This practice set contains high-yield board review questions covering key concepts in 7. Hand and Wrist. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
Question 5301
Topic: 7. Hand and Wrist
A 12-year-old patient with known Hurler syndrome presents for orthopedic evaluation. Which of the following upper extremity abnormalities is a hallmark finding of this specific condition?
Correct Answer & Explanation
. Trigger digits and joint contractures
Explanation
Patients with Hurler syndrome (MPS I) commonly develop severe joint contractures, particularly manifesting in the hands as trigger digits or a claw-hand deformity due to glycosaminoglycan deposition in the flexor tendons and joint capsules.
Question 5302
Topic: 7. Hand and Wrist
A 4-year-old girl is being evaluated for skeletal dysplasia. A hand radiograph is obtained.
What is the classic finding demonstrated in the hands of patients with Hurler syndrome (MPS I)?
Correct Answer & Explanation
. Proximal pointing of the metacarpals
Explanation
Dysostosis multiplex is the constellation of radiographic findings in MPS. In the hand, this typically presents as bullet-shaped phalanges and proximal pointing of the metacarpals.
Question 5303
Topic: Wrist & Carpus
What is the most common anatomic location for the presentation of a Chondromyxoid fibroma (CMF)?
Correct Answer & Explanation
. Proximal tibia metaphysis
Explanation
CMF most commonly occurs in the lower extremities, particularly around the knee. The proximal tibia metaphysis is the single most common location, accounting for a large percentage of cases.
Question 5304
Topic: 7. Hand and Wrist
When evaluating a patient with Mucopolysaccharidosis Type I (Hurler syndrome), which of the following hand/wrist manifestations is most commonly encountered and often necessitates surgical intervention?
Correct Answer & Explanation
. Carpal tunnel syndrome
Explanation
Carpal tunnel syndrome is extremely common in patients with MPS (especially types I, II, and V) due to the deposition of glycosaminoglycans within the flexor tenosynovium and flexor retinaculum. Prophylactic or early surgical release is frequently required to prevent irreversible median nerve damage.
Question 5305
Topic: 7. Hand and Wrist
A 10-year-old girl with multiple hereditary exostoses presents with a progressive forearm deformity. Which of the following is the most characteristic pattern of forearm deformity seen in this condition?
Correct Answer & Explanation
. Ulnar shortening with radial bowing and radial head dislocation
Explanation
Correct Answer: Ulnar shortening with radial bowing and radial head dislocationForearm deformities are common in Multiple Hereditary Exostoses (MHE). The distal ulna contributes a larger percentage to the overall longitudinal growth of the ulna compared to the distal radius. Osteochondromas at the distal ulna tether its growth, leading to disproportionate ulnar shortening. Because the radius continues to grow, it becomes relatively overgrown, leading to radial bowing, increased ulnar variance (positive), and eventually subluxation or dislocation of the radial head.
Question 5306
Topic: 7. Hand and Wrist
A 9-year-old girl with multiple hereditary exostoses presents with a progressive forearm deformity. Which of the following patterns of forearm deformity is most characteristic of this condition?
Correct Answer & Explanation
. Relative shortening of the ulna, radial bowing, and ulnar deviation of the hand.
Explanation
Correct Answer: Relative shortening of the ulna, radial bowing, and ulnar deviation of the hand.Forearm deformities are common in multiple hereditary exostoses (MHE). The distal ulnar physis contributes a significant portion of the longitudinal growth of the ulna and has a smaller cross-sectional area than the distal radius. Osteochondromas disproportionately affect the distal ulna, leading to premature growth arrest and relative shortening of the ulna. Because the radius continues to grow, it becomes bowed (usually bowing outward/radially) and the distal radial articular surface tilts ulnarly, leading to ulnar deviation of the carpus and hand. In severe cases, the radial head may dislocate proximally.
Question 5307
Topic: 7. Hand and Wrist
A neonate presents with a flat midface, prominent eyes, and severe foreshortening of all limbs. Radiographs demonstrate 'dumbbell-shaped' femora, coronal clefts in the vertebral bodies, and broad, short tubular bones of the hands. What is the most likely diagnosis?
Correct Answer & Explanation
. Kniest dysplasia
Explanation
Kniest dysplasia is a type II collagenopathy (COL2A1) characterized by classic radiographic findings of 'dumbbell-shaped' femora (due to extremely broad metaphyses) and coronal clefts in the vertebral bodies, alongside severe myopia and hearing loss.
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