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Question 7341

Topic: 6. Spine

Which of the following factors is considered in the Spinal Instability Neoplastic Score (SINS) to determine the need for surgical stabilization in spinal metastases?

. Primary tumor histology
. Presence of mechanical pain
. Patient's estimated life expectancy
. Tumor response to previous radiotherapy
. Presence of concurrent visceral metastases

Correct Answer & Explanation

. Presence of mechanical pain


Explanation

The SINS criteria evaluate six parameters: spinal location, pain (mechanical vs non-mechanical), bone lesion type (lytic/blastic), radiographic spinal alignment, vertebral body collapse, and posterolateral involvement. Mechanical pain is heavily weighted as a marker of instability.

Question 7342

Topic: 6. Spine

A 4-year-old girl is diagnosed with oligoarticular Juvenile Idiopathic Arthritis (JIA) affecting her right knee. She is ANA positive and Rheumatoid Factor negative. What is the most critical screening protocol she requires to prevent a severe complication?

. Annual echocardiogram
. Slit-lamp examination every 3-4 months
. Pulmonary function testing every 6 months
. Radiographs of the cervical spine annually
. Monthly monitoring of liver function tests

Correct Answer & Explanation

. Slit-lamp examination every 3-4 months


Explanation

Children with oligoarticular JIA who are ANA positive are at the highest risk for asymptomatic anterior uveitis. They require frequent slit-lamp examinations (typically every 3-4 months) to prevent permanent vision loss.

Question 7343

Topic: 6. Spine

A 35-year-old immigrant presents with back pain, night sweats, and lower extremity weakness. MRI of the thoracic spine demonstrates destruction of the anterior vertebral bodies of T8 and T9 with a large paraspinal abscess, while the intervening disc space is relatively preserved. What is the most likely causative organism?

. Staphylococcus aureus
. Mycobacterium tuberculosis
. Pseudomonas aeruginosa
. Brucella melitensis
. Salmonella typhi

Correct Answer & Explanation

. Mycobacterium tuberculosis


Explanation

Tuberculous spondylitis (Pott disease) typically involves the anterior aspects of two adjacent vertebral bodies and often spares the intervertebral disc until late in the disease process. A large, calcified paraspinal (cold) abscess is also highly characteristic.

Question 7344

Topic: 6. Spine

A 55-year-old patient with end-stage renal disease presents with diffuse bone pain. Radiographs of the spine demonstrate alternating bands of sclerosis and lucency parallel to the vertebral endplates. What is the primary pathophysiologic driver of this specific radiographic finding?

. Excessive unmineralized osteoid accumulation
. Inability to excrete serum calcium
. Secondary hyperparathyroidism stimulating osteosclerosis
. Aluminum toxicity from dialysate
. Beta-2 microglobulin amyloid deposition

Correct Answer & Explanation

. Secondary hyperparathyroidism stimulating osteosclerosis


Explanation

The "rugger jersey spine" is characteristic of renal osteodystrophy. It is caused by secondary hyperparathyroidism, where excess PTH stimulates osteoblastic activity leading to bands of osteosclerosis adjacent to the vertebral endplates.

Question 7345

Topic: 6. Spine

A 15-year-old boy with disproportionate short stature, frontal bossing, and rhizomelic shortening presents with severe leg pain and numbness after walking short distances. Genetic testing confirms a mutation in FGFR3. Which of the following is the most likely anatomic cause of his current symptoms?

. Foramen magnum stenosis
. Lumbar spinal stenosis due to decreased interpedicular distance
. Atlantoaxial instability
. Thoracic kyphosis
. Tethered cord syndrome

Correct Answer & Explanation

. Lumbar spinal stenosis due to decreased interpedicular distance


Explanation

Achondroplasia is caused by an FGFR3 mutation leading to short pedicles and decreased interpedicular distance in the lumbar spine. This classically results in claudication and spinal stenosis in adolescence or adulthood.

Question 7346

Topic: 6. Spine

A 6-year-old boy with a disproportionately short trunk, coxa vara, and barrel chest is diagnosed with spondyloepiphyseal dysplasia congenita (SEDC). Before he undergoes surgical correction of his coxa vara, which of the following screening examinations is most critical?

. Echocardiogram
. Renal ultrasound
. Flexion-extension cervical spine radiographs
. Pulmonary function tests
. DEXA scan

Correct Answer & Explanation

. Flexion-extension cervical spine radiographs


Explanation

Spondyloepiphyseal dysplasia congenita (SEDC) is a type II collagenopathy associated with odontoid hypoplasia and atlantoaxial instability. Preoperative cervical spine screening is critical to prevent catastrophic neurologic injury during intubation.

Question 7347

Topic: 6. Spine

A 5-year-old child with short-trunk dwarfism and severe myopia presents with a waddling gait. Radiographs show flattened vertebral bodies and delayed ossification of the capital femoral epiphyses. Prior to undergoing general anesthesia, what imaging study is most critical?

. Echocardiogram
. Cervical spine flexion-extension radiographs
. MRI of the lumbar spine
. CT of the temporal bones
. Dual-energy x-ray absorptiometry

Correct Answer & Explanation

. Cervical spine flexion-extension radiographs


Explanation

Spondyloepiphyseal Dysplasia (SED) Congenita is a type II collagenopathy (COL2A1) frequently associated with odontoid hypoplasia and atlantoaxial instability. Flexion-extension cervical spine radiographs are essential before general anesthesia to prevent catastrophic spinal cord injury.

Question 7348

Topic: 6. Spine

A 55-year-old female with end-stage renal disease presents with diffuse bone pain. Radiographs of her spine demonstrate sclerotic bands at the superior and inferior endplates of the vertebral bodies. Which of the following lab profiles is most consistent with her condition?

. Low calcium, low phosphorus, low PTH
. Low calcium, high phosphorus, high PTH
. High calcium, low phosphorus, high PTH
. Normal calcium, normal phosphorus, normal PTH
. High calcium, high phosphorus, low PTH

Correct Answer & Explanation

. Low calcium, high phosphorus, high PTH


Explanation

Renal osteodystrophy typically causes secondary hyperparathyroidism due to phosphate retention and renal failure to produce 1,25-OH vitamin D. This results in hypocalcemia, hyperphosphatemia, and elevated PTH, causing the classic "rugger jersey" spine appearance.

Question 7349

Topic: 6. Spine

A 4-year-old child with short-trunk dwarfism, severe myopia, and coxa vara is scheduled for a surgical procedure requiring general anesthesia. To prevent a catastrophic neurologic complication during intubation, which abnormality must be ruled out?

. Cervical facet ankylosis
. Odontoid hypoplasia causing atlantoaxial instability
. Foramen magnum stenosis
. Basilar invagination
. Subaxial cervical kyphosis

Correct Answer & Explanation

. Odontoid hypoplasia causing atlantoaxial instability


Explanation

Spondyloepiphyseal dysplasia congenita affects type II collagen and causes short-trunk dwarfism. Patients frequently have odontoid hypoplasia leading to atlantoaxial instability, strictly requiring cervical flexion-extension films before anesthesia.

Question 7350

Topic: 6. Spine

Which of the following radiographic findings is typically the earliest and most characteristic manifestation of Juvenile Idiopathic Arthritis in the cervical spine?

. Atlantoaxial rotatory subluxation
. Cervical kyphosis with anterior vertebral wedging
. Erosion of the dens (odontoid process)
. Ossification of the posterior longitudinal ligament (OPLL)
. Apophyseal joint ankylosis beginning at C2-C3

Correct Answer & Explanation

. Apophyseal joint ankylosis beginning at C2-C3


Explanation

The hallmark of JIA in the cervical spine is early ankylosis of the apophyseal (facet) joints, classically beginning at the C2-C3 level. This progressive fusion alters cervical biomechanics and frequently leads to compensatory subaxial subluxation.

Question 7351

Topic: 6. Spine

A 7-year-old child presents with a short trunk, coxa vara, and normal length hands and feet. Radiographs reveal flattened vertebral bodies. What is the most critical anatomical area to evaluate to prevent catastrophic neurologic injury in this patient?

. Thoracic spine
. Cervical spine
. Lumbar spine
. Sacrum
. Skull base

Correct Answer & Explanation

. Cervical spine


Explanation

Spondyloepiphyseal dysplasia congenita (caused by a COL2A1 mutation) is highly associated with odontoid hypoplasia, leading to atlantoaxial instability. Flexion-extension cervical spine radiographs are mandatory before any surgical procedure.

Question 7352

Topic: 6. Spine

A 14-year-old female with long-standing polyarticular JIA presents for preoperative clearance for a total hip arthroplasty. Which of the following cervical spine abnormalities is most characteristic of JIA and poses a significant intubation risk?

. Atlantoaxial rotatory fixation
. Ankylosis of the apophyseal (facet) joints
. Basilar invagination
. Odontoid hypoplasia
. Pseudarthrosis of C1-C2

Correct Answer & Explanation

. Ankylosis of the apophyseal (facet) joints


Explanation

JIA commonly affects the cervical spine, leading to early fusion (ankylosis) of the apophyseal joints, most frequently at C2-C3. This restricts neck extension and complicates airway management during general anesthesia.

Question 7353

Topic: 6. Spine

A 16-year-old female with long-standing polyarticular juvenile idiopathic arthritis is scheduled for a bilateral total hip arthroplasty. Before proceeding with intubation and surgery, which of the following is the most critical radiographic screening to perform?

. Chest radiograph
. Flexion-extension radiographs of the cervical spine
. Pelvic MRI
. Temporomandibular joint CT
. Echocardiogram

Correct Answer & Explanation

. Flexion-extension radiographs of the cervical spine


Explanation

Patients with polyarticular or systemic JIA frequently develop cervical spine involvement, including atlantoaxial instability and subaxial subluxation. Flexion-extension cervical spine radiographs are mandatory before any procedure requiring intubation to prevent catastrophic neurologic injury.

Question 7354

Topic: 6. Spine

A 12-year-old male presents with back pain and is found to have a 35-degree left-sided thoracic scoliotic curve. His neurological examination is normal. What is the most appropriate next step in evaluation?

. Immediate custom TLSO bracing
. Genetic testing for neurofibromatosis
. Total spine MRI
. CT scan of the thoracic spine
. Scoli-score genetic progression assay

Correct Answer & Explanation

. Total spine MRI


Explanation

Atypical scoliosis features, such as a left-sided thoracic curve, pain, or rapid progression, warrant a total spine MRI. This is crucial to rule out an intraspinal anomaly like a syringomyelia, tethered cord, or tumor.

Question 7355

Topic: 6. Spine

A 13-year-old female presents with a 45-degree progressive left thoracic scoliosis. An MRI reveals an Arnold-Chiari I malformation and a large cervical syringomyelia. What is the most appropriate sequencing of treatment?

. Posterior spinal fusion followed by posterior fossa decompression
. Posterior fossa decompression followed by observation of the scoliosis
. Concurrent spinal fusion and posterior fossa decompression
. Thoracolumbosacral orthosis (TLSO) bracing
. Suboccipital craniectomy followed immediately by anterior spinal fusion

Correct Answer & Explanation

. Posterior fossa decompression followed by observation of the scoliosis


Explanation

In patients with scoliosis secondary to an Arnold-Chiari malformation and syringomyelia, the neurosurgical abnormality must be addressed first. Decompression of the posterior fossa often leads to stabilization or improvement of the scoliosis.

Question 7356

Topic: 6. Spine

The pathogenesis of syringomyelia in patients with a Chiari I malformation is best explained by which of the following mechanisms?

. Ischemic necrosis of the central spinal cord
. Altered cerebrospinal fluid hydrodynamics at the foramen magnum
. Autoimmune destruction of myelin in the central canal
. Central canal obliteration by an ependymoma
. Viral infection of the anterior horn cells

Correct Answer & Explanation

. Altered cerebrospinal fluid hydrodynamics at the foramen magnum


Explanation

Chiari I malformations cause cerebellar tonsillar ectopia, obstructing normal CSF flow at the foramen magnum. This altered hydrodynamic pulsatile pressure forces CSF into the central canal of the spinal cord, forming a syrinx.

Question 7357

Topic: 6. Spine

Which of the following clinical features most strongly indicates the need for a total spine MRI to evaluate for syringomyelia or a neural axis abnormality in an adolescent presenting with scoliosis?

. Right thoracic curvature
. Positive family history of idiopathic scoliosis
. A left thoracic curve pattern
. Apical lordosis
. Curve progression of 5 degrees over 1 year

Correct Answer & Explanation

. A left thoracic curve pattern


Explanation

A left thoracic curve is an atypical pattern in adolescent idiopathic scoliosis and carries a higher association with neural axis abnormalities like syringomyelia or Chiari malformation, warranting an MRI.

Question 7358

Topic: 6. Spine

An 8-year-old boy with a 25-degree scoliosis is diagnosed with a large cervical syringomyelia associated with a Chiari I malformation. He undergoes successful suboccipital decompression. What is the most likely natural history of his scoliosis following the neurosurgical intervention?

. Rapid progression requiring immediate spinal fusion
. Stabilization or improvement of the curve
. Complete reversal of the curve to 0 degrees within 3 months
. Progression only if he reaches skeletal maturity
. Unpredictable behavior unlinked to the syrinx decompression

Correct Answer & Explanation

. Stabilization or improvement of the curve


Explanation

In children with syringomyelia-associated scoliosis, neurosurgical decompression of the syrinx (via treating the Chiari malformation) often leads to stabilization or improvement of the scoliotic curve, particularly if the curve is less than 30 degrees at the time of surgery.

Question 7359

Topic: 6. Spine

A 45-year-old man presents with progressive, painless swelling and instability of his right shoulder. He has a history of severe thermal burns on his hands without recalling the pain. What is the most appropriate initial diagnostic step to identify the underlying cause?

. Joint aspiration and culture
. MRI of the cervical spine
. Electromyography of the upper extremity
. CT of the chest
. Genetic testing for COL1A1

Correct Answer & Explanation

. MRI of the cervical spine


Explanation

The patient's presentation of a Charcot (neuropathic) shoulder with loss of pain and temperature sensation is highly suspicious for syringomyelia. An MRI of the cervical spine is the gold standard to visualize the syrinx.

Question 7360

Topic: 6. Spine

A 16-year-old boy is scheduled for surgical intervention of a left thoracic scoliotic curve measuring 45 degrees. Preoperative MRI reveals an Arnold-Chiari type I malformation and a large cervicothoracic syrinx. What is the most appropriate management strategy?

. Immediate posterior spinal fusion
. Anterior release followed by posterior spinal fusion
. Neurosurgical decompression of the posterior fossa prior to scoliosis surgery
. Placement of growing rods to avoid spinal cord stretch
. Bracing for 6 months followed by re-evaluation

Correct Answer & Explanation

. Neurosurgical decompression of the posterior fossa prior to scoliosis surgery


Explanation

In patients with scoliosis secondary to a syrinx and Chiari I malformation, neurosurgical decompression should be performed before deformity correction. This reduces the risk of neurologic injury and may stabilize the curve.