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Question 5621

Topic: 6. Spine

A 14-year-old female with long-standing polyarticular Juvenile Idiopathic Arthritis (JIA) is scheduled for a total hip arthroplasty. Before proceeding with endotracheal intubation, which of the following is the most critical preoperative radiographic evaluation?

. AP and lateral thoracic spine radiographs
. Flexion and extension lateral cervical spine radiographs
. CT scan of the temporomandibular joints
. AP pelvis radiograph to template leg length
. Chest radiograph to rule out interstitial lung disease

Correct Answer & Explanation

. Flexion and extension lateral cervical spine radiographs


Explanation

Patients with severe JIA are at high risk for cervical spine instability, particularly atlantoaxial subluxation. Flexion-extension cervical radiographs are mandatory before any procedure requiring intubation to prevent catastrophic neurologic injury.

Question 5622

Topic: 6. Spine

A 12-year-old boy presents with left-sided mid-back pain and is found to have a left thoracic scoliotic curve of 35 degrees. Abdominal reflexes are absent asymmetrically. What is the most appropriate next step in management?

. Prescribe a Boston brace and follow-up in 6 months
. Perform a whole-spine MRI to evaluate for neural axis abnormalities
. Schedule for posterior spinal fusion
. Order a standing AP pelvis radiograph to check for leg length discrepancy
. Reassure the parents that this is typical adolescent idiopathic scoliosis

Correct Answer & Explanation

. Perform a whole-spine MRI to evaluate for neural axis abnormalities


Explanation

A left thoracic curve, severe pain, and asymmetric or absent abdominal reflexes are "red flag" signs for an underlying neural axis abnormality, such as syringomyelia or a Chiari malformation. An MRI of the entire spine is mandatory.

Question 5623

Topic: 6. Spine

A 10-year-old girl is found to have a 45-degree right thoracic scoliosis and an enlarging cervicothoracic syrinx associated with a Chiari I malformation. What is the most appropriate management strategy?

. Immediate posterior spinal fusion, followed by syrinx observation
. Suboccipital decompression of the Chiari malformation first, then reassess the scoliosis
. Simultaneous posterior spinal fusion and syrinx shunting
. Bracing for the scoliosis until skeletal maturity, then syrinx decompression
. Isolated physical therapy for trunk strengthening

Correct Answer & Explanation

. Suboccipital decompression of the Chiari malformation first, then reassess the scoliosis


Explanation

When a significant syrinx or Chiari malformation is the underlying cause of scoliosis, neurosurgical decompression must be performed prior to any spinal fusion. In many young patients, the scoliotic curve will stabilize or even improve following decompression.

Question 5624

Topic: 6. Spine

A 50-year-old patient with known syringomyelia presents with massive, painless swelling and instability of the elbow joint.

The exact neuroanatomic lesion in syringomyelia that leads to this neuropathic joint typically originates in which structure of the spinal cord?

. Posterior columns
. Dorsal root ganglion
. Anterior horn cells
. Anterior white commissure
. Lateral corticospinal tract

Correct Answer & Explanation

. Anterior white commissure


Explanation

Syringomyelia typically begins as a cystic enlargement of the central canal, which first compresses the decussating spinothalamic fibers in the anterior white commissure. This causes the classic bilateral loss of pain and temperature sensation leading to neuropathic arthropathy.

Question 5625

Topic: 6. Spine

According to the Azouz classification for Dysplasia Epiphysealis Hemimelica, which of the following defines a "Classic" (Type 2) presentation?

. Involvement of a single epiphysis
. Involvement of more than one epiphysis within a single limb
. Bilateral symmetrical involvement of the lower extremities
. Generalized involvement of an entire lower limb including the pelvis
. Spine and flat bone involvement

Correct Answer & Explanation

. Involvement of a single epiphysis


Explanation

The Azouz classification divides DEH into three types. Type 1 is localized (one epiphysis), Type 2 is classic (more than one epiphysis in a single limb), and Type 3 is generalized (involving the entire limb).

Question 5626

Topic: 6. Spine

Which radiographic feature in a patient with NF1 is most predictive of rapid progression of dystrophic scoliosis?

. Thoracolumbar location with a long, sweeping curve
. Presence of rib penciling and severe vertebral scalloping
. Associated hyperlordosis of the lumbar spine
. A right thoracic curve in a female patient
. Lack of an associated plexiform neurofibroma

Correct Answer & Explanation

. Presence of rib penciling and severe vertebral scalloping


Explanation

Dystrophic scoliosis in NF1 is characterized by short, sharp curves, rib penciling, severe vertebral scalloping, and spindling of the transverse processes. These dystrophic features indicate a high risk for rapid curve progression, often necessitating early surgical intervention.

Question 5627

Topic: 6. Spine

A 10-year-old boy with NF1 presents with a short, sharply angulated thoracic scoliosis. Radiographs show rib penciling and severe vertebral scalloping. What is the most appropriate surgical management?

. Bracing until skeletal maturity
. Posterior spinal fusion alone
. Combined anterior and posterior spinal fusion
. Growing rod construct
. VEPTR insertion

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion


Explanation

Dystrophic scoliosis in NF1 features a short, sharp curve, rib penciling, and vertebral scalloping. Due to the high risk of pseudoarthrosis and curve progression, combined anterior and posterior spinal fusion is the standard of care.

Question 5628

Topic: 6. Spine

A 7-year-old child with Neurofibromatosis Type 1 (NF1) presents with a rapidly progressive, 55-degree thoracic scoliotic curve. Radiographs show severe apical vertebral scalloping, rib penciling, and a short, sharp curve pattern. What is the most appropriate definitive management?

. Thoracolumbosacral orthosis (TLSO) bracing for 23 hours a day.
. Observation with serial radiographs every 6 months until skeletal maturity.
. Posterior spinal fusion alone using pedicle screw constructs.
. Combined anterior and posterior spinal fusion.
. Growth-friendly constructs (e.g., VEPTR or growing rods) without fusion.

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion.


Explanation

Dystrophic scoliosis in NF1 is characterized by a short, sharp curve with severe bony changes like vertebral scalloping and rib penciling. Due to the high risk of rapid progression and pseudarthrosis, the gold standard treatment for severe dystrophic curves is a combined anterior and posterior spinal fusion.

Question 5629

Topic: 6. Spine
A 9-year-old child with NF1 presents with a significant spinal deformity. Reviewing the radiographs, all of the following are classic radiologic features of a dystrophic NF1 curve EXCEPT:
. Severe vertebral body scalloping
. Rib penciling
. Widened interpedicular distances (dural ectasia)
. Long, sweeping, C-shaped thoracic curve
. Spindling of the transverse processes

Correct Answer & Explanation

. Long, sweeping, C-shaped thoracic curve


Explanation

Dystrophic curves in NF1 are typically short, sharp, and angular, rather than long and sweeping. Vertebral scalloping, rib penciling, and widened pedicles due to dural ectasia are classic hallmark features.

Question 5630

Topic: 6. Spine



A 10-year-old boy with NF-1 presents with a 45-degree short, sharp thoracic scoliotic curve. Radiographs demonstrate rib penciling, vertebral scalloping, and severe rotation. What is the most appropriate definitive management?

. Observation until the curve reaches 50 degrees
. TLSO bracing for 23 hours a day
. Posterior spinal fusion alone
. Combined anterior and posterior spinal fusion
. Growing rod construct

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion


Explanation

This patient has dystrophic scoliosis associated with NF-1, characterized by rapid progression, short sharp curves, rib penciling, and vertebral scalloping. Combined anterior and posterior spinal fusion is required because isolated posterior fusions have unacceptably high pseudarthrosis and curve progression rates.

Question 5631

Topic: 6. Spine

A 10-year-old girl with NF1 presents with a rapidly progressing spinal deformity. Radiographs demonstrate short-segment, sharp angular curves with vertebral scalloping and rib penciling.

What is the most appropriate surgical strategy?

. Observation until skeletal maturity followed by fusion
. Bracing with a custom TLSO orthosis
. Posterior spinal fusion alone using pedicle screws
. Combined anterior and posterior spinal fusion
. Magnetically controlled growing rods placement

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion


Explanation

Dystrophic scoliosis in NF1 is characterized by severe, rapidly progressive, short-segment curves with dysplastic osseous features. Due to the high risk of pseudarthrosis and curve progression, a combined anterior and posterior spinal fusion is the recommended standard of care.

Question 5632

Topic: 6. Spine

A 9-year-old boy with Neurofibromatosis Type 1 (NF1) presents with a rapidly progressing thoracic scoliosis. Radiographs demonstrate a short, sharp curve with vertebral scalloping, severe apical wedging, and penciling of the ribs. Which of the following is the most appropriate surgical strategy for this specific type of curve?

. Bracing until skeletal maturity
. Posterior spinal fusion alone with pedicle screws
. Anterior and posterior spinal fusion
. Growing rod instrumentation
. Observation with serial radiographs

Correct Answer & Explanation

. Anterior and posterior spinal fusion


Explanation

Dystrophic scoliosis in NF1 is characterized by short, sharp curves, vertebral scalloping, and rib penciling. Due to a high risk of pseudoarthrosis and rapid curve progression, combined anterior and posterior spinal fusion is the recommended surgical treatment.

Question 5633

Topic: 6. Spine

A 12-year-old female with Neurofibromatosis Type 1 (NF1) presents with a rapidly progressive 45-degree thoracic scoliosis. Radiographs demonstrate "penciling" of three adjacent ribs, severe vertebral body scalloping, and pronounced rotation. What is the most appropriate surgical management?

. Thoracolumbosacral orthosis (TLSO) bracing
. Posterior spinal fusion alone
. Anterior and posterior spinal fusion
. Growing rod construct
. Vertebral body tethering (VBT)

Correct Answer & Explanation

. Anterior and posterior spinal fusion


Explanation

Dystrophic scoliosis in NF1 has a high risk of rapid curve progression and pseudarthrosis. An anterior and posterior spinal fusion is recommended to achieve solid arthrodesis and prevent progression.

Question 5634

Topic: 6. Spine

A 10-year-old female with Neurofibromatosis Type 1 presents with a rapidly progressive, short-segmented thoracic scoliosis. Radiographs reveal severe apical wedging, rib penciling, and evidence of dural ectasia. What is the most appropriate definitive management for this spinal deformity?

. Observation with serial radiographs
. Thoracolumbosacral orthosis (TLSO)
. Posterior spinal fusion alone
. Combined anterior and posterior spinal fusion
. Growth-friendly spinal instrumentation (e.g., growing rods)

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion


Explanation

Dystrophic scoliosis in NF1 has a very high rate of progression and pseudoarthrosis. Combined anterior and posterior spinal fusion is the standard of care to prevent pseudoarthrosis, hardware failure, and continued curve progression.

Question 5635

Topic: 6. Spine

A 14-year-old boy with a known history of Neurofibromatosis Type 1 undergoes a spine MRI due to worsening back pain and neurological symptoms, as shown in the provided clinical image.

Which of the following best describes the classical spinal manifestation demonstrated, which contributes to dystrophic spinal deformity in NF1?

. Anterior vertebral body wedging
. Posterior vertebral body scalloping secondary to dural ectasia
. Congenital block vertebra
. Dysplastic pars interarticularis
. Pedicle hypertrophy

Correct Answer & Explanation

. Posterior vertebral body scalloping secondary to dural ectasia


Explanation

A classic dystrophic change seen in NF1 is posterior vertebral body scalloping, frequently caused by dural ectasia. This weakens the structural integrity of the spinal column and strongly predisposes the patient to severe, rapidly progressive scoliosis.

Question 5636

Topic: 6. Spine

A 9-year-old girl with Neurofibromatosis Type 1 presents with a rapidly progressive spinal curvature. Which of the following radiographic findings is most characteristic of dystrophic scoliosis in this patient?

. Apical vertebral wedging and rib penciling
. Right thoracic curve with normal kyphosis
. Interpedicular narrowing without vertebral scalloping
. Spondylolisthesis at L5-S1
. Multilevel block vertebrae

Correct Answer & Explanation

. Apical vertebral wedging and rib penciling


Explanation

Dystrophic scoliosis in NF1 is characterized by short, sharp curves, severe apical vertebral wedging, rib penciling, enlarged foramina, and dural ectasia. These curves carry a high risk of rapid progression and often require early surgical spinal fusion.

Question 5637

Topic: 6. Spine

A 9-year-old girl with Neurofibromatosis Type 1 (NF1) presents with a rapidly progressive thoracic scoliosis. Radiographs demonstrate a short-segmented, sharply angulated curve with vertebral body scalloping, severe apical wedging, and rib penciling.

What is the most appropriate surgical management for this patient's spinal deformity?

. Thoracolumbosacral orthosis (TLSO) bracing until skeletal maturity
. Posterior spinal fusion alone with pedicle screw instrumentation
. Combined anterior and posterior spinal fusion
. Growing rod construct without definitive fusion
. Hemiepiphysiodesis on the convex side of the curve

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion


Explanation

Dystrophic scoliosis in NF1 is characterized by short, sharp curves, rib penciling, and vertebral scalloping. These curves have an extremely high risk of rapid progression and pseudoarthrosis, typically necessitating combined anterior and posterior spinal fusion for adequate, rigid stabilization.

Question 5638

Topic: 6. Spine

A 4-year-old girl is diagnosed with oligoarticular juvenile idiopathic arthritis (JIA). Laboratory testing reveals a high titer of antinuclear antibodies (ANA). Which of the following is the most critical routine screening required for this patient?

. Routine slit-lamp examination
. Echocardiogram
. Pulmonary function tests
. Renal ultrasound
. Spine radiography

Correct Answer & Explanation

. Routine slit-lamp examination


Explanation

Patients with early-onset oligoarticular JIA who are ANA positive are at the highest risk for developing chronic, asymptomatic anterior uveitis. Routine slit-lamp screening is mandatory to prevent permanent vision loss.

Question 5639

Topic: 6. Spine

A 35-year-old female with a long-standing history of polyarticular juvenile idiopathic arthritis is scheduled for a total hip arthroplasty. She has severe limitation in multiple joints. What is the most critical pre-operative imaging required prior to intubation?

. Chest computed tomography
. Flexion-extension cervical spine radiographs
. Thoracic spine magnetic resonance imaging
. Pelvic computed tomography
. Abdominal ultrasound

Correct Answer & Explanation

. Flexion-extension cervical spine radiographs


Explanation

Patients with polyarticular JIA frequently develop cervical spine instability, particularly at the C1-C2 level. Flexion-extension cervical radiographs are essential pre-operatively to evaluate for atlantoaxial subluxation before intubation.

Question 5640

Topic: 6. Spine

A 16-year-old patient with JIA controlled on Etanercept is scheduled for a posterior spinal fusion for scoliosis. According to current perioperative guidelines, how should this biologic medication be managed?

. Continue the medication uninterrupted through the surgical period
. Stop 1 week prior to surgery and restart immediately post-operatively
. Withhold the medication for 1 to 2 dosing cycles prior to surgery and restart after complete wound healing
. Increase the dose post-operatively to prevent disease flare
. Switch to high-dose oral methotrexate perioperatively

Correct Answer & Explanation

. Withhold the medication for 1 to 2 dosing cycles prior to surgery and restart after complete wound healing


Explanation

To minimize infection risk, biologic agents like Etanercept (a TNF inhibitor) should be withheld for 1-2 dosing intervals prior to major orthopedic surgery. They are typically restarted once the surgical wound has completely healed.