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Question 1901

Topic: Pediatric Hip

A 10-year-old girl with delayed fontanelle closure, open skull sutures, and supernumerary teeth is evaluated for a waddling gait. What is the most common orthopedic abnormality requiring surgical intervention in this syndrome?

. Scoliosis
. Atlantoaxial instability
. Coxa vara
. Genu valgum
. Recurrent patellar dislocations

Correct Answer & Explanation

. Coxa vara


Explanation

The patient has cleidocranial dysplasia. Coxa vara is a common finding and represents the most frequent orthopedic manifestation requiring surgical intervention, such as a valgus producing subtrochanteric osteotomy.

Question 1902

Topic: Pediatric Hip

A 7-year-old boy presents with bilateral hip pain and a waddling gait. Radiographs demonstrate small, fragmented proximal femoral epiphyses bilaterally and double-layered patellae. Spine radiographs are completely normal. What is the most likely diagnosis?

. Legg-Calve-Perthes disease
. Spondyloepiphyseal dysplasia congenita
. Multiple epiphyseal dysplasia
. Morquio syndrome
. Pseudoachondroplasia

Correct Answer & Explanation

. Multiple epiphyseal dysplasia


Explanation

Multiple epiphyseal dysplasia (MED) presents with delayed/fragmented epiphyses (often mimicking bilateral Perthes) and characteristic double-layered patellae. The spine is typically normal, distinguishing it from spondyloepiphyseal dysplasias.

Question 1903

Topic: Pediatric Hip

A 14-year-old boy presents for evaluation of shoulder hypermobility. He is able to touch his shoulders together anteriorly. Examination reveals a large, open anterior fontanelle and retained deciduous teeth. He is at increased risk for developing which of the following orthopedic conditions?

. Slipped capital femoral epiphysis
. Coxa vara
. Atlantoaxial instability
. Pathologic fractures of the diaphysis
. Early-onset osteoarthritis of the knee

Correct Answer & Explanation

. Coxa vara


Explanation

This patient has cleidocranial dysplasia (RUNX2 mutation), characterized by hypoplastic/absent clavicles, delayed fontanelle closure, and delayed dental eruption. Orthopedically, these patients have a high incidence of progressive developmental coxa vara.

Question 1904

Topic: Pediatric Hip

A 12-year-old obese male presents with left knee pain and a limp. Radiographs confirm a mild, stable slipped capital femoral epiphysis (SCFE) on the left. Which of the following is the strongest indication for prophylactic in situ pinning of the contralateral asymptomatic hip?

. Age greater than 14 years
. Presentation greater than 3 weeks from symptom onset
. Normal Body Mass Index (BMI)
. Concomitant diagnosis of hypothyroidism
. Grade 1 initial slip severity

Correct Answer & Explanation

. Concomitant diagnosis of hypothyroidism


Explanation

Endocrine disorders (such as hypothyroidism or renal osteodystrophy) significantly increase the risk of bilateral SCFE. Prophylactic pinning of the contralateral hip is highly recommended in these patients to prevent future displacement.

Question 1905

Topic: Pediatric Hip

A 4-month-old female is being treated in a Pavlik harness for developmental dysplasia of the hip (DDH). At her follow-up appointment, she demonstrates a loss of active knee extension on the affected side. What is the most likely cause of this finding?

. Obturator nerve palsy from excessive abduction
. Sciatic nerve palsy from excessive extension
. Peroneal nerve palsy from strap compression
. Femoral nerve palsy from excessive hip flexion
. Development of avascular necrosis of the femoral head

Correct Answer & Explanation

. Femoral nerve palsy from excessive hip flexion


Explanation

Femoral nerve palsy in a Pavlik harness is caused by excessive hip flexion. The harness should be adjusted or temporarily discontinued until nerve function recovers to prevent permanent deficits.

Question 1906

Topic: Pediatric Hip
An 8-year-old boy is diagnosed with Legg-Calvé-Perthes disease. According to the Herring Lateral Pillar classification, which radiographic finding places the patient in the poorest prognostic group (Group C)?
. No involvement of the lateral pillar
. Greater than 50% of the lateral pillar height is maintained
. Less than 50% of the lateral pillar height is maintained
. Presence of a subchondral radiolucent line (crescent sign)
. Metaphyseal cysts without lateral pillar collapse

Correct Answer & Explanation

. Less than 50% of the lateral pillar height is maintained


Explanation

The Herring Lateral Pillar classification predicts the outcome of Perthes disease. Group C is defined by the maintenance of less than 50% of the original height of the lateral pillar and correlates with the worst long-term outcomes.

Question 1907

Topic: Pediatric Hip

A 6-month-old girl is evaluated for asymmetric gluteal folds. An AP pelvis radiograph demonstrates a right acetabular index of 35 degrees and a superolaterally displaced femoral head. What is the best initial management for this patient?

. Placement in a Pavlik harness
. Closed reduction and spica casting
. Open reduction and spica casting
. Femoral varus derotational osteotomy
. Observation with repeat radiographs in 3 months

Correct Answer & Explanation

. Closed reduction and spica casting


Explanation

For Developmental Dysplasia of the Hip (DDH) presenting in children aged 6 to 18 months, closed reduction and spica casting under general anesthesia is the standard initial treatment. The Pavlik harness has an unacceptably high failure rate in infants over 6 months of age.

Question 1908

Topic: Pediatric Hip

A 12-year-old boy presents to the emergency department with acute, severe groin pain and is completely unable to bear weight on his left leg. Radiographs reveal a severe left slipped capital femoral epiphysis (SCFE). Because he is unable to bear weight, he is classified as having an unstable SCFE. Which complication is he at highest risk for developing compared to a patient with a stable SCFE?

. Chondrolysis
. Avascular necrosis (AVN) of the femoral head
. Contralateral slip progression
. Subtrochanteric femur fracture
. Septic arthritis

Correct Answer & Explanation

. Avascular necrosis (AVN) of the femoral head


Explanation

Unstable SCFE is defined by the inability to bear weight (with or without crutches) and carries a significantly higher risk of avascular necrosis (up to 47%) compared to stable SCFE. Urgent decompression and stabilization are prioritized to mitigate this devastating complication.

Question 1909

Topic: Pediatric Hip

A 10-year-old girl is diagnosed with multiple epiphyseal dysplasia (MED). Radiographs of her knees are likely to demonstrate which of the following pathognomonic findings?

. Double-layer patella
. Stippled epiphyses
. Erlenmeyer flask deformity
. Coxa vara with a short femoral neck
. Hitchhiker thumb

Correct Answer & Explanation

. Double-layer patella


Explanation

A double-layer or multi-layered patella on a lateral knee radiograph is highly characteristic of Multiple Epiphyseal Dysplasia (MED). This condition typically spares the spine and facial structures, distinguishing it from spondyloepiphyseal dysplasia.

Question 1910

Topic: Pediatric Hip

A 7-year-old child presents with retained deciduous teeth, delayed closure of cranial sutures, and hypermobility of the shoulders, allowing them to be brought together in the midline. Which of the following is the most common orthopedic manifestation requiring surgical intervention in this syndrome?

. Coxa vara
. Atlantoaxial instability
. Scoliosis
. Recurrent patellar dislocation
. Slipped capital femoral epiphysis

Correct Answer & Explanation

. Coxa vara


Explanation

Cleidocranial dysplasia is caused by a RUNX2 (CBFA1) mutation. While clavicular hypoplasia is classic, progressive coxa vara is the most common orthopedic issue requiring surgical intervention, often via a valgus-producing proximal femoral osteotomy.

Question 1911

Topic: Pediatric Hip

A 4-year-old boy is diagnosed with Spondyloepiphyseal Dysplasia Congenita (SEDC). He has a barrel chest, severe coxa vara, and a shortened trunk. The underlying genetic defect in this condition primarily affects which of the following structural proteins?

. Type I collagen
. Type II collagen
. Type X collagen
. Cartilage oligomeric matrix protein
. Fibroblast growth factor receptor

Correct Answer & Explanation

. Type II collagen


Explanation

Correct Answer: Type II collagenSpondyloepiphyseal Dysplasia Congenita (SEDC) is a type II collagenopathy, caused by mutations in the COL2A1 gene. Type II collagen is the primary structural protein in articular cartilage and the nucleus pulposus of intervertebral discs. Defects lead to abnormal epiphyseal development, platyspondyly, and severe joint deformities like coxa vara.

Question 1912

Topic: Pediatric Hip

A 7-year-old boy presents with waddling gait, knee pain, and short stature. Radiographs demonstrate delayed ossification of the capital femoral epiphyses bilaterally, mimicking Legg-Calve-Perthes disease, with a double-layered patella. What is the inheritance pattern and typical gene involved?

. Autosomal dominant, COMP gene
. Autosomal recessive, DTDST gene
. X-linked recessive, DMD gene
. Autosomal dominant, FBN1 gene
. Autosomal recessive, CBFA1 gene

Correct Answer & Explanation

. Autosomal dominant, COMP gene


Explanation

Multiple epiphyseal dysplasia (MED) is typically autosomal dominant and frequently caused by a mutation in the COMP (Cartilage Oligomeric Matrix Protein) gene. A double-layered patella is a classic radiographic sign of this dysplasia.

Question 1913

Topic: Pediatric Hip
A 9-year-old boy complains of bilateral knee pain and a waddling gait. Radiographs show small, irregular, and fragmented epiphyses, but normal metaphyses and a normal spine. Lateral radiographs of the knee reveal a 'double-layer' appearance of the patella. Which of the following conditions is most likely?
. Multiple Epiphyseal Dysplasia
. Spondyloepiphyseal Dysplasia Congenita
. Legg-Calvé-Perthes Disease
. Meyer Dysplasia
. Chondrodysplasia Punctata

Correct Answer & Explanation

. Multiple Epiphyseal Dysplasia


Explanation

Multiple Epiphyseal Dysplasia (MED) primarily affects the epiphyses, presenting with early-onset joint pain and a waddling gait. The 'double-layer' patella on a lateral radiograph is a pathognomonic finding for this condition, distinguishing it from other dysplasias.

Question 1914

Topic: Pediatric Hip

A 15-year-old male with delayed closure of cranial sutures and supernumerary teeth is evaluated for a unilateral limp. Examination reveals significant shoulder hypermobility. Which of the following hip abnormalities is most commonly associated with his condition?

. Slipped capital femoral epiphysis
. Coxa vara
. Developmental dysplasia of the hip
. Perthes-like osteonecrosis
. Protrusio acetabuli

Correct Answer & Explanation

. Coxa vara


Explanation

Cleidocranial dysplasia is an autosomal dominant condition caused by a RUNX2 (CBFA1) mutation. Skeletal manifestations typically include absent or hypoplastic clavicles, delayed skull ossification, and developmental coxa vara.

Question 1915

Topic: Pediatric Hip

A 12-year-old boy presents with bilateral hip and knee pain after physical activity. Radiographs reveal irregular, flattened epiphyses in the hips and knees, with a double-layer appearance of the patella on the lateral view. His height and spine radiographs are normal. What is the most likely diagnosis?

. Legg-Calve-Perthes disease
. Spondyloepiphyseal dysplasia
. Multiple epiphyseal dysplasia
. Chondrodysplasia punctata
. Pseudoachondroplasia

Correct Answer & Explanation

. Multiple epiphyseal dysplasia


Explanation

Multiple epiphyseal dysplasia (MED) affects the epiphyses of the appendicular skeleton, causing early-onset osteoarthritis and a classic double-layer patella on lateral radiographs. The spine is generally spared, differentiating it from spondyloepiphyseal dysplasia.

Question 1916

Topic: Pediatric Hip
A 6-year-old boy presents with a painless limp of 3 months duration. He has limited internal rotation and abduction of the right hip. Radiographs show sclerosis and fragmentation of the right capital femoral epiphysis. What is the primary etiology of this condition?
. Septic thrombosis of the femoral vein
. Idiopathic avascular necrosis of the capital femoral epiphysis
. Traumatic disruption of the ligamentum teres
. Slipped capital femoral epiphysis
. Hypercoagulable state leading to metaphyseal infarcts

Correct Answer & Explanation

. Idiopathic avascular necrosis of the capital femoral epiphysis


Explanation

Legg-Calvé-Perthes disease is an idiopathic avascular necrosis of the proximal femoral epiphysis in children. It typically presents between ages 4 and 8 with a painless limp and restricted hip motion, particularly in internal rotation and abduction.

Question 1917

Topic: Pediatric Hip

An obese 13-year-old boy presents with a limp and vague knee pain. Examination reveals obligatory external rotation of the hip during passive flexion. Radiographs confirm a slipped capital femoral epiphysis (SCFE). In which direction does the femoral neck typically displace relative to the capital epiphysis in this condition?

. Posterior and medial
. Anterior and lateral
. Posterior and lateral
. Anterior and medial
. Directly superior

Correct Answer & Explanation

. Anterior and lateral


Explanation

Correct Answer: Anterior and lateralIn a slipped capital femoral epiphysis (SCFE), the capital femoral epiphysis remains seated in the acetabulum while the femoral neck (metaphysis) displaces anteriorly and laterally. Although it is often conceptually described as the epiphysis slipping posteriorly and medially, the actual mechanical displacement involves the metaphysis moving anteriorly and laterally relative to the fixed epiphysis.

Question 1918

Topic: Pediatric Hip
A 7-year-old boy is diagnosed with Legg-Calvé-Perthes disease. According to the Herring Lateral Pillar classification, which radiographic finding places the patient in Group C, indicating a poorer prognosis?
. No involvement of the lateral pillar
. >50% maintenance of lateral pillar height
. <50% maintenance of lateral pillar height
. Subchondral fracture line extending to the lateral pillar
. Metaphyseal cysts

Correct Answer & Explanation

. <50% maintenance of lateral pillar height


Explanation

The Herring Lateral Pillar classification is a key prognostic indicator in Legg-Calvé-Perthes disease, assessed during the fragmentation stage. Group A has no lateral pillar involvement. Group B maintains >50% of lateral pillar height. Group C maintains <50% of lateral pillar height and is associated with a poorer outcome, often requiring surgical containment (e.g., femoral or pelvic osteotomy) in children over 8 years of age.

Question 1919

Topic: Pediatric Hip

A 6-week-old female infant undergoes a screening ultrasound for Developmental Dysplasia of the Hip (DDH) due to a breech presentation. The ultrasound report notes a Graf Type IIa hip. What does this classification indicate, and what is the standard management?

. Normal mature hip; no follow-up needed
. Physiologically immature hip; follow-up ultrasound in 4-6 weeks
. Subluxated hip; immediate Pavlik harness application
. Dislocated hip; closed reduction and spica casting
. Teratologic dislocation; open reduction required

Correct Answer & Explanation

. Physiologically immature hip; follow-up ultrasound in 4-6 weeks


Explanation

Correct Answer: Physiologically immature hip; follow-up ultrasound in 4-6 weeksIn the Graf classification for DDH ultrasound, a Type I hip is normal (alpha angle >60 degrees). Type IIa indicates a physiologically immature hip in an infant less than 3 months old (alpha angle 50-59 degrees). The standard management is observation and a follow-up ultrasound in 4-6 weeks, as the vast majority will mature normally without intervention. Type IIb is the same angle but in an infant >3 months, which requires treatment.

Question 1920

Topic: Pediatric Hip
A 13-year-old obese boy presents with a 3-week history of left groin pain and a limp. On physical examination, as the left hip is passively flexed, it obligatorily deviates into external rotation. Radiographs show that a line drawn along the superior border of the femoral neck does not intersect the lateral portion of the femoral epiphysis. What is the most likely diagnosis?
. Legg-Calvé-Perthes disease
. Slipped Capital Femoral Epiphysis (SCFE)
. Developmental Dysplasia of the Hip (DDH)
. Transient synovitis
. Femoroacetabular Impingement (FAI)

Correct Answer & Explanation

. Slipped Capital Femoral Epiphysis (SCFE)


Explanation

The clinical presentation of an obese adolescent with groin pain, obligatory external rotation during hip flexion (Drehmann sign), and a radiograph showing failure of Klein's line to intersect the epiphysis is pathognomonic for Slipped Capital Femoral Epiphysis (SCFE).