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6. Spine MCQs

Practice Set 290 of 379

This practice set contains high-yield board review questions covering key concepts in 6. Spine. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.

Question 5781

Topic: 6. Spine

A 6-year-old boy presents with mid-back pain. Radiographs reveal a "coin lesion" (vertebra plana) at T7 without posterior element involvement. Neurological exam is completely normal. What is the most appropriate initial management?

. Urgent surgical decompression and fusion
. Core needle biopsy and systemic chemotherapy
. Observation and extension bracing
. Intralesional corticosteroid injection
. Radiation therapy

Correct Answer & Explanation

. Observation and extension bracing

Explanation

Vertebra plana in a child without neurological deficit is classically associated with eosinophilic granuloma (Langerhans Cell Histiocytosis). It is typically self-limiting, and observation with bracing often leads to partial reconstitution of vertebral height.

Question 5782

Topic: 6. Spine

A 6-year-old boy presents with back pain and a normal neurologic exam. Radiographs reveal complete collapse of the T8 vertebral body (vertebra plana). Laboratory studies are within normal limits. Which of the following is the most appropriate initial management?

. Anterior corpectomy and fusion
. Posterior spinal fusion without decompression
. Systemic chemotherapy
. Observation and bracing
. Radiation therapy

Correct Answer & Explanation

. Observation and bracing

Explanation

Solitary vertebra plana in a child is strongly suggestive of Langerhans cell histiocytosis (eosinophilic granuloma). In neurologically intact patients, it is a self-limiting process that often reconstitutes over time, making observation with or without an extension brace the treatment of choice.

Question 5783

Topic: 6. Spine
A 6-year-old girl presents with mid-back pain. Radiographs of the thoracic spine demonstrate severe, uniform collapse of the T7 vertebral body with preservation of the adjacent disc spaces. A diagnosis of eosinophilic granuloma is suspected. What is the most appropriate initial management for this spinal lesion without neurological deficits?
. Anterior corpectomy and fusion
. Posterior spinal fusion with instrumentation
. Intralesional curettage and bone grafting
. Observation and symptomatic care with or without a brace
. Emergent radiation therapy

Correct Answer & Explanation

. Observation and symptomatic care with or without a brace

Explanation

Eosinophilic granuloma often presents as vertebra plana (Calvรฉ disease) in children. Without neurological compromise or significant instability, observation and conservative care is the standard, as the lesion typically heals and the vertebral body often reconstitutes.

Question 5784

Topic: Thoracolumbar Spine & Deformity

A newborn presents with short limbs, severe rigid clubfeet, a "hitchhiker" thumb, and cystic swelling of the pinnae. Cervical kyphosis is noted on radiographs. What is the underlying defective mechanism?

. Mutation in the fibroblast growth factor receptor 3
. Defect in a sulfate transport protein
. Mutation in the cartilage oligomeric matrix protein
. Defective synthesis of type X collagen
. Deficiency in carbonic anhydrase II

Correct Answer & Explanation

. Defect in a sulfate transport protein

Explanation

Diastrophic dysplasia is caused by a mutation in the SLC26A2 gene, resulting in defective intracellular sulfate transport. Clinical hallmarks include hitchhiker thumbs, severe clubfeet, and cauliflower ears.

Question 5785

Topic: 6. Spine

A 6-year-old with short stature, corneal clouding, and normal intelligence presents with progressive cervical myelopathy. Radiographs reveal generalized platyspondyly and severe odontoid hypoplasia. Urine analysis will most likely show elevated levels of which glycosaminoglycan?

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

Correct Answer & Explanation

. Keratan sulfate

Explanation

Morquio syndrome (Mucopolysaccharidosis Type IV) involves a deficiency of N-acetylgalactosamine-6-sulfatase (GALNS), leading to the accumulation of keratan sulfate. Odontoid hypoplasia causing cervical instability is a critical orthopedic manifestation.

Question 5786

Topic: 6. Spine

A 35-year-old male with achondroplasia presents with progressive neurogenic claudication and lower extremity weakness. What is the primary anatomic cause of spinal stenosis in this condition?

. Ligamentum flavum hypertrophy and massive facet joint cysts
. Decreased interpedicular distance and severely shortened pedicles
. High-grade isthmic spondylolisthesis at L4-L5
. Progressive vertebral body wedging and kyphosis
. Epidural lipomatosis secondary to obesity

Correct Answer & Explanation

. Decreased interpedicular distance and severely shortened pedicles

Explanation

Spinal stenosis in achondroplasia is primarily a bony developmental issue characterized by congenitally short pedicles and a progressive decrease in the interpedicular distance from the upper to lower lumbar spine.

Question 5787

Topic: 6. Spine

A 7-year-old child with a short trunk, coxa vara, and severe myopia requires surgical clearance for strabismus correction. Which preoperative imaging is most critical for the anesthesiologist?

. AP pelvis radiograph
. Flexion-extension lateral cervical spine radiographs
. Chest radiograph
. MRI of the lumbar spine
. Transthoracic echocardiogram

Correct Answer & Explanation

. Flexion-extension lateral cervical spine radiographs

Explanation

Spondyloepiphyseal dysplasia congenita (SEDC) involves defects in type II collagen (COL2A1) and is highly associated with odontoid hypoplasia. Flexion-extension cervical radiographs are critical to rule out atlantoaxial instability prior to intubation.

Question 5788

Topic: 6. Spine

A 6-year-old child presents with short-trunk dwarfism, corneal clouding, and severe genu valgum. Urine tests are positive for keratan sulfate. What is the most life-threatening orthopedic complication associated with this condition?

. Severe scoliosis causing cor pulmonale
. Atlantoaxial instability due to odontoid hypoplasia
. Malignant transformation of osteochondromas
. Premature closure of the triradiate cartilage
. Avascular necrosis of the femoral head

Correct Answer & Explanation

. Atlantoaxial instability due to odontoid hypoplasia

Explanation

Morquio syndrome (Mucopolysaccharidosis Type IV) is characterized by the accumulation of keratan sulfate. Odontoid hypoplasia is a hallmark finding, leading to severe atlantoaxial instability and the risk of fatal cervical myelopathy.

Question 5789

Topic: 6. Spine

A 14-year-old male presents with bilateral knee and hip pain. Radiographs show flattened and irregular epiphyses. A diagnosis of Multiple Epiphyseal Dysplasia (MED) is suspected over Spondyloepiphyseal Dysplasia (SED). Which radiographic finding strongly supports MED over SED?

. Severe platyspondyly
. Odontoid hypoplasia
. A normal appearance of the spine
. Coronal clefts in the vertebral bodies
. Anterior beaking of the lumbar vertebrae

Correct Answer & Explanation

. A normal appearance of the spine

Explanation

Multiple Epiphyseal Dysplasia (MED) and Spondyloepiphyseal Dysplasia (SED) both present with irregular epiphyses. However, MED selectively affects the epiphyses of long bones while largely sparing the spine (normal spine appearance), whereas SED prominently involves the spine (e.g., platyspondyly).

Question 5790

Topic: 6. Spine

A 7-year-old boy with Spondyloepiphyseal Dysplasia Congenita (SEDC) is scheduled for elective bilateral hip osteotomies. Due to his underlying condition, which of the following pre-operative evaluations is most critical?

. Echocardiogram to rule out aortic root dilation
. Renal ultrasound to evaluate for polycystic kidneys
. Pulmonary function tests to assess for severe restrictive lung disease
. Flexion-extension cervical spine radiographs to evaluate for atlantoaxial instability
. DEXA scan to assess bone mineral density

Correct Answer & Explanation

. Flexion-extension cervical spine radiographs to evaluate for atlantoaxial instability

Explanation

SEDC is a Type II collagenopathy (COL2A1 mutation) frequently associated with odontoid hypoplasia, which can lead to life-threatening atlantoaxial instability. Flexion-extension C-spine radiographs are mandatory prior to any procedure requiring intubation or general anesthesia.

Question 5791

Topic: 6. Spine

A 35-year-old male with achondroplasia presents with progressive neurogenic claudication and bilateral lower extremity weakness. What is the primary anatomical cause of spinal stenosis in patients with this condition?

. Massive hypertrophy of the ligamentum flavum
. Severe degenerative spondylolisthesis
. Shortened pedicles and decreased interpedicular distance
. Ossification of the posterior longitudinal ligament (OPLL)
. Congenital fusion of the lumbar vertebral bodies

Correct Answer & Explanation

. Shortened pedicles and decreased interpedicular distance

Explanation

In achondroplasia, defective endochondral ossification affects the neural arch development, leading to congenitally short pedicles and a progressive decrease in interpedicular distance from the upper to the lower lumbar spine, directly causing severe spinal stenosis.

Question 5792

Topic: Thoracolumbar Spine & Deformity

A 2-year-old child presents with coarse facial features, severe thoracolumbar kyphosis (gibbus deformity), corneal clouding, and hepatosplenomegaly. Urine analysis shows elevated levels of both dermatan sulfate and heparan sulfate. Which enzyme is deficient in this patient?

. Iduronate-2-sulfatase
. Alpha-L-iduronidase
. Heparan N-sulfatase
. Beta-galactosidase
. Arylsulfatase B

Correct Answer & Explanation

. Alpha-L-iduronidase

Explanation

Hurler Syndrome (Mucopolysaccharidosis Type I) is caused by a deficiency in alpha-L-iduronidase, leading to the accumulation of dermatan and heparan sulfate. Clinically, it is differentiated from Hunter syndrome (MPS II) by the presence of corneal clouding.

Question 5793

Topic: Thoracolumbar Spine & Deformity

A newborn presents with short-limbed dwarfism, hitchhiker thumbs, cauliflower ears, and severe rigid clubfeet. What is the most critical orthopedic complication that must be monitored closely during early infancy in this condition?

. Atlantoaxial rotatory subluxation
. Progressive cervical kyphosis
. Thoracolumbar scoliosis
. High-grade spondylolisthesis
. Basilar invagination

Correct Answer & Explanation

. Progressive cervical kyphosis

Explanation

The patient has diastrophic dysplasia (SLC26A2 mutation). Cervical kyphosis is common and can progress to severe spinal cord compression or resolve spontaneously, making close monitoring critical.

Question 5794

Topic: 6. Spine

A 45-year-old male with achondroplasia presents with progressively worsening neurogenic claudication. What is the primary anatomical etiology of spinal stenosis in this patient population?

. Ligamentum flavum hypertrophy
. Decreased interpedicular distance caudally and short pedicles
. Degenerative spondylolisthesis
. Severe facet joint osteoarthrosis
. Central disk herniation

Correct Answer & Explanation

. Decreased interpedicular distance caudally and short pedicles

Explanation

Spinal stenosis in achondroplasia is primarily driven by congenital narrowing of the spinal canal due to shortened pedicles and an abnormal decrease in interpedicular distance from L1 to L5.

Question 5795

Topic: 6. Spine

A 5-year-old child with short-trunk dwarfism, coxa vara, and myopia requires surgery for an umbilical hernia. Based on the likely diagnosis, what critical screening must be performed prior to general anesthesia?

. Echocardiogram to rule out aortic root dilation
. Flexion-extension cervical spine radiographs
. Pulmonary function tests
. Renal ultrasound
. MRI of the lumbar spine

Correct Answer & Explanation

. Flexion-extension cervical spine radiographs

Explanation

The presentation is classic for Spondyloepiphyseal Dysplasia Congenita (COL2A1 mutation). These patients have odontoid hypoplasia, leading to atlantoaxial instability, necessitating flexion-extension C-spine films before intubation.

Question 5796

Topic: 6. Spine

A 6-year-old boy presents with a short trunk, severe genu valgum, corneal clouding, and normal intelligence. Urine analysis shows elevated keratan sulfate. What specific cervical spine pathology is most characteristic of this condition?

. Foramen magnum stenosis
. Odontoid hypoplasia resulting in C1-C2 instability
. Progressive cervical kyphosis
. Klippel-Feil anomaly
. Occipitoatlantal assimilation

Correct Answer & Explanation

. Odontoid hypoplasia resulting in C1-C2 instability

Explanation

This describes Morquio Syndrome (Mucopolysaccharidosis Type IV). Odontoid hypoplasia is a hallmark of Morquio syndrome, creating a high risk of atlantoaxial instability and catastrophic spinal cord injury.

Question 5797

Topic: 6. Spine

An infant born with multiple large joint dislocations (bilateral knees, hips, and elbows), a depressed nasal bridge, and spatulate thumbs is diagnosed with Larsen syndrome. Which spinal abnormality must be urgently evaluated to prevent catastrophic myelopathy?

. Atlantoaxial rotatory subluxation
. Progressive cervical kyphosis
. Rapidly progressive thoracic scoliosis
. Lumbar hyperlordosis
. Diastematomyelia

Correct Answer & Explanation

. Progressive cervical kyphosis

Explanation

Patients with Larsen syndrome (FLNB mutation) have a high incidence of severe, progressive cervical kyphosis. This can lead to fatal or severely morbid spinal cord compression if not identified and treated early.

Question 5798

Topic: 6. Spine

Based on the lateral spine radiograph of this 17-year-old male patient presenting with short trunk dwarfism, what is the characteristic vertebral deformity shown?



. Bullet-shaped vertebrae
. Picture-frame vertebrae
. Champagne-bottle shaped vertebrae
. Rugger-jersey spine
. Bamboo spine

Correct Answer & Explanation

. Champagne-bottle shaped vertebrae

Explanation

Correct Answer: Champagne-bottle shaped vertebraeThe radiograph demonstrates the typical 'champagne-bottle' shaped vertebral bodies, which are a hallmark radiographic finding in Spondyloepiphyseal Dysplasia Tarda and Congenita. Progressive dorsolumbar kyphosis and platyspondyly are also commonly observed.

Question 5799

Topic: 6. Spine

A 6-year-old child with Spondyloepiphyseal Dysplasia Congenita is scheduled for elective lower extremity surgery. Which of the following preoperative evaluations is most critical to prevent a catastrophic neurological complication during anesthesia induction and intubation?

. Echocardiogram to assess for aortic root dilation
. Pulmonary function tests to assess restrictive lung disease
. Flexion-extension radiographs of the cervical spine
. MRI of the lumbar spine to rule out tethered cord
. Renal ultrasound to check for congenital anomalies

Correct Answer & Explanation

. Flexion-extension radiographs of the cervical spine

Explanation

Correct Answer: Flexion-extension radiographs of the cervical spinePatients with Spondyloepiphyseal Dysplasia Congenita commonly have an os odontoideum with or without atlantoaxial instability. Preoperative flexion-extension cervical spine radiographs are critical to evaluate for instability before intubation to prevent iatrogenic spinal cord injury.

Question 5800

Topic: 6. Spine

A 5-year-old boy diagnosed with Spondyloepiphyseal Dysplasia Congenita is being monitored in the orthopedic clinic. Which of the following spinal deformities is most likely to progress as he ages, requiring close observation?

. Cervical lordosis
. Dorsolumbar kyphosis
. Lumbar hyperlordosis
. Idiopathic-like thoracic scoliosis
. Spondylolisthesis at L5-S1

Correct Answer & Explanation

. Dorsolumbar kyphosis

Explanation

Correct Answer: Dorsolumbar kyphosisProgressive dorsolumbar kyphosis with platyspondyly and deformed vertebrae is a characteristic and progressive spinal deformity in patients with Spondyloepiphyseal Dysplasia Congenita, often requiring close clinical and radiographic monitoring.

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