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Question 921

Topic: Cervical Spine

A 7-year-old boy presents with severe neck pain and a 'cock robin' head tilt one week after undergoing a tonsillectomy. He resists any passive neck movement. Radiographs show a unilateral anterior displacement of the lateral mass of C1 on C2. What is the most likely diagnosis?

. Klippel-Feil syndrome
. Grisel syndrome
. Juvenile idiopathic arthritis
. Odontoid fracture
. Cervical osteomyelitis

Correct Answer & Explanation

. Grisel syndrome


Explanation

Grisel syndrome is a non-traumatic atlantoaxial rotatory subluxation associated with inflammatory conditions of the upper respiratory tract or recent ENT surgery. The 'cock robin' head position is a classic clinical finding.

Question 922

Topic: 6. Spine

A 5-year-old child with a mucopolysaccharidosis presents with decreasing exercise tolerance. Examination reveals short stature, knock-knees, and corneal clouding. Which of the following cervical spine abnormalities is most characteristic of this specific syndrome?

. Basilar invagination
. Odontoid hypoplasia and atlantoaxial instability
. Congenital cervical block vertebrae
. Os odontoideum
. Cervical kyphosis

Correct Answer & Explanation

. Odontoid hypoplasia and atlantoaxial instability


Explanation

Morquio syndrome (MPS IV) is highly associated with odontoid hypoplasia and ligamentous laxity, leading to atlantoaxial instability. This requires careful screening with flexion-extension radiographs to prevent catastrophic spinal cord injury.

Question 923

Topic: 6. Spine

A 6-month-old infant with achondroplasia presents with central apnea, failure to thrive, and hyperreflexia. What is the most likely underlying pathophysiology for these neurological findings?

. Atlantoaxial rotatory fixation
. Thoracolumbar kyphosis
. Foramen magnum stenosis
. Lumbar spinal stenosis
. Tethered spinal cord

Correct Answer & Explanation

. Foramen magnum stenosis


Explanation

Infants with achondroplasia are at high risk for foramen magnum stenosis, which can compress the cervicomedullary junction. This can present with central apnea, myelopathy, and sudden death, requiring urgent decompression.

Question 924

Topic: 6. Spine

A 15-year-old boy presents with back pain and a rigid thoracic kyphosis. Lateral standing radiographs demonstrate a thoracic kyphosis of 55 degrees. Which of the following radiographic findings is required to confirm the diagnosis of Scheuermann's disease?

. Anterior wedging of at least 5 degrees in 3 consecutive vertebrae
. Schmorl nodes in at least 2 consecutive vertebrae
. Loss of disc height in the lumbar spine
. Vertebra plana
. A single hemivertebra at the apex of the curve

Correct Answer & Explanation

. Anterior wedging of at least 5 degrees in 3 consecutive vertebrae


Explanation

The classic Sorensen criteria for Scheuermann kyphosis include anterior wedging of greater than or equal to 5 degrees in three or more consecutive vertebral bodies. Associated findings can include Schmorl nodes and endplate irregularities.

Question 925

Topic: 6. Spine

A 6-month-old infant is diagnosed with an infantile idiopathic scoliosis. The curve is left-sided and measures 30 degrees. The rib-vertebra angle difference (RVAD) of Mehta is measured. Which RVAD value most strongly predicts that the curve is progressive rather than resolving?

. Less than 10 degrees
. 10 to 15 degrees
. Greater than 20 degrees
. 0 degrees
. Negative value

Correct Answer & Explanation

. Greater than 20 degrees


Explanation

A Mehta Rib-Vertebral Angle Difference (RVAD) of greater than 20 degrees strongly predicts curve progression in infantile idiopathic scoliosis. An RVAD less than 20 degrees is generally associated with spontaneous resolution.

Question 926

Topic: 6. Spine

A 9-year-old girl with congenital scoliosis presents for preoperative planning before posterior spinal fusion. MRI reveals a split spinal cord separated by a bony spur at T10. What is the most appropriate management regarding the bony spur?

. Resection of the spur concurrently or prior to deformity correction
. Observation and standard scoliosis correction
. Resection of the spur only if neuro monitoring changes during correction
. Prophylactic laminectomy without spur excision
. In situ spinal fusion without deformity correction

Correct Answer & Explanation

. Resection of the spur concurrently or prior to deformity correction


Explanation

Diastematomyelia acts as a spinal cord tether. The bony or cartilaginous spur must be resected and the dura repaired, typically before or concurrently with deformity correction, to prevent neurologic injury during spinal straightening.

Question 927

Topic: 6. Spine

A 14-year-old boy with Marfan syndrome presents with a 45-degree thoracic scoliotic curve. He is Risser 1. Which of the following best describes the expected response to brace treatment and a common associated spinal anomaly?

. Excellent response to bracing; high risk for spondylolisthesis
. Poor response to bracing; high risk for dural ectasia
. Excellent response to bracing; high risk for syringomyelia
. Poor response to bracing; high risk for os odontoideum
. Moderate response to bracing; high risk for basilar invagination

Correct Answer & Explanation

. Poor response to bracing; high risk for dural ectasia


Explanation

Patients with Marfan syndrome tend to have a poor response to orthotic management for scoliosis and curves often progress to surgery. They have a high incidence of dural ectasia, which can complicate surgical intervention and cause chronic back pain.

Question 928

Topic: Thoracolumbar Spine & Deformity

A 13-year-old female presents with severe back pain, hamstring tightness, and a waddling gait. Radiographs show a Grade IV isthmic spondylolisthesis of L5 on S1. Which of the following physical examination findings is most characteristic of this condition?

. Positive Trendelenburg test
. Phalen sign
. Heart-shaped pelvis
. Palpable step-off and pelvic retroversion
. Absent patellar reflexes

Correct Answer & Explanation

. Palpable step-off and pelvic retroversion


Explanation

High-grade spondylolisthesis typically presents with hamstring tightness, a crouched or waddling gait, pelvic retroversion (a vertical sacrum), and a palpable step-off at the lumbosacral junction.

Question 929

Topic: 6. Spine

A 12-year-old boy with Duchenne muscular dystrophy has a rapidly progressive scoliosis measuring 40 degrees. He became wheelchair-bound 6 months ago. His forced vital capacity (FVC) is currently 45% of predicted. What is the most appropriate management?

. Rigid TLSO bracing
. Observation until the curve reaches 60 degrees
. Posterior spinal fusion to the pelvis
. Anterior spinal fusion
. Growing rod insertion

Correct Answer & Explanation

. Posterior spinal fusion to the pelvis


Explanation

In Duchenne muscular dystrophy, once a patient is wheelchair-bound and curves progress beyond 20-30 degrees, posterior spinal fusion to the pelvis is indicated. Surgery should be performed before the FVC drops below 30% to minimize severe pulmonary complications.

Question 930

Topic: 6. Spine

A 7-year-old boy with Spinal Muscular Atrophy (SMA) Type 2 presents with a severe, collapsing 70-degree thoracolumbar scoliosis. He is a non-ambulator. What is the most frequent major complication of untreated severe spinal deformity in this patient population?

. Spastic quadriparesis
. Cor pulmonale and severe restrictive lung disease
. Spontaneous vertebral fractures
. Syringomyelia
. Cauda equina syndrome

Correct Answer & Explanation

. Cor pulmonale and severe restrictive lung disease


Explanation

In SMA, collapsing scoliotic deformities lead to severe restrictive lung disease. The combination of respiratory muscle weakness and progressive thoracic deformity frequently leads to respiratory failure and cor pulmonale.

Question 931

Topic: Thoracolumbar Spine & Deformity

A 16-year-old male gymnast presents with a 3-month history of axial low back pain exacerbated by extension. Plain radiographs are normal. What is the most sensitive imaging modality to detect an early, acute stress reaction of the pars interarticularis?

. CT scan
. MRI with STIR sequences
. Bone scintigraphy
. Dynamic flexion-extension radiographs
. Ultrasound

Correct Answer & Explanation

. MRI with STIR sequences


Explanation

MRI with fluid-sensitive sequences (STIR or T2 fat-suppressed) is highly sensitive for detecting marrow edema indicative of an early/acute pars stress reaction. This detects the pathology before a frank fracture line is visible on CT.

Question 932

Topic: 6. Spine

A 14-year-old boy with spastic quadriplegic cerebral palsy presents with a 70-degree sweeping neuromuscular scoliosis and pelvic obliquity. The right hemipelvis is elevated, and he has a dislocated right hip. If surgical intervention is planned for both the hip and spine, what is the generally accepted staging sequence?

. Spinal fusion first, then hip reconstruction
. Hip reconstruction first, then spinal fusion
. Simultaneous hip reconstruction and spinal fusion only
. Hip arthrodesis followed by spinal tethering
. Spinal fusion only; hip dislocations in CP are never treated surgically

Correct Answer & Explanation

. Spinal fusion first, then hip reconstruction


Explanation

Severe pelvic obliquity from neuromuscular scoliosis alters the mechanical alignment of the acetabulum. Spinal fusion to the pelvis to correct obliquity is generally performed first to provide a stable foundation, decreasing the risk of recurrent hip dislocation post-reconstruction.

Question 933

Topic: 6. Spine
A 15-year-old with Osteogenesis Imperfecta Type III presents with progressive headaches, lower cranial nerve dysfunction, and hyperreflexia. Radiographs of the cervical spine demonstrate upward migration of the odontoid process above the Chamberlain line. What is the diagnosis?
. Atlantoaxial rotatory subluxation
. Basilar invagination
. Os odontoideum
. Cervical kyphosis
. Odontoid fracture

Correct Answer & Explanation

. Basilar invagination


Explanation

Basilar invagination is a recognized complication in severe Osteogenesis Imperfecta due to softening of the skull base, leading to upward migration of the odontoid. It can cause brainstem compression and lower cranial nerve palsies.

Question 934

Topic: 6. Spine

A 4-year-old boy presents with progressive cavovarus foot deformity on the right side and increasing clumsiness. Examination reveals an asymmetric patch of hair over the lumbosacral region and hyperreflexia in the right lower extremity. What is the most appropriate initial diagnostic study?

. EMG/NCS of the right lower extremity
. CT scan of the lumbar spine
. MRI of the entire neural axis
. Radiographs of the right foot
. Muscle biopsy

Correct Answer & Explanation

. MRI of the entire neural axis


Explanation

The combination of a progressive foot deformity, a cutaneous lumbosacral stigma (hairy patch), and upper motor neuron signs strongly suggests a tethered cord syndrome. An MRI of the spine is the gold standard diagnostic test.

Question 935

Topic: Thoracolumbar Spine & Deformity

A major indication for surgical decompression of an L1 burst fracture is:

. Loss of anterior body height of 60%
. Retropulsion of canal fragments to 50% of canal size
. Kyphosis of 15°
. Post-void residual of 450 mL
. Presence of a posterior lamina fracture

Correct Answer & Explanation

. Post-void residual of 450 mL


Explanation

Generalized treatment algorithms for burst fractures involving upper lumbar spine have relative indications for surgery that include 50% loss of height, 25% of kyphosis, and 50% canal compromise. Absolute indications for decompression include neurological deficits including a potential conus injury. Post-void residual of > 450 mL is suggestive of sacral root injury at the level of conus. Bradford suggests that anterior decompression of this injury has favorable outcome with frequent resolution or improvement of symptoms.

Question 936

Topic: 6. Spine

An injury associated with a type 1 fracture of the odontoid is:

. Concomitant fracture of the body at C 2
. Burst fracture of the lumbar spine
. Atlanto-occipital dislocation
. Rupture of the transverse ligament
. Associated Jefferson fracture of the ring of C 1

Correct Answer & Explanation

. Atlanto-occipital dislocation


Explanation

Type 1 fractures are a rare entity. They are frequently treated with immobilization with a hard collar if isolated. There have been numerous reports in the literature of a type 1 fracture of the odontoid being associated with an atlanto-occipital dislocation, and this injury must be suspected. The potential for missing atlanto-occipital dislocation may lead to a fatal outcome.

Question 937

Topic: Thoracolumbar Spine & Deformity

A type 3 traumatic spondylolisthesis of the axis, as classified by Levine and Edwards, is best treated with which of the following:

. Soft collar immobilization
. Hard Philadelphia cervical orthosis
. Halo vest immobilization
. Open reduction and operative posterior stabilization
. Gardner-Wells tongs application and awake reduction, then posterior stabilization

Correct Answer & Explanation

. Open reduction and operative posterior stabilization


Explanation

The Levine classification of traumatic spondylolisthesis or Hangman fractures involving C 2 in the type 3 injury has a combined bilateral facet dislocation at C 2-C 3 as well as the traumatic spondylolisthesis of the axis. Closed reduction could not be performed secondary to the traumatic spondylolisthesis at the C 2 isthmus.

Question 938

Topic: 6. Spine

A 35-year-old man presents 3 years after a motor vehicular trauma. It is now 3 years following operative stabilization of the spine at C 7. He complained of mild weakness in his right upper extremity at the biceps level and has corresponding parasthesias in the right thumb. The next step in the evaluation of this patient is:

. Anteroposterior lateral flexion extension radiographs of the cervical spine
. Computerized tomography scan of the cervical spine
. Magnetic resonance imaging of the cervical spine
. Physical therapy with range of motion and strengthening exercises of both upper extremities
. Anti-inflammatory medication for presumed tendonitis

Correct Answer & Explanation

. Magnetic resonance imaging of the cervical spine


Explanation

The patient is a 35-year old man has been stable since his injury. The most important evaluation for this individual would be magnetic resonance imaging to rule out potential cervical cord syrinx that has occurred given new onset weakness and sensory changes proximal to his injury.

Question 939

Topic: 6. Spine

A 55-year-old man with ankylosing spondylitis has a minor fall and is suffering with neck pain. Anteroposterior and lateral radiographs are negative with no evidence of fracture. He has no neurologic loss and has normal strength with the exception of severe restricted motion. Twelve hours following injury, he is found to have bilateral bicep and tricep weakness. The appropriate management and the work up of this individual is:

. Computerized tomography (CT) anteroposterior lateral radiographs of the cervical spine
. CT scan of the cervical spine
. Magnetic resonance imaging (MRI) of the cervical spine
. Bone scan of the MRI
. Electromyogram to better delineate all the nerve neuropathy

Correct Answer & Explanation

. Magnetic resonance imaging (MRI) of the cervical spine


Explanation

The patient is within 12 hours of having normal cervical spine films. Approximately one third of patients with ankylosing spondylitis incur occult injuries to the cervical spine that are not identified by plain films prior to kyphotic progression. A bone scan would delineate a fracture after 72 hours. However, the presence of progressive weakness should raise suspicion of a potential epidural hematoma. For this reason, magnetic resonance imaging would better delineate epidural hematoma.

Question 940

Topic: 6. Spine
A 2-year-old boy with a congenital heart anomaly has a 40° thoracolumbar curvature. Standing posteroanterior and lateral radiographs reveal vertebral anomalies indicative of congenital scoliosis. Which of the following patterns of congenital scoliosis has the worst prognosis for progression?
. Block vertebrae
. Unilateral unsegmented bar
. Fully segmented hemivertebra
. Unilateral unsegmented bar with a contralateral fully segmented hemivertebra
. Nonsegmented hemivertebra

Correct Answer & Explanation

. Unilateral unsegmented bar with a contralateral fully segmented hemivertebra


Explanation

Congenital spinal deformity is caused by structural abnormalities in the vertebrae that can result in asymmetric growth, such as scoliosis or kyphosis. It has been classified in 2 types. Type I involves defects of formation and type II involves defects of segmentation. However, in many instances, deformities can be a mixture of both. Defects of formation include segmented or unsegmented hemivertebrae and wedge vertebrae. Defects of segmentation include block vertebrae, unilateral bars, or unilateral bars with hemivertebrae. The potential for progression is dependent on the growth potential of the anomalies. The presence of healthy-appearing disks between the hemivertebra and its normal counterparts indicates good growth potential and risk for progression. A unilateral bar on the opposite side of a segmented hemivertebra acts as a tether on the concave side of the curve and has the most likelihood for progression. Children with congenital scoliosis also have a significant incidence of associated anomalies, both intraspinal and other organ systems. About 30% have a spinal dysraphism such as diastematomyelia, meningocele or lipoma. Other associated anomalies include Klippel-Feil syndrome (25%), genitourinary tract abnormalities (30%), cardiac defects (12%), and Sprengel's deformity (10%).