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6. Spine MCQs

Practice Set 53 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 1041

Topic: 6. Spine

An infant presents with idiopathic scoliosis. Which of the following factors indicates observation as the treatment of choice:

. Age younger than 1 year
. Rib-vertebral angle difference >20°
. Rib-vertebra phase 2
. C urve of 47°
. Left thoracic curve

Correct Answer & Explanation

. Rib-vertebral angle difference >20°

Explanation

Rib-vertebral angle difference >20° rib-vertebra phase 2, and curves >45° indicate a likely progression. The majority of infantile curves are left thoracic, so this is not a factor. A patient younger than 1 year of age has a higher chance of spontaneous resolution than an older patient. Therefore, absent other risk factors, observation is the initial recommend treatment.

Question 1042

Topic: Thoracolumbar Spine & Deformity

Which of the following factors predicts a lesser degree of impairment of pulmonary function in patients with adolescent idiopathic scoliosis:

. Increased C obb angle
. Decreased number of vertebrae in the curve
. Increased cephalad apex of the curve
. Decreased thoracic kyphosis
. Pectus excavatum in addition to the scoliosis

Correct Answer & Explanation

. Decreased number of vertebrae in the curve

Explanation

An increased Cobb angle, increased cephalad apex of the curve, decreased thoracic kyphosis, and pectus excavatum in addition to the scoliosis are factors that predict a greater degree of pulmonary impairment. A lower number of vertebrae in the curve signals less impairment.

Question 1043

Topic: 6. Spine
All except which of the following neurologic/muscular disorders can present undiagnosed in a patient with scoliosis:
. Friedreich's ataxia
. Charcot-Marie-Tooth disease
. Syringomyelia
. Duchenne muscular dystrophy
. Spinal cord tumor

Correct Answer & Explanation

. Friedreich's ataxia

Explanation

Scoliosis can be caused by any neurologic disorder that affects trunk balance. Scoliosis is seen in most patients with Friedreich's ataxia, a disorder in which patients present with an ataxic gait in preadolescence or adolescence. The curve becomes significant at about the same time as the ataxia. Scoliosis can also be seen in 10% of patients with Charcot-Marie-Tooth disease. This condition is characterized by cavus feet, intrinsic atrophy, and occasional hip dysplasia; no significant pain or clumsiness is present. Scoliosis is seen in many patients with syringomyelia. The syrinx and scoliosis both develop silently with no noticeable weakness until both the syrinx and the curve are significant. Spinal cord tumor may present with scoliosis in an ambulatory patient. Duchenne muscular dystrophy leads to scoliosis in most patients, but the patients become nonambulatory several years before the curve develops.

Question 1044

Topic: 6. Spine

A newborn is evaluated for multiple congenital joint dislocations involving the knees, hips, and elbows. Physical examination reveals spatulate thumbs, a depressed nasal bridge, and a prominent forehead. What is the most critical initial imaging study to obtain in this patient?

. Echocardiogram
. Renal ultrasound
. Cervical spine radiographs
. Cranial ultrasound
. MRI of the lumbar spine

Correct Answer & Explanation

. Cervical spine radiographs

Explanation

The patient has Larsen syndrome, which presents with multiple joint dislocations and characteristic spatulate thumbs. Cervical spine kyphosis and instability are common and can lead to severe neurologic compromise, making cervical radiographs critical.

Question 1045

Topic: Cervical Spine

A 6-year-old child presents with severe disproportionate short-trunk dwarfism, corneal clouding, and normal intelligence. Radiographs show universal platyspondyly and hypoplasia of the odontoid. The patient is deficient in which of the following enzymes?

. Alpha-L-iduronidase
. Iduronate sulfatase
. Galactosamine-6-sulfatase
. Glucocerebrosidase
. Sphingomyelinase

Correct Answer & Explanation

. Galactosamine-6-sulfatase

Explanation

Morquio syndrome (MPS IV) is characterized by normal intelligence, severe skeletal dysplasia, and odontoid hypoplasia predisposing to atlantoaxial instability. It is caused by a deficiency in the enzyme galactosamine-6-sulfatase.

Question 1046

Topic: 6. Spine

A 6-year-old boy presents with short stature, normal intelligence, knock-knees, and a short trunk. Radiographs reveal platyspondyly with central anterior beaking of the vertebral bodies. He is at greatest risk for which of the following potentially life-threatening orthopedic complications?

. Atlantoaxial instability
. Slipped capital femoral epiphysis
. Congenital pseudarthrosis of the clavicle
. Pathologic fractures of the femur
. Progressive restrictive thoracic scoliosis

Correct Answer & Explanation

. Atlantoaxial instability

Explanation

Morquio syndrome (MPS IV) is characterized by normal intelligence, platyspondyly with central anterior beaking, and severe atlantoaxial instability due to odontoid hypoplasia. These patients require careful cervical spine evaluation before any anesthesia or sports participation.

Question 1047

Topic: 6. Spine

A 9-year-old child presents with a waddling gait and bilateral knee pain. Radiographs reveal delayed, irregular ossification of the femoral heads and other epiphyses, but the spine appears completely normal.

Which of the following is true regarding this condition?

. It is typically inherited in an autosomal recessive manner
. The spine usually shows severe platyspondyly and anterior beaking
. It is most commonly caused by a mutation in the COMP gene
. Patients typically have severe intellectual disability
. It results from defective type I collagen synthesis

Correct Answer & Explanation

. It is most commonly caused by a mutation in the COMP gene

Explanation

Multiple Epiphyseal Dysplasia (MED) is characterized by irregular epiphyses with a normal spine (differentiating it from SED). The most common genetic cause is an autosomal dominant mutation in the COMP (Cartilage Oligomeric Matrix Protein) gene.

Question 1048

Topic: 6. Spine

A 12-year-old boy presents with bilateral knee pain and a waddling gait. Radiographs show delayed, irregular ossification of the epiphyses and a "double-layer" patella on the lateral view. Which of the following best describes this condition?

. It is an autosomal recessive disorder of type I collagen
. It is caused by a mutation in the COMP gene
. It typically presents with severe dwarfism and craniofacial anomalies
. It is associated with highly elevated alkaline phosphatase
. It commonly involves severe spinal deformities

Correct Answer & Explanation

. It is caused by a mutation in the COMP gene

Explanation

Multiple Epiphyseal Dysplasia (MED) can be caused by mutations in the COMP gene or type IX collagen. A "double-layer" patella is a classic pathognomonic radiographic sign, and spinal involvement is typically absent or very mild.

Question 1049

Topic: 6. Spine

A 6-year-old child with severe short-trunk dwarfism, a barrel chest, and cleft palate is diagnosed with spondyloepiphyseal dysplasia congenita. Which of the following orthopedic complications requires meticulous monitoring in this patient?

. Anterolateral tibial bowing
. Atlantoaxial instability
. Spondylolisthesis at L5-S1
. Madelung deformity
. Tarsal coalition

Correct Answer & Explanation

. Atlantoaxial instability

Explanation

SED congenita is a type II collagenopathy (COL2A1 mutation). Patients have a high incidence of odontoid hypoplasia leading to atlantoaxial instability, which requires careful monitoring to prevent catastrophic neurologic injury.

Question 1050

Topic: 6. Spine

A neonate presents with short-limbed dwarfism, rigid clubfeet, swelling of the pinnae ("cauliflower ears"), and abducted thumbs. What is the most common spinal deformity associated with this condition?

. Congenital muscular torticollis
. Atlantoaxial rotatory subluxation
. Severe rigid kyphoscoliosis
. Lumbar hyperlordosis
. Spondyloptosis

Correct Answer & Explanation

. Severe rigid kyphoscoliosis

Explanation

Diastrophic dysplasia (SLC26A2 mutation) presents with "hitchhiker" thumbs, cauliflower ears, and rigid clubfeet. The most common and challenging spinal deformity is progressive, severe rigid kyphoscoliosis, which often requires surgical intervention.

Question 1051

Topic: 6. Spine

A newborn presents with bilateral knee dislocations, bilateral hip dislocations, and clubfeet. The face exhibits a depressed nasal bridge and prominent forehead. What critical radiographic evaluation must be performed before any manipulative casting or surgery?

. Ultrasound of the kidneys
. Echocardiogram
. Cervical spine radiographs
. MRI of the brain
. Pelvic radiograph

Correct Answer & Explanation

. Cervical spine radiographs

Explanation

Larsen syndrome (FLNB mutation) is characterized by multiple joint dislocations and distinct facial features. Cervical kyphosis and instability are extremely common and can be lethal if unrecognized, making cervical spine radiographs mandatory before any manipulation.

Question 1052

Topic: 6. Spine

A 12-year-old boy with Duchenne muscular dystrophy has developed a 50-degree scoliotic curve. His forced vital capacity (FVC) is currently 40% of predicted. What is the recommended management?

. Observation until the curve reaches 70 degrees
. Rigid thoracolumbosacral orthosis (TLSO)
. Posterior spinal fusion extended to the pelvis
. Anterior spinal fusion only
. Insertion of vertical expandable prosthetic titanium ribs (VEPTR)

Correct Answer & Explanation

. Posterior spinal fusion extended to the pelvis

Explanation

Scoliosis in Duchenne muscular dystrophy is highly progressive and does not respond to bracing, which can further restrict pulmonary function. Early posterior spinal fusion to the pelvis is indicated when the curve exceeds 20-30 degrees and before pulmonary function severely declines.

Question 1053

Topic: 6. Spine

A 5-year-old child with achondroplasia presents with newly developed central sleep apnea, hyperreflexia, and clonus.

What is the most likely anatomical etiology of these neurological symptoms?

. Progressive thoracolumbar kyphosis
. Lumbar spinal stenosis
. Foramen magnum stenosis
. Atlantoaxial instability
. Tethered cord syndrome

Correct Answer & Explanation

. Foramen magnum stenosis

Explanation

Children with achondroplasia have abnormal endochondral ossification, which can lead to a narrowed foramen magnum. This stenosis can compress the cervicomedullary junction, resulting in central sleep apnea, hyperreflexia, sudden death, or high cervical myelopathy.

Question 1054

Topic: 6. Spine

Sprengel deformity is characterized by a high-riding, dysplastic scapula. It is often associated with an omovertebral bone or fibrous band. The omovertebral connection typically attaches from the cervical spine to which part of the scapula?

. Acromion
. Coracoid process
. Superomedial angle
. Inferior angle
. Glenoid neck

Correct Answer & Explanation

. Superomedial angle

Explanation

In Sprengel deformity, the omovertebral bone or fibrous connection typically runs from the spinous processes or lamina of the lower cervical vertebrae to the superomedial angle of the elevated scapula. Surgical resection of this connection (e.g., Woodward procedure) is often required for correction.

Question 1055

Topic: Thoracolumbar Spine & Deformity

A 15-year-old girl is experiencing progressive weakness. She is unable to abduct her arms above 100° (pic). She has a progressive lordosis. Her facial expression is flat, and she cannot smile. Her mother has the same constellation of findings. No scoliosis is present. She and her mother are able to walk independently. The most likely diagnosis is:

. Parsonage-Turner syndrome
. Friedreich ataxia
. Facioscapulohumeral dystrophy
. Bells palsy
. Nemaline rod myopathy

Correct Answer & Explanation

. Facioscapulohumeral dystrophy

Explanation

This patient most likely has facioscapulohumeral dystrophy. With a frequency of 1:20,000, it is a rare disorder inherited in an autosomal- dominant fashion. The genetic abnormality is found on chromosome 4, with a decreased number of D4Z4 tandem repeats, but this does not appear to code for a protein product. Scoliosis is not present. In this condition, selective weakness of the serratus anterior, trapezius, and rhomboid muscles is present. Therefore, the scapula is not effectively stabilized against the trunk during use.

Question 1056

Topic: 6. Spine

An 8-year-old girl with no history of fever, illness, or weakness presents with an increasing spinal deformity. She was born in Asia. The patient is neurologically normal. Based on radiographs (Slide 1) and magnetic resonance images (Slide 2), the most likely diagnosis is:

. Pyogenic spondylitis
. Tuberculous spondylitis
. C ompression fractures
. C ongenital kyphosis
. Scheuermann kyphosis

Correct Answer & Explanation

. C ongenital kyphosis

Explanation

This patient has congenital kyphosis. The relative disk destruction with vertebral preservation argues against tuberculosis. The anterior bony wedging is atypical for remote bacterial infection. Anterior fusion is not seen in patients with Scheuermann kyphosis (rarely after maturity) or in patients with compression fractures.

Question 1057

Topic: Thoracolumbar Spine & Deformity

Which of the following is not a common finding in patients with Prader- Willi syndrome:

. Short stature
. Developmental delay
. Scoliosis
. Osteopenia
. Arachnodactyly

Correct Answer & Explanation

. Arachnodactyly

Explanation

Prader-Willi syndrome is characterized by early hypotonia, short stature, severe obesity, developmental delay, scoliosis, and osteopenia.

Question 1058

Topic: 6. Spine

A 2-year-old child with Spinal Muscular Atrophy (SMA) type II presents for scoliosis evaluation. Which of the following medical therapies has recently revolutionized the treatment of SMA by modifying the SMN2 gene splicing to produce functional SMN protein?

. Eteplirsen
. Nusinersen
. Deflazacort
. Atalauren
. Riluzole

Correct Answer & Explanation

. Nusinersen

Explanation

Nusinersen (Spinraza) is an antisense oligonucleotide that alters the splicing of SMN2 pre-mRNA. This increases the production of fully functional SMN protein, significantly improving motor function in SMA patients.

Question 1059

Topic: 6. Spine

A 2-year-old child with achondroplasia presents with delayed motor milestones, hypotonia, and sleep apnea. Which of the following is the most critical anatomical area to evaluate immediately?

. Thoracolumbar junction
. Cervical spine for atlantoaxial instability
. Foramen magnum
. Lumbosacral spine for stenosis
. Genu varum

Correct Answer & Explanation

. Foramen magnum

Explanation

Foramen magnum stenosis is a life-threatening complication in infants and young children with achondroplasia. It can cause cervicomedullary compression, leading to central sleep apnea, hypotonia, and sudden death.

Question 1060

Topic: 6. Spine

A 9-year-old boy with Duchenne muscular dystrophy is referred for progressive neuromuscular scoliosis. At what point in the disease progression is posterior spinal fusion typically indicated?

. When the curve exceeds 15 degrees
. When the curve exceeds 20-30 degrees in a non-ambulatory patient
. Only when the patient requires continuous mechanical ventilation
. When forced vital capacity (FVC) drops below 20%
. Immediately upon loss of ambulation, regardless of curve size

Correct Answer & Explanation

. When the curve exceeds 20-30 degrees in a non-ambulatory patient

Explanation

In DMD, scoliosis usually progresses rapidly after wheelchair dependence. Spinal fusion is indicated for curves >20-30 degrees in non-ambulatory patients, ideally performed while the FVC is still >35% to minimize pulmonary complications.

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