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Question 441

Topic: Cervical Spine

A 62-year-old female with long-standing rheumatoid arthritis presents with neck pain and mild upper extremity clumsiness. Lateral flexion-extension radiographs of the cervical spine demonstrate atlantoaxial subluxation (AAS). Which of the following radiographic measurements is widely considered an absolute indication for surgical stabilization?

. Anterior atlantodental interval (ADI) of > 3 mm
. Posterior atlantodental interval (PADI) < 14 mm
. Anterior atlantodental interval (ADI) of > 5 mm
. Posterior atlantodental interval (PADI) > 18 mm
. C2-C3 subluxation of 2 mm

Correct Answer & Explanation

. Posterior atlantodental interval (PADI) < 14 mm


Explanation

In rheumatoid arthritis, the posterior atlantodental interval (PADI), also known as the space available for the cord (SAC), is the most reliable predictor of neurologic compromise and recovery. A PADI of less than 14 mm indicates critical spinal canal narrowing and is a strong indication for surgical stabilization, even if neurological symptoms are mild.

Question 442

Topic: Cervical Spine

In a patient with severe rheumatoid arthritis, which of the following cervical spine radiographic measurements is the most reliable predictor of impending neurologic deficit?

. Anterior atlanto-dens interval (ADI) greater than 3.5 mm
. Powers ratio greater than 1.0
. McGregor's line violation by the odontoid
. Posterior atlanto-dens interval (PADI) less than 14 mm
. Ranawat line measurement of 15 mm

Correct Answer & Explanation

. Posterior atlanto-dens interval (PADI) less than 14 mm


Explanation

The posterior atlanto-dens interval (PADI), also known as the space available for the cord (SAC), is the most reliable predictor of neurologic paralysis in rheumatoid atlantoaxial subluxation. A PADI of less than 14 mm strongly correlates with neurologic compromise.

Question 443

Topic: Cervical Spine

A 65-year-old male presents with deteriorating handwriting, frequent tripping, and hyperreflexia in all extremities. The Hoffmann sign is positive bilaterally. Which surgical approach is most appropriate for a patient with 4-level cervical spondylotic myelopathy, neutral sagittal alignment, and no significant neck pain?

. Anterior cervical discectomy and fusion (ACDF)
. Cervical corpectomy and fusion
. Posterior cervical laminectomy and fusion
. Cervical disc arthroplasty
. Posterior cervical laminoplasty

Correct Answer & Explanation

. Posterior cervical laminoplasty


Explanation

Posterior cervical laminoplasty is ideal for multi-level cervical spondylotic myelopathy in the setting of neutral or lordotic sagittal alignment with minimal axial neck pain. It preserves motion and avoids the morbidity of multi-level anterior surgery or posterior fusion.

Question 444

Topic: Cervical Spine

A 65-year-old female with long-standing rheumatoid arthritis presents with neck pain and mild hand clumsiness. Flexion-extension radiographs reveal 8 mm of atlantoaxial instability. What is the most critical radiographic parameter to evaluate her risk of impending neurologic deterioration?

. Anterior atlantodental interval (ADI)
. Posterior atlantodental interval (PADI)
. Ranawat line
. McGregor's line
. Wackenheim's clivus baseline

Correct Answer & Explanation

. Posterior atlantodental interval (PADI)


Explanation

The Posterior Atlantodental Interval (PADI), also known as the Space Available for the Cord (SAC), is the most reliable predictor of neurologic deficit in rheumatoid atlantoaxial subluxation. A PADI < 14 mm is a strong indication for surgical stabilization to prevent permanent neurologic deficit.

Question 445

Topic: Cervical Spine

A 45-year-old male sustains a burst fracture of the atlas (Jefferson fracture). Which of the following radiographic findings dictates the need for rigid stabilization (e.g., halo-vest or surgery) due to transverse ligament rupture?

. Combined lateral mass overhang of > 2 mm on open-mouth odontoid view
. Combined lateral mass overhang of > 6.9 mm on open-mouth odontoid view
. Prevertebral soft tissue swelling > 5 mm at C2
. Atlantodental interval (ADI) of 2 mm on lateral radiograph
. Loss of cervical lordosis on lateral radiograph

Correct Answer & Explanation

. Combined lateral mass overhang of > 6.9 mm on open-mouth odontoid view


Explanation

A combined lateral mass displacement (overhang) of greater than 6.9 mm on an open-mouth odontoid radiograph indicates rupture of the transverse alar ligament (Spence's rule). This implies significant C1-C2 instability.

Question 446

Topic: Cervical Spine

A 45-year-old male presents after a diving accident. Radiographs reveal a C1 burst fracture. An open mouth odontoid view demonstrates lateral displacement of the C1 lateral masses. A displacement greater than 6.9 mm indicates rupture of which of the following structures?

. Alar ligament
. Apical ligament
. Transverse ligament
. Tectorial membrane
. Ligamentum flavum

Correct Answer & Explanation

. Transverse ligament


Explanation

The Rule of Spence states that if the combined overhang of the C1 lateral masses on C2 is greater than 6.9 mm on an AP open-mouth radiograph, it implies a rupture of the transverse ligament. This indicates instability and may require surgical stabilization.

Question 447

Topic: Cervical Spine

A 25-year-old male presents to the emergency department after a diving accident. He is awake, alert, and cooperative. Examination reveals weakness in his hands and intrinsic muscles, but intact lower extremity function. Imaging shows a bilateral C6-C7 jumped facet. What is the most appropriate next step in management?

. Perform awake closed reduction with cranial skeletal traction
. Obtain an urgent MRI before attempting any reduction maneuvers
. Proceed immediately to posterior open reduction and stabilization
. Proceed immediately to anterior cervical discectomy and fusion
. Place the patient in a rigid cervical collar and observe for 24 hours

Correct Answer & Explanation

. Perform awake closed reduction with cranial skeletal traction


Explanation

In an awake, alert, and examinable patient with cervical facet dislocations, emergent closed reduction with skeletal traction is indicated and safe without a prior MRI. MRI is required prior to reduction primarily in comatose or unexaminable patients to rule out an extruded disc.

Question 448

Topic: Cervical Spine

A 5-year-old child presents with short-trunk dwarfism, significant genu valgum, and normal intelligence. Radiographs reveal platyspondyly and hypoplasia of the odontoid process. Urine testing shows elevated keratan sulfate. Which enzyme is most likely deficient?

. Alpha-L-iduronidase
. Galactosamine-6-sulfatase
. Iduronate sulfatase
. Arylsulfatase B
. Glucocerebrosidase

Correct Answer & Explanation

. Galactosamine-6-sulfatase


Explanation

Morquio syndrome (Mucopolysaccharidosis Type IV) is caused by a deficiency in galactosamine-6-sulfatase (Type IVA) or beta-galactosidase (Type IVB). Odontoid hypoplasia is a critical finding that necessitates early cervical spine evaluation for instability.

Question 449

Topic: Cervical Spine

A 6-year-old boy presents with a disproportionately short trunk, severe coxa vara, and an abnormal waddling gait. Cervical spine radiographs reveal odontoid hypoplasia. Genetic testing would most likely reveal a mutation affecting which of the following?

. Type I collagen
. Type II collagen
. Cartilage Oligomeric Matrix Protein
. Fibroblast Growth Factor Receptor 3
. Sulfate transporter

Correct Answer & Explanation

. Type II collagen


Explanation

Spondyloepiphyseal dysplasia congenita (SEDC) is caused by mutations in the COL2A1 gene, which encodes type II collagen. Affected patients frequently suffer from atlantoaxial instability secondary to odontoid hypoplasia.

Question 450

Topic: Cervical Spine

A 7-year-old girl with short stature, corneal clouding, and normal intelligence presents for evaluation. Radiographs show severe platyspondyly and hypoplasia of the odontoid. She is found to be deficient in which of the following enzymes?

. Alpha-L-iduronidase
. Iduronate sulfatase
. Galactose-6-sulfatase
. Arylsulfatase B
. Cathepsin K

Correct Answer & Explanation

. Galactose-6-sulfatase


Explanation

Morquio syndrome (MPS IV) is caused by a deficiency in galactose-6-sulfatase (Type A). Unlike Hurler or Hunter syndromes, patients with Morquio syndrome typically maintain normal intelligence but suffer from severe skeletal issues like cervical instability.

Question 451

Topic: Cervical Spine

A 6-year-old boy presents with short stature, severe genu valgum, and corneal clouding, but displays normal intelligence. Radiographs reveal generalized platyspondyly and marked odontoid hypoplasia. Which of the following is the specific enzyme deficient in this patient's condition?

. Alpha-L-iduronidase
. Iduronate-2-sulfatase
. N-acetylgalactosamine-6-sulfatase
. Arylsulfatase B
. Heparan N-sulfatase

Correct Answer & Explanation

. N-acetylgalactosamine-6-sulfatase


Explanation

This patient has Morquio syndrome A (MPS IV A), characterized by normal intelligence, severe skeletal dysplasia, and profound cervical instability due to odontoid hypoplasia. It is caused by a deficiency in N-acetylgalactosamine-6-sulfatase.

Question 452

Topic: Cervical Spine

A 6-year-old girl with Morquio syndrome (MPS IV) presents with progressive weakness and clumsiness in her hands. Cervical spine flexion-extension radiographs demonstrate severe atlantoaxial instability. What is the primary anatomic cause of this instability in Morquio syndrome?

. Odontoid hypoplasia combined with ligamentous laxity
. Odontoid hypoplasia combined with severe ligamentous hypertrophy
. Congenital absence of the transverse ligament
. Os odontoideum with normal ligamentous tension
. Anterior wedging of the C2 vertebral body

Correct Answer & Explanation

. Odontoid hypoplasia combined with ligamentous laxity


Explanation

Atlantoaxial instability in Morquio syndrome (MPS IV) is driven by a combination of severe odontoid hypoplasia and profound ligamentous laxity. This places patients at high risk for fatal cervicomedullary compression if not addressed with surgical fusion.

Question 453

Topic: Cervical Spine

A 6-year-old child presents with severe disproportionate short-trunk dwarfism, knock-knees, and normal intelligence. Radiographs reveal platyspondyly and odontoid hypoplasia. Laboratory analysis confirms a diagnosis of Morquio syndrome (MPS IV). Which of the following is the deficient enzyme in Type A of this disorder?

. Alpha-L-iduronidase
. Galactosamine-6-sulfatase
. Heparan N-sulfatase
. Arylsulfatase B
. Iduronate-2-sulfatase

Correct Answer & Explanation

. Galactosamine-6-sulfatase


Explanation

Morquio syndrome Type A is an autosomal recessive mucopolysaccharidosis caused by a deficiency in galactosamine-6-sulfatase. Unlike most other MPS types, intelligence is preserved, but severe skeletal deformities and atlantoaxial instability are hallmarks.

Question 454

Topic: Cervical Spine

A 6-year-old girl presents with short-trunk dwarfism, severe genu valgum, and generalized joint hyperlaxity. She has normal intelligence but exhibits corneal clouding. Cervical spine radiographs reveal odontoid hypoplasia with significant atlantoaxial instability.

Which of the following glycosaminoglycans abnormally accumulates in this patient's specific syndrome?

. Heparan sulfate and Dermatan sulfate
. Heparan sulfate only
. Keratan sulfate
. Chondroitin-6-sulfate
. Hyaluronic acid

Correct Answer & Explanation

. Keratan sulfate


Explanation

The patient's presentation of short-trunk dwarfism, normal intelligence, joint hyperlaxity, and odontoid hypoplasia is classic for Morquio syndrome (Mucopolysaccharidosis Type IV). This condition is caused by a deficiency in galactosamine-6-sulfatase or beta-galactosidase, leading to the specific accumulation of keratan sulfate.

Question 455

Topic: Cervical Spine

In a patient with advanced rheumatoid arthritis evaluating the cervical spine, which radiographic parameter indicates the highest risk for progressive neurologic deficit and dictates the need for surgical stabilization?

. Anterior atlantodental interval (ADI) of 3mm
. Posterior atlantodental interval (PADI) of less than 14mm
. Subaxial subluxation of 2mm
. Basilar invagination with Clark station 1
. Isolated loss of normal cervical lordosis

Correct Answer & Explanation

. Posterior atlantodental interval (PADI) of less than 14mm


Explanation

The PADI (space available for the cord) is the most reliable predictor of neurologic recovery and risk. A PADI of less than 14 mm is a strong indication for surgical intervention in rheumatoid atlantoaxial subluxation.

Question 456

Topic: Cervical Spine

A 60-year-old woman with long-standing rheumatoid arthritis presents with new-onset myelopathy. Flexion-extension cervical radiographs reveal an atlantodental interval (ADI) of 10 mm. Which ligament's failure is primarily responsible for this radiographic finding?

. Apical ligament
. Alar ligament
. Transverse ligament
. Posterior longitudinal ligament
. Ligamentum flavum

Correct Answer & Explanation

. Transverse ligament


Explanation

Atlantoaxial subluxation in rheumatoid arthritis is primarily due to the destruction and laxity of the transverse ligament. This is caused by the invasive synovial pannus at the atlanto-odontoid joint.

Question 457

Topic: Cervical Spine

In patients with mucopolysaccharidoses (MPS), upper cervical instability is a well-documented risk. The pathogenesis of odontoid hypoplasia and subsequent atlantoaxial instability in Morquio syndrome (MPS IV) is primarily due to the accumulation of which of the following?

. Dermatan sulfate
. Heparan sulfate
. Keratan sulfate
. Hyaluronic acid
. Chondroitin-4-sulfate

Correct Answer & Explanation

. Keratan sulfate


Explanation

Morquio syndrome (MPS IV) is caused by a defect in keratan sulfate degradation, leading to its accumulation in tissues. This disrupts normal chondrogenesis, particularly affecting the odontoid process and leading to hypoplasia and severe C1-C2 ligamentous instability.

Question 458

Topic: Cervical Spine

A 6-year-old child presents with extreme ligamentous laxity, short stature, and progressive severe kyphoscoliosis. Urine analysis reveals elevated levels of keratan sulfate. Radiographs demonstrate universal platyspondyly with anterior central beaking. Which of the following is an absolute surgical indication in this disorder?

. Progressive genu valgum
. Odontoid hypoplasia with neurological deficit
. Development of corneal clouding
. Subluxation of the radial head
. Progressive hearing loss

Correct Answer & Explanation

. Odontoid hypoplasia with neurological deficit


Explanation

Morquio syndrome (MPS IV) is characterized by keratan sulfate accumulation and extreme ligamentous laxity. Odontoid hypoplasia with atlantoaxial instability is a hallmark; any associated neurologic deficit warrants urgent cervical spine fusion.

Question 459

Topic: Cervical Spine

A 25-year-old male sustains a traumatic spondylolisthesis of the axis (Hangman's fracture) following an MVC. Imaging shows a bilateral pars fracture with 4 mm of translation and 15 degrees of angulation. According to the Levine and Edwards classification, what is the most appropriate initial management?

. Rigid cervical collar alone
. Halo vest immobilization following gentle traction
. Anterior cervical discectomy and fusion (ACDF)
. Posterior C1-C3 instrumented fusion
. Open reduction and internal fixation of the C2 pars

Correct Answer & Explanation

. Halo vest immobilization following gentle traction


Explanation

This describes a Type II Hangman's fracture (>3 mm translation, >11 degrees angulation) caused by hyperextension and axial loading followed by severe flexion. The initial management is gentle traction to reduce the deformity, followed by halo vest immobilization.

Question 460

Topic: Cervical Spine

Both Multiple Epiphyseal Dysplasia (Fairbank type) and Pseudoachondroplasia can be caused by mutations in the Cartilage Oligomeric Matrix Protein (COMP) gene. Which of the following clinical or radiographic features best distinguishes Pseudoachondroplasia from Multiple Epiphyseal Dysplasia?

. Presence of a normal facial appearance
. Severe metaphyseal irregularities and flaring
. Autosomal dominant inheritance pattern
. Development of early-onset osteoarthritis
. Presence of odontoid hypoplasia

Correct Answer & Explanation

. Severe metaphyseal irregularities and flaring


Explanation

Correct Answer: BWhile both MED and Pseudoachondroplasia can result from COMP gene mutations, Pseudoachondroplasia is generally more severe. The key distinguishing feature is that Pseudoachondroplasia involves significant metaphyseal irregularities, flaring, and shortening, in addition to epiphyseal dysplasia. MED is predominantly confined to the epiphyses with minimal to no metaphyseal involvement. Both conditions typically have a normal facial appearance (Option A), distinguishing them from true achondroplasia. Both are autosomal dominant (Option C) and lead to early osteoarthritis (Option D).