Head

Examination of the head must be done systematically, looking for asymmetry, indentations, and protuberances. Evaluation of the fontanels and cranial sutures should be performed with gentle palpation. The dimensions of the anterior fontanel should be carefully recorded [Pedroso et al., 2008]. The examiner should determine by observation and palpation the presence of frontal bossing, bulging fontanel, sutural synostosis or diastasis (separation), and unusual head shapes such as trigonocephaly, marked dolichocephaly or brachycephaly. Positional plagiocephaly has become increasingly common with the current “back to sleep” approach, and is the most common cause of abnormal head shape; it can often be distinguished from isolated craniostenosis by prominence of the contralateral forehead, which leads to a rhomboidal appearance [Bialocerkowski et al., 2008]. Unusual masses under the scalp and gross asymmetries of the skull should be sought.

The occipitofrontal circumference should always be measured. If the child becomes agitated, the measurement could be deferred until later in the examination (stage 3). The largest measured circumference incorporating nasion and inion should be recorded and plotted on a standardized graph of normative data, preferably incorporating serial measurements. The size of the anterior fontanel, which is typically closed by 12 months of age, should be recorded. The anterior fontanel pulsates in unison with the heartbeat, and becomes fuller or bulging when the child cries, and this must be distinguished from disease states in which there is increased intracranial pressure. The tenseness of the anterior fontanel should be evaluated when the child is sitting comfortably in an upright position. The posterior fontanel usually admits only a finger at time of birth and is usually closed by 2 months of age. Other fontanels are usually difficult to palpate, except in pathologic states. Occasionally, accessory fontanels may be found along the sutures, particularly the sagittal suture; these are usually benign variants. The head should be auscultated for the presence of unusual intracranial bruits. Intracranial bruits occur commonly in childhood, and cautious interpretation is advised; asymmetric bruits and those that can be suppressed by carotid artery suppression are frequently pathologic. Vascular abnormalities, such as vein of Galen malformations, can produce extremely loud bruits, and are often heralded by prominent scalp veins. The vein of Galen malformation is typically associated with increasing occipitofrontal circumference, high-output cardiac failure, and infant distress while in the supine position.

Cranial Nerves

Most of the examination of cranial nerve function of the infant and toddler can be completed by observation with minimal invasive procedures. More details concerning examination of each cranial nerve can be found in Chapter 2. Toys or colorful objects can facilitate the assessment of extraocular movements in young children. Visual fixation and pursuit will bring out nystagmus and strabismus. If the child appears uninterested in bright objects, the possibility of a visual defect or an underlying intellectual defect must be considered. Rolling eye movements and dysconjugate gaze suggest gross visual impairment. Double simultaneous stimulation (i.e., simultaneously bringing two bright objects into both temporal fields) normally causes the child to look from one object to the other; failure to take notice of one object may indicate homonymous hemianopsia. An opticokinetic tape (with repetitive bars or objects) should be drawn horizontally and then vertically across the child’s field of vision. An absent response results from lack of visual fixation or from gross impairment of vision. Unusual transient deviations of the eyes may occur in the first year of life [Echenne and Rivier, 1992].

A beam from a small flashlight should be directed at each eye to allow evaluation of pupil size (noting colobomas and anisocoria), pupillary responses and the red retinal reflex. To avoid frightening the child, the examiner could use the light in a playful manner such as directing the light beam at the child’s hand or abdomen while trying to avoid restraining the child or forcibly opening the eyes. There are many eye features to be noted, including symmetry of the palpebral fissures, relative size of the two globes, angulation of the eyes compared with other facial components (i.e., mongoloid or antimongoloid slant) and with the ears, cataracts, conjunctival telangiectases, colobomas of the iris, ptosis, proptosis, and malformed or eccentrically placed pupils. Hair color, patterning distribution, including unusual whorl patterns, and texture should be assessed.

The examiner should observe the child’s facial movements closely throughout the entire examination. Smiling at the child, tickling the child, or making unusual noises or facial grimaces often causes the child to smile or laugh, allowing observation of the nasolabial folds. Widening of the ipsilateral palpebral fissure or inability to bury the limbus when crying is indicative of facial nerve weakness. If facial weakness is found, one must distinguish central from peripheral cranial nerve VII dysfunction. The latter includes involvement of the muscle that raises the ipsilateral eyebrow. In the younger infant, sucking and rooting reflexes should be obtained. Tongue thrusting, drooling, and unusual shapes of the lips should be evident. Sometimes, the child can be induced to protrude the tongue if the examiner urges the child to imitate the examiner’s tongue movements. Deformity, atrophy, or abnormal positioning of the tongue can be observed. Tongue fasciculations should be evaluated with the tongue in the resting position, and by gently elevating the tongue with a depressor and examining the undersurface.

Basic responses to the sound made by a tuning fork, rubbing fingers together, ringing a bell, or using a toy noisemaker that generates noise at a modest volume may provide much information. The examiner must be careful not to confuse response to a visual cue (e.g., the movement needed to elicit noise from a toy) with response to the sound. Typically, the young infant orients toward the sound by turning the head with arrest of motor activity, while the child older than 6 months usually reaches for the noisemaker. If the child fails to perform satisfactorily, formal audiometry and auditory-evoked response testing should be obtained.

Motor Evaluation

As with all other parts of the evaluation, the motor examination begins with observation. Even before touching the child, the examiner should systematically observe the general posture and the symmetry of movements of arms and legs, and look for any gross discrepancies in muscle bulk or limb length. Definite hand preference (such as reaching across the midline to avoid using the contralateral hand) before 24 months suggests a central or peripheral nervous system impairment of the opposite hand and arm. In this situation, leg movement and use should be carefully studied to detect the presence of hemiparesis. Typically, there is also a decrease in spontaneous movement of the affected limb. Unusual posturing of the limbs may indicate paresis or evolving extrapyramidal disease.

Decreased muscle bulk may not be appreciated because of the large amount of subcutaneous fat, and muscle atrophy may be undetected. The examiner should be careful to palpate muscle mass beneath the fat and not to misinterpret the subcutaneous tissue as muscle.