Pediatric Examination and Board Review (14 page)

FIGURE 5-1.
Pectus excavatum. Adolescent with a pectus excavatum deformity. Note that the most pronounced sternal curvature is in the lower half. (Reproduced, with permission, from Doherty G. Current Diagnosis & Treatment: Surgery, 13th ed. New York: McGraw-Hill; 2010: Fig. 43-4.)

14.
(A)
The most common heart lesions occurring in patients with Marfan syndrome include mitral valve prolapse and dilatation of the aortic root due to abnormalities in the connective tissue as a result of a mutation in the fibrillin gene. Aortic stenosis because of a bicuspid aortic valve and coarctation of the aorta are commonly seen in patients with Turner syndrome, and left ventricular outflow tract obstruction may be seen in patients with severe forms of hypertrophic cardiomyopathy. The typical examination feature in a patient with mitral valve prolapse includes a systolic ejection click that varies in timing when the patient is standing versus when he is squatting. A diastolic murmur of mitral regurgitation may be heard in conjunction with more severe cases of mitral valve prolapse.

15.
(D)
In patients with Marfan syndrome, participation in activities is limited to mildly aerobic low-impact sports. This is to avoid precipitation of further aortic root dilation or rupture and to avoid retinal detachment. It is recommended that patients with Marfan syndrome undergo routine echocardiographic evaluation to assess aortic root dilation as well as routine ophthalmologic examinations. Betablockers are effective to decrease the progression of aortic root dilation.

16.
(A)
The American Heart Association recommends that a preparticipation physical examination screening include obtaining a family history for sudden cardiac death and a review of systems looking for a history of dizziness or syncope. Physical findings of Marfan syndrome, hypertension, decreased peripheral pulses, or a pathologic murmur such as the harsh systolic murmur described would indicate the need for further evaluation. The finding of a family history of diabetes, respiratory, sinus arrhythmia, or a single elevated blood pressure returning to normal at subsequent visits would not place this patient at risk for sudden death during sports.

S
UGGESTED
R
EADING

Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, eds.
Moss and
Adams Heart Disease in Infants, Children and Adolescents.
7th ed. Philadelphia, PA: Williams and Wilkins; 2007.

Braunwald E, Zipes DP, Libbey P, eds.
Braunwald: Heart Disease: A Textbook of Cardiovascular Medicine.
7th ed. Philadelphia, PA: WB Saunders; 2004.

Keane JF, Fyler DC, Lock JE, eds.
Nadas’ Pediatric Cardiology.
2nd ed. Philadelphia, PA: Hanley and Belfus; 2006.

Park M, ed.
Pediatric Cardiology for Practitioners.
5th ed. St. Louis, MO: Mosby; 2007.

Chapter 2

CRITICAL CARE

CASE 6: A 5-MONTH-OLD WITH APNEA AND CYANOSIS

A 5-month-old male infant is brought to the emergency department after he “stopped breathing for approximately 20 seconds and became blue around his lips.” He began breathing again after the mother “blew in his face.” There have been no other symptoms such as upper respiratory tract illness, fever, vomiting, diarrhea, or change in eating habits. He takes approximately 28-34 ounces of formula per day as well as some solids and has always eaten well, although he often “spits up” after eating.

When you first see this child, he is somewhat irritable. His physical examination is completely normal except that there is dried blood in both of the child’s nares. His room air oxygen saturation is 98%. It has been approximately 90 minutes since the baby “turned blue,” and the mother appears to be quite frightened by these events.

The family history is remarkable for a sibling’s death during sleep 2 years ago, subsequently labeled as so-called SIDS (sudden infant death syndrome).

SELECT THE ONE BEST ANSWER

1.
The screening test(s) that need to be performed emergently include(s)

(A) serum glucose and electrolytes

(B) a complete blood count (CBC) and differential

(C) an erythrocyte sedimentation rate and serum C-reactive protein (CRP) level

(D) examination of cerebrospinal fluid (CSF)

(E) a computed tomography (CT) scan of the head

2.
In addition to the stat screening test performed in question number 1, the initial workup should also include

(A) a CT scan of the head, chest radiograph, and electrocardiogram (ECG)

(B) an esophageal pH probe, thyroid functions, and an EEG

(C) an echocardiogram, head CT, and thyroid function tests

(D) an abdominal radiograph, head magnetic resonance imaging (MRI), and glucose tolerance test

(E) adrenocorticotropic hormone (ACTH) stimulation test, MRI of the brain, and chest radiograph

3.
Which characteristic of this event makes it an apparent life-threatening event (ALTE)?

(A) the fear it instilled in the mother

(B) the color change in the child

(C) the apnea as described

(D) B and C only

(E) A, B, and C

4.
The family history of the previous sibling that died from “SIDS”

(A) raises suspicion of nonaccidental trauma

(B) is a common family history in a child with an ALTE

(C) increases the likelihood of future ALTEs in this patient

(D) indicates that an extensive workup in this patient for metabolic disease is needed

(E) B and C

5.
In the emergency department, the child has normal electrolytes, coagulation profile, and CBC. What is the appropriate next intervention?

(A) the child should be discharged to home with a home monitor

(B) the child should be admitted to the hospital for a period of observation

(C) the child should be discharged with scheduled workup as an outpatient for head CT and pH probe

(D) the child should be admitted to the hospital for observation and workup including head CT, eye examination, and skeletal survey

(E) A and C

6.
This child is admitted and the workup performed is negative except for posterior rib fractures on the right of ribs 9 and 10. Which is the most correct statement?

(A) the rib fractures are secondary to old birth trauma and are not relevant to the current illness

(B) the rib fractures are secondary to the resuscitation efforts of the mother in the day of admission

(C) posterior rib fractures are clear signs of prior nonaccidental trauma and should lead to a social service evaluation and police report

(D) posterior rib fractures are found in otherwise normal children and occur as a result of routine handling of an infant

(E) anterior rib fractures are clear signs of prior nonaccidental trauma and should lead to a social service evaluation and police report

7.
The percentage of “SIDS” cases that is thought to be because of nonaccidental trauma is closest to

(A) 1-5%

(B) 6-20%

(C) 21-40%

(D) 41-55%

(E) 56-60%

8.
In the child admitted with ALTE for whom a diagnosis is not made (idiopathic), the examination was normal on admission and remained normal in the hospital. Discharge plans from the hospital should include

(A) home monitoring until there is no apnea for 6 weeks, and weekly visits to the pediatrician

(B) home monitoring for 2 years and daily home nursing visits

(C) routine health care and no monitoring because there is no evidence that home monitoring prevents later death

(D) routine health care and home monitoring for 6 months if apnea does not recur

(E) routine health monitoring and home monitoring until the age of 1 year

9.
In general, the siblings of a child with an ALTE with a negative workup and no suspicion of abuse as an etiology

(A) should receive polysomnography

(B) should be placed on a home apnea monitor if younger than 8 months old

(C) should receive immediate evaluation by an appropriate health-care provider

(D) should receive routine care by an appropriate health-care provider

(E) should receive home health visits weekly

10.
In children who present with ALTE, the workup most frequently results in demonstration of

(A) no abnormality

(B) gastroesophageal (GE) reflux

(C) seizure disorder

(D) a prolonged QT syndrome

(E) electrolyte disturbances

11.
Important aspects of the history of the ALTE is/are

(A) relationship to feeding