Menu

4. Pediatrics MCQs

Practice Set 210 of 334

This practice set contains high-yield board review questions covering key concepts in 4. Pediatrics. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.

Question 4181

Topic: 4. Pediatrics
A neonatologist requests orthopedic consultation for a newborn diagnosed with Osteogenesis Imperfecta (OI) based on multiple intrauterine fractures and profound long bone deformities. According to the Sillence classification, which type of OI is uniformly fatal in the perinatal period?
. Type I
. Type II
. Type III
. Type IV
. Type V

Correct Answer & Explanation

. Type II

Explanation

Sillence Type II Osteogenesis Imperfecta is the most severe form and is uniformly fatal in the perinatal period due to severe pulmonary hypoplasia and profound skeletal fragility.

Question 4182

Topic: 4. Pediatrics

A 3-year-old child presents with a short trunk, flat midface, prominent joints, hearing loss, and myopia. Radiographs reveal characteristic 'dumbbell-shaped' femora and coronal clefts in the vertebral bodies. Which gene is most likely mutated in this patient?

. COMP
. COL2A1
. SLC26A2
. FGFR3
. FLNB

Correct Answer & Explanation

. COL2A1

Explanation

The clinical and radiographic presentation is classic for Kniest Dysplasia, which is a Type II collagenopathy caused by mutations in the COL2A1 gene. 'Dumbbell-shaped' femora are characteristic.

Question 4183

Topic: 4. Pediatrics

A 6-month-old infant with achondroplasia exhibits hypotonia, frequent apneic episodes, and hyperreflexia in the lower extremities. Which of the following is the most appropriate next step in management?

. Flexion-extension C-spine X-rays
. MRI of the craniocervical junction
. CT of the temporal bones
. MRI of the lumbar spine
. Polysomnography alone

Correct Answer & Explanation

. MRI of the craniocervical junction

Explanation

Infants with achondroplasia are at high risk for foramen magnum stenosis, which causes cervicomedullary compression leading to sleep apnea, hypotonia, and hyperreflexia. Urgent MRI of the craniocervical junction is required.

Question 4184

Topic: 4. Pediatrics

A 4-year-old presents with short stature, a waddling gait, bilateral coxa vara, and genu varum. Radiographs display widened and irregular metaphyses, but perfectly normal epiphyses and spine. Genetic testing reveals a mutation in COL10A1. What is the diagnosis?

. Multiple Epiphyseal Dysplasia
. Spondyloepiphyseal Dysplasia
. Schmid Metaphyseal Chondrodysplasia
. Jansen Metaphyseal Chondrodysplasia
. Achondroplasia

Correct Answer & Explanation

. Schmid Metaphyseal Chondrodysplasia

Explanation

Schmid Metaphyseal Chondrodysplasia is caused by a mutation in COL10A1 (Type X collagen). It presents with coxa vara and genu varum, affecting only the metaphyses while sparing the epiphyses and spine.

Question 4185

Topic: Pediatric Upper Extremity & Spine

Which of the following describes the 'columns' of the distal humerus, critical for surgical planning?

. The medial and lateral epicondyles
. The anterior and posterior cortices of the humeral shaft
. The medial and lateral supracondylar ridges extending to the articular surface
. The capitellum and trochlea
. The anterior and posterior synovial compartments

Correct Answer & Explanation

. The medial and lateral supracondylar ridges extending to the articular surface

Explanation

The concept of medial and lateral columns is fundamental to the surgical management of distal humerus fractures. These columns consist of the medial and lateral supracondylar ridges that extend distally to articulate with the forearm bones. The articular surface (capitellum and trochlea) forms the 'tie arch' between these columns. Restoring the length, alignment, and rotation of these columns, and then reducing the articular fragments, is central to achieving stable fixation. The epicondyles are part of the columns, but not the columns themselves. The other options describe different anatomical parts or concepts.

Question 4186

Topic: Pediatric Upper Extremity & Spine

Which specific intra-articular fracture pattern often requires meticulous direct articular visualization and fine fragment reduction, sometimes necessitating a separate mini-approach, due to its common association with instability?

. Type A supracondylar fracture
. Type B1 lateral condyle fracture
. Type C3 comminuted bicondylar fracture
. Capitellar fracture (e.g., Bryan and Morrey Type I)
. Simple medial epicondyle avulsion

Correct Answer & Explanation

. Capitellar fracture (e.g., Bryan and Morrey Type I)

Explanation

Capitellar fractures (e.g., Bryan and Morrey Type I) are intra-articular coronal shear fractures of the capitellum. They are notoriously unstable and require meticulous direct articular visualization and often a separate mini-approach (e.g., lateral paratricipital or via a posterior approach with osteotomy) to achieve anatomical reduction and stable fixation (often with headless compression screws or small plates). They are often missed or underestimated on plain radiographs. Type A supracondylar and medial epicondyle avulsions are less complex articular injuries. C3 is complex overall, but capitellar itself is a distinct challenge.

Question 4187

Topic: 4. Pediatrics
A 10-year-old boy presents with a Salter-Harris Type II distal radius fracture. What is the characteristic feature of this fracture type?
. Fracture through the epiphysis
. Fracture through the metaphysis
. Fracture through the physis only
. Fracture through the physis and metaphysis
. Fracture through the physis and epiphysis

Correct Answer & Explanation

. Fracture through the physis and metaphysis

Explanation

The Salter-Harris classification describes physeal (growth plate) fractures. Type I: fracture through the physis only. Type II: fracture through the physis and extending into the metaphysis (fracture through the growth plate with a metaphyseal fragment, a 'Thurston Holland' fragment). This is the most common type. Type III: fracture through the physis and extending into the epiphysis (intra-articular). Type IV: fracture through the metaphysis, physis, and epiphysis (intra-articular). Type V: crush injury to the physis (rare, poor prognosis). Therefore, Type II involves both the physis and the metaphysis.

Question 4188

Topic: 4. Pediatrics

What is the approximate remodeling potential for angular deformity of a mid-diaphyseal forearm fracture in a 6-year-old child?

. Minimal remodeling, requiring anatomical reduction.
. Up to 5 degrees of remodeling.
. Up to 10 degrees of remodeling.
. Up to 15 degrees of remodeling.
. Significant remodeling, often correcting up to 20-30 degrees.

Correct Answer & Explanation

. Up to 15 degrees of remodeling.

Explanation

In children, remodeling potential for forearm fractures decreases with age and proximity to the physis. For mid-diaphyseal fractures in a 6-year-old, angular deformities of up to 10-15 degrees (Option C or D) can remodel well, especially if the fracture is proximal and the child has significant growth remaining. However, rotational deformities remodel poorly at any age. While 'significant' remodeling (Option E) can occur, a specific range like 10-15 degrees is more precise for mid-diaphyseal. For infants, even greater remodeling can occur, but less for older children/adolescents. Therefore, 10-15 degrees represents a reasonable threshold for a 6-year-old.

Question 4189

Topic: 4. Pediatrics

In a 4-year-old child with a minimally displaced greenstick fracture of the mid-diaphyseal radius and ulna, what is the most appropriate management strategy?

. Operative fixation with flexible IM nails.
. Open reduction and internal fixation with plates.
. Closed reduction under sedation to correct angulation, followed by long arm casting.
. Application of a short arm cast without reduction.
. Observation only, relying on excellent remodeling potential.

Correct Answer & Explanation

. Closed reduction under sedation to correct angulation, followed by long arm casting.

Explanation

For minimally displaced greenstick fractures withacceptableangulation in young children (e.g., <10 degrees in this age group), closed reduction under sedation (if needed to correct any unacceptable angulation) followed by a long arm cast (Option C) is the standard treatment. The significant remodeling potential in a 4-year-old allows for correction of minor residual deformities. Surgical fixation (A, B) is overkill. Short arm cast (D) is insufficient for diaphyseal forearm fractures. Observation (E) is generally not appropriate, as some form of immobilization is typically required even for 'minimally displaced' to protect healing.

Question 4190

Topic: 4. Pediatrics

A 6-year-old child sustains a both bones forearm fracture with 25 degrees of angulation. Which plane of angulation has the best remodeling potential?

. Volar angulation.
. Dorsal angulation.
. Radial angulation.
. Ulnar angulation.
. All planes remodel equally well.

Correct Answer & Explanation

. Volar angulation.

Explanation

Volar angulation (Option A) in the sagittal plane has superior remodeling potential compared to angulation in the coronal plane (radial or ulnar angulation) or rotational deformities. This is due to the inherent growth and remodeling capabilities of bone, which are more effective in correcting sagittal plane deformities, especially when the apex of the deformity is directed away from the joint. Remodeling is also better when closer to the physis and in younger children.

Question 4191

Topic: 4. Pediatrics

Which of the following is an absolute indication for operative fixation of a both bones forearm fracture in a 9-year-old child?

. Angulation greater than 15 degrees in the sagittal plane.
. Shortening greater than 1 cm.
. Complete displacement with inability to achieve acceptable closed reduction.
. Isolated ulna fracture with less than 5 degrees angulation.
. Greenstick fracture with less than 10 degrees angulation.

Correct Answer & Explanation

. Complete displacement with inability to achieve acceptable closed reduction.

Explanation

Complete displacement with inability to achieve or maintain an acceptable closed reduction (Option C) is an absolute indication for operative fixation in a 9-year-old child. While children have remodeling potential, severe displacement and instability, especially rotational, will lead to significant functional impairment. Angulation (A) and shortening (B) are relative indications depending on age and location. Isolated ulna fracture (D) or minimally angulated greenstick fracture (E) are often managed non-operatively in this age group.

Question 4192

Topic: Pediatric Hip

A 28-year-old female with a known history of an inherited chondrodysplasia presents with hip pain and a waddling gait. Review the provided radiograph. Which of the following proximal femoral deformities is most commonly associated with this condition in adulthood?



. Coxa valga
. Coxa vara
. Slipped capital femoral epiphysis
. Femoral retroversion
. Cam impingement

Correct Answer & Explanation

. Coxa vara

Explanation

Correct Answer: Coxa varaThe radiograph shows retarded ossification of the proximal femur, which is usually accompanied by coxa vara in the adult period for patients with Spondyloepiphyseal Dysplasia. This deformity contributes to the waddling gait and hip pain.

Question 4193

Topic: 4. Pediatrics

When differentiating Spondyloepiphyseal Dysplasia Congenita (SEDc) from Achondroplasia, which of the following clinical features is most characteristic of SEDc?

. Rhizomelic shortening of the limbs with a normal trunk length
. Disproportionate short stature with a markedly short trunk
. Frontal bossing and midface hypoplasia
. Autosomal dominant inheritance linked to FGFR3
. Trident hand deformity

Correct Answer & Explanation

. Disproportionate short stature with a markedly short trunk

Explanation

Correct Answer: Disproportionate short stature with a markedly short trunkSEDc is characterized by a short trunk due to a growth disorder of the spine (platyspondyly), whereas achondroplasia typically presents with a normal trunk length and rhizomelic (proximal) shortening of the limbs.

Question 4194

Topic: Pediatric Hip

A 28-year-old female with a history of a skeletal dysplasia presents with hip pain. Her pelvic radiograph is shown below. What is the primary deformity seen in the proximal femur that typically develops in this condition?


. Coxa valga
. Coxa vara
. Slipped capital femoral epiphysis
. Developmental dysplasia of the hip
. Femoral retroversion

Correct Answer & Explanation

. Coxa vara

Explanation

Correct Answer: Coxa varaThe radiograph demonstrates retarded ossification of the proximal femur, which is usually accompanied by coxa vara in older patients with spondyloepiphyseal dysplasia.

Question 4195

Topic: 4. Pediatrics

A 4-year-old child is being evaluated for short stature. Radiographs reveal platyspondyly and epiphyseal dysplasia of the femoral head. The child has a disproportionately short trunk. This presentation is most consistent with spondyloepiphyseal dysplasia congenita. Which of the following features most reliably distinguishes this condition from achondroplasia?

. Presence of a short trunk
. Autosomal dominant inheritance
. Frontal bossing
. Rhizomelic shortening of the limbs
. Trident hand deformity

Correct Answer & Explanation

. Presence of a short trunk

Explanation

Correct Answer: Presence of a short trunkSpondyloepiphyseal dysplasia congenita is associated with a short trunk due to a growth disorder of the spine (platyspondyly). In contrast, achondroplasia is characterized by a relatively normal trunk length and rhizomelic (proximal) shortening of the limbs.

Question 4196

Topic: 4. Pediatrics

A 5-year-old boy presents with disproportionate short stature, a short trunk, and platyspondyly. Radiographs show epiphyseal dysplasia of the femoral head. Genetic testing is most likely to reveal a mutation in which of the following genes?

. FGFR3
. COL1A1
. COL2A1
. COMP
. RUNX2

Correct Answer & Explanation

. COL2A1

Explanation

Correct Answer: CSpondyloepiphyseal dysplasia congenita (SEDc) is an inherited chondrodysplasia caused by a mutation in the COL2A1 gene, which encodes type II procollagen. This leads to a growth disorder of the spine and epiphyses, resulting in a short trunk, platyspondyly, and epiphyseal dysplasia. FGFR3 mutations are associated with achondroplasia, COL1A1 with osteogenesis imperfecta, COMP with pseudoachondroplasia, and RUNX2 with cleidocranial dysplasia.

Question 4197

Topic: Pediatric Hip

A 28-year-old female with a history of spondyloepiphyseal dysplasia presents with hip pain and a waddling gait. Her pelvic radiograph is shown below. Which of the following proximal femoral deformities is most commonly associated with this condition in adulthood?




. Coxa valga
. Coxa vara
. Slipped capital femoral epiphysis
. Femoral retroversion
. Cam impingement

Correct Answer & Explanation

. Coxa vara

Explanation

Correct Answer: BThe radiograph shows retarded ossification of the proximal femur, which is usually accompanied by coxa vara in the elderly period or adulthood in patients with spondyloepiphyseal dysplasia. This deformity contributes to the waddling gait and progressive arthropathy seen in these patients.

Question 4198

Topic: 4. Pediatrics

Which of the following clinical features best distinguishes spondyloepiphyseal dysplasia from achondroplasia?

. Rhizomelic limb shortening
. Short trunk with relatively normal limb length
. Severe intellectual disability
. Craniosynostosis
. Polydactyly

Correct Answer & Explanation

. Short trunk with relatively normal limb length

Explanation

Correct Answer: BSpondyloepiphyseal dysplasia is characterized by a short trunk due to a growth disorder of the spine, leading to disproportionate short stature where the trunk is short relative to the limbs. In contrast, achondroplasia is characterized by rhizomelic (proximal) limb shortening with a relatively normal trunk length.

Question 4199

Topic: 4. Pediatrics

A 5-year-old boy presents with disproportionate short stature, a short trunk, and progressive dorsolumbar kyphosis. Radiographs reveal platyspondyly and epiphyseal dysplasia of the femoral head. Genetic testing is most likely to reveal a mutation in which of the following genes?

. FGFR3
. COL1A1
. COL2A1
. COMP
. RUNX2

Correct Answer & Explanation

. COL2A1

Explanation

Correct Answer: COL2A1Congenital spondyloepiphyseal dysplasia (SEDC) is an inherited chondrodysplasia characterized by short stature and a short trunk due to a growth disorder of the spine and epiphyses. It is caused by a mutation in the COL2A1 gene, which encodes type II procollagen. FGFR3 mutations are associated with achondroplasia, COL1A1 with osteogenesis imperfecta, COMP with pseudoachondroplasia, and RUNX2 with cleidocranial dysplasia.

Question 4200

Topic: Pediatric Hip

A 28-year-old female with a known history of an inherited chondrodysplasia presents with hip pain and a waddling gait. Her pelvic radiograph is shown below. Which of the following deformities is most commonly associated with the delayed ossification of the proximal femur seen in this condition?


. Coxa valga
. Coxa vara
. Slipped capital femoral epiphysis
. Developmental dysplasia of the hip
. Femoroacetabular impingement

Correct Answer & Explanation

. Coxa vara

Explanation

Correct Answer: Coxa varaIn Spondyloepiphyseal Dysplasia, there is retarded ossification of the proximal femur in young patients, which is usually accompanied by the development of coxa vara in the older period, as seen on the radiograph of this 28-year-old female. This contributes to the progressive arthropathy and gait abnormalities.

Test Yourself

Switch to an interactive, timed exam simulation to truly master this topic.

On this Page