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Question 641

Topic: 4. Pediatrics

A 3-year-old girl is brought to the clinic due to noticeable anterolateral bowing of her left tibia. On physical exam, you note multiple cafe-au-lait spots and axillary freckling. What genetic mutation is most likely responsible for her condition?

. Type 1 collagen gene mutation
. Fibroblast growth factor receptor 3 (FGFR3) mutation
. Neurofibromin 1 (NF1) gene mutation
. Dystrophin gene mutation
. GNAS1 gene mutation

Correct Answer & Explanation

. Neurofibromin 1 (NF1) gene mutation


Explanation

Anterolateral bowing of the tibia (often progressing to pseudoarthrosis) and cafe-au-lait spots are classic signs of Neurofibromatosis Type 1. NF1 is caused by an autosomal dominant mutation in the neurofibromin 1 gene located on chromosome 17.

Question 642

Topic: Pediatric Hip

In the surgical management of an unstable slipped capital femoral epiphysis (SCFE) using a single cannulated screw, which complication is most directly related to unrecognized intra-articular hardware penetration?

. Avascular necrosis
. Chondrolysis
. Femoral neck fracture
. Slipped progression
. Cam impingement

Correct Answer & Explanation

. Chondrolysis


Explanation

Chondrolysis (rapid cartilage destruction) is strongly associated with unrecognized intra-articular hardware penetration during SCFE pinning. Careful fluoroscopic evaluation using the approach-withdraw technique helps prevent this.

Question 643

Topic: Pediatric Hip

A 4-month-old female infant is evaluated for asymmetric thigh folds. Ultrasound reveals a dysplastic left hip with an alpha angle of 50 degrees and a beta angle of 80 degrees. What is the most appropriate initial management?

. Pavlik harness
. Closed reduction and spica casting
. Open reduction and capsulorrhaphy
. Observation with repeat ultrasound in 3 months
. Pelvic osteotomy

Correct Answer & Explanation

. Pavlik harness


Explanation

For developmental dysplasia of the hip (DDH) in an infant under 6 months of age, the Pavlik harness is the gold standard initial treatment. It maintains the hip in flexion and abduction to safely promote acetabular remodeling.

Question 644

Topic: Pediatric Hip
A 6-year-old boy presents with a painless limp. Radiographs show sclerosis and flattening of the capital femoral epiphysis. According to the Herring lateral pillar classification, greater than 50% loss of lateral pillar height categorizes the hip into which group?
. Group A
. Group B
. Group C
. Group B/C
. Group D

Correct Answer & Explanation

. Group C


Explanation

In the Herring classification for Legg-Calvé-Perthes disease, a loss of more than 50% of the lateral pillar height defines Group C. This group carries the poorest prognosis regarding long-term hip joint congruity and function.

Question 645

Topic: 4. Pediatrics
You are asked to review a 28-year-old man who is thought to originate from Afghanistan who has presented to an immigration centre claiming asylum. He has been diagnosed with HIV for which HAART has been commenced. There is a past history of tuberculosis as a child for which he was successfully treated with triple anti-bacterial therapy. Over the past few days, he has developed a severe cough, productive of bloody sputum. On examination, he is pyrexial 37.8 °C, his BP is 105/70 mmHg, and pulse is 80 bpm and regular. There are scattered coarse crackles on auscultation of the chest. Investigations: Hb 11.0 g/dl, WCC 7.9 × 10^9/l, PLT 194 × 10^9/l, Na+ 137 mmol/l, K+ 4.2 mmol/l, Creatinine 115 μmol/l, CXR: Bilateral pleural effusions, widespread nodular consolidation. Which of the following is the most likely diagnosis?
. Invasive aspergillosis
. Pneumocystis jirovecii
. Pseudomonal pneumonia
. Staphylococcal pneumonia
. Tuberculosis

Correct Answer & Explanation

. Tuberculosis


Explanation

Given the patient's history of tuberculosis and recent initiation of HAART for HIV, the most likely diagnosis is immune reconstitution inflammatory syndrome (IRIS) leading to reactivation of latent tuberculosis.

Question 646

Topic: Pediatric Hip

A 6-month-old infant is diagnosed with developmental dysplasia of the hip (DDH) after failing 6 weeks of treatment in a Pavlik harness. Ultrasound confirms persistent dislocation. What is the most appropriate next step in management?

. Continue the Pavlik harness for an additional 6 weeks
. Closed reduction and spica casting under general anesthesia
. Immediate open reduction and femoral shortening osteotomy
. Application of a Denis Browne bar
. Observation until walking age

Correct Answer & Explanation

. Closed reduction and spica casting under general anesthesia


Explanation

If a Pavlik harness fails to achieve reduction in an infant with DDH after 3 to 4 weeks, continuing it is contraindicated due to the risk of 'Pavlik harness disease'. The next step is typically closed reduction and application of a hip spica cast under anesthesia.

Question 647

Topic: Pediatric Hip

A 13-year-old obese boy presents with a 3-week history of right groin pain and a limp. On examination, he walks with an antalgic gait, and his right hip goes into obligate external rotation when flexed. Radiographs confirm a slipped capital femoral epiphysis (SCFE). What is the standard surgical treatment?

. Closed reduction and spica casting
. In situ single screw fixation
. Proximal femoral derotational osteotomy
. Core decompression
. Open reduction and internal fixation with a dynamic hip screw

Correct Answer & Explanation

. In situ single screw fixation


Explanation

The classic presentation of SCFE includes obligate external rotation with hip flexion. The gold standard of treatment is in situ fixation across the physis, typically utilizing a single partially threaded cannulated screw, to prevent further slippage.

Question 648

Topic: Pediatric Upper Extremity & Spine
A 6-year-old girl falls onto her outstretched hand and sustains a Gartland type III supracondylar humerus fracture. On evaluation, the hand is well-perfused and warm with brisk capillary refill, but the radial pulse is absent. What is the most appropriate initial management?
. Immediate open vascular exploration by a vascular surgeon
. Closed reduction and percutaneous pinning of the fracture
. CT angiogram of the upper extremity
. Emergent volar fasciotomy
. Observation and casting in 90 degrees of flexion

Correct Answer & Explanation

. Closed reduction and percutaneous pinning of the fracture


Explanation

For a 'pink, pulseless' hand associated with a severely displaced supracondylar fracture, the standard initial step is urgent closed reduction and percutaneous pinning. The radial pulse frequently returns once the brachial artery is unkinked by the reduction.

Question 649

Topic: Pediatric Upper Extremity & Spine

During closed reduction and percutaneous pinning of a supracondylar humerus fracture, a healthy 6-year-old boy suddenly develops tachycardia, masseter muscle rigidity, and a rapidly rising end-tidal CO2. The anesthesiologist administers the definitive antidote. What is the mechanism of action of this life-saving drug?

. Inhibits calcium release from the sarcoplasmic reticulum via the ryanodine receptor
. Competitively blocks acetylcholine at the postsynaptic neuromuscular junction
. Stimulates central alpha-2 adrenergic receptors to reduce sympathetic outflow
. Directly vasodilates peripheral arterioles to rapidly dissipate core body heat
. Inhibits acetylcholinesterase to increase synaptic acetylcholine

Correct Answer & Explanation

. Inhibits calcium release from the sarcoplasmic reticulum via the ryanodine receptor


Explanation

The patient is experiencing malignant hyperthermia. The definitive treatment is Dantrolene, which works by inhibiting the ryanodine receptor (RyR1), thereby preventing the release of calcium from the sarcoplasmic reticulum and halting muscle hypermetabolism.

Question 650

Topic: Pediatric Hip

An obese 12-year-old boy presents with a 3-week history of right groin and knee pain, walking with an externally rotated limp. Radiographs demonstrate widening of the capital femoral physis with the epiphysis displaced posteriorly and inferiorly. Which of the following is the most feared complication directly associated with attempted forceful closed reduction of this condition?

. Chondrolysis
. Avascular necrosis (AVN)
. Slipped capital femoral epiphysis of the contralateral hip
. Femoroacetabular impingement (FAI)
. Leg length discrepancy

Correct Answer & Explanation

. Avascular necrosis (AVN)


Explanation

Forceful closed reduction of a slipped capital femoral epiphysis (SCFE) significantly increases the risk of avascular necrosis due to stretching or tearing of the tenuous retinacular vessels. In situ pinning is the standard of care to avoid this catastrophic complication.

Question 651

Topic: 4. Pediatrics

When managing an infant with congenital idiopathic clubfoot using the Ponseti method, what is the correct sequence of deformity correction?

. Cavus, Adductus, Varus, Equinus
. Adductus, Varus, Equinus, Cavus
. Equinus, Varus, Adductus, Cavus
. Varus, Cavus, Adductus, Equinus
. Cavus, Varus, Adductus, Equinus

Correct Answer & Explanation

. Cavus, Adductus, Varus, Equinus


Explanation

The Ponseti method sequentially corrects the deformities using the CAVE acronym: Cavus, Adductus, Varus, and finally Equinus. The cavus is corrected first by supinating the forefoot to align it with the hindfoot.

Question 652

Topic: Pediatric Hip

An obese 12-year-old boy presents with an inability to bear weight on his left leg and severe left hip pain. Radiographs show a posterior and inferior slip of the proximal femoral epiphysis. He is diagnosed with an unstable slipped capital femoral epiphysis (SCFE). Which complication is most highly associated with this specific presentation?

. Chondrolysis
. Leg length discrepancy
. Femoroacetabular impingement
. Avascular necrosis of the femoral head
. Nonunion

Correct Answer & Explanation

. Avascular necrosis of the femoral head


Explanation

Unstable SCFE (defined as the inability to bear weight even with crutches) has a high risk of avascular necrosis, with rates up to 50%. Prompt diagnosis and careful surgical management are required to minimize this risk.

Question 653

Topic: 4. Pediatrics

A 6-year-old boy sustains a severe extension-type supracondylar humerus fracture. Upon arrival, his hand is warm and pink, but the radial pulse is absent. After successful closed reduction and percutaneous pinning, the hand remains warm and pink with capillary refill less than 2 seconds, but the radial pulse remains unpalpable. What is the best next step?

. Observation and close monitoring
. Immediate vascular exploration
. CT angiography of the upper extremity
. Removal of pins and open reduction
. Administer intravenous heparin

Correct Answer & Explanation

. Observation and close monitoring


Explanation

A pink, pulseless hand after reduction of a pediatric supracondylar humerus fracture is generally managed with observation, as collateral circulation is adequate to perfuse the hand. Vascular exploration is typically indicated only if the hand becomes cold and white.

Question 654

Topic: 4. Pediatrics

A 6-year-old child presents with a pulseless, pale, and poorly perfused hand following a displaced extension-type supracondylar humerus fracture. After anatomical closed reduction and percutaneous pinning, the hand remains pulseless and poorly perfused. What is the next most appropriate step in management?

. Observe for 24 hours
. Perform a duplex ultrasound
. Remove the pins and attempt open reduction
. Perform an anterior approach to the elbow for vascular exploration
. Perform a sympathetic block

Correct Answer & Explanation

. Perform an anterior approach to the elbow for vascular exploration


Explanation

A dysvascular hand that remains poorly perfused after anatomical reduction of a supracondylar humerus fracture requires immediate surgical exploration. The anterior approach allows direct visualization and release of the entrapped brachial artery.

Question 655

Topic: Pediatric Hip

An 18-month-old child is diagnosed with untreated developmental dysplasia of the hip (DDH) on the right side. Which of the following is the most appropriate initial surgical management?

. Pavlik harness application
. Closed reduction and spica casting
. Open reduction with or without pelvic/femoral osteotomy
. Shelf acetabuloplasty
. Total hip arthroplasty

Correct Answer & Explanation

. Open reduction with or without pelvic/femoral osteotomy


Explanation

After 18 months of age, closed reduction is rarely successful due to severe soft tissue contractures and acetabular dysplasia. Open reduction, often combined with a pelvic or femoral osteotomy, is the standard of care to achieve stable concentric reduction.

Question 656

Topic: Pediatric Hip

A 13-year-old obese male presents with right thigh pain and a limp. Examination reveals obligate external rotation of the right hip during passive flexion. Radiographs confirm a severe, stable slipped capital femoral epiphysis (SCFE). What is the most appropriate definitive management?

. Spica casting
. In situ pinning with a single cannulated screw
. Open reduction and internal fixation with multiple screws
. Immediate subtrochanteric osteotomy
. Observation and non-weight bearing

Correct Answer & Explanation

. In situ pinning with a single cannulated screw


Explanation

In situ percutaneous pinning with a single central cannulated screw is the gold standard for a stable SCFE. This prevents further slippage while minimizing the risk of avascular necrosis and chondrolysis.

Question 657

Topic: Pediatric Hip

A 9-year-old boy presents with a 3-week history of right groin pain and an antalgic limp. His BMI is in the 25th percentile for his age. Radiographs confirm a mild stable slipped capital femoral epiphysis (SCFE). Given the patient's age and weight, which of the following is the most appropriate initial laboratory workup?

. Complete blood count and inflammatory markers
. Rheumatoid factor and antinuclear antibodies
. Thyroid-stimulating hormone (TSH) and free T4
. Serum calcium, phosphate, and alkaline phosphatase
. Parathyroid hormone (PTH) and vitamin D levels

Correct Answer & Explanation

. Thyroid-stimulating hormone (TSH) and free T4


Explanation

SCFE in children under 10 years of age, or in those whose weight is less than the 50th percentile, is highly associated with underlying endocrine disorders. Hypothyroidism is the most common endocrine etiology, making TSH and free T4 essential in the initial screening.

Question 658

Topic: Pediatric Hip

An obese 13-year-old boy presents with an obligatory external rotation of his right hip when it is passively flexed. Radiographs reveal a slipped capital femoral epiphysis (SCFE). Which of the following is the most likely direction of epiphyseal displacement relative to the femoral neck?

. Anterior and superior
. Anterior and inferior
. Posterior and inferior
. Posterior and superior
. Strictly medial

Correct Answer & Explanation

. Posterior and inferior


Explanation

In SCFE, the femoral epiphysis typically displaces posteriorly and inferiorly relative to the femoral neck. The clinical sign is obligate external rotation during hip flexion.

Question 659

Topic: Pediatric Hip

A 6-month-old infant is treated with a Pavlik harness for developmental dysplasia of the hip (DDH). Two weeks later, the baby exhibits decreased active extension of the left knee. What is the most likely complication?

. Avascular necrosis of the femoral head
. Femoral nerve palsy
. Obturator nerve palsy
. Sciatic nerve palsy
. Septic arthritis

Correct Answer & Explanation

. Femoral nerve palsy


Explanation

Excessive hip flexion in a Pavlik harness can compress the femoral nerve against the inguinal ligament. This leads to femoral nerve palsy, manifesting as decreased active knee extension.

Question 660

Topic: 4. Pediatrics

A 2-year-old child presents with bowed legs, costochondral junction enlargement, and delayed closure of the fontanelles. Radiographs show widened, cupped, and frayed metaphyses. Which zone of the physis is massively expanded in this condition?

. Reserve zone
. Proliferative zone
. Hypertrophic zone
. Zone of provisional calcification
. Primary spongiosa

Correct Answer & Explanation

. Zone of provisional calcification


Explanation

In rickets, there is a failure of mineralization in the zone of provisional calcification. This lack of mineralization prevents normal apoptosis of chondrocytes, leading to massive expansion of the hypertrophic zone.