Board Simulation: Development
Roberta E. Bauer
Mark H. Deis
QUESTIONS
1. An infant remains in a seated position when placed on your examination table by his mother. His body stiffens when you push him laterally with a gentle nudge on each shoulder. He falls backward without protecting himself when you gently push on his chest. When placed in a prone position, he immediately gets up on his hands and knees and rocks back and forth, but his mother reports that he does not yet crawl. When placed in a seated position, he picks up his mother’s keys and immediately transfers them to his other hand. His mother puts a small piece of cereal in front of him, and he picks it up between the palmar aspect of his thumb and the anatomic lateral aspect of his index finger. He turns toward you when you speak, but his mother reports that he does not seem to know his name, nor has he used any expressive word approximations. He has been babbling for approximately
2 months. He clearly enjoys your efforts to amuse him and displays no stranger anxiety. This infant is performing closest to the development expected at:
2 months. He clearly enjoys your efforts to amuse him and displays no stranger anxiety. This infant is performing closest to the development expected at:
a) 6 months
b) 7 months
c) 8 months
d) 9 months
e) This is not typical development for any age.
2. While obtaining a toddler’s history at her well-child visit, you note that she walks with an unsteady gait, taking as many as five steps at a time. Her mother reports that she has been walking for approximately 3 weeks. She also crawls quickly between objects in the room. She picks up raisins with a mature pincer grasp. Her mother tells you that they have recently installed safety locks on all the cabinets at home because the child is beginning to open the doors. When given a crayon, she first places its end in her mouth but then holds it in her fist and rubs it on paper, imitating you. She clearly responds to her name when called, and her mother believes that she is beginning to understand “no.” Her mother is able to keep her occupied by having her play with a toy from which figurines pop up when buttons are pressed. She imitates you when you play peek-a-boo with her during the physical examination, although she was clearly apprehensive when you first came near her. She has been using “dada” and “mama” nonspecifically for approximately 1 month, and her mother believes that she is beginning to use these words appropriately. This toddler is performing closest to the development expected at:
a) 10 months
b) 11 months
c) 12 months
d) 13 months
e) This is not typical development for any age.
3. A child is brought into your office for a well-child visit. He runs down the hallway and into the examination room, following his older sibling. He begins to climb up onto a chair in the room and screams in protest when his sibling pulls him down and sits there herself. His mother reports that without constant vigilance at home, he “invents new ways to cause trouble.” He is able to climb up the stairs but has not yet tried to walk up them. He is beginning to throw balls and other objects overhand but cannot quite yet kick a ball. He is able to feed himself, although he is quite messy at it. He loves to scribble. His favorite toy is one in which a small plastic hammer is used to activate various causeand-effect mechanisms. His mother estimates that he has a 15-word vocabulary, most of which consists of labels of familiar objects. He can point to his hair, eyes, and mouth when asked to do so. He again screams in protest and points to crackers that his sister has removed from their mother’s bag and has not yet shared with him. He also grunts and points to a familiar cartoon character painted on the wall, looking at his mother while doing so. His mother places him on her lap so you can examine him, and he cries at your approach. She asks him to remove his shoes, which he promptly does, throwing them to the floor. He then claps as you both cheer him. This child is performing closest to the development expected at:
a) 16 months
b) 18 months
c) 20 months
d) 22 months
e) This is not typical development for any age.
4. A child is brought in for assessment before entering preschool. The physical examination findings, including growth parameters, are typical for her age. Her mother reports that she can dress and undress by herself and is able to help with simple tasks at home, such as preparing the dinner table. She is able to ascend and descend stairs by placing one foot on each step, and recently she began riding a bicycle with training wheels. You draw various figures for her in an attempt to have her imitate you, and she is able to copy a circle and a cross, but not a square. Her speech is completely understandable. She is also able to relate a personal event and can identify four colors. This child is performing closest to the development expected at:
a) 36 months
b) 42 months
c) 48 months
d) 54 months
e) This is not typical development for any age.
Case for Questions 5-7
Amy is a 2-year-old girl who is brought to your office to be assessed for tantrums. She appears well, her physical assessment findings are normal, and her growth parameter values (height, weight, and head circumference) are in the 10th percentile. She has no stigmata of neurocutaneous diseases or syndromes. Her gross motor skills show her walking and running well, but she has a hard time walking backward when asked to, although you later see her doing it quite well when her brother chases her. She feeds herself with her fingers, can pick up her toys with her mother demonstrating, and lets her mother know what she wants by leading her to it or by pointing. She likes to play by herself and enjoys taking toys in and out of her toy box or her
mother’s cupboards. She enjoys throwing your blocks, but you can get her to stack only two. She scribbles well and prolifically and does not show a clear preference for her left or right hand. She missed her visit with you at 18 months because she had her booster at the health department, but she always passed her screening tests up to 1 year of age. Her mother says she becomes frustrated easily and does not respond to having the tantrums ignored. Your nurse could not get her to wear earphones or cooperate with the hearing screening.
mother’s cupboards. She enjoys throwing your blocks, but you can get her to stack only two. She scribbles well and prolifically and does not show a clear preference for her left or right hand. She missed her visit with you at 18 months because she had her booster at the health department, but she always passed her screening tests up to 1 year of age. Her mother says she becomes frustrated easily and does not respond to having the tantrums ignored. Your nurse could not get her to wear earphones or cooperate with the hearing screening.
5. Appropriate next steps in assessment might include:
a) Hearing screening
b) Vision screening
c) Formal developmental testing of all areas of function
d) Notifying a child protection agency regarding an inappropriate response to tantrums
e) Neurology referral
f) Magnetic resonance imaging of the brain
View Answer
Answer
The answer is a, b, and c. Amy is throwing temper tantrums, a normal issue at 2 years of age. Her mother has appropriately responded by trying to distract her and ignoring the behavior, but the strategies are not working because other frustrations are causing Amy’s tantrums. Her communication skills are significantly delayed. At 2 years of age, she is not using language to communicate her needs and has to rely on leading and pointing. If these were the only clues, we might suspect just a language disorder. With delayed expressive language, we might be concerned about hearing impairment, poor environmental stimulation and exposure to language modeling, pervasive developmental disorder of childhood, or dyspraxia. However, the description tells us that she is putting things into and taking them out of a box repeatedly, the kind of repetitive play that interests a 10- to 12-month-old, who is newly discovering that objects still exist even when you cannot see them. She does not understand verbal commands even with demonstrations (your request to walk backward or your nurse’s request to move her finger on the side where she hears the noise), and an assessment of her self-help skills shows her finger-feeding and scribbling abilities. We do not know whether she has any vision or hearing impairment, but we do know that her mother is describing delayed play, self-help, and language skills, and we need to be concerned about the global delays of MR. So, appropriate additional evaluations are a, b, and c. Notifying a child protection agency is not appropriate. Certainly if focal abnormalities were noted on the physical examination, if any questions arose of seizure activity, or if the history suggested a loss of milestones or degeneration, then a neurologic consult might be appropriate at this point, but Amy’s physical and neurologic examination findings and growth parameters are normal. An imaging study would not change the therapy in any direct way at this point and is not immediately indicated.
6. The history provided suggests which developmental delays?
a) Gross motor
b) Fine motor
c) Receptive language
d) Expressive language
e) Cognitive
f) Self-help areas
View Answer
Answer
The answer is b, c, d, e, and f. The history suggests delays in all areas except gross motor. Trisomy 21 is typically associated with a number of physical findings. A diagnosis of failure to thrive is not justified with normal growth parameters and absence of downward crossing of percentiles. High-frequency hearing loss may well present with expressive language delays, but in the absence of cognitive difficulties, it should not affect self-help and play skills so noticeably.
7. This history and physical examination findings are consistent with:
a) Trisomy 21
b) Mental retardation (MR)
c) Failure to thrive
d) High-frequency hearing loss secondary to birth injury
View Answer
Answer
The answer is b. MR is defined by significantly impaired performance on IQ testing (two standard deviations below the mean for age) coupled with impaired adaptive behavior. Therefore, for a diagnosis to be made, a child must undergo formal psychometric testing to measure the IQ. Such testing becomes stable and quite reliable past the age of 6 years and is not available before the age of 2 years. Until formal testing is available, the diagnosis of global developmental delay is appropriate. Notably, most children with MR do not have chromosomal disorders. The more profound the developmental delay, the more likely it is that a medical diagnosis is associated with the developmental issue, such as static encephalopathy, an inborn error of metabolism, or a chromosomal abnormality. In children with normal growth and no stigmata of minor or major malformation, the yield of metabolic studies is low. A family history of fetal wastage or MR should prompt a consideration of a chromosomal aberration or fragile X syndrome. Infants who are small for their gestational age or have microcephaly should be evaluated for congenital infection or anatomic abnormalities of the brain. Seizures and hard neurologic signs of upper motor neuron damage should prompt an evaluation for central nervous system injury or suggest the presence of a degenerative process. Milder forms of global developmental delay are common in fetal alcohol syndrome and familial inheritance of cognitive disorders. Two-year-olds who are not yet talking always warrant further evaluation.
Case for Questions 8-12
Ben is brought in for his 6-month visit. He is visually alert, but his eyes tend to drift toward his nose when he is tired. He is hard to hold according to his mother and has always been a “spitty baby.” The problem is worse when she tries to feed him solid food because it comes out before she can get him to swallow. He gags easily and had a full startle reaction when you dropped his chart accidentally. He has been growing poorly, presently at just below the 10th percentile for weight, although he started out at the 50th percentile. His head circumference is at the second percentile, and his length at the 10th. On pull-to-sit maneuver, his head lags behind his shoulders. He does not sit independently and tends to fall backward when he tries. When you support him in a sitting position, he holds his arms up beside his shoulders and holds his head upright but stiff. He bears weight in a supported standing position but does so on the tips of his toes, and he is arched from the tip of his head to his toes. In a prone position, he screams and cannot seem to get his arms forward to support himself. He is fisted most of the time and will not grasp your toys. His cry is different when he is hurt than when he is just fussy, but he cries a lot, and the cry is high-pitched and shrill. He does not seem to localize sounds, but his mother is sure that he can hear because he calms down when she sings to him, even if she is not holding him. His physical examination findings are abnormal for brisk deep tendon reflexes in all four extremities and sustained ankle clonus.
8. Historical features that might assist you include:
a) Prenatal history of drug or alcohol exposure
b) Perinatal history of prematurity, abruption, or asphyxia
c) Family history of progressive loss of strength and mobility
d) Multiple miscarriages and family members with congenital anomalies
View Answer
Answer
The answer is b. Ben presents with significant motor delay at the young age of 6 months, although we would suspect that his 2- and 4-month examination findings were also abnormal. Children can certainly show some delay in controlling conjugate gaze during the early months of life, but by 6 months, inwardly turning eyes are a cause for concern until proved otherwise. Some of the same insults that produce esotropia also affect the acquisition of motor control. Classically, the child with upper motor neuron damage is floppy early in life, and the resting tone then increases with age. By 6 months, delayed loss of primitive reflexes (the persistent startle when you dropped your chart), delayed acquisition of voluntary control of the head and trunk, delayed acquisition of protective reactions that allow independent sitting at least with propping, and an increase in deep tendon reflexes strongly suggest central nervous system injury. This concern is further supported by the child’s high-pitched cry, fussiness, gastroesophageal reflux, hyperactive gag when solid foods are introduced, and relative microcephaly. The history most suggestive of this presentation is b, a significantly asphyxiated or premature infant with massive periventricular leukomalacia or significant intraventricular hemorrhage. A prenatal history of drug abuse might support the presentation if poor prenatal care and nutrition, poor blood pressure control, and bleeding early on were present to a degree that interfered with intrauterine growth. Family disorders associated with progressive loss of strength are more likely to be muscular dystrophies, which do not present with spasticity but rather with weakness; demyelinating diseases, such as multiple sclerosis, which are reportable in the newborn to 6-month age range; or heritable neuropathies, which present at later ages. Fetal wastage and anomalies are more likely in children who are dysmorphic or excessively small at birth; Ben had been growing normally. Cerebral palsy is frequently associated with ocular muscle problems, bony deformities such as a dislocated hip or scoliosis, seizure disorders, and sensory disturbances such as myopia and hearing impairment.
9. Additional assessments might appropriately include:
a) Multidisciplinary evaluation by a physical therapist and an occupational therapist and oral-motor assessment by a speech therapist or an occupational therapist
b) Ophthalmology
c) Neurology
d) Audiology
e) Orthopaedics
f) All of the above
View Answer
Answer
The answer is f. All the additional assessments suggested in Question 9 might be appropriate.
10. The Denver Developmental Screening Examination is an appropriate tool to continuously assess this child’s cognitive, language, and self-help functioning.
a) True
b) False
View Answer
Answer
The answer is b. Ben is clearly experiencing motor delay, and at this point we strongly suspect an underlying diagnosis that categorically qualifies him for early intervention services; therefore, the Denver Developmental Screening Examination is not an appropriate screening tool for him. The norms were determined in children with normal motor ability to demonstrate their selfhelp and play behaviors and would not accurately reflect Ben’s potential ability to understand and comprehend. He should undergo specialized testing of his cognitive abilities with motor-free assessments, if possible.
11. This child can be referred to early intervention service coordination in his community even before a full diagnosis has been made.
a) True
b) False
12. This clinical picture is most consistent with:
a) Trisomy 15
b) MR
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