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Question 901

Topic: 6. Spine

A 6-year-old boy with Morquio syndrome (Mucopolysaccharidosis Type IV) presents with progressive weakness and hyperreflexia in all four extremities. Flexion-extension radiographs of the cervical spine will most likely show instability caused by which underlying anomaly?

. Odontoid hypoplasia
. Basilar invagination
. Congenital fusion of C2-C3
. Foramen magnum stenosis
. Absent transverse ligament

Correct Answer & Explanation

. Odontoid hypoplasia


Explanation

Morquio syndrome classically presents with severe ligamentous laxity and odontoid hypoplasia. This combination results in life-threatening atlantoaxial instability, often requiring prophylactic C1-C2 fusion.

Question 902

Topic: 6. Spine

A 13-year-old boy with Duchenne Muscular Dystrophy is wheelchair-bound and presents with a 45-degree neuromuscular scoliosis. To minimize perioperative mortality and respiratory complications, spinal fusion is typically indicated before which of the following pulmonary parameters falls below 35%?

. Functional residual capacity (FRC)
. Forced vital capacity (FVC)
. Forced expiratory volume in 1 second (FEV1)
. Peak expiratory flow rate (PEFR)
. Total lung capacity (TLC)

Correct Answer & Explanation

. Forced vital capacity (FVC)


Explanation

In Duchenne Muscular Dystrophy, a Forced Vital Capacity (FVC) below 30-35% significantly increases the risk of perioperative pulmonary complications. Surgery is ideally performed before respiratory decline reaches this critical threshold.

Question 903

Topic: 6. Spine

A 14-year-old non-ambulatory boy with spastic quadriplegic cerebral palsy presents with an 80-degree neuromuscular scoliosis and severe pelvic obliquity causing sitting imbalance and skin breakdown over the ischium. What is the standard surgical approach for this condition?

. Anterior spinal fusion from T10 to L4
. Posterior spinal fusion from T10 to the pelvis
. Posterior spinal fusion from the upper thoracic spine (T2 or T3) to the pelvis
. Growing rod instrumentation
. Posterior spinal fusion from T4 to L4

Correct Answer & Explanation

. Posterior spinal fusion from the upper thoracic spine (T2 or T3) to the pelvis


Explanation

In severe neuromuscular scoliosis due to CP with significant pelvic obliquity, fusion must cross the lumbosacral junction. The standard surgical treatment is posterior spinal fusion from the upper thoracic spine (T2-T3) to the pelvis to restore sitting balance.

Question 904

Topic: 6. Spine

A 7-year-old boy presents for evaluation of an asymptomatic spinal deformity. Standing radiographs demonstrate a 30-degree left-sided thoracic scoliosis. His neurological exam is entirely normal. Which of the following is the most appropriate next step in management?

. Initiate full-time TLSO bracing immediately
. Obtain a total spine MRI
. Schedule posterior spinal fusion
. Order genetic testing for Marfan syndrome
. Observe with repeat radiographs in 6 months

Correct Answer & Explanation

. Obtain a total spine MRI


Explanation

Atypical curve patterns, such as a left-sided thoracic curve or juvenile onset, carry a high incidence (up to 20%) of intraspinal axis abnormalities like syringomyelia or Chiari malformations. A total spine MRI is strictly indicated.

Question 905

Topic: 6. Spine

A 12-year-old girl presents with severe lower back pain and radiculopathy. Examination reveals a palpable step-off at the lumbosacral junction. Radiographs show a grade 4 L5-S1 dysplastic spondylolisthesis with a dome-shaped sacrum and a high slip angle. What is the safest and most reliable surgical treatment?

. L5 laminectomy alone
. TLSO bracing for 6 months
. In situ posterolateral fusion from L4 to S1
. Complete anatomic reduction and interbody fusion
. Pars interarticularis defect repair

Correct Answer & Explanation

. In situ posterolateral fusion from L4 to S1


Explanation

High-grade dysplastic spondylolisthesis has a significant risk of L5 or sacral nerve root injury with attempted aggressive anatomic reduction. In situ posterior spinal fusion extending from L4 to S1 is considered a safe and effective definitive treatment.

Question 906

Topic: Cervical Spine

A 10-year-old boy presents with neck pain after a minor fall from a trampoline. Lateral cervical spine radiographs show a rounded, corticated bone fragment situated superior to a hypoplastic dens. Flexion-extension views demonstrate 6 mm of anterior translation of C1 on C2. What is the most likely diagnosis?

. Acute Type II odontoid fracture
. Os odontoideum
. Hangman fracture
. Congenital absence of the transverse ligament
. Klippel-Feil anomaly

Correct Answer & Explanation

. Os odontoideum


Explanation

An os odontoideum appears as a smooth, corticated, rounded ossicle separated from a hypoplastic base of the dens, differentiating it from an acute type II fracture which would have irregular, uncorticated margins.

Question 907

Topic: 6. Spine

A 6-year-old girl presents with torticollis 10 days after undergoing a tonsillectomy. Her head is rotated to the right and tilted to the left. She refuses to move her neck due to pain. Neurologic exam is normal. Which of the following is the most likely diagnosis?

. Retropharyngeal abscess
. Atlantoaxial rotatory subluxation
. Congenital muscular torticollis
. Cervical spine osteomyelitis
. Spasmodic torticollis

Correct Answer & Explanation

. Atlantoaxial rotatory subluxation


Explanation

Grisel's syndrome is a non-traumatic atlantoaxial rotatory subluxation associated with inflammation of adjacent head and neck tissues. It typically occurs after upper respiratory infections or ENT procedures like tonsillectomy.

Question 908

Topic: 6. Spine

A 12-year-old boy with known Marfan syndrome presents with a 45-degree right thoracic scoliosis. Which of the following statements regarding the behavior and management of scoliosis in Marfan syndrome is most accurate?

. Bracing is highly successful and should be pursued vigorously
. Curves are typically flexible and correct easily with positioning
. High risk of curve progression exists, and bracing is generally ineffective
. Spinal deformity typically stabilizes after reaching a 30-degree Cobb angle
. Curves are usually isolated lumbar patterns requiring anterior fusion only

Correct Answer & Explanation

. High risk of curve progression exists, and bracing is generally ineffective


Explanation

Scoliosis in Marfan syndrome is known for being rigid, aggressive, and highly resistant to orthotic management. These patients have a high risk of continued progression and frequently require surgical stabilization.

Question 909

Topic: 6. Spine

An 8-year-old boy with Down syndrome presents with decreasing walking endurance and new-onset clumsiness. Neurological examination reveals hyperreflexia and a positive Babinski sign. Flexion-extension cervical radiographs show atlantoaxial instability. Which of the following radiographic measurements is the most critical threshold indicating the need for surgical stabilization?

. Atlanto-dens interval (ADI) greater than 5 mm
. Atlanto-dens interval (ADI) greater than 10 mm
. Space available for the cord (SAC) less than 14 mm
. Powers ratio greater than 1
. Basion-dental interval greater than 12 mm

Correct Answer & Explanation

. Space available for the cord (SAC) less than 14 mm


Explanation

In children with Down syndrome, the Space Available for the Cord (SAC) is the most reliable predictor of neurological risk. A SAC of less than 14 mm is a strong indication for surgical stabilization to prevent irreversible spinal cord injury.

Question 910

Topic: Thoracolumbar Spine & Deformity

A 10-year-old boy with known neurofibromatosis type 1 (NF1) presents with a 45-degree right thoracic scoliosis. Which of the following radiographic findings is most specifically associated with a high risk for rapid, unrelenting curve progression (dystrophic curve)?

. Apical vertebral rotation
. Vertebral scalloping
. Interpedicular narrowing
. Thickened ribs
. Spondylolysis at L5

Correct Answer & Explanation

. Vertebral scalloping


Explanation

Dystrophic curves in NF1 have a high propensity for rapid progression. Radiographic markers include vertebral scalloping (often due to dural ectasia), rib pencilling, spindling of transverse processes, and severe apical rotation.

Question 911

Topic: 6. Spine

A 6-year-old boy with Morquio syndrome (Mucopolysaccharidosis Type IV) is evaluated for decreasing ambulatory function and upper extremity weakness. What is the most likely cervical spine pathology responsible for his presentation?

. Foramen magnum stenosis
. Odontoid hypoplasia causing atlantoaxial instability
. Basilar invagination
. Cervical kyphosis from vertebral wedging
. Congenital cervical facet dislocation

Correct Answer & Explanation

. Odontoid hypoplasia causing atlantoaxial instability


Explanation

Morquio syndrome is classically associated with odontoid hypoplasia and ligamentous laxity, leading to profound C1-C2 instability. This must be monitored closely to prevent cervical myelopathy.

Question 912

Topic: 6. Spine

A 12-year-old boy presents with back pain and a left-sided thoracic scoliosis. Physical examination reveals absent abdominal reflexes on the left side but normal lower extremity strength. What is the most appropriate next step in management?

. Magnetic resonance imaging of the entire spine
. Computed tomography of the chest and spine
. Initiation of full-time TLSO bracing
. Immediate posterior spinal fusion
. Technetium-99m bone scan

Correct Answer & Explanation

. Magnetic resonance imaging of the entire spine


Explanation

Atypical scoliosis (left thoracic curve, presence of pain, or abnormal neurological signs like absent abdominal reflexes) raises suspicion for intraspinal anomalies such as syringomyelia or Chiari malformation. A total spine MRI is strictly indicated.

Question 913

Topic: 6. Spine

A 14-year-old girl with neurofibromatosis type 1 has a sharply angulated, dystrophic thoracic kyphoscoliosis measuring 65 degrees. There are no signs of myelopathy. What is the most reliable surgical strategy to achieve a solid arthrodesis and halt progression?

. Posterior spinal fusion with pedicle screw instrumentation
. In situ posterior fusion without instrumentation
. Anterior release followed by halo-gravity traction
. Combined anterior and posterior spinal fusion
. Growth-friendly distraction-based implants (VEPTR)

Correct Answer & Explanation

. Combined anterior and posterior spinal fusion


Explanation

Dystrophic curves in NF1 are highly unstable and prone to pseudarthrosis and curve progression if treated with posterior fusion alone. A combined anterior and posterior spinal fusion is the gold standard for severe dystrophic kyphoscoliosis.

Question 914

Topic: 6. Spine

An infant presents with multiple large joint dislocations (hips, knees, and elbows) and spatulate thumbs. A diagnosis of Larsen syndrome is suspected. Which of the following evaluations is most critical to perform early to prevent sudden infant death?

. Echocardiogram to rule out aortic root dilation
. Renal ultrasound to rule out agenesis
. Cervical spine radiographs to evaluate for severe kyphosis
. Skeletal survey to rule out osteogenesis imperfecta
. Cranial ultrasound for intraventricular hemorrhage

Correct Answer & Explanation

. Cervical spine radiographs to evaluate for severe kyphosis


Explanation

Larsen syndrome is characterized by multiple joint dislocations and a classic facies. Cervical kyphosis is common and can be rapidly progressive, leading to lethal cervical myelopathy if not identified and stabilized early.

Question 915

Topic: 6. Spine

A 6-month-old boy is diagnosed with infantile idiopathic scoliosis. Radiographs demonstrate a 35-degree left thoracic curve. Which of the following Rib-Vertebra Angle Difference (RVAD or Mehta's angle) measurements most strongly predicts that the curve will be progressive rather than resolving?

. Less than 10 degrees
. Greater than 20 degrees
. Greater than 40 degrees
. Greater than 60 degrees
. Greater than 80 degrees

Correct Answer & Explanation

. Greater than 20 degrees


Explanation

Mehta's Rib-Vertebra Angle Difference (RVAD) is critical in predicting the progression of infantile idiopathic scoliosis. An RVAD greater than 20 degrees generally indicates a progressive curve requiring intervention like serial casting.

Question 916

Topic: 6. Spine

A 15-year-old boy complains of mid-back pain. Lateral spine radiographs demonstrate a rigid thoracic kyphosis of 65 degrees. To formally diagnose classic Scheuermann's disease (type I), the radiographs must show anterior wedging of at least 5 degrees in how many consecutive vertebrae?

. 1 consecutive vertebra
. 2 consecutive vertebrae
. 3 consecutive vertebrae
. 4 consecutive vertebrae
. 5 consecutive vertebrae

Correct Answer & Explanation

. 3 consecutive vertebrae


Explanation

Sorensen's criteria for classic Scheuermann's kyphosis require anterior wedging of greater than 5 degrees in at least three consecutive vertebrae. It is also associated with Schmorl's nodes and endplate irregularities.

Question 917

Topic: 6. Spine

A 10-year-old female with congenital scoliosis is found to have diastematomyelia with a bony spur at T10 on MRI. She is scheduled for surgical deformity correction. How should the diastematomyelia be managed in relation to the deformity surgery?

. Resect the spur after the deformity correction is complete
. Resect the spur and detether the cord prior to or during the same setting as deformity correction
. Leave the spur intact as it provides spinal stability
. Treat with localized radiation prior to spinal fusion
. Perform a bypass fusion extending two levels above and below the spur

Correct Answer & Explanation

. Resect the spur and detether the cord prior to or during the same setting as deformity correction


Explanation

In diastematomyelia (split cord malformation), the cord is tethered by a bony or fibrous spur. To prevent acute neurological deficit during deformity correction, the tethering spur must be resected prior to any corrective spinal maneuvers.

Question 918

Topic: 6. Spine

A 7-year-old boy presents with severe neck pain and a persistent torticollis one week after an uncomplicated tonsillectomy. His head is rotated to the right and tilted to the left. He is afebrile with normal inflammatory markers. What is the most likely diagnosis?

. Cervical discitis
. Epidural abscess
. Atlantoaxial rotatory subluxation (Grisel's syndrome)
. Retropharyngeal abscess
. Klippel-Feil syndrome

Correct Answer & Explanation

. Atlantoaxial rotatory subluxation (Grisel's syndrome)


Explanation

Grisel's syndrome is an inflammatory non-traumatic atlantoaxial rotatory subluxation that typically follows an upper respiratory infection or head/neck surgery, such as a tonsillectomy.

Question 919

Topic: Cervical Spine

A 14-year-old boy is evaluated for neck pain following a minor tackle in football. Cervical radiographs reveal a round, smoothly corticated ossicle with a wide gap separating it from a hypoplastic odontoid peg. The anterior arch of C1 appears hypertrophied. What is the most likely diagnosis?

. Acute Type II odontoid fracture
. Os odontoideum
. Hangman's fracture
. Mach effect
. Persistent terminal ossicle

Correct Answer & Explanation

. Os odontoideum


Explanation

An os odontoideum presents as a smooth, corticated ossicle separate from a hypoplastic dens, often with hypertrophy of the anterior ring of C1. It indicates chronic instability rather than an acute fracture.

Question 920

Topic: 6. Spine

A 5-year-old girl is evaluated for an elevated left scapula and limited left shoulder abduction. Radiographs reveal a bony connection extending from the cervical spine spinous processes to the superomedial border of the scapula. What is the eponym for this specific anatomical structure?

. Klippel bone
. Omovertebral bone
. Coracoclavicular bar
. Cervicoscapular ligament
. Scapulovertebral syndesmosis

Correct Answer & Explanation

. Omovertebral bone


Explanation

An omovertebral bone is a fibrous, cartilaginous, or osseous connection between the cervical spine and the superior angle of the scapula, classically seen in Sprengel's deformity.