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7. Hand and Wrist MCQs

Practice Set 175 of 266

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 3481

Topic: 7. Hand and Wrist

Both Ellis-van Creveld syndrome and Jeune syndrome (asphyxiating thoracic dystrophy) can present with narrow thoraces, short limbs, and pelvic radiographic changes. Which of the following features is uniquely prominent in Ellis-van Creveld syndrome but typically absent in Jeune syndrome?

. Respiratory distress in infancy
. Shortened ribs and narrow thorax
. Postaxial polydactyly of the hands
. Congenital cardiac defects and ectodermal dysplasia
. Radiographic trident appearance of the acetabulum

Correct Answer & Explanation

. Congenital cardiac defects and ectodermal dysplasia

Explanation

While both conditions share skeletal features like narrow thoraces and a 'trident' pelvis, Ellis-van Creveld syndrome is uniquely distinguished by its ectodermal dysplasia (e.g., nail and tooth abnormalities) and a high rate of congenital cardiac defects.

Question 3482

Topic: 7. Hand and Wrist

Which of the following radiographic findings in the hands is highly specific to Ellis-van Creveld syndrome when accompanied by postaxial polydactyly?

. Delta phalanx of the thumb
. Madelung deformity
. Brachydactyly type D
. Triphalangeal thumb
. Synmetacarpalism (fusion of adjacent metacarpals)

Correct Answer & Explanation

. Synmetacarpalism (fusion of adjacent metacarpals)

Explanation

Synmetacarpalism, particularly fusion of the 5th and 6th metacarpals, is a classic radiographic sign in the hands of patients with Ellis-van Creveld syndrome. It typically accompanies the postaxial polydactyly.

Question 3483

Topic: 7. Hand and Wrist

A newborn presents with short-limbed dwarfism, postaxial polydactyly of the hands, and dysplastic nails. Echocardiogram reveals a single atrium. The underlying genetic mutation responsible for this syndrome most directly disrupts which of the following cellular pathways?

. Wnt/beta-catenin signaling
. Fibroblast growth factor receptor 3 (FGFR3) signaling
. Hedgehog (Hh) signaling in the primary cilia
. Transforming growth factor-beta (TGF-beta) signaling
. Notch signaling

Correct Answer & Explanation

. Hedgehog (Hh) signaling in the primary cilia

Explanation

Ellis-van Creveld syndrome is caused by mutations in the EVC1 or EVC2 genes, which localize to primary cilia. These proteins are critical positive regulators of Hedgehog (Hh) signal transduction.

Question 3484

Topic: 7. Hand and Wrist

A 4-year-old child with chondroectodermal dysplasia is undergoing radiographic evaluation for hand deformities. Which of the following findings is most commonly seen in the wrist and hand of patients with this condition?

. Preaxial polydactyly and scaphoid-trapezium fusion
. Postaxial polydactyly and capitate-hamate fusion
. Macrodactyly and absent radius
. Shortened first metacarpal and triquetral-lunate fusion
. Delta phalanx and absent thumb

Correct Answer & Explanation

. Postaxial polydactyly and capitate-hamate fusion

Explanation

Patients with Ellis-van Creveld syndrome classically present with postaxial polydactyly of the hands. Carpal anomalies are also common, specifically fusion of the capitate and hamate.

Question 3485

Topic: 7. Hand and Wrist

Both Ellis-van Creveld syndrome and Jeune syndrome share features of short-rib polydactyly and are considered ciliopathies. Which of the following clinical findings is a hallmark of Ellis-van Creveld syndrome that helps distinguish it from Jeune syndrome?

. Severe pulmonary hypoplasia leading to neonatal death
. Chronic renal failure in childhood
. Ectodermal dysplasia affecting nails and teeth
. Preaxial polydactyly
. Hepatic fibrosis

Correct Answer & Explanation

. Ectodermal dysplasia affecting nails and teeth

Explanation

Ellis-van Creveld syndrome is distinguished from other short-rib polydactyly syndromes like Jeune syndrome by the presence of ectodermal dysplasia, which manifests as dysplastic nails, teeth anomalies, and multiple oral frenula. Jeune syndrome is more closely associated with severe asphyxiating thoracic dysplasia and subsequent renal or hepatic disease.

Question 3486

Topic: 7. Hand and Wrist

A 2-year-old girl is diagnosed with a syndrome characterized by short limbs, a single atrium, and postaxial polydactyly. Upon oral examination, which of the following findings is most pathognomonic for her condition?

. Cleft palate
. Multiple labiogingival frenula
. Mandibular hypoplasia
. Bifid uvula
. Amelogenesis imperfecta

Correct Answer & Explanation

. Multiple labiogingival frenula

Explanation

The presence of multiple labiogingival frenula (often obliterating the mucobuccal fold) and neonatal teeth are classic oral manifestations of Ellis-van Creveld syndrome.

Question 3487

Topic: 7. Hand and Wrist



A patient presents with the classical clinical phenotype of Ellis-van Creveld syndrome. Based on the typical extremity manifestations associated with this condition, which of the following patterns of polydactyly is overwhelmingly present?

. Preaxial polydactyly of the hands and feet
. Postaxial polydactyly of the hands, rarely affecting the feet
. Central polydactyly involving the third digit
. Postaxial polydactyly of the feet, rarely affecting the hands
. Mirror-image polydactyly (ulnar dimelia)

Correct Answer & Explanation

. Postaxial polydactyly of the hands, rarely affecting the feet

Explanation

Ellis-van Creveld syndrome is classically associated with postaxial polydactyly (ulnar-sided) of the hands, occurring in nearly 100% of patients. Polydactyly of the feet is much less common, occurring in only about 10-25% of cases.

Question 3488

Topic: 7. Hand and Wrist

Radiographs of the hands and wrists in a patient with Ellis-van Creveld syndrome are most likely to demonstrate which of the following characteristic carpal anomalies?

. Scapholunate dissociation
. Capitate-hamate coalition
. Absence of the pisiform
. Triquetral-lunate coalition
. Avascular necrosis of the lunate

Correct Answer & Explanation

. Capitate-hamate coalition

Explanation

Capitate-hamate fusion or coalition is a hallmark radiographic finding in the wrists of patients with EVC syndrome. Other classic skeletal findings include postaxial polydactyly and cone-shaped epiphyses.

Question 3489

Topic: 7. Hand and Wrist

A newborn is evaluated for a suspected skeletal dysplasia. Physical examination reveals extra digits. In Ellis-van Creveld syndrome, what is the most consistent and classic pattern of polydactyly?

. Preaxial polydactyly of the hands only
. Postaxial polydactyly of the hands and occasionally the feet
. Central polydactyly (cleft hand) of the hands
. Preaxial polydactyly of the feet only
. Postaxial polydactyly of the hands with consistent preaxial polydactyly of the feet

Correct Answer & Explanation

. Postaxial polydactyly of the hands and occasionally the feet

Explanation

Postaxial polydactyly (ulnar-sided extra digits) is one of the classic tetrad features of EVC. It is present in the hands of nearly 100% of patients and occasionally in the feet.

Question 3490

Topic: 7. Hand and Wrist

A 55-year-old woman presents with generalized bone pain, nephrolithiasis, and abdominal discomfort. Hand radiographs are obtained. Which specific radiographic location of subperiosteal bone resorption is considered pathognomonic for her condition?

. Ulnar aspect of the distal phalanges
. Radial aspect of the middle phalanges
. Metacarpal heads
. Carpal bones
. Base of the first metacarpal

Correct Answer & Explanation

. Radial aspect of the middle phalanges

Explanation

Subperiosteal bone resorption on the radial aspect of the middle phalanges is the pathognomonic radiographic sign of hyperparathyroidism. Other findings include acro-osteolysis and a "salt-and-pepper" appearance of the skull.

Question 3491

Topic: 7. Hand and Wrist

A 48-year-old female with chronic kidney disease presents with generalized bone pain. Hand radiographs show bone resorption along the radial aspect of the middle phalanges of her index and middle fingers. What is the most likely underlying biochemical profile?

. High Calcium, Low Phosphorus, High PTH
. Low Calcium, High Phosphorus, High PTH
. Normal Calcium, Normal Phosphorus, High Alkaline Phosphatase
. High Calcium, High Phosphorus, Low PTH
. Low Calcium, Low Phosphorus, High PTH

Correct Answer & Explanation

. Low Calcium, High Phosphorus, High PTH

Explanation

Subperiosteal bone resorption on the radial aspect of the middle phalanges is pathognomonic for hyperparathyroidism. In the context of chronic kidney disease, this represents secondary hyperparathyroidism, characterized by low calcium, high phosphorus, and high PTH.

Question 3492

Topic: 7. Hand and Wrist

A 50-year-old female on hemodialysis complains of diffuse bone pain. Hand radiographs are obtained.

Which of the following radiographic findings is considered pathognomonic for her underlying metabolic bone disease?

. Subperiosteal resorption on the radial aspect of the middle phalanges
. Subchondral cysts of the distal interphalangeal joints
. Chondrocalcinosis of the metacarpophalangeal joints
. Periarticular osteopenia of the proximal interphalangeal joints
. Tuft resorption isolated to the thumb

Correct Answer & Explanation

. Subperiosteal resorption on the radial aspect of the middle phalanges

Explanation

Subperiosteal bone resorption on the radial aspect of the middle phalanges is the pathognomonic radiographic hallmark of hyperparathyroidism.

Question 3493

Topic: 7. Hand and Wrist

A 45-year-old female with end-stage renal disease presents with diffuse bone pain. A radiograph of her hand is obtained.

What is the pathognomonic radiographic finding of her bone disease at this anatomical site?

. Erosions with overhanging sclerotic edges
. Subperiosteal bone resorption on the radial aspect of the middle phalanges
. Periarticular osteopenia and marginal erosions
. Pencil-in-cup deformity of the distal interphalangeal joints

Correct Answer & Explanation

. Subperiosteal bone resorption on the radial aspect of the middle phalanges

Explanation

The patient has secondary hyperparathyroidism due to renal osteodystrophy. The pathognomonic radiographic hallmark of hyperparathyroidism is subperiosteal bone resorption, most classically observed on the radial aspects of the middle and proximal phalanges.

Question 3494

Topic: 7. Hand and Wrist

A 48-year-old female with chronic kidney disease presents with diffuse bone pain.

Hand radiographs demonstrate subperiosteal bone resorption. Where is this radiographic finding most classically located?

. Ulnar aspect of the proximal phalanges
. Radial aspect of the middle phalanges
. Radial aspect of the distal phalanges
. Ulnar aspect of the metacarpals
. Distal tufts of the distal phalanges

Correct Answer & Explanation

. Radial aspect of the middle phalanges

Explanation

The pathognomonic radiographic finding for hyperparathyroidism is subperiosteal bone resorption. This is most characteristically seen on the radial aspect of the middle phalanges of the index and middle fingers.

Question 3495

Topic: 7. Hand and Wrist

A 4-year-old child presents with painful, recurrent soft tissue swellings over the paraspinal muscles that progressively harden. Examination of the child's feet is most likely to reveal which of the following congenital anomalies?

. Polydactyly
. Macrodactyly
. Short, deviated first metatarsals (hallux valgus)
. Syndactyly of the second and third toes
. Rocker-bottom feet

Correct Answer & Explanation

. Short, deviated first metatarsals (hallux valgus)

Explanation

Fibrodysplasia Ossificans Progressiva (FOP) is characterized by progressive heterotopic ossification of muscle and soft tissues. A classic, almost pathognomonic, physical finding present at birth is a short, medially deviated great toe (congenital hallux valgus).

Question 3496

Topic: 7. Hand and Wrist

A 45-year-old patient with end-stage renal disease on hemodialysis presents with diffuse bone pain. Laboratory evaluation reveals hyperphosphatemia, hypocalcemia, and markedly elevated parathyroid hormone levels. Radiographs of the hands are most likely to show which of the following pathognomonic findings?

. Subperiosteal resorption of the radial aspect of the middle phalanges
. Diffuse osteosclerosis with a bone-in-bone appearance
. Looser zones in the metacarpals
. Erlenmeyer flask deformity of the distal radius
. Stippled calcification of the carpal bones

Correct Answer & Explanation

. Subperiosteal resorption of the radial aspect of the middle phalanges

Explanation

Renal osteodystrophy frequently results in secondary hyperparathyroidism. The pathognomonic radiographic finding for hyperparathyroidism is subperiosteal bone resorption, most classically seen on the radial aspect of the middle phalanges.

Question 3497

Topic: 7. Hand and Wrist

Which of the following clinical and genetic findings uniquely characterize Diastrophic Dysplasia compared to other short-limb dysplasias?

. Cauliflower ears, hitchhiker thumbs, and an SLC26A2 mutation
. Normal facies, atlantoaxial instability, and a COMP mutation
. Blue sclerae, basilar invagination, and a COL1A1 mutation
. Rhizomelic shortening, trident hands, and an FGFR3 mutation
. Polydactyly, narrow thorax, and an IFT80 mutation

Correct Answer & Explanation

. Cauliflower ears, hitchhiker thumbs, and an SLC26A2 mutation

Explanation

Diastrophic dysplasia is an autosomal recessive disorder caused by a defect in the SLC26A2 sulfate transporter. It is classically associated with cauliflower ears, hitchhiker thumbs, severe clubfeet, and cleft palate.

Question 3498

Topic: 7. Hand and Wrist

A 45-year-old female with a history of scaly skin plaques presents with asymmetric oligoarthritis and dactylitis of her right hand. Radiographs demonstrate central erosions of the proximal phalanx with distal bone proliferation creating a "pencil-in-cup" appearance. Which joint is classically most affected in this condition?

. Distal interphalangeal (DIP) joint
. Proximal interphalangeal (PIP) joint
. Metacarpophalangeal (MCP) joint
. Radiocarpal joint
. Carpometacarpal (CMC) joint

Correct Answer & Explanation

. Distal interphalangeal (DIP) joint

Explanation

Psoriatic arthritis frequently involves the distal interphalangeal (DIP) joints, distinguishing it from rheumatoid arthritis which typically spares them. The classic "pencil-in-cup" deformity is due to concurrent periarticular bone erosion and new bone formation.

Question 3499

Topic: 7. Hand and Wrist
A 45-year-old male with a history of psoriasis presents with asymmetric oligoarthritis and dactylitis. Radiographs of the hands reveal periarticular erosions and bone proliferation at the distal interphalangeal (DIP) joints. According to the CASPAR criteria, which of the following clinical findings is most specific to the diagnosis of Psoriatic Arthritis?
. Subcutaneous extensor surface nodules
. Positive Rheumatoid Factor (RF)
. Nail pitting and onycholysis
. Symmetric proximal interphalangeal (PIP) joint swelling
. Bilateral sacroiliitis

Correct Answer & Explanation

. Nail pitting and onycholysis

Explanation

Nail dystrophy, including pitting and onycholysis, is a hallmark of Psoriatic Arthritis (PsA) and a key component of the CASPAR criteria. Subcutaneous nodules and positive RF are characteristic of Rheumatoid Arthritis, not PsA.

Question 3500

Topic: 7. Hand and Wrist

A patient with advanced psoriatic arthritis presents with a 'telescoping' digit resulting from severe osteolysis of the phalanges. Which of the following terms correctly describes this classic deformity?

. Boutonniere deformity
. Swan neck deformity
. Main en lorgnette (Opera-glass hand)
. Ulnar drift
. Z-thumb deformity

Correct Answer & Explanation

. Main en lorgnette (Opera-glass hand)

Explanation

Main en lorgnette, or opera-glass hand, is a consequence of arthritis mutilans in severe psoriatic arthritis. It is caused by severe osteolysis leading to a telescoping, flail digit.

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