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Question 5241

Topic: 7. Hand and Wrist

When evaluating a patient with multiple intra-articular loose bodies, which of the following radiographic features best differentiates primary synovial chondromatosis from secondary synovial chondromatosis?

. Presence of joint space narrowing
. Loose bodies of uniform size and shape
. Subchondral cyst formation
. Fewer than 5 loose bodies present
. Predilection for the small joints of the hands

Correct Answer & Explanation

. Loose bodies of uniform size and shape


Explanation

Primary synovial chondromatosis typically presents with multiple loose bodies that are uniform in size and shape due to simultaneous metaplasia. Secondary synovial chondromatosis (e.g., from osteoarthritis) features fewer bodies of varying sizes.

Question 5242

Topic: 7. Hand and Wrist

A 10-year-old boy with Multiple Hereditary Exostoses (MHE) is being evaluated for progressive forearm deformity. Which of the following represents the most classic pattern of forearm deformity seen in this syndrome?

. Relative shortening of the radius, ulnar bowing, and ulnar subluxation
. Relative shortening of the ulna, radial bowing, and radial head dislocation
. Proximal radioulnar synostosis
. Overgrowth of the distal ulna leading to positive ulnar variance
. Madelung deformity with volar subluxation of the hand

Correct Answer & Explanation

. Relative shortening of the ulna, radial bowing, and radial head dislocation


Explanation

The classic forearm deformity in MHE includes an osteochondroma in the distal ulna causing relative ulnar shortening, secondary bowing of the radius, an increased ulnar tilt of the distal radius, and eventual radial head dislocation.

Question 5243

Topic: 7. Hand and Wrist
Trichorhinophalangeal syndrome (TRPS) Type III is phenotypically distinguished from TRPS Type I primarily by the presence of which of the following features?
. Multiple exostoses (osteochondromas)
. Severe generalized shortening of all metacarpals and phalanges
. Premature craniosynostosis
. Ocular involvement with blue sclerae
. Severe hearing and visual impairment

Correct Answer & Explanation

. Severe generalized shortening of all metacarpals and phalanges


Explanation

TRPS Type III is caused by specific, severe missense mutations in the TRPS1 gene. It is distinguished clinically from Type I by extreme short stature and severe brachydactyly affecting all metacarpals and phalanges. Multiple exostoses are characteristic of TRPS Type II.

Question 5244

Topic: 7. Hand and Wrist

A 7-year-old boy presents with sparse hair, a pear-shaped nose, and short stature. Hand radiographs show the following characteristic anomaly.

What is the radiographic hallmark seen in this syndrome's phalanges?

. Delta phalanx
. Acro-osteolysis
. Cone-shaped epiphyses
. Macrodactyly
. Symphalangism

Correct Answer & Explanation

. Cone-shaped epiphyses


Explanation

Trichorhinophalangeal syndrome (TRPS1) typically presents with cone-shaped epiphyses of the phalanges. Clinical features also include sparse hair, a bulbous nose, and short stature.

Question 5245

Topic: 7. Hand and Wrist

A 10-year-old girl is evaluated for characteristic hand deformities and facial features.

Radiographs of her hands reveal the classic skeletal hallmark of Trichorhinophalangeal syndrome. Which of the following describes this finding?

. Delta phalanges
. Carpal coalition
. Madelung deformity
. Cone-shaped epiphyses of the phalanges
. Bullet-shaped metacarpals

Correct Answer & Explanation

. Cone-shaped epiphyses of the phalanges


Explanation

The radiographic hallmark of TRPS1 is the presence of cone-shaped epiphyses at the bases of the middle phalanges, which often leads to clinodactyly and premature growth arrest.

Question 5246

Topic: 7. Hand and Wrist

Review the radiograph of the hand below.

The marked digital foreshortening (brachydactyly) in this patient with TRPS is primarily caused by which of the following pathophysiological mechanisms?

. Failure of endochondral ossification in the carpal bones
. Premature fusion of the central portion of the phalangeal growth plates
. Avascular necrosis of the metacarpal heads
. Failure of intramembranous ossification of the phalangeal shafts
. Progressive osteolysis of the distal phalangeal tufts

Correct Answer & Explanation

. Premature fusion of the central portion of the phalangeal growth plates


Explanation

The cone-shaped epiphyses seen in TRPS lead to premature, central fusion of the growth plates in the phalanges. This arrests longitudinal growth, resulting in the characteristic brachydactyly and angular deformities of the fingers.

Question 5247

Topic: 7. Hand and Wrist

A 9-year-old girl is evaluated for short stature and characteristic facial features, including a pear-shaped nose. Radiographs of her hands are obtained.

Which of the following radiographic findings is most characteristic of Trichorhinophalangeal syndrome (TRPS)?

. Acro-osteolysis of the distal tufts
. Cone-shaped epiphyses in the middle phalanges
. Metacarpal extreme shortening with a 'trident hand'
. Subperiosteal bone resorption
. Madelung deformity

Correct Answer & Explanation

. Cone-shaped epiphyses in the middle phalanges


Explanation

Cone-shaped epiphyses, most commonly affecting the middle phalanges, are the classic radiographic hallmark of Trichorhinophalangeal syndrome (TRPS). This leads to premature physeal closure and brachydactyly.

Question 5248

Topic: 7. Hand and Wrist

A 12-year-old girl with fine sparse hair and a bulbous, "pear-shaped" nose complains of painless deformity and swelling of her finger joints.

Based on the clinical presentation and typical radiographic findings of this syndrome, which of the following skeletal anomalies is the hallmark of this condition?

. Delta phalanx
. Cone-shaped epiphyses of the phalanges
. Metacarpal foreshortening
. Carpal coalition
. Avascular necrosis of the metacarpal heads

Correct Answer & Explanation

. Cone-shaped epiphyses of the phalanges


Explanation

Trichorhinophalangeal syndrome (TRPS) is caused by a mutation in the TRPS1 gene. The hallmark radiographic finding in the hands is cone-shaped epiphyses of the middle phalanges, which often lead to angular joint deviations.

Question 5249

Topic: 7. Hand and Wrist

A 12-year-old boy with multiple bony bumps around his knees and wrists is diagnosed with Multiple Hereditary Exostoses (MHE).

The pathogenesis of this condition involves a mutation in EXT1 or EXT2 genes, which leads to a defect in the synthesis of what molecular component?

. Type I collagen
. Heparan sulfate
. Hyaluronic acid
. Chondroitin sulfate
. Type X collagen

Correct Answer & Explanation

. Heparan sulfate


Explanation

EXT1 and EXT2 genes encode glycosyltransferases essential for the synthesis of heparan sulfate. Deficiency in heparan sulfate disrupts Indian Hedgehog (Ihh) signaling, causing abnormal chondrocyte proliferation.

Question 5250

Topic: 7. Hand and Wrist

A 14-year-old female with Multiple Hereditary Exostoses (MHE) presents with a visible forearm deformity. Which of the following descriptions represents the most common sequence of forearm deformity in this condition?

. Ulnar lengthening leading to a negative ulnar variance and scaphoid impaction
. Relative growth retardation of the ulna leading to secondary bowing of the radius and ulnar deviation of the hand
. Overgrowth of the distal radius causing radial deviation of the hand and isolated distal radioulnar joint subluxation
. Primary proximal radioulnar synostosis blocking supination
. Disproportionate radial shortening resulting in a secondary Madelung deformity

Correct Answer & Explanation

. Relative growth retardation of the ulna leading to secondary bowing of the radius and ulnar deviation of the hand


Explanation

Osteochondromas in MHE commonly affect the distal ulna due to its small cross-sectional area, causing growth retardation. This relative ulnar shortening leads to a tethering effect, causing secondary bowing of the radius, ulnar deviation of the hand, and potentially radial head dislocation.

Question 5251

Topic: Nerve & Tendon

A 45-year-old female presents with a deep intramuscular lipoma in the proximal anterior forearm near the supinator muscle. She reports insidious, progressive weakness in extending her thumb and fingers, without any sensory deficits. Which nerve is most likely compressed by this mass?

. Anterior interosseous nerve
. Posterior interosseous nerve
. Superficial radial nerve
. Ulnar nerve
. Median nerve

Correct Answer & Explanation

. Posterior interosseous nerve


Explanation

A mass near the supinator muscle (arcade of Frohse) can compress the posterior interosseous nerve (PIN). PIN compression results in motor weakness of thumb and finger extensors, without sensory loss (as the sensory component travels via the superficial radial nerve).

Question 5252

Topic: Wrist & Carpus

A 16-year-old boy with a known history of multiple hereditary exostoses (MHE) presents with worsening deformity of his left forearm and decreased range of motion. Radiographs reveal a characteristic forearm deformity associated with this condition. Which of the following best describes the typical pathoanatomy of the forearm in MHE?

. Radial shortening, ulnar bowing, and ulnar head dislocation
. Symmetric shortening of the radius and ulna with proximal radioulnar synostosis
. Ulnar shortening, secondary bowing of the radius, and radial head dislocation
. Overgrowth of the distal ulna leading to positive ulnar variance and TFCC tears
. Madelung deformity secondary to premature closure of the volar-ulnar distal radius physis

Correct Answer & Explanation

. Ulnar shortening, secondary bowing of the radius, and radial head dislocation


Explanation

Forearm deformity in MHE typically begins with relative shortening of the ulna due to osteochondroma formation. This tethering effect leads to secondary bowing of the radius and eventual dislocation of the radial head.

Question 5253

Topic: Nerve & Tendon

A 6-year-old boy sustains a displaced extension-type supracondylar humerus fracture after falling from monkey bars. Post-injury, he is unable to flex the interphalangeal joint of his thumb and the distal interphalangeal joint of his index finger. Which nerve is most likely injured?

. Ulnar nerve
. Radial nerve
. Anterior interosseous branch of the median nerve
. Posterior interosseous nerve
. Musculocutaneous nerve

Correct Answer & Explanation

. Anterior interosseous branch of the median nerve


Explanation

Correct Answer: Anterior interosseous branch of the median nerveThe median nerve is the most frequently injured nerve in extension-type supracondylar humerus fractures. Specifically, the anterior interosseous nerve (AIN), a motor branch of the median nerve, is highly susceptible. AIN palsy presents clinically as the inability to flex the IP joint of the thumb (flexor pollicis longus) and the DIP joint of the index finger (flexor digitorum profundus), resulting in an inability to make an 'OK' sign.

Question 5254

Topic: Nerve & Tendon

A 6-year-old boy falls from a playground structure and sustains a widely displaced extension-type supracondylar humerus fracture. Which of the following nerves is most frequently injured in this specific fracture pattern?

. Radial nerve
. Ulnar nerve
. Median nerve
. Axillary nerve
. Musculocutaneous nerve

Correct Answer & Explanation

. Median nerve


Explanation

Correct Answer: Median nerveThe median nerve, specifically its anterior interosseous nerve (AIN) branch, is the most frequently injured nerve in extension-type supracondylar humerus fractures. The radial nerve is the second most commonly injured, often seen with posteromedial displacement. The ulnar nerve is more commonly injured in flexion-type supracondylar fractures or iatrogenically during medial pin placement.

Question 5255

Topic: Hand Trauma & Infection

A 30-year-old carpenter presents with a swollen, throbbing index finger two days after a minor puncture wound. Of Kanavel's four cardinal signs for acute pyogenic flexor tenosynovitis, which clinical finding is generally considered the earliest and most sensitive indicator?

. Fusiform swelling of the entire digit
. A resting flexed posture of the digit
. Tenderness to palpation along the flexor tendon sheath
. Severe pain elicited by passive extension of the digit
. Erythema extending into the palm

Correct Answer & Explanation

. Severe pain elicited by passive extension of the digit


Explanation

Pain with passive extension is widely regarded as the earliest and most sensitive of Kanavel's four cardinal signs for pyogenic flexor tenosynovitis. The other signs are fusiform (sausage) swelling, a flexed resting posture, and tenderness over the flexor sheath.

Question 5256

Topic: 7. Hand and Wrist
A 32-year-old carpenter presents with progressive dorsal wrist pain and decreased grip strength. Radiographs show sclerosis and fragmentation of the lunate with proximal migration of the capitate (Lichtman Stage IIIB Kienbรถck disease). Ulnar variance is neutral and there is no diffuse radiocarpal arthritis. Which surgical intervention is most appropriate?
. Radial shortening osteotomy
. Proximal row carpectomy (PRC)
. Scaphoid-trapezium-trapezoid (STT) fusion
. Lunate excision and fascial interposition
. Total wrist arthrodesis

Correct Answer & Explanation

. Proximal row carpectomy (PRC)


Explanation

Lichtman Stage IIIB Kienbรถck disease is defined by lunate fragmentation with carpal collapse (evidenced by scaphoid rotary subluxation) without advanced radiocarpal arthritis. A proximal row carpectomy (PRC) or scaphocapitate fusion is the treatment of choice at this stage to salvage wrist motion.

Question 5257

Topic: 7. Hand and Wrist

Which of the following radiographic findings in the hands is most characteristic of Multiple Epiphyseal Dysplasia (MED)?

. Trident hand with short, thick metacarpals
. Madelung deformity of the wrist
. Delayed ossification of the carpal bones with short, stubby metacarpals and phalanges
. Proximal pointing of the metacarpals
. Arachnodactyly with camptodactyly

Correct Answer & Explanation

. Delayed ossification of the carpal bones with short, stubby metacarpals and phalanges


Explanation

Correct Answer: C (Delayed ossification of the carpal bones with short, stubby metacarpals and phalanges)Multiple Epiphyseal Dysplasia (MED) affects the epiphyses of long bones and the small bones of the hands and feet. Characteristic hand radiograph findings include delayed ossification of the carpal bones and brachydactyly (short, stubby metacarpals and phalanges). A 'trident hand' is classic for Achondroplasia. Madelung deformity is seen in Leri-Weill dyschondrosteosis. Proximal pointing of the metacarpals is a hallmark of Mucopolysaccharidoses (e.g., Hurler syndrome). Arachnodactyly is characteristic of Marfan syndrome.

Question 5258

Topic: 7. Hand and Wrist

A 10-year-old girl is evaluated for sparse hair, a bulbous pear-shaped nose, and shortened fingers. Hand radiographs demonstrate prominent cone-shaped epiphyses in the middle phalanges. A mutation in which of the following genes is most likely responsible for this specific epiphyseal anomaly?

. TRPS1
. COMP
. COL2A1
. EXT1
. GNAS

Correct Answer & Explanation

. TRPS1


Explanation

Trichorhinophalangeal syndrome (TRPS) is characterized by sparse hair, a bulbous nasal tip, and characteristic cone-shaped epiphyses in the hands. It is caused by mutations in the TRPS1 gene, which encodes a zinc-finger transcription factor.

Question 5259

Topic: 7. Hand and Wrist

A 9-year-old boy presents with sparse hair, a bulbous pear-shaped nose, and mild short stature. Radiographs of the hands demonstrate distinct cone-shaped epiphyses in the phalanges. Which of the following is the most likely diagnosis?

. Trichorhinophalangeal syndrome
. Ellis-van Creveld syndrome
. Cleidocranial dysplasia
. Chondrodysplasia punctata
. Diastrophic dysplasia

Correct Answer & Explanation

. Trichorhinophalangeal syndrome


Explanation

Trichorhinophalangeal syndrome (TRPS) is characterized by sparse hair, a bulbous nose, and cone-shaped epiphyses in the hands. It is an important epiphyseal dysplasia to recognize due to its highly specific radiographic and facial features.

Question 5260

Topic: 7. Hand and Wrist

A 6-year-old girl falls from monkey bars and sustains a widely displaced extension-type supracondylar humerus fracture. On presentation to the emergency department, her hand is pale, pulseless, and she is unable to make an "OK" sign with her thumb and index finger. Which of the following nerves is most likely injured, and what is the most appropriate next step in management?

. Radial nerve; immediate open exploration
. Ulnar nerve; closed reduction and percutaneous pinning
. Anterior interosseous nerve; closed reduction and percutaneous pinning
. Median nerve; immediate open exploration
. Anterior interosseous nerve; immediate open exploration

Correct Answer & Explanation

. Anterior interosseous nerve; closed reduction and percutaneous pinning


Explanation

Correct Answer: CThe inability to make an "OK" sign indicates an injury to the anterior interosseous nerve (AIN), a motor branch of the median nerve. The AIN is the most commonly injured nerve in extension-type supracondylar humerus fractures. A pulseless, pale hand (vascular compromise) in the setting of a displaced fracture requires urgent closed reduction and percutaneous pinning. Often, the pulse returns after anatomical reduction relieves tension on the brachial artery. Open exploration is reserved for cases where the hand remains dysvascular after adequate reduction.