Pediatrics Flashcards
A patient with a history of coarctation of the aorta presents to your clinic to establish care for knee pain. On exam you notice a webbed neck and broad chest. This patient most likely suffers from which of the following?
A Turner syndrome
B Klinefelter syndrome
C Edwards syndrome
D Down syndrome
Answer: A
Explanation:
• Turner syndrome involves a 45,XO karyotype, webbed neck, shield chest, and coarctation of the aorta.
• Down syndrome is trisomy 21 with upward slanting eyes, “simian” crease, cardiac disease (e.g. endocardial cushion defects).
• Klinefelter syndrome involves a male presenting with tall stature, gynecomastia, and small testicles, with an 47,XXY genotype.
• Edwards syndrome is not high-yield enough to know.
An 8 year-old male presents with gradual onset right hip pain for the past 1 month. He denies trauma, numbness, tingling, or weakness. He has no past medical history, and has maintained a normal body mass index (BMI). He suffered from an upper respiratory infection (URI) 2 weeks ago, but otherwise has been well. Hip x-rays demonstrate sclerosis of the femoral head. Which of the following is the most likely etiology of this condition?
A Trauma
B Poor blood supply
C Viral illness
D Movement of the femoral head upon the growth plate
Answer: B
Explanation:
• Legg-Calve-Perthes disease involves idiopathic loss of blood supply to the femoral head, leading to femoral head avascular necrosis (AVN), which can be seen on x-rays as femoral head sclberosis and ultimately femoral head collapse.
• Treatment may involve observation, rest, bracing, physical therapy, pain control, and/or orthopedic surgery.
• Slipped capital femoral epiphysis and transient synovitis would demonstrate sudden-onset hip pain, not gradual.
• This patient has not had trauma, per the question stem, and trauma would more likely cause sudden-onset pain regardless.
A 14 year-old obese male develops sudden-onset left hip groin pain. He walks with a trendelenburg gait. X-rays reveal 17% slippage of the femoral metaphysis under the epiphysis. What is the most appropriate next step?
A Pavlik harness
B Abduction brace
C Non-weight-bearing progressing to weight-bearing as tolerated
D Orthopedic surgery consult
Answer: D
Explanation:
• Adolescent obese males are at greatest risk for slipped capital femoral epiphysis (SCFE), in which the metaphysis of the femur slips under the epiphysis (like ice cream falling off a cone).
• These patients require orthopedic surgery (ORIF) and screening for endocrine abnormalities.
• SCFE is graded on the % slippage that occurs.
• Grade 1: <33% slippage.
• Grade 2: 33-50% slippage.
• Grade 3: >50% slippage.
• There is no Grade 4
You are examining a newborn baby. With the baby supine, you decide to flex both knees in order to compare how high and how far their knees extend. Which of the following is the name of this test?
A Pavlik
B Barlow
C Ortolani
D Galleazzi
Answer: D
Explanation:
• This question describes the Galleazzi test; by comparing both knees (how high and how far out they extend) you are essentially comparing the femur and tibial lengths of both legs.
• This is a test for congenital hip dislocation.
• If a dislocated hip is present, the knee will appear lower on the side of the dislocated hip.
• The Barlow test involves hip and knee flexion and adduction while applying a posteriorly directed force to dislocate the hip intentionally.
• The Ortolani test involves relocating a dislocated hip (via the Barlow maneuver) by abducting the thighs while applying an anterior and medially directed force upon the femur. The “clunk” of relocation is a positive test for developmental hip dysplasia/congenital hip dislocation.
A 14 year-old obese male develops sudden-onset left hip groin pain. He walks with a trendelenburg gait. X-rays reveal 40% slippage of the femoral metaphysis under the epiphysis. What is the grade of injury that can be assigned in this case, based on the x-ray findings?
A Grade 1
B Grade 2
C Grade 3
D Grade 4
Answer: B
Explanation:
• Adolescent obese malbes are at greatest risk for slipped capital femoral epiphysis (SCFE), in which the metaphysis of the femur slips under the epiphysis (like ice cream falling off a cone).
• These patients require orthopedic surgery (ORIF) and screening for endocrine abnormalities.
• SCFE is graded on the % slippage that occurs.
• Grade 1: <33% slippage.
• Grade 2: 33-50% slippage
• Grade 3: >50% slippage.
• There is no Grade 4.
You examine a newborn in the nursery and detect positive Galleazzi and Ortolani tests. Which of the following interventions is the most reasonable for this patient?
A Pavlik harness
B Van Ness rotation
C Physical therapy
D Observation
Answer: A
Explanation:
• The Galleazzi, Barlow, and Ortolani tests detect congenital hip dislocation.
• In these patients a Pavlik harness is recommended for a few months in order to keep the femurs in a flexed and abducted position so that the femur stays within the acetabulum and avascular necrosis (AVN) of the femoral head does NOT occur.
• Observation and PT without a Pavlik harness would risk AVN.
• Van Ness rotation is a surgery performed for patients with partial proximal femoral focal deficiency (PFFD).
At which of the following ages in normal development does a child typically begin to walk?
A 18 months
B 12 months
C 9 months
D 6 months
Answer: B
Explanation:
• 12 months of age is typically when a child begins to walk (give or take a month).
- A 16 year-old male develops bone cancer of the distal femur. This is most likely which of the following?
A Osteosarcoma
B Osteoma
C Multiple myeloma
D Ewing Sarcoma
Answer: A
Explanation:
• Osteosarcoma is the most common primary bone cancer overall, and is typically found in the knee.
A 16 year-old male develops bone cancer of the distal femur. This is most likely which of the following?
A Osteosarcoma
B Osteoma
C Multiple myeloma
D Ewing Sarcoma
Answer: A
Explanation:
• Osteosarcoma is the most common primary bone cancer overall, and is typically found in the knee.
A 9 year-old male presents with muscle spasms. On exam you notice overall muscular hypertrophy and difficulty relaxing his hand after a handshake. EMG reveals a “divebomber” finding in multiple muscles. Which of the following is the most reasonable next step?
A Lumbar puncture
B Repeat EMG in 6-12 months
C Muscle biopsy
D Physical therapy
Answer: D
Explanation:
• This patient presents with classic myotonia congenita, a genetic disorder resulting in myotonia, spasms, and muscle hypertrophy starting at birth, and made worse by cold weather.
• Patients are unable to smoothly grasp and release an object (trouble relaxing muscles after contraction).
• Often this condition can be managed by observation, activity modification, and physical therapy.
• Genetic testing a reasonable option for diagnosis, but muscle biopsy and lumbar puncture would NOT be required for this.
• Serial EMGs would yield little new information unless the patient was symptomatic with progressive weakness.
A 7 year-old male presents with progressive weakness and disability over the past year. He has been otherwise healthy and has sustained no trauma or recent infections. On exam you notice that during ambulation he exhibits a hyperlordotic posture, bilateral trendelenburg gait, and walks on his toes. Despite his weakness, his calves appear large. EMG reveals early recruitment in the muscles studied. Which of the following is the most likely etiology of this patient’s condition?
A Xp21 mutation
B Immune system cross-reactivity
C Intrauterine TORCH infection
D SMN1 mutation
Answer: A
Explanation:
• This patient presents with classic Duchenne Muscular Dystrophy (DMD), due to gene Xp21 mutation leading to essentially NO dystrophin protein being produced.
• This causes a severe progressive myopathy in early childhood in which the normal muscle tissue is replaced by fibrosis, starting with the neck flexors (this is why the calves appear large - calf pseudohypertrophy due to fibrosis).
• Myopathies cause early recruitment of muscle fibers on EMG, while neuropathic conditions cause decreased recruitment.
• Becker muscular dystrophy is due to low amounts of dystrophin (not complete absence), and is less severe with a later onset than early childhood.
• SMN1 mutation causes spinal muscular atrophy, which has 3 different types, the third of which could feasibly present similarly to this patient, but EMG would show decreased recruitment, NOT increased, due to anterior horn cell death.
• Immune system cross-reaction causes Guillain-Barre Syndrome, but in this patient without recent infection (URI or GI illness), and with NO sensory abnormalities. With EMG evidence of myopathy (NOT neuropathy) in this case, GBS is less likely.
A young boy speaks with 75% of his words being intelligible and walks up stairs using alternating feet (does not stand with two feet on a single stair), and who can successfully ride a tricycle, is likely approximately how many years old?
A 4
B 3
C 2
D 1
Answer: B
Explanation:
• At 3 years of age, a child should use 3-word sentences, have 75% intelligible speech, operate a tricycle, and walk upstairs using alternating feet.
A 15 year-old male with a history of obesity presents with sudden-onset left hip pain. He denies trauma. Hip x-rays demonstrate a widened growth plate and femoral head sliding off the growth plate by approximately 20%. Which of the following is the most appropriate treatment for this problem?
A Orthopedic surgery
B Physical therapy
C Bracing
D Observation
Answer: A
Explanation:
• Slipped capital femoral epiphysis (SCFE) involves sudden-onset hip/groin pain due to the femoral head sliding off the growth plate, typically in obese adolescent males.
• X-ray grading is defined by the percentage of slippage of the femoral head off the growth plate.
• Grade 1: 0-33% sliding.
• Grade 2: 34-50% sliding.
• Grade 3: >50% sliding.
• There is no grade 4.
• Treatment is orthopedic surgery.
Which of the following juvenile rheumatoid arthritis (JRA) diseases carries the worst prognosis? RF: rheumatoid factor.
A Polyarticular, RF+
B Polyarticular, RF-
C Pauciarticular, RF+
D Pauciarticular, RF-
Answer: A
Explanation:
• Polyarticular, RF+ Juvenile Rheumatoid Arthritis carries the worst prognosis and should be treated early.
What is the most common site for pressure injuries in children?
AHeel
BSacrum
CGreater trochanter
DOcciput
Answer: D
Explanation:
• In adults the sacrum is the most common location for developing pressure injuries (pressure ulcers).
• In children the occiput is the most common site.
An otherwise healthy 6 year-old boy complains of pain in the groin that extends distally into his medial thigh and medial knee. Imaging reveals avascular necrosis of the femoral head. After orthopedic consultation, physical therapy, reduced weight bearing, and bracing are recommended. In what position should the hip be placed?
AExternally rotated, slight hip adduction
BExternally rotated, hyperabduction
CExternally rotated, slight hip extension
DInternally rotated, slight hip flexion
Answer: B
Explanation:
• The likely diagnosis of avascular necrosis (AVN) of the hip in a child age 4-10 is Legg-Calve Perthes disease.
• This refers to idiopathic AVN of the femoral head in children. It often presents as hip pain that may radiate into the ipsilateral thigh or knee.
• The goal of bracing, when indicated, is to promote a hip position that places the femoral head completely within the acetabulum; this is attained with external rotation and hyperabduction.
- In the newborn nursery you decide to examine a baby. With the thighs abducted and flexed, you apply an anterior and medially directed force upon the femur and detect a palpable “clunk”. Which of the following is the name of this test?
A Pavlik
B Barlow
C Ortolani
D Galleazzi
Answer: C
Explanation:
• Barlow test: Involves hip and knee flexion and adduction while applying a posteriorly directed force to dislocate the hip intentionally
• Ortolani test: Involves relocating a dislocated hip (via the Barlow maneuver) by abducting the thighs while applying an anterior and medially directed force upon the femur. The “clunk” of relocation is a positive test for developmental hip dysplasia/congenital hip dislocation.
• Galleazzi test: By comparing both knees (how high and how far out they extend) you are essentially comparing the femur and tibial lengths of both legs. This is a test for congenital hip dislocation. If a dislocated hip is present, the knee will appear lower on the side of the dislocated hip.
In the newborn nursery you decide to examine a baby. With the thighs abducted and flexed, you apply an anterior and medially directed force upon the femur and detect a palpable “clunk”. Which of the following is the name of this test?
A Pavlik
B Barlow
C Ortolani
D Galleazzi
Answer: C
Explanation:
• Barlow test: Involves hip and knee flexion and adduction while applying a posteriorly directed force to dislocate the hip intentionally
• Ortolani test: Involves relocating a dislocated hip (via the Barlow maneuver) by abducting the thighs while applying an anterior and medially directed force upon the femur. The “clunk” of relocation is a positive test for developmental hip dysplasia/congenital hip dislocation.
• Galleazzi test: By comparing both knees (how high and how far out they extend) you are essentially comparing the femur and tibial lengths of both legs. This is a test for congenital hip dislocation. If a dislocated hip is present, the knee will appear lower on the side of the dislocated hip.
Regarding pediatric prosthetics, approximately how often should a left transradial prosthesis be prescribed between ages 14 and 20?
A Every 3 years
B Every 2 years
C Every 18 months
D Every 1 year
Answer: B
Explanation:
• Between ages 0-5 the prosthesis should be replaced approximately annually.
• Between ages 5-12 the prosthesis should be replaced approximately every 18 months.
• Between ages 12-21 the prosthesis should be replaced approximately every 2 years.
A 6 year-old female presents with pain of sudden-onset left hip pain. Her mother denies any history of trauma. Hip x-rays are negative for acute findings. She has no prior medical history and has maintained a normal body mass index (BMI). Her mother reports that the patient had a cough and runny nose 1 week ago, but has otherwise been well. Exam reveals intact strength, sensation, and lower limb reflexes. Which of the following is the most likely diagnosis?
A Slipped capital femoral epiphysis (SCFE)
B Septic arthritis
C Transient synovitis
D Avascular necrosis of the hip (AVN)
Answer: C
Explanation:
• This patient presents with classic transient synovitis.
• This involves sudden inflammation of the hip joint synovium, leading to sudden-onset hip pain; this commonly is due to a recent upper respiratory tract infection (URI) which “moves out” of the upper airways and into the hip, resulting in synovitis and pain.
• Hip x-rays are typically normal. Labs may show elevated erythrocyte sedimentation rate (ESR).
• Transient synovitis is usually self-limiting. Treatment involves rest, ice, NSAIDs, physical therapy.
• AVN of the hip would show x-ray changes of femoral head sclerosis, and the pain would be gradual onset.
• Septic arthritis would show fever, leukocytosis, swelling, and erythema.
• SCFE classically occurs in obese adolescent males with sudden-onset hip pain due to the femoral head sliding off the growth plate, and is diagnosed on hip x-rays.
A 16 year-old male develops bone cancer of the distal femur. This is most likely which of the following?
A Osteosarcoma
B Osteoma
C Multiple myeloma
D Ewing Sarcoma
Answer: A
Explanation:
• Osteosarcoma is the most common primary bone cancer overall, and is typically found in the knee.
Which of the following is the most common type of congenital limb deficiency?
A Right fibular hemimelia
B Left fibular hemimelia
C Right terminal transradial deficiency
D Left terminal transradial deficiency
Answer: D
Explanation:
• Left terminal transradial deficiency is the most common overall congenital limb deficiency.
○ It involves no limb being present beyond a transverse limb deficit across the radius.
• Fibular hemimelia in general is the most common lower limb congenital limb deficiency.
○ It involves the fibula bone itself being absent.
In the newborn nursery you decide to examine a baby. You flex and adduct both hips while applying a posteriorly directed force in an attempt to intentionally dislocate the hip. Which of the following is the name of this test?
A Pavlik
B Barlow
C Ortolani
D Galleazzi
Answer: B
Explanation:
• Barlow test involves hip and knee flexion and adduction while applying a posteriorly directed force to dislocate the hip intentionally.
• Ortolani test involves relocating a dislocated hip (via the Barlow maneuver) by abducting the thighs while applying an anterior and medially directed force upon the femur.
○ The “clunk” of relocation is a positive test for developmental hip dysplasia/congenital hip dislocation.
• Galleazzi test, by comparing both knees (how high and how far out they extend) you are essentially comparing the femur and tibial lengths of both legs.
○ This is a test for congenital hip dislocation.
○ If a dislocated hip is present, the knee will appear lower on the side of the dislocated hip.
