4. Pediatrics MCQs

The principal abnormality associated with Holt-Oram syndrome is:

The hereditary pattern for Holt-Oram syndrome is:

A 15-day-old boy presents with deformity of the right hand. The boy was delivered prematurely and underwent an urgent arterial switch for transposition of great vessels. The patient is in stable condition. He has a radial club hand, and because the radial head cannot be palpated, total absence of radius is suspected. The thumb is absent and the index finger has camptodactyly. The forearm is short compared to the left side, and the patient flexes his elbow upon stimulation. Spontaneous finger motion is also present. A thorough physical examination is performed and a set of investigations is ordered. The results are as follows: complete blood count 10,000/µL; platelet 254×10^3/µL; neutrophils 50%; Hb 14.2 g/dL; lymphocytes 40%; Hct 45%; and monocytes 10%. No renal abnormalities were noted on ultrasonogram of the abdomen. A radiograph of the spine is normal. The next step in the management of the radial club hand is:

A 15-day-old boy presents with deformity of the right hand. The boy was delivered prematurely and underwent an urgent arterial switch for transposition of great vessels. The patient is in stable condition. He has a radial club hand, and because the radial head cannot be palpated, total absence of radius is suspected. The thumb is absent and the index finger has camptodactyly. The forearm is short compared to the left side, and the patient flexes his elbow upon stimulation. Spontaneous finger motion is also present. A thorough physical examination is performed and a set of investigations is ordered. The results are as follows: complete blood count 10,000/µL; platelet 254×10^3/µL; neutrophils 50%; Hb 14.2 g/dL; lymphocytes 40%; Hct 45%; and monocytes 10%. No renal abnormalities were noted on ultrasonogram of the abdomen. A radiograph of the spine is normal. When the patient is 10 years old, he is not satisfied with the length of his forearm and wishes to lengthen it. Which of the following is not a satisfactory recommendation:

Which of the following conditions is present in patients with radial club hand but not in patients with ulnar club hand:

All of the following developmental anomalies are associated with ulnar club hand except:

Which of the following syndromes is associated with ulnar club hand:

A 1-year-old boy was born full-term and pregnancy was uneventful. However, the parents noticed deformity of the patient's forearm, which progressed with growth. The parents consulted a pediatric orthopaedic surgeon 4 months prior and were advised to observe the growth. Multiple investigations in the form of two-dimensional echogram, abdomen ultrasonography, radiographs of the spine, and complete blood work did not reveal any abnormalities. No genetic or syndromic abnormality was reported. A radiograph taken 4 months prior is shown.

A 4-month-old girl is placed in a Pavlik harness for developmental dysplasia of the hip. At a follow-up visit 2 weeks later, the parents report the infant has stopped kicking her left leg. On exam, she lacks active knee extension on the left. What is the most likely cause?

A 13-year-old obese boy presents with a 3-week history of right groin pain and a limp. He walks with an externally rotated foot. AP and frog-leg lateral pelvis radiographs confirm a mild stable slipped capital femoral epiphysis (SCFE) on the right. What is the most appropriate management?

A 6-week-old infant is being treated with a Pavlik harness for developmental dysplasia of the hip. At the 2-week follow-up, the mother reports the infant has stopped kicking the affected leg. On examination, active knee extension is absent but ankle movements are preserved. What is the most likely cause of this complication?

An 8-year-old child sustains a displaced extension-type supracondylar fracture of the humerus. After closed reduction and percutaneous pinning, examination reveals an inability to flex the interphalangeal joint of the thumb and the distal interphalangeal joint of the index finger. Which nerve structure is most likely injured?

A 13-year-old overweight boy presents with a limp and right groin pain for 3 weeks. On physical examination, as the right hip is passively flexed, the limb obligatorily rotates externally. What is the most likely diagnosis?

In the Ponseti method for the conservative management of idiopathic clubfoot, the sequence of deformity correction is strictly protocolized. Which of the following represents the correct order of correction?

A newborn infant presents with a claw hand deformity, an absent grasp reflex, but a completely preserved Moro reflex in the upper arm and shoulder. Examination also reveals mild ptosis and miosis on the ipsilateral side. Which nerve roots of the brachial plexus are predominantly involved in this injury?

Macrodactyly that is present at birth is termed:

Syndromes that may be associated with macrodactyly include:

A 2-year-old child is brought to your office for evaluation of a "big hand." Upon examination, you notice that the child has mildly enlarged ring and small fingers. There is full range of motion without instability. After examination of the patient, you recommend:

A 2-year-old child is brought to your office for evaluation of a "big hand." Upon examination, you notice that the child has mildly enlarged ring and small fingers. There is full range of motion without instability. The child's parents inform you that they would like you to amputate the affected digits as soon as possible. You should:

A 2-year-old child is brought to your office for evaluation of a "big hand." Upon examination, you notice that the child has mildly enlarged ring and small fingers. There is full range of motion without instability. After examination of the patient, you discuss the diagnosis of macrodactyly with the parents. The parents feel assured after your discussion of the disease process and your review of the radiographs. You should next see the patient: