Pediatric Examination and Board Review (44 page)

6.
(E)
Significant sensorineural hearing impairment could be etiologically related to congenital infections, such as rubella, CMV, toxoplasmosis, and syphilis. The peak vulnerability for viral teratogenesis occurs during the first trimester. Embryologically this occurs for the eighth nerve ganglion at 30 days, and cochlear duct and superior colliculus at 16 weeks.

7.
(A)
Significant sensorineural hearing impairment could be etiologically related to a genetic disorder (prolonged QT, Waardenburg, Usher, hypothyroidism, malformations of the cochlea), geneticmetabolic disorders (Hurler, Cockayne, osteogenesis imperfecta), and molecular disorders (connexin-26 mutations).

8.
(E)
Pituitary function tests are not indicated for the workup of deafness. However, if growth failure and/or septo-optic dysplasia is present, appropriate endocrine studies are indicated.

9.
(B)
Infants and toddlers benefit from sign language, Early Intervention, medical and genetic evaluation, and discussions with a pediatric neuro-otologist regarding cochlear implantation.

10.
(D)
Although 4-year-olds know colors, shapes, and words of songs and rhymes, they do not typically print their names or remember phone numbers until age 5 years.

11.
(E)
IDEA 97 provides an array of supports for children with disability, including transportation, augmentative communication aids, and both supports and modifications of the curriculum.

12.
(D)
The Americans with Disability Act provides for appropriate accommodations during testing. IDEA provides for free appropriate public education in the least restrictive environment with supports necessary to access learning.

13.
(C)
Children who are unable to sit, crawl, or pull to stand at age 5 years are unlikely to walk. All children who can sit at age 2 years can walk. Many children who sit at 4 years can ultimately walk. Casey did not sit at age 2 or 4 years.

14.
(D)
Casey’s motor control allows him to access switches but not manipulate objects. He will not be able to manipulate crayons if he cannot manipulate common objects like spoons.

Current public school law provides modifications and supports so that Casey can optimize his participation in learning activities with peers. His mother is appropriately ahead of the school system in her request for appropriate learning and educational supports.

The key developmental issue is whether or not the child can hear some speech with amplification. If he cannot, then total communication or coordination of cochlear implantations with intense aural rehabilitation is required.

S
UGGESTED
R
EADING

Cheney PD, Palmer FB. Cerebral palsy.
Ment Retard Dev Disabil Res Rev.
1997;3:109-219.

Gifford KA, Holmes MG, Berstein HH. Hearing loss in children.
Pediatr Rev.
2009;30(6):207-215.

Nelson KB. The epidemiology of cerebral palsy in term infants.
Ment Retard Dev Disabil Res Rev.
2002;8:146-150.

Palmer FB, Hoon AH. Cerebral Palsy. In: Augustyn M, Zuckerman B, Caronna EB, eds.
Developmental and Behavioral Pediatrics: A Handbook for Primary Care
, Philadelphia, PA: Lippincott, Williams & Wilkins; 2011:164-171.

Straub K, Orbutz JE. Effects of cerebral palsy on neuropsychological function.
J Dev Phys Disabil
. 2009;21(2):153-167.

Willis LM, Willis, KE. Hearing Loss and Deafness. In: Augustyn M, Zuckerman B, Caronna EB, eds.
Developmental and Behavioral Pediatrics: A Handbook for Primary Care
, Philadelphia, PA: Lippincott, Williams & Wilkins; 2011:242-249.

CASE 24: A 1-YEAR-OLD WITH EARLY HANDEDNESS

A 1-year-old boy named Adam presents with motor delay at a physical examination with a new doctor. The mother reports that he has been “left-handed” since 6 months of age. The mother worries that Adam is not able to do the things his older brother was able to do at a similar age.

Adam weighed 6 pounds at birth and was born at 38 weeks’ gestational age. There were no prenatal, perinatal, or postnatal concerns except for his motor delays. Records from his previous doctor note that a strong startle reflex (Moro) was elicited on several occasions in the first 2 months of life.

On physical examination, among other things, you note that his right hand is slightly smaller than his left. He smiles symmetrically. He is able to sit but when pushed to his right, he falls. When prone, his right arm cannot do a marine pushup. In vertical suspension, he weight-bears on his legs. His lower extremity reflexes and tone are normal. There are no birthmarks.

SELECT THE ONE BEST ANSWER

1.
What other examination findings might be present?

(A) ease with pronation and supination of the right arm

(B) ease transferring across the midline to take an object from the left hand

(C) difficulty doing a pincer with the right hand

(D) inability to finger-feed with his left hand

(E) C and D

2.
What type of motor disorder might this be?

(A) diplegic CP

(B) brachial plexus palsy

(C) hemiplegic CP

(D) Sturge-Weber syndrome

(E) none of the above

3.
All of the following supportive tests are indicated except

(A) MRI of brain

(B) electroencephalograph (EEG)

(C) Wood’s lamp examination

(D) urine culture for CMV

(E) plasma amino acid determination

4.
Adam’s mother asks you if the MRI and EEG are being ordered to make sure that Adam doesn’t have a brain tumor. What should you tell her?

(A) the reason we are ordering the MRI is to make sure there is no brain tumor; she is correct

(B) the reason we are ordering an MRI is to understand if there are differences between the right and left sides of Adam’s brain

(C) we are trying to find a brain lesion that could benefit from neurosurgery

(D) if the EEG reveals a focal discharge, then we can treat with anticonvulsants and not worry about motor delay

(E) all of the above

5.
Diagnostic studies are done. Adam’s MRI reveals a small left frontal porencephalic cyst. An EEG reveals no seizure activity. Plasma amino acids are normal. Wood’s lamp examination reveals no depigmented macules. An adult neurologist who is a family friend states there has been brain damage that is permanent and nothing can be done. Adam’s mother calls your office in crisis. Which of the following is true?

(A) all children with hemiplegic CP learn to walk

(B) if the lesion involves the left hemisphere, there is poor prognosis for speech

(C) if seizures occur, they are severe

(D) there may be difficulty with both visual fields

(E) Adam will require residential placement

6.
Adam’s mother read that hyperbaric oxygen treatments could help children with brain injury. She asks you to write a letter justifying this treatment to the insurance company. The responsibilities of primary care physicians to families with children with hemiplegic CP include

(A) Early Intervention referral

(B) intensive physical therapy (PT) to ensure walking

(C) orthopedic referral for consideration of inhibitive casting of the right upper extremity

(D) writing letters to the insurance companies/public aid to obtain authorization for hyperbaric oxygen treatments

(E) A and C

7.
At the age of 8 years, Adam’s intelligence was reevaluated using the Stanford-Binet Intelligence Scale IV Edition. The test resulted in an IQ of 42 (normal 100 ± 15). On the Vineland Adaptive Behavior Scales, he is at a 3- to 4-year developmental level. His articulation skills were at a 3
¹
/
₂
-year level. His receptive language skills were at a 4
¹
/
₂
-year level and his expressive language skills were at a 31/2-year level. What is Adam’s developmental diagnosis?

(A) mild mental retardation

(B) moderate mental retardation

(C) severe mental retardation

(D) learning disability

(E) none of the above

Questions 8 through 34. Prognostic Belief Scale. Based on the information provided in question 7, use the following probabilities to indicate the degree to which Adam will be able to perform the skills listed below when he matures into adulthood.

(A) 10% or less

(B) 25%

(C) 50%

(D) 90%

(E) 100%

8.
Dress and use the toilet independently

9.
Enter into a marriage contract

10.
Drink from a cup independently

11.
Cook a meal unsupervised

12.
Raise children

13.
Find his own way in unfamiliar surroundings