most commonly occurs in children in the first 2 years of life. A stretching and splinting program may correct the deformity in the first year of life, but nonsurgical management after age 3 years results in a success rate of only 50%. Release of the proximal annular pulley of the flexor sheath is recommended at this age.**
**REFERENCES: Tan AH, Lam KS, Lee EH: The treatment outcome of trigger thumb in children. J Pediatric Orthop B 2002;11:256-259.**
**Slakey JB, Hennrikus WL: Acquired thumb flexion contracture in children: Congenital trigger thumb. J Bone Joint Surg Br 1996;78:481-483.**
**Herring JA: Disorders of the upper extremity: Thumb dysplasia, in Herring JA (ed): Tachdjian’s Pediatric Orthopaedics, ed 3. Philadelphia, PA, WB Saunders, 2002, p 445.**
**93****. A 3-year-old boy had been treated with serial casting for a right congenital idiopathic clubfoot deformity. The parents are concerned because the child now walks on the lateral border of the right foot. Examination shows that the foot passively achieves a plantigrade position with neutral heel valgus and ankle dorsiflexion to 15 degrees. The forefoot inverts during active ankle dorsiflexion. Mild residual metatarsus adductus is present. Management should now consist of
1- additional serial casting.
2- a floor-reaction ankle-foot orthosis.
3- closing wedge cuboid osteotomy.
4- lateral transfer of the anterior tibialis tendon.
5- posterior tibial tendon transfer through the interosseous membrane to the
third metatarsal.
PREFERRED RESPONSE: 4**
**DISCUSSION: Dynamic midfoot supination that is the result of peroneal weakness is a common residual problem after cast correction or surgical reconstruction of a congenital idiopathic clubfoot. Dynamic supination is unlikely to resolve spontaneously. Most parents do not want to use brace support forever. Transfer of the posterior tibialis to the dorsum of the foot has shown poor results in clubfeet. Preferred treatments include: 1) transfer of the entire anterior tibialis tendon to the lateral cuneiform, or 2) split transfer of the anterior tibialis tendon to the cuboid or to the peroneus brevis tendon.**
**REFERENCES: Kuo KN, Hennigan SP, Hastings ME: Anterior tibial tendon transfer in residual dynamic clubfoot deformity. J Pediatr Orthop 2001;21:35-41.**
**Garceau GJ: Anterior tibial tendon transfer for recurrent clubfoot. Clin Orthop 1972;84:61-65.**
**Miller GM, Hsu JD, Hoffer MM, et al: Posterior tibial tendon transfer: A review of the literature and analysis of 74 procedures. J Pediatr Orthop 1982;2:363-370.**
**94****. Figures 43a and 43b show the clinical photographs of a 4-month-old child with bilateral popliteal pterygium. The fixed knee contractures measure 100 degrees bilaterally. What future treatment is most likely to successfully correct this deformity?
1- Serial casting of both knees weekly
2- Physiotherapy and dynamic splinting
3- Soft-tissue releases of the knees, including Z-plasties of skin, excision of fibrotic bands, hamstring lengthenings, and posterior knee capsulotomies
4- Femoral shortening osteotomies combined with soft-tissue releases of the knees (Z-plasties of skin, excision of fibrotic bands, hamstring lengthenings, and posterior knee capsulotomies)
5- Gradual correction with a circular external fixator without soft-tissue release
PREFERRED RESPONSE: 4**
**DISCUSSION: Congenital popliteal webbing with contractures of 60 degrees is a difficult deformity to correct. The anatomy of the web is of considerable importance. MRI can delineate the extent of the posterior fibrous band that often stretches from the ischium to the calcaneus. The sciatic nerve, usually shortened, most often runs just anterior to this fibrous band. For mild contractures of less than 20 degrees, nonsurgical management is usually adequate. Hamstring lengthening and postoperative splinting are usually sufficient for contractures of 20 degrees to
40 degrees. Moderate contractures of up to 60 degrees usually require Z-plasties in the popliteal fossa and postoperative serial casting to avoid undue tension on neurovascular structures. Contractures of more than 60 degrees require a femoral shortening osteotomy or gradual correction with an external fixator. However, rapid recurrence following fixator removal is common if formal soft-tissue procedures and postoperative splinting are not performed.**
**REFERENCES: Parikh SN, Crawford AH, Do TT, et al: Popliteal pterygium syndrome: Implications for orthopaedic management. J Pediatr Orthop B 2004;13:197-201.**
**Brunner R, Hefti F, Tgetgel JD: Arthrogrypotic joint contracture at the knee and foot: Correction with a circular frame. J Pediatr Orthop B 1997;6:192-197.**
**95****. A 15-year-old boy reports a 2-day history of progressive left buttock pain and severe limping. He denies any history of trauma or radiation of the pain. He has an oral temperature of 100.4 degrees F (38 degrees C). Examination reveals that the lumbar spine and left hip have unguarded motion. The abdomen is nontender. There is moderate tenderness of the left sacroiliac region with no palpable swelling. Pain is elicited when the left lower extremity is placed in the figure-4 position (FABER test). Laboratory studies show a peripheral WBC count of 11,500/mm** P3P**(normal to 10,500/mm** P3P**) and an erythrocyte sedimentation rate of 38 mm/h (normal up to 20 mm/h). Radiographs of the pelvis, hips, and lumbar spine are normal. A nucleotide bone scan (posterior view) is shown in Figure 44. Initial management should consist of
1- oral nonsteroidal anti-inflammatory drugs.
2- intravenous antistaphylococcal antibiotics.
3- incision and debridement of the retroperitoneal abscess.
4- incision and debridement of the left sacroiliac joint.
5- arthrotomy and irrigation of the left hip joint.
PREFERRED RESPONSE: 2**
**DISCUSSION: The symptoms, physical findings, and laboratory studies are most consistent with a diagnosis of infectious sacroiliitis, usually caused by Staphylococcus aureus. Initial radiographs will be normal, and the diagnosis of sacroiliitis is often delayed. A technetium
Tc 99m bone scan will localize the problem in 90% of patients but may occasionally give a
false-negative result in early cases. If suspicion is high, a gallium scan or MRI scan may help confirm the diagnosis of sacroiliitis. Needle aspiration of the sacroiliac joint is difficult; therefore, antibiotic selection is usually empiric or based on blood cultures. Sacroiliitis that is the result of connective tissue inflammatory disease is usually bilateral and without fever or leukocytosis. The lack of hip irritability, spinal rigidity, and abdominal tenderness helps to rule out other causes of limping with fever, such as psoas abscess, diskitis, and septic hip.
REFERENCES: Aprin H, Turen C: Pyogenic sacroiliitis in children. Clin Orthop
1993;287:98-106.
Osman AA, Govender S: Septic sacroiliitis. Clin Orthop 1995;313:214-219.**
**96****. A 12-month-old boy has right congenital fibular intercalary hemimelia with a normal contralateral limb. A radiograph of the lower extremities shows a limb-length discrepancy of 2 cm. All of the shortening is in the right tibia. Assuming that no treatment is rendered prior to skeletal maturity, the limb-length discrepancy will
most likely
1- remain 2 cm at maturity.
2- decrease slowly until the limb lengths equalize.
3- increase at a constant rate of 2 cm per year.
4- increase markedly because of complete failure of tibial growth.
5- increase slowly, with the right lower extremity remaining in proportion to the left lower extremity.
PREFERRED RESPONSE: 5**
**DISCUSSION: Many congenital limb deficiencies and bowing deformities result in growth retardation. If unilateral, a gradually progressive limb-length discrepancy will result; however, the proportional lengths of the lower extremities will remain at a relatively constant ratio. For example, if the right foot is at the level of the left knee at birth, this will still be true at maturity. This concept can be useful for early prediction of limb-length discrepancy by using a “multiplier method,” as described by Paley and associates. This method can facilitate early treatment decisions, such as the need for amputation, without having to wait for serial scanography measurements.**
**REFERENCES: Paley D, Bhave A, Herzenberg JE, et al: Multiplier method for predicting
limb-length discrepancy. J Bone Joint Surg Am 2000;82:1432-1446.**
**Moseley CF: A straight-line graph for leg length discrepancies. Clin Orthop 1978;136:33-40.**
**97****. What is the preferred treatment of a symptomatic curly toe deformity in a
6-year-old child?
1- Observation
2- Tape the toe to the adjacent toes for 8 weeks
3- Tenotomy of the flexor tendons
4- Transfer of the flexor tendons to the extensor mechanism
5- Arthrodesis of the proximal interphalangeal joint
PREFERRED RESPONSE: 3**
**DISCUSSION: While some curly toe deformities spontaneously improve in younger children, the deformity is likely to persist in a 6-year-old child. Taping techniques result in no change or only a temporary decrease in deformity. Studies have shown that simple flexor tenotomy is as effective as flexor tendon transfer. Arthrodesis is rarely indicated.**
**REFERENCES: Hamer A, 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.**
**98****. A 12-year-old girl who is Risser stage 3 has had intermittent mild midback pain for the past 4 weeks. The pain is worse after prolonged sitting and after carrying a heavy backpack at school. She occasionally takes acetaminophen, but the pain does not limit sport activities. Examination reveals a mild right rib prominence during forward bending. Neurologic examination is normal. Radiographs show a 20-degree right thoracic scoliosis with no congenital anomalies or lytic lesions. Management should consist of
1- back muscle stretching and reduced weight in the backpack.
2- consultation with a pain management specialist.
3- MRI of the thoracic spine.
4- a technetium Tc 99m bone scan.
5- a thoracolumbosacral orthosis.
PREFERRED RESPONSE: 1**
**DISCUSSION: Mild scoliosis is not a painful condition, but it usually presents during adolescence. Intermittent back pain is reported by 25% to 30% of adolescents whether or not scoliosis is present. Such pain is often attributed to muscle strain from tight muscles, poor posture, or heavy school backpacks. The clinician must distinguish typical pain (mild, intermittent, nonlimiting) from atypical pain. The latter requires more careful examination and imaging studies (bone scan or MRI) to determine the source of pain. The patient’s age and right thoracic curve pattern are typical for idiopathic scoliosis; therefore, imaging of the neuroaxis is not necessary to look for cord syrinx, tethering, or tumor. Brace treatment is not required for this small curve unless future progression is demonstrated.**
**REFERENCES: Ramirez N, Johnston CE, Browne RH: The prevalence of back pain in children who have idiopathic scoliosis. J Bone Joint Surg Am 1997;79:364-368.**
**Hollingworth P: Back pain in children. Br J Rheum 1996;35:1022-1028.**
**Siambanes D, Martinez JW, Butler EW, et al: Influence of school backpacks on adolescent back pain. J Pediatr Orthop 2004;24:211-217.**
**99****. What zone of the physis is widened in rickets?
1- Reserve
2- Proliferative
3- Hypertrophic
4- Maturation
5- Primary spongiosa
PREFERRED RESPONSE: 3**
**DISCUSSION: Rickets causes widening of the hypertrophic layer of the physis because of the failure of mineralization and vascular invasion. The other zones of the physis may be altered in other disease conditions but remain relatively unchanged in rickets.**
**REFERENCES: Hunziker EB, Schenk RK, Cruz-Orive LM: Quantitation of chondrocyte performance in growth-plate cartilage during longitudinal bone growth. J Bone Joint Surg Am 1987;69:162-173.**
**Iannotti JP: Growth plate physiology and pathology. Orthop Clin North Am 1990;21:1-17.**
**100****. A 7-year-old boy has had low back pain for the past 3 weeks. Radiographs reveal apparent disk space narrowing at L4-5. The patient is afebrile. Laboratory studies show a WBC count of 9,000/mm** P3P**and a C-reactive protein level of 10 mg/L. A lumbar MRI scan confirms the loss of disk height at L4-5 and reveals a small perivertebral abscess at that level. To achieve the most rapid improvement and to lessen the chances of recurrence, management should consist of
1- oral antibiotics.
2- IV antibiotics.
3- surgical drainage of the perivertebral abscess and IV antibiotics.
4- bed rest.
5- cast immobilization.
PREFERRED RESPONSE: 2**
**DISCUSSION: The patient has diskitis. Administration of IV antibiotics speeds resolution and minimizes recurrence. Bed rest and cast immobilization have been successfully used to treat this disorder but can be associated with prolonged recovery and frequent recurrence, even when oral antibiotics are administered. A perivertebral abscess seen in association with this condition usually resolves without surgery.**
**REFERENCES: Ring D, Johnston CE II, Wenger DR: Pyogenic infectious spondylitis in children: The convergence of discitis and vertebral osteomyelitis. J Pediatr Orthop
1995;15:652-660.**
**Crawford AH, Kucharzyk DW, Ruda R, et al: Diskitis in children. Clin Orthop 1991;266:70-79.**