Pediatric Orthopedics: OI & SMA Board Review MCQs

Question 1

A 9-year-old boy presents with a painful, swollen right forearm 3 weeks after a minor fall. Radiographs show a massive, mineralized mass surrounding a healing radial shaft fracture, initially raising suspicion for osteosarcoma. A biopsy confirms hype

Accepted Answer

IFITM5

Question 2

A 15-month-old child is evaluated for delayed motor milestones. He can sit independently but cannot pull to stand. Physical examination reveals proximal muscle weakness, absent patellar reflexes, and fine tremors of the tongue. Genetic testing confir

Accepted Answer

The number of SMN2 gene copies

Question 3

A 4-year-old girl with Osteogenesis Imperfecta Type III is admitted for her first cycle of intravenous pamidronate. Approximately 24 hours after the infusion, she develops a temperature of 38.8°C (101.8°F), myalgias, and irritability. Laboratory stud

Accepted Answer

Administer antipyretics and provide supportive care, as this is a self-limiting reaction.

Question 4

A 3-year-old boy with Spinal Muscular Atrophy Type II is started on risdiplam (Evrysdi), a newly approved daily oral medication to improve motor function. The parents ask the orthopedic surgeon how this medication works. The surgeon correctly explain

Accepted Answer

Modify the pre-mRNA splicing of the SMN2 gene to increase functional protein production.

Question 5

A 16-year-old male with severe Osteogenesis Imperfecta Type III presents with a 3-month history of worsening clumsiness, frequent falls, and new-onset tingling in his hands. On examination, he has hyperreflexia in his lower extremities, a positive Ho

Accepted Answer

MRI of the cervical spine and craniocervical junction.

Question 6

A 6-year-old non-ambulatory girl with Spinal Muscular Atrophy Type II is noted to have progressive right hip subluxation on annual surveillance radiographs. The hip is currently subluxated 40% but remains reducible. The primary biomechanical driver f

Accepted Answer

Stronger hip flexors and adductors overpowering weaker hip extensors and abductors.

Question 7

A 5-year-old boy with Osteogenesis Imperfecta Type IV undergoes bilateral femoral rodding with Fassier-Duval telescoping intramedullary nails to correct severe anterolateral bowing. Two years postoperatively, radiographs reveal that the right femur h

Accepted Answer

Failure of the rod components to telescope, leading to bone growth around the implant.

Question 8

A 12-year-old boy with Spinal Muscular Atrophy Type II presents with a progressive, 85-degree sweeping thoracolumbar neuromuscular scoliosis and a 25-degree pelvic obliquity. He is non-ambulatory and relies on a custom-molded wheelchair. He is schedu

Accepted Answer

To correct pelvic obliquity and provide a level, stable sitting base.

Question 9

A 7-year-old girl with a history of multiple low-energy fractures is noted by her dentist to have opalescent, amber-colored teeth that exhibit significant enamel attrition and dentin exposure. This dental manifestation is a direct result of a qualita

Accepted Answer

The sclera of the eye and tendons.

Question 10

A 6-month-old infant with Spinal Muscular Atrophy Type I is admitted to the pediatric intensive care unit with acute respiratory distress. The patient exhibits a classic "bell-shaped" chest and paradoxical breathing. This respiratory pattern is prima

Accepted Answer

Weakness of the intercostal muscles with relative sparing of the diaphragm.

Pediatric Orthopedics: OI & SMA Board Review MCQs

Pediatric Orthopedics: OI & SMA Board Review MCQs

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Pediatric Orthopedics: OI & SMA Board Review MCQs

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