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Question 12581

Topic: Lower Extremity Trauma

A 10-year-old boy of Ashkenazi Jewish descent presents with severe acute right hip pain. Radiographs demonstrate avascular necrosis of the right femoral head and a classic "Erlenmeyer flask" deformity of the distal femurs bilaterally. He also has significant splenomegaly. A defect in which of the following enzymes is the primary etiology?

. Beta-glucocerebrosidase
. Arylsulfatase A
. Hexosaminidase A
. Acid maltase
. Alpha-galactosidase A

Correct Answer & Explanation

. Beta-glucocerebrosidase


Explanation

The patient has Gaucher disease, an autosomal recessive lysosomal storage disorder caused by a deficiency in beta-glucocerebrosidase. The accumulation of glucocerebroside in macrophages expands the marrow cavity, causing bone pain crises, osteonecrosis, and Erlenmeyer flask deformities.

Question 12582

Topic: 2. Trauma

A 60-year-old male is undergoing staging for a newly diagnosed primary diffuse large B-cell lymphoma of the humerus. He presents to the emergency department with a displaced pathologic fracture through the lesion. What is the most appropriate management sequence?

. Amputation followed by systemic chemotherapy
. Immediate initiation of systemic chemotherapy, treating the fracture non-operatively in a brace
. Open reduction internal fixation with plates and screws followed by radiation alone
. Surgical stabilization (e.g., intramedullary nailing) followed by systemic chemotherapy and local radiation
. Neoadjuvant radiation to heal the fracture, followed by surgical resection

Correct Answer & Explanation

. Surgical stabilization (e.g., intramedullary nailing) followed by systemic chemotherapy and local radiation


Explanation

While uncomplicated primary bone lymphoma is treated medically, the presence of a displaced pathologic fracture necessitates prompt surgical stabilization. Post-operatively, the patient must proceed with the standard curative regimen of systemic chemotherapy and local radiation.

Question 12583

Topic: Pelvic & Acetabular Trauma
A 45-year-old male is brought to the trauma bay following a high-speed motorcycle collision. He has a mechanically unstable pelvis with an anteroposterior compression (APC) type III injury. A pelvic binder is placed, but he remains hemodynamically unstable despite a massive transfusion protocol. FAST exam is negative. What is the most appropriate next step in management?
. Application of an external fixator
. Immediate exploratory laparotomy
. Preperitoneal pelvic packing
. Bilateral internal iliac artery ligation
. CT angiography of the abdomen and pelvis

Correct Answer & Explanation

. Preperitoneal pelvic packing


Explanation

In a hemodynamically unstable patient with a pelvic ring injury and negative FAST, venous bleeding from the presacral plexus or cancellous bone is the most common source. Preperitoneal pelvic packing (or pelvic angiography depending on institutional protocol) is the immediate next step for hemorrhage control.

Question 12584

Topic: 2. Trauma

A 25-year-old male sustains a closed tibial shaft fracture. He complains of escalating pain out of proportion to the injury. On physical exam, he has decreased sensation in the first dorsal web space of the foot. Which fascial compartment is experiencing elevated pressure?

. Anterior compartment
. Lateral compartment
. Superficial posterior compartment
. Deep posterior compartment
. Peroneal compartment

Correct Answer & Explanation

. Anterior compartment


Explanation

The anterior compartment of the leg contains the deep peroneal nerve. Compression of this nerve due to compartment syndrome leads to paresthesias or sensory loss in its autonomous sensory zone, the first dorsal web space.

Question 12585

Topic: 2. Trauma
A 25-year-old man sustains a high-speed motor vehicle collision and presents with a vertically oriented (Pauwels type III) femoral neck fracture. Which of the following fixation constructs provides the greatest biomechanical stability for this specific fracture pattern?
. Three parallel cancellous screws
. Sliding hip screw with an anti-rotation screw
. Dynamic condylar screw
. Cephalomedullary nail
. Bipolar hemiarthroplasty

Correct Answer & Explanation

. Sliding hip screw with an anti-rotation screw


Explanation

A sliding hip screw combined with an anti-rotation screw provides superior biomechanical stability for highly unstable, vertically oriented (Pauwels III) femoral neck fractures. It better resists the extreme vertical shear forces compared to multiple cancellous screws.

Question 12586

Topic: Pelvic & Acetabular Trauma
A 45-year-old man sustains a severe crush injury resulting in an APC III pelvic ring disruption. He is hemodynamically unstable despite receiving 2L of crystalloid and 2 units of PRBCs. A FAST exam is negative, and a pelvic binder is in place. What is the most appropriate next step in management?
. Immediate open reduction and internal fixation of the pelvis
. Pelvic angiography with embolization or preperitoneal pelvic packing
. Exploratory laparotomy
. Retrograde urethrogram
. CT scan of the abdomen and pelvis

Correct Answer & Explanation

. Pelvic angiography with embolization or preperitoneal pelvic packing


Explanation

In a hemodynamically unstable patient with a pelvic ring injury and no other identified source of bleeding (negative FAST), the hemorrhage is likely retroperitoneal. Immediate pelvic angiography with embolization or preperitoneal packing is indicated.

Question 12587

Topic: 2. Trauma

A 32-year-old man with a closed tibial shaft fracture presents with pain out of proportion to his injury and severe pain with passive toe stretch. His blood pressure is 110/80 mmHg, and his intracompartmental pressure is measured at 55 mmHg. What is the delta pressure, and what is the correct action?

. 25 mmHg; perform emergent four-compartment fasciotomies
. 25 mmHg; elevate the leg above the heart and observe
. 55 mmHg; continue to monitor closely
. 55 mmHg; perform emergent fasciotomies
. 30 mmHg; administer IV pain medication and reassess in 1 hour

Correct Answer & Explanation

. 55 mmHg; perform emergent fasciotomies


Explanation

Delta pressure is calculated as Diastolic BP minus Compartment Pressure (80 - 55 = 25 mmHg). A delta pressure of less than 30 mmHg is an absolute indication for emergent four-compartment fasciotomies due to acute compartment syndrome.

Question 12588

Topic: 2. Trauma
A 35-year-old man is brought to the trauma bay after a motorcycle crash. His blood pressure is 70/40 mmHg. Pelvic radiographs demonstrate an anteroposterior compression type III (APC III) pelvic ring injury with 4 cm of symphyseal diastasis. What is the most appropriate immediate orthopedic intervention?
. Emergent anterior external fixation
. Application of a pelvic binder centered over the iliac crests
. Application of a pelvic binder centered over the greater trochanters
. Pelvic angiography and embolization
. Open reduction and internal fixation of the pubic symphysis

Correct Answer & Explanation

. Application of a pelvic binder centered over the greater trochanters


Explanation

In hemodynamically unstable patients with open-book pelvic ring injuries, a pelvic binder should be immediately applied centered over the greater trochanters to reduce pelvic volume and control venous bleeding. Placement over the iliac crests is ineffective and can worsen the deformity.

Question 12589

Topic: 2. Trauma

A 24-year-old man presents with persistent radial-sided wrist pain 6 months after a fall. Imaging reveals a proximal pole scaphoid nonunion with avascular necrosis (AVN) of the proximal fragment. There is no evidence of radiocarpal arthritis. What is the most appropriate surgical treatment?

. Vascularized bone graft (e.g., 1,2 ICSRA) via a dorsal approach
. Non-vascularized iliac crest bone graft via a volar approach
. Proximal row carpectomy
. Scaphoid excision and four-corner fusion
. Percutaneous headless compression screw fixation without bone grafting

Correct Answer & Explanation

. Vascularized bone graft (e.g., 1,2 ICSRA) via a dorsal approach


Explanation

Proximal pole scaphoid nonunions complicated by AVN have a poor healing rate with standard grafting and are best treated with vascularized bone grafting, typically the 1,2 intercompartmental supraretinacular artery (ICSRA) graft. The dorsal approach allows direct access to the proximal pole and blood supply.

Question 12590

Topic: 2. Trauma

A 28-year-old man sustains a closed right tibial shaft fracture. Twelve hours later, he develops severe calf pain out of proportion to the injury and paresthesias in the first dorsal web space. His diastolic blood pressure is 70 mmHg and his anterior compartment pressure is measured at 45 mmHg. What is the most appropriate management?

. Elevate the leg above heart level and reassess pressures in 2 hours
. Administer intravenous analgesia and apply a bivalved long leg cast
. Emergent four-compartment fasciotomy via two incisions
. Immediate intramedullary nailing of the tibia without fasciotomy
. Emergent single-incision fasciotomy of the anterior compartment only

Correct Answer & Explanation

. Emergent four-compartment fasciotomy via two incisions


Explanation

The patient has acute compartment syndrome, confirmed by a delta pressure (Diastolic BP - Compartment Pressure) of 25 mmHg, which is below the 30 mmHg diagnostic threshold. The gold standard treatment is an emergent four-compartment fasciotomy, typically via dual medial and lateral incisions.

Question 12591

Topic: 2. Trauma

A 9-year-old boy is incidentally found to have a centrally located radiolucent lesion in the proximal humerus without cortical breach or fracture. He is completely asymptomatic. What is the most appropriate initial management?

. Curettage and bone grafting
. Aspiration and methylprednisolone injection
. Observation and serial radiographs
. Prophylactic internal fixation
. Radiofrequency ablation

Correct Answer & Explanation

. Observation and serial radiographs


Explanation

Asymptomatic, incidentally discovered unicameral bone cysts without impending fracture criteria are best managed with observation and serial radiographs, as many will spontaneously resolve after skeletal maturity.

Question 12592

Topic: 2. Trauma

A 25-year-old woman presents with a painless bump on her shin. Radiographs demonstrate an expansive, intramedullary diaphyseal lesion of the tibia with a "ground-glass" appearance and bowing deformity. What is the recommended treatment for an impending fracture in this lesion?

. Curettage and cancellous bone grafting
. Intramedullary nailing
. Plate fixation with cortical strut graft
. Radiation therapy
. Amputation

Correct Answer & Explanation

. Intramedullary nailing


Explanation

For impending or actual fractures in fibrous dysplasia, intramedullary nailing is the preferred fixation method because it spans the entire bone. Cancellous bone grafts are typically resorbed and replaced by dysplastic bone, leading to high failure rates.

Question 12593

Topic: 2. Trauma

A 5-year-old boy with a known COL1A2 mutation sustains his fourth diaphyseal femur fracture. His previous fractures were treated with cast immobilization, leading to progressive anterolateral bowing. Which of the following is the gold standard surgical intervention for this patient?

. Open reduction and internal fixation with dynamic compression plates
. External fixation with gradual deformity correction
. Percutaneous pinning with Kirschner wires
. Multiple osteotomies and telescoping intramedullary rodding
. Closed reduction and spica casting

Correct Answer & Explanation

. Multiple osteotomies and telescoping intramedullary rodding


Explanation

Osteogenesis imperfecta leads to brittle bones prone to fracture and progressive bowing. The standard of care for recurrent fractures and deformity is realignment via multiple osteotomies stabilized by telescoping intramedullary rods to accommodate patient growth.

Question 12594

Topic: 2. Trauma

What is the primary pathophysiologic mechanism responsible for the development of primary synovial chondromatosis?

. Fragmentation of osteophytes
. Osteochondral fracture nonunion
. Benign metaplasia of the subsynovial connective tissue
. Infectious seeding of the joint space
. Autoimmune destruction of hyaline cartilage

Correct Answer & Explanation

. Benign metaplasia of the subsynovial connective tissue


Explanation

Primary synovial chondromatosis is caused by benign metaplasia of the subsynovial connective tissue into cartilage nodules. Secondary synovial chondromatosis occurs due to fragmentation of osteophytes or articular cartilage in the setting of osteoarthritis.

Question 12595

Topic: 2. Trauma
An 8-year-old boy trips and falls, sustaining a pathologic fracture through a centrally located, radiolucent lesion in the proximal humerus diaphysis. Radiographs demonstrate a 'fallen leaf' sign. What is the most appropriate initial management?
. Immediate intralesional curettage and bone grafting
. Immediate flexible intramedullary nailing
. Immobilization in a sling to allow the fracture to heal
. Corticosteroid injection into the cyst
. Wide en bloc resection of the affected humeral segment

Correct Answer & Explanation

. Immobilization in a sling to allow the fracture to heal


Explanation

The clinical picture describes a unicameral bone cyst (UBC) with a pathologic fracture. The initial management is non-operative immobilization to allow the fracture to heal; up to 15-20% of UBCs will spontaneously consolidate and resolve following a fracture.

Question 12596

Topic: 2. Trauma

A patient is evaluated for suspected Complex Regional Pain Syndrome (CRPS) Type I six months after an ankle fracture. According to the Budapest Criteria for the clinical diagnosis of CRPS, the patient must report at least one symptom in three of four categories. Which of the following is NOT one of the designated categories?

. Sensory (e.g., hyperalgesia or allodynia)
. Vasomotor (e.g., temperature or skin color asymmetry)
. Sudomotor/Edema (e.g., sweating changes or asymmetric edema)
. Motor/Trophic (e.g., decreased range of motion, motor dysfunction, trophic changes)
. Radiographic (e.g., patchy periarticular osteopenia on plain film)

Correct Answer & Explanation

. Radiographic (e.g., patchy periarticular osteopenia on plain film)


Explanation

The Budapest Criteria for CRPS rely entirely on clinical signs and symptoms across four categories: Sensory, Vasomotor, Sudomotor/Edema, and Motor/Trophic. Radiographic findings like osteopenia are supportive but are not part of the formal clinical criteria.

Question 12597

Topic: 2. Trauma

Progressive Diaphyseal Dysplasia (Camurati-Engelmann disease) is a rare sclerosing bone dysplasia. Which of the following is the most common clinical presentation in childhood?

. Proximal muscle weakness, limb pain, and a waddling gait
. Multiple recurrent fractures from minor trauma
. Blue sclerae and dentinogenesis imperfecta
. Progressive hearing loss and frontal bossing
. Disproportionate short stature with rhizomelic shortening

Correct Answer & Explanation

. Proximal muscle weakness, limb pain, and a waddling gait


Explanation

Camurati-Engelmann disease classically presents with bone pain, delayed walking, proximal muscle weakness, and a waddling gait. Recurrent fractures are characteristic of Osteogenesis Imperfecta, not Camurati-Engelmann.

Question 12598

Topic: 2. Trauma

Circular external fixation (e.g., Ilizarov frame) is often utilized for complex Charcot foot reconstruction. Which of the following is the most frequent complication associated with this surgical technique?

. Deep vein thrombosis
. Aseptic nonunion of the arthrodesis site
. Pin tract infection
. Neurovascular bundle transection
. Catastrophic frame breakage

Correct Answer & Explanation

. Pin tract infection


Explanation

Pin tract infections are the most common complication of circular external fixation in diabetic foot reconstruction, occurring in 30% to 50% of patients. Fortunately, the majority are superficial and respond well to local wound care and oral antibiotics.

Question 12599

Topic: Lower Extremity Trauma

A radiograph of a 20-year-old female taken for a minor knee sprain incidentally reveals dense, longitudinal striations in the metaphyses of the distal femur and proximal tibia. The patient is otherwise healthy and asymptomatic. This radiographic finding is characteristic of which of the following skeletal dysplasias?

. Osteopathia striata
. Melorheostosis
. Osteopoikilosis
. Pycnodysostosis

Correct Answer & Explanation

. Osteopathia striata


Explanation

Correct Answer: Osteopathia striataOsteopathia striata (Voorhoeve disease) is a benign, usually asymptomatic skeletal dysplasia characterized radiographically by dense, longitudinal linear striations in the metaphyses and diaphyses of long bones, and sometimes a fan-like appearance in the ilium. It is caused by mutations in the WTX (AMER1) gene and is inherited in an X-linked dominant pattern. It is often an incidental finding and requires no treatment.

Question 12600

Topic: Lower Extremity Trauma

A 28-year-old female with disproportionate short stature reports progressive, bilateral knee pain. She has a history of mild short-limbed dwarfism but normal spine radiographs. Lateral knee radiographs reveal a distinctive bony anomaly. Which of the following is the pathognomonic finding for her likely condition?

. Erlenmeyer flask deformity of the distal femur
. Bipartite patella at the superolateral pole
. Double-layer patella
. Popliteal exostosis
. Dysplastic medial femoral condyle

Correct Answer & Explanation

. Double-layer patella


Explanation

Multiple Epiphyseal Dysplasia (MED) can be caused by mutations in COMP, MATN3, or type IX collagen genes. A double-layer patella visualized on the lateral radiograph is a highly characteristic and pathognomonic finding for MED.