"How to Do a Clinical Examination"
How to Do a Clinical Examination? History taking, physical examination and screening Adapted from J. 0. Forfar HISTORY Clinical examination in paediatrics relies on the classic principles of history taking and physical examination, which must be appropriate to the circumstances of the child, supplemented by such supplementary investigations as are necessary. The history has often to be obtained from parents or attendants and the examiner has to be alert to the possibility of secondary bias which they may introduce and of preconceived judgments based on limited knowledge or folklore. Firstly, parents should be encouraged to give a spontaneous account of a child's illness subject to such curbs on irrelevance or verbosity as the examiner deems expedient and secondly the examiner can ask specific questions to amplify and clarify the parents' description. Older children can provide much history, usually give an accurate account of their symptoms and answer questions directly without bias. The examiner has to decide before or during the history taking whether it is desirable for the child to be present. Children can be divided into age groups: Neonatal 1st 4 weeks of life Infant 1st year Preschool 2 to 4+ years School 5 to 15 years Childhood 1 to 15 years Adolescent 13-19 years HISTORY OF PRESENT ILLNESS Specific points for enquiry or amplification are: Age and sex. Note date of birth. Symptoms of abnormalities complained of and their duration. The precise order of symptoms including any repeated episodes (e.g. asthma or epilepsy). Changes noted since the onset of illness contrasting present with previous condition. Activity or apathy may be gauged by the child's movements in pram or cot, performance in normal household activities, interest in people and things, 1 willingness to walk to school or shops, tiring easily or returning early from play. Feeding and appetite. Temporarily or persistently impaired (this may lead to an assessment of daily caloric intake which with babies can usually be assessed in terms of intake of milk, amount of sugar and other elements, and with older children by analyzing a recorded daily diet), food fads or dislikes; unusual parental ideas regarding diet; daily intake of vitamin supplements? Difficulty in swallowing is commonly functional rather than organic and related to attempts to persuade the child to eat against his will with resultant choking and gagging. The child with organic dysphagia may swallow food but will regurgitate it shortly thereafter. Thirst. If present, assess the total daily intake of fluid and output of urine. Vomiting. Amount; frequency; duration; effortless or projectile; nature of vomitus (e.g. stained with bile or blood); an isolated symptom or associated with abdominal pain (e.g. appendicitis), distension (e.g obstruction), screaming (e.g. intussusception), diarrhoea, constipation, pyrexia or impairment of consciousness? In a baby the possibility of rumination necessitates questions regarding observed movements of the mouth and glottis prior to vomiting. Abdominal pain is probably thought to occur much more frequently than it actually does in babies due to their propensity for drawing up their legs and crying as a result of pain wherever it occurs. Toddlers, asked frequently if they have a 'sore tummy' in the presence of any upset, may come to use this term for pain at any site. With abdominal pain ascertain its nature; timing; duration; constancy or intermittency; site; aggravation by breathing or movement; relationship to food, bowel movement or micturition; association with anorexia, diarrhoea, melaena, constipation, vomiting, sore throat, cough or purpura. Abdominal distension. Generalized or localized, past or present, duration, associated pain? State of bowels and character of the stools. Normally infants pass several semi-solid mustard-coloured stools per day. Ascertain the frequency of bowel movement, character of the stools (hard or soft, watery, accompanied by mucus, blood streaked or mixed with blood, bulky, dark or pale, floating on water, malodorous), presence of involuntary faecal soiling (encopresis), pain or crying on defaecation (e.g. anal fissure)? Loss of, or gain in, weight. Has the child become thinner or fatter judged by general appearance, recent looseness or tightness of clothing, wasting, swelling or puffiness? Discharge from eyes, ears, nose or other sites. Purulent, watery or blood stained, profuse or scanty, continuous or intermittent? Sore throat. Babies and toddlers do not complain of sore throat although parents often assume this to be present (e.g. when there is refusal of food). Older children can localize pain. Cough. Ascertain duration; character; dry or moist; paroxysmal; more severe by day or night; disturbing of sleep; associated with pain, whoop, vomiting, chest pain, wheeze, nasal discharge; accompanied by sputum (swallowed or expectorated, watery, mucoid, mucopurulent or blood stained). 2 Breathlessness. Present only on activity (exercise tolerance) or at rest, persistent or intermittent, of gradual or sudden onset, exercise induced, nocturnal or diurnal, associated with cough, cyanosis or breath holding? Cry or voice. Any change noted? Mouth breathing. Mouth habitually open or shut, snores? Stridor. Continuous or intermittent, inspiratory or expiratory, duration? Wheeze. Inspiratory or expiratory, continuous or intermittent, precipitating factors, duration? Abnormalities of the breath. Sweet smell (acetone) or fetor? Other localized pain. Extent, nature, degree of severity, intermittent or continuous, duration and direction of radiation? Localized swellings. Site, size, colour, consistency, presence or absence of local pain, tenderness, duration? Rashes or other skin lesions. Site, colour, number and size of lesions; vesicles, ulcers, papules, macules, petechiae, itch? Jaundice. Time of onset, duration, any abnormality in the stools or urine, vomiting? Cyanosis. Peripheral or cental, persistent or intermittent, affected by environmental temperature? Pallor. Intermittent or persistent (many children are naturally-pale but pallor tends to cause anxiety in parents)? State of the musculature. Normal active movements or not? Does the mother feel the limbs to be stiff (hypertonicity or spas-ticity), or 'floppy', slipping through her hands on lifting (hypotonicity)? Changes in posture or in walk. Of long duration or recently developed, holding the body in any unusual way (e.g. torticollis or scoliosis); abnormality in gait (examine wear in shoes)? Co-ordination. Dropping things, spilling from a cup, impairment of fine movement (e.g. writing or buttoning clothing)? Involuntary movements. Nature of these, the same movement repeated or different movements, any injury suffered as a result of the movements, aggravated by emotional stress? States of reduced consciousness. Degree, premonitory symptoms or aura, duration, subsequent awareness of events, injury sustained? Convulsions and fits. State of the child prior to the convulsion, any precipitating factor (e.g. pyrexia), any premonitory symptoms, type of movement observed, duration of various stages, state of consciousness, loss of posture, incontinence, uprolling of the eyes, biting of the tongue or other injury, sleep or headache afterwards? Speech. Delay in onset or loss of speech, change in character, difficulty in comprehension or expression? Defects in vision. Able to follow a moving object with eyes, difficulty in reading or in distance vision, colliding with objects, colour blindness? Headache. Site, manner of onset, severity, and accompanying symptoms (e.g. vomiting)? Younger children seldom complain of headache. Hearing. Parental appreciation of hearing loss; unresponsive-ness or inattentiveness; any evidence of mental retardation, behaviour disorder or circumstance suggesting organic or functional deafness? Dysuria. Pain, burning or cry related to micturition? Frequency of micturition. How frequent, any recent change in pattern? Bed wetting and incontinence. Always present or recently developed; any polyuria, dysuria, frequency of micturition, or thirst; do particular circumstances produce the symptoms and what is the parental reaction to them (e.g. punishment or ridicule)? 3 Volume of urine. Evidence of actual volume passed (parents tend to overestimate the amount of urine passed per day). Character of the urine. Colour (e.g. amber, red, smoky, like tea or Pepsicola), abnormal odour? Manipulative ability. Ability to handle objects, use spoon, knife and fork, dress and undress himself, tie shoe laces (c. 6 years), write. Behaviour and mood (best discussed in the absence of the child). Active or hyperactive; quiet or lethargic; loquacious or silent; given to 'cocktail party' chatter (common in hydrocephalus); disobedient; aggressive; negative; reluctant to go to school; refusing food; withdrawn; averse to social activities; resistance to bedding; reluctant to sleep; fearful of the dark; subject to nightmares or night terrors, temper tantrums, nail biting and thumb sucking; carefree or anxious; whining, nagging, demanding attention; fastidious or careless; 'highly strung' or placid; crying too readily; jealous; volatile or stolid; speech disturbed? Relation- ship with parents, siblings, schoolmates and teachers? Treatment already given must be ascertained. Selectivity of questioning. Not all of the questions indicated above will be asked in every instance; some will be secondary questions dependent on positive answers to primary questions. Better to ask too many rather than too few questions: the more extensive the questioning the more likely are forgotten points of history to be uncovered. PREVIOUS HISTORY Mostly a history about past illness in a child has to be obtained from parents, guardians or others. Baby books, photographs, health visitor and infant clinic records may help. Birth history An account of the mother's pregnancy and the birth is a necessary part of history taking in the case of an infant or young child. Ascertain illnesses which the mother had before or during pregnancy, exposure to drugs or radiation, hydramnios, hypertension, oedema, albuminuria, threatened abortion or antepartum haemorrhage, length of gestation, duration of labour (prolonged or precipitate), type of delivery (high forceps and breech carry particular risk). Note the infant's birthweight, state at delivery (e.g. Apgar score), and postnatal history regarding events such as convulsions, breathing difficulties, cyanosis, jaundice, vomiting, infection, special or intensive care. Feeding Nature of the feed, its composition, volume, frequency, any additional supplements and the state of the appetite? Previous illnesses Diagnosis, dates of occurrence, duration and severity? Contact with infectious disease Include contact with animals. Residence abroad Country and any particular hazards it holds? Prophylactic inoculations 4 Inoculations received and any reaction to them? FAMILY HISTORY Parents' ages, present state of health, past health and possible consanguinity? Previous stillbirths or miscarriages (mothers who have had difficulty in conceiving and have had abortions, stillbirths, infant deaths or abnormal children are more likely to have children who suffer from congenital abnormalities and cerebral palsy)? Past or present illnesses or deaths (with cause of death) of siblings? Illnesses of other relatives or occupants of the house? Hereditary traits require enquiry regarding a much wider circle of relatives. DEVELOPMENTAL HISTORY This involves the time of achievement of milestones of motor, vision/fine motor, social/adaptive and hearing/language progress (see Fig. 2.19). Note any evidence of dissociated development (e.g. delay in reaching linguistic compared with motor milestones would raise the question of hearing impairment or speech disorder, standing holding on to furniture before sitting cerebral diplegia, normal manipulative ability combined with retarded postural milestones ataxic cerebral palsy). Approximately 15% of mentally retarded children reach normal motor milestones during infancy. SOCIAL AND ENVIRONMENTAL HISTORY With most disorders it is necessary to build up a picture of the child's social and cultural environment; to appreciate fears and stresses both at home (e.g. parental attitudes, separation and divorce, absence of a parent, illness or chronic disability in the family, jealousy at the arrival of a new baby, the possible death of a near relative) and at school (e.g. a change of school, over-rigid discipline or bullying); to judge intelligence and ability as they affect capacity to meet the demands of communal living and education; to ascertain the occupation of the father, the size and condition of the child's home. Inadequately explained injuries or neglected appearance may raise the possibility of child abuse. PHYSICAL EXAMINATION The examination of infants and children is an art demanding qualities of understanding, sympathy, patience and at times finesse and subtlety. The paediatric patient who enters the consulting room or is ill in bed may be a bawling infant whom nothing will pacify, a toddler clinging to his mother and burying his tearful face in her lap at the slightest movement of the examiner towards him, a more robust young man of early school age who stoutly and persistently resists all attempts to remove his clothing, particularly his trousers, an uninhibited hyperactive child who moves rapidly round the room deploying his destructive interest against toys, instruments or the examiner's papers, or an apprehensive schoolgirl who just retains her self-control during questioning but recoils in terror at the production of a sphygmomanometer or ophthalmoscope. RELATIONSHIP OF EXAMINER TO CHILD AND PARENTS The child should be placed where he wishes, be it on the parent's knee or on a chair by himself. Removal of clothing or a visit to the examination couch may come later. The examiner must remain patient and confident even if provoked and not be too conscious of his own dignity. Impatience and irascibility will deprive him of information which 5 might be available. His demeanor should be friendly, sociable, tolerant, good-natured and restrained as he listens, observes, notes and judges. He should encourage the mother and child no matter how the latter behaves. Loud noise tends to alarm children; a soft persuasive voice is more likely to be effective than stentorian exhortation. Conversation should be attuned to the intellectual and social level of the patient. Simple explanations should be given about what is being done and disturbing or painful procedures kept to the end. GENERAL INSPECTION Clinical examination of a child begins from the moment of first meeting. A glance will reveal the state of consciousness. Much may be learned from first impressions of the child's appearance, demeanor, state of nutrition, reaction to the environment, relationship with parents, conversation, speech, cry, size relative to age, state of nutrition, state of activity, posture, overt deformity, injury or haemorrhage. 1. The facies may indicate pain or anxiety, the blankness of mental retardation, wasting (Fig. 2.4n) or the spasmodic movement of the tic. Weight loss or dehydration may be revealed by hollow cheeks and sunken eyes, oedema by periorbital swelling (Fig. 2.4q). Mouth breathing, jaundice or cyanosis may be present. Some disorders e.g. mongolism, cretinism (Fig. 2.4c), gargoylism (Fig. 2.4f), de Lange's syndrome (Fig. 2.4e) and sometimes tetanus (Fig. 2.4o) may be diagnosed immediately by the characteristic facies which they exhibit. Rashes call for appreciation of colour, size, distribution and nature. The skin may be dry and loose in dehydration with loss of elasticity on lifting up skin folds, loose but not inelastic in weight loss, ulcerated, infected, dry, ichthyotic, angiomatous, abnormally pigmented, sweating abnormally, shiny and tense, pitting on pressure, showing localized swellings (Fig. 2.4d) or striae. 2. The nails may be bitten and show abnormalities including deficient formation, ridging, abnormal curving, infection. Finger clubbing may be present. 3. Disturbance of rate or pattern of breathing or dyspnoea may be visible. Abnormal sounds such as high pitched cry, cough, wheeze, stridor or whoop may be heard. 4. Abnormal smells such as acetone or the mousy odour of phenylketonuria may be detected. 5. Posture may be abnormal as in opisthotonus, kyphosis or torticollis. A few moments may tell much of a child's psychological makeup and intelligence. Is he nervous, excitable, distractable, withdrawn, intelligent or stupid? What is his emotional relationship with his parents? PHYSICAL MEASUREMENTS Weight Over the first few days of life physiological weight loss of up to 15% of bodyweight with return to birthweight (BW) by 7 to 10 days is common followed by weight gain of approximately 25 g per day up to 3 months. Expected weight in kilograms can be calculated as follows: 6 First year: during 1st 4 months: BW + (age in months x 0.8) during 2nd 4 months: BW + (age in months X 0.7) during 3rd 4 months: BW + (age in months x 0.6) (Infants normally double their birthweight by 5 months and treble it by a year) Between 1 and 9 years add 4 to the age in years and multiply by 2 Between 9 and 12 years treble the age in years. One standard deviation from these figures = 12^% of their value. Length (p. 391) At birth, length is approximately 50 cm (20 in), at 6 months 68 cm (27 in), at 1 year 75 cm (30 in), at 2 years 85 cm (34 in), at 3 years 95 cm (37 in) and at 4 years 100 cm (39 in). Over the next 8 years there is an annual increase of approximately 5.5 cm (2 in). One standard deviation from these figures = 4% of their value. In infancy, because of the difficulty of measuring crown-heel length accurately, crown-rump length is the most representative measurement of length (see Fig. 8.36). The mean crown-rump length at birth is 34 cm (13 in), at 2 weeks 34.5 cm (13s in) and at 6 months 43.5 cm (17 in). Body proportions and shape change with age (Fig. 2.2). Measurement of the arms should be from the tip of the acro-mium to the tip of the middle finger and of the legs from the anterior superior iliac spine to the internal malleolus. Coincidental measurements of length and weight can be used to calculate 'weight for length' which may be of value in the Stature divided into quarters Marfan's syndrome, or as nutrition indices — length for age, weight for age or weight for length below the 5th centile would indicate undernutrition in the absence of any disease process. Newborn 2 5 Age in years Fig. 2.2 Body proportions at different ages. Head circumference (p. 416) 7 At birth 35 cm (14 in), 6 months 43.5 cm (17 in), 1 year 46.5 cm (18 in), 2 years 49 cm (19 in): Increases annually by approximately half a centimetre from 2 to 7 years and by one-third of a centimetre from 8 to 12 years. One standard deviation from these figures = 2^% of their value. A cranial hemicircumference less on one side than the other may indicate hemiplegia on the contralateral side. Temperature Pyrexia is a common concomitant of disease in the child but hypothermia may also be so, particularly in the newborn. The infant's temperature can be taken in the groin with the thigh flexed on the abdomen (normal = 37°C or 98.4°F) or in the rectum (normal = 37.5°C or 99.5°F). In older children the axilla is more suitable. Low reading thermometers covering the range 29-43°C (85-109°F) are necessary in assessing hypothermia and should be used routinely in paediatric practice. Premature infants have temperatures 1°F or so lower than full-term infants. Blood pressure The normal auscultatory method can be carried out over the age of 3 years but is more difficult in younger children in whom sedation may be necessary. The cuff width should be two-thirds of the upper arm (cuffs of 5 cm (2 in) and 7.5 cm (3 in) are available). Auscultatory blood pressure determination in the legs is difficult. The palpatory method (systolic pressure) may be employed with babies and toddlers and where neither of these methods is possible the flush method may be used. A suitably sized cuff is applied loosely round the upper arm or thigh, the limb below the elbow or knee is blanched either by manual compression or an elastic bandage and the cuff inflated above 200 mmHg. The compression is then released and the cuff pressure lowered slowly, the point at which the blanched area flushes being noted. The flush pressure is midway between systolic and diastolic pressures. Non-invasive automatic monitoring methods of measuring blood pressure are unreliable in small sick infants in whom the most reliable method is a transducer 'attached to an intra-arterial catheter (Weindling 1989). Average blood pressures (mmHg) are: Newborn (range of flush method) 35-85 Infancy (systolic/diastolic) 80/55 Preschool child (systolic/diastolic) 85/60 School child (systolic/diastolic) 90/60 EXAMINATION OF INDIVIDUAL SYSTEMS AND REGIONS Individual systems can be examined in a standard sequence but in practice this is seldom appropriate. It is better to begin with the system which is likely to reveal the most information and to leave to the last disturbing procedures and systems likely to yield least information. 8 HEAD AND NECK Cranium Pathological cranial shapes are illustrated in Figure 2.3. Note colour, quantity and character of the hair and level of the hair line. The anterior fontanelle measures approximately 2.5 cm (1 in) x 2.5 cm at birth and does not close until 18 months (delay may be seen in rickets, increased intracranial tension or Microcephaly (d) Scaphocephaly. (e) Hydrocephaly (f) Plagiocephaly. (g) Caput succedaneum. (h) Cephalhaematoma. Fig. 2.3 Head shapes. visually and by palpation. The posterior fontanelle measures 0.5 cm (i in) in diameter at birth and closes shortly thereafter. Cranial sutures such as the sagittal and coronal are easily palpable at birth but the edges are not widely separated (e.g. as in raised intracranial pressure). Sutures may be prematurely closed and the edges heaped up in cranial synostosis. Scalp veins may be distended (Fig. 2.4t). Craniotabes (seen in prematurity and rickets) is a reduction in the rigidity of the cranial bones which can be indented by finger pressure over the parieto-occipital region. 9 Structural defects such as lacunae or areas of thickening may be detected by palpation. Cranial bruits may be audible on auscultation over the vertex, occipital or temporal regions. After the fontanelle has closed a 'cracked pot sign (raised intracranial pressure) may be elicited by a sharp tap over the cranium. Ears The external auricle may be deformed, the meatus narrowed or absent or the ears low set (one-third of the external auricle should be above a horizontal line at eye level). Low-set ears are often associated with other congenital abnormalities. Auriscopic examination (the speculum should be appropriate to the size of the infant) may reveal wax or purulent discharge (if necessary clear with a metal loop or cotton wool on an orange stick), a tympanic membrane (ear drum) which is dusky, bulging, retracted or perforated. Distortion of the cone of light which normally extends forward from the tip of the handle of the malleus usually indicates infection, undue prominence of the malleus retraction of the drum. Perforations are most likely in the upper part of the drum. Eyes Distance apart may be measured in terms of inner canthal distance, mid-pupillary distance or outer canthal distance. Age and racial differences can be offset by using the canthal index (100 x inner canthal distance divided by the outer canthal distance), which is 38 mm (SD 2.4) for boys and 38.5 mm (SD 2.4) for girls. The eyes are wide apart (telecanthus) in acrocephalo-syn-dactyly (Apert), hypertelorism, Waardenburg's syndrome and craniofacial dysostoses such as Crouzon's. They may be close spaced (e.g. Down's syndrome) or displaced downwards (the 'sunset sign') in hydrocephalus Ptosis is drooping of the eyelids. The colour of the mucous membrane of the lower eyelid may give some indication of anaemia. The eyeballs may be prominent (exophthal-mos), sunken (enophthalmos), enlarged (buphthalmos) and may exhibit nystagmus, squint (strabismus), conjunctivitis, icterus, haemorrhage, congenital defects (e.g. colobomata or aniridia), .abnormal iris pigmentation, Brushfield's spots (small whitish inclusions in the iris — often present in Down's syndrome), opacities of the cornea, lens or intraocular chambers. The pupils may be different in size (e.g. amblyopic eye pupil larger than normal); they should react to light and accommodation. Vision in babies can be tested crudely by observing whether they follow an object held 18 in in front of the face and moved from side to side through an arc of 30°. Ophthalmoscopic examination is best carried out in a dim room. The examiner's eye should be as near as possible to the ophthalmoscope and his head placed so that it does not obstruct the gaze of the child's other eye. If observer and patient each have normal vision no lens ('0') is necessary. The need to use a plus (+) lens indicates hypermetropia, a minus (—) lens myopia. An examiner with a refractive error should correct this with a lens of the ophthalmoscope. To get the young child to look in a fixed direction get him to look with some expectancy in the required direction and periodically fulfil his expectations, e.g. 'tell me when the torch flashes' — a helper being prepared to flash the torch at the appropriate moment. Babies looking backwards over the mother's shoulder tend to open their eyes. Dilatation of the pupils is not usually necessary routinely but for full examination 1% hom-atropine may be used. 10 Ophthalmoscopy may reveal opacities of the media, refractive errors, retinal haemorrhage, venous en-gorgement, papilloedema with blurring of the disc edges or filling of the optic cup with vessels coming into focus in front of the disc, pallor of the disc, exudate, choreoretinitis, abnormal pigmentation, cherry-red spots or choroidal tubercles.In older children it is usually possible to test visual fields against the examiner's fields, each looking straight at the other's pupil 18 inches apart, the examiner moving a cotton wool pledge on the end of an orange stick midway between himself and the patient to map the periphery of the patient's field as judged against his own. Nose The nose may show evidence of infection or allergy in the form of a purulent or watery discharge. Movement of the alae nasi may indicate respiratory difficulty. The nasal mucosa may be pale, congested, watery or dry; the septum may be deflected; polyps may be present. By closing off the mouth, blockage of the nasal passages may be demonstrated. Mouth The lips may show oedema, pallor, cyanosis or ulceration. The mouth may be examined with the co-operation of the child or without it (Fig. 2.5). Mucosae may be moist, dry, well coloured or pale, ulcerated, blistered, bleeding or purpuric, exhibiting the white curd-like plaques of thrush which will not easily scrape off, or Koplik's spots (measles) on the buccal mucosa. The gums may be ulcerated or hypertrophied (e.g. phenytoin). The teeth can be observed for number, whether primary or secondary, size and shape, pigmentation, caries and enamel defect. The average times of eruption of the teeth are as follows: Deciduous (primary) teeth Eruption Shedding central incisors lower 7th month 75 months upper Lateral incisors lower 9th month 86 month upper first molars 12th month 100 month canines 18th month 122 month second molars 24th month 109 month The teeth in the lower jaw are shed earlier than those in the upper and girls' teeth earlier than boys' teeth. Permanent dentition Eruption First molars Lower 6th year Upper Central incisors Lower 6th year Upper 6th to 7th year 11 Lateral incisors Lower 7th year Upper 8th year Canines Lower 10th year Upper 10th to 11th year First premolars Upper 10th year Lower 10th to 11th year Second Upper 10th year premolars Lower 10th to 11th year Second molars Lower 11th to 12th year Upper 11th to 12th year Third molars 20th year Tongue: size, shape, 'geographic' appearance Palate: cleft or high arched, any abnormality of the uvula? If the child is willing to say 'ah' examination of the posterior pharynx is easy; otherwise induce the gag reflex by touching the posterior pharyngeal wall with a spatula and taking advantage of the few brief moments during which the posterior pharynx is revealed on gagging (Fig. 2.5). Tonsils: size, presence of infection, exudate, pitting, peritonsillar swelling? Posterior phalangeal wall: inflammation, postnasal discharge, presence of lymphoid tissue? Fig. 2.5 Examining the mouth — uncooperative child. Neck Examine from front and back noting any shortening, webbing (Fig. 2.4r), torticollis (Fig. 2.4n), head retraction or neck stiffness (by passive flexion), limitation of flexion (ask child to put nose on knees while sitting with knees drawn up), abnormal swellings (e.g. lymph glands, thyroid, cystic hygroma (Fig. 2.4b) — trans-illuminable). A sternomastoid tumour (early infancy) is a hard visible nodule within the body of the sternomastoid muscle. Lymph nodes 12 Enlargement may occur in the anterior and posterior cervical triangles, occipital and submental (lateral to salivary glands) regions — as well as axillary, epitrochlear and inguinal regions. Ascertain tenderness, inflammation, fluctuation and size. CHEST AND LUNGS Inspection The cross-section of the infant's chest is circular compared with elliptical for the older child or adult. This imposes limitations on expansion so that respiration is diaphragmatic and abdominal and dyspnoea is reflected in both respiratory and abdominal movements. In infancy over-inflation of the chest (e.g. in bronchiolitis) is most evident in the upper half of the chest anteriorly. Chronic emphysema can cause an increase in the anteroposterior diameter of the chest (pigeon chest) which is normally less than the lateral diameter. Chest circumference ranges from 32 ± 5 cm at birth to 75 ± 16 cm at 14 years. Chest expansion measured at the nipple line between full inspiration and expiration can be measured in co-operative older children. It should be 4 cm (U in) or more. Asymmetry is best detected by watching chest movement on taking a deep breath. Visible deformities of the chest include pectus excavatum, Harrison's sulcus (at insertion of diaphragm), praecordial bulging, thickening of the costochondral junctions (rickety rosary), the dinner fork deformity of the costochondral junction (scurvy) and anterior prominence of the ribs on one side with frontal skull prominence on the same side (the 'squint baby' syndrome). The respiratory rate must be measured when the infant is at peace, not crying, struggling or feeding. Upper limits of normal for various ages are as follows: 40 per minute 0 to 2 years 30 per minute 2 to 6 years 25 per minute 6 to 10 years 20 per minute over 10 years Respiratory rhythm may be disturbed in time and amplitude particularly in premature and asphyxiated babies. Infection can cause respiratory inversion — the respiratory cycle changing from inspiration/expiration/pause to expiration/inspiration/pause, often described by the mother as a 'catch' in the infant's breathing. The normally longer inspiratory phase of respiration may be exceeded by a lengthened expiratory phase (e.g. in asthma). The pliable chest wall of the infant readily reflects changes in intrathoracic pressure, increased inspiratory effort or obstruction of air flow resulting in intercostal indrawing and costal margin recession. Abnormal respiratory noises include inspiratory and expiratory stridor, wheeze, cough and grunting respiration. Palpation The chief landmarks of the child's chest are shown in Figure 2.6. Palpation with the palm of the hand may reveal a cardiac thrill, palpable rhonchi, the crepitant sensation of subcutaneous emphysema, local tenderness or swelling. Due to the mobility of the trachea in infancy and childhood tracheal displacement is not a reliable sign. Axillary lymph nodes should be examined by abducting the arm from the trunk, inserting the fingers into the axilla and palpating, with the arm replaced beside the trunk. 13 Percussion Percussion landmarks are shown in Figure 2.6. The percussion note is more resonant in the child than in the adult and percussion should be lighter: significant impairment usually means extensive consolidation or fluid in the chest. Hyperresonance occurs in over-inflation, emphysema or pneumothorax; reduced cardiac dullness is a valuable concomitant sign of these. Auscultation The breath sounds in infants and children are harsh (broncho vesicular). The inspiratory sound is normally two to three times longer than the expiratory and followed by a pause. Breath sound intensity may be reduced in conditions such as bronchiolitis, emphysema, pneumothorax and pleural effusion and increased with consolidation or collapse where an affected lobe or segment collapses against a bronchus giving rise to bronchial breathing (sounds similar to those heard on auscultation over the trachea). Rhonchi and coarse crepitations are usually associated with infection or bronchospasm, fine crepitations with infection. The wheezing sounds associated with asthma are high pitched and musical. Pleural friction is a creaky leathery sound giving the impression of being close to the ear. 14 Landmarks in a child's chest: (a) anterior, (b) posterior, (c) right lateral, (d) left lateral. Vocal resonance, heard on auscultation of the chest when the patient speaks (e.g. says 'one two three'), may be diminished (e.g. pleural effusion) or increased (e.g. consolidation). If whispering is easily audible on auscultation whispering pectoriloquy is present. Abnormal signs in the cardiovascular system such as displacement of the apex beat may be due to respiratory disease. CARDIOVASCULAR SYSTEM Inspection 15 Cardiovascular disease may be accompanied by a range of general signs such as poor physical development, squatting, dyspnoea, tachypnoea, central cyanosis, oedema finger clubbing and distension of superficial veins. Cardiac enlargement may be associated with praecordial bulging and abnormal pulsation — right ventricular hypertrophy causing increased pulsation in the central and superior parts of the praecordium, left ventricular hypertrophy increased pulsation and visible lifting of the apical praecordium. The position of the apex beat is an important landmark (Fig. 2.6). Jugular venous pressure (increased if above the level of the manu-brium sterni) can be assessed in older children but this is rarely practicable in infants due to their shortness of the neck, mobility and lack of co-operation. Palpation Applying the palm of the hand to the chest, thrills, increased praecordial pulsation (apical in left ventricular hypertrophy and basal and right sided in right ventricular hypertrophy) and diastolic shock (in the pulmonary area in pulmonary hypertension) may be felt. The apex beat, normally in the fourth or fifth intercostal space within the mid-clavicular line, can be identified at the point of maximum impulse. The pulse can be examined at the wrist (radial) or inguinal region (femoral). Sinus arrhythmia (increase in rate on inspiration with decrease on expiration) is common in most children. A bounding pulse is usually associated with lesions causing shunting (e.g. VSD or PDA), a weak pulse with restriction of left-sided cardiac outflow (e.g. aortic stenosis), a collapsing (water hammer) pulse with wide pulse pressure (difference between systolic and diastolic pressures) (e.g. aortic regurgitation). The latter is best felt with four fingers laid across the pulse with the arm elevated. In coarction of the aorta the femoral pulses may be absent, or delayed compared with the radial, and in older children pulsating collateral vessels may be detected in the scapular region. Percussion The thin chest wall of the child makes cardiac percussion of more value than in the adult. The right cardiac border does not normally extend beyond the right sternal edge: the upper border is at the level of the second intercostal space (Fig. 2.6). In conjunction with the position of the apex beat (and the character of praecordial pulsation) these assessments are of some value in determining cardiac size and/or displacement. Diminished or absent cardiac dullness is found in emphysema and pneumothorax. Auscultation The ranges for heart rate in infancy and childhood are: Newborn Infant Preschool child School child 70/120 80/160 75/120 70/110 Auscultation should be carried out over the areas shown in Figure 2.6. Auscultatory assessment concerns cardiac rhythm, heart sounds, and murmurs. Sinus arrhythmia (see above) is normal in children: triple or gallop rhythm is usually associated with cardiac failure: irregular irregularity (e.g. due to extra systoles or, rarely in childhood, atrial fibrillation) may occur. Close splitting of the second heart sound in the pulmonic area (often present on inspiration and absent on expiration) is common in normal children: pathological splitting is wider and does nor vary with respiration. The commonly heard and often physiological third heart sound is differentiated from a split second sound by being heard best at the apex, widely separated from the second heart sound and of lesser intensity. An ejection click may be associated with stenosis of a valve. There is diminished intensity of heart sounds in cardiac failure or pleural effusion; and of the pulmonic second sound in pulmonary stenosis and aortic second sound in aortic stenosis. Cardiac hypertrophy accentuates heart sounds. Description of murmurs should include site (Fig. 2.6), intensity (0-6) with point of maximum intensity, timing (systolic — pan, early or late, or diastolic — early diastolic, mid-diastolic or presystolic) (Fig. 2.7), 16 propagation (mitral systolic murmurs radiate to the left axilla, aortic systolic to the neck, aortic regurgitant down the left sternal edge) and variation with position. Coarctation of the aorta may produce a murmur audible over the back. A venous hum is a continuous loud murmur at the root of the neck abolished by pressure over the jugular vein or placing the child in the head-down position. A pericardial friction rub is a to and fro leathery sound appearing on auscultation to be close to the examiner's ear. Cardiovascular disorder may also be revealed in other systems, e.g. by hepatic enlargement in cardiac failure. ABDOMEN Inspection Disease may reveal itself by distension with a shiny tense abdominal skin, a scaphoid abdomen with lax wrinkled skin or by distended abdominal wall veins. Absent abdominal wall movement on respiration mav indicate a disorder such as peritonitis. Visible peristalsis may be seen in obstruction (e.g. in the epigastrium in pyloric stenosis): obstruction low down in the gut may cause a ladder pattern. A distended bladder may show as a rounded swelling in the suprapubic region. Umbilical abnormalities include hernia, omphalocele, infection or discharge. Other hemiae may be present and inguinal lymph glands may be enlarged. Palpation Should be carried out with warm hands usually with the examiner seated beside the child lying recumbent or held on the mother's knee, head supported, knees and hips flexed. Crying children relax the abdominal muscles during inspiration allowing the examiner a brief moment when palpation is possible. Tenderness should be sought using gentle palpation followed by deeper palpation, while simultaneously looking for evidence of pain in the form of facial grimacing. Rebound tenderness (sudden withdrawal of the hand on deep palpation) may confirm doubtful tenderness. Guarding of the abdominal wall may be localized or generalized and in severe degrees may amount to boarding. Palpation of a pyloric tumour (start with the stomach empty and give a feed) is carried out as in Figure 2.8: the tumour may harden and relax intermittently and be impalpable when relaxed. Fig. 2.8 Palpation of pyloric tumour in pyloric stenosis The lower border of the liver is normally 1 cm below the costal margin in infants and children and can be felt rolling under the finger as it descends during inspiration (Fig. 2.9). Liver span, measured in the mid-clavicular line, is the distance between the upper border of the liver (Fig. 2.6) determined by percussion and the lower edge. At a body weight of 20 kg the mean span (±2 SD) is 8 cm (±1.8 cm), and at 60 kg 10.2 cm (±2.0 cm). An enlarged spleen is felt in the left hypochondrium possibly extending into the left iliac fossa in infancy and the right in older children. In lesser degrees of splenic enlargement the dp can be felt descending superficially below the costal margin on deep inspiration (Fig. 2.10). The kidneys can be palpated bimanually at the end of forced expiration (Fig. 2.11). A distended bladder may reach the umbilicus. Tumours (e.g. Wilms' or 17 neuroblastoma) may be felt and the soft sausage-shaped tumour of intussusception be found in the right upper quadrant during relaxation A strangulated hernia (e.g. inguinal) may be hard, irreducible and very tender. With ascites a fluid thrill may be detected, the wave caused by flicking the abdominal wall on one side being transmitted to a hand-held flat on the other side, with the hand (hypothenar edge) of a second person placed on the abdomen in the longitudinal axis of the patient to suppress any transmission of shock waves through the fat of the abdominal wall. Enlarged inguinal lymph glands may be palpable. Fig. 2.9 Palpation of liver. Fig. 2.10 Palpation of spleen. Fig. 2.11 Palpation of kidney. Percussion When supine, dullness due to free fluid may be present in both flanks but on turning the patient on to one side disappears from the upper side while the level of dullness rises over the lower side (shifting dullness — allow half a minute for change to take place). A distended bladder will cause increased dullness in the suprapubic area. Intra-abdominal masses may impair percussion. Auscultation Tinkling, crackling bowel sounds are normally audible intermittently over the abdomen, are accentuated with increased peristalsis (e.g. obstruction) and absent with ileus (e.g. peritonitis). Examination of stools Note colour, consistency and the presence of abnormalities such as blood, mucus or pus. Bright red blood suggests bleeding from the lower alimentary tract, melaena from the 18 upper while streaking suggests local bleeding (e.g. anal fissure). Threadworms may be noted on the stool. Rectal examination Use a finger appropriate to the size of the patient to detect the tone of the anal sphincter; faecal masses, including their character; gripping of the finger (e.g. anal stenosis, or narrowed segment in Hirschsprung's disease), intrapelvic masses or undue pain. Note material on the withdrawn finger (e.g. blood). Genitalia Female genitalia may show labial adhesions, clitoral enlargement, abnormal discharge, abnormality of the vaginal introitus (e.g. sexual abuse) or urethra. In the male note the size of the penis and any developmental abnormality such as hypospadias, phimosis or prepucial infection (balanitis). The testes should be examined for presence in the scrotum (in a warm room with warm hands as cold will cause retraction), abnormality in size and shape: if palpable in the inguinal canal see if they can be manoeuvred gently into the scrotum. Transillumination may reveal the nature of a scrotal swelling (e.g. hydrocele is transilluminable, solid tumour is not). The assessment of secondary sexual characteristics is described in Chapter 8. NERVOUS SYSTEM The nervous system can be examined in the older child with the same exactitude as in the adult but in the infant and young child has not achieved the functional precision with which it operates later; intelligent co-operation is also likely to be lacking. Neurological deficits are often more easily observed in the course of everyday activities than on formal examination: they may reveal neglect of a limb (e.g. hemiplegia), strabismus, clumsiness, inco-ordination, intention tremor, ataxia and neglect of sound. Developmental assessment is largely the assessment of the maturation of the nervous system (Ch. 9). Inspection After infancy it is usually possible to test specific aspects of neurological function and intelligence separately but during infancy neurological and intellectual disorders tend to express themselves in terms of disturbed motor function. A neurological lesion may be appreciated as impaired intelligence and vice versa. Note state of consciousness, such as degree of alertness, interest, memory for events, hyperexckability, hyperirritability, unrespon-siveness, drowsiness, semi-consciousness or unconsciousness. Disorders of posture and movement may result from neurological disease. In recumbency is he too inactive and is posture unduly affected by gravity (hypotonia)? Are there normal limb, trunk and head movements or abnormal movements (e.g. the writhing movements of choreo-athetosis, intention tremor or the repetitive involuntary movement of tics) or purposeless roving movements of the eyes (blindness)? Does he sit and stand? Are there any convulsive movements, generalized, localized, or the 'salaam' attacks of myoclonic epilepsy (in which the sitting infant suddenly falls forward or drops his head)? Is there any incoordination, clumsiness, abnormality of gait (e.g the broad-based groping walk of ataxia; stiff-legged scissoring gait of spasticity with difficulty in putting the heel to the 19 ground; the outward flinging and stiffness of one leg with adduction, elbow flexion and limited swinging of the arm on the same side seen in hemiplegia; or the staggering gait of cerebellar disturbance)? Are there any palsies (e.g. facial (Fig. 2.4m) or Erb's, or wrist drop). Cry and speech may be affected (e.g. the high-pitched cry of cerebral injury, the peculiarly specific cry of the cri-du-chat syndrome, delayed speech in mental retardation and autism, lack of speech intelligibility in mental retardation or cerebral palsy, aphonia in chorea, stammering, monotony and lack of expression in deafness and certain types of cerebral damage). Range of vocabulary, language and ideas give some indication of intelligence. Cranial nerves With younger children lack of co-operation will diminish ability to test the cranial nerves although with practice observation of the child's movements may enable a limited examination to be made. 1st (olfactory) nerve. Only older children have the experience and ability to distinguish smells such as orange, lemon or chocolate. 2nd (optic) nerve. Can the infant and toddler see and follow (later name) objects? For older children use Snellen types. Test visual fields. Ophthalmoscopic examination. 3rd (oculomotor), 4th (trochlear) and 6th (abducens) nerves. Test eye movements in all directions (the common abnormalities are inability to deviate the eyes fully upward or abduct the eyes fully). 3rd nerve paralysis causes ptosis, lateral deviation of the eye (unopposed action of 6th nerve) and pupillary dilatation; 4th nerve paralysis (often along with 3rd nerve paralysis) diplopia on looking downwards and medially (e.g. going down stairs), 6th nerve paralysis (of lateral rectus muscle) loss of abduction of the eyes (paralytic squint) on looking to the affected side. Paralysis of the sympathetic innervation of the pupil (usually damage to the cervical sympathetic chain) results in a contracted pupil (mei-osis) still reacting to light and accommodation, along with ptosis, enophthalmos and loss of sweating (anhidrosis) on the affected side of the face (Homer's syndrome). 5th (trigeminal) nerve. Test sensation over the area supplied by the sensory branch — forehead, cheek and lower jaw — and by the corneal and jaw reflexes; motor function by palpating contraction in the masseter muscle when the patient clenches the teeth. 7th (facial) nerve. Upper motor neurone lesions affect the lower part only of one side of the face so that the teeth cannot be shown effectively and the lips formed for whistling; lower motor neurone lesions affect the whole of one side of the face so that, in addition to the above, eye closure, forehead wrinkling and smilling are affected (Fig. 2.4m). 8th (auditory) nerve. Damage to the auditory branch impairs hearing (see Chapter 9 for testing): damage to the vestibular branch may result in impaired balance and positional nystagmus (following sudden rotating movement of the head). 9th (glossopharyngeal) and 10th (vagus) nerves. Test their motor function by asking the patient to say 'ah' — paralysis on the affected side results in the palate being drawn to the healthy side or merely lack of movement. X-ray palatography may help. For diagnosis of paralysis of the recurrent laryngeal branch of the vagus see Chapter 14. Enlarged adenoids may also limit palatal movement. llth (spinal accessory) nerve. Demonstrate trapezius weakness by exerting downward pressure on the patient's shoulders as he tries to shrug them. 20 12th (hypoglossal) nerve. Paralysis affects the same side of the tongue which deviates to the affected side on protrusion, or waggling shows limited movement towards one side. Muscle tone Handling of the child and passive movements of limbs indicate muscle tone. Muscle softness, floppiness, excessive laxity of joints (with hyperextensibility of the digits, elbows and knees) are the features of hypotonia; excessive firmness of muscles, stiffness and limited mobility on passive movement indicate hypertonia. Spas-ticity in the upper limbs can be tested by extending the arm fully at the elbow, supinating the forearm and seeing if there is a pro-nator flick when the limb is released. Hypertonia may be 'clasp knife' (stretching the muscle beyond a certain range results in sudden relaxation of tone), generalized or regionalized (e.g. adduction of the thighs and shortening of the tendo- Achilles with plantar flexion of the foot — Little's disease). Motor power Assessed by observing capacity for muscular activity and ability to overcome resistance (by examiner) to various movements. Co-ordination Specific tests such as the finger-nose test or the picking up of small objects (see Fig. 9.2) may examine this. Dysdiadochokinesis (inability to carry out rapid repetitive movements such as patting the back of the hand or asymmetry of footsteps heard on running) is a feature of ataxic cerebral palsy. A tendency to run on the toes suggests cerebral diplegia. A hemiplegic posturing of the limb on one side may be most obvious on running. Sensation Pin prick, normally causing a withdrawal response, can be used to test sensory loss. In older children sensation can be tested by light touch (cotton wool), two-point discrimination (compass points normally recognized 2-3 cm apart), temperature appreci- ation (hot or cold water in test tubes), positional sense (ask him to state the position of a joint passively moved and screened from him, e.g. 'is the toe pointing up or down'?), rombergism (ability to maintain balance with eyes shut), vibration sense (detection of vibration of a tuning fork applied to a bony point) and astereog-nosis (inability to tell by touch the nature of a familiar object such as a coin placed in the hand). Cutaneous nerve segments (Fig. 2.12) can be used to judge levels of cord damage in respect of sensory loss. 21 Fig. 2.12 Sensory nerve segments (reproduced from Folia Medica, Geigy Scientific Table by permission of Geigy J.R. Basle, S.A., Switzerland). Reflexes In early infancy there are a number of primitive reflexes peculiar to this period of life (Table 2.1) whose absence or persistence beyond the time of normal disappearance often has pathological significance (Fig. 2.17). After infancy the main superficial and deep reflexes (Table 2.1) used in neurological examination, and their segmental innervations, are: Biceps jerk C5 Supinator jerk C6 Triceps jerk C6, C7 Abdominal reflex Th 8-12 Cremaster reflex L1 Knee jerk L3, L4 Ankle jerk L5, S1 Plantar response S1 Other neurological signs 22 In Kemig's sign extension of the flexed knee with the child supine and hips at a right angle is resisted or impossible. In Brudzinski's sign flexion of the head produces flexion of the knees and thighs. In impairment of straight leg raising, the leg, with the knee fully extended, cannot be raised to a right angle with the patient supine. 'Balancing' movements reflect labyrinthine function (Fig 2.23). HIGHER NERVOUS ACTIVITIES Past history, place in school, relationship with peers and routine clinical examination may reveal much about higher nervous function; accurate assessment will involve psychometric testing and formal speech and language analysis. LOCOMOTOR SYSTEM Examination of the nervous system and of the locomotor system may be closely interrelated. Fractures Fractures may be visible or suspected because of deformity, crep-itus (which should not be actively elicited), pain on movement and local tenderness — which could be due to other conditions such as bruising or osteomyelitis. Deformities of the trunk and neck These may be spinal such as scoliosis (best recognized when the child bends forward to touch his toes (Fig. 23.23) or suspected if skin creases in the flanks are asymmetrical and fail to disappear when the spine is passively flexed to the opposite side), hypnosis (anterior bowing) or lordosis (posterior bowing). Ribs may be absent. With torticollis (Fig. 2.4n) the head is tilted towards the affected side, the chin points in the opposite direction and rotation of the head away from the affected side may be limited. Deformities of limbs Normally children are mildly bow-legged before the age of 2years and knock kneeed between of 2 and 12 vears: thereafter the legs straighten spontaneously. Deformities may consist of absence of bones (e.g. radius with severe flexion and lateral twisting of the hand and wrist, or phocomelia), shortening or deformity of bones, increased carrying angle at the elbow (Turner's syndrome — Fig. 2.4r), incurving of the little finger (Down's syndrome), the 'dinner fork' deformity of the wrists with the hands outstretched (chorea) and flexion deformities (e.g. arthrogryposis multiplex congenita). Lower limbs deformities include genu valgum and genu varum, talipes equinovarus (club foot), talipes calaneo valgus, metatarsus varus, absence of part of a limb, shortening or unequal development (e.g. hemiatrophy or hemihypertrophy). Excessive joint mobility is seen in hypotonia, Ehlers-Danlos syndrome and dia-strophic dwarfism. Measurement of limb length is discussed on p. 22. Finger shortening (brachydactyly; e.g. Down's and the Ellis van Creveld syndrome) or lengthening (arachnodactyly; e.g. Marfan's syndrome) may be expressed in terms of finger length relative to palm length — the middle finger length (tip to proximal crease) is 43% of the total hand length (tip of the middle finger to the distal palmar crease). Muscles May be wasted, hypertrophied (e.g. pseudohypertrophic muscular dystrophy) or absent (e.g. Poland's syndrome). Fasciculation or fibrillary movements may be noted in the tongue, thenar eminence or elsewhere. Muscle tone . 23 Fig. 2.13 Normal labyrinthine reflexes at the age of 7 months. The infant makes reflex 'balancing' movements with the arms as he is tilted from side to side. Table 2.1 Neonatal reflexes How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited Primitive reflexes Cranial nerve reflexes (relevant cranial nerves) Sucking (IX, X, On feeding Birth When General XII) voluntary neurological control of depression, feeding hypotonia, achieved at immaturity or 6—9 months bulbar palsy Swallowing (IX, On feeding Birth When General X, XII) voluntary neurological control of depression, feeding hypotonia, achieved at 6-9 immaturity or months bulbar palsy 24 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited Rooting (V) On light contact Birth General with infant's neurological cheek the infant depression, turns towards hypotonia, the point of immaturity or contact bulbar palsy Glabella (V, VII) A sharp tap on Birth Variable Apathy or facial the glabella persistence palsy if absent; produces accentuated in momentary hyperexcitability tight closure of the eyes Head turn to light With infant Several General (II) supine light weeks neurological from a diffuse depression, source is hypotonia, ? allowed to fall impaired vision on one side and then on the other side of the infant's face and he turns his head to the light (the infant must be in a quiet relaxed state) Pupillary (II, also Shade one eye Birth General III and V) with the hand neurological for a moment or depression, ? two and then impaired vision withdraw it Optic blink (II) Shine a bright Birth General light suddenly neurological at the eyes depression, ? impaired vision Doll's eye (III, Clap the hands Birth 2 weeks Ophthalmic IV, VI) about 30 cm muscle palsies from the (ophthalmoplegia) infant's head. Avoid producing an air stream across the face. Rapid habituation — 25 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited no response to test — normally achieved Acoustic blink (a) Hold baby After a few ? impaired (VIII) upright with days hearing examiner's hands under infant's arms. Spin round so that baby turns with examiner, first one direction then other (the head should turn towards direction in which baby is being turned) Labyrinthine (b) Baby Birth Disturbed (rotation) (VIII, similarly held vestibular also III, IV, VII) but head held function or firmly by ophthalmoplegia examiner's forefinger and middle finger which, on each side, are extended upwards against the side of the baby's head. Similar rotation (the baby's eyes should turn towards the turning direction) Gag (IX, X, XII) Touch posterior Birth General pharynx, e.g. neurological with spatula depression or bulbar palsy Cough reflexes Clap the hands Weak for General (IX, X, XIII) about 30 cm several neurological from the weeks after depression 26 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited infant's head. birth Avoid producing an air stream across the face. Rapid habituation — no response to test — normally achieved Cutaneous reflexes Palmar/foot Place the Birth 2-3 months General grasp examiner's (palmar), 7-8 neurological forefinger in the months (foot) depression, palm/sole of the hypotonia: infant's hemisyndromes, hand/foot which Erb's palsy or then closes clavicular round the fracture. May examiner's persist beyond finger normal time in maintaining a spastic cerebral grip palsy Abdominal Stroke a pin or 7 days Absence does not thumbnail from necessarily imply the side to the abnormality centre of the abdomen (a response is only possible if muscles are fully relaxed) Anal Contraction of Birth Damage to sacral the external cord (e.g. spina anal sphincter bifida) when the skin round it is stroked with a pin 27 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited Cremaster Stroking the 10 days Absent in spinal medial side of cord lesion the thigh with a pin or thumbnail results in pulling up of the testes Withdrawal On pricking the Birth Hemisyndromes; sole of the foot absent or weak in with a pin spina bifida and there is rapid after breech flexion of the delivery with hip, knee and extended legs foot Plantar Stroking the Birth Defects of lower (Babinski) foot along the spinal cord. lateral side with Hemisyndromes pin or thumbnail produces dorsal flexion of the big toe and spreading of the other toes in infancy (and plantar flexion of the other toes in older children who are walking). Do not mistake a grasp reflex of the foot for a flexor plantar response Extensor reflexes Asymmetrical With baby in 1 month 3-5 months Medullary or tonic neck supine position spinal cord rotate the head damage. Readily to one side. elicited in This produces immature infant. increased tone May persist in, and partial beyond normal 28 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited extension of, time in cerebral the arm and leg palsy on the side to which the head is rotated, and there may be flexion of the arm and leg on the contralateral side Crossed extensor Passively A few days 1 month Absent in lesions reflex extend one of spinal cord and lower limb weak in pressing the peripheral nerve knee down, and damage with a pin stimulate after birth the sole of the foot of this fixated leg. Flexion and slight adduction of the unstimulated lower limb normally occurs Trunk Stroke a pin or 5-6 days 7-8 days Hemisyndromes; incurvation thumbnail along spinal cord (Galant) the damage — with paravertebral indication of line about 3 cm segmental level from the midline from the shoulder to the buttock (the back should curve with the concavity to the stimulated side) Perez Elicited as for 5-6 days As for trunk Galant but by incurvation reflex stroking over central vertebral spines (the infant 29 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited arches backwards, the buttocks rise and the anus dilates) Moro Hold baby in Birth 2-3 months General supine position neurological with depression, shoulders and hypotonia. back supported Prolongation of on left tonic phase oi hand and arm of response in the examiner immaturity. and head Hemisyndromes, (occiput) on the fractured clavicle right hand. or humerus. May Allow the head persist beyond to fall back normal time in suddenly cerebral palsy (catching it again with the right hand after it has fallen a short distance) while the rest of the body remains supported. The arms rapidly abduct and come together again with an embracing movement and the legs flex Hand opening Stroke the Birth 2-3 months General dorsum of the neurological hand depression. Hemisyndrome Progression Hold infant in 4 days 2 months General Walking/stepping standing neurological position and depression, place foot on a hypotonia: paresis flat surface. The of lower limbs leg extends to take the infant's 30 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited weight (supporting reaction), the opposite leg flexes then extends, and as it takes weight the original Placing With the baby 4 days 5-9 months General held upright neurological between the depression hands of the examiner the dorsal part of the foot is brought lightly in contact with the edge of the table. Normally the baby flexes knee and hip and places foot on the table Crawling Infant prone. 4 days 4 months General Crawling neurological movements depression may occur spontaneously but can be reinforced by the examiner pressing his thumb gently into the sole of the infant's foot (the crawling reflex is more easily elicited in the immature infant than the walking reflex) Tendon reflexes Jaw jerk Sharp tap on 2 days Absent in brain examiner's stem lesions or 31 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited index finger 5th cranial nerve placed on damage; patient's chin below the lip Biceps A tap on the 2 days exaggerated in examiner's hyperexcitability, finger placed on e.g. the biceps hypocalcaemia muscle causes contraction of the muscle Knee jerk A tap on the 2 days Absent in general patellar tendon neurological with the knee in depression and the flexed hypotonia; position exaggerated in produces hyperexcitability quadriceps contraction Ankle With infant Birth Absent in general prone, knee neurological slightly flexed depression and and the fore hypotonia; pan of the foot exaggerated in held lightly in hyperexcitability the examiner's hand a tap over the tendo- Achillis produces plantar flexion of the foot (response better felt than seen) Ankle clonus Rapid Birth Absent in general dorsiflexion of neurological the foot with depression and the examiner's hypotonia; hand on the exaggerated in distal part of the hyperexcitability sole produces a succession of rapid contractions of the calf muscle 32 How elicited Usual time of Possible Time of disappearance significance if appearance of those abnormal reflexes which are time limited (only briefly sustained in normal infant) Joints Examination will include assessment of joint temperature relative to other body areas, range of movement, swelling, local tenderness or pain on active or passive movement. Dislocation may cause gross deformity. In the knee, synovial effusion is likely to give rise to patellar tap — with the knee joint extended and the thigh gripped just above the patella, the patella, on being sharply depressed, is felt to tap against the underlying bone. Dislocation may cause gross deformity. Congenital dislocation of the hips (CDH) should be diagnosed as soon as possible after birth, not after the child begins to walk when the classical late signs (shortening, external rotation of the limb, asymmetry of thigh folds and limited abduction) have developed. Ortolani's and Barlow's tests are used in diagnosis: the thighs are abducted while the middle finger of each hand presses the greater trochanter forward (Fig. 2.14); if the hip is dislocated the femoral head during abduction can be felt to slip forward with a 'clunk' (similar to the sensation of changing gear in a car and to be differentiated from the common non-significant minor muscular clicks). An extension of the test consists in applying pressure backwards and outwards with the thumb on the inner side of the thigh as abduction is commenced. If the femoral head slips out over the posterior Up of the acetabulum and returns when pressure is released and abduction continues the hip is un- stable. These tests should not be repeated more than necessary. Dislocation may be confirmed by radiology or ultrasound. In anteroposterior radiographs, with the hips and knees extended, thighs medially rotated and abducted 45° from the midline, a line along the centre of the femoral shaft lies outside the upper lip of the acetabulum (Fig. 2.15). Examination for congenital dislocation of the hip. 33 Fig. 2.15 X-ray — congenital dislocation of the hip. Hips abducted 45° from the midline. Line drawn through the centre of the shaft of the femur lies outside the upper edge of the acetabulum. EXAMINATION OF THE NEWBORN Gross physical abnormalities are obvious at birth but other manifestations of disease tend to be less specific. Examination has a threefold purpose — recognizing (1) physical defects, (2) disease and (3) establishing a baseline to which later clinical assessments can relate. MEDICAL HISTORY OF PARENTS, SIBLINGS AND OTHER RELATIVES The mother's previous pregnancies may indicate factors such as prolonged involuntary infertility, habitual abortion or frequent fetal loss implying higher risk of congenital abnormality. Parents, siblings or other relatives may suffer, or have suffered, from dis- orders which carry hereditary risks. Certain maternal diseases may have profound influence on the fetus. The social history may imply disadvantage. BIRTH HISTORY Note birth rank and maturity, evidence of fetal distress, early rupture of the membranes, induction of labour, prolonged or rapid labour, type of delivery, obstetric estimate of gestation, birthweight, Apgar score, apnoea, need for resuscitation, special or intensive care. GESTATION AGE Important in making judgements concerning diagnosis and management this may be assessed by (1) mother's 'dates' (p. 87), (2) obstetric examination (p. 87), (3) ultrasound measurements of fetal growth (p. 89), (4) clinical examination of the infant. Gestation age may be assessed using external characteristics, muscle tone and primitive reflexes (Table 2.2). Dubowitz & Dubowitz (1977) have derived a score based on (a) neurological criteria and (b) external criteria convertible into gestation age (Fig. 2.16). A more rapid method relies on skin colour and texture, breast development and ear firmness (Parkin et al 1976). THE TRANSITION PERIOD 34 Respiration, which may be irregular in rate and depth in the immediate neonatal period, should be established within half a minute of delivery. Diffuse motor activity at birth peaks at half to one hour then diminishes and the infant often passes into sleep. ROUTINE EXAMINATION Usually carried out on the first day of life and again on discharge when signs not evident on the first day may be evident (e.g. the murmur of ventricular septal defect). Measurements Weight, length (crown-heel or crown-rump), skull circumference, respiration rate and temperature are standard. Inspection Gross deformities such as excessive cranial moulding, caput suc-cedaneum, cephalhaematoma, abnormal facies (e.g. Down's syndrome or renal agenesis (Fig. 2.4a)), spina bifida, absent or deformed limbs, tumour masses (e.g. sacrococcygeal teratoma), herniae (e.g. umbilical) may be evident. Posture and movement (see below). Healthy skin will be pink and elastic; unhealthy may be cracked and parchment-like (placental insufficiency), abnormally pallid (fetal exsanguination), cyanosed (asphyxia or severe congenital heart disease), shiny (oedema), jaundiced, desquamating (e.g. Ritter's disease), loose and inelastic (prematurity), dry and turgorless (dehydration), blemished (angiomata, naevi or milia) or pustular (infection). Neonatal urticaria (erythema toxicum), pet-echiae or ecchymosis (consumption coagulopathy), Mongolian blue spots (patchy accumulations of pigment over the lumbar region and buttocks) and rarer disorders such as Harlequin colour change (the body pale on one side and deep red on the other with a clear line of demarcation between) or the 'collodion baby' may occur. Dermal sinuses may be present in the region of the coccyx. Cyanosis or pallor may be evident. The umbilical cord and stump may show an omphalocele, bleeding, infection or other discharges. A single umbilical artery (1% of singletons, higher in twins) is sometimes associated with severe congenital abnormalities. Note the shape of the head and charac- ter and size of the anterior fontanelle. Prematurely born infants tend to develop side-to- side flattening due to their readily mould-able skulls. The eyes are usually closed but pupillary shape and size can be examined. Nystagmus (if transitory unimportant, but if persistent probably pathological), microphthalmia, eyeball enlargement (buphthalmos — Fig. 2.4u), corneal clouding, cataract (Fig. 2.4i), defects of the iris (coloboma or aniridia) may be present. Eye movements may be absent (ophthalmoplegia). The mouth may reveal deformities such as cleft palate. The mucous membranes may be moist, dry, ulcerated or infected. The jaw may be small and retracted (micrognathia — Fig. 2.4p). Epstein's pearls (nodules just lateral to the midline on the hard palate) are normal. Facial asymmetry may be noted (in the neo-nate the corner of the mouth is actively depressed on crying so that in facial palsy the depressed side is the normal). Inspect the chest for asymmetry, the pattern and rate of respiration, evidence of respiratory distress (tachypnoea, grunting, intercostal and costal margin recession), respiratory depression (slowness and shallowness of respiration), praecordial bulging or abnormal praecordial pulsation, breast enlargement or secretion of milk. Note any abnormal shape of the abdomen (e.g. scaphoid, distended, showing peristaltic waves) and any impairment of normal abdominal respiration. Watch feeding for vigour and co-ordination of sucking, swallowing and possible vomiting. 35 Note any imperforation, malposition or abnormal structure (e.g. lax and patulous) of the anus or structural abnormality of the urethra (e.g. hypospadias) or genitalia (e.g. scrotal swelling or testicular abnormality). In the newborn female, particularly the premature, the clitoris and labia minora are usually prominent. The cry may be abnormal (e.g. stridulous or high pitched as in cerebral irritation). Hiccup is common in normal newborn infants. Palpation Handling will reveal muscle tone. Note the size and tension of the anterior fontanelle, degree of closure of the posterior fontanelle, state of the sutures (widening or overriding). Oedema pits on pressure; sclerema neonatorum presents as lard-like hardening of subcutaneous tissue, subcutaneous fat necrosis as areas of rubbery consistency usually on the neck posteriorly and outer aspect of the thighs, surgical emphysema as a crepitant sensation over the root of neck and thorax. Palpation and handling will reveal abnormal praecordial pulsation, position of the apex beat, enlarged viscera and other masses, mobility and range of movements of joints (cf. Ortolani manoeuvre), position of the testes, possibly fractures. Percussion May reveal lung hyperresonance (e.g. lobar emphysema), lung consolidation, cardiac displacement and possibly size, fluid in the abdomen. Auscultation Reveals breath sounds and associated adventitious sounds, cardiac murmurs (often transitory in the first few days of life), bowel sounds (bowel sounds in the chest are unique in diaphragmatic hernia), cranial bruits. Excreta The nature and manner of passage of meconium; the frequency, consistency, colour, odour and possible blood staining of the stools; the visual, chemical and bacteriological examination of the urine may be important. Instrumental procedures Laryngoscopy (frequently used for intubation); ophthalmoscopy (detection of early retrolental fibroplasia; retinal bleeding, exudate or defect; cataract and corneal opacity); auriscopic examination; transillumination (e.g. hydranencephaly or hydrocele); lumbar puncture; subdural tapping; suprapubic aspiration of the bladder; blood culture and culture of various other discharges. NEUROLOGICAL ASSESSMENT Neurological assessment of the newborn infant gives an indication of the presence or likely development of disorders such as cerebral palsy, microcephaly, hydrocephaly, the 'clumsy child' and intellectual impairment. Behavioural characteristics: was breathing established spontaneously at birth or intubation required? What is the state of consciousness, response to stimuli, amount of spontaneous movement (or lack of it)? Have there been convulsions (localized or generalized). Are sucking and swallowing impaired (e.g. in bulbar palsy) necessitating 36 tube feeding? Are there normal crying responses to hunger and pain? Is there a normal state of alertness, or apathy? Does sleep occupy the normal 20 out of 24 hours? Posture in the newborn is one of flexion; the arm will remain flexed at the elbow on 'pulling to sit', the popliteal angle remains at 90° on straight leg raising (in contrast dorsiflexion of the foot against the tibia reveals the diminished extensor tone — Table 9.1), sudden passive extension (stretch) and then release of the elbow will result in forearm recoil; in the prone position the lower limbs are flexed under the abdomen, the arms flexed at the elbows and adducted beside the trunk (Fig. 9.1); on ventral suspension the dangling limbs remain flexed (Table 9.1). Disturbance of the normal postural flexion is likely to indicate neurological dysfunction. In generalized hypotonia the infant is'floppy' with a 'flat' posture in recumbency. Regional hypotonia is seen for instance in the bilateral lower limb paresis of spina bifida or in Erb's palsy. In generalized hypertonia (extensor hypertonus) the legs are usually more affected than the arms, the trunk tends to be opisthotonic and the extensor tone in the neck results in seemingly good head control in ventral suspension but poor head control on 'pulling to sit'. In more severe cases, the arms are also involved, the position being one of decerebrate rigidity. In hypertonia static postures may be interrupted by 'cycling' movements of the lower limbs, 'doggy paddling' of the upper and generalized 'jitteriness': tendon reflexes are usually ex- aggerated. Transient or persistent hemisyndromes may occur in which one side of the body may show increased or diminished tone relative to the other (tonic hemisyndrome); reflexes, particularly the extensor reflexes, such as the asymmetrical tonic neck reflex (Fig. 2.17b), may be accentuated unilaterally (reflex hemisyndrome) or one side may be paralysed (paralytic hemisyndrome). Spontaneous movements exhibited by the newborn infant include rotation of the head, movement of the arms, sucking the thumb, movement of the lower limbs (more than the arms). SCREENING Early presymptomatic diagnosis may enable defects which might otherwise become irremediable to be cured or ameliorated and can also provide a basis for genetic counselling. Screening may be universal (U), selective (S) or research. NEONATAL SCREENING Clinical screening (U) Parental and family history may reveal history indicating likely or possible genetically determined disorders and maternal disorders conducive to fetal damage. Relevant screening elements in the birth history include a period of gestation of less than 36 weeks; fetal asphyxia; asphyxia defined as a period of 15 min or more before respiration is established, a 5-min Apgar score of less than 3, a blood pH of 7.2 or less or a pCO; of more than 8 kilopascals (60 mmHg); postmaturity (42 weeks or more); a birth order of 5 or more in offspring of unskilled or semi-skilled workers; a serum bilirubin level higher than 340 /Amol/1 (20 mg/dl); precipitate, prolonged or difficult delivery, especially breech. Physical examination may reveal the defects indicated in 'Examination of the newborn' above. Haematological screening (S) This usually includes ABO and Rh blood group incompatibilities and Coombs testing of Rh-negative mothers and their offspring. In certain populations it might include screening for sickle cell disease and thalassaemia. 37 Metabolic screening The technique most commonly used is the extended Guthrie test (U) (blood obtained by skin prick and allowed to dry on filter paper; p. 1176) which will reveal phenylketonuria (diagnosable from the third day of life provided the infant has received several protein- containing feeds), tyrosinaemia (transient or permanent), histidinaemia (the Guthrie test reveals only about half the cases), maple syrup urine disease, homocystinuria, valinaemia and galactosaemia. Chromatography (U) in addition to the above will reveal hyperglycinaemia, hyperprolinaemia and hydroxyprolinae-mia. Urine examination (U) which will reveal phenylketonuria, tyrosinaemia, homocystinuria and galactosaemia can also be used in screening. Screening for hypothyroidism (U) using assay of TSH or T4 on filter paper blood samples is now widely practised. Selective screening may be carried out where glucoses-phosphate dehydrogenase deficiency is prevalent and under certain circumstances blood sugar screening for hypoglycaemia (S) using Dextrostix, creatine phosphokinase screening (blood dried on filter paper) for muscular dystrophy (S) and IgM screening for congenital infection (S). Screening for hereditary angioneurotic oedema (c-1-esterase deficiency), alpha- 1- antitrypsin deficiency and hyperlipidaemia (cholesterol and lipoprotein levels) has been carried out on an experimental basis. Treatable conditions detected on screening in the neonatal period include cleft lip and palate, congenital heart disease, congenital dislocation of the hips, talipes, undescended testes, somecases of cerebral palsy, some cases of mental retardation, cretinism, Rh incompatibility, phenylketonuria, maple syrup urine disease, tyrosinaemia, galactosaemia and hyperlipidaemia. PRESCHOOL SCREENING Many of the tests indicated above can be applied in the preschool period but may be too late then. However, retarded development may only become evident in the preschool period following developmental screening (as opposed to developmental assessment). Figure 2.19 illustrates a scheme developed for use in Scotland (Barber et al 1976). Treatable conditions discoverable on preschool screening include congenital heart disease, deafness, squint, refractive errors of vision, undescended testes, cataract, rickets, child abuse, epilepsy, certain types of intellectual impairment, certain developmental defects, scabies and other skin disorders. PRESCHOOL DEVELOPMENTAL SCREENING 1. The following charts, providing for routine examinations at 5 standard times (and 3 months, 6 months, 1 year and 2 years if necessary), show development as a staircase and allow for variation within the normal range. 2. Each screening test comprises two items from each of gross motor, vision/fine motor, social/adaptive and hearing/language categories. Equipment 1 and 2 in. cubes, rattle, box of small beads, box of pins, large light-coloured ball, \ in. ball, practice golf ball on string, paper and pencils, cups and spoon, miniature tea set, wooden steps (if no stairs convenient), bell. Reed test (Royal National Institute for the Blind). Method of use 1. The developmental level is represented by a cross on each chart placed on the vertical line arising from the child's age at the time of examination (in the first year of life allow for premature birth, e.g. infant born at 34/52 gestation subtract 6/52 from chronological age). 38 2. When a child is tested at any of the 9 standard ages noted above (a) give both tests for the age — success in both: mark cross on level with upper horizontal line success in one: mark cross on level with lower horizontal line failure in both: proceed to (b) (b) give pair of tests for next — success in both: mark cross on younger standard age level with upper horizontal line success in one: mark cross on level with lower horizontal line failure in both: move to pair of tests for next younger age 3. When a child is not exactly at test age, give test for nearest standard age and mark cross vertically above child's actual age. 4. When a child is exactly between test ages, give tests for next age above child's age in first instance. 5. Review (e.g. in 2 months) if two or more of the marks plotted on any screening test fall between the lower step lines and the dotted lines. 6. Assessment is indicated if any one of the four charts has a mark below the dotted line or there is marked discrepancy between the charts. SCHOOL-AGE SCREENING Most of the defects discovered by neonatal and preschool screening, if not so discovered, will have revealed themselves by school age. Additional screening or ascertainment procedures relevant to this time are audiometric and visual testing, ascertainment of speech, language and reading (dyslexia) disorders, intelligence testing, screening for cleanliness and infestation. Urine screening with dipslides for unsuspected urinary tract infection in schoolgirls has some advocates. (Note: age scale is not linear. If child is not standard age plot age as accurately as possible using scale on chart.) 39