Pediatric Examination and Board Review (43 page)

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.

Parker S, Magee T. Colic. In: Augustyn M, Zuckerman B, Caronna EB, eds.
Developmental and Behavioral Pediatrics: A Handbook for Primary Care
, Philadelphia, PA: Lippincott, Williams & Wilkins; 2011:182-186.

Piper MC, Darrah J.
Motor Assessment of the Developing Infant
. Philadelphia, PA: WB Saunders; 1994:114-139.

CASE 23: A 4-YEAR-OLD BOY WITH MOTOR DISABILITY PREPARING TO TRANSITION TO PUBLIC SCHOOL KINDERGARTEN

Casey is a 4-year-old boy getting ready for transition to public school. He has been diagnosed with CP.

Birth history: Pregnancy was complicated by a placental abruption at 36 weeks’ gestation. This occurred during a sailing party in a rural area. It took approximately 6 hours to reach an emergency department and an additional 3 hours to bring together anesthesiology and obstetrics for a cesarean delivery. Casey weighed 7 pounds at birth. The neurologist informed the parents that Casey did suffer brain damage; however, there was a chance that future disability could be minimal.

Developmental history: At 5 months, Casey entered an Early Intervention program. As an infant, he was alert and responsive to social interactions, but his motor control and coordination were poor. By the age of 4 years he could use a touch talker to communicate basic needs and could use a switch to direct his powered wheelchair. His parents, particularly his mother, are very involved with his learning and have a positive partnership with his preschool. An intelligent and well-educated person, his mother communicates and listens to the professionals and provides useful input regarding Casey’s learning styles and ways to promote his development.

Physical examination reveals an alert, social boy in a wheelchair. He laughed when you told him that your favorite summer food was ice cream. His growth is at the 25th percentile. His neurologic examination reveals full extraocular movements, difficulty with putting his tongue to lips, chin, or cheeks on request, and inability to repeat common sounds or words. He can indicate his needs with a picture board. His lower extremity reflexes are 3+ at the knees, and sustained clonus is elicited at the ankles. With eye gaze, he identifies shapes and colors and has prepositional concepts and counting skills.

As Casey prepares his transition to a public school setting, his mother has requested a team meeting with the physical therapist, teacher, and psychologist. She says that Casey has many more capabilities than are shown by his motor skills. She is dissatisfied with timed assessments of Casey and wants the staff to use modifications that will accurately reflect Casey’s abilities. She states that Casey should be mainstreamed with a “sameage” kindergarten class.

SELECT THE ONE BEST ANSWER

1.
What characteristic features are not necessary for considering the diagnosis of hypoxemic ischemic encephalopathy?

(A) cord pH lower than 7.1

(B) severe respiratory depression

(C) neonatal seizures

(D) multisystem organ failure

(E) serially abnormal neurologic examination performed in the first 72 hours

2.
What types of associated neurodevelopmental disorders rarely accompany CP attributed to hypoxemic ischemic encephalopathy?

(A) epilepsy

(B) mental retardation

(C) learning disability

(D) hearing impairment

(E) feeding disability

3.
If a child with CP has a hearing impairment, what is least likely to contribute to the hearing loss phenotype?

(A) peak bilirubin of 15 mg/dL in the newborn period requiring phototherapy for 24 hours

(B) family history of early childhood hearing loss

(C) gray forelock in his grandmother

(D) use of extracorporeal membrane oxygenation (ECMO) for persistent fetal circulation after meconium aspiration syndrome

(E) all of the above are likely to contribute to the hearing impairment

4.
If audiologic testing at 10 months revealed a severe and possibly profound bilateral hearing loss with mild abnormalities on tympanometry and the child had no motor or developmental delays, all of the following developmental communication outcomes are likely except

(A) ability to learn sign language

(B) difficulty in learning oral language compared with non–hearing-impaired peers

(C) no speech delay, if the middle ear abnormality is corrected

(D) recognition of spoken words, if cochlear implantation surgery has taken place

(E) A and C are both exceptions

5.
When aided binaurally by hearing aids and when given intensive speech/language therapy and audiologic training, increased attention to sound is noted. A follow-up audiogram reveals aided responses in the mild range at 250 Hz and moderate at 500 to 4000 Hz. What might be the consequences of improved speech recognition?

(A) turning to loud sounds

(B) immediately beginning to talk in words

(C) learning to say some single-word approximations

(D) imitating the sounds that animals make

(E) beginning to like the music in a children’s television show

6.
Each of the following first-trimester illnesses are commonly associated with hearing loss in the newborn except

(A) toxoplasmosis

(B) CMV

(C) syphilis

(D) rubella

(E) influenza A

7.
All of the following syndromes are associated with severe sensorineural hearing loss except

(A) Klinefelter syndrome

(B) Waardenburg syndrome

(C) Hurler syndrome

(D) Usher syndrome

(E) prolonged QT syndrome

8.
All of the following diagnostic tools may be helpful in identifying the etiology of hearing loss except

(A) computed tomography (CT) scan of temporal bones

(B) molecular test of connexin-26 mutations

(C) dilated funduscopic examination

(D) electrocardiogram (ECG)

(E) pituitary function tests

9.
Of the following family actions, which is least likely to be helpful?

(A) having all family members learn sign language

(B) sending child to an out-of-state residential school

(C) enrolling child in Early Intervention

(D) testing other adult family members

(E) exploring options for cochlear implants

10.
When using a touch talker, what output by Casey might indicate that he is above average in developmental skills?

(A) correctly identifying colors

(B) correctly naming shapes

(C) knowing the next word in a song

(D) typing his full name and phone number

(E) none of the above

11.
What are the appropriate support services at kindergarten entry that are included in IDEA 1997 (Individual with Disability Education Act), the federal law specifying evaluation and support for children with disabilities?

(A) wheelchair bus transportation

(B) augmentative communication services

(C) adapted physical education

(D) classroom aide

(E) all of the above

12.
All of the following are key features of IDEA 1997 except

(A) free public education

(B) least restrictive environment

(C) rehabilitation services that increase a child’s ability to learn

(D) no modifications during standardized testing

(E) no exceptions; all of the above are key features

13.
At the age of 5 years, Casey can count to 20, knows the alphabet, can match sounds with letters, and loves books like
The Cat in the Hat
. All of the following are reasonable short-term goals for Casey except

(A) learning to read

(B) learning addition and subtraction

(C) learning to walk

(D) going to adaptive aquatics

(E) A and D

14.
The least important goals for Casey in kindergarten include

(A) learning to take turns in groups

(B) following the teacher’s directions

(C) making friends

(D) learning how to write with a pencil in cursive

(E) B and C

ANSWERS

1.
(A)
Casey survived an unanticipated catastrophic event. It is important to realize that both the lay public and the American legal system often think that all at-risk obstetric events can be known and effectively prevented and that all neurologic sequelae result in total disability. To attribute CP clinically to hypoxemia and ischemia, there must be a clustering of neonatal indicators, such as blood pH less than 7 and neonatal respiratory depression, as well as associated disorders, such as neonatal seizures and organ dysfunction (cardiac, renal, bone marrow, hepatic) accompanied by sequentially abnormal neurologic examinations.

2.
(C)
Although Casey had severe motor sequelae, he was spared major cognitive, visual, auditory, and learning sequelae.

3.
(A)
Family history, ECMO, and Mendelian disorders like Waardenburg syndrome (partial albinism, medial displacement of inner canthi, and deafness in 25% follows autosomal dominant inheritance) are known etiologies for congenital hearing loss.

4.
(C)
Mild tympanographic abnormalities do not cause hearing loss of more than 70 db. Children with severe to profound hearing loss can learn signs or recognize some speech with cochlear implants. They have delays in learning oral language. Although a 25-db hearing loss can occur with persistent middle ear effusion, middle ear surgery alone will not correct the audiogram to normal.

5.
(C)
Improved speech recognition is initially associated with learning to make speech sounds, especially single words or single-word approximations. A child with moderate hearing loss will turn to loud sounds, imitate sounds of animals, and begin to like children’s songs.