Question 4821
Topic: 4. PediatricsWhich zone of the physis (growth plate) is the primary site of cellular abnormality in a patient with achondroplasia?
Correct Answer & Explanation
. Reserve zone
Practice Set 242 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.
Which zone of the physis (growth plate) is the primary site of cellular abnormality in a patient with achondroplasia?
. Reserve zone
A 6-week-old female infant is currently being treated with a Pavlik harness for Developmental Dysplasia of the Hip (DDH). During her 2-week follow-up appointment, the mother reports that the infant is no longer kicking her left leg. On examination, the infant exhibits a lack of spontaneous knee extension on the left side, though ankle and toe movements are intact. What is the most appropriate next step in management?
. Adjust the anterior strap to increase hip flexion
A 12-year-old obese male presents with a 4-week history of left groin pain and a limp. He is diagnosed with a stable left slipped capital femoral epiphysis (SCFE) and undergoes in situ pinning. Prophylactic pinning of the contralateral asymptomatic hip is widely debated but is most strongly indicated in patients with which of the following underlying conditions?
. Type 1 Diabetes Mellitus
. AP radiograph; the lateral pillar retains <50% of its original height
. Full tibialis anterior tendon transfer (TATT) to the lateral cuneiform
A 6-year-old girl with spastic quadriplegic cerebral palsy (GMFCS Level V) is evaluated in the multidisciplinary hip surveillance clinic. Her AP pelvis radiograph demonstrates a Reimers migration percentage (MP) of 55% on the right hip with associated acetabular dysplasia. What is the most widely accepted surgical intervention for this degree of subluxation?
. Adductor and iliopsoas tenotomies alone
A 2-year-old boy is brought to the clinic for bilateral bowing of the lower extremities. The physician is trying to differentiate between physiological genu varum and infantile Blount's disease. Measurement of the metaphyseal-diaphyseal (MD) angle of Drennan on standing AP radiographs is performed. Which of the following MD angles most strongly indicates a high likelihood of progression to true infantile Blount's disease?
. 5 degrees
A 4-year-old female with blue sclerae and a history of multiple long bone fractures after minimal trauma is diagnosed with Osteogenesis Imperfecta (OI). Genetic testing is ordered. The primary pathophysiology of this condition is a mutation in the COL1A1 or COL1A2 genes leading to the substitution of a crucial amino acid in the triple helix of Type I collagen. Which of the following amino acids is abnormally substituted in this disorder?
. Proline
Achondroplasia is the most common form of skeletal dysplasia and short-limb dwarfism. The underlying genetic defect is an activating mutation in the Fibroblast Growth Factor Receptor 3 (FGFR3) gene. In which zone of the epiphyseal growth plate does this mutation primarily exert its inhibitory effect?
. Reserve (resting) zone
. Café-au-lait spots
. Central, Posteromedial, Anterolateral
A pediatric orthopedic surgeon is discussing ambulation prognosis with the parents of a child born with myelomeningocele (spina bifida). The infant has a documented neurologic level of L4. Assuming appropriate orthopedic care and bracing are provided, what is the most likely lifelong ambulatory potential for this patient?
. Household ambulator only, relying on a wheelchair for community transport
A 15-month-old child undergoes open reduction for a neglected developmental dysplasia of the hip (DDH) via an anterior approach. During the procedure, several intra-articular anatomical obstacles to reduction are encountered and addressed. Which of the following structures is located most inferiorly and must be incised to allow the femoral head to seat concentrically in the true acetabulum?
. Ligamentum teres
. Medial circumflex femoral artery
A 6-month-old infant is brought to the emergency department for irritability and swelling of the right knee. Radiographs reveal a distal femoral fracture characterized by a thin layer of metaphyseal bone avulsed at the periphery of the physis. Which of the following best describes the specificity of this injury for non-accidental trauma (child abuse)?
. Low specificity; commonly caused by normal infant rolling
. Walks using a handheld mobility device (e.g., walker or crutches) in most indoor settings and uses wheeled mobility for long distances
A 5-year-old child with spastic quadriplegic cerebral palsy presents for a routine visit. Gross Motor Function Classification System (GMFCS) level is V. Radiographs show a migration percentage of 55% bilaterally with an acetabular index of 35 degrees. What is the most appropriate next step in management?
. Observation with repeat radiographs in 1 year
A 4-year-old boy presents with a recurrent clubfoot deformity after successful initial Ponseti casting and Achilles tenotomy in infancy. The mother notes he drops his foot and walks on the lateral border. Examination reveals dynamic supination during the swing phase of gait. Passive range of motion allows the foot to be brought to neutral. What is the most appropriate management?
. Repeat Ponseti casting followed by a lateral sliding calcaneal osteotomy
A 12-year-old obese boy presents to the emergency department with severe acute left hip pain after slipping on ice. He is completely unable to bear weight, even with crutches. Radiographs show a severe left Slipped Capital Femoral Epiphysis (SCFE). Which of the following complications is he at greatest risk for compared to a patient who presents with an ability to bear weight?
. Chondrolysis
. Maintenance of lateral pillar height greater than 50%