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Early identification of infants at risk for

developmental disabilities

Laurel M. Bear, MDa,b,*aMedical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI 53226, USA

b

Child Development Center, Childrens Hospital of Wisconsin, PO Box 1997 MS#744, Milwaukee,WI 53201, USA

Early recognition of infants at risk for development disability is important.

The early identification of a developmental delay is a challenge to all physicians,

however. It is difficult to differentiate between infants who are lagging behind in

skill acquisition but will achieve the usual developmental milestones and infants

who are truly deviating from the expected pattern. Identifying children in the first

year of life provides the opportunity for early referral for interventional services

and diagnosis. The physician can supply factual information and devise a realisticapproach for the care of those conditions and then counsel parents regarding

possible outcomes for their child. Children with chronic medical conditions and

children growing up with precarious social and environmental circumstances are

at risk for developmental delay. Early diagnosis of at-risk infants is possible once

a thorough history is obtained, a complete physical examination is performed,

and an assessment of the functional developmental level is secured.

Perinatal history

A thorough perinatal history offers vital information that, when analyzed,

provides important clues to possible risk factors that may have a later impact on a

childs functioning. Not only should gestational age, birth weight, and Apgar

scores be obtained but also pertinent pregnancy information should be reviewed.

Most importantly, was there prenatal care before the birth of the child? Early

support of a pregnancy leads to earlier maternal and fetal intervention and

improved fetal outcome. By definition, a high-risk pregnancy places the mother

or infant at higher risk for medical complications. Some maternal circumstances

0031-3955/04/$ see front matterD 2004 Elsevier Inc. All rights reserved.

doi:10.1016/j.pcl.2004.01.015

* Child Development Center, Childrens Hospital of Wisconsin, PO Box 1997 MS#744, Mil-

waukee, WI 53201.

E-mail address: [email protected]

Pediatr Clin N Am 51 (2004) 685701


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have an impact on the medical and developmental functioning of the infant,

including inadequate or excessive maternal weight gain, diabetes, lupus, seizure

disorder, and other chronic diseases. Increasing maternal age has a direct cor-relation regarding the increased prevalence of chromosomal anomalies. Some

of the entities that have a direct influence on infant outcome include placental

insufficiency, infection, preterm birth, multiple gestation, fetal demise in a mul-

tiple pregnancy, illegal drug use, prescription medication use, and alcohol and

tobacco use. Cocaine-exposed infants have been found to have significant

cognitive deficits and a doubling of the rate of developmental delay during the

first 2 years of life, although contradictory studies have suggested that there was

no direct effect on cognitive outcome but indirect effects were mediated through

the home environment [13].Some factors affect delivery and have an impact on a neonates well-being.

The length of laboreither prolonged or precipitousis important to note.

Friedman ***characterized normal labor by studying normal nulliparous and

multiparous women during routine labor. From these studies he developed the

concept of a dilatation curve. Labor was divided into a latent phase, active phase,

and second stage (Fig. 1) [4]. There are marked individual variations in the length

of labor; however, the average length of the first stage of labor can range from

7 hours in a nulliparous woman to 4 hours in a parous woman. The second or

active stage usually lasts from 50 minutes in a nulliparous woman to 20 minutesin a multiparous woman. A short or long second stage of labor can have an affect

on fetal well-being. Cesarean section itself does not place a baby at high risk;

Fig. 1. Composite of the average dilitation curve for nulliparous labor.

L.M. Bear / Pediatr Clin N Am 51 (2004) 685701686


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however, the reason for the cesarean section may. Were fetal heart rate problems,

infection, or a failure to progress the reason for the surgical delivery? An op-

erative delivery may lead to transient tachypnea of the newborn. This entitywas first described in 1966 by Avery ***and was believed to be caused by the

delayed absorption of fetal lung fluid [5]. Passage of heavy, thick meconium

before or at the time of delivery may indicate fetal distress, and it places the in-

fant at higher risk for the development of meconium aspiration syndrome. Me-

conium is found in 8% to 20% of deliveries and is usually light (meconium

staining). Passage of meconium is a poor predictor of later neurologic problems.

In the large National Perinatal Collaborative study, less than 0.5% of infants with

a birth weight more than 2500 g and meconium staining had neurologic problems

[6]. Fetal hypoxia, as measured directly by scalp pH or indirectly by fetal heartrate tracing, sets the stage for possible neonatal complications but is not a specific

predictor of later problems.

Neonatal considerations

The Apgar score, devised by the anesthesiologist Dr. Virginia Apgar in

1952, was one of the first objective, clinical measures used to assess a newborns

well-being. It consists of five observable measures: heart rate, respiratory effort,color, muscle tone, and response to noxious stimuli. The Apgar score was de-

veloped as a tool to assess the condition of an infant at birth. A low Apgar score

is not synonymous with asphyxia. Asphyxia implies fetal hypercarbia and hy-

poxemia that can lead to metabolic acidosis. Medications, congenital anomalies,

gestational age, and acute events all may have an impact on the Apgar score. The

1-minute Apgar score indicates if ongoing intervention is needed. A low Apgar

score correlates poorly with later development of cerebral palsy. Nelson and

Ellenberg ****found that 55% of children with cerebral palsy had Apgar scores

of 7 to 10 at 1 minute, and 73% of children with cerebral palsy scored 7 to 10 at5 minutes. Of the children who had Apgar scores of 0 to 3 at 10 minutes or later

and survived, 80% were free of major handicap at early school age [710].

It is important to assess accurately a newborn infant by determining its

gestational age and recording the growth parameters. The newborns growth

parameters reflect the in utero environment. Newborns are classified according to

their birth weight as small for gestational age (less than the tenth percentile),

average, or large for gestational age (more than the ninetieth percentile). The

National Center for Health Statistics revised the individual growth charts in 2000.

These charts incorporated newer data from five national health examinationsurveys. There are some minor differences in the percentile lines. Most of the

differences are found in the charts for infants and there are differences at the outer

percentiles (ie, smallest and largest children) [11].

Maternal diabetes and maternal obesity are well-known predisposing factors in

the development of large-for-gestational-age infants. Other factors that are

associated with macrosomic infants include (1) large size of parents, particularly

L.M. Bear / Pediatr Clin N Am 51 (2004) 685701 687


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obesity in the mother, (2) multiparity, (3) maternal age, (4) prolonged gestation,

(5) male fetus, (6) previous large infant, and (7) race and ethnicity [1215].

The incidence of congenital anomalies and intellectual and developmental de-lay is statistically more common in preterm and term infants of high birth weight.

Obstetric complications continue to occur in this population, including birth

trauma, intrauterine growth restriction, and intrapartum asphyxia. The infant of a

mother with diabetes displays its own set of medical complications, including

neonatal respiratory distress, hypoglycemia, hypocalcemia, hyperviscosity (poly-

cythemia) syndrome, cardiomyopathy, and congenital anomalies [1619].

Small-for-gestational-age infants are at risk for increased adverse affects in

the newborn period. These infants have a birth weight less than the tenth

percentile, taking into consideration the population-specific birth weight andthe gestational age. The proportionate reduction in head and body size has

been termed symmetrical growth restriction. Asymmetrical growth restriction

refers to the relative sparing of head size in proportion to the smaller body size.

By definition, intrauterine growth-restricted infants have had a rate of fetal

growth that was less than normal, which produces an infant who is small for

gestational age. Problems in this group of infants include perinatal depression, hy-

pothermia, hypoglycemia, polycythemia-hyperviscosity syndrome, and immune

dysfunction. These children remain at risk for compromised health and devel-

opment throughout their childhood. This is particularly evident as far as somaticgrowth is concerned. Catch-up growth is usually evident by 4 months of age if it

is going to occur. From 4 months onward, the velocity of growth remains

constant and these children remain small [20]. More troublesome are the long-

term studies that suggest that these infants can demonstrate lower cognitive

performance, suboptimal school achievement, and behavioral or mental health

problems [2123].

A spectrum of hypoxic-ischemic encephalopathy exists. It is usually classified

as mild, moderate, or severe [24]. Infants who manifest mild degrees (ie, normal

neurologic examination at the time of discharge) rarely exhibit neurodevelop-mental sequelae. Infants with a more moderate degree of level of encephalopathy

have a 20% to 40% chance of an abnormal outcome. One hundred percent of

babies with severe encephalopathy demonstrate an abnormal outcome [2529].

The criteria for hypoxic-ischemic encephalopathy are as follows: (1) an Apgar

score of 0 to 3 for more than 5 minutes, (2) an umbilical artery metabolic acidosis

or mixed respiratory-metabolic academia with pH less than 7, (3) newborn

neurologic sequelae (eg, seizures, hypotonia, or coma), and (4) multiorgan sys-

tem dysfunction.

The initial concerns surrounding premature birth relate to survival. Chancesfor survival increase from 22 through 33 weeks gestation. At each gestational

age, a lower birth weight carries a higher risk of mortality [30]. Premature infants

may experience a wide range of complications, including chronic lung dis-

ease, periventricular leukomalacia, sepsis, retinopathy of prematurity, intraven-

tricular hemorrhage, necrotizing enterocolitis, hearing deficits, and birth defects

[31]. These neonatal morbidities have an increasing adverse effect on neuro-



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logic, developmental, neurosensory, and functional outcomes with decreasing

birth weight.

Vohr et al reported on the neurodevelopmental, neurosensory, and functionaloutcomes of 1151 extremely low birth weight (4011000 g) survivors cared for

in the 12 participating centers of the National Institute of Child Health and Hu-

man Development Neonatal Research Network. They identified medical, social,

and environmental factors associated with these outcomes. Twenty-five percent

of the 1480 surviving infants evaluated at 18 months of age had an abnormal

neurologic examination, 37% had a Bayley II mental developmental index of less

than 70, and 29% had a psychomotor developmental index of less than 70. They

Box 1. Risk category for developmental delay by medical diagnosis

High risk

1. Birth weight less than 1250 g

2. 30 weeks gestation or less

3. Intraventricular hemorrhage/periventricular leukomalacia

4. Severe perinatal asphyxia5. Severe neurologic problems

6. Bronchopulmonary dysplasia that requires home oxygen

7. Complex congenital/cyanotic heart disease

8. Abnormal neurologic examination at discharge

9. Significant feeding problems/requirement of gavage feeding

10. Intracranial pathology: congenital or acquired

11. Extracorporeal membrane oxygenation

12. Diaphragmatic hernia

13. Persistent pulmonary hypertension of the newborn/requiredinhaled nitric oxide/oscillatory ventilator

14. Significant circulatory failure

15. Congenital viral infection (HIV, TORCH)

16. Prolonged or persistent hypoglycemia

17. Multiple/major congenital anomalies and genetic disorders

Moderate risk

1. Birth weight between 1250 and 1500 g

2. Prolonged ventilation and high-frequency ventilation

3. Surgical: cloacal anomalies/gastroschisis/omphalocele

4. Tracheostomy

5. Metabolic disorders



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found that neurologic, developmental, neurosensory, and functional morbidities

increased with decreasing birth weight. The factors significantly associated with

increased neurodevelopmental morbidity included chronic lung disease, grades3 to 4 intraventricular hemorrhage and periventricular leukomalacia, steroids for

chronic lung disease, necrotizing enterocolitis, and male gender. The factors

significantly associated with decreased morbidity included increased birth

weight, female gender, higher maternal education, and white race [32]. Compro-

mised neurologic status affects cognitive function and later school performance.

Emerging studies of this extremely low birth weight population reveal sig-

nificantly poorer school performance, with particular difficulty in the area of

mathematics. Even children without neurologic impairments have scores that are

significantly lower on cognitive and achievement measures. Social and environ-mental factors, including maternal level of education, contribute to outcome

(Box 1) [3337].

Developmental history

A childs development should follow a predictable pattern. Reviewing a

childs history of acquisition of developmental milestones offers a way to detect

deviations from normal. A developmental history is usually organized bydomains of development. Areas to be included are gross motor skills, fine

motor skills, social interaction, language, and self-help. These domains are not

mutually exclusive, and a childs behaviors can be thought of as belonging

to more than one domain. For example, a 9-month-old child requires social

maturity, motor planning and performance, and symbolic language skills to

wave bye-bye. Anticipatory guidance guidelines offer information on the

timing and standard deviation for normal development. The Bright Futures

publication identifies several desirable health, social, and developmental out-

comes as a result of the implementation of the health supervision. By eliciting adevelopmental history at each visit, the pediatrician has an opportunity to create

a supportive relationship with the parents and educate the family on normal

development of their child.

Social history

The environment in which the family resides has an impact on the develop-

ment of the child. Parental education, financial resources, marriage status/livingarrangements, and family support structures all have an impact on the outcome

of the family. Poverty is one of the primary predictors of poor health outcomes

for children. The child-rearing practices of persons who live in poverty are

more likely to lack some of the elements that are fundamental for optimal

outcomes. Fundamental needs that may not be met include literacy, stimulation,

parental time, books, appropriate educational toys, guidance, role models, and



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high expectations [3842]. Caldwell and Bradley developed a psychometrically

valid inventory to describe the quality of the home environment: the HOME

scale (Home Observation for Measurement of the Environment). This is a45-item (infants and toddlers) or 55-item (preschool children) observational

assessment [43].

Family history

A concise, systematic approach to obtaining a complete family history can

provide useful information. The history should encompass three generations:

(1) patient and siblings, (2) parents and their siblings, and (3) grandparents and

their siblings. The usual record consists of medical conditions. If there have been

deaths of family members, the age at death and the cause of death should be

noted. Specific questions should be asked regarding illnesses that have affected

more than one family member, congenital anomalies, and genetic disorders

within the nuclear and extended family unit. The interview also should focus

on the educational achievement of the adults, school difficulties of family

members, and speech, language, learning, and developmental problems.

Physical examination

When performing the physical examination [44], knowledge of normal

anatomy and its variants helps the physician recognize significant findings.

The basic techniques of physical examination should be used and fine tuned to

recognize any subtle physical clues that might be overlooked. Fetal embryogene-

sis and development is a highly intricate process, with parallel and sequential

growth taking place. Any alterations at a specific time may affect more than one

organ system. A well- known example is the association of ear deformities and

kidney anomalies. The ear and kidney have active growth at the same time, sointrauterine influences are reflected on both organs. Parents are highly sensitive

observers of their children, and their concerns should be addressed openly.

Quiet observation of the child can lead to a wealth of information. Look

for subtle differences between opposing sides of the body, face, or extremities.

Unusual or abnormal postures and positioning can lead you to the site of pa-

thology. Torticollis can be suspected by the abnormal tilt or rotation of a babys

head at rest. Passive and active range of motion can confirm this diagnosis.

A complete head-to-toe examination is important to identify abnormalities that

may have an impact on a childs development. The examination may begin withaccurate measurement of growth parameters. Weight, height, and head circum-

ference should be documented routinely and plotted on standard growth forms.

When a child is born prematurely, the adjusted age may be used for plotting these

growth parameters. It is often practical to plot growth parameters at the

chronologic age, however, with the understanding that these parameters are at

a lower percentile than if the child were plotted at the adjusted age. To calculate



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the adjusted age, the amount of prematurity is subtracted from the chronologic

age. The adjusted age should be used when evaluating the developmental level

of infants and young children.During the course of the physical examination deviations should be noted.

Identify, as clearly as possible, any abnormal features. It is often helpful to know

if the baby resembles a family member. Skull shape can vary somewhat among

racial groups, and small degrees of asymmetry can be seen in normal individuals.

More marked asymmetry is often caused by intrauterine deformation or possibly

craniosynostosis. The head circumference provides a rough estimate of brain size.

Conditions such as microcephaly and macrocephaly may indicate an underlying

brain anomaly.

Placement of a childs ears is best judged in relation to other landmarks on thehead and face. Low-set ears and posterior rotation of the ear are common

abnormalities. The junction of the upper pinna to the scalp should be at the

same level as the outer canthus of the eye. The upper limit of the pinna should be

at the level of the eyebrow, whereas the lower level of the pinna should be at the

level of the alae nasi. Preauricular pits or tags may occur in normal individuals,

and variations in the form of a helix are also relatively common.

In general, the face is divided into thirds, with the distance between the inner

canthi of both eyes comparable to the distance between the inner and outer canthi

of each eye. The slant of the palpebral fissure varies, and there are ethnicvariations of eye shape and lid placement. Clefts within the eyelid are considered

abnormal. A white pupil or abnormal placement of the light reflex is always

abnormal. Marked strabismus may be either divergent or convergent in one or

both eyes. If there is a constant deviation after 5 months of age, it requires

examination by a specialist. Pendular nystagmus is a sensorial deficit that always

requires referral. Bilateral lid ptosis may indicate a neuromuscular abnormality.

In the center of the face are only two structures: the nose and philtrum. A

change in length of one usually necessitates a compensatory change in the other.

For example, a short nose often results in a long philtrum. The columella is thetissue that connects the nose tip to the face. This structure, along with the nose tip

and alae nasi, provides an overall picture of the end of the nose. Nasal length and

nasal width have considerable familial and ethnic variation, which makes it

difficult to recognize abnormalities in this region.

The perioral region is evaluated by inspection. The size, symmetry, and shape

of the mouth, lips, and nasal labial folds should be reviewed along with the

contour of the chin and cheeks. Often abnormalities of nervous innervation to the

lower face may be demonstrated when the child cries or laughs. The intraoral

cavity should be examined for patency of palate and its configuration. A cleftpalate is the most common major mouth malformation. There are two classes of

palatal clefts: those that occur in isolation and those that occur in association with

a cleft lip.

When examining the neck it is important to demonstrate head position at rest.

Abnormal hypertonia of neck extensors is demonstrated by an exaggeration of

the space between the posterior neck and table surface when the infant is supine.



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The relative width and length should be assessed. Neck mobility, including ex-

tension, flexion, and rotation, is evaluated by passive and active range of motion.

A short neck usually is the result of an absence or malformation of one or morecervical vertebrae, and neck mobility is then limited, particularly during flexion.

Rotational activity is more often limited with torticollis.

The shoulders superiorly and lower rib inferiorly delineate the chest. General

shape of the thorax and nipple placement and symmetry should be noted. One of

the minor variants that is often seen is pectus excavatum (funnel chest). This

condition may be of cosmetic concern but usually causes no physiologic

problems. Shield chest is an unusually broad and often short upper thorax and

is frequently associated with wide-spaced nipples.

The back is first inspected for general configuration and symmetry. Specialattention should be paid to the lower back, where sacral dimples or other midline

abnormalities may be found. If an abnormality is found, it must be differentiated

from a minor or major degree of spinal dysraphism. The chance of an underlying

defect increases if found above the natal cleft. It may be necessary to separate the

buttocks to view the entire spine.

Examination of the heart is accomplished through inspection, palpation, and

auscultation. Congenital heart defects can occur in isolation or in association with

other anomalies. Children with these defects may experience inadequate weight

gain secondary to poor caloric intake and an increase in metabolic rate. The childcan experience a concurrent decrease in strength and stamina, with a delay in the

acquisition of motor milestones. Early intervention encourages normal develop-

mental patterns until the heart defect can be corrected [4547].

The abdominal examination begins with a brief inspection, and one should

pay close attention to symmetry, muscle tone, and the presence of major or minor

defects in the abdominal wall. Children who have had surgical procedures that

required an abdominal approach experience disruption of their abdominal

musculature and may display a decrease in abdominal flexor muscle strength.

Special attention should be paid to the umbilicus. Umbilical hernias can rangewidely in size, and often the abdominal wall defect is considerably smaller than

the hernial sac. A palpable defect smaller than 1 cm in diameter could be

considered a normal variant and should close spontaneously within 2 to 3 years.

The genitourinary system is a complex system. The complexity is second only

to that of the face and brain. Disruption in this area can be accompanied by

changes within the pelvis. Bladder extrophy is one such condition in which the

hips are often widely abducted with the pelvis tilted. Sitting skills may be

delayed. It is important to partner with a surgeon to optimize motor skills without

compromising surgical correction. Inspection of the female external genitalia isusually the only examination that is required. Internal inspection should be

reserved for patients with a strong suspicion of anomalies. Examination of male

genitalia involves inspection and palpation. Location of both testes should be

documented. When either the testis or penis seems unusually small or large,

referral to a pediatric urologist is warranted for further evaluation. When

ambiguous genitalia are discovered, it is important to note the size and textures



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of the various structures. The location of the urethral opening also should be

documented. Investigation should be undertaken to determine the presence or

absence of the vaginal introitus. The possible labia majora should be palpated formasses that may represent a testis or ova testis.

The skin is the largest organ in the body; many cutaneous abnormalities have

been described [48]. Many of these skin anomalies are localized and benign,

whereas others may provide valuable clues to underlying medical or neurologic

conditions. Areas of hyper- or hypopigmentation, changes in elasticity, and

discrete lesions may lead to a significant medical diagnosis. The texture of the

hair, placement of the hairline, presence of hair in the pubic or axillary area, and

unusual patches of hair elsewhere on the body should be documented. Fine hair

covering the bodylanugois often found in many infants. Nail color, size,shape, and thickness should be elucidated by physical inspection. Fingernails

should be inspected from the dorsal surface and end-on.

Examination of the extremities is best performed with the child in a recumbent

or sitting position. Limb length and bulk are judged. A difference of more than

1 cm in leg length eventually produces a pelvic tilt [49]. Palpation of the limbs

determines size and consistency of the muscles. When any asymmetry is

suspected, referral for accurate measurement and evaluation is warranted.

Examination of the large joints of the body should accompany the limb

examination and should include rotation, flexion, extension, and active rangeof motion. During inspection of the hands and feet, syndactyly, joint laxity, and

presence or absence of a plantar arch should be looked for. Increased flexibility

across various joints is often seen in various ethnic groups and on a familial basis.

Excessive hyperextensibility, particularly of small joints, along with creased

elasticity of the skin is seen in Ehlers-Danlos syndrome.

The Ortolani maneuver should be performed at routine intervals during an

infants life. A marked asymmetry of hip abduction warrants referral for further

evaluation. Ultrasonic evaluation is best performed between 2 and 6 weeks of

age but can be used up to 4 months of age. Once an infant is 4 months of age,the plain radiograph becomes the imaging modality of choice because the femoral

head is ossified [50].

Subtle changes in a neurologic examination may have enormous significance

in eventual development of that child. A complete neurologic examination should

be performed at regular intervals. Evaluation of the nervous system provides

information regarding motor function and sensory integrity. Neurologic findings

can vary depending on the patients state of alertness and desire to interact with

the environment. For example, immediately after a feeding, the neonatal reflexes

tend to be less distinct and muscle tone may seem diminished compared withfindings just before a feeding.

The cranial nerves are used to elicit some basic primitive responses. Evalu-

ation should include the ability for the child to fix and follow and the ability to

suck, swallow, and hear. Some of the primitive reflexes that should dissipate over

the first 8 months of life include the palmar grasp, rooting/sucking, moro, and

atonic neck reflex. Hands may remain closed until 2 months of age, and thumb



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adduction should be checked for. The atonic neck reflex can be present until

3 months of age and should dissipate between 3 and 6 months of age. With the

resolution of primitive reflexes, various postural reflexes are then elucidated.These reflexes include neck righting, parachute, and the Landau maneuver.

The peripheral deep tendon reflexes are synonymous with the stretch reflex.

They describe a neuronal arc that consists of a muscle contraction with the

impulse traveling to the dorsal route ganglia. The efferent side consists of the

motor neuron along with its terminal structures that innervate the muscle. Central

influences can modify the response of the motor neuron. The elicitation of a

motor response means that the arc is intact and conducting impulses. The absence

of response does not necessarily indicate an abnormal state because neural

influences may suppress the reflex. Reflexes that should be checked includethe biceps, triceps, patellar, and quadriceps reflexes, which are graded by the

degree of response elicited. When no response is forthcoming, the grade is

zero. If a reflex response is elicited by reinforcement, then the arc is intact,

although possibly inhibited. A 1+ response is in the low normal range; 2+ is

considered a normal or average response; 3+ is a brisk response and may indicate

disease; a 4+ response is very brisk and is associated with clonus and shows

evidence of disease.

The Babinski sign is considered by some researchers to be the most important

sign in neurology. When present after the age of 12 to 16 months, it indicatesdysfunction of the corticospinal motor system. The Babinski sign is referred to as

being present or absent, not positive or negative. An absent Babinski reveals

intact neurologic function and consists of flexion of the forefoot with adduction

of the toes. The presence of a Babinski sign is an abnormal response and is seen

with dorsiflexion of the great toe and fanning of the others. This is often

accompanied by withdrawal at the knee and hip.

It is crucial to have a solid understanding of normal developmental landmarks

to assess the state of nervous system function at the various ages in infancy.

Motor skills are generally acquired in relatively constant sequence during the first2 years of life. This sequential attainment of milestones cements the evidence of

nervous system integrity. Factors unrelated to brain disease may hinder the

orderly appearance of developmental milestones. Maternal or sensory deprivation

may result in deficits in the motor and speech realms. Prematurity, serious

generalized disease, and recurrent infections can have an impact and retard

development during the first year of life. This may occur with an intact nervous

system. As with all of pediatrics, the neurologic examination in an infant must be

performed in a less systemized fashion than that of an adult. Quiet observation

can yield a wealth of information, such as information in the areas of symmetryof limb movement, organization of ocular movements, and degree of interest in

and an awareness of the babys surroundings. The ability to suck, cry appropri-

ately, and move passively can be obtained [51].

The Moro reflex should be a part of the examination of any infant. It is

normally present from birth until 3 to 4 months of age. During this 4-month

period, the intensity of the response should diminish. If the Moro reflex persists



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in a full-term infant past 4 months of age, neurologic disease should be suspected.

If it is present at 6 months of age, it almost always indicates a significant disorder.

Absence of the response in a neonate does not necessarily indicate brain diseaseand is often difficult to elicit in premature infants. An asymmetric response

may suggest hemiparesis, injury to the brachial plexus, or a fracture of the

clavicle or humerus.

Rooting and sucking reflexes are more active if the child has not been recently

fed. Persistence of these reflexes beyond 4 months of age suggests bilateral brain

dysfunction. Chewing or mouthing movements may indicate an underlying

neurologic disorder.

The infant should blink in response to a bright light, and the pupils should

constrict simultaneously. Conjugate movements develop rapidly after birth; how-ever, infants are often unable to follow objects during the first few weeks of

life. By 3 months of age an infant should be able to fix and follow a red ring.

The newborn infants hands are held in a fisted position during much of the

awake period. The hands tend to open and become more relaxed after 4 weeks

of age. Persistence of a specific posture in one hand after 2 months of age may

indicate an emerging spastic hemiparesis. The grasp reflex is symmetrical and

present at birth and should persist for 2 to 3 months.

The asymmetric tonic neck reflex, or fencing pose, is a normal response

between 1 and 5 months of age. The infant should be able to overcome thisresponse (nonobligate). Persistence of this reflex or response that persists beyond

6 months of age or is an obligate response is considered abnormal.

Stepping movements are obtained by supporting a child in an upright position

with the bottom of his or her feet in firm contact with a table. This response lasts

for 3 to 4 weeks and provides evidence of neurologic integrity. While holding the

child in this position, an assessment of muscle tone also can be made. Scissoring

of the legs is always considered abnormal.

By 6 months of age, a normal infant no longer should show evidence of these

primitive reflexes. Persistence of these reflexes without a good explanationrequires further investigation.

Performing the Amiel-Tison angles completes neurologic examination [52].

These measurements of passive tone are similar to what is obtained in the

newborn examination. The angles that are tested include the scarf sign, popliteal

angle, adductor angle, heel-to-ear maneuver, and foot dorsiflexion. There are

age-appropriate standards for measuring the various angles. As a child grows

from infancy to young childhood, he or she becomes more flexible with an

increase in measured angles. Asymmetry between the two sides can be elicited.

An assessment of muscle tone can be obtained while performing these maneu-vers (Fig. 1).

The scarf sign is performed with the child in the supine position. In a quiet

alert state, with the childs back flat against a hard surface, the wrist is grasped

and swept across the chest until resistance is felt. Three possible positions are

obtained: (1) the elbow does not reach the midline, (2) the elbow passes the

midline, (3) the arm encircles the neck.



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The popliteal angle is obtained with the child in the supine position with the

buttocks on the table surface. With the hands placed over the childs knees, the

thighs are flexed laterally and then the lower leg is extended. The angle ismeasured based on the measurement between the thigh and calf. Both legs are

measured simultaneously. A difference between the two sides of 10 to 20

indicates significant asymmetry.

The adductor angle is obtained with the infant in the supine position, legs ex-

tended and gently abducting the lower extremities. The angle is measured by

drawing an imaginary line along both inner thighs with the vertex at the symphy-

sis pubis. If this angle is bisected, the two angles should be relatively equal.

The heel-to-ear maneuver requires a measurement with both legs extended and

then moved toward the head. The angle is measured from the flat surface alongthe back of the leg to the infants heel. The pelvis should not lift off the table. If

it is not possible to extend the lower limbs because the hips are in a fixed

hyperflexed posture, then this is considered an abnormal sign.

Foot dorsiflexion is measured with the leg extended and the foot flexed onto

the shin. Pressure should be applied to the sole of the foot. The dorsum of the

foot and the anterior aspect of the leg form the angle. The usual range of 60 to

70 persists throughout infancy. A slow and rapid angle should be measured.

A difference of more than 10 indicates an abnormal exaggerated stretch re-

flex (Fig. 2).

Fig. 2. Normal pattern of passive tone within first year of life. (Adapted from Amiel-Tison C. A method

for neurological evaluation within the first year of lige. Curr Probl Pediatr 1976;7:1; with permission.)



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Developmental screening tools

Numerous developmental tools can be used for screening infants and young

children. It is important that the screening tool be easy to administer and is

accurate between evaluations. The aim of a developmental screening tool is to

identify children who need a more comprehensive evaluation. Some of the more

well-known tools include the Denver Developmental Assessment, the Capute

Scales (Cat/Clams), and the Bayley Infant Neurodevelopmental Screener. If a

child was born more than 2 weeks before the expected date of delivery, the

adjusted age should be used. A developmental quotient can be calculated for each

domain. For example, at a chronologic age of 13 months a child is not able to

perform a specific age-appropriate developmental task but is able accomplish the

tasks at the 10-month level. The child is functioning at 77% of the expected level.

A developmental quotient of 70% indicates a significant delay.

The Denver Developmental Screening Test [53] was designed as a screening

tool for apparently normal children from birth to 6 years of age. It has 125 well-

standardized, easily administered test items in a one-page format. This test

encompasses four general areas of development: fine motor, gross motor,

personal and social skills, and language. The items are arranged in these four

sections. When two or more delays are noted (ie, the child refuses an item or it

falls completely to the left of the age line), the child is considered to have failed

the screening and more definitive developmental assessment must be undertaken.

The Denver-II screening test is known to have modest sensitivity and specificity

depending on the interpretation of questionable results.

The Capute Scales [54] are an assessment tool that examines visual motor and

problem-solving and language abilities. CLAMS stands for clinical linguistic and

auditory milestones. It was developed, standardized, and validated for language

development from birth to 36 months of age. The CAT (clinical adaptive test)

examines problem-solving items from birth to 36 months. Items are recorded as

yes for pass and no for failed. If two consecutive months are scored as

yes, a basal age is determined. Items at the next higher level are then

administered until two consecutive levels of no responses are obtained.

The Bayley Infant Neurodevelopmental Screener [55] is a tool to assess

children in blocks of age from birth until 24 months of age. It is used primarily as

a screening instrument; however, it also can be used as a surveillance instrument.

Each group of items assesses fine motor, gross motor, language, and social

interactive and play skills. The child receives a point if the item is accomplished

and a zero if it is not. The child is placed in one of three categories: low,

moderate, or high risk for developmental delay. This screening tool is easy to

administer and takes approximately 10 minutes to administer, and the adminis-

tration format is easy to follow.

Recently, screening tools have been developed that respond to parental

concerns. By using these tools, parents become active participants in the care

of their child. These concerns have proven to be highly predictive of true

problems [56,57]. These parental report questionnaires have good psychometric



15/17

properties and have been standardized on diverse populations and provide

accurate information about development. They have the added benefit of

requiring much less direct time from the primary care provider. Some of theinstruments include the Parents Evaluation of Developmental Status [58], Ages

and Stages Questionnaires [59], and the Child Development Inventories [60].

Summary

Early identification of infants at risk for developmental delay is of the utmost

importance to initiate appropriate intervention. Although early detection can be a

challenge, the primary care practitioner is in the ideal position to recognize andrefer these children. Early recognition requires an in-depth knowledge of the

childs history, general physical examination, and developmental level and an

understanding of the expected developmental precursors of a skill. Referral to

appropriate interventional resources leads to a formalized developmental and

neurologic evaluation. If necessary, the development of an interdisciplinary

comprehensive plan of remediation can occur and a definitive diagnosis can be

made. If no significant problem is found, a decision to provide expectant

observation is warranted.

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