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Being disrespectful or rude about parental choicetonotvaccinatetheirchildrisksthelossofanytherapeuticrelationship withthefamily. Vitalsigns It is necessary to interpret the vital signs according to the age of a particular child. Theseidentifyandflag abnormal age-appropriate vital signs, in order to escalate the level of care for childrenwithpotentiallycriticalillness. ReflectiononthePracticeofPaediatric Emergency the practice of paediatric emergency can be immensely rewarding. The opportunitytoplaygamesinthecontextofassessingpatientsisawonderfulway to spend a working day. However, great care and attention are required to identify those children with serious or life-threatening illness from those who haveaviralillness. Anunhurried,calmapproachwithserialreviewandclose, careful follow-up can minimise the likelihood of missing a serious diagnosis. By reading this textbook you are on a journey to be well positioned to recogniseandmanagethesickchildandhopefullychangetheoutcomeforthe better. This group of patients can be very complex, with numerous medical and psychosocial issues and a baseline abnormal examination. The parents are the best advocate for their child, and they know their child better than anybody else. Chronic illness brings with it a range of stressors for the child and family, includingthosesurroundingpainfulproceduressuchasvenousaccess. Aswith any child, but especially in this population who are likely to be subjected to multipleproceduresovertime,itiscriticaltominimisethetraumasurrounding potentially painful procedures. Tools used may include distraction, parental presence, play therapy, positioning on a parent, topical anaesthesia, pharmaceutical analgesia, or procedural sedation. Inadequate pain management is known to have long-term negative effects on children, includingdiminishingeffectsofadequateanalgesiaforsubsequentproceduresor needle phobia, whichmakefutureproceduresmoretraumaticfor the childand moredifficultfortheclinician. Children may have associated impairments including epilepsy or intellectual, speech,visualorhearingimpairment. Causative organisms of pneumonia are similar to other children, though anaerobesmayalsobeinvolvedinthesettingofpossibleaspiration. Note that chest X-rays will be difficult to interpret in those with severe scoliosis. Contributing factors includereducedfibreandfluidintake,reducedmobilityanddifficultyachieving optimal toileting posture, and prescription of anticholinergic or opiate drugs which increase transit time. Management of spasticity includes physiotherapy and splinting, as well as medical management, with surgeryreservedforseverecases. Itisimportantthatemergencyphysiciansare awarethathipdislocationsandpathologicalfracturesaremorecommoninthis population and must be considered in the patient who presents with irritability withnoothercausefound. Commonmedicationsusedincerebralpalsy the medications used to manage spasticity may be unfamiliar to emergency practitioners. Baclofen has poororalbioavailability,sointrathecalbaclofenisincreasinglyusedfor severe generalised spasticity or dystonia. Itoccursduetoadefectinthe closureoftheneuraltubeduringthefirstmonthofpregnancy,leavingthespinal cord exposed and able to protrude through the open part of the spine. Patients with spina bifida have varying degrees of disability, including paralysis or weakness in the legs, bowel and bladder incontinence, hydrocephalus and specific learning difficulties. Symptoms vary depending on the position of the neural tube opening along the spine and on how much of the spinal cord or meningesprotrudethroughtheopening. The end result is irreversible airway damagewithbronchiectasisandrespiratoryfailureinmostpatients. Patients with the disease will have raised concentrations of sodium and chloride to >60 mmol L. The initial screen is for raised concentrations of immunoreactivetrypsinogen,withfurthertestingasindicated. Early diagnosis and aggressive nutritional support improve growth and allow genetic counselling for the family; however, it may notimprovepulmonaryoutcomes. Socialandpsychologicalsupportforpatientandfamily: Complicationsmanagedintheemergency department Respiratory Prompt and aggressive treatment of infective exacerbations is crucial to maintaininglungfunction,improvingqualityoflife,andprolongingsurvival.

Until the airway is both secured and protected, this is best done by in-line immobilization, as use of a stiff cervical collar makes intubation difficult. Conventionally, in-line immobilization is performed with the practitioner standing at the head of the casualty, holding the head on both sides with the hands and maintaining it in a neutral position, in line with the neck and torso. This can make airway management difficult, with the inline immobilizer squatting awkwardly to one side. This effectively immobilizes the cervical spine, but makes examination of the posterior neck difficult, and is uncomfortable for a tall practitioner. Once the airway is secured and protected, the trinity of stiff collar, head blocks and tape should be implemented. As the level of consciousness decreases, so does muscle tone, and the pharynx collapses around the glottis, obstructing the airway. In the supine position, the tongue drops backwards, plugging the glottis anteriorly. Airway obstruction can be sudden or insidious, and partial or complete, but will result in damaging hypoxia and hypercarbia, which are particularly dangerous in a head-injured casualty. Maxillofacial trauma Disruption of the facial bones allows the face to fall back, compressing and obstructing the pharynx. This is associated with soft tissue swelling and bleeding, which further obtund the airway. Typically, these patients need to sit up to allow the face to fall away from the pharynx and open up the airway. Signs can be subtle; contusion over the larynx with a hoarse voice, coughing of bright red blood and surgical emphysema should alert the practitioner to the likelihood of sudden airway obstruction. Inhalational burns Inhaling super-heated air burns the airway and can result in rapid development of swelling and airway obstruction. Signs such as facial burns, smoke staining and singed nasal hair suggest an inhalational burn, requiring early and expert intubation. Use of accessory muscles of ventilation; casualty classically sitting forward splinting chest, and using neck and shoulder muscles to aid breathing. Listen in haemorrhage and swelling, which compresses, distorts and obstructs the upper airway. This can progress rapidly and make tracheal intubation impossible and surgical airway difficult. This pulls the jaw and pharyngeal structures forward off the posterior pharyngeal wall and glottis, and opens up the airway. Jaw thrust this is a more assertive manoeuvre that is Feel for passage of air through mouth and nose with palm of hand; very sensitive for detecting air flow. Palpation of the trachea in supra-sternal notch will detect the deviation associated with a tension pneumothorax. All these techniques can be performed without extending the head and compromising an unstable cervical spine. Bare hands techniques and the use of pharyngeal airways are used together to pull the pharyngeal tissues and tongue off the posterior pharyngeal wall and away from the glottis, opening up the airway. All the non-surgical airway manoeuvres described are applicable to children, but require some modification in technique to accommodate their anatomical and physiological differences. Surgical cricothyroidotomy is not recommended in children under 12 years of age, as the cricoid cartilage can be damaged, leading to tracheal collapse. Using the thenar eminences to provide a counterpoint on the maxillae, the mandible is lifted up and forwards to open up the airway as with chin lift.

The small cuboidal bones also grow by interstitial cartilage proliferation and appositional (periosteal) bone formation. After the end of bone growth (which varies for different bones) no further increase in size occurs, but bone and joint remodelling continues throughout life. In diarthrodial joints (freely movable, synovial joints) this is hyaline cartilage, which is ideally suited to permit low-friction movement and to accommodate both compressive and tensile forces. In synarthroses, where greater resistance to shearing forces is needed, the interface usually consists of tough fibrocartilage (e. For all its solidity, it is in a continuous state of flux, its internal shape and structure changing from moment to moment in concert with the normal variations in mechanical function and mineral exchange. All modulations in bone structure and composition are brought about by cellular activity, which is regulated by hormones and local factors; these agents, in turn, are controlled by alterations in mineral ion concentrations. Disruption of this complex interactive system results in systemic changes in mineral metabolism and generalized skeletal abnormalities. The matrix Type I collagen fibres, derived from tropocollagen molecules produced by osteoblasts, make up over 80 per cent of the unmineralized matrix. Their functions have not been fully elucidated but they appear to be involved in the regulation of bone cells and matrix mineralization. Osteocalcin is produced only by osteoblasts and its concentration in the blood is, to some extent, a measure of osteoblastic activity. A number of growth factors have now been identified; they are produced by the osteoblasts and some of them, acting in combination, have a regulatory effect on bone cell development, differentiation and metabolism. It was originally found by Marshall Urist in 1964 (Urist, 1965) and is now produced in purified form from bone matrix. It has been shown to have the important property of inducing the differentiation of progenitor cells into cartilage and thereafter into bone. It is now produced commercially and is being used to enhance osteogenesis in bone fusion operations (Rihn et al. The interface between bone and osteoid can be labelled by administering tetracycline, which is taken up avidly in newly mineralized bone and shows as a fluorescent band on ultraviolet light microscopy. In mature bone the proportions of calcium and phosphate are constant and the molecule is firmly bound to collagen. While the collagenous component lends tensile strength to bone, the crystalline mineral enhances its ability to resist compression. Unmineralized matrix is known as osteoid; in normal life it is seen only as a thin layer on surfaces where active new bone formation is taking place, but the proportion of osteoid to mineralized bone increases significantly in rickets and osteomalacia. Lying in their bony lacunae, they communicate with each other and with the surface lining cells by slender cytoplasmic processes. It has also been suggested that they are sensitive to mechanical stimuli and communicate information and changes in stress and strain to the active osteoblasts (Skerry et al. Ultimately the ageing osteocytes are phagocytosed during osteoclastic bone resorption and remodelling. Osteoclasts these large multinucleated cells are the principal mediators of bone resorption. Osteoblasts Osteoblasts are concerned with bone formation and osteoclast activation. They are derived from mesenchymal precursors in the bone marrow and the deep layer of the periosteum.

Suppurative complications of streptococcal infection include peritonsillar abscess, sinusitis and otitis media. The principal concerns, however, are with the non-suppurative complications: rheumatic fever and glomerulonephritis. Acute tonsillitis can cause airway obstruction, particularly with pre-existing tonsillar hypertrophy, and may even warrantadmissionformonitoring. Historyandexamination Presentation is usually with unilateral sore throat or neck pain, which is often severe. Amuffled(orhot potato) voice, trismus and ipsilateral ear pain help to differentiate peritonsillar abscess from severe pharyngitis/tonsillitis. Examination findings include cervical lymphadenopathy, unilateral tonsillar erythema,bulgingofthesuperioraspectofthetonsilanduvulardeviationtothe opposite side. Investigations the majority of cases are diagnosed clinically with definitive confirmation on needleaspirationofpus. Microbiological identification of abscess contents probably does not alter management. Treatment Initial treatment is rehydration, analgesia and antibiotics (penicillin). Acute drainage is generally recommended, with intra-oral drainage, abscess tonsillectomy or needle aspiration. Needle aspiration alone would appear to be effective in a majority of cases (>90%). This should be performed by an appropriately trained specialist owing to the risk of complications, including punctureofthecarotidartery. Uncommon but dangerous complications include infection extension to the parapharyngeal space,airwayobstructionandaspirationofpuscausingpneumonia. Post-tonsillectomyhaemorrhage Introduction Tonsillectomy remains a very commonly performed procedure. Secondary (delayed) haemorrhage occurs after the first 24 hours following surgery and complicatesabout0. Risk factors include bleeding tendencies and patients who have a history of chronictonsillitis,precedingsurgery. History Bleeding is usually obvious, although occasionally will be swallowed and not immediately apparent. A history of bleeding or bruising tendency should be soughtalthoughideallywillhavebeenidentifiedpriortosurgery. Examination Initial assessment should focus on signs of shock or haemodynamic compromise. Subsequent examination of the tonsillar fauces for evidence of activebleedingisthenimportant. Investigations Fullbloodcountexaminationtomonitorforadropinhaemoglobinandtaking blood for cross match is recommended in any significant post-tonsillectomy bleed. Coagulation profile testing rarely demonstrates abnormality but is warrantedinmajorhaemorrhage. Severebleedingwillrequireremovaloftheclottoallow application of adrenaline (epinephrine)-soaked gauze (1:10,000) directly onto thebleedingpoint. ThegauzeisheldinplacewithMagillsforceps,andpressure is applied laterally to the wall of the mouth. Complications Post-tonsillectomy bleeding is potentially life threatening, largely from hypovolaemicshock. Occasionally, higher impact trauma including motor vehicle accidents will produce severe injury requiring maxillofacial reconstructive surgery and early problems of haemostasis and airway compromise. History Makespecificenquirywithregardtothetimeandmechanismoftraumaandthe nature of the dentition prior to injury. This includes the number of deciduous teeththatwerepresentandthepresenceofanysecondarydentition. Determine whether any teeth have been avulsed and their current location and method of storage. Examination the physical examination should review all orofacial structures with careful scrutiny, digital palpation and observation of normal function. Externally and internally the orofacial region should exhibit consistent symmetry, and any departure from this, be it an area of altered facial soft tissue architecture associatedwithaswellingoralteredbonyarchitectureassociatedwithafracture, willbeimportant.

Some veer towards the purely descriptive; others go into almost obsessive detail based on topographical and morphological features. Their usefulness lies in the elaboration of an agreed terminology which will aid communication and permit sensible auditing of the results of various forms of treatment. Ulnar deficiency Hypoplasia of the distal end of the ulna is usually seen as part of a generalized dysplasia, but occasionally it occurs alone. The radius is bowed (as if growth is tethered on the ulnar side) and the radial head may dislocate; the wrist is deviated medially. The forearm deformity is not as marked as in radial deficiency but overall function is severely restricted. Over time, the mobile pseudarthrosis may become painful, particularly with overhead activities and on direct pressure, but shoulder dysfunction itself is unusual. Radio-ulnar Synostosis this is often associated with a posterolateral dislocation of the radial head. Clinically there is complete loss of pronation and supination, although some children appear to maintain some forearm rotation due to laxity of the wrist and elbow. Forearm rotation cannot be regained with surgery but improvement in the resting position of the forearm (and hence of the hand) can be achieved. Transverse deficiency of the arm Transverse deficiency of the distal part of the arm will leave a simple stump below a normal elbow. Cleft hand A central defect of the hand is more common than an ulnar post-axial deficiency. If associated with cleft foot, the ectrodactyly may be an autosomal dominant condition but with variable penetrance affecting boys more frequently than girls. Complex reconstructions can be considered but the balance between appearance and function must be remembered. This can be dealt with by limb lengthening procedures or, if shortening is very marked, by adding a distal orthosis. Since the hip permits normal weightbearing, this condition also can be managed by limb lengthening operations. The most widely used classification is that of Aitkin, as illustrated in Figure 8. Coxa vara with moderate shortening of the shaft can be dealt with by corrective osteotomy and limb lengthening. Severe degrees of coxa vara, sometimes associated with pseudoarthrosis of the femoral neck, may result in marked shortening of the femur. In the worst cases most of the femoral shaft is miss- Pseudarthrosis of the clavicle this almost always affects the right side (except in cases of dextrocardia! Whilst occasional familial autosomal dominant cases have been described, the true aetiology is unknown; other theories such as 8. Type C: the femoral head and neck are absent and the acetebulum is under-developed. Congenital coxa vara is not included in this classification although it may also be a variant of the same disorder (see Chapter 19). If the deformity is bilateral and symmetrical, walking is possible and some individuals acquire remarkable agility; however, they may still seek treatment to overcome the severe cosmetic problem. However, the trick is easier, and looks better, in drawings than in real life and the procedure is seldom done nowadays. Though unhappy with his appearance, because the lower limb defects were symmetrical he was able to get about remarkably well. Tibial deficiency Tibial dysplasia is very rare: several forms exist and the condition may be associated with other limb anomalies. Prognosis, and hence treatment, depend on the quality of the knee joint: if there is no ability for knee extension, a proximal amputation must be considered. If the ankle cannot be reconstructed a distal amputation may be required and a fibula transfer may extend the useful portion of the tibia. In other cases reconstruction using limb lengthening techniques may be applicable.