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 5821
Topic: 6. Spine
You are examining a newborn diagnosed with Spondyloepiphyseal Dysplasia Congenita. In addition to monitoring the skeletal system and cervical spine, what other specialized screening must be routinely performed throughout this patient's childhood?
Correct Answer & Explanation
. Dilated fundoscopic exams for retinal detachment
Explanation
Severe myopia and a high risk of retinal detachment are classic extraskeletal manifestations of SED Congenita due to Type II collagen defects in the vitreous humor. Routine ophthalmologic screening is essential to prevent blindness.
Question 5822
Topic: 6. Spine
In a 14-year-old male with Spondyloepiphyseal Dysplasia Tarda, what is the most characteristic pathognomonic finding on a lateral radiograph of the lumbar spine?
Correct Answer & Explanation
. A hump-shaped mound of bone on the central/posterior half of the vertebral endplates
Explanation
The pathognomonic radiographic feature of SED Tarda is a hump-shaped mound of bone accumulating on the posterior or central portion of the superior and inferior vertebral endplates.
Question 5823
Topic: 6. Spine
The mutation in Spondyloepiphyseal Dysplasia Congenita impairs the normal structural integrity of hyaline cartilage. Which of the following intervertebral disc components is also primarily composed of this defective protein?
Correct Answer & Explanation
. Nucleus pulposus
Explanation
The nucleus pulposus is rich in Type II collagen, making it highly susceptible to the COL2A1 mutation seen in SED Congenita, contributing to the spinal manifestations of the disease.
Question 5824
Topic: Cervical Spine
Morquio syndrome and Spondyloepiphyseal Dysplasia Congenita both present with short trunk dwarfism and atlantoaxial instability. Which of the following clinical features reliably distinguishes Morquio syndrome from SED Congenita on physical examination?
Correct Answer & Explanation
. Presence of corneal clouding
Explanation
Corneal clouding is a classic feature of Morquio syndrome (a mucopolysaccharidosis), but it is absent in SED Congenita. Both conditions exhibit normal intelligence, short trunk dwarfism, and odontoid hypoplasia.
Question 5825
Topic: 6. Spine
Which of the following represents the most immediately life-threatening complication of Spondyloepiphyseal Dysplasia Congenita in early childhood?
Correct Answer & Explanation
. Cervical myelopathy from atlantoaxial instability
Explanation
Odontoid hypoplasia is very common in SED Congenita, leading to atlantoaxial instability. If unrecognized, this can cause life-threatening cervical myelopathy or sudden death, especially during intubation or trauma.
Question 5826
Topic: 6. Spine
What spinal deformity is most frequently associated with Spondyloepiphyseal Dysplasia Congenita and typically progresses during childhood, often requiring surgical fusion?
Correct Answer & Explanation
. Thoracolumbar kyphoscoliosis
Explanation
Thoracolumbar kyphoscoliosis is a very common spinal deformity in SED Congenita. It frequently progresses throughout childhood due to vertebral dysplasia and ligamentous laxity, often necessitating surgical stabilization.
Question 5827
Topic: 6. Spine
Based on the lateral spine radiograph showing characteristic heaped-up bone on the posterior vertebral endplates, a 14-year-old boy is diagnosed with SED Tarda.
What is the inheritance pattern for the most common form of this disease?
Correct Answer & Explanation
. X-linked recessive
Explanation
The image describes SED Tarda, classically showing posterior humping of the vertebral bodies. The most common genetic variant (TRAPPC2 mutation) is inherited in an X-linked recessive pattern.
Question 5828
Topic: 6. Spine
A 5-year-old child with spondyloepiphyseal dysplasia congenita (SEDC) is scheduled to undergo a proximal femoral valgus osteotomy for severe coxa vara. Which of the following preoperative evaluations is absolutely critical before proceeding with general anesthesia?
Correct Answer & Explanation
. Flexion-extension radiographs of the cervical spine
Explanation
Patients with SEDC have a high incidence of odontoid hypoplasia, which can lead to atlantoaxial instability. Flexion-extension cervical spine radiographs are mandatory prior to any procedure requiring intubation or neck manipulation to avoid catastrophic spinal cord injury.
Question 5829
Topic: 6. Spine
A 10-year-old girl with Spondyloepiphyseal Dysplasia Congenita presents with increasing lower extremity weakness, hyperreflexia, and a positive Babinski sign. Cervical radiographs reveal an atlantodens interval (ADI) of 8 mm. What is the primary pathoanatomic etiology of her cervical instability?
Correct Answer & Explanation
. Odontoid hypoplasia
Explanation
Atlantoaxial instability in SEDC is primarily due to odontoid hypoplasia (a manifestation of the epiphyseal dysplasia), rather than purely ligamentous laxity as seen in Down syndrome. Symptomatic myelopathy with an ADI >5 mm requires posterior C1-C2 fusion.
Question 5830
Topic: 6. Spine
In a patient with Spondyloepiphyseal Dysplasia Tarda (SEDT), which of the following radiographic spinal abnormalities is considered the hallmark pathognomonic finding?
Correct Answer & Explanation
. Heaped-up or humped bone at the central and posterior portions of the vertebral endplates
Explanation
The pathognomonic radiographic finding for SEDT is the presence of a 'heaped-up' or 'hump-shaped' mound of bone in the central and posterior aspects of the superior and inferior vertebral endplates. Anterior beaking is more characteristic of Morquio syndrome.
Question 5831
Topic: 6. Spine
A patient with ankylosing spondylitis (AS) is being considered for biologics. AS is strongly associated with HLA-B27. Which cytokine pathway is often targeted in AS given its role in enthesitis and bone formation/resorption imbalance characteristic of the disease?
Correct Answer & Explanation
. IL-17 pathway
Explanation
The IL-17 pathway, particularly cytokines produced by Th17 cells (IL-17 and IL-22), is increasingly recognized as central to the pathogenesis of ankylosing spondylitis (AS). IL-17 plays a critical role in promoting inflammation at entheses (where ligaments and tendons attach to bone), driving osteoproliferation (syndesmophytes), and contributing to bone erosion. Consequently, biologics targeting IL-17 (e.g., secukinumab, ixekizumab) are effective treatments for AS. While TNF-alpha is also important and targeted, IL-17 is a more specific answer for the described pathology.
Question 5832
Topic: 6. Spine
A 14-year-old competitive gymnast complains of progressive low back pain, worse with extension and gymnastics activities. Radiographs show a defect in the pars interarticularis of L5. What is the most likely diagnosis?
Correct Answer & Explanation
. Spondylolysis
Explanation
The clinical presentation (low back pain in an adolescent athlete, especially one performing repetitive hyperextension activities like gymnastics) combined with a radiographic defect in the pars interarticularis is classic for spondylolysis. Spondylolisthesis implies anterior slippage of one vertebra over another, which can result from bilateral spondylolysis, but the initial defect is spondylolysis. Lumbar disc herniation is rare in this age group unless from acute trauma, and Scheuermann's kyphosis affects the thoracic spine primarily.
Question 5833
Topic: Thoracolumbar Spine & Deformity
In the context of adult spinal deformity, which of the following radiographic parameters is most strongly correlated with health-related quality of life (HRQOL) and patient-reported outcomes?
Correct Answer & Explanation
. Sagittal vertical axis (SVA)
Explanation
Sagittal vertical axis (SVA), which measures the plumb line from C7 relative to the sacral promontory, is widely considered the most important radiographic parameter correlating with health-related quality of life (HRQOL) and patient-reported outcomes in adult spinal deformity. Patients with positive sagittal imbalance (forward lean) tend to have significantly worse pain and functional scores. While Cobb angle, PI, and PT are also important, SVA directly reflects global sagittal balance and its impact on energy expenditure and posture.
Question 5834
Topic: 6. Spine
A 70-year-old male with symptomatic severe lumbar spinal stenosis, refractory to conservative management, is considering surgical decompression. Which of the following co-morbidities carries the highest risk for adverse outcomes following lumbar spine surgery?
Correct Answer & Explanation
. Current smoking
Explanation
Current smoking is consistently identified as one of the most significant modifiable risk factors for adverse outcomes following lumbar spine surgery. It is associated with higher rates of surgical site infection, impaired wound healing, pseudarthrosis (nonunion), increased pain, and overall worse patient-reported outcomes. While other comorbidities like obesity, diabetes, and cardiovascular disease increase risk, smoking is particularly detrimental to fusion and healing processes.
Question 5835
Topic: 6. Spine
A 58-year-old male with a history of ankylosing spondylitis (AS) is undergoing a bilateral THA for end-stage arthritis. He previously developed significant heterotopic ossification (HO) after a spinal fusion. What is the most effective prophylactic regimen to prevent severe HO in this patient?
Correct Answer & Explanation
. Postoperative radiation therapy (e.g., 700 cGy in a single dose) delivered within 24-72 hours of surgery.
Explanation
Patients with ankylosing spondylitis, especially those with a history of HO after other surgeries, are at extremely high risk for severe heterotopic ossification after THA. While NSAIDs (like indomethacin) are a standard prophylaxis for moderate-risk patients, they may not be sufficient for very high-risk individuals. Radiation therapy is highly effective for HO prophylaxis. The most effective protocol for high-risk patients, such as those with AS, is a single dose of 700 cGy delivered to the hip, typically within 24-72 hourspostoperatively. Preoperative radiation is also effective but may delay surgery and might have slightly different logistical challenges. However, the timing (postoperative, within 24-72 hours) is critical for optimal efficacy. Corticosteroids and vitamin K antagonists are not standard or effective for HO prophylaxis. Therefore, postoperative radiation therapy is the most robust and evidence-based approach for this high-risk patient.
Question 5836
Topic: 6. Spine
A 55-year-old active male undergoes primary total hip arthroplasty (THA) for severe osteoarthritis. Postoperatively, he experiences recurrent anterior dislocations despite adequate component positioning (cup inclination 40°, anteversion 15°). Further evaluation reveals a 'flatback' deformity in the lumbar spine, with a diminished lumbar lordosis and a sacral slope of 25° in standing, decreasing to 10° in sitting. Which of the following adjustments would be most appropriate during a revision surgery to address his recurrent dislocations related to his spinal-pelvic mechanics?
Correct Answer & Explanation
. Place the acetabular component in a more anteriorly tilted position (increased functional anteversion in sitting) or use a dual-mobility construct.
Explanation
Patients with stiff spinal deformities, particularly 'flatback' (diminished lumbar lordosis, low sacral slope), are at higher risk for hip dislocation after THA. In standing, these patients often have a posterior pelvic tilt, but in sitting, they demonstrate a significant anterior pelvic tilt. This anterior pelvic tilt in sitting effectively reduces the functional anteversion of the acetabular component, increasing the risk of anterior dislocation. To compensate for this, the acetabular component needs to be placed in a position that provides increased effective anteversion in the sitting position, which means positioning it with more anteversion than traditional guidelines, or with a more anterior tilt. A dual-mobility construct also offers increased jump distance and stability. Increasing anteversion and inclination excessively (Option A) can lead to posterior impingement or instability. Decreasing anteversion and inclination (Option B) would worsen the problem. A constrained liner is an option for recurrent dislocation but may not fully address the underlying spinal-pelvic kinematics causing the problem and comes with its own risks. A larger femoral head increases jump distance but might not be sufficient for severe spinopelvic malalignment. Therefore, placing the acetabular component in a more anteriorly tilted position (increased functional anteversion in sitting) or using a dual-mobility construct directly addresses the kinematic challenges posed by the 'flatback' deformity.
Question 5837
Topic: 6. Spine
A 50-year-old male with severe ankylosing spondylitis and a fused, kyphotic spine ('chin-on-chest' deformity) requires bilateral total hip arthroplasties. What is the single most critical consideration during intraoperative positioning and component placement for successful THA in this patient to optimize postoperative function and prevent dislocation?
Correct Answer & Explanation
. Accounting for the patient's altered functional pelvic tilt in their typical standing/sitting posture
Explanation
Patients with severe ankylosing spondylitis and a fused kyphotic spine have a fixed sagittal imbalance, which significantly alters their functional pelvic tilt. In their typical standing or sitting posture, the pelvis is often in a functionally retroverted position relative to anatomical landmarks. If components are implanted based on standard anatomical landmarks without considering this altered functional pelvic tilt, the patient may be at high risk for impingement and dislocation postoperatively. For example, a 'standard' anteverted cup in an anatomically neutral pelvis could become highly anteverted functionally when the spine is fused in kyphosis. Therefore, it is critical to anticipate the patient's functional pelvic tilt and adjust acetabular component anteversion and inclination accordingly. Performing a spinal osteotomy (E) before THA is a major procedure and not always necessary or feasible. Other options are less critical than understanding functional pelvic tilt.
Question 5838
Topic: Thoracolumbar Spine & Deformity
A 74-year-old female with a prior long segment lumbar fusion (T10-pelvis) for scoliosis is scheduled for a THA. How does her altered spinopelvic biomechanics influence acetabular component positioning?
Correct Answer & Explanation
. The stiff pelvis cannot roll back during hip flexion, requiring increased cup anteversion to prevent posterior dislocation
Explanation
A fused lumbar spine cannot flex (loss of posterior pelvic tilt/rollback) to accommodate hip flexion, placing the patient at a high risk for posterior dislocation. The acetabular component should be placed in increased anteversion and inclination to compensate for this stiffness.
Question 5839
Topic: 6. Spine
A newborn is diagnosed with congenital scoliosis secondary to a fully segmented hemivertebra in the thoracic spine. Because congenital scoliosis occurs due to abnormal somite development during embryogenesis, screening for associated anomalies is mandatory. Which of the following sets of screening tests is most appropriate?
Correct Answer & Explanation
. Renal ultrasound, echocardiogram, and entire spine MRI
Explanation
Congenital scoliosis is highly associated with VACTERL anomalies. Renal anomalies (unilateral kidney, duplication) occur in up to 20-30%, requiring a renal ultrasound. Cardiac anomalies require an echocardiogram. Intraspinal anomalies (tethered cord, syringomyelia, diastematomyelia) are seen in up to 30%, mandating an entire spine MRI.
Question 5840
Topic: 6. Spine
A 14-year-old female gymnast complains of insidious onset, mechanical low back pain that worsens with back extension. Radiographs of the lumbar spine show a defect in the pars interarticularis of L5. The classic 'Scotty dog with a collar' sign is best appreciated on which specific radiographic projection?
Correct Answer & Explanation
. Oblique view
Explanation
Spondylolysis is a defect in the pars interarticularis, common in adolescent athletes subjected to repetitive hyperextension (like gymnasts). The pars defect looks like a 'collar' on the neck of the 'Scotty dog,' which is the classic radiographic appearance seen on the 45-degree oblique view of the lumbar spine.
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